Summary
A patient initially presenting typical symptoms of idiopathic sudden deafness later developed disabling episodic vertigo, which led to translabyrinthine eighth nerve transection. Morphological examination of the removed cochlear nerve specimen revealed a demyelinating process in the neuroglial portion of the nerve. The major part of the peripheral, neurolemmal portion of the cochlear nerve was normal. The inferior vestibular nerve was fibrotic. The major part of the superior vestibular nerve was normal. Some of its peripheral bundles showed increased endoneurial fibrosis. It is suggested that a demyelinating process was the cause of the patient's symptoms.
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References
Adams RD (1959) A comparison of the morphology of the human demyelinative diseases and experimental “allergic” encephalomyelitis. In: Kies MW, Alford EC (eds) Allergic encephalomyelitis. Thomas, Springfield/IL, pp 183–209
Barany R (1913) Zur Differentialdiagnose zwischen Acusticustumor und multipler Sklerose mit bulbären Sitz. Monatsschr Ohrenheilkd 47: 693–694
Beal DD, Hemenway WG, Lindsay JR (1967) Inner ear pathology of sudden deafness. Arch Otolaryngol 85: 591–598
Cohen MM, Lessell S, Wolf PA (1979) A prospective study of the risk of developing multiple sclerosis in uncomplicated optic neuritis. Neurology 29: 208–213
Foix C, Bariety M (1926) A propos de trois cas de sclerose en plaques a debut vestibulaire. Rev D'Oto Neuro Ocul 4: 641–654
Gussen R (1976) Sudden deafness of vascular origin: A human temporal bone study. Ann Otol Rhinol Laryngol 85: 94–100
Hallberg OE (1956) Sudden deafness of obscure origin. Laryngoscope 66: 1237–1267
Hess K (1888) Über einen Fall von multipler Sklerose des Centralnervensystems. Arch Psychiatr 19: 64–87
Kornhuber HH (1976) Zur Differentialdiagnose des Schwindels. Arch Oto-rhinolaryngol 212: 339–349
Lampert PW (1965) Demyelination and remyelination in experimental allergic encephalomyelitis. J Neuropathol Exp Neurol 24: 371–385
Leidler R (1917) Über die Beziehungen der multiplen Sklerose zum zentralen Vestibularapparat. Monatsschr. Ohrenheilkd 51: 249–270
Lindsay JR (1959) Sudden deafness due to virus infection. Arch Otolaryngol 69: 13–18
Lindsay JR (1973) Profound childhood deafness: Inner ear pathology. Ann Otol Rhinol Laryngol [Suppl 5] 82: 1–121
Mackiewicz J (1945) Menière's syndrome in disseminated sclerosis. Acta Otolaryngol Orient 1: 17–20
Nadol JB, Weiss AD, Parker SW (1975) Vertigo of delayed onset after sudden deafness. Ann Otol Rhinol Laryngol 84: 841–846
Prineas J (1975) Pathology of the early lesion in multiple sclerosis. Hum Pathol 6: 531–554
Prineas JW, Wright RG (1978) Macrophages, lymphocytes, and plasma cells in the perivascular compartment in chronic multiple sclerosis. Lab Invest 38: 409–421
Prineas JW, Connell F (1978) The fine structure of chronically active multiple sclerosis plaques. Neurology [Suppl 9/2] 28: 68–75
Raine CS, Snyder DH, Valsamis MP, Stone SH (1974) Chronic experimental allergic encephalomyelitis in inbred guinea pigs — An ultrastructural study. Lab Invest 31: 369–380
Raine CS, Traugorr U, Stone SH (1978) Chronic relapsing experimental allergic encephalomyelitis: CNS plaque development in unsuppressed and suppressed animals. Acta Neuropathol (Berl) 43: 43–53
Sando I, Harada T, Loehr A, Sobel JH (1977) Sudden deafness: Histopathologic correlation in temporal bone. Ann Otol Rhinol Laryngol 86: 269–279
Schuknecht HF, Benitez J, Beekhuis J, Igarashi M, Singleton G, Rüedi L (1962) The pathology of sudden deafness. Laryngoscope 72: 1142–1157
Schuknecht HF, Kimura RS, Naufal PM (1973) The pathology of sudden deafness. Acta Otolaryngol (Stockh) 76: 75–96
Schumacher GA (1976) Demyelinating diseases as a cause for vertigo. Arch Otolaryngol 85: 537–538
Snyder DH, Valsamis MP, Stone SH, Raine CS (1975) Progessive demyelination and reparative phenomena in chronic experimental allergic encephalomyelitis. J Neuropathol Exp Neurol 34: 209–221
Stone SH, Lerner EM (1965) Chronic disseminated allergic encephalomyelinitis in guinea pigs. Ann NY Acad Sci 122: 227–241
Ward PH, Cannon D, Lindsay JR (1965) The vestibular system in multiple sclerosis. Laryngoscope 75: 1031–1047
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Ylikoski, J., House, J.W. Demyelinating disease as the assumed cause of hearing loss and vertigo. Arch Otorhinolaryngol 230, 161–170 (1981). https://doi.org/10.1007/BF00456145
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DOI: https://doi.org/10.1007/BF00456145