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Online EHDI Case 6 (Questions without 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.


1. What are your thoughts?

2. What are your recommendations?

Click <HERE> for answers to Part 1

Part 2

3 weeks later (Tommy is now 5 months 1 week) and you received a phone call from the audiologist who performed the comprehensive evaluation on Tommy. The results indicate a bilateral moderate to severe sensorineural hearing loss (SNHL). The following are the specific findings:

  • OAEs are absent bilaterally
  • Acoustic emmittance testing reveal normal middle ear function bilaterally 
  • (Tympanometry) with elevated reflex threshold in both ears.
  • Diagnostic ABR reveal responses ranging from 45-80 dB across the frequency range from 500 to 4000 Hz for both ears.

1. What are some of the reasons Tommy would have passed this newborn hearing screening with his sensorineural hearing loss?

2. According to JCHI 2007, what are some of the risk factors that are listed as greater concerns for late onset hearing loss?

Click <HERE> for answers to Part 2

Part 3

Tommy returns for his 9 months old check up. He continues to grow and develop well. Soon after his diagnosis, he started to receive early intervention services and was fitted with hearing aids; He has now accepted his hearing aids and wears them during all waking hours. A repeat ABR had been completed a couple months ago which confirmed the severity of the loss without any more
progression. The family is pleased with the observation that they are seeing at home and the way Tommy consistently responds to sounds when he has his hearing aids on.

He was recently seen by his audiologist again and behavioral testing (Visual Reinforcement Audiometry) was obtained reliably and revealed similar thresholds compared to his last ABR.

The medical work up is also well underway and medical results are back. The MRI did not reveal any anomalies, but genetic tests revealed a mutation in the GJB2 gene, encoding the connexin 26 (Cx26) protein at the 13q12 location.


1. What are your thoughts now?

2. What are the main teaching points of this case?

3. How can you, as a primary care provider, monitor for late onset hearing loss?

Click <HERE> for answers to Part 3


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