• Check hearing screening results for all newborns in your practice. B
• Refer all newborns who fail screening for audiologic and medical evaluation and diagnosis before 3 months of age. B
• Refer infants with diagnosed hearing loss for early intervention services no later than 6 months of age. B
• Educate families about services and resources available to them and their hearing-impaired child. B
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
Congenital permanent hearing loss occurs in about 3 of every 1000 births.1 Undiagnosed hearing loss can result in speech-language, academic, social, and other developmental delays. Until about 20 years ago, most children with hearing loss were not diagnosed until about 3 years of age.2 By that age, opportunities for effective intervention to help these children develop communication skills were often delayed, and many children remained seriously disabled.
In this enlightened age, when newborn hearing screening is nearly universal (92%), the prospects for children with hearing impairments are brighter—but not as bright as they could be.3 That’s because more than half the newborns with positive screens are lost to follow-up.3 Too many remain “lost,” without a diagnosis or access to services, until they show up at school without the language skills they need to keep up, academically or socially, with their classmates.
The medical home can help
The American Academy of Pediatrics (AAP) and the Health Resources Services Administration aggressively promote the concept of the medical home as the best locus for coordinating the care of children with special needs, and the American Academy of Family Physicians (AAFP) has endorsed the medical home concept.2,4 According to the AAFP’s Joint Statement of Principles, the medical home is responsible for coordinating care across all elements of the health care system and the patient’s community.4 Most physicians in a recent survey believed the medical home should be responsible for coordinating services and guiding families in the development of intervention plans for children with hearing loss.5 Family physicians who provide a medical home for infants and young children are in an ideal position to ensure that children with hearing loss are not lost to follow-up and that they receive the services they need to lead healthy lives.
What follow-up entails
According to the 2007 Position Paper of the Joint Committee on Infant Hearing, a body composed of representatives from the AAP, the American Academy of Audiology, the American Speech-Language-Hearing Association, and other professional organizations concerned with hearing loss, an effective program to mitigate the impact of hearing loss should follow this timetable6:
- By 1 month of age, all infants should receive hearing screening. (Of note: In 2008, the US Preventive Services Task Force issued a B recommendation for universal hearing screening of all newborns.7)
- By 3 months of age, hearing loss should be diagnosed.
- Within 1 month of diagnosis, hearing aids should be fitted for infants whose parents choose hearing aids.
- As soon as possible after diagnosis—but no later than 6 months of age—infants with confirmed, permanent hearing loss should receive early intervention services.6
Intervention services should include medical and surgical evaluation, evaluation for hearing aids, and then cochlear implants for those with severe-to-profound hearing loss who do not benefit from hearing aids. Communication assessment and therapy should also be considered. The goal of intervention is to help infants with hearing loss develop communication competence, social skills, emotional well-being, and positive self-esteem.6
The CLINICAL TOOL provides a detailed overview of the early hearing detection and intervention (EHDI) process outlined by the Joint Committee and a checklist of roles and responsibilities for physicians serving as medical homes for children with hearing loss and their families. Unfortunately, many primary caregivers report that their medical training did not prepare them to guide families through this process.8-10 This article is intended to provide the additional information caregivers have requested to help them meet these obligations.
The early hearing detection and intervention process6
|Prenatal period||Birth – 1 month||By 3 months||No later than 6 months|
|Education for parents about the importance of newborn hearing screening and the distinction between screening and diagnosis||Hearing screening for all infants in hospital or at audiologist out-patient facility||Infants with positive screens are diagnosed by an audiologist with auditory brainstem response testing||Physician and parents monitor developmental milestones and experience with hearing aids|
|Auditory brainstem response screening for all NICU infants ≥5 days of age||Hearing loss is ruled out or confirmed||Meeting with family and early intervention personnel to develop an individualized family service plan|
|Rescreening for all infants with risk factors who are hospitalized within 1 month of discharge||Audiologist shares results with family, medical home, and the early hearing detection and intervention coordinator||Individualized family service plan is in place, as mandated by federal law under the individuals with disabilities education Act|
|Infants are linked to a medical home before discharge by the hospital||Families are counseled regarding diagnosis and follow-up and given educational materials||Early intervention services are instituted in accordance with the individualized family service plan|
|Screening results are given to families and to the baby’s medical home||Audiologist recommends treatment in the medical home or referral to an ear, nose, and throat specialist for medical evaluation and treatment||Together, audiologist and family develop expectations for hearing aids|
|Families are counseled about screening results and follow-up||Audiologist and/or physician provides referrals for genetic counseling and ophthalmologic consultation||If hearing aids are unsuccessful, families are counseled about cochlear implants|
|Audiologist alerts parents and the medical home that child may need hearing aids and early intervention services||If family wishes, audiologist or medical home makes a referral to a cochlear implant team|
|Audiologist or physician counsels parents on the test results, treatments, and communication options: aural-oral, total communication, and sign language||Medical clearance and insurance authorizations are obtained for cochlear implants|
|Reports from all involved providers and agencies are transmitted to state early hearing detection and intervention coordinator|
|Checklist for family physicians|
|____ Encourage all families to have their baby’s hearing screened||____ Review screening results||____ Review audiologic diagnostic evaluation results||____ Coordinate early intervention services|
|____ Explain screening procedures||____ Make referrals for outpatient screening and audiologic diagnostic evaluation by an audiologist||____ Ensure that an audiologic reevaluation has been completed||____ Confer with audiologist on child’s progress with hearing aids and consideration of cochlear implants|
|____ Assess for family history of hearing loss||____ Ensure all infants hospitalized after discharge are rescreened||____ Review findings from otolaryngology and audiologic consultations with family||____ Provide families with basic information about cochlear implants|
|____ Provide informational materials about newborn hearing screening||____ Assess risk factors for hearing loss, including congenital and delayed-onset types||____ Encourage family to comply with professionals’ recommendations and stress importance of keeping appointments||____ Counsel families about the risks and benefits of cochlear implants|
|____ Answer family’s questions about newborn screening or hearing loss||____Ensure that screening results have been transmitted to state early hearing detection and intervention coordinator||____ Refer for genetic counseling and ophthalmologic consultation||____ Make referral to cochlear implant team|
|____ Provide families with preliminary information on amplification and communication options||____ Encourage families to comply with professionals’ recommendations|
|____Confirm that families have received informational materials on screening and follow-up||____ Make referral to audiologist for hearing aids||____Ensure all reports are transmitted to state early hearing detection and intervention coordinator|
|____ Ensure hearing aids are fitted within 1 month of diagnosis||____ Monitor developmental milestones|
|____ Provide medical clearance, insurance authorization, and referral for hearing aids and early intervention services|
|____ Ensure otolaryngology and audiologic results have been transmitted to state early hearing detection and intervention coordinator|
|NICU, neonatal intensive care unit.|