Causative factors and epidemiology of bilateral vestibulopathy in 255 patients

Ann Neurol. 2007 Jun;61(6):524-32. doi: 10.1002/ana.21105.


Objective: To determine the causative factors and epidemiology of bilateral vestibulopathy (BV).

Methods: This is a retrospective review of 255 patients (mean age, 62 +/- 16 years) with BV diagnosed in our dizziness unit between 1988 and 2005. All patients had undergone a standardized neurophthalmological and neurootological examination, electronystagmography with caloric irrigation, cranial magnetic resonance imaging or computed tomography (n = 214), and laboratory tests.

Results: Sixty-two percent of the study population were male subjects. Previous vertigo attacks had occurred in 36%, indicating a sequential manifestation. The definite cause of BV was determined in 24% and the probable cause in 25%: The most common causes were ototoxic aminoglycosides (13%), Menière's disease (7%), and meningitis (5%). Strikingly, 25% exhibited cerebellar signs. Cerebellar dysfunction was associated with peripheral polyneuropathy in 32% compared with 18% in BV patients without cerebellar signs. Hypoacusis occurred bilaterally in 25% and unilaterally in 6% of all patients. It appeared most often in patients with BV caused by Cogan's syndrome, meningitis, or Menière's disease.

Interpretation: The cause of BV remains unclear in about half of all patients despite intensive examinations. A large subgroup of these patients have associated cerebellar dysfunction and peripheral polyneuropathy. This suggests a new syndrome that may be caused by neurodegenerative or autoimmune processes.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Aminoglycosides / adverse effects
  • Autoantibodies / blood
  • Causality
  • Cerebellar Diseases / epidemiology*
  • Cerebellar Diseases / physiopathology
  • Child
  • Comorbidity
  • Disease Progression
  • Female
  • Germany / epidemiology
  • Hearing Disorders / blood
  • Hearing Disorders / epidemiology*
  • Hearing Disorders / immunology
  • Humans
  • Male
  • Meniere Disease / epidemiology*
  • Meningitis / epidemiology*
  • Middle Aged
  • Retrospective Studies
  • Sex Distribution
  • Syndrome
  • Vestibular Diseases / epidemiology*
  • Vestibular Diseases / etiology
  • Vestibular Diseases / physiopathology*


  • Aminoglycosides
  • Autoantibodies