Case Study

A 29-year-old patient gave birth to a healthy infant two days ago. You visit her and her newborn in the hospital where the first-time mother tells you that her baby is scheduled to have a hearing test in the newborn nursery. She asks you whether this is a routine test or whether she should be worried her baby has hearing loss.

Case Study Questions

1. Which of the following responses is/are appropriate to answer the patient's question about screening for hearing loss in newborns?

   A. The U.S. Preventive Services Task Force (USPSTF) recommends screening for hearing loss in all newborn infants.

   B. Screening is not necessary unless a risk factor assessment determines the infant is at high risk of hearing loss.

   C. Newborns who are screened for hearing loss are more likely to receive earlier diagnosis and treatment than newborns who are not screened.

   D. Risk factors for permanent bilateral congenital hearing loss include neonatal intensive care unit admission for two or more days and a family history of hereditary childhood sensorineural hearing loss.

2. Which one of the following describes the most common sequence of screening tests used to detect hearing loss in newborns?

   A. Auditory brainstem response (ABR) is performed first, followed by otoacoustic emissions (OAEs) in all newborns.

   B. OAEs are performed first, followed by ABR in all newborns.

   C. ABR is performed first, followed by OAEs only in newborns who do not pass the ABR.

   D. OAEs are performed first, followed by ABR only in newborns who do not pass the OAEs.

   E. Both tests are performed in all newborns, and the order does not matter.

3. For infants who are diagnosed with congenital hearing loss, which one of the following statements about treatment interventions and outcomes is correct?

   A. Cochlear implants are the most effective treatment for mild to moderate hearing loss.

   B. Early detection of hearing loss in newborns has not been shown to affect language outcomes.

   C. Early intervention should include evaluation for hearing aids, medical and surgical evaluation, and communication assessment and therapy.

   D. The harms associated with the use of cochlear implants include a high risk of meningitis.

   E. The overall risks of complications of treatment are estimated to be at least moderate.

Answers

1. The correct answers are A, C, and D. The USPSTF recommends screening for hearing loss in all newborn infants. The USPSTF found good evidence that screening newborns for hearing loss is highly accurate and leads to earlier identification and treatment of infants with hearing loss.

The USPSTF recommends universal screening of all newborns (as opposed to targeted screening of newborns who are considered to be at high risk) because 50 percent of children with permanent bilateral congenital hearing loss have no identifiable risk factors. Risk factors known to be associated with permanent congenital hearing loss include neonatal intensive care unit admission for two or more days; several congenital syndromes; family history of hereditary childhood sensorineural hearing loss; craniofacial abnormalities; and certain congenital infections.

2. The correct answer is D. The most common sequence of tests is a two-step screening process in which OAEs are performed first, followed by ABR in those newborns who do not pass the OAEs. Any newborn who has a positive screening test for hearing loss should receive further audiologic evaluation and follow-up after discharge. Confirmatory testing is necessary before a diagnosis of permanent bilateral congenital hearing loss can be made.

3. The correct answer is C. Early intervention for hearing loss should be designed to meet the individual needs of the infant and the family, including evaluation for hearing aids or other amplification or sensory devices, medical and surgical evaluation, and communication assessment and therapy.

Cochlear implants are deemed most appropriate for treatment of severe-to-profound hearing loss, and are considered only after inadequate response to hearing aids. Complications of cochlear implant surgery include increased risk of meningitis; however, the overall risks associated with cochlear implants are thought to be small.