The discovery of the involvement of other deafness genes, including FOXI1 (MIM #601093), KCNJ10 (MIM # 602208) and GJB2 (MIM #121011) [9–11] in combination with SLC26A4 monoallelic mutation has proposed the existence of digenic inheritance pattern in PDS and EVA. This evidence concerns the gene SLC26A4 and deafness.