USH1C and Usher syndrome: One form of Usher syndrome results from mutations in USH1C, which encodes Harmonin, a scaffolding protein that localizes to auditory hair cells and is essential for normal hair bundle morphogenesis and mechanotransduction (Verpy et al. 2000; Boeda et al. 2002; Lefevre et al. 2008; Grillet et al. 2009).