[Niikawa-Kuroki syndrome. Which characteristics must the HNO doctor consider in its diagnosis]

Niikawa-Kuroki syndrome (Kabuki make-up syndrome) is a congenital disorder with characteristic facial features and possibly anomalies of the skeletal system and internal organs. There is an increasing number of reports of patients with combined hearing impairment, inner ear deformities or sensorineural hearing impairment. In addition, the patients often suffer from therapy-resistant chronic otitis media. In addition to multiple cardiac and renal deformities, our 3 year old patient has a hearing impairment due to chronic otitis media with chronic otorrhea, and requires a hearing aid. A high-definition CT scan of the petrosal bone revealed, for the first time in a patient with Niikawa-Kuroki syndrome, a large vestibular aqueduct syndrome and deformities of the vestibular system. We examine the problems involved with treating chronic otorrhea in chronic otitis media and providing patients with BTE hearing aids.