Sudden sensorineural hearing loss: when ophthalmology meets otolaryngology.
OBJECTIVE
Sudden sensorineural hearing loss is a perplexing entity in otology. Susac's syndrome (also called retinocochleocerebral vasculopathy) is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear, and usually affects women in young adulthood. We describe the clinical aspects, radiographic findings, and management of one such case.


METHODS
A 30-year-old woman was admitted to the hospital because of sudden onset of bilateral deafness and headache. During her hospitalization, she developed discrete right hemiparesis and hypoesthesia.


RESULTS
Magnetic resonance imaging revealed multiple signal hyperintensities and atrophy of the corpus callosum. The differential diagnosis was a myelinating condition, such as multiple sclerosis or acute demyelinating encephalomyelitis.


CONCLUSIONS
Retinal fluorescein angiography helped the diagnosis of Susac's syndrome.
ENT Department, CHR-Citadelle, Liege, Belgium.
S
S
Karelle
L
L
Demanez
P F
PF
Zangerle
P
P
Blaise
G
G
Moonen
A L
AL
Poirrier
D000328:Adult; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D055955:Susac Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy