Table I.

Clinical characteristics of the family members.

Subject	Age (years)	Sex	Age at onset (years)	Pure tone test	Degree of hearing loss	DPOAE	Binaural ABR threshold (dBnHL)
I1	78	Male	47	Binaural full frequency sensorineural deafness	High severity on both sides	Binaural full frequency extraction in abnormal range	110
II2	56	Male	54	Binaural sensorineural deafness	Moderate on both sides	Binaural full frequency extraction in abnormal range	50
II3	61	Female	52	Binaural mixed deafness	Severe on both sides	Binaural full frequency extraction in abnormal range	70
II4	56	Male	43	Binaural sensorineural deafness	Severe on both sides	Binaural full frequency extraction in abnormal range	100
II6	56	Male	50	Binaural mixed deafness	Severe on both sides	Binaural full frequency extraction in abnormal range	70
III2	35	Female	32	Sensorineural deafness in right ear, high-frequency hearing loss in left ear	Normal hearing threshold	Binaural partial frequency extraction in abnormal range	30
III9	31	Male	31	Binaural sensorineural deafness with high frequency	Normal hearing threshold	Binaural partial frequency extraction in abnormal range	30
III14	32	Female	31	Binaural sensorineural deafness with high frequency	Normal hearing threshold	Binaural partial frequency extraction in abnormal range	40

DPOAE, Distortion Product Otoacoustic Emission; ABR, Auditory Brainstem Response; dBnHL, decibel normal hearing level.