title: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with sudden sensorineural hearing loss.
abstract: 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.
authors.affiliation: Department of Otolaryngology, Norfolk and Norwich University Hospital, Norwich, UK. john.phillips@mac.com
authors.forename: John S
authors.initials: JS
authors.lastname: Phillips
authors.forename: Jacquelyn A
authors.initials: JA
authors.lastname: King
authors.forename: Siddharthan
authors.initials: S
authors.lastname: Chandran
authors.forename: Peter R
authors.initials: PR
authors.lastname: Prinsley
authors.forename: David
authors.initials: D
authors.lastname: Dick
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone / Q000379:methods; D046589:CADASIL / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D005260:Female; D003639:Hearing Loss, Sudden / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010375:Pedigree