ATP6V1B2 and autosomal dominant deafness - onychodystrophy syndrome: Although mutant mouse model derived from pathogenic variant of DDOD syndrome have been previously generated to mimic clinical patients,[10] the role of the Atp6v1b2 gene in HCs remained unclear due to functional compensation induced by premature termination codon (PTC) mutations in mice.[17] To address this issue, we generated conditional knockout mice with a targeted deletion of the Atp6v1b2 gene specifically in HCs.