Hearing loss is a common sensory and genetically heterogeneous disorder. Approximately 1:1000 children is born deaf or develops severe to profound hearing loss in early childhood due to genetic defects. About 70% of these cases are non-syndromic and, in most cases, follow an autosomal recessive mode of inheritance. Autosomal recessive non-syndromic hearing loss (ARNSHL), also known as DFNB, accounts for up to 80% of non-syndromic hereditary hearing loss. To date, more than 100 gene loci have been implicated in DFNB. DFNB7, also known as DFNB11, is a form of profound, congenital, neurosensory, non-syndromal deafness.