What is Aural Atresia / Canal Stenosis
Aural atresia refers to the absence an external ear canal. When someone has aural atresia, there is a high incidence of malformation of the external ear and middle ear also, but the inner ear and auditory nerve are frequently normal. It is important not to forget the "normal" ear in patients who have one-sided Atresia as approximately 25% of these individuals may have a hearing loss in their best side also.
A narrowed ear canal (i.e. one where the eardrum can be viewed, but the canal is narrower than normal) is sometimes referred to as a stenotic canal, or canal stenosis.
Aural atresia most commonly effects just one ear (unilateral aural atresia), but can occur both ears (bilateral aural atresia).
Atresia is most frequently isolated, but can be a symptom of a larger syndrome, such as Treacher Collins, Goldenhar, Crouzon's, Alpert's, Pfeiffer, Klippel-Feil, BOR (Branchio-Oto-Renal) also known as Melnick-Fraser, 18-q chromosome, as well as Hemifacial Microsomia.
The surgery to create a normal sized ear canal from either a stenotic canal or complete aural atresia is known as an "atresia repair" and is sometimes also referred to as a canalplasty.