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