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Online EHDI Case 6 (Part 1 with Answers)

Online EHDI Case 6: Late-Onset Bilateral Progressive Sensorineural Hearing Loss

Part 1

Tommy is 4 months 2 weeks old. He was born at 39 weeks gestation. There is no family history of childhood hearing impairment. He passed his Universal Newborn Hearing Screening (UNHS) a few days after birth before being discharged. Otoacoustic emissions (OAEs) had first been administered but Tommy failed that test. Subsequently, a screening (aka: automated) Auditory Brainstem Response (aABR) was then administered that he passed in both ears.

Tommy is accompanied to today’s visit by his parents. You saw him a month earlier for his 3 months check up and at that time he was doing well and in overall good health. He continues to nurse well and often (every 2 hours). He is surprisingly very alert and gets excited when he sees the dog or his siblings.

His parents are here today because they are concerned about his hearing abilities. Their concerns come from observation that, even though Tommy remains stimulated by visual cues, he seems to be regressing when noises and sounds are involved. Mom reports that, 2 weeks ago, Tommy had startled to the sound of a fire truck passing by with its siren on. Yesterday, he did not seem to have a reaction to the sound of a police siren until he actually saw the car and it’s flashing lights.

Upon examination, Tommy is physically doing well and within the norms. Otoscopic examination revealed healthy outer and middle ear bilaterally.

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1. What are your thoughts?

• Infants will not react to sound the same way we do until they are roughly in their 7th month of age. Therefore, when parents are reporting lack of responses to sound, it is important to remember the child’s developmental age. (Developmental milestones handout- should be available from previous cases)
• However, all children need to be monitored with developmental surveillance, including parental/caregivers report, even for those who pass their hearing screening at birth. Additionally, it is recommended that a comprehensive audiologic assessment be conducted at least at 24-30 months of age for those children with risk factors.
• In Tommy’s case, what the parents are reporting is regression of Tommy’s responses to sound stimuli. Even though he is young, Tommy seemed to be responding appropriately (startling, jerking, eye movements) just a few weeks ago. More recently, he has not.
• Tommy passed his newborn hearing screen and today his ears look clear upon otoscopic examination. However, hearing thresholds can change and, as stipulated by JCIH 2007 Position Statement, “caregiver concerns regarding hearing” is a risk indicator associated with permanent hearing loss. Therefore, this parental report is an indication for referral for a comprehensive audiologic evaluation.

2. What are your recommendations?

• Tommy should be referred for a complete audiologic evaluation immediately. It is important to remember that this child is already 4 months old. By the recommendations of the JCIH 2007 Position Statement, 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 (including early intervention, assistive devices and medical work up). Also, because of his young age, a complete audiologic evaluation (including a diagnostic Auditory Brainstem Response (ABR)) would require sedation/anesthesia once Tommy reaches about 6 months of age. It is therefore important to keep those guidelines in mind to ensure that proper referrals are made on time. To ensure fast service, you should contact the local pediatric audiology facility to ensure that Tommy will be seen as soon as possible in view of his age and to avoid the need for sedation/anesthesia ABR.
• You will be in touch with the family when you have received the results of the evaluation.

 

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