As suggested by a genotype–phenotype correlation study, CDH23 null alleles cause USH1D, characterized by congenital profound HL, vestibular dysfunction, and late-onset retinitis pigmentosa, whereas most missense variations cause non-syndromic HL (DFNB12)8,9,12–15, with a wide phenotype spectrum ranging from congenital severe-to-profound HL to late-onset high-frequency HL16. Here, CDH23 is linked to Hodgkins lymphoma.