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MT-RNR1&2
A2755G
A2755G-F1
C2639A
C3093G
C3093G-F1
G2492A
G2647A
G3010A
G3054A
G3090A
T2336C
T2336C-F1
T2648C
T2806A
T3098C
A735G
A735G-F2
A735G-F1
A745G
A747G
A801G
A856G
A1027G
A1331G
A1374G
A1453G
A1517C
A1555G
A1555G-F9
A1555G-F10
A1555G-F11
A1555G-F353
A1555G-F323
A1555G-F302
A1555G-F228
A1555G-F227
A1555G-F186
A1555G-F185
A1555G-F226
A1555G-F183
A1555G-F112
A1555G-F182
A1555G-F225
A1555G-F111
A1555G-F66
A1555G-F181
A1555G-F110
A1555G-F65
A1555G-F136
A1555G-F222
A1555G-F109
A1555G-F63
A1555G-F120
A1555G-F133
A1555G-F102
A1555G-F108
A1555G-F61
A1555G-F119
A1555G-F125
A1555G-F70
A1555G-F2
A1555G-F221
A1555G-F58
A1555G-F100
A1555G-F107
A1555G-F1
A1555G-F24
A1555G-F118
A1555G-F124
A1555G-F56
A1555G-F69
A1555G-F23
A1555G-F81
A1555G-F55
A1555G-F99
A1555G-F106
A1555G-F22
A1555G-F7
A1555G-F116
A1555G-F123
A1555G-F53
A1555G-F21
A1555G-F68
A1555G-F80
A1555G-F4
A1555G-F220
A1555G-F83
A1555G-F95
A1555G-F32
A1555G-F98
A1555G-F104
A1555G-F20
A1555G-F31
A1555G-F12
A1555G-F114
A1555G-F27
A1555G-F122
A1555G-F29
A1555G-F26
A1555G-F67
A1555G-F28
A1555G-F71
A1555G-F82
A1555G-F92
A1555G-F93
A1555G-F96
A1555G-F97
A1555G-F103
A1555G-F113
A1555G-F121
C792T
C869T
C1192A
C1192T
C1226G
C1310T
C1494T
C1494T-F11
C1494T-F10
C1494T-F9
C1494T-F8
C1494T-F7
C1494T-F6
C1494T-F5
C1494T-F4
C1494T-F3
C1494T-F2
C1494T-F1
C1537T
C1556T
G988A
T669C
T669C-F2
T721C
T850C
T921C
T961G
T961G-F2
T990C
T1180G
T1391C
T1413C
T1452C
A839G
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A1555G
# **General Information** | **Position** | **1555** | **Variant** | **m.1555A\>G** | **Locus** | **MT\-RNR1** | **RNA** | **12S rRNA** | | --- | --- | --- | --- | --- | --- | --- | --- | | **Homoplasmy** | \+ | **Heteroplasmy** | \+ | **mitoTIP** | N/A | **Pathogenicity** | Cfrm \[P] | "Pathogenicity" status is derived from published literature or predictions from bioinformatics tools. Researchers and clinicians are cautioned that additional data and/or analysis may still be necessary to confirm the pathological significance of these variants. For more information, please see [Mitofam Info](https://mitofam.com/doc/385/). # **Pedigree Information** The **m.1555A\>G** variant in MT\-RNR1 has been reported in 371 pedigrees. To date, 943 carriers have been reported. Homoplasmy was reported in 747/943 carriers (79\.2%), and 693/943 carriers (73\.5%) were affected. In one unaffected carrier, a marked tissue\-specific difference was observed, with higher mutation loads in saliva (10\.6%) than in blood (5\.1%). The main clinical manifestations among affected carriers included PTA R, profound, hearing\-impaired, slope, profound hearing loss, hearing loss, severe hearing loss, moderate hearing loss, and other features. | **No.** | **Position** | **Variant** | **Family ID** | **Nationality** | **Diseases** | **Unaffected Maternal Relatives** | **Affected Maternal Relatives** | **Publication Year** | **PMID/Ref** | **Note** | | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | | 1 | 1555 | m.1555A\>G | [A1555G\-F1](https://mitofam.com/doc/1522/) | Spain | Nonsyndromic sensorineural hearing loss | 5 | 16 | 1997 | [9040738](https://pubmed.ncbi.nlm.nih.gov/9040738/) | Family 1 | | 2 | 1555 | m.1555A\>G | [A1555G\-F2 ](https://mitofam.com/doc/1523/)| Spain | Nonsyndromic sensorineural hearing loss | 1 | 6 | 1997 | [9040738](https://pubmed.ncbi.nlm.nih.gov/9040738/) | Family 2 | | 3 | 1555 | m.1555A\>G | A1555G\-F3 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 14 | 0 | 2005 | [15708009](https://pubmed.ncbi.nlm.nih.gov/15708009/) | Family BJ101 | | 4 | 1555 | m.1555A\>G |[ A1555G\-F4](https://mitofam.com/doc/1524/) | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 11 | 1 | 2005 | [15708009](https://pubmed.ncbi.nlm.nih.gov/15708009/) | Family BJ102 | | 5 | 1555 | m.1555A\>G | A1555G\-F5 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 19 | 0 | 2005 | [15708009](https://pubmed.ncbi.nlm.nih.gov/15708009/) | Family BJ103 | | 6 | 1555 | m.1555A\>G | A1555G\-F6 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 13 | 0 | 2005 | [15708009](https://pubmed.ncbi.nlm.nih.gov/15708009/) | Family BJ104 | | 7 | 1555 | m.1555A\>G |[ A1555G\-F7](https://mitofam.com/doc/1525/) | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 5 | 9 | 2005 | [16168391](https://pubmed.ncbi.nlm.nih.gov/16168391/) | Family BJ105 | | 8 | 1555 | m.1555A\>G | A1555G\-F8 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 12 | 5 | 2005 | [16168391](https://pubmed.ncbi.nlm.nih.gov/16168391/) | Family BJ106 | | 9 | 1555 | m.1555A\>G | [A1555G\-F9 ](https://mitofam.com/doc/1526/)| China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 7 | 9 | 2005 | [16152638](https://pubmed.ncbi.nlm.nih.gov/16152638/) | with G7444A | | 10 | 1555 | m.1555A\>G | [A1555G\-F10](https://mitofam.com/doc/1527/) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 22 | 11 | 2006 | [16955413](https://pubmed.ncbi.nlm.nih.gov/16955413/) | Family BJ107 | | 11 | 1555 | m.1555A\>G | [A1555G\-F11](https://mitofam.com/doc/1528/) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 6 | 9 | 2006 | [16955413](https://pubmed.ncbi.nlm.nih.gov/16955413/) | Family BJ108 | | 12 | 1555 | m.1555A\>G | [A1555G\-F12 ](https://mitofam.com/doc/1529/)| China | Aminoglycoside\-induced and nonsyndromic hearing loss | 3 | 5 | 2006 | [16955413](https://pubmed.ncbi.nlm.nih.gov/16955413/) | Family BJ109 | | 13 | 1555 | m.1555A\>G | A1555G\-F13 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 16 | 0 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD1 | | 14 | 1555 | m.1555A\>G | A1555G\-F14 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 19 | 1 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD2 | | 15 | 1555 | m.1555A\>G | A1555G\-F15 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 15 | 0 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD3 | | 16 | 1555 | m.1555A\>G | A1555G\-F16 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 18 | 5 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD4 | | 17 | 1555 | m.1555A\>G | A1555G\-F17 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 30 | 0 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD5 | | 18 | 1555 | m.1555A\>G | A1555G\-F18 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 6 | 1 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD6 | | 19 | 1555 | m.1555A\>G | A1555G\-F19 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 27 | 2 | 2007 | [17341440](https://pubmed.ncbi.nlm.nih.gov/17341440/) | Family WZD7 | | 20 | 1555 | m.1555A\>G | [A1555G\-F20](https://mitofam.com/doc/1530/) | China | LHON; A1555G co\-occurrence | 10 | 3 | 2007 | [17452034](https://pubmed.ncbi.nlm.nih.gov/17452034/) | Family WZ16; with T14484C | | 21 | 1555 | m.1555A\>G | [A1555G\-F21](https://mitofam.com/doc/1531) | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 9 | 9 | 2007 | [17723226](https://pubmed.ncbi.nlm.nih.gov/17723226/) | Family WZD11; with G11696A | | 22 | 1555 | m.1555A\>G | [A1555G\-F22](https://mitofam.com/doc/1532) | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 8 | 4 | 2008 | [18386806](https://pubmed.ncbi.nlm.nih.gov/18386806/) | Family WZD8 | | 23 | 1555 | m.1555A\>G | [A1555G\-F23](https://mitofam.com/doc/1533) | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 8 | 4 | 2008 | [18386806](https://pubmed.ncbi.nlm.nih.gov/18386806/) | Family WZD9 | | 24 | 1555 | m.1555A\>G | [A1555G\-F24 ](https://mitofam.com/doc/1534)| China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 4 | 3 | 2008 | [18386806](https://pubmed.ncbi.nlm.nih.gov/18386806/) | Family WZD10 | | 25 | 1555 | m.1555A\>G | A1555G\-F25 | China | LHON; A1555G co\-occurrence | ND | ND | 2008 | [18775412](https://pubmed.ncbi.nlm.nih.gov/18775412/) | with G11778A | | 26 | 1555 | m.1555A\>G | [A1555G\-F26 ](https://mitofam.com/doc/1535)| China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 9 | 6 | 2009 | [19376484](https://pubmed.ncbi.nlm.nih.gov/19376484/) | Family WZD35 | | 27 | 1555 | m.1555A\>G |[ A1555G\-F27](https://mitofam.com/doc/1536) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 8 | 9 | 2012 | [22475488](https://pubmed.ncbi.nlm.nih.gov/22475488/) | | | 28 | 1555 | m.1555A\>G | [A1555G\-F28](https://mitofam.com/doc/1537) | China | Maternally inherited hearing loss | 3 | 6 | 2018 | [30272361](https://pubmed.ncbi.nlm.nih.gov/30272361/) | with A4317G | | 29 | 1555 | m.1555A\>G | [A1555G\-F29](https://mitofam.com/doc/1538) | Spain | Leigh syndrome; sensorineural hearing loss | 3 | 10 | 2020 | [32867169](https://pubmed.ncbi.nlm.nih.gov/32867169/) | | | 30 | 1555 | m.1555A\>G | A1555G\-F30 | Democratic Republic of the Congo | Nonsyndromic sensorineural deafness with variable age at onset | ND | ND | 1996 | [8800928](https://pubmed.ncbi.nlm.nih.gov/8800928/) | Family 1 | | 31 | 1555 | m.1555A\>G | [A1555G\-F31](https://mitofam.com/doc/1539) | Democratic Republic of the Congo | Nonsyndromic sensorineural deafness with variable age at onset | 2 | 1 | 1996 | [8800928](https://pubmed.ncbi.nlm.nih.gov/8800928/) | Family 3 | | 32 | 1555 | m.1555A\>G | [A1555G\-F32](https://mitofam.com/doc/1540) | Israel | Maternally inherited nonsyndromic deafness with A1555G | 9 | 9 | 1996 | [8817331](https://pubmed.ncbi.nlm.nih.gov/8817331/) | | | 33 | 1555 | m.1555A\>G | A1555G\-F33 | Greece | Sensorineural bilateral hearing loss cohort | ND | ND | 2011 | [21725156](https://pubmed.ncbi.nlm.nih.gov/21725156/) | | | 34 | 1555 | m.1555A\>G | A1555G\-F34 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S1 | | 35 | 1555 | m.1555A\>G | A1555G\-F35 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S2 | | 36 | 1555 | m.1555A\>G | A1555G\-F36 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S3 | | 37 | 1555 | m.1555A\>G | A1555G\-F37 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S4 | | 38 | 1555 | m.1555A\>G | A1555G\-F38 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S5 | | 39 | 1555 | m.1555A\>G | A1555G\-F39 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S6 | | 40 | 1555 | m.1555A\>G | A1555G\-F40 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S7 | | 41 | 1555 | m.1555A\>G | A1555G\-F41 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S8 | | 42 | 1555 | m.1555A\>G | A1555G\-F42 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S9 | | 43 | 1555 | m.1555A\>G | A1555G\-F43 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S10 | | 44 | 1555 | m.1555A\>G | A1555G\-F44 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S11 | | 45 | 1555 | m.1555A\>G | A1555G\-F45 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S12 | | 46 | 1555 | m.1555A\>G | A1555G\-F46 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S13 | | 47 | 1555 | m.1555A\>G | A1555G\-F47 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S14 | | 48 | 1555 | m.1555A\>G | A1555G\-F48 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S15 | | 49 | 1555 | m.1555A\>G | A1555G\-F49 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S16 | | 50 | 1555 | m.1555A\>G | A1555G\-F50 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S17 | | 51 | 1555 | m.1555A\>G | A1555G\-F51 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S18 | | 52 | 1555 | m.1555A\>G | A1555G\-F52 | Spain | Maternally inherited progressive sensorineural deafness | ND | ND | 1998 | [9490575](https://pubmed.ncbi.nlm.nih.gov/9490575/) | Pedigree S19 | | 53 | 1555 | m.1555A\>G |[ A1555G\-F53](https://mitofam.com/doc/1541/) | Belgium | Non\-syndromic hearing loss | 2 | 0 | 2008 | [18790089](https://pubmed.ncbi.nlm.nih.gov/18790089/) | | | 54 | 1555 | m.1555A\>G | A1555G\-F54 | Belgium | Non\-syndromic hearing loss | ND | ND | 2008 | [18790089](https://pubmed.ncbi.nlm.nih.gov/18790089/) | | | 55 | 1555 | m.1555A\>G | [A1555G\-F55](https://mitofam.com/doc/1542) | China | nonsyndromic/aminoglycoside\-associated hearing loss | 23 | 5 | 2011 | [21504270](https://pubmed.ncbi.nlm.nih.gov/21504270/) | | | 56 | 1555 | m.1555A\>G | [A1555G\-F56](https://mitofam.com/doc/1543) | United States | Restrictive cardiomyopathy | 2 | ND | 1999 | [9915970](https://pubmed.ncbi.nlm.nih.gov/9915970/) | | | 57 | 1555 | m.1555A\>G | A1555G\-F57 | Chinese cohort | Aminoglycoside\-induced deafness / ototoxicity | ND | ND | 1993 | [8285309](https://pubmed.ncbi.nlm.nih.gov/8285309/) | | | 58 | 1555 | m.1555A\>G | [A1555G\-F58](https://mitofam.com/doc/1544) | South Africa | Streptomycin\-induced sensorineural deafness | 24 | 10 | 1997 | [9391883](https://pubmed.ncbi.nlm.nih.gov/9391883/) | | | 59 | 1555 | m.1555A\>G | A1555G\-F59 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 1 | 3 | 2007 | [17637808](https://pubmed.ncbi.nlm.nih.gov/17637808/) | Family 1 | | 60 | 1555 | m.1555A\>G | A1555G\-F60 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 22 | 26 | 2007 | [17637808](https://pubmed.ncbi.nlm.nih.gov/17637808/) | Family 2 | | 61 | 1555 | m.1555A\>G | [A1555G\-F61](https://mitofam.com/doc/1545) | Belgium | Unexplained abnormal fatigue; mitochondrial dysfunction | ND | ND | 2018 | [29336589](https://pubmed.ncbi.nlm.nih.gov/29336589/) | | | 62 | 1555 | m.1555A\>G | A1555G\-F62 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 1 | | 63 | 1555 | m.1555A\>G | [A1555G\-F63](https://mitofam.com/doc/1546) | China | Maternally inherited nonsyndromic/aminoglycoside\-associated hearing loss | 4 | 16 | 2013 | [23774020](https://pubmed.ncbi.nlm.nih.gov/23774020/) | Family FE163; with A4317G | | 64 | 1555 | m.1555A\>G | A1555G\-F64 | Israel | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | ND | 11 | 1999 | [9950117](https://pubmed.ncbi.nlm.nih.gov/9950117/) | | | 65 | 1555 | m.1555A\>G | [A1555G\-F65](https://mitofam.com/doc/1547) | Tunisia | nonsyndromic hearing loss | 2 | 3 | 2006 | [16406239](https://pubmed.ncbi.nlm.nih.gov/16406239/) | | | 66 | 1555 | m.1555A\>G | [A1555G\-F66](https://mitofam.com/doc/1548) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 4 | 7 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD31; with G15927A | | 67 | 1555 | m.1555A\>G | [A1555G\-F67](https://mitofam.com/doc/1549) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 4 | 7 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD32; with G15927A | | 68 | 1555 | m.1555A\>G |[ A1555G\-F68](https://mitofam.com/doc/1550) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 13 | 13 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD33; with G15927A | | 69 | 1555 | m.1555A\>G |[ A1555G\-F69](https://mitofam.com/doc/1551) | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 9 | 9 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD34; with G15927A | | 70 | 1555 | m.1555A\>G | [A1555G\-F70](https://mitofam.com/doc/1552) | China | aminoglycoside\-induced and nonsyndromic hearing loss | 7 | 1 | 2020 | [32400865](https://pubmed.ncbi.nlm.nih.gov/32400865/) | Family 32400865 | | 71 | 1555 | m.1555A\>G | [A1555G\-F71](https://mitofam.com/doc/1553) | China | aminoglycoside\-induced and nonsyndromic hearing loss | 5 | 2 | 2020 | [32400865](https://pubmed.ncbi.nlm.nih.gov/32400865/) | Family 32400865 | | 72 | 1555 | m.1555A\>G | A1555G\-F72 | Poland | nonsyndromic/aminoglycoside\-associated hearing loss | ND | 2 | 2011 | [21621438](https://pubmed.ncbi.nlm.nih.gov/21621438/) | | | 73 | 1555 | m.1555A\>G | A1555G\-F73 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 9 | 1 | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ201 | | 74 | 1555 | m.1555A\>G | A1555G\-F74 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 11 | 1 | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ202 | | 75 | 1555 | m.1555A\>G | A1555G\-F75 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 11 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ203 | | 76 | 1555 | m.1555A\>G | A1555G\-F76 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 8 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ204 | | 77 | 1555 | m.1555A\>G | A1555G\-F77 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 15 | 1 | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ205 | | 78 | 1555 | m.1555A\>G | A1555G\-F78 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 21 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ206 | | 79 | 1555 | m.1555A\>G | A1555G\-F79 | Japan | aminoglycoside\-induced deafness family context; functional cybrid study | ND | ND | 1996 | [8687424](https://pubmed.ncbi.nlm.nih.gov/8687424/) | | | 80 | 1555 | m.1555A\>G | [A1555G\-F80](https://mitofam.com/doc/1554) | Italy | Nonsyndromic sensorineural hearing loss | 1 | 6 | 1998 | [9779807](https://pubmed.ncbi.nlm.nih.gov/9779807/) | Family A | | 81 | 1555 | m.1555A\>G | [A1555G\-F81](https://mitofam.com/doc/1555) | Italy | Nonsyndromic sensorineural hearing loss | 0 | 1 | 1998 | [9779807](https://pubmed.ncbi.nlm.nih.gov/9779807/) | Family B | | 82 | 1555 | m.1555A\>G | [A1555G\-F82](https://mitofam.com/doc/1556) | Germany | nonsyndromic hearing impairment | 1 | 3 | 2002 | [11857751](https://pubmed.ncbi.nlm.nih.gov/11857751/) | Family 11857751 | | 83 | 1555 | m.1555A\>G | [A1555G\-F83](https://mitofam.com/doc/1557) | Poland | Aminoglycoside\-induced sensorineural hearing loss | 0 | 1 | 2002 | [11857751](https://pubmed.ncbi.nlm.nih.gov/11857751/) | | | 84 | 1555 | m.1555A\>G | A1555G\-F84 | Poland | Aminoglycoside\-induced sensorineural hearing loss | ND | 0 | 2002 | [11857751](https://pubmed.ncbi.nlm.nih.gov/11857751/) | | | 85 | 1555 | m.1555A\>G | A1555G\-F85 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 86 | 1555 | m.1555A\>G | A1555G\-F86 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 87 | 1555 | m.1555A\>G | A1555G\-F87 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 88 | 1555 | m.1555A\>G | A1555G\-F88 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 89 | 1555 | m.1555A\>G | A1555G\-F89 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 90 | 1555 | m.1555A\>G | A1555G\-F90 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 91 | 1555 | m.1555A\>G | A1555G\-F91 | Japan | nonsyndromic and aminoglycoside\-associated sensorineural hearing loss | ND | ND | 2004 | [14755216](https://pubmed.ncbi.nlm.nih.gov/14755216/) | | | 92 | 1555 | m.1555A\>G | [A1555G\-F92](https://mitofam.com/doc/1558) | China | Aminoglycoside\-induced hearing impairment in a maternal pedigree | 7 | 2 | 2022 | [36292680](https://pubmed.ncbi.nlm.nih.gov/36292680/) | | | 93 | 1555 | m.1555A\>G |[ A1555G\-F93](https://mitofam.com/doc/1559) | China | Nonsyndromic hearing impairment in a maternal pedigree; m.4394C\>T modifier also present | 4 | 5 | 2022 | [36292680](https://pubmed.ncbi.nlm.nih.gov/36292680/) | | | 94 | 1555 | m.1555A\>G | A1555G\-F94 | China | aminoglycoside\-associated sensorineural hearing loss | ND | ND | 1996 | [8797567](https://pubmed.ncbi.nlm.nih.gov/8797567/) | | | 95 | 1555 | m.1555A\>G | [A1555G\-F95](https://mitofam.com/doc/1560) | Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | 2 | 4 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 96 | 1555 | m.1555A\>G | [A1555G\-F96 ](https://mitofam.com/doc/1561/)| Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | ND | 3 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 97 | 1555 | m.1555A\>G | [A1555G\-F97](https://mitofam.com/doc/1562) | Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | 2 | 4 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 98 | 1555 | m.1555A\>G | [A1555G\-F98](https://mitofam.com/doc/1563) | Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | 1 | 4 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 99 | 1555 | m.1555A\>G | [A1555G\-F99 ](https://mitofam.com/doc/1564)| Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | 1 | 3 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 100 | 1555 | m.1555A\>G |[ A1555G\-F100](https://mitofam.com/doc/1565) | Spain | Nonsyndromic hearing loss with heteroplasmic and homoplasmic A1555G carriers | 2 | 2 | 2003 | [12920080](https://pubmed.ncbi.nlm.nih.gov/12920080/) | | | 101 | 1555 | m.1555A\>G | A1555G\-F101 | China | Pediatric nonsyndromic hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 102 | 1555 | m.1555A\>G | [A1555G\-F102](https://mitofam.com/doc/1566) | Spain | Nonsyndromic sensorineural hearing loss with heteroplasmic A1555G | 5 | 5 | 2008 | [17999439](https://pubmed.ncbi.nlm.nih.gov/17999439/) | Family S292 | | 103 | 1555 | m.1555A\>G | [A1555G\-F103 ](https://mitofam.com/doc/1567)| Serbia | Leber hereditary optic neuropathy | 3 | 2 | 2020 | [32991883](https://pubmed.ncbi.nlm.nih.gov/32991883/) | | | 104 | 1555 | m.1555A\>G | [A1555G\-F104](https://mitofam.com/doc/1568) | Spain | Postlingual nonsyndromic hearing loss and unaffected A1555G carriers | 6 | 9 | 2006 | [16631122](https://pubmed.ncbi.nlm.nih.gov/16631122/) | | | 105 | 1555 | m.1555A\>G | A1555G\-F105 | Tunisia | Nonsyndromic hearing loss | ND | ND | 2008 | [18325329](https://pubmed.ncbi.nlm.nih.gov/18325329/) | | | 106 | 1555 | m.1555A\>G | [A1555G\-F106](https://mitofam.com/doc/1569) | Japan | Nonsyndromic sensorineural hearing loss/deafness, tinnitus; no aminoglycoside exposure reported | 90 | 32 | 2004 | [15179218](https://pubmed.ncbi.nlm.nih.gov/15179218/) | | | 107 | 1555 | m.1555A\>G | [A1555G\-F107 ](https://mitofam.com/doc/1570)| Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | ND | ND | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 108 | 1555 | m.1555A\>G | [A1555G\-F108](https://mitofam.com/doc/1571) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 3 | 0 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 109 | 1555 | m.1555A\>G | [A1555G\-F109](https://mitofam.com/doc/1572) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 1 | 2 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 110 | 1555 | m.1555A\>G |[ A1555G\-F110](https://mitofam.com/doc/1573) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 0 | 2 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 111 | 1555 | m.1555A\>G | [A1555G\-F111](https://mitofam.com/doc/1574) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 1 | 0 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 112 | 1555 | m.1555A\>G |[ A1555G\-F112](https://mitofam.com/doc/1575) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 3 | 2 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 113 | 1555 | m.1555A\>G | [A1555G\-F113 ](https://mitofam.com/doc/1576)| Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 2 | 0 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 114 | 1555 | m.1555A\>G | [A1555G\-F114](https://mitofam.com/doc/1577) | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | 0 | 4 | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 115 | 1555 | m.1555A\>G | A1555G\-F115 | Italy | Isolated idiopathic/nonsyndromic sensorineural hearing loss; some aminoglycoside\-associated | ND | ND | 2008 | [18215147](https://pubmed.ncbi.nlm.nih.gov/18215147/) | | | 116 | 1555 | m.1555A\>G | [A1555G\-F116](https://mitofam.com/doc/1578/) | Finland | Familial sensorineural hearing impairment; no aminoglycoside use in Family A | 13 | 7 | 2000 | [10854117](https://pubmed.ncbi.nlm.nih.gov/10854117/) | Family A | | 117 | 1555 | m.1555A\>G | A1555G\-F117 | Finland | Aminoglycoside\-induced hearing impairment | ND | 0 | 2000 | [10854117](https://pubmed.ncbi.nlm.nih.gov/10854117/) | Family B | | 118 | 1555 | m.1555A\>G | [A1555G\-F118](https://mitofam.com/doc/1579/) | China | Nonsyndromic/aminoglycoside\-associated hearing impairment; modifier m.4317A\>G also present | 6 | 16 | 2018 | [29348176](https://pubmed.ncbi.nlm.nih.gov/29348176/) | Family WZD91; with A4317G | | 119 | 1555 | m.1555A\>G | [A1555G\-F119](https://mitofam.com/doc/1580/) | China | Aminoglycoside\-associated severe hearing loss in four matrilineal relatives; other members normal hearing | 2 | 3 | 2018 | [29348176](https://pubmed.ncbi.nlm.nih.gov/29348176/) | Family WZD92 | | 120 | 1555 | m.1555A\>G |[ A1555G\-F120](https://mitofam.com/doc/1581) | China | Aminoglycoside\-induced and nonsyndromic hearing loss; modifier tRNAThr G15927A | 3 | 8 | 2008 | [18930888](https://pubmed.ncbi.nlm.nih.gov/18930888/) | Family WZD31 | | 121 | 1555 | m.1555A\>G | [A1555G\-F121](https://mitofam.com/doc/1582) | Israel | Nonsyndromic deafness / aminoglycoside susceptibility model; no aminoglycoside exposure in listed subjects | 5 | 4 | 2000 | [10915767](https://pubmed.ncbi.nlm.nih.gov/10915767/) | | | 122 | 1555 | m.1555A\>G | [A1555G\-F122](https://mitofam.com/doc/1583) | China | Maternally inherited nonsyndromic hearing loss; aminoglycoside\-induced profound hearing loss in exposed members | ND | ND | 2004 | [14699607](https://pubmed.ncbi.nlm.nih.gov/14699607/) | | | 123 | 1555 | m.1555A\>G | [A1555G\-F123](https://mitofam.com/doc/1584) | Japan | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | 1 | 0 | 1997 | [9111378](https://pubmed.ncbi.nlm.nih.gov/9111378/) | Family 3 | | 124 | 1555 | m.1555A\>G |[ A1555G\-F124 ](https://mitofam.com/doc/1585)| Japan | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | 0 | 3 | 1997 | [9111378](https://pubmed.ncbi.nlm.nih.gov/9111378/) | Family 5 | | 125 | 1555 | m.1555A\>G | [A1555G\-F125](https://mitofam.com/doc/1586) | Japan | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | 6 | 11 | 1997 | [9111378](https://pubmed.ncbi.nlm.nih.gov/9111378/) | Family 1 | | 126 | 1555 | m.1555A\>G | A1555G\-F126 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | 0 | 1 | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G8 | | 127 | 1555 | m.1555A\>G | A1555G\-F127 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G29 | | 128 | 1555 | m.1555A\>G | A1555G\-F128 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G38 | | 129 | 1555 | m.1555A\>G | A1555G\-F129 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G43 | | 130 | 1555 | m.1555A\>G | A1555G\-F130 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G101 | | 131 | 1555 | m.1555A\>G | A1555G\-F131 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G104 | | 132 | 1555 | m.1555A\>G | A1555G\-F132 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G136 | | 133 | 1555 | m.1555A\>G | [A1555G\-F133](https://mitofam.com/doc/1587) | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | 0 | 1 | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G144 | | 134 | 1555 | m.1555A\>G | A1555G\-F134 | Poland | Nonsyndromic and aminoglycoside\-induced hearing loss | ND | ND | 2010 | [20353758](https://pubmed.ncbi.nlm.nih.gov/20353758/) | Family G244 | | 135 | 1555 | m.1555A\>G | A1555G\-F135 | United Kingdom | Suspected mitochondrial disorder; partial genetic diagnosis from MT\-RNR1 m.1555A\>G | ND | 0 | 2021 | [34732400](https://pubmed.ncbi.nlm.nih.gov/34732400/) | Family 17 | | 136 | 1555 | m.1555A\>G | [A1555G\-F136](https://mitofam.com/doc/1588) | China | Maternally inherited sensorineural hearing loss with A1555G | ND | ND | 2008 | [18282333](https://pubmed.ncbi.nlm.nih.gov/18282333/) | Family 18282333 | | 137 | 1555 | m.1555A\>G | A1555G\-F137 | United States | Pediatric nonsyndromic sensorineural hearing loss | ND | ND | 2004 | [15286157](https://pubmed.ncbi.nlm.nih.gov/15286157/) | | | 138 | 1555 | m.1555A\>G | A1555G\-F138 | China | Sensorineural hearing impairment with homoplasmic A1555G | 14 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD13 | | 139 | 1555 | m.1555A\>G | A1555G\-F139 | China | Sensorineural hearing impairment with homoplasmic A1555G | 5 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD14 | | 140 | 1555 | m.1555A\>G | A1555G\-F140 | China | Sensorineural hearing impairment with homoplasmic A1555G | 20 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD15 | | 141 | 1555 | m.1555A\>G | A1555G\-F141 | China | Sensorineural hearing impairment with homoplasmic A1555G | 11 | 4 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD16 | | 142 | 1555 | m.1555A\>G | A1555G\-F142 | China | Sensorineural hearing impairment with homoplasmic A1555G | 16 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD17 | | 143 | 1555 | m.1555A\>G | A1555G\-F143 | China | Sensorineural hearing impairment with homoplasmic A1555G | 9 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD18 | | 144 | 1555 | m.1555A\>G | A1555G\-F144 | China | Sensorineural hearing impairment with homoplasmic A1555G | 11 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD19 | | 145 | 1555 | m.1555A\>G | A1555G\-F145 | China | Sensorineural hearing impairment with homoplasmic A1555G | 14 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD20 | | 146 | 1555 | m.1555A\>G | A1555G\-F146 | China | Sensorineural hearing impairment with homoplasmic A1555G | 21 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD21 | | 147 | 1555 | m.1555A\>G | A1555G\-F147 | China | Sensorineural hearing impairment with homoplasmic A1555G | 17 | 11 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD22 | | 148 | 1555 | m.1555A\>G | A1555G\-F148 | China | Sensorineural hearing impairment with homoplasmic A1555G | 18 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD23 | | 149 | 1555 | m.1555A\>G | A1555G\-F149 | China | Sensorineural hearing impairment with homoplasmic A1555G | 7 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD24 | | 150 | 1555 | m.1555A\>G | A1555G\-F150 | China | Sensorineural hearing impairment with homoplasmic A1555G | 9 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD25 | | 151 | 1555 | m.1555A\>G | A1555G\-F151 | China | Sensorineural hearing impairment with homoplasmic A1555G | 4 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD26 | | 152 | 1555 | m.1555A\>G | A1555G\-F152 | China | Sensorineural hearing impairment with homoplasmic A1555G | 4 | 2 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD27 | | 153 | 1555 | m.1555A\>G | A1555G\-F153 | China | Sensorineural hearing impairment with homoplasmic A1555G | 21 | 2 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD28 | | 154 | 1555 | m.1555A\>G | A1555G\-F154 | China | Sensorineural hearing impairment with homoplasmic A1555G | 7 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD29 | | 155 | 1555 | m.1555A\>G | A1555G\-F155 | China | Sensorineural hearing impairment with homoplasmic A1555G | 3 | 4 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD30 | | 156 | 1555 | m.1555A\>G | A1555G\-F156 | China | Sensorineural hearing impairment with homoplasmic A1555G | 14 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD35 | | 157 | 1555 | m.1555A\>G | A1555G\-F157 | China | Sensorineural hearing impairment with homoplasmic A1555G | 13 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD36 | | 158 | 1555 | m.1555A\>G | A1555G\-F158 | China | Sensorineural hearing impairment with homoplasmic A1555G | 21 | 8 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD37 | | 159 | 1555 | m.1555A\>G | A1555G\-F159 | China | Sensorineural hearing impairment with homoplasmic A1555G | 9 | 12 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD38 | | 160 | 1555 | m.1555A\>G | A1555G\-F160 | China | Sensorineural hearing impairment with homoplasmic A1555G | 13 | 2 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD39 | | 161 | 1555 | m.1555A\>G | A1555G\-F161 | China | Sensorineural hearing impairment with homoplasmic A1555G | 14 | 5 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD40 | | 162 | 1555 | m.1555A\>G | A1555G\-F162 | China | Sensorineural hearing impairment with homoplasmic A1555G | 31 | 5 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD41 | | 163 | 1555 | m.1555A\>G | A1555G\-F163 | China | Sensorineural hearing impairment with homoplasmic A1555G | 6 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD42 | | 164 | 1555 | m.1555A\>G | A1555G\-F164 | China | Sensorineural hearing impairment with homoplasmic A1555G | 60 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD43 | | 165 | 1555 | m.1555A\>G | A1555G\-F165 | China | Sensorineural hearing impairment with homoplasmic A1555G | 13 | 0 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD44 | | 166 | 1555 | m.1555A\>G | A1555G\-F166 | China | Sensorineural hearing impairment with homoplasmic A1555G | 8 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD45 | | 167 | 1555 | m.1555A\>G | A1555G\-F167 | China | Sensorineural hearing impairment with homoplasmic A1555G | 9 | 4 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD46 | | 168 | 1555 | m.1555A\>G | A1555G\-F168 | China | Sensorineural hearing impairment with homoplasmic A1555G | 20 | 4 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD47 | | 169 | 1555 | m.1555A\>G | A1555G\-F169 | China | Sensorineural hearing impairment with homoplasmic A1555G | 11 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD48 | | 170 | 1555 | m.1555A\>G | A1555G\-F170 | China | Sensorineural hearing impairment with homoplasmic A1555G | 23 | 6 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD49 | | 171 | 1555 | m.1555A\>G | A1555G\-F171 | China | Sensorineural hearing impairment with homoplasmic A1555G | 7 | 7 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD50 | | 172 | 1555 | m.1555A\>G | A1555G\-F172 | China | Sensorineural hearing impairment with homoplasmic A1555G | 11 | 6 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD51 | | 173 | 1555 | m.1555A\>G | A1555G\-F173 | China | Sensorineural hearing impairment with homoplasmic A1555G | 16 | 2 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD52 | | 174 | 1555 | m.1555A\>G | A1555G\-F174 | China | Sensorineural hearing impairment with homoplasmic A1555G | 11 | 5 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD53 | | 175 | 1555 | m.1555A\>G | A1555G\-F175 | China | Sensorineural hearing impairment with homoplasmic A1555G | 15 | 9 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD54 | | 176 | 1555 | m.1555A\>G | A1555G\-F176 | China | Sensorineural hearing impairment with homoplasmic A1555G | 13 | 1 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD55 | | 177 | 1555 | m.1555A\>G | A1555G\-F177 | China | Sensorineural hearing impairment with homoplasmic A1555G | 9 | 7 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD56 | | 178 | 1555 | m.1555A\>G | A1555G\-F178 | China | Sensorineural hearing impairment with homoplasmic A1555G | 16 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD57 | | 179 | 1555 | m.1555A\>G | A1555G\-F179 | China | Sensorineural hearing impairment with homoplasmic A1555G | 4 | 3 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD58 | | 180 | 1555 | m.1555A\>G | A1555G\-F180 | China | Sensorineural hearing impairment with homoplasmic A1555G | 18 | 12 | 2009 | [19818876](https://pubmed.ncbi.nlm.nih.gov/19818876/) | Family WZD59 | | 181 | 1555 | m.1555A\>G |[ A1555G\-F181](https://mitofam.com/doc/1589) | Japan | Maternally inherited hearing loss with A1555G | 3 | 6 | 2002 | [12031626](https://pubmed.ncbi.nlm.nih.gov/12031626/) | Family 1 | | 182 | 1555 | m.1555A\>G | [A1555G\-F182](https://mitofam.com/doc/1590) | Japan | Maternally inherited hearing loss with A1555G | 14 | 5 | 2002 | [12031626](https://pubmed.ncbi.nlm.nih.gov/12031626/) | Family 2 | | 183 | 1555 | m.1555A\>G | [A1555G\-F183](https://mitofam.com/doc/1591) | Japan | Maternally inherited hearing loss with A1555G | 10 | 3 | 2002 | [12031626](https://pubmed.ncbi.nlm.nih.gov/12031626/) | Family 3 | | 184 | 1555 | m.1555A\>G | A1555G\-F184 | Portugal | Pediatric sensorineural hearing loss; syndromic case with m.1555A\>G | ND | ND | 2012 | [22567359](https://pubmed.ncbi.nlm.nih.gov/22567359/) | | | 185 | 1555 | m.1555A\>G |[ A1555G\-F185](https://mitofam.com/doc/1592) | China | De novo heteroplasmic A1555G carrier family; severe deafness in another child explained by GJB2 rather than A1555G | 2 | 0 | 2022 | [35614445](https://pubmed.ncbi.nlm.nih.gov/35614445/) | | | 186 | 1555 | m.1555A\>G | [A1555G\-F186](https://mitofam.com/doc/1593) | Portugal | Mitochondrial disease cohort with three homoplasmic MT\-RNR1 m.1555A\>G patients | ND | ND | 2024 | [38465286](https://pubmed.ncbi.nlm.nih.gov/38465286/) | Family 38465286 | | 187 | 1555 | m.1555A\>G | A1555G\-F187 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE003 | | 188 | 1555 | m.1555A\>G | A1555G\-F188 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE007 | | 189 | 1555 | m.1555A\>G | A1555G\-F189 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE008 | | 190 | 1555 | m.1555A\>G | A1555G\-F190 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE0128 | | 191 | 1555 | m.1555A\>G | A1555G\-F191 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE019 | | 192 | 1555 | m.1555A\>G | A1555G\-F192 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE020 | | 193 | 1555 | m.1555A\>G | A1555G\-F193 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE036 | | 194 | 1555 | m.1555A\>G | A1555G\-F194 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE081 | | 195 | 1555 | m.1555A\>G | A1555G\-F195 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE122 | | 196 | 1555 | m.1555A\>G | A1555G\-F196 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE141 | | 197 | 1555 | m.1555A\>G | A1555G\-F197 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE154 | | 198 | 1555 | m.1555A\>G | A1555G\-F198 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE160 | | 199 | 1555 | m.1555A\>G | A1555G\-F199 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE163 | | 200 | 1555 | m.1555A\>G | A1555G\-F200 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE300 | | 201 | 1555 | m.1555A\>G | A1555G\-F201 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE304 | | 202 | 1555 | m.1555A\>G | A1555G\-F202 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE317 | | 203 | 1555 | m.1555A\>G | A1555G\-F203 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family FE350 | | 204 | 1555 | m.1555A\>G | A1555G\-F204 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB038 | | 205 | 1555 | m.1555A\>G | A1555G\-F205 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB048 | | 206 | 1555 | m.1555A\>G | A1555G\-F206 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB052 | | 207 | 1555 | m.1555A\>G | A1555G\-F207 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB076 | | 208 | 1555 | m.1555A\>G | A1555G\-F208 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB078 | | 209 | 1555 | m.1555A\>G | A1555G\-F209 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB079 | | 210 | 1555 | m.1555A\>G | A1555G\-F210 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB094 | | 211 | 1555 | m.1555A\>G | A1555G\-F211 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB111 | | 212 | 1555 | m.1555A\>G | A1555G\-F212 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB126 | | 213 | 1555 | m.1555A\>G | A1555G\-F213 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family NB137 | | 214 | 1555 | m.1555A\>G | A1555G\-F214 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX019 | | 215 | 1555 | m.1555A\>G | A1555G\-F215 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX022 | | 216 | 1555 | m.1555A\>G | A1555G\-F216 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX025 | | 217 | 1555 | m.1555A\>G | A1555G\-F217 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX028 | | 218 | 1555 | m.1555A\>G | A1555G\-F218 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX037 | | 219 | 1555 | m.1555A\>G | A1555G\-F219 | China | pediatric nonsyndromic hearing impairment cohort | ND | ND | 2011 | [21205314](https://pubmed.ncbi.nlm.nih.gov/21205314/) | Family ZX047 | | 220 | 1555 | m.1555A\>G | [A1555G\-F220 ](https://mitofam.com/doc/1594)| Japan | sporadic/progressive sensorineural hearing loss with A1555G\-positive daughters | 2 | 0 | 1996 | [8973709](https://pubmed.ncbi.nlm.nih.gov/8973709/) | | | 221 | 1555 | m.1555A\>G | [A1555G\-F221](https://mitofam.com/doc/1595) | Israel | symptomatic and asymptomatic members of a large family carrying A1555G; cybrid/functional study | ND | ND | 2001 | [11230176](https://pubmed.ncbi.nlm.nih.gov/11230176/) | | | 222 | 1555 | m.1555A\>G | [A1555G\-F222 ](https://mitofam.com/doc/1596)| Turkey | prelingual nonsyndromic profound deafness; first proband also had right parietal infarct | 4 | 0 | 2003 | [12655418](https://pubmed.ncbi.nlm.nih.gov/12655418/) | Family 1 | | 223 | 1555 | m.1555A\>G | A1555G\-F223 | Turkey | prelingual nonsyndromic profound deafness | ND | ND | 2003 | [12655418](https://pubmed.ncbi.nlm.nih.gov/12655418/) | Family 1 | | 224 | 1555 | m.1555A\>G | A1555G\-F224 | Turkey | prelingual nonsyndromic profound deafness with sibling family history | ND | 1 | 2003 | [12655418](https://pubmed.ncbi.nlm.nih.gov/12655418/) | Family 1 | | 225 | 1555 | m.1555A\>G | [A1555G\-F225](https://mitofam.com/doc/1597) | Finland | large multigenerational family with m.1555A\>G and SSBP1 start\-loss modifier segregating with hearing loss | 5 | 7 | 2018 | [29182774](https://pubmed.ncbi.nlm.nih.gov/29182774/) | | | 226 | 1555 | m.1555A\>G | [A1555G\-F226](https://mitofam.com/doc/1598) | Finland | large multigenerational family with m.1555A\>G and SSBP1 start\-loss modifier segregating with hearing loss | 2 | 4 | 2018 | [29182774](https://pubmed.ncbi.nlm.nih.gov/29182774/) | Family A | | 227 | 1555 | m.1555A\>G | [A1555G\-F227](https://mitofam.com/doc/1599) | Finland | large multigenerational family with m.1555A\>G and SSBP1 start\-loss modifier segregating with hearing loss | 3 | 6 | 2018 | [29182774](https://pubmed.ncbi.nlm.nih.gov/29182774/) | Family B | | 228 | 1555 | m.1555A\>G | [A1555G\-F228](https://mitofam.com/doc/1600) | Finland | large multigenerational family with m.1555A\>G and SSBP1 start\-loss modifier segregating with hearing loss | 4 | 0 | 2018 | [29182774](https://pubmed.ncbi.nlm.nih.gov/29182774/) | Family C | | 229 | 1555 | m.1555A\>G | A1555G\-F229 | United Kingdom | unrelated hearing\-impaired m.1555A\>G carrier used as comparator | 0 | 0 | 2018 | [29182774](https://pubmed.ncbi.nlm.nih.gov/29182774/) | | | 230 | 1555 | m.1555A\>G | A1555G\-F230 | United States | aminoglycoside\-induced sensorineural hearing loss | 0 | 1 | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 231 | 1555 | m.1555A\>G | A1555G\-F231 | Japan | aminoglycoside\-induced postlingual/profound sensorineural hearing loss; Figure 1 Patient\-1 pedigree | 52 | 10 | 2001 | [11174059](https://pubmed.ncbi.nlm.nih.gov/11174059/) | | | 232 | 1555 | m.1555A\>G | A1555G\-F232 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 233 | 1555 | m.1555A\>G | A1555G\-F233 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 234 | 1555 | m.1555A\>G | A1555G\-F234 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 235 | 1555 | m.1555A\>G | A1555G\-F235 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 236 | 1555 | m.1555A\>G | A1555G\-F236 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 237 | 1555 | m.1555A\>G | A1555G\-F237 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 238 | 1555 | m.1555A\>G | A1555G\-F238 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 239 | 1555 | m.1555A\>G | A1555G\-F239 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 240 | 1555 | m.1555A\>G | A1555G\-F240 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 241 | 1555 | m.1555A\>G | A1555G\-F241 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 242 | 1555 | m.1555A\>G | A1555G\-F242 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 243 | 1555 | m.1555A\>G | A1555G\-F243 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 244 | 1555 | m.1555A\>G | A1555G\-F244 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 245 | 1555 | m.1555A\>G | A1555G\-F245 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 246 | 1555 | m.1555A\>G | A1555G\-F246 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 247 | 1555 | m.1555A\>G | A1555G\-F247 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 248 | 1555 | m.1555A\>G | A1555G\-F248 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 249 | 1555 | m.1555A\>G | A1555G\-F249 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 250 | 1555 | m.1555A\>G | A1555G\-F250 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 251 | 1555 | m.1555A\>G | A1555G\-F251 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 252 | 1555 | m.1555A\>G | A1555G\-F252 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 253 | 1555 | m.1555A\>G | A1555G\-F253 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 254 | 1555 | m.1555A\>G | A1555G\-F254 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 255 | 1555 | m.1555A\>G | A1555G\-F255 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 256 | 1555 | m.1555A\>G | A1555G\-F256 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 257 | 1555 | m.1555A\>G | A1555G\-F257 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 258 | 1555 | m.1555A\>G | A1555G\-F258 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 259 | 1555 | m.1555A\>G | A1555G\-F259 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 260 | 1555 | m.1555A\>G | A1555G\-F260 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 261 | 1555 | m.1555A\>G | A1555G\-F261 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 262 | 1555 | m.1555A\>G | A1555G\-F262 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 263 | 1555 | m.1555A\>G | A1555G\-F263 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 264 | 1555 | m.1555A\>G | A1555G\-F264 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 265 | 1555 | m.1555A\>G | A1555G\-F265 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 266 | 1555 | m.1555A\>G | A1555G\-F266 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 267 | 1555 | m.1555A\>G | A1555G\-F267 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 268 | 1555 | m.1555A\>G | A1555G\-F268 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 269 | 1555 | m.1555A\>G | A1555G\-F269 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 270 | 1555 | m.1555A\>G | A1555G\-F270 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 271 | 1555 | m.1555A\>G | A1555G\-F271 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 272 | 1555 | m.1555A\>G | A1555G\-F272 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 273 | 1555 | m.1555A\>G | A1555G\-F273 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 274 | 1555 | m.1555A\>G | A1555G\-F274 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 275 | 1555 | m.1555A\>G | A1555G\-F275 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 276 | 1555 | m.1555A\>G | A1555G\-F276 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 277 | 1555 | m.1555A\>G | A1555G\-F277 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 278 | 1555 | m.1555A\>G | A1555G\-F278 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 279 | 1555 | m.1555A\>G | A1555G\-F279 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 280 | 1555 | m.1555A\>G | A1555G\-F280 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 281 | 1555 | m.1555A\>G | A1555G\-F281 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | | | 282 | 1555 | m.1555A\>G | A1555G\-F282 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD1 | | 283 | 1555 | m.1555A\>G | A1555G\-F283 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD2 | | 284 | 1555 | m.1555A\>G | A1555G\-F284 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD3 | | 285 | 1555 | m.1555A\>G | A1555G\-F285 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD4 | | 286 | 1555 | m.1555A\>G | A1555G\-F286 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD5 | | 287 | 1555 | m.1555A\>G | A1555G\-F287 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD6 | | 288 | 1555 | m.1555A\>G | A1555G\-F288 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD7 | | 289 | 1555 | m.1555A\>G | A1555G\-F289 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD8 | | 290 | 1555 | m.1555A\>G | A1555G\-F290 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD9 | | 291 | 1555 | m.1555A\>G | A1555G\-F291 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD10 | | 292 | 1555 | m.1555A\>G | A1555G\-F292 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD31 | | 293 | 1555 | m.1555A\>G | A1555G\-F293 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD32 | | 294 | 1555 | m.1555A\>G | A1555G\-F294 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD33 | | 295 | 1555 | m.1555A\>G | A1555G\-F295 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD34 | | 296 | 1555 | m.1555A\>G | A1555G\-F296 | China | Nonsyndromic or aminoglycoside\-induced hearing loss | ND | 0 | 2010 | [20100600](https://pubmed.ncbi.nlm.nih.gov/20100600/) | Family WZD11 | | 297 | 1555 | m.1555A\>G | A1555G\-F297 | Japan | Maternally transmitted aminoglycoside\-induced deafness / otosensitivity | ND | ND | 1993 | [8414970](https://pubmed.ncbi.nlm.nih.gov/8414970/) | | | 298 | 1555 | m.1555A\>G | A1555G\-F298 | Japan | Maternally transmitted aminoglycoside\-induced deafness / otosensitivity | ND | ND | 1993 | [8414970](https://pubmed.ncbi.nlm.nih.gov/8414970/) | | | 299 | 1555 | m.1555A\>G | A1555G\-F299 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 8 | 5 | 2007 | [17637808](https://pubmed.ncbi.nlm.nih.gov/17637808/) | Family 3 | | 300 | 1555 | m.1555A\>G | A1555G\-F300 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 6 | 4 | 2007 | [17637808](https://pubmed.ncbi.nlm.nih.gov/17637808/) | Family 4 | | 301 | 1555 | m.1555A\>G | A1555G\-F301 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 14 | 8 | 2007 | [17637808](https://pubmed.ncbi.nlm.nih.gov/17637808/) | Family 5 | | 302 | 1555 | m.1555A\>G | [A1555G\-F302](https://mitofam.com/doc/1601) | Denmark | Sensorineural hearing impairment with A1555G | 3 | 0 | 2002 | [12372057](https://pubmed.ncbi.nlm.nih.gov/12372057/) | | | 303 | 1555 | m.1555A\>G | A1555G\-F303 | Denmark | Sensorineural hearing impairment with A1555G | ND | ND | 2002 | [12372057](https://pubmed.ncbi.nlm.nih.gov/12372057/) | | | 304 | 1555 | m.1555A\>G | A1555G\-F304 | Japan | aminoglycoside\-induced postlingual/profound sensorineural hearing loss; Figure 1 Patient\-2 pedigree | 2 | 2 | 2001 | [11174059](https://pubmed.ncbi.nlm.nih.gov/11174059/) | | | 305 | 1555 | m.1555A\>G | A1555G\-F305 | Japan | aminoglycoside\-induced postlingual/profound sensorineural hearing loss; Figure 1 Patient\-3 pedigree | 4 | 4 | 2001 | [11174059](https://pubmed.ncbi.nlm.nih.gov/11174059/) | | | 306 | 1555 | m.1555A\>G | A1555G\-F306 | Japan | aminoglycoside\-induced postlingual/profound sensorineural hearing loss; Figure 1 Patient\-4 pedigree | 7 | 3 | 2001 | [11174059](https://pubmed.ncbi.nlm.nih.gov/11174059/) | | | 307 | 1555 | m.1555A\>G | A1555G\-F307 | Indonesia | Non\-syndromic sensorineural deafness; A1555G carrier | ND | ND | 2003 | [12955586](https://pubmed.ncbi.nlm.nih.gov/12955586/) | | | 308 | 1555 | m.1555A\>G | A1555G\-F308 | Indonesia | Non\-syndromic sensorineural deafness; A1555G carrier | ND | ND | 2003 | [12955586](https://pubmed.ncbi.nlm.nih.gov/12955586/) | | | 309 | 1555 | m.1555A\>G | A1555G\-F309 | Indonesia | Non\-syndromic sensorineural deafness; A1555G carrier | ND | ND | 2003 | [12955586](https://pubmed.ncbi.nlm.nih.gov/12955586/) | | | 310 | 1555 | m.1555A\>G | A1555G\-F310 | Indonesia | Non\-syndromic sensorineural deafness; A1555G carrier | ND | ND | 2003 | [12955586](https://pubmed.ncbi.nlm.nih.gov/12955586/) | | | 311 | 1555 | m.1555A\>G | A1555G\-F311 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 11 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ207 | | 312 | 1555 | m.1555A\>G | A1555G\-F312 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 20 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ208 | | 313 | 1555 | m.1555A\>G | A1555G\-F313 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 9 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ209 | | 314 | 1555 | m.1555A\>G | A1555G\-F314 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 23 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ210 | | 315 | 1555 | m.1555A\>G | A1555G\-F315 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 11 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ211 | | 316 | 1555 | m.1555A\>G | A1555G\-F316 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 10 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ212 | | 317 | 1555 | m.1555A\>G | A1555G\-F317 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 12 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ213 | | 318 | 1555 | m.1555A\>G | A1555G\-F318 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 19 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ214 | | 319 | 1555 | m.1555A\>G | A1555G\-F319 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 24 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ215 | | 320 | 1555 | m.1555A\>G | A1555G\-F320 | China | Aminoglycoside\-induced sensorineural hearing loss/deafness | 13 | ND | 2006 | [16375862](https://pubmed.ncbi.nlm.nih.gov/16375862/) | Family BJ216 | | 321 | 1555 | m.1555A\>G | A1555G\-F321 | China | Aminoglycoside\-induced and nonsyndromic hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | Family A | | 322 | 1555 | m.1555A\>G | A1555G\-F322 | China | Aminoglycoside\-induced and nonsyndromic hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | Family B | | 323 | 1555 | m.1555A\>G | [A1555G\-F323 ](https://mitofam.com/doc/1602)| China | Aminoglycoside\-induced and nonsyndromic hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | Family C | | 324 | 1555 | m.1555A\>G | A1555G\-F324 | China | Aminoglycoside\-induced hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 325 | 1555 | m.1555A\>G | A1555G\-F325 | China | Pediatric nonsyndromic hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 326 | 1555 | m.1555A\>G | A1555G\-F326 | China | Aminoglycoside\-induced hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 327 | 1555 | m.1555A\>G | A1555G\-F327 | China | Aminoglycoside\-induced hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 328 | 1555 | m.1555A\>G | A1555G\-F328 | China | Aminoglycoside\-induced hearing loss | ND | ND | 2005 | [15841390](https://pubmed.ncbi.nlm.nih.gov/15841390/) | | | 329 | 1555 | m.1555A\>G | A1555G\-F329 | France | Mitochondrial disease cohort with homoplasmic MT\-RNR1 m.1555A\>G | ND | ND | 2013 | [23847141](https://pubmed.ncbi.nlm.nih.gov/23847141/) | | | 330 | 1555 | m.1555A\>G | A1555G\-F330 | France | Mitochondrial disease cohort with homoplasmic MT\-RNR1 m.1555A\>G | ND | ND | 2013 | [23847141](https://pubmed.ncbi.nlm.nih.gov/23847141/) | | | 331 | 1555 | m.1555A\>G | A1555G\-F331 | France | Mitochondrial disease cohort with homoplasmic MT\-RNR1 m.1555A\>G | ND | ND | 2013 | [23847141](https://pubmed.ncbi.nlm.nih.gov/23847141/) | | | 332 | 1555 | m.1555A\>G | A1555G\-F332 | China | Nonsyndromic hearing loss | ND | 2 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree FE451 | | 333 | 1555 | m.1555A\>G | A1555G\-F333 | China | Nonsyndromic hearing loss | 7 | ND | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree FE386 | | 334 | 1555 | m.1555A\>G | A1555G\-F334 | China | Nonsyndromic hearing loss | 13 | 1 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL013 | | 335 | 1555 | m.1555A\>G | A1555G\-F335 | China | Nonsyndromic hearing loss | 2 | ND | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL157 | | 336 | 1555 | m.1555A\>G | A1555G\-F336 | China | Nonsyndromic hearing loss | 2 | 3 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL041 | | 337 | 1555 | m.1555A\>G | A1555G\-F337 | China | Nonsyndromic hearing loss | ND | ND | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree ZX077 | | 338 | 1555 | m.1555A\>G | A1555G\-F338 | China | Nonsyndromic hearing loss | 7 | 3 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree FE487 | | 339 | 1555 | m.1555A\>G | A1555G\-F339 | China | Nonsyndromic hearing loss | 14 | 3 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL008 | | 340 | 1555 | m.1555A\>G | A1555G\-F340 | China | Nonsyndromic hearing loss | 4 | 2 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL077 | | 341 | 1555 | m.1555A\>G | A1555G\-F341 | China | Nonsyndromic hearing loss | 8 | 5 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WL108 | | 342 | 1555 | m.1555A\>G | A1555G\-F342 | China | Nonsyndromic hearing loss | 7 | 3 | 2015 | [26361786](https://pubmed.ncbi.nlm.nih.gov/26361786/) | Pedigree WLT173 | | 343 | 1555 | m.1555A\>G | A1555G\-F343 | Japan | Sensorineural hearing loss | ND | ND | 2017 | [28320335](https://pubmed.ncbi.nlm.nih.gov/28320335/) | with T7511C | | 344 | 1555 | m.1555A\>G | A1555G\-F344 | Japan | Sensorineural hearing loss | ND | ND | 2017 | [28320335](https://pubmed.ncbi.nlm.nih.gov/28320335/) | with T7511C | | 345 | 1555 | m.1555A\>G | A1555G\-F345 | Japan | Sensorineural hearing loss | ND | ND | 2017 | [28320335](https://pubmed.ncbi.nlm.nih.gov/28320335/) | with T7511C | | 346 | 1555 | m.1555A\>G | A1555G\-F346 | Japan | Sensorineural hearing loss | ND | ND | 2017 | [28320335](https://pubmed.ncbi.nlm.nih.gov/28320335/) | with T7511C | | 347 | 1555 | m.1555A\>G | A1555G\-F347 | Spain | intellectual disability; A1555G carrier; deafness absent; visual loss reported | ND | ND | 2018 | [29340697](https://pubmed.ncbi.nlm.nih.gov/29340697/) | | | 348 | 1555 | m.1555A\>G | A1555G\-F348 | Spain | autism spectrum disorder with intellectual disability; A1555G carrier; deafness absent | ND | ND | 2018 | [29340697](https://pubmed.ncbi.nlm.nih.gov/29340697/) | | | 349 | 1555 | m.1555A\>G | A1555G\-F349 | Spain | autism spectrum disorder with intellectual disability; deafness; A1555G carrier | ND | ND | 2018 | [29340697](https://pubmed.ncbi.nlm.nih.gov/29340697/) | | | 350 | 1555 | m.1555A\>G | A1555G\-F350 | South Korea | Non\-syndromic hearing loss due to MT\-RNR1 m.1555A\>G; patient\-derived fibroblast model | 0 | 0 | 2026 | [42115265](https://pubmed.ncbi.nlm.nih.gov/42115265/) | | | 351 | 1555 | m.1555A\>G | A1555G\-F351 | South Korea | Non\-syndromic hearing loss due to MT\-RNR1 m.1555A\>G; patient\-derived fibroblast model | 0 | 0 | 2026 | [42115265](https://pubmed.ncbi.nlm.nih.gov/42115265/) | | | 352 | 1555 | m.1555A\>G | A1555G\-F352 | Japan | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | 0 | 0 | 1997 | [9111378](https://pubmed.ncbi.nlm.nih.gov/9111378/) | Family 2 | | 353 | 1555 | m.1555A\>G | [A1555G\-F353](https://mitofam.com/doc/1603/) | Japan | Aminoglycoside\-induced and nonsyndromic sensorineural hearing loss | 0 | 2 | 1997 | [9111378](https://pubmed.ncbi.nlm.nih.gov/9111378/) | Family 4 | | 354 | 1555 | m.1555A\>G | A1555G\-F354 | United States | Aminoglycoside\-induced sensorineural hearing loss | ND | ND | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 355 | 1555 | m.1555A\>G | A1555G\-F355 | United States | Aminoglycoside\-induced sensorineural hearing loss | ND | ND | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 356 | 1555 | m.1555A\>G | A1555G\-F356 | United States | Aminoglycoside\-induced sensorineural hearing loss | 0 | 0 | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | with C1525T | | 357 | 1555 | m.1555A\>G | A1555G\-F357 | United States | Aminoglycoside\-induced sensorineural hearing loss | 0 | 0 | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 358 | 1555 | m.1555A\>G | A1555G\-F358 | United States | Aminoglycoside\-induced sensorineural hearing loss | 0 | 0 | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 359 | 1555 | m.1555A\>G | A1555G\-F359 | United States | Aminoglycoside\-induced sensorineural hearing loss | 0 | 2 | 1997 | [9164619](https://pubmed.ncbi.nlm.nih.gov/9164619/) | | | 360 | 1555 | m.1555A\>G | A1555G\-F360 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 2 | | 361 | 1555 | m.1555A\>G | A1555G\-F361 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 3 | | 362 | 1555 | m.1555A\>G | A1555G\-F362 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 4 | | 363 | 1555 | m.1555A\>G | A1555G\-F363 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 5 | | 364 | 1555 | m.1555A\>G | A1555G\-F364 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 6 | | 365 | 1555 | m.1555A\>G | A1555G\-F365 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 7 | | 366 | 1555 | m.1555A\>G | A1555G\-F366 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 8 | | 367 | 1555 | m.1555A\>G | A1555G\-F367 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 9 | | 368 | 1555 | m.1555A\>G | A1555G\-F368 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 10 | | 369 | 1555 | m.1555A\>G | A1555G\-F369 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 11 | | 370 | 1555 | m.1555A\>G | A1555G\-F370 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 12 | | 371 | 1555 | m.1555A\>G | A1555G\-F371 | Japan | Sensorineural hearing loss | ND | ND | 1998 | [9887373](https://pubmed.ncbi.nlm.nih.gov/9887373/) | Family 13 | # **Carrier Information** | **No.** | **Position** | **Variant** | **Family ID** | **Carrier ID** | **Type (Fam/De novo/Uninf)** | **Gender (F/M)** | **Proband (Y/N)** | **Affected (Y/N)** | **Die/Alive(D/A)** | **Age** | **Blood Mutant Level** | **Muscle Mutant level** | **Urine Mutant level** | **Other Mutant level** | **Clinical Features** | **Note** | | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | | 1 | 1555 | m.1555A\>G | A1555G\-F2 | A1555G\-F2\-III5 | Fam | F | Y | Y | A | 23 | Homo | / | / | / | Bilateral sensorineural hearing loss since age 1\-2 | | | 2 | 1555 | m.1555A\>G | A1555G\-F2 | A1555G\-F2\-II2 | Fam | M | N | Y | A | 54 | 85% | / | / | / | Bilateral sensorineural deafness | Maternal uncle | | 3 | 1555 | m.1555A\>G | A1555G\-F2 | A1555G\-F2\-II4 | Fam | F | N | Y | A | 45 | 91% | / | / | / | Progressive bilateral sensorineural hearing loss since age 15 | Mother of proband; worsened after streptomycin | | 4 | 1555 | m.1555A\>G | A1555G\-F2 | A1555G\-F2\-III6 | Fam | F | N | Y | A | 20 | 94% | / | / | / | Progressive hearing loss since age 10 | Sister of proband | | 5 | 1555 | m.1555A\>G | A1555G\-F2 | A1555G\-F2\-III2 | Fam | F | N | Y | A | ND | \>99% | / | / | / | Clinically deaf | homoplasmic/\>99% A1555G by Figure 2 | | 6 | 1555 | m.1555A\>G | A1555G\-F3 | A1555G\-F3\-III7 | Fam | M | Y | Y | A | 30 | Homo | / | / | / | Profound hearing impairment | Original carrier ID: BJ101\-III7; aminoglycoside\-induced | | 7 | 1555 | m.1555A\>G | A1555G\-F4 | A1555G\-F4\-III4 | Fam | F | Y | Y | A | 27 | Homo | / | / | / | Severe hearing impairment | Original carrier ID: BJ102\-III4; without aminoglycoside history | | 8 | 1555 | m.1555A\>G | A1555G\-F5 | A1555G\-F5\-III9 | Fam | M | Y | Y | A | 22 | Homo | / | / | / | Severe hearing impairment | Original carrier ID: BJ103\-III9; aminoglycoside\-induced | | 9 | 1555 | m.1555A\>G | A1555G\-F6 | A1555G\-F6\-III7 | Fam | F | Y | Y | A | 23 | Homo | / | / | / | Profound hearing impairment | Original carrier ID: BJ104\-III7; aminoglycoside\-induced | | 10 | 1555 | m.1555A\>G | A1555G\-F7 | A1555G\-F7\-III2 | Fam | F | Y | Y | A | 18 | Homo | / | / | / | Severe bilateral sensorineural hearing impairment; PTA about 80/86 dB; slope audiogram | Original carrier ID: BJ105\-III\-2; after streptomycin exposure at age 17 for tuberculosis | | 11 | 1555 | m.1555A\>G | A1555G\-F8 | A1555G\-F8\-III9 | Fam | M | Y | Y | A | 41 | Homo | / | / | / | Sensorineural hearing impairment since age 15; PTA about 72/70 dB; slope audiogram | Original carrier ID: BJ106\-III\-9; no aminoglycoside exposure reported | | 12 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-III3 | Fam | F | Y | Y | A | 26 | Homo | / | / | / | Nonsyndromic hearing impairment | Carrying G7444A; Aminoglycoside\-induced | | 13 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III5 | Fam | M | Y | Y | A | 48 | Homo | / | / | / | Severe hearing loss | Original carrier ID: BJ107\-III5; after streptomycin exposure | | 14 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-III13 | Fam | F | Y | Y | A | 34 | Homo | / | / | / | Severe hearing loss | Original carrier ID: BJ108\-III13; after streptomycin exposure | | 15 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-III7 | Fam | F | Y | Y | A | 21 | Homo | / | / | / | Profound hearing loss | Original carrier ID: BJ109\-III7; after gentamycin exposure | | 16 | 1555 | m.1555A\>G | A1555G\-F13 | A1555G\-F13\-IV1 | Fam | M | Y | Y | A | 12 | Homo | / | / | / | Severe hearing impairment | Original carrier ID: WZD1\-IV1; after streptomycin/gentamycin at age 10 months | | 17 | 1555 | m.1555A\>G | A1555G\-F14 | A1555G\-F14\-IV1 | Fam | M | Y | Y | A | 13 | Homo | / | / | / | Severe hearing loss | Original carrier ID: WZD2\-IV1; after streptomycin at age 8 months | | 18 | 1555 | m.1555A\>G | A1555G\-F15 | A1555G\-F15\-III1 | Fam | F | Y | Y | A | 27 | Homo | / | / | / | Severe hearing loss | Original carrier ID: WZD3\-III1; after gentamycin at age 2 years | | 19 | 1555 | m.1555A\>G | A1555G\-F16 | A1555G\-F16\-III1 | Fam | M | Y | Y | A | 21 | Homo | / | / | / | Severe hearing loss | Original carrier ID: WZD4\-III1; after streptomycin/gentamycin at age 2 years | | 20 | 1555 | m.1555A\>G | A1555G\-F17 | A1555G\-F17\-III1 | Fam | M | Y | Y | A | 18 | Homo | / | / | / | Profound hearing loss | Original carrier ID: WZD5\-III1; after gentamycin at age 18 months | | 21 | 1555 | m.1555A\>G | A1555G\-F18 | A1555G\-F18\-III1 | Fam | F | Y | Y | A | 19 | Homo | / | / | / | Profound hearing loss | Original carrier ID: WZD6\-III1; after streptomycin/gentamycin at age 3 years | | 22 | 1555 | m.1555A\>G | A1555G\-F19 | A1555G\-F19\-IV1 | Fam | M | Y | Y | A | 19 | Homo | / | / | / | Profound hearing loss | Original carrier ID: WZD7\-IV1; after streptomycin at age two weeks | | 23 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-IV1 | Fam | F | Y | Y | A | 17 | Homo | / | / | / | Moderate bilateral sensorineural hearing impairment | Original carrier ID: WZD11\-IV1; Carrying G11696A | | 24 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III1 | Fam | M | Y | Y | A | ND | Homo | / | / | / | Profound bilateral hearing impairment; PTA about 98/98 dB | Original carrier ID: WZD8\-III\-1; aminoglycoside exposure | | 25 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-IV1 | Fam | M | Y | Y | A | 22 | Homo | / | / | / | Profound bilateral hearing impairment; PTA about 97/100 dB | Original carrier ID: WZD9\-IV\-1; aminoglycoside exposure | | 26 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-III2 | Fam | F | Y | Y | A | 27 | Homo | / | / | / | severe hearing impairment; PTA about 67/82 dB | Original carrier ID: WZD10\-III\-2 | | 27 | 1555 | m.1555A\>G | A1555G\-F25 | A1555G\-F25\-III10 | Fam | M | Y | Y | A | ND | Homo | / | / | / | LHON | Carrying G11778A; with G11778A | | 28 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III1 | Fam | F | Y | Y | A | 34 | Homo | / | / | / | Profound bilateral hearing impairment since age 8 | | | 29 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-III2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | | | 30 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-IV2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | | | 31 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-IV4 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | mother of V\-2 | | 32 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-IV6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | | | 33 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-IV7 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | | | 34 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-V1 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | | | 35 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-V2 | Fam | F | Y | Y | A | 23 | Homo | Homo | / | / | Leigh syndrome and sensorineural hearing loss | | | 36 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-V3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 37 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-V4 | Fam | M | N | Y | A | 19 | Homo | / | / | Homo(F) | Leigh syndrome and sensorineural hearing loss | | | 38 | 1555 | m.1555A\>G | A1555G\-F29 | A1555G\-F29\-V5 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 39 | 1555 | m.1555A\>G | A1555G\-F30 | A1555G\-F30\-P1 | Fam | ND | ND | Y | A | ND | / | / | / | Homo(ND) | Hearing loss | Representative pedigree Family 1; mutation\-positive aggregate includes deaf and reportedly normal members | | 40 | 1555 | m.1555A\>G | A1555G\-F31 | A1555G\-F31\-II2 | Fam | F | ND | N | A | ND | / | / | / | Homo(ND) | Healthy | normal senile hearing deficiency | | 41 | 1555 | m.1555A\>G | A1555G\-F31 | A1555G\-F31\-III4 | Fam | F | ND | Y | A | ND | / | / | / | Homo(ND) | Deafness developed at age 17\. | | | 42 | 1555 | m.1555A\>G | A1555G\-F31 | A1555G\-F31\-IV5 | Fam | F | ND | Y | A | ND | / | / | / | Homo(ND) | Congenital deafness; born with jaundice and mentally retarded. | | | 43 | 1555 | m.1555A\>G | A1555G\-F31 | A1555G\-F31\-V1 | Fam | F | ND | N | A | ND | / | / | / | Homo(ND) | Healthy | | | 44 | 1555 | m.1555A\>G | A1555G\-F32 | A1555G\-F32\-II1 | Fam | ND | ND | Y | A | 20\-40 | / | / | / | Homo(lymphoblastoid cell line) | Ten with congenital, severe\-to\-profound or other hearing loss | maternally related members | | 45 | 1555 | m.1555A\>G | A1555G\-F32 | A1555G\-F32\-II2 | Fam | ND | ND | N | A | 20\-60 | / | / | / | Homo(lymphoblastoid cell line) | Healthy | | | 46 | 1555 | m.1555A\>G | A1555G\-F33 | A1555G\-F33\-P1 | Uninf | ND | ND | Y | A | ND | Positive | / | / | / | Sensorineural hearing loss | A1555G\-positive patients in Greek sensorineural hearing\-loss cohort; Two | | 47 | 1555 | m.1555A\>G | A1555G\-F34 | A1555G\-F34\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S1 | | 48 | 1555 | m.1555A\>G | A1555G\-F35 | A1555G\-F35\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S2 | | 49 | 1555 | m.1555A\>G | A1555G\-F36 | A1555G\-F36\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S3 | | 50 | 1555 | m.1555A\>G | A1555G\-F37 | A1555G\-F37\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S4 | | 51 | 1555 | m.1555A\>G | A1555G\-F38 | A1555G\-F38\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S5 | | 52 | 1555 | m.1555A\>G | A1555G\-F39 | A1555G\-F39\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S6 | | 53 | 1555 | m.1555A\>G | A1555G\-F40 | A1555G\-F40\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S7 | | 54 | 1555 | m.1555A\>G | A1555G\-F41 | A1555G\-F41\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S8 | | 55 | 1555 | m.1555A\>G | A1555G\-F42 | A1555G\-F42\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S9 | | 56 | 1555 | m.1555A\>G | A1555G\-F43 | A1555G\-F43\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S10 | | 57 | 1555 | m.1555A\>G | A1555G\-F44 | A1555G\-F44\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S11 | | 58 | 1555 | m.1555A\>G | A1555G\-F45 | A1555G\-F45\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S12 | | 59 | 1555 | m.1555A\>G | A1555G\-F46 | A1555G\-F46\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S13 | | 60 | 1555 | m.1555A\>G | A1555G\-F47 | A1555G\-F47\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S14 | | 61 | 1555 | m.1555A\>G | A1555G\-F48 | A1555G\-F48\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S15 | | 62 | 1555 | m.1555A\>G | A1555G\-F49 | A1555G\-F49\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S16 | | 63 | 1555 | m.1555A\>G | A1555G\-F50 | A1555G\-F50\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S17 | | 64 | 1555 | m.1555A\>G | A1555G\-F51 | A1555G\-F51\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S18 | | 65 | 1555 | m.1555A\>G | A1555G\-F52 | A1555G\-F52\-P1 | Fam | ND | ND | Y | A | ND | Homo | / | / | / | Progressive sensorineural deafness | Spanish pedigree S19 | | 66 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-I1 | Uninf | F | N | Y | A | 73 | 70\.60% | / | / | / | Moderate bilateral sensorineural deafness | | | 67 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II1 | Fam | F | N | Y | A | 53 | 97\.90% | / | / | / | Severe mixed type deafness | | | 68 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II3 | Fam | F | N | N | A | 48 | 85\.30% | / | / | / | Healthy | | | 69 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II5 | Fam | F | N | Y | A | 44 | 70\.70% | / | / | / | Mild bilateral sensorineural deafness | | | 70 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II7 | Fam | F | N | Y | A | 41 | 49\.80% | / | / | / | High\-frequency hearing loss | | | 71 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II9 | Fam | F | N | Y | A | 38 | 49\.60% | / | / | / | High\-frequency hearing loss | | | 72 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-II11 | Fam | F | N | Y | A | 35 | 49\.50% | / | / | / | High\-frequency hearing loss | | | 73 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III1 | Fam | F | Y | Y | A | 28 | Homo | / | / | / | Profound mixed type deafness | | | 74 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III2 | Fam | M | N | Y | A | 26 | Homo | / | / | / | Moderate bilateral sensorineural deafness | | | 75 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III3 | Fam | M | N | Y | A | 24 | Homo | / | / | / | Severe mixed type deafness | | | 76 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III4 | Fam | M | N | Y | A | 20 | Homo | / | / | / | Severe mixed type deafness | | | 77 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III5 | Fam | F | N | N | A | 26 | Homo | / | / | / | Healthy | | | 78 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III6 | Fam | F | N | Y | A | 20 | Homo | / | / | / | Severe mixed type deafness | | | 79 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III7 | Fam | F | N | Y | A | 16 | Homo | / | / | / | Profound mixed type deafness | | | 80 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III8 | Fam | F | N | Y | A | 13 | Homo | / | / | / | Moderate bilateral sensorineural deafness | | | 81 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III9 | Fam | M | N | N | A | 11 | 14\.80% | / | / | / | Healthy | | | 82 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III10 | Fam | F | N | N | A | 4 | 11\.90% | / | / | / | Healthy | | | 83 | 1555 | m.1555A\>G | A1555G\-F27 | A1555G\-F27\-III11 | Fam | M | N | N | A | 9 | 19\.40% | / | / | / | Healthy | | | 84 | 1555 | m.1555A\>G | A1555G\-F53 | A1555G\-F53\-II1 | Fam | F | Y | Y | ND | ND | / | / | / | Homo(ND) | Hearing loss | Original carrier ID: A1555G\-subject\-1; aminoglycoside exposure reported | | 85 | 1555 | m.1555A\>G | A1555G\-F54 | A1555G\-F54\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | Homo(ND) | Hearing loss | Original carrier ID: A1555G\-subject\-2; aminoglycoside exposure unknown | | 86 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-II3 | Fam | M | N | Y | ND | 55 years | Positive | / | / | / | profound hearing loss; onset 50 years; PTA left/right 90/94 | | | 87 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-II8 | Fam | F | N | Y | ND | 63 years | Positive | / | / | / | profound hearing loss; onset 53 years; PTA left/right 96/93 | | | 88 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-II10 | Fam | F | N | Y | ND | 60 years | Positive | / | / | / | profound hearing loss; onset 58 years; PTA left/right 78/115 | | | 89 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-III7 | Fam | M | N | Y | ND | 35 years | Positive | / | / | / | profound hearing loss; onset 30 years; PTA left/right 78/91 | | | 90 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-III10 | Fam | F | N | Y | ND | 30 years | Positive | / | / | / | profound hearing loss; onset 21 years; PTA left/right 99/100 | Carrying A4317G | | 91 | 1555 | m.1555A\>G | A1555G\-F28 | A1555G\-F28\-III12 | Fam | F | Y | Y | ND | 28 years | Positive | / | / | / | Profound hearing loss; PTA left/right 108/108 | onset 1 year; gentamycin exposure reported at age 15 with irreversible impairment two weeks later | | 92 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-III12 | Fam | M | N | Y | ND | ND | Homo | / | / | / | prelingual nonsyndromic hearing loss; poor speech | | | 93 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-III14 | Fam | M | N | Y | ND | ND | Homo | / | / | / | prelingual nonsyndromic hearing loss; poor speech | | | 94 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-IV5 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Severe high\-frequency hearing loss | aminoglycoside injection history | | 95 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-IV7 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Severe high\-frequency hearing loss | aminoglycoside injection history | | 96 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-IV15 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Severe high\-frequency hearing loss | | | 97 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-V3 | Fam | M | Y | Y | ND | ND | Homo | / | / | / | Total hearing loss | after aminoglycoside injection | | 98 | 1555 | m.1555A\>G | A1555G\-F56 | A1555G\-F56\-III2 | Fam | F | Y | Y | A | 35 | 57% | 55% | / | 60%(F); 89%(heart) | Restrictive cardiomyopathy with heart failure | | | 99 | 1555 | m.1555A\>G | A1555G\-F56 | A1555G\-F56\-IV1 | Fam | F | N | N | A | 11 | 95% | / | / | / | Healthy | | | 100 | 1555 | m.1555A\>G | A1555G\-F56 | A1555G\-F56\-IV2 | Fam | F | N | N | A | 7 | 50% | / | / | / | Healthy | | | 101 | 1555 | m.1555A\>G | A1555G\-F57 | A1555G\-F57\-P1 | Uninf | ND | Y | Y | ND | 13\-21 years cohort range; individual age ND | Homo | / | / | / | Sporadic severe/profound deafness | aminoglycoside\-induced; no family history reported | | 102 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-II1 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 103 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-II6 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 104 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-II7 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 105 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-II8 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 106 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-II9 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 107 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III1 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 108 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III41 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 109 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III42 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 110 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III43 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 111 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III45 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 112 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III47 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 113 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III49 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 114 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III50 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 115 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III51 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Deaf | after streptomycin therapy | | 116 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-IV4 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 117 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-IV9 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 118 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-IV10 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 119 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-IV11 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 120 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III52 | Fam | M | ND | Y | ND | ND | / | / | / | / | Deaf | after streptomycin therapy | | 121 | 1555 | m.1555A\>G | A1555G\-F58 | A1555G\-F58\-III53 | Fam | M | ND | Y | ND | ND | / | / | / | / | Deaf | after streptomycin therapy | | 122 | 1555 | m.1555A\>G | A1555G\-F59 | A1555G\-F59\-P1 | Fam | F | Y | Y | ND | diagnosed 9 months | Homo | / | / | / | Bilateral profound hearing loss | Original carrier ID: Family\-1\-proband; after gentamycin overdose at 15 days | | 123 | 1555 | m.1555A\>G | A1555G\-F60 | A1555G\-F60\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Nonsyndromic hearing loss | Original carrier ID: Family\-2\-proband; maternally inherited; Family 2 | | 124 | 1555 | m.1555A\>G | A1555G\-F61 | A1555G\-F61\-II1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Noted as aminoglycoside\-deafness associated | Original carrier ID: S2; abnormal fatigue with mitochondrial abnormalities; A1555G | | 125 | 1555 | m.1555A\>G | A1555G\-F61 | A1555G\-F61\-I2 | Uninf | F | N | ND | ND | ND | Homo | / | / | / | ND | Original carrier ID: S2; phenotype not described in extracted text | | 126 | 1555 | m.1555A\>G | A1555G\-F62 | A1555G\-F62\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-1\-sampled\-subject; with A1555G | | 127 | 1555 | m.1555A\>G | A1555G\-F360 | A1555G\-F360\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-2\-sampled\-subject; with A1555G | | 128 | 1555 | m.1555A\>G | A1555G\-F361 | A1555G\-F361\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-3\-sampled\-subject; with A1555G | | 129 | 1555 | m.1555A\>G | A1555G\-F362 | A1555G\-F362\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-4\-sampled\-subject; with A1555G | | 130 | 1555 | m.1555A\>G | A1555G\-F363 | A1555G\-F363\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-5\-sampled\-subject; with A1555G | | 131 | 1555 | m.1555A\>G | A1555G\-F364 | A1555G\-F364\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-6\-sampled\-subject; with A1555G | | 132 | 1555 | m.1555A\>G | A1555G\-F365 | A1555G\-F365\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-7\-sampled\-subject; with A1555G | | 133 | 1555 | m.1555A\>G | A1555G\-F366 | A1555G\-F366\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-8\-sampled\-subject; with A1555G | | 134 | 1555 | m.1555A\>G | A1555G\-F367 | A1555G\-F367\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-9\-sampled\-subject; with A1555G | | 135 | 1555 | m.1555A\>G | A1555G\-F368 | A1555G\-F368\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-10\-sampled\-subject; with A1555G | | 136 | 1555 | m.1555A\>G | A1555G\-F369 | A1555G\-F369\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-11\-sampled\-subject; with A1555G | | 137 | 1555 | m.1555A\>G | A1555G\-F370 | A1555G\-F370\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-12\-sampled\-subject; with A1555G | | 138 | 1555 | m.1555A\>G | A1555G\-F371 | A1555G\-F371\-P1 | Fam | ND | ND | Y | ND | ND | Positive | / | / | / | Sensorineural hearing loss associated | Original carrier ID: Family\-13\-sampled\-subject; with A1555G | | 139 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III3 | Fam | M | Y | Y | A | 22 years; onset 2 years | Homo | / | / | / | Extremely severe hearing loss; PTA L95/R104; sloping audiogram | Carrying A4317G; proband; aminoglycoside history; wears hearing aid | | 140 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-I2 | Uninf | F | N | Y | A | 74 years; onset 40 years | Homo | / | / | / | severe hearing loss; PTA L81/R85; flat audiogram | Carrying A4317G | | 141 | 1555 | m.1555A\>G | A1555G\-F64 | A1555G\-F64\-P1 | Fam | M | Y | Y | ND | 2 (onset after streptomycin; evaluation age not stated) | Homo | / | / | / | Became deaf at age 2 years | after streptomycin | | 142 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-V1 | Fam | F | N | N | ND | 11 | / | / | / | / | Healthy | | | 143 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-V2 | Fam | F | N | N | ND | 18 | / | / | / | / | Healthy | | | 144 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-V3 | Fam | M | Y | Y | ND | 14 | / | / | / | / | Congenital profound deafness | Aminoglycoside exposure reported | | 145 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-V4 | Fam | M | N | Y | ND | 11 | / | / | / | / | Congenital profound deafness | | | 146 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-V5 | Fam | M | N | Y | ND | 16 | / | / | / | / | Unilateral deafness in high frequency | | | 147 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-III1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 3 | | 148 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-III6 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 2 | | 149 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-I2 | Uninf | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 150 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-II4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 151 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-II9 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 152 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-IV2 | Fam | F | Y | Y | ND | 17 | Homo | / | / | / | Severe hearing loss | after gentamycin exposure at age 2 | | 153 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-III2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | after aminoglycoside exposure | | 154 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-III5 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | after aminoglycoside exposure | | 155 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-III7 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure | | 156 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 157 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-II4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 158 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-V1 | Fam | M | Y | Y | ND | 12 | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 2 | | 159 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | after gentamycin exposure at age 10 | | 160 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure | | 161 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV15 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure | | 162 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV17 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure | | 163 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-II2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 164 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 165 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III8 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 166 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 167 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 168 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 169 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV5 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 170 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV9 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 171 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV14 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure | | 172 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III2 | Fam | M | Y | Y | ND | 15 | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 4 | | 173 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure | | 174 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 175 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 176 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III5 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 177 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III12 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure | | 178 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II6 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure | | 179 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III7 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure | | 180 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III14 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure | | 181 | 1555 | m.1555A\>G | A1555G\-F70 | A1555G\-F70\-III3 | Fam | M | N | Y | ND | 21 | / | / | / | / | Profound hearing loss | no aminoglycoside exposure reported | | 182 | 1555 | m.1555A\>G | A1555G\-F70 | A1555G\-F70\-III5 | Fam | F | Y | Y | ND | 25 | / | / | / | / | Profound hearing loss | aminoglycoside exposure at age 10 | | 183 | 1555 | m.1555A\>G | A1555G\-F71 | A1555G\-F71\-II10 | Fam | F | N | Y | ND | 77 | / | / | / | / | Severe hearing loss | aminoglycoside exposure reported | | 184 | 1555 | m.1555A\>G | A1555G\-F71 | A1555G\-F71\-III9 | Fam | F | Y | Y | ND | 41 | / | / | / | / | Profound hearing loss | aminoglycoside exposure reported | | 185 | 1555 | m.1555A\>G | A1555G\-F71 | A1555G\-F71\-IV4 | Fam | F | N | Y | ND | 16 | / | / | / | / | Severe hearing loss | no aminoglycoside exposure reported | | 186 | 1555 | m.1555A\>G | A1555G\-F72 | A1555G\-F72\-P1 | Fam | M | Y | Y | ND | 3 (onset age) | / | / | / | / | Hearing loss, PTA R73/L80 | Original carrier ID: G104; gentamicin exposure | | 187 | 1555 | m.1555A\>G | A1555G\-F73 | A1555G\-F73\-III4 | Fam | M | Y | Y | ND | 17 | Homo | / | / | / | Profound HL; PTA 95/95 | gentamicin at age 6 | | 188 | 1555 | m.1555A\>G | A1555G\-F74 | A1555G\-F74\-III3 | Fam | M | Y | Y | ND | 22 | Homo | / | / | / | Profound HL; PTA 95/95 | streptomycin/kanamycin at age 2 | | 189 | 1555 | m.1555A\>G | A1555G\-F75 | A1555G\-F75\-III6 | Fam | F | Y | Y | ND | 19 | Homo | / | / | / | Profound HL; PTA 100/100 | gentamicin at age 1 | | 190 | 1555 | m.1555A\>G | A1555G\-F76 | A1555G\-F76\-III3 | Fam | M | Y | Y | ND | 17 | Homo | / | / | / | Profound HL; PTA 95/95 | gentamicin/streptomycin at age 3 | | 191 | 1555 | m.1555A\>G | A1555G\-F77 | A1555G\-F77\-III5 | Fam | M | Y | Y | ND | 17 | Homo | / | / | / | Severe HL; PTA 90/86 | streptomycin at age 1\.5 | | 192 | 1555 | m.1555A\>G | A1555G\-F78 | A1555G\-F78\-III17 | Fam | M | Y | Y | ND | 15 | Homo | / | / | / | Profound HL; PTA 90/100 | gentamicin at age 1\.5 | | 193 | 1555 | m.1555A\>G | A1555G\-F311 | A1555G\-F311\-III10 | Fam | F | Y | Y | ND | 16 | Homo | / | / | / | Profound HL; PTA 93/93 | gentamicin at age 3 | | 194 | 1555 | m.1555A\>G | A1555G\-F312 | A1555G\-F312\-III14 | Fam | M | Y | Y | ND | 8 | Homo | / | / | / | Profound HL; PTA 100/100 | gentamicin at age 1 | | 195 | 1555 | m.1555A\>G | A1555G\-F313 | A1555G\-F313\-IV4 | Fam | M | Y | Y | ND | 12 | Homo | / | / | / | Severe HL; PTA 85/85 | gentamicin/kanamycin at age 1 | | 196 | 1555 | m.1555A\>G | A1555G\-F314 | A1555G\-F314\-III5 | Fam | M | Y | Y | ND | 18 | Homo | / | / | / | Severe HL; PTA 75/78 | gentamicin/streptomycin at age 1 | | 197 | 1555 | m.1555A\>G | A1555G\-F315 | A1555G\-F315\-III12 | Fam | M | Y | Y | ND | 19 | Homo | / | / | / | Severe HL; PTA 85/89 | streptomycin at age 2 | | 198 | 1555 | m.1555A\>G | A1555G\-F316 | A1555G\-F316\-III3 | Fam | M | Y | Y | ND | 13 | Homo | / | / | / | Severe HL; PTA 85/85 | gentamicin at age 1\.5 | | 199 | 1555 | m.1555A\>G | A1555G\-F317 | A1555G\-F317\-III1 | Fam | M | Y | Y | ND | 12 | Homo | / | / | / | Severe HL; PTA 85/90 | gentamicin at age 1\.5 | | 200 | 1555 | m.1555A\>G | A1555G\-F318 | A1555G\-F318\-III9 | Fam | M | Y | Y | ND | 18 | Homo | / | / | / | Profound HL; PTA 100/100 | gentamicin/kanamycin/streptomycin at age 1 | | 201 | 1555 | m.1555A\>G | A1555G\-F319 | A1555G\-F319\-IV10 | Fam | F | Y | Y | ND | 15 | Homo | / | / | / | Profound HL; PTA 100/100 | gentamicin/streptomycin at age 9 | | 202 | 1555 | m.1555A\>G | A1555G\-F320 | A1555G\-F320\-III11 | Fam | M | Y | Y | ND | 24 | Homo | / | / | / | Profound HL; PTA 92/91 | gentamicin/streptomycin at age 1 | | 203 | 1555 | m.1555A\>G | A1555G\-F79 | A1555G\-F79\-P1 | Fam | F | N | ND | ND | 69 | / | / | / | Homo(F) | ND | Original carrier ID: 95\-119; aminoglycoside\-induced; Mother of a Japanese deafness patient; donor's own hearing phenotype not reported | | 204 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-III1 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Hearing\-impaired | apparently homoplasmic A1555G in blood | | 205 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-IV5 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Hearing\-impaired | Fig. 1 symbol appears open/unaffected; in Results text | | 206 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-II3 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 207 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-III2 | Fam | M | ND | Y | ND | ND | / | / | / | / | Moderate hearing loss | affected child of confirmed\-positive mother II\-3 | | 208 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-III4 | Fam | M | ND | Y | ND | ND | / | / | / | / | Profound hearing loss | affected child of confirmed\-positive mother II\-3 | | 209 | 1555 | m.1555A\>G | A1555G\-F80 | A1555G\-F80\-III6 | Fam | F | ND | Y | ND | ND | / | / | / | / | Moderate hearing loss | affected child of confirmed\-positive mother II\-3 | | 210 | 1555 | m.1555A\>G | A1555G\-F81 | A1555G\-F81\-V13 | Fam | F | Y | Y | ND | 3 (onset age) | Homo | / | / | / | Profound hearing loss from age 3 | Proposita; apparently homoplasmic A1555G in blood | | 211 | 1555 | m.1555A\>G | A1555G\-F81 | A1555G\-F81\-V11 | Fam | F | ND | Y | ND | 18 (onset/exposure) | Homo | / | / | / | Profound hearing loss | after streptomycin exposure; apparently homoplasmic A1555G in blood | | 212 | 1555 | m.1555A\>G | A1555G\-F82 | A1555G\-F82\-I1 | Uninf | F | N | Y | ND | ND | Homo | / | / | / | Mild high\-frequency hearing impairment | No aminoglycoside exposure | | 213 | 1555 | m.1555A\>G | A1555G\-F82 | A1555G\-F82\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Mild to moderate high\-frequency hearing impairment | No aminoglycoside exposure | | 214 | 1555 | m.1555A\>G | A1555G\-F82 | A1555G\-F82\-II3 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Moderate bilateral hearing impairment | No aminoglycoside exposure | | 215 | 1555 | m.1555A\>G | A1555G\-F82 | A1555G\-F82\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | No aminoglycoside exposure | | 216 | 1555 | m.1555A\>G | A1555G\-F85 | A1555G\-F85\-P1 | Uninf | F | Y | Y | ND | 75 | / | / | / | / | SNHL; PTA R68\.3/L85\.0 | streptomycin exposure; onset age 37 | | 217 | 1555 | m.1555A\>G | A1555G\-F86 | A1555G\-F86\-P1 | Fam | F | Y | Y | ND | 47 | / | / | / | / | SNHL; PTA R65/L65 | streptomycin exposure; onset age 5 | | 218 | 1555 | m.1555A\>G | A1555G\-F87 | A1555G\-F87\-P1 | Fam | F | Y | Y | ND | 35 | / | / | / | / | SNHL; PTA R56\.7/L45\.0 | no aminoglycoside exposure; onset age 34 | | 219 | 1555 | m.1555A\>G | A1555G\-F88 | A1555G\-F88\-P1 | Fam | M | Y | Y | ND | 44 | / | / | / | / | SNHL; PTA R81\.7/L73\.3 | no aminoglycoside exposure; onset age 0 | | 220 | 1555 | m.1555A\>G | A1555G\-F89 | A1555G\-F89\-P1 | Fam | M | Y | Y | ND | 39 | / | / | / | / | SNHL; PTA R50/L41\.7 | no aminoglycoside exposure; onset age 10 | | 221 | 1555 | m.1555A\>G | A1555G\-F90 | A1555G\-F90\-P1 | Fam | F | Y | Y | ND | 37 | / | / | / | / | SNHL; PTA R91\.7/L90\.0 | no aminoglycoside exposure; onset age 6 | | 222 | 1555 | m.1555A\>G | A1555G\-F91 | A1555G\-F91\-P1 | Fam | F | Y | Y | ND | 37 | / | / | / | / | SNHL; PTA R58\.3/L53\.3 | no aminoglycoside exposure; onset age 20 | | 223 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III7 | Fam | ND | ND | ND | ND | ND | Homo | / | / | / | ND | aminoglycoside exposure; matrilineal relative; severity not individually extracted; aminoglycoside: Yes | | 224 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III11 | Fam | ND | ND | ND | ND | ND | Homo | / | / | / | ND | aminoglycoside exposure; matrilineal relative; duplicate OCR mention possible; aminoglycoside: Yes | | 225 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-III1 | Fam | ND | ND | Y | ND | ND | Homo | / | / | / | Profound hearing loss | aminoglycoside: Yes | | 226 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-III3 | Fam | ND | ND | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 227 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-III8 | Fam | ND | ND | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 228 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-II2 | Fam | ND | ND | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | aminoglycoside: No reported exposure | | 229 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-IV2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | sister of proband; aminoglycoside: Yes; gentamicin at age 1 | | 230 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-II6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 231 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-II9 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 232 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III14 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 233 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III15 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | aminoglycoside: No reported exposure | | 234 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III10 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | explicitly tested affected female; aminoglycoside: No reported exposure | | 235 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III17 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | aminoglycoside: No reported exposure | | 236 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III11 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | aminoglycoside: No reported exposure | | 237 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III7 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | explicitly tested; aminoglycoside: No reported exposure | | 238 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II2 | Fam | F | ND | N | ND | 46 | Homo | / | / | / | Healthy | | | 239 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II3 | Fam | F | ND | N | ND | 45 | Homo | / | / | / | Healthy | | | 240 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II5 | Fam | M | ND | N | ND | 39 | Homo | / | / | / | Healthy | | | 241 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II6 | Fam | M | ND | N | ND | 37 | Homo | / | / | / | Healthy | | | 242 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II8 | Fam | F | ND | Y | ND | 59 | Homo | / | / | / | Profound hearing loss | explicitly tested affected female; aminoglycoside: No | | 243 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-II10 | Fam | M | ND | N | ND | 45 | Homo | / | / | / | Healthy | | | 244 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III4 | Fam | M | ND | N | ND | 28 | Homo | / | / | / | Healthy | | | 245 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III9 | Fam | M | ND | Y | ND | 38 | Homo | / | / | / | Profound hearing loss | aminoglycoside: Yes | | 246 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III11 | Fam | F | ND | Y | ND | 36 | Homo | / | / | / | Profound hearing loss | aminoglycoside: Yes | | 247 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III12 | Fam | F | ND | Y | ND | 31 | Homo | / | / | / | Severe hearing loss | aminoglycoside: No | | 248 | 1555 | m.1555A\>G | A1555G\-F92 | A1555G\-F92\-III7 | Fam | F | Y | Y | ND | 38 | Homo | / | / | / | Bilateral hearing loss; PTA right 106 dB / left 86 dB; profound sloping hearing impairment | Female; proband; after gentamycin at age 19 | | 249 | 1555 | m.1555A\>G | A1555G\-F92 | A1555G\-F92\-II8 | Fam | F | N | Y | ND | 66 | Homo | / | / | / | PTA right 105 dB / left 70 dB; profound sloping hearing impairment | Affected female; onset age 31; gentamycin exposure | | 250 | 1555 | m.1555A\>G | A1555G\-F92 | A1555G\-F92\-III4 | Fam | F | N | Y | ND | 42 | Homo | / | / | / | PTA right 21 dB / left 92 dB; severe sloping hearing impairment | Affected female; onset age 20; kanamycin exposure | | 251 | 1555 | m.1555A\>G | A1555G\-F93 | A1555G\-F93\-III7 | Fam | F | Y | Y | ND | 25 | Homo | / | / | / | PTA right 85 dB / left 71 dB; severe sloping hearing impairment | Female proband; onset age 24; no aminoglycoside exposure reported; also carries m.4394C\>T | | 252 | 1555 | m.1555A\>G | A1555G\-F93 | A1555G\-F93\-II6 | Fam | F | N | Y | ND | 50 | Homo | / | / | / | PTA right 30 dB / left 30 dB; mild flat hearing impairment | Affected female; onset age 45; no aminoglycoside exposure reported; also carries m.4394C\>T | | 253 | 1555 | m.1555A\>G | A1555G\-F93 | A1555G\-F93\-II8 | Fam | F | N | Y | ND | 49 | Homo | / | / | / | PTA right 54 dB / left 57 dB; moderate sloping hearing impairment | Affected female; onset age 22; gentamycin exposure; also carries m.4394C\>T | | 254 | 1555 | m.1555A\>G | A1555G\-F94 | A1555G\-F94\-P1 | Fam | F | Y | Y | A | 24 | / | / | / | / | Moderate to severe sensorineural hearing loss; normal vestibular testing | Original carrier ID: 24\-year\-old\-Chinese\-woman; Streptomycin exposure; after short\-term streptomycin exposure; reported A1555G; tissue/load not stated | | 255 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II1 | Fam | M | ND | N | A | 32 | 9\.57% | / | / | / | Healthy | | | 256 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II2 | Fam | F | ND | N | A | 31 | 9\.31% | / | / | / | Healthy | | | 257 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-I2 | Uninf | F | ND | Y | A | 66 | 61\.03% | / | / | / | Sensorineural hearing loss affecting all frequencies | streptomycin exposure; history of | | 258 | 1555 | m.1555A\>G | A1555G\-F96 | A1555G\-F96\-II1 | Fam | M | ND | Y | A | 24 | 70\.93% | / | / | / | Mild high\-frequency sensorineural hearing loss | monozygotic twin; no reported aminoglycoside exposure | | 259 | 1555 | m.1555A\>G | A1555G\-F96 | A1555G\-F96\-II2 | Fam | M | ND | Y | A | 24 | 64\.48% | / | / | / | Mild high\-frequency sensorineural hearing loss | monozygotic twin; no reported aminoglycoside exposure | | 260 | 1555 | m.1555A\>G | A1555G\-F96 | A1555G\-F96\-II3 | Fam | M | ND | Y | A | 20 | 91\.76% | / | / | / | Sloping hearing loss at medium and high frequencies; bilateral tinnitus | no reported aminoglycoside exposure | | 261 | 1555 | m.1555A\>G | A1555G\-F96 | A1555G\-F96\-I2 | Uninf | F | ND | Y | A | 45 | 70\.00% | / | / | / | Sensorineural hearing loss affecting all frequencies; positional vertigo | no reported aminoglycoside exposure | | 262 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II1 | Fam | F | ND | Y | A | 29 | 94\.74% | / | / | / | Sloping hearing loss at medium and high frequencies | no reported aminoglycoside exposure | | 263 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II3 | Fam | F | ND | Y | A | 26 | 96\.15% | / | / | / | Sensorineural hearing loss affecting all frequencies | no reported aminoglycoside exposure | | 264 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-I2 | Uninf | F | ND | Y | A | 55 | 100% | / | / | / | Hearing loss | Homoplasmic carrier with hearing loss; streptomycin exposure; history of | | 265 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II5 | Fam | F | ND | N | A | 19 | 100% | / | / | / | Healthy | | | 266 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II6 | Fam | M | ND | N | A | 18 | 100% | / | / | / | Healthy | | | 267 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-II5 | Fam | F | ND | Y | A | 52 | 87\.30% | / | / | / | Moderate/severe high\-frequency sensorineural hearing loss | no reported aminoglycoside exposure | | 268 | 1555 | m.1555A\>G | A1555G\-F99 | A1555G\-F99\-III2 | Fam | F | ND | N | A | ND | 4\.79% | / | / | / | Healthy | | | 269 | 1555 | m.1555A\>G | A1555G\-F99 | A1555G\-F99\-IV1 | Fam | F | ND | Y | A | 5 | 3\.75% | / | / | / | Mild U\-shaped hearing loss with low\-level | heteroplasmy | | 270 | 1555 | m.1555A\>G | A1555G\-F100 | A1555G\-F100\-II3 | Fam | M | ND | N | A | 30 | 3\.84% | / | / | / | Healthy | | | 271 | 1555 | m.1555A\>G | A1555G\-F100 | A1555G\-F100\-II4 | Fam | F | ND | N | A | 25 | 18\.37% | / | / | / | Healthy | | | 272 | 1555 | m.1555A\>G | A1555G\-F100 | A1555G\-F100\-I2 | Uninf | F | ND | Y | A | 61 | 52\.14% | / | / | / | Sloping hearing loss at medium and high frequencies. | | | 273 | 1555 | m.1555A\>G | A1555G\-F101 | A1555G\-F101\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric nonsyndromic hearing\-loss | Original carrier ID: Subject\-3; subject \#3; no aminoglycoside exposure; co\-occurs with 961insC | | 274 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-I2 | Uninf | F | ND | N | D | 85 | 23\.1% | / | / | / | Healthy | | | 275 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-II1 | Fam | F | ND | Y | A | 55 | 89\.9% | / | / | / | Mild hearing loss; onset unknown | | | 276 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-II4 | Fam | F | ND | N | A | 57 | 90\.2% | / | / | 89\.1%(saliva) | Healthy | | | 277 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-II6 | Fam | F | ND | N | A | 59 | 33\.3% | / | / | 24\.7%(saliva) | Healthy | | | 278 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-IV9 | Fam | M | Y | Y | ND | ND | Homo | / | / | / | LHON | Male proband with | | 279 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-IV8 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 280 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-III7 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Mother | | 281 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-III6 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Symptomatic mutation carrier; uncle | | 282 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-III3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 283 | 1555 | m.1555A\>G | A1555G\-F103 | A1555G\-F103\-III4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 284 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II1 | Fam | F | ND | Y | ND | 21 | Homo | / | / | / | Mild hearing loss; PTA 25/25 | Original carrier ID: 1; onset 8; aminoglycoside: No | | 285 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II2 | Fam | F | ND | Y | ND | 46 | Homo | / | / | / | Profound hearing loss; PTA 110/110 | Original carrier ID: 2; onset 8; aminoglycoside: Streptomycin | | 286 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II3 | Fam | M | ND | Y | ND | 46 | Homo | / | / | / | Severe hearing loss; PTA 75/75 | Original carrier ID: 3; onset 16; aminoglycoside: No | | 287 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II4 | Fam | M | ND | Y | ND | 40 | Homo | / | / | / | Profound hearing loss; PTA 110/116; tinnitus and dizziness | Original carrier ID: 4; onset 9; aminoglycoside: No | | 288 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II5 | Fam | M | ND | Y | ND | 37 | Homo | / | / | / | Moderate hearing loss; PTA 50/61 | Original carrier ID: 5; onset 2; aminoglycoside: No | | 289 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II6 | Fam | M | ND | Y | ND | 25 | Homo | / | / | / | Moderate hearing loss; PTA 44/40; tinnitus | Original carrier ID: 6; onset 10; aminoglycoside: Streptomycin | | 290 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II7 | Fam | F | ND | Y | ND | 36 | Homo | / | / | / | Severe hearing loss; PTA 85/70 | Original carrier ID: 7; onset 3; aminoglycoside: No | | 291 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II8 | Fam | M | ND | Y | ND | 31 | Homo | / | / | / | Moderate hearing loss; PTA 60/60; tinnitus | Original carrier ID: 8; onset 15; aminoglycoside: No | | 292 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II9 | Fam | F | ND | Y | ND | 39 | Homo | / | / | / | Profound hearing loss; PTA 100/100; tinnitus | Original carrier ID: 9; onset 10; aminoglycoside: No | | 293 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II10 | Fam | F | ND | Y | ND | 35 | Homo | / | / | / | Moderate hearing loss; PTA 50/55 | Original carrier ID: 10; onset 13; aminoglycoside: No | | 294 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II11 | Fam | F | ND | Y | ND | 37 | Homo | / | / | / | Severe hearing loss; PTA 80/90 | Original carrier ID: 11; onset 1; aminoglycoside: No | | 295 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II12 | Fam | M | ND | Y | ND | 32 | Homo | / | / | / | Moderate hearing loss; PTA 55/60 | Original carrier ID: 12; onset 1; aminoglycoside: No | | 296 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II13 | Fam | F | ND | Y | ND | 25 | Homo | / | / | / | Severe hearing loss; PTA 90/90 | Original carrier ID: 13; onset 7; aminoglycoside: No | | 297 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II14 | Fam | M | ND | Y | ND | 27 | Homo | / | / | / | Profound hearing loss; PTA 100/100; tinnitus | Original carrier ID: 14; onset 14; aminoglycoside: No | | 298 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II15 | Fam | F | ND | Y | ND | 42 | Homo | / | / | / | Severe hearing loss; PTA 85/80; tinnitus and dizziness | Original carrier ID: 15; onset 10; aminoglycoside: Streptomycin | | 299 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II16 | Fam | F | ND | Y | ND | 46 | Homo | / | / | / | Profound hearing loss; PTA 95/95; tinnitus | Original carrier ID: 16; onset 8; aminoglycoside: No | | 300 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II17 | Fam | F | ND | Y | ND | 27 | Homo | / | / | / | Profound hearing loss; PTA not recorded | Original carrier ID: 17; onset 4; aminoglycoside: Streptomycin | | 301 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II18 | Fam | F | ND | Y | ND | 57 | Homo | / | / | / | Moderate hearing loss; PTA 45/40; tinnitus | Original carrier ID: 18; onset 20; aminoglycoside: No | | 302 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II19 | Fam | F | ND | N | ND | 9 | Homo | / | / | / | Healthy | Original carrier ID: 19 | | 303 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II20 | Fam | F | ND | N | ND | 53 | Homo | / | / | / | Healthy | Original carrier ID: 20 | | 304 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II21 | Fam | M | ND | N | ND | 12 | Homo | / | / | / | Healthy | Original carrier ID: 21 | | 305 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II22 | Fam | F | ND | N | ND | 28 | Homo | / | / | / | Healthy | Original carrier ID: 22 | | 306 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II23 | Fam | F | ND | N | ND | 30 | Homo | / | / | / | Healthy | Original carrier ID: 23 | | 307 | 1555 | m.1555A\>G | A1555G\-F104 | A1555G\-F104\-II24 | Fam | F | ND | N | ND | 30 | Homo | / | / | / | Healthy | Original carrier ID: 24 | | 308 | 1555 | m.1555A\>G | A1555G\-F105 | A1555G\-F105\-P1 | Fam | ND | ND | Y | ND | ND | / | / | / | / | Nonsyndromic hearing loss | Original carrier ID: Patient\-lane\-5; A1555G\-positive patient | | 309 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V10 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Hearing loss | exact age not reported; Proband; in large maternal hearing\-loss family; indicated in Fig. 1/Fig. 2 | | 310 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV19 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | heterozygous GJB2 299\-300delAT without significant hearing loss | | 311 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II1 | Fam | M | Y | Y | ND | 54 | Homo | / | / | / | Moderate isolated slowly progressive SNHL; down\-sloping bilateral audiogram | Original carrier ID: Patient\-1; after streptomycin exposure; onset 27 years | | 312 | 1555 | m.1555A\>G | A1555G\-F108 | A1555G\-F108\-II1 | Fam | F | Y | Y | ND | 34 | Homo | / | / | / | Mild\-to\-moderate isolated slowly progressive SNHL; flat audiogram | Original carrier ID: Patient\-2; onset age 15; no aminoglycoside exposure | | 313 | 1555 | m.1555A\>G | A1555G\-F109 | A1555G\-F109\-II1 | Fam | F | Y | Y | ND | 66 | Homo | / | / | / | Profound bilateral SNHL; bilateral labyrinth areflexia | Original carrier ID: Patient\-3; Aminoglycoside exposure reported; after streptomycin; onset age 24 | | 314 | 1555 | m.1555A\>G | A1555G\-F110 | A1555G\-F110\-II1 | Fam | F | Y | Y | ND | 36 | Homo | / | / | / | Severe bilateral SNHL | Original carrier ID: Patient\-4; Aminoglycoside exposure reported; onset age 18; aminoglycoside exposure; no known | | 315 | 1555 | m.1555A\>G | A1555G\-F111 | A1555G\-F111\-II1 | Fam | F | Y | Y | ND | 40 | Homo | / | / | / | Severe bilateral SNHL | Original carrier ID: Patient\-5; onset age 19; no aminoglycoside exposure | | 316 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II1 | Fam | F | Y | Y | ND | 50 | Homo | / | / | / | Moderate bilateral down\-sloping SNHL | Original carrier ID: Patient\-6; onset in childhood; no aminoglycoside exposure | | 317 | 1555 | m.1555A\>G | A1555G\-F113 | A1555G\-F113\-II1 | Fam | F | Y | Y | ND | 49 | Homo | / | / | / | Severe bilateral down\-sloping SNHL; onset age 6; type 2 diabetes and hypertension | Original carrier ID: Patient\-7 | | 318 | 1555 | m.1555A\>G | A1555G\-F114 | A1555G\-F114\-II1 | Fam | M | Y | Y | ND | 66 | Homo | / | / | / | Profound bilateral down\-sloping SNHL; type 2 diabetes | Original carrier ID: Patient\-8; onset in childhood; no aminoglycoside exposure | | 319 | 1555 | m.1555A\>G | A1555G\-F115 | A1555G\-F115\-P1 | Fam | M | Y | Y | ND | 71 | Homo | / | / | / | Profound bilateral down\-sloping SNHL | Original carrier ID: Patient\-9; onset in childhood; worsened after streptomycin at age 28 | | 320 | 1555 | m.1555A\>G | A1555G\-F117 | A1555G\-F117\-P1 | Fam | ND | Y | Y | ND | ND | / | / | / | / | Definite hearing impairment | Original carrier ID: Family\-B\-proband; aminoglycoside\-induced | | 321 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III3 | Fam | M | Y | Y | ND | ND | Homo | / | / | / | Hearing impaired | Original carrier ID: WZD91\-III\-3; Carrying A4317G; proband; WZD91; in Figure 1D | | 322 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III1 | Fam | M | N | Y | ND | 24 | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-1; Carrying A4317G; matrilineal relative; cell\-line donor; age 24 | | 323 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III9 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-9; Carrying A4317G; matrilineal relative | | 324 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III17 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-17; Carrying A4317G; matrilineal relative | | 325 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV5 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-IV\-5; Carrying A4317G | | 326 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV10 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-IV\-10; Carrying A4317G | | 327 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-III13 | Fam | F | N | Y | ND | 35 | Positive | / | / | / | Severe aminoglycoside\-associated hearing loss | Original carrier ID: WZD92\-III\-13; A1555G\-only cell\-line donor; age 35 | | 328 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-III17 | Fam | F | N | N | ND | 28 | Positive | / | / | / | Healthy | Original carrier ID: WZD92\-III\-17 | | 329 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-III18 | Fam | M | N | N | ND | 31 | Positive | / | / | / | Healthy | Original carrier ID: WZD92\-III\-18 | | 330 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-II2 | Fam | F | N | Y | ND | 55 | Homo | / | / | / | Severe hearing loss; flat audiogram | no aminoglycoside exposure; onset age 28 | | 331 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-III4 | Fam | F | N | Y | ND | 28 | Homo | / | / | / | Moderate hearing loss; slope audiogram | no aminoglycoside exposure | | 332 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-III5 | Fam | M | N | Y | ND | 31 | Homo | / | / | / | Severe hearing loss; slope audiogram | aminoglycoside exposure; onset age 6 | | 333 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-III7 | Fam | M | N | Y | ND | 33 | Homo | / | / | / | Profound hearing loss; slope audiogram | aminoglycoside exposure; onset age 5 | | 334 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-IV2 | Fam | F | Y | Y | ND | 17 | Homo | / | / | / | Profound hearing loss; ridge audiogram | aminoglycoside exposure; onset age 2 | | 335 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-IV1 | Fam | F | N | N | ND | 14 | Homo | / | / | / | Healthy | | | 336 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II1 | Fam | ND | N | Y | ND | 20\-60 range | / | / | / | / | Deaf\-mute with congenital severe deafness | Original carrier ID: F6H; No aminoglycoside exposure | | 337 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II2 | Fam | ND | N | Y | ND | 20\-60 range | / | / | / | / | Deaf\-mute with congenital severe deafness | Original carrier ID: F7D; No aminoglycoside exposure | | 338 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II3 | Fam | ND | N | Y | ND | 20\-60 range | / | / | / | / | Deaf\-mute with congenital severe deafness | Original carrier ID: F12F; No aminoglycoside exposure | | 339 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II4 | Fam | ND | N | Y | ND | 20\-60 range | / | / | / | / | Severe hearing loss developed at age 20 | Original carrier ID: F7C; No aminoglycoside exposure | | 340 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II5 | Fam | ND | N | Y | ND | 20\-60 range | / | / | / | / | Moderate early\-onset hearing loss | Original carrier ID: F12J; No aminoglycoside exposure | | 341 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II6 | Fam | ND | N | N | ND | 20\-60 range | / | / | / | / | Healthy | Original carrier ID: F7H; No aminoglycoside exposure | | 342 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II7 | Fam | ND | N | N | ND | 20\-60 range | / | / | / | / | Healthy | Original carrier ID: F12C; No aminoglycoside exposure | | 343 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II8 | Fam | ND | N | N | ND | 20\-60 range | / | / | / | / | Healthy | Original carrier ID: F12D; No aminoglycoside exposure | | 344 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II9 | Fam | ND | N | N | ND | 20\-60 range | / | / | / | / | Healthy | Original carrier ID: F12E; No aminoglycoside exposure | | 345 | 1555 | m.1555A\>G | A1555G\-F121 | A1555G\-F121\-II10 | Fam | ND | N | N | ND | 20\-60 range | / | / | / | / | Healthy | Original carrier ID: F12H; No aminoglycoside exposure | | 346 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-IV6 | Fam | ND | Y | ND | ND | ND | Homo | / | / | / | ND | exact hearing severity not text\-extracted; in large maternal hearing\-loss family; Affected patient | | 347 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-IV4 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-IV\-4 | | 348 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-IV5 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-IV\-5 | | 349 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-IV11 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-1\-IV\-11; aminoglycoside injection | | 350 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-IV13 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-IV\-13 | | 351 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V4 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-1\-V\-4; aminoglycoside injection | | 352 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V6 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss; loud\-sound exposure history. | Original carrier ID: Family\-1\-V\-6 | | 353 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V8 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-V\-8 | | 354 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V11 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-V\-11 | | 355 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V13 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-V\-13 | | 356 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V15 | Fam | F | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-V\-15 | | 357 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V19 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-V\-19 | | 358 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-V23 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-1\-V\-23 | | 359 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI9 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-1\-VI\-9; aminoglycoside injection | | 360 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI11 | Fam | F | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-VI\-11 | | 361 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI12 | Fam | F | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-VI\-12 | | 362 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI13 | Fam | M | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-VI\-13 | | 363 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI18 | Fam | M | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-VI\-18 | | 364 | 1555 | m.1555A\>G | A1555G\-F125 | A1555G\-F125\-VI19 | Fam | F | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-1\-VI\-19 | | 365 | 1555 | m.1555A\>G | A1555G\-F352 | A1555G\-F352\-IV6 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-2\-IV\-6; aminoglycoside injection | | 366 | 1555 | m.1555A\>G | A1555G\-F123 | A1555G\-F123\-III7 | Fam | F | ND | N | A | ND | Homo | / | / | / | Healthy | Original carrier ID: Family\-3\-III\-7 | | 367 | 1555 | m.1555A\>G | A1555G\-F123 | A1555G\-F123\-IV2 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Progressive hearing loss after one aminoglycoside injection at age 3 | Original carrier ID: Family\-3\-IV\-2 | | 368 | 1555 | m.1555A\>G | A1555G\-F353 | A1555G\-F353\-II6 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-4\-II\-6; aminoglycoside injection | | 369 | 1555 | m.1555A\>G | A1555G\-F353 | A1555G\-F353\-III1 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-4\-III\-1; aminoglycoside injection | | 370 | 1555 | m.1555A\>G | A1555G\-F353 | A1555G\-F353\-III2 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-4\-III\-2 | | 371 | 1555 | m.1555A\>G | A1555G\-F124 | A1555G\-F124\-II4 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-5\-II\-4 | | 372 | 1555 | m.1555A\>G | A1555G\-F124 | A1555G\-F124\-III4 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss. | Original carrier ID: Family\-5\-III\-4 | | 373 | 1555 | m.1555A\>G | A1555G\-F124 | A1555G\-F124\-III14 | Fam | F | ND | Y | A | ND | Homo | / | / | / | Hearing loss | Original carrier ID: Family\-5\-III\-14; aminoglycoside injection | | 374 | 1555 | m.1555A\>G | A1555G\-F124 | A1555G\-F124\-IV1 | Fam | M | ND | Y | A | ND | Homo | / | / | / | Progressive hearing loss without clear aminoglycoside injection; repeated vertigo attacks | Original carrier ID: Family\-5\-IV\-1 | | 375 | 1555 | m.1555A\>G | A1555G\-F126 | A1555G\-F126\-II1 | Fam | F | Y | Y | ND | onset 17 | Homo | / | / | / | Hearing loss; PTA 66/70 | streptomycin exposure; family history yes (1 affected maternal relative) | | 376 | 1555 | m.1555A\>G | A1555G\-F127 | A1555G\-F127\-P1 | Uninf | F | Y | Y | ND | onset 4 | Homo | / | / | / | Hearing loss; PTA 70/58 | Original carrier ID: G29; gentamicin exposure; no family history | | 377 | 1555 | m.1555A\>G | A1555G\-F128 | A1555G\-F128\-P1 | Uninf | F | Y | Y | ND | onset 9 | Homo | / | / | / | Hearing loss; PTA 56/59 | Original carrier ID: G38; no aminoglycoside exposure; no family history | | 378 | 1555 | m.1555A\>G | A1555G\-F129 | A1555G\-F129\-P1 | Uninf | F | Y | Y | ND | onset 10 | Homo | / | / | / | Hearing loss; PTA 70/74 | Original carrier ID: G43; gentamicin exposure; no family history | | 379 | 1555 | m.1555A\>G | A1555G\-F130 | A1555G\-F130\-P1 | Uninf | M | Y | Y | ND | onset 3 | Homo | / | / | / | Hearing loss; PTA 85/84 | Original carrier ID: G101; no aminoglycoside exposure; no family history | | 380 | 1555 | m.1555A\>G | A1555G\-F131 | A1555G\-F131\-P1 | Fam | M | Y | Y | ND | onset 3 | Homo | / | / | / | Hearing loss; PTA 73/80 | Original carrier ID: G104; gentamicin exposure; family history yes (2 affected maternal relatives) | | 381 | 1555 | m.1555A\>G | A1555G\-F132 | A1555G\-F132\-P1 | Uninf | M | Y | Y | ND | onset 7 | Homo | / | / | / | Hearing loss; PTA 49/44 | Original carrier ID: G136; amikacin exposure; no family history | | 382 | 1555 | m.1555A\>G | A1555G\-F133 | A1555G\-F133\-I2 | Uninf | F | Y | Y | ND | onset 6 | Homo | / | / | / | Hearing loss; PTA not available | gentamicin exposure; family history yes (1 affected maternal relative) | | 383 | 1555 | m.1555A\>G | A1555G\-F134 | A1555G\-F134\-P1 | Uninf | F | Y | Y | ND | onset 15 | Homo | / | / | / | Hearing loss; PTA 108/112 | Original carrier ID: G244; gentamicin exposure; no family history | | 384 | 1555 | m.1555A\>G | A1555G\-F135 | A1555G\-F135\-P1 | Fam | F | Y | ND | ND | 18 | Homo | / | / | / | ND | Original carrier ID: Family\-17\-proband; Suspected mitochondrial disorder; MT\-RNR1 diagnosis contributed partially; subject\-specific; RNR1 phenotype not detailed | | 385 | 1555 | m.1555A\>G | A1555G\-F4 | A1555G\-F4\-III1 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss | brother; after gentamicin exposure at age 3 | | 386 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-II2 | Fam | F | N | Y | ND | 77 | Homo | / | / | / | Hearing status: severe | onset: 30; aminoglycoside exposure: Yes | | 387 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-II6 | Fam | F | N | Y | ND | 67 | Homo | / | / | / | Hearing status: profound | onset: 40; aminoglycoside exposure: Yes | | 388 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-II8 | Fam | F | Y | Y | ND | 61 | Homo | / | / | / | Hearing status: mild | onset: 58; aminoglycoside exposure: No | | 389 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III1 | Fam | M | N | Y | ND | 38 | Homo | / | / | / | Hearing status: severe; onset: congenital | aminoglycoside exposure: No | | 390 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III3 | Fam | F | N | Y | ND | 40 | Homo | / | / | / | Hearing status: severe; onset: congenital | aminoglycoside exposure: No | | 391 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III5 | Fam | M | N | Y | ND | 48 | Homo | / | / | / | Hearing status: moderate | onset: 10; aminoglycoside exposure: Yes | | 392 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III7 | Fam | M | N | Y | ND | 51 | Homo | / | / | / | Hearing status: mild | onset: 4; aminoglycoside exposure: No | | 393 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III9 | Fam | M | N | Y | ND | 54 | Homo | / | / | / | Hearing status: mild | onset: 7; aminoglycoside exposure: Yes | | 394 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III11 | Fam | M | N | Y | ND | 56 | Homo | / | / | / | Hearing status: mild | onset: 1; aminoglycoside exposure: Yes | | 395 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III13 | Fam | F | N | Y | ND | 49 | Homo | / | / | / | Hearing status: severe | onset: 18; aminoglycoside exposure: Yes | | 396 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III17 | Fam | M | N | Y | ND | 51 | Homo | / | / | / | Hearing status: mild | onset: 15; aminoglycoside exposure: No | | 397 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III21 | Fam | F | N | Y | ND | 45 | Homo | / | / | / | Hearing status: severe | onset: 3; aminoglycoside exposure: Yes | | 398 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III23 | Fam | M | N | Y | ND | 46 | Homo | / | / | / | Hearing status: mild | onset: 38; aminoglycoside exposure: No | | 399 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III25 | Fam | F | N | Y | ND | 42 | Homo | / | / | / | Hearing status: profound | onset: 16; aminoglycoside exposure: Yes | | 400 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-III27 | Fam | F | N | Y | ND | 36 | Homo | / | / | / | Hearing status: profound | onset: 8 months; aminoglycoside exposure: Yes | | 401 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-IV2 | Fam | F | N | N | ND | 17 | Homo | / | / | / | Healthy | onset: NA; aminoglycoside exposure: No | | 402 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-IV7 | Fam | M | N | N | ND | 25 | Homo | / | / | / | Healthy | onset: NA; aminoglycoside exposure: No | | 403 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-IV13 | Fam | M | N | N | ND | 17 | Homo | / | / | / | Healthy | onset: NA; aminoglycoside exposure: No | | 404 | 1555 | m.1555A\>G | A1555G\-F136 | A1555G\-F136\-IV14 | Fam | F | N | N | ND | 10 | Homo | / | / | / | Healthy | onset: NA; aminoglycoside exposure: No | | 405 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-I2 | Uninf | F | N | N | ND | 72 | Homo | / | / | / | Healthy | Carrying G7444A; onset: NA; aminoglycoside exposure: No | | 406 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II4 | Fam | F | N | Y | ND | 49 | Homo | / | / | / | Hearing status: moderate | Carrying G7444A; onset: 27; aminoglycoside exposure: Yes | | 407 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II6 | Fam | F | N | N | ND | 47 | Homo | / | / | / | Healthy | Carrying G7444A; onset: NA; aminoglycoside exposure: No | | 408 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II8 | Fam | F | N | N | ND | 44 | Homo | / | / | / | Healthy | Carrying G7444A; onset: NA; aminoglycoside exposure: No | | 409 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II9 | Fam | M | N | Y | ND | 41 | Homo | / | / | / | Hearing status: severe | Carrying G7444A; onset: 8; aminoglycoside exposure: Yes | | 410 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II11 | Fam | M | N | Y | ND | 41 | Homo | / | / | / | Hearing status: mild | Carrying G7444A; onset: ND; aminoglycoside exposure: No | | 411 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II13 | Fam | M | N | Y | ND | 38 | Homo | / | / | / | Hearing status: profound | Carrying G7444A; onset: 2; aminoglycoside exposure: Yes | | 412 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-II14 | Fam | M | N | Y | ND | 31 | Homo | / | / | / | Hearing status: profound | Carrying G7444A; onset: 3; aminoglycoside exposure: Yes | | 413 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-III5 | Fam | M | N | Y | ND | 19 | Homo | / | / | / | Hearing status: profound | Carrying G7444A; onset: 1; aminoglycoside exposure: Yes | | 414 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-III7 | Fam | M | N | Y | ND | 24 | Homo | / | / | / | Hearing status: profound | Carrying G7444A; onset: 2; aminoglycoside exposure: Yes | | 415 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-III10 | Fam | M | N | N | ND | 18 | Homo | / | / | / | Healthy | Carrying G7444A; onset: NA; aminoglycoside exposure: No | | 416 | 1555 | m.1555A\>G | A1555G\-F9 | A1555G\-F9\-III15 | Fam | M | N | N | ND | 8 | Homo | / | / | / | Healthy | Carrying G7444A; onset: NA; aminoglycoside exposure: No | | 417 | 1555 | m.1555A\>G | A1555G\-F137 | A1555G\-F137\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Mild hearing loss | Original carrier ID: subject\_21; no aminoglycoside exposure; GJB2 absent | | 418 | 1555 | m.1555A\>G | A1555G\-F138 | A1555G\-F138\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 15; penetrance incl aminoglycosides 6\.7%, excl aminoglycosides 0%; family average onset ND; haplogroup D4; proband; Han Chinese pediatric | | 419 | 1555 | m.1555A\>G | A1555G\-F139 | A1555G\-F139\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 6; penetrance incl aminoglycosides 16\.7%, excl aminoglycosides 0%; family average onset ND; haplogroup D4a; proband; Han Chinese pediatric | | 420 | 1555 | m.1555A\>G | A1555G\-F140 | A1555G\-F140\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 22; penetrance incl aminoglycosides 9\.1%, excl aminoglycosides 4\.5%; family average onset 5; haplogroup F1a; proband; Han Chinese pediatric | | 421 | 1555 | m.1555A\>G | A1555G\-F141 | A1555G\-F141\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 16; penetrance incl aminoglycosides 31\.3%, excl aminoglycosides 31\.3%; family average onset 12; haplogroup N9a3; proband; Han Chinese pediatric | | 422 | 1555 | m.1555A\>G | A1555G\-F142 | A1555G\-F142\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 17; penetrance incl aminoglycosides 5\.9%, excl aminoglycosides 0%; family average onset ND; haplogroup D5b1; proband; Han Chinese pediatric | | 423 | 1555 | m.1555A\>G | A1555G\-F143 | A1555G\-F143\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 10; penetrance incl aminoglycosides 10%, excl aminoglycosides 0%; family average onset ND; haplogroup D5a; proband; Han Chinese pediatric | | 424 | 1555 | m.1555A\>G | A1555G\-F144 | A1555G\-F144\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 12; penetrance incl aminoglycosides 8\.3%, excl aminoglycosides 0%; family average onset ND; haplogroup D5a; proband; Han Chinese pediatric | | 425 | 1555 | m.1555A\>G | A1555G\-F145 | A1555G\-F145\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 16; penetrance incl aminoglycosides 12\.5%, excl aminoglycosides 0%; family average onset ND; haplogroup R9b; proband; Han Chinese pediatric | | 426 | 1555 | m.1555A\>G | A1555G\-F146 | A1555G\-F146\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 25; penetrance incl aminoglycosides 16%, excl aminoglycosides 12%; family average onset 15; haplogroup D4; proband; Han Chinese pediatric | | 427 | 1555 | m.1555A\>G | A1555G\-F147 | A1555G\-F147\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying T5802C; family index; family size 29; penetrance incl aminoglycosides 41\.4%, excl aminoglycosides 31%; family average onset 14; haplogroup D4; proband; Han Chinese pediatric | | 428 | 1555 | m.1555A\>G | A1555G\-F148 | A1555G\-F148\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 22; penetrance incl aminoglycosides 18\.2%, excl aminoglycosides 13\.6%; family average onset 18; haplogroup D4; proband; Han Chinese pediatric | | 429 | 1555 | m.1555A\>G | A1555G\-F149 | A1555G\-F149\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 11; penetrance incl aminoglycosides 36\.4%, excl aminoglycosides 36\.4%; family average onset 12; haplogroup D5a; proband; Han Chinese pediatric | | 430 | 1555 | m.1555A\>G | A1555G\-F150 | A1555G\-F150\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 13; penetrance incl aminoglycosides 30\.8%, excl aminoglycosides 23\.1%; family average onset 16; haplogroup F1a; proband; Han Chinese pediatric | | 431 | 1555 | m.1555A\>G | A1555G\-F151 | A1555G\-F151\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 6; penetrance incl aminoglycosides 33\.3%, excl aminoglycosides 0%; family average onset ND; haplogroup M10a; proband; Han Chinese pediatric | | 432 | 1555 | m.1555A\>G | A1555G\-F152 | A1555G\-F152\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 7; penetrance incl aminoglycosides 42\.9%, excl aminoglycosides 0%; family average onset ND; haplogroup M7b1; proband; Han Chinese pediatric | | 433 | 1555 | m.1555A\>G | A1555G\-F153 | A1555G\-F153\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 24; penetrance incl aminoglycosides 12\.5%, excl aminoglycosides 8\.3%; family average onset 16; haplogroup N9a; proband; Han Chinese pediatric | | 434 | 1555 | m.1555A\>G | A1555G\-F154 | A1555G\-F154\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 8; penetrance incl aminoglycosides 12\.5%, excl aminoglycosides 0%; family average onset ND; haplogroup D4; proband; Han Chinese pediatric | | 435 | 1555 | m.1555A\>G | A1555G\-F155 | A1555G\-F155\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 8; penetrance incl aminoglycosides 62\.5%, excl aminoglycosides 50%; family average onset 11; haplogroup N9a; proband; Han Chinese pediatric | | 436 | 1555 | m.1555A\>G | A1555G\-F156 | A1555G\-F156\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 15; penetrance incl aminoglycosides 6\.7%, excl aminoglycosides 0%; family average onset ND; haplogroup F1; proband; Han Chinese pediatric | | 437 | 1555 | m.1555A\>G | A1555G\-F157 | A1555G\-F157\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 15; penetrance incl aminoglycosides 13\.3%, excl aminoglycosides 0%; family average onset ND; haplogroup D4h; proband; Han Chinese pediatric | | 438 | 1555 | m.1555A\>G | A1555G\-F158 | A1555G\-F158\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 30; penetrance incl aminoglycosides 30%, excl aminoglycosides 20%; family average onset 16; haplogroup F2; proband; Han Chinese pediatric | | 439 | 1555 | m.1555A\>G | A1555G\-F159 | A1555G\-F159\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 22; penetrance incl aminoglycosides 59\.1%, excl aminoglycosides 50%; family average onset 11; haplogroup D5; proband; Han Chinese pediatric | | 440 | 1555 | m.1555A\>G | A1555G\-F160 | A1555G\-F160\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 16; penetrance incl aminoglycosides 18\.8%, excl aminoglycosides 0%; family average onset ND; haplogroup M7; proband; Han Chinese pediatric | | 441 | 1555 | m.1555A\>G | A1555G\-F161 | A1555G\-F161\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying T12338C; family index; family size 20; penetrance incl aminoglycosides 30%, excl aminoglycosides 25%; family average onset 12; haplogroup F2; proband; Han Chinese pediatric | | 442 | 1555 | m.1555A\>G | A1555G\-F162 | A1555G\-F162\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 37; penetrance incl aminoglycosides 16\.2%, excl aminoglycosides 5\.4%; family average onset 30; haplogroup N9a; proband; Han Chinese pediatric | | 443 | 1555 | m.1555A\>G | A1555G\-F163 | A1555G\-F163\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 7; penetrance incl aminoglycosides 14\.3%, excl aminoglycosides 0%; family average onset ND; haplogroup G; proband; Han Chinese pediatric | | 444 | 1555 | m.1555A\>G | A1555G\-F164 | A1555G\-F164\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 62; penetrance incl aminoglycosides 3\.2%, excl aminoglycosides 3\.2%; family average onset 9; haplogroup F1a1; proband; Han Chinese pediatric | | 445 | 1555 | m.1555A\>G | A1555G\-F165 | A1555G\-F165\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 14; penetrance incl aminoglycosides 7\.1%, excl aminoglycosides 0%; family average onset ND; haplogroup B4; proband; Han Chinese pediatric | | 446 | 1555 | m.1555A\>G | A1555G\-F166 | A1555G\-F166\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying G5821A; family index; family size 12; penetrance incl aminoglycosides 33%, excl aminoglycosides 25%; family average onset 15; haplogroup C; proband; Han Chinese pediatric | | 447 | 1555 | m.1555A\>G | A1555G\-F167 | A1555G\-F167\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 14; penetrance incl aminoglycosides 35\.7%, excl aminoglycosides 28\.6%; family average onset 13; haplogroup D5a; proband; Han Chinese pediatric | | 448 | 1555 | m.1555A\>G | A1555G\-F168 | A1555G\-F168\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 25; penetrance incl aminoglycosides 20%, excl aminoglycosides 12%; family average onset 14; haplogroup N9a3; proband; Han Chinese pediatric | | 449 | 1555 | m.1555A\>G | A1555G\-F169 | A1555G\-F169\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 15; penetrance incl aminoglycosides 26\.7%, excl aminoglycosides 6\.7%; family average onset 18; haplogroup B4a1; proband; Han Chinese pediatric | | 450 | 1555 | m.1555A\>G | A1555G\-F170 | A1555G\-F170\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 30; penetrance incl aminoglycosides 23\.3%, excl aminoglycosides 13\.3%; family average onset 10; haplogroup F2; proband; Han Chinese pediatric | | 451 | 1555 | m.1555A\>G | A1555G\-F171 | A1555G\-F171\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying C12224T; family index; family size 15; penetrance incl aminoglycosides 53\.3%, excl aminoglycosides 46\.7%; family average onset 13; haplogroup D5; proband; Han Chinese pediatric | | 452 | 1555 | m.1555A\>G | A1555G\-F172 | A1555G\-F172\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying A14693G; family index; family size 18; penetrance incl aminoglycosides 38\.9%, excl aminoglycosides 27\.8%; family average onset 15; haplogroup Y2; proband; Han Chinese pediatric | | 453 | 1555 | m.1555A\>G | A1555G\-F173 | A1555G\-F173\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 19; penetrance incl aminoglycosides 15\.8%, excl aminoglycosides 0%; family average onset ND; haplogroup D4b; proband; Han Chinese pediatric | | 454 | 1555 | m.1555A\>G | A1555G\-F174 | A1555G\-F174\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 17; penetrance incl aminoglycosides 35\.3%, excl aminoglycosides 11\.8%; family average onset 21; haplogroup F2; proband; Han Chinese pediatric | | 455 | 1555 | m.1555A\>G | A1555G\-F175 | A1555G\-F175\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 25; penetrance incl aminoglycosides 40%, excl aminoglycosides 20%; family average onset 18; haplogroup A; proband; Han Chinese pediatric | | 456 | 1555 | m.1555A\>G | A1555G\-F176 | A1555G\-F176\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 15; penetrance incl aminoglycosides 13\.3%, excl aminoglycosides 0%; family average onset ND; haplogroup D5a; proband; Han Chinese pediatric | | 457 | 1555 | m.1555A\>G | A1555G\-F177 | A1555G\-F177\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 17; penetrance incl aminoglycosides 47\.1%, excl aminoglycosides 29\.4%; family average onset 13; haplogroup D5b; proband; Han Chinese pediatric | | 458 | 1555 | m.1555A\>G | A1555G\-F178 | A1555G\-F178\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | family index; family size 20; penetrance incl aminoglycosides 20%, excl aminoglycosides 10%; family average onset 20; haplogroup F3b; proband; Han Chinese pediatric | | 459 | 1555 | m.1555A\>G | A1555G\-F179 | A1555G\-F179\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying G7444A; family index; family size 8; penetrance incl aminoglycosides 50%, excl aminoglycosides 37\.5%; family average onset 13; haplogroup B4b1c; proband; Han Chinese pediatric | | 460 | 1555 | m.1555A\>G | A1555G\-F180 | A1555G\-F180\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Hearing\-impaired / | Carrying G7444A; family index; family size 31; penetrance incl aminoglycosides 41\.9%, excl aminoglycosides 35\.5%; family average onset 12; haplogroup B4; proband; Han Chinese pediatric | | 461 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-III5 | Fam | M | Y | Y | ND | 44 | / | / | / | / | Deafness | after streptomycin at age 17 | | 462 | 1555 | m.1555A\>G | A1555G\-F182 | A1555G\-F182\-III16 | Fam | F | Y | Y | ND | 27 | / | / | / | / | Hearing loss at age 5 | after streptomycin | | 463 | 1555 | m.1555A\>G | A1555G\-F183 | A1555G\-F183\-III2 | Fam | F | Y | Y | ND | 19 | / | / | / | / | Tinnitus/hearing loss | after streptomycin exposure | | 464 | 1555 | m.1555A\>G | A1555G\-F184 | A1555G\-F184\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | / | Syndromic sensorineural hearing loss with ataxia and failure to thrive. | Original carrier ID: m1555\_patient | | 465 | 1555 | m.1555A\>G | A1555G\-F185 | A1555G\-F185\-II2 | De novo | F | N | N | ND | 31 | 28\.68% | / | / | / | Healthy | | | 466 | 1555 | m.1555A\>G | A1555G\-F186 | A1555G\-F186\-II1 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Parkinsonism | in mitochondrial disease cohort | | 467 | 1555 | m.1555A\>G | A1555G\-F186 | A1555G\-F186\-II2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Neurological symptoms plus deafness. | | | 468 | 1555 | m.1555A\>G | A1555G\-F186 | A1555G\-F186\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Neurological symptoms plus deafness. | | | 469 | 1555 | m.1555A\>G | A1555G\-F187 | A1555G\-F187\-IV1 | Fam | M | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 98/98 dB; profound | Original carrier ID: FE003\-IV\-1; slope; aminoglycoside: Yes | | 470 | 1555 | m.1555A\>G | A1555G\-F188 | A1555G\-F188\-IV6 | Fam | M | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 100/100 dB; profound | Original carrier ID: FE007\-IV\-6; slope; aminoglycoside: Yes | | 471 | 1555 | m.1555A\>G | A1555G\-F189 | A1555G\-F189\-III7 | Fam | F | Y | Y | ND | 10 | Homo | / | / | / | PTA R/L 58/78 dB; severe | Original carrier ID: FE008\-III\-7; slope; aminoglycoside: No | | 472 | 1555 | m.1555A\>G | A1555G\-F190 | A1555G\-F190\-IV1 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 102/98 dB; profound | Original carrier ID: FE0128\-IV\-1; slope; aminoglycoside: Yes | | 473 | 1555 | m.1555A\>G | A1555G\-F191 | A1555G\-F191\-IV1 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 67/82 dB; severe | Original carrier ID: FE019\-IV\-1; slope; aminoglycoside: Yes | | 474 | 1555 | m.1555A\>G | A1555G\-F192 | A1555G\-F192\-III15 | Fam | M | Y | Y | ND | 8 | Homo | / | / | / | PTA R/L 81/74 dB; severe | Original carrier ID: FE020\-III\-15; slope; aminoglycoside: Yes | | 475 | 1555 | m.1555A\>G | A1555G\-F193 | A1555G\-F193\-III1 | Fam | M | Y | Y | ND | 10 | Homo | / | / | / | PTA R/L 58/49 dB; moderate | Original carrier ID: FE036\-III\-1; slope; aminoglycoside: No | | 476 | 1555 | m.1555A\>G | A1555G\-F194 | A1555G\-F194\-III1 | Fam | M | Y | Y | ND | 16 | Homo | / | / | / | PTA R/L 50/56 dB; moderate | Original carrier ID: FE081\-III\-1; slope; aminoglycoside: Yes | | 477 | 1555 | m.1555A\>G | A1555G\-F195 | A1555G\-F195\-III2 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 71/53 dB; moderate | Original carrier ID: FE122\-III\-2; slope; aminoglycoside: Yes | | 478 | 1555 | m.1555A\>G | A1555G\-F196 | A1555G\-F196\-III1 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 24/30 dB; mild | Original carrier ID: FE141\-III\-1; slope; aminoglycoside: No | | 479 | 1555 | m.1555A\>G | A1555G\-F197 | A1555G\-F197\-III1 | Fam | F | Y | Y | ND | 18 | Homo | / | / | / | PTA R/L 61/60 dB; moderate | Original carrier ID: FE154\-III\-1; slope; aminoglycoside: No | | 480 | 1555 | m.1555A\>G | A1555G\-F198 | A1555G\-F198\-III1 | Fam | F | Y | Y | ND | 5 | Homo | / | / | / | PTA R/L 61/74 dB; severe | Original carrier ID: FE160\-III\-1; slope; aminoglycoside: No | | 481 | 1555 | m.1555A\>G | A1555G\-F199 | A1555G\-F199\-III3 | Fam | M | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 110/99 dB; profound | Original carrier ID: FE163\-III\-3; flat; aminoglycoside: Yes | | 482 | 1555 | m.1555A\>G | A1555G\-F200 | A1555G\-F200\-II12 | Fam | F | Y | Y | ND | 3 | Homo | / | / | / | PTA R/L 110/105 dB; profound | Original carrier ID: FE300\-II\-12; slope; aminoglycoside: Yes | | 483 | 1555 | m.1555A\>G | A1555G\-F201 | A1555G\-F201\-II2 | Fam | F | Y | Y | ND | 3 | Homo | / | / | / | PTA R/L 100/80 dB; profound | Original carrier ID: FE304\-II\-2; slope; aminoglycoside: Yes | | 484 | 1555 | m.1555A\>G | A1555G\-F202 | A1555G\-F202\-III10 | Fam | M | Y | Y | ND | 4 | Homo | / | / | / | PTA R/L 94/93 dB; profound | Original carrier ID: FE317\-III\-10; slope; aminoglycoside: Yes | | 485 | 1555 | m.1555A\>G | A1555G\-F203 | A1555G\-F203\-III1 | Fam | F | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 100/100 dB; profound | Original carrier ID: FE350\-III\-1; flat; aminoglycoside: Yes | | 486 | 1555 | m.1555A\>G | A1555G\-F204 | A1555G\-F204\-III1 | Fam | M | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 90/87 dB; profound | Original carrier ID: NB038\-III\-1; flat; aminoglycoside: Yes | | 487 | 1555 | m.1555A\>G | A1555G\-F205 | A1555G\-F205\-III2 | Fam | F | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 78/81 dB; severe | Original carrier ID: NB048\-III\-2; slope; aminoglycoside: No | | 488 | 1555 | m.1555A\>G | A1555G\-F206 | A1555G\-F206\-III2 | Fam | F | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 120/102 dB; profound | Original carrier ID: NB052\-III\-2; flat; aminoglycoside: No | | 489 | 1555 | m.1555A\>G | A1555G\-F207 | A1555G\-F207\-III1 | Fam | M | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 118/118 dB; profound | Original carrier ID: NB076\-III\-1; flat; aminoglycoside: Yes | | 490 | 1555 | m.1555A\>G | A1555G\-F208 | A1555G\-F208\-III2 | Fam | F | Y | Y | ND | 6 | Homo | / | / | / | PTA R/L 110/117 dB; profound | Original carrier ID: NB078\-III\-2; flat; aminoglycoside: Yes | | 491 | 1555 | m.1555A\>G | A1555G\-F209 | A1555G\-F209\-III1 | Fam | M | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 102/102 dB; profound | Original carrier ID: NB079\-III\-1; flat; aminoglycoside: Yes | | 492 | 1555 | m.1555A\>G | A1555G\-F210 | A1555G\-F210\-III2 | Fam | F | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 117/117 dB; profound | Original carrier ID: NB094\-III\-2; flat; aminoglycoside: Yes | | 493 | 1555 | m.1555A\>G | A1555G\-F211 | A1555G\-F211\-III2 | Fam | F | Y | Y | ND | 3 | Homo | / | / | / | PTA R/L 83/86 dB; severe | Original carrier ID: NB111\-III\-2; slope; aminoglycoside: Yes | | 494 | 1555 | m.1555A\>G | A1555G\-F212 | A1555G\-F212\-III2 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 84/92 dB; profound | Original carrier ID: NB126\-III\-2; slope; aminoglycoside: No | | 495 | 1555 | m.1555A\>G | A1555G\-F213 | A1555G\-F213\-III1 | Fam | F | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 111/115 dB; profound | Original carrier ID: NB137\-III\-1; slope; aminoglycoside: No | | 496 | 1555 | m.1555A\>G | A1555G\-F214 | A1555G\-F214\-II2 | Fam | F | Y | Y | ND | 5 | Homo | / | / | / | PTA R/L 59/62 dB; moderate | Original carrier ID: ZX019\-II\-2; slope; aminoglycoside: Yes | | 497 | 1555 | m.1555A\>G | A1555G\-F215 | A1555G\-F215\-III3 | Fam | M | Y | Y | ND | 2 | Homo | / | / | / | PTA R/L 101/102 dB; profound | Original carrier ID: ZX022\-III\-3; flat; aminoglycoside: Yes | | 498 | 1555 | m.1555A\>G | A1555G\-F216 | A1555G\-F216\-III14 | Fam | M | Y | Y | ND | 1 | Homo | / | / | / | PTA R/L 113/108 dB; profound | Original carrier ID: ZX025\-III\-14; flat; aminoglycoside: Yes | | 499 | 1555 | m.1555A\>G | A1555G\-F217 | A1555G\-F217\-IV1 | Fam | F | Y | Y | ND | 3 | Homo | / | / | / | PTA R/L 87/87 dB; severe | Original carrier ID: ZX028\-IV\-1; slope; aminoglycoside: No | | 500 | 1555 | m.1555A\>G | A1555G\-F218 | A1555G\-F218\-II7 | Fam | M | Y | Y | ND | 5 | Homo | / | / | / | PTA R/L 30/27 dB; mild | Original carrier ID: ZX037\-II\-7; flat; aminoglycoside: No | | 501 | 1555 | m.1555A\>G | A1555G\-F219 | A1555G\-F219\-III1 | Fam | M | Y | Y | ND | 6 | Homo | / | / | / | PTA R/L 78/79 dB; severe | Original carrier ID: ZX047\-III\-1; slope; aminoglycoside: No | | 502 | 1555 | m.1555A\>G | A1555G\-F220 | A1555G\-F220\-II1 | Fam | F | Y | Y | A | 48 | Homo | / | / | / | Progressive bilateral sensorineural hearing loss beginning in early teens | | | 503 | 1555 | m.1555A\>G | A1555G\-F220 | A1555G\-F220\-II2 | Fam | F | N | N | A | 19 | Positive | / | / | / | Healthy | | | 504 | 1555 | m.1555A\>G | A1555G\-F220 | A1555G\-F220\-II3 | Fam | F | N | N | A | 15 | Positive | / | / | / | Healthy | | | 505 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II1 | Fam | ND | ND | ND | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | ND | Original carrier ID: F6H; symptomatic family member; detailed phenotype not provided | | 506 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II2 | Fam | ND | ND | ND | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | ND | Original carrier ID: F7C; symptomatic family member; detailed phenotype not provided | | 507 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II3 | Fam | ND | ND | ND | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | ND | Original carrier ID: F7D; symptomatic family member; detailed phenotype not provided | | 508 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II4 | Fam | ND | ND | ND | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | ND | Original carrier ID: F12G; symptomatic family member; detailed phenotype not provided | | 509 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II5 | Fam | ND | ND | ND | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | ND | Original carrier ID: F12J; symptomatic family member; detailed phenotype not provided | | 510 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II6 | Fam | ND | ND | N | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | Healthy | Original carrier ID: F6C | | 511 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II7 | Fam | ND | ND | N | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | Healthy | Original carrier ID: F12D | | 512 | 1555 | m.1555A\>G | A1555G\-F221 | A1555G\-F221\-II8 | Fam | ND | ND | N | ND | ND | / | / | / | Homo(lymphoblastoid/cybrid) | Healthy | Original carrier ID: F12H | | 513 | 1555 | m.1555A\>G | A1555G\-F222 | A1555G\-F222\-II4 | Fam | M | Y | Y | A | 11 | Homo | / | / | / | Profound prelingual deafness | cerebral infarct; aminoglycoside exposure positive; Central Anatolia | | 514 | 1555 | m.1555A\>G | A1555G\-F222 | A1555G\-F222\-I2 | Uninf | F | N | N | A | 48 | Homo | / | / | / | Healthy | | | 515 | 1555 | m.1555A\>G | A1555G\-F222 | A1555G\-F222\-II2 | Fam | F | N | N | A | 22 | Homo | / | / | / | Healthy | | | 516 | 1555 | m.1555A\>G | A1555G\-F222 | A1555G\-F222\-II3 | Fam | M | N | N | A | 20 | Homo | / | / | / | Healthy | | | 517 | 1555 | m.1555A\>G | A1555G\-F222 | A1555G\-F222\-III1 | Fam | F | N | N | A | 3 | Homo | / | / | / | Healthy | | | 518 | 1555 | m.1555A\>G | A1555G\-F223 | A1555G\-F223\-P1 | Fam | M | Y | Y | ND | 4 | Homo | / | / | / | Profound prelingual deafness | antibiotic exposure \+/; parenteral; Southeastern Anatolia | | 519 | 1555 | m.1555A\>G | A1555G\-F224 | A1555G\-F224\-P1 | Fam | F | Y | Y | ND | 17 | Homo | / | / | / | Profound prelingual deafness | family history positive in sibling; antibiotic exposure \+/; parenteral; Western Anatolia | | 520 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III6 | Fam | M | ND | ND | ND | ND | Homo | / | / | / | ND | Original carrier ID: III\-6\-P1; affected individual; SSBP1 c.3G\>A heterozygous; father in Family D; fibroblast line established | | 521 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III5 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Hearing loss; SSBP1 c.3G\>A heterozygous; patient fibroblast line established. | Original carrier ID: III\-5\-P2 | | 522 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III10 | Fam | M | ND | ND | ND | 38 | Homo | / | / | / | ND | Original carrier ID: III\-10\-P4; affected individual; SSBP1 c.3G\>A heterozygous; father in Family E; muscle biopsy showed mtDNA maintenance abnormalities | | 523 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III8 | Fam | F | ND | Y | ND | 41 | Homo | / | / | / | Hearing loss; SSBP1 c.3G\>A heterozygous; muscle biopsy at age 41 showed mtDNA maintenance abnormalities. | Original carrier ID: III\-8\-P5 | | 524 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV4 | Fam | M | ND | ND | ND | ND | Homo | / | / | / | ND | Original carrier ID: IV\-4\-Family\-A; affected individual used for exome sequencing | | 525 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV2 | Fam | F | ND | ND | ND | ND | Homo | / | / | / | ND | Original carrier ID: IV\-2\-Family\-B; affected individual used for exome sequencing | | 526 | 1555 | m.1555A\>G | A1555G\-F229 | A1555G\-F229\-P3 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Hearing\-impaired | A1555G; SSBP1 wild type; Unrelated carrier | | 527 | 1555 | m.1555A\>G | A1555G\-F230 | A1555G\-F230\-P1 | Fam | F | Y | Y | ND | 40 | Homo | / | / | / | SNHL suspected age 2 and diagnosed age 7; also SNHL | Original carrier ID: 004; Greek ancestry; streptomycin exposure; after streptomycin; sister | | 528 | 1555 | m.1555A\>G | A1555G\-F354 | A1555G\-F354\-P1 | Fam | F | Y | Y | ND | 44 | Homo | / | / | / | Multiple deaf | Original carrier ID: 011; English/Irish ancestry; streptomycin exposure after leg surgeries at ages 3\-5; family members; after streptomycin | | 529 | 1555 | m.1555A\>G | A1555G\-F355 | A1555G\-F355\-P1 | Fam | F | Y | Y | ND | 43 | Homo | / | / | / | Immediate SNHL; two maternal first cousins deaf | Original carrier ID: 014; Italian ancestry; after streptomycin for fever at age 3 | | 530 | 1555 | m.1555A\>G | A1555G\-F356 | A1555G\-F356\-P1 | Uninf | F | Y | Y | ND | 81 | Homo | / | / | / | Slowly progressive SNHL; constant tinnitus | Original carrier ID: 025; Mexican ancestry; after streptomycin at age 47; no family history; also C1525T reported | | 531 | 1555 | m.1555A\>G | A1555G\-F357 | A1555G\-F357\-P1 | Uninf | M | Y | Y | ND | 66 | Homo | / | / | / | Progressive SNHL diagnosed 17 years | Original carrier ID: 034; Puerto Rican ancestry; after streptomycin exposure; no family history | | 532 | 1555 | m.1555A\>G | A1555G\-F358 | A1555G\-F358\-P1 | Uninf | F | Y | Y | ND | 57 | Homo | / | / | / | Immediate tinnitus; progressive SNHL | Original carrier ID: 039; Chinese/Japanese/Caucasian ancestry; after streptomycin for pneumonia at age 17; no family history | | 533 | 1555 | m.1555A\>G | A1555G\-F359 | A1555G\-F359\-P1 | Fam | F | Y | Y | ND | 20 | Homo | / | / | / | SNHL | Original carrier ID: 040; Vietnamese ancestry; after streptomycin for malaria at age 4; brother; mother reported SNHL | | 534 | 1555 | m.1555A\>G | A1555G\-F231 | A1555G\-F231\-P1 | Fam | M | Y | Y | ND | 50 | / | / | / | / | Total deafness at age 45 | Original carrier ID: Patient\-1; streptomycin for lung tuberculosis; exposure in 1969 at age 23; family history positive | | 535 | 1555 | m.1555A\>G | A1555G\-F304 | A1555G\-F304\-P1 | Fam | F | Y | Y | ND | 62 | / | / | / | / | Total deafness at age 30 | Original carrier ID: Patient\-2; streptomycin for lung tuberculosis; exposure in 1957 at age 22; family history positive | | 536 | 1555 | m.1555A\>G | A1555G\-F305 | A1555G\-F305\-P1 | Fam | F | Y | Y | ND | 76 | / | / | / | / | Total deafness at age 75 | Original carrier ID: Patient\-3; isepamicin sulfate for pneumonia; kidney dysfunction; exposure at age 75; family history positive | | 537 | 1555 | m.1555A\>G | A1555G\-F306 | A1555G\-F306\-P1 | Fam | F | Y | Y | ND | 75 | / | / | / | / | Total deafness at age 65 | Original carrier ID: Patient\-4; streptomycin for skin eruption; exposure in 1967 at age 45; family history positive | | 538 | 1555 | m.1555A\>G | A1555G\-F7 | A1555G\-F7\-III3 | Fam | ND | N | ND | A | ND | Homo | / | / | / | ND | Original carrier ID: BJ105\-III\-3; matrilineal relative; aminoglycoside exposure reported for the affected relative group | | 539 | 1555 | m.1555A\>G | A1555G\-F20 | A1555G\-F20\-II4 | Fam | F | N | N | A | 52 | Homo | / | / | / | Healthy | Original carrier ID: WZ16\-II\-4; reported as normal in Table 1 | | 540 | 1555 | m.1555A\>G | A1555G\-F20 | A1555G\-F20\-III3 | Fam | F | N | Y | A | 18 | Homo | / | / | / | Visual impairment/LHON; moderate severity | Original carrier ID: WZ16\-III\-3; onset age 15; Table 1 | | 541 | 1555 | m.1555A\>G | A1555G\-F20 | A1555G\-F20\-III4 | Fam | F | Y | Y | A | 14 | Homo | / | / | / | Visual impairment/LHON; mild bilateral hearing impairment also described | Original carrier ID: WZ16\-III\-4; proband with | | 542 | 1555 | m.1555A\>G | A1555G\-F20 | A1555G\-F20\-III6 | Fam | M | N | Y | A | 11 | Homo | / | / | / | Visual impairment/LHON; moderate severity | Original carrier ID: WZ16\-III\-6; onset age 10; Table 1 | | 543 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-II6 | Fam | M | N | Y | A | ND | Homo | / | / | / | Profound hearing impairment | Original carrier ID: WZD8\-II\-6; aminoglycoside exposure | | 544 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-II4 | Fam | F | N | Y | A | ND | Homo | / | / | / | Severe hearing impairment | Original carrier ID: WZD8\-II\-4; no aminoglycoside exposure stated for this individual | | 545 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-II2 | Fam | F | N | Y | A | ND | Homo | / | / | / | Moderate hearing impairment | Original carrier ID: WZD8\-II\-2; no aminoglycoside exposure stated for this individual | | 546 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-II8 | Fam | F | N | Y | A | ND | Homo | / | / | / | Moderate hearing impairment | Original carrier ID: WZD8\-II\-8; no aminoglycoside exposure stated for this individual | | 547 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-II2 | Fam | F | N | Y | A | ND | Homo | / | / | / | Hearing impairment without reported | Original carrier ID: WZD9\-II\-2; aminoglycoside exposure | | 548 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III7 | Fam | F | N | Y | A | ND | Homo | / | / | / | Profound hearing impairment without reported | Original carrier ID: WZD9\-III\-7; aminoglycoside exposure | | 549 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III2 | Fam | F | N | Y | A | ND | Homo | / | / | / | Severe hearing impairment without reported | Original carrier ID: WZD9\-III\-2; aminoglycoside exposure | | 550 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III4 | Fam | F | N | Y | A | ND | Homo | / | / | / | Severe hearing impairment without reported | Original carrier ID: WZD9\-III\-4; aminoglycoside exposure | | 551 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-II2 | Fam | F | N | Y | A | ND | Homo | / | / | / | Profound hearing impairment | Original carrier ID: WZD10\-II\-2; after streptomycin exposure; exact pedigree label ambiguous in OCR text | | 552 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-III3 | Fam | F | N | Y | A | ND | Homo | / | / | / | Severe hearing impairment | Original carrier ID: WZD10\-III\-3; sister; no aminoglycoside exposure reported | | 553 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-IV1 | Fam | M | N | Y | A | ND | / | / | / | / | Severe hearing loss | Original carrier ID: WZD10\-IV\-1; Son; no aminoglycoside exposure reported | | 554 | 1555 | m.1555A\>G | A1555G\-F232 | A1555G\-F232\-V1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 103/99 dB; slope; profound | Original carrier ID: HA062\-V\-1; Aminoglycoside exposure reported | | 555 | 1555 | m.1555A\>G | A1555G\-F233 | A1555G\-F233\-IV12 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/93 dB; slope; profound | Original carrier ID: HA087\-IV\-12; Aminoglycoside exposure reported | | 556 | 1555 | m.1555A\>G | A1555G\-F234 | A1555G\-F234\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 93/100 dB; slope; profound | Original carrier ID: HA101\-III\-1; Aminoglycoside exposure reported | | 557 | 1555 | m.1555A\>G | A1555G\-F235 | A1555G\-F235\-IV2 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 108/103 dB; flat; profound | Original carrier ID: HA102\-IV\-2; Aminoglycoside exposure reported | | 558 | 1555 | m.1555A\>G | A1555G\-F236 | A1555G\-F236\-V3 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/99 dB; slope; profound | Original carrier ID: HA121\-V\-3; Aminoglycoside exposure reported | | 559 | 1555 | m.1555A\>G | A1555G\-F237 | A1555G\-F237\-III11 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/101 dB; slope; profound | Original carrier ID: HA146\-III\-11; Aminoglycoside exposure reported | | 560 | 1555 | m.1555A\>G | A1555G\-F238 | A1555G\-F238\-IV6 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 88/94 dB; slope; profound | Original carrier ID: HZ023\-IV\-6; Aminoglycoside exposure reported | | 561 | 1555 | m.1555A\>G | A1555G\-F239 | A1555G\-F239\-III12 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/98 dB; slope; profound | Original carrier ID: HZ142\-III\-12; Aminoglycoside exposure reported | | 562 | 1555 | m.1555A\>G | A1555G\-F240 | A1555G\-F240\-IV1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/88 dB; slope; profound | Original carrier ID: LC021\-IV\-1; Aminoglycoside exposure reported | | 563 | 1555 | m.1555A\>G | A1555G\-F241 | A1555G\-F241\-IV1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 101/92 dB; slope; profound | Original carrier ID: LC027\-IV\-1; Aminoglycoside exposure reported | | 564 | 1555 | m.1555A\>G | A1555G\-F242 | A1555G\-F242\-IV2 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 100/103 dB; slope; profound | Original carrier ID: LC063\-IV\-2; Aminoglycoside exposure reported | | 565 | 1555 | m.1555A\>G | A1555G\-F243 | A1555G\-F243\-IV9 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 97/103 dB; slope; profound | Original carrier ID: LS058\-IV\-9; Aminoglycoside exposure reported | | 566 | 1555 | m.1555A\>G | A1555G\-F244 | A1555G\-F244\-IV17 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 92/98 dB; audiogram U; profound | Original carrier ID: QZ036\-IV\-17; Aminoglycoside exposure reported | | 567 | 1555 | m.1555A\>G | A1555G\-F245 | A1555G\-F245\-IV7 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 88/97 dB; flat; profound | Original carrier ID: QZ069\-IV\-7; Aminoglycoside exposure reported | | 568 | 1555 | m.1555A\>G | A1555G\-F246 | A1555G\-F246\-IV5 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 97/97 dB; slope; profound | Original carrier ID: QZ085\-IV\-5; Aminoglycoside exposure reported | | 569 | 1555 | m.1555A\>G | A1555G\-F247 | A1555G\-F247\-IV5 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 106/105 dB; slope; profound | Original carrier ID: QZ097\-IV\-5; Aminoglycoside exposure reported | | 570 | 1555 | m.1555A\>G | A1555G\-F248 | A1555G\-F248\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 93/94 dB; slope; profound | Original carrier ID: QZ107\-IV\-1; Aminoglycoside exposure reported | | 571 | 1555 | m.1555A\>G | A1555G\-F249 | A1555G\-F249\-IV12 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/95 dB; slope; profound | Original carrier ID: RA036\-IV\-12; Aminoglycoside exposure reported | | 572 | 1555 | m.1555A\>G | A1555G\-F250 | A1555G\-F250\-III5 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 106/108 dB; slope; profound | Original carrier ID: SX003\-III\-5; Aminoglycoside exposure reported | | 573 | 1555 | m.1555A\>G | A1555G\-F251 | A1555G\-F251\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 93/92 dB; audiogram U; profound | Original carrier ID: SX012\-III\-1; Aminoglycoside exposure reported | | 574 | 1555 | m.1555A\>G | A1555G\-F252 | A1555G\-F252\-IV8 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 103/106 dB; slope; profound | Original carrier ID: SX052\-IV\-8; Aminoglycoside exposure reported | | 575 | 1555 | m.1555A\>G | A1555G\-F253 | A1555G\-F253\-IV5 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/102 dB; slope; profound | Original carrier ID: SX070\-IV\-5; Aminoglycoside exposure reported | | 576 | 1555 | m.1555A\>G | A1555G\-F254 | A1555G\-F254\-IV11 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 104/102 dB; slope; profound | Original carrier ID: SX072\-IV\-11; Aminoglycoside exposure reported | | 577 | 1555 | m.1555A\>G | A1555G\-F255 | A1555G\-F255\-IV3 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 100/102 dB; slope; profound | Original carrier ID: SX082\-IV\-3; Aminoglycoside exposure reported | | 578 | 1555 | m.1555A\>G | A1555G\-F256 | A1555G\-F256\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/103 dB; slope; profound | Original carrier ID: WC012\-IV\-1; Aminoglycoside exposure reported | | 579 | 1555 | m.1555A\>G | A1555G\-F257 | A1555G\-F257\-IV7 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 92/93 dB; profound | Original carrier ID: YJ042\-IV\-7; Aminoglycoside exposure reported; slope; homozygous GJB2 235delC reported | | 580 | 1555 | m.1555A\>G | A1555G\-F258 | A1555G\-F258\-IV8 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 105/109 dB; slope; profound | Original carrier ID: YJ052\-IV\-8; Aminoglycoside exposure reported | | 581 | 1555 | m.1555A\>G | A1555G\-F259 | A1555G\-F259\-III2 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 96/98 dB; audiogram U; profound | Original carrier ID: YQ016\-III\-2; Aminoglycoside exposure reported | | 582 | 1555 | m.1555A\>G | A1555G\-F260 | A1555G\-F260\-III2 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/108 dB; flat; profound | Original carrier ID: YQ021\-III\-2; Aminoglycoside exposure reported | | 583 | 1555 | m.1555A\>G | A1555G\-F261 | A1555G\-F261\-III2 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 93/91 dB; slope; profound | Original carrier ID: YQ023\-III\-2; Aminoglycoside exposure reported | | 584 | 1555 | m.1555A\>G | A1555G\-F262 | A1555G\-F262\-IV5 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 96/98 dB; audiogram U; profound | Original carrier ID: YQ052\-IV\-5; Aminoglycoside exposure reported; homozygous GJB2 235delC reported | | 585 | 1555 | m.1555A\>G | A1555G\-F263 | A1555G\-F263\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 99/101 dB; slope; profound | Original carrier ID: FE003\-IV\-1; Aminoglycoside exposure reported | | 586 | 1555 | m.1555A\>G | A1555G\-F264 | A1555G\-F264\-IV6 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 100/103 dB; flat; profound | Original carrier ID: FE008\-IV\-6; Aminoglycoside exposure reported | | 587 | 1555 | m.1555A\>G | A1555G\-F265 | A1555G\-F265\-III3 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 78/97 dB; flat; severe | Original carrier ID: HA167\-III\-3; Aminoglycoside exposure reported | | 588 | 1555 | m.1555A\>G | A1555G\-F266 | A1555G\-F266\-IV4 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 75/85 dB; flat; severe | Original carrier ID: HZ171\-IV\-4; Aminoglycoside exposure reported | | 589 | 1555 | m.1555A\>G | A1555G\-F267 | A1555G\-F267\-IV2 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 73/90 dB; slope; severe | Original carrier ID: LC07\-IV\-2; Aminoglycoside exposure reported | | 590 | 1555 | m.1555A\>G | A1555G\-F268 | A1555G\-F268\-III14 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 86/94 dB; slope; severe | Original carrier ID: LS099\-III\-14; Aminoglycoside exposure reported | | 591 | 1555 | m.1555A\>G | A1555G\-F269 | A1555G\-F269\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 79/88 dB; slope; severe | Original carrier ID: LS160\-III\-1; Aminoglycoside exposure reported | | 592 | 1555 | m.1555A\>G | A1555G\-F270 | A1555G\-F270\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 79/92 dB; slope; severe | Original carrier ID: LS192\-IV\-1; Aminoglycoside exposure reported | | 593 | 1555 | m.1555A\>G | A1555G\-F271 | A1555G\-F271\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 74/74 dB; audiogram U; severe | Original carrier ID: QZ081\-IV\-1; Aminoglycoside exposure reported | | 594 | 1555 | m.1555A\>G | A1555G\-F272 | A1555G\-F272\-III6 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 84/83 dB; slope; severe | Original carrier ID: RA035\-III\-6; Aminoglycoside exposure reported | | 595 | 1555 | m.1555A\>G | A1555G\-F273 | A1555G\-F273\-VI1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 78/74 dB; flat; severe | Original carrier ID: RA060\-VI\-1; Aminoglycoside exposure reported | | 596 | 1555 | m.1555A\>G | A1555G\-F274 | A1555G\-F274\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 56/93 dB; flat; severe | Original carrier ID: RA098\-IV\-1; Aminoglycoside exposure reported | | 597 | 1555 | m.1555A\>G | A1555G\-F275 | A1555G\-F275\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 71/83 dB; flat; severe | Original carrier ID: WC002\-IV\-1; Aminoglycoside exposure reported | | 598 | 1555 | m.1555A\>G | A1555G\-F276 | A1555G\-F276\-IV16 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 74/90 dB; slope; severe | Original carrier ID: YJ049\-IV\-16; Aminoglycoside exposure reported | | 599 | 1555 | m.1555A\>G | A1555G\-F277 | A1555G\-F277\-IV1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 88/73 dB; slope; severe | Original carrier ID: FE019\-IV\-1; Aminoglycoside exposure reported | | 600 | 1555 | m.1555A\>G | A1555G\-F278 | A1555G\-F278\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 89/83 dB; slope; severe | Original carrier ID: FE020\-III\-1; Aminoglycoside exposure reported | | 601 | 1555 | m.1555A\>G | A1555G\-F279 | A1555G\-F279\-IV6 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 70/68 dB; slope; moderate | Original carrier ID: QZ09\-IV\-6; Aminoglycoside exposure reported | | 602 | 1555 | m.1555A\>G | A1555G\-F280 | A1555G\-F280\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 56/61 dB; slope; moderate | Original carrier ID: FE036\-III\-1; Aminoglycoside exposure reported | | 603 | 1555 | m.1555A\>G | A1555G\-F281 | A1555G\-F281\-V7 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 8/62 dB; mild sudden/asymmetric hearing loss | Original carrier ID: SX018\-V\-7; Aminoglycoside exposure reported; flat; Table 3; separate note in text | | 604 | 1555 | m.1555A\>G | A1555G\-F282 | A1555G\-F282\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 68/77 dB; rising; severe | Original carrier ID: WZD1\-IV\-1; Aminoglycoside exposure reported | | 605 | 1555 | m.1555A\>G | A1555G\-F283 | A1555G\-F283\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 85/85 dB; rising; severe | Original carrier ID: WZD2\-IV\-1; Aminoglycoside exposure reported | | 606 | 1555 | m.1555A\>G | A1555G\-F284 | A1555G\-F284\-III1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 80/92 dB; slope; severe | Original carrier ID: WZD3\-III\-1; Aminoglycoside exposure reported | | 607 | 1555 | m.1555A\>G | A1555G\-F285 | A1555G\-F285\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 85/85 dB; slope; severe | Original carrier ID: WZD4\-III\-1; Aminoglycoside exposure reported | | 608 | 1555 | m.1555A\>G | A1555G\-F286 | A1555G\-F286\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 92/98 dB; slope; profound | Original carrier ID: WZD5\-III\-1; Aminoglycoside exposure reported | | 609 | 1555 | m.1555A\>G | A1555G\-F287 | A1555G\-F287\-III1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 103/105 dB; slope; profound | Original carrier ID: WZD6\-III\-1; Aminoglycoside exposure reported | | 610 | 1555 | m.1555A\>G | A1555G\-F288 | A1555G\-F288\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 102/103 dB; slope; profound | Original carrier ID: WZD7\-IV\-1; Aminoglycoside exposure reported | | 611 | 1555 | m.1555A\>G | A1555G\-F289 | A1555G\-F289\-III1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/98 dB; slope; profound | Original carrier ID: WZD8\-III\-1; Aminoglycoside exposure reported | | 612 | 1555 | m.1555A\>G | A1555G\-F290 | A1555G\-F290\-IV1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 97/100 dB; slope; profound | Original carrier ID: WZD9\-IV\-1; Aminoglycoside exposure reported | | 613 | 1555 | m.1555A\>G | A1555G\-F291 | A1555G\-F291\-III2 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 67/82 dB; slope; severe | Original carrier ID: WZD10\-III\-2; Aminoglycoside exposure reported | | 614 | 1555 | m.1555A\>G | A1555G\-F292 | A1555G\-F292\-III1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 98/98 dB; slope; profound | Original carrier ID: WZD31\-III\-1; Aminoglycoside exposure reported | | 615 | 1555 | m.1555A\>G | A1555G\-F293 | A1555G\-F293\-IV2 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 75/84 dB; flat; severe | Original carrier ID: WZD32\-IV\-2; Aminoglycoside exposure reported | | 616 | 1555 | m.1555A\>G | A1555G\-F294 | A1555G\-F294\-V1 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 102/98 dB; slope; profound | Original carrier ID: WZD33\-V\-1; Aminoglycoside exposure reported | | 617 | 1555 | m.1555A\>G | A1555G\-F295 | A1555G\-F295\-III2 | Uninf | M | Y | Y | A | ND | Homo | / | / | / | PTA R/L 105/109 dB; slope; profound | Original carrier ID: WZD34\-III\-2; Aminoglycoside exposure reported | | 618 | 1555 | m.1555A\>G | A1555G\-F296 | A1555G\-F296\-IV1 | Uninf | F | Y | Y | A | ND | Homo | / | / | / | PTA R/L 58/75 dB; slope; moderate | Original carrier ID: WZD11\-IV\-1; Aminoglycoside exposure reported | | 619 | 1555 | m.1555A\>G | A1555G\-F297 | A1555G\-F297\-P1 | Fam | ND | ND | Y | A | ND | / | / | / | Positive(H) | Hearing loss | Japanese AGD pedigree Family 1; aminoglycoside\-induced; affected relatives | | 620 | 1555 | m.1555A\>G | A1555G\-F298 | A1555G\-F298\-P1 | Fam | ND | ND | Y | A | ND | / | / | / | Positive(H) | Hearing loss | Japanese AGD pedigree Family 2; aminoglycoside\-induced; affected relatives | | 621 | 1555 | m.1555A\>G | A1555G\-F299 | A1555G\-F299\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Nonsyndromic hearing loss | Original carrier ID: Family\-3\-proband; maternally inherited | | 622 | 1555 | m.1555A\>G | A1555G\-F300 | A1555G\-F300\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Nonsyndromic hearing loss | Original carrier ID: Family\-4\-proband; maternally inherited | | 623 | 1555 | m.1555A\>G | A1555G\-F301 | A1555G\-F301\-P1 | Fam | ND | Y | Y | ND | ND | Homo | / | / | / | Nonsyndromic hearing loss | Original carrier ID: Family\-5\-proband; maternally inherited | | 624 | 1555 | m.1555A\>G | A1555G\-F302 | A1555G\-F302\-II1 | Fam | F | Y | Y | ND | 38 | / | / | / | / | sensorineural hearing impairment since childhood; moderate hearing loss | Original carrier ID: case\-1 | | 625 | 1555 | m.1555A\>G | A1555G\-F302 | A1555G\-F302\-II2 | Fam | F | N | N | ND | 18 | / | / | / | / | Healthy | | | 626 | 1555 | m.1555A\>G | A1555G\-F302 | A1555G\-F302\-II3 | Fam | F | N | N | ND | 12 | / | / | / | / | Healthy | | | 627 | 1555 | m.1555A\>G | A1555G\-F302 | A1555G\-F302\-II4 | Fam | F | N | N | ND | 4 | / | / | / | / | Healthy | | | 628 | 1555 | m.1555A\>G | A1555G\-F303 | A1555G\-F303\-P1 | Fam | M | Y | Y | ND | 41 | / | / | / | / | left\-sided moderate hearing loss | Original carrier ID: case\-2 | | 629 | 1555 | m.1555A\>G | A1555G\-F82 | A1555G\-F82\-II1 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Mild high\-frequency hearing impairment | | | 630 | 1555 | m.1555A\>G | A1555G\-F7 | A1555G\-F7\-II15 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Original carrier ID: BJ105\-II\-15; aminoglycoside exposure; matrilineal relative | | 631 | 1555 | m.1555A\>G | A1555G\-F185 | A1555G\-F185\-III2 | Fam | M | N | N | ND | 1 | 7\.25% | / | / | / | Healthy | | | 632 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-III2 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 633 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-III6 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 634 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-IV3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 635 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-IV9 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 636 | 1555 | m.1555A\>G | A1555G\-F55 | A1555G\-F55\-V9 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 637 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III4 | Fam | M | N | N | A | ND | Homo | / | / | / | Healthy | | | 638 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III6 | Fam | M | N | N | A | ND | Homo | / | / | / | Healthy | | | 639 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III7 | Fam | F | N | N | A | ND | Homo | / | / | / | Healthy | | | 640 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III9 | Fam | F | N | Y | A | 49 | Homo | / | / | / | Bilateral sensorineural deafness; two affected children. | | | 641 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III10 | Fam | F | N | N | A | ND | Homo | / | / | / | Healthy | | | 642 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-III12 | Fam | F | Y | Y | A | 54 | Homo | / | / | / | Bilateral sensorineural deafness since age 18 | no aminoglycoside exposure before onset | | 643 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV18 | Fam | M | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 644 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV19 | Fam | M | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 645 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV20 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 646 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV21 | Fam | M | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 647 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV26 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | child of III\-9 | | 648 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV27 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | child of III\-9 | | 649 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV28 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 650 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV29 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 651 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV30 | Fam | F | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 652 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV34 | Fam | M | N | ND | A | ND | Homo | / | / | / | ND | child of the proband; onset age within IV\-34 to IV\-38 group was 6\-14 years; no aminoglycoside exposure | | 653 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV35 | Fam | M | N | ND | A | ND | Homo | / | / | / | ND | child of the proband; onset age within IV\-34 to IV\-38 group was 6\-14 years; no aminoglycoside exposure | | 654 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV36 | Fam | M | N | ND | A | ND | Homo | / | / | / | ND | child of the proband; onset age within IV\-34 to IV\-38 group was 6\-14 years; no aminoglycoside exposure | | 655 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV37 | Fam | F | N | ND | A | ND | Homo | / | / | / | ND | child of the proband; onset age within IV\-34 to IV\-38 group was 6\-14 years; no aminoglycoside exposure | | 656 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-IV38 | Fam | F | N | ND | A | ND | Homo | / | / | / | ND | child of the proband; onset age within IV\-34 to IV\-38 group was 6\-14 years; no aminoglycoside exposure | | 657 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-V12 | Fam | M | N | Y | A | ND | Homo | / | / | / | Clinically deaf | homoplasmic A1555G by Figure 1 solid symbol | | 658 | 1555 | m.1555A\>G | A1555G\-F1 | A1555G\-F1\-V13 | Fam | F | N | N | A | ND | Homo | / | / | / | Healthy | | | 659 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-I2 | Uninf | F | N | N | ND | ND | / | / | / | / | Healthy | | | 660 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II2 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 661 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II3 | Fam | M | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 662 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II4 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | | | 663 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II5 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 664 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II6 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 665 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II7 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | | | 666 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II8 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 667 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II9 | Fam | F | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 668 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-II10 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 669 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III1 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 670 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III2 | Fam | M | N | Y | ND | ND | / | / | / | / | Profound hearing impairment diagnosed between ages 4 and 5 | brother of the two probands | | 671 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III3 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | | | 672 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III4 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | | | 673 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III5 | Fam | F | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 674 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III6 | Fam | M | Y | Y | ND | ND | / | / | / | / | Profound hearing impairment diagnosed between ages 4 and 5 | no aminoglycoside use in Family A; Figure 1 arrow proband | | 675 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III7 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | | | 676 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III8 | Fam | F | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 677 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III9 | Fam | F | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 678 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III10 | Fam | M | Y | Y | ND | ND | / | / | / | / | Profound hearing impairment diagnosed between ages 4 and 5 | no aminoglycoside use in Family A; Figure 1 arrow proband | | 679 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III11 | Fam | M | N | Y | ND | ND | / | / | / | / | High\-frequency hearing impairment | | | 680 | 1555 | m.1555A\>G | A1555G\-F116 | A1555G\-F116\-III12 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | | | 681 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-II2 | Fam | F | N | Y | ND | ND | / | / | / | / | Deafness | Streptomycin\-induced; onset age 30 | | 682 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-II3 | Fam | F | N | Y | ND | 69 | / | / | / | / | Bilateral deafness | without aminoglycoside history; onset age 60; muscle biopsy showed mitochondrial abnormalities | | 683 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-III1 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 684 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-III3 | Fam | F | N | Y | ND | ND | / | / | / | / | Bilateral deafness | without aminoglycoside history; onset age 45 | | 685 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-III7 | Fam | M | N | Y | ND | ND | / | / | / | / | Bilateral deafness | without aminoglycoside history; onset age 13 | | 686 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-IV3 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 687 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-IV4 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 688 | 1555 | m.1555A\>G | A1555G\-F181 | A1555G\-F181\-IV5 | Fam | F | N | Y | ND | ND | / | / | / | / | Bilateral deafness | without aminoglycoside history; onset age 18 | | 689 | 1555 | m.1555A\>G | A1555G\-F182 | A1555G\-F182\-II11 | Fam | F | N | Y | ND | ND | / | / | / | / | Sensorineural hearing loss of unknown cause; onset age 50\. | | | 690 | 1555 | m.1555A\>G | A1555G\-F182 | A1555G\-F182\-III15 | Fam | M | N | Y | ND | ND | / | / | / | / | Sensorineural hearing loss of unknown cause; onset age 8\. | | | 691 | 1555 | m.1555A\>G | A1555G\-F182 | A1555G\-F182\-III17 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 692 | 1555 | m.1555A\>G | A1555G\-F183 | A1555G\-F183\-II1 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 693 | 1555 | m.1555A\>G | A1555G\-F183 | A1555G\-F183\-III1 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | pedigree | | 694 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-IV41 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 695 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-IV43 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 696 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-IV46 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 697 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-IV47 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 698 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-V130 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 699 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-VI5 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 700 | 1555 | m.1555A\>G | A1555G\-F122 | A1555G\-F122\-VI6 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Affected patient in Nanjing family; individual severity/onset not separately reported | | 701 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II3 | Fam | M | ND | Y | A | 28 | 75\.26% | / | / | / | Sloping/high\-frequency sensorineural hearing loss | no reported aminoglycoside exposure | | 702 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II4 | Fam | M | ND | Y | A | 27 | 90\.43% | / | / | / | Moderate/severe high\-frequency sensorineural hearing loss | no reported aminoglycoside exposure | | 703 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II5 | Fam | F | ND | Y | A | 26 | 96\.60% | / | / | / | Moderate/severe high\-frequency sensorineural hearing loss | no reported aminoglycoside exposure | | 704 | 1555 | m.1555A\>G | A1555G\-F95 | A1555G\-F95\-II6 | Fam | M | ND | Y | A | 25 | 69\.78% | / | / | / | Moderate/severe high\-frequency sensorineural hearing loss | no reported aminoglycoside exposure | | 705 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II2 | Fam | M | ND | Y | A | 28 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with severe sensorineural hearing loss | | 706 | 1555 | m.1555A\>G | A1555G\-F97 | A1555G\-F97\-II4 | Fam | M | ND | Y | A | 22 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 707 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-II1 | Fam | M | ND | Y | A | 63 | 0 | / | / | / | Bilateral sensorineural hearing loss | peripheral blood tested negative for A1555G | | 708 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-II3 | Fam | F | ND | N | A | ND | 0 | / | / | / | Healthy | peripheral blood tested negative for A1555G | | 709 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-III2 | Fam | M | ND | Y | A | 28 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 710 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-III3 | Fam | F | ND | Y | A | 27 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 711 | 1555 | m.1555A\>G | A1555G\-F98 | A1555G\-F98\-III4 | Fam | M | ND | Y | A | 25 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 712 | 1555 | m.1555A\>G | A1555G\-F99 | A1555G\-F99\-II1 | Fam | F | ND | ND | A | ND | 100% | / | / | / | ND | Homoplasmic carrier; affected symbol in pedigree | | 713 | 1555 | m.1555A\>G | A1555G\-F99 | A1555G\-F99\-II2 | Fam | F | ND | ND | A | ND | 100% | / | / | / | ND | Homoplasmic carrier; affected symbol in pedigree | | 714 | 1555 | m.1555A\>G | A1555G\-F100 | A1555G\-F100\-II1 | Fam | M | ND | Y | A | 35 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 715 | 1555 | m.1555A\>G | A1555G\-F100 | A1555G\-F100\-II2 | Fam | M | ND | Y | A | 34 | 100% | / | / | / | Sensorineural hearing loss | Homoplasmic carrier with sensorineural hearing loss | | 716 | 1555 | m.1555A\>G | A1555G\-F307 | A1555G\-F307\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | Homo(ND) | Non\-syndromic sensorineural deafness; group I Makassar patient. | Original carrier ID: D12 | | 717 | 1555 | m.1555A\>G | A1555G\-F308 | A1555G\-F308\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | Homo(ND) | Non\-syndromic sensorineural deafness; group I Makassar patient. | Original carrier ID: D27 | | 718 | 1555 | m.1555A\>G | A1555G\-F309 | A1555G\-F309\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | / | Non\-syndromic sensorineural deafness; group I Makassar patient. | Original carrier ID: D65 | | 719 | 1555 | m.1555A\>G | A1555G\-F310 | A1555G\-F310\-P1 | Uninf | ND | Y | Y | ND | ND | / | / | / | / | Non\-syndromic sensorineural deafness; group I Makassar patient. | Original carrier ID: D75 | | 720 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-III2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 721 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-III8 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 722 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV1 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 723 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV4 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 724 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV5 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 725 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV7 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 726 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV9 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 727 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV10 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 728 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV11 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 729 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV12 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 730 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV26 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 731 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV33 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 732 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV34 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 733 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-IV35 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 734 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V1 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 735 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V2 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 736 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V7 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 737 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V11 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 738 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V12 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 739 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V15 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 740 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V16 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 741 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V17 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 742 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V18 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 743 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V19 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 744 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V21 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 745 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V23 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 746 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V24 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 747 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V26 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 748 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V27 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 749 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V50 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 750 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V51 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 751 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V52 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 752 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-V53 | Fam | ND | N | ND | ND | ND | Homo | / | / | / | ND | Phenotype unresolved/unknown symbol in Fig. 1 | | 753 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI2 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing loss and/or tinnitus phenotype indicated | detailed degree/onset summarized in Fig. 2; by Fig. 1 symbol | | 754 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI3 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 755 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI5 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 756 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI6 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 757 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI12 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 758 | 1555 | m.1555A\>G | A1555G\-F106 | A1555G\-F106\-VI13 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Fig. 1 open symbol | | 759 | 1555 | m.1555A\>G | A1555G\-F65 | A1555G\-F65\-IV1 | Fam | F | N | Y | ND | 38 | / | / | / | / | High\-frequency deafness. | | | 760 | 1555 | m.1555A\>G | A1555G\-F321 | A1555G\-F321\-IV1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Hearing impaired with | Original carrier ID: IV\-1\-Subject\-21; Fig.2 Family A proband IV\-1 / subject \#21; aminoglycoside exposure | | 761 | 1555 | m.1555A\>G | A1555G\-F322 | A1555G\-F322\-IV1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Hearing impaired with | Original carrier ID: IV\-1\-Subject\-63; Fig.2 Family B proband IV\-1 / subject \#63; aminoglycoside exposure | | 762 | 1555 | m.1555A\>G | A1555G\-F323 | A1555G\-F323\-III1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Hearing impaired with | Original carrier ID: III\-1\-Subject\-27; Fig.2 Family C proband III\-1 / subject \#27; aminoglycoside exposure | | 763 | 1555 | m.1555A\>G | A1555G\-F323 | A1555G\-F323\-III2 | Fam | M | Y | Y | ND | ND | Homo | / | / | / | Hearing impaired with | Original carrier ID: III\-2\-Subject\-9; Fig.2 Family C proband III\-2 / subject \#9; aminoglycoside exposure | | 764 | 1555 | m.1555A\>G | A1555G\-F324 | A1555G\-F324\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric profound hearing\-loss | Original carrier ID: Subject\-56; aminoglycoside\-induced; subject \#56 | | 765 | 1555 | m.1555A\>G | A1555G\-F325 | A1555G\-F325\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric nonsyndromic hearing\-loss | Original carrier ID: Subject\-131; subject \#131; no aminoglycoside exposure | | 766 | 1555 | m.1555A\>G | A1555G\-F326 | A1555G\-F326\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric profound hearing\-loss | Original carrier ID: Subject\-132; aminoglycoside\-induced; subject \#132 | | 767 | 1555 | m.1555A\>G | A1555G\-F327 | A1555G\-F327\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric profound hearing\-loss | Original carrier ID: Subject\-133; aminoglycoside\-induced; subject \#133 | | 768 | 1555 | m.1555A\>G | A1555G\-F328 | A1555G\-F328\-P1 | Uninf | ND | ND | Y | ND | ND | Homo | / | / | / | Pediatric profound hearing\-loss | Original carrier ID: Subject\-134; aminoglycoside\-induced; subject \#134 | | 769 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-II2 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 770 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-II4 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 771 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-II6 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 772 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III3 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 773 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III13 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; starred aminoglycoside\-exposed symbol, likely the duplicated III\-11 text mention | | 774 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III16 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 775 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III24 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 776 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-III29 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 777 | 1555 | m.1555A\>G | A1555G\-F10 | A1555G\-F10\-IV10 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ107 Figure 1; no individual age/severity reported | | 778 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-I2 | Uninf | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 779 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-II1 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 780 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-II5 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 781 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-II9 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; starred aminoglycoside\-exposed symbol | | 782 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-III2 | Fam | F | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 783 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-III3 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 784 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-III14 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; starred aminoglycoside\-exposed symbol | | 785 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-IV1 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 786 | 1555 | m.1555A\>G | A1555G\-F11 | A1555G\-F11\-IV2 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ108 Figure 1; no individual age/severity reported | | 787 | 1555 | m.1555A\>G | A1555G\-F12 | A1555G\-F12\-III6 | Fam | M | N | ND | ND | ND | Homo | / | / | / | ND | Affected matrilineal relative in BJ109 Figure 1; filled but not individually described in text | | 788 | 1555 | m.1555A\>G | A1555G\-F21 | A1555G\-F21\-III6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Moderate hearing impairment | Carrying G11696A; sloping\-shaped audiometric pattern; no aminoglycoside exposure reported | | 789 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-II2 | Fam | M | ND | Y | A | 55 | 81\.1% | / | / | / | Mild hearing loss | onset 30 years; aminoglycoside exposure | | 790 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III1 | Fam | M | ND | Y | A | 32 | 89\.4% | / | / | / | Severe hearing loss | onset 9 years; aminoglycoside exposure | | 791 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III2 | Fam | F | ND | Y | A | 29 | 91\.1% | / | / | / | Profound hearing loss | onset 5 years; aminoglycoside exposure | | 792 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III3 | Fam | M | ND | Y | A | 32 | 96\.1% | / | / | 95\.7%(saliva) | Severe hearing loss | onset 9 years; aminoglycoside exposure | | 793 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III4 | Fam | M | ND | N | A | 20 | 1\.4% | / | / | 2\.3%(saliva) | Healthy | | | 794 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III5 | Fam | F | ND | N | A | 35 | 5\.1% | / | / | 10\.6%(saliva) | Healthy | | | 795 | 1555 | m.1555A\>G | A1555G\-F102 | A1555G\-F102\-III6 | Fam | F | ND | Y | A | 32 | 6\.1% | / | / | 11\.8%(saliva) | Moderate unilateral hearing loss; onset unknown | | | 796 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II2 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing A1555G\-positive brother/relative in unlabelled Fig.1B | | 797 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II3 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired | A1555G\-positive brother/relative; aminoglycoside marker visible in Fig.1B | | 798 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing A1555G\-positive sister/relative in unlabelled Fig.1B | | 799 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II5 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired symbol | Original carrier ID: P1\-YoungerRelative\-1; A1555G\-positive younger relative/nephew in Fig.1B | | 800 | 1555 | m.1555A\>G | A1555G\-F107 | A1555G\-F107\-II6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired symbol | Original carrier ID: P1\-YoungerRelative\-2; A1555G\-positive younger relative in Fig.1B | | 801 | 1555 | m.1555A\>G | A1555G\-F108 | A1555G\-F108\-II2 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing mother carrying A1555G | | 802 | 1555 | m.1555A\>G | A1555G\-F108 | A1555G\-F108\-II3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing sister carrying A1555G | | 803 | 1555 | m.1555A\>G | A1555G\-F108 | A1555G\-F108\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing daughter carrying A1555G | | 804 | 1555 | m.1555A\>G | A1555G\-F109 | A1555G\-F109\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | A1555G\-positive daughter with SNHL after aminoglycoside exposure | | 805 | 1555 | m.1555A\>G | A1555G\-F109 | A1555G\-F109\-II3 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Sensorineural hearing loss | A1555G\-positive daughter with SNHL | | 806 | 1555 | m.1555A\>G | A1555G\-F109 | A1555G\-F109\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | | | 807 | 1555 | m.1555A\>G | A1555G\-F110 | A1555G\-F110\-II2 | Fam | M | N | Y | ND | 3 | Homo | / | / | / | Severely hearing\-impaired | Original carrier ID: TwinSon\-1; aminoglycoside exposure; neonatal; twin son | | 808 | 1555 | m.1555A\>G | A1555G\-F110 | A1555G\-F110\-II3 | Fam | M | N | Y | ND | 3 | Homo | / | / | / | Severely hearing\-impaired | Original carrier ID: TwinSon\-2; aminoglycoside exposure; neonatal; twin son | | 809 | 1555 | m.1555A\>G | A1555G\-F111 | A1555G\-F111\-II2 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing daughter carrying A1555G | | 810 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II2 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired; one had severe bilateral loss | brother; after streptomycin | | 811 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II3 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired carrying | A1555G; brother | | 812 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing sister carrying A1555G | | 813 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II5 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing son carrying A1555G | | 814 | 1555 | m.1555A\>G | A1555G\-F112 | A1555G\-F112\-II6 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Normal\-hearing son carrying A1555G | | 815 | 1555 | m.1555A\>G | A1555G\-F113 | A1555G\-F113\-II2 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | Normal\-hearing son of confirmed A1555G\-positive mother; not molecularly tested | | 816 | 1555 | m.1555A\>G | A1555G\-F113 | A1555G\-F113\-II3 | Fam | M | N | N | ND | ND | / | / | / | / | Healthy | Normal\-hearing son of confirmed A1555G\-positive mother; not molecularly tested | | 817 | 1555 | m.1555A\>G | A1555G\-F114 | A1555G\-F114\-II2 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired carrying | A1555G; brother | | 818 | 1555 | m.1555A\>G | A1555G\-F114 | A1555G\-F114\-II3 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired carrying | A1555G; brother | | 819 | 1555 | m.1555A\>G | A1555G\-F114 | A1555G\-F114\-II4 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired carrying | A1555G; brother | | 820 | 1555 | m.1555A\>G | A1555G\-F114 | A1555G\-F114\-II5 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing\-impaired carrying | A1555G; brother | | 821 | 1555 | m.1555A\>G | A1555G\-F53 | A1555G\-F53\-II2 | Fam | ND | N | N | ND | ND | / | / | / | Homo(ND) | Healthy | Normally hearing; no aminoglycoside exposure | | 822 | 1555 | m.1555A\>G | A1555G\-F53 | A1555G\-F53\-II3 | Fam | ND | N | N | ND | ND | / | / | / | Homo(ND) | Healthy | Normally hearing; no aminoglycoside exposure | | 823 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Moderate hearing loss | without aminoglycoside exposure; G15927A modifier | | 824 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-III2 | Fam | M | N | Y | ND | ND | Homo | / | / | Homo(ND) | Severe hearing loss | without aminoglycoside exposure; G15927A modifier | | 825 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-III4 | Fam | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Moderate hearing loss | without aminoglycoside exposure; G15927A modifier | | 826 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Severe hearing loss | without aminoglycoside exposure; G15927A modifier | | 827 | 1555 | m.1555A\>G | A1555G\-F67 | A1555G\-F67\-III4 | Fam | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Moderate hearing loss | without aminoglycoside exposure; G15927A modifier | | 828 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Profound hearing loss | Gentamicin exposure; after gentamycin exposure; G15927A modifier | | 829 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III2 | Fam | F | N | N | ND | 30 | Homo | / | / | / | Healthy | | | 830 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III3 | Fam | M | N | Y | ND | 32 | Homo | / | / | / | Profound hearing loss | Aminoglycoside exposure reported; onset age 6; aminoglycoside: No | | 831 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III5 | Fam | M | N | N | ND | 29 | Homo | / | / | / | Healthy | | | 832 | 1555 | m.1555A\>G | A1555G\-F26 | A1555G\-F26\-III10 | Fam | F | N | Y | ND | 35 | Homo | / | / | / | Profound hearing loss | Aminoglycoside exposure reported; onset age 1; aminoglycoside: Yes | | 833 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-3 | | 834 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III4 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-4 | | 835 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III5 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-5 | | 836 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III7 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-7 | | 837 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-II4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-II\-4 | | 838 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III5 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-III\-5 | | 839 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III6 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-III\-6 | | 840 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-III8 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-III\-8 | | 841 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-IV2 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-IV\-2 | | 842 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-IV3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-IV\-3 | | 843 | 1555 | m.1555A\>G | A1555G\-F23 | A1555G\-F23\-IV4 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD9\-IV\-4 | | 844 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-II3 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD10\-II\-3 | | 845 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-III4 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD10\-III\-4 | | 846 | 1555 | m.1555A\>G | A1555G\-F24 | A1555G\-F24\-IV2 | Fam | F | N | N | ND | ND | / | / | / | / | Healthy | Original carrier ID: WZD10\-IV\-2 | | 847 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-I2 | Uninf | F | N | Y | ND | ND | Homo | / | / | Homo(ND) | Hearing impaired by filled | pedigree symbol; no age/PTA reported; G15927A modifier | | 848 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-II3 | Fam | M | N | Y | ND | ND | Homo | / | / | Homo(ND) | Hearing impaired by filled | pedigree symbol; no age/PTA reported; G15927A modifier | | 849 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-II4 | Fam | M | N | Y | ND | ND | Homo | / | / | Homo(ND) | Hearing impaired by filled | pedigree symbol; no age/PTA reported; G15927A modifier | | 850 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-II5 | Fam | M | N | N | ND | ND | Homo | / | / | Homo(ND) | Healthy | pedigree symbol; no age/PTA reported; G15927A modifier | | 851 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-III2 | Fam | F | N | Y | ND | 35 | Homo | / | / | Homo(ND) | Profound hearing loss; ridge audiogram | aminoglycoside exposure; onset age 3; G15927A modifier | | 852 | 1555 | m.1555A\>G | A1555G\-F120 | A1555G\-F120\-IV3 | Fam | F | N | N | ND | ND | Homo | / | / | Homo(ND) | Healthy | pedigree symbol; no age/PTA reported; G15927A modifier | | 853 | 1555 | m.1555A\>G | A1555G\-F133 | A1555G\-F133\-II1 | Fam | F | N | Y | ND | ND | / | / | / | / | Hearing impairment | specific phenotype not individualized; by Fig. 1A affected symbol | | 854 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-II2 | Fam | F | N | Y | A | 44 years; onset ND | Homo | / | / | / | moderate hearing loss; PTA L63/R52; sloping audiogram | Carrying A4317G | | 855 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-II3 | Fam | M | N | Y | A | 47 years; onset 5 years | Homo | / | / | / | Severe hearing loss; PTA L85/R88; sloping audiogram | Carrying A4317G; aminoglycoside history | | 856 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-II6 | Fam | F | N | Y | A | 55 years; onset ND | Homo | / | / | / | moderate hearing loss; PTA L41/R41; sloping audiogram | Carrying A4317G | | 857 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-II8 | Fam | F | N | Y | A | 52 years; onset 2 years | Homo | / | / | / | moderate hearing loss; PTA L83/R53; trough audiogram | Carrying A4317G | | 858 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III1 | Fam | M | N | Y | A | 24 years; onset ND | Homo | / | / | / | moderate hearing loss; PTA L59/R58; sloping audiogram | Carrying A4317G | | 859 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III7 | Fam | M | N | Y | A | 34 years; onset ND | Homo | / | / | / | severe hearing loss; PTA L71/R73; sloping audiogram | Carrying A4317G | | 860 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III9 | Fam | M | N | Y | A | 32 years; onset ND | Homo | / | / | / | moderate hearing loss; PTA L55/R61; sloping audiogram | Carrying A4317G | | 861 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III12 | Fam | F | N | Y | A | 29 years; onset ND | Homo | / | / | / | moderate hearing loss; PTA L58/R65; trough audiogram | Carrying A4317G | | 862 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III13 | Fam | M | N | Y | A | 15 years; onset 0 years | Homo | / | / | / | Extremely severe hearing loss; PTA L88/R97; sloping audiogram | Carrying A4317G; aminoglycoside history | | 863 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III15 | Fam | F | N | Y | A | 30 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L40/R35; flat audiogram | Carrying A4317G | | 864 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III16 | Fam | F | N | Y | A | 27 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L43/R32; notched audiogram | Carrying A4317G | | 865 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III17 | Fam | F | N | Y | A | 23 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L42/R31; notched audiogram | Carrying A4317G | | 866 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III18 | Fam | F | N | Y | A | 19 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L31/R29; sloping audiogram | Carrying A4317G | | 867 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-III19 | Fam | F | N | N | D | ND | Homo | / | / | / | Healthy | Fig. 1; death slash shown | | 868 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-IV5 | Fam | M | N | N | A | ND | Homo | / | / | / | Healthy | Fig. 1 | | 869 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-IV6 | Fam | F | N | Y | A | 10 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L30/R33; flat audiogram | Carrying A4317G | | 870 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-IV7 | Fam | M | N | N | A | ND | Homo | / | / | / | Healthy | Fig. 1 | | 871 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-IV8 | Fam | F | N | Y | A | 9 years; onset ND | Homo | / | / | / | mild hearing loss; PTA L32/R33; flat audiogram | Carrying A4317G | | 872 | 1555 | m.1555A\>G | A1555G\-F63 | A1555G\-F63\-IV9 | Fam | F | N | N | A | ND | Homo | / | / | / | Healthy | Fig. 1 | | 873 | 1555 | m.1555A\>G | A1555G\-F329 | A1555G\-F329\-P1 | Uninf | F | Y | Y | ND | onset 1\-16 years | / | / | / | Homo(ND) | Skeletal myopathy; hypertrophic cardiomyopathy; deafness | CNS dementia; white\-matter MRI; RRF/COX\-negative/lipidosis muscle histology; normal respiratory\-chain analysis in muscle | | 874 | 1555 | m.1555A\>G | A1555G\-F330 | A1555G\-F330\-P2 | Uninf | M | Y | Y | ND | onset \<1 year | / | / | / | Homo(ND) | psychomotor retardation; skeletal myopathy/hypotonia; intrauterine and postnatal growth failure; normal brain MRI; increased lactate/pyruvate; decreased CI/CIII/CIV/CV in muscle/fibroblasts | | | 875 | 1555 | m.1555A\>G | A1555G\-F331 | A1555G\-F331\-P3 | Uninf | M | Y | Y | ND | onset 1\-16 years | / | / | / | Homo(ND) | optic atrophy; peripheral neuropathy; postnatal growth failure; cerebellar atrophy; normal respiratory\-chain analysis in muscle | | | 876 | 1555 | m.1555A\>G | A1555G\-F332 | A1555G\-F332\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: FE451\-proband; arrowed affected proband in Supplement Fig. S1 | | 877 | 1555 | m.1555A\>G | A1555G\-F333 | A1555G\-F333\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: FE386\-proband; arrowed affected proband in Supplement Fig. S1 | | 878 | 1555 | m.1555A\>G | A1555G\-F334 | A1555G\-F334\-P1 | Fam | F | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL013\-proband; arrowed affected proband in Supplement Fig. S1; aminoglycoside exposure history; asterisk indicates | | 879 | 1555 | m.1555A\>G | A1555G\-F335 | A1555G\-F335\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL157\-proband; arrowed affected proband in Supplement Fig. S1 | | 880 | 1555 | m.1555A\>G | A1555G\-F336 | A1555G\-F336\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL041\-proband; arrowed affected proband in Supplement Fig. S1; aminoglycoside exposure history; asterisk indicates | | 881 | 1555 | m.1555A\>G | A1555G\-F337 | A1555G\-F337\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: ZX077\-proband; arrowed affected proband in Supplement Fig. S1 | | 882 | 1555 | m.1555A\>G | A1555G\-F338 | A1555G\-F338\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: FE487\-proband; arrowed affected proband in Supplement Fig. S1 | | 883 | 1555 | m.1555A\>G | A1555G\-F339 | A1555G\-F339\-P1 | Fam | F | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL008\-proband; arrowed affected proband in Supplement Fig. S1 | | 884 | 1555 | m.1555A\>G | A1555G\-F340 | A1555G\-F340\-P1 | Fam | F | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL077\-proband; arrowed affected proband in Supplement Fig. S1; aminoglycoside exposure history; asterisk indicates | | 885 | 1555 | m.1555A\>G | A1555G\-F341 | A1555G\-F341\-P1 | Fam | M | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WL108\-proband; arrowed affected proband in Supplement Fig. S1; aminoglycoside exposure history; asterisk indicates | | 886 | 1555 | m.1555A\>G | A1555G\-F342 | A1555G\-F342\-P1 | Fam | F | Y | Y | ND | ND | Positive | / | / | / | Nonsyndromic hearing loss | Original carrier ID: WLT173\-proband; arrowed affected proband in Supplement Fig. S1 | | 887 | 1555 | m.1555A\>G | A1555G\-F343 | A1555G\-F343\-P1 | Fam | M | Y | Y | ND | 3 years; onset 3 years | Homo | / | / | / | SNHL; severe/severe; tinnitus Yes; vertigo No; diabetes No | steeply sloping/steeply sloping; progression Yes; speech discrimination not tested; DPOAE not tested; criteria Yes | | 888 | 1555 | m.1555A\>G | A1555G\-F344 | A1555G\-F344\-P1 | Fam | F | Y | Y | ND | 27 years; onset 21 years | Homo | / | / | / | SNHL; moderate/moderate; steeply sloping/steeply sloping; progression Yes; tinnitus Yes; vertigo No; diabetes No; speech discrimination 55%/55%; DPOAE no response except 1 kHz; criteria Yes | | | 889 | 1555 | m.1555A\>G | A1555G\-F345 | A1555G\-F345\-P1 | Fam | M | Y | Y | ND | 13 years; onset 13 years | Homo | / | / | / | SNHL; severe/severe; steeply sloping/steeply sloping; progression Yes; tinnitus Yes; vertigo No; diabetes No; speech discrimination 20%/55%; bilateral DPOAE no response; criteria Yes | | | 890 | 1555 | m.1555A\>G | A1555G\-F346 | A1555G\-F346\-P1 | Uninf | F | Y | Y | ND | 46 years; onset 0 years | Homo | / | / | / | SNHL; severe/severe; tinnitus No; vertigo No; diabetes No | gently sloping/steeply sloping; progression Yes; speech discrimination 80%/80%; DPOAE not tested; criteria No | | 891 | 1555 | m.1555A\>G | A1555G\-F347 | A1555G\-F347\-P1 | Uninf | M | ND | Y | ND | 33 | Homo | / | / | / | Visual loss present; constipation reported | Original carrier ID: ID018; ID\-group subject; predisposed to aminoglycoside ototoxicity | | 892 | 1555 | m.1555A\>G | A1555G\-F348 | A1555G\-F348\-P1 | Uninf | F | ND | Y | ND | 43 | Homo | / | / | / | Epilepsy and constipation reported | Original carrier ID: A043; ASD\-group subject; predisposed to aminoglycoside ototoxicity | | 893 | 1555 | m.1555A\>G | A1555G\-F349 | A1555G\-F349\-P1 | Uninf | F | ND | Y | ND | 54 | Homo | / | / | / | Deafness present; epilepsy and constipation reported | Original carrier ID: A054; ASD\-group subject | | 894 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-I2 | Uninf | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired maternal ancestor | Original carrier ID: WZD91\-I\-2; Carrying A4317G; WZD91 | | 895 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-II2 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-II\-2; Carrying A4317G; matrilineal relative | | 896 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-II3 | Fam | M | N | Y | ND | 47 | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-II\-3; Carrying A4317G; matrilineal relative; cell\-line donor age 47 | | 897 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-II6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-II\-6; Carrying A4317G; matrilineal relative | | 898 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-II8 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-II\-8; Carrying A4317G; matrilineal relative | | 899 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III7 | Fam | M | N | Y | ND | 34 | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-7; Carrying A4317G; matrilineal relative; cell\-line donor age 34 | | 900 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III12 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-12; Carrying A4317G; matrilineal relative | | 901 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III13 | Fam | M | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-13; Carrying A4317G; matrilineal relative | | 902 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III15 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-15; Carrying A4317G; matrilineal relative | | 903 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III19 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-19; Carrying A4317G; matrilineal relative | | 904 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III20 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-III\-20; Carrying A4317G; matrilineal relative | | 905 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-III21 | Fam | F | N | N | D | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-III\-21; Carrying A4317G; matrilineal relative | | 906 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV6 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-IV\-6; Carrying A4317G; matrilineal relative | | 907 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV7 | Fam | F | N | Y | ND | ND | Homo | / | / | / | Hearing impaired WZD91 | Original carrier ID: WZD91\-IV\-7; Carrying A4317G; matrilineal relative | | 908 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV8 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-IV\-8; Carrying A4317G | | 909 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV9 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-IV\-9; Carrying A4317G | | 910 | 1555 | m.1555A\>G | A1555G\-F118 | A1555G\-F118\-IV11 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD91\-IV\-11; Carrying A4317G | | 911 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-II8 | Fam | F | Y | Y | ND | ND | Positive | / | / | / | Severe aminoglycoside\-associated hearing loss | Original carrier ID: WZD92\-II\-8; WZD92 proband sequenced by whole mtDNA analysis | | 912 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-III14 | Fam | M | N | Y | ND | ND | / | / | / | / | Severe aminoglycoside\-associated hearing loss in target\-positive maternal line | Original carrier ID: WZD92\-III\-14; individual molecular load not reported; WZD92 | | 913 | 1555 | m.1555A\>G | A1555G\-F119 | A1555G\-F119\-III19 | Fam | M | N | Y | ND | ND | / | / | / | / | Severe aminoglycoside\-associated hearing loss in target\-positive maternal line | Original carrier ID: WZD92\-III\-19; individual molecular load not reported; WZD92 | | 914 | 1555 | m.1555A\>G | A1555G\-F350 | A1555G\-F350\-P1 | Uninf | ND | Y | Y | ND | ND | Homo | / | / | \~90%(F) | Non\-syndromic hearing loss; cochlear implantation. | Original carrier ID: A1555G\-1 | | 915 | 1555 | m.1555A\>G | A1555G\-F351 | A1555G\-F351\-P1 | Uninf | ND | Y | Y | ND | ND | Homo | / | / | \~90%(F) | Non\-syndromic hearing loss; cochlear implantation. | Original carrier ID: A1555G\-2 | | 916 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III1 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 917 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III2 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | | | 918 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III3 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 919 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III4 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 920 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III7 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 921 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III9 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | | | 922 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III11 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | | | 923 | 1555 | m.1555A\>G | A1555G\-F225 | A1555G\-F225\-III12 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | | | 924 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV1 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-1\-Family\-A | | 925 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV2 | Fam | F | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-2\-Family\-A | | 926 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV3 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-3\-Family\-A | | 927 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV5 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-5\-Family\-A | | 928 | 1555 | m.1555A\>G | A1555G\-F226 | A1555G\-F226\-IV6 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-6\-Family\-A | | 929 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV1 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-1\-Family\-B | | 930 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV3 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-3\-Family\-B | | 931 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV4 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-4\-Family\-B | | 932 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV5 | Fam | M | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-5\-Family\-B | | 933 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV6 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-6\-Family\-B | | 934 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV7 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-7\-Family\-B | | 935 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV8 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-8\-Family\-B | | 936 | 1555 | m.1555A\>G | A1555G\-F227 | A1555G\-F227\-IV9 | Fam | M | ND | Y | ND | ND | Homo | / | / | / | Hearing loss | Original carrier ID: IV\-9\-Family\-B | | 937 | 1555 | m.1555A\>G | A1555G\-F228 | A1555G\-F228\-IV1 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-1\-Family\-C | | 938 | 1555 | m.1555A\>G | A1555G\-F228 | A1555G\-F228\-IV2 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-2\-Family\-C | | 939 | 1555 | m.1555A\>G | A1555G\-F228 | A1555G\-F228\-IV3 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-3\-Family\-C | | 940 | 1555 | m.1555A\>G | A1555G\-F228 | A1555G\-F228\-IV4 | Fam | F | ND | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: IV\-4\-Family\-C | | 941 | 1555 | m.1555A\>G | A1555G\-F83 | A1555G\-F83\-I1 | Uninf | F | ND | Y | ND | ND | Homo | / | / | / | Severe hearing impairment | Gentamicin exposure; after gentamycin exposure | | 942 | 1555 | m.1555A\>G | A1555G\-F83 | A1555G\-F83\-I2 | Uninf | F | ND | Y | ND | ND | Homo | / | / | / | Severe hearing impairment | Gentamicin exposure; after gentamycin exposure | | 943 | 1555 | m.1555A\>G | A1555G\-F84 | A1555G\-F84\-P1 | Uninf | ND | Y | Y | ND | ND | Homo | / | / | / | Severe hearing impairment | Aminoglycoside exposure reported; aminoglycoside exposure | For abbreviations and other usage instructions, please see [Mitofam Info](https://mitofam.com/doc/385/).
Haoying Chen
2026年6月30日 10:52
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