Usefulness of BAER studies in the early diagnosis of Pelizaeus-Merzbacher disease.
Brainstem auditory evoked responses (BAERs) were markedly abnormal in a 1-month-old infant with Pelizaeus-Merzbacher disease. Wave II occurred at a prolonged latency, and all of the following waves were absent bilaterally. BAER is useful for early diagnosis in this disease.
D001933:Brain Stem / Q000503:physiopathology; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis*; D004576:Electromyography; D005072:Evoked Potentials, Auditory; D006801:Humans; D007231:Infant, Newborn; D008297:Male