Congenital Atresia

Congenital atresia is the maldevelopment or non-development of the ear canal.

It can either occur on its own or along with other abnormalities involving the head and neck. It most commonly occurs on one side, but can occur on both sides. When it does occur on one side, it is usually on the right.

The primary issue with ear canal development problems is that hearing is often affected. About 30 percent of children with one-sided hearing loss will fail one grade in their twelve years of school. It is important to assess children who have congenital atresia for any hearing issues. Their performance at school should be closely monitored.

Assessment

Once the child is diagnosed (usually at birth), a special hearing test (ABR) has to be performed to assess the function of the hearing organ of the inner ear (cochlea) in the malformed ear. The children with congenital atresia will often have a malformed outer ear (auricle) as well.

Treatment

There are two options for repair of the outside of the ear:

• Prosthetic ear. A prosthetic ear can be made as a mirror image of the opposite ear and anchored to the head (using titanium screws placed surgically).
• Ear reconstruction. Reconstruction of the ear using rib cartilage generally requires three to four procedures over several months to reach its final stage.

There are three options for hearing rehabilitation for children with ear canal development problems:

• Bone conduction hearing aid. This device is a hearing aid that sends the sounds to the ear by vibrating the bone behind the ear. The device comes in a head band or similar device that keeps the hearing aid tightly against the bone behind the ear. This device does not require surgery.
• Bone-anchored hearing aid (BAHA). The BAHA is also a bone conduction device, but it is anchored to the head using a titanium screw. This device will give the best hearing result. BAHA is FDA approved for children five years of age or older.
• Canal reconstruction. This surgical procedure reopens the ear canal and lines the new ear canal with skin that is taken from the leg. A CT scan of the temporal bone (bone surrounding the ear) is required to assess the anatomy of the ear prior to making a decision on surgery.
  
  The hearing results in this procedure will never be quite perfect since children with congenital atresia of the ear will not have an ear drum (tympanic membrane) and the small bones behind the ear drum (see anatomy), will not be normal.
  
  Therefore the normal sound conduction mechanism does not function perfectly even if the ear canal is opened. The main risk of this procedure is that the ear canal may close at some point in the child's lifetime as a result of an infection or inflammation in the ear.

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