If your child was born with microtia, you may be struggling to understand why. How did this condition develop? Is microtia genetic? Did you do something to cause your child’s condition? Although scientists still have a lot to learn about the causes of microtia, Beverly Hills specialists Dr. Youssef Tahiri and Dr. John Reinisch want parents to know that this rare condition is not their fault. As researchers work diligently to learn more about the underlying causes of microtia, there is still a large amount of information available about the condition. Parents can help their children by learning more about microtia, its effects, and the various treatment options available. To speak to a microtia expert and explore various state-of-the-art treatments, contact Dr. Tahiri and Dr. Reinisch today.
Definition
Microtia literally means small ear. Children with this rare condition have a small, abnormally-shaped or absent external ear. Most children have this condition only on one side (unilateral), however it can occur on both sides (bilateral). Typically, microtia only involves the outer ear, but can also cause difficulty hearing and determining the direction of sound. In some cases, a child with microtia will have no hearing in the affected ear, due to the absence of the ear canal. This is called aural atresia. A child with microtia and aural atresia in both ears will be significantly hard of hearing and require a hearing aid. Children with aural atresia typically also have incomplete formation of the small middle ear bones and may lack an eardrum.
Dr. Tahiri and Dr. Reinisch specialize in microtia surgery for children. Patients from all over the USA and the world visit their Beverly Hills office to discuss microtia and surgical treatment.
Understanding Microtia
Microtia is a congenital condition, which causes a child to be born with a small, malformed, or missing ear. For 90 percent of microtia patients, the condition only affects one ear. Because microtia is so rare, it is rarely detected on prenatal ultrasounds. The condition can also come as a surprise to parents because children with microtia rarely have a family history of the irregularity. Microtia is especially rare in the U.S., where only 1 in every 8,000-10,000 children is born with this condition each year. Incidents of microtia are higher in other countries, however, particularly in Central and South America, and China sees more instances of microtia than anywhere else in the world. Dr. Tahiri and Dr. Reinisch see patients from around the world. Their institute is globally recognized, thanks to their innovations and advanced surgery for aesthetic correction and hearing restoration.
Stages of Microtia
There are four stages, or grades, of microtia:
Dr. Tahiri and Dr. Reinisch are renowned for their ability to treat all four stages of microtia.
Effects of Microtia
Children with all stages of microtia may face harassment and bullying, particularly at school. However, the most obvious difficulty for microtia patients is the decreased hearing ability resulting from a narrowed or missing ear canal. Although patients can still hear out of the unaffected ear, diminished hearing can make it difficult to detect individual sounds in noisy environments. When a patient can only hear on one side, the brain has difficulty filtering out background noise and determining which direction noises are coming from. This diminished hearing can cause trouble for patients, especially in school and social settings. In some cases, it can also lead to speech impairment.
Learn more about treatment options
Fortunately, Dr. Tahiri and Dr. Reinisch offer advanced treatment for both the cosmetic problems and the hearing difficulties associated with microtia. Their innovative MEDPOR® (or OMNIPORE®) technique allows them to create an external ear structure using a child’s own tissues – a procedure which offers a number of benefits. They also works with an otologist to enhance hearing and, if possible, create a new ear canal to more fully restore this sense. Contact Dr. Tahiri and Dr. Reinisch to learn more about microtia and their renowned treatment options.
HEARING CONSIDERATIONS
Background
Hearing loss associated with CFM has been well described in individuals with microtia and EAC atresia. Patients with microtia and malformed ears are typically diagnosed at birth and should undergo audiological testing. Hearing loss in these patients is typically conductive in nature due to external ear abnormalities, aural atresia and Eustachian tube dysfunction. Sensorineural hearing loss is less common but may also be present in individuals with CFM due to inner ear anomalies seen in these patients.
Hearing consideration
Microtia is accompanied by congenital absence or stenosis of the external auditory canal with variable middle ear anomalies this causes a significant conductive hearing loss. Children with unilateral microtia seem to develop normally. Parents usually do not notice a significant difference between their young children and their siblings without microtia. This is the reason why unilateral hearing loss has been undertreated by many otolaryngologists and usually ignored by plastic surgeons treating microtia.
However, when children with microtia are older, and communication becomes more sophisticated, hearing issues become more obvious. Sound localization and hearing comprehension especially in noisy environments become more difficult.
Patients with microtia tend to turn their unaffected ear in the direction of sound, because the head itself casts a sound shadow, which reduces comprehension in the unturned normal ear.
Once a child is 2.5 years of age, a computer tomography (CT) scan of the temporal bones can allow an otologist to determine if an atresia patient is a possible candidate for a canalplasty. The otologist needs to view the actual scan rather than the radiologist’s report. The Jahrsdorfer 10-point grading scale is commonly used to predict surgical candidacy depending on key features seen on the CT scan.
The treatment of hearing loss by an otologist should be coordinated with the surgeon responsible for the outer ear surgery. Traditionally, atresia repair is done after the costal cartilage ear reconstruction, since almost all otologists perform the ear canal reconstructions through a posterior mastoid skin incision to access the temporal bone. That approach compromises the blood supply of the mastoid skin that is used to cover the cartilage framework. However, prior atresia repair does not jeopardize the success of ear reconstruction using an implant covered by a vascularized fascia flap. With an alloplastic reconstruction, atresia repair is best done before or at the same time as the ear reconstruction as the canal helps with positioning of the reconstructed ear. Also, early atresia repair potentially improves hearing at a critical period of early brain development.
If the patient is not a candidate for atresia repair and a functional canal, a faux-canal can be created at the time of microtia reconstruction.
To speak to a microtia expert and explore various state-of-the-art treatments, contact Dr. Tahiri and Dr. Reinisch today.
Atresia Repair
One option for improving hearing is surgical correction of the anatomical malformation. Atresiaplasty was first described by Thomson in 1843. His technique has been modified and improved over the past 175 years.
The degree of hearing improvement obtained with atresiaplasty is associated with higher Jahrsdorfer scores. Patients with craniofacial syndromes, such as Treacher-Collins or craniofacial microsomia, are often poor surgical candidates due to poorly developed middle ear structures reflected in lower Jahrsdorfer scores. Selection of appropriate surgical candidates for atresiaplasty and meticulous surgical technique is essential to achieving optimal patient outcomes.
For patients who are favorable candidates for atresiaplasty based on hearing and radiographic assessments, the goals of atresiaplasty, expected outcomes and anticipated postoperative care should be discussed. The main goals of atresiaplasty are to create a skin lined ear canal and a mobile tympanic membrane that is coupled to a mobile ossicular chain. Hearing may improve in cases of favorable anatomy. Post-operatively, patients with a patent ear canal and a well-defined postauricular sulcus can potentially be fit with ear level amplification. Patients who undergo atresiaplasty will typically require ongoing otologic management to clean the ear canal.
Patients and parents need to consider their options for hearing management in the context of their preferences for microtia management.
One advantage of the Medpor or Omnipore ear reconstruction is that Atresia reconstruction can be performed before the ear reconstruction or at the same time as the ear reconstruction. When an atresiaplasty is performed at the same time of the ear reconstruction, it is called a Combined Atresia Microtia (CAM reconstruction) and our team has the most experience in the world.
Bone Anchored Hearing Aid
BAHA stands for Bone Anchoring Hearing Aid. It is a surgically implantable system for treatment of hearing loss.
It has three parts:
A Baha implant enhances natural bone transmission as a pathway for sound to travel to the inner ear, bypassing the external auditory canal and middle ear.
If the patient is not a candidate for an Atresia repair, a BAHA can easily be placed at the time of microtia reconstruction. A BAHA is placed using the same incision we use for the ear reconstruction; thus, no new incisions are made. Moreover, we use the latest form of BAHA, it is a BAHA 5, which works with a magnet and has no external screw coming out the scalp.
The advantages having a BAHA placed at the same time as the reconstruction include:
Dr. Tahiri and Dr. Reinisch specialize in microtia surgery for children. Patients from all over the USA and the world visit their Beverly Hills office to discuss microtia and surgical treatment.
FREQUENTLY ASKED QUESTIONS
When your child has microtia, it is easy to be overwhelmed with the number of surgical options available. Dr. Tahiri and Dr. Reinisch educate parents regarding the different treatments, as well as the advantages and disadvantages of each. We want you and your child to be comfortable with the treatment that you select, and we will guide you as you make important decisions that will affect your little one’s future.
You may be unaware of the range of medical specialists involved with microtia treatment. You may also have concerns regarding how to talk to your child about the condition. Perhaps you are wondering why you should choose surgery for your child when he or she is perfectly happy with a small ear. Maybe you are confused about why this congenital deformity was not picked up during a prenatal ultrasound. Dr. Tahiri and Dr. Reinisch are always happy to answer your questions and address your concerns in detail.
Did I do something during pregnancy that caused the Microtia?
Microtia is found in children at birth and is usually an isolated condition. As of today, no real causes of Microtia (other than genetic syndromes involving approximately 5%) were identified. In some children, microtia occurs along with other facial abnormalities, such as hemifacial microsomia, which involves the under development of one side of the face, including the ear. In very rare cases, hemifacial microsomia can occur on both sides of the face at once.
Mothers often ask me if they did anything during pregnancy that caused microtia. To date and to our knowledge, microtia is not caused by something that the mother did during pregnancy. So, it is important for parents to not feel guilty. Also, it is important to remember, that through surgical advances, microtia can be successfully treated via a single procedure in the outpatient setting.
It seems that ear reconstruction is a cosmetic procedure; why would I put my child through a surgery?
As far as I am concerned, the definition of a cosmetic procedure is improving something that is normal. The ear does not only have a cosmetic function but has also a functional aspect.
Reconstructing an ear helps:
The microtic ear was not picked up during my ultrasound. Why?
Various anomalies can be detected by the ultrasound technician. Ultrasound technicians often focus on major organs and external ears are not routinely checked during an ultrasound. It may be easier to detect microtic ears with new high definition 3D ultrasounds.
Why should I choose for my child Medpor ear reconstruction instead of a rib cartilage ear reconstruction?
The traditional method of ear reconstruction has utilized an ear framework made from rib cartilage. Refinements of the cartilage procedure over the years have reduced the number of needed surgical stages. However, the amount of required harvested cartilage has increased, pushing back the age of reconstruction until 10 years of age or older. Reconstruction at an older age, and its usual multiple stages, have made microtia reconstruction with autologous cartilage a more arduous physical and psychological endeavor for both children and their parents. If the final cosmetic result of the constructed ear is not ideal, the entire reconstructive journey can be disappointing.
The use of an alloplastic framework covered by a thin temporo-parietal fascia flap offers several advantages over the traditional method of cartilage reconstruction. Since ears reach 85% of adult size by 3.5 years, ear reconstruction can be performed at a younger age since the need for sufficient costal cartilage is not a factor. Other advantages of a fascia covered alloplastic framework over the traditional rib cartilage technique include minimal patient discomfort, single outpatient procedure, and better ear definition and projection.
At what age should my child have a CT scan and what type of CT scan should be done?
In order to evaluate a child for this life improving intervention, it has become necessary to perform CT scans of the temporal bone at a much earlier age than previously. Scans at 2.5 years are recommended. The CT scan will allow us to determine if your child is a candidate for Atresia repair but also, it allow us to rule out the presence of a Cholesteatoma.
Scan parameters are those used for standard temporal bone studies (1 mm cuts or less with bone windows, usually in a manipulable voxel format without contrast).
How long is the surgery?
The surgery takes approximately 6 hours. Prior to starting the surgery, there is approximately a preparation phase lasting 1hour to 1 ½ hour. During this phase, anesthesia is being administered, we prepare the head, braid the hair if necessary, plan the surgery, make the surgical markings and prepare your child for the procedure.
Is the surgery painful?
Although it is a long surgery and it could seem painful, it is actually not. Patients tolerate it very well. It is done as an outpatient procedure and often, patients require only some minimal pain medications for the first 2 days after surgery. The surgery mostly involves skin and soft tissues. It does not involve muscle or bone and thus, the pain is relatively minimal.
Does the body react to the Medpor or Omnipore implant?
No. The Medpor implant is made of porous high-density polyethylene. This is a completely inert material and will not be rejected by the body.
Are there different types of implants?
Indeed, over the years various implants came on the market. Medpor, Omnipor and Supor are the three most commonly used implants. They are all great implants. In our experience, there are no advantages using one over the other. They are all good. All implants got refined over the years to provide the best reconstruction.
What is the post-operative care?
The first post-operative appointment occurs at post-op day #2 or #3. The absorptive dressing is removed during this appointment. If fluid is noticed beneath the scalp at that time, it can be easily drained percutaneously with a butterfly needle. The silicone ear splint is left in place for a total of two weeks post-operatively. During that period, the head should be kept dry and patient should not sleep on the side of surgery to avoid pressure on the reconstructed ear.
For young children, parents should sleep with the child to make sure they do not inadvertently turn and sleep on the operated side.
At two weeks post-operatively, the silicone ear mold is removed and the ear and head are washed with shampoo in the office. Parents are shown how to wash it gently with their fingers on a daily fashion. A new silicone ear mold is made for the patient to use at night for the following four months. A light coating of ointment is applied on the ear before applying the ear mold for the next week.
The third post-operative visit occurs after three weeks. Washing can now be done with a gauze pad to encourage removal of the dissolving chromic sutures. Parents are taught how to make the silicone ear mold, which will be worn every night for the following four months (parents are provided with the silicone mold packets). This helps to protect the ear, but more importantly, it helps to maintain the projection of the ear.
What is the recovery period?
Patients are usually back to normal after the second day after surgery. As long as the patient keep the head dry while the mold is in place and avoid contact sports, there are no activities limitations. Often, our patients come from all over the world and stay few weeks in Los Angeles. They end up going roller blading by the beach, they go to the zoo, museums and take part of a lot of outdoor activities.
Where is the surgery performed?
Since this surgery is performed as an outpatient procedure, it is often performed at a surgical center specialized in microtia reconstruction. Those surgical centers are very experienced with treating patients with microtia and their care is second to none. If the patient has a genetic syndrome such as Treacher Collins Syndrome or other significant comorbidities such as cardiac or pulmonary problems, the surgery is performed at the Cedars Sinai Main Hospital.
Who will be the anesthesiologist?
Either at the surgery center or at the main hospital, we work closely with the same group of board certified pediatric anesthesiologist who are part of the Cedars Sinai Anesthesia Group. Those pediatric anesthesiologists are very experienced and very comfortable treating patients with microtia. We work with them very regularly and they are very familiar with the Medpor ear reconstruction procedure.
What are the most common complications?
Complications most often occur within the first few weeks of surgery. They are rare however. The most common complication would be a small area of exposure of the implant. This means that the tissues in a small area did not survive and the implant is exposed. The rate of this complication is less than 4%. This complication is treated via small revision surgery aimed at covering the implant.
Other complications are rare and include infection, implant fracture, implant migration. Those complications are very rare and their rate is less than 1 percent.
Moreover, to maintain an adequate posterior sulcus, it is important to wear the silicone mold at night for the first four months. We have seen patients who have lost some ear projection because they were not compliant with wearing the mold at night in the first four months post-operatively.
What are the post-operative limitations?
Ideally, the patient should limit sports for the first few weeks following Medpor ear reconstruction (four to six weeks). Following that period, the patient is free to resume his/her regular activities, while wearing normal sport-specific protection.
If my child had previously a cartilage ear reconstruction for which a TPF flap was used, can a Medpor ear reconstruction still be performed?
This is a very good question. In the rare circumstances that a TPF flap is not available (if it was harvested to cover a rib cartilage ear, of if the TPF vessels were injured from a prior surgery or if a Medpor reconstruction was performed and failed), then an occipital fascia flap can be used to perform a Medpor ear reconstruction. This occipital fascia flap is harvested from the back of the head and is used to cover the implant.
Thus, if a TPF flap is not available, it is still possible to perform a Medpor ear reconstruction.
What are the disadvantages of the Medpor or Omnipore ear reconstruction?
The main disadvantage of the Medpor ear reconstruction, is that it is not flexible and does not bend like a normal ear. This is seen no matter what implant you use (Medpor, Omnipor or Supor). One has to keep in mind that rib cartilage ear reconstruction does not bend either. The other disadvantage of the Medpor ear reconstruction is that if it is not secured properly, the ear can descend slightly from its original position (2-5mm). To prevent that, we used a soft tissue leash to suspend the ear at the appropriate position.
