External auditory canal atresia (EACA) in children

What is external auditory canal atresia (EACA) in children?

EAC is typically discovered at birth during the newborn examination, but occurs during the first trimester. A genetic cause has been identified in some patients.

EACA is a feature of several syndromes including:

• Treacher-Collins

• Hemifacial Microsomia

• Goldenhar Syndrome

How is external auditory canal atresia (EACA) in children diagnosed?

Patients with EAC atresia are typically referred by their primary care doctor to an otolaryngologist (ENT specialist).

• A complete history and physical exam typically reveals microtia and atresia.

• A CT scan is often obtained once a child is 3 to 4 years old to evaluate the patient’s temporal bone anatomy.

How is external auditory canal atresia (EACA) in children treated?

Atresiaplasty (canaloplasty)

This involves the creation of a new ear canal and ear drum with possible reconstruction of the ear bones. This procedure is performed no sooner than 4 to 5 years of age unless a patient has bilateral atresia. Management of the new ear canal after surgery can be challenging in younger patients secondary to cooperation issues. 

Most surgeons prefer to address the malformed external ear prior to performing an atresiaplasty. Performing an atresiaplasty prior to repairing the external ear is sometimes preferred by surgeons who use an implant as opposed to rib cartilage