Inner Ear, Autoimmune Disease
- Author: Neeraj N Mathur, MBBS, MS; Chief Editor: Arlen D Meyers, MD, MBA more...
Background
In 1979, McCabe first described a cohort of patients with idiopathic, rapidly progressive bilateral sensorineural hearing loss (SNHL). These patients' hearing improved after treatment with corticosteroids, thereby suggesting an autoimmune pathogenesis. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as true vertigo, generalized imbalance, and ataxia, may be present.
Inner ear. Pathophysiology
The term autoimmune inner ear disease (AIED) implies a direct attack of the immune system upon an endogenous inner ear antigen. Most of the evidence linking the immune system to cochleovestibular dysfunction is indirect; therefore, immune-mediated inner ear disease may be a preferred term. AIED is a clinical diagnosis based on its distinct clinical course, immune test results, and treatment response. The most important diagnostic finding is improvement in hearing observed with a trial of immunosuppressants.
Specific criteria for idiopathic progressive bilateral sensorineural hearing loss (IPBSNHL) include bilateral SNHL of at least 30 dB at any frequency with progression in at least one ear, defined as a threshold shift that is greater than 15 dB at any frequency or 10 dB at 2 or more consecutive frequencies or a significant change in discrimination score. This definition excludes patients with sudden SNHL occurring in less than 24 hours, which more likely is due to a microvascular or viral etiology.
A certain subset of patients with presumed Ménière disease (idiopathic endolymphatic hydrops) actually may have Ménière syndrome, in which the underlying pathophysiology is immune mediated. Typically, Ménière disease is initially diagnosed in these patients; however, fluctuating hearing loss in the contralateral ear develops later. This change may prompt a workup for AIED. Hughes et al found that approximately one half of their patients with AIED have manifestations of autoimmune Ménière syndrome.[1]
Epidemiology
Frequency
United States
Because the existence of autoimmune inner ear disease (AIED) has been recognized only since 1979, incidence is difficult to determine. Recent studies in the literature from large referral centers are based on relatively small sample sizes of patients who fit the criteria for diagnosis of AIED. As diagnostic tests for the condition become more specific and more is known about AIED, more patients will be identified who have an autoimmune basis for inner ear symptoms.
Sex
The condition has been suggested to be more common in female patients who may or may not have concomitant systemic autoimmune disease than in male patients.
Age
In most patients, initial onset of symptoms occurs at age 20-50 years. Cases in pediatric patients are uncommon.
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.
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].
Harris JP, Sharp PA. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss. Laryngoscope. May 1990;100(5):516-24. [Medline].
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].
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].
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].
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].
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].
McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol. Sep-Oct 1979;88(5 Pt 1):585-9. [Medline].
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].
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].
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].
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].
Luetje CM. Theoretical and practical implications for plasmapheresis in autoimmune inner ear disease. Laryngoscope. Nov 1989;99(11):1137-46. [Medline].
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].
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].
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].
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].
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].
Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum. Oct 2004;34(2):544-8. [Medline].
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].
Dornhoffer JL, Arenberg JG, Arenberg IK, Shambaugh GE Jr. Pathophysiological mechanisms in immune inner ear disease. Acta Otolaryngol Suppl. 1997;526:30-6. [Medline].
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].
Harris JP, Ryan AF. Fundamental immune mechanisms of the brain and inner ear. Otolaryngol Head Neck Surg. Jun 1995;112(6):639-53. [Medline].
Rauch SD. Clinical management of immune-mediated inner-ear disease. Ann N Y Acad Sci. Dec 29 1997;830:203-10. [Medline].
Soliman AM. Immune-mediated inner ear disease. Am J Otol. Nov 1992;13(6):575-9. [Medline].
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].
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].

