Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice

► We investigated auditory effects of Cdh23 variants in both C57BL/6J and CBA/CaJ genetic backgrounds. ► The CBA/CaJ-derived Cdh23 allele lessens hearing loss and decreases hair cell loss in a C57BL/6J genetic background. ► The C57BL/6J-derived Cdh23 allele has no effect on hearing loss or hair cell death in a CBA/CaJ genetic background. ► Deleterious alleles at other loci in addition to Cdh23 must contribute to the accelerated hearing loss of C57BL/6J mice. ► Mice of the C57BL/6J and C57BL/6N substrains have identical hearing loss profiles.