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Online EHDI Case 5 (Part 1 With Answers)

Online EHDI Case 5: A NICU Graduate with Neural Hearing Loss

Part 1

Samantha is 3 months old. She is visiting her pediatrician today for her 3 months old check up visit. She is accompanied by her parents. Samantha was born at 32 weeks gestation. She was delivered via c-section and was admitted to the NICU for 4 weeks because she was born with severe birth depression which required vigorous resuscitation and had some evidence of hypoxic ischemic encephalopathy (HIE) in the neonatal period.

She passed her universal newborn hearing screening (UNHS) at the hospital using the otoacoustic emissions test (OAEs) before she was discharged. Her parents report that Samantha is nursing well and has between 6 to 8 wet diapers a day and a few bowel movements.

Upon examination, Samantha is well and within her developmental age norms.

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1. Was the newborn hearing screening handled properly? 

 No- Samantha was born prematurely and was admitted to the neonatal intensive care unit (NICU) for more than 5 days.

 In the new Joint Committee on Infant Hearing (JCIH) 2007 (link to: http://www.pediatrics.org/cgi/content/full/120/4/898, the distinction is made between infants from the well nurseries and infants who are admitted to the NICU that calls for two separate hearing screening protocols.

    • Infants who are admitted to the NICU for more than 5 days are to have a screening Auditory Brainstem Response (aABR) included as part of their screenings so that neural hearing loss will not be missed:
http://www.babyhearing.org/HearingAmplification/Causes/Neuropathy.asp

http://www.pediatrics.org/cgi/content/full/120/4/898).Please note that many authors in the literature will use the term auditory neuropathy/dys-synchrony or AN/AD instead of neural hearing loss. (This may not be technically correct as discussed later in this case) For infants who do not pass the screening ABR in the NICU, referral should be made directly to an audiologist for screening and, when indicated, comprehensive evaluation including diagnostic ABR.

    • Infants who have graduated from the NICU represent 10% of the newborn population and infants who stay in the NICU for more than 5 days are considered to be “High Risk NICU”. Had Samantha been in the NICU for only 4 days, she would have had been considered a “low” risk and OAEs testing would have been consistent with JCIH 2007 (link to: http://www.pediatrics.org/cgi/content/full/120/4/898), recommendations….however, a neural loss would still be missed because OAEs are not sufficient to identify a neural loss.

 It is also important to remember that this child is already 3 months old. By the recommendations of the JCIH 2007 ((link to: http://www.pediatrics.org/cgi/content/full/120/4/898), diagnosis should be made by 3 months of age and habilitation should be in place by the time the child is 6 months of age.

 Also, in the event that a complete audiologic evaluation is needed, sedation/anesthesia typically is not needed for children under 6 months of age; so it is important to keep those guidelines in mind to ensure that proper referrals are made on time. 

 Did you also know….JCIH 2007 (link to: http://www.pediatrics.org/cgi/content/full/120/4/921)), recommends that infants who pass their neonatal hearing screening but have risk indicators (Appendix 2- Risk indicators) should have at least one audiology assessment by 24-30 months or sooner if concerns are expressed before that age.

2. What are your recommendations at this time?

 Refer Samantha for audiologic evaluation. At this time, she needs to be referred directly to a pediatric audiologist for complete evaluation since she is already 3 months old.

 The test should include otoacoustic emissions (OAEs), diagnostic auditory brainstem response (ABR) monitoring and acoustic immittance measurement (to include tympanometry and acoustic reflex measurements).

 Provide the parents with a brochure about developmental milestones (link to the CHP brochure) so they become familiar with age appropriate responses. It is sometimes easy for a parent to become overly concerned about their child’s lack of responses to a sound which seems “loud” to the parent. This brochure will help them understand what type of response to sound is expected at certain age. For instance, an infant who responds at a level which is considered to be “loud” for an adult with normal hearing is actually responding appropriately for her age. So it should not be surprising that an infant would not startle to a sound which to you sounds loud. Also at this young age, infants, when sleeping, are deep asleep and noises, rather than movements, may not wake them. But concerns by parents should be investigated to ensure that there is no hearing loss.

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