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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

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
Hearing loss: diagnosis and management.
Lasak JM, Allen P, McVay T, Lewis D
Prim Care 2014 Mar;41(1):19-31. Epub 2013 Nov 18 doi: 10.1016/j.pop.2013.10.003. PMID: 24439878

Recent clinical studies

Etiology

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
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
Neuroanatomical and resting state EEG power correlates of central hearing loss in older adults.
Giroud N, Hirsiger S, Muri R, Kegel A, Dillier N, Meyer M
Brain Struct Funct 2018 Jan;223(1):145-163. Epub 2017 Jul 22 doi: 10.1007/s00429-017-1477-0. PMID: 28735495
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 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

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
Central deafness: a review of past and current perspectives.
Musiek FE, Chermak GD, Cone B
Int J Audiol 2019 Oct;58(10):605-617. Epub 2019 May 8 doi: 10.1080/14992027.2019.1606458. PMID: 31066317
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

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
Treatment of congenital cytomegalovirus infection: implications for future therapeutic strategies.
Nassetta L, Kimberlin D, Whitley R
J Antimicrob Chemother 2009 May;63(5):862-7. Epub 2009 Mar 14 doi: 10.1093/jac/dkp083. PMID: 19287011Free 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
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
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
Neuroanatomical and resting state EEG power correlates of central hearing loss in older adults.
Giroud N, Hirsiger S, Muri R, Kegel A, Dillier N, Meyer M
Brain Struct Funct 2018 Jan;223(1):145-163. Epub 2017 Jul 22 doi: 10.1007/s00429-017-1477-0. PMID: 28735495
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

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
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
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
Neuroanatomical and resting state EEG power correlates of central hearing loss in older adults.
Giroud N, Hirsiger S, Muri R, Kegel A, Dillier N, Meyer M
Brain Struct Funct 2018 Jan;223(1):145-163. Epub 2017 Jul 22 doi: 10.1007/s00429-017-1477-0. PMID: 28735495
Central presbycusis: a review and evaluation of the evidence.
Humes LE, Dubno JR, Gordon-Salant S, Lister JJ, Cacace AT, Cruickshanks KJ, Gates GA, Wilson RH, Wingfield A
J Am Acad Audiol 2012 Sep;23(8):635-66. doi: 10.3766/jaaa.23.8.5. PMID: 22967738Free PMC Article

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
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
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

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