[tri-med] Re: Ears to hear and all that

----- Original Message ----- 
From: "Kraig Warnemuende"
>> except that there seems to be hard plate behind the ear drums.

Not sure if this is the same as Alex - Alex has a "lump" behind his ear drum
and that you can see when you look at his ear drum - in actual fact its the
bones of his ear (confirmed with CT scans and an ENT who went in and had a
look to see hwta the lump was). His ear drum is slightly misplaced and the
bones (stapes) are deformed. Normally you can't see the bones behind the ear
drum - but with Alex they are misplaced in such a way that they rest against
the ear drum and you can see it as a "lump". It contributes to his hearing
loss.

>>Also, I think it was Karen who
> mentioned "glue ear" recently and I was wondering what that was.

The ears have eustachian tubes which allows fluid from the middle ear to
drain into the throat. When the eustachian tubes don't drain correctly (very
common in small children even those with no trisomy) the fluid builds up
inside the ear. The fluid build up happens a lot when they have colds or get
ear infections. Over time it drains some, but not completely and so just
gets "thick" like glue (hence the term glue ear). If it gets left it can set
like concrete and actually cause permanent, irreversible hearing loss (of
the conductive variety - this Alex also has).

When there is fluid in the ear it causes deafness - its like hearing under
water, it often also causes "crackling" in hearing as well (I suffered from
glue ear as an adult because of allergies - its like someone crackling a
chip packet in your ear all the time - annoying big time). In our kids
because of low tone, small ear and oral structures, poor eating etc etc the
eustachian tubes often don't drain properly so they are predisposed to
developing glue ear.

Temporary fluid in the ear isn't a problem - but when it occurs over a long
time it causes problems with the fluid going to glue (glue ear) and results
in hearing loss and delayed speech, and these may be permanent.

You often can't see glue ear just by looking at the ear - its behind the ear
drum and its not necessarily infected. A common misconception is that glue
ear causes ear infections - in reality ear infections cause the fluid to
build up quicker than it can drain away. The fluid can get infected, but you
can have fluid in the ear with no infection. An ear infection makes the drum
look red, but glue ear or chronic fluid in the ear doesn't effect the look
of the ear drum, only the movement of the ear drum.

Alex rarely got ear infections - but he had very chronic glue ear due to low
tone and oral motor issues (not being able to suck and swallow). Alex's ear
infections usually came from reflux (when babies throw up sometimes food is
forced back UP the eustachian tubes into the middle ear) When this happens
it almost always sets up an ear infection. Alex didn't vomit often (he had
silent reflux) - but when he did I could guarantee an ear infection by
morning - once he got the fundo all ear infections stopped.

To diagnose glue ear they do what they call a tympanogram (sp?) (tymps is
the slang). With this they place a small probe in the ear and it bounces a
sound off the ear drum and measures the reflection back. Flat tymps (a flat
line on the graph) means that the ear drum isn't moving so there is probably
fluid behind the ear drum. In normal tymps the ear drums are flexible and
the result on the graph is a "hill" or curve.

The problem with tymps in our kids is that because they have small ear
canals, curvy ear canals and or misplaced ear drums (that is not completely
centred) tymps often bounce off the ear canal, not the ear drum - which
gives you a false tymp result. Alex always got normal looking tymps when he
was little because of this (he actually has very large ear canals unlike
most of our kids but they are VERY curvy) when in fact he had massive glue
ear. As a result by the time the ENT got in there the damage was permanent.

An ENT can also detect fluid in the ear by using a special auroscope (sp?
again) - its a typical thing that they look in the ear with but it has a
bulb on it so that they can squirt a little bit of air in as well and watch
and see if the ear drum moves. However a lot of our kids have such small ear
canals that they can't even get an auroscope in. They can however use a
special microscope in some cases to get around this.

Treatment for glue ear can take many forms - conservative treatment means
leaving it be and simply using hearing aids to compensate for the hearing
loss. Using anti-histamines or drugs like bisovlin over a long period of
time to dry out the fluid, or more aggressively putting in vent tubes
(called grommets in some countries). Beware I am not a fan of vent tubes -
they caused Alex more problems than they were worth!!!

Vent tubes are small bits of plastic that look like a very miniature cotton
reel. They are set in the ear drum to allow fluid to come back out. They
also allow water in so usually you get told not to allow water in their ears
so precautions need to be taken with hair washing, bathing etc - modern
thought is that clean water is OK though, just not soapy or pool water.

Vent tubes allow the fluid to drain out through the ear drum rather than
just through the eustachian tube. They are usually temporary, to give the
eustachian tubes time to develop. But with our kids they can need repeated
sets (one set typically last 6 months to 18 months depends on how fast our
kids grow) The problem with repeated sets is that the tube placement itself
can cause scarring of the ear drum, which means that the ear drum doesn't
move properly, and that in itself causes a hearing loss. In some kids (eg
Devon) they can leave permanent holes in the ear drums.

When Alex had vent tubes he actually got more infections than when he didn't
have them. And they were putrid ear infections - he would have masses of
putrid smelling gunk pour out of his ear and down his neck, and I mean it
STANK. When a child has an ear infection with vent tubes they can't wear
hearing aides, and so his development was worse with the vent tubes than
without. I will admit that a big part of the problem was probably the fact
that Alex was allegic to the vent tubes, well allergic to the tubes in as
much as he is allergic to latex and the surgeon used latex gloves when
placing the tubes leaving some residue on the tubes which he constantly
reacted too.

It was all these putrid ear infections that actually led us to the latex
allergy diagnosis.

Alex also developed granulation around the vent tubes which meant that his
ears were constantly bleeding which scared a lot of people to see. I made
the ENT remove the vent tubes after about 12 months. Normally the tubes just
fall out, but I wouldn't wait.

Prof Gibson placed Alex's vent tubes when he did a hearing test called an
electrocohleography - a special hearing test where they place the electrodes
directly on the cochlear and then measure the waves like in an ABR to see if
the hearing loss is conductive (related to the middle ear) or neural
(realted to nerve conduction past the middle ear). Its supposed to be the
gold standard of hearing tests - but its also not common because they
actually have to go into the ear to do it, so it requires surgery to do the
hearing test. It was done with Alex because of inconsistent results with
other hearing tests and because we knew he needed grommets so it was done at
the same time as the grommet placement. When Prof Gibson did the echog there
was so much glue that it almost invalidated the test. He physically removed
a lot of it, but he also admitted that there was permanent damage as a lot
had set like concrete. (which prevents the bones from moving to conduct
sound)

Anyway - thats a looong explanation. The reality is that our kids are
predisposed to fluid in the ear because of the small ears, reflux and low
tone associated with the T-18. This can cause progressive hearing loss over
time. As a result our kids should have regular hearing tests (every 6 to 12
months) to monitor for any hearing loss - even if they have a normal result
when they are small. Similarly if they get an abnormal result they should
also have regular hearing tests to monitor for any improvement in the
hearing loss. Fluid build up or the development of glue is not something
that can be seen visually, and with our kids its often not possible to see
the ear drum anyway because of the small ear canals. Similarly tymps are not
always reliable - so a hearing test is better (but even these can be
innaccurate). The big problem with our kids is that they often need ABR's -
which usually mean sedation, so its a catch 22. They can be done without
sedation though and you can insist on this. Alex's ABR's were always done
without sedation (I would just get him to go to sleep for the test by
keeping him up all night the night before) - but it requires much
co-operation with the audiologist and time (we often only got one ear done
at a time).

I also add to all this that I am very much a fan of aiding kids - Alex
wasn't aided until he was 2 and even then he was aided very conservatively.
I believe that his hearing issues have been a huge factor in his
developmental delays. Recent tests support this (the hearing in one ear has
not developed past infant type hearing - that is it has not matured). Its
only recently (like the last 3 months) because of the results of these
latest very complex tests that they have upped the power of his aides, but
we have lost so much time, and also allowed many of these possibly
reversible problems to become permanent. Regrets - yes. Mothers instinct has
been right all along and I should have been more aggressive with the idiots
he had as audiologists (long history of battle there). I miss our ENT who
always said that a doctor was in big trouble, and shouldn't be called a
doctor, if they didn't listen to the parents.

"Love's the only house big enough for all the pain in the world"
Martina McBride

Keep Looking For Rainbows!!
Karen, Mum to Alex (8 years, T-18 Mosaic)

                  Building ___ooOOoo__ Rainbows
                       www.trisomyonline.org
                  Families Helping Families On-line

Other related posts: