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>In my son's case, his CT scan showed enlarged vestibular aqueducts (along

>with Mondini malformations), which is also connected to progressive losses.

Dixie,

Petya too has enlarged vestibular aqueducts. At one point I was told that

they were a Mondini malformation. That is why they say Petya possibly has

Mondini. Sometime ago I was told this seems to be a seperate syndrome.

Does anyone have updated information on enlarged vestibular aqueducts? Were

you saying the Mondini is connected to progressive loss or the enlarged

vestibular aqueducts?

Barb

>Barb,

> I haven't had the bone conduction test done yet, but the hearing clinic

>wants to do it next time we go in ~ late September since the University is

on

>Summer break right now. What exactly is a bone conduction test anyway? My

>ENT said " I can tell you right now that she's going to have a hearing loss "

>but I was too shocked to ask him to explain himself. I'm so brave! Would

he

>say that because of calcium deposits on the eardrum that he said are

>permanent? Her eardrums had been retracted for quite a while because of

all

>the fluid behind her ears, maybe that's why he said that....who knows.

The bone conduction test tells you if there is a senso neural loss. It

bypasses the middle ear and thus the conductive component. This is a

permanent hearing loss. I am suprised that this was not done at the same

time as the other audiogram.

I don't have any experience with calcium deposits on the eardrum, perhaps

someone else knows.

Barb

>to her ears. Since she's only almost 3 years old, it's kind of hard to

tell

>when she's hearing the sounds during the audiogram because she loses

interest

>very quickly and then starts ignoring everybody. Are ABR's more accurate?

It shouldn't take that long to get an audiogram if you have someone

experienced in testing chidren. How is she responding to the testing? By

looking at the toys or dropping blocks in a bucket? What has the examiner

said about her reliability? Where are you when the testing is going on? I

am always on the side of the booth with the audiologist so I can read the

board as she is doing the testing.

Barb

>

>used to spend hours reading those magazines. They really helped me to

>I would imagine it to be really difficult to teach preschoolers, fourth and

>fifth graders all in one class. Even if you had " teacher of the year " , I

>cant imagine it being very effective. I'm sorry to hear there are no

>qualified interpreters there. If the cost of living werent so high there,

>I'd consider moving back there-its a beautiful place!

>Terry

I had a difficult time too imagining the effectiveness of a class like this.

My daughter is in a self-contained classroom for the hearing impaired. Last

year the class was one kindergarten, two second grade, 4 third grade, and 3

fifth grade. This is the best placement that Petya has ever had.

The kindergartner had a one on one aide. Two of the fifth graders and one

second grader were mainstreamed all day. The rest were in and out of the

room for varying times during the day. Ususally there were no more than 3

or 4 kids in the room at any one time. Petya gets lots of one on one

attention. Her education is truly individualized. I am more amazed at the

teacher's organizational abilities, getting kids to all the different places

that they go to during the day with an interperter and on time than I am

with her ability to teach to so many different levels. She was named the

county's teacher of the year last year. That was the first time a special

education teacher had recieved such an honor. So I would say this situation

can work quite well with the right teacher.

Barb

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My daughter has been diagnosed with Mondini malformation as well and enlarged

vestibular aqueducts. We are told she will eventually lose all hearing. Does

anyone

know if Mondini patients can be implanted successfully and if there is any

evidence

that progressive loss is inevitable? Thanks for your help.

gprogers wrote:

>

>

> >In my son's case, his CT scan showed enlarged vestibular aqueducts (along

> >with Mondini malformations), which is also connected to progressive losses.

>

> Dixie,

>

> Petya too has enlarged vestibular aqueducts. At one point I was told that

> they were a Mondini malformation. That is why they say Petya possibly has

> Mondini. Sometime ago I was told this seems to be a seperate syndrome.

> Does anyone have updated information on enlarged vestibular aqueducts? Were

> you saying the Mondini is connected to progressive loss or the enlarged

> vestibular aqueducts?

>

> Barb

>

> >Barb,

> > I haven't had the bone conduction test done yet, but the hearing clinic

> >wants to do it next time we go in ~ late September since the University is

> on

> >Summer break right now. What exactly is a bone conduction test anyway? My

> >ENT said " I can tell you right now that she's going to have a hearing loss "

> >but I was too shocked to ask him to explain himself. I'm so brave! Would

> he

> >say that because of calcium deposits on the eardrum that he said are

> >permanent? Her eardrums had been retracted for quite a while because of

> all

> >the fluid behind her ears, maybe that's why he said that....who knows.

>

> The bone conduction test tells you if there is a senso neural loss. It

> bypasses the middle ear and thus the conductive component. This is a

> permanent hearing loss. I am suprised that this was not done at the same

> time as the other audiogram.

>

> I don't have any experience with calcium deposits on the eardrum, perhaps

> someone else knows.

>

> Barb

>

> >to her ears. Since she's only almost 3 years old, it's kind of hard to

> tell

> >when she's hearing the sounds during the audiogram because she loses

> interest

> >very quickly and then starts ignoring everybody. Are ABR's more accurate?

>

> It shouldn't take that long to get an audiogram if you have someone

> experienced in testing chidren. How is she responding to the testing? By

> looking at the toys or dropping blocks in a bucket? What has the examiner

> said about her reliability? Where are you when the testing is going on? I

> am always on the side of the booth with the audiologist so I can read the

> board as she is doing the testing.

>

> Barb

>

> >

> >used to spend hours reading those magazines. They really helped me to

>

> >I would imagine it to be really difficult to teach preschoolers, fourth and

> >fifth graders all in one class. Even if you had " teacher of the year " , I

> >cant imagine it being very effective. I'm sorry to hear there are no

> >qualified interpreters there. If the cost of living werent so high there,

> >I'd consider moving back there-its a beautiful place!

> >Terry

>

> I had a difficult time too imagining the effectiveness of a class like this.

> My daughter is in a self-contained classroom for the hearing impaired. Last

> year the class was one kindergarten, two second grade, 4 third grade, and 3

> fifth grade. This is the best placement that Petya has ever had.

>

> The kindergartner had a one on one aide. Two of the fifth graders and one

> second grader were mainstreamed all day. The rest were in and out of the

> room for varying times during the day. Ususally there were no more than 3

> or 4 kids in the room at any one time. Petya gets lots of one on one

> attention. Her education is truly individualized. I am more amazed at the

> teacher's organizational abilities, getting kids to all the different places

> that they go to during the day with an interperter and on time than I am

> with her ability to teach to so many different levels. She was named the

> county's teacher of the year last year. That was the first time a special

> education teacher had recieved such an honor. So I would say this situation

> can work quite well with the right teacher.

>

> Barb

>

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

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Hi My son Robby has a mondini malformation and enlarged vestibular

aquaducts.He has the nucleus 24 and all of the electrodes were able to

be inserted.But they did have to deactivate some due to facial nerve

stimulation, but this could change.He was implanted in June and turned

on in July.He just recently started responding.He responds more now than

he ever did with hearing aids.If you do decide to go with the implant,

please be aware of the risk of a spinal fluid leak with the

surgery.People with mondini have a greater risk of this happening.It

happened to my son and he had one of the best surgeons in the country.He

was in intensive care for five days, but he pulled throgh fine with no

lasting complications.Good luck, and email e any time if you want to

talk.Also, please know that the risks with the surgery are very

minimal.My son just seems to have lots of luck and it's not always

good.LOL.

Sherri Porter

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In a message dated 08/16/1999 7:30:13 AM Pacific Daylight Time, syed@...

writes:

<< > It shouldn't take that long to get an audiogram if you have someone

> experienced in testing chidren. How is she responding to the testing? By

> looking at the toys or dropping blocks in a bucket? What has the examiner

> said about her reliability? Where are you when the testing is going on? I

> am always on the side of the booth with the audiologist so I can read the

> board as she is doing the testing. >>

They were trying to have her put pegs in a pegboard and watching her facial

expressions. They would ask her if she heard a birdie...when they played the

tone her eyes would light up and she's say " You hella a boo boo? " (You hear

a birdie?) so we would know she heard something. Other times she'd just say

" Not wuucking " because she didn't hear anything. I usually sit right behind

Lexi in the sound proof booth so I can help get her interesting in " playing "

if she loses interest too quickly.

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> My daughter has been diagnosed with Mondini malformation as well and

enlarged

> vestibular aqueducts. We are told she will eventually lose all hearing.

Does anyone

> know if Mondini patients can be implanted successfully and if there is any

evidence

> that progressive loss is inevitable? Thanks for your help.

Mondini most certainly be implanted. My son has mondini's and he has an

implant. !hat I described last week (the bone not forming correctly) often

goes along with Mondini's and this may be why you were told her loss would

be progressive.

From the British Journal of Audiology:

http://www.ent-news.com/vol6_3.dir/cur_lit.dir/article19.html

Mondini dysplasia is a congenital malformation of the inner ear. The purpose

of this study was to retrospectively review the audiological findings in 5

patients with Mondini dysplasia who were fitted with a Nucleus 22 cochlear

implant. There were 4 adult patients and 1 six year old girl. All subjects

had a severe/profound hearing loss. Sound field aided results were typically

around 60dB(A) at low frequencies and considerably worse at high

frequencies. Implanted thresholds were in the 30-40dB(A) range. These

results fall within the typical range obtained from other cochlear implant

patients. Speech tasks and environmental sound tests after surgery showed

significant improvement over pre-surgery results. The threshold of detection

for both speech and environmental sounds was found to be as good in Mondini

patients as in cochlear implant patients with no inner ear malformation.

These findings indicate that Mondini dysplasia is not a contra-indication

for multichannel cochlear implantation.

Kay

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Barb,

My understanding is that enlarged vestibular aqueducts are a now considered

a separate syndrome (EVA syndrome for short). The syndrome is seen in

persons without Mondini malformations as well as in persons with them.

Mondini malformations can be associated with progressive losses. EVAs are

also associated with progression of hearing loss, particularly after blows

to the head, which can cause sudden drops in hearing.

BTW, there are various types of cochlear malformations identified as

Mondini. Most, I think, involve fewer turns in the cochlea. In my sons

case, he has all of the turns, but the last turn is not partitioned.

Dixie

>>In my son's case, his CT scan showed enlarged vestibular aqueducts (along

>>with Mondini malformations), which is also connected to progressive losses.

>

>Dixie,

>

>Petya too has enlarged vestibular aqueducts. At one point I was told that

>they were a Mondini malformation. That is why they say Petya possibly has

>Mondini. Sometime ago I was told this seems to be a seperate syndrome.

>Does anyone have updated information on enlarged vestibular aqueducts? Were

>you saying the Mondini is connected to progressive loss or the enlarged

>vestibular aqueducts?

>

>Barb

>

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It depends on the type of malformation. In my son's case, his Mondini is

incomplete partitioning of the last turn of the cochlea, and when diagnosed,

the doctor said the good news is that he would be an excellent candidate for

a CI if his hearing drops. If your daughter's cochlea is malformed in such

a way that the cochlea does not have all the turns in it, it might be more

difficult.

From my reading, it appears that progressive loss is not inevitable, but

does happen in these cases. But I'm certainly not an expert...

Dixie

>

>

>My daughter has been diagnosed with Mondini malformation as well and enlarged

>vestibular aqueducts. We are told she will eventually lose all hearing.

Does anyone

>know if Mondini patients can be implanted successfully and if there is any

evidence

>that progressive loss is inevitable? Thanks for your help.

>

>

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Share on other sites

>My understanding is that enlarged vestibular aqueducts are a now considered

>a separate syndrome (EVA syndrome for short). The syndrome is seen in

>persons without Mondini malformations as well as in persons with them.

>Mondini malformations can be associated with progressive losses. EVAs are

>also associated with progression of hearing loss, particularly after blows

>to the head, which can cause sudden drops in hearing.

>

>BTW, there are various types of cochlear malformations identified as

>Mondini. Most, I think, involve fewer turns in the cochlea. In my sons

>case, he has all of the turns, but the last turn is not partitioned.

>

>Dixie

>>

Thanks for this info, Dixie. Our doctor at UVA wants to see Petya's scans.

Our next appointment is in January but the scans are in Washington state.

So I am in the process of having them sent out here. The report says the

cochlea is normally shaped but it does not say anything about the

partitions. Also it says that the vestibular aqueducts are slightly

enlarged. However I read somewhere that there is often a mistake made by

doctors on the interpretation of how enlarged they have to be to cause a

problem. I wish I could remember where I heard that. I will be looking

forward to having another opinion on her scans.

Barb

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