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Online EHDI Case 2 (Part 1 With Answers)

Online EHDI Case 2: A Child with Profound Hearing Loss

Part 1

History: Ismael Zawdi is a 2-month old infant who comes to your practice for the first time for well child care. He was a full term infant with an uncomplicated pregnancy, labor, and delivery. Birth weight was 3.2 kg. In the newborn period he did not pass his otoacoustic emissions testing (OAE). As instructed, his family took him for repeat testing at the hospital and again he did not pass. He then went to a local audiologist for Auditory Brainstem Responses (ABR). He had no responses up to 90 decibels in the right ear. Responses were not obtained for the left ear. Ismael is healthy, well grown, and in all other ways doing well. The family history includes a maternal cousin with hearing loss.
Ismael’s parents are from Saudi Arabia and have lived in the United States for almost 2 years. His father is a graduate student in mechanical engineering. His mother is college educated but does not work outside the home at this time. They both speak excellent English. They seem to have a good grasp on information they have received about hearing loss.
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1. What is your interpretation of his hearing status?

• The ABR suggests that Ismael has a permanent profound sensori-neural hearing loss (SNHL).
• Without amplification, this degree of hearing impairment precludes detecting anything except very loud sounds.
• Even with hearing aids that could potentially make some environmental sounds detectable, the child will not be able to hear most speech sounds.
• Ismael, therefore, may have extreme difficulty detecting and interpreting conversational speech. Thus, he is at significant risk for a severe disorder in spoken language.

2. What should Ismael’s family do next?

• Ismael and his family must follow-up with an audiologist to be fitted for hearing aids. [UNHS flow sheet]
• Given that Ismael was born in the United States, he is eligible for Medical Assistance to help finance the hearing aids regardless of his parents’ financial or immigration status. [http://www.phlp.org/Loophole%20guide%202-04.pdf]
• He should be referred to Early Intervention services, as per the protocol in PA.
[http://www.dpw.state.pa.us/Child/EarlyIntervention/ ]
• His family should be offered information about hearing loss. [Parent Handout]
• His parents should be offered information about family support.
[http://www.parentlinks.org/index_files/Page1148.htm
• He should be seen for a medical evaluation of his hearing loss, including genetic testing. However, given the number of appointments and issues at the present time, this step may be postponed to the next visit.
• The child should be carefully monitored over the first 6 months with a hearing aid. If he does not begin to respond to sound, he may be a candidate for a Cochlear Implant. [For more information on cochlear implantation, see http://www.nidcd.nih.gov/health/hearing/coch.asp ]

3. How does the family’s ethnic and cultural background affect your approach?

• This family is from Saudi Arabia. It will be important to ask the family about the roles and responsibilities of each parent in their culture to determine how best to support the child. Important questions are as follows:
    o Will both parents be reliably attending visits to the physician and audiologist?
    o If the father is not available for a visit, how will the family proceed? Could another family member or friend accompany Ismael’s mother to a visit?
    o Are there issues about transportation?
    o Will Ismael’s mother be able to accept an Early Intervention specialist into her home? Should that specialist be a woman?
• Another issue is what language to expose the child to. The timing about when to discuss this issue depends on the family’s understanding of the basics of hearing loss, their emotional responses, their support systems, and their ability to carry out the plan.

Additional Info

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