---
title: Susac syndrome
description: "Susac syndrome is a rare autoimmune microangiopathy affecting the brain, retina, and inner ear. It leads to a triad of encephalopathy, branch retinal artery occlusions, and hearing loss. ## Clinical features Symptoms may appear together or sequentially. - Confusion, cognitive…"
url: "https://bluefinvision.com/knowledge-base/topics/conditions-diseases/susac-syndrome/"
updated: "2025-12-16"
category: "Conditions & Diseases"
---

# Susac syndrome

Susac syndrome is a rare autoimmune microangiopathy affecting the brain, retina, and inner ear. It leads to a triad of encephalopathy, branch retinal artery occlusions, and hearing loss.

## Clinical features

Symptoms may appear together or sequentially.

- Confusion, cognitive changes, and headaches from brain involvement
- Visual field defects from branch retinal artery occlusions seen on fluorescein angiography
- Sensorineural hearing loss, often low-frequency
- Characteristic 'snowball' lesions in the corpus callosum on MRI

## Treatment

Early aggressive immunosuppression improves long-term outcome.

- High-dose steroids followed by steroid-sparing agents such as IVIG, mycophenolate, or cyclophosphamide
- Close collaboration between neurology, rheumatology, and ophthalmology
- Hearing and visual rehabilitation where deficits persist
- Long-term monitoring for relapses