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Electrophysiological Investigation of the Auditory System in Friedreich’s Ataxia

Published online by Cambridge University Press:  18 September 2015

M.J. Taylor ,
J.B. McMenamin ,
E. Andermann  and
G.V. Watters
M.J. Taylor
Affiliation:
Division of Neurology, Montreal Children’s Hospital; the Department of Pediatrics, University of Toronto; the Division of Neurology, the Hospital for Sick Children, Toronto and the Department of Neurogenetics, Montreal Neurological Institute
J.B. McMenamin
Affiliation:
Division of Neurology, Montreal Children’s Hospital; the Department of Pediatrics, University of Toronto; the Division of Neurology, the Hospital for Sick Children, Toronto and the Department of Neurogenetics, Montreal Neurological Institute
E. Andermann
Affiliation:
Division of Neurology, Montreal Children’s Hospital; the Department of Pediatrics, University of Toronto; the Division of Neurology, the Hospital for Sick Children, Toronto and the Department of Neurogenetics, Montreal Neurological Institute
G.V. Watters
Affiliation:
Division of Neurology, Montreal Children’s Hospital; the Department of Pediatrics, University of Toronto; the Division of Neurology, the Hospital for Sick Children, Toronto and the Department of Neurogenetics, Montreal Neurological Institute
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Abstract:

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Auditory brainstem responses (ABRs) and cortical auditory evoked responses (AERs) were studied in a series of 16 Friedreich’s ataxia patients who varied in age, degree of clinical involvement and duration of the disorder. The ABRs were markedly abnormal in all but the youngest patient, and the abnormalities reflected the severity and duration of the disease. The latencies of the AERs were significantly longer in the Friedreich’s ataxia patients compared to normal controls, suggesting cortical as well as peripheral involvement of the auditory system. These data are discussed in terms of the neuropathology of the disorder and the similarities with the other sensory systems in Friedreich’s ataxia patients.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 131 - 135
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

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