Anotia/Microtia

Anotia and microtia are birth defects of a baby’s ear.

Anotia

microtia

What are anotia and microtia?

Anotia and microtia are birth defects of a baby’s ear. Anotia
happens when the external ear (the part of the ear that can be
seen) is missing completely. Microtia happens when the external
ear is small and not formed properly.
Anotia/microtia usually happens during the first few weeks of
pregnancy. These defects can vary from being barely noticeable
to being a major problem with how the ear formed. Most of the
time, anotia/microtia affects how the baby’s ear looks, but
usually the parts of the ear inside the head (the inner ear) are not
affected. However, some babies with this defect also will have a
narrow or missing ear canal.

Types of Microtia
There are four types of microtia, ranging from Type 1 to Type 4.
Type 1 is the mildest form, where the ear retains its normal
shape, but is smaller than usual. Type 4 is the most severe type
where all external ear structures are missing —anotia. This
condition can affect one or both ears. However, it is more
common for babies to have only one affected ear.1-2

Occurrence
Because the severity of microtia ranges from mild to severe,
researchers have a hard time estimating how many babies in the
United States are affected. Recently, state birth defects tracking
systems have estimated that anotia/microtia range from less
than 1 in 10,000 live births to about 5 in 10,000 live births.3

Causes and Risk Factors

The causes of anotia/microtia among most infants are unknown.
Some babies have anotia/microtia because of a change in their
genes. In some cases, anotia/microtia occurs because of an
abnormality in a single gene, which can cause a genetic
syndrome. Another known cause for anotia/microtia is taking a
medicine called isotretinoin (Accutane®) during pregnancy. This
medicine can lead to a pattern of birth defects, which often
includes anotia/microtia. These defects also are thought to be
caused by a combination of genes and other factors, such as the
things the mother comes in contact with in the environment or
what the mother eats or drinks or certain medicines she uses
during pregnancy.

Like many families of children with a birth defect, CDC wants to

find out what causes them. Understanding the factors that are
more common among babies with a birth defect will help us learn
more about the causes. CDC funds the Centers for Birth Defects
Research and Prevention, which collaborate on large studies
such as the National Birth Defects Prevention Study (NBDPS;
births 1997-2011) and the Birth Defects Study To Evaluate
Pregnancy exposureS (BD-STEPS; began with births in 2014), to
understand the causes of and risks for birth defects, including
anotia/microtia.

Recently, CDC reported on important findings about some factors

that increase the risk of having a baby with anotia or microtia:


Diabetes ― Women who have diabetes before they get
pregnant have been shown to be more at risk for having a
baby with anotia/microtia, compared to women who did not
have diabetes.4
 
Maternal diet—Pregnant women who eat a diet lower in
carbohydrates and folic acid might have an increased risk
for having a baby with microtia, compared to all other
pregnant women.5

CDC continues to study birth defects, such as anotia/microtia,

and how to prevent them. If you are pregnant or thinking about
becoming pregnant, talk with your doctor about ways to increase
your chances of having a healthy baby.

Diagnosis
Anotia/microtia are visible at birth. A doctor will notice the
problem by just examining the baby. A CT or CAT scan (special x-
ray test) of the baby’s ear can provide a detailed picture of the
ear. This will help the doctor see if the bones or other structures
in the ear are affected. A doctor will also perform a thorough
physical exam to look for any other birth defects that may be
present.

Treatment
Treatment for babies with anotia/microtia depends on the type or
severity of the condition. A healthcare provider or hearing
specialist called an audiologist will test the baby’s hearing to
determine any hearing loss in the ear(s) with the defect. Even a
hearing loss in one ear can hurt school performance. 6 All
treatment options should be discussed and early action may
provide better results. Hearing aids may be used to improve a
child’s hearing ability and to help with speech development.

Surgery is used to reconstruct the external ear. The timing of

surgery depends on the severity of the defect and the child’s age.
Surgery is usually performed between 4 and 10 years of age.
Further treatment may be necessary if the child has other birth
defects present.

In the absence of other conditions, children with anotia/microtia

can develop normally and lead healthy lives. Some children with
anotia/microtia may have issues with self-esteem if they are
concerned with visible differences between themselves and
other children. Parent-to-parent support groups can prove to be
useful for new families of babies with birth defects of the head
and face, including anotia/microtia.

Other Resources
The views of these organizations are their own and do not reflect
the official position of CDC.

 Children’s Craniofacial Association (CCA)

CCA addresses the medical, financial, psychosocial,
emotional, and educational concerns relating to craniofacial
conditions.
 The National Craniofacial Association (FACES)
FACES is dedicated to assisting children and adults who
have craniofacial disorders resulting from disease,
accident, or birth.
References
1. Canfield MA, Langlois PH, Nguyen LM, Scheuerle AE.
Epidemiologic features and clinical subgroups of
anotia/microtia in Texas. Birth Defects Research (Part A):
Clinical and Molecular Teratology. 2009;85:905-913.
2. Shaw GM, Carmichael SL, Kaidarova Z, Harris JA.
Epidemiologic characteristics of anotia and microtia in
California, 1989-1997. Birth Defects Research (Part A):
Clinical and Molecular Teratology. 2004;70:472-475.
3. National Birth Defects Prevention Network. Selected birth
defects data from population-based birth defects
surveillance programs in the United States, 2004-2008.
Birth Defects Research (Part A): Clinical and Molecular
Teratology. 2011;91:1076-1149.
4. Correa A, Gilboa SM, Besser LM, Botto LD, Moore CA, Hobbs
CA. Diabetes mellitus and birth defects. American Journal of
Obstetrics and Gynecology. 2008;199:237.e1–9.
5. Ma C, Shaw GM, Scheuerle AE, Canfield MA, Carmichael SL,
and National Birth Defects Prevention Study. Association of
microtia with maternal nutrition. Birth Defects Research
(Part A): Clinical and Molecular Teratology. 2012;(94):1026-
1032.
6. Kuppler K, Lewis M, Evans AK. A review of unilateral hearing
loss and academic performance: Is it time to reassess
traditional dogmata? International Journal of Pediatrics and
Otorhinolaryngology. 2013;77: 617-622.