Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report

Abstract Background Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease that can results in a variety of neurological deficits. We describe a very rare presentation of MMD in the form of bilateral sudden sensorineural hearing loss after a second attack of subcortical cerebral haemo...

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Main Author: Mohamed Nabil Elmarawany
Format: Article
Language:English
Published: SpringerOpen 2023-08-01
Series:Egyptian Journal of Neurosurgery
Subjects:
Online Access:https://doi.org/10.1186/s41984-023-00212-1
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author Mohamed Nabil Elmarawany
author_facet Mohamed Nabil Elmarawany
author_sort Mohamed Nabil Elmarawany
collection DOAJ
description Abstract Background Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease that can results in a variety of neurological deficits. We describe a very rare presentation of MMD in the form of bilateral sudden sensorineural hearing loss after a second attack of subcortical cerebral haemorrhage. Case presentation A 44-year-old female presented with bilateral sudden sensorineural hearing loss (SSNHL) secondary to an acute intracerebral haemorrhage in the left temporoparietal region. She had a previous haemorrhagic event in the right temporoparietal region two years ago. The two haemorrhagic events have apparently led to interruption of the auditory pathways bilaterally, resulting in sudden complete deafness. Digital subtraction angiography was done, and it showed bilateral occlusion of the internal carotid arteries, with the abnormal anastomotic vascular network at the base of the brain characteristic for MMD. Conclusions Subcortical hemorrhage due to MMD is a potential cause of bilateral SSNHL that can be diagnosed with early proper imaging. Bilateral damage of the auditory pathways is the likely cause of cortical deafness in this clinical scenario.
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spelling doaj.art-1bff93f442c4496990ecc1e79d15dfe02023-08-06T11:09:39ZengSpringerOpenEgyptian Journal of Neurosurgery2520-82252023-08-013811610.1186/s41984-023-00212-1Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case reportMohamed Nabil Elmarawany0Department of Neurosurgery, Faculty of Medicine, Menoufia UniversityAbstract Background Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease that can results in a variety of neurological deficits. We describe a very rare presentation of MMD in the form of bilateral sudden sensorineural hearing loss after a second attack of subcortical cerebral haemorrhage. Case presentation A 44-year-old female presented with bilateral sudden sensorineural hearing loss (SSNHL) secondary to an acute intracerebral haemorrhage in the left temporoparietal region. She had a previous haemorrhagic event in the right temporoparietal region two years ago. The two haemorrhagic events have apparently led to interruption of the auditory pathways bilaterally, resulting in sudden complete deafness. Digital subtraction angiography was done, and it showed bilateral occlusion of the internal carotid arteries, with the abnormal anastomotic vascular network at the base of the brain characteristic for MMD. Conclusions Subcortical hemorrhage due to MMD is a potential cause of bilateral SSNHL that can be diagnosed with early proper imaging. Bilateral damage of the auditory pathways is the likely cause of cortical deafness in this clinical scenario.https://doi.org/10.1186/s41984-023-00212-1MoyamoyaDeafnessSudden sensorineural hearing loss
spellingShingle Mohamed Nabil Elmarawany
Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
Egyptian Journal of Neurosurgery
Moyamoya
Deafness
Sudden sensorineural hearing loss
title Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
title_full Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
title_fullStr Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
title_full_unstemmed Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
title_short Bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with Moyamoya disease: case report
title_sort bilateral sudden sensorineural hearing loss due to subcortical cerebral hemorrhage in a patient with moyamoya disease case report
topic Moyamoya
Deafness
Sudden sensorineural hearing loss
url https://doi.org/10.1186/s41984-023-00212-1
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