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Susac syndrome: outcome of unilateral cochlear implantation

Published online by Cambridge University Press:  12 April 2011

N Grover ,
O J H Whiteside  and
J D Ramsden
N Grover*
Affiliation:
Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
O J H Whiteside
Affiliation:
Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
J D Ramsden
Affiliation:
Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
*
Address for correspondence: Ms Nancy Grover, Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Headington, Oxford OX3 9DU, UK Fax: +44 (0)1865 231091 E-mail: [email protected]
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Abstract

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.

Keywords

Susac SyndromeCochlear ImplantsDiagnosis
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 125 , Issue 8 , August 2011 , pp. 856 - 858
Copyright
Copyright © JLO (1984) Limited 2011

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