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

American Journal of Medical Genetics. Part a
Kelley M DodsonWalter E Nance

Abstract

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.

References

Nov 1, 1978·Archives of Neurology·J M BicknellP Turner
Jul 1, 1992·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·S M Parnes, S A Weaver
Sep 1, 1991·Journal of Neuropathology and Experimental Neurology·A H Koeppen, R C Borke
May 1, 1988·Journal of Neuropathology and Experimental Neurology·A H Koeppen, M P Dentinger
Aug 11, 1988·The New England Journal of Medicine·D RigamontiR F Spetzler
Aug 1, 1995·Brain : a Journal of Neurology·J M FearnleyP Rudge
Sep 1, 1994·Movement Disorders : Official Journal of the Movement Disorder Society·Y RiverA Reches
Nov 1, 1993·Annals of Neurology·A H KoeppenR E Thach
Mar 1, 1993·Neurosurgery·A KumarK G TerBrugge
Apr 11, 1996·The New England Journal of Medicine·M GunelR P Lifton
Dec 1, 1996·The Journal of Laryngology and Otology·R M Irving, J M Graham
Jan 1, 1997·The Journal of Laryngology and Otology·M L Castelli, A Husband
Oct 15, 1998·Nature Genetics·L Van LaerG Van Camp
Nov 3, 1998·QJM : Monthly Journal of the Association of Physicians·A W GerbigT Hunziker
Oct 26, 1999·Neurology·M MascalchiC A Tassinari
Oct 12, 2000·American Journal of Human Genetics·A K LalwaniA N Mhatre
Jul 2, 2002·The Annals of Otology, Rhinology, and Laryngology·Shin-Ichi IshimotoMiwako Kimura
Aug 10, 2002·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Ingeborg J M DhoogeBart Vinck
Jan 25, 2003·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·S U KaleA Shehu
Aug 1, 1964·Journal of Neurology, Neurosurgery, and Psychiatry·B E TOMLINSON, J N WALTON
Jul 1, 1960·Journal of Neuropathology and Experimental Neurology·L IWANOWSKI, J OLSZEWSKI

❮ Previous
Next ❯

Citations

Apr 27, 2012·American Journal of Otolaryngology·Kelley M DodsonArti Pandya
Dec 18, 2009·American Journal of Otolaryngology·Noriko Yoshikawa, Barry E Hirsch
Feb 12, 2008·Otolaryngologic Clinics of North America·Michele B St Martin, Barry E Hirsch
Oct 20, 2006·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Deniz KonyaM Necmettin Pamir
Jun 27, 2006·American Journal of Otolaryngology·Bridget HathawayBarton Branstetter
Mar 21, 2006·The Neurologist·Basil Ridha, Keith A Josephs
Jul 7, 2011·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Sarah A SydlowskiJon Shallop
May 15, 2013·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Sarah A SydlowskiR Steven Ackley
Jun 13, 2018·Frontiers in Neurology·Aran YooJorge C Kattah

❮ Previous
Next ❯

Related Concepts

Related Feeds

Ataxias (MDS)

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on ataxia here.

Ataxia

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on ataxia here.

Ataxias

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on different types of ataxias here.

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.