Pathogenic sequence alterations of the SLC26A4 gene, which encodes for the anion exchanger pendrin (OMIM *605646), lead to EVA in the context of autosomal recessive Pendred syndrome (OMIM #274600) or non-syndromic deafness DFNB4 (OMIM #600791) (Everett et al. 1997). This evidence concerns the gene SLC26A4 and autosomal recessive nonsyndromic hearing loss 4.