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Hearing loss

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Also known as:
  • HEAR
  • congenital hearing loss
  • deafness

The ear is very complex and made up of multiple parts, including the outer ear, the middle ear, the inner ear, the hearing (acoustic) nerve and the auditory system (which processes sound as it travels from the ear to the brain). When these individual components work together properly, a child is able to hear and process the sounds of their environment. Hearing loss can occur when any of these parts are not working. Hearing loss can affect a child’s ability to develop speech, language and social skills.  However, with early identification and intervention, children with hearing loss are less likely to experience these challenges.

Hearing is important to children because this is how they learn to communicate. Babies start hearing sounds before they are born. After birth, babies watch their parent's faces and hear them speak. The baby's hearing system continues to develop everyday. At three months old, babies will smile when spoken to, and at six months old they will begin to babble and imitate certain sounds. If your baby can not hear, this process of development will be slowed down and can be detrimental to the cognitive development of your baby.

Most hospitals perform the newborn hearing screen before the baby is discharged from the hospital. To find out if your baby was screened contact your baby's doctor - the results of the screen will be in your baby's medical records. You can also contact the hospital your baby was born at and ask if a newborn hearing screen is regularly performed. You can also call 866-997-4327 for more information or check out your state's page to find your local Early Hearing Detection and Intervention Coordinator. 

 

Follow-Up Testing

If your baby’s newborn hearing screen was out of the normal range, your baby’s doctor or the state screening program will contact you to arrange for your baby to have additional testing. It is important to remember that an out-of-range screening result does not necessarily mean that your child has hearing loss. Between 2 and 10 percent of all babies across the United States do not pass their first hearing screen, but very few of these babies have permanent hearing loss. Babies can fail the newborn hearing screening due to vernix in the ear canal, fluid in the middle ear, or because of movement or crying during the test. However, since a few babies actually do have hearing loss, it is very important that you go to your follow-up appointment for a confirmatory test.

All babies who do not pass their newborn hearing screen are referred, or directed for treatment, to an audiologist for a complete audiology (hearing) evaluation. A baby who does not pass the newborn hearing screen is one whose hearing does not respond to the newborn hearing screen. The audiologist will perform a few tests to determine if hearing loss is present and if so, the degree and type of hearing loss. An audiologist is someone who has a masters or doctorate degree that specializes in hearing loss. An audiologist can be your greatest asset in determining proper treatment for your baby's hearing loss. The audiologist will work closely with your pediatrician and early intervention specialists to ensure that your baby receives proper treatment.

Some of the tests that an audiologist may use include:

  • Auditory Brainstem Response (ABR) Test - This test evaluates the auditory brain stem (the part of the nerve that carries sound from the ear to the brain) and the brain’s response to sound.  During this test, miniature earphones are placed in the ear and sounds are played. Band-Aid-like electrodes are placed along your baby’s head to detect the brain’s response to the sounds.  If your baby’s brain does not respond to all the sounds, your baby could have hearing trouble.
  • Otoacoustic Emissions (OAE) Test - This test determines if certain parts of your baby’s ear respond appropriately to sound. During the test, a miniature earphone and microphone are placed in the ear and sounds are played.  When a baby has normal hearing, an echo will reflect back into the ear canal. This echo is measured by the microphone.  If the microphone doesn’t receive the echo, your child may have hearing loss.
  • Brain Audiometry Evaluation – This test evaluates if all parts of the ear are working correctly.  The audiologist will play a sound in your baby’s ear and then watch for changes in your baby’s behavior. A baby with full hearing will generally respond by sucking a pacifier, quieting, or searching for the sound.

The audiologist may recommend your baby for a genetics evaluation.  This evaluation will help your baby’s doctor to determine the cause of hearing loss.  The doctor will ask you questions about your family health history and pregnancy history and examine your baby for symptoms that are commonly associated with syndromic hearing loss. The doctor may then take a small sample of your baby’s blood for genetic testing. Genetic testing can help confirm if there is a genetic explanation for your baby’s hearing loss and determine the chances of having another baby with genetic hearing loss.

The results of each baby's newborn hearing screening is reported to his or her respsective state's EHDI programs. The data from the screening, as well as any follow up testing, can be used for research purposes. The Centers for Disease Control and Prevention (CDC) works with each state's EHDI program to conduct the Hearing Screening, as well as a Follow-Up Survey. The CDC uses this information to assess the impact of the promotion of infant hearing screening, if timely follow-up evaluations are occuring, and how successful early intervention services are. The CDC also funds and regulates hearing loss surveillance and research.


About Hearing loss

Early Signs

Newborn hearing screening is designed to detect hearing loss as early as possible. However, a few babies can pass a hearing test and still have hearing loss. Some develop hearing loss later in childhood due to illness or certain genetic conditions. Even if your child has passed a hearing screen before, it is important to look out for the following signs:

  • Does not startle at loud noises
  • Does not turn to the source of a sound
  • Does not say single words, such as “dada” or “mama” by 1 year of age
  • Turns head when he or she sees you but not if you only call out his or her name
  • Seems to hear some sounds but not others

If your baby has any of these signs, be sure to contact your baby’s doctor immediately.

Treatment

Each baby’s diagnosis is different. These differences can be related to the degree of the hearing loss, the type of the hearing loss and the cause of the hearing loss. Your child’s treatment and intervention plan will vary according to his or her individual needs. Basic information on hearing loss including how the ear works, types of hearing loss, audiograms, and simulations of varying levels of hearing loss is available through Boys Town National Research Hospital website, My Baby’s Hearing: All About Hearing Loss (*Español: Todo Sobre La Perdida Auditiva)

In general, a treatment plan for hearing loss includes close monitoring and regular follow-up. It can change as your child grows older and begins school. Some treatment and intervention options include the following:

Early Intervention

Early intervention services are available to help children with hearing loss gain important speech, language, and social skills. Babies who are diagnosed with hearing loss through newborn screening should be referred to early intervention services as soon as possible. Research shows that children identified with hearing loss who begin services before 6 months old develop language (spoken or signed) on a par with their hearing peers. Click here to contact your state Early Intervention coordinator.

Technology

There are many technologies available that don’t cure hearing loss, but help your child use any hearing they may have. Your baby’s doctor and audiologist can work together to create the best plan for your child.

Learn more about treatment and intervention services for hearing loss from the CDC Center on Birth Defects and Development Disabilities and My Baby's Hearing.

Communication Options

Professionals in education, other parents and adults who are deaf or hard of hearing all have strong feelings about the best way for your baby to learn and communicate. As parents, however, you have the right and responsibility to decide what's best to help your child communicate with others and succeed in school. It's important to gather information on all communication approaches when making this important decision, including sign language, listening and spoken language, or a combination of visual and auditory methods. Click here to learn more about all of these options.

Information about specific communication options are provided below:

American Sign Language (ASL)

National Institute on Deafness and Other Communication Disorders

Handspeak

Laurent Clerc National Deaf Education Center Language and Literacy

Auditory Oral

Oral Deaf Education

Auditory Verbal

Alexander Graham Bell Association for the Deaf and Hard of Hearing Auditory Verbal Method

What is Auditory-Verbal Therapy?

Cued Speech

National Cued Speech Association

CuedSpeech.com

Expected Outcomes

Children with hearing loss whose hearing loss is identified early and receive immediate treatment develop better speech and language skills and have fewer developmental delays.  Early intervention can also lead to better performance in school.

It is important to identify babies with hearing loss because children begin learning as soon as they are born. One way they learn is by listening to the sounds and language in their environment. Studies show that children with hearing loss who get help early develop better language skills than those who do not.

Causes

More than 50% of hearing loss is believed to have a genetic cause. Genetic hearing loss may be inherited from one or both parents, or it could be the result of a new genetic change in the child. Hearing loss can be syndromic or nonsyndromic.  Genetic hearing loss also follows an autosomal recessive (77%), autosomal dominant (22%), or X-linked recessive (1%) pattern of inheritance.

In most cases, genetic hearing loss is nonsyndromic. This means that the only symptom a baby has is hearing loss. However, a small portion of babies with genetic hearing loss has other features that suggest their hearing loss is just one part of a bigger condition (syndromic hearing loss).  For example, babies with hearing loss and a white patch of hair may have a genetic condition called Waardenburg syndrome.

When hearing loss is an autosomal recessive genetic condition, a child must inherit two copies of the non-working gene, one from each parent, in order to have the condition. The parents of a child with an autosomal recessive condition each carry one copy of the non-working gene, but typically do not show signs and symptoms of the condition. While having a child with hearing loss is rare, when both parents are carriers, they can have more than one child with the condition. Learn more about autosomal recessive inheritance.

When hearing loss is an autosomal dominant genetic condition, a child only needs one copy of the non-working gene for hearing loss, from either parent, in order to have the condition. While having a child with hearing loss is rare, when one or both parents has hearing loss, they can have more than one child with the condition.  Learn more about autosomal dominant inheritance.

When hearing loss is an X-linked recessive genetic condition, a male must inherit one copy of the non-working gene from his mother to have the condition. A female must inherit two copies of the non-working gene, one from each parent, in order to have the condition. In X-linked conditions, the gene is carried on the X sex chromosome, and males are affected more than females. While having a child with hearing loss is rare, when one or both parents carry the non-working gene for hearing loss, they can have more than one child with the condition. Learn more about X-linked recessive inheritance.

25% of hearing loss is caused by environmental factors.  Some infections a mother could have while pregnant can cause her baby to have hearing loss. Hearing loss can also develop when there are complications during the birth of a baby or if a child develops a certain illness.

For some children, the cause of their hearing loss is unknown. 


Support for Hearing loss

Support Services

Support groups can help connect families who have a child or other family member with hearing loss with a supportive community of people who have experience and expertise in living with the condition. These organizations offer resources for families, affected individuals, health care providers, and advocates.

For a list of additional resources please visit the NCHAM links to family support resources.

Accessing Care

Work with your baby’s doctor to determine the next steps for your baby’s care.  Your baby’s doctor may help you coordinate care with early intervention services, an audiologist, or other medical resources in your community.

There are many great resources that can provide you and your baby’s doctor with information and resources. The CDC has fact sheets, brochures, and parent guides about the importance of early screening and the treatment of newborn hearing loss.

The Maternal Child Health Bureau (MCHB), the Health Resources and Services Administratoin (HRSA), and health literacy researchers at Lousiana State University developed fact sheets about your baby's first hearing test and how finding hearing loss early can make a big difference in your baby's life.

The National Center for Hearing Assessment and Management seeks to ensure that all babies and toddlers with hearing loss are identified as early as possible and provided with timely and appropriate audiological, educational, and medical care. To get more information about this organization’s resources for parents visit NCHAM.

Because some forms of hearing loss are genetic, you may want to talk with a genetics specialist. A genetic counselor or geneticist can help you understand the causes of the condition, discuss genetic testing for hearing loss, and understand what this diagnosis means for other family members and future pregnancies. Speak with your baby’s doctor about getting a referral. The ‘Clinic Services Search Engine’ offered by the National Coordinating Center for the Regional Genetic and Newborn Screening Service Collaboratives and the ‘Find a Genetic Counselor’ tool on the National Society of Genetic Counselors (NSGC) website are two good places for you or your pediatrician to use in identifying local specialists.

Families' Experiences

Through newborn screening, Sam was diagnosed with hearing loss before he was a week old. With a hearing aid and treatment from a speech therapist, Sam is doing well. This video features Sam’s story in describing the importance of newborn screening for hearing loss.

Amanda and Ashley were diagnosed with hearing loss through newborn screening when they were two days old. With the benefit of early treatment, these twin sisters are now succeeding in school. Read their story.

When parents learn that their child has hearing loss, they may feel confused and worried. The mother in this story writes about her hopes and expectations for her daughter’s future.

Read what other parents have to say about raising a child with hearing loss in a section entitled “Parent Talk” on the My Baby’s Hearing Loss website. Parents of children with hearing loss discuss expectations for their children as well as family discipline and involvement.


States Screening for Hearing loss

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