In summary, our study demonstrated the function of GJB2 in cochlear development from embryos to adults, thus suggesting an avenue for the treatment of DFNB1A patients such as restoration of GJCs through overexpression of GJB2 or GJB6 [49, 56] or correction of mutant GJB2 through postnatal genome editing rather than through germ cell editing [57–60]. Here, GJB2 is linked to autosomal recessive nonsyndromic hearing loss 1A.