This aspect is fundamental for NSHL, where, apart from the relevant contribution of the GJB2 gene and, in some populations of GJB6 gene, both responsible for ~50% of all AR cases [14,15,16], and of STRC deletions (accounting for 1% to 5% of HL cases [17]), no other worldwide primary players have been identified. Here, GJB2 is linked to nodular sclerosis classical Hodgkin lymphoma.