Table 1.

The clinical information of the patients.

Proband number	Age	Gender	Age onset	Hearing impairment	ABR	DPOAE	Tympanogram	MRI/CT
1	2 yr	F	0	Profound	85 dB	Absent	As(L)/A(R)	Normal
2	1 yr	M	0	Profound	105 dB	Absent	A(L)/A(R)	Normal

ABR: auditory brainstem response; DPOAE: distortion product otoacoustic emissions; A: A type; As: As type.