Congenital atresia is the maldevelopment or non-development of the ear canal. It may occur on its own or associated with other abnormalities of 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 more commonly on the right side.
The main problem with the development problems of the ear canal is the hearing problem associated with it. It has been found that 30% of children with a one sided hearing loss will fail one grade in their 12 years of school. Therefore, it is greatly important to assess children with a congenital atresia of the ear for hearing issues and to monitor them closely in school.
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. There are 2 options for repair of the outside of the ear.
1. A 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).
2. Reconstruction of the ear using cartilage. Reconstruction of the ear using rib cartilage generally requires 3-4 procedures over several months to reach its final stage.
There are 3 options for hearing rehabilitation for children with ear canal development problems:
1. 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 a surgery.
2. BAHA. This device is also a bone conduction device but it gets anchored to the head using a titanium screw. Click here for more information about the BAHA device. This device will give the best hearing result. The device is FDA approved for children 5 years of age or older.
3. Reconstruction of the ear canal (congenital atresia repair). This surgical procedure reopens the ear canal and lines the new ear canal with skin (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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