The Beethoven (Bth) mouse is a natural model with hearing impairment that carries the Tmc1 (NM_028953.2) missense variant (p.M412K, c.T1235A), which is orthologous to that found in Family 1304, a DFNA36 family we previously described (Zhao et al., 2014). The gene discussed is TMC1; the disease is Hearing impairment.