Cogan’s syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis (IK) and vestibuloauditory dysfunction . Associations between CS and systemic vasculitis, as well as aortitis, also exist [2-4].
The pathology, pathogenesis, clinical manifestations, and treatment of Cogan’s syndrome will be presented here.
Limited autopsy data derived from temporal bone specimens of patients with CS reveal a range of findings, including [5-7]:
- Lymphocyte and plasma cell infiltration of the spiral ligament
- Endolymphatic hydrops
- Degenerative changes in the organ of Corti
- Extensive new bone formation in the inner ear
- Demyelination and atrophy of the vestibular and cochlear branches of the eighth cranial nerve
Histopathologic examination of corneal tissue from patients with IK reveals lymphocytic and plasma cell infiltration in the deeper layers .