Auditory function in adrenomyeloneuropathy

Joseph P. Pillion, Hugo W. Moser, Gerald V. Raymond

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)24-29
Number of pages6
JournalJournal of the neurological sciences
Issue number1-2
StatePublished - Jun 15 2008

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology


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