title: Electrical stimulation of the auditory nerve via cochlear implants in patients with auditory neuropathy.
abstract: Auditory neuropathy (AN) is a term used to describe an auditory disorder in which there is evidence of normal outer hair cell function (otoacoustic emissions and/or cochlear microphonics) and poor function of the auditory nerve (absent or highly distorted auditory brain stem response starting with wave I). Many of these patients have evidence of generalized peripheral nerve disease, leading to an assumption that the peripheral portion of the auditory nerve is the most likely site of lesion. A small group of these patients has received cochlear implants, and the majority of them achieve average to above-average performance. Although this outcome may seem incongruous with neural disease, average performance by patients with AN may be a result of the reintroduction of neural synchrony by electrical stimulation and/or the fact that most deaf patients have poor nerve survival. Although cochlear implants are promising for deaf patients with AN, more study of the disorder is needed.
authors.affiliation: Children's Auditory Research and Evaluation Center, House Ear Institute, Los Angeles, California, USA.
authors.forename: Yvonne S
authors.initials: YS
authors.lastname: Sininger
authors.forename: Patricia
authors.initials: P
authors.lastname: Trautwein
mesh_terms: D000328:Adult; D000367:Age Factors; D002607:Charcot-Marie-Tooth Disease / Q000150:complications; D002648:Child; D003054:Cochlear Implants; D003056:Cochlear Nerve / Q000502:physiology; D004558:Electric Stimulation; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications; D015417:Hereditary Sensory and Motor Neuropathy / Q000150:complications; D006801:Humans; D000160:Vestibulocochlear Nerve Diseases / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*