This article has reviewed the genetic disorder, neurofibromatosis 2. The history, nomenclature, genetic etiology, epidemiology, diagnostic criteria, pathogenesis, and presentation of neurofibromatosis 2 have been given. A related but distinctly different disorder, neurofibromatosis 1, has also been described. The diagnostic evaluation of neurofibromatosis 2 has been discussed with an emphasis on the importance of early diagnosis. An analysis of the surgical treatment of bilateral acoustic neuromas as well as nonsurgical therapeutic alternatives has been presented. The results and complications in the operative management of 86 acoustic tumors in 49 patients with neurofibromatosis 2 have been described in detail. The poor immediate hearing results and the even poorer long-term hearing results have been examined in light of the invasive histopathology of these tumors. It has been emphasized that patients with neurofibromatosis 2 and asymptomatic family members require comprehensive, multidisciplined, long-term management.
|Original language||English (US)|
|Number of pages||21|
|Journal||Otolaryngologic Clinics of North America|
|State||Published - 1992|
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