Microtia literally means small ear. Children with this rare condition have a small, abnormally shaped, or missing 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.
Discovering that their child has microtia can be overwhelming for most parents. The fear of the unknown, coupled with concerns about the child’s future, can be daunting.
Questions about the condition, possible complications, and how this condition will impact daily life are natural and valid.
For adults living with the condition, the situation is quite similar.
An ear deformity like microtia can be a lifelong challenge, affecting self-esteem and quality of life. But it’s important not to lose sight of hope for a rewarding life or to feel isolated.
Dr. Youssef Tahiri and his team are here to offer support.
Renowned as a world-renowned pediatric plastic and craniofacial surgeon and adult reconstructive surgery specialist, Dr. Youssef Tahiri is dedicated to helping patients with microtia navigate the challenges of this condition with compassion and expertise.
When patients work with our team at the Los Angeles Ear and Craniofacial Center, they can be confident they will receive the most comprehensive care possible— helping parents understand the anomaly and its complexities to provide insight into the microtia treatment options available.
Take your first step toward repair and healing. Call (310) 255-4476 or email team@tahiriplasticsurgery.com to schedule a free Skype consultation.
Microtia is a congenital condition that 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 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 sees patients from around the world. Their institute is globally recognized, thanks to their innovations and advanced surgery for aesthetic correction and hearing restoration.
There are four stages, or grades, of microtia:
The root cause of microtia remains largely unknown. However, some experts propose a genetic basis for this congenital condition.
Research suggests that approximately 5% of microtia cases occur when there’s a family history of this condition, with siblings of affected individuals at slightly elevated risk.
However, it’s crucial to note that 95% of children born with microtia have no family history of the condition. For this reason, medical experts aren’t convinced that microtia is a hereditary condition.
A consultation with a geneticist is recommended to get a more definitive understanding of the genetic factors involved.
Genetic evaluation can help determine if microtia is linked to a hereditary condition and serve as a guide for subsequent medical management.
The Centers for Disease Control and Prevention (CDC) defines a “disability” as any condition that makes it difficult for an individual to perform certain activities or interact with the world.
While microtia itself doesn’t necessarily constitute a disability, associated hearing loss often does.
Hearing loss may be grounds for eligibility for various disability benefits depending on its degree of impact on an individual’s life.
For this reason, comprehensive support is essential, which can include the following:
Like many congenital disorders, microtia is relatively, affecting approximately one in 8,000 to 10,000 newborns.
Males are diagnosed with microtia twice as frequently as females, and the condition is more prevalent in certain ethnic groups, including Asians, Pacific Islanders, and Hispanics.
In most cases, microtia affects only one ear, usually the right ear. However, occurrences that impact both ears (bilateral microtia) have been observed in around 20% of cases.
The physical manifestations of microtia vary significantly among individuals. Symptoms can range from a smaller-than-normal ear to the absence of the external ear (anotia).
Due to this variability, a precise diagnosis is critical for determining the most suitable course of treatment.
Depending on the condition’s severity, medical intervention may involve prostheses or surgical procedures like cartilage grafts.
Understanding the potential implications of microtia is essential for individuals and families affected by this congenital condition.
This knowledge empowers informed decision-making regarding treatment and management strategies.
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.
While many individuals with microtia experience no other health complications, it’s crucial to be aware of potential associated conditions.
Approximately 50% of cases occur alongside hemifacial microsomia, also referred to as Goldenhar syndrome and craniofacial microsomia (CFM).
This craniofacial disorder affects the facial structure, usually the ear and jaw. However, the eye, cheek, and neck may also be affected.
Some clinical presentations characteristic of Goldenhar syndrome are empty eye sockets, facial paralysis, and missing jawbones.
Aside from Goldenhar syndrome, other possible complications of microtia include facial nerve weakness, cleft lip or palate, and, in rarer instances, cardiac and urological abnormalities.
Microtia is generally diagnosed at birth due to the visible abnormality of the outer ear. While prenatal ultrasounds can sometimes indicate potential issues, a definitive diagnosis is usually made after delivery.
Healthcare providers often examine the internal ear structures using CT (computed tomography) scans to assess the condition’s extent and identify any potential underlying problems.
CT scans provide detailed images of the ear, which can be valuable in diagnosing microtia in children.
However, it’s typically discouraged until the child is old enough due to the potential risks of radiation exposure.
The optimal age for a CT scan to diagnose microtia diagnosis is around two and a half years, when the child’s skull, particularly the temporal bone, is close to its adult size.
Fortunately, Dr. Tahiri offers advanced treatment for both the cosmetic problems and the hearing difficulties associated with microtia.
Their innovative porous polyethylene ear (PPE) implants, which include MEDPOR™, OMNIPORE™, and the latest iteration SUPOR™, allow them to create an external ear structure using a child’s own tissues—a technique that offers a number of benefits.
These implants use high-density porous biomaterial that can match the shape and projection of a normal ear. They also eliminate the need for ear pinning surgery, reducing the number of surgical stages.
Dr. Tahiri 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 to learn more about microtia and their renowned treatment options.
Understanding the relationship between microtia and hearing loss is crucial for early intervention and optimal development.
This section will explore hearing assessment, treatment options, and the importance of a comprehensive approach involving healthcare professionals.
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.
Microtia is accompanied by the congenital absence or stenosis of the external auditory canal with variable middle ear anomalies, which causes 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 many otolaryngologists have undertreated unilateral hearing loss, which is 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 canaloplasty.
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 position 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 today.
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 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 Dr. Tahiri’s PPE implant technique 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 as the ear reconstruction, it is called a Combined Atresia Microtia (CAM reconstruction), and our team has the most experience in the world.
BAHA stands for Bone Anchoring Hearing Aid. It is a surgically implantable system for the 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 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 of the scalp.
The advantages of having a BAHA placed at the same time as the reconstruction include:
Ear prosthetics offer a nonsurgical alternative or temporary solution for individuals with microtia.
These artificial ears, usually crafted from silicone, are designed to closely mimic the appearance of an ear, including the earlobe. Attachment methods include adhesives or magnetic implants.
While ear prosthetics provide an aesthetic benefit, they require ongoing maintenance. In other words, they may not be a suitable option for all individuals with microtia, especially children.
Surgical ear reconstruction is a standard treatment option for microtia. This procedure aims to create an ear that resembles a natural ear in appearance and function.
Depending on the procedure, multiple stages of surgery are often required.
During the surgical process, surgeons create a new ear framework using cartilage harvested from the patient’s ribs or synthetic materials.
This framework is then covered with the patient’s tissue for a natural appearance.
Meanwhile, Dr. Tahiri’s PPE implant method can be performed in a single outpatient procedure. Skin grafts are usually taken from the back of the patient’s scalp, resulting in minimal to no visible scarring.
The optimal timing for microtia surgery is typically between six and nine.
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%) have been identified.
In some children, microtia occurs along with other facial abnormalities, such as hemifacial microsomia, which involves the underdevelopment 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.
The optimal time for your child to undergo microtia repair depends on the procedure.
For traditional ear reconstruction techniques, such as those that utilize rib cartilage, the recommended age is between 8 and 10, as children within this age range have sufficient rib cartilage.
However, Dr. John Reinisch designed a procedure that utilizes biomaterial or nonhuman chest cartilage and allows children as young as three years old to have the surgery—the porous polyethylene ear (PPE) implant technique.
The primary benefit of pursuing an early treatment like MEDPOR™, OMNIPORE™, and SUPOR™, particularly before school age or five years old, is that the child may avoid harassment and bullying at school.
Ultimately, the best decision must be made in consultation with a qualified healthcare provider.
Contact Dr. Tahiri to determine the optimal timing for PPE implants and other microtia repair options.
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:
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.
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 temporoparietal 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, a single outpatient procedure, and better ear definition and projection.
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 it will also 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).
The surgery takes approximately 6 hours. Prior to starting the surgery, there is approximately a preparation phase lasting 1 hour to 1 ½ hours.
During this phase, anesthesia is administered, we prepare the head, braid the hair if necessary, plan the surgery, make the surgical markings, and prepare your child for the procedure.
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.
No. PPE implants (MEDPOR™, OMNIPORE™, and SUPOR™) are made of porous high-density biomaterial. This is a completely inert material and will not be rejected by the body.
Indeed, over the years various implants came on the market. MEDPOR™, OMNIPORE™, and the latest innovation SUPOR™ are the three most commonly used implants. They are all great implants.
In our experience, there are no advantages to using one over the other. They are all good. All implants were refined over the years to provide the best reconstruction.
The first post-operative appointment occurs on 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 the 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 in 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 the 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.
Patients are usually back to normal after the second day after surgery. As long as the patient keeps the head dry while the mold is in place and avoids contact sports, there are no activity limitations.
Often, our patients come from all over the world and stay a few weeks in Los Angeles. They end up going rollerblading by the beach, they go to the zoo and museums and take part in a lot of outdoor activities.
Since this surgery is performed as an outpatient procedure, it is often performed at a surgical center specializing 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.
Either at the surgery center or at the main hospital, we work closely with the same group of board-certified pediatric anesthesiologists 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 PPE implant procedure.
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 was 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, and 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.
Ideally, the patient should limit sports for the first few weeks following the PPE implant procedure (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 the PPE implant technique 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, or if the TPF vessels were injured from prior surgery, or if a PPE implant was performed and failed), then an occipital fascia flap can be used to perform a MEDPOR™, OMNIPORE™, or SUPOR™ 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 PPE implant procedure.
The main disadvantage of PPE implants is that they’re not flexible and do not bend like a normal ear. This is seen no matter what implant you use (MEDPOR™, OMNIPORE™, or SUPOR™).
One has to keep in mind that rib cartilage ear reconstruction does not bend either.
The other disadvantage of the PPE implant 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.
Dr. Tahiri specializes in microtia surgery for children. Patients from all over the USA and the world visit his Beverly Hills office to discuss microtia and surgical treatment.
While there is no definitive cure for microtia, meaning it cannot be completely reversed, medical intervention can lead to significant improvements.
Various treatment options exist to correct the physical aspects of microtia. For instance, plastic surgery via PPE implants can improve the appearance of an affected ear.
At the same time, surgical interventions, such as ear canal reconstruction and bone-anchored hearing aids (BAHAs), can enhance hearing and improve the overall quality of life for individuals with microtia.
Despite ongoing research, there’s no known way to prevent microtia, as the exact cause remains unidentified.
However, certain lifestyle factors can potentially influence the risk.
Avoiding excessive coffee and alcohol consumption and the use of specific medications, such as retinoic acids like Accutane (isotretinoin)—a drug to treat severe acne—during pregnancy is recommended as a precautionary measure.
Parents should consult a healthcare provider immediately if they suspect their child may have microtia.
Early diagnosis enables prompt development of a comprehensive treatment plan, which, depending on the severity, can include aesthetic correction and hearing restoration.
The timeline for resuming regular activities after microtia surgery hinges on the specific procedures performed and the child’s overall recovery.
However, we recommend that the patient limit strenuous activity, such as sports, for four to six weeks following the PPE implant procedure.
Fortunately, Dr. Youssef Tahiri and his medical team will facilitate post-operative care, including providing guidelines on when it’s safe for a child to resume regular activities.
Aside from the typical hearing loss, some individuals with microtia experience health complications, such as:
However, it’s essential to note that microtia is a condition with varying severity. With early medical intervention, most individuals with microtia can expect to enjoy good overall health.
A diagnosis of microtia can understandably raise many questions. While the Los Angeles Ear and Craniofacial Center team will happily address all concerns, parents are encouraged to proactively gather information to effectively care for their child.
Critical questions to ask the child’s physician include the following:
