Audiological profiling of 198 individuals with auditory neuropathy spectrum disorder

Abstract

Objective: The study aimed at exploring the audiological characteristics and the relationship between speech identification scores with cortical evoked potential measures and hearing aid benefit in individuals with auditory neuropathy spectrum disorder (ANSD). Study design: We initiated a retrospective study of patients who were diagnosed as having ANSD. In 198 patients with ANSD, patient history, risk factors, degree and configuration of hearing loss, speech identification scores in quiet and noise, otoacoustic emissions (OAE), immittance evaluation, auditory brainstem responses (ABR), long latency responses (LLR) and hearing aid benefit were assessed. Results: The results showed that the majority of the patients had bilateral ANSD with a female to male ratio of 1.25:1 with the majority having a rising configuration of hearing loss. OAEs were present in most with absent stapedial reflexes and ABR. The study also showed that individuals with ANSD do obtain benefit from hearing aids and it is related to the presence of LLR. Conclusions: The present study emphasizes the audiological characteristics of a large group with ANSD. Among the management options, hearing aids were found to be useful in only a few of the patients and other rehabilitation options, such as cochlear implants, need to be explored in future studies. Currently, the determining factors for benefit with hearing aid are speech identification scores and cortical auditory evoked potentials.