Auditory neuropathy spectrum disorder (ANSD) is a specific form of
hearing loss
Hearing loss is a partial or total inability to Hearing, hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to Language ...
defined by the presence of normal or near-normal
otoacoustic emission
An otoacoustic emission (OAE) is a sound that is generated from within the inner ear. Having been predicted by Austrian astrophysicist Thomas Gold in 1948, its existence was first demonstrated experimentally by British physicist David Kemp in 197 ...
s (OAEs) but the absence of normal
middle ear reflexes and severely abnormal or completely absent
auditory brainstem response
The auditory brainstem response (ABR), also called brainstem evoked response audiometry (BERA), is an auditory evoked potential extracted from ongoing electrical activity in the brain and recorded via electrodes placed on the scalp. The measured r ...
(ABRs).
Individuals presenting with this recently recognised hearing loss appear to display sporadic windows of hearing and not. Very few (1 in 14) will go on to develop normal speech and language but with poor
speech perception
Speech perception is the process by which the sounds of language
Language is a structured system of communication. The structure of a language is its grammar and the free components are its vocabulary. Languages are the primary means by wh ...
in background noise and in others, no speech perception and therefore language development is possible.
The condition was originally termed
auditory neuropathy Auditory neuropathy (AN) is a hearing disorder in which the outer hair cells of the cochlea are present and functional, but sound information is not transmitted sufficiently by the auditory nerve to the brain. Hearing loss with AN can range from nor ...
(AN) and in 2001 as Auditory Neuropathy / Auditory Dys-synchrony (AN/AD)
(to include those cases where no true
neuropathy
Peripheral neuropathy, often shortened to neuropathy, is a general term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord. Damage to peripheral nerves may impair sensation, movement, gland, or o ...
was apparent). In 2008 at a meeting convened at Lake Como in Italy (Guidelines Development
Conference on the Identification and Management
of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy,
June 19–21, 2008), a group of leading authorities on the condition reached a consensus and renamed it as auditory neuropathy spectrum disorder.
See also
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Audiologist
Audiology (from Latin , "to hear"; and from Greek , ''-logia'') is a branch of science that studies hearing, balance, and related disorders. Audiologists treat those with hearing loss and proactively prevent related damage. By employing vario ...
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Auditory brainstem response
The auditory brainstem response (ABR), also called brainstem evoked response audiometry (BERA), is an auditory evoked potential extracted from ongoing electrical activity in the brain and recorded via electrodes placed on the scalp. The measured r ...
*
Auditory neuropathy Auditory neuropathy (AN) is a hearing disorder in which the outer hair cells of the cochlea are present and functional, but sound information is not transmitted sufficiently by the auditory nerve to the brain. Hearing loss with AN can range from nor ...
*
Auditory processing disorder
Auditory processing disorder (APD), rarely known as King-Kopetzky syndrome or auditory disability with normal hearing (ADN), is a neurodevelopmental disorder affecting the way the brain processes auditory information. Individuals with APD usually ...
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Cochlear implant
A cochlear implant (CI) is a surgically implanted neuroprosthesis that provides a person who has moderate-to-profound sensorineural hearing loss with sound perception. With the help of therapy, cochlear implants may allow for improved speech und ...
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Hearing aids
A hearing aid is a device designed to improve hearing by making sound audible to a person with hearing loss. Hearing aids are classified as medical devices in most countries, and regulated by the respective regulations. Small audio amplifiers su ...
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Otoacoustic emissions
An otoacoustic emission (OAE) is a sound that is generated from within the inner ear. Having been predicted by Austrian astrophysicist Thomas Gold in 1948, its existence was first demonstrated experimentally by British physicist David Kemp (physici ...
*
Sensorineural hearing impairment
Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the vestibulocochlear nerve (cranial nerve VIII). SNHL accounts for about 90% of rep ...
References
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External links
Alice's Story - A Blog Dedicated to a Beautiful Little Girl Who Has Ears That Need New Batteries
Hearing
Audiology