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.