Short attacks of vertigo leading to gradual deterioration of function on a permanent basis.
The best characterized condition is Cogan's syndrome, which is associated with progressive hearing loss, tinnitus, vertigo and interstitial keratitis.
Other ocular signs such as conjunctivitis, uveitis, scleritis and choroiditis have also been described.
A longer interval between hearing loss and ocular symptoms may be commoner in males.
Investigations
No autoantibodies have been identified.
Brain MRI may show non-specific white-matter hyperintensities in 10% of patients.
Treatment
Little data available: typically treated with steroids or azathioprine or TNF-α antagonists.