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Deafness

MedGen UID:
4155
•Concept ID:
C0011053
•
Finding; Finding
Synonym: Loss of hearing

Definition

An inherited or acquired condition characterized by the inability to hear in one or both ears. [from NCI]

Professional guidelines

PubMed

Alport Syndrome: Achieving Early Diagnosis and Treatment.
Kashtan CE
Am J Kidney Dis 2021 Feb;77(2):272-279. Epub 2020 Jul 22 doi: 10.1053/j.ajkd.2020.03.026. PMID: 32712016
Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss.
Herrera M, García Berrocal JR, García Arumí A, Lavilla MJ, Plaza G; Grupo de Trabajo de la Comisión de Audiología de la SEORL
Acta Otorrinolaringol Esp (Engl Ed) 2019 Sep-Oct;70(5):290-300. Epub 2018 Aug 6 doi: 10.1016/j.otorri.2018.04.010. PMID: 30093087
Wolfram Syndrome: Diagnosis, Management, and Treatment.
Urano F
Curr Diab Rep 2016 Jan;16(1):6. doi: 10.1007/s11892-015-0702-6. PMID: 26742931Free PMC Article

Recent clinical studies

Etiology

A Nationwide Study Examining Deafness Among Hospitalized Adults.
Harris CM, Kotwal S, Wright SM
Am J Audiol 2021 Jun 14;30(2):275-280. Epub 2021 Apr 6 doi: 10.1044/2021_AJA-20-00156. PMID: 33823115
Deafness and Vestibulopathy in Cerebellar Diseases: a Practical Approach.
Barsottini OG, Pedroso JL, Martins CR Jr, França MC Jr, Albernaz PM
Cerebellum 2019 Dec;18(6):1011-1016. doi: 10.1007/s12311-019-01042-4. PMID: 31154624
Inattentional deafness in music.
Koreimann S, Gula B, Vitouch O
Psychol Res 2014;78(3):304-12. Epub 2014 Mar 21 doi: 10.1007/s00426-014-0552-x. PMID: 24652341
Pedophilia and deafness.
Vernon M, Rich S
Am Ann Deaf 1997 Oct;142(4):300-11. doi: 10.1353/aad.2012.0258. PMID: 9351300
Nosology of deafness.
Jacobson JT
J Am Acad Audiol 1995 Jan;6(1):15-27. PMID: 7696675

Diagnosis

Molecular diagnosis of hereditary deafness and application of stepwise testing strategy.
Zeng BP, Xu HG, Mao L, Tang WX
Yi Chuan 2023 Jan 20;45(1):29-41. doi: 10.16288/j.yczz.22-206. PMID: 36927636
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
Genetics of hearing and deafness.
Angeli S, Lin X, Liu XZ
Anat Rec (Hoboken) 2012 Nov;295(11):1812-29. Epub 2012 Oct 8 doi: 10.1002/ar.22579. PMID: 23044516Free PMC Article
Deafness: diagnosis and management.
Tonkin JP
Med J Aust 1990 Jun 18;152(12):659-63. doi: 10.5694/j.1326-5377.1990.tb125426.x. PMID: 2198446
Word-deafness revisited.
Lancet 1986 Jun 28;1(8496):1477-8. PMID: 2873285

Therapy

AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial.
Lv J, Wang H, Cheng X, Chen Y, Wang D, Zhang L, Cao Q, Tang H, Hu S, Gao K, Xun M, Wang J, Wang Z, Zhu B, Cui C, Gao Z, Guo L, Yu S, Jiang L, Yin Y, Zhang J, Chen B, Wang W, Chai R, Chen ZY, Li H, Shu Y
Lancet 2024 May 25;403(10441):2317-2325. Epub 2024 Jan 24 doi: 10.1016/S0140-6736(23)02874-X. PMID: 38280389
AAV-Mediated Gene Therapy Restores Hearing in Patients with DFNB9 Deafness.
Qi J, Tan F, Zhang L, Lu L, Zhang S, Zhai Y, Lu Y, Qian X, Dong W, Zhou Y, Zhang Z, Yang X, Jiang L, Yu C, Liu J, Chen T, Wu L, Tan C, Sun S, Song H, Shu Y, Xu L, Gao X, Li H, Chai R
Adv Sci (Weinh) 2024 Mar;11(11):e2306788. Epub 2024 Jan 8 doi: 10.1002/advs.202306788. PMID: 38189623Free PMC Article
Tympanostomy Tubes or Medical Management for Recurrent Acute Otitis Media.
Hoberman A, Preciado D, Paradise JL, Chi DH, Haralam M, Block SL, Kearney DH, Bhatnagar S, Muñiz Pujalt GB, Shope TR, Martin JM, Felten DE, Kurs-Lasky M, Liu H, Yahner K, Jeong JH, Cohen NL, Czervionke B, Nagg JP, Dohar JE, Shaikh N
N Engl J Med 2021 May 13;384(19):1789-1799. doi: 10.1056/NEJMoa2027278. PMID: 33979487Free PMC Article
Gene therapy for hearing loss.
Omichi R, Shibata SB, Morton CC, Smith RJH
Hum Mol Genet 2019 Oct 1;28(R1):R65-R79. doi: 10.1093/hmg/ddz129. PMID: 31227837Free PMC Article
Saline irrigation for allergic rhinitis.
Head K, Snidvongs K, Glew S, Scadding G, Schilder AG, Philpott C, Hopkins C
Cochrane Database Syst Rev 2018 Jun 22;6(6):CD012597. doi: 10.1002/14651858.CD012597.pub2. PMID: 29932206Free PMC Article

Prognosis

Effects of delayed versus immediate umbilical cord clamping in reducing death or major disability at 2 years corrected age among very preterm infants (APTS): a multicentre, randomised clinical trial.
Robledo KP, Tarnow-Mordi WO, Rieger I, Suresh P, Martin A, Yeung C, Ghadge A, Liley HG, Osborn D, Morris J, Hague W, Kluckow M, Lui K, Soll R, Cruz M, Keech A, Kirby A, Simes J; APTS Childhood Follow-up Study collaborators
Lancet Child Adolesc Health 2022 Mar;6(3):150-157. Epub 2021 Dec 8 doi: 10.1016/S2352-4642(21)00373-4. PMID: 34895510
A Nationwide Study Examining Deafness Among Hospitalized Adults.
Harris CM, Kotwal S, Wright SM
Am J Audiol 2021 Jun 14;30(2):275-280. Epub 2021 Apr 6 doi: 10.1044/2021_AJA-20-00156. PMID: 33823115
Association Between Oxygen Saturation Targeting and Death or Disability in Extremely Preterm Infants in the Neonatal Oxygenation Prospective Meta-analysis Collaboration.
Askie LM, Darlow BA, Finer N, Schmidt B, Stenson B, Tarnow-Mordi W, Davis PG, Carlo WA, Brocklehurst P, Davies LC, Das A, Rich W, Gantz MG, Roberts RS, Whyte RK, Costantini L, Poets C, Asztalos E, Battin M, Halliday HL, Marlow N, Tin W, King A, Juszczak E, Morley CJ, Doyle LW, Gebski V, Hunter KE, Simes RJ; Neonatal Oxygenation Prospective Meta-analysis (NeOProM) Collaboration
JAMA 2018 Jun 5;319(21):2190-2201. doi: 10.1001/jama.2018.5725. PMID: 29872859Free PMC Article
Linking Deafness and Dementia: Challenges and Opportunities.
Lin VYW, Black SE
Otol Neurotol 2017 Sep;38(8):e237-e239. doi: 10.1097/MAO.0000000000001408. PMID: 28806332
The Genetic Basis of Nonsyndromic Hearing Loss in Indian and Pakistani Populations.
Yan D, Kannan-Sundhari A, Vishwanath S, Qing J, Mittal R, Kameswaran M, Liu XZ
Genet Test Mol Biomarkers 2015 Sep;19(9):512-27. Epub 2015 Jul 17 doi: 10.1089/gtmb.2015.0023. PMID: 26186295Free PMC Article

Clinical prediction guides

Development and Validation of the Spanish AzBio Sentence Corpus.
Rivas A, Perkins E, Rivas A, Rincon LA, Litvak L, Spahr T, Dorman M, Kessler D, Gifford R
Otol Neurotol 2021 Jan;42(1):154-158. doi: 10.1097/MAO.0000000000002970. PMID: 33885261
Cochlear implantation in patients with ossified cochleas.
Yan T, Zong F, Ma X, Xu X, Chen W, Song Z, Han X, Wang X, Zhang H
Am J Otolaryngol 2019 Mar-Apr;40(2):183-186. Epub 2018 Dec 8 doi: 10.1016/j.amjoto.2018.12.004. PMID: 30551897
Alport syndrome: a unified classification of genetic disorders of collagen IV α345: a position paper of the Alport Syndrome Classification Working Group.
Kashtan CE, Ding J, Garosi G, Heidet L, Massella L, Nakanishi K, Nozu K, Renieri A, Rheault M, Wang F, Gross O
Kidney Int 2018 May;93(5):1045-1051. Epub 2018 Mar 16 doi: 10.1016/j.kint.2017.12.018. PMID: 29551517
Clinical trials, ototoxicity grading scales and the audiologist's role in therapeutic decision making.
King KA, Brewer CC
Int J Audiol 2018 Sep;57(sup4):S89-S98. Epub 2017 Dec 23 doi: 10.1080/14992027.2017.1417644. PMID: 29276851Free PMC Article
Pathogenesis and prenatal diagnosis of human cytomegalovirus infection.
Revello MG, Gerna G
J Clin Virol 2004 Feb;29(2):71-83. doi: 10.1016/j.jcv.2003.09.012. PMID: 14747024

Recent systematic reviews

Betahistine in Ménière's Disease or Syndrome: A Systematic Review.
Van Esch B, van der Zaag-Loonen H, Bruintjes T, van Benthem PP
Audiol Neurootol 2022;27(1):1-33. Epub 2021 Jul 7 doi: 10.1159/000515821. PMID: 34233329
Cochlear Implantation in Children With Single-Sided Deafness: A Systematic Review and Meta-analysis.
Benchetrit L, Ronner EA, Anne S, Cohen MS
JAMA Otolaryngol Head Neck Surg 2021 Jan 1;147(1):58-69. doi: 10.1001/jamaoto.2020.3852. PMID: 33151295Free PMC Article
Drug-associated adverse events in the treatment of multidrug-resistant tuberculosis: an individual patient data meta-analysis.
Lan Z, Ahmad N, Baghaei P, Barkane L, Benedetti A, Brode SK, Brust JCM, Campbell JR, Chang VWL, Falzon D, Guglielmetti L, Isaakidis P, Kempker RR, Kipiani M, Kuksa L, Lange C, Laniado-Laborín R, Nahid P, Rodrigues D, Singla R, Udwadia ZF, Menzies D; Collaborative Group for the Meta-Analysis of Individual Patient Data in MDR-TB treatment 2017
Lancet Respir Med 2020 Apr;8(4):383-394. Epub 2020 Mar 17 doi: 10.1016/S2213-2600(20)30047-3. PMID: 32192585Free PMC Article
Topical antibiotics for chronic suppurative otitis media.
Brennan-Jones CG, Head K, Chong LY, Burton MJ, Schilder AG, Bhutta MF
Cochrane Database Syst Rev 2020 Jan 2;1(1):CD013051. doi: 10.1002/14651858.CD013051.pub2. PMID: 31896168Free PMC Article
Saline irrigation for allergic rhinitis.
Head K, Snidvongs K, Glew S, Scadding G, Schilder AG, Philpott C, Hopkins C
Cochrane Database Syst Rev 2018 Jun 22;6(6):CD012597. doi: 10.1002/14651858.CD012597.pub2. PMID: 29932206Free PMC Article

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