Auditory neuropathy

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Auditory neuropathy (AN) is a variety of hearing loss in which the outer hair cells within the cochlea are present and functional, but sound information is not faithfully transmitted to the auditory nerve and brain properly. Also known as Auditory Neuropathy/Auditory Dys-synchrony (AN/AD) or Auditory Neuropathy Spectrum Disorder (ANSD).

A neuropathy usually refers to a disease of the peripheral nerve or nerves, but the auditory nerve itself is not always affected in Auditory Neuropathy Spectrum Disorders.[1]

Possible Sites of Lesion[edit]

Neuron: Hair cell
Gray931.png
Three rows of Outer Hair Cells in the Organ of Corti, one row of Inner Hair Cells.
Gray's p.1057

Based on clinical testing of subjects with auditory neuropathy, the disruption in the stream of sound information has been localized to one or more of three probable locations: the inner hair cells of the cochlea, the synapse between the inner hair cells and the auditory nerve, or a lesion of the ascending auditory nerve itself.[2]

Diagnosing Auditory Neuropathy[edit]

Diagnosis is possible after a test battery, that must necessarily include the following: the auditory brainstem response and otoacoustic emissions. Auditory brainstem response should be tested with both polarities (helps in identifying Cochlear Microphonics).[3]

Auditory neuropathy is diagnosed when a person has present Otoacoustic Emissions and/or Cochlear Microphonics in combination with absent or abnormal Auditory Brainstem Response.[4] Patients with Auditory Neuropathy Spectrum Disorders have to date never been shown to have normal middle ear muscle reflexes at 95 decibel dB or less despite having normal otoacoustic emissions.[5]

Auditory Neuropathy can occur spontaneously, or in combination with diseases like Charcot-Marie-Tooth disease and Friedreich's ataxia.

It appears that regardless of the audiometric pattern(hearing thresholds) or of their function on traditional speech testing in quiet the vast majority of sufferers have very poor hearing in background noise situations.[6]

Residual Auditory Function[edit]

When testing the auditory system, there really is no characteristic presentation on the audiogram.

When diagnosing someone with auditory neuropathy, there is no characteristic level of functioning either. People can present relatively little dysfunction other than problems of hearing speech in noise, or can present as completely deaf and gaining no useful information from auditory signals.

Hearing aids are sometimes prescribed, with mixed success.[7]

Some people with auditory neuropathy obtain cochlear implants, also with mixed success.[7][8]

Screening[edit]

Universal Newborn Hearing Screenings (UNHS) is mandated in a majority of the United States. Auditory neuropathy is sometimes difficult to catch right away, even with these precautions in place. Parental suspicion of a hearing loss is a trustworthy screening tool for hearing loss, too; if it is suspected, that is sufficient reason to seek a hearing evaluation from an audiologist.[1]

In most parts of Australia, hearing screening via AABR testing is mandated, meaning that essentially all congenital (i.e., not those related to later onset degenerative disorders) auditory neuropathy cases should be diagnosed at birth.

See also[edit]

References[edit]

  1. ^ Amatuzzi M, Liberman MC, Northrop C (October 2011). "Selective inner hair cell loss in prematurity: a temporal bone study of infants from a neonatal intensive care unit". J. Assoc. Res. Otolaryngol. 12 (5): 595–604. doi:10.1007/s10162-011-0273-4. PMC 3173554. PMID 21674215. 
  2. ^ Starr, A.; Picton, T.W.; Sininger, Y.; Hood, L.J.; Berlin, C.I. (1996). "Auditory Neuropathy". Brain. 119 (3): 741–753. doi:10.1093/brain/119.3.741. PMID 8673487. 
  3. ^ Roush, P. (2008). "Auditory Neuropathy Spectrum Disorder, Evaluation and Management.". The Hearing Journal 61 (11): 36–41. 
  4. ^ Vlastarakos PV, Nikolopoulos TP, Tavoulari E, Papacharalambous G, Korres S (August 2008). "Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management". Int. J. Pediatr. Otorhinolaryngol. 72 (8): 1135–50. doi:10.1016/j.ijporl.2008.04.004. PMID 18502518. 
  5. ^ Berlin CI, Hood LJ, Morlet T, et al. (September 2005). "Absent or elevated middle ear muscle reflexes in the presence of normal otoacoustic emissions: a universal finding in 136 cases of auditory neuropathy/dys-synchrony". J Am Acad Audiol 16 (8): 546–53. doi:10.3766/jaaa.16.8.3. PMID 16295241. 
  6. ^ Zeng FG, Liu S (April 2006). "Speech perception in individuals with auditory neuropathy". J. Speech Lang. Hear. Res. 49 (2): 367–80. doi:10.1044/1092-4388(2006/029). PMID 16671850. 
  7. ^ a b Roush P, Frymark T, Venediktov R, Wang B (December 2011). "Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature". Am J Audiol 20 (2): 159–70. doi:10.1044/1059-0889(2011/10-0032). PMID 21940978. 
  8. ^ Carvalho AC, Bevilacqua MC, Sameshima K, Costa Filho OA (2011). "Auditory neuropathy/Auditory dyssynchrony in children with Cochlear Implants". Braz J Otorhinolaryngol 77 (4): 481–7. doi:10.1590/S1808-86942011000400012. PMID 21860975. 

Further reading[edit]

  • Manchaiah VK, Zhao F, Danesh AA, Duprey R (February 2011). "The genetic basis of auditory neuropathy spectrum disorder (ANSD)". Int. J. Pediatr. Otorhinolaryngol. 75 (2): 151–8. doi:10.1016/j.ijporl.2010.11.023. PMID 21176974. 
  • Vlastarakos PV, Nikolopoulos TP, Tavoulari E, Papacharalambous G, Korres S (August 2008). "Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management". Int. J. Pediatr. Otorhinolaryngol. 72 (8): 1135–50. doi:10.1016/j.ijporl.2008.04.004. PMID 18502518. 

External links[edit]