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Updated: Jul 10, 2026
Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice
Published on: May 10, 2019
Discrepancy between auditory brainstem responses, auditory steady-state responses, and auditory behavior in two
Manabu Tanaka1, Shin-Ichiro Hamano, Hideaki Sakata
1Division of Neurology, Saitama Children's Medical Center, Saitama, Japan. a1099655@pref.saitama.lg.jp
Abstract:
We reported two cases of Pelizaeus-Merzbacher disease. Both cases visited our hospital manifesting horizontal nystagmoid movements present from birth, and delayed motor development. Magnetic resonance imaging of the brain showed diffuse dysmyelination of the cerebral white matter, and auditory brainstem response showed waves I and II but absence of all subsequent components. Conditioned orientation reflex (COR) audiometry showed poor reactions in an infantile case whose development was severely retarded, and who spoke no meaningful words. Auditory steady-state response (ASSR) was a helpful tool for identifying her auditory ability; thereafter, her communication skills improved naturally. The other case was mildly developmentally retarded, and the results of COR audiometry and ASSR were considered the same level. The discrepancy between results of these hearing tests may arise under the influence of developmental level of the case.
Insights
This study highlights Pelizaeus-Merzbacher disease (PMD) in two cases, noting congenital nystagmus and developmental delays. Auditory Steady-State Response (ASSR) proved valuable for assessing hearing in a severely affected infant, improving communication.
Area of Science:
- Neuroscience
- Genetics
- Pediatrics
Background:
- Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked hypomyelinating disorder.
- It is characterized by significant neurological deficits, including motor and cognitive impairments.
Observation:
- Two cases of PMD presented with congenital horizontal nystagmus and delayed motor development.
- Brain MRI revealed diffuse cerebral white matter dysmyelination.
- Auditory Brainstem Response (ABR) showed specific wave pattern abnormalities.
Findings:
- Conditioned Orientation Reflex (COR) audiometry indicated poor auditory responses in a severely affected infant.
- Auditory Steady-State Response (ASSR) effectively identified auditory ability in the infant, leading to improved communication.
- In a mildly affected case, COR and ASSR results were comparable, suggesting developmental level influences test interpretation.
Implications:
- ASSR is a crucial tool for evaluating auditory function in infants with PMD.
- Early identification of auditory ability can facilitate communication development in affected children.
- Understanding the impact of developmental status on audiological assessments is vital for accurate diagnosis and management.
