Auditory neuropathy spectrum disorder (ANSD) is a specific form of

hearing loss Hearing loss is a partial or total inability to 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 acquire spo ...

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 19 ...

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) or brainstem auditory evoked potentials (BAEPs) or brainstem auditory evoked responses (BAERs) is an auditory evoked potential extracted from ongoing ...

(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 are heard, interpreted, and understood. The study of speech perception is closely linked to the fields of phonology and phonetics in linguistics and cognitive psychology and percept ...

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. The cause may be several dysfunctions ...

(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, refers to damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerve fibers are affected. Neuropa ...

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.

References

External links

Alice's Story - A Blog Dedicated to a Beautiful Little Girl Who Has Ears That Need New Batteries
Hearing Audiology {{neuroscience-stub

See also

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External links