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. Author manuscript; available in PMC: 2015 May 15.
Published in final edited form as: J Med Genet. 2009 Apr;46(4):272–276. doi: 10.1136/jmg.2008.060947

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 / / /