Microtia-Associated Syndromes

  • Sunil S. Tholpady
  • Michael W. Chu


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].



Oculo-auriculo-vertebral spectrum


Online Mendelian Inheritance in Man


Treacher Collins syndrome


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Sunil S. Tholpady
    • 1
    • 2
  • Michael W. Chu
    • 2
  1. 1.Riley Hospital for Children, Department of SurgeryIndianapolisUSA
  2. 2.Indiana University, Department of SurgeryIndianapolisUSA

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