Screening tools and recommendations for screening children for hearing loss

Although most permanent sensorineural hearing loss is detected through universal hearing screening in the newborn nursery, hearing loss may begin after the perinatal period (late-onset) and be progressive. The 1-3-6 Rule is crucial for early detection and intervention of hearing loss, which allows for improved speech and language development in deaf and hard-of-hearing children. Universal newborn screening programs aim to complete the hearing screening process in all infants by age 1 month, identify all babies with hearing loss by age 3 months, and provide interventions by age 6 months. [Joint: 2019] The Joint Committee on Infant Hearing encourages states already meeting the 1-3-6 benchmarks to work towards a more assertive 1-2-3 timeline so infants have access to language earlier, and audiologic testing can be completed in a natural sleep state, not needing sedation. Hearing Loss and Deafness has detailed diagnoses and management information.

The American Academy of Audiology advises school-based screening in preschool, kindergarten, and grades 1, 3, 5, and either 7 or 9. [American: 2011] The American Academy of Pediatrics (AAP) recommends subjective assessment of hearing (and assessment of risk for hearing loss) at all well-child examinations and objective hearing screening at school entry. When possible, the AAP also advises objective office-based screening for children 4, 5, 6, 8, 10, 13, 16, and 20 years old. Given the risk of hazardous noise exposure in adolescents, it is recommended to perform audiometry screening in adolescents that includes 6,000 and 8,000 Hz high frequencies. The AAP Periodicity Schedule recommends high-frequency screening once between ages 11-14, once between ages 15-17, and again between ages 18-21.

Regardless of the newborn hearing screen status, the Joint Committee on Infant Hearing recommends referral for audiologic evaluation for any child with delays in speech development or auditory skills, along with referral if there is any parental concern regarding speech or hearing.

Developmental abnormalities, poor cognitive function, or behavioral problems (e.g., autism/developmental delay) may preclude accurate results on routine audiometric screening. Referral to a pediatric audiologist with the necessary equipment and expertise to test infants and young children should be considered. If the audiologic evaluation is concerning, referral to an otolaryngologist would be the next step.

The following algorithms provide further guidance.

• Office Visit Hearing Assessment Algorithm (AAP): One-page algorithm accounting for risk factors and screen results [Harlor: 2009]
• Algorithm for Pediatric Medical Home Providers (AAP) ( 243 KB): One-page algorithm for screening, evaluation, and intervention of infants 0-6 months old; from the American Academy of Pediatrics Early Hearing Detection and Intervention (EHDI) program.

Risk factors that may warrant screening beyond that routinely recommended include:

• Caregiver or clinician concern regarding speech, language, or hearing delay based on surveillance or developmental screening
• Family history of permanent childhood hearing loss
• Birth weight <1500 g
• Neonatal intensive care ≥5 days (although some recommend any NICU length of stay) [Garinis: 2018]
• A history of ECMO (5-28% of ECMO survivors had hearing loss on follow-up testing) [Garinis: 2018]
• Hyperbilirubinemia requiring exchange transfusion
• Ototoxic medications >5 days, including aminoglycosides and glycopeptide antibiotics (gentamicin and tobramycin), loop diuretics (furosemide), and chemotherapy
• Mechanical ventilation >5 days
• Intrauterine TORCH infections, such as cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis
• Conditions associated with hearing loss - There are over 400 syndromes associated with hearing loss, such as neurofibromatosis type 2, osteopetrosis, and syndromes, such as Usher, Waardenburg, CHARGE, Alport, Pendred, and Jervell and Lange-Nielson.
• Neurodegenerative disorders, such as Hunter syndrome, or sensory-motor neuropathies, such as Friedreich ataxia and Charcot-Marie-Tooth syndrome [Harlor: 2009]
• Culture-positive postnatal infections associated with sensorineural hearing loss, including confirmed bacterial and viral (especially herpes viruses and varicella) and meningitis
• Head trauma, especially basal skull/temporal bone fracture that requires hospitalization
• Recurrent/persistent otitis media with effusion >3 months (or less if any other risk factors, such as speech delay) [Rosenfeld: 2016]

The Joint Committee also recommends that an AABR screen be performed prior to discharge (even if the newborn hearing screen was passed prior to re-admission) for newborns who are re-admitted in the first month of life for conditions that are associated with hearing loss, such as sepsis and hyperbilirubinemia requiring an exchange transfusion.

The following table from the Joint Committee on Infant Hearing’s report (2019) [Joint: 2019] provides recommendations on when to re-screen infants based on specific risk factors for hearing loss.

Risk Factors for Early Childhood Hearing Loss: Guidelines for Infants who Pass the Newborn Hearing Screen

Note. AAP = American Academy of Pediatrics; ABR = auditory brainstem response; AABR = automated auditory brainstem response. * Infants at increased risk of delayed onset or progressive hearing loss **Infants with toxic levels or with a known genetic susceptibility remain at risk ***Syndromes (Van Camp & Smith, 2016) ****Parental/caregiver concern should always prompt further evaluation.

See Early Childhood Hearing Outreach Initiative (NCHAM) for information on screening young children with otoacoustic emissions in the office. [Liming: 2016]

Newborns in the nursery who fail otoacoustic emission (OAE) screening can be re-screened by auditory brainstem response (AABR), if available, before discharge from the newborn nursery. If the AABR is passed, the infant has a passed newborn hearing screen and should be re-screened per guidelines detailed elsewhere in this article or as concerns arise. If the infant fails the AABR screen (either primarily or as the re-screen) or is unable to access an AABR, they may be re-screened as an outpatient prior to 1 month of age or referred for formal audiologic evaluation prior to 3 months (next steps vary from state to state).

1-3-6 Guidance for Medical Home Providers (AAP) ( 257 KB) has a helpful flowchart for next steps if the hearing screen is not passed in the first 3 months of life. Note that infants admitted to the NICU should only be screened by AABR or ABR modalities, given their risk of neural hearing loss. In some states, targeted Congenital Cytomegalovirus (CMV) testing is mandated for infants not passing their newborn hearing screening, while in others, universal CMV testing is completed as part of the newborn metabolic screen. Be sure to know the requirements in your state and ensure that necessary testing has been completed. If there is no mandated CMV testing, consider if this makes sense for your practice. Read more information at Newborn Screening (National CMV Foundation).

Furthermore, any child who does not pass a subsequent hearing screen should have a formal evaluation by a licensed audiologist. Children who are found to have hearing loss necessitate a comprehensive medical evaluation. [Joint: 2019] It is important to perform a thorough physical exam, looking for stigmata of genetic conditions or abnormalities of the head and neck areas, such as cleft lip or palate, heterochromia of the irises, and microcephaly, among others. Consider work-up for congenital cytomegalovirus infection and look for cardiac or renal anomalies. Primary care providers should request copies of the audiology report with the audiologist’s recommendations for further diagnostic evaluation. This report will also be sent to the state EHDI program. Once a child is determined to have hearing loss, a referral to the early intervention program is recommended within 7 days, and the JCIH recommends referral within 48 hours. [Joint: 2019]

Primary care clinicians can educate families on the connection between hearing and language development to help parents understand the importance of follow-up with appointments and early intervention services. The primary care provider should continue to monitor the child’s development and intervene as necessary.

Hearing screening is typically a simple evaluation providing “pass/fail” results. It is commonly performed in the newborn nursery or birthing center, primary care clinic, school, and daycare settings. People other than audiologists may be trained to provide hearing screening, including nurses, early intervention specialists, medical technicians, medical assistants, and teachers. Failed hearing screens should trigger more extensive diagnostic testing performed by a licensed (preferably pediatric) audiologist. Passing results usually do not require further hearing evaluations until the next recommended screening unless there is a strong index of suspicion or a known risk factor for late-onset hearing loss.

The most common methods used to test children’s hearing include:

• Otoacoustic emission (OAE) testing
• Auditory brainstem response (ABR) testing or brainstem auditory-evoked response (BAER)
• Pure-tone testing/audiogram

It is important for the primary care physician to remember that although OAEs and ABRs test the “structural integrity” of a child’s hearing, the behavioral pure-tone audiogram is the true measure of a child’s hearing ability. As Harlor and Bower note, “[e]ven if ABR or OAE test results are normal, hearing cannot be definitively considered normal until a child is mature enough for a reliable behavioral audiogram to be obtained. " [Harlor: 2009]

If a primary care office has screening equipment, it should be calibrated yearly. Those administering the screen should be trained appropriately. [Stewart: 2019]

Otoacoustic Emission (OAE)

Auditory Brainstem Response (ABR) / Brainstem Auditory Evoked Response (BAER) Testing

Audiometry

For failed hearing screens, diagnostic testing (“hearing evaluation”) should be performed only by a licensed audiologist trained in testing children and has access to the testing equipment needed to evaluate children. Children with special health care needs are best served by a pediatric audiologist with experience in working with this population. Early Intervention programs usually offer hearing screening or testing, as will state programs focused on children with special health care needs and/or disabilities. Newborn screening programs can also guide you to reliable resources, particularly those focused on following up on abnormal newborn screens.

The cost of hearing testing can range from <$100 for a screening evaluation to >$700 for a full diagnostic evaluation, depending on the comprehensiveness of testing. Sedated procedures will cost more because medical staff is needed to provide safe sedation with patient monitoring. In many cases, insurance and other third-party payers will cover the cost of testing. Co-pays and deductibles will apply, so each family should check their insurance benefits to understand the cost before testing is performed. Some insurance plans will require a referral from a physician.

Normal hearing is essential for normal speech and language development in children. Even mild or fluctuating hearing loss can impact a child’s speech and language development. Though most children are now screened for hearing loss as newborns, it is important to retest hearing when there are concerns about hearing or speech/language development. Failure to meet the developmental milestones below should raise concern.

The Joint Committee on Infant Hearing’s most recent 2019 report offers recommendations for audiologic evaluation referrals. Pertinent patient populations who should be referred for audiologic evaluation include:

Pediatric Otolaryngology (ENT) (see RI providers [7]): The AAP strongly recommends referral to otolaryngology for further evaluation and to help determine the cause of hearing loss.

Speech - Language Pathologists (see RI providers [33]): Providers can also refer patients to speech-language pathology and genetics for further testing.

Pediatric Ophthalmology (see RI providers [8]): It is also recommended to refer to ophthalmology, given the association of certain eye conditions (such as Usher syndrome) with hearing loss.

Pediatric Nephrology (see RI providers [10]), Pediatric Neurology (see RI providers [18]), Developmental - Behavioral Pediatrics (see RI providers [12])Depending on associated conditions, providers can consider referrals to cardiology, nephrology, neurology, and developmental pediatrics.

Early Intervention for Children with Disabilities/Delays (see RI providers [13]): If hearing loss is detected in the first 3 years of life, children should also be referred to early intervention. See Service Providers below for referral information.