LOXHD1 and nodular sclerosis classical Hodgkin lymphoma: NSHL typified by a down-sloping audiogram configuration suggests LOXHD1 involvement, and the phenotype and inheritance patterns are similar to those seen with SLC26A4 and TMPRSS3. In our study, most cases showed congenital HL (19/21, 90.5%) and bilateral symmetric down-sloping audiogram configurations (10/15, 66.7%) that were in accordance with previous reports (Kim et al., 2021; Bai et al., 2020).