The Cogan I Syndrome - A Diagnosis by Exclusion

Introduction The Cogan I syndrome is defined by ocular and audiovestibular symptoms. It’s classified in a typical and atypical form by the presence of accessory symptoms and time of progression. While ocular symptoms are usually regredient, inner ear affection can lead to deafness. Typical is an interstitial keratitis and Meniere like fluctuating inner ear affection. Cogan Syndrom is considered to belong to rheumatic disorders by the complex of symptoms and high antibody titers against HSP/-SSA/DEP1, found in some case control studies.

Results A 61 years old patient presented with staggering vertigo, fluctuating hearing loss, Uveitis posterior and high erythrocyte sedimentation rate. He suffered the previous years from interstitial keratitis, Menière like symptoms, arthralgia. myalgia and weigth loss. Secondary diagnoses are coronary heart disease, portal hypertension, a smouldering multiple myeloma and epidural hygroma after liquor puncture. A spontaneous cerebrospinal fluid leak syndrome, cerebral disorders, polyneuropathia and further rheumatic diseases as well as infectious diseases like Syphilis were excluded. So we diagnosed a Cogan I Syndrome.

Conclusion This Case Report shows impressive the long way from onset of symptoms to diagnosis through exclusion of differential diagnosis and the necessity of higher publicity of this seldom syndrome.

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Publication History

Article published online:
10 June 2020