HOXA1 and Bosley-Salih-Alorainy syndrome: Homozygous mutations of HOXA1 are associated with the autosomal recessive Athabascan Brainstem Dysgenesis Syndrome (ABDS) and the Bosley-Salih-Alorainy Syndrome (BSAS) characterized by sensorineural deafness due to the absence or aplasia of the cochlea (Tischfield et al., 2005; Bosley et al., 2008).