抄録
We have reported two patients with Kabuki make-up syndrome who exhibited mild sensorineural hearing loss. It has been reported that the hearing loss in cases of Kabuki makeup syndrome is conductive and caused by otitis media. The hearing loss in our own cases was suspected of being sensorineural and congenital, because the patients had no history of labyrinthitis or meningitis which acttected the inner ear. We suggest in the Kabuki makeup syndrome, congenital sensorineural hearing loss might be present and that the pathogenesis ofthis hearing loss might be caused by abnormal embryogenesis.