Required for postnatal maturation of the hair bundle and long-term survival of hair cells and spiral ganglion. Expressed in adult lung, brain and testis. In the inner ear, it is expressed in the inner and outer hair cells of the organ of Corti. Also expressed in cochlear spiral ganglion neurons. Defects in GIPC3 underlie the age-related hearing loss 5 (ahl5) and juvenile audiogenic monogenic seizure (jams1) phenotypes. Ahl5 mice show irregular structure of the stereocilia bundle of outer and inner hair cells, late-onset degeneration of the organ of Corti. The spiral ganglion exhibits a severe loss of neurons. Belongs to the GIPC family.