Bernhardt D, Veltmann R, Dörwald R, Huth F
Dtsch Med Wochenschr. 1976 Mar 5;101(10):373-7. doi: 10.1055/s-0028-1104091.
A 34-year-old woman died of left-heart failure due to combinated aortic-valve disease three years after manifestation of Cogan's syndrome characterized by sudden inner-ear deafness, loss of equilibrium, interstitial keratitis, and progressive loss of vision during pregnancy. At necropsy there was evidence of recurrent endocarditis of the aortic valves with stenosis and regurgitation, severe angitis of the thoracic aorta with marked secondary sclerosing changes as the cause of the heart failure. Angitis within the optical fasciculus and stato-acoustic nerve was the cause of the vestibular and optical defects. Primary changes in the visual and auditory cortices, the retina and inner ear were excluded as causes. There was also acute membrano-proliferative glomerulonephritis, which may have been coincidental.
一名34岁女性，在出现以突发内耳性耳聋、平衡失调、间质性角膜炎以及孕期视力进行性丧失为特征的科根综合征三年后，死于由合并主动脉瓣疾病导致的左心衰竭。尸检发现有主动脉瓣反复感染性心内膜炎伴狭窄和反流，胸主动脉严重血管炎并伴有显著的继发性硬化改变，这是心力衰竭的原因。视束和听神经内的血管炎是前庭和视觉缺陷的原因。排除了视觉和听觉皮层、视网膜及内耳的原发性病变作为病因。此外还存在急性膜增生性肾小球肾炎，可能为巧合。
