Table 1.
Subjects | Age | Age onset | Hearing loss | ABR | DPOAE | AR | MRI |
---|---|---|---|---|---|---|---|
IV-2 | 57 yr | − | Normal | − | − | − | Normal |
IV-4 | 50 yr | 0 | Profound | 95 dB | + | + | Normal |
IV-5 | 47 yr | 0 | Profound | 95 dB | + | + | Normal |
V-3 | 19 yr | 0 | Profound | 90 dB | + | + | Normal |
V-4 | 17 yr | 0 | Profound | 90 dB | + | + | Normal |
+: presence; −: absence.