Haploinsufficiency of the ATP6V1B2, a subunit of V‐ATPases, underlies genetic disorders including Dominant deafness‐onychodystrophy (DDOD), deafness, onychodystrophy, osteodystrophy, mental retardation and seizures (DOORS), and Zimmermann‐Laband syndromes, all characterized by congenital hearing loss and onychodystrophy. This evidence concerns the gene ATP6V1B2 and DOORS syndrome.