McDonald T J, Vollertsen R S, Younge B R
Laryngoscope. 1985 Jun;95(6):650-4. doi: 10.1288/00005537-198506000-00003.
The experience at the Mayo Clinic with 18 patients who had Cogan's syndrome is reviewed. Typically, the illness began with systemic symptoms. The ocular and audiovestibular symptoms included ocular discomfort and redness, photophobia, and fluctuating sensorineural deafness with imbalance. Each patient had interstitial keratitis; the ocular symptoms periodically recurred but responded to therapy. Thirteen patients who had fluctuating bilateral sensorineural deafness subsequently suffered total bilateral deafness. Two patients who are unilaterally deaf have good residual hearing. Two other patients regained hearing within normal limits after treatment with corticosteroids. No hearing symptoms were observed in one patient who had severe vertigo and whose condition is stable. Seventeen patients had vertigo: 11 have no labyrinthine function, 1 had absent responses on vestibular testing initially but currently demonstrates good responses, and the 5 others have remained stable with diminished vestibular function. If Cogan's syndrome is diagnosed early and treatment with corticosteroids is initiated promptly, hearing can be stabilized; otherwise, the prognosis for auditory function is poor.
本文回顾了梅奥诊所18例柯根综合征患者的诊疗经历。通常，该病始于全身症状。眼部和视听前庭症状包括眼部不适、发红、畏光，以及伴有失衡的波动性感音神经性耳聋。每位患者均患有间质性角膜炎；眼部症状会周期性复发，但对治疗有反应。13例患有波动性双侧感音神经性耳聋的患者随后出现双侧全聋。2例单侧耳聋患者仍保留较好的残余听力。另外2例患者在接受皮质类固醇治疗后听力恢复至正常范围。1例患有严重眩晕且病情稳定的患者未出现听力症状。17例患者有眩晕症状：11例无迷路功能，1例最初前庭测试无反应，但目前反应良好，另外5例前庭功能减退但病情保持稳定。如果柯根综合征能早期诊断并及时开始使用皮质类固醇治疗，听力可得到稳定；否则，听觉功能预后较差。
