Defects in SLC26A4, which encodes the anion (chloride/iodide, chloride/bicarbonate) transporter Pendrin, can cause nonsyndromic DFNB4 deafness with enlargement of the vestibular aqueduct (EVA) and Pendred syndrome [12,13]. Here, SLC26A4 is linked to autosomal recessive nonsyndromic hearing loss 4.