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Online-EHDI Case 1 (Part 3 Answers to Questions)

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

Part 3

Jamiel and Mrs. Sweager return for a scheduled 6-month well child visit. He is wearing hearing aids on both ears. His mother reports that when he got the aids, 2 months ago, she saw increased interest in sounds in the environment, including her voice. He now reliably turns to the source of sound. He is enrolled in early intervention services. A speech and language pathologist and/or hearing therapist comes to the family’s home once a week to work with Jamiel and Mrs. Sweager on receptive and expressive language skills. She is pleased with his progress because he seems to respond to his name specifically and has begun to babble “baba”. In response to your question about how well he tolerates the aids, she mentions that he never attempted to take them off of his ears. In all other ways, he is developing normally and growing well. His physical examination remains normal.


1. How do you assess the progress in this case to date?

• This case proceeded very smoothly. The child completed his screening by 1 month of age and his diagnostic evaluation by 3 months of age, in accordance with Healthy People 2010 and state benchmarks. [UNHS Flow sheet]
• He has also received definitive treatment with amplification and Early Intervention by 6 months of age, another PA and Healthy People 2010 benchmark. [UNHS Flow sheet]
• The initial indications are that he is using sound information more effectively than before he wore the hearing aids.
• The family should be offered a referral for genetic evaluation in order to determine whether there is a known etiology to this hearing loss and to determine the risks that these parents might have another child with hearing loss.
o In about 50% of cases of severe to profound deafness, the cause is genetic.
o The hearing loss may be associated with a recognizable syndrome involving other medical or physical findings in addition to the hearing loss. It may also be “nonsyndromic”, meaning that there are no other significant features besides the hearing loss. In most cases where the hearing loss is genetic, there is not a definitive syndrome and the hearing loss is an isolated genetic condition.
o There are several modes of inheritance: autosomal dominant, autosomal recessive, X-linked (dominant or recessive), multifactorial, or chromosomal. The precise mode of inheritance determines the risks of recurrence.
o The most common cause of non-syndromic deafness is cased by a defective gap junction beta 2 (GJB2) gene, which has instructions for a protein, Connexin 26 (CX26). This condition has an autosomal recessive pattern of inheritance.

2. What do you do next?

• Close monitoring of progress is part of the Medical Home []
o The child should get close follow-up by his primary medical team, including routine and preventive care as well as attention to the issues of hearing loss.
o The Medical Home should obtain reports from the EI team and the audiologist and in other ways serve as the care coordinator.
o Close follow-up should include routine monitoring of the child’s development and behavior with early attention if developmental delays or behavior problems arise. .

3. What type of communication options would you suggest and recommend to this family? (i.e. verbal v. sign language v. total communication)

• The parents must now consider what is the best option for communication. The best option for communication is a personal matter. The decision is influenced by the child’s degree of hearing loss, the pattern of hearing loss, the family’s preferences, and related issues.
• The options include:
o Oral-aural education: This approach teaches children to use residual hearing with amplification and to lip reading. The emphasis is on receiving verbal input and producing verbal output. If children can use these modalities adequately, then this option allows communication with the majority of people in the society.
o Sign language: American Sign Language or ASL is the official language used amongst the Deaf community and culture in North America. Other countries have their own sign language. Bilingual-bicultural education emphasizes sign language as the natural language for children who are deaf and de-emphasizes speech. This is often the choice for deaf children of deaf parents. For more information on ASL, visit
o Total communication: This method is the integration of speech with sign language and maybe useful for children with severe hearing loss who cannot learn readily from the verbal environment.
• Encourage parents to discuss all options with their families and therapists.

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