Recently I had to counsel a young couple who just had the birth of their first child. Their baby was noted to have an abnormal ear on one side. Otherwise the baby was healthy and had no other medical problems.
The news of having a newborn baby with an abnormality is devastating for the parents. It is a normal reaction as one tries to cope with the fact that there is ‘something wrong’. It often takes time for the parents as well as the attending doctor to help the parents to accept the condition of their baby.
What is microtia?
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Microtia is a condition when there is abnormal development of the outer ear that results in a small or underdeveloped pinna. It is a congenital condition that occur one in every 6,000 births. It occurs more often in boys than girls. It typically involves one ear but can occur in both ears. Patients may have narrowed or absent ear canal (canal atresia).
The hearing of the affected ear is reduced because of the abnormal ear canal. Usually the inner ear (cochlea) is normal. Hearing rehabilitation is important if both ears are involved.
What assessments are necessary at birth?
Microtia is not a life threatening condition. However it can be feature of a syndrome such as Goldenhar syndrome, hemifacial microsomia or Treacher Collins syndrome. The pediatrician will normally assess for any syndromic features and associated anomalies soon after birth.
The hearing of the affected ear is usually reduced because the structures in the middle ear can be affected as well. In children with unilateral microtia, hearing in the other ear is usually normal. However a hearing test must be done to confirm this. A baby with normal hearing in only one ear will usually develop speech and language as other babies with no hearing problems.
If both ears are affected, a hearing aid is fitted as early as possible. A bone conduction hearing aid is recommended and it is often worn on a headband in a very young child.
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What is the management plan for the child?
If the initial assessments done at birth show that the microtia is an isolated problem and that hearing issues are resolved then the child’s developmental milestones should be normal.
A computed scan of the temporal bone (ear) is usually done when the child reaches 4-5 years old. This scan will show the status of the middle ear structures such as the ossicles and the size of the middle ear cavity. It will also show if there is cholesteatoma present. Cholesteatoma is accumulation of dead skin trapped within the absent ear canal. If cholesteatoma exists, the ENT surgeon would perform an ear exploration surgery to remove the cholesteatoma.
When is ear cosmetic surgery considered?
Ear (pinna) reconstruction is considered when the child is of school-going age. The child’s ear reaches adult size around the age of 6 years. Any surgery performed before this age will result in mismatched ears.
There are a few treatment options available for ear cosmetic surgery:
- Ear reconstruction using rib graft
The pinna can be reconstructed using the patient’s rib cartilage as a graft. The rib cartilage is harvested and carved into the shape of the pinna before implanting under the skin. This surgery is usually performed at an older age around 10 years old to ensure adequate rib cartilage size. Ear reconstruction surgery is done in stages with 3 to 6 months apart between each procedure.
- Prosthetic ears
The child can be fitted with false ear which can be fixed onto the skull through implants and fixtures. This surgery can be performed when the child is around 7 years old. The false ear is sculptured from a silicone material and colored according to the patient’s skin tone. The surgery is usually performed in 2 stages.
Is there surgery to improve the hearing?
Surgery to improve hearing is performed after the pinna reconstruction surgery is completed. The canal reconstruction surgery involves drilling out the bone to form a new ear canal and lining the canal with split skin graft. The hearing outcome of this surgery depends on the condition of the middle and inner ear structures. The ENT surgeon would advise if the patient is suitable for this surgery based on the CT scan images.
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