Children With Special Health Care NeedsPhone:(801)584-8284 TollFree:(800)829-8200

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Early Hearing Detection and Intervention (EHDI)

Hearing loss is one of the most common birth defects, occurring at a rate of 1 out of 300 babies. The first months and years of life are a critical period for brain development, including speech and language skills, social and emotional development, and academic success. Early identification of hearing loss and appropriate intervention ensures a child will have the opportunity to reach their maximum potential.

Utah Early Hearing Detection and Intervention (EHDI) Program Goals:

  • All newborns receive hearing screening before discharge or before 10 days of age if born out of hospital.
  • For infants that do not pass, repeat the hearing screening no later than 14 days of age.
  • If infants do not pass the second hearing screening:
    1. Test for congenital Cytomegalovirus (CMV) infection before 21 days of age.
    2. Complete a diagnostic hearing evaluation by a pediatric audiologist before 3 months of age.
  • If infants are diagnosed with hearing loss:
    1. Enroll infant into Early Intervention services before 6 months of age.

Parents

Many babies with hearing loss will react to loud sounds, cry, and babble. The only way to know if your baby’s hearing is normal is to have the screening done with special equipment.

baby receiving hearing testingInitial Newborn Hearing Screening
All babies born in Utah will have a hearing screening before they leave the hospital. If your baby was born out of hospital, your baby should have their hearing screened before 10 days of age.

Be sure to know the results of your baby’s hearing screening. The results will be a “pass” or a “refer”.

How will my baby be screened?

There are two tests which may be used to screen a baby’s hearing. Both tests are safe and do not hurt.

Otoacoustic Emissions (OAE)
Soft sounds are directed into the ear through a tiny earphone. When a normal inner ear (hearing organ) receives a sound, it sends a "sound" (emission) back out. A special microphone measures these responses. If a baby has hearing loss, little to no "sound" or emission may be measured. This means further testing is necessary.

Automated Auditory Brainstem Response (AABR)
Soft clicking sounds are played through earphones into the baby’s ears. Band-aid like sensors placed on the baby’s head measure the brain’s response to these soft sounds. The machine compares the response from the baby being tested to a “normal” response for babies. If there is a good match, the baby passes the screening. If the match is not close enough, the baby will not pass the screening.

These tests may be used alone or in combination. Both tests are accurate and reliable. Some hospitals use a 2-step process. Babies are first screened with OAE, and those who do not pass the OAE are then given the AABR.

What if my baby does not pass the first screening?

If your baby did not pass their initial hearing screening, it does not mean there is hearing loss, it just means further investigation is needed. It is very important to have your baby rescreened before 14 days of age.

What if my baby does not pass the re-screening?

If your baby does not pass the second hearing screening, be sure to follow-up with your pediatrician or primary care physician right away so that your baby is tested for congenital Cytomegalovirus (CMV) before 21 days of age. CMV testing is time-sensitive. CMV is a common virus that infects people of all ages; however, when it occurs during pregnancy, the baby can become infected, potentially causing damage to the brain, eyes, and/or inner ears. CMV causes 1/3 of all childhood hearing loss.

Congenital CMV testing is simple and painless, and is accomplished using a urine or saliva sample (the inside of your baby’s cheek is swabbed). Your baby will also be referred for a diagnostic hearing evaluation with a pediatric audiologist.

A diagnostic hearing evaluation should be completed before 3 months of age by a pediatric audiologist. A pediatric audiologist has expertise in developmentally appropriate test techniques, general child development, and parent counseling regarding infants and children with hearing loss.

Utah Guide to Pediatric Audiologists (PDF | Interactive Map)

What if my child is diagnosed with hearing loss?

If hearing loss is confirmed, it is recommended that infants and children be fit with appropriate amplification, i.e. hearing aid(s), and enrolled in Early Intervention as soon as possible, ideally prior to 6 months of age.  

After hearing loss has been identified, your child will likely be followed closely the first several years in order to monitor for changes in hearing or progressive/worsening hearing loss. Your audiologist will recommend your child’s hearing evaluation schedule based on their case history, but likely every 3 to 6 months. If you notice any changes in your child’s hearing, an evaluation should be conducted as soon as possible (do not wait until your next regularly scheduled appointment).

An audiologist is the only professional qualified to select and fit all forms of amplification for children (hearing aids, FM systems, cochlear implants, and other assistive listening devices). It is important to select a pediatric audiologist with experience managing infants and children with hearing loss.

 

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Physicians

All newborns should be screened before discharge or if born out of the hospital before 10 days of age. If the baby does not pass, a rescreen should be completed before 14 days of age. If the baby does not pass a second hearing screening, the baby should be referred to their primary care physician for CMV testing and must be referred to a Pediatric Audiologist for diagnostic hearing testing.

Utah is a two-stage hearing screening state. This means that a baby may be tested twice (stage 1= inpatient and stage 2 = outpatient) before further diagnostic hearing testing is required. 90% of infants who fail the inpatient screening will go on to pass the outpatient screening. The other 10% are at a higher risk for permanent hearing loss.

Do not minimize the importance of hearing screening by saying that it is likely just fluid. Any child that does not pass a hearing screening is at higher risk for a hearing loss; there is a possibility of hearing loss even in the presence of fluid and further testing is needed.

What does hearing screening entail?

There are two tests which may be used to screen a baby’s hearing. Both tests are safe and do not hurt and require that the infant is calm and quiet.

Otoacoustic Emissions (OAE)
Soft sounds are directed into the ear through a tiny earphone.  When a normal inner ear (hearing organ) receives a sound, it sends a "sound" (emission) back out.  A special microphone measures these responses.  If a baby has hearing loss, little to no "sound" or emission may be measured. This means further testing is necessary.

Automated Auditory Brainstem Response (AABR)
Soft clicking sounds are played through earphones into the baby’s ears. Band-aid like sensors placed on the baby’s head measure the brain’s response to these soft sounds. The machine compares the response from the baby being tested to a “normal” response for babies. If there is a good match, the baby passes the screening. If the match is not close enough, the baby refers on the screening.

These tests may be used alone or in combination. Both tests are accurate and reliable.  Some hospitals use a 2-step process.  Babies are first screened with OAE, and those who do not pass the OAE are then given the AABR.

NICU Hearing Screening Guidelines

Newborns who have been in the NICU for more than 5 days must have an Automated ABR to rule out a neural hearing loss. (Utah Newborn Hearing Screening Standards and JCIH Recommendations)

If hearing screening was missed or the family has not returned for the rescreen, it is essential for the primary care provider (PCP) to educate the family on the importance of this testing.  

If newborn hearing screening is performed in a physician’s office, state law requires that results be sent to the State EHDI Program (no parental release is required).  Please fax to (801) 536-0492 or email ehdi@utah.gov.

What happens if the baby does not pass hearing screening?

If a baby does not pass two hearing screenings, they should be referred to a Pediatric Audiologist for diagnostic testing and to complete CMV testing.

If a baby is diagnosed with hearing loss, they should be referred to agencies capable of providing specialized early intervention services for hearing impaired children and to appropriate medical specialists: ENT (required), genetics (recommended), ophthalmology, neurology or other specialists as needed.

What does CMV testing require?

This testing is time-sensitive and must be completed before the infant is 21 days old (to rule out a congenital infection vs acquired).  This test must be performed using either urine or saliva. Blood and cerebrospinal fluid are not sensitive and should not be used.  If testing has not been completed prior to 21 days of age please contact the Utah EHDI Program at (801) 584-8215.

CMV testing results (both positive and negative) are required to be reported to the Utah EHDI Program. Please fax to (801) 584-8492 or email ehdi@utah.gov.

What happens if the baby is positive for CMV?

If an infant has a positive CMV test, they should undergo a complete diagnostic audiologic evaluation as soon as possible.  Frequent audiologic re-assessment is also needed to promptly identify and treat progressive hearing loss.  The frequency of this testing should be determined by the child’s audiologist.

A general recommendation for all babies with congenital CMV (regardless of their newborn hearing screening results) is to have a hearing re-assessment every 3 months in the first three years of life, and then every six months through age six years; however, each child should be considered on an individual basis as timing of assessments may need to be more frequent or altered based on antiviral therapy, rehabilitation needs, pediatric audiologist guidance, or parent concerns.

To date, there is no FDA approved drug to treat congenital CMV infection. There is limited data on the use of antiviral medications in infants with symptomatic congenital CMV infection.  Studies are ongoing to determine what types of therapy are of greatest benefit to CMV-infected infants.  Infants with suspected congenital CMV infections should be evaluated by physicians who specialize in these infections.

More information on CMV is found here.

 

Forms

Resources

Hospitals

photo of newborns holding handsGuidelines for conducting well-baby hearing screenings
  • Test no sooner than 12 hours after birth or as close to discharge as possible.
  • Test when infant is quiet or sleeping.
  • Ensure a quiet environment for testing, away from background noise.

If a newborn does not pass the screening it is acceptable to repeat the screening no more than two times during the same session using the same technology.

Guidelines for conducting hearing screenings in the NICU

Newborns who have been in the NICU for more than 5 days must have an Automated ABR to rule out a neural hearing loss. (Utah Newborn Hearing Screening Standards and JCIH Recommendations)

Communicating hearing screening results to families

  • Provide results of the screening, whether the newborn passed or did not pass the hearing screening, and explain what the results mean. Follow EHDI Screener Guidelines and Scripts.
  • Results should be conveyed verbally and in writing to the parents.
  • If the infant did not pass the initial screening, do not minimize the importance of the rescreen.
  • Schedule the repeat hearing screening appointment prior to discharge if an infant does not pass the initial screening.
  • If the initial screening was not completed (the baby was missed or results were incomplete) prior to discharge, it is the birth facility's responsibility to follow-up with the family and schedule outpatient screening.
  • Provide all parents with information on milestones of normal auditory, speech and language development in children.

CMV Mandate Responsibility

  • It is the hospital screening program’s responsibility to report newborn hearing screening results to the primary care provider (PCP), the family and the State EHDI program..
  • If the baby does not pass inpatient screening, a CMV fax form must be completed and sent to the PCP to notify of the failed screening.
  • If the baby returns and does not pass their outpatient screening, a CMV fax form and physician letter must be sent to the PCP to notify of the need for CMV testing, as well as to the State EHDI Program ehdi@utah.gov.
Forms Resources

Midwives

photo of baby being heldBirth attendants are required by law to assure initial hearing screening occurs no later than 10 days of age. If a newborn does not pass the hearing screening, a follow-up screening should be performed no later than 14 days of age.

If the newborn fails their initial hearing screen and the screen was performed more than 14 days after birth OR if the newborn fails their rescreen, the newborn should immediately be referred to their Primary Care Physician (PCP) for congenital Cytomegalovirus (CMV) testing.

A CMV fax form should be completed and sent to the primary care provider (PCP) and to Utah EHDI (801) 536-0492. CMV testing is time-sensitive and must be completed before 21 days of age in order to rule-out congenital vs. acquired CMV. CMV testing is simple and painless, and is accomplished using a urine or saliva sample (the inside of your baby’s cheek is swabbed).

Forms Resources

Audiologists

photo of a child with cochlear implant playingAs an Audiologist, you play a key role in the EHDI Process:
  • Confirm CMV testing results.
  • Complete diagnostic audiologic evaluation prior to 3 months of age (JCIH Recommendations).
  • Report the results to the child's Primary Care Physician (PCP) and Fax to (801) 584-8492 (Utah EHDI Program)
  • Make a referral to Baby Watch/Early Intervention for children, under the age of three, who fail two hearing screenings and/or are diagnosed with permanent hearing loss within 7 days of diagnosis.
Forms Resources

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