Defects in SLC26A4 are among the most frequent causes (up to 10%) of early-onset autosomal recessive hearing loss (arHL); non-syndromic DFNB4 (MIM: 600,791) and Pendred syndrome (MIM: 274,600) (Sloan-Heggen et al. 2016). This evidence concerns the gene SLC26A4 and autosomal dominant nonsyndromic hearing loss.