title: Demyelination of vestibular nerve axons in unilateral Ménière's disease.
abstract: We conducted a study to determine whether vestibular nerves in patients with unilateral Ménière's disease whose symptoms are refractory to medical management exhibit neuropathologic changes. We also endeavored to determine whether retrocochlear abnormalities are primary or secondary factors in the disease process. To these ends, we obtained vestibular nerve segments from five patients during retrosigmoid (posterior fossa) neurectomy, immediately fixed them, and processed them for light and electron microscopy. We found that all five segments exhibited moderate to severe demyelination with axonal sparing. Moreover, we noted that reactive astrocytes produced an extensive proliferation of fibrous processes and that the microglia assumed a phagocytic role. We conclude that the possible etiologies of demyelination include viral and/or immune-mediated factors similar to those seen in other demyelinating diseases, such as multiple sclerosis and Guillain-Barré syndrome. Our findings suggest that some forms of Ménière's disease that are refractory to traditional medical management might be the result of retrocochlear pathology that affects the neuroglial portion of the vestibular nerve.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond, USA.
authors.forename: Robert F
authors.initials: RF
authors.lastname: Spencer
authors.forename: Aristides
authors.initials: A
authors.lastname: Sismanis
authors.forename: Jefferson K
authors.initials: JK
authors.lastname: Kilpatrick
authors.forename: Wayne T
authors.initials: WT
authors.lastname: Shaia
mesh_terms: D000328:Adult; D000368:Aged; D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D001707:Biopsy, Needle; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008575:Meniere Disease / Q000175:diagnosis / Q000601:surgery*; D008854:Microscopy, Electron; D008875:Middle Aged; D011379:Prognosis; D012494:Sampling Studies; D012720:Severity of Illness Index; D014725:Vestibular Nerve / Q000473:pathology* / Q000648:ultrastructure*