eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Autoimmune Disease

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

Introduction

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.

Inner ear.

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Inner ear.

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

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.

Clinical

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.

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.

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.2
    • 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.3
  • 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.

More on Inner Ear, Autoimmune Disease

Overview: Inner Ear, Autoimmune Disease
Differential Diagnoses & Workup: Inner Ear, Autoimmune Disease
Treatment & Medication: Inner Ear, Autoimmune Disease
Follow-up: Inner Ear, Autoimmune Disease
Multimedia: Inner Ear, Autoimmune Disease
References
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References

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  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].

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  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].

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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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