In this study, we reported (1) the prevalence of MYO7A-associated HL for each of ADNSHL, ARNSHL, and USH1B in a large Japanese HL cohort, (2) the detailed clinical characteristics of each set of patients including the onset age, severity of HL, progressiveness of HL, and other associated symptoms (tinnitus, vertigo, visual symptoms), and (3) genotype–phenotype correlations for variant type and clinical phenotype. The gene discussed is MYO7A; the disease is Hodgkins lymphoma.