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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with sudden sensorineural hearing loss

Published online by Cambridge University Press:  16 March 2006

John S Phillips ,
Jacquelyn A King ,
Siddharthan Chandran ,
Peter R Prinsley  and
David Dick
John S Phillips
Affiliation:
Department of Otolaryngology, Norfolk and Norwich University Hospital, Norwich, UK
Jacquelyn A King
Affiliation:
Department of Audiology, Norfolk and Norwich University Hospital, Norwich, UK
Siddharthan Chandran
Affiliation:
Cambridge Centre for Brain Repair, Cambridge, UK
Peter R Prinsley
Affiliation:
Department of Otolaryngology, Norfolk and Norwich University Hospital, Norwich, UK
David Dick
Affiliation:
Department of Neurology, Norfolk and Norwich University Hospital, Norwich, UK
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Abstract

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CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an autosomal dominant angiopathy characterized by recurrent cerebrovascular events, migraine and dementia. We describe a case of sensorineural hearing loss as the presenting feature of this condition. We have found no previous reports in the world literature of CADASIL presenting with a sudden sensorineural hearing loss. The significance of questioning a patient with regard to family history is exemplified in this case.

Keywords

Hearing LossSensorineuralDementia, Multi-Infarct
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 119 , Issue 2 , February 2005 , pp. 148 - 151
Copyright
© 2005 Royal Society of Medicine Press