Ear
Congenital anomalies
Congenital anomalies - general
Author: Nat Pernick, M.D.
Last author update: 1 October 2013
Last staff update: 17 April 2021
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Ear [title] congenital anomalies review[ptyp]
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
