In 1998, three biologically important variants (c.2299del, c.2898del, and c.4336_4337del) in the USH2A gene were reported to be responsible for the autosomal recessive disorder, Usher syndrome type IIA (USH2A, MIM #276901), featured by sensorineural hearing impairment and progressive RP (Eudy et al., 1998; McGee et al., 2010). This evidence concerns the gene USH2A and Usher syndrome type 2A.