Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with sudden sensorineural hearing loss.
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.
Phillips
John S
JS
Department of Otolaryngology, Norfolk and Norwich University Hospital, Norwich, UK. john.phillips@mac.com
King
Jacquelyn A
JA
Chandran
Siddharthan
S
Prinsley
Peter R
PR
Dick
David
D
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