Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

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iSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

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Bilateral Vestibulopathy in Supercial Siderosis

Bilateral Vestibulopathy in Superficial Siderosis Sang-Yeon Lee1, Dong-Han Lee1, Yun Jung Bae2, Jae-Jin Song1, Ji Soo Kim3* Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS. Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration. Methods: Six patients diagnosed with SS by magnetic resonance imaging (MRI) were enrolled. Their medical records and radiological findings were retrospectively reviewed, particularly in terms of progression of the vestibulocochlear manifestations and the radiological characteristics.

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Vestibular function in superficial siderosis

Vestibular deficits due to SS have rarely been reported
in the otolaryngological literature because early reports
noted the selective deposition of hemosiderin around
the CNS and/or the 8th nerve in contact with the
cerebrospinal fluid, most notably the cerebellum, brainstem,
lining of the ventricles, and spinal cord [1,4].
These deposits around CNS structures and/or the 8th
nerve were considered to be the changes most responsible
for the disequilibrium of SS.

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