Auditory function in adrenomyeloneuropathy
- 作者：Joseph P. Pillion ; Hugo W. Moser ; Gerald V. Raymond
- 关键词：Auditory brainstem response (ABR) ; Acoustic reflex ; Adrenomyeloneuropathy (AMN) ; Masking level difference (MLD) ; Word recognition ; Very long chain fatty acids (VLCFA)
- 刊名：Journal of the Neurological Sciences
- 出版年：2008
- 期刊代码：178_0022510x
- 类别：med
- 出版时间：15 June 2008
- 卷：269
- 期：1-2
- 页码：24-29
- 文件大小：419 K
摘要
Auditory brainstem responses (ABR), ipsilateral and contralateral acoustic reflexes and the masking level difference for speech (MLD) were studied in 29 patients with adrenomyeloneuropathy (AMN). Abnormalities were seen for all ABR components with Waves V and III affected to the greatest degree. For male patients with AMN, the I–III, III–V and I–V interpeak latency intervals were abnormal for a majority of patients. For female patients with AMN, the I–V and III–V interpeak latency intervals were abnormal for a majority of patients with the I–III interval less affected. Contralateral acoustic reflexes were elevated or absent for approximately 50%of ears. Ipsilateral acoustic reflexes were abnormal for 25%of ears. MLDs were significantly reduced in 72%of patients. When considered in terms of the earliest ABR wave abnormality, the earlier components of the ABR (i.e., Waves III and I) were the initial components impaired for the majority of ears. Word recognition in quiet was relatively unimpaired for all subjects. Despite the presence of marked ABR abnormalities, patients with AMN denied the presence of significant difficulty hearing.