Gap junction channels in the mammalian cochlea are primarily formed by Cx26 and Cx30 (Ahmad et al., 2003) and mutations of the genes encoding for these two proteins (Gjb2 and Gjb6, respectively) are associated with the most common form of prelingual congenital hearing impairment in humans (DFNB1) (Xu and Nicholson, 2013). Here, GJB6 is linked to autosomal recessive nonsyndromic hearing loss 1A.