Microtia & Atresia: Where Do I Begin?

All of us have had different experiences during and after the deliveries of our little ones. I know some families are not told much at all following the delivery of their child. Nothing about Microtia or Atresia is explained to them, even when they inquire about their little one’s ear. Some families have told me that they had already been at home with their child for a number of months before their child was ever diagnosed with Microtia and Atresia. In fact, our support group has quite a few adult members who have either diagnosed themselves based on what they have researched on line or members who are forty years of age or older who discovered that they have Microtia just from joining our support group. Can you imagine growing up all of these years wondering about your ear and not understanding why you couldn’t hear or maybe why you have balance issues? I cry every time I receive an e-mail from a family or an adult saying that they had no idea that their child or themselves, as an adult now, had Microtia and or Aresia. It is just nice to be able to put a name to it and even more importantly, know that you are not alone. There are many adults and children who have Microtia and Atresia!

Here is also a wonderful document, “Integrated microtia and aural atresia management,” that sets the gold standard guideline of care for microtia and atresia that can be found at the following link.

The below seven steps will help you know where to begin following the delivery of your child:

Congratulations on the arrival of your family’s newest addition! Please enjoy your little one while you can because they grow up so fast. Soon, you will learn that everything is going to be okay. You will also realize that your child will be able to live a happy and healthy, normal life.

Step One:

Following the delivery of your child, while still in the hospital, your pediatrician or neonatologist will suggest that your child have a renal ultrasound and an EKG conducted. The reason for checking the kidneys (renal) and the heart (cardiogram) is because the ears, kidneys, and heart all develop during the 1st trimester of your pregnancy. So, if it appears that there is an issue with your child’s ears, your pediatrician will also want to make sure the kidneys and heart have fully developed and are fine. One of the main causes for concern with the heart is sometimes children born with Microtia and Atresia, Hemifacial Microsomia, Treacher Collins, Goldenhar Syndrome, even Down’s Syndrome may have tiny holes or cysts present in the heart that often go away shortly following birth. A pediatrician will want to make sure everything is fine and rule out any long term risks or concerns.

Step Two:

Following the delivery of your child, while still in the hospital, your child should have a newborn hearing screening conducted. This screening is a hearing test that your child will either pass or fail in each ear. If your child fails the hearing screening in one ear, you will then be referred to see an audiologist and an ENT for further testing. Your pediatrician should suggest scheduling appointments with both an ENT and an audiologist.

You may also contact the EHDI Organization (Early Hearing Detection Intervention) for answers about who your state contact is and also the law regarding newborn hearing screenings:  http://www.asha.org/Advocacy/federal/Early-Hearing-Detection-and-Intervention/    Here is also an article explaining how many parents still do not receive follow-up information or further details on what to do next that could help their children’s hearing loss after their infant fails the newborn hearing screening.  Please click on the following link to learn more:  http://www.ucdmc.ucdavis.edu/publish/news/injuryprevention/6058

Step Three:

When scheduling an appointment with your audiologist, request to have an ABR Test. An ABR (Auditory Brain Response) test is a test where the audiologist will hook up electrodes to your child’s forehead and behind each ear that will measure the electronic responses that will be recorded from the hearing nerves and brain. The results of this test should be able to tell you how much of a hearing loss your child has, so you know how much he/she is hearing or not hearing in decibels (dB). In addition to the ABR test, your audiologist will also conduct other hearing tests such as:

AEP (auditory evoked potential evaluations)
ASSR (auditory steady state response evaluations)
SAT (speech awareness threshold)
VRA (visual reinforcement audiometry)
DP(OAE) and the TE(OAE) which are both auto-acoustic evaluations.

Step Four:

Schedule an appointment with an ENT (ear-nose-throat specialist) or a pediatric Otolaryngologist. This appointment should help you learn more about Microtia and Atresia or any other associated syndromes or disorders your child may have. Your ENT should also be able to tell you which grade of Microtia your child has based on looking at your child. Inquire about keeping an eye on ear infections and what kind of auditory concerns you should have for your child due to his/her hearing loss. Also, inquire about assisted hearing devices such as bone anchored auditory devices on a soft band head band (known as a BAHA, bone anchored hearing aid). Now, go back to your audiologist and inquire about trying on a BAHA and getting a loaner or trial BAHA to wear so you can decide if you would like to purchase one. You must be very clear about the BAHA being on a soft band head band otherwise, your audiologist and ENT will think you are talking about the bone anchored device that gets implanted and you can not have a BAHA implanted until the age of five (mandated by the FDA).

Step Five:

Learn if your baby should have a CAT scan.

Step Six:

Learn about the risk of ear infections.

Step Seven:

Now that you have learned a little bit more about your child’s Microtia and Atresia, hearing loss, and hearing aid options, you can begin doing more research on other options that your child has. Some of these options are:

Do Nothing Option (keep your little ear): Begin thinking about your child and his/her ear and if you would like to consider ear reconstructive surgery or raise your child to accept his/her little ear with lots of supportive and confidence. Your child will live a perfectly normal life with a little ear or little ears.

Canalplasty Surgery (Atresia Repair) – An option for hearing restoration besides a BAHA or hearing aid.

Ear Reconstructive Surgery: There are two surgical techniques. 1. Rib Graft. 2. Medpor.

Additional Facial Plastic Surgery

Begin contacting calalplasty, rib graft, medpor and maxillofacial/craniofacial surgeons so that you can learn more about the options for your child and also begin planning for surgery if surgery is something you are considering. By making contact with surgeons and doctors early on, your and your child will have a better idea of when some surgeries can begin and when you may have to wait for others to take place.

Prosthetic Ear: Be thinking about the option for a prosthetic ear instead of having surgery. Prosthetic ears today look amazing and very real. It is an excellent alternative to having ear reconstructive surgery.

  • If for some reason, no one contacts you or tells you any of the above or makes any suggestions as to who to see regarding your child’s Microtia and Atresia, please feel free to reach out to the following helpful resources:

Schools for the Deaf and Blind

The House Research Institute:  www.houseearclinic.org

The Hearing Loss Association:  www.hearingloss.org

Child Find – Early Intervention Services:  www.cde.state.co.us/early/childfind

Birth 2 3 Organization – Early Intervention Services:  www.birth23.org

Global Foundation for Children with Hearing Loss:  www.childrenwithhearingloss.org

Contact your local Children’s Hospital

Contact Vanderbilt Universitywww.MicrotiaandAtresiaCare.com

Contact Standord University:  http://www.stanfordchildrens.org/en/service/microtia

Contact the EAA (Educational Audiology Association):  https://edaud.org/

Contact NCHAM (National Center for Hearing Assessment and Management) :  https://www.infanthearing.org/

Contact ASHA (American Speech-Language Hearing Association):  http://www.asha.org/

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