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Autoimmune Disease of the Inner Ear Clinical Presentation

  • Author: Neeraj N Mathur, MBBS, MS; Chief Editor: Arlen D Meyers, MD, MBA  more...
 
Updated: Mar 04, 2016
 

History

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  • 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

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  • 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.[6]
    • 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.[7]
  • 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 Director-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: Royal Society of Medicine, Indian Medical Association, Association of Otolaryngologists of India, Cochlear Implant Group of India, National Academy of Medical Sciences (India), Neuro-Otological and Equilibriometric Society of India

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Peter S Roland, MD Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director, Clinical Center for Auditory, Vestibular, and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Chief of Pediatric Otology, Children’s Medical Center of Dallas; President of Medical Staff, Parkland Memorial Hospital; Adjunct Professor of Communicative Disorders, School of Behavioral and Brain Sciences, Chief of Medical Service, Callier Center for 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 Auditory Society, The Triological Society, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons, American Neurotology Society, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Otological Society

Disclosure: Received honoraria from Alcon Labs for consulting; Received honoraria from Advanced Bionics for board membership; Received honoraria from Cochlear Corp for board membership; Received travel grants from Med El Corp for consulting.

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, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;RxRevu;SymbiaAllergySolutions<br/>Received income in an amount equal to or greater than $250 from: Symbia<br/>Received from Allergy Solutions, Inc for board membership; Received honoraria from RxRevu for chief medical editor; Received salary from Medvoy for founder and president; Received consulting fee from Corvectra for senior medical advisor; Received ownership interest from Cerescan for consulting; Received consulting fee from Essiahealth for advisor; Received consulting fee from Carespan for advisor; Received consulting fee from Covidien for consulting.

Additional Contributors

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, Illinois State Medical Society, American Neurotology Society, American College of Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases 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
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