Question:
A 6-week-old infant with history of neonatal jaundice presents with parental concern of poor response to sound. Initial OAE screening at birth was normal. What is the next best investigation?
A 6-week-old infant with history of neonatal jaundice presents with parental concern of poor response to sound. Initial OAE screening at birth was normal. What is the next best investigation?
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Auditory Neuropathy Spectrum Disorder (ANSD) Profile:
$\bullet$ Outer Hair Cells intact $\rightarrow$ Normal OAE Present Cochlear Microphonic.
$\bullet$ Auditory Nerve Brainstem impaired $\rightarrow$ Absent or Abnormal ABR/BERA.
Classic cause: Neonatal Hyperbilirubinemia Kernicterus! Always screen high-risk neonates with ABR!
$\bullet$ Outer Hair Cells intact $\rightarrow$ Normal OAE Present Cochlear Microphonic.
$\bullet$ Auditory Nerve Brainstem impaired $\rightarrow$ Absent or Abnormal ABR/BERA.
Classic cause: Neonatal Hyperbilirubinemia Kernicterus! Always screen high-risk neonates with ABR!
Updated On: Sep 3, 2026
- Repeat OAE
- Brainstem Evoked Response Audiometry (BERA/ABR)
- Behavioral observation audiometry
- Impedance audiometry
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The Correct Option is B
Solution and Explanation
Concept:
Neonatal hyperbilirubinemia is a major neurotoxic risk factor for the auditory system, having a specific selective predilection for injuring the cochlear nuclei in the brainstem and the eighth cranial nerve (vestibulocochlear nerve).
This distinct clinical pathology is recognized as Auditory Neuropathy Spectrum Disorder (ANSD).
Explanation:
• Universal newborn hearing screening employs two main physiological technologies: Otoacoustic Emissions (OAE) and Automated Auditory Brainstem Response (AABR).
• Otoacoustic Emissions (OAE): Measures sound waves generated by the contractile motion of outer hair cells (OHCs) in the cochlea. It only assesses the pre-neural cochlear amplifier mechanism.
• Auditory Brainstem Response (ABR BERA): Measures electrophysiological neural conduction along the eighth cranial nerve through the brainstem auditory pathways (cochlear nucleus, superior olivary complex, lateral lemniscus, and inferior colliculus).
• In bilirubin-induced auditory neurotoxicity (ANSD), the outer hair cells of the cochlea remain entirely intact and functional (hence, OAE is completely normal "pass"), while synaptic transmission from inner hair cells or neural conduction along the auditory nerve/brainstem is severely disrupted.
• Consequently, relying solely on OAE in infants with hyperbilirubinemia or NICU stays will falsely miss ANSD.
• Therefore, Diagnostic Brainstem Evoked Response Audiometry (BERA/ABR) is the mandatory next investigation to evaluate neural auditory pathway function.
Final Answer:
Brainstem Evoked Response Audiometry (BERA/ABR) is the next best investigation to diagnose auditory neuropathy spectrum disorder in an infant with hyperbilirubinemia and normal OAE.
Neonatal hyperbilirubinemia is a major neurotoxic risk factor for the auditory system, having a specific selective predilection for injuring the cochlear nuclei in the brainstem and the eighth cranial nerve (vestibulocochlear nerve).
This distinct clinical pathology is recognized as Auditory Neuropathy Spectrum Disorder (ANSD).
Explanation:
• Universal newborn hearing screening employs two main physiological technologies: Otoacoustic Emissions (OAE) and Automated Auditory Brainstem Response (AABR).
• Otoacoustic Emissions (OAE): Measures sound waves generated by the contractile motion of outer hair cells (OHCs) in the cochlea. It only assesses the pre-neural cochlear amplifier mechanism.
• Auditory Brainstem Response (ABR BERA): Measures electrophysiological neural conduction along the eighth cranial nerve through the brainstem auditory pathways (cochlear nucleus, superior olivary complex, lateral lemniscus, and inferior colliculus).
• In bilirubin-induced auditory neurotoxicity (ANSD), the outer hair cells of the cochlea remain entirely intact and functional (hence, OAE is completely normal "pass"), while synaptic transmission from inner hair cells or neural conduction along the auditory nerve/brainstem is severely disrupted.
• Consequently, relying solely on OAE in infants with hyperbilirubinemia or NICU stays will falsely miss ANSD.
• Therefore, Diagnostic Brainstem Evoked Response Audiometry (BERA/ABR) is the mandatory next investigation to evaluate neural auditory pathway function.
Final Answer:
Brainstem Evoked Response Audiometry (BERA/ABR) is the next best investigation to diagnose auditory neuropathy spectrum disorder in an infant with hyperbilirubinemia and normal OAE.
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