Cochlear Implant Patients with Superficial Siderosis

2009 ◽  
Vol 20 (06) ◽  
pp. 348-352 ◽  
Author(s):  
Sarah A. Sydlowski ◽  
Michael J. Cevette ◽  
Jon Shallop ◽  
David M. Barrs
Keyword(s):  
Central Nervous System ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Case Reports ◽  
Superficial Siderosis ◽  
Viable Option ◽  
Retrospective Case ◽  
Fatal Disease ◽  
The Central Nervous System

Background: Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. Purpose: To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation. Research Design: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease. Results: Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option. Conclusions: It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.

Superficial siderosis of the central nervous system after brachial plexus injury

1994 ◽  
Vol 80 (5) ◽  
pp. 931-934 ◽  
Author(s):  
Virginio Bonito ◽  
Cristina Agostinis ◽  
Stefano Ferraresi ◽  
Carlo Alberto Defanti
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Brachial Plexus ◽  
Brachial Plexus Injury ◽  
Superficial Siderosis ◽  
Content Type ◽  
Initial Symptoms ◽  
The Central Nervous System ◽  
Fine Print

✓ Superficial siderosis is a rare condition characterized by deposition of hemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. A case is presented of severe superficial siderosis of the central nervous system in a 51-year-old man who had suffered a brachial plexus injury at the age of 20 years. The diagnosis was made by means of magnetic resonance imaging 16 years after the initial symptoms, which comprised bilateral hearing loss and anosmia. Subarachnoid bleeding was due to traumatic pseudomeningocele of the brachial plexus, a very unusual cause of superficial siderosis. This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease.

Hearing loss and vertigo in superficial siderosis of the central nervous system

2004 ◽  
Vol 25 (2) ◽  
pp. 142-149 ◽  
Author(s):  
Dominique Vibert ◽  
Rudolf Häusler ◽  
Karl-Olof Lövblad ◽  
Gerhard Schroth
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Superficial Siderosis ◽  
The Central Nervous System

Superficial Siderosis of the Central Nervous System: A Neglected Cause of Sensorineural Hearing Loss

1992 ◽  
Vol 107 (1) ◽  
pp. 69-77 ◽  
Author(s):  
Steven M. Parnes ◽  
Susan A. Weaver
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Imaging Techniques ◽  
Cranial Nerves ◽  
Sensorineural Hearing ◽  
Superficial Siderosis ◽  
System A ◽  
The Central Nervous System

Superficial siderosis of the central nervous system is a rare, often unrecognized condition in which there is a deposition of intracellular and extracellular hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves as a result of chronic bleeding in the subarachnoid space. On the basis of our cases and a review of the literature, the majority of patients manifest sensorineural hearing loss in addition to cerebellar dysfunction, pyramidal tract signs, and progressive mental deterioration. Recent imaging techniques—particularly CT and MRI—can define the presence of iron deposition, confirming the diagnosis ante mortem. From post mortem specimens, histologic sections demonstrate severe damage to the eighth nerve, with microglial metabolism of iron. Cases will be presented to illustrate these points.

Cochlear Implantation in a Patient with Superficial Siderosis of the Central Nervous System

2002 ◽  
Vol 23 (4) ◽  
pp. 468-472 ◽  
Author(s):  
Ingeborg J. M. Dhooge ◽  
Eddy De Vel ◽  
Henri Urgell ◽  
Stéphane Gallégo ◽  
Bart Vinck
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cochlear Implantation ◽  
Superficial Siderosis ◽  
The Central Nervous System

Cochlear implantation in superficial siderosis

1996 ◽  
Vol 110 (12) ◽  
pp. 1151-1153 ◽  
Author(s):  
Richard M. Irving ◽  
John M. Graham
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Cochlear Implantation ◽  
Superficial Siderosis ◽  
Nervous System Disorder ◽  
Central Nervous System Disorder ◽  
Hearing Rehabilitation ◽  
System Disorder ◽  
Careful Assessment

AbstractSuperficial siderosis is a rare central nervous system disorder characterized by deafness, ataxia, and pyramidal signs. The hearing loss is believed to be predominantly neural and is usuallly progressive and bilateral. Careful assessment is therefore necessary to determine the best approach to hearing rehabilitation. A case is presented of usperficial siderosis in a young woman who has benegited significantly from cochlear implantation using the Nucleus dvice.

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