To date, 12 genes (CDH23, CEP250, CIB2, CLRN1, DFNB31, GPR98, HARS, MYO7A, PCDH15, USH1C, USH1G and USH2A) have been reported to be responsible for Usher syndrome and three additional loci have been mapped. Here, USH2A is linked to Usher syndrome.