Pathogenic variants in USH2A explain in general 8-19% of cases with autosomal recessive retinitis pigmentosa (arRP) (OMIM: #613809)1,2, 57–90% of Usher syndrome type 2 (USH2) (OMIM: #276901) cases and ~50% of Usher syndrome (USH) cases3,4. Here, USH2A is linked to Usher syndrome.