Table 1. The clinical features and the genetic study of the composite Chinese family with assortative mating.

Number	Sex	Age at onset	Hearing test (BC1/ASSR2)	Audiogram profiles	mtDNA 12S rRNA 1555A>G	MYH14		WFS1 c.449C>T (p.A150 V)
						c.541G>A (p.A181 T)	c.2594C>T (p.T865 M)
IA:1	Male	-	L3∶20 dB; R4∶25 dB	normal	wt	wt5/wt	wt/wt	wt/wt
IA:2	Female	The fourth decade	L:51 dB; R:55 dB	sloping	Homoplasmic	wt/wt	wt/wt	wt/wt
IIA:1	Male	The first decade	L:>100 dB; R:>100 dB	sloping	Homoplasmic	wt/wt	wt/wt	wt/wt
IIA:2	Male	The fourth decade	L:70 dB; R:70 dB	sloping	Homoplasmic	wt/wt	wt/wt	wt/wt
IIIA:1	Female	-	L:20 dB; R:20 dB	normal	wt	wt/wt	wt/wt	wt/wt
IIIA:2	Male	-	L:20 dB; R:23 dB	normal	wt	wt/wt	wt/wt	wt/wt
IIA:3	Male	The first decade	L:90 dB; R:98 dB	sloping	Homoplasmic	wt/wt	wt/wt	wt/wt
IIA:4	Female	-	L:25 dB; R:20 dB	normal	Homoplasmic	wt/wt	wt/wt	wt/wt
IIA:5	Male	-	L:20 dB; R:20 dB	normal	wt	wt/wt	wt/wt	wt/wt
IIIA:3	Female	-	L:23 dB; R:20 dB	normal	Homoplasmic	wt/wt	wt/wt	wt/wt
IIIA:4	Female	-	L:25 dB; R:25 dB	normal	Homoplasmic	wt/wt	wt/wt	wt/wt
IIIA:5	Female	-	L:23 dB; R:20 dB	normal	Homoplasmic	wt/wt	wt/wt	wt/wt
IB:1	Male	-	L:25 dB; R:23 dB	normal	wt	wt/wt	wt/wt	wt/wt
IB:2	Female	The first decade	L:70 dB; R:75 dB	U-shape	wt	mt6/wt	wt/wt	wt/wt
IIB:1	Male		L:20 dB; R:20 dB	normal	wt	wt/wt	wt/wt	wt/wt
IIB:2	Male	The first decade	L:85 dB; R:81 dB	U-shape	wt	mt/wt	wt/wt	wt/wt
IIB:3	Female	The first decade	L:85 dB; R:80 dB	U-shape	wt	mt/wt	wt/wt	wt/wt
IIIB:1	Female	The first decade	L:83 dB; R:>100 dB	U-shape	wt	mt/wt	mt/wt	wt/wt
IIIB:2	Female	The first decade	L:80 dB; R:80 dB	U-shape	wt	mt/wt	mt/wt	wt/wt
IIC:1	Female	congenital	L:90 dB; R:90 dB	sloping	wt	wt/wt	wt/wt	mt/wt
IIIC:1	Female	congenital	L:90 dB; R:85 dB	sloping	wt	wt/wt	wt/wt	mt/wt
IIIC:2	Male	congenital	L:>100 dB; R:>100 dB	-	wt	wt/wt	wt/wt	mt/wt
IIIC:3	Male	congenital	L:>100 dB; R:>100 dB	-	wt	wt/wt	wt/wt	mt/wt

¹Bone conduction;

²Auditory steady state response;

³left ear;

⁴Right ear;

⁵wild type;

⁶mutant allele.