OTOLOGIC MANIFESTATIONS OF NEUROFIBROMATOSIS

A chart review of 440 patients with neurofibromatosis seen at the Chil dren's Hospital of Philadelphia Neurofibromatosis Clinic between 1980 and 1991 was performed to look for otologic manifestations. Neurofibro matosis 1 was present in 434 patients, and neurofibromatosis 2 was pre sent in 6. A total of 31 otologic abnormalities related to neuro fibro matosis were found in 28 patients. Neurofibromas of the external ear, including the pinna and the external auditory canal, were the most com mon finding. Middle ear neurofibromas were found in two patients. Acou stic neuromas were seen exclusively in patients believed to have neuro fibromatosis 2, One cerebellopontine angle neurofibrosarcoma was found in a patient with neurofibromatosis 1.