The variants of solute carrier family 26 member 4 (SLC26A4) gene (MIM #605,646), also named the PDS gene, described as the second leading cause after the gap junction protein β 2 (GJB2) gene (MIM #121,011), were responsible for autosomal recessive NSHL (DFNB4, MIM #600,791) and syndromic deafness characterized by congenital sensorineural hearing loss, abnormalities of the cochlea, and goiter named as Pendred syndrome (PS, MIM #274,600) [3, 4]. This evidence concerns the gene SLC26A4 and Pendred syndrome.