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Updated: Aug 18, 2026

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with
John S Phillips1, Jacquelyn A King, Siddharthan Chandran
1Department of Otolaryngology, Norfolk and Norwich University Hospital, Norwich, UK. john.phillips@mac.com
Insights
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) can present with sudden sensorineural hearing loss. This case highlights the importance of family history in diagnosing rare neurological conditions.
Area of Science:
- Neurology
- Genetics
- Otolaryngology
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare genetic disorder.
- It typically manifests with cerebrovascular events, migraine, and cognitive decline.
Observation:
- A case study detailing a patient whose initial symptom was sensorineural hearing loss.
- This presentation is unusual, with no prior documented instances in medical literature.
Findings:
- The study reports the first known case of CADASIL presenting primarily with sudden sensorineural hearing loss.
- This finding expands the known clinical spectrum of CADASIL.
Implications:
- Highlights the importance of thorough patient history, particularly family history, in diagnosing complex neurological disorders.
- Suggests that hearing loss may be an underrecognized early symptom of CADASIL, prompting further investigation.
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.
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