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Related Experiment Video

Updated: Jul 12, 2026

Neurobehavioral Assessments in a Mouse Model of Neonatal Hypoxic-ischemic Brain Injury
08:32

Neurobehavioral Assessments in a Mouse Model of Neonatal Hypoxic-ischemic Brain Injury

Published on: November 24, 2017

Preservation of brainstem neurophysiological function in hydranencephaly.

S A Counter1

  • 1Neurology Department, Harvard Medical School, Massachusetts General Hospital, Harvard Biological Laboratories, Cambridge, MA 02138, United States. allen_counter@harvard.edu

Journal of the Neurological Sciences
|August 28, 2007
PubMed
Summary

This study assessed neurophysiological function in hydranencephaly using evoked potentials. Results indicate intact brainstem auditory pathways, suggesting preserved unilateral auditory function despite absent cortical responses.

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Area of Science:

  • Neuroscience
  • Clinical Electrophysiology

Background:

  • Hydranencephaly is a rare congenital disorder characterized by the absence of cerebral hemispheres.
  • Assessing central neurophysiological function in such cases is crucial for understanding residual brain function.

Purpose of the Study:

  • To evaluate the preservation of central neurophysiological function in a patient with hydranencephaly.
  • To correlate electrophysiological findings with clinical observations of auditory responses.

Main Methods:

  • Utilized a comprehensive panel of evoked potentials: brainstem auditory evoked responses (BAER), auditory middle latency responses (MLR), cortical auditory evoked responses (CER), electroretinograms (ERG), visual evoked responses (VER), and somatosensory evoked responses (SER).
  • Stimulation included auditory (ear-specific), visual (strobe flash), and somatosensory (median and tibial nerves).

Main Results:

  • Normal BAERs were observed for right ear stimulation, but abnormal for the left.
  • Auditory MLR and CER were absent bilaterally.
  • Strobe ERGs were grossly normal, while strobe VERs were abnormal.
  • SER revealed significant conduction defects in the median nerve pathways and rostral to the lower spinal cord for tibial nerves.

Conclusions:

  • Brainstem electrophysiological measures demonstrated functional auditory afferent tracts and nuclei.
  • The findings suggest intact unilateral auditory function, supporting clinical observations of behavioral auditory responses in hydranencephaly, despite the absence of cortical processing.