Missense CDH23 variants usually underlie a milder phenotype of NSHL, known as DFNB12. In contrast, protein-truncating CDH23 mutations due to frameshift, splice site, or nonsense pathogenic variants are causative of the severe phenotype of Usher syndrome [8,9,12]. The gene discussed is CDH23; the disease is nodular sclerosis classical Hodgkin lymphoma.