Table 1.
Summary of clinical and audiological data of studied patients
| Subject | Age (Year) |
Hearing level |
Audiogram shape |
OAE | Onset | Mutation | |
|---|---|---|---|---|---|---|---|
| Family 438 |
IV-9 | 22 | Bilateral- moderate |
Flat | Absent | C/P | c.1958delC (p.Pro653LeufsX13) |
|
| |||||||
| Family 766 |
V-1 | 35 | Bilateral- profound |
Flat | Absent | C/P | c.4467dupC (p.Ile1490HisfsX19 |
| V-3 | 30 | Bilateral- severe |
Flat | Absent | C/P | ||
| V-4 | 16 | Right profound, left severe |
Right scoop, Left flat | Absent | C/P | ||
| V-6 | 14 | Bilateral- profound |
Fragmentary shape |
Absent | C/P | ||
|
| |||||||
| Family 796 |
IV-2 | 17 | Bilateral- severe | Flat | Present | C/P | c.4718T>C (p.Ile1573Thr) |
| IV-3 | 13 | Bilateral- moderate |
Sloping | Present | C/P | ||
| IV-4 | 11 | Bilateral- moderate |
Sloping | Present | C/P | ||
| IV-5 | 9 | Bilateral- mild |
Flat | Present | C/P | ||
C/P: Congenital or prelingual