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NEUROLOGY 1983;33:1071
© 1983 American Academy of Neurology

Early abnormalities of brainstem auditory evoked potentials in Friedreich's ataxia

Evidence of primary brainstem dysfunction

Bahman Jabbari, MD, Daniel M. Schwartz, PhD, Donna M. MacNeil, MA and Steven B. Coker, MD

Department of Neurology of the Uniformed Services University of the Health Sciences, Bethesda, MD, and the Neurology Service of Walter Reed Hospital, Washington, DC (Drs. Jabbari and Coker) and from the Audiology Department (Dr. Schwartz and Ms MacNeil) of Walter Reed.

We studied five children with classic Friedreich's ataxia, using an audiologic test battery to determine the primary site of auditory dysfunction. None of the children had any hearing complaints, and all were tested soon after onset of symptoms. The audiologic test battery consisted of brainstem auditory evoked potential test, tympanometry, and acoustic reflex measurements. The results indicated that the brainstem was the primary site of auditory dysfunction.

Address correspondence and reprint requests to Dr. Jabbari, P.O. Box 310, Walter Reed Army Medical Center, Washington, DC 20012.

Accepted for publication December 4, 1982.




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