Cogan’s Syndrome

Epidemiology

  • Very rare disorder

Physiology

  • Systemic vasculitis

Diagnosis

  • xxx

Clinical

(ear and eye involvement nearly always begin within several months of each other)

  • Interstitial Keratitis (most common pattern of ocular involvement):
    • Also can see uveitis, episcleritis, scleritis, retinal vasculitis, panopthalmitis
  • Vestibuloauditory Symptoms/Sensorineural Hearing Loss: 50% of cases develop some permanent hearing loss
    • Also can see tinnitus, vertigo, oscillopsia
  • Large Vessel Vasculitis (resembling Takayasu’s Arteritis): occurs in 15% of cases
    • Aortitis

Treatment

  • Topical Steroids: for interstitial keratitis
  • Steroids with or without Cyclophosphamide: indicated for sensorineural hearing loss

References

  • xxx