Table 1 Clinical features of individuals with sensorineural hearing loss in this family
From: A novel MYH14 mutation in a Chinese family with autosomal dominant nonsyndromic hearing loss
Examinations | II-1 | II-5 | III-1 |
|---|---|---|---|
PTA threshold (dB HL) | 56.25 / 58.75 (left/right) | 50.00 / 51.25 (left/right) | 56.25 / 58.75 (left/right) |
SRS (%) | 64 / 64 (left/right) | 92 / 88 (left/right) | 36 / 32 (left/right) |
Tympanometry | “A” type | “A” type | “A” type |
ABR threshold (dB nHL) | 70 (both) | 70/60 (left/right) | 60 (both) |
DPOAE | Absent (both) | Absent (both) | Absent (both) |
Cochlear microphone | No elicited | No elicited | No elicited |
Vestibular bithermal calorid test | Normal | Normal | Labyrinth reactivity lower (8.4°/s) |
cVEMP | Normal | Normal | No wave |
oVEMP | Low-amplitude in both ears | Low-amplitude in left and normal in right | N1 and P1 waves recorded only in right ear |
Optic nerve electroretinogram | Normal | Normal | Normal |
Temporal bone HRCT | Normal | Normal | Normal |
Brain MRI | Normal | Normal | Normal |