How to treat. Treatments vary and include observation, excision, intralesional injections, cryotherapy, enzyme therapy, silicone gel application, and irradiation.23 Recurrence is common; no therapy has been proven to be universally superior or preferred.
Disruption of embryologic development (failed invagination of the external auditory canal) can lead to a stenotic or absent ear canal (aural atresia). Aural atresia is also often associated with fusion of the incus and malleus. This condition occurs predominantly in males. Unilateral atresia is more common than bilateral atresia, and the right ear is more often involved than the left.24
Microtia is the incomplete development of the pinna leading to a small or deformed pinna. Microtia can be unilateral or bilateral. As with atresia, microtia more commonly affects males and, if unilateral, the right side is more often affected than the left. Microtia can occur in isolation but is often associated with genetic syndromes such as Treacher Collins syndrome and craniofacial microsomia (Goldenhar syndrome). When microtia is identified (typically at birth or early infancy), audiologic testing and a thorough physical examination for evidence of associated defects should be performed. Consult with an audiologist, clinical geneticist, or pediatric otolaryngologist.
Pre-auricular pits (sinuses) are tiny indentations anterior to the helix and superior to the tragus. While pre-auricular pits are more common on the right side, they are bilateral in 25% to 50% of cases.25 Pre-auricular pits occur in up to 1% of white children, 5% of black children, and 10% of Asian children.25 Children with this condition should undergo formal audiologic testing as their risk for hearing loss is higher compared with the general population.26
The branchio-oto-renal syndrome (associated with pre-auricular pits and hearing loss) also features structural defects of the ear, renal anomalies and/or nasolacrimal duct stenosis or fistulas. If this syndrome is suspected, renal ultrasound imaging is warranted. Other indications for renal ultrasound in patients with a pre-auricular pit are any dysmorphic feature, a family history of deafness, an auricular malformation, or a maternal history of gestational diabetes.27 Pre-auricular pits do not require surgery unless they drain chronically or become recurrently infected. Complete surgical excision is the treatment of choice in these cases.
Mark Stephens, MD, 1850 Park Avenue, State College, PA 16801; [email protected]