A dominant or a recessive mutation of the human ortholog, TMC1 causes a progressive hearing loss (DFNA36) or a profound congenital deafness (DFNB7/B11), for which Beethoven (Bth) and deafness (dn) are mouse models, respectively (Kurima et al. 2002; Vreugde et al. 2002).These examples and more others highlight the importance of mutant mouse in this process (Rhodes et al. 2004; Longo-Guess et al. 2007; Lewis et al. 2009; Carpinelli et al. 2013; Miller et al. 2013), providing valuable insight into gene functions. This evidence concerns the gene TMC1 and deafness.