FRMPD4 and sensorineural hearing loss disorder: In mice, <i>Frmpd4</i> knockout resulted in high-frequency hearing loss and cochlear abnormalities consistent with the human phenotype.<h4>Conclusions</h4>Our findings expand the phenotypic spectrum of <i>FRMPD4</i> to include non-syndromic sensorineural hearing loss and establish its evolutionarily conserved role in auditory function.