Problem with baby’s hearing? An intervention checklist

The Journal of Family Practice. 2011 February;60(2):E1-E7

February 1, 2011|The Journal of Family Practice

Victoria A. Williams, BA;Carole E. Johnson, PhD, AUD

It’s time to make sure infants with positive screens for hearing loss get the follow-up treatment they need—and deserve. This tool can help.

Getting an early start: Newborn screening

Ideally, intervention should begin before a child is born. When parents come in for prenatal visits, talk to them about the importance of newborn hearing screening. Tell them to expect that their baby will be screened at the birth hospital or at an outpatient audiology facility, and that this screening should be done by the time the baby is 1 month of age.

Tell parents their baby’s hearing will be tested with automated screening equipment that measures otoacoustic emissions from the baby’s ears or auditory brainstem response (ABR) to sound, both measurements that correlate with a child’s hearing and auditory behavior. Infants in well-baby nurseries can be screened by either technology. Infants in neonatal intensive care units (NICUs) for more than 5 days should be screened with ABR technology, which is better able to pick up neural losses.⁶ NICU babies frequently are at higher risk for neural hearing loss including auditory neuropathy/dyssynchrony, a condition that may account for about 8% of pediatric hearing losses annually.³

Be sure to explain to expectant couples that a positive screen indicates only that a problem may exist. It is not equivalent to a diagnosis of hearing loss, which is usually made by an audiologist.

The medical home’s role. The medical home should make sure that infants who fail an initial or secondary hospital screening and those who were missed or born outside the hospital are referred for outpatient screening. Newborns who fail initial screening should have both ears rescreened before hospital discharge, even if only one ear had failed previously.⁶ Additionally, any infant readmitted to the hospital within the first month of life who has a condition associated with potential hearing loss should be rescreened before discharge, preferably with an ABR.⁶ Risk factors associated with permanent-congenital, delayed-onset, or progressive hearing loss in children are listed in the TABLE.

TABLE
Risk indicators associated with hearing loss in childhood

Caregiver concerns* about hearing, speech, language, or developmental delay Family history* of permanent childhood hearing loss Neonatal intensive care for ≥5 days, or with any of the following: - Extracorporeal membrane oxygenation* - Assisted ventilation - Exposure to ototoxic medications such as gentamicin and tobramycin or loop diuretics such as furosemide - Hyperbilirubinemia requiring exchange transfusion In utero infections such as cytomegalovirus,* herpes, rubella, syphilis, and toxoplasmosis Craniofacial anomalies, including those that involve the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies Physical findings, such as white forelock, that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss Syndromes associated with hearing loss or progressive or late-onset hearing loss,* such as neurofibromatosis and osteopetrosis, as well as Usher, Waardenburg, Alport, Pendred, or Jervell and Lange-Nielson syndromes Neurodegenerative disorders* such as Hunter syndrome, or sensory motor neuropathies such as Friedreich ataxia and Charcot-Marie-Tooth syndrome Culture-positive postnatal infections associated with sensorineural hearing loss,* including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis Head trauma, especially basal skull/temporal bone fractures* that require hospitalization Chemotherapy*
These indicators are of greater concern for delayed-onset hearing loss. Source:* American Academy of Pediatrics, Joint Committee on Infant Hearing. Pediatrics. 2007.⁶

Caregiver concerns* about hearing, speech, language, or developmental delay
Family history* of permanent childhood hearing loss
Neonatal intensive care for ≥5 days, or with any of the following:
- Extracorporeal membrane oxygenation*
- Assisted ventilation
- Exposure to ototoxic medications such as gentamicin and tobramycin or loop diuretics such as furosemide
- Hyperbilirubinemia requiring exchange transfusion
In utero infections such as cytomegalovirus,* herpes, rubella, syphilis, and toxoplasmosis
Craniofacial anomalies, including those that involve the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies
Physical findings, such as white forelock, that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss
Syndromes associated with hearing loss or progressive or late-onset hearing loss,* such as neurofibromatosis and osteopetrosis, as well as Usher, Waardenburg, Alport, Pendred, or Jervell and Lange-Nielson syndromes
Neurodegenerative disorders* such as Hunter syndrome, or sensory motor neuropathies such as Friedreich ataxia and Charcot-Marie-Tooth syndrome
Culture-positive postnatal infections associated with sensorineural hearing loss,* including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis
Head trauma, especially basal skull/temporal bone fractures* that require hospitalization
Chemotherapy*

*These indicators are of greater concern for delayed-onset hearing loss.
Source: American Academy of Pediatrics, Joint Committee on Infant Hearing. Pediatrics. 2007.⁶

Reporting screening results
Screening results are useful only if they are transmitted to caregivers and families. Breakdowns in transmission are a persistent problem for the early hearing detection and intervention process.² The process is facilitated when hospitals make sure that all babies and their families are linked to a medical home at the time of discharge.

Hospital personnel should also be responsible for providing screening results to families in a face-to-face meeting. Medical home providers should review screening results again with parents and answer any questions that might have arisen after the initial hospital stay. Screening results should be given to parents in a sensitive manner, and patient education materials should be provided in parents’ native language, written at an appropriate reading level. Because hospital staff may not be trained to do this properly, it is important that medical home providers oversee the process and address any parental concerns.²

Screening results should also be reported by the birth hospital to the state’s EHDI coordinator, part of the national tracking and surveillance system funded by the Centers for Disease Control and Prevention to ensure that all children with hearing loss achieve communication and social skills commensurate with their cognitive abilities. ¹¹ You can find contact information for your state coordinator at www.cdc.gov/ncbddd/ehdi/documents/EHDI_contact.pdf.

Diagnosing hearing loss

When an infant who fails newborn screening testing arrives at the medical home, the urgent next step is to make sure he or she is referred to a pediatric audiologist for a complete audiologic diagnostic evaluation. Advising parents to “wait and see” is not appropriate; researchers have identified that response as a major obstacle to successful follow-up.² The audiologic evaluation should be done by the time the baby is 3 months of age and should be performed by a pediatric audiologist specializing in diagnosis and management of young children with hearing loss.^4-6

References

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