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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

The natural history, clinical outcomes, and genotype-phenotype relationship of otoferlin-related hearing loss: a systematic, quantitative literature review.
Ford CL, Riggs WJ, Quigley T, Keifer OP Jr, Whitton JP, Valayannopoulos V
Hum Genet 2023 Oct;142(10):1429-1449. Epub 2023 Sep 7 doi: 10.1007/s00439-023-02595-5. PMID: 37679651Free PMC Article
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

Recent clinical studies

Etiology

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
Type 2 Diabetes and Hearing Impairment.
Helzner EP, Contrera KJ
Curr Diab Rep 2016 Jan;16(1):3. doi: 10.1007/s11892-015-0696-0. PMID: 26699766
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

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/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
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

Therapy

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
Trigonelline: a plant alkaloid with therapeutic potential for diabetes and central nervous system disease.
Zhou J, Chan L, Zhou S
Curr Med Chem 2012;19(21):3523-31. doi: 10.2174/092986712801323171. PMID: 22680628
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

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
Auditory neuropathy/dys-synchrony: diagnosis and management.
Berlin CI, Hood L, Morlet T, Rose K, Brashears S
Ment Retard Dev Disabil Res Rev 2003;9(4):225-31. doi: 10.1002/mrdd.10084. PMID: 14648814

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
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
Genetics of isolated auditory neuropathies.
Del Castillo FJ, Del Castillo I
Front Biosci (Landmark Ed) 2012 Jan 1;17(4):1251-65. doi: 10.2741/3984. PMID: 22201801
The mitochondrial connection in auditory neuropathy.
Cacace AT, Pinheiro JM
Audiol Neurootol 2011;16(6):398-413. Epub 2011 Jan 22 doi: 10.1159/000323276. PMID: 21266802

Recent systematic reviews

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
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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