Auditory neuropathies: Understanding their pathogenesis to illuminate intervention strategies

摘要

Purpose of review: For lack of therapy targeting sensorineural hearing loss, hearing-impaired patients must be fitted with sound-amplifying hearing aids or cochlear implants, successfully in a majority of cases. Yet failures are often found among auditory neuropathies. Recent findings: Auditory neuropathies are a class of conditions characterized by disrupted spike synchrony in auditory pathways despite reasonably preserved hearing sensitivity: amplification by hearing aids is inadequate and electrical stimulation of the auditory nerve may not improve discharge synchrony. Summary: Among the already partially understood pathogenetic frameworks, this article reviews physiological reasons why some rehabilitation procedures can restore neural synchrony, whereas others either fail or might even increase the damage, and what tests could help predict the outcome of intervention.