Pinard Minola, Chertkow Howard, Black Sandra, Peretz Isabelle
Department of Clinical Neurosciences, Sir Mortimer Davis/Jewish General Hospital and McGill University, Montréal, Canada.
Neurocase. 2002;8(1-2):40-55. doi: 10.1093/neucas/8.1.40.
AL, a woman with an acquired disturbance of auditory processing beginning in the second decade, was originally diagnosed as having pure word deafness. Recent analysis with a wide range of stimuli suggests that her comprehension deficit also extends to a subset of musical and non-verbal environmental sounds. The perceptual demands of the different auditory stimuli appear to account for part of the apparent material specificity. Additionally, over the years, the presumed temporal lobe cortical pathology has been supplemented by a mild to moderate, peripheral low-frequency hearing loss and evidence of dysfunction in lower level auditory processing pathways. The current peripheral dysfunction closely resembles cases recently labeled as auditory neuropathy. The diagnosis of pure word deafness should not be based on a limited set of auditory stimuli; additionally, a careful assessment using modern audiological techniques should be performed to evaluate peripheral auditory functions.
AL是一位从二十多岁开始出现后天听觉处理障碍的女性，最初被诊断为患有纯词聋。近期对多种刺激的分析表明，她的理解缺陷还延伸至一部分音乐和非言语环境声音。不同听觉刺激的感知要求似乎是造成明显材料特异性的部分原因。此外，多年来，推测的颞叶皮质病变已被轻度至中度的外周低频听力损失以及较低水平听觉处理通路功能障碍的证据所补充。目前的外周功能障碍与最近被称为听觉神经病的病例非常相似。纯词聋的诊断不应基于一组有限的听觉刺激；此外，应使用现代听力学技术进行仔细评估，以评估外周听觉功能。
