We were able to identify the cellular expression pattern, subcellular location, and characterize the phenotype in two knockout mouse models of Klhdc7b. The very similar phenotype in two mouse models generated and analyzed at two different institutions and mouse facilities, possibly with different noise levels, on different backgrounds with respect to a known age-related hearing loss mutation, strengthens the case for a specific hearing loss phenotype caused by the loss of KLHDC7B. This evidence concerns the gene KLHDC7B and presbycusis.