Involved in hearing and vision as member of the USH2 complex . Necessary for elongation and maintenance of inner and outer hair cell stereocilia in the organ of Corti in the inner ear . Involved in the maintenance of the hair bundle ankle region, which connects stereocilia in cochlear hair cells of the inner ear . In retina photoreceptors, required for the maintenance of periciliary membrane complex that seems to play a role in regulating intracellular protein transport .