Am J Med Genet. 2004 Sep 15;130A(1):22.  Related Articles, Links  

Superficial siderosis: A potentially important cause of genetic as well as
non-genetic deafness.

Dodson KM, Sismanis A, Nance WE.

Department of Otolaryngology/Head and Neck Surgery, Virginia Commonwealth
University School of Medicine and Health Sciences, Medical College of Virginia
Campus, Richmond, Virginia.

Superficial siderosis is an important disease that is increasingly being
recognized as a cause of sensorineural hearing loss. Hemosiderin, resulting
from repeated episodes of subarachnoid bleeding, is deposited preferentially on
the surface of the eighth nerve, cerebellum, and brain stem as a consequence of
glial catabolism of ferritin within those structures. This deposition
eventually results in destruction and demyelination within the central nervous
system, leading to the cardinal clinical findings of superficial siderosis:
hearing loss, ataxia, and myelopathy. This mechanism may contribute to the
pathogenesis of several forms of genetic deafness, and should be considered as
a diagnostic possibility in cases of late onset deafness even in the absence of
an overt history of subarachnoid bleeding. Copyright 2004 Wiley-Liss, Inc.

PMID: 15368490 [PubMed - in process]

--------------------------------------------------------------------------
------

Who loves ya.
Tom