(Non-Development of the Ear
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
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
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
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
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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Appointment with Dr. Djalilian, Please Call
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