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Microtia

  • Overview

    "While it can be upsetting to see your child with an abnormal ear, it's good to keep in mind that most children still have some hearing out of the ear and that the ear can be repaired."

    When your baby was born, your first emotion was probably joy. Then, you (or your doctor) noticed something was slightly wrong: Your baby’s ear seemed abnormally small, malformed or possibly even missing.

    You likely wondered, “Why my baby?” or “What did I do wrong?”

    It’s common for parents to have questions like this when their baby is born with a congenital anomaly or birth defect. And while it can be upsetting to see your child with an abnormal ear, it’s good to keep in mind that most children still have some hearing out of the affected ear and that the ear can be repaired. We’ve put together some information that can help you learn about what microtia is—and how our experts at Children’s Hospital Boston can help you.

    Here’s what you need to know about microtia:

    • Microtia is a congenital condition in which a baby is born without one or both external ears or with an ear deformity.
    • Microtia can affect one (unilateral) or both (bilateral) ears.
    • The unilateral form is much more common, occurring in about 90 percent of affected children..
    • Microtia occurs in one in about 6,000 babies.
    • In most cases, there is only about a 40 percent reduction in hearing of the affected ear. Most children with microtia have normally developed inner ear canals with an absence of the external ear.
    • Surgeons can surgically construct a new ear for your child.

    For more information about treatment options, see our Treatment & Care section.

    How Boston Children's Hospital approaches microtia
    At Children’s, our surgeons are skilled at both repairing malformed ears and building new ears. Our dedicated Microtia Program is within our Department of Plastic and Oral Surgery, which performs more than 2,000 surgeries a year and cares for over 13,000 outpatient visitors annually.

    Surgeons in our Microtia Program typically perform an autologous (using your child’s own tissue) reconstruction.

    In this procedure, a new ear is made from your child’s rib cartilage and placed under the skin on the side of the scalp where the ear would be. In addition, the surgeon refines and repositions the ear lobe and reconstructs the contour of the ear. For more information on this procedure, see our Treatment & Care section.

    Help for hearing loss
    Children’s Diagnostic Audiology Program offers expert evaluation and management of infants, children and adolescents with various degrees of hearing loss.



    Reviewed by Arin K. Greene, MD, MMSc

    ©Children’s Hospital Boston; posted in 2011

  • In-Depth

    It’s certainly upsetting to see your baby born with a malformed ear.

    However, the good news is that most children with microtia still have some hearing out of the affected ear. And, as your child gets older, our plastic surgeons can reconstruct his ear.
    Most important, most kids with microtia don’t have other associated health problems—and go on to lead normal, happy lives.

    Microtia is divided into four classifications based on how the ear is affected:

    Grade 1—This is the mildest form of microtia.The affected ear is smaller than normal, but all the parts of the outer ear exist.?

    Grade 2—In this type of microtia, many of the normal features of the outer ear are missing.?

    Grade 3—This is the most common form of microtia.
    Here, the affected ear consists simply of a vertical appendage of skin and cartilage.
    Typically, there is no external auditory canal.?

    Anotia—This is the most severe type of microtia.
    If your child has anotia, it means that one (or both) of his ears is absent.
    Even in this type of microtia, the inner ear may be relatively normal and your child may still be able to hear out of the affected ear.

    Why is microtia a problem?
    Microtia looks worse than it is. In fact, most children who have microtia have a relatively normal inner ear, which means that—even if there’s no outer ear at all—they can still hear.

    Microtia can be a cosmetic problem, but even the social effects of having a malformed ear can be mitigated if you talk honestly with your child to educate her about her condition—and give her plenty of emotional support.

    About our Department of Pediatric Plastic Surgery
    Read all about how our department helps children with conditions like microtia.


     

    Causes?
    What causes microtia? We don't really know.

    Microtia occurs when there is a problem with development of the ear when a baby is still an embryo.
    Occasionally, microtia occurs due to an inherited defect, but more often than not, it occurs spontaneously with no family history.
    Microtia can also occur as part of a spectrum of syndromes, especially hemifacial microsomia.

    Why couldn't we see it on the ultrasound?
    Most ultrasound technicians focus on your baby’s internal organs and skeletal structure.
    As ultrasound technology becomes more accurate, however, microtia can sometimes be identified before your baby is born.

    How common is microtia?
    Microtia occurs in one in 6,000 babies. It’s more common in boys than in girls.
    The chance of an affected parent transmitting this to their child is usually small (less than about 6 percent), but there are some families who may carry a gene for microtia.

    Signs and symptoms
    What are the symptoms of microtia??The signs and symptoms are clear: A baby is born without one or both external ears or with an ear deformity.

    Questions to ask your doctor?
    You and your family are key players in your child’s medical care. It’s important that you share your observations and ideas with your child’s health care provider and that you understand your provider’s recommendations.??If your child was born with microtia and you’ve set up an appointment, you probably already have some ideas and questions on your mind. But at the appointment, it can be easy to forget the questions you wanted to ask. It’s often helpful to jot them down ahead of time so that you can leave the appointment feeling like you have the information you need.??You may want to suggest that your child write down what he wants to ask his health care provider, too.??Some of the questions you may want to ask include:

    • What sort of surgical options are there?
    • Are there alternatives to surgery?
    • How well will our child be able to hear?
    • What can we do to help prepare our child for questions about her ear?
    • Where can we go for further information?

    FAQ

    Q: How common is microtia?
    A: Microtia occurs in one in about 6,000 babies.

    Q: How will microtia affect my child’s hearing?
    A: It’s reasonable to assume that a child with microtia would not be able to hear out of the affected ear.

    However, in most cases, there is only about a 40 percent reduction in hearing of the affected ear.

    Most children with microtia have normally developed inner ear canals with an absence of the external ear.

    Q: What happens if my child has hearing loss?
    A: If your child has a hearing problem related to his microtia, your audiologist and pediatrician will arrange for your child to be seen by an otolaryngologist (ear, nose and throat specialist) and will guide you to other services to help your child learn to communicate.

    Our Diagnostic Audiology team will:

    • monitor the hearing loss
    • provide counseling to you and your family regarding the hearing loss
    • recommend hearing aids and ways to maximize the use of what hearing your child does have
    • refer your child for additional language or developmental assessments
    • direct you to community resources for educational or financial assistance
    • refer your child to Habilitative Audiology to learn about the option of a cochlear implant if your child has a severe to profound hearing loss in both ears

    Coping and Support

    At Children’s Hospital Boston, we understand that you may have a lot of questions when your child is diagnosed with microtia. Will my child be able to hear? What do we do next? We’ve provided some answers to those questions in the following pages, but there are also a number of other resources to help you and your family through this time.

    • Children’s Center for Families is dedicated to helping families locate the information and resources they need to better understand their child’s particular condition and take part in their care. All patients, families and health professionals are welcome to use the Center’s services at no extra cost. The center is open Monday through Friday from 8 a.m. to 7 p.m., and on Saturdays from 9 a.m. to 1 p.m. Please call 617-355-6279 for more information.

    Children’s Behavioral Medicine Clinic helps children who are being treated on an outpatient basis at the hospital—as well as their families—understand and cope with their feelings about:

    • being sick
    • facing uncomfortable procedures
    • handling pain
    • taking medication
    • preparing for surgery
    • changes in friendships and family relationships
    • managing school while dealing with an illness
    • grief and loss

    Visit the Behavioral Medicine Clinic page or call 617-355-6688 to learn more. ?
    The Experience Journal was designed by Children’s psychiatrist-in-chief David DeMaso, MD, and members of his team. This online collection features thoughts, reflections and advice from kids and caregivers about going through cardiac disease, heart transplants and many other medical experiences.?

    Children’s Pediatric Psychiatry Consultation Service is made up psychologists, psychiatrists, social workers and other mental health professionals who understand the unique circumstances of hospitalized children and their families. They work with children who have been admitted to the hospital—and their family members—and operate on a referral basis. For more information, visit the Pediatric Psychiatry Consultation Service homepage. If you are interested in setting up an appointment, please speak to your child’s treating clinician.

    Children’s Department of Psychiatry offers a free booklet, “Helping Your Child with Medical Experiences: A Practical Parent Guide” (Please note that Adobe Acrobat is required to view and download the guide.) Topics in the booklet include:

    • talking to your child about his or her condition
    • preparing for surgery and hospitalization
    • supporting siblings
    • taking care of yourself during your child’s illness
    • adjusting to life after treatment

    Children’s chaplaincy is a source of spiritual support for parents and family members. Our program includes nearly a dozen clergy members—representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions—who will listen to you, pray with you and help you observe your own faith practices during your child’s treatment.?
    On our Children’s For Patients and Families site, you can read all about:

    • getting to Children’s
    • accommodations
    • navigating the hospital experience
    • resources that are available to your family at Children’s

    What is the long-term outlook for my child?
    Most children with microtia go on to lead normal, happy lives.

    When your child gets older, our plastic surgeons can construct the affected ear, so that it looks relatively normal.

    Surgical procedures usually don’t begin until your child is at least 6 for two reasons:

    We recommend waiting so that your child has enough rib cartilage for surgeons to use to reconstruct his ear.
    Also, when your child is 6, his ear will about its adult size, so surgeons can maximize symmetry with his other ear.

    Our doctors can also treat your child for hearing loss if necessary.

  • Tests

    How is microtia diagnosed?
    Because microtia is a congenital condition, your child's malformed ear will be obvious at birth, and therefore diagnosed by the doctor immediately upon physical examination.

    Doctors also recommend an Auditory Brainstem Response Evaluation soon after every child’s birth to evaluate babies’ inner ear function on both sides.

    What is an Auditory Brainstem Response Evaluation (ABR)?
    An ABR is a type of test that evaluates how well sounds travel along the hearing nerve pathways from the ear to the brainstem.

    There are two main types of hearing loss:

    Conductive hearing loss—problems transmitting sound from the outer ear (where sound is collected) to the inner ear.
    This is the predominant type of hearing loss that’s associated with microtia.

    Sensorineural hearing loss—problems with the nerves connecting the inner ear to the brain. In the inner ear, tiny hairs on the cochlea act as a neural pathway, transmitting through the inner ear. Usually, problems with these hairs on the cochlea are responsible for sensorineural hearing loss.
    Sensorineural hearing loss is present in 10 to 15 percent of children with microtia.

    How does it work?
    An ABR test is a sleep EEG hearing test, which shows the softest sounds your child's ears can detect at various pitches. The ABR compares changes in brain activity to the timing of repetitive sounds to determine whether the particular intensity of sound can be heard.

    How is the test performed?
    There will be three or four small stickers on your child's head, connected to leads going into a computer. Sounds will be presented through an earphone to each ear separately while a computer analyzes the changes in the brain wave pattern in response to sounds.

    Your child should be sleeping for the duration of the test. Young children under the age of 6 months are not usually sedated, and the test is performed while your baby is naturally sleeping, following a feeding. Children older than 6 months are usually sedated.

    The test is not painful or uncomfortable in any way, but it is necessary for your child to be asleep in order to obtain clear recordings during the test.

    You will be in the room with your child during the test, and the results will be explained immediately afterward.

    How long does an ABR test take?
    ABR evaluations, both sedated and unsedated, take two to three hours to complete.
    Important:

    Please follow these feeding instructions before your child’s ABR test.

    Under 6 months of age:
    no solid food, milk or formula for four hours before the test

    6 months or older:
    no solid food, milk or formula for six hours before the test

    For all children:
    Clear liquids such as water, breast milk or apple juice should be given until two hours before the test.
    Please bring your child awake but very sleepy.

    Hearing clearly
    If your child has significant hearing loss because of his microtia, Children’s Diagnostic Audiology Program can help.

    Specially trained audiologists conduct diagnostic tests to:

    • assess hearing disorders
    • provide guidance to families of children with hearing loss
    • recommend appropriate care for hearing loss
  • Undoubtedly, you were distressed when you saw your baby's malformed ear. That's understandable—no parent wants his or her child to be born with a congenital anomaly.

    At Boston Children's Hospital, we view the diagnosis as a starting point: Now we're able to begin the process of treating any hearing loss your child may have—and making plans for reconstructive surgery.

    At Children's, we consider you and your child integral parts of the care team and not simply recipients of care. You and your care team will work together to customize a plan of care for your child.

    How will my child's microtia be treated?
    Treatment of microtia usually involves reconstructive surgery to rebuild the outer ear.

    This is a very delicate procedure that requires expertise in pediatric plastic surgery.

    Surgical procedures usually begin when your child is at least 6 years old for two reasons:

    We recommend waiting so that your child has enough rib cartilage for surgeons to use to reconstruct his ear.

    Also, at 6 years old, his ear will approximately its adult size, so surgeons can maximize symmetry with his other ear.

    What's the surgery like?
    Surgeons in Children's Microtia Program typically perform an autologous reconstruction.

    Autologous reconstruction—using your child's own tissue
    A new ear is made from your child's rib cartilage and placed under the skin on the side of the scalp where the ear would be.

    Because this process uses your child's own tissue, it will continue to grow as your child grows.
    In addition, if the ear becomes injured, it can heal itself.?
    This reconstruction process involves between two and four separate surgeries depending on your child's type of microtia. The typical surgeries are as follows:

    • first operationharvesting and carving rib cartilage from your child to build the framework of the ear
    • second operation—refining and repositioning the ear lobe and reconstructing the contour of the ear canal
    • third operation—elevating or lifting of the ear

    We usually space these procedures out by six months each, so that your child can heal before the next stage.

    If doctors decide that your child needs surgery on her middle or inner ear, this is done after the outer ear reconstruction is finished.

    Some hospitals perform reconstructions using synthetic materials. At Children's, we prefer the autologous procedure. However, plans of care are developed on a case-by-case basis and after speaking with our specialists, alloplastic reconstruction may also be considered.

    What if my child has bilateral microtia?
    Treatment for the much less common bilateral microtia (microtia of both ears) is a more complicated for various reasons—but the general idea is the same.

    Plastic surgeons will reconstruct your child's ears so that they look relatively normal.

    Are there alternatives to surgery?
    While we believe that the most effective way to correct microtia is with reconstructive surgery, there are a few other options:

    Prosthetic ear—A realistic ear can be attached to your child's skull.
    The prosthetic ear will never feel like a part of your child's body (and will need to be removed at night), which may make this a less-than-ideal solution for some children.

    Ear implant—This procedure involves minor surgery and can achieve realistic-looking results.
    This surgery may be a good option if too much scarring prevents an autologous ear construction.

    No treatment—If your child has a relatively mild form of microtia, you may opt to not have any reconstructive surgery done.

    Your doctor can best advise you which is the best option for your child.

    What happens if my child has a hearing loss?
    If your child has a hearing problem related to his microtia, your audiologist and pediatrician will arrange for your child to be seen by an otolaryngologist (ear, nose and throat specialist) and will guide you to other services to help your child learn to communicate.
    Our Diagnostic Audiology team will:

    • monitor the hearing loss
    • provide counseling to you and your family regarding the hearing loss
    • recommend hearing aids and ways to maximize the use of what hearing your child does have
    • refer your child for additional language or developmental assessments
    • direct you to community resources for educational or financial assistance
    • refer your child to Habilitative Audiology to learn about the option of a cochlear implant if your child has a severe to profound hearing loss in both ears

    Supportive care
    We want to maximize the safety and effectiveness of whatever therapy you and your child's doctor decide upon.

    Supportive care involves preventing and treating infections and side effects of treatment to keep your child as comfortable as possible while we're working to reconstruct her ear.

    Follow-up care
    A schedule of follow-up care will be determined by your child's physician and other members of your care team to monitor your child.

    Breaking the silence
    In very severe cases of microtia where the inner ear is also affected, a child may not be able to hear at all. A cochlear implant is an electronic device designed to provide the sensation of hearing to people who are deaf. The Cochlear Implant Team at Children's has placed nearly 500 cochlear implants in kids, giving them the tools they need to understand language, so they can learn to communicate effectively and confidently. Read a story about a 6-year-old girl's introduction to a world of sound.
  • Research & Innovation

    The mission of Children’s Hospital Boston’s Plastic Surgery laboratory is to study pediatric plastic surgical problems and to develop improved treatments for those conditions. We are interested in translational basic research that has direct clinical applicability. Our basic science questions are derived from clinical experience, and our aim is to translate the knowledge gained in the laboratory back to the clinic to improve patient care.

    Read about our research laboratory.

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