The Ush1c gene, which encodes the protein harmonin located in the stereocilia and near the ribbon synapse in auditory and vestibular hair cells, was first identified as the cause of the USH1C subtype of USH1 disease.[4] We previously generated and characterized a Ush1c knockout (KO) mouse model,[5] in which the first four exons of the Ush1c gene were deleted and replaced with a reporter gene. The gene discussed is USH1C; the disease is Usher syndrome type 1C.