U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Auditory neuropathy spectrum disorder

MedGen UID:
751969
•Concept ID:
C2732267
•
Disease or Syndrome
Synonyms: Auditory Neuropathy; Auditory Neuropathy Spectrum Disorder; Auditory neuropathy with dys-synchrony
SNOMED CT: Auditory neuropathy spectrum disorder (443805006); Auditory neuropathy with dys-synchrony (443805006)

Definition

A hearing disorder characterized by impaired transmission of signals through the auditory nerve, resulting in mild to severe hearing loss and poor speech perception. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Auditory neuropathy spectrum disorder

Professional guidelines

PubMed

Twelve years of neonatal hearing screening: audiological and etiological results.
Acke FRE, De Vriese C, Van Hoecke H, De Leenheer EMR
Eur Arch Otorhinolaryngol 2022 Jul;279(7):3371-3378. Epub 2021 Aug 31 doi: 10.1007/s00405-021-07060-5. PMID: 34463816
Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.
Mahomva C, Liu YC, Raol N, Anne S
Otolaryngol Head Neck Surg 2022 May;166(5):964-969. Epub 2021 Jul 27 doi: 10.1177/01945998211029836. PMID: 34311620
The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder.
Pham NS
Curr Opin Otolaryngol Head Neck Surg 2017 Oct;25(5):396-399. doi: 10.1097/MOO.0000000000000390. PMID: 28708635

Recent clinical studies

Etiology

Prevalence, risk factors, and audiological characteristics of auditory neuropathy.
Almishaal AA, Saleh S, Alferaih H, Alhelo O
Int J Audiol 2022 Dec;61(12):1018-1026. Epub 2021 Dec 20 doi: 10.1080/14992027.2021.2014074. PMID: 34928752
Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.
Mahomva C, Liu YC, Raol N, Anne S
Otolaryngol Head Neck Surg 2022 May;166(5):964-969. Epub 2021 Jul 27 doi: 10.1177/01945998211029836. PMID: 34311620
Does riboflavin depletion cause auditory neuropathy spectrum disorder in at risk neonates?
James AL, Chakraborty P, Tomlinson C, Harrison RV
Int J Pediatr Otorhinolaryngol 2020 Oct;137:110238. Epub 2020 Jul 2 doi: 10.1016/j.ijporl.2020.110238. PMID: 32896351
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Unilateral auditory neuropathy spectrum disorder.
Zhang QJ, Lan L, Shi W, Wang DY, Qi Y, Zong L, Li Q, Wang H, Ding HN, Li N, Han B, Wang QJ
Acta Otolaryngol 2012 Jan;132(1):72-9. Epub 2011 Nov 10 doi: 10.3109/00016489.2011.629630. PMID: 22073929

Diagnosis

Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.
Mahomva C, Liu YC, Raol N, Anne S
Otolaryngol Head Neck Surg 2022 May;166(5):964-969. Epub 2021 Jul 27 doi: 10.1177/01945998211029836. PMID: 34311620
A nonsense TMEM43 variant leads to disruption of connexin-linked function and autosomal dominant auditory neuropathy spectrum disorder.
Jang MW, Oh DY, Yi E, Liu X, Ling J, Kim N, Sharma K, Kim TY, Lee S, Kim AR, Kim MY, Kim MA, Lee M, Han JH, Han JJ, Park HR, Kim BJ, Lee SY, Woo DH, Oh J, Oh SJ, Du T, Koo JW, Oh SH, Shin HW, Seong MW, Lee KY, Kim UK, Shin JB, Sang S, Cai X, Mei L, He C, Blanton SH, Chen ZY, Chen H, Liu X, Nourbakhsh A, Huang Z, Kang KW, Park WY, Feng Y, Lee CJ, Choi BY
Proc Natl Acad Sci U S A 2021 Jun 1;118(22) doi: 10.1073/pnas.2019681118. PMID: 34050020Free PMC Article
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article
Auditory neuropathy spectrum disorder: a review.
Norrix LW, Velenovsky DS
J Speech Lang Hear Res 2014 Aug;57(4):1564-76. doi: 10.1044/2014_JSLHR-H-13-0213. PMID: 24686491

Therapy

Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.
Mahomva C, Liu YC, Raol N, Anne S
Otolaryngol Head Neck Surg 2022 May;166(5):964-969. Epub 2021 Jul 27 doi: 10.1177/01945998211029836. PMID: 34311620
Identification of Perinatal Risk Factors for Auditory Neuropathy Spectrum Disorder.
West AN, Kuan EC, Peng KA
Laryngoscope 2021 Mar;131(3):671-674. Epub 2020 Jul 1 doi: 10.1002/lary.28904. PMID: 32609896
Acquired auditory neuropathy spectrum disorder after malaria treated with quinine.
Brough H
Trop Doct 2020 Jul;50(3):246-248. Epub 2020 Apr 14 doi: 10.1177/0049475520917236. PMID: 32290759
Contemporary surgical issues in paediatric cochlear implantation.
Wasson JD, Briggs RJ
Int J Audiol 2016;55 Suppl 2:S77-87. Epub 2016 Jun 7 doi: 10.1080/14992027.2016.1184765. PMID: 27266370
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article

Prognosis

Identification of Perinatal Risk Factors for Auditory Neuropathy Spectrum Disorder.
West AN, Kuan EC, Peng KA
Laryngoscope 2021 Mar;131(3):671-674. Epub 2020 Jul 1 doi: 10.1002/lary.28904. PMID: 32609896
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article
Auditory neuropathy spectrum disorder: a review.
Norrix LW, Velenovsky DS
J Speech Lang Hear Res 2014 Aug;57(4):1564-76. doi: 10.1044/2014_JSLHR-H-13-0213. PMID: 24686491
Unilateral auditory neuropathy spectrum disorder.
Zhang QJ, Lan L, Shi W, Wang DY, Qi Y, Zong L, Li Q, Wang H, Ding HN, Li N, Han B, Wang QJ
Acta Otolaryngol 2012 Jan;132(1):72-9. Epub 2011 Nov 10 doi: 10.3109/00016489.2011.629630. PMID: 22073929

Clinical prediction guides

Cochlear implantation in children with auditory neuropathy spectrum disorder: an updated systematic review.
Sahwan M, Abdelsamad Y, Alasfoor F, Alfayez F, Binkhamis G, Nichani J
Eur Arch Otorhinolaryngol 2024 Mar;281(3):1149-1162. Epub 2023 Aug 28 doi: 10.1007/s00405-023-08194-4. PMID: 37638998
Identification of Perinatal Risk Factors for Auditory Neuropathy Spectrum Disorder.
West AN, Kuan EC, Peng KA
Laryngoscope 2021 Mar;131(3):671-674. Epub 2020 Jul 1 doi: 10.1002/lary.28904. PMID: 32609896
Clinical role of electrocochleography in children with auditory neuropathy spectrum disorder.
Fontenot TE, Giardina CK, Teagle HF, Park LR, Adunka OF, Buchman CA, Brown KD, Fitzpatrick DC
Int J Pediatr Otorhinolaryngol 2017 Aug;99:120-127. Epub 2017 Jun 5 doi: 10.1016/j.ijporl.2017.05.026. PMID: 28688553Free PMC Article
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder.
Sharma A, Cardon G
Hear Res 2015 Dec;330(Pt B):221-32. Epub 2015 Jun 10 doi: 10.1016/j.heares.2015.06.001. PMID: 26070426Free PMC Article
Auditory neuropathy spectrum disorder: a review.
Norrix LW, Velenovsky DS
J Speech Lang Hear Res 2014 Aug;57(4):1564-76. doi: 10.1044/2014_JSLHR-H-13-0213. PMID: 24686491

Recent systematic reviews

Cochlear implantation in children with auditory neuropathy spectrum disorder: an updated systematic review.
Sahwan M, Abdelsamad Y, Alasfoor F, Alfayez F, Binkhamis G, Nichani J
Eur Arch Otorhinolaryngol 2024 Mar;281(3):1149-1162. Epub 2023 Aug 28 doi: 10.1007/s00405-023-08194-4. PMID: 37638998
Is auditory neuropathy an appropriate term? A systematic literature review on its aetiology and pathogenesis.
Burdo S, Di Berardino F, Bruno G
Acta Otorhinolaryngol Ital 2021 Dec;41(6):496-506. Epub 2021 Nov 26 doi: 10.14639/0392-100X-N0932. PMID: 34825666Free PMC Article
Cochlear Implant Behavioral Outcomes for Children With Auditory Neuropathy Spectrum Disorder: A Mini-Systematic Review.
Myers K, Nicholson N
Am J Audiol 2021 Sep 10;30(3):777-789. Epub 2021 Jul 23 doi: 10.1044/2021_AJA-20-00175. PMID: 34297601
Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation.
Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL
Int J Pediatr Otorhinolaryngol 2015 Dec;79(12):1980-7. Epub 2015 Oct 17 doi: 10.1016/j.ijporl.2015.10.006. PMID: 26545793
Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature.
Roush P, Frymark T, Venediktov R, Wang B
Am J Audiol 2011 Dec;20(2):159-70. Epub 2011 Sep 22 doi: 10.1044/1059-0889(2011/10-0032). PMID: 21940978

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...