Re: Fixation or Fusion of Middle Ear Bones

[Guest guest]

,

It was weird to read your post. When Madelyn went for her last ABR the

audiologist told me that her's was sloped different than the normal way it is

suppose to slope. She didn't explain it to me but said her's was backwards or

reverse. It will be interesting to see what people respond back to you.

Elaine D.

[Guest guest]

There is not really a distinctive " look " to an audiogram of a kid with fused

middle ear bones. What you will see is gap between the air tests and the bone

tests (presuming that the conductive loss is worse than the sensineural loss, if

any). The gap is generally lower in the lower frequencies, and higher in the

upper frequencies. Patty's gap starts at about 35 dB at 250 hZ and increases to

50-55 dB at 6K.

Her fused bones were detached 21 months ago. We suspect they have refused, but

audiograms and cat scans don't reveal that conclusively, only surgery does.

We're passing on the surgery for now since she is hearing aided at 15 dB.

If you want to actually see what Patty's audiogram looked like before her

surgery, I could fax it to you -- e-mail me your fax # privately if you like.

Sheri

[Guest guest]

Sheri,

How did you find that here bones were fused to begin with. We suspect

that this may be our daughters problem but we have been told that the only

way to know is to go in and look. Could you please give me more information

about what you have been through and what happened with surgery. Did a CAT

scan or an MRI show anything? Or was it just the difference in the bone

conduction and the behavioral audiogram. did a tympanogram show anything?

Thanks

Sheri #2

[Guest guest]

In our case, it was fairly simple. The source of Patty's hearing loss is known

(Branchio-Oto-Renal Syndrome) which has about a 10 % incidence of fused and

misshappen middle ear bones. She did have a cat scan and many, many audiograms.

Everyone we've dealt with has told us that those can only indicate the

possibility of fusion, nothing short of surgery is conclusive. Since the cat

scan and audiograms indicated the possibility of fusion, and she has a syndrome

where fusion is common, we decided to proceed with the surgery.

We did her worst ear first. The surgery was scheduled for 2 hours and ended up

going almost 5 because of the mess they found when they got in there. Not only

were all the bones fused into one misshappen bone, the whole chunk had fused to

the back of her ear drum (which they had to completely replace). Patty lost

some of her sense of taste as a result of the surgery because one of her nerves

could not be saved.

Otosclerosis is a little different, I think -- this is more like an arthritis of

the middle ear bones, if I remember correctly. There is some good info on it at

http://www.med.umn.edu/otol/library/otoscler.htm

If the bones refuse, there is a procedure called a stapedectomy where they can

replace the stapes with a prosthetic. We're waiting until Patty is older to let

her decide if she wants to try this or not.

Sheri

[Guest guest]

Dear Sheri,

I have a few more questions if you don't mind. If I am getting too

personal let me know. What kind of hearing Loss does your daughter have?

And what are the other things other then fusion of middle ear bones that

occur in the syndrome that your daughter has? My husband and I are trying to

decide if we want to run the risk of going in surgically. It is a difficult

decision. my daughter has a moderate low to mid frequency loss but

is normal in the highs. My husband has the same type of loss but it is more

mild then moderate. They otologist said he's would describe his loss as

Otosclorisis but could not be sure unless they did surgery. He said that he

would wear aids before he would let anyone operate on him. He has also

started to complains of sensitive hearing in some frequencies.

Sheri

[Guest guest]

Sheri -

I too am interested in learning more about your daughter's situation as

that with my boys, the ENT is thinking their situation is otosclerosis,

which is all related to those little middle ear bones and how they may

or may not be moving. Thanks for sharing.

Sparks

Sdunnstern@... wrote:

> From: Sdunnstern@...

>

> Sheri,

> How did you find that here bones were fused to begin with. We

> suspect

> that this may be our daughters problem but we have been told that the

> only

> way to know is to go in and look. Could you please give me more

> information

> about what you have been through and what happened with surgery. Did

> a CAT

> scan or an MRI show anything? Or was it just the difference in the

> bone

> conduction and the behavioral audiogram. did a tympanogram show

> anything?

> Thanks

> Sheri #2

> -----------------------------------------------------------------------

>

> -----------------------------------------------------------------------

> All messages posted to this list are private and confidential. Each

> post is the intellectual property of the author and therefore subject

> to copyright restrictions.

[Guest guest]

Patty has a mixed loss -- the sensinerual component is mostly mid-range (1K to

4K) and is mild, but the conductive loss is across the board and adds an

additional 35-50 db which bumps her up to mod-severe in her right ear and

moderate in her left. BOR syndrome has a hodgepodge of other symptoms which

appear inconsistently even in folks who do test positive for the EYA1 gene.

Patty has unilateral microtia and canal stenosis (which means her right ear and

canal is about half the size of her left), and she has some kidney

abnormalities. Other common symptoms of BOR include " pits " (which look like

acne scars) on or around the ears, and fistulas (which I think look like bumps)

on the neck.

Our decision to have the surgery was easy because she already had to have

surgery to enlarge her ear canal, so they were " in the neighbourhood " .

Unfortunately, she has had just about every complication in the book. The canal

closed back up, the middle ear bones appear to have refused, she's all but

rejected the ear drum replacement, and she lost the chorda tympani nerve (at

least her facial nerve was unaffected). Except for the nerve, this all happened

over 6-21 months post surgery, the surgery was initially successful!! Rather

than re-do the sugery, we went for the aids, and we have been extremely pleased

with those results.

Sheri

[Guest guest]

Sheri,

Thanks for sharing, I appreciate the information along with the website

you sent.

Sparks

sbyrne1281@... wrote:

> From: sbyrne1281@...

>

>

> In our case, it was fairly simple. The source of Patty's hearing loss

> is known (Branchio-Oto-Renal Syndrome) which has about a 10 %

> incidence of fused and misshappen middle ear bones. She did have a

> cat scan and many, many audiograms. Everyone we've dealt with has

> told us that those can only indicate the possibility of fusion,

> nothing short of surgery is conclusive. Since the cat scan and

> audiograms indicated the possibility of fusion, and she has a syndrome

> where fusion is common, we decided to proceed with the surgery.

>

> We did her worst ear first. The surgery was scheduled for 2 hours and

> ended up going almost 5 because of the mess they found when they got

> in there. Not only were all the bones fused into one misshappen bone,

> the whole chunk had fused to the back of her ear drum (which they had

> to completely replace). Patty lost some of her sense of taste as a

> result of the surgery because one of her nerves could not be saved.

>

> Otosclerosis is a little different, I think -- this is more like an

> arthritis of the middle ear bones, if I remember correctly. There is

> some good info on it at

> http://www.med.umn.edu/otol/library/otoscler.htm

>

> If the bones refuse, there is a procedure called a stapedectomy where

> they can replace the stapes with a prosthetic. We're waiting until

> Patty is older to let her decide if she wants to try this or not.

>

> Sheri

>

> -----------------------------------------------------------------------

>

> -----------------------------------------------------------------------

> All messages posted to this list are private and confidential. Each

> post is the intellectual property of the author and therefore subject

> to copyright restrictions.