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Auditory neuropathy

MedGen UID:
338895
•Concept ID:
C1852271
•
Disease or Syndrome
Synonym: Auditory dys-synchrony
 
Monarch Initiative: MONDO:0021944
OMIM®: 561000
OMIM® Phenotypic series: PS609129

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 MONDO]

Professional guidelines

PubMed

Auditory neuropathy: from etiology to management.
Huang Y, Yang J, Duan M
Curr Opin Otolaryngol Head Neck Surg 2022 Oct 1;30(5):332-338. Epub 2022 Jul 18 doi: 10.1097/MOO.0000000000000829. PMID: 35939320
Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management.
Hood LJ
Otolaryngol Clin North Am 2015 Dec;48(6):1027-40. Epub 2015 Aug 18 doi: 10.1016/j.otc.2015.06.006. PMID: 26296649
Neonatal hearing screening: what we have achieved and what needs to be improved.
Nikolopoulos TP
Int J Pediatr Otorhinolaryngol 2015 May;79(5):635-7. Epub 2015 Feb 16 doi: 10.1016/j.ijporl.2015.02.010. PMID: 25758196

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
Auditory nerve disease and auditory neuropathy spectrum disorders.
Kaga K
Auris Nasus Larynx 2016 Feb;43(1):10-20. Epub 2015 Jul 22 doi: 10.1016/j.anl.2015.06.008. PMID: 26209259
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
Auditory dyssynchrony or auditory neuropathy: understanding the pathophysiology and exploring methods of treatment.
Nikolopoulos TP
Int J Pediatr Otorhinolaryngol 2014 Feb;78(2):171-3. Epub 2013 Dec 24 doi: 10.1016/j.ijporl.2013.12.021. PMID: 24380663
Familial auditory neuropathy.
Wang Q, Gu R, Han D, Yang W
Laryngoscope 2003 Sep;113(9):1623-9. doi: 10.1097/00005537-200309000-00041. PMID: 12972945

Diagnosis

Auditory Neuropathy/Auditory Synaptopathy.
Hood LJ
Otolaryngol Clin North Am 2021 Dec;54(6):1093-1100. Epub 2021 Sep 15 doi: 10.1016/j.otc.2021.07.004. PMID: 34535280
Auditory brainstem response.
Eggermont JJ
Handb Clin Neurol 2019;160:451-464. doi: 10.1016/B978-0-444-64032-1.00030-8. PMID: 31277868
Auditory neuropathy--neural and synaptic mechanisms.
Moser T, Starr A
Nat Rev Neurol 2016 Mar;12(3):135-49. Epub 2016 Feb 19 doi: 10.1038/nrneurol.2016.10. PMID: 26891769
Auditory neuropathy.
Starr A, Rance G
Handb Clin Neurol 2015;129:495-508. doi: 10.1016/B978-0-444-62630-1.00028-7. PMID: 25726287
Electrophysiologic auditory tests.
Legatt AD
Handb Clin Neurol 2015;129:289-311. doi: 10.1016/B978-0-444-62630-1.00017-2. PMID: 25726276

Therapy

Ocular Biomarkers of Riboflavin Transporter Deficiency.
Bulas S, Bedoukian EC, O'Neil EC, Krantz ID, Yum SW, Liu GT, Aleman TS
J Neuroophthalmol 2023 Mar 1;43(1):110-115. Epub 2022 Aug 2 doi: 10.1097/WNO.0000000000001678. PMID: 35921603
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
Auditory neuropathy/Auditory dyssynchrony in children with Cochlear Implants.
de Carvalho ACM, Bevilacqua MC, Sameshima K, Filho OAC
Braz J Otorhinolaryngol 2011 Jul-Aug;77(4):481-487. doi: 10.1590/S1808-86942011000400012. PMID: 21860975Free PMC Article
Auditory neuropathy/dys-synchrony and its perceptual consequences.
Rance G
Trends Amplif 2005;9(1):1-43. doi: 10.1177/108471380500900102. PMID: 15920648Free PMC Article

Prognosis

Auditory and verbal skills development post-cochlear implantation in Mandarin children with auditory neuropathy: a follow-up study.
Hu J, Zhou X, Guo Y, Liu Y, Li Y, Jin X, Zhou Y, Wang X, Zheng Z, Shi J, Liu P, Zheng J, Zhang J, Liu H
Acta Otolaryngol 2022 Feb;142(2):175-181. Epub 2022 Jan 27 doi: 10.1080/00016489.2022.2026465. PMID: 35085477
Hearing Loss and Failed Newborn Hearing Screen.
Dedhia K, Graham E, Park A
Clin Perinatol 2018 Dec;45(4):629-643. Epub 2018 Sep 24 doi: 10.1016/j.clp.2018.07.004. PMID: 30396409
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

Clinical prediction guides

Auditory neuropathy in mice and humans with Friedreich ataxia.
Rance G, Carew P, Winata L, Sale P, Delatycki M, Sly D
Ann Clin Transl Neurol 2023 Jun;10(6):953-963. Epub 2023 Apr 14 doi: 10.1002/acn3.51777. PMID: 37060174Free PMC Article
A comprehensive review of the effects of caffeine on the auditory and vestibular systems.
Ghahraman MA, Farahani S, Tavanai E
Nutr Neurosci 2022 Oct;25(10):2181-2194. Epub 2021 Apr 22 doi: 10.1080/1028415X.2021.1918984. PMID: 33888039
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
Familial auditory neuropathy.
Wang Q, Gu R, Han D, Yang W
Laryngoscope 2003 Sep;113(9):1623-9. doi: 10.1097/00005537-200309000-00041. PMID: 12972945

Recent systematic reviews

Mitochondrial Disease and Hearing Loss in Children: A Systematic Review.
Roesch S, O'Sullivan A, Zimmermann G, Mair A, Lipuš C, Mayr JA, Wortmann SB, Rasp G
Laryngoscope 2022 Dec;132(12):2459-2472. Epub 2022 Feb 21 doi: 10.1002/lary.30067. PMID: 35188226Free PMC Article
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
Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases.
Merkus P, Di Lella F, Di Trapani G, Pasanisi E, Beltrame MA, Zanetti D, Negri M, Sanna M
Eur Arch Otorhinolaryngol 2014 Jan;271(1):3-13. Epub 2013 Feb 13 doi: 10.1007/s00405-013-2378-3. PMID: 23404468

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