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Susac syndrome: outcome of unilateral cochlear implantation

  • N Grover (a1), O J H Whiteside (a1) and J D Ramsden (a1)

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.

Copyright

Corresponding author

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: nancybatta@yahoo.com

References

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1Do, HT, Fisch, C, Francois, E. Susac syndrome: report of four cases and review of the literature. Am J Neuroradiol 2004;25:382–8
2Aubart-Cohen, F, Klein, I, Alexandra, JF, Bodaghi, B, Doan, S, Fardeau, C et al. Long-term outcome in Susac syndrome. Medicine 2007;86:93102
3Green, KM, Bhatt, YM, Mawman, DJ, O'Driscoll, MP, Saeed, SR, Ramsden, RT et al. Predictors of audiological outcome following cochlear implantation in adults. Cochlear Implants Int 2007;8:111
4Susac, JO. Susac's syndrome: the triad of microangiopathy of brain and retina with hearing loss in young women. Neurology 1994;44:591–3
5Roeser, MM, Driscoll, CLW, Shallop, JK, Gifford, RH. Susac syndrome – a report of cochlear implantation and review of otologic manifestations in twenty-three patients. Otol Neurotol 2008;30:3440
6Turner, BW, Digre, KB, Shelton, C. Susac syndrome. Otolaryngol Head Neck Surg 1998;118:866–7
7Papo, T, Biousse, V, Lehoang, P, Fardeau, C, N'Guyen, N, Huong, DL et al. Susac syndrome. Medicine 1998;77:311
8Bateman, ND, Johnson, IJ, Gibbin, KP. Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss. J Laryngol Otol 1997;111:1072–4

Keywords

Susac syndrome: outcome of unilateral cochlear implantation

  • N Grover (a1), O J H Whiteside (a1) and J D Ramsden (a1)

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