Neural Hearing Loss: Mechanisms, Diagnosis and Treatment Horizons.

Abstract

Neural hearing loss, characterized by dysfunction of the auditory nerve, including the spiral ganglion neurons (SGNs) and/or their synaptic connections, is increasingly recognized as a critical contributor to auditory deficits across diverse conditions, including Auditory Neuropathy Spectrum Disorder (ANSD), presbycusis, and noise-induced hearing loss (NIHL). It is possible that neural hearing loss is underdiagnosed, due to the lack of clinical tools with sufficient sensitivity and specificity to detect poor neural health. Current interventions, such as hearing aids and cochlear implants (CIs), primarily target sensory deficits and offer limited benefit in cases of significant neural compromise. Therapeutically, there is a growing shift towards biologically driven strategies aimed at restoring neural function. Recent developments in novel therapies, including pharmacological, gene-based, neurotrophic, and cell-based approaches, have opened new possibilities demonstrating the potential to protect, repair, and/or replace damaged SGNs, and re-establish auditory pathways. This perspectives article explores the evolving understanding of neural hearing loss, emphasizing its complex pathophysiology and the limitations of current diagnostic and therapeutic approaches, while highlighting how a diverse range of emerging solutions are moving closer to clinical application.