Familial case_15 carries a novel frameshift deletion [c.162delG; p.(Lys55Serfs*25)] in SMPX gene (NM_014332.2), known for causing dominant X-linked NSHL (Table 1), The proband presents post-lingual bilateral symmetric severe to profound medium-high frequencies HL, while the mother shows a monolateral profound to severe HL (Figure 4A). This evidence concerns the gene SMPX and nodular sclerosis classical Hodgkin lymphoma.