Research in Vestibular Science

Case Reports

A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis: Gyuman Lee, Youngmin Mun, Dae Bo Shim; Res Vestib Sci. 2023;22(3):83-88. Published online September 15, 2023; DOI: https://doi.org/10.21790/rvs.2023.22.3.83; Correction in: Res Vestib Sci 2023;22(4):137

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Abstract PDF: Superficial siderosis (SS) is a rare neurodegenerative condition characterized by hemosiderin deposition in the central nervous system, which sometimes leads to sensorineural hearing loss and vestibular dysfunction. This case report details the diagnosis and treatment of a 63-year-old female patient presenting with a yearlong history of postural instability and recent symptoms of sudden right-sided hearing loss and imbalance. Physical examination and imaging revealed bilateral deafness and infratentorial SS. Treatment, including medication, vestibular exercises, high-dose steroids, and hyperbaric oxygen therapy, led to a significant alleviation of dizziness but no improvement in hearing. This case emphasizes the need for clinicians to consider SS in patients presenting with sudden-onset hearing loss and dizziness, even without prior any medical history or trauma, to accurately identify the underlying cause.

Superficial Siderosis with Peripheral Dizziness: Report of 2 Cases: Tae-Hoon Kim, Jin-Hyuk Huh, Moon-Suh Park, Jae-Yong Byun; Res Vestib Sci. 2018;17(2):60-66. Published online June 15, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.2.60

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Superficial siderosis (SS) of the central nervous system is a rare disease, which is caused by the accumulation of iron from the hemoglobin in the superficial layer of the brain, spinal cord, and central parts of cranial nerves. The etiology of SS is the accumulation of hemosiderin in the subarachnoid space due to chronic or repeated hemorrhage resulting in progressive and irreversible neurological dysfunction. The cause of the disease is aneurysm, trauma, tumor, and vascular malformation. In most cases, the cause of bleeding is unknown. Clinical features include sensorineural hearing loss, cerebellar ataxia, and myelopathy. Until now, magnetic resonance imaging (MRI) has only been diagnosed and there is no standardized treatment. We will investigate clinical features and MRI findings of SS disease in the central nervous system using 2 patient cases.

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A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis
Gyuman Lee, Youngmin Mun, Dae Bo Shim
Research in Vestibular Science.2023; 22(3): 83. CrossRef

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