Forty-two unrelated individuals and two siblings (12-0878, 12-0880) with Usher syndrome were screened for mutations in the Usher genes CDH23, CLRN1, DFNB31, GPR98, MYO7A, PCDH15, USH1C, USH1G, and USH2A. Biallelic mutations could be identified in 35 individuals and monoallelic mutations in eight individuals. Here, ADGRV1 is linked to Usher syndrome.