Disease: Newborn Infant Hearing Screening

    What is a newborn infant hearing screening program?

    Newborn infant hearing screening programs are designed to identify hearing loss in infants shortly after birth. All states have implemented these screening protocols within hospitals and birthing clinics. Most hearing screening tests are done prior to discharge from the hospital or birthing clinics.

    Typically, nurses or medical assistants are trained extensively on how to operate automated equipment for testing infants. Prior to discharge, each newborn has his/her hearing tested. If, for some reason, the newborn does not pass the screen, a rescreen is usually done. If the infant does not pass the second hearing test, he/she is referred to a specialist for further testing.

    Specialists who are experts at testing hearing are called audiologists. Audiologists have had training that emphasizes diagnostic hearing testing techniques as well as hearing rehabilitation of children and adults. Their postgraduate academic training requires a minimum of a master's degree.

    Why is it important to screen for hearing loss in all newborn infants?

    Significant hearing loss is the most common disorder at birth. Approximately 1%-2% of newborns are affected.

    Several national committees, including the National Institutes of Health, the American Academy of Otolaryngology/Head and Neck Surgery, and the American Academy of Pediatrics, have recommended that hearing loss in infants be identified, and when possible treated, prior to 6 months of age. This recommendation is based on studies that have shown that children identified with hearing loss prior to 6 months of age have a better chance of developing skills equivalent to their peers by the time they enter kindergarten. Children not identified until later (for example, it is very common to first identify hearing impaired children at age 2 to 3 years) may ultimately suffer from irreversible and permanent impairments in speech, language, and cognitive abilities when compared to their peers.

    Prior to the implementation of hearing screen programs, it was customary to only test those newborns who had known significant risk factors for hearing loss. This group included infants whose mothers suffered from illness during pregnancy, those who had a family history of hearing loss, or those who were exposed to drugs known to affect hearing. In addition, infants with the following conditions were included for hearing screening:

    • low birth weight and/or prematurity, or oxygen deprivation or breathing difficulties at birth;
    • high bilirubin levels (yellow color);
    • syndromes associated with hearing loss;
    • abnormal head or face structures;
    • infections such as cytomegalovirus, syphilis, herpes, or toxoplasmosis; or
    • low Apgar scores (which assess several health factors at one and again at five minutes after birth).

    However, despite the testing of all infants who fell into this "high-risk registry," over half of all newborns with hearing loss were missed!

    In order to identify this large group of hearing-impaired infants not identified with current testing protocols, it is now recommended that all newborns have a hearing test prior to discharge from the hospital. The goal of this program is to identify all hearing-impaired infants at an early age, thereby increasing these children's chance at healthy and more productive lives.

    How common is hearing loss in infants?

    Most permanent hearing loss is due to damage/malfunction of the nerve that transmits sound from the inner ear to the brain (auditory nerve). For those infants in whom a cause is determined, approximately half have a genetic condition and the remaining half have an acquired condition to explain their hearing loss.

    What are some of the causes of hearing loss in the newborn?

    Hearing loss in a newborn can be caused by a number of conditions. Some of the known risk factors include high bilirubin levels (jaundice), drugs that are toxic to the ears (for example, medicines that are given to the newborn to battle a serious infection may damage hearing as a side effect), prolonged mechanical ventilation, low Apgar scores, meningitis, prematurity, and/or low birth weight. Malformed structures in the middle or outer ear can also lead to hearing loss. Viral illness during the pregnancy, such as rubella (German measles) or cytomegalovirus (CMV), can be passed to the newborn and result in hearing loss. Hearing loss can sometimes be inherited in abnormal genes passed from the parents to the newborn or be the result of a gene mutation that occurred during fetal development. Genetic counseling is often recommended for parents to determine if heredity is the cause of the hearing loss. In approximately half of all cases of hearing loss, the cause is never determined.

    How is hearing in infants tested?

    Hearing in infants can be tested using two different methods: the auditory brainstem response (ABR) evaluations or the otoacoustic emission (OAE) measures. Both tests are accurate, noninvasive, automated, and do not require any observable response from the infant. Which test is used depends on the screening program's choice of instrumentation and training. For a screening tool, both methods are extremely effective. There are, however, some distinct differences in how the hearing is measured using an ABR versus an OAE.

    What is an ABR test?

    In order to process sounds, electrical impulses are transmitted through nerves from our ears to the brainstem at the base of the brain. An auditory brainstem response (ABR) is a physiological measure of the brainstem's response to sound. It tests the integrity of the hearing system from the ear to the brainstem. The test is performed by placing four to five electrodes on the infant's head, after which a variety of sounds is presented to the infant through small earphones. As the hearing nerve fires, the sound stimulus travels up to the brain. This electrical activity generated by the nerve can be recorded by the electrodes and is represented as waveforms on a computer screen. The audiologist can then present different loudness levels of each sound and determine the softest levels at which the infant can hear. For infant-screening purposes, only one sound is used to test the hearing, commonly referred to as a "click." The click is a grouping of several sounds to test a wider area of the hearing organ at one time. The click is typically presented at a loud level and a soft one. If a healthy response is recorded, then the infant has "passed" the hearing screen. Testing usually takes five to 15 minutes to complete.

    What is an OAE evaluation?

    An otoacoustic emission test (OAE) measures an acoustic response that is produced by the inner ear (cochlea), which in essence bounces back out of the ear in response to a sound stimulus. The test is performed by placing a small probe that contains a microphone and speaker into the infant's ear. As the infant rests quietly, sounds are generated in the probe. Once the cochlea processes the sound, an electrical stimulus is sent to the brainstem. In addition, there is a second and separate sound that does not travel up the nerve but comes back out into the infant's ear canal. This "byproduct" is the otoacoustic emission. The emission is then recorded with the microphone probe and represented pictorially on a computer screen. The audiologist can determine which sounds yielded a response/emission and the strength of those responses. If there is an emission present for those sounds that are critical to speech comprehension, then the infant has "passed" the hearing screen. Testing generally takes about five to eight minutes.

    OAEs and ABRs, is one test better than the other?

    Both tests have advantages and disadvantages when used for screening, and depending on the program and experience of the audiologist, either one can be utilized successfully. The OAE is easy and cost-effective. However, the false-positive rate (for example, an infant fails a hearing test but actually has normal hearing) may be higher for an OAE than for an ABR. The false-positive rate for ABR testing is approximately 4% when testing is done during the first three days of life. The false positive rate for OAE testing is 5%-21% for testing done during the first three days of life. This large variation between ABR and OAE testing is commonly felt to reflect the OAE testing device's increased sensitivity to residual amniotic fluid and vernix that is commonly found in the neonate's ear canal.

    The two tests, however, rely on different mechanisms of hearing for the screening. For in-depth testing and a complete hearing evaluation of infants, these tests work best together as a complement to each other.

    What does it mean when an infant does not pass the hearing screen?

    A newborn who fails an initial hearing screen may not necessarily have a permanent hearing loss or a hearing loss at all. There are many possible reasons why an infant may fail a hearing screening test. One common reason is that fluid from the birth may still be present in the ear canal. This fluid blocks the sound stimulus, preventing it from reaching the inner ear, and therefore causes the newborn to fail. Similarly, fluid in the middle-ear space behind the eardrum (a common site for infection in children) can also block the sound stimulus and lead to a false failed test. After these problems resolve, the infant usually passes the rescreen. Therefore, it is important to have at least one week between the initial hearing screen and the rescreen to allow the newborn a chance to "dry out."

    Another possible reason for a false failure is excessive noise or movement from the infant during the test. The responses that are recorded with an ABR or OAE are very, very small. Any movement or crying from the infant can prevent the equipment from detecting the response. Therefore, it is important that the newborn is quiet or sleeping for the hearing screen. Feeding the infant just prior to the screening is often very helpful. Although neither test is painful, they are novel experiences for the newborn and can be momentarily upsetting.

    If it becomes evident that an infant has a hearing loss, then a full diagnostic exam is necessary to determine the type and amount of hearing loss.

    What is the difference between a hearing screen and a diagnostic hearing test?

    The basic difference between a diagnostic test and a hearing screen is the amount of information gathered during the session. For example, if an infant fails the hearing screen, it is unknown if there truly is a hearing loss, how much hearing loss is present, or whether or not the hearing loss is permanent or correctable. A diagnostic test can usually answer these questions. The diagnostic test session is understandably longer and requires more interaction with the infant. Typically, a more extensive ABR is conducted, using a variety of test stimuli. OAEs are also performed to cross-check the results of the ABR. To complete a thorough test, the infant needs to sleep in the office for upward of 45 minutes. The more information that can be collected, the more complete the results will be.

    If an infant does not pass a hearing screen in the hospital, what happens next?

    Most hospital screening programs will refer infants who failed the initial screening test to a secondary center that specializes in more complete testing for diagnosis. Sometimes, simple problems, such as too much residual amniotic fluid and vernix in the ear canal, will resolve prior to the rescreen and the infant will pass the second test. The rescreen is an important step in determining whether the infant is able to hear, so it should not be taken lightly.

    If an infant does not pass the rescreen, then a full diagnostic test will be necessary. This test may be completed at the rescreen site or at another facility, depending on several factors.

    Why is it important to screen for hearing loss in all newborn infants?

    Significant hearing loss is the most common disorder at birth. Approximately 1%-2% of newborns are affected.

    Several national committees, including the National Institutes of Health, the American Academy of Otolaryngology/Head and Neck Surgery, and the American Academy of Pediatrics, have recommended that hearing loss in infants be identified, and when possible treated, prior to 6 months of age. This recommendation is based on studies that have shown that children identified with hearing loss prior to 6 months of age have a better chance of developing skills equivalent to their peers by the time they enter kindergarten. Children not identified until later (for example, it is very common to first identify hearing impaired children at age 2 to 3 years) may ultimately suffer from irreversible and permanent impairments in speech, language, and cognitive abilities when compared to their peers.

    Prior to the implementation of hearing screen programs, it was customary to only test those newborns who had known significant risk factors for hearing loss. This group included infants whose mothers suffered from illness during pregnancy, those who had a family history of hearing loss, or those who were exposed to drugs known to affect hearing. In addition, infants with the following conditions were included for hearing screening:

    • low birth weight and/or prematurity, or oxygen deprivation or breathing difficulties at birth;
    • high bilirubin levels (yellow color);
    • syndromes associated with hearing loss;
    • abnormal head or face structures;
    • infections such as cytomegalovirus, syphilis, herpes, or toxoplasmosis; or
    • low Apgar scores (which assess several health factors at one and again at five minutes after birth).

    However, despite the testing of all infants who fell into this "high-risk registry," over half of all newborns with hearing loss were missed!

    In order to identify this large group of hearing-impaired infants not identified with current testing protocols, it is now recommended that all newborns have a hearing test prior to discharge from the hospital. The goal of this program is to identify all hearing-impaired infants at an early age, thereby increasing these children's chance at healthy and more productive lives.

    How common is hearing loss in infants?

    Most permanent hearing loss is due to damage/malfunction of the nerve that transmits sound from the inner ear to the brain (auditory nerve). For those infants in whom a cause is determined, approximately half have a genetic condition and the remaining half have an acquired condition to explain their hearing loss.

    What are some of the causes of hearing loss in the newborn?

    Hearing loss in a newborn can be caused by a number of conditions. Some of the known risk factors include high bilirubin levels (jaundice), drugs that are toxic to the ears (for example, medicines that are given to the newborn to battle a serious infection may damage hearing as a side effect), prolonged mechanical ventilation, low Apgar scores, meningitis, prematurity, and/or low birth weight. Malformed structures in the middle or outer ear can also lead to hearing loss. Viral illness during the pregnancy, such as rubella (German measles) or cytomegalovirus (CMV), can be passed to the newborn and result in hearing loss. Hearing loss can sometimes be inherited in abnormal genes passed from the parents to the newborn or be the result of a gene mutation that occurred during fetal development. Genetic counseling is often recommended for parents to determine if heredity is the cause of the hearing loss. In approximately half of all cases of hearing loss, the cause is never determined.

    How is hearing in infants tested?

    Hearing in infants can be tested using two different methods: the auditory brainstem response (ABR) evaluations or the otoacoustic emission (OAE) measures. Both tests are accurate, noninvasive, automated, and do not require any observable response from the infant. Which test is used depends on the screening program's choice of instrumentation and training. For a screening tool, both methods are extremely effective. There are, however, some distinct differences in how the hearing is measured using an ABR versus an OAE.

    What is an ABR test?

    In order to process sounds, electrical impulses are transmitted through nerves from our ears to the brainstem at the base of the brain. An auditory brainstem response (ABR) is a physiological measure of the brainstem's response to sound. It tests the integrity of the hearing system from the ear to the brainstem. The test is performed by placing four to five electrodes on the infant's head, after which a variety of sounds is presented to the infant through small earphones. As the hearing nerve fires, the sound stimulus travels up to the brain. This electrical activity generated by the nerve can be recorded by the electrodes and is represented as waveforms on a computer screen. The audiologist can then present different loudness levels of each sound and determine the softest levels at which the infant can hear. For infant-screening purposes, only one sound is used to test the hearing, commonly referred to as a "click." The click is a grouping of several sounds to test a wider area of the hearing organ at one time. The click is typically presented at a loud level and a soft one. If a healthy response is recorded, then the infant has "passed" the hearing screen. Testing usually takes five to 15 minutes to complete.

    What is an OAE evaluation?

    An otoacoustic emission test (OAE) measures an acoustic response that is produced by the inner ear (cochlea), which in essence bounces back out of the ear in response to a sound stimulus. The test is performed by placing a small probe that contains a microphone and speaker into the infant's ear. As the infant rests quietly, sounds are generated in the probe. Once the cochlea processes the sound, an electrical stimulus is sent to the brainstem. In addition, there is a second and separate sound that does not travel up the nerve but comes back out into the infant's ear canal. This "byproduct" is the otoacoustic emission. The emission is then recorded with the microphone probe and represented pictorially on a computer screen. The audiologist can determine which sounds yielded a response/emission and the strength of those responses. If there is an emission present for those sounds that are critical to speech comprehension, then the infant has "passed" the hearing screen. Testing generally takes about five to eight minutes.

    OAEs and ABRs, is one test better than the other?

    Both tests have advantages and disadvantages when used for screening, and depending on the program and experience of the audiologist, either one can be utilized successfully. The OAE is easy and cost-effective. However, the false-positive rate (for example, an infant fails a hearing test but actually has normal hearing) may be higher for an OAE than for an ABR. The false-positive rate for ABR testing is approximately 4% when testing is done during the first three days of life. The false positive rate for OAE testing is 5%-21% for testing done during the first three days of life. This large variation between ABR and OAE testing is commonly felt to reflect the OAE testing device's increased sensitivity to residual amniotic fluid and vernix that is commonly found in the neonate's ear canal.

    The two tests, however, rely on different mechanisms of hearing for the screening. For in-depth testing and a complete hearing evaluation of infants, these tests work best together as a complement to each other.

    What does it mean when an infant does not pass the hearing screen?

    A newborn who fails an initial hearing screen may not necessarily have a permanent hearing loss or a hearing loss at all. There are many possible reasons why an infant may fail a hearing screening test. One common reason is that fluid from the birth may still be present in the ear canal. This fluid blocks the sound stimulus, preventing it from reaching the inner ear, and therefore causes the newborn to fail. Similarly, fluid in the middle-ear space behind the eardrum (a common site for infection in children) can also block the sound stimulus and lead to a false failed test. After these problems resolve, the infant usually passes the rescreen. Therefore, it is important to have at least one week between the initial hearing screen and the rescreen to allow the newborn a chance to "dry out."

    Another possible reason for a false failure is excessive noise or movement from the infant during the test. The responses that are recorded with an ABR or OAE are very, very small. Any movement or crying from the infant can prevent the equipment from detecting the response. Therefore, it is important that the newborn is quiet or sleeping for the hearing screen. Feeding the infant just prior to the screening is often very helpful. Although neither test is painful, they are novel experiences for the newborn and can be momentarily upsetting.

    If it becomes evident that an infant has a hearing loss, then a full diagnostic exam is necessary to determine the type and amount of hearing loss.

    What is the difference between a hearing screen and a diagnostic hearing test?

    The basic difference between a diagnostic test and a hearing screen is the amount of information gathered during the session. For example, if an infant fails the hearing screen, it is unknown if there truly is a hearing loss, how much hearing loss is present, or whether or not the hearing loss is permanent or correctable. A diagnostic test can usually answer these questions. The diagnostic test session is understandably longer and requires more interaction with the infant. Typically, a more extensive ABR is conducted, using a variety of test stimuli. OAEs are also performed to cross-check the results of the ABR. To complete a thorough test, the infant needs to sleep in the office for upward of 45 minutes. The more information that can be collected, the more complete the results will be.

    If an infant does not pass a hearing screen in the hospital, what happens next?

    Most hospital screening programs will refer infants who failed the initial screening test to a secondary center that specializes in more complete testing for diagnosis. Sometimes, simple problems, such as too much residual amniotic fluid and vernix in the ear canal, will resolve prior to the rescreen and the infant will pass the second test. The rescreen is an important step in determining whether the infant is able to hear, so it should not be taken lightly.

    If an infant does not pass the rescreen, then a full diagnostic test will be necessary. This test may be completed at the rescreen site or at another facility, depending on several factors.

    Source: http://www.rxlist.com

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