Regarding the petrous bone malformations, we found a significant correlation between the SLC26A4 mutations group and the presence of Mondini Malformations rather than isolated EVA, with Mondini Malformation more likely to be present in the M3 group, followed by M2, M1 and then the M0 group and isolated EVA more frequent in patients of the M0 group, then M1, M2 and, finally, the M3 group of patients. Here, SLC26A4 is linked to Incomplete partition of the cochlea type II.