Hearing loss is one of the most common human sensory defects, affecting approximately one out of 1000 children at birth. In more than half of the cases, the defect can be attributed to a genetic cause. In most of the cases (80%), hereditary hearing loss is categorized as non-syndromic, based on the absence of other symptoms. Isolated deafness forms that are transmitted in the recessive mode (DFNB) are the most frequent (85%) and the most severe. To date, few dozens of genes and gene loci have been implicated in DFNB.