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Microtia-Associated Syndromes

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Modern Microtia Reconstruction

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Abstract

External ear development occurs from derivations of the first (mandibular) and second (hyoid) pharyngeal arches, with the first pharyngeal cleft responsible for the external auditory meatus. At approximately 5 weeks of age, each arch develops three elevations that form into the six hillocks of His that create very specific auricular structures. From the mandibular arch, the three hillocks form the tragus, helix, and cymba concha, while the three hillocks from the hyoid arch form the concha, antihelix, and antitragus. The ear is attached to the skull by anterior and posterior extrinsic as well as intrinsic ligaments, external (auricularis anterior, superior, and posterior) and internal (helicis major and minor, tragicus, antitragicus, and the transverse and oblique) muscles, skin, and the external auditory canal cartilage. The ear lies in a horizontal posterior position relative to the mandible and only achieves its rotated and elevated position during mandibular and facial growth. The ear itself is composed of neural crest cells, which are a specialized cell type responsible for much of facial development and migrate a large distance to make their cognate structures [1–4].

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Abbreviations

OAVS:

Oculo-auriculo-vertebral spectrum

OMIM:

Online Mendelian Inheritance in Man

TCS:

Treacher Collins syndrome

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Author information

Authors and Affiliations

  1. Riley Hospital for Children, Department of Surgery, Indianapolis, IN, USA

    Sunil S. Tholpady

  2. Indiana University, Department of Surgery, Indianapolis, IN, USA

    Sunil S. Tholpady & Michael W. Chu

Authors
  1. Sunil S. Tholpady
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  2. Michael W. Chu
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Editor information

Editors and Affiliations

  1. Keck School of Medicine, University of Souther California, Los Angeles, CA, USA

    John F. Reinisch

  2. Plastic and Reconstructive Surgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA

    Youssef Tahiri

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Tholpady, S.S., Chu, M.W. (2019). Microtia-Associated Syndromes. In: Reinisch, J., Tahiri, Y. (eds) Modern Microtia Reconstruction. Springer, Cham. https://doi.org/10.1007/978-3-030-16387-7_4

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  • DOI: https://doi.org/10.1007/978-3-030-16387-7_4

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