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A single nucleotide variant (SNV) of the cadherin 23 gene ( Cdh23   c.753A  ), common to many inbred mouse strains, accelerates age-related hearing loss (AHL) and can worsen auditory phenotypes of other mutations. We used homologous recombination in C57BL/6 NJ (B6N) and 129S1/SvImJ (129S1) embryonic stem cells to engineer mouse strains with reciprocal single base pair substitutions (B6- Cdh23   c.753A > G   and 129S1- Cdh23   c.753G > A  ). We compared ABR thresholds and cochlear pathologies of these SNV mice with those of congenic (B6.129S1- Cdh23   Ahl +  and 129S1.B6- Cdh23   ahl  ) and parental (B6N and 129S1) strain mice. Results verified the protective effect of the  Cdh23   c.753G   allele, which prevented high frequency hearing loss in B6 mice to at least 18 months of age, and the AHL-inducing effect of the  Cdh23   c.753A   allele, which worsened hearing loss in 129S1 mice. ABR thresholds differed between 129S- Cdh23   c.753A   SNV and 129S1.B6- Cdh23   ahl   congenic mice, and a linkage backcross involving these strains localized a Chr 10 QTL contributing to the difference. These results illustrate the large effects that strain background and congenic regions have on the hearing loss associated with  Cdh23   c.753  alleles. Importantly, the B6- Cdh23   c.753G  strain can be used to eliminate the confounding influence of the  Cdh23   c.753A  variant in hearing studies of B6 mice and mutant mice on the B6 background.

Effects of Cdh23 single nucleotide substitutions on age-related hearing loss in C57BL/6 and 129S1/Sv mice and comparisons with congenic strains