The key is an ophthalmological examination and evidence of BRAOs along with hearing loss and encephalopathy
- Chronic inflammatory disease of unknown origin affects young adults
- Autoimmune reaction against an unknown common autoantigen in the cornea and inner ear and can develop an aortitis.
- Systemic symptoms - fever, headache, joint pain, and myalgia.
- Bilateral sensorineural hearing loss and Vestibular symptoms
- Inflammatory ocular manifestations - keratitis
- Rarely a severe systemic vasculitis with aortitis.
- Red eye, ear pain, vertigo, episcleritis, scleritis, uveitis, vitritis, choroiditis
- Bloods: mildly elevated ESR
- Slit lamp examination
Susac's syndrome. Though examination should readily distinguish the two disorders, the physician should consider Susac's syndrome and look for the triad of
- 1) hearing loss
- 2) branched retinal artery occlusions
- 3) encephalopathy.
- Consider ophthalmology review and systemic corticosteroids for ocular involvement
- Systemic disease - Prednisolone 1 mg/kg po once/day is begun as soon as possible and continued for 2 to 6 mo. Some clinicians add cyclophosphamide, methotrexate, or cyclosporine for recalcitrant cases.