Nonsyndromic deafness was reported in only two families so far: patients from the original DFNB18 family were homozygous for a “leaky” splice-site mutation (IVS12+5G>C) that might affect the splicing of exons 11 and 12 [9], and patients from a Chinese family were homozygous for a missense change (p.Pro608Arg) but no functional assay was performed to prove the pathogenicity of this change [21]. Here, USH1C is linked to deafness.