Bilateral Vestibulopathy in Superficial Siderosis
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 sens...
Main Authors: | , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Frontiers Media S.A.
2018-06-01
|
Series: | Frontiers in Neurology |
Subjects: | |
Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2018.00422/full |
id |
doaj-4f9346403ea94324a67084cb5bae027d |
---|---|
record_format |
Article |
spelling |
doaj-4f9346403ea94324a67084cb5bae027d2020-11-24T21:09:30ZengFrontiers Media S.A.Frontiers in Neurology1664-22952018-06-01910.3389/fneur.2018.00422355733Bilateral Vestibulopathy in Superficial SiderosisSang-Yeon Lee0Dong-Han Lee1Yun Jung Bae2Jae-Jin Song3Ji Soo Kim4Ja-Won Koo5Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaDepartment of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaDepartment of Radiology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaDepartment of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaDepartment of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaDepartment of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South KoreaBackground: 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.Results: All six patients had SNHL. Five of them exhibited progressive hearing loss over years, which was asymmetric in four. On their most recent evaluations, patients showed cerebellar ataxia with combined central and peripheral vestibulopathy on both sides (n = 4), a bilateral peripheral vestibulopathy (n = 1) or isolated central vestibulopathy (n = 1). Notably, the former four patients showed an evolution of isolated central vestibulopathy into combined central and peripheral vestibulopathy. Hypo-intense lesions on T2 weighted MRIs were evident around the cerebellum in all patients, but such lesions were observed around the brainstem in five and the CN VIII in four. The cochlea-vestibular dysfunction generally progressed asymmetrically, but no left-right asymmetry was evident on MRI.Conclusions: SS typically presents as bilaterally asymmetric, progressive cochleovestibular dysfunction with cerebellar ataxia. The pattern of vestibular dysfunction is usually combined central and peripheral vestibulopathy on both sides. Thus, precise identification of audiovestibular dysfunction and central signs is essential in SS, and patients with SS should undergo regular, comprehensive neurotological evaluation to optimize their treatments and prognosis.https://www.frontiersin.org/article/10.3389/fneur.2018.00422/fullsuperficial siderosisvertigohearing losscerebellar ataxiavestibulopathy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sang-Yeon Lee Dong-Han Lee Yun Jung Bae Jae-Jin Song Ji Soo Kim Ja-Won Koo |
spellingShingle |
Sang-Yeon Lee Dong-Han Lee Yun Jung Bae Jae-Jin Song Ji Soo Kim Ja-Won Koo Bilateral Vestibulopathy in Superficial Siderosis Frontiers in Neurology superficial siderosis vertigo hearing loss cerebellar ataxia vestibulopathy |
author_facet |
Sang-Yeon Lee Dong-Han Lee Yun Jung Bae Jae-Jin Song Ji Soo Kim Ja-Won Koo |
author_sort |
Sang-Yeon Lee |
title |
Bilateral Vestibulopathy in Superficial Siderosis |
title_short |
Bilateral Vestibulopathy in Superficial Siderosis |
title_full |
Bilateral Vestibulopathy in Superficial Siderosis |
title_fullStr |
Bilateral Vestibulopathy in Superficial Siderosis |
title_full_unstemmed |
Bilateral Vestibulopathy in Superficial Siderosis |
title_sort |
bilateral vestibulopathy in superficial siderosis |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Neurology |
issn |
1664-2295 |
publishDate |
2018-06-01 |
description |
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.Results: All six patients had SNHL. Five of them exhibited progressive hearing loss over years, which was asymmetric in four. On their most recent evaluations, patients showed cerebellar ataxia with combined central and peripheral vestibulopathy on both sides (n = 4), a bilateral peripheral vestibulopathy (n = 1) or isolated central vestibulopathy (n = 1). Notably, the former four patients showed an evolution of isolated central vestibulopathy into combined central and peripheral vestibulopathy. Hypo-intense lesions on T2 weighted MRIs were evident around the cerebellum in all patients, but such lesions were observed around the brainstem in five and the CN VIII in four. The cochlea-vestibular dysfunction generally progressed asymmetrically, but no left-right asymmetry was evident on MRI.Conclusions: SS typically presents as bilaterally asymmetric, progressive cochleovestibular dysfunction with cerebellar ataxia. The pattern of vestibular dysfunction is usually combined central and peripheral vestibulopathy on both sides. Thus, precise identification of audiovestibular dysfunction and central signs is essential in SS, and patients with SS should undergo regular, comprehensive neurotological evaluation to optimize their treatments and prognosis. |
topic |
superficial siderosis vertigo hearing loss cerebellar ataxia vestibulopathy |
url |
https://www.frontiersin.org/article/10.3389/fneur.2018.00422/full |
work_keys_str_mv |
AT sangyeonlee bilateralvestibulopathyinsuperficialsiderosis AT donghanlee bilateralvestibulopathyinsuperficialsiderosis AT yunjungbae bilateralvestibulopathyinsuperficialsiderosis AT jaejinsong bilateralvestibulopathyinsuperficialsiderosis AT jisookim bilateralvestibulopathyinsuperficialsiderosis AT jawonkoo bilateralvestibulopathyinsuperficialsiderosis |
_version_ |
1716758184889155584 |