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Focal absence of the external ear

MedGen UID:
867495
•Concept ID:
C4021874
•
Anatomical Abnormality
HPO: HP:0400003

Definition

Absence of a localized portion of the ear that cannot be described by a more precise term (e.g., absent ear lobe). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal absence of the external ear

Professional guidelines

PubMed

Successful treatment of erythematous-squamous disorders of the external auditory canal with tacrolimus and clotrimazole in otic oil: Our experience in 25 patients.
Navarro-Triviño FJ, Ruiz-Villaverde R
Dermatol Ther 2020 Nov;33(6):e14471. Epub 2020 Nov 4 doi: 10.1111/dth.14471. PMID: 33112026
ACR Appropriateness Criteria(®) Tinnitus.
Expert Panel on Neurologic Imaging:, Kessler MM, Moussa M, Bykowski J, Kirsch CFE, Aulino JM, Berger KL, Choudhri AF, Fife TD, Germano IM, Kendi AT, Kim JH, Luttrull MD, Nunez D Jr, Shah LM, Sharma A, Shetty VS, Symko SC, Cornelius RS
J Am Coll Radiol 2017 Nov;14(11S):S584-S591. doi: 10.1016/j.jacr.2017.08.052. PMID: 29101995
Osseointegrated alloplastic versus autogenous ear reconstruction: criteria for treatment selection.
Wilkes GH, Wolfaardt JF
Plast Reconstr Surg 1994 Apr;93(5):967-79. doi: 10.1097/00006534-199404001-00011. PMID: 8134490

Recent clinical studies

Etiology

Global age-sex-specific mortality, life expectancy, and population estimates in 204 countries and territories and 811 subnational locations, 1950-2021, and the impact of the COVID-19 pandemic: a comprehensive demographic analysis for the Global Burden of Disease Study 2021.
GBD 2021 Demographics Collaborators
Lancet 2024 May 18;403(10440):1989-2056. Epub 2024 Mar 11 doi: 10.1016/S0140-6736(24)00476-8. PMID: 38484753Free PMC Article
Residual Cholesteatoma After Endoscopic-Assisted Canal Wall-Up Tympanomastoidectomy: A Randomized Controlled Trial.
Noschang Lopes da Silva M, Selaimen da Costa S, André Selaimen F, da Costa Huve F, Lang Silva A, Dias Toshiaki Koga F, Martins-Costa LL, Bernard Rosa Nery M, Zanardini M, Sperling N
Otol Neurotol 2022 Aug 1;43(7):803-807. doi: 10.1097/MAO.0000000000003575. PMID: 35878636
Pharmacokinetics of Single Dose Lidocaine and Epinephrine Following Iontophoresis of the Tympanic Membrane in a Double-Blinded Randomized Trial.
England LJ, Syms CA 3rd, York C
Otol Neurotol 2021 Aug 1;42(7):1095-1103. doi: 10.1097/MAO.0000000000003128. PMID: 33859140Free PMC Article
Protein profile of ear auricle cartilage and the important role of ITGB1/PTK2 in microtia.
Dong W, Jiang H, He L, Pan B, Lin L, Song Y, Yang Q
Int J Pediatr Otorhinolaryngol 2020 Oct;137:110235. Epub 2020 Jul 12 doi: 10.1016/j.ijporl.2020.110235. PMID: 32896350
Temporal bone carcinoma: Treatment patterns and survival.
Seligman KL, Sun DQ, Ten Eyck PP, Schularick NM, Hansen MR
Laryngoscope 2020 Jan;130(1):E11-E20. Epub 2019 Mar 15 doi: 10.1002/lary.27877. PMID: 30874314Free PMC Article

Diagnosis

ACR Appropriateness Criteria(®) Tinnitus.
Expert Panel on Neurologic Imaging:, Kessler MM, Moussa M, Bykowski J, Kirsch CFE, Aulino JM, Berger KL, Choudhri AF, Fife TD, Germano IM, Kendi AT, Kim JH, Luttrull MD, Nunez D Jr, Shah LM, Sharma A, Shetty VS, Symko SC, Cornelius RS
J Am Coll Radiol 2017 Nov;14(11S):S584-S591. doi: 10.1016/j.jacr.2017.08.052. PMID: 29101995
Evaluation of linear accelerator-based stereotactic radiosurgery in the management of glomus jugulare tumors.
Sager O, Beyzadeoglu M, Dincoglan F, Gamsiz H, Demiral S, Uysal B, Oysul K, Dirican B, Sirin S
Tumori 2014 Mar-Apr;100(2):184-8. doi: 10.1177/030089161410000211. PMID: 24852863
Congenital cholesteatoma in identical twins.
Al Balushi T, Naik JZ, Al Khabori M
J Laryngol Otol 2013 Jan;127(1):67-9. Epub 2012 Dec 10 doi: 10.1017/S0022215112002757. PMID: 23217274
Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review.
Markou K, Karasmanis I, Vlachtsis K, Petridis D, Nikolaou A, Vital V
Am J Otolaryngol 2008 Mar-Apr;29(2):142-6. doi: 10.1016/j.amjoto.2007.04.005. PMID: 18314029
Malignant external otitis and osteomyelitis of the base of the skull.
Chandler JR
Am J Otol 1989 Mar;10(2):108-10. PMID: 2735380

Therapy

Residual Cholesteatoma After Endoscopic-Assisted Canal Wall-Up Tympanomastoidectomy: A Randomized Controlled Trial.
Noschang Lopes da Silva M, Selaimen da Costa S, André Selaimen F, da Costa Huve F, Lang Silva A, Dias Toshiaki Koga F, Martins-Costa LL, Bernard Rosa Nery M, Zanardini M, Sperling N
Otol Neurotol 2022 Aug 1;43(7):803-807. doi: 10.1097/MAO.0000000000003575. PMID: 35878636
Pharmacokinetics of Single Dose Lidocaine and Epinephrine Following Iontophoresis of the Tympanic Membrane in a Double-Blinded Randomized Trial.
England LJ, Syms CA 3rd, York C
Otol Neurotol 2021 Aug 1;42(7):1095-1103. doi: 10.1097/MAO.0000000000003128. PMID: 33859140Free PMC Article
Evaluation of linear accelerator-based stereotactic radiosurgery in the management of glomus jugulare tumors.
Sager O, Beyzadeoglu M, Dincoglan F, Gamsiz H, Demiral S, Uysal B, Oysul K, Dirican B, Sirin S
Tumori 2014 Mar-Apr;100(2):184-8. doi: 10.1177/030089161410000211. PMID: 24852863
Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases.
Wenson SF, Jessup CJ, Johnson MM, Cohen LM, Mahmoodi M
J Cutan Pathol 2012 Feb;39(2):263-9. Epub 2011 Nov 12 doi: 10.1111/j.1600-0560.2011.01812.x. PMID: 22077601
Malignant external otitis and osteomyelitis of the base of the skull.
Chandler JR
Am J Otol 1989 Mar;10(2):108-10. PMID: 2735380

Prognosis

New Method Using Local Affected Cartilage and Flap for the Cleft in Question Mark Ear.
Wang T, Yu X, Jiang H, Pan B
Aesthetic Plast Surg 2024 Jun;48(11):2034-2041. Epub 2024 Apr 27 doi: 10.1007/s00266-024-04030-0. PMID: 38676769
Global age-sex-specific mortality, life expectancy, and population estimates in 204 countries and territories and 811 subnational locations, 1950-2021, and the impact of the COVID-19 pandemic: a comprehensive demographic analysis for the Global Burden of Disease Study 2021.
GBD 2021 Demographics Collaborators
Lancet 2024 May 18;403(10440):1989-2056. Epub 2024 Mar 11 doi: 10.1016/S0140-6736(24)00476-8. PMID: 38484753Free PMC Article
Temporal bone carcinoma: Treatment patterns and survival.
Seligman KL, Sun DQ, Ten Eyck PP, Schularick NM, Hansen MR
Laryngoscope 2020 Jan;130(1):E11-E20. Epub 2019 Mar 15 doi: 10.1002/lary.27877. PMID: 30874314Free PMC Article
Evaluation of linear accelerator-based stereotactic radiosurgery in the management of glomus jugulare tumors.
Sager O, Beyzadeoglu M, Dincoglan F, Gamsiz H, Demiral S, Uysal B, Oysul K, Dirican B, Sirin S
Tumori 2014 Mar-Apr;100(2):184-8. doi: 10.1177/030089161410000211. PMID: 24852863
Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review.
Markou K, Karasmanis I, Vlachtsis K, Petridis D, Nikolaou A, Vital V
Am J Otolaryngol 2008 Mar-Apr;29(2):142-6. doi: 10.1016/j.amjoto.2007.04.005. PMID: 18314029

Clinical prediction guides

Global age-sex-specific mortality, life expectancy, and population estimates in 204 countries and territories and 811 subnational locations, 1950-2021, and the impact of the COVID-19 pandemic: a comprehensive demographic analysis for the Global Burden of Disease Study 2021.
GBD 2021 Demographics Collaborators
Lancet 2024 May 18;403(10440):1989-2056. Epub 2024 Mar 11 doi: 10.1016/S0140-6736(24)00476-8. PMID: 38484753Free PMC Article
Residual Cholesteatoma After Endoscopic-Assisted Canal Wall-Up Tympanomastoidectomy: A Randomized Controlled Trial.
Noschang Lopes da Silva M, Selaimen da Costa S, André Selaimen F, da Costa Huve F, Lang Silva A, Dias Toshiaki Koga F, Martins-Costa LL, Bernard Rosa Nery M, Zanardini M, Sperling N
Otol Neurotol 2022 Aug 1;43(7):803-807. doi: 10.1097/MAO.0000000000003575. PMID: 35878636
Successful treatment of erythematous-squamous disorders of the external auditory canal with tacrolimus and clotrimazole in otic oil: Our experience in 25 patients.
Navarro-Triviño FJ, Ruiz-Villaverde R
Dermatol Ther 2020 Nov;33(6):e14471. Epub 2020 Nov 4 doi: 10.1111/dth.14471. PMID: 33112026
Temporal bone carcinoma: Treatment patterns and survival.
Seligman KL, Sun DQ, Ten Eyck PP, Schularick NM, Hansen MR
Laryngoscope 2020 Jan;130(1):E11-E20. Epub 2019 Mar 15 doi: 10.1002/lary.27877. PMID: 30874314Free PMC Article
Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases.
Wenson SF, Jessup CJ, Johnson MM, Cohen LM, Mahmoodi M
J Cutan Pathol 2012 Feb;39(2):263-9. Epub 2011 Nov 12 doi: 10.1111/j.1600-0560.2011.01812.x. PMID: 22077601

Recent systematic reviews

Questioning the value of stalk drilling after external auditory canal osteoma excision: case series, literature review, and meta-analysis.
Argaman A, Oron Y, Handzel O, Abu-Eta R, Muhanna N, Halpern D, Ungar OJ
Eur Arch Otorhinolaryngol 2024 Jan;281(1):51-59. Epub 2023 Jun 19 doi: 10.1007/s00405-023-08074-x. PMID: 37335347

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