ATP6V1B2 and autosomal dominant deafness - onychodystrophy syndrome: To further investigate interventions for Atp6v1b2‐associated hearing loss, we established a hair cell‐specific conditional knockout mouse model (Atp6v1b2fl/f;Atoh1Cre/+) that accurately recapitulates the congenital severe to profound hearing loss phenotype seen in DDOD syndrome patients.