In particular, Six1-deficient mice show severe developmental defects in the ear and kidney, among other organs, which strongly resemble BOR syndrome symptoms (Laclef et al., 2003b; Zheng et al., 2003; Ozaki et al., 2004; Xu et al., 2003; Bosman et al., 2009). The gene discussed is SIX1; the disease is BOR syndrome.