Autosomal recessive inheritance contributes to 80% of nonsyndromic hereditary hearing loss, and mutations in the gene encoding the gap junction protein Connexin 26 (GJB2 – DFNB1) are present in about 50% of the recessive cases; autosomal dominant inheritance is observed in 10–20% of the cases, and X-linked inheritance in 2–3%. Here, GJB2 is linked to hearing loss disorder.