首诊为听力下降的脑白质病患者影像学及临床特征分析

目的：初步探讨首诊为听力下降的脑白质病患者影像学及临床特征。方法：回顾性分析以听力下降为首诊原因的7例脑白质病患者的头颅MRI及临床特征，包括病变发生的部位、形态、范围及血浆极长链脂肪酸水平（VLCFA）测定。结果：2例患者头颅MRI显示双侧侧脑室后角旁对称分布长T1长T2信号，呈蝶翼状改变，其余5例患者头颅MRI显示在脑白质内不同部位异常长T1长T2信号。发生部位多见于额叶白质内，其次为双侧侧脑室后角旁；7例患者均表现为小斑片状影；2例单发生于侧脑室后角旁，1例单发生于额叶，其余5例发生于2个或2个以上部位。3例患者VLCFA测定在正常范围，其余4例患者家属拒绝测定。结论：以听力下降为首诊原因的脑白质病患者应常规行头颅MRI检查，头颅MRI检查可清晰显示异常白质，以免遗漏已经存在的病变。

Analysis of imaging and clinical feature of leukoencephalopathy with hearing loss first complaint

Objective:To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.Method:The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed,including their place,shape,range and blood plasma very-long-chain fatty acid(VLCFA).Result:The head MRI of 2 cases shows symmetry distributed long T_1 and T_2 signal like butterfly aliform in white matter beside both cornu posterious ventriculi lateralis.The head MRI of 5 cases shows abnormal long T_1 and T_2 signal in different part in Alba.The pathological changes often located in white matter of frontal lobe and secondly beside both eornu posterious ventriculi lateralis.The shape shows little patching in 7 cases.The range only shows beside cornu posterious ventriculi lateralis in 2 cases.The range only shows in frontal lobe in 1 cases.Two or more than two parts shows in the rest 5 cases.The result of VLCFA is normal in 3 cases.The rest 4 cases refused to have examination of VLCFA. Conclusion:The patient who firstly complained of hearing loss should be routinely done head MRI.The head MRI imaging could clearly show abnormal white matter in order to avoid omitting existent pathological changes.