Very rare. It is an ocular and vestibular vasculitis the origin of which is unknown, probably immunological (presence of antibodies directed against antigens of the inner ear or antibodies against heat stroke protein 70), affecting mostly young adults.

This syndrome is known as:

-        'typical' when it combines a non-syphilitic interstitial keratitis and bilateral audio-vestibular impairment similar to Meniere's syndrome (which is unilateral!) with an evolution from hearing loss to deafness within less than 2 years between the onset of vestibular and ocular manifestations. 

-        'atypical' if the ocular involvement is different (episcleritis, iritis, choroiditis, optic neuritis) and the delay between the ocular and audiovestibular symptoms  is longer than 2 years.

Another organ is affected in 2/3 of cases: cardiovascular symptoms (aortitis with microaneurysms, risk of aortic insufficiency), neurological disorders (hemiplegia, T.I.A.), mucocutaneous or digestive (melena). The clinical picture looks like as systemic vasculitis in 1/3 of cases.

Treatment: corticosteroids, sometimes plasmapheresis or immunosuppressants (rituximab, tocilizumab, etc) in case of resistance to corticosteroid therapy.

Anesthetic implications: 

side-effects of corticosteroid therapy; echocardiography: aortic valve; hearing loss or deafness

References : 

-         Greco A, Gallo A, Fusconi M, Magliulo G et al. 
Cogan’s syndrome: an autoimmune inner ear disease. 
Autoimmunity Reviews 2013; 12: 396-400.

-         Jancatova D, Zelenik K, Kominek P, Matousek P. 
Atypical Cogan’s syndrome : a case report and summary of current treatment options.
Int J Pediatr Otorhinolaryngol 2015; 79: 428-31.

-         Singer O. 
Cogan and Behçet syndromes. 
Rheum Dis Clin N Am 2015; 41: 75-91.

Updated: October 2019