In a recent American study including 1119 deafness probands, a cohort made up of 62.3% autosomal recessive cases (Sloan-Heggen et al. 2016) used a targeted sequencing approach and the most commonly implicated genes were GJB2, MYH9, OTOA, PCDH15, SLC26A4, STRC, TMC1, TMPRSS3 and USH2A. Interestingly, Sloan-Heggen et al. 2016 reported the p.(Ala163Val) allele in a patient with congenital hearing loss; however, in a compound heterozygous state with a second CLDN14 allele (p.P28L). Here, CLDN14 is linked to hearing loss disorder.