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Updated: Jun 1, 2026
Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities
Published on: January 29, 2014
Auditory impairment in infants at risk for bilirubin-induced neurologic dysfunction
Steven M Shapiro1, Gerald R Popelka
1Division of Child Neurology, Department of Neurology, Medical College of Virginia Campus, Virginia Commonwealth University Medical Center, Richmond, VA 23298-0211, USA. sshapiro@vcu.edu
Insights
Bilirubin toxicity causes auditory neuropathy spectrum disorder, damaging the auditory system from the brainstem to neural centers, but not the cochlea. Auditory measures can diagnose this, and interventions like cochlear implants may help.
Area of Science:
- Neuroscience
- Audiology
- Toxicology
Background:
- Kernicterus, a bilirubin-induced brain injury, can affect auditory pathways.
- Auditory neuropathy spectrum disorder (ANSD) is characterized by impaired neural signal transmission from the cochlea to the brain.
Purpose of the Study:
- To differentiate classical and subtype kernicterus using auditory measures.
- To elucidate the progression and characteristics of bilirubin-induced auditory neural damage.
- To assess the diagnostic utility of auditory measures for bilirubin neurotoxicity.
Main Methods:
- Utilized physiological auditory measures, including auditory-evoked potentials and cochlear integrity tests.
- Analyzed auditory system damage progression from brainstem to higher neural centers.
- Distinguished between cochlear and neural auditory function.
Main Results:
- Bilirubin toxicity primarily affects the auditory system at the brainstem and VIII cranial nerve levels, sparing the cochlea.
- Auditory neural damage manifests as timing deficits, dyssynchrony, reduced responses, or response elimination.
- Auditory measures effectively diagnose bilirubin-induced auditory neural damage.
Conclusions:
- Auditory neuropathy spectrum disorder is a comprehensive description of bilirubin-induced auditory neural damage.
- Independent cochlear and auditory neural function assessments are crucial for diagnosis.
- Interventions such as cochlear implants show potential effectiveness in managing this condition.
Abstract:
Classical and subtypes of kernicterus associated with bilirubin toxicity can be differentiated in part with physiological auditory measures that include auditory-evoked potentials and measures of cochlear integrity. The combination of these auditory measures suggests that bilirubin exposure results in auditory system damage initially at the level of the brainstem, progressing to the level of the VIII cranial nerve and then to greater neural centers. There is no evidence of neural damage at the level of the cochlea. Auditory neural damage from bilirubin toxicity ranges from neural timing deficits, including neural firing delays and dyssynchrony, to neural response reduction and even elimination of auditory neural responses. This condition is comprehensively described as auditory neuropathy spectrum disorder. Independent measures of cochlear function and auditory neural function up to the level of the brainstem can effectively diagnose auditory neural damage resulting from bilirubin neurotoxicity. Intervention, including cochlear implants can be effective.
