Klippel-Trenaunay-Weber syndrome with labyrinthine bony overgrowth and mixed hearing loss, a case report.

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of (1) capillary malformations, (2) venous malformations, and (3) soft tissue or bony hypertrophy. There exists one report of a patient with KTS and an associated sensorineural hearing loss. We describe an adolescent girl with KTS and AV fistulas who was found to have a bony overgrowth extending from the cochlea into the middle ear cavity and an ipsilateral mixed hearing loss. In both of these patients, there were obvious KTS deformities involving the face. We suggest conducting hearing screening in patients with KTS and obvious head involvement.