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Atresia of external auditory canal and conductive deafness

MedGen UID:
502760
Concept ID:
C3502361
Disease or Syndrome
Synonym: Atresia of External Auditory Canal and Conduction Deafness
 
Monarch Initiative: MONDO:0007170
OMIM®: 108760

Clinical features

From HPO
Conductive hearing impairment
MedGen UID:
9163
Concept ID:
C0018777
Disease or Syndrome
An abnormality of vibrational conductance of sound to the inner ear leading to impairment of sensory perception of sound.
Atresia of the external auditory canal
MedGen UID:
78613
Concept ID:
C0266597
Congenital Abnormality
Absence or failure to form of the external auditory canal.
Stenosis of the external auditory canal
MedGen UID:
140758
Concept ID:
C0395837
Finding
An abnormal narrowing of the external auditory canal.
Aplasia/Hypoplasia of the middle ear
MedGen UID:
867171
Concept ID:
C4021529
Finding
Aplasia or developmental hypoplasia of all or part of the middle ear.
Delayed pneumatization of the mastoid process
MedGen UID:
867257
Concept ID:
C4021617
Finding
An abnormally reduced degree of pneumatization (i.e., formation of air cells) in the mastoid process with respect to age-dependent norms.

Professional guidelines

PubMed

Tsai Do BS, Bush ML, Weinreich HM, Schwartz SR, Anne S, Adunka OF, Bender K, Bold KM, Brenner MJ, Hashmi AZ, Keenan TA, Kim AH, Moore DJ, Nieman CL, Palmer CV, Ross EJ, Steenerson KK, Zhan KY, Reyes J, Dhepyasuwan N
Otolaryngol Head Neck Surg 2024 May;170 Suppl 2:S1-S54. doi: 10.1002/ohn.750. PMID: 38687845
Michels TC, Duffy MT, Rogers DJ
Am Fam Physician 2019 Jul 15;100(2):98-108. PMID: 31305044
Dougherty W, Kesser BW
Otolaryngol Clin North Am 2015 Dec;48(6):955-74. Epub 2015 Sep 8 doi: 10.1016/j.otc.2015.06.007. PMID: 26360369

Recent clinical studies

Etiology

Rooijers W, Tio PAE, van der Schroeff MP, Padwa BL, Dunaway DJ, Forrest CR, Koudstaal MJ, Caron CJJM
Int J Oral Maxillofac Surg 2022 Oct;51(10):1296-1304. Epub 2022 Feb 3 doi: 10.1016/j.ijom.2022.01.005. PMID: 35125269
Hong R, Du Q, Pan Y
AJNR Am J Neuroradiol 2020 Jun;41(6):1076-1080. Epub 2020 May 28 doi: 10.3174/ajnr.A6576. PMID: 32467182Free PMC Article
Evans MA, Sugihara EM, Chan EY
Otol Neurotol 2017 Sep;38(8):1149-1152. doi: 10.1097/MAO.0000000000001487. PMID: 28727650
Braun T, Hempel JM, Berghaus A
Dtsch Arztebl Int 2014 Feb 7;111(6):92-8. doi: 10.3238/arztebl.2014.0092. PMID: 24622605Free PMC Article
Mazita A, Zabri M, Aneeza WH, Asma A, Saim L
J Laryngol Otol 2011 Nov;125(11):1116-20. Epub 2011 Aug 16 doi: 10.1017/S0022215111002052. PMID: 21846418

Diagnosis

Michels TC, Duffy MT, Rogers DJ
Am Fam Physician 2019 Jul 15;100(2):98-108. PMID: 31305044
Ho ML
Neuroimaging Clin N Am 2019 Feb;29(1):57-92. doi: 10.1016/j.nic.2018.09.005. PMID: 30466645
Kaga K
Adv Otorhinolaryngol 2014;75:20-3. Epub 2013 Oct 11 doi: 10.1159/000350596. PMID: 24135344
Parisier SC, Bent JP 3rd
Otolaryngol Clin North Am 1999 Jun;32(3):457-69. doi: 10.1016/s0030-6665(05)70145-6. PMID: 10393779
Weissman JL
Radiology 1996 Jun;199(3):593-611. doi: 10.1148/radiology.199.3.8637972. PMID: 8637972

Therapy

Alsebayel MM, Abaalkhail FA, Alsebayel FM, Alissa DA, Al-Jedai AH, Elsiesy H
Am J Case Rep 2018 May 3;19:523-526. doi: 10.12659/AJCR.908433. PMID: 29720579Free PMC Article
Mukherjee P, Cheng K, Flanagan S, Greenberg S
Eur Arch Otorhinolaryngol 2017 Aug;274(8):3021-3028. Epub 2017 May 24 doi: 10.1007/s00405-017-4618-4. PMID: 28540513
Braun T, Hempel JM, Berghaus A
Dtsch Arztebl Int 2014 Feb 7;111(6):92-8. doi: 10.3238/arztebl.2014.0092. PMID: 24622605Free PMC Article
Cremers CWRJ
Adv Otorhinolaryngol 2007;65:278-284. doi: 10.1159/000098843. PMID: 17245059
da Cunha Ferreira S, de Melo Tavares de Lima MA
Braz J Otorhinolaryngol 2006 May-Jun;72(3):414-8. doi: 10.1016/s1808-8694(15)30978-2. PMID: 17119781Free PMC Article

Prognosis

Yang J, Zhao C, Liu Y, Gao M, Ren R, Wang D, Huang Z, Zhao S
J Otolaryngol Head Neck Surg 2020 Aug 8;49(1):57. doi: 10.1186/s40463-020-00452-3. PMID: 32771065Free PMC Article
Tamplen M, Schwalje A, Lustig L, Alemi AS, Miller ME
Laryngoscope 2016 Jun;126(6):1440-5. Epub 2015 Sep 11 doi: 10.1002/lary.25659. PMID: 26360798
Bouhabel S, Arcand P, Saliba I
Int J Pediatr Otorhinolaryngol 2012 Feb;76(2):272-7. Epub 2011 Dec 15 doi: 10.1016/j.ijporl.2011.11.020. PMID: 22177319
Eelkema EA, Curtin HD
Semin Ultrasound CT MR 1989 Jun;10(3):195-212. PMID: 2697337
De la Cruz A, Linthicum FH Jr, Luxford WM
Laryngoscope 1985 Apr;95(4):421-7. PMID: 3884941

Clinical prediction guides

Ng JH, Kuthubutheen J
Otol Neurotol 2022 Oct 1;43(9):e1020-e1023. Epub 2022 Sep 1 doi: 10.1097/MAO.0000000000003676. PMID: 36047684
Volgger V, Schießler IT, Müller J, Schrötzlmair F, Pollotzek M, Hempel JM
Eur Arch Otorhinolaryngol 2022 May;279(5):2345-2352. Epub 2021 Jun 26 doi: 10.1007/s00405-021-06938-8. PMID: 34173875Free PMC Article
Jeon JW, Christensen J, Chisholm J, Zalewski C, Rasooly M, Dempsey C, Magnani A, Frischmeyer-Guerrerio P, Brewer CC, Kim HJ
Otolaryngol Head Neck Surg 2022 Feb;166(2):357-362. Epub 2021 May 11 doi: 10.1177/01945998211008899. PMID: 33971761Free PMC Article
Nishiyama T, Oishi N, Ogawa K
Int J Pediatr Otorhinolaryngol 2021 Mar;142:110628. Epub 2021 Jan 16 doi: 10.1016/j.ijporl.2021.110628. PMID: 33494042
Michels TC, Duffy MT, Rogers DJ
Am Fam Physician 2019 Jul 15;100(2):98-108. PMID: 31305044

Recent systematic reviews

Rooijers W, Tio PAE, van der Schroeff MP, Padwa BL, Dunaway DJ, Forrest CR, Koudstaal MJ, Caron CJJM
Int J Oral Maxillofac Surg 2022 Oct;51(10):1296-1304. Epub 2022 Feb 3 doi: 10.1016/j.ijom.2022.01.005. PMID: 35125269

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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