Taken together, our results suggest that U-TOPTM HL Genotyping Kit Ver2 for the screening of 11 variants from five deafness genes, comprised of two ANSD genes (OTOF and ATP1A3) and three SNHL genes (MPZL2, COCH, and TMC1), can be used as a reliable screening tool in the Korean population with a distinctive auditory phenotype. Here, ATP1A3 is linked to sensorineural hearing loss disorder.