Table 1.

Summary of all clinical symptoms of five patients with Usher syndrome type IIC and individuals V:15 and V:16 with atypical hearing loss.

Individual	genotype	hearing loss	fundoscopic examination	ERG	OCT	ENG
V:1	del/del	moderate-severe across all frequencies	loss of vision	/	/	/
V:7	del/del	moderate-severe across all frequencies	retinitis pigmentosa	cone degeneration	increased inner retinal thickness	directional preponderance 27% left, unilateral weakness 36% left
V:8	del/del	moderate-severe across all frequencies	retinitis pigmentosa	cone degeneration	/	directional preponderance 35% right
V:10	del/del	moderate-severe across all frequencies	retinitis pigmentosa	/	/	directional preponderance 13% right
V:11	del/del	moderate-severe across all frequencies	retinitis pigmentosa	/	/	normal
V:15	wt/wt	severe-profound in mid to high frequencies	normal	/	/	/
V:16	wt/wt	severe-profound in mid to high frequencies	normal	/	/	/