Wang Lin'e, Zhang Daoxing, Ma Qingxue, Xue Qiming, Xie Yanchen
Department of Otorhinolaryngology, Beijing Friendship Hospital, Capital University of Medical Science, Beijing, 100050, China.
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2009 Nov;23(22):1014-7.
To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.
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).
The head MRI of 2 cases shows symmetry distributed long T, and T2 signal like butterfly aliform in white matter beside both cornu posterior ventriculi lateralis. The head MRI of 5 cases shows abnormal long T1 and T2 signal in different part in Alba. The pathological changes often located in white matter of frontal lobe and secondly beside both cornu posterior ventriculi lateralis. The shape shows little patching in 7 cases. The range only shows beside cornu posterior 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.
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.
探讨以听力减退为首发症状的白质脑病的影像学及临床特征。
回顾性分析7例以听力减退为首发症状的白质脑病患者的头颅MRI及临床特征，包括其部位、形态、范围及血极长链脂肪酸（VLCFA）情况。
2例患者头颅MRI显示双侧侧脑室后角旁白质呈蝶翼状对称性分布长T1、T2信号。5例患者头颅MRI显示脑白质不同部位长T1、T2信号异常。病变常位于额叶白质，其次为双侧侧脑室后角旁。7例患者形态均呈小片状。范围方面，2例仅见于双侧侧脑室后角旁，1例仅见于额叶，其余5例见于两个或两个以上部位。3例患者VLCFA结果正常，其余4例拒绝行VLCFA检查。
对于以听力减退为首发症状的患者，应常规行头颅MRI检查。头颅MRI成像可清晰显示白质异常，以免漏诊存在的病变。
