* This information was gathered by directly speaking with surgeons, families and individuals who have already had surgery, and through personal research. The remaining information has come from noted sources.
What is Atresia?
Atresia, (also known as aural atresia) is the absence or closure of the external auditory ear canal. The middle ear bones (incus, stapes, and malleus) may be malformed including the narrowing of the ear canal, known as canal stenosis. Atresia is Latin for absence of an opening. An Atretic ear is often sealed off by the skull with no opening (canal) being present. Atresia is a congenital birth defect. There are varying degrees of middle ear bone abnormalities such as: a.) the incus, stapes and malleus all may be present but are not functioning for one reason or another b.) one or more of these three bones may be missing, not connected to the oval window and or are not present in the middle ear structure at all c.) all three of these bones may be present however are misshaped or malformed. A majority of the time, the Cochlea still functions and the auditory nerve is intact. There usually will be a hearing loss to some degree mainly because of having no ear canal allowing sound to travel through to the inner ear. If your child has unilateral Atresia in most cases the non-Atretic ear may have normal hearing. However, sometimes the non-Atretic ear may be affected with a hearing loss as well.
What do I do now that my child has Atresia?
Before you even leave the hospital following the delivery of your child, the hospital or newborn hearing screening should be conducted on your child. If your child is having trouble hearing out of their Atretic, ear he/she will typically refer on the hearing test and it will be suggested that you schedule an appointment with an audiologist and an ENT for further review of your child’s hearing. An ENT can confirm that your child has Atresia and check to see that there are no other potential problems such as “swollen” glandualar problems (in neck and head), a cholosteatoma, ear infections, chance of an ear opening in the Atretic ear, if your child has canal stenosis (narrowing of the ear canal) in the non-Atretic ear, and to just help you learn more about Atresia and generally make sure that your child is healthy from an ENT perspective. The audiologist appointment that should be scheduled is called an ABR (Auditory Brainstem Response) test. T his test will help the audiologist determine how severe the hearing loss is that your child has. This test is conducted through a software program that reads the electrical activity from the brain when sound and vibration are presented to your child by connecting a series of electrodes to their skull and ear. This feedback will determine approximately at what dB level your child can hear at in their Atretic ear and how well the non-Atretic ear can hear as well. The ABR test along with a series of other tests including an OAE test (Otoacoustic Emissions) test will help you understand how well your child can hear overall. It is also important to continue taking your child to the audiologist three to four times per year to keep a close watch on his/her hearing making sure the hearing does not degrade or worsen.
Will my child be prone to having more ear infections and congestion because of Atresia? No, not necessarily.
Some children with Atresia may appear to experience more congestion. However, many children who do not have Atresia have congestion problems as well. Sometimes, we as parents, tend to look for reasons to point our finger at such as: “Our child has Atresia and this must be the reason why they are sick all of the time.” This can be labeled as jumping to conclusions. In fact, some parents may find that the siblings of the Atretic child may tend to either get sick more often or have more ear infections when compared to their child with Atresia. The best thing to consider is taking your child to the pediatrician, audiologist, or ENT every three to four times per year to keep an eye on any potential ear infections. Staying on top of things and being proactive will help prevent any potential problems that could happen later on if gone undetected. If you are concerned about your child obtaining ear infections from swimming you can use proactive measures to prevent potential ear infections by using a swim cap, swimming ear plugs, or by applying “swimmers ear” ear drops after swimming to help dry out the ear.
* It is also important to take your child to an ENT to have his/her ears checked for a Cholesteatoma (a growth caused by excess skin cells that can permanently damage hearing inside of the ear (if gone undetected) by pushing up against the ear drum and inner ear bones). This may cause your child irritability or may appear as an ear ache or ear infection. This is something that should be ruled out. A cholesteatoma can grow back after being removed, but can be permanently removed. The only way to rule out a cholesteatoma is to have a CT scan conducted.
Information on Cholesteatomas:
Cholesteatomas are excess growths of skin that can become a tumor (benign or non cancerous)growing from the inside of the ear to the middle ear. They can be dangerous and very painful to anyone who has one. They can destroy hearing with symptoms of pus or an unpleasant smelling substance, oozing fluid or recurring discharge coming from the ear. Cholesteatomas can erode bone and cause many different problems. There is also a difference between a canal cholesteatoma in the setting of aural stenosis (a narrow ear canal) and an acquired cholesteatoma in an ear in the ear drum and ear bones. Some cholesteatomas develop in time and some are congenital and a child is born with one. However, they can be removed. Although they may grow back they can be removed again and hopefully permanently.
The below is a link that shows images and provides information on cholesteatomas:
My child has been diagnosed with canal stenosis, what does this mean?
Canal Stenosis is the narrowing of an ear canal and is a milder form of Atresia. Instead of the canal being absent it is simply narrower than the normal canal or closed. Sometimes, stenosis may involve the same complications as Atresia with malformed or missing middle ear bones. There may also be a conductive hearing loss present as well. However, canal stenosis may just mean that your child has a narrow ear canal and they can still hear just fine. It is important to take your child to an audiologist to conduct hearing tests to make sure the hearing that is present does not worsen and that your child is hearing fine. It is also very important to take your child to an ENT to have their ear cleaned (every six months) as the narrow canal may not typically allow a typical sized cotton swab in for cleaning purposes.
Can someone with Aural Atresia get ear infections? Yes.
Below is a letter from one of the top canalplasty surgeons in the world explaining why someone with Atresia can still get ear infections:
Dear Ms Tumblin:
Children with Atresia can get ear infections. The bacteria or virus that starts an ear infection gains access to the middle ear through the Eustachion Tube – which those with Atresia have. With an ear infection, the pain we associate with the acute otitis media is caused by the eardrum bulging outward with the pressure from the infection and pus in the middle ear. Since there is no eardrum, children with Atresia may not feel pain with an infection (sometimes there is a mild ache but nowhere near the usual pain with AOM). A fever of unknown origin or dizziness or meningitis can all be sudden signs of an ongoing infection and should be considered by a physician treating a child with Atresia who exhibits one of these symptoms. A CT scan is about the only way to tell if there is fluid in the middle ear. This still takes judgement as both soft tissue, cholesteatoma, and fluid all look the same on CT scan. If a child has a scan showing air in the middle ear space at one point (proving the ET is open) and then a repeat scan with no air with a clinical picture consistent with an ear infection, it is very likely that is what is going on. If a patient has a scan showing no air, the provider is unsure whether this is the way it has always been since birth or whether there was an air containing middle ear space that now has fluid or infection contained in it.
Dr. JOSEPH ROBERSON
Phone 650.462-3149 (Jessica Dana)
Can someone having Atresia experience ear pressure when flying? No, not necessarily.
Most infants and or toddlers who fly will have a 50/50 chance of acting up on the flight. This is not always due to experiencing pressure in the ear drum from the flight or just because they have Atresia. Often times, they are not adjusting to so many people being present around them and are unfamiliar with the plane cabin as it is not their natural environment. Along with excitement during travel, kids may act out due to sleepiness and irritability or just fear in general of flying and not understanding what is going on. Any Atretic child or adult may experience pressure build up just as much as any other child or adult who does not have Atresia. The best rule of thumb is to just be prepared and to take precautions such as: when traveling with an infant give him/her some Tylenol before take off, have them suckling on a bottle during take off, and during the landing of the flight. If you are an adult or older child you may also choose to chew gum to help relieve pressure build up. There are also special ear plugs for flying…they are called “Ear Plane” and they can be found at CVS, Walgreens and Walmart. However, these stores may only carry adult sizes. You can Google these ear plugs and order the children’s sizes on line.
Is there a surgical procedure available that can help restore my hearing? Yes.
This surgery is known as Canalplasty or (Atresia repair). Canalplasty is a surgical procedure where an ear canal can be reconstructed with a skin graft and if the middle ear bones can be manipulated by the surgeon, hearing can be restored within normal range hearing or close to it. Even if one or two of the inner ear bones are malformed or missing it is possible that your child can still be a candidate for canalplasty through the surgical implantation of prosthetic bones. Either way, your child’s surgeon can determine if your child is a candidate for either one of these surgical procedures. A CT would have to be taken in order for the surgeon to determine if you are a candidate for the procedure.
Why should my child have Atresia repair?
Children and adults having Atresia experience difficulty in hearing in more ways than one. Atresia affects hearing by preventing children and adults from hearing background noise such as in restaurants, in the cafeteria, or at conferences and concerts. Atresia also affects the inability to locate sound in space. For example, if you call to your child when background sound is present, they may not hear you across a room due to not being able to locate where the sound is coming from. This is called locating sound in space. Once Atresia surgery has been performed it will greatly improve your child’s ability to locate sound in space and to hear better with background noises and in loud and noisy environments such as in restaurants, at parties, at concerts, in classrooms like PE and band, in the lunch room cafeteria, and in the car with the window down while having a conversation. Atresia repair can help children and adults live easier everyday lives.
How do I know my child is a candidate for Atresia repair (Canalplasty)?
First of all, a child must have some development of the middle ear bones and an open space (oval window into the inner ear “tympanic membrane.” If a child does not have this space in the middle ear, the ear bones cannot vibrate and hearing can not be restored through Atresia repair. Atresia repair candidacy is based from reading a CT scan that is scored on a scale between 1 and 10 with 10 representing a 100% chance of restoring hearing to below a 25dB level, where 20dB is considered within normal hearing range. The score of 10, 9, and 8 suggests that your child can be recommended for Atresia repair. The score of 7 or 6 suggests that Atresia repair is possible but only at a 60% to 70% chance of restoring hearing to within a decent hearing dB level. Scores of 5 to 0 suggest that Atresia repair should not be recommended. However, at this level it is still possible for your child to hear through the use of an implantable BAHA (Bone Anchored Hearing Aid) or a Vibrant Soundbridge Middle Ear Implantable System. Also, if your child has what is called a nerve hearing loss your child can not be a candidate for Atresia repair due to the cochlea most-likely not functioning. At this point, your child may be a candidate for a Cochlear implant, but only if they have no hearing in both ears. If one ear still hears, you can not be a candidate for a Cochlear implant.
How is Canalplasty determined?
Below is a basis that each canalplasty surgeon goes off of in order to determine if you are a candiate for canalplasty when reading a CT scan. The grading system that is used is called the Jahrsdoerfer Grading Scale for Congenital Aural Atresia/Anatomical Structure Score or the “J” scale. It was created by Dr. Jahrsdoerfer. Usually, what makes an individual a candidate is if they receive a total score of 7 or more out of 10 (with 10 being the optimal score achieved based on ear structure (middle/inner).
Scoring for the Jahrsdoerfer Grading System:
Stapes bone 2
Oval window open 1
Middle ear space 1
Facial nerve 1
Malleus-incus complex 1
Mastoid pneumatization 1
Incus-stapes connection 1
Round window 1
External ear 1
Total Possible Score 10
Sometimes, if the score is a 6 or 7, or if the facial nerve is in the way, the adult or child seeking Atresia repair may not be considered a candidate for canalplasty. However, the surgeon may suggest the use of a “bone anchored auditory implant” or an “osteo-integrated bone-conduction device” (BAHA system). There is always a chance for prosthetic repair where only the surgeon can determine if he/she can still perform canalplasty and restore the hearing in that adult or child through the use of prosthetic bones that can be used in place of the incus, stapes, or malleus. This can only be determined by the surgeon and based on each individuals ear structure, grading system results, and what that surgeon believes he/she can do personally to restore the hearing for that individual.
When should I have a CT scan taken of my child’s ear?
It is suggested to have a CAT Scan taken closer to the time of planning for Atresia repair surgery and Microtia ear reconstruction. Keep in mind that if you have a CT scan taken too early of your child’s ear the scan may not show all of the current bone structures due to developmental changes that occur with age (primarily between the critical developmental years of 1 and 3 years of age). It is suggested that if planning to have Medpor ear reconstructive surgery with Atresia repair that a CT be taken of your child no earlier than at 2.5 years of age. If you are planning to have Rib Graft ear reconstructive surgery with Atresia repair, it is suggested to have your child’s CT taken around 5 or 6 years of age or just prior to surgery for planning purposes. Most hospitals do not request a CT scan following the birth of your child unless there are other accompanying syndromes, disorders, or conditions in addition to Microtia and Atresia that the doctor would prefer to rule out or detect. Again, if a CT is taken too early in development, it is possible that every detail in the scan may not be clearly visible thus putting your child at risk for needing an additional CT scan later. If your doctor is requesting that your child have a CT scan shortly after birth or during a younger age, ask if it isn’t something that can just wait until later when your child is older, mainly because of the concern for radiation exposure to your child.
If my child is underweight or small in stature should I wait to have Atresia surgery performed? Yes.
It is possible that your surgeon will suggest waiting another year or so just to make sure that your child is ready for Atresia surgery. A couple of things determine if your child should wait a bit for surgery. They are: Your child may have to meet a minimum weight limit in order to be placed under anesthesia for long amounts of time during surgery. The surgeon may ask your child to wait if he/she believes your child’s inner and middle ear structures may be prone to change because of your child’s inner ear bones still developing at a rate that could risk shifting of the bones following surgery. Also, if considering having the combined Atresia repair and Medopor surgery, again if your child does not meet the minimum weight of 28 lbs, you may be asked to way a little bit until they can meet the weight limit. This decision may also be determined with the help of the Medpor or Rib Graft surgeon and the parents. Illnesses and poor health (colds, flu, high white blood cell count) may also postpone these surgeries..
Will the hearing that was restored during Atresia repair decrease overtime or change?
The goal is to not have the hearing worsen once being repaired other than with what comes with old age. However, there are some situations that may affect restored hearing which are: the canal collapses – this can occur if Atresia repair is performed at too young of an age. There is no sound evidence as to why this only happens to some children and not others who have had Atresia repair performed at ages between 3 years and 12 years of age. Whether hearing is restored at age 3 or at age 12, there are still cases today where the canals collapse. This is commonly due to developmental stages caused by bony growths as the child grows older. As the child’s skull structure grows and changes the reconstructed ear canal can tear due to growth changes and the bones that have been restored may shift and lose their function during growth. Some suggest waiting until after the pubescent years for Atresia repair because of this exact reason. Again, the collapsing of the ear canal does not affect or happen to every child and there is no way to predict that this will happen. Another situation that may affect restored hearing would be if your child was in a collision or accident where they have suffered a concussion. There are some precautions that can help keep the canals from collapsing following surgery which are: the use of a plug “sponges” to help keep the canal from collapsing while the newly reconstructed ear canal is healing and the use of steroidal cream that can keep the ear canal open when applied following surgery (only if there is a threat of the canal closing).
* If the hearing is not restored following Atresia surgery and continues to worsen or not improve at all, there are a couple options: you can choose to do nothing if your child has unilateral atresia and still has one functioning biological ear with good hearing, attempt a revision surgery to improve the hearing in the Atretic ear once again, consider having a “bone anchored auditory implantable device” or “osteo-integrated bone conduction device” surgically implanted, or choose to wear a conventional hearing aid that can easily be worn in the new canal that has already been constructed (the Vibrant Soundbridge system).
Can my child get ear infections following Atresia repair? Yes.
A child can get an ear infections in the ear that has Atresia repair just as they would in the non-Atretic ear. Your child will not be prone to developing ear infections any more than having the occasional ear infection. It is very important to maintain the Atretic ear following surgery and throughout the years by taking your child to an ENT at least twice a year for routine maintenance checks and microscopic cleanings to remove dead cellular tissue build up. After having Atresia repair, the newly reconstructed ear canal will not secrete cerumen (ear wax), but instead, it will slough off dead skin cells that will need to be removed. This will help keep an eye on any typical ear infections that may develop and maintain your quality of hearing. It is also important to take your child to the audiologist at least twice a year in order to keep track of your child’s hearing and to monitor any changes in hearing.
Can my child swim after having Atresia repair? Yes.
However, no child should swim any time soon following surgery in order for the ear to heal completely. Your child’s surgeon will suggest to you how long your child should stay out of water whether it be for one month following surgery or three months following surgery. This can also be determined from post op follow-up visits. It is advised to put “swimmers ear” ear drops in your child’s ears every time after swimming in order to help dry out the canals so that an ear infection does not develop or so the water in the ear does not build up and begin to bother your child.
Will my child be sensitive to louder noises following Atresia repair? Yes and No.
Yes, your child may experience sensory overload or become extra sensitive to louder noises at first following surgery only because your child will be adjusting to his/her new hearing. This can also happen with a BAHA when it is worn at first. Your child may act out at times or become irritated when there is a loud noise and then be fine in the moments later after the loud noise has gone. In time, your child will adjust and this will get better as he/she gets used to their new hearing.
What happens following Atresia repair?
After the bandages come off and the risk of infection is minimal or gone and healing is complete your child can enjoy the miracle of hearing. There are a few things to keep in mind following canalplasty: following Rib Graft or Medpor surgery you may notice that the reconstructed ear may have a larger ear canal when compared to the non-Atretic/non-Microtic ear. The canal is man made and will be slightly larger, but hardly noticeable even when compared to an ear that may have been diagnosed with canal stenosis. It is important to go to the post op follow-up appointments and have the surgeon make sure everything is healing correctly and completely. It is important to listen to the suggestions and recommendations that the surgeon provides you with following surgery. The surgeon is the expert here and knows your child’s ear best, literally inside and out. Even if you take your child to another surgeon or ENT specialist who did not perform the surgery, they know ears and this is what their advanced medical degree is in. It is important to have regularly scheduled hearing tests, check ups and exams with both your audiologist and ENT. Always protect your hearing by keeping away from loud noises and protecting your hearing with the appropriate ear gear and ear plugs. Especially, around noisy work environments or during extracurricular activities such as hunting and shooting or near fireworks during seasonal celebrations. Bottom line, enjoy your hearing and protect it always. It is amazing at how far advancements in medical technology have come that can help us in everyday life and help us to live our lives to the fullest.
- When being troubled with questions about your ear or your hearing always seek the advice of a medical professional.
- If you are not satisfied with the advice from that medical professional seek a second or even a third opinion so that you can compare notes to help make the best decision.
- If an ENT or surgeon specialist refuses to help you maintain your ear that has been operated on by another surgeon, seek the advice of the original surgeon as to who to go to and ask who he/she would be willing to recommend.
- If your ENT or current medical professional you travel to for maintenance visits does not feel comfortable with maintaining your child’s ear encourage him/her to contact the surgeon who performed the surgery for suggestions on how to maintain your ear or ask for another surgeon/ENT that they can recommend in your area.
- It is important to do your own research.
- The more you learn and the more you know will only help you make the best decisions.