Nevertheless, in many populations of European and Asian descent, pathogenic variants in GJB2 (connexin 26 gene) and GJB6 are major contributors to autosomal recessive NSHI (ARNSHI) (Chan and Chang, 2014), with the GJB6-D13S1830 deletion identified in up to 9.7%, as the second biggest genetic etiology of NS deafness in the European populations (del Castillo et al., 2002; del Castillo et al., 2003). The gene discussed is GJB2; the disease is deafness.