Congenital Ear Anomaly


Congenital Ear Anomaly, Congenital Ear Malformation, Low-Set Ears, Low Set Ears, Congenital Small Ears, Microtia, Pre-auricular Skin Tag

  • Definitions
  1. Low-Set Ears
    1. Pinna falls below the horizontal level of the medial canthus of the eye (or line drawn from the lateral canthus to the occipital protuberance)
    2. Ear Pinna is rotated posteriorly
    3. Caused by several congenital disorders (see below)
    4. Associated with Hearing Deficit (obtain Newborn Hearing Screening) and genitourinary anomalies (obtain renal Ultrasound)
  2. Microtia (Congenital Small Ears)
    1. Small, undeveloped pinna or absent pinna in the case of anotia
    2. May be associated with Hearing Deficit (obtain Newborn Hearing Screening)
  3. Pre-auricular Skin Tags, ear pits, fissures or sinuses
    1. Growths or defects at the anterior Ear Pinna
    2. May be associated with Hearing Deficits (obtain Newborn Hearing Screening)
    3. Renal Ultrasound not indicated unless other dysmorphic features, Teratogen Exposure, deafness Family History or maternal diabetes
  • Causes
  • Low-Set Ears
  1. Trisomy 21 (Down Syndrome)
  2. Trisomy 18
  • Causes
  • Microtia
  1. CHARGE Syndrome
  2. Goldenhar Syndrome
  3. Treacher-Collins Syndrome