Susac syndrome: outcome of unilateral cochlear implantation.
Summary of "Susac syndrome: outcome of unilateral cochlear implantation."
Objective: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.
Affiliation
Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK.
Journal Details
This article was published in the following journal.
Name: The Journal of laryngology and otology
ISSN: 1748-5460
Pages: 1-3
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21481296
- DOI: http://dx.doi.org/10.1017/S0022215111000600
Medical and Biotech [MESH] Definitions
Cochlear Implantation
Surgical insertion of an electronic hearing device (COCHLEAR IMPLANTS) with electrodes to the COCHLEAR NERVE in the inner ear to create sound sensation in patients with residual nerve fibers.
Cochlear Nerve
The cochlear part of the 8th cranial nerve (VESTIBULOCOCHLEAR NERVE). The cochlear nerve fibers originate from neurons of the SPIRAL GANGLION and project peripherally to cochlear hair cells and centrally to the cochlear nuclei (COCHLEAR NUCLEUS) of the BRAIN STEM. They mediate the sense of hearing.
Hearing Loss, Central
Hearing loss due to disease of the AUDITORY PATHWAYS (in the CENTRAL NERVOUS SYSTEM) which originate in the COCHLEAR NUCLEI of the PONS and then ascend bilaterally to the MIDBRAIN, the THALAMUS, and then the AUDITORY CORTEX in the TEMPORAL LOBE. Bilateral lesions of the auditory pathways are usually required to cause central hearing loss. Cortical deafness refers to loss of hearing due to bilateral auditory cortex lesions. Unilateral BRAIN STEM lesions involving the cochlear nuclei may result in unilateral hearing loss.
Auditory Brain Stem Implantation
Surgical insertion of an electronic hearing device (AUDITORY BRAIN STEM IMPLANTS) with electrodes to the cochlea nucleus in the BRAIN STEM rather than to the inner ear as in COCHLEAR IMPLANTATION.
Susac Syndrome
A rare disorder consisting of microangiopathy of brain, retina, and inner ear ARTERIOLES. It is characterized by the clinical triad of encephalopathy, BRANCH RETINAL ARTERY OCCLUSION and VERTIGO/hearing loss.
PubMed Articles
Abstract Conclusion: The cochlear implant was beneficial as an attempt to restore hearing and improve communication abilities in this patient with profound sensorineural hearing loss secondary to Susa...
Long-Term Evaluation of Cochlear Implantation in Cogan Syndrome.
Purpose: The evaluation of long-term results of cochlear implantation in patients with typical Cogan syndrome. Procedures: The medical records of approximately 3,000 patients who underwent cochlear im...
Delayed cochlear implantation after surgical labyrinthectomy.
Background:Cochlear implantation has been used to rehabilitate profoundly deafened adults for more than 25 years. However, surgical labyrinthectomy is often considered a contraindication to cochlear i...
Cochlear Implantation in Brown-Vialetto-Van-Laere syndrome.
Objective:To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome.Patients:Two adult patients (a brother and sister) with post-li...
Characteristics of Susac syndrome: a review of all reported cases.
In Susac syndrome, occlusions of microvessels-presumed to be mediated by an autoimmune response to an as yet unknown antigen-lead to a characteristic clinical triad of CNS dysfunction, branch retinal...
Clinical Trials
Children's Bilateral Cochlear Implantation in Finland
The purpose of this study is to evaluate short and long term outcomes (benefits and side-effects) of bilateral cochlear implantation in Finnish children.
Bilateral Cochlear Implantation in Children With the MED-EL Cochlear Implant
The purpose of this study is to evaluate and document longitudinal efficacy in young children implanted bilaterally with MED-EL COMBI 40+ / PULSARCI100/SONATATI100 cochlear implant systems...
Balance in Children With Cochlear Implants
The purpose is to test the balance of children with unilateral,bilateral cochlear implants and patients having unilateral implants before,bilateral implants after surgery,using the Bruinin...
Relationship Between Auto NRT and Behavioural T & C Levels With the Nucleus Freedom Cochlear Implant
Cochlear implants require programming on an individual basis to provide appropriate levels of electrical stimulation. This program, or "map", is placed in the speech processor of the cochl...
Iowa/Nucleus 10/10 mm and Nucleus Freedom Feasibility Study
The purpose of this study is to evaluate whether the Iowa/Nucleus 10/10 mm in one ear, in conjunction with a Nucleus Freedom implant in the other ear can provide useful binaural hearing in...
