LA22.0

ICD-11 MMS 2026-01

Microtia

Description

Microtia is a congenital malformation of variable severity of the external and middle ear. Both hereditary factors (evidence for familial craniofacial microsomia and patterns suggestive of multifactorial inheritance) and vascular accidents are involved in the etiology of the disease. Specific causative factors also can include maternal rubella during the first trimester of pregnancy. Microtia commonly involves the external canal and middle ear; hence, hearing can be affected. Microtia may present within a spectrum of branchial arch defects (hemifacial microsomia, craniofacial microsomia) or may manifest as an independent malformation. The microtic auricle consists of a disorganised remnant of cartilage attached to a variable amount of soft tissue lobule.

Index terms

  • Microtia
  • congenital small ears
  • hypoplasia of ear
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  • Microtia Type 1
  • Microtia Type 2
  • Microtia Type 3

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA22.1 – Anotia
  • LA22.2 – Aplasia or hypoplasia of external auditory canal
  • LA22.3 – Structural developmental anomalies of ear ossicles
  • LA22.4 – Structural developmental anomalies of inner ear
  • LA22.Y – Other specified structural developmental anomalies of ear causing hearing impairment
  • LA22.Z – Structural developmental anomalies of ear causing hearing impairment, unspecified

Foundation entity

Microtia – http://id.who.int/icd/entity/2005415414

Allowed postcoordination axes

Part of: LA22: Structural developmental anomalies of ear causing hearing impairment › 20: Developmental anomalies