Hattori H, Inada H, Tanaka K, Niihira S, Seto T, Matsuoka O, Isshiki G
Department of Pediatrics, Osaka City University Medical School.
No To Hattatsu. 1998 Sep;30(5):387-93.
Auditory brainstem responses (ABR) and other evoked potentials were studied in four patients with the Wolfram syndrome. In three cases ABR was abnormal in the early stage of the disease. There was no responses or only the wave V with prolonged latency at a stimulation level of 80 dBnHL. A prolongation of the I-V interpeak latency (IPL) was also revealed at a stimulation level of 105 dBnHL. The remaining patient showed shortening of the I-V IPL. The visual evoked potentials showed prolonged peak latency in three cases, and the median nerve short latency somatosensory evoked potentials were normal in two cases. These ABR findings indicated not only sensory neuronal hearing loss but also a degenerative change in the brain stem in the Wolfram syndrome.
对4例沃夫勒姆综合征患者进行了听觉脑干反应（ABR）及其他诱发电位研究。3例患者在疾病早期ABR异常。在80 dBnHL的刺激水平下无反应或仅出现波V潜伏期延长。在105 dBnHL的刺激水平下也发现I-V峰间期（IPL）延长。其余1例患者I-V IPL缩短。3例患者视觉诱发电位显示峰潜伏期延长，2例患者正中神经短潜伏期体感诱发电位正常。这些ABR结果表明，沃夫勒姆综合征不仅存在感觉神经元性听力损失，还存在脑干的退行性改变。
