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Birth defects

  • Birth defects are health conditions present at birth.
  • They can cause problems in how the body works.
  • About 120,000 babies born each year have a birth defect.
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Hearing loss

Hearing loss is when you can’t hear sound in one or both ears. It can range from mild to complete:

  • Mild, you can’t hear soft speech
  • Severe, you can’t hear very loud sounds
  • Complete, you can’t hear anything at all. Complete hearing loss is also called deafness.

Hearing loss is a common birth defect. A birth defect is a health condition that is present at birth. Birth defects change the shape or function of one or more parts of the body. They can cause problems in overall health, how the body develops, or in how the body works. Nearly 3 in 1,000 babies (about 12,000) are born with some kind of hearing loss in this country each year. 

Hearing loss that is present at birth is called congenital hearing loss. Hearing loss also can develop later in babies or during childhood or adulthood. 

What are signs of hearing loss?

Signs of hearing loss in your baby can include:

  • Not being startled by loud sounds
  • Not turning toward the sound of a voice 
  • Not imitating sounds after he’s 6 months old 
  • Not babbling by the time he’s 1 year old
  • Not using single words or following simple directions by the time he’s 18 months old
  • Not using simple, two-word sentences by age 2 

If your baby shows signs of hearing loss at any time, call her provider to get your baby’s hearing checked. 

What are common types of hearing loss?

Your baby’s auditory system is the system in the body that helps her hear. It includes the ears and the parts of the brain that make sense of sounds. Problems in these parts of the auditory system may cause hearing loss:

  • Outer ear. This includes the part of the ear on the outside of the head, the ear canal and the outside of the eardrum. The eardrum separates the outer and middle ear. 
  • Middle ear. This is made up of the inside of the eardrum and three small bones called ossicles. Sound coming into the ear moves through the ear canal to the eardrum, causing the eardrum to vibrate (move back and forth quickly). When the eardrum vibrates, it moves the ossicles, which help sound move to the inner ear.
  • Inner ear. This is made up of the cochlea (a curled tube filled with liquid) and nerves that change sound vibrations into signals that travel to the brain through the auditory nerve. The auditory nerve connects the inner ear to the brain.

Common types of hearing loss include: 

  • Conductive hearing loss. This happens when there’s a problem in the outer or middle ear that slows or prevents sound waves from passing through. Problems may include a blockage in the ear canal, damage to the ear drum or ossicles, or fluid in the middle ear. This kind of hearing loss often is temporary and can be treated.
  • Sensorineural hearing loss. This happens when there’s a problem with the inner ear. It may happen when the nerves of the inner ear are damaged. This kind of hearing loss is permanent. \
  • Mixed hearing loss. This is a combination of conductive and sensorineural hearing loss. This kind of hearing loss is permanent. 

How do you know if your baby has hearing loss?

The Centers for Disease Control and Prevention (CDC) recommends that all babies get screened for hearing loss before they’re 1 month old. Most babies get their hearing checked as part of newborn screening before they leave the hospital. Newborn screening checks for serious but rare conditions at birth. It includes blood, hearing and heart screening. 

If your baby doesn’t pass his newborn hearing screening, it doesn’t always mean he has hearing loss. He may just need to be screened again. If your baby doesn’t pass a second time, it’s very important that he gets a full hearing test as soon as possible and before he’s 3 months old. 

A full hearing test can help your baby’s health care providers diagnose hearing loss. If your baby has hearing loss, getting treatment right away is important. Every state has an Early Hearing Detection and Intervention (EDHI) program that works to help children with hearing loss and their families. It can help with full hearing tests and other services for your baby. Find your local EDHI program through the National Center for Hearing Assessment and Management website. 

What hearing tests are part of newborn screening?

Your baby has one of these two hearing screening tests as part of newborn screening: 

  • Automated auditory brain stem response (also called AABR). This test uses patches (also called electrodes) and a computer to check how the auditory nerve reacts to sound. Your baby wears the patches on his head and tiny earphones in his ears. Your baby’s provider sends clicking sounds through the earphones and the patches measure how your baby’s auditory nerve reacts. The provider can’t change the test settings. and the test only gives a “pass” or “fail” report. Your baby can be asleep for this test.
  • Otoacoustic emissions (also called OAE). This test checks how the inner ear responds to sound. Your baby’s provider places a small earphone in your baby’s ear. The earphone is connected to a computer. Your baby’s provider sends a soft clicking sound into the ear through the earphone. The soft clicking sounds should echo in your baby’s ear canal. If there’s no echo, your baby may have hearing loss. Your baby can be asleep for this test.

What other kinds of hearing tests can babies have? 

If your baby doesn’t pass his ABR or OAE test in newborn screening, her provider refers her to an audiologist. This is someone with special training to diagnose and treat hearing loss in newborns, children and adults. 

An audiologist gives your baby a full hearing test to check for hearing loss. She may use these tests: 

  • Diagnostic auditory brain stem response (also called diagnostic ABR). This test can be used for babies and children. It’s the most common hearing test used to find hearing loss in babies younger than 6 months. Your baby wears patches on his head and the audiologist sends sounds into your baby’s ear through earphones.  It’s similar to the AABR test, but it gives more information and only an audiologist can do the test. The audiologist can change settings, like sound levels, during the test to check for and treat hearing loss. For example, your provider use information from the test to fit your child with hearing aids. A hearing aid is a small electronic device that your baby wears in or behind the ear. It makes some sounds louder and helps your baby hear spoken words. 
  • Visual response audiometry (also called VRA). This test is used with children between 6 months and 2½ years old. It checks to see how well your baby responds (turns towards) sounds. Your baby wears earphones, and the audiologist plays sounds through them into the ear. If your baby turns towards sound, he sees a fun image, like a toy or a flashing light. 

How can hearing loss affect your baby?

Early screening, diagnosis and early treatment can help children with hearing loss develop speech, language and social skills. Without early treatment, hearing loss can lead to:

  • Delayed or limited language and speech development. For example, babies and children with hearing loss may have trouble understanding things that other people say, learning new words and saying words the right way. Children with untreated hearing loss may have poor communication skills.
  • Low self-esteem. Children may have low self-esteem (feel badly about themselves) if their hearing loss causes learning problems or makes it hard for them to be social with other children. 

What causes hearing loss?

We’re not sure what causes all forms of hearing loss, but these possible causes include:

Genes. Genes are parts of your body’s cells that store instructions for the way your body grows and works. Genes are passed from parents to children. Genes may play a role in about half of the cases of hearing loss in babies and children. If you or your partner has a family history of hearing loss, you may want to speak with a genetic counselor before getting pregnant. This is a person who is trained to help you understand about how genes, birth defects and other medical conditions run in families, and how they can affect your health and your baby's health.

There are two kinds of genetic hearing loss:

  1. Syndromic. The hearing loss happens with other birth defects, such as Pendred syndrome. Pendred syndrome affects hearing, the sense of balance and the thyroid gland (a gland in your body that makes hormones).
  2. Non-syndromic (also called isolated or undifferentiated). This is when hearing loss is the only birth defect a baby has. 

Viruses and infections during pregnancy. Having these conditions during pregnancy may cause hearing loss in your baby:

  • Cytomegalovirus (also called CMV). This is a kind of herpes virus that can cause a sore throat, fever, swollen glands and fatigue (feeling tired all the time).
  • Herpes. This is a large group of viruses that can cause different kinds of diseases and health problems. 
  • Rubella (also called German measles). This is an infection that causes mild flu-like symptoms and a rash on the skin. 
  • Syphilis. This is a sexually transmitted disease (also called STD) that begins with a sore in the genital area.
  • Toxoplasmosis. This is an infection caused by a parasite that can cause problems like headache, fatigue or fever. 

Premature birth or low birthweight. Premature birth is birth that happens too early, before 37 weeks of pregnancy. Low birthweight is when a baby is born weighing less than 5 pounds, 8 ounces. 

Infections after birth. Ear infections can cause temporary hearing loss. But if your baby gets ear infections often that don’t respond to treatment, the infections can cause permanent hearing loss. Other childhood infections, such as meningitis, measles or chickenpox, also can cause hearing loss.

Other conditions after birth. These conditions also can cause hearing loss:

  • Head injuries
  • Taking some medicines, like an antibiotic called streptomycin 
  • Buildup of ear wax
  • Buildup of fluid behind the eardrum
  • Ear infections that scar the ear drum
  • Objects stuck in the ear canal, like food, toys or pieces of crayon
  • Being around loud sounds, like the sounds of machines

How is hearing loss treated? 

Treatment depends on your baby’s overall health and the cause of the hearing loss. Early treatment starting when your baby’s as young as 6 months old can help her develop language skills without delay. Treatment may include:

  • Medicines. Medicines to treat ear infections may include antibiotics, which kill infections caused by bacteria.
  • Surgery. Surgery can sometimes correct problems with the structure of the outer and middle ears.
  • Ear tubes. These are tiny tubes placed through the ear drum to allow air into the middle ear and prevent fluids from building up behind the ear drum. If your child has hearing loss from fluid buildup and ear infections, or she gets a lot of ear infections (about four or more in 1 year), she may need ear tubes.
  • Learning American Sign Language (also called ASL). People who use sign language communicate using hand shapes, direction and motion of the hands, along with facial expressions. ASL is the first language for many Americans who are deaf. 
  • Speech therapy. This is therapy to teach your child how to speak more clearly or communicate in other ways. Speech-language pathologists (also called speech therapists) are professionals that can help children learn how to make sounds and improve their voices. They also can teach sign language and help children learn how to be social and interact with others. 
  • Hearing aid. Babies as young as 4 weeks old can benefit from a hearing aid. But even the best hearing aids may not help a baby if she has severe or complete hearing loss.
  • Cochlear implant. This is a small electronic device that can help some babies with severe or complete hearing loss. One part of the implant sits on the outside of the head, behind the ear. During surgery, your baby’s provider places the second part beneath her skin behind the ear, with wires threaded into the inner ear. Most children who get cochlear implants are between 2 and 6 years old, but babies as young as 1 year may get one. A cochlear implant doesn't give a baby complete hearing, but it can give a baby a sense of sound. Hearing through a cochlear implant is different from normal hearing. Specialists, like audiologists and speech therapists, can help your baby learn to hear through the implant and develop her speech, language and social skills. 

For more information

Early Hearing Detection and Intervention (EHDI) Program
Individuals with Disabilities Education Improvement Act 2004 (IDEA 2004)
Hearing loss treatment and intervention services

Last reviewed June 2014

Call your provider now if your baby

  • Has a temperature above 100.4º F
  • Has trouble breathing or is hard to waken
  • Has blood in her vomit or stool
  • Has yellowish skin or eyes
  • Is having a seizure

Frequently Asked Questions

Can dad's exposure to chemicals harm his future kids?

Dad's exposure to harmful chemicals and substances before conception or during his partner's pregnancy can affect his children. Harmful exposures can include drugs (prescription, over-the-counter and illegal drugs), alcohol, cigarettes, cigarette smoke, chemotherapy and radiation. They also include exposure to lead, mercury and pesticides.

Unlike mom's exposures, dad's exposures do not appear to cause birth defects. They can, however, damage a man's sperm quality, causing fertility problems and miscarriage. Some exposures may cause genetic changes in sperm that may increase the risk of childhood cancer. Cancer treatments, like chemotherapy and radiation, can seriously alter sperm, at least for a few months post treatment. Some men choose to bank their sperm to preserve its integrity before they receive treatment. If you have a question about a specific exposure, contact the Organization of Teratology Information Specialists at www.otispregnancy.org.

Can Rh factor affect my baby?

The Rh factor may be a problem if mom is Rh-negative but dad is Rh-positive. If dad is Rh-negative, there is no risk.

If your baby gets her Rh-positive factor from dad, your body may believe that your baby's red blood cells are foreign elements attacking you. Your body may make antibodies to fight them. This is called sensitization.

If you're Rh-negative, you can get shots of Rh immune globulin (RhIg) to stop your body from attacking your baby. It's best to get these shots at 28 weeks of pregnancy and again within 72 hours of giving birth if a blood test shows that your baby is Rh-positive. You won't need anymore shots after giving birth if your baby is Rh-negative. You should also get a shot after certain pregnancy exams like an amniocentesis, a chorionic villus sampling or an external cephalic version (when your provider tries to turn a breech-position baby head down before labor). You'll also want to get the shot if you have a miscarriage, an ectopic pregnancy or suffer abdominal trauma.

Does cleft lip or cleft palate cause dental problems?

A cleft lip or cleft palate that extends into the upper gums (where top teeth develop) can cause your baby to have certain dental problems, including:

  • Missing teeth
  • Too many teeth
  • Oddly shaped teeth
  • Teeth that are out of position around the cleft

Every baby with a cleft lip or palate should get regular dental checkups by a dentist with experience taking care of children with oral clefts. Dental problems caused by cleft lip or palate usually can be fixed. If needed, your baby can get ongoing care by a team of experts, including:

  • A dentist
  • An orthodontist to move teeth using braces
  • An oral surgeon to reposition parts of the upper jaw, if needed, and to fix the cleft

See also: Cleft lip and cleft palate

 

Does cleft lip or cleft palate cause ear problems?

Cleft lip does not cause ear problems.

Babies with cleft palate, however, are more likely than other babies to have ear infections and, in some cases, hearing loss. This is because cleft palate can cause fluid to build up in your baby’s middle ear. The fluid can become infected and cause fever and earache. If fluid keeps building up with or without infection, it can cause mild to moderate hearing loss.

Without treatment , hearing loss can affect your baby’s language development and may become permanent.

With the right care, this kind of hearing loss is usually temporary. Your baby’s provider may recommend:

  • Having your baby’s ears checked regularly for fluid buildup
  • Medicines for treating fluid buildup and ear infections
  • Ear tubes if your baby has fluid in his ears over and over again. Ear tubes are tiny tubes that are inserted into the eardrum to drain the fluid and help prevent infections.

See also: Cleft lip and cleft palate

Does cleft lip or cleft palate cause problems with breastfeeding?

Babies with only a cleft lip usually don’t have trouble breastfeeding. Most of the time, they can breastfeed just fine. But they may need some extra time to get started.

Babies with cleft lip and palate or with isolated cleft palate can have:

  • Trouble sucking strong enough to draw milk through a nipple
  • Problems with gagging or choking
  • Problems with milk coming through the nose while feeding

Most babies with cleft palate can’t feed from the breast. If your baby has cleft palate, he can still get the health benefits of breastfeeding if you feed him breast milk from a bottle. Your provider can show you how to express (pump) milk from your breasts and store breast milk.

Your baby’s provider can help you start good breastfeeding habits right after your baby is born. She may recommend:

  • Special nipples and bottles that can make feeding breast milk from a bottle easier
  • An obturator. This is a small plastic plate that fits into the roof of your baby’s mouth and covers the cleft opening during feeding.

See also: Cleft lip and cleft palate, Breastfeeding

Does cleft lip or cleft palate cause speech problems?

Children with cleft lip generally have normal speech. Children with cleft lip and palate or isolated cleft palate may:

  • Develop speech more slowly
  • Have a nasal sound when speaking
  • Have trouble making certain sounds

Most children can develop normal speech after having cleft palate repair. However, some children may need speech therapy to help develop normal speech.

See also: Cleft lip and cleft palate

What are choroid plexus cysts?

The choroid plexus is the area of the brain that produces the fluid that surrounds the brain and spinal cord. This is not an area of the brain that involves learning or thinking. Occasionally, one or more cysts can form in the choroid plexus. These cysts are made of blood vessels and tissue. They do not cause intellectual disabilities or learning problems. Using ultrasound, a health care provider can see these cysts in about 1 in 120 pregnancies at 15 to 20 weeks gestation. Most disappear during pregnancy or within several months after birth and are no risk to the baby. They aren't a problem by themselves. But if screening tests show other signs of risk, they may indicate a possible genetic defect. In this case, testing with higher-level ultrasound and/or amniocentesis may be recommended to confirm or rule out serious problems.

What if I didn't take folic acid before pregnancy?

If you didn’t take folic acid before getting pregnant, it doesn't necessarily mean that your baby will be born with birth defects. If women of childbearing age take 400 micrograms of folic acid every day before and during early pregnancy, it may help reduce their baby’s risk for birth defects of the brain and spin called neural tube defects (NTDs). But it only works if you take it before getting pregnant and during the first few weeks of pregnancy, often before you may even know you’re pregnant.

Because nearly half of all pregnancies in the United States are unplanned, it's important that all women of childbearing age (even if they're not trying to get pregnant) get at least 400 micrograms of folic acid every day. Take a multivitamin with folic acid before pregnancy. During pregnancy, switch to a prenatal vitamin, which should have 600 micrograms of folic acid.

Last reviewed November 2012

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