Deafness or hearing loss can be present at birth or develop as your baby grows. Hearing loss is a group of conditions that make it more difficult for your baby to hear and understand sounds.

Hearing loss can be caused by a change in the ear or a change in the part of the brain that processes sounds. Your baby could have one of these changes or a mix of the two.

Hearing loss can be mild, moderate, severe, or profound. Your baby may be deaf or hard of hearing in one ear (unilateral) or both ears (bilateral). Their hearing may get better or worse over time (fluctuating) or stay the same over time (stable). The changes to their ears or brain lead to the signs and symptoms of this condition.

Any hearing loss that makes it difficult for babies to hear speech can affect language development and communication skills. It is important to find out if your baby is deaf or hard of hearing as early as possible.

While the term “hearing loss” is most commonly used by the health care system, it is important to understand that using the words “deaf” or “hard of hearing” is socially acceptable and often preferred. Often when children with hearing loss grow older, or as adults, they will identify themselves as “D/deaf” or “hard of hearing.” The Deaf community views the term “Deaf” as a positive word and includes any person who experiences life with a hearing loss.

About half of babies are deaf or hard of hearing because of changes in their genes. These genes give the body instructions on how to form the parts of the ear and brain that allow us to hear. When genetic hearing loss does not have any other associated health issues in the body, it is called nonsyndromic hearing loss. Genetic hearing loss that is part of a syndrome that causes other health issues is called syndromic hearing loss.

Other possible causes of deafness or hearing loss include:

• Infections in the mother during pregnancy such as cytomegalovirus, herpes, toxoplasmosis, or rubella
• Environmental causes such as exposure to too much/too loud noise, infections that occur in the baby after birth, head trauma, or certain medications
• Complications after birth

In addition, babies who are born too early (premature) have a higher chance of becoming deaf or hard of hearing than babies born at full term.

Deafness resulting from changed genes means a baby likely inherited the condition from their biological (birth) parents.

Babies can inherit deafness and hearing loss in different ways, depending on which genes are responsible.

• Hearing loss inherited in an autosomal recessive pattern means that babies are deaf or hard of hearing when each parent passes down a nonworking gene to their baby. Only babies with two nonworking genes—one from the mom and one from the dad—are deaf or hard of hearing.
  • People with one working copy and one nonworking copy of the gene are called carriers.
  • Carriers do not become deaf or hard of hearing from their one nonworking gene. However, they may pass down a nonworking copy of the gene to their children. Carriers may become deaf or hard of hearing from environmental factors (aging, exposure to noise, infections, etc.) just like individuals who do not carry a nonworking gene.
  • If two parents are carriers, they have a 1 in 4 chance of having a child who is deaf or hard of hearing.
  • Carriers often do not know they are carriers before having a child who is deaf or hard of hearing. In most cases, families have no history of hearing loss until the birth of a child who is deaf or hard of hearing.
  • Parents who already have a child who is deaf or hard of hearing still have a 1 in 4 chance of having another child who is deaf or hard of hearing. This 1 in 4 chance stays the same for all future children.
• When hearing loss is caused by genes that are inherited in an autosomal dominant pattern, babies are deaf or hard of hearing when only one parent passes down a nonworking gene to their baby. Babies with one nonworking gene—one from the mom or one from the dad—are deaf or hard of hearing. In these cases, one of the parents may also be deaf or hard of hearing.
• When hearing loss is caused by genes that are inherited in an X-linked recessive pattern, babies inherit hearing loss on their X chromosome.
  • Girls have two X chromosomes, so only girls with two nonworking genes—one from the mom and one from the dad— are deaf or hard of hearing.
  • Boys only have one X chromosome, so boys with only one nonworking gene—one from mom— are deaf or hard of hearing. X-linked recessive hearing loss is more common in boys.
• Genetic counselors and medical geneticists can help families learn about genetic hearing loss.

Even if your baby had a “Pass” result at birth, at a later time they could start to show signs or symptoms of hearing loss. It is important to take your baby to their health care provider to see if their hearing is changing over time.

The following signs of hearing loss often appear shortly after birth but can also appear later in childhood.

• Your baby seems to hear some sounds but not others
• Your baby does not react to sound
  • They do not turn to see what is making a sound
  • They turn to look at you if they see you, but not if you call to them
• Your baby startles frequently or easily when approached from behind
• Your baby does not say single words like “dada” or “mama” by the time they are 1 year old
• Your baby uses his or her eyes intensely to ‘look’ for information, or appears to always be reading your lips.

Newborn hearing screening uses earbuds that are connected to a special device to check your baby’s hearing while your baby is calm and in a quiet room. The hearing screening is not painful, and many babies sleep through the few minutes needed to do the screening.

There are two ways to screen a newborn for hearing status. Your baby may have one or both screens. One screen uses a small earbud and microphone to check for echoes in response to sound made in your baby’s ear (otoacoustic emissions, OAE). Another screen uses earphones and stickers with sensors to see how your baby’s brain responds to sound (auditory brainstem response, ABR). Your baby will get either a “Pass” result or a “Fail” (sometimes called a “Refer”) result.

What Happens After an Out-of-Range Screening Result?

In hearing screening an out-of-range screening result is called a “Fail” or "Refer" result. If your baby’s hearing screening gives a “Refer” result, the person doing the hearing screen will let you know right after the screen. Together, you will discuss next steps and follow-up plans.

Some babies who have typical hearing may have a “Refer” hearing screening result, this is usually because they still have fluid or other buildup in their ears. A “Refer” result means that your baby needs more follow-up screening and possible testing.

Your baby may need the following tests after a “Refer” hearing screening result:

• Another hearing screen (this may be done at the birth facility, by your baby’s primary health care provider, or by an audiologist)
• A more complete, full hearing test (audiology evaluation)

Your baby may also get the following tests after a “Refer” hearing screening result or after being found to be deaf or hard of hearing. These tests can help determine the cause of your baby’s hearing loss.

• Genetic testing using a blood sample to look for genetic causes of hearing loss
• Testing for infections at birth that can cause hearing loss (like cytomegalovirus)

You should complete any recommended follow-up testing as soon as possible, but before your baby is 3 months old. Babies who are deaf or hard of hearing can have trouble with learning speech, language, and social skills if their hearing loss is not found early.

False-positive newborn hearing screening results for hearing loss can and do happen. The screening results can be affected by material or fluid in the ear, or by movement or crying during the screen.

Babies with a “Pass” hearing result on newborn hearing screenings may also still become deaf or hard of hearing later in infancy, childhood, or adulthood. It is important to watch your baby for signs of hearing loss.

Newborn hearing screening helps babies who are deaf or hard of hearing. If your baby has a “Fail” result, follow up with your health care provider quickly. It is important to follow their instructions. Your baby may benefit from intervention right away, even if they are not showing signs or symptoms. In some cases, your baby’s health care provider may decide it is best to watch (monitor) your baby to decide next steps. Careful monitoring and early intervention will give your baby the best opportunities for addressing their hearing status.

It is important to talk to your health care provider about available interventions for your baby. The goal of intervention is to allow babies who are deaf or hard of hearing develop language and learn how to communicate, either by improving their hearing or by providing other language and communication tools.

Opportunities may include one or more of the following:

• American Sign Language
• Cued Speech
• Medical interventions
  • Surgery
• Technology
  • Hearing aids
  • Cochlear implants
  • Other assistive devices

Children who are deaf or hard of hearing and receive early and ongoing educational and audiological services have fewer developmental delays and develop better communication skills. All children can begin learning language at birth, so the sooner hearing loss is identified, the better the outcome for your child.