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.
Get full access to this article
View all access options for this article.
References
1.
Per-LeeJ, ClairmontA: Acoustic neuroma in von Recklinghausen's disease. South Med J69:844847, 1976.
2.
KanterWR, EldridgeR, FabricantR, AllenJC, KoerberT: Central neurofibromatosis with bilateral acoustic neuroma: Genetic, clinical and biochemical distinctions from peripheral neurofibromatosis. Neurology30:851859, 1980.
HarkinJ, ReedR: Atlas of tumor pathology, series 2, fasc 3, Tumors of the Peripheral Nervous System.Bethesda, Md, 1969, Armed Forces Institute of Pathology, pp 9295.
5.
YoungD: Bilateral acoustic neuromas in a large kindred. JAMA214:347353, 1980.
6.
MoyesPD: Case reports and technical note. Familial bilateral acoustic neuroma affecting 14 members from four generations. J Neurosurg29:7882, 1968.
7.
RubensteinL: Atlas of tumor pathology, series 2, fasc 6, Tumors of the Central Nervous System.Bethesda, Md, 1972, Armed Forces Institute of Pathology, pp 204206, 302-308.
8.
HoltGR: E.N.T. manifestations of von Recklinghausen's disease. Laryngoscope88:16171632, 1978.
