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Otologic manifestations of neurofibromatosis
Author(s) -
Smullen Sean,
Willcox Thomas,
Wetmore Ralph,
Zackai Elaine
Publication year - 1994
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1288/00005537-199406000-00003
Subject(s) - neurofibromatosis , medicine , cerebellopontine angle , neurofibroma , pinna , auditory canal , neurofibromatoses , audiology , café au lait spot , ear canal , dermatology , surgery , radiology , magnetic resonance imaging
A chart review of 440 patients with neurofibromatosis seen at the Children's Hospital of Philadelphia Neurofibromatosis Clinic between 1980 and 1991 was performed to look for otologic manifestations. Neurofibromatosis 1 was present in 434 patients, and neurofibromatosis 2 was present in 6. A total of 31 otologic abnormalities related to neurofibromatosis were found in 28 patients. Neurofibromas of the external ear, including the pinna and the external auditory canal, were the most common finding. Middle ear neurofibromas were found in two patients. Acoustic neuromas were seen exclusively in patients believed to have neurofibromatosis 2. One cerebellopontine angle neurofibrosarcoma was found in a patient with neurofibromatosis 1.