External, Middle, and Inner Ear

  • Frank G. Garritano
  • Vito C. Quatela


The embryogenesis of the external, middle, and inner ear is a highly complex process. The external and middle ear are largely formed as part of the branchial apparatus, whereas the inner ear develops separately from the otic placode. Deviations from the normal pattern of embryogenesis at varying stages of development can give rise to a wide variety of congenital malformations. Because of how closely these structures are linked, a malformation affecting the external ear can also frequently affect the middle and internal ear and vice versa. A clear knowledge of the processes that lead to these congenital malformations helps inform our understanding of these pathologic conditions.


  1. 1.
    Harris J, Källén B, Robert ES. The epidemiology of anotia and microtia. Med Genet. 1996;33(10):809.CrossRefGoogle Scholar
  2. 2.
    Ang GS, Simpson SA, Reddy AR. Mycophenolate mofetil embryopathy may be dose and timing dependent. Am J Med Genet A. 2008;146A(15):1963.CrossRefGoogle Scholar
  3. 3.
    Llano-Rivas I, González-del Angel A, del Castillo V, Reyes R, Carnevale A. Microtia: a clinical and genetic study at the National Institute of Pediatrics in Mexico City. Arch Med Res. 1999;30(2):120.CrossRefGoogle Scholar
  4. 4.
    Scheinfeld NS, Silverberg NB, Weinberg JM, Nozad V. The preauricular sinus: a review of its clinical presentation, treatment, and associations. Pediatr Dermatol. 2004;21(3):191.CrossRefGoogle Scholar
  5. 5.
    Teunissen EB, Cremers WR. Classification of congenital middle ear anomalies. Report of 144 ears. Ann Otol Rhinol Laryngol. 1993;102(8):606–12.CrossRefGoogle Scholar
  6. 6.
    Swartz JD, Faerber EN. Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import. Am J Roentgenol. 1985;144(3):501–6.CrossRefGoogle Scholar
  7. 7.
    Joshi VM, Navlekar SK, Kishore GR. CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics. 2012;32(3):683–98.CrossRefGoogle Scholar
  8. 8.
    Jackler RK. Congenital malformations of the inner ear. In: Cummings CW, Flint PW, Harker LA, et al., editors. Cummings otolaryngology: head and neck surgery. 4th ed. Elsevier Mosby: Philadelphia, PA; 2005. p. 4413–4.Google Scholar
  9. 9.
    Ozgen B, Oguz KK, Atas A, Sennaroglu L. Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 2009;30(4):774–80.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Frank G. Garritano
    • 1
  • Vito C. Quatela
    • 2
    • 3
  1. 1.Facial Plastic and Reconstructive SurgeryThe Permanente Medical GroupOaklandUSA
  2. 2.Quatela Center for Plastic SurgeryRochesterUSA
  3. 3.University of RochesterRochesterUSA

Personalised recommendations