TheGJB2 p.[V27I; E114G] variant may also additively impair GJB2 function, as the channel activities of homozygous p.[V27I; E114G] CX26 gap junctions has been previously shown to be reduced.11 Thus, it seems reasonable that carriers of the simple heterozygous c.262G>C could be asymptomatic,3 while carriers of c.262G>C in compound heterozygosity along with any other deafness-related variants such as c.235delC, p.V27I, etc. could experience HL, like in our study and a previous NSHL case.3 Hence, the pathogenic effects of theseGJB2 variants could be additive. The gene discussed is GJB2; the disease is deafness.