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Updated: Jun 18, 2026
Behavioral Assessment of Hearing in 2 to 4 Year-old Children: A Two-interval, Observer-based Procedure Using Conditioned Play-based Responses
Published on: January 23, 2017
[The diagnosis of auditory neuropathy in young children]
1Key Laboratory of Otolaryngology Head and Neck Surgery, Capital Medical University, Ministry of Education, Beijing Tongren Hospital of Capital Medical University, Beijing Institute of Otolaryngology, Beijing 100005, China. moly@trhos.com
Insights
Auditory neuropathy (AN) in children is often identified by abnormal auditory brainstem responses (ABRs) but present otoacoustic emissions. Further testing, including cochlear nerve MRI, is crucial for accurate diagnosis and management.
Area of Science:
- Pediatric Audiology
- Neuroscience
- Otoacoustic Emissions
Context:
- Auditory neuropathy (AN) presents a diagnostic challenge in infants and young children.
- Distinguishing AN from other hearing impairments requires comprehensive audiological evaluation.
- Severely abnormal auditory brainstem responses (ABRs) with present otoacoustic emissions are key indicators.
Purpose:
- To analyze the clinical data of children diagnosed with auditory neuropathy.
- To evaluate the utility of various audiological tests in identifying AN.
- To determine the necessity of further investigations like MRI for AN diagnosis.
Summary:
- This study retrospectively analyzed 48 children with abnormal click ABRs and present cochlear microphonics (CMs) and/or distortion product otoacoustic emissions (DPOAEs).
- Results showed absent click ABRs in most ears, but present CMs were universal, while DPOAEs were preserved in only 41.7%.
- Four children were diagnosed with cochlear nerve deficiency (CND) via inner ear MRI, highlighting its importance.
Impact:
- Routine CM testing is recommended for children with abnormal ABRs to detect AN.
- Behavioral hearing cannot be reliably predicted by auditory evoked responses alone.
- MRI is essential for evaluating cochlear nerve development when AN is suspected.
Objective:
To present the clinical data of a group of children with audiological profile of auditory neuropathy.
Method:
Forty-eight infants and young children who had severely abnormal click ABRs along with present CMs and/or DPOAEs were included in this retrospective study. Click ABRs, CMs, DPOAEs, tympanograms, behavioral thresholds and inner ear MRI were analysed.
Result:
Fourty children present with bilateral audiological profile of AN, 8 were unilateral. Most of the ears (68.2%) were of absent click ABRs at the maximum presentation level of 100 dB nHL. However, some of them had repeatable Wave V at very high presentation levels. All ears showed present CMs while only 41.7% showed preserved DPOAEs. Four children were diagnosed as CND by further investigation of inner ear MRI.
Conclusion:
It is recommended that CM testing be carried out routinely in children with severely abnormal click ABRs so as not to miss out AN; Behavioral hearing can not be predicted by auditory evoked responses; if audiological profile of AN is detected, further MRI exploration of well-developed cochlear nerve is necessary.
