Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis

Acta Otolaryngol. 2006 May;126(5):548-52. doi: 10.1080/00016480500437369.

Abstract

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Audiometry, Evoked Response
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • C-Reactive Protein / metabolism
  • Diagnosis, Differential
  • Diuretics, Osmotic / administration & dosage
  • Dose-Response Relationship, Drug
  • Drug Therapy, Combination
  • Ear Diseases / diagnosis
  • Ear Diseases / drug therapy
  • Ear Diseases / etiology*
  • Ear Diseases / immunology
  • Ear, External*
  • Endolymphatic Hydrops / complications*
  • Endolymphatic Hydrops / diagnosis
  • Endolymphatic Hydrops / drug therapy
  • Endolymphatic Hydrops / immunology
  • Female
  • Hearing Loss, Sensorineural / diagnosis
  • Hearing Loss, Sensorineural / drug therapy
  • Hearing Loss, Sensorineural / etiology*
  • Hearing Loss, Sensorineural / immunology
  • Humans
  • Immunoglobulin M / blood
  • Immunosuppressive Agents / administration & dosage
  • Isosorbide / administration & dosage
  • Meniere Disease / diagnosis
  • Meniere Disease / drug therapy
  • Meniere Disease / etiology*
  • Meniere Disease / immunology
  • Middle Aged
  • Polychondritis, Relapsing / diagnosis
  • Polychondritis, Relapsing / drug therapy
  • Polychondritis, Relapsing / etiology*
  • Polychondritis, Relapsing / immunology
  • Prednisone / administration & dosage
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy
  • Primary Myelofibrosis / immunology
  • Thalidomide / administration & dosage
  • Treatment Outcome

Substances

  • Diuretics, Osmotic
  • Immunoglobulin M
  • Immunosuppressive Agents
  • Thalidomide
  • C-Reactive Protein
  • Prednisone
  • Isosorbide