Sawaishi Y, Tomita Y, Mito T
Division of Child Neurology, Tottori University School of Medicine, Yonago.
No To Hattatsu. 1990 May;22(3):223-9.
Seventeen patients with no auditory brainstem evoked response (ABR) who suffered from various neurological disorders were reported. We evaluated the possibilities of co-existent brainstem lesions in addition to the peripheral impairment in the auditory pathway, by assessing neurological findings and other laboratory examinations, including cranial CT and electrically elicited blink reflex. Patients who showed cranial nerve symptoms other than that of the acoustic nerve or abnormal postural reflexes were suspected to have brainstem dysfunction. It was difficult, however, to exclude the influence from the dysfunction in the more central level CNS. Definite brainstem atrophy was revealed radiologically only in one case who was at the end stage of the degenerative disease. Blink reflex was studied in eleven cases, four of whom revealed abnormal responses, also suggesting brainstem dysfunction. All the five cases, consistent with these abnormal laboratory findings, had shown severe delay in motor development. Other five patients who showed rather good auditory behavior were considered to have 'desynchronization' response to ABR in the auditory pathway at the peripheral level. Many pathophysiological conditions may be involved in the phenomenon of absent ABR, which should be carefully evaluated from the viewpoints of clinical neurology.
报告了17例无听觉脑干诱发电位（ABR）的患者，他们患有各种神经系统疾病。我们通过评估神经学检查结果和其他实验室检查，包括头颅CT和电诱发眨眼反射，来评估除听觉通路外周损伤外并存脑干病变的可能性。表现出除听神经外的颅神经症状或异常姿势反射的患者被怀疑有脑干功能障碍。然而，很难排除中枢神经系统更高级别功能障碍的影响。仅在1例处于退行性疾病末期的患者中，影像学显示有明确的脑干萎缩。对11例患者进行了眨眼反射研究，其中4例显示异常反应，也提示脑干功能障碍。与这些异常实验室检查结果一致的所有5例患者均表现出严重的运动发育迟缓。其他5例听觉行为较好的患者被认为在听觉通路外周水平对ABR有“失同步”反应。ABR缺失现象可能涉及多种病理生理状况，应从临床神经学角度仔细评估。
