Information about Microtia Atresia

As was mentioned in the previous post, We do not want Clara Rose to define herself by her ears—they are a part of who she is but not all that she is—and this is true for all of us.

There is much debate in the Special Needs adoption community about whether or not to share information about an adopted child having a special need, especially if it isn’t visible. Some parents view it as a matter of protecting their child’s privacy. We, Wim and I, understand and respect this reasoning but we have different beliefs. We believe that taking this approach will also run the risk of a child feeling shame for their medical condition—that it is a secret to be kept and not shared because something is wrong.

Nothing is wrong with Clara Rose. Her ears are different from most of our ears, but when you think about it don’t we all have different ears, different noses, etc.

Wim and I recognize that there is likely some curiousity among family and friends about Microtia Atresai. It certainly sounds different—neither of us had ever heard of it until we started researching medical conditions. During this research we learned from other parents that this is very easy to handle and is likely why folks don’t hear too much about it. It is too much of a non-issue that it isn’t really talked about. We are and will most likely continue to be happy to answer questions from folks, but don’t expect us to dwell upon it. As all parents know, there is much more about kids to focus on—like all the fun things they do to keep us hip and cool, and most definitely—on our toes!

This post contains information about Microtia Atresia for those who want to know more, causes, what is it, treatment, and a few pictures from the web. In terms of treatment, we aren’t sure at this point what we will do. At this point, I think I prefer the rib graft procedure described below, but Clara Rose will most likely be involved in making this decision when she a little bit older (8-10 years old). Some people may feel that is too big of a decision for a young child to make, but they are her ears after all so she should have some say in the whole process.

Clara Rose has bilateral Microtia Atresia, meaning the condition affects both of her ears. One ear more so than the other ear. Her Right ear is Stage 3. We are not sure if her left ear is Stage 1 or Stage 2, but she does have an outer ear canal for that ear. We do not know if it is narrow or not. We do not have readable results from a hearing test, but there were some comments in her growth report indicating that she has some hearing.

Obviously there are some unknowns—as with the addition of any child to a family, whether by birth, adoption, or step kids through marriage.

What causes Microtia Atresia?

The occurrence of microtia is usually a random, sporadic event, and it is not caused by anything the mother did before or during the pregnancy.

Researchers theorize that Microtia is not hereditary but is caused by tissue ischemia (decreased blood flow) resulting from an obliterated artery during fetus development One doctor notes that s/he has treated 10-12 patients with microtia who have a proven, identical twin with non-malformed ears.

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 (Microtia) and middle ear also, but the inner ear and auditory nerve are frequently normal. 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), but can occur both ears (bilateral).Atresia can be a symptom of a larger syndrome, such as Treacher Collins, Crouzon's, Alpert's, Preiffer, Klippel-Feil, BOR (Branchio-Oto-Renal), 18-q chromosome, as well as Hemifacial Microsomia.What is Aural Atresia? - http://www.microtia.com/whatis.html The Microtia And Aural Atresia Division of the IRPS - http://www.irps.net/microtiainfo.html EMedicine – Aural Atresia - http://author.emedicine.com/ped/topic167.htm

What is Microtia?

Microtia literall translates from the Latin to mean “small ear” Microtia varies from the complete absence of the ear (which is referred to as anotia) to a somewhat normal but small ear. Visit http://www.earsurgery.com/pages/variations.html to see good pictures of the various grades of microtia (Figure 5).Microtia Statistics:• Nearly twice as frequent in males as in females• Averages occurrence is 1 in 6,000 when averaged across all ethnic groups• Approximately 60 % unilateral right, 30 % unilateral left, 10 % bilateral
Microtia (meaning 'Small ear') is a congenital deformity of the pinna (outer ear). It can be unilateral (one side only) or bilateral (affecting both sides). It occurs in 1 out of about 8,000-10,000 births. In unilateral microtia, the right ear is most typically affected.

There are four grades of microtia [2]:

Grade I: A slightly small ear with identifiable structures and a small but present external ear canal

Grade II: A partial or hemi-ear with a closed off or stenotic external ear canal producing a conductive hearing loss

Grade III: Absence of the external ear with a small peanut vestige structure and an absence of the external ear canal and ear drum

Grade IV: Absence of the total ear or anotia.
Treatment

Grade III is most common, and can be corrected by surgery. Typically, testing is first done to determine if the inner ear is intact and hearing is normal. If hearing is normal, the next step (if a canal is not visible externally) will be to determine if a canal exists, by CT scan. For younger patients, this is done under sedation. Age when outer ear surgery can be attempted depends on the technique chosen (see below). The earliest age surgery can be attempted is age 3 for Medpor and 6 for Rib Cartilage Grafts. However the vast majority of surgeons, including the world experts in ear reconstruction recommend waiting until a later age, such as 8-10 when the ear is full adult size.

Options

There are two separate issues in microtia surgery:

Auricular reconstruction to restore the visual appearance and form of the outer ear
Repair of atresia or application of a bone-anchored hearing aid BAHA to restore hearing.
Unilateral deafness is not generally considered a serious disability, especially when the person is able to adjust to it from birth. In general, there is little to gain from an intervention to enable hearing in the microtic ear, except in bilateral microtia. However, children with untreated unilateral hearing loss are eight to ten times more likely to have to repeat a grade in school.[citation needed] If surgery or aids are not used, special steps should be taken to ensure that the child is accessing and understanding all of the verbal information presented in school settings. Age for BAHA implantation depends on whether you are in Europe (18 months) or the US (age 5).[citation needed] If the child is under the age for surgical implantation, the BAHA can be worn on a headband

For auricular reconstruction, there are four different options:

1. Rib Cartilage Graft Reconstruction: This surgery should be performed only by specialists in the technique.[citation needed] It involves sculpting the patient's own rib cartilage into the form of an ear. Although the cartilage is the patient's own living tissue, the reconstructed ear does not continue to grow as the child does. The procedure is therefore usually performed after 8 years of age, one reason being to ensure that the rib cage is large enough to provide the donor material necessary, but also so that the opposite (normal) ear has reached almost full adult size. This is a two-stage surgery. The major advantage of this surgery is that the patient's own tissue is used for the reconstruction.

2. Reconstruct the ear using a polyethylene plastic implant: This is a two-stage surgery that can start around age 3. This surgery should only be performed by experts in the techniques involved as complications are difficult to correct.[citation needed]

3. Ear Prosthesis: This surgery is ideally suited to those who are awaiting rib cartilage reconstruction (adhesive prosthesis) or in whom other techniques have failed (bone anchored prosthesis or adhesive prosthesis). A craniofacial prosthesis or auricular (ear) prosthesis is custom made by an anaplastologist to mirror the other ear. Prosthetic ears can appear very realistic. They require a few minutes of daily care. They are typically made of silicone, which is colored to match the patient's skin and can be attached using either adhesive or with titanium screws inserted into the skull to which the prosthetic is attached with a magnetic or bar/clip type system. These screws are the same as the BAHA (bone anchored hearing aid) screws and can be placed simultaneously. The optimal age to begin wearing an ear prosthesis is between the age of 6 and 9. The child should be mature enough to want the prosthesis and to help care for it.[1] A person with grade I & II microtia need not consider an ear prosthesis. A person with grade IV (anotia) is best suited for an ear prosthesis. A person with grade III construction has three options for reconstruction.

1. Leave the skin tags for future surgical reconstructive purposes and make an adhesive retained prosthesis over the top of the existing ear. The advantage is that you can keep your reconstructive options open and you get more definition in the ear than a surgical approach. This allows you to try out the prosthetic approach without burning any bridges. It is also the least expensive approach. The disadvantage is that the ear is typically placed lower and more forward and tends to appear more bulky than the other ear. It will give you a good outline though.

Placement is more difficult than prosthetics option '2'. If you like the look of the prosthesis and want to make it more permanent, you can proceed with option '3'.

2. Remove the skin tags and use an adhesive retained ear. The advantage over prosthetic option '1' is that your prosthetic ear can be a near identical mirror image of your other ear. Placement is much easier as well because the skin is flat. The disadvantage is that options for surgical reconstruction are reduced by removing the skin tags.

3. Remove the skin tags and simultaneously place the implants for a prosthesis. The visual results are very similar to prosthetic option '2'. The advantage is that placement of the ear becomes even easier and you eliminate the costs of adhesive and the associated daily care. The disadvantage is again that you limit your surgical reconstructive options significantly in placing the titanium screws.

4. Soft tissue reconstruction: The reasoning for soft tissue reconstruction is that surgically reconstructed ears using rib-graft or implants are sensitive, though the sensation is not the same as for normal ears due to the transplant of skin from other areas of the body.

Classifications of Microtia:
Microtia is divided into separate classifications (the classic microtia is considered Grade III).

Anotia
Anotia technically means no ear. This ear deformity, the most severe form of microtia may at times be called anotia.

Grade 1 Microtia
The ear is smaller than normal although most of the features of a normal ear such as a well-defined lobule, helix and anti-helix are present. This can occur with or without an external auditory canal.

Grade 2 Microtia
The normal features of the ear are missing. There is still a lobule and a remnant of helix and antihelix.

Grade 3 Microtia
"The Classic Microtia": The ear consists of a vertical skin appendage with a malformed lobule (earlobe) on the lower end. There is usually firm tissue at the upper end which is made up of a disorganized cartilaginous vestige. The lower end is usually a piece of lobular tissue which will be the future earlobe when reconstructed. Usually there is no external auditory canal (atresia).