Atresia in general is the absence or closure of a natural passage of the body. Aural atresia refers to the absence an external ear canal. When someone has aural atresia, there is a high incidence of malformation of the external ear and middle ear also, but the inner ear and auditory nerve are frequently normal. In most cases an eardrum (a middle ear structure) does not develop and then middle ear bones may be malformed as well. A narrowed ear canal (i.e. one where the eardrum can be viewed, but the canal is narrower than normal) is sometimes referred to as a stenotic canal, or canal stenosis. Aural atresia most commonly affects just one ear (unilateral aural atresia), but can occur in both ears (bilateral aural atresia). The surgery to create a normal sized ear canal from either a stenotic canal or complete aural atresia is known as an “atresia repair” and is sometimes also referred to as a canalplasty.
The ear can be broken down into 3 components: internal, middle and external portions. Microtia refers to deformity of the external portion of the ear and is present from birth. Microtia is graded on a scale of from 1-4:
Grade 1: Small ear with mostly normal ear anatomy, the ear canal may or may not be affected.
Grade 2: Characterized by some ear anatomy, including a developed tragus. The bottom 1/3 of the ear is usually normal, no ear canal or a narrow canal.
Grade 3: Most common form of microtia, characterized by small, undeveloped ear remnants and no canal.
Grade 4: Also referred to as anotia, no ear remnants or canal present.
The ear canal is always affected with Aural Atresia if the outer ear malformation is graded 2-4. Also, the outer ear can be normal while the ear canal is affected with atresia. Never is there a situation where a normal ear canal is present and no outer ear develops.
Evidence supporting the importance of binaural (two-eared) hearing continues to surface. Strong data shows children with a unilateral hearing loss have disadvantages in hearing in noisy environments and with sound localization, developing normal language, work and school performance and several other areas. The brain system that provides these functions develops in the first 5-10 years of life causing us to advocate hearing restoration at a young age. Frequently, if hearing is not provided in the early years, the brain cannot develop fully at a later time, limiting achievement and function in later life. Commonly, these disadvantages may begin to surface as a child’s demands in school increase around 4th grade-AFTER the system has failed to develop. A unilateral hearing loss child may be able to hear, process and perform very well in quiet environments. In environments, such as a noisy classroom, the same child’s hearing and performance can drop nearly 50% without any intervention. Children with unilateral hearing loss are over 10 times more likely than their binaurally equipped peers to fail a grade, have more behavioral problems, and have a greater need for educational assistance.
The recommendation for nonintervention because “one ear is sufficient” is no longer justifiable. In almost all situations, we can provide hearing to an ear affected with Atresia or Atresia/Microtia. Several options are available for use to achieve hearing. Read more about this topic
The California Ear Institute, which has some of the most experienced atresia repair surgeons in the world, can undertake atresia surgery in children as young as 3. There are other conditions, such as a cholesteatoma, that warrant earlier intervention. It is preferred that atresia surgery not be performed prior to a child reaching 30 pounds and 3 years of age.
Audiology Workup: While several tests cannot be performed with atresia, tests that evaluate the status of the cochlea and hearing nerve are necessary to determine if the inner ear has formed correctly. It is important not to forget the “normal” ear in patients who have one-sided atresia, as approximately 27% of these individuals may have a hearing loss in their non-atresia side. A full workup on both sides is advised. CT: a CT of the temporal bone at 2.5 years of age is recommended. It is possible that CT’s have been performed on children with atresia prior to this age to address different concerns beyond the ears. For the purpose of determining whether or not a child is a candidate for atresia repair, it is not recommended for the scan be performed before 2.5 years unless there is evidence of a cholesteatoma. SEE FAQs BELOW FOR MORE ON CHOLESTEATOMAS
Receptive language development (understanding of what is said to you) and expressive language development (ability to speak) proceeds rapidly in the first stages of life, with a majority developing before the age of 5. We recommend bone-conduction devices, usually in the form of a Bone Anchored Hearing Aid (BAHA) softband device, as soon as possible after diagnosis of atresia to stimulate the inner ear affected with a hearing loss from Atresia. This is to be used prior to surgical correction or until reaching the age where conversion to a more permanent device can take place. More information on device options – Click Here
To evaluate the middle ear, a CT scan of the temporal bone needs to be performed. While the risk of radiation in today’s scanners is much smaller than prior scans, we recommend every effort to be made to get the scan needed in one attempt and to avoid having to repeat scanning.
In some areas, miniCAT scanners are available. These have greatly reduced radiation exposure and allow children to sit upright and avoid sedation in some instances (9% of the usual radiation dose of most commonly used CT scanners). At the California Ear Institute offices in Palo Alto, miniCAT services are available for our patients. These studies can be performed and results interpreted within a matter of minutes.
If not accessible, a standard CT can be performed with sedation under general anesthesia for young children. Pediatric hospitals tend to be the best resource for these scans. Here are guidelines for your physician ordering the CT.
A CT gives a picture of the middle and inner ear anatomy not visible by physical exam. The CT can be read and given a grade, applying the Jahrsdoerfer scale. This 10-point grading system developed for determining surgical candidacy of atresia patients, can loosely determine chances that atresia repair surgery would bring hearing up to the normal range (0-30dB).
Scores of 8 or 9 Atresia repair is recommended
Scores of 6 or 7 Atresia repair is possible and dependent on specific patient
Scores of 0 to 5 Atresia repair is generally not recommended, unless there is concurrent sensorineural hearing loss and the need to wear traditional hearing aids is essential to restore hearing or in some patients who have atresia in both ears.
While the J-scale is one of the best methods for guiding recommendations for atresia, it certainly is not a complete algorithm as there are some other factors important to consider, such as unilateral v. bilateral atresia, the benefit of sound awareness and the ability for the localization of sound.
Each person’s situation is different and should be fully evaluated by a surgeon with experience in treating atresia and microtia patients. It is imperative the surgeon who is considering surgery review the CT scan personally.
A CT scan also determine the presence of a cholesteatoma, which is a common condition associated with atresia.
If the patient is not a good atresia repair candidate, there are other avenues that can provide hearing. The hearing loss produced by lack of an ear canal and eardrum can be ‘bypassed’ with vibrations in the bone surrounding the inner ear, which in turn stimulates the hearing nerve. For information on the latest technology, see Device Options.
A cholesteatoma is a destructive growth of keratinized squamous epithelial cells (skin cells), often described as a skin cyst, located in the base of the skull (mastoid) and/or middle ear. Cholesteatomas have a higher incidence in children with atresia and those with small or partial ear canals.
Cholesteatomas grow over time and have the potential of eroding through bone. If erosion of the middle ear ossicles and/or inner ear space occurs, hearing (or the potential for hearing in cases of atresia) can be permanently compromised. Other possible complications arising from cholesteatomas include dizziness, facial paralysis and infection of the brain (meningitis), which can potentially be fatal.
People with normal ear canal anatomy often experience painless drainage from the ear as an initial symptom but patients with atresia or partial canals may not show any signs or symptoms of a cholesteatoma until it becomes infected. At this point, intervention is emergent. A pit where the ear canal should have formed may be a sign of buried cholesteatoma.
The standard of care is to surgically excise the cholesteatoma after diagnosis in almost all cases to prevent the risks associated with leaving it untreated.
Atresia/Microtia is frequently isolated, meaning that a patient with atresia has no other health problems. In our experience in working with atresia patients around the world, we have found atresia microtia to be a component with an underlying syndrome in approximately 8% of patients. These syndromes include: Treacher Collins, Hemifacial Microsomia, Goldenhar, Crouzon’s, Pfeiffer, Apert, Nager/Miller, Klippel-Feil, Brachio-Oto-Renal or Melnick-Fraser, Pierre Robin and CHARGE syndrome. A thorough workup should be performed by a child’s pediatrician or geneticist. Jaw or facial abnormalities are more frequent and may be seen in over 20% of patients with atresia. Jaw and face experts are part of our treatment team at CEI Medical Group. Evaluation includes the assessment and potential treatment planning to address all of these disorders when present.
At the end of ever surgery, patients wake up with no pain due to long-acting pain medication placed in the tissues while they are asleep. After several hours, mild pain may develop. The pain experienced is much less than one might expect. While prescription strength pain medication is prescribed for use if needed, we find that most patients’ pain is simply controlled with Tylenol. It is common for kids to be completely without discomfort within days.
No. The process of determining whether or not a person with atresia is a good candidate for atresia repair is a very selective process. In recommending surgery, statistics show that there is a high likelihood of achieving results that bring a patient’s hearing into the normal range for a properly selected atresia patient (0-30dB). While there are some variables that we have been able to overcome (i.e. PORP in the cases of a fibrous incudostapedial joint) with experience in treating this condition, surgical outcomes are not always predictable. In formulating an individual treatment plan, chances of success and failure are discussed openly.
With Medpor and rib graft reconstruction, plastic surgeons will attempt to make the ear look as true to a normal ear as possible. However, attempts of recreating the appearance of a canal are limited by working with the soft tissue superficial to the bone that failed to allow for canal formation in the first place. While the indication is solely for a more true aesthetic appearance, creating a partial canal is a request we often get as Dr. Roberson is specialty trained in skull-based surgery. More frequently performed in tandem with the placement of implantable devices for hearing of an affected ear (i.e. BAHA or VSB), the desire of making a true partial canal can be fulfilled. Hearing outcomes will not be affected either way. In some cases, an ear canal can be created that we know will not produce good hearing by itself but can allow a hearing aide to be used to return hearing a functionally normal range.
At the California Ear Institute, a team of trained professionals has been hand selected to care for patients diagnosed in atresia and microtia. We care very much about the health and well-being of our patients and seek to extend the best care possible. Emailing us at firstname.lastname@example.org is the easiest way to establish contact. Contact Us
Yes, there are several other mechanisms to provide hearing to an ear that has a normally formed inner ear. Individual plans for each patient are developed and described before any treatment is started at CEI. Dr. Roberson and/or his team provide information and answer questions so that you can make a fully informed decision. Our website [atresiarepair.com] is dedicated to helping you understand some of these options.
Infection is a possible complication of any surgery, atresia surgery included. Also, atresia repaired ears can get infections like any normal ear. Many lines of defense are in place, keeping the likelihood of an infection developing very low. If an infection was to develop, treatment may range from antibiotic eardrops to oral antibiotics to surgical intervention, in very rare cases.
One thing to keep in mind is that a surgically repaired ear is still at risk for developing middle ear infections (otitis media) just as a regular ear would. Poor eustachian tube function, repeat colds and upper respiratory infections, exposure to cigarette smoke and environmental pollutants, craniofacial abnormalities and allergies that place all at higher risk for developing ear infections certainly extend to atresia patients. Preventative action (i.e. allergy prophylaxis) should be taken when possible. Attentive monitoring and treatment of middle ear infections of both reconstructed and normal ears is warranted by a primary care physician or local ENT.
A second stage surgery for the placement of a middle ear bone prosthesis is indicated in some patients. This decision is made based on hearing testing results. Patients who are a candidate for the second surgery are those that show a lack of adequate improvement in the hearing after surgery.
In certain cases, the decision to place a prosthesis is made at the initial surgery if it is clear that the bones will not be functional for sound conduction. Unfortunately, CT results and intraoperative exam are not 100% in being able to predict hearing outcomes. The complications for which an additional surgery may be recommended include lateralization of the eardrum, an infection not responding to antibiotics, perforation of the ear drum and mucosalization of the canal (also known as “wet ear canal”). Frequently, these complications are managed without needing to intervene surgically.
A surgically constructed ear canal does not have the capability to clean itself like a regular ear canal does. Because of this, regular cleanings performed under microscopic examination must be performed by a local ENT to remove the debris that builds up in the canal. Failure to do so places the patient at increased risk for developing an infection. The time between these cleanings varies from patient to patient but in the first year, every 3-4 months is often recommended. Cleanings can usually be spaced out to every 6-12 months or longer following the first year after surgery but will be determined by a local ENT based on debris production.
California Ear Institute physicians work with other ENTs around the world for detailed post-operative follow-up. Only if serious complications arise will a patient have to return to our facilities. We recommend a hearing test be performed around 4 months after surgery and ask for those results to be emailed to us.
Swimming can resume once the canal is completely healed and you have been cleared to do so, usually 6-8 weeks after surgery. In patients prone to having residual water in the canal after water exposure, we often advise placing several drops of rubbing alcohol in the canal after swimming to prevent infection.
The canal needs to be kept dry for the first 6-8 weeks following the surgery. Keeping the canal dry can pose a challenge, especially when young, active children are involved. During bath time, placing a cotton ball coated lightly in Vaseline over the entrance of the canal can help prevent water from entering.
During the stay in Palo Alto, we typically see patients within a day or two after surgery and every week following for post-op appointments until the return home.
There is packing placed in the ear canal during surgery that needs to be removed following a specific schedule. You will be given your post op appointments before surgery but these may be adjusted depending on the healing process, as every person heals at a different rate, so flexibility is important. Drops will need to be placed in the ear starting at the 1 week post-op appointment and for the following 3-4 weeks. Once the canal is healed, a silicone-based mold is made that will be worn every night for 4 months or until directed to discontinue. Drops will continue to be used at night while the mold is being worn. Use of the mold has drastically reduced the incidence of narrowing of the created canal. Please note, a new mold for the canal should be made at CEI after the Medpor. For patients who have Medpor reconstruction following canal surgery, we strongly request a visit to CEI before returning home for us to make this mold under the microscope.
In 2008, with a new technique, we started taking the skin grafts from the scalp for atresia repair. Read more about this technique here. Formerly, these skin grafts came from either the abdomen or the thigh. Using these areas created some discomfort for the patients and sometimes caused scarring. The skin grafts taken from the scalp are so thin, hair growth on the scalp is unaffected and no hair grows in the canal. They are much less painful for the patient and they do not leave a visible scar. Hair generally grows back very quickly on the scalp, as the hair follicles are not damaged in the process.
There is a single small skin graft (about 2 inches by 3 inches) taken for lining the canal when the atresia repair surgery is happening by itself. A slightly larger skin graft (about 3 inches by 4 ½ inches) is used for the canal portion of CAM procedures, in addition to the grafts needed for the outer ear reconstruction. The grafts for the outer ear during the CAM are most often taken from the groin area and/or the back of the normal ear in single sided cases.
The complication rate for both the Medpor and canal is increased by significantly when the Medpor is performed prior to the canal. Because a plastic surgeon’s work is done superficially, he or she is unable to determine where the canal should be placed in relation to the patient’s middle ear. In some cases, loss of the Medpor prosthesis has occurred when the ear canal is created after Medpor surgery. Note an ear canal procedure can safely be done after Medpor in many cases remains by very experienced surgeons but we highly recommended that a person undergo atresia repair either before or at the same time of Medpor reconstruction.
It is recommended that there be at a minimum four months between canal surgeries in children who have bilateral atresia. Ideally, there would be at least 6 months of healing time between an atresia repair and Medpor reconstruction performed on the same ear.
In atresia patients, the external auditory canal failed to form properly in the first few weeks in the uterus. Instead of a canal extending from the outside to the middle ear, bone and in some cases soft tissue fills this space.
During surgery, this soft tissue and bone is delicately removed by special instruments to create a pathway down to the middle ear bones. The middle ear bones are examined and in some cases need to be replaced with a prosthesis, based upon evaluation of their mechanical capabilities. [Note: because of the minute size of these structures, the world’s best CT scanners are not even powerful enough to determine the connection between the middle ear bones—something we can only know for sure at surgery.
An eardrum is created from muscle fascia and draped over the middle ear bones. This tissue heals and becomes a living, functioning eardrum very similar to a normal eardrum. A skin graft is taken from the scalp for lining of the newly created canal and to surface the top of the new eardrum.
The tissue is sutured to the existing skin around the newly made canal and is packed with antibiotic gel foam and sponges that will be removed in post op visits.
One benefit of the California Ear Institute being a private institution as opposed to large educational facility is that you can be assured that Dr. Roberson will be performing the surgery in all atresia cases. An experienced staff of surgical techs, nurses, PA’s, anesthesiologists and doctors make up the team created to extend the best possible care to patients.
It is recommended that patients undergoing atresia surgery (canalplasty) stay at least 2 weeks, preferably 3 weeks after surgery as complication risks are minimized with close monitoring during recovery. For patients having the CAM procedure, a 4-week minimum stay is advised and travel will be required to Los Angeles for follow up appointments during a portion of that time.
We have found that it is important that the newly made canals and/or ears are monitored very closely within the first several weeks following surgery in order to maximize surgical outcomes.
We highly advise that follow up appointments with your local ENT be made before your return home to ensure continuity of care. We will provide detailed instructions for your ENT regarding the post op process and they will always be able to contact us directly if further questions remain.
All patients are scheduled for a pre operative appointment with our staff at the California Ear Institute the day before surgery (or on a Friday if the surgery falls on a Monday.) An audiology appointment will be scheduled in conjunction with this appointment. In certain circumstances, a CT may also be performed at this time.
Costs for atresia repair surgery is highly variable and depends on the complexity of the surgery and the patient’s insurer. There are three sources of billing for atresia repair surgery:
The facility, which bills based on the amount of time the surgery takes. The facility will also bill for prosthetics, should titanium replacements for the ossicles be required
The anesthesiologist, who bills based on the length of the surgery
The surgeon’s fee, which depends on the procedures performed and the complexity of the procedures
In the United States, insurers will inform you what they will pay for surgery based on CPT codes. For domestic patients, our staff can help provide you with those codes and/or work with your insurance company to determine the estimated reimbursement from the insurance company for the cost of the surgery.
For international patients, CPT codes do not apply and we have established fees for the facility, anesthesiology and surgeons.
For all patients, a $5,000 deposit is required to reserve a surgery date, with the total cost of the surgery due 3 weeks prior to surgery date. We have had some international patients who live in countries with socialized medical care in which atresia repair is not available. Our staff can also provide documentation as needed if this applies to you.
Please contact us for more information regarding the financials of surgery.
In late January, 2008, Dr. Joseph Roberson and Dr. John Reinisch joined to perform the world’s first procedure in which atresia repair and medpor auricular reconstruction were combined into a single surgical procedure. The results for a CAM (combined atresia microtia procedure), appear to be as good as the traditional two or occasionally three procedures based on our work in the first 100 patients. Dr. Roberson has now been part of over 120 CAM procedures since 2008 with two other surgeons. Several additional factors are taken into consideration when determining whether or not a person is a good candidate for the CAM procedure and should be discussed in more detail with Dr. Roberson and staff.
There are currently two major approaches to the outer ear reconstruction. One technique uses an autologous rib graft. Rib graft auricular reconstruction generally begins around age 6 and requires 3-4 surgical procedures (for unilateral microtia).
Another technique in microtia repair uses porous polyethylene (known as a Medpor) in place of the rib. Medpor auricular reconstruction surgical can start as early as age 3 years. The Medpor out ear is placed in one operative procedure but at times minor surgical revisions are needed after the first surgery as directed by the Medpor surgeon for optimal outcomes.
One very important detail is that with the Medpor approach, atresia repair is done BEFORE the outer ear surgeries and with the rib graft approach, atresia repair is done AFTER the outer ear surgeries. In selected patients (in a technique first described at the California Ear Institute), both an ear canal and outer ear can be created in a Combined Atresia Microtia Repair (CAM) using the Medpor technique for the outer ear reconstruction.
Risks with atresia surgery include infection (<1%), injury to inner ear (<1%), facial nerve injury (<1%) and risks associated with anesthesia. Complications that can develop include stenosis of the newly created canal, lateralization of the newly created eardrum, external auditory canal or middle ear infections and perforations. Rarely do these require surgical intervention. Preventative measures are taken, such as use of antibiotic eardrops and a custom fit mold worn at night, that greatly minimize the risk of these complications.
Reconstruction of severely malformed middle ear bones is required in ~13% of cases for optimization of hearing outcomes. In many cases, this reconstruction can be done at the first surgery. In some cases, a second surgery is required. Read more about Fibrous IS Joint Abstract.