Reversible cochlear disorders with normal vestibular functions in three cases with Wegener's granulomatosis
Reversible cochlear disorders with normal vestibular functions in three cases with Wegener's granulomatosis
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韦格纳肉芽肿病三例前庭功能正常的可逆性耳蜗疾病
DOI：
10.1016/j.anl.2011.03.010
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发表时间：
2011
影响因子：
1.7
通讯作者：
Naito Y
中科院分区：
文献类型：
--
作者：
Yamazaki H;Fujiwara K;Shinohara S;Kikuchi M;Kanazawa Y;Kurihara R;Kishimoto I;Naito Y
韦格纳肉芽肿病(WG)患者常伴有听力损失，但其确切的发病机制尚不清楚。我们经历了3例WG患者，其首发症状为双侧进行性混合性听力损失(包括传导性和感觉神经性)，一年后出现全身症状。根据抗中性粒细胞胞浆抗体(ANCA)阳性的血清学和病变的病理结果以及全身症状，他们被诊断为WG。虽然它们在ANCA的类型和全身损害方面不同，但都显示出相当可逆的耳蜗病，前庭功能正常。此外，他们最初的耳科表现有相同的特征：(1)鼓膜增厚伴搏动性浆液性鼓室积液，(2)言语辨别能力差，(3)激素依赖性听力改变。他们在椅子前庭-眼反射(VOR)测试和冷空气热量测试中没有表现出明显的前庭异常，即使他们有严重的听力损失。在这些结果的基础上，我们推测产生耳蜗内电位的血管纹血管炎可能导致这些可逆性耳蜗特异性功能障碍。
Patients with Wegener's granulomatosis (WG) often suffer from hearing loss, but its precise mechanisms have not been well understood. We experienced 3 WG cases whose initial symptoms were bilateral progressive mixed (both conductive and sensorineural) hearing loss, followed by systemic symptoms one year later. They were diagnosed as WG based on positive serology of anti-neutrophil cytoplasmic antibodies (ANCAs) and pathologic findings of affected lesions in addition to systemic symptoms. Although they were different in the type of ANCAs and systemic lesions, all showed considerably reversible cochlear disorders with normal vestibular functions. Moreover, their initial otologic manifestations shared same characteristic features, (1) thick ear drums with pulsatile serous intratympanic effusion, (2) poor speech discrimination ability, and (3) steroid-dependent changes of hearing levels (HLs). They exhibited no significant vestibular abnormalities in chair vestibule-ocular reflex (VOR) testing and cold air caloric tests even when they had severe hearing loss. On the basis of these results, we hypothesized that vasculitis of stria vascularis which generates endocochlear potential might cause these reversible cochlear-specific dysfunctions.