Here we employed HEI-OC1 cells (Kalinec et al., 2003; Kalinec G. M. et al., 2016) that were derived from the progenitor epithelium of the organ of Corti from P7 mice as a tissue-relevant cell model to assess several Cx26 mutants that have been associated with non-syndromic and syndromic hearing loss. Here, GJB2 is linked to hearing loss disorder.