Aided Performance in Individuals with Late-Onset Auditory Neuropathy Spectrum Disorder (ANSD) - A Pilot Study

Deepthi L, Dr. Satish Kumaraswamy, Nandhu S Mohan

Abstract: Auditory neuropathy spectrum disorder ANSD is a condition characterized by distorted transmission of auditory signals from the inner ear to the auditory nerve and brainstem. In this disorder, outer hair cell function within the cochlea remains normal, while auditory nerve function is disrupted. The disruption is believed to result from desynchronized or reduced neural activity. The term ANSD was suggested considering the varied affected loci rather than a specific site of damage. This study aims to explore the aided speech perception in individuals with late-onset ANSD using different hearing aid configurations. The effect of amplification on speech perception, including the use of acoustic enhancement strategies, is investigated to provide appropriate management for this challenging condition.

Keywords: Late-onset ANSD, acoustic enhancement strategies, hearing aid, aided speech perception