Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

Castelli, Monica; Husband, A. D.

doi:10.1017/s0022215100136448

Cited by 18 publications

(9 citation statements)

References 16 publications

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“…These patterns may well reflect the tonotopic localization of axons transmitting the high frequency sound at the surface of the auditory nerve. However, the hearing loss may initially be asymmetric and/or unilateral [Parnes and Weaver, 1992;Fearnley et al, 1995;Longridge et al, 1996;Castelli and Husband, 1997;Li et al, 2001;Kale et al, 2003]. Neurotologic testing shows a retrocochlear pattern of impairment, ranging from increased wave latencies to an undetectable Auditory Brainstem Response (ABR) [Yamana et al, 2001;Dhooge et al, 2002].…”

Section: Discussionmentioning

confidence: 99%

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

Dodson

Sismanis

Nance

2004

American J of Med Genetics Pt A

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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.

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Section: Discussionmentioning

confidence: 99%

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

Dodson

Sismanis

Nance

2004

American J of Med Genetics Pt A

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“…The most common clinical manifestationsinclude bilateral sensorineural hearing loss (SNHL) (95%) and ataxia (88%). [6,7] The other features include bilateral hemiparesis, bladderdisturbances, anosmia, dementia and headache. [1,2] The treatment is directed at the underlying cause; cochlear transplantation may be required for SNHL.…”

Section: Casementioning

confidence: 99%

Superficial siderosis

et al. 2009

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Case 1An elderly patient presented with insidious onset of sensorineural hearing loss. The past history was unremarkable except for insidious onset of weakness of left lower limb. Physical examination revealed left hemiparesis with upgoing left plantar. Magnetic resonance imaging (MRI) of brain revealed a large flow void in the interpeduncular cistern suggestive of aneurysm arising from the top of the basilar artery. In addition, hypointense signal was noted (prominent on T2 and FLAIR images) on the surface of both cerebral hemispheres, the pons, cerebellar folia and medulla. The final diagnosis was superficial siderosis secondary to leaking basilar artery aneurysm [ Figures 1 and 2].Case 2 A middle-aged female, with complaints of progressive ataxia and bilateral sensorineural hearing loss was referred for MRI of brain. The patient had significant medical history of adult onset seizure disorder well controlled with anti-epileptic drugs. Physical examination revealed senorineural deafness and limb and gait ataxia. Computed tomography (CT) showed a small intraparenchymal hyperdense lesion in the right parietal region suggestive of hematoma. MRI of brain revealed a typical popcorn lesion in right parietal region with heterogeneous signal intensity on T1, T2 and FLAIR images with fluid levels suggestive of a cavernous angioma. In addition, hypointense signal was noted (especially on T2 images) coating the cerebral sulci, pons, midbrain and medulla. Mild cerebellar cortical atrophy was also noted [ Figures 3 and 4]. The final diagnosis was superficial siderosis secondary to cavernous malformation.Superficial siderosis is the result of chronic and recurrent subarachnoid hemorrhage and the causes include: Figure 1: Coronal T2 images show hypointense signal on the surfaces of the sulci due to hemosiderin staining

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“…This rarely encountered disease first described by Noetzel [2] is characterized by deposition of hemosiderin on the surface of the brain, cerebellum, brain stem, cranial nerves and spinal cord. SSCN may be manifested as a progressive sensorineural hearing loss (SNHL), cerebellar dysfunction, pyramidal tract signs, or mental retardation [1,[3][4][5]. Since the eighth cranial nerve is invariably darkly pigmented and atrophied histopathologically in comparison with the adjacent facial nerve [6], SNHL is thought to be caused by retrocochlear abnormalities [1].…”

Section: Introductionmentioning

confidence: 99%

Superficial Siderosis of the Central Nervous System

Takasaki¹,

Tanaka²,

Shigeno³

et al. 2000

ORL

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This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.

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Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

Cited by 18 publications

References 16 publications

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

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

Superficial siderosis

Superficial Siderosis of the Central Nervous System

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