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.
Kabarity et al. (1981) examined a 13 year old male within an Arabic family with 13 individuals affected with heritable "uncomplicated" childhood deafness at the Genetic Counseling Centre in Kuwait University. Pedigree analysis revealed 9 relatives (4 males and 5 females), all affected likewise, and an affected boy and girl who passed away from unrelated reasons. All cases were offspring from first cousin marriages, which comprised 79.5% of the unions in this family. Additional studies showed that the family migrated to Kuwait from Al-Sawiah village near Nablus city (Palestine) in 1954 where consanguineous marriage is fairly widespread for cultural and social reasons.
Al-Kandari and Alshuaib (2007) studied 200 normal and 15 high-risk neonates in Kuwait to detect permanent hearing loss. Different incidence rates of permanent hearing loss were uncovered in the two groups. Only 1% of the neonates in the normal group had profound sensorineural hearing loss, while another 1% had moderate sensorineural hearing loss. However, in the high-risk group, as much as 27% and 20% of the neonates had severe and moderate sensorineural hearing loss, respectively. Al-Kandari and Alshuaib (2007) recommended nationwide newborn hearing screening programs in Kuwait and other neighboring countries for early detection of permanent hearing loss.