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

Abstract

Objective: To enrich clinical information of auditory neuropathy spectrum disorder.

Methods: Forty-eight infants and young children with severely abnormal auditory brainstem responses (ABRs) along with preserved distortion product otoacoustic emissions (DPOAEs) and/or cochlear microphonics (CMs) were included in this retrospective study. Click ABRs, CMs, DPOAEs, behavioral thresholds and tympanograms were established.

Results: Audiological information of 88 ears (40 cases were bilateral and 8 unilateral) that met the inclusion criteria were analyzed. Most of the ears (68.2%) had absent click ABRs at the maximum presentation level of 100 dB nHL, while other ears had repeatable wave V with lowest thresholds of 80 dB nHL. Behavioral hearing of 23 cases ranged from mild (n = 1), moderate (n = 2) to severe and profound loss (n = 20). CMs were present in all ears in spite of those with middle ear pathology, while DPOAEs were present only in 40% of the affected ears.

Conclusion: Diverse results of audiological assessments are shown in this group of children. Concurrent pathologies may make auditory neuropathy spectrum disorder undetected. Absent or severely abnormal ABR along with present CMs are the most reliable measures for detecting this disorder at this stage.