Frequently Asked Questions

Frequently Asked Questions About Infant Hearing As pediatricians we need to know that an infant with hearing loss may present in our office and respond to environmental sounds and even our spoken voice, depending on the type of loss, the degree of loss and the frequencies affected. Such responses only confirm that hearing loss is truly an invisible disability. Even mild losses will impact speech and language learning. Physicians should listen carefully to parents who express concerns about speech and hearing. As you may recall in 1999 the American Academy of Pediatrics published a policy statement entitled, Newborn and Infant Hearing Loss: Detection and Intervention. This policy statement highlights the Academy’s recommendations in support of universal newborn hearing screening and can be found on the Academy’s website at http://www.aap.org/policy/re9846.html. More that twothirds of the states, including Florida, have passed legislation mandating universal newborn hearing screening, however, this legislation is seldom ideal in terms of providing optimal benefits for children and families. In Florida newborn hearing screening is mandated and birth hospitals report the hearing screening results on the Florida Newborn Screening Specimen Card. Results of newborn hearing screening are provided on the Newborn Screening Results Summary provided by hospitals to physicians of record at the time of birth. Refer to the this summary for information to be included in the newborn screening results letters to be sent to birthing hospitals and physicians. Q&A A brief overview of the physician’s role in the follow-up of infants who do not pass newborn hearing screening follows. This information provides a common knowledge base for managing the health and development of these young patients.  What should a primary care physician do if a newborn doesn’t pass the hospital-based hearing screening? o It is important to understand the prevalence of newborn hearing loss and the accuracy of initial hospital screening. If a newborn is never screened before hospital discharge, the child has one in a 650 chance of having an undetected congenital hearing loss. But, depending on the screening test used, the child who fails the initial screening may have a risk of confirmed hearing loss as high as one in 10! It is reasonable to reassure families that their own child may be shown to have normal hearing, but it is critical that every newborn who fails initial screening return for follow-up screening and confirmatory testing if indicated. This may be a joint responsibility shared with the hospital and/or an audiologist to whom the child was referred, but nothing is as powerful as a contact from the physician if for any reason the child has not received their follow-up hearing test within 30 days of birth. o Standard of care recommended:  Completion of follow up screening within 1 month of age.  When the child fails the follow-up screen, completion of an audiological evaluation by 3 months of age.  Immediate referral to an audiologist to obtain otoacoustic emissions and/or auditory evoked response evaluation.  If child is under treatment for middle ear effusion an audiological evaluation must be completed within 3 months of initial newborn hearing screening referral, regardless of middle ear status, to rule out sensorineural hearing impairment.  Children should not return to the follow-up screening site once they have received a second hearing screen.  Test environments that are too noisy or who have insufficiently trained personnel should not perform initial or follow-up screening.  The audiologist is required to refer a child with confirmed permanent hearing loss to the local Early Steps early intervention program for family-centered services and to the primary care physician or otolaryngologist for medical management and medical clearance for amplification.  Referral sites should consist only of an audiologist who has the capability to perform hearing evaluation on children 0-36 months of age. o Link to AAP Algorithm: Universal Newborn Hearing Screening, Diagnosis, and Intervention - Guidelines for Pediatric Medical Home Providers http://www.medicalhomeinfo.org/screening/Screen%20Materials/Algorithm .pdf o As with any other area of healthcare there are legal risks involved when standard of care is not followed. There have been two legal suits in Florida regarding cases where the physician did not refer a child who had been identified through newborn hearing screening on for audiological evaluation.  When should the infant’s hearing be rescreened? o Many hospitals perform a second screen for newborns failing the first screen. This is accomplished prior to discharge. In these cases the audiologist will retest the child’s otoacoustic emissions prior to performing additional diagnostic tests. In the minority of hospitals that only perform screening only once, infants should return to the hospital or go to a community pediatric audiologist for rescreening within the first few weeks of life. It is a reasonable goal to have every infant with hearing loss identified by two months of age. Any delay in this process deprives the developing brain of the auditory stimulus it requires for normal language development. Even profoundly deaf newborns can benefit from effective use of communication strategies and amplification starting at one to three months of age. Also, outpatient rescreening or diagnostic testing requires the infant to very quiet or asleep. Every month that passes decreases the likelihood that the infant will be in a quiet state at the time of audiologic evaluation. Infants older than 3 months may require sedation to perform the testing.  What should the physician do to encourage parents to follow up on obtaining audiologic evaluations? o Link to AAP: When a Newborn Doesn’t Pass the Hearing Screen: How Medical and Other Professionals Can Help Increase the Number of Infants Who Return for a Follow-Up Evaluation http://www.aap.org/healthtopics/visiohhearing.cfm What does rescreening typically involve? o Rescreening may initially be performed with the same screening techniques used in the hospital, either otoacoustic emission testing (OAE) or automated auditory brainstem response testing (AABR). These screening tests are “physiologic” in nature of their measurement, rather that “behavioral” testing which requires the observation of a behavioral response in the infant. o Link to AAP: Hearing and Newborn Screening Information for Parents http://www.medem.com/medlb/article_detaillb_for_printer.cfm?article_ID= ZZZS5I9Y65D&sub_cat=1 o Link to AAP: Hearing and Understanding Screening Results Information for Parents http://www.medem.com/medlb/article_detaillb_for_printer.cfm?article_ID= ZZZ88BR175D&sub_cat=1  What should parents be told if their newborn does not pass the screening test in the hospital? o First, parents should be reassured. Most infants who do not pass the hospital-based test are eventually shown to have normal hearing. But parents should not be complacent – every baby who fails the initial screen must return for follow-up. As many as one in ten children who are identified during newborn hearing screening are confirmed as having hearing loss and over half of them have no identifiable hearing loss risk  factors. If the child is subsequently identified as having a congenital hearing loss, parents should know that early intervention has been shown to have dramatic results, with subsequent language development at nearnormal levels. Parents will never need to say, “If only I had known sooner.”  What should a physician do if a child is discharged before screening can occur? o Each licensed birth center that provides maternity and newborn care services is responsible for providing hearing screening services to all newborns within the first 30 days of life (FS 383.145). Immediate referral for hearing screening within the first 30 days of life is performed by hearing screening personnel at the birth hospital, an audiologist, or appropriately performed under the supervision of a physician. Screening shall result in a conclusive pass or refer status. Children not passing screening procedures in one or both ears shall be referred immediately for full audiological evaluation. Children should not return to the screening site once they have received an appropriately performed hearing screening test. Test environments that are too noisy or that have insufficiently trained personnel should not perform initial screening or rescreening. Parents or legal guardians may waive the right to hearing screening for their newborn. o Link to AAP Algorithm: Universal Newborn Hearing Screening, Diagnosis, and Intervention - Guidelines for Pediatric Medical Home Providers http://www.medicalhomeinfo.org/screening/Screen%20Materials/Algorithm .pdf  What should a physician do if a child has risk factors for hearing loss? o Action required for infants with identified high risk factors for hearing loss: The risk factors identified during newborn hearing screening are known to be associated with late onset hearing loss that will cause delays in communication development. Referral for a hearing evaluation performed by an audiologist specializing in children, age birth to 3 years needs to be considered at every routine medical visit until age 3 years. Referral should be made when there is parental concern about hearing or indication of delay in communication development. Communication development monitoring using a formal screening measure (i.e., Early Language Milestones – ELM) at every growth and development check-up at intervals no greater than 6 months is recommended until age 3 years. Physicians are required by Federal law 34 CFR§303.321d to refer children with evident or suspected developmental delays or impairments to their local early intervention program http://www.cms-kids.com/ContactUs/EIPdir.pdf. Referrals must be made within 2 working days of when the child is identified with an evident disorder or impairment that could affect their early development. o Link to AAP Algorithm: Universal Newborn Hearing Screening, Diagnosis, and Intervention - Guidelines for Pediatric Medical Home Providers http://www.medicalhomeinfo.org/screening/Screen%20Materials/Algorithm .pdf  What about the infant who only fails the screening in one ear? o Even though unilateral hearing loss may be a less severe condition than bilateral hearing loss, these infants also deserve prompt follow-up. Children with unilateral hearing loss have ten times the risk of children with two normal hearing ears to experience difficulties at school age. Effective early intervention services to the families can minimize future problems. Equally important, it has been found that one in seven of these children have progression of hearing loss in the ear that initially passed screening. In addition to rescreening, any child with a confirmed unilateral hearing loss must be followed closely over time to assure that the condition does not evolve into a bilateral hearing loss. If a unilateral hearing loss is confirmed, the parents can be counseled about how to maximize the child’s language development by being sure that the auditory stimuli are reaching the better ear effectively. What can parents expect in an audiologic evaluation if the infant does not pass the rescreen? o Most importantly, the primary care physician should make sure that all follow-up and confirmatory testing is performed by a pediatric audiologist with experience testing infants and fitting them with hearing aids, even if this requires extra travel for the infant and family. You can determine who is a qualified pediatric audiologist by asking how many infants that provider has seen in that month, and whether he or she has expertise in using physiologic and behavioral methods to test hearing in infants. A list of pediatric audiologists by county can be found at http://www.cmskids.com/ContactUs/PedAudiolst.pdf. Neurologists also use Auditory Brainstem Responses to evaluate the integrity of the brainstem in specific neurological disorders. This assessment is inherently different than the pediatric audiologists’ assessment using ABR to evaluate infant hearing, thus referral to a neurologist for an infant hearing assessment is NOT appropriate. o Explain to parents that they can expect the audiologist to administer a battery of painless tests to assess the integrity of the auditory system from the outer ear through the inner ear and even the brain stem. This confirmatory testing is also performed using ”physiologic” testing, typically including a standard diagnostic Auditory Brainstem Response (ABR, also  know as BAER), can include performance of steady state ABR (also known as ASSR), along with additional OAE testing. The ABR can include different types of stimuli and asses the system for air conduction and bone conduction. Testing for middle ear function appropriate to young infants will also be done. The presence of hearing loss is often confirmed on the first or second test appointment however, the test battery may require several visits to fully gather the information needed to completely characterize the hearing loss. Behavioral testing techniques are reliable after an infant is 6 to 12 months old – far too late to allow for early intervention and early use of amplification, but these test techniques should become a part of the test battery used in the ongoing assessment on the infant with hearing loss. o Florida Audiologic Assessment Guidelines for Infants and Toddlers http://www.cms-kids.com/SHINE/Aud_assmt_guidelines_8-23-04.pdf  Where can I find a list of pediatric audiologists for my county? http://www.cms-kids.com/ContactUs/PedAudiolst.pdf  If a child is confirmed to have congenital hearing loss, what else should the primary care physician do? o Address family concerns. Families need help understanding this new medical development. Work closely with the audiologist to better understand the nature and degree of the hearing loss. Pediatricians should interface with professionals in early intervention programs to assist families in obtaining services to help them address the developmental needs of the infant with hearing loss. o Arrange for a complete evaluation by an otolaryngologist or otologist with experience working with infants and young children. Some children require further evaluation to assess the potential of progressive hearing loss, and children for whom hearing aids are recommended require medical clearance. o Due to recent discoveries of the increasing number of genetic syndromes among children with congenital hearing loss, it is recommended that these families also be referred to a medical geneticist with experience in the field of congenital hearing loss. Thirty percent of hearing loss is of uncertain etiology and there are more than 200 syndromic and non-syndromic forms of hearing loss that have been identified; about 20 percent will have associated clinical findings. o Link to AAP: Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss http://www.aap.org/healthtopics/visionhearing.cfm For genetic consultation families can be referred to:  University of Florida, Gainesville (352) 392-4104  University of South Florida, Tampa (727) 892-4237  University of Miami, Miami (305) 243-6848 o Every affected newborn should have a complete evaluation by a pediatric ophthalmologist to assure that the visual stimuli to the brain are in no way compromised, and to assess for any associated eye anomalies or genetic syndromes with both visual and auditory impairment. Children with hearing impairment have a higher proportion of visual disorders than their peers with normal hearing. o After hearing loss is confirmed, physicians need to be involved in the following:  Coordinating services with the Individuals with Disabilities Education Act Part C agency in their local area. Part C agencies are responsible for Child Find and intervention for children with disabilities. The Part C agency in Florida is under the Department of Health, Children’s Medical Services. These services are provided under the administration of the Early Steps State Office (850-245-4200).  Monitoring middle ear status to avoid further compromise to hearing.  Monitoring developmental milestones is critical as 30-40 percent of children with hearing loss will demonstrate multiple disabilities or delays. Children receiving Early Steps early intervention services will receive monitoring of their developmental skills.  What can a family expect when they are contacted by the early intervention program – what services are offered? o One component of early intervention services provided by Early Steps programs is specific to meeting the unique needs of children with hearing loss and their families. This component is called SHINE, or Serving Hearing Impaired Newborns Effectively. Any child with a confirmed hearing loss up to age 3 years can receive SHINE services. SHINE provides information and support to parents and caregivers in the comfort of their home or another preferred environment. The SHINE provider shares information about hearing loss, amplification use, cochlear implants, communication and auditory skill development, and provides unbiased information on communication choices parents have in regard to speaking, signing, and/or cueing to their child. SHINE providers have an ongoing role with the family in monitoring communication development and discussing the child’s progress with the family. Even though hearing loss affects speech production, services in the first years of life are best provided by a specialist in hearing impairment. Parents can choose to have specialists in hearing impairment serve their child and family in addition to receiving SHINE services so that a good match is found of the child’s communication development needs and the parent’s desire for communication development options. o Florida Early Steps Service Locations http://www.cmskids.com/ContactUs/EIPdir.pdf  Tell me more about the legal requirement for me to refer children with identified disability conditions (including permanent hearing loss) to the local early intervention program? o Physicians are required by Federal law 34 CFR§303.321d http://www.csped.com/laws_regs/idea300-304/part303/ to refer children with evident or suspected developmental delays or impairments to their local Early Steps intervention program. Referrals must be made within 2 working days of when the child is identified with an evident disorder or impairment that could affect their early development. Contact information for local Early Steps programs can be obtained from http://www.cmskids.com/ContactUs/EIPdir.pdf or by calling (850)-245-4200. What does audiologic habilitation mean? What does it entail? o Audiologic habilitation includes hearing aid fitting. Hearing aid fitting proceeds when evaluations from audiology, otolaryngology and other medical evaluations have been completed and the parent is in agreement with this recommendation. The fitting should take place in the first 2-3 months of life. Early hearing aid fitting is possible based on physiologic testing alone to provide maximum access to the acoustic features of speech in a safe listening range. What should a physician do to identify late onset hearing loss in children? o A proportion of children who do not have risk factors and who pass newborn hearing screening subsequently experience late onset hearing loss. Not all hearing loss risk factors can be effectively identified in the newborn nursery, therefore, the primary medical care provider has the responsibility to identify potential risk factors and to refer to audiology for hearing evaluation if a child has one or more hearing loss risk factors and a concern about hearing or communication development arises. The hearing loss risk factors that are associated with late-onset hearing loss are:  congenital infection (neonatal herpes, CMV, HIV, rubella, syphilis, toxoplasmosis)  stigmata or other findings associated with a syndrome known to include sensorineural or conductive hearing loss or eustachian tube dysfunction    neurodegenerative disorders such as Hunter syndrome or sensory motor neuropathies such at Friedreich’s ataxia and Chacot-Marie-Tooth syndrome  postnatal infections associated with sensorineural hearing loss including bacterial meningitis  head trauma  syndromes associated with progressive hearing loss such as neurofibromatosis, osteopetrosis, and Usher’s syndrome  recurrent or persistent otitis media with effusion for at least 3 months. o The 2000 Joint Committee on Infant Hearing report specified risk factors http://www.infanthearing.org/newsletter/v3i1/jcih.html Links from State and National Hearing-Related Organizations Florida Speech and Hearing Association http://www.flasha.org/ Florida Academy of Audiology http://www.audiologyonline.com/associations/userpages/faa/index.asp Florida Society of Otolarnygology, Head and Neck Surgery http://www.fsohns.org/about_fsohns.asp National Center for Hearing Assessment and Management (NCHAM) http://www.infanthearing.org Marion Downs National Center for Infant Hearing http://www.colorado.edu/slhs/mdnc/ AAP Links Related to Newborn Hearing Screening and Follow Up The National Center for Medical Home Initiatives: Newborn and Infant Hearing Screening Activities http://www.medicalhomeinfo.org/screening/hearing.html Algorithm: Universal Newborn Hearing Screening, Diagnosis, and Intervention Guidelines for Pediatric Medical Home Providers http://www.medicalhomeinfo.org/screening/Screen%20Materials/Algorithm.pdf Minimizing False-Positives in Universal Newborn Hearing Screening: A Simple Solution http://www.aap.org/mrt/mfpu.htm A12 - page physicians' guide to the procedure accompanies each pack of Newborn Hearing Screening and Your Baby. http://www.aap.org/bst/showdetl.cfm?&DID=15&Product_ID=3741 My baby’s been referred for a second hearing screening test or for follow up hearing testing http://www.babyhearing.org/ When A Newborn Doesn't Pass the Hearing Screen: How Medical and Other Professionals Can Help Increase the Number of Infants Who Return for a FollowUp Evaluation http://www.aap.org/healthtopics/visionhearing.cfm Talking with Parents about Hearing Loss http://www.medicalhomeinfo.org/screening/EHDI/Descripton%20of%20EHDI%20 Program/TennesseeWhatDoIsayID2.pdf Hearing and Newborn Screening Information for Parents http://www.medem.com/medlb/article_detaillb_for_printer.cfm?article_ID=ZZZS5I 9Y65D&sub_cat=1 Hearing and Understanding Screening Results Information for Parents http://www.medem.com/medlb/article_detaillb_for_printer.cfm?article_ID=ZZZ88 BR175D&sub_cat=1 Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss http://www.aap.org/healthtopics/visionhearing.cfm We suggest the following information be put in a sidebar or highlighted in some other way Childhood Hearing: A Sound Foundation in the Medical Home The online module Childhood Hearing: A Sound Foundation in the Medical Home is now available on www.PediaLink.org for registration. The module content was developed by nationally and internationally renowned pediatricians and audiologists, and is presented in an interactive and informative manner, including engaging vignettes, multimedia, and resources you can personalize and download for your own office. You can participate in this module from the convenience of your own office or home. With this course, members can: -Assess their knowledge with comprehensive pre and post tests -Learn about: Different types of hearing loss Screening methods for hearing loss Medical and genetic risk factors The importance of early recognition and ongoing surveillance The role of the medical home in hearing screening -Download clinical tools and reference materials Beginning October 1, 2004, all AAP members will be able to register for only $50 (normally a $75 value). This limited-time, limited quantity offer expires November 30, 2004. To take advantage of this extraordinary opportunity, please register at www.pedialink.org. As part of your participation in this offer you may be asked to provide feedback about this eLearning activity. Special thanks to Mary Pat Moeller, Ph.D, Betty Vohr, MD, Judy Gravel, Ph.D, Mark Goldstein, MD, Daniel Choo, MD, Becky Harris, Bob Englander, and the NCBDDD/CDC Early Hearing Detection and Intervention Team for devoting their time and effort in developing this module. This module is funded through a cooperative agreement with the National Center on Birth Defects and Developmental Disabilities at the Centers for Disease Control and Prevention. If you have any questions regarding this module, please contact Yulee Lee at ylee@aap.org