Otopathology in idiopathic Dandy's syndrome

Otol Neurotol. 2013 Aug;34(6):1099-103. doi: 10.1097/MAO.0b013e3182814e74.


Background: Dandy's syndrome, or bilateral vestibular hypofunction and oscillopsia, may cause chronic disequilibrium aggravated by head movement or in the presence of reduced light. It may be secondary to ototoxicity, central nervous system tumors, Ménière's syndrome, infections, or trauma or may be idiopathic.

Objective: To describe the temporal bone histopathology in one individual with idiopathic Dandy's syndrome.

Materials and methods: Temporal bones from 1 individual were removed at autopsy and studied using light and Nomarski microscopy.

Results: In this case, the otopathology demonstrated vestibular atelectasis of the membranous labyrinth of the superior, lateral, and posterior semicircular canals but not the utricle or saccule bilaterally. The findings also included mild hair cell loss in the cristae of all semicircular canals and of the utricular and saccular maculae and severely reduced neuronal count in Scarpa's ganglion bilaterally. There was also a scattered loss of inner and outer hair cells throughout the cochlea and moderate-to-severe loss of cochlear neurons bilaterally.

Conclusion: We have reported the histopathologic findings in a case of idiopathic Dandy's syndrome. Both temporal bones showed vestibular atelectasis of all three semicircular canals, preservation of normal saccule and utricle, and severe reduction of the neuronal population in Scarpa's ganglion bilaterally. Both ears also showed substantial degeneration of the spiral ganglion of the cochleas. Severe Scarpa's ganglion degeneration was also noted in the only other case of idiopathic Dandy's Syndrome in the literature. However, that other case had no evidence of vestibular atelectasis and had normal hearing.

Publication types

  • Case Reports

MeSH terms

  • Audiometry
  • Autopsy
  • Cell Count
  • Cochlea / pathology
  • Disease Progression
  • Ear, Inner / pathology*
  • Evoked Potentials, Auditory, Brain Stem / physiology
  • Fatal Outcome
  • Head Movements
  • Hearing Loss, Sensorineural / etiology
  • Hearing Loss, Sensorineural / pathology
  • Humans
  • Male
  • Middle Aged
  • Neuroepithelial Cells / pathology
  • Postural Balance
  • Saccule and Utricle / pathology
  • Semicircular Canals / pathology
  • Syndrome
  • Temporal Bone / pathology
  • Vestibular Nerve / pathology
  • Vestibulocochlear Nerve Diseases / pathology*