A1555G-F68

**Figure 1\. Pedigree diagram for family A1555G\-F68\.**

![](/media/202607/A1555G-F68-pedigree_20260701022355913334.png#size=100%&align=left)

# Pedigree Information

| **No.** | **Position** | **Variant** | **Family ID** | **Nationality** | **Diseases** | **Unaffected Maternal Relatives** | **Affected Maternal Relatives** | **Publication Year** | **PMID/Ref** | **Note** |
| --- | --- | --- | --- | --- | --- | --- | --- | --- | --- | --- |
| 1 | 1555 | m.1555A\>G | A1555G\-F68 | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 13 | 13 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD33; with G15927A |

The **m.1555A\>G** variant in MT\-RNR1 was reported in family A1555G\-F68 from China with aminoglycoside\-induced and nonsyndromic hearing loss. The pedigree record reported 13 unaffected and 13 affected maternal relatives, and the carrier table includes 14 listed carriers. Homoplasmy was reported in all listed carriers; 14/14 carriers were affected, and the main clinical manifestation among affected carriers was profound hearing loss, severe hearing loss, moderate hearing loss, mild hearing loss.

# 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\-F68 | A1555G\-F68\-V1 | Fam | M | Y | Y | ND | 12 | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 2 |
| 2 | 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 |
| 3 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure |
| 4 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV15 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure |
| 5 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV17 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Profound hearing loss | without aminoglycoside exposure |
| 6 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-II2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 7 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 8 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III8 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 9 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-III11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 10 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 11 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV2 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 12 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV5 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 13 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV9 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 14 | 1555 | m.1555A\>G | A1555G\-F68 | A1555G\-F68\-IV14 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure |

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