Some representative deafness genes that have been identified include the Alport syndrome (COL4A3, COL4A4 or COL4A5 genes), branchio-oto-renal syndrome (EYA1 gene), Mohr-Tranebjaerg syndrome (TIMM8A gene), Pendred syndrome (SLC26A4 gene), Jervell and Lange-Nielsen Syndrome (KVLQT1 and KCNE1 genes), Usher syndrome with its several types, Norrie disease (NDP gene), DFNB1 (GJB2 gene), DFN3 (POU3F4 gene), DFNB4 (SLC26A4 gene), DFNA6/14 (WFS1 gene), and several others [3,4]. The gene discussed is POU3F4; the disease is Jervell and Lange-Nielsen syndrome.