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Microtia, third degree

MedGen UID:
866820
Concept ID:
C4021174
Anatomical Abnormality
Synonyms: Abnormal shape/structure of ear; Third-degree microtia
 
HPO: HP:0011267

Definition

Presence of some auricular structures, but none of these structures conform to recognized ear components. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicrotia, third degree

Conditions with this feature

Ablepharon macrostomia syndrome
MedGen UID:
395439
Concept ID:
C1860224
Disease or Syndrome
Ablepharon-macrostomia syndrome (AMS) is a congenital ectodermal dysplasia characterized by absent eyelids, macrostomia, microtia, redundant skin, sparse hair, dysmorphic nose and ears, variable abnormalities of the nipples, genitalia, fingers, and hands, largely normal intellectual and motor development, and poor growth (summary by Marchegiani et al., 2015).

Recent clinical studies

Etiology

Siegert R
Adv Otorhinolaryngol 2010;68:95-107. Epub 2010 May 3 doi: 10.1159/000314565. PMID: 20442564
Siegert R
Laryngoscope 2003 Nov;113(11):2021-7; discussion 2028-9. doi: 10.1097/00005537-200311000-00031. PMID: 14603067
Rotenberg BW, James AL, Fisher D, Anderson J, Papsin BC
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):273-9. doi: 10.1016/s0165-5876(02)00252-5. PMID: 12443817
Siegert R, Weerda H
Laryngoscope 2001 Apr;111(4 Pt 1):708-14. doi: 10.1097/00005537-200104000-00026. PMID: 11359144
Lambert PR
Ann Otol Rhinol Laryngol 1988 Nov-Dec;97(6 Pt 1):641-9. doi: 10.1177/000348948809700612. PMID: 3202566

Diagnosis

Bogusiak K, Arkuszewski P, Skorek-Stachnik K, Kozakiewicz M
J Craniofac Surg 2014 Jan;25(1):177-83. doi: 10.1097/SCS.0000000000000387. PMID: 24406574
Rotenberg BW, James AL, Fisher D, Anderson J, Papsin BC
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):273-9. doi: 10.1016/s0165-5876(02)00252-5. PMID: 12443817

Therapy

Romo T 3rd, Reitzen SD
Facial Plast Surg 2008 Jan;24(1):120-8. doi: 10.1055/s-2008-1037453. PMID: 18286440
Rotenberg BW, James AL, Fisher D, Anderson J, Papsin BC
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):273-9. doi: 10.1016/s0165-5876(02)00252-5. PMID: 12443817

Prognosis

Siegert R
Adv Otorhinolaryngol 2010;68:95-107. Epub 2010 May 3 doi: 10.1159/000314565. PMID: 20442564
Rotenberg BW, James AL, Fisher D, Anderson J, Papsin BC
Int J Pediatr Otorhinolaryngol 2002 Dec 2;66(3):273-9. doi: 10.1016/s0165-5876(02)00252-5. PMID: 12443817
Lambert PR
Ann Otol Rhinol Laryngol 1988 Nov-Dec;97(6 Pt 1):641-9. doi: 10.1177/000348948809700612. PMID: 3202566

Clinical prediction guides

Bogusiak K, Arkuszewski P, Skorek-Stachnik K, Kozakiewicz M
J Craniofac Surg 2014 Jan;25(1):177-83. doi: 10.1097/SCS.0000000000000387. PMID: 24406574
Siegert R
Adv Otorhinolaryngol 2010;68:95-107. Epub 2010 May 3 doi: 10.1159/000314565. PMID: 20442564
Siegert R
Laryngoscope 2003 Nov;113(11):2021-7; discussion 2028-9. doi: 10.1097/00005537-200311000-00031. PMID: 14603067
Lambert PR
Ann Otol Rhinol Laryngol 1988 Nov-Dec;97(6 Pt 1):641-9. doi: 10.1177/000348948809700612. PMID: 3202566

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