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Online-EHDI Case 1 (Part 2 Answers to Questions)

      Case 1: A Healthy Newborn That Does Not Pass the Hearing Screen


                                                             Part 2

Jamiel returns for his 2-month well child visit. Mrs. Sweager says that after your visit she returned to the hospital where the baby was born and he had a second Otoacoustic Emissions (OAE) testing. He did not pass that test. He was referred to an audiologist who did a diagnostic Auditory Brainstem Response (ABR) test. The test revealed that Jamiel has a bilateral moderate to severe Sensorineural Hearing Loss (SNHL) with greater loss in the high frequencies. Another visit with the audiologist is planned in 2 weeks for a hearing aid fitting. His mother said she is doubtful about the accuracy of the tests. Jamiel often makes some cooing sounds and he startles with loud noises, such as when the family dog barks or an ambulance with its siren on goes past their house. However, she no longer feels as sad and overwhelmed as she had been earlier.

Jamiel is otherwise doing well. He eats with gusto and follows a regular sleep schedule. He smiles socially. Physical examination reveals a well developed, well nourished 2 months old. Height, weight and head circumference are all 50-75%. The physical exam is entirely normal. She asks how this type of hearing loss might affect her child. She is worried about her ability to afford hearing aids, especially if the child pushes them off and loses them.
1. What are the implications of a moderate to severe sensorineural hearing loss (SNHL)?

• Hearing sensitivity is measured in Decibels Hearing Level (dB HL). Hearing thresholds are determined for different sound frequencies, measured in Hertz (Hz). For children, decibel measurements from 0 to up to 15 dB are considered to fall within normal range of hearing. Anything higher than that is abnormal. [Sample audiogram]
• Levels of hearing loss:
o A moderate SNHL means that the amount of energy required for the person to detect sound is about 40 to 55 dB HL. A moderate to severe SNHL means that the amount of energy required for the person to detect sound is about 56-69 dB.
o Jamiel’s results indicate that he hears at the moderate level in the lower frequencies and in the severe range in the higher frequencies.
• Conversational speech is typically at a 50 to 55 dB level. Therefore, a person with a moderate to severe loss who is not using a hearing aid will not hear conversational speech.

2. How do you interpret the cooing and startles?

• Children may coo and even begin babbling if they do not have normal hearing. However, they require normal hearing to sustain their sound production. Auditory feedback seems to be necessary to sustain cooing and babbling.
• Children may startle because loud noises are >90 dB HL or above their hearing threshold.
• Often environmental clues about loud noises, such as other people turning their heads to the source of sound vibration, signal the infant to look.

3. How can you help this child and family at this time?

• Create a medical home for the child and family []. Provide comprehensive, compassionate, continuous care for the child and family.
• Provide Mrs. Sweager with information about hearing loss and amplification. [Parent handout]
• Offer to assist with the treatment planning.
o The earlier the child receives hearing aids, the better. Early treatment allows children to detect and interpret sounds that they might otherwise learn to suppress and ignore.
o Educating an older child to pay attention to sound is much more challenging than allowing the child to learn to attend to sounds at a young age. Babies usually tolerate hearing aids better than do toddlers and young preschoolers.
• Encourage her to keep her appointment with the audiologist. Explain that the parents will need to make periodic visits to the audiologist for on-going monitoring and management and reassure them that following this plan will improve their child’s overall development.
o Do what you can to help the parents to develop a trusting relationship with their child’s audiologist.
o Hearing aids will need to be worn daily. Evaluate the family’s understanding of this feature. Encourage their participation in all steps of the protocol.
• Refer Jamiel to Early Intervention (EI). [] This is an important step in the protocol for children with hearing loss.
• If the parents are interested, provide them with information about parent-to-parent support. Link to

4. What are options for funding hearing aids?

• Different states have established funding mechanisms for hearing aids.
• In the Commonwealth of Pennsylvania, Medical Assistance will fund the purchase of hearing aids. The funding is not based on parents’ assets. Medical Assistance will cover purchase and maintenance of amplification regardless of parental income.
• Application for Medical Assistance requires many steps. The process begins at the
County Assistance office. For details on this procedure you can provide families with this handout: []

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