Correlating auditory evoked and brainstem histologic abnormalities in infantile Gaucher's disease.

@article{Lacey1984CorrelatingAE,
  title={Correlating auditory evoked and brainstem histologic abnormalities in infantile Gaucher's disease.},
  author={D. J. Lacey and Korn{\'e}l Terpl{\'a}n},
  journal={Neurology},
  year={1984},
  volume={34 4},
  pages={539-41}
}
In an infant with Gaucher's disease, brainstem auditory evoked responses were abnormal on three separate occasions. Only waves I and II were typically present at standard intensity settings, although a third peak was elicited at 88 dB. Histologic studies demonstrated absence of neurons in the cochlear nuclei and a hypoplastic superior olivary complex. This neurophysiologic-pathologic correlation supports contentions that both brainstem auditory evoked response (BAER) waves I and II arise from… CONTINUE READING

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