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Focused Issue of This Month Vestibular Neuritis and Bilateral Vestibulopathy Kwang- Dong Choi, MD Department of Neurology, Pusan National University Medical School E - mail : choikwangdong@hanmail.net Eui-Kyung Goh, MD Department of Otolaryngology, Pusan National University Medical School E - mail : gohek@pusan.ac.kr J Korean Med Assoc 2008; 51(11): 992-1006 Abstract Vestibular neuritis is the second most common cause of peripheral vestibular vertigo. The key signs and symptoms are the acute onset of sustained rotatory vertigo without hearing loss, postural imbalance with Romberg's sign, and peripheral type nystagmus. Head thrust and caloric tests show ipsilateral hyporesponsiveness, but hearing test shows normal. Either an inflammation of the vestibular nerve or labyrinthine ischemia was proposed as a cause of vestibular neuritis. Recovery after vestibular neuritis is usually incomplete. Despite the assumed viral cause, the effects of corticosteroids, antiviral agents, or the two in combination are uncertain. Bilateral vestibulopathy is a rare disorder of the peripheral labyrinth or the eighth nerve. The most frequent etiologies include ototoxicity, autoimmune disorders, meningitis, neuropathies, sequential vestibular neuritis, cerebellar degeneration, tumors, and miscellaneous otological diseases. The two key symptoms are unsteadiness of gait and oscillopsia associated with head movements or when walking. The diagnosis is made with the simple bedside tests for defective vestibulo-ocular reflex (head thrust and dynamic visual acuity tests). Bilateral vestibulopathy is confirmed by the absence of nystagmus reaction to both caloric and rotatory chair tests. The spontaneous recovery is relatively rare and incomplete. Vestibular rehabilitation is supportive of the improvement, but the efficacy of physical therapy is limited. Keywords: Vestibular neuritis; Bilateral vestibulopathy; Peripheral vestibulopathy 992

Vestibular Neuritis and Bilateral Vestibulopathy 10 * Recording of horizontal eye movement of the left eye Recording of vertical eye movement of the left Recording of torsional eye movement of the left eye Figure 1. Spontaneous nystagmus in a patient with right vestibular neuritis. The fast phases of mixed torsional-horizontal nystagmus directs toward the intact ear. The nystagmus increases with gaze in the direction of nystagmus, and decreases with gaze in the opposite direction. Visual fixation markedly suppress the nystagmus. 993

Choi KDGoh EK 994

Vestibular Neuritis and Bilateral Vestibulopathy Figure 2. Bithermal caloric tests show a complete canal paresis in the left ear in a patient with left vestibular neuritis. 995

Choi KDGoh EK Figure 3. Rotatory chair test shows increased phase lead and the asymmetry with normal gain of the vestibulo-ocular reflex in a patient with left vestibular neuritis. Figure 4. Ocular torsion to the left side in a patient with left vestibular neuritis. 996

Vestibular Neuritis and Bilateral Vestibulopathy Figure 5. Normal vestibular-evoked myogenic potentials in a patient with right vestibular neuritis. A B Figure 6. Posturographic data in a patient with vestibular neuritis. The scores of condition 4, 5, 6 are decreased. 997

Choi KDGoh EK 998

Vestibular Neuritis and Bilateral Vestibulopathy 999

Choi KDGoh EK Table 1. Commonly used antivertiginous and antiemetic drugs Drug Dosage Action Anticholinergics Muscarine antagonist Scopolamine (Transderm Scop) 0.6 mg po q 4~6 h or transdermal patch: 1 q 3 days Antihistamines Dimenhydrinate 50 mg po q 4~6 h or Histamine (H1) antagonist (Dramamine) im q 4~6 h or 100 mg suppository q 8~10 h Muscarine antagonist Meclizine 25 mg po q 4~6 h Histamine (H1) antagonist (Antivert, Bonine) Muscarine antagonist Promethazine 15 or 50 mg po q 4~6 h or Histamine (H1) antagonist (Phenergan) im q 4~6 h or suppository q 4~6 h Dopamine (D2) antagonist Phenothiazine Prochlorperazine 5 or 10 mg po q 4~6 h or im q 6 h or Muscarine antagonist (Compazine) 25 mg suppository q 12 h Dopamine (D2) antagonist Butyrophenone Droperidol 2.5 or 5 mg im q 12 h Muscarine antagonist (Inapsine) Dopamine (D2) antagonist Benzodiazepines Diazepam (Valium) 5 or 10 mg po bid-qid Im q 4~6 h or iv q 4~6 h GABA A agonist Clonazepam (Klonopin) 0.5 mg po tid GABA A agonist 1000

Vestibular Neuritis and Bilateral Vestibulopathy 1001

Choi KDGoh EK Table 2. Causes of bilateral vestibulopathy Idiopathic Ototoxicity Meningitis (labyrinthine infection) Tumors Autoimmune disorders Cerebellar degeneration Neuropathies Bilateral sequential vestibular neuritis Bilateral Meniere's syndrome Congenital malformation Familial vestibulopathy Labyrinthine ischemia Gentamicin & other antibiotics, anticancer chemotherapy Antihistamines (meclizine, phenergan) Antidepressant (Amitriptyline, other TCA agents) Aspirin & NSAID Diazepam, alprazolam, lorazepam Verapamil and other CCAs Furosemide Quinine & related drugs Streptococcus aureus, N. menigitidis, S. pneumoniae, M. tuberculosis, HIV Neurofibromatosis (bilateral acoustic neuromas), NHL, Leptomeningeal metastasis Cogan's syndrome, Behcet's disease, SLE, RA, PAN Wegener's granulomatosis, Giant cell arteritis, Antiphospholipid syndrome Friedrich's ataxia, Charcot - Marie syndrome Vit. B12 deficiency, Hereditary sensory and autonomic neuropathy (HSMN IV), Nutritional (beri beri), Neurosarcoidosis Usher's syndrome 1002

Vestibular Neuritis and Bilateral Vestibulopathy Figure 7. Bithermal caloric tests show markedly decreased respones to warm and cold stimulation bilaterally in a patient with bilateral vestibulopathy. 1003

Choi KDGoh EK Figure 8. Rotatory chair test shows increased phase lead and decreased gain of the vestibulo-ocular reflex in a patient with bilateral vestibulopathy. 1004

Vestibular Neuritis and Bilateral Vestibulopathy 11. Leigh RJ, Zee DS, eds. The neurology of eye movements. 4th ed. New York: Oxford University, 2006: 20-81. 12. Goldberg JM, Fernandez C. Physiology of peripheral neurons innervating semicircular canals of the squirrel monkey. I. Resting discharge and response to constant angular acceleration. J Neurophysiol 1971; 34: 635-660. 13. Miles FA, Braitman DJ. Long-term adaptive changes in primate vestibuloocular reflex. II. Electrophysiological observations on semicircular canal primary afferents. J Neurophysiol 1980; 43: 1426-1436. 14. Baloh RW. Clinical practice. Vestibular neuritis. N Engl J Med 2003; 348: 1027-1032. 15. Rinne T, Bronstein AM, Rudge P, Gresty MA, Luxon LM. Bilateral loss of vestibular function. Acta Otolaryngol 1995; 520: 247-250. 16. Kroenke K, Hoffman RM, Einstadter D. How common are various causes of dizziness?: A critical review. S Med J 2000; 93: 160-167. 17. Nedzelski JM, Barber HO, McIlmoyl L. Diagnosis in a dizziness unit. J Otolaryngol 1986; 15: 101-104. 18. Baloh RW, Halmagyi GM, eds. Disorders of the vestibular system. New York: Oxford University, 1996: 318-327. 19. Choi KD, Oh SY, Kim HJ, Koo JW, Cho BM, Kim JS. Recovery of vestibular imbalances after vestibular neuritis. Laryngoscope 2007; 117: 1307-1312. 10. Safran AB, Vibert D, Issoua D, Hausler R. Skew deviation after vestibular neuritis. Am J Ophthalmol 1994; 118: 238-245. 11. Furman JM, Cass SP, eds. Vestibular disorders: a case-study approach. 3rd ed. New York: FA Davis, 2003: 21-29. 12. Halmagyi GM, Curthoys IS. A clinical sign of canal paresis. Arch Neurol 1988; 45: 737-739. 13. Schubert MC, Tusa RJ, Grine LE, Herdman SJ. Optimizing the sensitivity of the head thrust test for identifying vestibular hypofunction. Phys Ther 2004; 84: 151-158. 14. Cremer PD, Halmagyi GM, Aw ST, Curthoys IS, McGarvie LA, Todd MJ, Black RA, Hannigan IP. Semicircular canal plane head impulses detect absent function of individual semicircular canals. Brain 1998; 121: 699-716. 15. Colebatch JG, Halmagyi GM, Skuse NF. Myogenic potentials generated by a click-evoked vestibulocollic reflex. J Neurol Neurosurg Psychiatry 1994; 57: 190-197. 16. Murofushi T, Halmagyi GM, Yavor RA, Colebatch JG. Absent vestibular evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior vestibular nerve involvement? Arch Otolaryngol Head Neck Surg 1996; 122: 845-848. 17. Hilding DA, Kanda T, House WF. Vestibular neuronitis and small acoustic neuroma: electron microscopic observations. Otol Clin N Am 1968; 1: 305-318. 18. Schuknecht HF, Kitamura K. Second Louis H. Clerf lecture, Vestibular neuritis. Ann Otol Rhinol Laryngol Suppl 1981; 90: 1-19. 19. Hirata T, Sekitani T, Okinaka Y, Matsuda Y. Serovirological study of vestibular neuronitis. Acta Otolaryngol Suppl 1989; 468: 371-373. 20. Kim JS, Lopez I, DiPatre PL, Liu F, Ishiyama A, Baloh RW. Internal auditory artery infarction: clinicopathologic correlation. Neurology 1999; 52: 40-44. 21. Fetter M, Dichgans J. Vestibular neuritis spares the inferior division of the vestibular nerve. Brain 1996; 119: 755-763. 22. Hotson JR, Baloh RW. Acute vestibular syndrome. N Engl J Med 1998; 339: 680-685. 23. Newman-Toker DE, Kattah JC, Alvernia JE, Wang DZ. Normal head impulse test differentiates acute cerebellar strokes from vestibular neuritis. Neurology 2008; 70: 2378-2385. 24. Selesnick SH, Jackler RK, Pitts LW. The changing clinical presentation of acoustic tumors in the MRI era. Laryngoscope 1993; 103: 431-436. 25. Ishikawa K, Edo M, Togawa K. Clinical observation of 32 cases of vestibular neuronitis. Acta Otolaryngol Suppl 1993; 503: 13-15. 26. Okinaka Y, Sekitani T, Okazaki H, Miura M, Tahara T. Progress of caloric response of vestibular neuronitis. Acta Otolaryngol Suppl 1993; 503: 18-22. 27. Bergenius J, Perols O. Vestibular neuritis: a follow-up study. Acta Otolaryngol 1999; 119: 895-899. 28. Meran A, Pfaltz CR. The acute vestibular paralysis. Arch Otorhinolaryngol 1975; 209: 229-244. 29. Cameron SA, Dutia MB. Lesion-induced plasticity in rat vestibular nucleus neurones dependent on glucocorticoid receptor activation. J Physiol 1999; 518: 151-158. 1005

Choi KDGoh EK 30. Kitahara T, Kondoh K, Morihana T, Okumura S, Horii A, Takeda N, Kubo T. Steroid effects on vestibular compensation in human. Neurol Res 2003; 25: 287-291. 31. Strupp M, Zingler VC, Arbusow V, Niklas D, Maag KP, Dieterich M, Bense S, Theil D, Jahn K, Brandt T. Methylprednisolone, valacyclovir, or the combination for vestibular neuritis. N Engl J Med 2004; 351: 354-361. 32. Cooksey FS. Rehabilitation in vestibular injury. Proc R Soc Med 1946; 39: 273-278. 33. Viber D, liard P, Hausler R. Bilateral idiopathic loss of peripheral vestibular function with normal hearing. Acta Otolaryngol 1995; 115: 611-615. 34. Dandy WE. The surgical treatment of Meniere's disease. Surg Gynecol Obstet 1941; 72: 421-425. 35. Bhansali SA, Stockwell CW, Bojrab DI. Oscillopsia in patients with loss of vestibular function. Otolaryngol Head Neck Surg 1993; 109: 120-126. 36. Chamber BR, Mai M, Barber HO. Bialteral vestibular loss, oscillopsia, and the cervico-ocular reflex. Otolaryngol Head Neck Surg 1985; 93: 403-407. 37. Mayer E, Bernstein J, Fostiropolous G. Salicylate ototoxicity. A clinical study. N Engl J Med 1965; 273: 587. 38. Rinne T, Bronstein AM, Rudge P, Gresty MA, Luxon LM. Bilateral loss of vestibular function: clinical findings in 53 patients. J Neurol 1998; 245: 314-321. 39. McGath JH, Barber HO, Stoyanoff S. Bilateral vestibular loss and oscillopsia. J Otolaryngol 1989; 18: 218-221. 40. Furman JM, Kamerer DB. Rotational responses in patients with bilateral caloric reduction. Acta Otolaryngol 1989; 108: 355-361. 41. Rinne T, Bronstein AM, Rudge P, Gresty MA, Luxon LM. Bilateral loss of vestibular function. Acta Otolaryngol 1995; 520: 247-250. 42. Gillespie MB, Minor LB. Prognosis in bilateral vestibular hypofunction. Laryngoscope 1999; 109: 35-41. Peer Reviewers Commentary 1006