In this study, the detection capabilities of a pathological genotype in patients with mild, moderate, and moderate-to-severe congenital bilateral SNHL were 83% in our cohort without relatives (193/233): 68% (159/233) of cases were caused by mutations in the GJB2 gene, 9% (22/233) of cases were caused by mutations in the STRC gene and 5% (12/233) of cases were linked to the USH2A gene. The gene discussed is USH2A; the disease is sensorineural hearing loss disorder.