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Studies on pediatric patients with absent auditory brainstem response (ABR) later components
1National Center of Neurology and Psychiatry, National Institute of Mental Health, Chiba, Japan.
Insights
Pediatric patients with abnormal auditory brainstem responses (ABR) often exhibit severe neurological deficits. Missing later ABR waves indicates significant brainstem and neurological dysfunction in children.
Area of Science:
- Pediatric Neurology
- Neurophysiology
- Auditory Neuroscience
Background:
- Auditory Brainstem Response (ABR) is crucial for assessing auditory pathway function.
- ABR abnormalities can indicate underlying neurological conditions in children.
- Limited components of ABR (wave I or I-II) suggest significant neural pathway disruption.
Purpose of the Study:
- To analyze the clinical characteristics of pediatric patients with limited ABR components (wave I or I-II).
- To determine the incidence of such ABR abnormalities in a pediatric population.
- To correlate ABR findings with neurological status and diagnoses.
Main Methods:
- Clinical analysis of eleven pediatric patients with specific ABR patterns.
- Review of audiological and neurological examination data.
- Calculation of the incidence of ABR abnormalities in 1,650 pediatric patients.
Main Results:
- The incidence of wave I or I-II ABR abnormality was 0.67% in the studied pediatric cohort.
- All eleven patients presented with severe mental retardation.
- Nine patients had convulsions, eight showed mental/motor deterioration, and seven had disturbed consciousness, including four in coma.
Conclusions:
- Limited ABR components (wave I or I-II) in pediatric patients are associated with severe neurological impairments.
- These findings highlight the significance of later ABR waves for assessing brainstem and broader neurological function.
- Patients lacking later ABR components often display profound neurological abnormalities beyond the brainstem.
Abstract:
Eleven pediatric patients with only wave I or waves I and II of their ABR were clinically analyzed. The clinical diagnoses of these patients were as follows: 1) anoxic encephalopathy in two cases; 2) neonatal asphyxia in one; 3) infantile Gaucher's disease in one; 4) mitochondrial encephalomyopathy in one; 5) suspected Pelizaeus-Merzbacher disease in three; 6) degenerative disease of unknown etiology in two (presumptive diagnosis were progressive supranuclear palsy and dentate-rubro-pallido-Luysian atrophy); and 7) infantile spasms with congenital malformation of the brain and bones in one. The incidence of the patients with this type of ABR abnormality was 0.67% among 1,650 of our pediatric patients whose ABRs were examined because of audiological or neurological problems. All eleven patients showed severe mental retardation. Nine of the eleven had convulsions and likewise, eight of eleven showed deterioration in mental and/or motor activities. Furthermore seven of eleven had disturbed consciousness and four of these seven were in deep coma. Other brainstem and bulbar signs and symptoms were frequently found in these patients. In our series, patients without the later components of ABR manifested marked neurological abnormalities inside and outside the brainstem.