The GJB6 gene was first described as a causative in a rare dominant form of deafness, DFNA3, and its implication in NSHL were ascertained through the identification of two large deletions, del(GJB6-D13S1830) of size 309 kb and del(GJB6-D13S1854) of size 232 kb, which truncate the GJB6 gene [6, 7]. This evidence concerns the gene GJB2 and deafness.