Congenital ossicular anomalies

Last revised by Rohit Sharma on 15 Dec 2018

Congenital anomalies of the ossicles are most frequently associated with external ear abnormalities also, although they can occur in isolation. 

Clinical presentation

These anomalies result in conductive hearing loss.


When bilateral, they are most frequently genetic with autosomal dominant inheritance. Unilateral anomalies are usually sporadic.

Isolated ossicular anomalies can be classified as:

  • class I: stapedial ankylosis
  • class II: stapedial ankylosis with other associated anomalies
  • class III: ossicular anomalies without stapedial ankylosis
  • class IV: oval/round dysplasia

They are associated with numerous syndromes including:

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Cases and figures

  • Ossicles Annotated
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