Screening for congenital and acquired deafness and hearing loss (HL) represents a significant responsibility for clinicians in all newborn units and medical homes. Approximately 1 to 3 infants of 1,000 born in a well infant nursery setting and 2 to 4 of 100 born in a NICU have significant bilateral HL. Outcomes in various developmental domains depend on the early recognition and subsequent intervention for HL. Developmental delay is present in 30% to 40% of children with HL; the goal of hearing screening (HS) and prompt intervention is to optimize a child’s language acquisition, cognitive development, literacy, social-emotional maturity and academic success. This article reviews the most up-to-date standards and practices to aid medical providers in the early detection and intervention for pediatric HL.In the United States, universal screening begins in the neonatal period. According to the American Academy of Pediatrics (AAP) Joint Commission on Infant Hearing (JCIH), all infants should be screened for congenital HL before 1 month of age. Most infants are screened in nursery or NICU settings, except for those born outside of a medical facility. For the latter population or for infants with no documented HS, screening should be completed by the medical home before 1 month of age.In a well nursery setting, most HSs are performed by otoacoustic emission (OAE) testing. During an OAE screen, a sensitive microphone in the ear canal records the cochlear responses (“acoustic emissions”) to acoustic stimuli, thereby testing the peripheral auditory system from the outer/middle ear (conduction zones) to the cochlear outer hair cells (sensory organ). A “pass” indicates that hearing is functional to the level of the cochlea. Children may fail this test if the system is impeded along any point. Thus, a “fail” will not distinguish between true congenital HL and reversible or transient abnormalities of the outer/middle ear (a false-positive). Excess wax buildup in the outer ear, middle ear effusions, or cochlear dysfunction may all result in a failed screen. A failed initial screen by OAE may be repeated before hospital discharge or followed by a complementary test known as an auditory brainstem response (ABR). ABR screening records neural activity generated in the cochlea and sent via neural pathways through the auditory nerve (cranial nerve VIII) to the brain stem in response to acoustic stimuli via an earphone. Because the cochlea is involved with both OAE and ABR, each screen can detect cochlear (sensory) HL; similarly, each test is also affected by outer/middle ear dysfunction that may be present. Infants who pass the ABR after a failed OAE have passed their newborn HS. However, ABR alone detects neural dysfunction of the auditory nerve and/or brainstem auditory pathway. A failed initial ABR may be repeated before discharge with another ABR. Rescreen by OAE after ABR is inappropriate because the child may have neural HL that will not be identified on OAE. Compared with ABR, OAE screening is less costly, takes less time to complete, and is less invasive (because it does not require electrode placement), making it the most widely used initial screen for HL in a nursery setting.All infants admitted to the NICU for more than 5 days are considered to be at high risk for neural HL and are screened by ABR. Failed ABR screening in the NICU should result in immediate referral to an audiologist for diagnostic evaluation of bilateral hearing ability, even if ABR detected unilateral HL. Notably, if an infant is readmitted to an inpatient setting (well nursery or NICU) before 1 month of age and has a condition that may be associated with HL (Table 1), that infant should have repeated HS by ABR before discharge.Any failed newborn HS should result in a comprehensive audiological evaluation before 3 months of age, and early intervention services should commence no later than 6 months of age for those who are found to have HL.Outside of the newborn period, the JCIH recommends that infant HS should occur regularly in the medical home. Clinicians should assess for the presence of risk factors for congenital or delayed-onset HL that may not have been picked up by the newborn HS. Infants who pass initial newborn HS but have risk factors for HL (Table 1) should have a diagnostic audiology evaluation no later than 24 to 30 months of age. In addition, routine in-office HS should include objective screening of developmental milestones at ages 9, 18, 24, and 30 months by a validated global screening tool (such as the Ages & Stages Questionnaires), middle ear examination (with pneumatic otoscopy as necessary to establish diagnosis of effusion), evaluation of apparent hearing skills in the examination room through observation, and consideration of any caregiver concerns about hearing, language, or communication. Objective communication delay as well as physician or parental concern should prompt immediate referral for speech-language pathology and audiologic evaluation to rule out HL. Because otitis media and effusion are associated with low-frequency HL, the presence of effusion for 3 months in children with risk factors for HL warrants referral for audiometry. Children with effusion without risk factors or clinical concern for HL should be evaluated again at 3- to 6-month intervals for resolution or clinical change.Once a diagnosis of HL is made, federal guidelines require that referral to an early intervention program be initiated within 2 days of confirmation of HL for children younger than 3 years.In keeping with the AAP Periodicity Schedule, audiometry screens should take place in the medical home during childhood at ages 4, 5, 6, 8, and 10 years. Risk assessments should occur during the interim years. Standard HS typically includes low-frequency tones (<3,000 Hz) at these ages because the development of low-frequency HL is more common and often associated with a history of otitis media and effusion. Due to greater sensitivity in detecting high-frequency HL, adolescents should be screened with audiometry that includes high frequencies (6,000 and 8,000 Hz) once during early (11–14 years), middle (15–17 years), and late (18–21 years) adolescence. Approximately 1 in 6 adolescents in the United States has high-frequency HL. This is thought to be related to acoustic trauma, and physicians should counsel patients on the risks of HL associated with headphone/ear bud use and environmental exposures.Studies have been conducted to evaluate the effectiveness of the previously mentioned nationwide guidelines. Currently, more than 95% of infants in the United States are screened for congenital HL before hospital discharge. However, only approximately 50% of infants among those who fail initial HS ultimately receive the appropriate evaluation and services after discharge. Programs for children with HL should include speech-language pathology services, audiology, and access to educators of the deaf, and they may require referral to genetics or otolaryngology, among other subspecialties. Referrals for amplification devices/implants or reconstructive surgery may also be needed. Given the gap that exists between recognition and intervention, primary care providers must focus on ensuring adequate follow-up and coordination of care for all children with risk or concern for HL after HS. Developmental and academic outcomes ultimately hinge on early detection and coordination of care for the child with HL.Comments: HL can range from mild to profound and from unilateral to bilateral; be categorized as conduction, sensory, or neural HL; and be due to multiple etiologies. This is a major public health problem, and early intervention can maximize educational outcomes and social functioning for children who are affected. Dr C. Everett Koop, past US Surgeon General, encouraged identification and treatment of HL to be included in Healthy People 2000 goals for our nation. Federal agencies and national organizations, including the National Institutes of Health, the Centers for Disease Control and Prevention (CDC), the Maternal and Child Health Bureau, the AAP, the JCIH, and others, along with Congress have banded together to develop policy statements and road maps to address this important issue. Mandates have emphasized strategies for screening to provide early identification, followed by interventions such as sign language and other communication strategies, personal amplification, or cochlear implants that are individualized to each child. To accomplish these goals the screening needs to take place as outlined in this In Brief in conjunction with a finely tuned team approach with collaboration between information shared from the nursery or NICU discharge staff to primary care providers and timely referrals to audiologists to confirm the HL, otolaryngologists to identify the etiology of the HL and assist with interventions, and other subspecialists, including geneticists. Families of children and the children with HL have benefited from support groups with adult and child peers. It truly takes a village and a commitment among all involved to be successful in these programs and assist children with HL to obtain the services they need to reach their full potential.