Autoimmune Disease of the Inner Ear Clinical Presentation

Updated: Sep 27, 2018
  • Author: Neeraj N Mathur, MBBS, MS, DNB(ENT), MNAMS, FAMS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Presentation

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. [10]

    • 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. [11]

    • A study by Suchan et al detected antibodies against inner ear antigen in 52% of subjects with idiopathic progressive SNHL, 44% of subjects with Ménière disease, and none of the study’s healthy subjects. [12]

  • 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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