Otoscelerosis diagnosis?

[anewman]

My daughter is 5 years old and her specialist just diagnosed her with otosclersois. He said he has never heard of a child having this disease. He said it is rare even in teenage years. I am wondering what kid of progression this is going to make and what I need to look for through the years?

[Reidan]

I too have Otosclerosis. I knew I had a hearing loss since my early teens. And even younger I remember teachers discussing it. I am now 44 and just started wearing hearing aids. So my hearing loss progression was very slow.

But, no two people have the exact same results from Otoscelerosis. I would be tempted to seek a 2nd, and even a 3rd, opinion. Try to find an ENT who specializes in children.

[Um bongo]

Originally Posted by Reidan

I too have Otosclerosis. I knew I had a hearing loss since my early teens. And even younger I remember teachers discussing it. I am now 44 and just started wearing hearing aids. So my hearing loss progression was very slow.

But, no two people have the exact same results from Otoscelerosis. I would be tempted to seek a 2nd, and even a 3rd, opinion. Try to find an ENT who specializes in children.

Your loss isn't a 'classic' shape for it, but there are some losses where it is positively indicated. With your air-bone gap, I take it they have reviewed all the obvious stuff.....

Come to think of it I know a UK dispenser with that loss, I'll see if I can find out what the suspect source is.

[Reidan]

Um Bongo, I saw three different ENT's about it over more than 20 years. Even had a Stapedotomy 2 years ago to try and correct it. That was a failure and offered no improvement. But thankfully did not make my hearing worse.

As you said my hearing loss is not typicall. I would like to hear what your associate has to say.



Anewman, has the specialist recommended surgery to correct it? Or are they recommending going straight to hearing aids?

The surgery to correct Otosclerosis is to replace the Stapes bone on the inner ear with a titanium and ceramic implant. I would suspect that the surgery would need to be done after the person has reached adult hood as the titanium implant will not grow like the living tissue of the bone. I have no knowledge to back this up, just speculation. But that speculation is what makes me ask what the doctors have recommended.

Do you have your daughters hearing test results? If you can post them here the Audiologist on this site, such as Um Bongo, can review the numbers and offer some feed back.

You asked what to expect for her over the years. As I said in my previous post. All users have individual cases, so my experiences may or may not reflect your daughters experience to date, or what she will face in the future. I will just tell you what mine has been like.

My personal experience has not been "bad" as I do have a mild case of Otosclerosis. It took me over 30 years to progress to the point where I needed hearing aids. I did not wake up one morning or have some traumatic experience that resulted in my hearing loss. To me things always seemed normal. My hearing was what my brain was used too. So in some ways it was hard for me to even accept that I had a hearing loss.

For me the realization that my hearing had degraded to the point where I finally needed to do something was not any single thing, but a collection of them that finally added up. Not being able to hear in business meetings. Having to turn the TV up so loud that it was uncomfortable for others to be in the same room. Not understanding conversations correctly. Etc. I did resist longer than I should have. And I did go through surgery to try and correct it before I gave up and got hearing aids. That ties back to what I said that to me my hearing seemed normal.


As for her being so young to be diagnosed with Otosclerosis. I too was diagnosed with a hearing loss when I was young. I do not remember the exact age. But I do remember that my first hearing test was done while I was in elementary school. They put on a pair of headphones and did the tone test. The results were that I had a very mild hearing loss.

Not proud to admit it, but my parents did nothing about it. I was never taken to see any doctors at that age for a professional evaluation. I guess they just assumed that since I was not suffering and was able to talk they did not need to take any action. So I am glad to see that you are taking your daughters diagnosis seriously.

[anewman]

at this point because she is so young they are recommending hearing aids/ the loss is abouy 50% and is only in the right ear. There is alot of genetics working against her. Alot of my husbands side has otosclerosist

[james22]

Otosclerosis can lead to conductive or sensorineural hearing problems. The main type of listening to reduction in otosclerosis is conductive hearing problems (CHL) where by sounds get to the ear drum but they are incompletely transferred through the ossicular chain in the center ear, and therefore partly neglect to get to the inner ear (cochlea).

[Overoaked]

I recommend that you investigate the possibility starting your daughter on fluoride supplements to potentially reduce the rate of otoscelerosis progression. Very inportant: Note that I said "investigate the possibility."

Do your own research and make your own decision. You can start by reading some fluoride discussion threads on this forum. The most important step will be to discuss this possibility with an ear-specialist MD (not just an audiologist), preferrably one who does stapedectomy (Sp?) surgery.

I'm a retired-age man with severe inherited-genetic otoscelerosis, which started affecting my hearing about 30 years ago. I've had three stapedectomy operations and wear two HAs. About six years ago, my MD (who is an internationally recognized ear surgeon) recommend Calcium Fluoride, which I have been taking even though I'm generally a skeptic regarding supplements and their claimed benefits.

My guess is that fluoride will be of little benefit for me at my age, but it might benefit your daughter because she has so many years in front of her.

[lohearn]

Originally Posted by anewman

My daughter is 5 years old and her specialist just diagnosed her with otosclersois. He said he has never heard of a child having this disease. He said it is rare even in teenage years. I am wondering what kid of progression this is going to make and what I need to look for through the years?

My son was diagnosed with otosclerosis at age 12 or 13. We noticed probably as early as age 7 or 8 a weird behavior he had. We live next a boat ramp with a decent sized parking lot. In the off season when few cars are there the kids use the parking lot to play in. It was either the first or second autumn we lived in the house, making my son either 7 or 8, when I first noticed what he did when we called for him.

I would stand at the end of my driveway and call his name, he would hear his name being called and he would rotate in a circle to look for the source of the sound. When he saw me at the end of the driveway he would come home. If he was in the neighborhood or out of sight and I called for him, he would arrive home and ask "did you call me?' since his friends would tell him "your mom wants you". One day I was talking to my neighbor in her yard, I saw my son down the street and called to him. he looked towards our driveway and I wasn't there, he looked confused. I called to him again, he started to look around until he spotted me in the neighbors yard.

Because his right ear works perfectly, he could hear me when I called him. But, because his left ear is bad ear he, like your daughter, no longer has stereo hearing and can't pinpoint where the sound is coming from. He had a hearing aid, wore it off and on until one day he said "it's a hassle and doesn't really help me". He did OK with his hearing in school, he would ask for a seat on the left side of the classroom, so his bad ear would be toward the wall and his right ear faced into the classroom.

He lost his hearing aid, thank goodness it came with one free replacement. The replacement went swimming one day, it dried out and still worked. He always complained that it sounded "funny", not like how his right ear heard things. At this time my son is now 18 and does not wear a hearing aid. He's become very good at compensating for his hearing loss. The progression of his otosclerosis has been very slow, at one point there was almost no change in his audiogram over a 4 year period. He is due to go have his hearing tested again, it's been 18 months for him. He says he has noticed a change.

I don't have otosclerosis, but I do have a one sided hearing loss. I'm not sure what your daughter's audiogram looks like, but you can see mine in my signature. I lost my hearing suddenly when I had an accident in January 2011. At first I struggled, a lot, to get by day to day. Now, after 18 months, I have adapted quite nicely and most people I meet for the first time have absolutely no idea that I have any hearing issue.

How does your daughter get along? Does she seem to have difficulty in day to day life? I would get a second opinion, preferably as someone else said with a pediatric ENT. Honestly, depending on her audiogram I would not rush to get her a hearing aid just yet. There are so many factors to consider before taking that step. In hindsight, I wish I hadn't laid out the funds for my son's hearing aid. I know she's young so she doesn't really "get it" so to speak. Observe her, especially in social situations to see how she does communicating with other kids. Educate her, give her the knowledge that she has an ear that deosn't work right. To her it's "normal", she knows nothing else at this point. Kids are amazingly adaptable. Good luck!

[Suzanne]

I am 56 years old. My hearing has been declining for many years. I took care of my mother (with alzheimer's) for the past ten years and so i put off doing anything until now. Anyway, my recent hearing test, of which I do not understand much of is as follows:

250 500 1k 2k 3k 4k 6k 8k

RT 65 65 60 55 50 55 50 35
LT 55 60 55 50 50 55 40 35

SRT: BC: unaided discrimination
RT 55 75 @ 2k RT 92% 80 db
LT 50 75 @ 2k LT 92% 75 db

Tympanometry and acoustic reflex tests
RT / LT
ECV: 1.24ml / 1.28
MEP: na / -10
SC: 1.0 / 0.26
GRAD: 1.0 / 0.48
TW: 1.0 / 110

Results: SAL test to estimate air-bone gaps because of masking dilemma. Audiometry shows bilateral mixed hearing loss. Tympanometry shows no mobility in the right ear and low mobility in the left ear.

Acoustic responses are absent. Word recognition is good.

I currently have a hearing aid on my better ear (at my request while I waited 2+ months to see ENT). Audiologist will give one for other ear if too much time lapses betw seeing the referral doctor. As she clearly can tell I have difficulty hearing in the "real" world.
ENT said hearing aid and or surgery but referred me to a Doctor who does the recommended surgery. My brother has otosclerosis and they are leaning in that direction. Based on my test result do I in fact appear a likely candidate for this surgery?