Contemporary management of neurofibromatosis

Richard T. Miyamoto, Karen L. Roos, Robert L. Campbell, Robert M. Worth

Research output: Contribution to journalArticle

19 Scopus citations

Abstract

The neurofibromatoses are two distinct entities with different genetic origins. The phenotypic expressions and required treatments are different. The devastating nature of neurofibromatosis-2 may be more effectively controlled through the application of advanced imaging techniques and contemporary neurotologic procedures. The most common manifestation of neurofibromatosis-2 is that of bilateral acoustic neuromas. The eventual total bilateral sensorineural deafness associated with this condition can be obviated in selected cases if the diagnosis is established early. Follow-up data are reported for three patients in whom hearing was preserved in at least one ear. When removal with hearing preservation is not possible, subtotal tumor removal with decompression of the internal auditory canals may delay progression of hearing loss. A new approach to tumors of the pterygomaxillary fossa that have extended to the middle cranial fossa has been successfully applied and is described.

Original languageEnglish (US)
Pages (from-to)38-43
Number of pages6
JournalAnnals of Otology, Rhinology & Laryngology
Volume100
Issue number1
DOIs
StatePublished - Jan 1991

Keywords

  • Acoustic neuroma
  • Genetic disorders
  • Neurofibromatosis
  • Von Recklinghausen's disease

ASJC Scopus subject areas

  • Otorhinolaryngology

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