Vestibulo-Cochlear Dysfunction in Migrating Disseminated Multiple Cranial Neuropathy

Abstract

Multiple cranial nerve palsy can occur in various intracranial or juxtacranial diseases including neoplasm, granuloma, angiopathy and meningeal diseases, or in acute or chronic relapsing polyneuropathy of demyelinating nature. The latter condition is characterized by symmetrical or bilateral, and predominantly motor nerve involvement. In 1967, the author described a peculiar, multiple, cranial nerve palsy and proposed the descriptive designation of migrating, disseminated, multiple, cranial neuropathy. Since then, 9 cases have been reported from Japan and 3 case series have been known from Thailand, Sri-Lanka and Finland. The cranial nerve palsy in this disease is acute in onset, usually self-limited in course, and migrates with latent interval. All of the sensori-motor cranial nerves II-XII may be involved. Repeated and careful examination and long-term follow-up study failed to disclose any evidence of systemic or local underlying disease processes. In this report, 10 of our own cases are described. Paralysis of the vestibulo-cochlear nerve was found in 5 patients. Neurological and neuro-otological studies disclosed that hearing loss was of sensori-neural type and the vestibular dysfunction was due to peripheral origin. One of these was found to have benign paroxysmal positional vertigo. Attention should be given to those presenting with vestibulo-cochlear symptoms and signs, especially with a tendency to relapse, in relation to migrating, disseminated, multiple, cranial neuropathy. © 1983, The Society of Practical Otolaryngology. All rights reserved.