Ear
Congenital anomalies
Congential - other
Author: Nat Pernick, M.D.
Last author update: 1 October 2013
Last staff update: 12 March 2025
Copyright: 2002-2025, PathologyOutlines.com, Inc.
PubMed Search: Ear congenital
Cite this page: Pernick N. Congential - other. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earcongenitalother.html. Accessed April 1st, 2025.
Definition / general
- Congenital anomalies of the ear are common (1 per 6,000 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
Accessory tragi
- See Accessory tragi
Heterotopia / choristoma
Definition / general
Case reports
Radiology images
Images hosted on other servers:
Additional references
- WHO classification includes salivary gland choristoma and glial choristoma
- Heterotopia: mass of tissue normal to the site in abnormal location
- Choristoma: mass of tissue foreign to the site
- Hamartoma: mass of tissue normal to site in haphazard arrangement
- Middle ear choristomas include salivary gland tissue and neuroglial tissue
- Salivary gland choristomas usually occur in women, associated with facial nerve and ossicle anomalies, suggesting a second branchial arch developmental anomaly; have mucinous and serous elements similar to submandibular and sublingual glands
- Neuroglial choristomas are often actually an acquired encephalocele with herniation of brain into middle ear and mastoid; treatment is surgical, although tissue may adhere to facial nerve; determine relationship to CNS structures at operation or radiographically, not by histology (Laryngoscope 2000;110:1731, Ann Diagn Pathol 2004;8:252)
- Cartilaginous choristoma of external ear canal: may be relatively common (Otolaryngol Head Neck Surg 2005;133:786)
Case reports
- 14 year old girl with salivary gland choristoma of the middle ear (Laryngoscope 2006;116:1033)
- 50 year old man with glial choristoma of middle ear and mastoid bone (J Korean Med Sci 2004;19:155)
- Patient with salivary gland choristoma of the middle ear (Arch Pathol Lab Med 1982;106:39)
Radiology images
Images hosted on other servers:
Mass-like lesion with soft tissue density
Additional references
