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Neurofibromatosis. 1989;2(1):47-52.

Bilateral internal auditory canal enlargement without acoustic nerve tumor in von Recklinghausen neurofibromatosis.

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  • 1Department of Otolaryngology, University of Tokyo, Japan.

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.

PMID:
2517023
[PubMed - indexed for MEDLINE]
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