TY - JOUR
T1 - Superficial siderosis of the central nervous system
T2 - An underdiagnosed cause of sensorineural hearing loss and ataxia
AU - Pribitkin, E. A.
AU - Rondinella, L.
AU - Rosenberg, S. I.
AU - Yousem, D. M.
PY - 1994
Y1 - 1994
N2 - 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.
AB - 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.
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M3 - Article
C2 - 8579152
AN - SCOPUS:0028338241
SN - 0192-9763
VL - 15
SP - 415
EP - 418
JO - American Journal of Otology
JF - American Journal of Otology
IS - 3
ER -