Profoundly deaf baby reacts to sounds, even voices?

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linzx08

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I wasn't sure where to put this since I'm new here.

My son was diagnosed profoundly deaf at 2 months old. He had absent OAEs and his ABRs showed absolutely nothing at any level. My husband insisted he could hear. Heck, we even had videos of him actually startling to loud noises with arms and legs flailing around and his eyes opening to a loud "hey". As most doctors would think, we were told we were in denial. We are not though... my husband's best friend is deaf, my aunt works at an institute for the deaf, and we are friends with many deaf people... It wasn't something "new" to us, unexpected yes, but nothing that after a few days we couldn't handle, but his actions did not match his tests.

His audiologists and speech therapists dismissed us until he got his hearing aids. Being profoundly deaf, he was to have no benefit and was a CI candidate (we were/will take this route, but he will also learn ASL so he has the best of both worlds). He blinked profusely with hearing aids on. They were turned down several times that day and we left with them being set for normal to moderately/severe sloping loss. He is currently 4 months old, and the last call was for him to not wear his hearing aids at all until he can do booth testing. In the time he had his aids on, he began to flinch more and more to noise and could wake from a clap across the room.

WITHOUT his hearing aids, he can wake to his name being said or the "bop" sound they do to test HA/CI. He blinks to every bop sound if said repeatedly. We have taken outside factors in consideration, such as movement, air, vibrations... and the reaction is still there. His last therapy session began preparing him for booth testing. She shook a series of shakers behind him including a maraco, a strip of light bells, a bottle of rocks, and he turned towards every single one of them.

He has had 3 ABRs by 2 different people at 2 different places, countless OAEs, normal tympanograms. Everything stays the same. Our audiologists and speech therapists told us they have never saw this happen or even heard of it happening. My husband and I have talked to several specialists and people in the deaf community and no one says this is even close to remotely possible. Both hearing and deaf people that we know tell us there is no way he is profound.

I just want to know what is going wrong. PLEASE if anyone knows anything about ABRs being wrong or having a reaction to even voices and being profoundly deaf, please help us. I want him to wear his aids if he needs them and I want him to learn sign if he needs it (well, he will learn that more than likely even if he has perfect hearing). Any theories or anything will be great because everyone has told us they can't tell us anything about him right now or have no idea what to do. He has no other issues besides his ABR/OAE readings, developmentally he is right on track!
 
I have heard of kids who tested as profound on ABR, but then proved to be more HOH.
Plus even profound kids may still have some residual hearing. Profound doesn't always mean deep profound.
I had an ABR as part of a research study. It indicated that I'm severe/profound when in fact my loss is more moderately severe (dipping to severe/profound in a couple of frequencies)
 
Adam's Momma was our member for quite a while here, and sought advice and her son attended deaf preschool.

She quit when later testing at a university hospital revealed that Adam was not actually deaf at all.

He had some other problems, but deafness was not one of them.
 
I am severe to profound deaf. (severe (nearly profound) in one ear, profound in the other.

Depending on how loud it is, and how deep the voice is, sometimes I can hear voices in the same room. But not distinctly.

I can hear it better with hearing aids, but again, not distinctly, unless I'm looking a person in the face (speechreading). Even then, I miss a lot of words.

Then there's also the definition of profound. Proufound starts at 90 db and goes down into the depths of nothing hearing anything at all. Some sounds I don't hear at all, some sounds I do. It all counts as profound.

Your child's hearing is different than another deaf person's. No two deaf people will be exactly the same in what they can hear.

Heck, I read somewhere that most culturally Deaf people do hear something.

But don't get the idea that it's anything like what hearing people hear.

Or like Bottesini said, there could be something else going on.

I just hope that if it is indeed deafness, that it will be accepted and accommodated by everyone.
 
Because of the Audiologist I see (who specializes in communication issues as well - I have APD), I actually have heard of quite a few infants and toddlers who've failed repeated ABRs and other hearing assessments - but react to sound.

A few have A.N. , Some apparently just process sound in a very atypical way that makes the tests either very inaccurate, or entirely useless.

I also know of at least one child who was determined to be bilaterally completely deaf, implanted (one side) with a CI, and then at age 3 found to have perfect hearing on the non implanted side and what was referred to as "significant residual hearing" on the implanted side.
 
When my Meniere's kicks in, I can hear someone with a bad muffler a mile away. Without Meniere's, nothing.
 
DeafBadger said:
I am severe to profound deaf. (severe (nearly profound) in one ear, profound in the other.

Depending on how loud it is, and how deep the voice is, sometimes I can hear voices in the same room. But not distinctly.

I can hear it better with hearing aids, but again, not distinctly, unless I'm looking a person in the face (speechreading). Even then, I miss a lot of words.

Then there's also the definition of profound. Proufound starts at 90 db and goes down into the depths of nothing hearing anything at all. Some sounds I don't hear at all, some sounds I do. It all counts as profound.

Your child's hearing is different than another deaf person's. No two deaf people will be exactly the same in what they can hear.

Heck, I read somewhere that most culturally Deaf people do hear something.

But don't get the idea that it's anything like what hearing people hear.

Or like Bottesini said, there could be something else going on.

I just hope that if it is indeed deafness, that it will be accepted and accommodated by everyone.
Click to expand...

Yes, very good post! I mean there IS a reason why even severe and profound kids weren't identified until we were toddlers........
 
Thank you all for your replies. I just saw them. We are still no where close to answers. We do some sign at home, some talking. I know different people have different loss even if categorized the same. He recently had his first MRI/CT scan and everything structural wise was there. He also had a booth test with reactions as low as 30dbs. He is only 6 months and the audiologists said she could see he wanted to turn to more sounds, but at 6 months he just isn't there yet.

I have heard of very few cases of an ABR being this drastically wrong. From what I've found, I've only read two or three like this. It is frustrating because absolutely no one can tell us if he can hear or if he can't. We've heard more than one audiologists tell us, "I'm not saying he can hear, but I'm not saying he can't." We are at one of the best places we could possibly be. It is not a place that pushes implants and hearing aids, and they don't push spoken language by itself. From the beginning, they have informed us of at least 5 or 6 communication methods from sign to cued speech to purely spoken language... all with or without CIs.

I just need to know which route we need to focus on more. So I want to add and ask this, have any one you had or know someone with profound ABRs, but reacts to ALL frequencies and sounds in the booth? Again the frequencies went as low as 30db (which is consider a "normal" hearing threshhold for the booth). All sounds were reacted to by 70db.
 
i understand sound taken in through mastoid bones,i hear our cat but is psycological because he hounding me,his pitch has changed if fact it hurting me,it making other people cringe so guess something in it,whatched documenarty about cats hearing Scale A on piano and some try to find key themselves(hmm) they said this could be break through in years to come for some deaf tuning in to the world scale A
 
Good for you! You are one of the few parents in this world willing to get a CI and teach them ASL! I'm sick of all the Audist bullshit about "CI are miracle cures! Durrrrr... ASL is gonna make them stupid or something!" Good thing you have made the choice to integrate them with our culture, while also doing what you think is best for them! However, I would wait on the CI until he/she is old enough to understand and have some sort of say in this. Imagine if you couldn't join the football or wrestling team because of a decision you parents made for you before you could even walk! Would you like that? I'm not saying CIs are bad, they are great (I'm probably the only one here who will admit that), but at least give them some form of a say before you go ahead and make a life changing decision for your child!
 
Again, the flaw of early detection/screening is that it really can't tell you WHAT the baby/profoundly/severely mentally disabled kid actually HEARS!
It's possible he is HOH, but is compensating very well.......Or maybe the ABR is wrong.
 
I had something like that where my parents didn't get an answer on the ABRs until I was finally confirmed with a mild/moderate loss *I thank CREC for that cause we would've been in your boat LOL*
 
linzx08 said:
I wasn't sure where to put this since I'm new here.

My son was diagnosed profoundly deaf at 2 months old. He had absent OAEs and his ABRs showed absolutely nothing at any level. My husband insisted he could hear. Heck, we even had videos of him actually startling to loud noises with arms and legs flailing around and his eyes opening to a loud "hey". As most doctors would think, we were told we were in denial. We are not though... my husband's best friend is deaf, my aunt works at an institute for the deaf, and we are friends with many deaf people... It wasn't something "new" to us, unexpected yes, but nothing that after a few days we couldn't handle, but his actions did not match his tests.

His audiologists and speech therapists dismissed us until he got his hearing aids. Being profoundly deaf, he was to have no benefit and was a CI candidate (we were/will take this route, but he will also learn ASL so he has the best of both worlds). He blinked profusely with hearing aids on. They were turned down several times that day and we left with them being set for normal to moderately/severe sloping loss. He is currently 4 months old, and the last call was for him to not wear his hearing aids at all until he can do booth testing. In the time he had his aids on, he began to flinch more and more to noise and could wake from a clap across the room.

WITHOUT his hearing aids, he can wake to his name being said or the "bop" sound they do to test HA/CI. He blinks to every bop sound if said repeatedly. We have taken outside factors in consideration, such as movement, air, vibrations... and the reaction is still there. His last therapy session began preparing him for booth testing. She shook a series of shakers behind him including a maraco, a strip of light bells, a bottle of rocks, and he turned towards every single one of them.

He has had 3 ABRs by 2 different people at 2 different places, countless OAEs, normal tympanograms. Everything stays the same. Our audiologists and speech therapists told us they have never saw this happen or even heard of it happening. My husband and I have talked to several specialists and people in the deaf community and no one says this is even close to remotely possible. Both hearing and deaf people that we know tell us there is no way he is profound.

I just want to know what is going wrong. PLEASE if anyone knows anything about ABRs being wrong or having a reaction to even voices and being profoundly deaf, please help us. I want him to wear his aids if he needs them and I want him to learn sign if he needs it (well, he will learn that more than likely even if he has perfect hearing). Any theories or anything will be great because everyone has told us they can't tell us anything about him right now or have no idea what to do. He has no other issues besides his ABR/OAE readings, developmentally he is right on track!
Click to expand...

Given the situation, and his age, I would tread lightly and not rush to surgery until you know exactly what he's hearing and wait a little longer until someone has answers for you so you know what you're dealing with, and what his range of loss is, on paper...

Laura
 
Thank you all! We are currently NOT eligible for CIs anymore due to his booth testing. I'm at a Children's Hospital and even if he had no response, we would have to go through a 6 month hearing aid trial to be sure he would not benefit from, if we choose too. Our HEAR Center offers therapy sessions for not only speech and hearing, but for cued speech, SEE, and ASL, as well. CIs are not something they throw at us, but it is presented as an option.

He will receive a CI before he is 2 IF it is proven his hearing loss is severe/profound. The reason we have chosen that is that is the optimal window. When he's older, if he chooses not to live with it, that is fine. We will all know ASL/SEE and it will be easy to transition.

As far as sports go, we're from the south so football is a BIG deal. We have researched and they now make helmets specifically for CIs. I know a teenager who has had one since he 2, just got his second at 16. He is our states current top baseball recruit and the Yankees have scouted him. Having a CI does not limit children, neither does being deaf. My son is still deaf at the end of the day regardless of his CI, but I do choose to give him both opportunities as early as possible.

I currently have a degree in elementary education, and for a long time I wasn't sure what I wanted to further my degree in. My husband and I decided I would go to graduate school for teaching children with hearing impairments. I figure either way, it will be great to have knowledge since I do live in an area with not only a school for the deaf, but a public school with kids with CIs. I would love to teach not only verbally, but also be able to sign the lesson. I definitely feel if my son's ABR was wrong somehow (they don't work for 8% of the population... I called the company, this is what I was told), I was led to choose this degree to help children whose parent do not give them access to all of the options.
 
Bottesini said:
Adam's Momma was our member for quite a while here, and sought advice and her son attended deaf preschool.

She quit when later testing at a university hospital revealed that Adam was not actually deaf at all.

He had some other problems, but deafness was not one of them.
Click to expand...

I still speak to Adam's Momma. He still has hearing loss but nowhere near as much as originally thought.
 
I tested as profoundly deaf as a young child but am not deaf at all. I am autistic so I respond to sounds and hear sounds differently but definitely not deaf....
 
Lissa said:
I still speak to Adam's Momma. He still has hearing loss but nowhere near as much as originally thought.
Click to expand...

Refresh my memory on Adam's Momma? But yeah......I think even with ABR it can be really hard to tell how bad hearing issues are. There is STILL time thou. I actually think that the optiminal time for implantation is based on kids who absoloutly have very very poor residual hearing. There's still time...heck kids just 15 years ago still developed spoken language with CIs...Not to mention that severe and profound kids still respond decently with HAs........there's still time...don't panic.
 
That is why I don't approved of having CI for surgery for very young children like babies or toddlers. It is a lot better to wait until it is definitely for sure that your child is hearing/hard of hearing or deaf. I don't see the rush to put hearing aid or CI on them so early. If he is hard of hearing or deaf, then all you have to do is accept him for his deafness. The medical doctors and surgeons had to jump into making the babies get CIs so early. What is the rush? Give the babies and toddlers a chance to go to their babyhood. Hearing is not important when it come to hard of hearing or deafness. It is not the end of the world for goodness sake. Just keep on signing baby signs or ASL. If he can hear without the aids, then you don't have to worry about that. Mistakes happen. **shaking my head** :(
 
Is the above various comments- suggest one be very accurate in the condition of DEAFness. How can one "switch back and forth" from actual/real DEAFness to "hard of hearing/profound loss?

I don't think it is "possible".
 
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