Bilateral vestibulopathy in superficial siderosis

Sang Yeon Lee, Dong Han Lee, Youn Jung Bae, Jae Jin Song, Ji Soo Kim, Ja-Won Koo

Research output: Contribution to journalArticleResearchpeer-review

Abstract

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.

Original languageEnglish
Article number422
JournalFrontiers in Neurology
Volume9
Issue numberJUN
DOIs
StatePublished - 6 Jun 2018

Fingerprint

Siderosis
Hemosiderin
Cerebellar Ataxia
Sensorineural Hearing Loss
Vestibulocochlear Nerve
Magnetic Resonance Imaging
Spinal Nerves
Cochlea
Peripheral Nerves
Hearing Loss
Cerebellum
Brain Stem
Medical Records
Bilateral Vestibulopathy
Spinal Cord
Iron
Hemorrhage
Brain

Keywords

  • Cerebellar ataxia
  • Hearing loss
  • Vertigo
  • Vestibulopathy
  • superficial siderosis

Cite this

Lee, Sang Yeon ; Lee, Dong Han ; Bae, Youn Jung ; Song, Jae Jin ; Kim, Ji Soo ; Koo, Ja-Won. / Bilateral vestibulopathy in superficial siderosis. In: Frontiers in Neurology. 2018 ; Vol. 9, No. JUN.
@article{519a457ff93d45fcbb4dc355957efd7c,
title = "Bilateral vestibulopathy in superficial siderosis",
abstract = "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.",
keywords = "Cerebellar ataxia, Hearing loss, Vertigo, Vestibulopathy, superficial siderosis",
author = "Lee, {Sang Yeon} and Lee, {Dong Han} and Bae, {Youn Jung} and Song, {Jae Jin} and Kim, {Ji Soo} and Ja-Won Koo",
year = "2018",
month = "6",
day = "6",
doi = "10.3389/fneur.2018.00422",
language = "English",
volume = "9",
journal = "Frontiers in Neurology",
issn = "1664-2295",
publisher = "Frontiers Media S.A.",
number = "JUN",

}

Bilateral vestibulopathy in superficial siderosis. / Lee, Sang Yeon; Lee, Dong Han; Bae, Youn Jung; Song, Jae Jin; Kim, Ji Soo; Koo, Ja-Won.

In: Frontiers in Neurology, Vol. 9, No. JUN, 422, 06.06.2018.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Bilateral vestibulopathy in superficial siderosis

AU - Lee, Sang Yeon

AU - Lee, Dong Han

AU - Bae, Youn Jung

AU - Song, Jae Jin

AU - Kim, Ji Soo

AU - Koo, Ja-Won

PY - 2018/6/6

Y1 - 2018/6/6

N2 - 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.

AB - 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.

KW - Cerebellar ataxia

KW - Hearing loss

KW - Vertigo

KW - Vestibulopathy

KW - superficial siderosis

UR - http://www.scopus.com/inward/record.url?scp=85048117098&partnerID=8YFLogxK

U2 - 10.3389/fneur.2018.00422

DO - 10.3389/fneur.2018.00422

M3 - Article

VL - 9

JO - Frontiers in Neurology

JF - Frontiers in Neurology

SN - 1664-2295

IS - JUN

M1 - 422

ER -