In three patients with deaf‐blindness, disease was found to be due to “non‐Usher” gene mutations: Quantification of NGS reads in a Saudi patient from a consanguineous family, apparently affected by USH1, revealed a homozygous deletion of OTOA, a gene known to be associated with autosomal recessively inherited deafness, DFNB22. Because the patient's retinal phenotype (deep pigment deposits along the vascular arcades, subretinal fibrosis; delayed, depressed, and simplified scotopic flash response in the ERG) appeared compatible with a recessive NR2E3‐related dystrophy (Khan et al. Here, OTOA is linked to Blindness.