Including some most frequently associated genes such as GJB2, GJB3, GJB6, SLC26A4, KCNQ4, DFNA5, SLC26A5, MYO1A, MYO7A, MYH15A, CDH23 etc. behind the congenital NSHL [6–9]. The gene discussed is GJB3; the disease is nodular sclerosis classical Hodgkin lymphoma.