pediatrics
Congenital Ear Abnormalities
Introduction
Ears can be absent, deformed, or incompletely developed at birth. (See also Introduction to Congenital Craniofacial and Musculoskeletal Abnormalities and Microtiaandexternal auditory canal atresia(which causes conductive hearing loss) involve the external ear. These malformations, which frequently coexist, are often identified at or soon after birth. Occasionally, school-based screening tests identify a partially occluded external auditory canal in children with a normal pinna. Low-set earsare ears positioned below where ears are typically positioned on the head. In low-set ears, the top of the pinna is positioned below the horizontal line connecting the outer corners of the eyes. This abnormality is associated with a number of genetic syndromes and often with developmental delays. Ear pitsandear tagsare minor anomalies typically located in front of the ear. Patients with these anomalies should be evaluated for hearing loss and for other congenital anomalies (eg, kidney anomalies with ear pits in branchio-oto-renal syndrome). The rate of kidney anomalies is increased in people with ear pits, so renal ultrasonography should be considered. Physical examination and CT of the temporal bone are necessary to evaluate possible additional bony malformations. This photo shows unilateral microtia. © Springer Science+Business Media This child has low-set ears and other facial characteristics of r(18) syndrome. © Springer Science+Business Media This photo shows a child with two ear pits. The black arrow points to the more common preauricular pit, and the white arrow points to a less common helical pit adjacent to the notch in the helix. © Springer Science+Business Media An ear tag (arrow) is a congenital abnormality. © Springer Science+Business Media 