Superficial siderosis of the central nervous system

An underdiagnosed cause of sensorineural hearing loss and ataxia

E. A. Pribitkin, L. Rondinella, S. I. Rosenberg, David Mark Yousem

Research output: Contribution to journalArticle

Abstract

Superficial siderosis of the central nervous system (CNS) is a rare but ultimately fatal disorder characterized by progressive hearing loss, ataxia, and dementia. Symptoms are caused by the deposition of hemosiderin in the CNS as a result of recurrent or persistent extravasation of blood into the cerebrospinal fluid. Sites most commonly involved include the cerebellum, brain stem, and eighth cranial nerve. In the past, diagnosis was most often made at autopsy, and this may account for the lack of reported cases in the otolaryngologic literature and the presumed rarity of the disorder. Now, diagnosis is confirmed by characteristic magnetic resonance imaging findings and treatment is targeted toward the source of bleeding. The diagnosis of superficial CNS siderosis should be entertained in all patients presenting with sensorineural hearing loss and ataxia.

Original languageEnglish (US)
Pages (from-to)415-418
Number of pages4
JournalAmerican Journal of Otology
Volume15
Issue number3
StatePublished - 1994
Externally publishedYes

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Siderosis
Sensorineural Hearing Loss
Ataxia
Central Nervous System
Vestibulocochlear Nerve
Hemosiderin
Hearing Loss
Cerebellum
Brain Stem
Cerebrospinal Fluid
Dementia
Autopsy
Magnetic Resonance Imaging
Hemorrhage
Therapeutics

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Superficial siderosis of the central nervous system : An underdiagnosed cause of sensorineural hearing loss and ataxia. / Pribitkin, E. A.; Rondinella, L.; Rosenberg, S. I.; Yousem, David Mark.

In: American Journal of Otology, Vol. 15, No. 3, 1994, p. 415-418.

Research output: Contribution to journalArticle

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