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Auditory testing


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I am an audiologist working with a child who has a mild-to-moderate hearing loss and wears behind-the-ear hearing aids. Experience has shown improved test results for kids with CdLS by age two. This child does not respond well in the sound booth and would need to be sedated for a Brainstem Auditory evoked Response (ABR). He is a sedation risk. When would you do the ABR, since it is a risk? When should we give up hope of improved hearing? What are audiologists finding with CdLS and how are the children doing?

Answer of our experts

I am not an audiologist but I will give you what information I can, based on my experiences. Not all children with CdLS show a marked improvement on their hearing tests, although many of them go from severe losses to normal hearing by age two. I cannot respond to the question about when to do the ABR, particularly since this child is a sedation risk. Most children have the ABR during their first year. Some of the hearing results may be due to an auditory neuropathy, although I am not aware of any tests indicating this is the case in this syndrome. Based on what I have learned with other children, the improvement in hearing seems to occur by age 3 years.

I would think that checking the ABR would be helpful from the therapy point of view. Even though there is a sedation risk, you could do the sedation with a pediatric anesthesiologist, who could always contact the pediatric anesthesiologist on our Clinical Advisory Board for advice.

MG/TK 7-13-10

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Recommendation(s)

Ears and hearing

R39
Hearing should be assessed in individuals with CdLS at an early age and should be followed up over time. Those with severe sensorineural hearing loss should be assessed for auditory neuropathy.
R40
Regular eye (ophthalmologic) and ear, nose and throat (otolaryngologic) evaluations are recommended in adults with CdLS.
R41
Otitis media (middle ear infections) with fluid build up and sinusitis in individuals with CdLS should be considered and treated according to the national guidelines for the general population.

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