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Immune-Mediated Inner Ear Disease

  • Yuri Agrawal
  • Howard W. Francis

Abstract

Immune-mediated inner ear disease (IMIED) is the preferred term for the syndrome involving cochlear dysfunction, leading to degrees of sensorineural hearing loss and vertigo. Although vasculitis has been inferred to be the cause of IMIED in some cases, histopathological proof of vascu-litis involving the inner ear in this condition is lacking. The condition does appear to be mediated in some fashion by the immune system; hence, the name IMIED. IMIED can occur in association with a recognized autoimmune condition such as Sjögren's syndrome, Wegener's granulomatosis, or giant cell arteritis, or as an isolated entity in which no other organ dysfunction is evident. The onset of hearing loss in IMIED is rapid compared with other entities that lead to auditory dysfunction. Hearing loss in IMIED occurs over the course of weeks to months. Patients with IMIED-associated hearing loss have problems with both hearing acuity and word discrimination. Following conversations in noisy environments (e.g., in crowded restaurants) may be particularly challenging. For patients with IMIED, vestibular problems that result from cochlear damage can be as challenging as the auditory dysfunction, if not more so. Up to 70% of patients diagnosed with IMIED demonstrate clinical improvement following glucocorticoid treatment. Prednisone (1 mg/kg/day) is a reasonable starting dose for the treatment of rapidly progressive sensorineural hearing loss secondary to IMIED. If the patient regains significant auditory and vestibular function within 2 weeks, tapering of the prednisone dose can begin in a taper designed to discontinue the medication over 2 months. For patients with severe or recurrent audiovestibular dysfunction secondary to IMIED, it is not clear whether long-term courses of high-dose glucocorticoids and additional immunosuppressive agents are effective in restoring inner ear function. Such treatment approaches should be used cautiously.

Keywords

Hearing Loss Giant Cell Arteritis Sensorineural Hearing Loss Relapse Polychondritis Endolymphatic Hydrops 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Billings PB, Keithley EM, Harris JP. Evidence linking the 68 kilodalton antigen identified in progressive sensorineural hearing loss patient sera with heat shock protein 70. Ann Otol Rhinol Laryngol. 1995;104(3):181–8PubMedGoogle Scholar
  2. Bloch DB, San Martin JE, Rauch SD, Moscicki RA, Bloch KJ. Serum antibodies to heat shock protein 70 in sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 1995;121(10):1167–71PubMedCrossRefGoogle Scholar
  3. Bovo R, Aimoni C, Martini A. Immune-mediated inner ear disease. Acta Otolaryngol. 2006;126(10):1012–21PubMedCrossRefGoogle Scholar
  4. Disher MJ, Ramakrishnan A, Nair TS, et al Human autoantibodies and monoclonal antibody KHRI-3 bind to a phylogenetically conserved inner-ear-supporting cell antigen. Ann N Y Acad Sci. 1997;830: 253–65PubMedCrossRefGoogle Scholar
  5. Harris JP, Weisman MH, Derebery JM, et al Treatment of corticoster-oid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. JAMA. 2003;290(14):1875–83PubMedCrossRefGoogle Scholar
  6. Kikuchi M. On the “sympathetic otitis”. Zibi Rinsyo Kyoto. 1959; 52:600Google Scholar
  7. Kornblut AD, Wolff SM, deFries HO, Fauci AS. Wegener's granuloma-tosis. Otolaryngol Clin N Am. 1982a;15(3):673–83Google Scholar
  8. Kornblut AD, Wolff SM, Fauci AS. Ear disease in patients with Wegener's granulomatosis. Laryngoscope 1982b;92(7 Pt 1): 713–17CrossRefGoogle Scholar
  9. Matteson EL, Fabry DA, Facer GW, et al Open trial of methotrexate as treatment for autoimmune hearing loss. Arthritis Rheum. 2001;45(2): 146–50PubMedCrossRefGoogle Scholar
  10. Mccabe BF Autoimmune sensorineural hearing loss. Ann otol Rhinol Laryngol. Sep-Oct 1979;88 (5pt1):585–589Google Scholar
  11. Minor L, Carey J, Lustig LR. Disorders of balance. In: Lustig LR, Niparko JK, editors. Clinical neurotology: diagnosing and managing disorders of hearing, balance and the facial nerve. New York: Martin Dunitz; 2003. p. 225–44Google Scholar
  12. Minor L, Zee DS. Clinical evaluation of the patient with dizziness. In: Lustig LR, Niparko JK, editors. Clinical neurotology: diagnosing and managing disorders of hearing, balance and the facial nerve. New York: Martin Dunitz; 2003. p. 81–110Google Scholar
  13. Moscicki RA, San Martin JE, Quintero CH, Rauch SD, Nadol JB Jr, Bloch KJ. Serum antibody to inner ear proteins in patients with progressive hearing loss. Correlation with disease activity and response to corticosteroid treatment. JAMA. 1994;272(8):611–16PubMedCrossRefGoogle Scholar
  14. Nair TS, Kozma KE, Hoefling NL, et al Identification and characterization of choline transporter-like protein 2, an inner ear glycoprotein of 68 and 72 kDa that is the target of antibody-induced hearing loss. J Neurosci. 2004;24(7):1772–9PubMedCrossRefGoogle Scholar
  15. Nair TS, Raphael Y, Dolan DF, et al Monoclonal antibody induced hearing loss. Hear Res. 1995;83(1–2):101–13PubMedCrossRefGoogle Scholar
  16. Pletcher SD, Cheung SW. Syphilis and otolaryngology. Otolaryngol Clin N Am. 2003;36(4):595–605, viCrossRefGoogle Scholar
  17. Roeser R. Audiology desk reference: a guide to the practice of audiol-ogy. 1 ed. New York: Thieme; 1996Google Scholar
  18. Ruckenstein MJ. Autoimmune inner ear disease. Curr Opin Otolaryngol Head Neck Surg. 2004;12(5):426–30PubMedCrossRefGoogle Scholar
  19. Sajjadi H, Paparella MM. Meniere's disease. Lancet 2008;372 (9636):406–14PubMedCrossRefGoogle Scholar
  20. Schindler JS, Niparko JK. Imaging quiz case 1. Transverse temporal bone fractures (left) with subsequent progressive SNHL, consistent with sympathetic cochleolabyrinthitis. Arch Otolaryngol Head Neck Surg. 1998;124(7):814, 816–8Google Scholar
  21. Schubert MC, Minor LB. Vestibulo-ocular physiology underlying vestibular hypofunction. Phys Ther. 2004;84(4):373–85PubMedGoogle Scholar
  22. Stone JH, Francis HW. Immune-mediated inner ear disease. Curr Opin Rheumatol. 2000;12(1):32–40PubMedCrossRefGoogle Scholar
  23. ten Cate WJ, Bachor E. Autoimmune-mediated sympathetic hearing loss: a case report. Otol Neurotol. 2005;26(2):161–5CrossRefGoogle Scholar
  24. Trune DR, Kempton JB, Mitchell CR, Hefeneider SH. Failure of elevated heat shock protein 70 antibodies to alter cochlear function in mice. Hear Res. 1998;116(1–2):65–70PubMedCrossRefGoogle Scholar
  25. Yeom K, Gray J, Nair TS, et al Antibodies to HSP-70 in normal donors and autoimmune hearing loss patients. Laryngoscope. 2003;113(10): 1770–6PubMedCrossRefGoogle Scholar
  26. Zeitoun H, Beckman JG, Arts HA, et al Corticosteroid response and supporting cell antibody in autoimmune hearing loss. Arch Otolaryngol Head Neck Surg. 2005;131(8):665–72PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • Yuri Agrawal
    • 1
  • Howard W. Francis
    • 2
  1. 1.Johns Hopkins UniversityDepartment of OtolaryngologyBaltimoreUSA
  2. 2.Otolaryngology — Head and Neck SurgeryJohns Hopkins University School of MedicineBaltimoreUSA

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