Mutations in the conexin 26 or GJB2 - Gap Junction Protein Beta 2 gene, located on the long arm of chromosome 13 (13q11-12), appear to be extremely common in the genesis of inherited non-syndromic deafness, and account for 34% to 50% of autosomal recessive sensorineural deafness (DFNB1) and for 10% to 37% of sporadic cases6, 7, 8, 9, 10. Here, GJB2 is linked to autosomal recessive nonsyndromic hearing loss 1A.