title: Bilateral internal auditory canal enlargement without acoustic nerve tumor in von Recklinghausen neurofibromatosis.
abstract: The common (and definitive) intracranial tumors in patients with one form of neurofibromatosis (NF), bilateral acoustic NF (NF-2), are bilateral acoustic neuromas. In this study, we present 2 cases with what appears to be another form of NF, namely, NF-1, who showed bilateral enlargement of the internal auditory canals. Bilateral caloric responses were remarkably impaired in both cases; a unilateral sensorineural hearing loss was observed in 1. No tumor was demonstrated in the enlarged internal auditory canals in either case. Although the pathophysiology remains uncertain, some patients with NF-1 show abnormal cochleovestibular function in association with bilateral internal auditory canal enlargement without an acoustic tumor.
authors.affiliation: Department of Otolaryngology, University of Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Kitamura
authors.forename: T
authors.initials: T
authors.lastname: Senba
authors.forename: A
authors.initials: A
authors.lastname: Komatsuzaki
mesh_terms: D002648:Child; D004424:Ear Canal / Q000473:pathology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D009464:Neuroma, Acoustic