Funded by the PA Department of Health
Approximately 1 to 3 babies per 1,000 are born with a hearing loss. Of babies with hearing loss, about 90% are born to hearing parents.
Universal newborn hearing screening was developed to ensure that all babies are screened at birth to identify potential hearing loss. Babies should be screened no later than 1 month of age, diagnosed no later than 3 months of age and receive early intervention no later than 6 months of age.
What is the role of the primary care physician in the Early Hearing Detection and Intervention Program?
The primary care provider (PCP) in cooperation with the audiologist directs and coordinates, as needed, the evaluation and referral process within the child’s medical home.
How can I learn more about newborn hearing screening and early childhood hearing loss?
Review one or all of the 6 case studies that describe a patient scenario, the questions to consider and how to handle. Click here to learn more
How is the newborn hearing screening performed?
The two screening technologies currently available are Auditory Brainstem Response (ABR) and Otoacoustic Emissions (OAE).
If my patient did not pass his/her hearing screening, what should I do?
A baby who did not pass his/her hearing screening should be referred to a pediatric audiologist for an outpatient follow-up diagnostic evaluation that typically begins with a second hearing screening. It is important that parents understand that this does not necessarily mean their baby has a hearing loss. Information about hearing screening should be provided to parents in a professional and compassionate manner while stressing the importance of prompt and appropriate follow-up. Hearing screenings should be completed before an infant is one month of age and a full diagnostic audiologic evaluation should be completed before 3 months of age.
What specialists should I refer my patient to if he/she is diagnosed with a hearing loss?
Referrals to appropriate medical specialists such as an otolaryngologist and geneticist* may be indicated by the diagnostic audiologic evaluation. * The majority of children with hearing loss have a genetic component even if they do not have a syndrome or dysmorphic features.
Additional medical referrals may need to be made to neurology, cardiology, nephrology and developmental pediatrics. See “What are the risk factors for late-onset and early childhood hearing loss?” for a list of syndromes commonly associated with hearing loss.
How do I help families find parent support?
Hands & Voices Guide By Your Side of Pennsylvania (GBYS) is a support program for families of infants and toddlers who are diagnosed with a hearing loss. GBYS gives families, who recently learned their child is deaf or hard of hearing, an opportunity to talk and meet face-to-face with a Parent Guide, a trained and experienced parent of a child who is deaf or hard of hearing. The program is available to all Pennsylvania families with children up to age 3 at no cost. Read full details about Hands & Voices Guide By Your Side of Pennsylvania
Parent to Parent of Pennsylvania provides support to families of children with special health care needs, including children with hearing loss, by linking them to a parent who has a child with the same special health care need. Learn more at the Parent to Parent of Pennsylvania website
What are the risk factors for late-onset and early childhood hearing loss?
The Joint Committee on Infant Hearing (JCIH) recommends in the JCIH Position Statement 2007 that infants who have a risk factor for late onset hearing loss should have at least one comprehensive audiologic evaluation by 24- to 30- months of age. Testing should also occur any time there is parent, caregiver or professional concern regarding communication development.
What is the PCP’s role in reducing the number of infants who do not pass the newborn hearing screening and who are considered lost to follow up?
The PCP and primary care medical home play an important role in ensuring that timely follow-up and the appropriate documentation of that follow-up occurs when an infant does not pass the newborn hearing screening or does not receive a newborn hearing screening. Without the active assistance of the medical home, the infant may be considered “lost” in the early hearing detection and intervention (EHDI) system, which undermines the potential benefits of newborn hearing screening. A “wait and see” approach is never appropriate. An infant who does not pass his/her newborn hearing screening has a potential developmental emergency.
Specific actions the medical home can take to reduce the number of infants who do not pass their newborn hearing screening and do not receive follow-up care, or follow-up care is not reported back to the PA Department of Health, are summarized in 2 new tools from the AAP:
- 1-3-6 Newborn Hearing Screening Checklist – This handy tool for monitoring patient status in the hearing screening, rescreening, diagnostic and early intervention process can be integrated into patient charts to facilitate monitoring and follow-up.
- Reducing Loss to Follow-Up/Documentation in Newborn Hearing Screening: Guidelines for Medical Home Providers – This algorithm provides a visual decision-making platform for primary care providers.
What are the best practices for identifying hearing loss in babies and children?
For all babies entering your practice:
➢ Have a system in place in your office to ensure that the results of the hospital hearing screening are available in your office chart in a timely fashion. This is true whether the baby passes or does not pass that hospital screen. This should occur before the baby’s first visit within days of hospital discharge. The results should be obtained directly from the hospital before the baby’s first visit. Relying solely on a verbal report from the family is not a replacement for obtaining the results on a hospital discharge summary or from the attending physician in the hospital. Strategies that may assist in this process include:
- Work with birthing hospitals to develop a system to directly transmit important birth data (including hearing screening results) directly into your office electronic record.
- If your practice sees the baby in the hospital, ensure that hearing screening results are obtained and brought to the office by the practice member seeing the baby in the hospital.
- If your practice does NOT see the baby in the hospital, develop a relationship with the hospital attending physician whereby a newborn summary, including the results of the hospital hearing screening, is available to you by the time of the first visit.
- Regardless of how screening results arrive at your office, have a system in place to populate your office chart with hospital hearing screening results prior to the child’s first visit.
➢ Know the screening technologies used in the birthing hospitals from which you receive patients. OAE technology assesses cochlear function while automated ABR technology assesses neural pathways beyond the cochlea. While most babies will have hearing loss due to cochlear dysfunction, some babies, especially those requiring NICU care, are at risk for neural hearing loss. In babies with neural hearing loss, cochlear function may be normal and they may pass an OAE screen and still have significant hearing loss.
➢ Educate office staff that automated ABR screening may miss cases of mild hearing loss and that mild hearing loss may still have a significant impact on longer term language development.
➢ Implement a policy for ensuring that all hearing screening results (both positive and negative) are discussed with families over the course of the first few visits. This discussion should emphasize the need for follow-up of positive screens (see “For babies entering your practice after not passing their hospital newborn hearing screen” below) and the need to educate parents regarding developmental surveillance that may indicate a hearing loss for babies passes their hospital hearing screening.
For babies entering your practice after not passing their hospital newborn hearing screen:
➢ Know the hospital policies for follow-up of babies who do not pass the hospital screen. There are many possibilities, including:
- The family returning to the hospital newborn nursery for an outpatient rescreen;
- The family being referred to an audiology facility to receive either an outpatient rescreen or a full diagnostic evaluation;
- The family being told only to talk to you about what follow-up is necessary.
Just as with the hospital screen, if the family has been referred to an audiology facility or is returning to the hospital for follow-up, it is critical that you obtain the results of this evaluation. Outpatient screening must be complete and documented in your office before one month of age.
➢ Develop a standardized method for explaining to families why follow-up of a screen that is not passed is important. A semi-scripted discussion can accomplish this in a way that ensures families take the screening seriously and obtain a prompt follow-up while also providing reassurance. Rather than saying things like “it is probably just fluid”, assure families that even if hearing loss is detected, technology and treatment options are available, but the sooner the follow-up is completed, the better off their child will be.
➢ Ensure a diagnostic evaluation is done before 3 months of age on all babies who do not pass their newborn hearing screening but ideally this should be completed as soon as possible after birth. In most cases, it will be the responsibility of the primary care physician to make this referral for the diagnostic evaluation. Have a working relationship with an audiology practice that is qualified to perform a full diagnostic evaluation on infants. Not all audiology practices have this capability so it is vital that you inquire about their ability to do so. EHDI-PALS, a national online directory of audiology practices that is maintained by the CDC, is a valuable source for this information (http://www.ehdipals.org/).
➢ Develop a system in the office that allows staff to track if the appointment for a diagnostic evaluation is complete. This system should alert the office if the appointment is not kept so that the family can be contacted and reminded of the need for follow-up. Developing such a system will require that a diagnostic code be developed and recorded for babies that do not pass their newborn hearing screening. This will allow your medical record system to identify those patients needing a diagnostic evaluation.
➢ Dedicate a single office staff person to track patients in need of follow-up after a newborn hearing screening that is not passed.
➢ Ensure results of the diagnostic evaluation are available in your office and documented in the chart in a timely fashion. Ensure there is an office policy to have families return to the office to discuss the results of any diagnostic evaluation.
➢ Be aware that the PA Department of Health receives notification of all babies who do not pass their newborn hearing screening and tracks these cases to ensure appropriate follow-up. Working together with the Department of Health can increase the rates of follow-up for families reluctant to do so.
For babies entering your practice after passing their hospital hearing screen:
➢ Discuss results of the newborn hearing screening with parents.
➢ Identify factors that place a baby at risk of hearing loss in the future. These factors include (Asterisk * indicates risk factors are of greater concern for delayed onset hearing loss):
- Parent or caregiver concern*
- Family history*
- Neonatal intensive care of >5 days
- Congenital infection
- Craniofacial anomalies
- Syndromes associated with hearing loss or progressive or late-onset hearing loss*
- Postnatal infections (meningoencephatitis)
- Head trauma (especially basal skull/temporal bone fracture*)
For a detailed list of risk factors for late-onset and early childhood hearing loss go to http://www.paearlyhearing.org/pcp/risk-factors
➢ Develop an individualized plan for following babies who have risk factors. At a minimum, this should include a follow-up audiological evaluation within the first year of life. Some risk factors, however, may demand an earlier evaluation (see risk factors above with asterisk*).
➢ Modify your electronic medical record or paper chart as a way to prompt a discussion of risk factors and give a place to document that it occurred.
➢ Develop a diagnostic code for those patients identified “at risk” for hearing loss. This will allow a way to track those patients who require follow up. The responsibility for tracking should also be given to a single office staff person.
➢ Act on parental concern about hearing loss since it is a risk factor that should result in a prompt referral for evaluation. Never ignore parental concern or dissuade a family from receiving an evaluation because the baby passed the newborn hearing screening and, therefore, “could not have a problem with their hearing”.
➢ Consider purchasing an office OAE machine and train staff in its use. Proficiency in performing office OAE could allow routine follow up screening of infants even if risk factors do not exist. As noted above, however, this technology should not be used for NICU graduates due to their risk of neural hearing loss.
For babies entering your practice without having received a hospital hearing screen:
➢ Question families as to reasons why the screening was not completed. If there is hesitation on the part of the family, discussion must occur emphasizing the value of early diagnosis and safety of screening technologies.
➢ Make arrangements immediately to have a hearing screening performed and the results sent to the office as soon as possible.
➢ Discuss developing quality measures with the birthing hospital to reduce the risk of errors if failure to screen was the result of an error made in the hospital.
For babies diagnosed with hearing loss:
➢ Provide family support and assist with coordination of care after diagnosis of hearing loss. This is the primary function of the primary care physician though the PCP does not have to become an expert in managing all aspects of care for children who are deaf or hard of hearing. However, the functions of support and coordination must be recognized as a vital component of this care.
➢ Make a referral to audiology, as well as appropriate referrals to otolaryngology, ophthalmology and genetics in order to investigate possible etiologies for hearing loss.These referrals may or may not come directly from the primary care office but the office must assure that are made.
➢ Obtain results from these referrals and be available to discuss the results with the family.
➢ Ensure that a referral is made promptly to Early Intervention services and that a line of communication is open between EI and all the various medical providers. This may require having the family sign a release of medical information and records that facilitate the transfer of information among all services.
➢ Refer parents to PA Guide By Your Side, a program that provides trained parental support by parents who themselves have children with hearing loss (For more information go to http://www.paearlyhearing.org/families/diagnosed/item/70).
PA EHDI website
PA Guide By Your Side (parent support)
Risk Factors for Late-Onset and Early Childhood Hearing Loss
EHDI-PALS (online directory of audiology practices)
My Baby’s Hearing
National Center for Hearing Assessment and Management (NCHAM) at Utah State University
Hearing Loss in Children
January 31, 2017 Let’s Talk Webinar “Best Practices for Identifying and Monitoring Babies and Children for Early Childhood Hearing Loss”
This webinar is presented by Bob Cicco, MD, FAAP and features the stories of 3 parents with a child who is deaf or hard of hearing. The Best Practices for Identifying and Monitoring Babies and Children for Early Childhood Hearing Loss was developed through surveying practices in PA on the follow-up care provided to patients who did not pass their newborn hearing screening. The parent stories highlight many of these best practices which can be implemented to by pediatricians and their office staff to provide optimal care management of children with potential or diagnosed hearing loss. To begin the webinar, use the following link: http://paaap.adobeconnect.com/p29y6i0op62/
The Newborn Hearing Screening and Intervention Program of the PA Department of Health (PA DOH) works with physicians to ensure that babies receive a hearing screening no later than 1 month of age and that babies who do not pass their hearing screening receive a diagnostic evaluation by an audiologist no later than 3 months of age.
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