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Early detection of hearing status in children is critical to prevent the significant detrimental effect on cognitive development it can have if not appropriately addressed.
Early detection of hearing status in children is critical to prevent the significant detrimental effect on cognitive development it can have if not appropriately addressed. Current guidelines by the Joint Committee on Infant Hearing (JCIH) recommend what is commonly referred to as the 1-3-6 time line: All infants should receive a hearing screen before age 1 month; those who do not pass should receive a full pediatric audiology diagnostic evaluation before age 3 months; and those identified as deaf or hard of hearing should receive special services (such as early childhood intervention, access to visual and spoken language, and amplification) no later than age 6 months.
In a session titled “Listen up! Early detection of hearing loss” at the American Academy of Pediatrics (AAP) National Conference and Exhibition on September 19, Rachel St. John, MD, director of the Family-Focused Center for Deaf and Hard of Hearing Children at Children’s Health in Dallas, Texas, and associate professor, Department of Otolaryngology, at the University of Texas (UT) Southwestern Medical Center, Dallas, discussed some of the practical applications of the recommendations by the JCIH.
Updates to these recommendations are forthcoming and support an even tighter time line for hearing screening and evaluation. “The new recommendation is that if the 1-3-6 time line is being met, practitioners should aim for a 1-2-3 time line in order to maximize early intervention for optimal language development,” said St. John.
She emphasized that the overarching goal of these recommendations is to ensure that infants and children who are deaf or hard of hearing have access to the earliest and most complete language learning as possible, which encompasses presenting multiple opportunities for language learning including both spoken and sign language.
“We know from a growing body of research that early identification of hearing status and provision of support for language learning has a statistically significant effect on overall cognitive development, and lack of early language access can have detrimental and permanent developmental effects,” St. John said.
St. John also spoke about new recommendations for the evaluation of hearing in children who pass their newborn screen but have risk factors for late onset hearing loss. The new recommendations state that, in general, children with risk factors have a full audiologic evaluation by age 9 months. However, this time line varies according to the etiology of the hearing loss, with earlier and more frequent audiologic evaluation recommended for children with known etiologies that cause rapid and/or progressive changes, such as congenital cytomegalovirus.
St. John emphasized the benefit primary care practitioners can give to families by being aware of the time lines for newborn hearing screening, as well as by paying attention to expressive language milestone development and, importantly, concerns a parent or guardian may express about their child’s hearing. “Parental concern is an extremely sensitive indicator of possible hearing changes,” she said, “and children whose parents express such concerns should be referred immediately for a full audiology evaluation.”
Tremendous progress has been made in the United States since the 1993 National Institutes of Health Conference recommended screening newborns for hearing loss. In 2017, over 98% of newborns in the United States are screened. However, screening is only 1 step in the pathway to a successful early hearing detection and intervention (EHDI) outcome.
The American Academy of Pediatrics and Joint Committee on Infant Hearing EHDI recommendation is to screen by 1 month, diagnose by 3 months, and begin early intervention no later than 6 months. Some states are more successful in achieving this goal than others. Reasons for this include socioeconomic barriers, distance barriers, tracking challenges, and lack of trained professionals or adequate services.
Why is this important for the primary care provider (PCP)? The majority of family-centered care coordination needed for a successful outcome occurs after discharge from the birthing hospital and requires an effective partnership between the PCP and parents, as well as the key team of specialists needed to achieve success including audiology, otolaryngology, genetics, ophthalmology, and early intervention services offering access to both teachers of the deaf and speech language pathologists.
The process of referral and services provision is most effective in improving the language, behavior, literacy, and academic outcomes if EHDI 1-3-6 is done quickly and seamlessly. The first months of life are a period of incredibly rapid brain growth and babies are born ready to learn. Therefore, access to language, either spoken or sign language, is urgent. Studies consistently identify that the earlier the infant is diagnosed and receives early intervention, the more improved the outcomes. In addition, not all infants are identified by newborn screening, as shown by a doubling of children identified with a permanent hearing loss by school age. This is why postdischarge continued surveillance is so important.
So, how can the PCP achieve a supportive learning partnership with a family to facilitate timely access to diagnosis, amplification, and early intervention for hearing loss while respecting the choices of the informed and supported family?
· Be informed of the needs of the infant and family, and services available.
· Follow up with family to confirm that the infant is completing the EHDI process.
· Discuss options for communication as needed.
· Make certain the infant is receiving appropriate early intervention services.
· Continue active surveillance of hearing and language skills for all infants.
· Follow recommendations for follow-up of infants with risk factors for hearing loss.
· Establish a practice goal of moving EHDI 1-3-6 to EHDI 1-2-3.
-Betty R. Vohr, MD, is professor of Pediatrics at the Warren Alpert Medical School of Brown University, Providence, Rhode Island, and medical director of the Neonatal Follow-up Clinic in the Department of Pediatrics at Women & Infants Hospital of Rhode Island, Providence.