A1555G-F66

**Figure 1\. Pedigree diagram for family A1555G\-F66\.**

![](/media/202607/A1555G-F66-pedigree_20260701022338271133.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\-F66 | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 4 | 7 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD31; with G15927A |

The **m.1555A\>G** variant in MT\-RNR1 was reported in family A1555G\-F66 from China with aminoglycoside\-induced and nonsyndromic hearing loss. The pedigree record reported 4 unaffected and 7 affected maternal relatives, and the carrier table includes 8 listed carriers. Homoplasmy was reported in all listed carriers; 8/8 carriers were affected, and the main clinical manifestation among affected carriers was profound hearing loss, severe hearing loss, moderate 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\-F66 | A1555G\-F66\-III1 | Fam | F | Y | Y | ND | ND | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 3 |
| 2 | 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 |
| 3 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-I2 | Uninf | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 4 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-II4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 5 | 1555 | m.1555A\>G | A1555G\-F66 | A1555G\-F66\-II9 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 6 | 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 |
| 7 | 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 |
| 8 | 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 |

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