Table I.
Summary of clinical data for maternal members in the Chinese pedigree examined for mutations
| Subject | Gender | Age at test (yrs) | Age at onset (yrs) | Use of aminoglycoside | PTA(dB) Right Ear | PTA (dB) Left Ear | Audiometric configuration | Level of hearing impairment |
|---|---|---|---|---|---|---|---|---|
| I-2 | F | 72 | - | No | 25 | 26 | - | Normal |
| II-4 | F | 49 | 27 | Yes | 67 | 55 | Sloping | Moderate |
| II-6 | F | 47 | - | No | 17 | 10 | - | Normal |
| II-8 | F | 44 | - | No | 15 | 17 | - | Normal |
| II-9 | M | 41 | 8 | Yes | 90 | 90 | Sloping | Severe |
| II-11 | M | 41 | - | No | 28 | 28 | Sloping | Mild |
| II-13 | M | 38 | 2 | Yes | 110 | 110 | Flat | Profound |
| II-14 | M | 31 | 3 | Yes | 93 | 98 | Sloping | Profound |
| III-3 | F | 26 | 1 | Yes | 108 | 97 | Flat | Profound |
| III-4 | F | 21 | 1 | Yes | 100 | 100 | Flat | Profound |
| III-5 | M | 19 | 1 | Yes | 105 | 100 | Flat | Profound |
| III-6 | M | 17 | 3 | Yes | 27 | 28 | Sloping | Mild |
| III-7 | M | 24 | 2 | Yes | 87 | 95 | Sloping | Profound |
| III-8 | M | 19 | - | No | 17 | 20 | - | Normal |
| III-9 | F | 21 | - | No | 15 | 15 | - | Normal |
| III-10 | M | 18 | - | No | 13 | 18 | - | Normal |
| III-11 | F | 18 | - | No | 12 | 5 | - | Normal |
| III-12 | M | 16 | - | Yes | 17 | 15 | - | Normal |
| III-13 | F | 19 | - | No | 0 | 5 | - | Normal |
| III-14 | M | 15 | - | No | 10 | 7 | - | Normal |
| III-15 | M | 8 | - | No | 5 | 10 | - | Normal |