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Autoimmune endolymphatic hydrops: five-year review.

G B Hughes1, B P Barna, S E Kinney

  • 1Department of Otolaryngology and Communicative Disorders, Cleveland Clinic Foundation, OH 44106.

Otolaryngology--Head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery
|March 1, 1988
PubMed
Summary
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Autoimmune endolymphatic hydrops, often bilateral, affects over half of autoimmune inner ear disease patients. Treatment with prednisone and lifestyle changes showed improvement in hearing and vestibular function for many, suggesting medical management is key.

Area of Science:

  • Otolaryngology
  • Immunology
  • Neurology

Background:

  • Autoimmune inner ear disease (AIED) can manifest as endolymphatic hydrops.
  • Autoimmune endolymphatic hydrops (AEH) presents a diagnostic and therapeutic challenge.

Purpose of the Study:

  • To review the clinical experience with AEH over five years.
  • To evaluate treatment outcomes for hearing and vestibular function in AEH patients.

Main Methods:

  • Retrospective review of 52 patients with AIED, focusing on 27 with AEH.
  • Treatment included dietary changes, diuretics, prednisone, and in refractory cases, cytotoxic drugs or lymphocytoplasmapheresis.
  • Audiometric and vestibular follow-up averaged 23 months, assessed using American Academy of Otolaryngology--Head and Neck Surgery guidelines.

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Main Results:

  • AEH was diagnosed in 52% of AIED patients, frequently bilateral.
  • Hearing improved or stabilized in 9 of 12 patients; vestibular function changes mirrored auditory outcomes.
  • Medical therapy was the primary treatment approach.

Conclusions:

  • AEH should be considered in bilateral inner ear symptoms unresponsive to conventional therapy or with positive immune markers.
  • Medical management, including prednisone and potentially cytotoxic agents, is recommended for AEH.
  • Further research is needed on surgical interventions for medically refractory cases.