4 year old lost hearing and speech but passed BAER

[Maukamakai]

My son had tubes placed following 4+ months of fluid after OM at age 3.5. Hearing/health returned to normal. Very concise and articulate language skills from an early age. 6 months later hearing loss recurred with long-term congestion and softening speech. ENT confirmed tubes had fallen out and should be replaced with long-term (T) tubes and adenoidectomy. This was done in January 09 with immediate recovery in health, breathing, congestion, etc.. Hearing, however seemed to worsen dramatically. Since follow-up in late Jan, we've had multiple tympanometry tests which he passes but fails behavioral response tests. MRI was clear, recent BAER was normal. Neuorology work-up indicates unknown hearing loss with no other neuro/developmental/behavioral issues. Basically we have a perfectly healthy, normal child who just can't hear or speak anymore. Gross/fine motor skills excelelnt, academic skills excellent, great balance. He has embraced sign language as his primary form of communication but still no explanation of why he quite rapidly lost his residual hearing and subsequently lost all speech. He still uses his voice to make sounds as a means of getting a hearing person's attention but is unable to articulate any words.

ANY IDEAS?!?! This has been beyond frustruating. We've heard alot of what his condition isn't but no one can tell us what it is. Could his tubes be distorting the sound enough that he can actually hear the sounds but can't make out what they are? APD has been ruled out. Would it be worth removing the tubes? We've requested hearing aids but the audiologists don't feel that he will respond to them, which is reasonable. We're going to try anyway but would love to hear ANY thoughts.

[ENTMD]

That is quite the story.  Very sorry.  It sounds like they have been doing a very thorough job in working this up.  The tubes themselves are not likely to be the problem here (in or out).  They may be helping him in terms of preventing otitis (and the discomfort/pain that can accompany it) and they would be worth leaving if for that reason alone.  There is no reason to think that your decisions about his treatment before this happened had anything to do with it.  It sounds like you did everything right.

Very little is known about this condition, as you already know.  Hearing aids and implants won't help, since this is typically not a "volume" issue; it's a processing issue.  Somehow, something in the auditory nerve and/or hearing centers in the brain are not working correctly.  Sorry that I don't have any new ideas to offer.

[Wear/a/Jimmy]

From an Audiologist perspective:


I would recomend more testing. Specifically:

Tympanometry
OAE
ASSR

an ABR/BAER is usually done with a "click" stimulus that has a band with of 2-4K. In other words an ABR does not show an accurate representation of your childs hearing ability. It is like measuring a persons ankle to make a pair of pants. The ankle measurement only tells a fraction of the story.

Another good point to mention is that ASSR testing can be done under sedation.

Once the ASSR test are done, and once all the audiometric data is compiled then it can be determined if the hearing loss is cochlear based or not.

Right now, the results suggests something called cochlear neuropathy or a neurologically based issue. That said, there have been cochlear implants done for children with cochlear neuropathies with good results.  (I can dig up the research on that if you would like)

Also, who did the testing? Was it an Audiologist or a techy. Because in my experience (over 10 years) I can tell you it makes a difference.

But I do agree with the MD, I can not see any relationship between periodic conductive hearing losses/OME leading to your childs condition.