Other Names for this Disease
- Auditory dys-synchrony
Friedrich ataxia, Stevens-Johnson syndrome, Ehlers-Danlos syndrome, and Charcot-Marie-Tooth syndrome. The exact underlying cause of auditory neuropathy is not completely understood; however, researchers have proposed many possible explanations including damage to the hair cells of the inner ears, faulty connections between the hair cells and the auditory nerve (the nerve connecting the inner ear to the brain), damage to the auditory nerve, and/or a combination of these abnormalities. In many cases, auditory neuropathy occurs sporadically in people with no family history of the condition; however, the condition does run in some families suggesting that genetic factors may play a role in some cases. Treatment varies based on the severity of the condition but aims to improve hearing (i.e. hearing aids and cochlear implants) and communication skills.Auditory neuropathy is a rare condition that can affect a person's ability to hear. Although sounds enter the inner ear normally, signals from the inner ear to the brain are not transmitted properly. As a result, the condition may be associated with mild to severe hearing loss and poor speech-perception abilities (difficulty understanding speech clearly). Auditory neuropathy can be associated with other neurological disorders such as
Last updated: 12/28/2015
- Auditory Neuropathy. National Institute on Deafness and Other Communication Disorders. March 2003; http://www.nidcd.nih.gov/health/hearing/pages/neuropathy.aspx#g.
- Wayne T Shaia, MD. Auditory Neuropathy. Medscape Reference. November 2015; http://emedicine.medscape.com/article/836769-overview.
- Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL. Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. Int J Pediatr Otorhinolaryngol. October 2015; pii: S0165-5876(15)00513-3:[Epub ahead of print].
- The National Institute on Deafness and Other Communication Disorders (NIDCD) conducts and supports biomedical and behavioral research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. Click on the link to view information on this topic.
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- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
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