Microtia

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.

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.