Autoimmune Disease of the Inner Ear Clinical Presentation

  • Author: Neeraj N Mathur, MBBS, MS; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Apr 2, 2012
 

History

  • Hearing loss: The hallmark of immune-mediated inner ear disease is sensorineural hearing loss (SNHL), which usually is bilateral and occurs rapidly over weeks to months.
  • Fluctuation: Sensorineural loss can fluctuate and stabilize at a certain level, or it can progress without fluctuation.
  • Laterality: Bilateral hearing loss occurs in most patients (79%). Occasionally, only one ear is involved initially, with the contralateral ear developing hearing loss later. In bilateral cases, audiometric thresholds can be symmetric or asymmetric.
  • Speech discrimination scores: Discrimination scores often are poor in immune-mediated inner ear disease. Therefore, in cases of unilateral or bilateral-asymmetric disease, include diagnostic imaging and serologic studies in the workup to exclude retrocochlear disease and syphilitic inner ear disease.
  • Vestibular symptoms: Approximately 50% of patients complain of vestibular symptoms typical of Ménière disease. Vestibular symptoms can include disequilibrium, ataxia, motion intolerance, positional vertigo, and episodic vertigo.
  • Tinnitus and aural fullness: As many as 25-50% of patients also have symptoms of tinnitus and aural fullness, which can fluctuate in severity.
  • Systemic autoimmune disease: Coexisting systemic autoimmune disease occurs in 15-30% of patients. Diagnoses include rheumatoid arthritis, ulcerative colitis, systemic lupus erythematosus, and polyarteritis nodosa.
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Physical

Findings from physical examination of the ear usually are normal in patients with immune-mediated inner ear disease. Occasionally, associated systemic autoimmune diseases can affect the external ear skin or middle ear mucosa.

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Causes

  • Association with type I immune reaction involving immunoglobulin E (IgE)–mediated response
    • Solimon postulated that histamine-induced vasodilation of endolymphatic sac vasculature may result in endolymphatic hydrops because of impaired fluid transport.
    • A large percentage of patients treated with immunotherapy for inhalant allergies demonstrated improvement in vertigo and other symptoms of Ménière disease, which suggests an association between IgE-mediated disease and inner ear dysfunction.
  • Production of autoantibodies to inner ear antigen
    • Yoo et al reported that rodents injected with type II collagen developed new-onset SNHL and pathologic cochlear changes that appear to be immune mediated.[4]
    • Harris and Sharp used Western-blot analysis to identify a 68-kd antibody present in the serum of 35% of their patients with idiopathic progressive SNHL. This antibody targeted a bovine inner ear antigen, suggesting an autoimmune basis for hearing loss.[5]
  • Production of immune complexes
    • In a series of 30 patients with Ménière disease, 96% had elevated levels of circulating immune complexes compared with 20% of control subjects.
    • Patients with systemic lupus erythematosus have evidence of circulating immune complexes and multiple autoantibodies. Reports exist of SNHL associated with systemic lupus erythematosus. Likewise, reports of patients with Wegener granulomatosis cite SNHL in association with vasculitis of the cochlear and endolymphatic sac arteries.
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Contributor Information and Disclosures
Author

Neeraj N Mathur, MBBS, MS  Professor, Department of ENT and Head Neck Surgery, Vardhman Mahavir Medical College and Associated Safdarjang Hospital; Professor, Delhi University and Indraprastha University, India

Neeraj N Mathur, MBBS, MS is a member of the following medical societies: Association of Otolaryngologists of India, Cochlear Implant Group of India, Indian Medical Association, National Academy of Medical Sciences, India, Neuro-Otologic and Equlibriometric Society of India, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert A Battista, MD, FACS  Assistant Professor of Otolaryngology, Northwestern University, The Feinberg School of Medicine; Physician, Ear Institute of Chicago, LLC

Robert A Battista, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, and Illinois State Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Peter S Roland, MD  Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development

Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Alcon Labs Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear Corp Honoraria Board membership; Med El Corp travel grants Consulting; Foresight Consulting fee Consulting

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA  Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Medvoy Ownership interest Management position; Cerescan Imaging Consulting; Headwatersmb Consulting fee Consulting; Venturequest Royalty Consulting

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Shelley Jaquish, MD, and William L Meyerhoff, MD, PhD, to the development and writing of this article.

References

  1. Hughes GB, Barna BP, Calarese LH. Immunologic Disorders of the Inner Ear. In: Bailey BJ, ed. Head and Neck Surgery-Otolaryngology. Philadelphia, Pa: Lippincott; 1993:1833-1842.

  2. Berlinger NT. Meniere's disease: new concepts, new treatments. Minn Med. Nov 2011;94(11):33-6. [Medline].

  3. Huang NC, Sataloff RT. Autoimmune inner ear disease in children. Otol Neurotol. Feb 2011;32(2):213-6. [Medline].

  4. Yoo TJ, Tomoda K, Stuart JM, Cremer MA, Townes AS, Kang AH. Type II collagen-induced autoimmune sensorineural hearing loss and vestibular dysfunction in rats. Ann Otol Rhinol Laryngol. May-Jun 1983;92(3 Pt 1):267-71. [Medline].

  5. Harris JP, Sharp PA. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss. Laryngoscope. May 1990;100(5):516-24. [Medline].

  6. Dayal VS, Ellman M, Sweiss N. Autoimmune inner ear disease: clinical and laboratory findings and treatment outcome. J Otolaryngol Head Neck Surg. Aug 2008;37(4):591-6. [Medline].

  7. 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. Aug 24-31 1994;272(8):611-6. [Medline].

  8. Rauch SD, San Martin JE, Moscicki RA, Bloch KJ. Serum antibodies against heat shock protein 70 in Menière's disease. Am J Otol. Sep 1995;16(5):648-52. [Medline].

  9. Gong SS, Yu DZ, Wang JB. Relationship between three inner ear antigens with different molecular weights and autoimmune inner ear disease. Acta Otolaryngol. Jan 2002;122(1):5-9. [Medline].

  10. Cao MY, Deggouj N, Gersdorff M, Tomasi JP. Guinea pig inner ear antigens: extraction and application to the study of human autoimmune inner ear disease. Laryngoscope. Feb 1996;106(2 Pt 1):207-12. [Medline].

  11. Pathak S, Goldofsky E, Vivas EX, Bonagura VR, Vambutas A. IL-1ß is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease. J Immunol. Feb 1 2011;186(3):1870-9. [Medline]. [Full Text].

  12. McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol. Sep-Oct 1979;88(5 Pt 1):585-9. [Medline].

  13. Sismanis A, Thompson T, Willis HE. Methotrexate therapy for autoimmune hearing loss: a preliminary report. Laryngoscope. Aug 1994;104(8 Pt 1):932-4. [Medline].

  14. Harris JP, Weisman MH, Derebery JM, et al. Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. JAMA. Oct 8 2003;290(14):1875-83. [Medline].

  15. Cohen S, Shoup A, Weisman MH, Harris J. Etanercept treatment for autoimmune inner ear disease: results of a pilot placebo-controlled study. Otol Neurotol. Sep 2005;26(5):903-7. [Medline].

  16. Matteson EL, Choi HK, Poe DS, et al. Etanercept therapy for immune-mediated cochleovestibular disorders: a multi-center, open-label, pilot study. Arthritis Rheum. Jun 15 2005;53(3):337-42. [Medline].

  17. Luetje CM. Theoretical and practical implications for plasmapheresis in autoimmune inner ear disease. Laryngoscope. Nov 1989;99(11):1137-46. [Medline].

  18. Parnes LS, Sun AH, Freeman DJ. Corticosteroid pharmacokinetics in the inner ear fluids: an animal study followed by clinical application. Laryngoscope. Jul 1999;109(7 Pt 2):1-17. [Medline].

  19. Silverstein H. Use of a new device, the MicroWick, to deliver medication to the inner ear. Ear Nose Throat J. Aug 1999;78(8):595-8, 600. [Medline].

  20. Swan EE, Mescher MJ, Sewell WF, Tao SL, Borenstein JT. Inner ear drug delivery for auditory applications. Adv Drug Deliv Rev. Dec 14 2008;60(15):1583-99. [Medline].

  21. Hamid M, Trune D. Issues, indications, and controversies regarding intratympanic steroid perfusion. Curr Opin Otolaryngol Head Neck Surg. Oct 2008;16(5):434-40. [Medline].

  22. Haynes DS, O'Malley M, Cohen S, Watford K, Labadie RF. Intratympanic dexamethasone for sudden sensorineural hearing loss after failure of systemic therapy. Laryngoscope. Jan 2007;117(1):3-15. [Medline].

  23. Bowman CA, Linthicum FH Jr, Nelson RA, Mikami K, Quismorio F. Sensorineural hearing loss associated with systemic lupus erythematosus. Otolaryngol Head Neck Surg. Feb 1986;94(2):197-204. [Medline].

  24. Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum. Oct 2004;34(2):544-8. [Medline].

  25. Derebery MJ, Rao VS, Siglock TJ, Linthicum FH, Nelson RA. Menière's disease: an immune complex-mediated illness?. Laryngoscope. Mar 1991;101(3):225-9. [Medline].

  26. Dornhoffer JL, Arenberg JG, Arenberg IK, Shambaugh GE Jr. Pathophysiological mechanisms in immune inner ear disease. Acta Otolaryngol Suppl. 1997;526:30-6. [Medline].

  27. Harris JP. Immunology of the inner ear: evidence of local antibody production. Ann Otol Rhinol Laryngol. Mar-Apr 1984;93(2 Pt 1):157-62. [Medline].

  28. Harris JP, Ryan AF. Fundamental immune mechanisms of the brain and inner ear. Otolaryngol Head Neck Surg. Jun 1995;112(6):639-53. [Medline].

  29. Rauch SD. Clinical management of immune-mediated inner-ear disease. Ann N Y Acad Sci. Dec 29 1997;830:203-10. [Medline].

  30. Soliman AM. Immune-mediated inner ear disease. Am J Otol. Nov 1992;13(6):575-9. [Medline].

  31. Tomiyama S, Harris JP. The role of the endolymphatic sac in inner ear immunity. Acta Otolaryngol. Mar-Apr 1987;103(3-4):182-8. [Medline].

 
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