Audiological results in a group of children with auditory neuropathy spectrum disorder

Lingyan Mo1, Fei Yan, Hui Liu

  • 1Key Laboratory and Department of Otolaryngology, Head and Neck Surgery, Capital Medical University, Beijing, PR China.

Insights

Auditory neuropathy spectrum disorder in children is complex. Absent auditory brainstem responses (ABRs) with present cochlear microphonics (CMs) are key indicators for diagnosis.

Area of Science:

  • Pediatric Audiology
  • Neuroscience
  • Otolaryngology

Background:

  • Auditory neuropathy spectrum disorder (ANSD) presents diagnostic challenges in infants and young children.
  • Understanding the audiological profile is crucial for early intervention.

Purpose of the Study:

  • To provide detailed clinical information on ANSD in a pediatric cohort.
  • To identify reliable audiological markers for ANSD detection.

Main Methods:

  • Retrospective analysis of 48 infants and young children with abnormal auditory brainstem responses (ABRs) and present otoacoustic emissions (OAEs) or cochlear microphonics (CMs).
  • Evaluation included click ABRs, CMs, distortion product otoacoustic emissions (DPOAEs), behavioral thresholds, and tympanograms.

Main Results:

  • 88 ears analyzed; 68.2% had absent click ABRs. Repeatable wave V ABRs were observed at 80 dB nHL in others.
  • Behavioral hearing loss ranged from mild to profound.
  • CMs were consistently present, while DPOAEs were found in only 40% of ears.

Conclusions:

  • ANSD exhibits diverse audiological findings in children.
  • Concurrent pathologies can mask ANSD.
  • Absent or abnormal ABRs combined with present CMs are reliable indicators for diagnosing ANSD.
Abstract

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