Road Map for Microtia/Atresia Care
It can be difficult to navigate the diagnostic and therapeutic plans for microtia and aural atresia. And to add even more complexity, every child is unique.
We seek to provide a comprehensive plan for you and your child with microtia and aural atresia. The years shown represent guidelines only...it is never too late to make forward progress on the road for your microtia and aural atresia care.
Congratulations! The birth or adoption of your child is an occasion for great celebration. We can work through the microtia road map, soon. But first, celebrate your new baby, knowing that children with microtia are beautiful, intelligent, and resilient.
It is often helpful and relieving to parents to meet and to discuss this road map for microtia and aural atresia. It can be confusing and daunting at first. This meeting starts the process of getting to know each other and setting up a care plan for your child.
Microtia comes in four grades of severity. Dr. Jabbour will grade your child's microtia, look for other associated anomalies, and order any diagnostic tests that are required.
It is important to assess a child's inner ear hearing with an auditory brainstem response (ABR). In infants, this can often be done while napping. The heart and the kidneys develop at the same time of the ear, and we have demonstrated that it is important to assess their structure by ultrasound (see research article). It is not required, but can be helpful, to consider a visit with a geneticist, who can also carefully assess if your child's microtia and/or aural atresia is an isolated anomaly or associated with a known craniofacial syndrome.
At six months of age, your child is beginning to be strong enough to sit up without assistance. This is a good time to initiate use of a bone conduction hearing aid, to allow sound to be processed through the affected inner ear. At UPMC Children's Hospital of Pittsburgh, we most often use BAHA devices.
We will get to know each other well, over the years. Dr. Jabbour will see your child every year to assess hearing, speech, and the health and safety of the unaffected ear (for children with single-sided microtia). We typically wait until about 5 years of age to obtain a CT scan to assess the anatomy of the affected ear and to assess for candidacy for aural atresia surgery (making and ear canal and ear drum).
In preparation for the first stage of surgery, it is helpful to make a 3D printed model of the ear that we seek to reconstruct. For patients with only one side affected, a mirror image of a 3D model of the unaffected ear is made and surgical cutting guides are custom designed by Dr. Jabbour. These are sterilizable and used in the first stage of surgery, which involves using the cartilage portions of the 6th, 7th, and 8th rib to sculpt the shape of the ear to match the 3D model and to insert it in the desired position for the ear, to achieve symmetry to the face.
For children with both ears affected, a model ear can be chosen as a target for the first side. The completed first side will serve as a model for the second side.
The goal of the second stage of surgery is to provide projection to the ear away from the head and to provide room for glasses. This is accomplished by elevating the cartilage, advancing the skin behind the ear, and placing a full thickness graft to line the back of the ear. The cartilage of the ear is supported by a wedge of porous polyethylene, called Su-Por. This is an excellent time to place a BAHA or Osia bone conduction hearing device, as we have excellent access for placement without the need for any additional incisions or surgeries.
For those who are excellent candidates for atresia repair, consideration can be given to a subsequent operation to open an ear canal and to make an ear drum. The ear drum is made from a small piece of lining tissue of muscle, called fascia. The ear canal and functional ear drum are lined with a split thickness skin graft from the leg or upper arm.
The decision of whether to pursue atresia repair or to use a bone conduction hearing device can be a difficult one; Dr. Jabbour will discuss the pros and cons of each with you, in detail, so that you can make an informed decision.
