eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Autoimmune Disease: Follow-up

Author: Neeraj N Mathur, MBBS, MS, Professor, Department of Ear, Nose and Throat, Lady Hardinge Medical College and Associated Smt SK and Kalawati, Saran Children's Hospital, University of Delhi, India; Professor and Head, Department of Ear, Nose and Throat, BP Koirala Institute of Health Sciences, Nepal
Contributor Information and Disclosures

Updated: Oct 16, 2009

Follow-up

Prognosis

  • Fifty percent of patients with Autoimmune ear disease have an excellent response to steroids. Those with vestibular symptoms only are in particular responsive to steroids. Those with systemic disease have a lower response rate.4
  • Patients who demonstrate improvement in hearing in response to immunosuppressive therapy have a better prognosis than those who do not improve. The natural history of untreated immune-mediated inner ear disease is unknown at this time.

Patient Education

  • Inform patients with immune-mediated inner ear disease that regular follow-up care with their otolaryngologist is necessary to correlate subjective hearing fluctuations with objective audiometric data. This information helps guide immunosuppressive therapy, which needs to be monitored closely by a rheumatologist or immunologist.
  • For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Ménière Disease.

Miscellaneous

Medicolegal Pitfalls

  • Informed consent about the possible risks of immunosuppressive therapy in the management of immune-mediated inner ear disease is necessary before treatment.
  • Perform workup on patients with a history consistent with bilateral Ménière disease to evaluate for an autoimmune etiology before considering surgery for vestibular symptoms. A workup with results that are positive for an autoimmune etiology may lead to a trial of immunosuppressive therapy and, thereby, avoid the potential morbidity associated with surgery.
  • Perform workup such as serologic or imaging studies to exclude conditions such as otosyphilis or acoustic neuroma before immunosuppressive therapy is instituted.
  • Patient counseling may be helpful regarding the variable and often poor response to medical therapy for AIED. Patients need to know that immune-mediated inner ear disease is not a uniform disease with a simple diagnosis and treatment. The disease often results in long-term disability due to progressive and often permanent hearing loss. The response to aggressive immunosuppressive therapy can also be disappointing because of variable response rates and high risk of side effects.
 
Acknowledgments

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.



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References
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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. 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].

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

  4. 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].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

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

  10. 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].

  11. 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].

  12. 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].

  13. 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].

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

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. 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].

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

  21. 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].

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

  23. 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].

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

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

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

  27. 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].

  28. 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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Further Reading

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Keywords

Ménière disease, inner ear, autoimmune disease, idiopathic endolymphatic hydrops, Ménière syndrome, bilateral sensorineural hearing loss, SNHL, autoimmune inner ear disease, AIED, immune-mediated inner ear disease

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Contributor Information and Disclosures

Author

Neeraj N Mathur, MBBS, MS, Professor, Department of Ear, Nose and Throat, Lady Hardinge Medical College and Associated Smt SK and Kalawati, Saran Children's Hospital, University of Delhi, India; Professor and Head, Department of Ear, Nose and Throat, BP Koirala Institute of Health Sciences, Nepal
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.

Medical Editor

Robert A Battista, MD, FACS, Assistant Professor of Otolaryngology, Northwestern University Medical School; 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear corp Honoraria Board membership; Med El corp travel grants Consulting

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, 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, Department of Otolaryngology-Head and Neck Surgery, 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 unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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