Trio-WES analysis identified a known pathogenic variant (c.1516C>T; p.R506*) in the ATP6V1B2 gene (GenBank: NM_001693) from four affected individuals with hearing loss and onychodystrophy, i.e., the affected mother and son in family 1 and the affected father and daughter in family 2. This evidence concerns the gene ATP6V1B2 and hearing loss disorder.