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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

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

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
Does riboflavin depletion cause auditory neuropathy spectrum disorder in at risk neonates?
James AL, Chakraborty P, Tomlinson C, Harrison RV
Int J Pediatr Otorhinolaryngol 2020 Oct;137:110238. Epub 2020 Jul 2 doi: 10.1016/j.ijporl.2020.110238. PMID: 32896351
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
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

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/dyssynchrony revisited.
Menten K, Van Eynde C, Desloovere C, Wouters J
B-ENT 2014;10(2):105-12. PMID: 25090808

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
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
The role of corticosteroids in infants and children with bacterial meningitis.
Bahal N, Nahata MC
DICP 1991 May;25(5):542-5. doi: 10.1177/106002809102500515. PMID: 2068839

Prognosis

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
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
Auditory neuropathies: understanding their pathogenesis to illuminate intervention strategies.
Giraudet F, Avan P
Curr Opin Neurol 2012 Feb;25(1):50-6. doi: 10.1097/WCO.0b013e32834f0351. PMID: 22185903

Clinical prediction guides

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
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
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
Auditory neuropathies: understanding their pathogenesis to illuminate intervention strategies.
Giraudet F, Avan P
Curr Opin Neurol 2012 Feb;25(1):50-6. doi: 10.1097/WCO.0b013e32834f0351. PMID: 22185903
Fetal alcohol syndrome. Hearing, speech, language, and vestibular disorders.
Church MW, Abel EL
Obstet Gynecol Clin North Am 1998 Mar;25(1):85-97. doi: 10.1016/s0889-8545(05)70359-4. PMID: 9547761

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
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
Does cochlear implantation improve speech recognition in children with auditory neuropathy spectrum disorder? A systematic review.
Humphriss R, Hall A, Maddocks J, Macleod J, Sawaya K, Midgley E
Int J Audiol 2013 Jul;52(7):442-54. Epub 2013 May 24 doi: 10.3109/14992027.2013.786190. PMID: 23705807

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