Medically reviewed by Dr. Youssef Tahiri
Is your child struggling with hearing loss? One possible cause is a condition known as aural atresia, which is characterized by underdeveloped ear canals.
Learning of your child’s aural atresia diagnosis can be difficult, especially when you know you did everything to support a healthy pregnancy. We understand the fear and uncertainty you could be feeling.
You might be asking questions such as, “Will my child be able to communicate and develop normally?” or “Are there further complications with the condition I need to worry about?”
While researching the condition can be helpful, the overwhelming amount of information available online can make it challenging to decide the best course of action for your child.
That’s why we’re here—to offer guidance and support. Like you, we want only the best for your child, and we’ll do everything possible to help our patients lead a fulfilling life.
Our medical team specializes in treating aural atresia—with a track record of positive outcomes and a commitment to providing the most comprehensive care. We will ensure that your child receives the compassionate care they deserve.
At the Los Angeles Ear and Craniofacial Center, Dr. Youssef Tahiri, a world-renowned craniofacial plastic and reconstructive surgeon, and his dedicated team of experts can provide you with everything you need to know about this congenital disorder and offer innovative treatment options.
We’re determined to help you navigate this challenging journey.
For state-of-the-art facilities and a treatment plan tailored to your precious one’s unique condition, you can entrust your child’s care with us.
Our team of medical specialists will happily support and guide you and your child so they can live a fuller life despite their condition.
“I have been communicating with Dr. Tahiri and [his] office staff since 2020. My daughter, who [has] bilateral microtia and atresia, finally just had her left ear reconstruction. This office worked tirelessly with [the] insurance [company] and with me, Mom. Always responding in a timely fashion and always kind. I cannot say enough about our experience.
The surgery center is immaculate, [and] the staff [is] very professional and nice. The doctor himself is AMAZING!!! We are so thrilled with her big ear and looking forward to [having] the other side [done]. The doctor has a gift. He is an artist and so great with kids. Five stars all around.”
Kate Faroni
You can schedule a free Skype consultation by calling (310) 255-4476 or emailing team@tahiriplasticsurgery.com. You can also visit our center at 9033 Wilshire Blvd, Suite 200, Beverly Hills, California 90211.
Imagine the laughter of friends, the rustling of leaves, the sweet lullaby of another person’s voice muted or muffled—that’s what living with aural atresia is like.
There are two types of aural atresia: acquired and congenital (a condition from birth).
The former usually results from an inflammatory process, trauma, or complication following otologic surgery, a range of procedures designed to treat conditions affecting the inner, middle, and external ear.
Meanwhile, congenital aural atresia is a birth disability that occurs when the external auditory canal (ear canal) doesn’t develop properly or may be absent completely.
Think of it this way: Your child’s ear canal is their doorway to the world of sound. But instead of being wide open, it remained closed, narrow (stenosis), or, in some cases, blocked by trapped skin (cholesteatoma).
Whether your child’s aural atresia is acquired or congenital, the condition can lead to profound conductive hearing loss.
If left untreated, these hearing challenges can persist throughout your kid’s life and, in some cases, may cause delayed language development and learning disabilities.
For this reason, prompt diagnosis and early medical, sometimes surgical, intervention is necessary to give your baby the best chance of normal hearing and a normal life.
Aural atresia is a relatively rare condition. It affects roughly 1 in 10,000 to 20,000 babies. In other words, children have a 0.01% to 0.005% chance of developing the condition.
Moreover, aural atresia often manifests unilaterally, with some studies finding a predilection or bias for the right ear.
Children with unilateral aural atresia can learn to speak and understand language without significant issues if their unaffected ear has normal hearing and development.
However, they’re more susceptible to delayed language development. After all, monaural hearing (hearing with only one ear) can make it challenging to process sounds accurately. Issues one may encounter include:
It’s also worth noting that aural atresia is more common in boys. Some researchers point to a 2.5 times higher likelihood of the condition occurring in males than females.
In many cases, aural atresia is also accompanied by other congenital disorders, like microtia, which is characterized by missing, abnormally shaped, or small ears.
Aural atresia is a complex condition. Hence, it’s essential to understand its pathophysiology or the changes associated with it.
To better imagine what your child is dealing with and its potential impact on their life, you must first know how our ears develop in the womb.
Here’s a timeline of what happens under normal circumstances:
During this stage, the three layers of the tympanic membrane (ear drum) also begin to develop.
These tissues are believed responsible for growing and shaping the external ear (auricle).
If something goes wrong at any point of this timeline, such as the epithelial plug not disappearing during the 28th week of pregnancy, your child may develop congenital aural atresia.
When your child is born without an external ear canal, they have aural atresia. But when they’re born with a narrow ear canal, they have ear canal stenosis.
A blocked or narrow ear canal prevents sound waves from reaching the inner ear properly. To better understand what it’s like to have either condition, imagine listening to a conversation through a thick wall.
Children with aural atresia and stenosis often have difficulties understanding speech, especially in noisy environments, which some studies suggest can pose learning challenges.
They may also struggle to localize sounds, like figuring out where a car is coming from, for example, which is amplified if your kid has bilateral atresia or when both of their ears are affected.
It’s hard not to blame yourself if your child is born with aural atresia. However, the debate on its exact cause is ongoing, with medical professionals believing that the origin is often multifactorial.
One of the proposed factors is embryological development disruption, particularly concerning the first pharyngeal cleft—the anatomical space responsible for the development of the external auditory canal.
Note that the first pharyngeal cleft bridges the first and second branchial arches, which give rise to the:
It’s for this reason that children with aural atresia often have other craniofacial abnormalities that result from first and second branchial arch disruption, such as:
Aural atresia can vary in severity. The condition’s symptoms are pronounced in some cases but not as much in others.
However, aural atresia does have distinct clinical presentations that allow you to determine if your child has it. These signs include:
Do understand, however, that aural atresia can manifest as severely narrow external auditory canals.
Indicators of speech and language delay, which can be potential symptoms of aural atresia, include:
If one of your child’s external auditory canals is narrow but still has normal hearing nerves, a bone conduction device may be sufficient to address their hearing challenges.
However, surgical intervention may be required to maximize hearing restoration if your child has severe aural atresia symptoms, especially if their condition is bilateral.
For this reason, comprehensive diagnosis is critical, especially if you want to ensure your child receives the appropriate treatment for their unique condition.
This process generally involves hearing tests, mainly auditory brainstem response tests, within the first few months of your baby’s life.
If your child’s hearing results show that their unaffected ear is normal and they’re expected to develop speech and language normally, they may forego further testing until they’re six months old.
However, if the test results indicate abnormal hearing in your child with aural atresia, they may need additional hearing tests to ensure the regular function of their unaffected ear.
Other diagnostic options include temporal bone CT (computed tomography) scans. This imaging test gives doctors a detailed look at the lower part of your child’s skull to detect anomalies in the ear.
Unfortunately, according to the Jahrsdoerfer grading scale, temporal bone CT scans should only be performed when your kid is at least five years old or if a surgeon recommends immediate intervention and requests these tests as part of aural atresia surgery.
It’s worth noting, however, that some experts like Dr. Tahiri allow your child to get a CT scan as early as two and a half years. After all, the earlier the diagnosis, the sooner you understand your options.
Regardless, the Jahrsdoerfer grading scale measures the severity of your child’s congenital aural atresia based on the following criteria:
Doctors use this system to determine the best treatment options. The more parts of your child’s ear are unaffected by the condition, the higher their Jahrsdoerfer grade, with 10 being the highest.
While doctors have made significant strides in understanding aural atresia, they cannot pinpoint its exact cause.
However, the medical community believes genetics plays a substantial role, especially considering that aural atresia often occurs alongside other syndromes associated with ear malformations.
For instance, Treacher Collins syndrome, a condition typically associated with aural atresia, can be diagnosed by testing if the affected individual has a mutated TCOF1 gene.
The TCOF1 gene produces the treacle protein necessary for your child’s facial cartilage, bone, and connective tissue development.
A mutation disrupting treacle production can cause developmental abnormalities like anotia (missing external ear) and aural atresia.
Meanwhile, Crouzon syndrome, which sometimes causes aural atresia, occurs because of FGFR2 or FGFR3 gene mutations.
These genetic codes are responsible for fibroblast growth factor receptors (FGFRs), which are critical for your child’s developmental and adult cells.
Aural atresia may also manifest if your child has any of the following congenital conditions:
For this reason, doctors consider genetic testing as a viable diagnostic tool for syndromic patients—those with aural atresia and other extra-craniofacial features.
We understand that an aural atresia diagnosis can be difficult news to hear. Don’t worry. Although the prognosis for aural atresia varies on the severity, most children develop appropriate hearing and speech.
Yes, you should expect your child to experience some degree of hearing loss, which some studies indicate ranges between 40dB (decibels) and 60dB. However, early intervention can minimize this range to 25dB to 35dB.
For this reason, getting a comprehensive diagnosis is critical for your primary care physician to determine the best possible treatment options for your child.
Here’s where Dr. Youssef Tahiri and the Los Angeles Ear and Craniofacial Center come in.
Using state-of-the-art facilities and world-renowned expertise in plastic and reconstructive surgery, our team of dedicated and compassionate professionals can deliver comprehensive care tailored to your child’s needs.
So, if you want a clearer picture of what your child is dealing with and the options that will allow them to overcome their condition, we’re the right team to talk to.
You can get a thorough diagnosis from Dr. Tahiri by calling (310) 255-4476, emailing team@tahiriplasticsurgery.com, or visiting our clinic at 9033 Wilshire Blvd, Suite 200, Beverly Hills, California 90211.
Alternatively, you can send photographs of your child’s aural atresia symptoms via our contact form for Dr. Tahiri to evaluate.
Aural atresia is a congenital condition that inhibits inner ear development. In some cases, the inner ear is missing entirely.
As a result, sound waves are not able to travel through the ear to vibrate the eardrum, hearing bones, and inner ear fluid.
Thus, the auditory nerve cannot transmit signals to the brain, where these signals would be registered as sounds.
The International Institute for Microtia Repair is the only practice that combines aural atresia repair and microtia treatment into a single outpatient surgery.
If your child suffers from aural atresia, he or she may be a candidate for ear canal reconstruction. Dr. Tahiri will take a CT scan of the inner ear to make sure that he or she has a healthy cochlea, which is necessary for successful surgery.
The majority of patients meet this requirement. These images will also reveal whether the inner ear bones have fused together, another condition that will limit candidacy for surgical repair.
Children must be at least two-and-a-half years old to receive a CT scan. Dr. Tahiri will consult with Dr. Robertson, as well as an audiologist and other specialists, to determine your child’s eligibility for treatment.
When children are very young, they are going through a critical developmental stage. As they hear and process new sounds, it stimulates their brains and strengthens neurological function.
You can compare this process to learning a new language or learning to play the piano. Children adopt these skills much more easily than adults. Therefore, it is important to treat aural atresia as early as possible.
However, in most cases, we will not perform surgery until your little one is at least three years old.
The unfortunate reality is that aural atresia can sometimes come with unwanted baggage—other congenital disorders—as if the condition itself wasn’t enough.
For this reason, managing aural atresia and the accompanying hearing challenges can be complex. It may require a multidisciplinary approach involving these professionals:
After all, the severity of your child’s congenital disability, the underlying cause of their aural atresia, and whether they have other genetic problems or autoimmune disorders affect the required intervention.
Fortunately, you don’t have to build a care team for your child alone. Our staff will happily recommend and connect you with the healthcare professionals you need to give your precious little one the best odds of leading a fulfilling life.
Aural atresia was described as early as 2,000 BC. However, the earliest reports of surgical procedures to treat the condition didn’t appear until the late 1940s and 1950s.
So, for a long time, individuals with aural atresia didn’t have effective treatment options, leaving many to suffer in silence—literally and figuratively. Fortunately, those days are gone.
Today, various surgical and nonsurgical avenues are available for your child to overcome aural atresia and its side effects. These treatment options include the following:
Not every child with aural atresia needs surgery to help with their hearing and speech. In fact, you can expect your child’s development to be normal as long as their other ear isn’t impaired.
Unfortunately, every child’s aural atresia case is unique. Some children struggle with monaural hearing, impacting their ability to process sound and communicate effectively.
That’s why getting hearing and speech assessments, such as auditory brainstem response tests, is critical, as it allows you to determine the best measures to improve your baby’s life.
This step may include behavioral testing (audiogram), which allows doctors to gauge your child’s reaction to speech and other sounds as early as six months.
Contact our office today to find out if your child qualifies for aural atresia surgery or if they can improve their hearing and speech through alternative, nonsurgical means.
Schedule a free Skype consultation with our multilingual team by calling (310) 255-4476 or emailing team@tahiriplasticsurgery.com.
If your child with microtia is a candidate for ear reconstruction surgery, you generally have two primary options: rib cartilage or synthetic framework.
Traditional ear reconstruction utilizes rib cartilage grafted from your child to develop an ear framework.
Due to recent advancements, your kid only needs to undergo a minimum of two surgical procedures—one to graft the necessary materials and another to install the new framework—to restore their ear.
Unfortunately, this ear reconstruction method often involves harvesting a substantial amount of cartilage, disqualifying children under 10 from getting the procedure.
The drawback to this delayed intervention is that the procedure will now require more surgical stages, which can be physically and psychologically taxing on children and their parents.
Worse is that ear reconstruction utilizing a rib cartilage framework doesn’t guarantee satisfactory results.
Enter synthetic ear frameworks like Dr. Tahiri’s porous polyethylene (PPE) implants, which include MEDPOR™, OMNIPORE™, and the latest iteration, SUPOR™.
Unlike rib cartilage-based ear reconstruction, our PPE method creates ear frameworks from synthetic, biomaterial chest cartilage, allowing your child to get the procedure as early as three years of age.
A synthetic framework also involves only a single outpatient procedure, promises minimal patient discomfort, and boasts better outcomes than frameworks made from rib cartilage.
When Dr. Tahiri creates this synthetic framework, you can be confident that your child will have a natural-looking ear, increased self-confidence, and improved hearing for a lifetime.
“Dr. Tahiri is an amazing surgeon. We’ve known him for over five years, and he has been supportive over the years. He came to numerous microtia and atresia events that our family helped host.
He patiently walked us through the entire surgical process and always answered our questions. My son had ear reconstruction surgery two months ago and also had the Oticon Ponto abutment procedure. We are beyond thankful for Dr. Tahiri, [his team], and the K and B [Surgical] Center.
They all together took care of our son like their own. We would recommend Dr. Tahiri again and again to all prospective patients. We have met multiple surgeons, and we knew he was the one the moment we met all those years ago.”
Christine Bayan
Call (310) 255-4476 or email team@tahiriplasticsurgery.com to learn more about Dr. Tahiri’s revolutionary MEDPOR™, OMNIPORE™, and SUPOR™ ear reconstruction implants.
A trusted otologist (Dr. Roberson) will perform ear canal reconstruction, also known as canalplasty.
Dr. Tahiri will perform the cosmetic aspects of the procedure, typically using a polyethylene MEDPOR™, OMNIPORE™, or SUPOR™ implant to create an outer ear framework.
Your treatment team will carefully plan the procedure during an initial consultation. The surgery itself will take place under general anesthesia.
To ensure your child’s complete safety, we work with experienced pediatric anesthesiologists. Your child’s surgeon will first create an opening where the ear canal would normally be located.
He or she will line this opening with a skin graft and, if necessary, reposition the inner ear bones to ensure better hearing.
In some cases, the doctor may need to create a new eardrum as well, using an additional skin graft.
Once this process is complete, Dr. Tahiri will reconstruct the outer ear, typically using the MEDPOR™, OMNIPORE™, or SUPOR™ technique. These combined procedures can restore hearing as well as aesthetics.
Combined atresia microtia (CAM) surgery is a rather extensive process. Though it is an outpatient surgery, we will still need to monitor your child’s progress and healing.
In most cases, your little one will have the first follow-up one to two days after surgery. The second appointment will usually take place two weeks later.
We know that many patients come from long distances to receive our care. We also partner with the local Ronald McDonald House to provide long-term housing.
If your child does not qualify for ear canal reconstruction, we can recommend an alternative treatment.
These options will not restore hearing as successfully. Nonetheless, they can improve auditory processing and language development:
Bone-anchored hearing aids (BAHAs) are excellent tools to help your child with aural atresia overcome hearing challenges.
Unfortunately, your child must be around five years old to use them. BAHAs involve anchoring a titanium implant in the skull bone behind the ear.
Fortunately, your child doesn’t need to wait that long to receive aid. Enter headband hearing devices. This nonsurgical option can be worn when your baby is a few months old.
Like BAHAs, headband hearing aids utilize bone conduction sound processors (BCSPs) to transmit sound waves from your little one’s skull bone to their inner ear minus the surgery.
The malformations caused by aural atresia can range between stenotic (<4mm diameter) and atretic ear canals (completely closed).
If your child has an ear canal but still has difficulty hearing, they can leverage hearing aids that focus on amplifying sound.
There are several different types of these sound-boosting hearing aids available, including the following:
The best type of hearing aid for your child with congenital aural atresia will depend on their specific needs and preferences.
If you need help navigating the world of hearing aids, contact us. Our compassionate team at the Los Angeles Ear and Craniofacial Center will happily discuss the most appropriate options for your child’s case.
A congenital disorder like aural atresia and its accompanying hearing loss can have profound effects on your child’s life.
The good news is that the proper treatment and medical team can help your little angel overcome the challenges of living with aural atresia and enjoy a long and happy life.
Here’s where the PPE implant technique and the Los Angeles Ear and Craniofacial Center team, which Dr. Youssef Tahiri leads, come in.
Since Dr. John Reinisch created them, the MEDPOR™, OMNIPORE™, or SUPOR™ techniques have been used in over 250,000 successful surgeries, helping children worldwide regain a sense of normalcy.
Although the PPE implant method contributes significantly to positive patient outcomes, our team also plays a substantial role in that, too.
After all, our compassionate and professional staff can guide you throughout all of the stages of your child’s treatment, including coordinating:
“It has been a privilege dealing with Dr. T and his staff. Being from Canada, I have had nothing but frustration and despair when it comes to microtia repair.
The moment I met Dr. T (via Zoom), I felt seen and heard. He answered all of my questions and gave me lots of information in order to make the best decision. Dr. T is friendly, kind, and incredibly talented.
The whole process—from initial consultation to post-surgery—has been easy and straightforward. I had surgery in January 2023, and the result is amazing.”
Julia Lambert
If you want to ensure your child with aural atresia and other congenital anomalies like microtia receives the most comprehensive care with the best possible outcomes, contact our office today.
The worst mistake you or any parent can make when their child has aural atresia or other congenital disorders is not knowing your enemy.
After all, understanding what you’re up against can give you the tools and knowledge to help you make informed decisions about your baby’s condition.
So, aside from potential hearing and speech difficulties, what else should you know about aural atresia? Here are some critical points:
As a parent of a child with aural atresia, you must have a robust management system in place. Here are some tips for navigating this journey:
A malformation in your baby’s external ear correlates to potential middle or inner ear abnormalities.
For this reason, medical professionals recommend that all newborns with outer ear malformations, including aural atresia, undergo hearing screening.
After all, early identification of hearing loss is critical for determining appropriate treatment options for your child’s unique situation and ensuring their normal hearing and speech development.
If you suspect your child has aural atresia, you must act as swiftly as possible. Fortunately, our staff is always available on the other end of the line to assist you with your concerns.
Give your child the most comprehensive care by calling (310) 255-4476, emailing team@tahiriplasticsurgery.com, or visiting us at 9033 Wilshire Blvd, Suite 200, Beverly Hills, California 90211.
