Perturbations within the MYO6 domain have been unequivocally implicated in both autosomal recessive non-syndromic hearing loss (DFNB37) and autosomal dominant non-syndromic hearing loss (DFNB22) (Melchionda et al., 2001; Ahmed et al., 2003). The gene discussed is MYO6; the disease is autosomal dominant nonsyndromic hearing loss.