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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
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

New trends in the prevention of occupational noise-induced hearing loss.
Sliwinska-Kowalska M
Int J Occup Med Environ Health 2020 Oct 20;33(6):841-848. Epub 2020 Sep 29 doi: 10.13075/ijomeh.1896.01600. PMID: 32994587
Long-term Follow-up of a Patient With Auditory Neuropathy and Normal Hearing Thresholds.
White-Schwoch T, Anderson S, Kraus N
JAMA Otolaryngol Head Neck Surg 2020 May 1;146(5):499-501. doi: 10.1001/jamaoto.2019.4314. PMID: 32163113
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
International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient.
Liming BJ, Carter J, Cheng A, Choo D, Curotta J, Carvalho D, Germiller JA, Hone S, Kenna MA, Loundon N, Preciado D, Schilder A, Reilly BJ, Roman S, Strychowsky J, Triglia JM, Young N, Smith RJ
Int J Pediatr Otorhinolaryngol 2016 Nov;90:251-258. Epub 2016 Sep 15 doi: 10.1016/j.ijporl.2016.09.016. PMID: 27729144
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

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
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--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

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
New trends in the prevention of occupational noise-induced hearing loss.
Sliwinska-Kowalska M
Int J Occup Med Environ Health 2020 Oct 20;33(6):841-848. Epub 2020 Sep 29 doi: 10.13075/ijomeh.1896.01600. PMID: 32994587
A juvenile ALS-like phenotype dramatically improved after high-dose riboflavin treatment.
Carreau C, Lenglet T, Mosnier I, Lahlou G, Fargeot G, Weiss N, Demeret S, Salachas F, Veauville-Merllié A, Acquaviva C, Nadjar Y
Ann Clin Transl Neurol 2020 Feb;7(2):250-253. Epub 2020 Feb 5 doi: 10.1002/acn3.50977. PMID: 32022482Free 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
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

Prognosis

Cochlear Implant Outcomes in Patients with Neurosarcoidosis.
Ebode D, Cohen-Aubart F, Trunet S, Ferrary E, Lahlou G, Mosnier I
Audiol Neurootol 2021;26(6):454-460. Epub 2021 Apr 6 doi: 10.1159/000514479. PMID: 33823505
New trends in the prevention of occupational noise-induced hearing loss.
Sliwinska-Kowalska M
Int J Occup Med Environ Health 2020 Oct 20;33(6):841-848. Epub 2020 Sep 29 doi: 10.13075/ijomeh.1896.01600. PMID: 32994587
Long-term Follow-up of a Patient With Auditory Neuropathy and Normal Hearing Thresholds.
White-Schwoch T, Anderson S, Kraus N
JAMA Otolaryngol Head Neck Surg 2020 May 1;146(5):499-501. doi: 10.1001/jamaoto.2019.4314. PMID: 32163113
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
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

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
Cochlear implantation in patient with Charcot-Marie-Tooth disease.
Kobayashi M, Yoshida T, Sugimoto S, Teranishi M, Hara D, Kimata Y, Sone M
Auris Nasus Larynx 2021 Apr;48(2):327-330. Epub 2020 Apr 5 doi: 10.1016/j.anl.2020.03.003. PMID: 32265048
New trends in the prevention of occupational noise-induced hearing loss.
Sliwinska-Kowalska M
Int J Occup Med Environ Health 2020 Oct 20;33(6):841-848. Epub 2020 Sep 29 doi: 10.13075/ijomeh.1896.01600. PMID: 32994587
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
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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