Brainstem auditory evoked responses in leukodystrophies

Rachel Ochs, Omkar N. Markand, William E. Demyer

Research output: Contribution to journalArticle

72 Scopus citations


Brainstem auditory evoked responses (BAERs) were recorded in seven patients with Pelizaeus-Merzbacher leukodystrophy (PMD), two with adrenoleukodystrophy (ALD), and one with metachromatic leukodystrophy (MLD). BAERs were altered in all patients, and the alterations were severe in 9 of the 10 patients. A patient with ALD who as yet had no neurologic symptoms showed only minimal abnormality of the BAERs, consisting of prolongation of the latency of wave V. In the remaining nine patients, only wave I generated in the extramedullary portion of the eighth nerve was recorded with or without a wave II. Subsequent components (III through VII) were absent. No abnormality of BAERs was observed in the 10 known carriers of PMD. The combination of BAERs and EEG is helpful in differentiating leukodystrophy from progressive gray matter diseases.

Original languageEnglish (US)
Pages (from-to)1089-1093
Number of pages5
Issue number8
StatePublished - Aug 1979

ASJC Scopus subject areas

  • Clinical Neurology

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    Ochs, R., Markand, O. N., & Demyer, W. E. (1979). Brainstem auditory evoked responses in leukodystrophies. Neurology, 29(8), 1089-1093.