Hagiwara N, Harashima S, Tukamoto H, Horiuchi T
Kyushu University Graduate School of Medical Sciences, Fukuoka-city.
Ryumachi. 2001 Oct;41(5):858-63.
A 54 year-old man who had been diagnosed as Behcet's disease since 1985 was admitted due to sypmtoms of fever, bilateral chronic hearing loss and repeated sudden left deafness after a partial laparoscopic gastrectomy for IIa type of early gastric cancer. Pure-tone audiometry, Bekesy audiometry, speech audiogram and auditory brain stem response revealed sensorineural hearing loss. The findings of magnetic resonance imaging, magnetic resonance angiography, single photon emission computed tomography and positron emission tomography ruled out a central nervous system disorder. The presence of an elevated C-reactive protein level, von Willebrand factor and plasmin alpha 2-plasmin inhibitor complex suggested vasculitis to be involved in the development of hearing loss. Although pulse-dose methylprednisolone therapy effectively arrested the acute progression of hearing loss, repeated audiograms showed that a modest dosage of oral prednisolone failed to maintain such improvement. However, after performing high-dose cyclophosphamide (CY) therapy, a significant improvement in the hearing loss (more than 10 dB) was observed. As a result, CY is thus considered to be a potentially important treatment for sensorioneural hearing loss.
一名自1985年起被诊断为白塞病的54岁男性，因发热、双侧慢性听力损失以及在接受IIa型早期胃癌的部分腹腔镜胃切除术后反复出现突发性左耳失聪症状而入院。纯音听力测定、贝凯西听力测定、言语听力图和听觉脑干反应显示为感音神经性听力损失。磁共振成像、磁共振血管造影、单光子发射计算机断层扫描和正电子发射断层扫描的结果排除了中枢神经系统疾病。C反应蛋白水平、血管性血友病因子和纤溶酶α2-纤溶酶抑制剂复合物升高表明血管炎与听力损失的发生有关。尽管脉冲剂量甲泼尼龙疗法有效地阻止了听力损失的急性进展，但反复的听力图显示，中等剂量的口服泼尼松龙未能维持这种改善。然而，在进行大剂量环磷酰胺（CY）治疗后，观察到听力损失有显著改善（超过10分贝）。因此，CY被认为是治疗感音神经性听力损失的一种潜在重要疗法。
