Susac syndrome: outcome of unilateral cochlear implantation.

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.

• Authors: N Grover, O J H Whiteside, J D Ramsden
• Journal: The Journal of laryngology and otology (J Laryngol Otol) Vol. 125 Issue 8 Pg. 856-8 (Aug 2011) ISSN: 1748-5460 [Electronic] England
• PMID: 21481296 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Audiometry, Pure-Tone
• Cochlear Implantation
• Diagnosis, Differential
• Female
• Hearing Loss, Sensorineural (diagnosis, rehabilitation, surgery)
• Humans
• Magnetic Resonance Imaging
• Susac Syndrome (diagnosis, pathology, surgery)
• Treatment Outcome