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Autoimmune inner ear disease--a real entity?

B P Barna1, G B Hughes

  • 1Department of Neurological Surgery, Cleveland Clinic Foundation, Ohio, USA.

Clinics in Laboratory Medicine
|October 8, 1997
PubMed
Summary
This summary is machine-generated.

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Sudden, progressive inner ear disease may involve immune responses, but is currently termed "immune-mediated" due to lack of definitive autoimmune markers. Further research is needed for accurate diagnosis and treatment.

Area of Science:

  • Otolaryngology
  • Immunology
  • Autoimmune Diseases

Background:

  • Evidence suggests immune system involvement in sudden, rapidly progressive, bilateral inner ear disease.
  • Inner ear contains immune cells and mediators; animal models show damage after immunization with inner ear tissue.
  • Human sensorineural hearing loss (SNHL) can occur with systemic immune diseases and improve with immunosuppression.

Purpose of the Study:

  • To evaluate the evidence for autoimmune mechanisms in sudden-onset, rapidly progressive, bilateral inner ear disease.
  • To differentiate between

Main Methods:

  • Review of existing evidence, including animal models and human studies.
  • Clinical assessment and laboratory testing to rule out other causes.
  • Investigational immunoassays for antibodies to inner ear proteins and hsp 70.

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Main Results:

  • While evidence supports immune involvement, definitive autoimmune criteria are not met.
  • Inner ear damage can occur from immune responses to irrelevant antigens.
  • Histopathology and specific antigen identification in humans are still lacking.

Conclusions:

  • Clinical inner ear disease with immune involvement is termed "immune-mediated" (IMIED), not definitively "autoimmune."
  • IMIED is likely a heterogeneous group of diseases with common endpoints.
  • Investigational immunoassays show promise but require further validation for sensitivity and specificity.