Microtia – Frequently Asked Questions

1. What is microtia?
Microtia is a birth deformity of the ear where the outer ear and the middle are do not fully develop. The word microtia comes from Latin words micro and otia, meaning ‘little ear.’

2. How common is it?
Microtia occurs roughly once in 7,500 births in the United States, but ranges wildly depending on the ethnic group in question. Generally speaking, the occurrence is more often in Hispanics and Asians than in African-Americans and Caucasians. In addition, some indigenous groups in Mexico, the US, and South America have higher rates of occurrence, around one in 1,000 births. In the Japanese/Korean communities the rate has been about one in 2,100. In the overall Caucasian community the rate is only about one in 20,000.

3. How common is it to have one versus both ears affected?
In 90% of microtia, only one ear is affected, and right-sided microtia is twice as common as left-sided microtia. Microtia affects both ears only in 10% of the cases.

4. Any differences between genders?
About 65% of the microtia cases involve boys, 35% girls.

5. What does microtia look like and what are the levels of severity?
There are four grades of microtia:

• Grade 1: Minimal deformity, only a slightly smaller than normal ear and ear canal.
• Grade 2: A partial, miniature ear with a closed off ear canal producing some hearing loss.
• Grade 3: Only a small peanut-like trace of an external ear and an absence of the ear canal and ear drum. This type of microtia is most common, and can be corrected by surgery
• Grade 4: Complete absence of the ear, also called anotia, which is Latin for ‘no ear.’

6. How is hearing affected?
Most children with microtia in one ear hear normally with the unaffected ear. Typically, hearing with the Grade 3 microtia ear is lessened by 50%, feeling as if the ear canal was filled with wax.

7. What is atresia?
Atresia is a condition in which a passage in the body is abnormally closed or absent. In the case of the ear, atresia is the absence or underdevelopment of the ear canal and other middle ear structures. Most of the time, microtia is accompanied by atresia. This is because the outer ear and the middle ear develop from one common block of tissue simultaneously while the child is growing in the womb, at 6-10 weeks of age.

8. Is microtia inherited?
Yes, it can be inherited, but not always. The chances of having a child with microtia are higher if there is microtia in the immediate family of the parents. However, fewer than 15% of the microtia cases have a positive family history. When two people with no microtia in the family history have a child, the risk of that child’s having microtia is the same as any child born—one in about 7,500. Then, if the child born has microtia, the risk of the subsequent children of the same parents having microtia jumps up to one in 25. Also, when a person with microtia conceives a child, that child has a about one in 25 chance of having microtia. In conclusion, if there is microtia in the immediate family (parent, sibling, grandparents, aunts, uncles), the chances jump from roughly one in 7,500 to roughly one in 25.

9. Are there any other medical problems associated with microtia?
Most microtia patients have no other abnormalities. Nevertheless, it is fairly common for microtia to be associated with other congenital abnormalities, especially on the face. About a quarter of microtia patients have underdeveloped jaws and overlying soft tissues on the microtia side of the face, making that side appear flattened or distorted. The condition is called hemifacial microsomia and comes in varying degrees. In its weakest form, hemifacial microsomia is barely noticeable. However, in its full genetic expression, it includes defects on the external and middle ear, underdeveloped upper and lower jaws, cheekbone, and temporal bones, mouth lengthened on one side, facial nerve weakness, and atrophy of facial muscles. In most cases, hemifacial microsomia can be corrected with either jaw surgery or orthodontics.

Roughly 10% of my microtia cases may eventually need jaw surgery, but most mild cases of hemifacial microsomia can be corrected with orthodontics.

10. How does the child become aware of microtia?
Normally, the child starts comparing sides in the mirror or a photo, noting a ‘little ear’ as early as at three years old. However, most children do not identify the small ear as a problem until they go to school or kindergarten.

11. How should the parents talk to the child about microtia?
Once the child discovers the little ear, parents can say that the child was born that way, and that the little ear can be repaired by the doctor. It goes a long way to relieve any anxiety. It is also important for the parents not to make a big deal out of the little ear, for family anxieties are easily transmitted to the child. Trying to cover the affected ear with hair or hats only makes the child feel insecure about it. It is best to let the little ear be as it is, exposed like a fully developed ear, and let the child know that doctor is going to repair it to make it like the other ear when he/she is old enough. When the child knows that the ear will be repaired later, it is easier to adjust.

12. What is the best age to have microtia repair surgery?
The optimal age to have surgery varies, because it is a matter of balancing rib growth, ear growth, and the child’s psychological readiness.

First, as a surgeon, I need the child’s own rib cartilage to construct the ear. A normal child should weigh at least 40 pounds to have a rib cartilage developed enough so I can safely extract a small piece for the ear construction.

Second, our ears are 85%-90% grown at roughly five years of age, and it is most sensible to repair the microtia ear when it can be sized close to a fully grown ear. The first attempt to reconstruct an ear has the best chances to look like the other ear. Further attempts to alter or fix an already reconstructed ear are technically much more challenging and require a very experienced surgeon. As an exception, in cases where microtia includes both ears, I operate a little earlier. This is because there have been studies to show that the impaired hearing from microtia on both ears may hinder the child’s brain development.

Third, from the psychological perspective, if the child wants to have the surgery, the preparation, the various stages of the surgery, and post-operative care are all made easier for everyone involved. Staged surgical ear reconstruction takes months—in surgeries and recovery times—and it surely helps when the child embarks on the adventure with a sense of challenge and persistence, even heroism. In other words, if the child does not at least consent to surgery at five or six years of age, it may make sense to wait until the attitude changes and he/she is more co-operative.

Finally, completing the surgery before the child starts first grade makes it logistically easier not having to deal with absences and recovery periods during school year.

13. What kind of doctors fix microtia?
The best doctors to repair microtia are those that are qualified and experienced in fixing both the outer and the inner ears. Doctors in otolaryngology (board certified in otolaryngology) are best trained to work with the inner ear, the ear canal and the middle ear structures.

Plastic surgeons (board certified in plastic surgery) are best trained to construct the external ear. Therefore, your best choice is a surgeon with both of those qualifications. Nevertheless, many surgeons with only one or the other specialty training can still repair microtia.

14. Why is choosing the best doctor so important in microtia repair?
Because the first microtia repair surgery attempt is your best chance to get the best results. If you have to go back to another doctor to fix a poorly reconstructed ear, it takes even better than the best doctor to do a good job. You want someone experienced in not only carving out the cartilage and designing the ear, but someone who can do it quickly, efficiently, and effectively. It takes precise aesthetic sense and extreme dexterity to be able to carve out a three-dimensional structure out of a living tissue to match the patients other ear, a mirror image. In addition, the surgeon must be able insert this living tissue creation inside the patient’s stretched skin, place it properly in a short amount of time, having a good intuition during the operation regarding any individual adjustments needed. Once done, the surgery is unforgiving.

15. Is microtia surgery covered by insurance?
Microtia surgery is not considered a cosmetic surgery, but rather a reconstruction of a missing body part. It is almost always covered by medical insurance. Even reputable HMOs may allow the reconstruction by Dr. Jones if they recognize the limited number of surgeons qualified for these procedures. If you discuss the situation with your insurance carrier, please be sure to refer to the procedure as reconstructive, not cosmetic or even plastic, surgery. It is critical that you obtain your carrier’s pre-approval prior to the surgery. The process to gain approval can sometimes take several months, and we recommend you begin the approval process at least six months prior to the planned surgery date. Dr. Jones’s office staff will help you if you have difficulty. Different insurance carriers and insurance policies pay varying amounts toward the surgery, and it is only by direct inquiry to your carrier that you can determine their policy.

16. What is a plastic ear?
Ears can be constructed either from rib cartilage or plastic. The main difference and the associated advantages and disadvantages come from the fact that one ear is created from the patient’s own tissue (rib cartilage) and the other ear from artificial material, either silastic or medpore plastic. I do not use plastic to reconstruct ears because of the many complications associated with it.

17. Why do you prefer using rib cartilage over plastic for reconstructing ears?
Natural tissue, such as sculptured rib, has several advantages over plastic implants. First of all, natural tissue is acceptable to the patient under many circumstances where the body would reject an artificial ear. Plastic ear implants are notorious for not only rejections, but also for infections and healing problems. In addition, glue-on or snap-on artificial ears can be pulled off. Can you imagine the embarrassment of your child, if the class clown yanked off the ear, holding it up to the classmates? Due to the risk of the ear coming off, sports participation with artificial ears may be limited. On the other hand, the plastic ear is taken off every night and put on in the morning for the rest of the child’s life. It is a hassle. Once implanted, a rib graft ear retains some sensation on the skin, whereas a plastic ear has no sensation at all. Another advantage of an ear made from rib cartilage is that the natural tissue changes color in various situations. While a plastic ear may match the adjacent skin color well most of the time, it does not blush or flush as one’s face normally does when excited, cold, or embarrassed. Nor does an artificial ear suntan. In the end, while a plastic ear color may be matched well for photography, it becomes artificial looking in the real world due to its inability to change color naturally.

18. Is it possible to use somebody else’s—say the mother’s—or an animal’s rib cartilage? Since kidney, heart, and liver transplants are quite commonly used; why not cartilage transplants?
It is possible to use another person’s or even a mouse’s cartilage. However, the patient’s body would not accept that cartilage as his/her own, rejecting it as if it were a foreign intruder. To combat these foreign antigens (toxins) and what they might do in the body, I would need to put the patient on special drugs. These drugs have some serious side effects that are not reasonable unless the transplanted organ is vital for living.

19. What are skin tags, and is there something we should do for the deformed area prior to surgery?
Not really, though I would not recommend wrestling or boxing. These sports have high incidences of ear trauma.

20. Should the child limit sports activities after microtia surgery?
Not really, though I would not recommend wrestling or boxing. These sports have high incidences of ear trauma.

21. How much pain is there after the rib cartilage surgery?
The pain should be minimal, because I numb the patient with both short-term and long-term acting local anesthetics. This approach diminishes most of the chest pains immediately following the surgery and through the first post-operative day. The child is usually up and playing on the second day after surgery.

22. Should we expect complications after surgery?
Not really. Dr. Jones’s ear reconstruction patients have a complication rate less than 1%.

23. Is it possible to have the reconstructed ear pierced?
Yes, Dr. Jones can do the piercing during the last stage of the ear reconstruction. Please talk to him about this prior to the surgery, and he will discuss the details with you.

24. Does the reconstructed ear grow?
Yes, an ear constructed from the patient’s own rib cartilage becomes part of the person and grows with him/her. On the other hand, an ear made from artificial material or plastic does not grow.