Das Cogan-Syndrom: Zwei Fallberichte

Background. Cogan's disease is a diagnostic challenge. The typical form of this chronic inflammatory disease is characterized by non-syphilitic interstitial keratitis and acute audio-vestibular impairment. Case reports. The first patient had "typical" symptoms of Cogan's syndrome with recurrent keratoconjunctivitis, severe hearing loss and vertigo. Systemic cardiovascular manifestations occurred in the further course of the disease with multiple vascular stenoses of the carotids and vessels of the lower limbs. The second patient had"atypical"symptoms of Cogan's disease with progressive bilateral hearing loss and vestibular deficits, unilateral conjunctivitis and orbital swelling and unspecific symptoms, such as fever and headaches. Therapy with systemic and local corticoids was successful in both cases concerning ocular and systemic symptoms. The first patient had received additional therapy with methotrexate, but had already become deaf before therapy had started. Cochlear implantation was successfully carried out. In the second patient hearing loss was stationary under therapy and the patient could be provided with hearing aids. Conclusion. Because of obligatory cochleo-vestibular symptoms the otorhinolaryngologist has a key position in diagnosis and therapy of this rare entity. Early diagnosis is a crucial prognostic factor concerning preservation or restitution of hearing function.