Auditory neuropathy spectrum disorder

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Auditory neuropathy spectrum disorder
a)Normal signal intensity in bilateral centrum semiovale -left and periventricular/subcortical white matter- right b)normal anatomy- left ,small cochlea nerve Cn white arrow, normal size IAC Fn, superior Vsn and inferior Vin vestibular nerves green arrows-middle, Cn symmetrically small right c) No ragged red fibres d) ragged blue fibres e) targetoid fibres, f) nicotinamide adenine dinucleotide-tetrazolium reductase, g) no reduction of cytochrome-c-oxidase
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Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (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 in background noise and in others, no speech perception and therefore language development is possible.

The condition was originally termed auditory neuropathy (AN) [1] and in 2001 as Auditory Neuropathy / Auditory Dys-synchrony (AN/AD) [2] (to include those cases where no true neuropathy 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.[3]

See also


  1. Starr, A.; Picton, T.W.; Sininger, Y.; Hood, L.J.; Berlin, C.I. (1996). "Auditory neuropathy". Brain. 119: 741–753. doi:10.1093/brain/119.3.741. PMID 8673487.
  2. Berlin, C.I.; Hood, L.J.; Rose, K. (2001). "On renaming auditory neuropathy as auditory dys-synchrony" (PDF). Audiology Today. 13: 15–17. Archived from the original (PDF) on 2020-11-24. Retrieved 2020-12-17.
  3. Berlin, C.I.; Morlet, T.; Hood, L.J. (2008). "Management of Individuals With Auditory Neuropathy Spectrum Disorder". Archived from the original on 2016-03-05. Retrieved 2020-12-17. {{cite journal}}: Cite journal requires |journal= (help)

External links