Susac syndrome: outcome of unilateral cochlear implantation

2011 ◽  
Vol 125 (8) ◽  
pp. 856-858 ◽  
Author(s):  
N Grover ◽  
O J H Whiteside ◽  
J D Ramsden
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Cochlear Implantation ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Ophthalmological Examination ◽  
Resonance Imaging ◽  
Susac Syndrome ◽  
Retinal Artery

AbstractObjective:Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patients. We present a case of successful hearing rehabilitation by cochlear implantation in a young woman with this syndrome.Clinical presentation:A 36-year-old woman presented with neurological symptoms suggestive of encephalitis. She subsequently developed vestibulocochlear symptoms. The diagnosis was confirmed upon magnetic resonance imaging and fluorescein angiography, which showed multiple peripheral retinal arterial occlusions. Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound sensorineural hearing loss.Intervention:A left cochlear implant was placed, with a good outcome.Conclusion:In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways.

scholarly journals Susac syndrome misdiagnosed as multiple sclerosis with exacerbation by interferon beta therapy

2017 ◽  
Vol 31 (2) ◽  
pp. 207-212 ◽  
Author(s):  
Hussein Algahtani ◽  
Bader Shirah ◽  
Muhammad Amin ◽  
Eyad Altarazi ◽  
Hashem Almarzouki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Interferon Beta ◽  
Young Patients ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Susac Syndrome ◽  
Retinal Artery

Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. She had the classic magnetic resonance imaging appearance, with normal magnetic resonance imaging of the spinal cord and absence of oligoclonal bands in the cerebrospinal fluid. Our patient responded well to treatment with a combination therapy and discontinuation of interferon beta-1a. Our observations raise awareness about the importance of the early and correct diagnosis of Susac syndrome, which usually affects young patients, with an excellent prognosis if treated aggressively at an early stage of the disease. Susac syndrome is underdiagnosed and is not uncommonly misdiagnosed as multiple sclerosis. Susac syndrome is a great mimicker of multiple sclerosis, and establishing diagnostic criteria for this syndrome is very useful. In any patient presenting with a progressive disabling neurological disorder associated with callosal lesions and/or hearing loss, and/or visual loss especially in women, Susac syndrome should be suspected.

scholarly journals Cerebral magnetic resonance imaging of coincidental infarction and small vessel disease in retinal artery occlusion

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yong Dae Kim ◽  
Jun Yup Kim ◽  
Young Joo Park ◽  
Sang Jun Park ◽  
Sung Hyun Baik ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Brain Imaging ◽  
Small Vessel Disease ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Resonance Imaging ◽  
Cerebral Magnetic Resonance Imaging ◽  
Vessel Disease ◽  
Retinal Artery

AbstractThere are several reports in the literature on the association between non-arteritic retinal artery occlusion (NA-RAO) and acute ischemic stroke. We investigated the burden of small vessel disease (SVD) and cerebral coincident infarction observed on cerebral magnetic resonance imaging (MRI) in patients with newly diagnosed NA-RAO. In this retrospective, observational, case-series study, consecutive patients with NA-RAO who underwent cerebral MRI within one month of diagnosis between September 2003 and October 2018 were included. The classification of NA-RAO was based on ophthalmologic and systemic examinations. We also investigated the co-incident infarction and burden of underlying SVD, which were categorized as white matter hyperintensity lesion (WMH), cerebral microbleeds (CMB), and silent lacunar infarction (SLI). Among the 272 patients enrolled in the study, 18% presented co-incident infarction and 73% had SVD, which included WMH (70%), CMB (14%), and SLI (30%). Co-incident infarction, WMH, and SLI significantly increased with age: co-incident infarction was observed in 8% of young (< 50 years) patients and 30% of old (≥ 70 years) patients. The embolic etiology of RAO (large artery atherosclerosis, cardioembolism, and undetermined etiology) was significantly associated with the prevalence of SVD (82%: 70%: 64%, P = 0.002) and co-incident infarction (30%: 19%: 8%; P = 0.009). Therefore, high co-incidence of acute cerebral infarction and underlying SVD burden warrant careful neurologic examination and appropriate brain imaging, followed by management of NA-RAO. Urgent brain imaging is particularly pertinent in elderly patients with NA-RAO.

scholarly journals Susac syndrome presenting with acute hemibody paraesthesia

2019 ◽  
Vol 8 ◽  
pp. 204800401984468
Author(s):  
Philip Campbell ◽  
Diego Kaski ◽  
Tabish A Saifee
Keyword(s):  
Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Stroke Management ◽  
Susac Syndrome ◽  
Hyperacute Stroke ◽  
Appropriate Treatment ◽  
Retinal Artery ◽  
Clinical Triad

Susac syndrome is an orphan disease characterised by encephalopathy, branch retinal artery occlusion and sensorineural hearing loss. As the clinical triad is rarely present at symptom onset, it is often initially misdiagnosed and appropriate treatment is often delayed. Herewith, we report a case of Susac syndrome in a 47-year-old man presenting with acute hemisensory loss and highlight the challenges of early diagnosis, particularly relevant in the era of hyperacute stroke management.

Results, Hearing Rehabilitation, and Follow-up with Magnetic Resonance Imaging after Tympanomastoid Exenteration, Obliteration, and External Canal Overclosure for Severe Chronic Otitis Media

2007 ◽  
Vol 116 (9) ◽  
pp. 705-711 ◽  
Author(s):  
Katrien Ketelslagers ◽  
Thomas Somers ◽  
Bert De Foer ◽  
Andrzej Zarowski ◽  
Erwin Offeciers
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Otitis Media ◽  
Cochlear Implantation ◽  
Conductive Hearing Loss ◽  
Chronic Otitis Media ◽  
Resonance Imaging ◽  
Conductive Hearing

Objectives: We sought to evaluate the results, auditory rehabilitation, and follow-up with magnetic resonance imaging (MRI) after tympanomastoid exenteration with obliteration of the mastoid cavity and overclosure of the external ear canal in patients with severe chronic otitis media that was resistant to medical therapy and conventional surgery and was associated with a profound sensorineural or severe conductive hearing loss. Methods: Twenty-nine patients were analyzed and underwent this surgical technique. Twelve patients had, during the same or later stage, either cochlear implantation, fixture implantation for a bone-anchored hearing aid, or middle ear implantation. For follow-up control of the obliterated cavity, delayed gadolinium-enhanced, T1-weighted MRI in combination with non-echo planar imaging diffusion weighted sequences were used. Results: No patient had recurrent otorrhea after an average follow-up period of 4.75 years. One patient had a residual cholesteatoma as shown by new MRI techniques, and this was successfully resected. One patient developed complications 6 months after 1-stage tympanomastoid exenteration and cochlear implantation. Conclusions: This technique is very useful in selected patients with severe chronic otitis media that is resistant to medical therapy and surgery and is associated with a profound sensorineural or severe conductive hearing loss. New sequences in MRI are used for postoperative follow-up of these obliterated cavities and seem reliable for the detection of residual or recurrent cholesteatoma. Middle ear implantation and cochlear implantation can be relatively safely performed in these patients in a second stage.

A Young Man With Visual Field Loss, Decreased Hearing, and Confusion

2021 ◽  
pp. 168-170
Author(s):  
M. Tariq Bhatti ◽  
Eric R. Eggenberger ◽  
Marie D. Acierno ◽  
John J. Chen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Visual Field ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Susac Syndrome ◽  
Retinal Artery ◽  
Signal Changes ◽  
The Brain ◽  
Field Defect

A 27-year-old man noted imbalance and staggering when walking. Vertigo, nausea, vomiting, and mild fever developed. This was presumed to be due to an inner ear infection, and antibiotics were prescribed. He began experiencing intermittent left face and arm numbness, bilateral hearing loss and tinnitus. Audiography indicated low-frequency hearing loss in both ears, left worse than right. He reported headaches and neck stiffness, and his family noticed that he was moody, easily aggravated, and confused, with slow mentation. Magnetic resonance imaging showed patchy, nodular, leptomeningeal enhancement involving both cerebral hemispheres and the posterior fossa, with scattered hyperintense T2 signal changes of the internal capsule and prominent abnormal signal changes in the corpus callosum. Cerebrospinal fluid analysis was remarkable for a markedly increased protein concentration and white blood cells. Eye examination showed 20/20 vision in both eyes with a superior visual field defect in the right eye. Retinal whitening was noted in the vascular distribution of the inferotemporal arcade. Intravenous fluorescein angiography showed delayed filling in this region consistent with a branch retinal artery occlusion and scattered areas of arteriolar wall hyperfluorescence. A diagnosis of Susac syndrome was made on the basis of the branch retinal artery occlusion, magnetic resonance imaging findings, and hearing deficit. Intravenous methylprednisolone was given, followed oral prednisone, which resulted in substantial improvement in headaches and cognition. Cyclophosphamide was also started at the same time as intravenous methylprednisolone. A new visual field defect developed due to a branch retinal artery occlusion in the left eye, which prompted initiation of intravenous immunoglobulin and transition from cyclophosphamide to rituximab. He had no recurrent branch retinal artery occlusions or other relapses of his underlying Susac syndrome on this treatment regimen. Susac syndrome was initially described as a microangiopathy of the brain and retina. It is an idiopathic autoimmune disorder that primarily affects the brain, eye, and inner ear.

Cochlear implantation after selective vestibular nerve section

2013 ◽  
Vol 127 (3) ◽  
pp. 311-313 ◽  
Author(s):  
C Martens ◽  
A Csillag ◽  
M Davies ◽  
P Fagan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Case Report ◽  
Magnetic Resonance ◽  
Cochlear Implantation ◽  
World Literature ◽  
Vestibular Nerve ◽  
Cochlear Nerve ◽  
Resonance Imaging ◽  
Nerve Section

AbstractIntroduction:Vestibular nerve section is a highly effective procedure for the control of vertigo in patients with Ménière's disease. However, hearing loss is a possible complication. If hearing loss occurs after vestibular nerve section, magnetic resonance imaging should make it possible to establish the presence or absence of an intact cochlear nerve.Method:Case report and review of the world literature concerning cochlear implantation after vestibular nerve section.Case report:We present a patient who developed subtotal hearing loss after vestibular nerve section. Magnetic resonance imaging was used to verify the presence of an intact cochlear nerve, enabling successful cochlear implantation.Conclusion:To our knowledge, this is the first reported case of cochlear implantation carried out after selective vestibular nerve section. Given recent advances in cochlear implantation, this case indicates that it is essential to make every effort to spare the cochlear nerve if vestibular nerve section is required. If hearing loss occurs after vestibular nerve section, magnetic resonance imaging should be undertaken to establish whether the cochlear nerve is intact.

scholarly journals Intralabyrinthine Schwannomas: Disease Presentation, Tumor Management, and Hearing Rehabilitation

2019 ◽  
Vol 80 (02) ◽  
pp. 196-202 ◽  
Author(s):  
Baishakhi Choudhury ◽  
Matthew Carlson ◽  
Daniel Jethanamest
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Hearing Rehabilitation ◽  
Rare Tumors ◽  
Auditory Rehabilitation ◽  
Tumor Management

AbstractIntralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

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