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Microtia

Last revised by Arlene Campos ◉ on 12 Jul 2024

Citation, DOI, disclosures and article data

Citation:

Weerakkody Y, Campos A, Walizai T, et al. Microtia. Reference article, Radiopaedia.org (Accessed on 03 Dec 2024) https://doi.org/10.53347/rID-10710

DOI:

https://doi.org/10.53347/rID-10710

Permalink:

https://radiopaedia.org/articles/10710

rID:

10710

Article created:

11 Sep 2010, Yuranga Weerakkody ◉

Disclosures:

At the time the article was created Yuranga Weerakkody had no recorded disclosures.

View Yuranga Weerakkody's current disclosures

Last revised:

12 Jul 2024, Arlene Campos ◉

Disclosures:

At the time the article was last revised Arlene Campos had no financial relationships to ineligible companies to disclose.

View Arlene Campos's current disclosures

Revisions:

10 times, by 10 contributors - see full revision history and disclosures

Systems:

Head & Neck

Sections:

Classifications

Synonyms:

Micro-otia

Microtia refers to a small pinna of the ear, when is it completely absent it is termed anotia.

Epidemiology

The estimated incidence at around 1:9000 live births. It is more common in males and there is a recognized right sided predilection.

Pathology

Associations

Microtia can be associated with a wide spectrum of inner and middle ear anomalies ¹. Isolated microtia is relatively uncommon. The most frequent associations include:

Wider organ associations include:

renal anomalies
- cross-fused ectopia
- renal agenesis

Syndromic associations include:

Classification

A number of classification of microtia have been described^6-10. The classification proposed by Weerda in 1998⁹, which is built upon earlier classifications by Marx ¹⁰, Rogers and Tanzer, is probably most widely used, and divides microtia into three grades based on increasing severity ⁹:

first degree (Marx grade I)
- slightly smaller ear than normal (>2 SD below mean)
- all structure are identifiable and essentially normal
second degree (Marx grade II)
- small l (>2 SD below mean)
- some features are abnormal while other are recognizable
third degree (Marx grade III)
- absence of the external ear with a small deformed vestigial structure with no recognisable features.
- often associated with external acoutic canal atresia
anotia (sometimes referred to as grade IV) ⁷
- complete absence of the external ear (anotia)

References

1. Mayer TE, Brueckmann H, Siegert R et-al. High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol. 1997;18 (1): 53-65. AJNR Am J Neuroradiol (abstract) - Pubmed citation
2. Swartz JD, Faerber EN. Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import. AJR Am J Roentgenol. 1985;144 (3): 501-6. AJR Am J Roentgenol (abstract) - Pubmed citation
3. Calzolari F, Garani G, Sensi A et-al. Clinical and radiological evaluation in children with microtia. Br J Audiol. 1999;33 (5): 303-12. - Pubmed citation
4. Takegoshi H, Kaga K, Kikuchi S, Ito K. Facial Canal Anatomy in Patients with Microtia: Evaluation of the Temporal Bones with Thin-Section CT. Radiology. 2002;225(3):852-8. doi:10.1148/radiol.2253010942 - Pubmed
5. Marc Lemmerling, Spyros S. Kollias. Radiology of the Petrous Bone. (2004) ISBN: 9783540423942 - Google Books
6. Zhang T, Bulstrode N, Chang K et al. International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction. J Int Adv Otol. 2019;15(2):204-8. doi:10.5152/iao.2019.7383 - Pubmed
7. Luquetti D, Heike C, Hing A, Cunningham M, Cox T. Microtia: Epidemiology and Genetics. Am J Med Genet A. 2012;158A(1):124-39. doi:10.1002/ajmg.a.34352 - Pubmed
8. Hunter A, Frias J, Gillessen-Kaesbach G, Hughes H, Jones K, Wilson L. Elements of Morphology: Standard Terminology for the Ear. Am J Med Genet A. 2009;149A(1):40-60. doi:10.1002/ajmg.a.32599 - Pubmed
9. Weerda H. Classification of Congenital Deformities of the Auricle. Facial Plast Surg. 1988;5(5):385-8. doi:10.1055/s-2008-1064778 - Pubmed
10. Marx H. Die Missbildungen des Ohres. In: Henke F, Lubarsh O, eds. Handbuch der Spez Path Anatomie Histologie. Berlin, Germany: Springer; 1926:620-625. Google Scholar

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