We identified ten cases with bi-allelic variants in the Usher syndrome-related genes, USH2A, MYO7A, CDH23, and ADGRV1. Usher syndrome is a clinically and genetically heterogeneous autosomal-recessive disorder characterized by sensorineural hearing deficiencies and later development of progressive retinitis pigmentosa. Here, CDH23 is linked to Usher syndrome.