@entity9465 is a @entity395 of unknown origin characterized by @entity2194 and vestibuloauditory symptoms. Typically, young adults suffer from @entity1036 and @entity393 . Few cases (around 150) have been published and thus it is difficult to determine the percentage of @entity1 with underlying @entity74 such as @entity13571 . The variety of systemic manifestations is large and includes fever, @entity381 , @entity85 . @entity13571 can be seen in around 10% of cases and may involve the large vessels, appearing as @entity1333 with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While @entity288 have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf @entity1 has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two @entity1 with a wide clinical spectrum of symptoms from @entity2194 and ears to a @entity1333 affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.

@entity9465 : organ-specific XXXX ? A report of two cases and review of the literature.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2194 :: ('MESH:D005128', 'Disease') :: ['inflammatory eye disease', 'local disease restricted to the eyes'] @entity13571 :: ('MESH:D056647', 'Disease') :: ['Systemic vasculitis', 'systemic vasculitis'] @entity1036 :: ('MESH:D007634', 'Disease') :: ['interstitial keratitis and sudden onset of tinnitus'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['widespread vasculitis', 'Takayasu-like vasculitis'] @entity381 :: ('MESH:D013163', 'Disease') :: ['splenomegaly'] @entity74 :: ('MESH:D030342', 'Disease') :: ['systemic disorders'] @entity393 :: ('MESH:D034381', 'Disease') :: ['hearing loss'] @entity395 :: ('MESH:D035583', 'Disease') :: ['rare disorder'] @entity85 :: ('MESH:D009140', 'Disease') :: ['lymphadenopath, and musculoskeletal complaints'] @entity9465 :: ('MESH:D055952', 'Disease') :: ["Cogan's syndrome"] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity13571 :: (MESH:D056647,Disease) :: ['autoimmune disease or systemic vasculitis']
