A1555G-F69

**Figure 1\. Pedigree diagram for family A1555G\-F69\.**

![](/media/202607/A1555G-F69-pedigree_20260701022404687374.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\-F69 | China | Aminoglycoside\-induced and nonsyndromic hearing loss | 9 | 9 | 2008 | [18820594](https://pubmed.ncbi.nlm.nih.gov/18820594/) | Family WZD34; with G15927A |

The **m.1555A\>G** variant in MT\-RNR1 was reported in family A1555G\-F69 from China with aminoglycoside\-induced and nonsyndromic hearing loss. The pedigree record reported 9 unaffected and 9 affected maternal relatives, and the carrier table includes 10 listed carriers. Homoplasmy was reported in all listed carriers; 10/10 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\-F69 | A1555G\-F69\-III2 | Fam | M | Y | Y | ND | 15 | Homo | / | / | / | Profound hearing loss | after gentamycin exposure at age 4 |
| 2 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II4 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Severe hearing loss | without aminoglycoside exposure |
| 3 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II3 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 4 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II11 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 5 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III5 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 6 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III12 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Moderate hearing loss | without aminoglycoside exposure |
| 7 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-II6 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure |
| 8 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III7 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure |
| 9 | 1555 | m.1555A\>G | A1555G\-F69 | A1555G\-F69\-III14 | Fam | ND | N | Y | ND | ND | Homo | / | / | / | Mild hearing loss | without aminoglycoside exposure |
| 10 | 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 |

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