Bilateral facial nerve schwannomas.
Facial nerve schwannoma is an uncommon tumor and bilateral facial nerve tumors are extremely rare. A case is presented in which neuromas affecting the intra-canalicular and labyrinthine portions of both facial nerves occurred. Radiologic assessment demonstrated the origin of these tumors. Eventual tumor involvement of the sole functioning cochlea resulted in the development of total hearing loss. Management entailed symptomatic care and surgical resection. Auditory rehabilitation was attempted using cochlear implantation, but results have not been satisfactory. Genetic screening identified a mutation in the NF2 gene. It is proposed that this patient's condition should be considered a variant of neurofibromatosis 2 and that bilateral facial neuromas should be included in the clinical criteria for this condition.
Fenton
J E
JE
Department of Otology, Clinique Bachaumont, Paris, France.
Morrin
M M
MM
Smail
M
M
Sterkers
O
O
Sterkers
J M
JM
D000328:Adult; D003390:Cranial Nerve Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D016057:Evoked Potentials, Auditory, Brain Stem; D005154:Facial Nerve / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009442:Neurilemmoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D016518:Neurofibromatosis 2 / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D014057:Tomography, X-Ray Computed