locus account for about half of the cases of European ancestry [12],[15]. Among hundreds of known causal mutations in the GJB2/GJB6 locus [14], the 35delG mutation in GJB2 is the most common, with an allele frequency of 1.25% in European Americans [16], but hundreds of other point mutations in GJB2 as well as a 342-kb deletion encompassing GJB6 also represent known causal variants [17],[18].
