There are some controversy as to whether Oto
sclerosis can produce a sensory neural
hearing impairment in this
presentation.
We hope to show that indeed
can produce a sensory neural
hearing loss,
that the diagnosis can be made and that
there is treatment that will prevent the progression
of the century neural component of the law.
This first slide is of a 58 year old
woman who first began to develop her
hearing loss at the age of 28.
She had a family history of hearing loss.
You can see that the otis sclerosis surrounds the
cochlear.
This is her audio graham.
Shortly before death,
there's no hearing in the left ear and almost
total loss of hearing in the right ear.
Was the otis sclerosis,
the cause of the century neural hearing loss?
I believe it was.
This is an Otis sclerotic lesion adjacent
to the organ of corti.
In this particular case,
we see that the spiral ligament and the organ of
corti appear normal.
However,
this patient had a 40 decibel
bone conduction threshold.
The question is,
did the otis sclerosis produce the sensory neural hearing
loss and in this case,
how
further evidence that otis sclerosis can produce
sensory neural hearing loss.
Is illustrated by this slide,
the upper columns indicate the bone
construction threshold above each
ear.
The lower columns indicate the area of the
spiral ligament involved by otis
sclerosis.
The limits of the bone conduction,
oscillator on the audio meter.
Are 70 decibels.
Hence,
we could not measure that above that level.
This is the uh case that I
just showed you of the massive otis
sclerosis involving the cochlear and
notice the amount of Otis sclerosis
relative to the other cases.
As the amount of otis sclerosis involving the
spiral ligament increases,
the bone conduction threshold increases
in order to rule out the possibility of presby
acoustics accounting for the loss in this group of
patients.
I plotted the age from 30 to
83 on the bottom of the
graph,
on the top is the degree of sensory neural
hearing loss.
And again,
this is the patient with the massive otis sclerosis,
but we can see there is no regularity to the
progression of the hearing loss.
This indicates to me that in these cases the otis
sclerosis was responsible for the sensory
neuron loss.
In most cases,
taking the same group of patients and Plotting the
two Ears separately,
each column representing a pair
of ears.
We see that where the otis sclerosis was
greater at the area of the spiral Ligament,
one in 1 ear,
the amount of sensory neural hearing loss was greater.
with the exception of two cases.
In those individuals who had large amounts of
otis sclerosis,
and in those two cases the difference of the amount of
Otis sclerosis was not too great.
This is a graph of 36
patients who had unilateral clinical older
sclerosis,
the height of the column represents the difference in the
bone conduction thresholds in the air
with the auto sclerosis and without
they are arranged along the bottom of
the chart to show the ages of the
patient and we see there is no real relationship
between the age and the degree of the difference of
the bone conduction thresholds and the two ears.
But rather that it is apparently Due to
the fact that one ear has Otis sclerosis and the other
does not.
This is an audio gram of a patient who had
unilateral clinical otis sclerosis and a
unilateral sensory neural hearing loss.
This is the over window of the area with the
clinical otis sclerosis.
After a state appendectomy,
we can see the over window filled with a gel foam
membrane and an auto sclerotic focus here
near but not involving the cochlear.
However,
in a different portion of the cochlear,
we can see that the focus does involve the area of the
spiral ligament.
This is the other ear of the same patient,
This is the staples foot plate and we see no otis
sclerosis adjacent to it.
However,
in the coakley a there was a rather
large auto sclerotic focus.
This would indicate to me the possibility that this
is one here in which the sensory neural hearing
loss was due to sclerosis
Because of cases such as this and there are three
in our temporal bone collection.
We have suspected that there are patients with a purely
sensory neural hearing loss without evidence of
clinical Oto sclerosis,
but possibly the losses due to cochlear otis
sclerosis.
We have examined 140 patients
who were suspected of having cochlear otis
sclerosis by polly tomography and found that
half of them showed evidence of possible
cochlear otis sclerosis.
We will discuss this shortly.
This audio graham shows the typical audio
metric findings in this group of patients.
That is the type to Bekasi a high
sissy score,
low tone decay and relatively good
discrimination scores.
This is the audio gram of a patient with a
unilateral clinical otis sclerosis.
This is the bone conduction in the affected
ear and the air conduction in the
left ear.
The air and bone were superimposed.
This is the temporal bone of the ear.
With the mixed hearing loss.
We see three distinct fosse of otis sclerosis,
one in the oval window and involving the endoscopy um
of the cochlea,
another adjacent to the internal carotid
artery and the third involving the
internal auditory canal.
In the other air.
There are no otis sclerotic fosse presents,
although both Ears had a sensory neural hearing
loss.
The ear with the Otis sclerosis had a greater
loss and we were unable to measure
2000 cycles because of the limits of the
bone conduction oscillator.
This is a slide again showing an auto
sclerotic focus adjacent to the spiral
ligament but apparently a normal appearing
organ of corti.
Why does this patient have a sensory neural hearing
loss,
which the patient did have a threshold of 50
decibels savants and Brett
low and France have demonstrated license
um at the advancing edge of the Oto sclerotic
focus.
This is what they feel causes the normal bone to
be absorbed and replaced by the Oto
sclerotic focus.
These license soames excrete Osti elliptic
enzymes.
It's conceivable that when these enzymes are liberated
into the inner ear spaces,
they can be a toxic influence
on the organ of corti in interfere with its
normal metabolism.
It's always been strange to me that otis
sclerosis could replace normal bone without
advancing osteoclasts.
However,
as we'll see,
they always occur in the center of the lesion rather
than in the advancing edge.
So this is one of the possible causes of the
sensory neural hearing loss found with
cochlear.
Otis sclerosis.
Rudy many years ago described
vascular shunts between the Oto sclerotic
focus and the inner ear.
He felt that this caused a venous stasis
of the circulation in the inner ear,
a build up of metabolites in the inner ear
fluids,
and this could have caused a sensory neural hearing loss
without evidence of damage to the hair cells.
If circulation is impaired to any organ,
the pathologist tell us that lamelo bone
can be laid down in that organ,
for instance,
in an ectopic pregnancy,
gone to brickell in the lungs etcetera.
Here we see a shunt between the inner ear and
the Oto sclerotic focus and the otis
sclerosis goes to this point this is
lamelo bone that has been laid down within the
lumen of the cochlear,
which I feel is probably the end result
of hypoxia or anoxia of
the cochlear.
This is the anterior portion of the
cochlea of a patient with clinical otis sclerosis
and a rather severe sensory neural hearing loss.
We see a normal spiral ligament,
An organ of corti.
Mystery of vascular Harris appears normal.
This is the post here half of the same
cochlear with a large auto sclerotic
focus adjacent to it.
It appears to be a very active focus as it's
quite cellular.
We see a number of abnormalities here.
The spiral ligament is highly ionized
and in fact classified in this area,
possibly due to hypoxia as a result
of the otis sclerotic focus,
I feel that perhaps Rudy.
She can also explain
changes such as this by shunning the arterial
blood from the coakley a.
To the O.
To sclerotic focus,
much like an aneurysm,
as we know,
an aneurysm or a large hemangioma in a
child will inhibit the growth of a limb.
Perhaps the shunning of the blood from
the spiral ligament into the auto sclerotic focus
produces hypoxia,
highland ization and ossification,
or perhaps in combination with
the protea,
elliptic enzymes that savants
and coasts have described another
abnormality we see here is a distortion of
the wall of the cochlear.
Is it possible that as the otis sclerosis impinges
upon the coakley.
It distorts it loosens the bachelor
membrane and interferes with the propagation
of the traveling wave up the rest of the
cochlea.
This shows the measurement of the two
ears in this case.
This was the case previously reported that had auto
sclerosis in one ear adjacent to the spiral
ligament and not in the other.
And we see that there is a difference in the
width of the bachelor membrane in the ear with
the Oto sclerosis involving the cochlear capsule.
Because of this,
we designed a model wherein we
could loosen the bachelor membrane at
four points along it.
This was copied after Becky sees and condors
model and we found that if we loosened the
bachelor membrane near what corresponds to
the basal turn of the cochlear,
that it did indeed decrease the
amplitude of the bachelor membrane
throughout the entire length of the cochlear.
This could account for a mechanical cause
of the sensory neural hearing loss in cochlear otis
sclerosis as we saw in the
typical audio gram of a patient with cochlear otis
sclerosis,
the configuration of the test is flat
rather than a high tone loss.
As we would expect to see with presby acoustics,
noise induced hearing loss is or drug
toxicity in summary,
then,
I am convinced that otis sclerosis can produce a
sensory neural hearing loss in clinical otis
sclerosis,
but very possibly in those individuals with a
progressive sensory neural hearing loss and a
family history of hearing loss,
particularly if there's a Schwartz he signed visible.
The mechanisms that produce this are not as yet
quite clear and very probably there are
more than one that is toxic and
influences on the organ of corti
vascular disturbances and distortion of
the cochlear capsule.
Assuming that otis sclerosis can produce the
sensory neural hearing loss.
Is it possible to make the diagnosis other than by
the history and physical examination and audio
metric tests val Vasari some years
ago demonstrated by polly tomography
that otis sclerosis can be visualized
by polly tomography.
However,
he had no histological evidence to show
this.
This next slide is of a
polit um of a temporal bone
following death.
And we see at the apex of the cochlear a
rarefied area,
this is the temporal bone of this
patient and in the same area we see it
Oto spongy idiotic lesion.
That is the early phase of photo sclerosis.
This is the other ear of the same patient and we see
it's rarefied but slightly proliferated here
and this is the temporal bone of that patient showing an
auto spongy,
idiotic lesion in the same area.
There's also some in the oval window but it wasn't visible
on that cut of the polyp tone.
This is the polyp tome of a normal
cochlear to orient you here is the
apex of the cochlear and the base will turn.
The important thing to notice is that the base will turn in the
normal is relatively translucent.
This is the temporal bone of a patient with
clinical otis sclerosis.
This small white area is part of the prosthesis.
in the oval window.
The basal turn is not clear.
This is the polyp tome of this same temporal bone
after death.
And we'll notice that there is a density
in the basal turn of the cochlear.
This is the temporal bone of that patient and will notice that
the Oto sclerotic lesion occupies the entire
width of the cochlear with some projection into the
basal turn.
This is the artist's rendition of the extent
of that otis sclerotic lesion,
and we can see that it projects slightly into the lumen of
the basal turn.
Here is the other ear of that patient before
death.
And we noticed that the basal turn is not quite as
dense as in the other ear,
but still not quite translucent as in
the normal.
This is the polyp tome of the same temporal
bone and we don't see the golf ball
density here in the basal turn that we saw in the other
ear.
Here is the odor sclerotic lesion in this temporal
bone.
And we see it occupies just the third the distance
or the thickness of the cochlear capsule,
and again the artist's rendition of the
size of the lesion being limited just to
the oval window area.
Therefore it is possible to make the diagnosis
of Otis sclerosis by polly tomography.
There are other conditions that can produce sensory neural
losses in patients with clinical otis
sclerosis and we must keep this in mind.
This slide is of the temporal bone
of a patient with clinical otis sclerosis,
a normal Coakley a with some preparation
artifacts.
Here is the oval window of that patient with a
relatively large otis sclerotic
focus.
If savants and Brett lows theories are correct.
Perhaps a focus limited to the
vestibule could secrete enough toxins into
the inner ear to produce a sensory neural hearing
loss.
Is this patient did not have sclerosis
involving the cochlear.
This is the audio graham on this here and we see
that the bone conduction threshold is low normal.
This is a polyp tone of this
year and again we see a relatively normally
appearing cochlear.
This is the polit um showing the vestibular area
which is normal size and notice the lateral
semicircular canal is normal.
Inside This is the audio Graham
on the other ear and we'll notice there is a greater
dip at 2000 than we saw in the
other ear.
The patient underwent a staple deck to me and had a rather
severe Drop in the bone conduction threshold.
At two 1000.
This is the polyp tome of this other ear and the coakley
appears abnormal in that we cannot see any odysseus
spiral laminar.
Coakley appears as a
typical of Mondini deformity and
this is the vestibule of the same here,
we see it's quite large and the lateral semicircular
canal is about twice its normal
size and this is the temporal
bone of this patient and we see that
the Aussie,
a spiral amina,
is lacking.
There's very poor support for the memory nous portions
of the cochlea.
Whether or not this is a preparation all
artifact or actually a rupture of the vascular
membrane secondary to The surgery that produced the
drop at 2000 cycles.
I cannot determine.
Here is another patient with clinical Otis sclerosis
seen in about 1967
and here is the other ear.
The patient returned five years later with
this audio Graham.
Because of this X rays were made and revealed
an enlarged internal auditory canal on the
side with the advanced hearing loss
and indeed a polit um X ray
revealed a filling defect of the internal
auditory canal due to an acoustic neuroma.
Mhm.
Another condition that can produce sensory neuro loss
in conjunction with Otis sclerosis is Meniere's
disease.
This patient had clinical otis sclerosis in
both ears,
but a greater loss in this year.
Some years previously had had episodic
vertigo and fluctuating hearing loss.
It was felt After two or 3 years of no vertigo
that state appendectomy could be undertaken
safely.
But the assumption was incorrect for the hearing
became worse.
Thus we must keep in mind.
The other possible causes of progressive
sensory neuron loss and individuals with
clinical otis sclerosis.
We use poly tomography to establish the
diagnosis in all cases with a
progressive sensory neuro component
to the clinical otis sclerosis.
Picture the
diagnosis of cochlear otis sclerosis is
now important for we feel there is a
treatment that will prevent the progression of the
sensory neural hearing loss,
Shambaugh and others in Chicago.
Some years ago suggested on the basis of some
epidemiologic studies that
sodium fluoride would prevent the progression
of the sensory neural hearing loss by changing
the auto spongy,
idiotic or immature Phase two,
The Auto Sclerotic or Mature Phase.
They based their judgment of the change of
the lesion on polly tomography.
I felt,
however,
that this was a nebulous way of making
this determination as it's very difficult to make
exactly the same exposure months
apart on each patient,
We therefore decided to use strontium
85 a radioactive isotope with a
very short half life to try and
determine the effectiveness of
sodium fluoride.
six of our patients had had drill out
procedures at the time of statehood ectomy
improved their hearing,
but subsequently there was a loss
of the hearing.
We had known for years that a subsequent drill
out would result in temporary hearing
improvement again,
but it would again be lost.
We contacted these patients and stated that
possibly by doing a third
operation and putting them on sodium fluoride,
we might prevent the regrowth regrowth of the bone.
We would give them strontium 85 a week prior
to the surgery to determine the activity
of the growing auto sclerotic bone
Strontium 85 is picked up by
osteoblasts and bone but not normal bone.
This slide shows the results of
our analysis at the time of surgery.
The foot plate was removed and dry.
These patients,
as I stated,
had been given strontium 85.
A week prior to surgery.
At the same time,
a piece of bone from the canal wall was removed.
We found that There was as
much as 34 times as much radio
activity in the foot plate as in
the canal bone.
The mean being 3-1,
none of these patients had a regression of their
hearing,
and so at a second operation,
the same procedure was carried out at this
time,
we found out that the radio activity in the foot plate
varied from 5 to 1 to 1 to
0,
with a mean of 1.5 to one.
This indicated to us that the sodium fluoride had
indeed decreased the radio activity
in the foot play.
A microscopic analysis of this
show that in at least half of the
cases the auto spongy,
chaotic foot plate in the first year
showed a change in the second year to an auto
sclerotic foot plate,
thereby indicating the sodium fluoride had an
effect on the photo sclerotic
focus the soft tissue detail is lost
here because it was necessary to drive these
specimens in order to weigh them and determine the
amount of radio activity.
This is another graph showing this change
in that the percent decrease in
activity between the 1st and 2nd
surgeries showed a
maximum change of
150
and a minimum change of 24 with a medium
of 82%.
What we're trying to do here is to convert the auto
spongy idiotic focus into the auto
sclerotic focus.
Every auto sclerotic focus has
both types of photos.
I this shows
the active Oto spongy chaotic
phase.
It's very cellular.
The marrow spaces are occupy more
area than the bony areas.
We can see an active osteoclasts here.
This is the auto sclerotic phase.
The barrel spaces are much less cellular.
The bone occupies the largest area of the
specimen,
and this is what we hope the sodium fluoride will
do is change the Oto spongy idiotic lesion
to the auto sclerotic lesion.
This graph represents 34 patients
who had been followed over a period of two years and
gradually showed an increase in their bone conduction
threshold at this point were placed on sodium
fluoride and it slowed down the progression of
the sensory neural hearing loss but did not
necessarily halted.
A certain group of these patients who had the
strontium 85 study,
in spite of the fact that there was less radio activity
in the second ear,
both foot plates appeared auto sclerotic.
At the first and the second surgery,
there were two cases in which the
lesion was auto spongy chaotic at the first
surgery.
And at the second surgery and yet at the second surgery,
the radio activity was decreased.
Yeah,
there was one case in which the first foot plate
was auto sclerotic and the second auto spongy,
chaotic.
And this was one of the few patients that showed an
increase in the radio activity in the
second tier.
One patient did not receive her sodium
fluoride as she misunderstood that she was to
continue taking it and her first foot plate
was neurotic,
as was the second.
These are the composite audio g of two
patients.
This was one of the failures with sodium
fluoride.
This shows the patient's hearing loss over a period of
10 years and at this point was placed on
sodium fluoride but continued to lose the hearing.
This patient was placed on sodium fluoride at this
point for a period of six months
and the hearing stabilized and then the fluoride was
discontinued.
Her hearing dropped precipitously and at this point
she was placed back on fluoride and her hearing
improved somewhat.
We have not seen many patients Improved,
but we've had one show as much as 25
decibels improvements.
This is a composite of those patients who were in
the strontium 85 study showing the
amount of decrease of radio activity between
the 1st and 2nd gear.
Only one of them showing an increase.
This is the group that had the auto spongy,
chaotic foot plate at the first surgery and auto
sclerotic at the second.
This is the group that had the auto sclerotic foot plate
at both the 1st and 2nd surgeries and in
spite of that,
we see there was considerable decrease in the radio
activity.
This was one case that showed
an auto spongy idiotic lesion and then an auto
sclerotic lesion.
And in spite of that there was a decrease in the
activity.
This is the patient who did not receive the
sodium fluoride and we see there was an increase in the
radio activity.
This is the dosage that we
use for the treatment of our patients.
It's been pretty well nationally accepted.
I keep my patients on this dosage for a period
of six months at the end of this time,
the hearing is retested.
If the hearing level is stable,
I reduced the dosage to three times weekly
in another six months to twice a week and finally to
once a week.
Occasionally one will find a patient in which the hearing
loss increases when the dose is reduced.
In this case,
the patient is put back on the dose that he was
receiving before and maintained on that
for a year,
the hearing is rechecked and if there is no change again,
an attempt is made to reduce the amount of
sodium fluoride.
In summary,
I'm convinced that otis sclerosis can produce
a sensory neural hearing loss,
the exact mechanism of the cause
of the hearing loss is as yet undetermined.
We can make the diagnosis of cochlear otis
sclerosis by polly tomography If
such equipment is not available,
a family history in a patient
with a progressive sensory neural hearing
loss and preferably a Schwartz,
he sign,
is a good indication that that patients flat
progressive sensory neural hearing loss may be due
to otis sclerosis.
I feel that the treatment with sodium
fluoride and calcium is innocuous.
I have yet to see a serious complication,
and I treat all patients
in whom I've made the diagnosis of cochlear otis
sclerosis as I have just outlined.