621 S. New Ballas Rd, Tower A, Ste 281
St Louis, MO 63141
Tel. 314-251-4772  Fax 314-251-5772

Other Congenital Ear Problems

Ear deformities can be mild or severe. For more severe deformities, such as microtia & anotia, ear reconstruction surgery is generally needed to correct your child's ear deformity and help them regain their self-confidence.

Overview

Congenital ear deformities range from mild to severe.  Mild deformities generally result in minor problems with the shape of the external ear.  If detected in the first days of life, mild deformities may be correctable without surgery, using ear molding techniques.  We are proud to offer infant ear molding using the EarWell system.  If minor ear shape abnormalities are noticed after a child is 4-6 weeks old, surgery may be recommended when your child is in grade school to reshape the ear and give it a more form. 

When there is a significant deformity or complete absence of the external ear, this is referred to as microtia (small, malformed ear) or anotia (absence of the ear).  Correction of microtia/anotia generally requires surgery. 

Classification of microtia

Although there are various grading systems for microtia, one of the most commonly used systems grades microtia on a scale of 1-4, with grade 1 being the mildest form and grade 4 being the most severe.

  • Grade 1:  The external ear is small and the auricle retains most of its normal structure. The external auditory meatus is usually present.
  • Grade 2:  The external ear is moderately anomalous. The auricle can be hook-, S-, or question mark shaped in appearance.
  • Grade 3:  The external is a rudimentary soft tissue structure with no cartilage; the auricle does not have a normal appearance.
  • Grade 4:  Anotia, or complete lack of the external ear.

In the more severe forms of microtia, there is usually absence of the ear canal (also called atresia) and some degree of conductive hearing loss.  Depending on the type and severity of hearing loss, a conductive hearing aid may be recommended for your child.

Incidence of microtia/anotia

Microtia occurs in approximately 1 in 8,000 babies, even when the parents do everything right.  It occurs more commonly in boys.  The vast majority of cases (70-80%) affect only one side.  The right side is affected more often than the left.

Cause of microtia/anotia

Nobody can say with certainty what causes microtia.  Most experts believe that there may be a disturbance in blood flow to the developing ear at around 6 weeks gestation, while the baby is still in the womb.  Research has also shown that there is an association between microtia and exposure to certain medications, including thalidomide and accutane.  There is also likely a genetic component.  Microtia is also observed in certain craniofacial conditions and syndromes, such as hemifacial microsomia, Nager variant and Treacher Collins syndrome.

Treatment options for microtia/anotia

There are 3 main options for treating microtia/anotia:

1.  Ear reconstruction using your child’s own cartilage:  This reconstructive technique, also called autologous reconstruction, is a 2-3 stage process.  In the first stage, the abnormal ear cartilage is removed and your child’s rib cartilage is used to build a new cartilage framework for the ear.  This framework is then implanted beneath the skin.  The earlobe, which is often not in its correct place, may also be rotated into a more normal position at this first stage.  In the second stage, which takes place several months later, the ear framework which was buried under the scalp, is elevated, and a skin graft is used to line the space behind the ear.  This stage creates a posterior ear sulcus (the normal space behind the ear) and helps the ear project or “stick-out”, the way that it should.  In the third and final stage, a small hollow is created where the ear canal normally sits.  This surgery does not create a new ear canal, only the appearance of one.  The total time from beginning the process to completion is 6-9 months.

Ear reconstruction using your child’s cartilage is usually not done until a child reaches 6-7 years of age.  This allows for your child to grow big enough to ensure that there will be enough rib cartilage to use to build your child’s ear.  It also allows your child to mature enough that he or she can participate in the process. 

Advantages of this reconstructive technique include:

  • Durability – Your child’s new ear should last a life-time.
  • Low infection risk – Since your child’s ear is made from his or her own tissue, it is more resistant to infection and other problems than plastic ear frameworks.
  • Good to excellent appearance – The ultimate appearance will depend on your surgeon’s experience.

Disadvantages of this technique:

  • A second surgical site – The need to remove cartilage requires a second incision and may result in some subtle contour irregularities of the chest where the cartilage is taken.
  • More post-surgical discomfort – This relates to the need to remove cartilage.

 2.  Ear reconstruction using a synthetic, plastic framework:  This reconstructive technique uses a pre-made ear framework made from porous polyethylene (Medpor) instead of a framework made from your child’s own cartilage.  The framework is covered by skin and temporoparietal fascia, the tough layer of tissue overlying the temporalis muscle.  Although creation of the framework is made much easier by using a synthetic frame, covering the framework with fascia and skin may be more challenging.  A second stage is later required to elevate the ear.  Total time from beginning to completion is also approximately 6 months.

Ear reconstruction using Medpor implants is generally done around the same age as cartilage reconstruction.  Patient maturity is the most important consideration.

Advantages of this reconstructive technique include:

  • Good to excellent appearance – Since the frameworks are pre-fabricated, they require no skill on the part of the surgeon to create.
  • No second surgical site – This likely means there will be less discomfort following surgery.
  • Fewer surgeries (possibly) – Although, in theory, there should be fewer surgeries required to do a MedPor ear reconstruction, this is only true if there are no complications.  Complication rates are higher, however, with this type of reconstruction.

Disadvantages of this technique:

  • Higher complication rate – Plastic implants are more likely to erode through the skin and become exposed.  Exposure may the result of trauma or injury or may occur simply with normal “wear”.  When this occurs, the implant becomes infected and must be removed.
  • Lifelong risk of infection – A synthetic implant never becomes “part of you” as does your own tissue.  Your body recognizes the MedPor framework as foreign and creates a capsule around it.  Since it is not fully integrated into your child, it may become infected if your child develops a minor infection elsewhere, such as a skin abscess or even a tooth infection.  This risk never goes away, no matter how long ago your child’s ear reconstruction was done.
  • Not likely to last a lifetime – MedPor reconstructions are not as durable as autologous reconstructions and are unlikely to last throughout a child’s whole life.  This means that your child is likely to require another reconstructive surgery at some point.  With each surgery, the reconstruction is more difficult and the results are likely not as good.
  • A larger scar on the scalp – The incision needed to harvest the temporoparietal fascia is larger and may be visible, especially if your child keeps a short hair style.

3.  Ear prosthesis:  Some families choose not to undergo a multi-stage surgery to reconstruct the ear, opting instead for a prosthetic ear.  Ear prosthetics are synthetic ears that are removable.  They are typically “snapped-on” to a metallic post that is implanted in the skull.

Advantages of this reconstructive technique include:

  • No additional surgical sites or scars are required.
  • Appearance in this technique can be excellent.

Disadvantages of this technique:

  • Your child will never feel that the ear is part of them.
  • The ear will need to be removed and put back on at nights.
  • There may be color mismatch if your child tans in the summer of as the prosthetic ages.
  • Need for replacement - If the ear is lost or worn-out, it will need to be replaced.  This results in a recurring expense over time.
  • Potential for healing problems where the metallic post is anchored.

For the reasons detailed above, it is our preference to do cartilage reconstructions in the children we treat.  With this technique, our patients get excellent results that are durable with the least long-term risk and the least need for repeat surgeries or fabrication of new ears.