Expert: Harriet B Jacobster, Au.D. - 10/15/2008

Question
QUESTION: My 5 year old has been diagnosed with a sensoneural hearing loss. Well actually, we're going back for a confirmation of this in 2 weeks. They want to do one more hearing test. He failed the one at the pediatrician, and one at the ENT, and we'll do one more.  

They said his problem is a permanent hearing loss, and is not a mechanical issue. They tried bypassing using this special gear on his head during the testing and said it's neurological in nature.

He has trouble hearing the bottom 35% of the tones, but it is moderate, so it's not completely gone.  She said he probably won't need hearing aides.

They want to do EKG, CAT scan, bloodwork, which we will schedule after the test in two weeks.

I'm wondering: in your experience, when you find this sort of loss generally progressive in nature?  What are the odds that it will get worse?  If it stays the same it's not that bad. . . The doctor also said that there is a disorder called Jervell and Lange-Nielsen syndrome but he indicated that most kids with that are more profoundly deaf.

Guess what I'm asking is how much to worry. LOL

any information you have is appreciated.

ANSWER: Hi Christine,
At five years of age, even a mild hearing loss can cause problems with cognitive and language development. There are many syndromes that can have hearing loss as a symptom; is there a particular reason the ENT picked JLN? My advice is to continue with the tests and find out as much as possible.  As far as knowing if the loss is progressive, that too is hard to know without more information about the cause.  Once the tests are complete, and if a cause is found, then you can proceed with appropriate treatment.  Personally, even with a mild sensorineural hearing loss, I would think of fitting your child with hearing aids.
Hope this helps.
Harriet B. Jacobster, AuD
Board Certified in Audiology

---------- FOLLOW-UP ----------

QUESTION: I'm not sure why he picked JLN. . . I guess because we're not seeing other things that would make us think he has some of the other causes such as skin discoloration or Down's syndrome.  In your experience, does JLN generally only appear with more severe hearing loss?  That was what I found on the 'net.  Thanks for your help. Our audiologist didn't feel he'd need hearing aides, but we still need to learn more. His loss in his other ear is only slight so maybe that's why. We'll discuss all our options after we do the CT scan, etc etc.

Answer
Hi Christine,
JLN is not common and I personally have only seen one or two cases.  The profound hearing loss is present from birth.  I noticed that the doctor also wants to do an EKG...is there a problem with your son's heart as well?  That may account for your doctor thinking it was JLN since cardio problems are part of the syndrome. But, there are literally thousands of syndromes that have hearing loss as a symptom and there are even more "non-syndromic" causes for hearing loss.  Again, with an untreated, non-reversible sensorineural hearing loss in a 5 year old, there can be far-reaching consequences.  You may want to consider a Speech Language evaluation as well as a Cognitive one as well to determine his levels and need for intervention.  
Hope this helps.
Harriet B. Jacobster, AuD
Board Certified in Audiology