Mace has been diagnosed with Bilateral Microtia Atresia. A real mouthful and we can imagine that you have never heard of it. Just like Mace’s parents. In the meantime they have learned a lot about it thanks to the doctors and several supportgroups, and they are aware of what has to be done to help Mace get is ears. But first a short explanation on Mace’s condition.
Microtia is a congenital deformity. The word microtia comes from Greek and literaly means ‘small ear’ (micro = small; ous = ear). The ear is smaller, more shallow, folded or even absent. Microtia occurs in 1 of about 8,000-10,000 births. Usually (in 90% of the cases) this occurs on one side (unilateral) but sometimes both ears are affected. There are more boys than girls with microtia. There are four grades used to determine the severity of microtia.
The exact cause of microtia is still unknown. Studies have revealed that the problem which leads to microtia occurs in the 8th week of the pregnancy. Microtia commonly occurs in combination with atresia because the ear canal and the outer ear are developed together during pregnancy. If there is a problem during this development, both the outer ear as well as the ear canal are affected.
Microtia is most of the time not inheritable. When one child or parent in a family has the deformity, the odds of having another child with microtia is increased by 5%. When 2 of the current children have microtia, the odds are increased by 15%.
A human is unable to hear perfectly without an ear or ear canal. This is the case with most children who have microtia. The ear canal, eardrum, and the inner ear with the ossicles are not intact therefore normal hearing is impossible. The cochlea is present in most cases and fully functional. This is the part of the inner ear where sound waves are converted to a nerve signal, which eventually travels to the brain. This makes it possible to rehabilitate the hearing by means of a so-called bone implant with a bone conducting hearing aid.
Atresia means absent. This indicates that the ear canal is not present. So there is no entrance for sound traveling towards the inner ear. However, the cochlea and middle ear may be present.
Bilateral means two-sided or mutually binding. In addition, it is a term from the anatomy to indicate that something is present in two body parts.
What is the situation with Mace?
Mace has no outer ear (pinna) and there are small cartilage remains. Through an audiological examination with Mace, it was determined that he has at least one working cochlea and suffers 60db hearing loss. Mace therefore has no ears and cannot perceive speech.
On the pictures you can see Mace wearing a softband with a BAHA (Bone Anchored Hearing Aid) attached to it. The BAHA ensures that sound reaches the inner ear through bone conduction and he can hear sound. When his family communicates with mace they use sign language to support their speech. This will stimulate Mace to learn and use both ways of communication.
Ears for Mace
We really want to give Mace ears. THis may sound simple but it definitely is not. In addition, the surgery is very expensive. This is the reason we started this foundation. You can read what it takes to help Mace with ears on the page: Medical procedure. Do you want to help us get this done? All help is very much appreciated. You can help Mace by donating. Each donation is a step closer to our goal.