Individuals carrying a heterozygous SIX1 mutation were reported to have a very different clinical outcomes, ranging from being non-syndromic bilateral hearing loss to Branchio-otic (BO) syndrome and to Branchio-otic-renal (BOR) syndrome (Salam et al., 2000; Ruf et al., 2004; Mosrati et al., 2011). The gene discussed is SIX1; the disease is hearing loss disorder.