ICD-11 definition : 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 disorganized
remnant of cartilage attached to a variable amount of soft tissue lobule.;
ICD-11 synonym : congenital small ears; hypoplasia of ear;
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 disorganized
remnant of cartilage attached to a variable amount of soft tissue lobule.