A1555G-F22

**Figure 1\. Pedigree diagram for family A1555G\-F22\.**

![](/media/202607/A1555G-F22-pedigree_20260701021702468002.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\-F22 | China | Aminoglycoside\-induced and nonsyndromic hearing impairment | 8 | 4 | 2008 | [18386806](https://pubmed.ncbi.nlm.nih.gov/18386806/) | Family WZD8 |

The **m.1555A\>G** variant in MT\-RNR1 was reported in family A1555G\-F22 from China with aminoglycoside\-induced and nonsyndromic hearing impairment. The pedigree record reported 8 unaffected and 4 affected maternal relatives, and the carrier table includes 9 listed carriers. Homoplasmy was reported in all listed carriers; 5/9 carriers were affected, and the main clinical manifestation among affected carriers was profound bilateral hearing impairment, pta about 98, 98 db, profound hearing impairment, severe hearing impairment, moderate hearing impairment.

# 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\-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 |
| 2 | 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 |
| 3 | 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 |
| 4 | 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 |
| 5 | 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 |
| 6 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III3 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-3 |
| 7 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III4 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-4 |
| 8 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III5 | Fam | F | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-5 |
| 9 | 1555 | m.1555A\>G | A1555G\-F22 | A1555G\-F22\-III7 | Fam | M | N | N | ND | ND | Homo | / | / | / | Healthy | Original carrier ID: WZD8\-III\-7 |

For abbreviations and other usage instructions, please see [Mitofam Info](https://mitofam.com/doc/385/).