It has been shown that mice models that lacked the Slc26a4 gene (Slc26a4−/−) did not express Kcnj10 and that lack of this protein led to the loss of the endocochlear potential via endolymphatic acidification and Ca2+ absorption inhibition [25,26] which may be the direct cause of deafness in Pendred syndrome. This evidence concerns the gene KCNJ10 and Pendred syndrome.