Current topics in hearing research: Deafferentation and threshold independent hearing loss

Hearing research findings in recent years have begun to change how we think about hearing loss and how we consider the risk of auditory damage from noise exposure. These findings include evidence of noise-induced cochlear damage in the absence of corresponding permanent threshold elevation or evidence of hair cell loss. Animal studies in several species have shown that noise exposures that produce robust but only temporary threshold shifts can permanently damage inner hair cell synaptic ribbons. This type of synaptic degeneration has also been shown to occur as a result of aging in animals and humans. The emergence of these data has motivated a number of clinical studies aimed at identifying the perceptual correlates associated with synaptopathy. The deficits believed to arise from synaptopathy include poorer hearing in background noise, tinnitus and hyperacusis (loudness intolerance). However, the findings from human studies have been mixed. Key questions remain as to whether synaptopathy reliably produces suprathreshold perceptual deficits or whether it serves as an early indicator of auditory damage with suprathreshold deficits emerging later as a function of further cochlear damage. Here, we provide an overview of both human and animal studies that explore the relationship among inner hair cell damage, including loss of afferent synapses, auditory thresholds, and suprathreshold measures of hearing.