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Case Reports
. 2011 Oct;32(8):e63-5.
doi: 10.1097/MAO.0b013e318210b719.

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome: a histopathologic case report

David J Szmulewicz  1 , Saumil N Merchant, Gabor Michael Halmagyi
Affiliations
Case Reports

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome: a histopathologic case report

David J Szmulewicz et al. Otol Neurotol. 2011 Oct.
No abstract available

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Figures

FIG. 1
FIG. 1
A, Low-power view of the left ear (near-mid modiolar section) showing an atrophic vestibular nerve and a normal-appearing cochlear nerve. The cochlear turns, saccule, and utricle seemed normal. B, High-power view of the left vestibular nerve within the internal auditory canal. The nerve was severely atrophic, but there was no evidence of inflammation or vasculitis. There was severe loss of Scarpa's ganglion cells. C, Crista of the left posterior semicircular canal showing a good population of hair cells and supporting cells in the neuroepithelium. There was a reduction of dendrites within the stroma. D, Facial nerve from the right ear. There was severe diminution of the number of geniculate ganglion cells. CSF indicates cerebrospinal fluid.
See this image and copyright information in PMC

References

    1. Szmulewicz, et al. Neuropathy: an integral component of cerebellar ataxia neuropathy vestibular areflexia syndrome. Neurology. Reviewed with a request for revision.
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    1. Migliaccio AA, Halmagyi GM, McGarvie LA, et al. Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign. Brain. 2004;127:280–293. - PubMed
    1. Merchant SN, Nadol JB. Schuknecht's Pathology of the Ear. 3rd edition PMPH-USA Inc; Shelton, CT: 2010.
    1. Kuntzer, et al. Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies) Muscle Nerve. 2004;30:255–268. - PubMed

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