Sensorineural hearing loss (SNHL) is the most prevalent form of sensory impairment in humans, with an approximate prevalence of 1:1000 live births. At least 50% of the reported cases are hereditary and among these, autosomal recessive deafness (DFNB) is the most frequent and severe form of deafness. Autosomal recessive prelingual deafness is known to be genetically highly heterogeneous. DFNB1A (Deafness, Autosomal Recessive 1A) is caused by homozygous or compound heterozygous mutation in the GJB2 gene. Hereditary forms of hearing impairment caused by GJB2 mutations are the frequent sensory disorders registered among newborns in various human populations.