Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder.

OBJECTIVE

The objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6).

STUDY DESIGN

Case series.

SETTING

Single tertiary care academic medical center.

SUBJECTS AND METHODS

All pediatric patients with a confirmed diagnosis of ANSD on Auditory Brainstem Response (ABR) testing who presented to a single tertiary medical center between September 2008 and September 2018 were included. Only patients that underwent Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS) and/or Ling 6 Sound Test (Ling 6) were included in the study. Audiologic testing performed after cochlear implantation was excluded.

RESULTS

60 pediatric patients with ANSD were analyzed. There were 10 patients included in the study with documented hearing aid use who underwent IT-MAIS and/or Ling 6 testing. Average IT-MAIS score improved by 20.4% after initial or extended trial of amplification. Similarly, average Ling 6 score improved from 3.6 to 4.8 after initial or extended trial of amplification. The four patients who did not receive amplification had higher average IT-MAIS and Ling 6 scores.

CONCLUSION

In most children with ANSD, IT-MAIS and Ling 6 Sound Test scores improved with initial hearing aid use and over time with extended hearing aid use. Long-term prospective, multi-institutional studies are needed to determine the impact of the natural history of ANSD, comorbidities, and socioeconomic variables on auditory function testing results in children with ANSD using hearing aids.