IE: scaffolding and cochlear development; R: scaffolding
USH3A
3q25.1
USH3A/clarin-1
IE and R: probable role in synapsis transport*
USH3B
20q
−/−
Unknown
USH: usher syndrome; DFNB: autosomal recessive deafness; DFNA: autosomal dominant deafness; RP: retinitis pigmentosa; IE: inner ear; R: retina.
*A role in the retinal and inner ear synapses as been proposed for all the USH proteins. This remains to be elucidated.