Neural Hearing Loss
Neural hearing loss is a type of sensorineural hearing loss in which the problem lies in the auditory nerve or the neural pathways from the cochlea to the brain, rather than in the cochlea's hair cells. In cochlear hearing loss, the outer or inner hair cells are damaged. Neural hearing loss specifically involves damage to or dysfunction of the spiral ganglion neurons or higher auditory pathways.
One condition within this category is auditory neuropathy spectrum disorder (ANSD), in which the cochlear hair cells may function normally (shown by present otoacoustic emissions) but the auditory nerve does not transmit signals reliably. This results in poor speech understanding even when tones can be detected at normal or near-normal levels. Neural hearing loss can be difficult to manage with conventional hearing aids. Cochlear implants have shown benefit for some individuals with auditory neuropathy.
Diagnosis requires a combination of audiological tests including OAE, auditory brainstem response (ABR), and pure tone audiometry.
