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Idiopathic Dandy's syndrome

C A Syms1, J W House

  • 1Wilford Hall Medical Center, San Antonio, Texas, USA.

Otolaryngology--Head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery
|January 1, 1997
PubMed
Summary

Dandy's syndrome, caused by bilateral vestibular loss, presents with visual and gait issues. Idiopathic cases show more women and normal hearing, with limited rehabilitation success in both known and unknown cause groups.

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Area of Science:

  • Neurology
  • Otolaryngology
  • Vestibular System Disorders

Background:

  • Dandy's syndrome, characterized by visual
  • jumbling
  • and gait instability, results from bilateral vestibular nerve section or loss.
  • Peripheral vestibular system dysfunction leads to reduced or absent caloric responses.

Purpose of the Study:

  • To investigate differences between Dandy's syndrome patients with known etiologies and those with idiopathic causes.
  • To compare clinical characteristics, audiological findings, and symptom outcomes in these patient groups.

Main Methods:

  • Retrospective review of 105 Dandy's syndrome patients diagnosed between 1984 and 1994.
  • Data collection included presenting symptoms, physical examination, audiometry, electronystagmography, laboratory results, and etiology.
  • Comparison of patients with known causes (e.g., Meniere's disease, ototoxicity, trauma) versus those with no identified cause.

Main Results:

  • 32% of patients had idiopathic Dandy's syndrome.
  • Idiopathic cases showed a higher proportion of women (68% vs. 41%) and normal audiograms (53% vs. 19%) compared to known cause groups.
  • Symptom improvement rates were low, with 28% in known cause groups and 40% in idiopathic groups, indicating rehabilitation challenges.

Conclusions:

  • Idiopathic Dandy's syndrome exhibits distinct demographic and audiological profiles.
  • Vestibular loss significantly impacts quality of life, with limited spontaneous or rehabilitation-driven symptom recovery.
  • Further research into the underlying mechanisms and improved management strategies for Dandy's syndrome is warranted.

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