Expert: Harriet B Jacobster, Au.D. - 10/23/2008

Question
I have a brother that has been deaf since birth. I believe it had something to do with his thyroid glands, and I was told that is one of the things doctors check now when babies are born. My question is how we would go about trying to correct this now. He is 54 years old. How much would something like that cost?

Answer
Hi Pam,
Does your brother have Pendred syndrome? Pendred syndrome is an inherited syndrome which consists of deafness from the time of birth and enlargement of the thyroid gland (goiter) in the front of the neck. Pendred syndrome is a result of a defect in the production of the thyroid hormone, an essential hormone for normal body metabolism. I really do not know if anything can be done at this point in time.  For this, you need to consult an ENT or endocrinologist. Even if the thyroid problem is handled, my concern is for the auditory system.  Since your brother has been deaf since birth, his auditory center is most likely atrophied and it is very possible that sound would not be able to reach his brain. Of course, it is possible that with extensive habilitation, he may hear a little.  He needs to consult the ENT for a complete battery of auditory testing which should include Auditory Brainstem Responses, possibly Nystagmography as well, and imaging of the inner ear. I can't even guess about cost; this is an issue for insurance.
Hope this helps.
Harriet B. Jacobster, AuD
Board Certified in Audiology