Familial central neurofibromatosis.
The condition of central neurofibromatosis, while well documented in neurology literature, has tended to be amalgamated into acoustic neuromas associated with peripheral neurofibromatosis (or Recklinghausen's disease) in otolaryngology literature. The history of a unique family that has demonstrated four generations of bilateral acoustic neuromas has been outlined to show that central neurofibromatosis is a distinct entity related but not identical in clinical and biochemical aspects to Recklinghausen's disease. This article in intended to acquaint the otolaryngologist with the presentation and management of central neurofibromatosis.
D000158:Acoustic Impedance Tests; D000293:Adolescent; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology; D005072:Evoked Potentials, Auditory; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000235:genetics* / Q000503:physiopathology; D009464:Neuroma, Acoustic / Q000235:genetics* / Q000503:physiopathology; D010375:Pedigree