U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Central hearing loss

MedGen UID:
9162
•Concept ID:
C0018776
•
Disease or Syndrome
Synonyms: Central Hearing Loss; Hearing Loss, Central
SNOMED CT: Central hearing loss (68467004); Central deafness (68467004)
 
Monarch Initiative: MONDO:0020707

Definition

Hearing loss resulting from disorders of the central nervous system auditory pathways. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral hearing loss

Professional guidelines

PubMed

Newborn Screening.
Pappas KB
Pediatr Clin North Am 2023 Oct;70(5):1013-1027. doi: 10.1016/j.pcl.2023.06.003. PMID: 37704344
Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia.
Haffner D, Emma F, Eastwood DM, Duplan MB, Bacchetta J, Schnabel D, Wicart P, Bockenhauer D, Santos F, Levtchenko E, Harvengt P, Kirchhoff M, Di Rocco F, Chaussain C, Brandi ML, Savendahl L, Briot K, Kamenicky P, Rejnmark L, Linglart A
Nat Rev Nephrol 2019 Jul;15(7):435-455. doi: 10.1038/s41581-019-0152-5. PMID: 31068690Free PMC Article
Dizziness: Approach to Evaluation and Management.
Muncie HL, Sirmans SM, James E
Am Fam Physician 2017 Feb 1;95(3):154-162. PMID: 28145669

Recent clinical studies

Etiology

Clinical and genetic architecture of a large cohort with auditory neuropathy.
Wang H, Guan L, Wu X, Guan J, Li J, Li N, Wu K, Gao Y, Bing D, Zhang J, Lan L, Shi T, Li D, Wang W, Xie L, Xiong F, Shi W, Zhao L, Wang D, Yin Y, Wang Q
Hum Genet 2024 Mar;143(3):293-309. Epub 2024 Mar 8 doi: 10.1007/s00439-024-02652-7. PMID: 38456936Free PMC Article
Hearing screening in neonates with hyperbilirubinemia.
Ezzeldin ZM, Sharaf E, Hamdy HS, Abdelwahab Selim YA
Int J Pediatr Otorhinolaryngol 2021 Mar;142:110591. Epub 2020 Dec 23 doi: 10.1016/j.ijporl.2020.110591. PMID: 33360868
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
Cochlear implantation in unique pediatric populations.
Hang AX, Kim GG, Zdanski CJ
Curr Opin Otolaryngol Head Neck Surg 2012 Dec;20(6):507-17. doi: 10.1097/MOO.0b013e328359eea4. PMID: 23128686Free PMC Article

Diagnosis

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
Hearing and Ageing.
Guerrieri M, Di Mauro R, Di Girolamo S, Di Stadio A
Subcell Biochem 2023;103:279-290. doi: 10.1007/978-3-031-26576-1_12. PMID: 37120472
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 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

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
Association Between Central and Peripheral Age-Related Hearing Loss and Different Frailty Phenotypes in an Older Population in Southern Italy.
Sardone R, Castellana F, Bortone I, Lampignano L, Zupo R, Lozupone M, Griseta C, Dibello V, Seripa D, Guerra V, Donghia R, Logroscino G, Solfrizzi V, Quaranta N, Ferrucci L, Giannelli G, Panza F
JAMA Otolaryngol Head Neck Surg 2021 Jun 1;147(6):561-571. doi: 10.1001/jamaoto.2020.5334. PMID: 33570584Free PMC Article
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
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

Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
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
Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder.
de Carvalho GM, Ramos P, Arthur C, Guimarães A, Sartorato E
J Int Adv Otol 2016 Apr;12(1):8-15. doi: 10.5152/iao.2016.2232. PMID: 27340976
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 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

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
Evidence for independent peripheral and central age-related hearing impairment.
Bao J, Yu Y, Li H, Hawks J, Szatkowski G, Dade B, Wang H, Liu P, Brutnell T, Spehar B, Tye-Murray N
J Neurosci Res 2020 Sep;98(9):1800-1814. Epub 2020 May 16 doi: 10.1002/jnr.24639. PMID: 32415883
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

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
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
Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review.
Fernandes NF, Morettin M, Yamaguti EH, Costa OA, Bevilacqua MC
Braz J Otorhinolaryngol 2015 Jan-Feb;81(1):85-96. Epub 2014 Oct 22 doi: 10.1016/j.bjorl.2014.10.003. PMID: 25458263Free PMC Article
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

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

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • 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...