Mutations of the genes encoding OTOG (otogelin, DFNB18B), OTOGL (otogelin-like, DFNB84B), and TECTA (α-tectorin, DFNA8/12, DFNB21) have been associated with hearing loss in humans, with occasional reports of vestibular hyporeflexia or vertigo in patients with OTOG or TECTA defects. The gene discussed is OTOG; the disease is hearing loss disorder.