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Susac syndrome.

Menachem Gross1, Eyal Banin, Ron Eliashar

  • 1Department of Otolaryngology/Head & Neck Surgery, Hadassah-Hebrew University Hospital, Jerusalem, Israel. drgrossm@hotmail.com

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|July 9, 2004
PubMed
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Susac syndrome is a rare condition affecting the brain, ears, and eyes. Early recognition of its neurological, auditory, and visual symptoms is crucial for timely diagnosis and treatment.

Area of Science:

  • Neurology
  • Ophthalmology
  • Otolaryngology

Background:

  • Susac syndrome is a rare, poorly understood condition.
  • It involves microangiopathy affecting the brain, cochlea, and retina.
  • Diagnosis can be challenging due to overlapping symptoms with other conditions.

Observation:

  • A 50-year-old woman presented with neurological symptoms, bilateral sensorineural hearing loss, and retinal artery occlusion.
  • Initial diagnosis was sudden sensorineural hearing loss, but bilateral symptoms emerged later.
  • The patient underwent comprehensive evaluation including MRI, audiometry, and fluorescein angiography.

Findings:

  • The patient experienced recurrent neurological episodes, vertigo, tinnitus, and bilateral hearing loss.
  • Magnetic resonance imaging (MRI) studies, audiometric tests, and retinal fluorescein angiography were performed.

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  • Treatment involved initial corticosteroids followed by other immunosuppressive agents.

    • Implications:

    • Susac syndrome presents a diagnostic challenge, with a differential diagnosis including autoimmune diseases, vasculitis, and multiple sclerosis.
    • Otolaryngologists must be aware of vestibulocochlear manifestations.
    • Multidisciplinary evaluation is essential for accurate diagnosis and management of Susac syndrome.