Congenital anomalies

Congenital anomalies - general

Last author update: 1 October 2013
Last staff update: 17 April 2021

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PubMed Search: Ear [title] congenital anomalies review[ptyp]

Nat Pernick, M.D.
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Cite this page: Pernick N. Congenital anomalies - general. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earcongenital.html. Accessed June 3rd, 2023.
Definition / general
  • Common (1 per 6000 births); higher incidence in Japanese and Navajo Indians
  • More common in males
  • 90% are unilateral
  • Either isolated or with other abnormalities
  • Cosmetic or functional
  • Anotia: complete absence of external ear
  • Microtia: mild to severe deformity; associated with other defects
First branchial cleft anomalies
  • Cysts, sinuses, abscesses (Emerg Med J 2003;20:103) and fistulas near external ear; may be pre, post or infraauricular; also at angle of jaw, ear lobe, in external auditory canal or parotid gland
  • Fistulas may connect skin with external auditory canal
  • Includes duplication of external auditory canal (Int J Pediatr Otorhinolaryngol 2005;69:255)
  • 1 - 8% of branchial defects
  • Usually middle aged women
  • Classified by Work as Type I or II (Laryngoscope 1972;82:1581)
  • Type I: keratinizing squamous epithelium without adnexae (ectoderm only, duplicates membranous external auditory canal)
  • Type II: keratinized squamous epithelium with adnexa and cartilage (ectoderm and mesoderm, duplicates external auditory canal and pinna)
  • Treatment: excision; often recurs
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