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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

Epidemiology, aetiology and diagnosis of congenital hearing loss via hearing screening of 153 913 newborns.
Yoshimura H, Okubo T, Shinagawa J, Nishio SY, Takumi Y, Usami SI
Int J Epidemiol 2024 Apr 11;53(3) doi: 10.1093/ije/dyae052. PMID: 38609324Free PMC Article
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

Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.
Ramanathan D, Mahomva C, Goldberg D, Liu YC, Anne S, Lyle W
Am J Otolaryngol 2023 Mar-Apr;44(2):103753. Epub 2022 Dec 20 doi: 10.1016/j.amjoto.2022.103753. PMID: 36577168
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
Longitudinal outcomes of early implantation in children with auditory neuropathy spectrum disorder (ANSD).
Chisholm K, Gibbons A, Psarros C, Bate K, Gardner-Berry K
Cochlear Implants Int 2010 Jun;11 Suppl 1:169-75. doi: 10.1179/146701010X12671177818588. PMID: 21756606

Diagnosis

Preparing for Otoferlin gene therapy trials: A survey of NHS Paediatric Audiology and Cochlear Implant services on diagnosis and management of Auditory Neuropathy Spectrum Disorder.
Spyridakou C, Chan J, Tan J, Clement E, Nash R, Hall A, Mehta N, Schilder AG
Int J Pediatr Otorhinolaryngol 2024 Feb;177:111870. Epub 2024 Jan 27 doi: 10.1016/j.ijporl.2024.111870. PMID: 38290274
Auditory Neuropathy: Bridging the Gap Between Hearing Aids and Cochlear Implants.
Yawn RJ, Nassiri AM, Rivas A
Otolaryngol Clin North Am 2019 Apr;52(2):349-355. Epub 2019 Feb 12 doi: 10.1016/j.otc.2018.11.016. PMID: 30765091
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

Preparing for Otoferlin gene therapy trials: A survey of NHS Paediatric Audiology and Cochlear Implant services on diagnosis and management of Auditory Neuropathy Spectrum Disorder.
Spyridakou C, Chan J, Tan J, Clement E, Nash R, Hall A, Mehta N, Schilder AG
Int J Pediatr Otorhinolaryngol 2024 Feb;177:111870. Epub 2024 Jan 27 doi: 10.1016/j.ijporl.2024.111870. PMID: 38290274
Predicting the Impact of OTOF Gene Missense Variants on Auditory Neuropathy Spectrum Disorder.
Dmitriev DA, Shilov BV, Polunin MM, Zadorozhny AD, Lagunin AA
Int J Mol Sci 2023 Dec 7;24(24) doi: 10.3390/ijms242417240. PMID: 38139069Free PMC Article
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
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
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

Targeted next generation sequencing reveals OTOF mutations in auditory neuropathy spectrum disorder.
Chen K, Liu M, Wu X, Zong L, Jiang H
Int J Pediatr Otorhinolaryngol 2018 Dec;115:19-23. Epub 2018 Sep 14 doi: 10.1016/j.ijporl.2018.09.008. PMID: 30368385
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
Longitudinal outcomes of early implantation in children with auditory neuropathy spectrum disorder (ANSD).
Chisholm K, Gibbons A, Psarros C, Bate K, Gardner-Berry K
Cochlear Implants Int 2010 Jun;11 Suppl 1:169-75. doi: 10.1179/146701010X12671177818588. PMID: 21756606

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
Predicting the Impact of OTOF Gene Missense Variants on Auditory Neuropathy Spectrum Disorder.
Dmitriev DA, Shilov BV, Polunin MM, Zadorozhny AD, Lagunin AA
Int J Mol Sci 2023 Dec 7;24(24) doi: 10.3390/ijms242417240. PMID: 38139069Free 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
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

Auditory and Speech Outcomes of Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Systematic Review and Meta-Analysis.
Bo D, Huang Y, Wang B, Lu P, Chen WX, Xu ZM
Ann Otol Rhinol Laryngol 2023 Apr;132(4):371-380. Epub 2022 May 2 doi: 10.1177/00034894221092201. PMID: 35499129
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

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