Carrier Rates in the Midwestern United States for GJB2 Mutations Causing Inherited Deafness

Abstract
Congenital severe-to-profound deafness affects 0.05% to 0.1% of children in the United States. In most of these families, there is no history of hearing loss, and a definitive etiology is only rarely established in the deaf child. The majority of cases are ascribed to unknown or presumed genetic factors. Estimates of the proportion of deafness due to genetic factors vary from 20% to 76%.1-4