The protein encoded by LHFPL5 is a member of a superfamily of tetraspan proteins, which includes the claudin tight junction proteins, gap junction proteins, peripheral myelin, and epithelial membrane proteins as well as calcium channel-like proteins. Several genes of this superfamily have been reported to be necessary for hearing in either humans or mice, or both. LHFPL5 is expressed transiently in inner ear hair cell stereociliary bundles. This finding is suggestive that LHFPL5 is essential for normal function of the human cochlea.