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Abnormal cochlea morphology

MedGen UID:
871363
Concept ID:
C4025858
Anatomical Abnormality
Synonym: Abnormality of cochlea
 
HPO: HP:0000375

Definition

An abnormality of the cochlea. [from HPO]

Conditions with this feature

Hearing loss, autosomal dominant 75
MedGen UID:
1713569
Concept ID:
C5394059
Disease or Syndrome
DFNA75 is characterized by adult onset of moderate to severe, mid to high frequency hearing loss, progressing to involvement of all frequencies (Xia et al., 2019).

Professional guidelines

PubMed

Wang B, Dai WJ, Cheng XT, Liuyang WY, Yuan YS, Dai CF, Shu YL, Chen B
J Zhejiang Univ Sci B 2019 Feb.;20(2):156-163. doi: 10.1631/jzus.B1800224. PMID: 30666849Free PMC Article
Heman-Ackah SE, Roland JT Jr, Haynes DS, Waltzman SB
Otolaryngol Clin North Am 2012 Feb;45(1):41-67. doi: 10.1016/j.otc.2011.08.016. PMID: 22115681
Singleton GT
Otolaryngol Head Neck Surg 1986 Apr;94(4):426-9. doi: 10.1177/019459988609400403. PMID: 3086801

Recent clinical studies

Etiology

Mey K, Muhamad AA, Tranebjaerg L, Rendtorff ND, Rasmussen SH, Bille M, Cayé-Thomasen P
Laryngoscope 2019 Nov;129(11):2574-2579. doi: 10.1002/lary.27319. PMID: 31633822
Sennaroğlu L, Bajin MD
Balkan Med J 2017 Sep 29;34(5):397-411. Epub 2017 Aug 25 doi: 10.4274/balkanmedj.2017.0367. PMID: 28840850Free PMC Article
Liberman MC, Kujawa SG
Hear Res 2017 Jun;349:138-147. Epub 2017 Jan 10 doi: 10.1016/j.heares.2017.01.003. PMID: 28087419Free PMC Article
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Read AP, Newton VE
J Med Genet 1997 Aug;34(8):656-65. doi: 10.1136/jmg.34.8.656. PMID: 9279758Free PMC Article

Diagnosis

Legatt AD
J Clin Neurophysiol 2018 Jan;35(1):25-38. doi: 10.1097/WNP.0000000000000421. PMID: 29298210
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
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
Coelho DH, Roland JT Jr
Otolaryngol Clin North Am 2012 Feb;45(1):91-110. doi: 10.1016/j.otc.2011.08.019. PMID: 22115684
Maitland CG
Curr Neurol Neurosci Rep 2001 Sep;1(5):486-91. doi: 10.1007/s11910-001-0111-x. PMID: 11898560

Therapy

Verheij E, Elden L, Crowley TB, Pameijer FA, Zackai EH, McDonald-McGinn DM, Thomeer HGXM
AJNR Am J Neuroradiol 2018 May;39(5):928-934. Epub 2018 Mar 15 doi: 10.3174/ajnr.A5588. PMID: 29545254Free PMC Article
Liberman MC, Kujawa SG
Hear Res 2017 Jun;349:138-147. Epub 2017 Jan 10 doi: 10.1016/j.heares.2017.01.003. PMID: 28087419Free PMC Article
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Rance G
Trends Amplif 2005;9(1):1-43. doi: 10.1177/108471380500900102. PMID: 15920648Free PMC Article
Papsin BC
Laryngoscope 2005 Jan;115(1 Pt 2 Suppl 106):1-26. doi: 10.1097/00005537-200501001-00001. PMID: 15626926

Prognosis

da Costa Monsanto R, Knoll RM, de Oliveira Penido N, Song G, Santos F, Paparella MM, Cureoglu S
Otolaryngol Head Neck Surg 2022 Feb;166(2):363-372. Epub 2021 Apr 20 doi: 10.1177/01945998211008911. PMID: 33874787
Proctor RD, Gawne-Cain ML, Eyles J, Mitchell TE, Batty VB
Cochlear Implants Int 2013 Jan;14(1):2-6. doi: 10.1179/1754762811Y.0000000029. PMID: 23340090
Saihan Z, Webster AR, Luxon L, Bitner-Glindzicz M
Curr Opin Neurol 2009 Feb;22(1):19-27. doi: 10.1097/wco.0b013e3283218807. PMID: 19165952
Merchant SN, Durand ML, Adams JC
ORL J Otorhinolaryngol Relat Spec 2008;70(1):52-60; discussion 60-2. Epub 2008 Feb 1 doi: 10.1159/000111048. PMID: 18235206Free PMC Article
Read AP, Newton VE
J Med Genet 1997 Aug;34(8):656-65. doi: 10.1136/jmg.34.8.656. PMID: 9279758Free PMC Article

Clinical prediction guides

Sennaroğlu L, Bajin MD
Balkan Med J 2017 Sep 29;34(5):397-411. Epub 2017 Aug 25 doi: 10.4274/balkanmedj.2017.0367. PMID: 28840850Free PMC Article
Mathur P, Yang J
Biochim Biophys Acta 2015 Mar;1852(3):406-20. Epub 2014 Dec 4 doi: 10.1016/j.bbadis.2014.11.020. PMID: 25481835Free PMC Article
Van Abel KM, Carlson ML, Link MJ, Neff BA, Beatty CW, Lohse CM, Eckel LJ, Lane JI, Driscoll CL
Laryngoscope 2013 Aug;123(8):1957-66. Epub 2013 Jan 18 doi: 10.1002/lary.23928. PMID: 23335152
Merchant SN, Durand ML, Adams JC
ORL J Otorhinolaryngol Relat Spec 2008;70(1):52-60; discussion 60-2. Epub 2008 Feb 1 doi: 10.1159/000111048. PMID: 18235206Free PMC Article
Read AP, Newton VE
J Med Genet 1997 Aug;34(8):656-65. doi: 10.1136/jmg.34.8.656. PMID: 9279758Free PMC Article

Recent systematic reviews

Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG
Otolaryngol Head Neck Surg 2017 May;156(5):783-793. Epub 2017 Apr 4 doi: 10.1177/0194599817696502. PMID: 28374626
Kaplan AB, Kozin ED, Puram SV, Owoc MS, Shah PV, Hight AE, Sethi RKV, Remenschneider AK, Lee DJ
Int J Pediatr Otorhinolaryngol 2015 Mar;79(3):310-315. Epub 2014 Dec 15 doi: 10.1016/j.ijporl.2014.11.023. PMID: 25577282Free PMC Article
Van Abel KM, Carlson ML, Link MJ, Neff BA, Beatty CW, Lohse CM, Eckel LJ, Lane JI, Driscoll CL
Laryngoscope 2013 Aug;123(8):1957-66. Epub 2013 Jan 18 doi: 10.1002/lary.23928. PMID: 23335152
Pakdaman MN, Herrmann BS, Curtin HD, Van Beek-King J, Lee DJ
Otolaryngol Head Neck Surg 2012 Feb;146(2):180-90. Epub 2011 Dec 1 doi: 10.1177/0194599811429244. PMID: 22140206

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