WEBVTT 00:00.000 --> 00:30.000 position:50% align:top line:0% *This machine-generated transcript may have errors. If remediation or a manually-generated transcript is needed, please contact NLM Support at https://support.nlm.nih.gov.* 00:31.940 --> 00:34.420 There are some controversy as to whether Oto 00:34.420 --> 00:37.190 sclerosis can produce a sensory neural 00:37.190 --> 00:39.710 hearing impairment in this 00:39.710 --> 00:40.760 presentation. 00:40.760 --> 00:43.490 We hope to show that indeed 00:43.490 --> 00:46.440 can produce a sensory neural 00:46.440 --> 00:47.360 hearing loss, 00:48.040 --> 00:50.860 that the diagnosis can be made and that 00:50.860 --> 00:53.610 there is treatment that will prevent the progression 00:53.620 --> 00:56.460 of the century neural component of the law. 00:57.130 --> 01:00.060 This first slide is of a 58 year old 01:00.060 --> 01:02.960 woman who first began to develop her 01:02.960 --> 01:05.170 hearing loss at the age of 28. 01:05.830 --> 01:08.590 She had a family history of hearing loss. 01:09.840 --> 01:12.520 You can see that the otis sclerosis surrounds the 01:12.520 --> 01:13.120 cochlear. 01:13.410 --> 01:14.890 This is her audio graham. 01:14.890 --> 01:16.250 Shortly before death, 01:17.000 --> 01:19.920 there's no hearing in the left ear and almost 01:19.920 --> 01:22.250 total loss of hearing in the right ear. 01:22.840 --> 01:24.450 Was the otis sclerosis, 01:24.450 --> 01:27.380 the cause of the century neural hearing loss? 01:27.900 --> 01:29.190 I believe it was. 01:32.140 --> 01:34.940 This is an Otis sclerotic lesion adjacent 01:34.950 --> 01:37.060 to the organ of corti. 01:37.310 --> 01:38.780 In this particular case, 01:38.780 --> 01:41.590 we see that the spiral ligament and the organ of 01:41.590 --> 01:43.150 corti appear normal. 01:43.840 --> 01:44.450 However, 01:44.450 --> 01:47.150 this patient had a 40 decibel 01:47.150 --> 01:48.960 bone conduction threshold. 01:49.640 --> 01:50.650 The question is, 01:51.110 --> 01:54.030 did the otis sclerosis produce the sensory neural hearing 01:54.030 --> 01:55.280 loss and in this case, 01:55.290 --> 01:55.910 how 02:00.040 --> 02:02.920 further evidence that otis sclerosis can produce 02:02.920 --> 02:04.450 sensory neural hearing loss. 02:04.450 --> 02:06.360 Is illustrated by this slide, 02:06.840 --> 02:09.280 the upper columns indicate the bone 02:09.280 --> 02:11.960 construction threshold above each 02:11.970 --> 02:12.760 ear. 02:12.910 --> 02:15.310 The lower columns indicate the area of the 02:15.310 --> 02:18.200 spiral ligament involved by otis 02:18.200 --> 02:19.050 sclerosis. 02:19.520 --> 02:21.120 The limits of the bone conduction, 02:21.120 --> 02:23.390 oscillator on the audio meter. 02:23.390 --> 02:24.880 Are 70 decibels. 02:25.840 --> 02:26.340 Hence, 02:26.340 --> 02:28.750 we could not measure that above that level. 02:28.750 --> 02:31.600 This is the uh case that I 02:31.600 --> 02:34.050 just showed you of the massive otis 02:34.050 --> 02:36.790 sclerosis involving the cochlear and 02:36.790 --> 02:39.200 notice the amount of Otis sclerosis 02:39.210 --> 02:40.950 relative to the other cases. 02:41.540 --> 02:44.110 As the amount of otis sclerosis involving the 02:44.110 --> 02:45.810 spiral ligament increases, 02:46.140 --> 02:48.610 the bone conduction threshold increases 02:49.360 --> 02:52.130 in order to rule out the possibility of presby 02:52.130 --> 02:54.880 acoustics accounting for the loss in this group of 02:54.890 --> 02:55.580 patients. 02:56.120 --> 02:58.770 I plotted the age from 30 to 02:58.780 --> 03:01.270 83 on the bottom of the 03:01.270 --> 03:01.930 graph, 03:02.160 --> 03:05.160 on the top is the degree of sensory neural 03:05.160 --> 03:05.860 hearing loss. 03:05.860 --> 03:06.380 And again, 03:06.380 --> 03:09.100 this is the patient with the massive otis sclerosis, 03:09.370 --> 03:11.840 but we can see there is no regularity to the 03:11.840 --> 03:13.500 progression of the hearing loss. 03:13.940 --> 03:16.790 This indicates to me that in these cases the otis 03:16.790 --> 03:19.650 sclerosis was responsible for the sensory 03:19.650 --> 03:20.320 neuron loss. 03:20.320 --> 03:21.460 In most cases, 03:22.930 --> 03:25.680 taking the same group of patients and Plotting the 03:25.680 --> 03:27.030 two Ears separately, 03:27.030 --> 03:29.990 each column representing a pair 03:29.990 --> 03:30.850 of ears. 03:32.750 --> 03:35.460 We see that where the otis sclerosis was 03:35.460 --> 03:38.350 greater at the area of the spiral Ligament, 03:38.350 --> 03:39.730 one in 1 ear, 03:40.040 --> 03:42.770 the amount of sensory neural hearing loss was greater. 03:42.950 --> 03:44.970 with the exception of two cases. 03:44.970 --> 03:47.730 In those individuals who had large amounts of 03:47.730 --> 03:48.760 otis sclerosis, 03:49.330 --> 03:52.240 and in those two cases the difference of the amount of 03:52.240 --> 03:54.760 Otis sclerosis was not too great. 03:57.530 --> 03:59.950 This is a graph of 36 03:59.950 --> 04:02.730 patients who had unilateral clinical older 04:02.730 --> 04:03.460 sclerosis, 04:04.230 --> 04:07.100 the height of the column represents the difference in the 04:07.100 --> 04:09.960 bone conduction thresholds in the air 04:09.960 --> 04:12.360 with the auto sclerosis and without 04:13.100 --> 04:15.860 they are arranged along the bottom of 04:15.860 --> 04:18.710 the chart to show the ages of the 04:18.710 --> 04:21.540 patient and we see there is no real relationship 04:21.550 --> 04:24.480 between the age and the degree of the difference of 04:24.480 --> 04:27.230 the bone conduction thresholds and the two ears. 04:27.610 --> 04:30.400 But rather that it is apparently Due to 04:30.650 --> 04:33.540 the fact that one ear has Otis sclerosis and the other 04:33.540 --> 04:34.250 does not. 04:35.690 --> 04:38.250 This is an audio gram of a patient who had 04:38.940 --> 04:41.620 unilateral clinical otis sclerosis and a 04:41.620 --> 04:44.320 unilateral sensory neural hearing loss. 04:46.800 --> 04:49.750 This is the over window of the area with the 04:49.770 --> 04:51.370 clinical otis sclerosis. 04:51.370 --> 04:52.870 After a state appendectomy, 04:52.880 --> 04:55.620 we can see the over window filled with a gel foam 04:55.620 --> 04:58.160 membrane and an auto sclerotic focus here 04:58.620 --> 05:00.790 near but not involving the cochlear. 05:01.340 --> 05:01.800 However, 05:01.800 --> 05:03.630 in a different portion of the cochlear, 05:03.630 --> 05:06.620 we can see that the focus does involve the area of the 05:06.620 --> 05:07.550 spiral ligament. 05:10.030 --> 05:12.380 This is the other ear of the same patient, 05:12.390 --> 05:15.170 This is the staples foot plate and we see no otis 05:15.170 --> 05:16.960 sclerosis adjacent to it. 05:18.140 --> 05:18.690 However, 05:18.690 --> 05:21.350 in the coakley a there was a rather 05:21.350 --> 05:23.450 large auto sclerotic focus. 05:24.440 --> 05:27.120 This would indicate to me the possibility that this 05:27.130 --> 05:30.020 is one here in which the sensory neural hearing 05:30.020 --> 05:32.960 loss was due to sclerosis 05:34.100 --> 05:37.060 Because of cases such as this and there are three 05:37.060 --> 05:38.790 in our temporal bone collection. 05:39.340 --> 05:42.250 We have suspected that there are patients with a purely 05:42.250 --> 05:44.900 sensory neural hearing loss without evidence of 05:45.640 --> 05:47.010 clinical Oto sclerosis, 05:47.020 --> 05:49.560 but possibly the losses due to cochlear otis 05:49.560 --> 05:50.260 sclerosis. 05:50.940 --> 05:53.930 We have examined 140 patients 05:54.640 --> 05:57.020 who were suspected of having cochlear otis 05:57.020 --> 05:59.850 sclerosis by polly tomography and found that 05:59.850 --> 06:02.430 half of them showed evidence of possible 06:02.630 --> 06:04.100 cochlear otis sclerosis. 06:04.360 --> 06:05.950 We will discuss this shortly. 06:06.540 --> 06:09.150 This audio graham shows the typical audio 06:09.150 --> 06:11.560 metric findings in this group of patients. 06:12.140 --> 06:14.870 That is the type to Bekasi a high 06:14.870 --> 06:15.940 sissy score, 06:16.260 --> 06:18.830 low tone decay and relatively good 06:18.840 --> 06:20.370 discrimination scores. 06:21.940 --> 06:24.200 This is the audio gram of a patient with a 06:24.200 --> 06:26.810 unilateral clinical otis sclerosis. 06:26.820 --> 06:29.570 This is the bone conduction in the affected 06:29.570 --> 06:32.560 ear and the air conduction in the 06:32.560 --> 06:33.150 left ear. 06:33.150 --> 06:35.260 The air and bone were superimposed. 06:37.400 --> 06:39.900 This is the temporal bone of the ear. 06:39.900 --> 06:41.300 With the mixed hearing loss. 06:41.310 --> 06:44.250 We see three distinct fosse of otis sclerosis, 06:44.250 --> 06:47.200 one in the oval window and involving the endoscopy um 06:47.200 --> 06:48.140 of the cochlea, 06:48.370 --> 06:50.840 another adjacent to the internal carotid 06:50.840 --> 06:53.680 artery and the third involving the 06:53.680 --> 06:55.360 internal auditory canal. 06:56.940 --> 06:57.910 In the other air. 06:57.910 --> 07:00.560 There are no otis sclerotic fosse presents, 07:00.940 --> 07:03.670 although both Ears had a sensory neural hearing 07:03.670 --> 07:04.120 loss. 07:04.130 --> 07:06.640 The ear with the Otis sclerosis had a greater 07:06.640 --> 07:08.910 loss and we were unable to measure 07:08.910 --> 07:11.800 2000 cycles because of the limits of the 07:11.800 --> 07:13.300 bone conduction oscillator. 07:13.900 --> 07:16.340 This is a slide again showing an auto 07:16.340 --> 07:18.890 sclerotic focus adjacent to the spiral 07:18.890 --> 07:21.620 ligament but apparently a normal appearing 07:21.620 --> 07:22.580 organ of corti. 07:23.040 --> 07:25.820 Why does this patient have a sensory neural hearing 07:25.820 --> 07:26.180 loss, 07:26.180 --> 07:28.910 which the patient did have a threshold of 50 07:28.910 --> 07:31.860 decibels savants and Brett 07:31.860 --> 07:34.690 low and France have demonstrated license 07:34.690 --> 07:37.420 um at the advancing edge of the Oto sclerotic 07:37.430 --> 07:37.850 focus. 07:38.650 --> 07:41.600 This is what they feel causes the normal bone to 07:41.600 --> 07:44.270 be absorbed and replaced by the Oto 07:44.270 --> 07:45.460 sclerotic focus. 07:45.840 --> 07:48.470 These license soames excrete Osti elliptic 07:48.480 --> 07:49.260 enzymes. 07:49.890 --> 07:52.760 It's conceivable that when these enzymes are liberated 07:52.760 --> 07:54.860 into the inner ear spaces, 07:54.860 --> 07:57.380 they can be a toxic influence 07:57.510 --> 08:00.240 on the organ of corti in interfere with its 08:00.240 --> 08:01.500 normal metabolism. 08:02.050 --> 08:04.540 It's always been strange to me that otis 08:04.540 --> 08:07.140 sclerosis could replace normal bone without 08:07.140 --> 08:09.230 advancing osteoclasts. 08:09.470 --> 08:09.910 However, 08:09.910 --> 08:10.590 as we'll see, 08:10.590 --> 08:13.520 they always occur in the center of the lesion rather 08:13.520 --> 08:15.550 than in the advancing edge. 08:15.800 --> 08:18.530 So this is one of the possible causes of the 08:18.530 --> 08:21.120 sensory neural hearing loss found with 08:21.130 --> 08:21.670 cochlear. 08:21.670 --> 08:22.660 Otis sclerosis. 08:23.540 --> 08:25.980 Rudy many years ago described 08:25.980 --> 08:28.640 vascular shunts between the Oto sclerotic 08:28.650 --> 08:31.160 focus and the inner ear. 08:31.740 --> 08:34.580 He felt that this caused a venous stasis 08:34.860 --> 08:37.350 of the circulation in the inner ear, 08:37.360 --> 08:40.180 a build up of metabolites in the inner ear 08:40.180 --> 08:40.770 fluids, 08:41.010 --> 08:43.950 and this could have caused a sensory neural hearing loss 08:44.140 --> 08:46.950 without evidence of damage to the hair cells. 08:48.740 --> 08:51.480 If circulation is impaired to any organ, 08:51.480 --> 08:54.470 the pathologist tell us that lamelo bone 08:54.530 --> 08:57.200 can be laid down in that organ, 08:57.210 --> 08:57.880 for instance, 08:57.890 --> 08:59.660 in an ectopic pregnancy, 09:00.110 --> 09:03.010 gone to brickell in the lungs etcetera. 09:03.290 --> 09:06.260 Here we see a shunt between the inner ear and 09:06.260 --> 09:09.050 the Oto sclerotic focus and the otis 09:09.050 --> 09:11.860 sclerosis goes to this point this is 09:11.860 --> 09:14.510 lamelo bone that has been laid down within the 09:14.510 --> 09:15.980 lumen of the cochlear, 09:16.320 --> 09:19.070 which I feel is probably the end result 09:19.290 --> 09:21.780 of hypoxia or anoxia of 09:22.110 --> 09:23.100 the cochlear. 09:25.440 --> 09:27.880 This is the anterior portion of the 09:27.880 --> 09:30.850 cochlea of a patient with clinical otis sclerosis 09:30.850 --> 09:33.560 and a rather severe sensory neural hearing loss. 09:34.020 --> 09:35.740 We see a normal spiral ligament, 09:35.740 --> 09:36.870 An organ of corti. 09:37.240 --> 09:39.550 Mystery of vascular Harris appears normal. 09:40.400 --> 09:42.840 This is the post here half of the same 09:42.840 --> 09:45.500 cochlear with a large auto sclerotic 09:45.500 --> 09:46.960 focus adjacent to it. 09:46.970 --> 09:49.660 It appears to be a very active focus as it's 09:49.660 --> 09:50.620 quite cellular. 09:51.040 --> 09:53.770 We see a number of abnormalities here. 09:54.150 --> 09:56.350 The spiral ligament is highly ionized 09:57.240 --> 09:59.660 and in fact classified in this area, 10:00.340 --> 10:03.090 possibly due to hypoxia as a result 10:03.100 --> 10:04.860 of the otis sclerotic focus, 10:05.540 --> 10:07.080 I feel that perhaps Rudy. 10:07.080 --> 10:09.360 She can also explain 10:09.740 --> 10:12.600 changes such as this by shunning the arterial 10:12.600 --> 10:14.390 blood from the coakley a. 10:14.390 --> 10:14.980 To the O. 10:14.980 --> 10:16.090 To sclerotic focus, 10:16.090 --> 10:17.560 much like an aneurysm, 10:18.240 --> 10:18.900 as we know, 10:18.900 --> 10:21.710 an aneurysm or a large hemangioma in a 10:21.720 --> 10:24.150 child will inhibit the growth of a limb. 10:25.240 --> 10:27.960 Perhaps the shunning of the blood from 10:28.580 --> 10:31.310 the spiral ligament into the auto sclerotic focus 10:31.310 --> 10:32.950 produces hypoxia, 10:33.340 --> 10:35.720 highland ization and ossification, 10:35.880 --> 10:38.540 or perhaps in combination with 10:38.540 --> 10:39.800 the protea, 10:39.800 --> 10:42.360 elliptic enzymes that savants 10:42.740 --> 10:45.230 and coasts have described another 10:45.230 --> 10:48.130 abnormality we see here is a distortion of 10:48.130 --> 10:49.450 the wall of the cochlear. 10:49.890 --> 10:52.830 Is it possible that as the otis sclerosis impinges 10:52.830 --> 10:53.840 upon the coakley. 10:53.840 --> 10:56.290 It distorts it loosens the bachelor 10:56.290 --> 10:59.290 membrane and interferes with the propagation 10:59.290 --> 11:01.790 of the traveling wave up the rest of the 11:01.790 --> 11:02.460 cochlea. 11:05.660 --> 11:08.430 This shows the measurement of the two 11:08.430 --> 11:09.710 ears in this case. 11:09.710 --> 11:12.550 This was the case previously reported that had auto 11:12.550 --> 11:15.080 sclerosis in one ear adjacent to the spiral 11:15.080 --> 11:16.990 ligament and not in the other. 11:17.240 --> 11:20.020 And we see that there is a difference in the 11:20.020 --> 11:22.940 width of the bachelor membrane in the ear with 11:22.940 --> 11:25.860 the Oto sclerosis involving the cochlear capsule. 11:26.440 --> 11:27.400 Because of this, 11:27.400 --> 11:30.240 we designed a model wherein we 11:30.240 --> 11:32.920 could loosen the bachelor membrane at 11:32.930 --> 11:34.390 four points along it. 11:34.400 --> 11:37.120 This was copied after Becky sees and condors 11:37.130 --> 11:39.950 model and we found that if we loosened the 11:39.950 --> 11:42.940 bachelor membrane near what corresponds to 11:42.940 --> 11:44.540 the basal turn of the cochlear, 11:44.940 --> 11:47.620 that it did indeed decrease the 11:47.620 --> 11:49.980 amplitude of the bachelor membrane 11:50.350 --> 11:52.730 throughout the entire length of the cochlear. 11:53.340 --> 11:56.070 This could account for a mechanical cause 11:56.320 --> 11:59.160 of the sensory neural hearing loss in cochlear otis 11:59.160 --> 12:01.920 sclerosis as we saw in the 12:01.930 --> 12:04.820 typical audio gram of a patient with cochlear otis 12:04.820 --> 12:05.500 sclerosis, 12:05.510 --> 12:08.460 the configuration of the test is flat 12:08.840 --> 12:10.530 rather than a high tone loss. 12:10.530 --> 12:13.020 As we would expect to see with presby acoustics, 12:13.640 --> 12:15.930 noise induced hearing loss is or drug 12:15.930 --> 12:18.030 toxicity in summary, 12:18.030 --> 12:18.440 then, 12:18.860 --> 12:21.670 I am convinced that otis sclerosis can produce a 12:21.670 --> 12:24.080 sensory neural hearing loss in clinical otis 12:24.080 --> 12:24.800 sclerosis, 12:25.190 --> 12:28.190 but very possibly in those individuals with a 12:28.190 --> 12:31.110 progressive sensory neural hearing loss and a 12:31.110 --> 12:33.410 family history of hearing loss, 12:33.410 --> 12:36.260 particularly if there's a Schwartz he signed visible. 12:37.340 --> 12:40.320 The mechanisms that produce this are not as yet 12:40.330 --> 12:43.150 quite clear and very probably there are 12:43.150 --> 12:45.650 more than one that is toxic and 12:45.660 --> 12:47.860 influences on the organ of corti 12:48.340 --> 12:51.290 vascular disturbances and distortion of 12:51.290 --> 12:52.540 the cochlear capsule. 12:53.140 --> 12:55.740 Assuming that otis sclerosis can produce the 12:55.740 --> 12:57.210 sensory neural hearing loss. 12:57.210 --> 13:00.160 Is it possible to make the diagnosis other than by 13:00.160 --> 13:03.120 the history and physical examination and audio 13:03.120 --> 13:06.110 metric tests val Vasari some years 13:06.110 --> 13:08.640 ago demonstrated by polly tomography 13:08.980 --> 13:11.580 that otis sclerosis can be visualized 13:11.790 --> 13:13.460 by polly tomography. 13:13.840 --> 13:14.430 However, 13:14.430 --> 13:17.120 he had no histological evidence to show 13:17.120 --> 13:17.450 this. 13:18.070 --> 13:20.630 This next slide is of a 13:20.630 --> 13:23.050 polit um of a temporal bone 13:23.350 --> 13:24.290 following death. 13:24.290 --> 13:26.720 And we see at the apex of the cochlear a 13:26.720 --> 13:28.060 rarefied area, 13:29.610 --> 13:32.410 this is the temporal bone of this 13:32.410 --> 13:34.730 patient and in the same area we see it 13:35.440 --> 13:37.280 Oto spongy idiotic lesion. 13:37.280 --> 13:40.150 That is the early phase of photo sclerosis. 13:42.250 --> 13:45.220 This is the other ear of the same patient and we see 13:45.220 --> 13:48.170 it's rarefied but slightly proliferated here 13:50.040 --> 13:52.990 and this is the temporal bone of that patient showing an 13:52.990 --> 13:53.760 auto spongy, 13:53.760 --> 13:55.680 idiotic lesion in the same area. 13:55.990 --> 13:58.980 There's also some in the oval window but it wasn't visible 13:58.980 --> 14:01.100 on that cut of the polyp tone. 14:03.210 --> 14:05.710 This is the polyp tome of a normal 14:05.710 --> 14:08.660 cochlear to orient you here is the 14:09.040 --> 14:11.460 apex of the cochlear and the base will turn. 14:11.460 --> 14:14.200 The important thing to notice is that the base will turn in the 14:14.200 --> 14:16.960 normal is relatively translucent. 14:18.440 --> 14:21.050 This is the temporal bone of a patient with 14:21.060 --> 14:22.520 clinical otis sclerosis. 14:22.520 --> 14:25.260 This small white area is part of the prosthesis. 14:25.260 --> 14:26.370 in the oval window. 14:26.680 --> 14:28.730 The basal turn is not clear. 14:30.040 --> 14:32.840 This is the polyp tome of this same temporal bone 14:33.140 --> 14:34.060 after death. 14:34.800 --> 14:37.730 And we'll notice that there is a density 14:37.740 --> 14:39.620 in the basal turn of the cochlear. 14:42.640 --> 14:45.600 This is the temporal bone of that patient and will notice that 14:45.610 --> 14:48.510 the Oto sclerotic lesion occupies the entire 14:48.510 --> 14:51.470 width of the cochlear with some projection into the 14:51.470 --> 14:52.360 basal turn. 14:54.710 --> 14:57.540 This is the artist's rendition of the extent 14:57.550 --> 14:59.430 of that otis sclerotic lesion, 14:59.430 --> 15:02.350 and we can see that it projects slightly into the lumen of 15:02.350 --> 15:03.360 the basal turn. 15:05.440 --> 15:08.280 Here is the other ear of that patient before 15:08.280 --> 15:08.740 death. 15:08.750 --> 15:11.400 And we noticed that the basal turn is not quite as 15:11.400 --> 15:13.170 dense as in the other ear, 15:13.510 --> 15:16.440 but still not quite translucent as in 15:16.440 --> 15:17.000 the normal. 15:18.740 --> 15:21.730 This is the polyp tome of the same temporal 15:21.730 --> 15:23.830 bone and we don't see the golf ball 15:24.140 --> 15:27.080 density here in the basal turn that we saw in the other 15:27.080 --> 15:27.560 ear. 15:27.940 --> 15:30.640 Here is the odor sclerotic lesion in this temporal 15:30.640 --> 15:30.980 bone. 15:30.980 --> 15:33.900 And we see it occupies just the third the distance 15:33.910 --> 15:36.110 or the thickness of the cochlear capsule, 15:37.020 --> 15:39.840 and again the artist's rendition of the 15:39.840 --> 15:42.690 size of the lesion being limited just to 15:42.690 --> 15:44.600 the oval window area. 15:44.930 --> 15:47.880 Therefore it is possible to make the diagnosis 15:47.880 --> 15:50.350 of Otis sclerosis by polly tomography. 15:51.130 --> 15:54.100 There are other conditions that can produce sensory neural 15:54.100 --> 15:56.710 losses in patients with clinical otis 15:56.710 --> 15:59.140 sclerosis and we must keep this in mind. 15:59.520 --> 16:02.290 This slide is of the temporal bone 16:02.360 --> 16:05.060 of a patient with clinical otis sclerosis, 16:05.240 --> 16:07.870 a normal Coakley a with some preparation 16:08.040 --> 16:09.020 artifacts. 16:09.840 --> 16:12.770 Here is the oval window of that patient with a 16:12.780 --> 16:15.370 relatively large otis sclerotic 16:15.380 --> 16:15.860 focus. 16:17.420 --> 16:20.200 If savants and Brett lows theories are correct. 16:20.210 --> 16:22.960 Perhaps a focus limited to the 16:22.960 --> 16:25.830 vestibule could secrete enough toxins into 16:25.830 --> 16:28.690 the inner ear to produce a sensory neural hearing 16:28.690 --> 16:29.060 loss. 16:29.060 --> 16:31.740 Is this patient did not have sclerosis 16:31.740 --> 16:32.950 involving the cochlear. 16:33.370 --> 16:36.370 This is the audio graham on this here and we see 16:36.370 --> 16:39.210 that the bone conduction threshold is low normal. 16:41.540 --> 16:44.530 This is a polyp tone of this 16:44.540 --> 16:47.310 year and again we see a relatively normally 16:47.310 --> 16:48.330 appearing cochlear. 16:51.350 --> 16:54.130 This is the polit um showing the vestibular area 16:54.130 --> 16:56.690 which is normal size and notice the lateral 16:56.690 --> 16:58.940 semicircular canal is normal. 16:58.940 --> 17:01.910 Inside This is the audio Graham 17:01.910 --> 17:04.660 on the other ear and we'll notice there is a greater 17:04.660 --> 17:07.550 dip at 2000 than we saw in the 17:07.550 --> 17:08.270 other ear. 17:10.710 --> 17:13.400 The patient underwent a staple deck to me and had a rather 17:13.400 --> 17:15.980 severe Drop in the bone conduction threshold. 17:15.990 --> 17:16.960 At two 1000. 17:18.840 --> 17:21.490 This is the polyp tome of this other ear and the coakley 17:21.490 --> 17:24.390 appears abnormal in that we cannot see any odysseus 17:24.390 --> 17:25.310 spiral laminar. 17:25.710 --> 17:27.370 Coakley appears as a 17:28.310 --> 17:31.210 typical of Mondini deformity and 17:31.210 --> 17:33.340 this is the vestibule of the same here, 17:33.340 --> 17:36.330 we see it's quite large and the lateral semicircular 17:36.330 --> 17:38.760 canal is about twice its normal 17:38.940 --> 17:41.490 size and this is the temporal 17:41.490 --> 17:44.380 bone of this patient and we see that 17:44.380 --> 17:44.910 the Aussie, 17:44.910 --> 17:46.030 a spiral amina, 17:46.030 --> 17:46.960 is lacking. 17:47.170 --> 17:50.170 There's very poor support for the memory nous portions 17:50.170 --> 17:51.060 of the cochlea. 17:51.540 --> 17:53.750 Whether or not this is a preparation all 17:53.750 --> 17:56.580 artifact or actually a rupture of the vascular 17:56.580 --> 17:59.560 membrane secondary to The surgery that produced the 17:59.560 --> 18:01.350 drop at 2000 cycles. 18:01.740 --> 18:02.960 I cannot determine. 18:05.840 --> 18:08.730 Here is another patient with clinical Otis sclerosis 18:08.730 --> 18:11.170 seen in about 1967 18:12.940 --> 18:14.640 and here is the other ear. 18:15.730 --> 18:18.590 The patient returned five years later with 18:18.590 --> 18:19.660 this audio Graham. 18:22.740 --> 18:25.700 Because of this X rays were made and revealed 18:25.700 --> 18:28.690 an enlarged internal auditory canal on the 18:28.690 --> 18:31.370 side with the advanced hearing loss 18:31.560 --> 18:34.080 and indeed a polit um X ray 18:34.080 --> 18:37.050 revealed a filling defect of the internal 18:37.050 --> 18:39.770 auditory canal due to an acoustic neuroma. 18:40.340 --> 18:40.670 Mhm. 18:41.340 --> 18:44.340 Another condition that can produce sensory neuro loss 18:44.340 --> 18:47.010 in conjunction with Otis sclerosis is Meniere's 18:47.010 --> 18:47.670 disease. 18:48.040 --> 18:50.760 This patient had clinical otis sclerosis in 18:50.760 --> 18:51.400 both ears, 18:51.400 --> 18:53.160 but a greater loss in this year. 18:53.540 --> 18:56.280 Some years previously had had episodic 18:56.280 --> 18:58.580 vertigo and fluctuating hearing loss. 18:58.950 --> 19:01.890 It was felt After two or 3 years of no vertigo 19:01.890 --> 19:04.580 that state appendectomy could be undertaken 19:04.580 --> 19:05.240 safely. 19:05.460 --> 19:08.330 But the assumption was incorrect for the hearing 19:08.330 --> 19:09.260 became worse. 19:10.310 --> 19:11.940 Thus we must keep in mind. 19:11.940 --> 19:14.780 The other possible causes of progressive 19:14.780 --> 19:17.280 sensory neuron loss and individuals with 19:17.290 --> 19:18.850 clinical otis sclerosis. 19:19.740 --> 19:22.250 We use poly tomography to establish the 19:22.250 --> 19:24.680 diagnosis in all cases with a 19:24.680 --> 19:26.760 progressive sensory neuro component 19:27.440 --> 19:29.780 to the clinical otis sclerosis. 19:29.790 --> 19:32.250 Picture the 19:32.250 --> 19:35.000 diagnosis of cochlear otis sclerosis is 19:35.000 --> 19:37.440 now important for we feel there is a 19:37.440 --> 19:40.200 treatment that will prevent the progression of the 19:40.200 --> 19:41.670 sensory neural hearing loss, 19:42.640 --> 19:44.610 Shambaugh and others in Chicago. 19:44.610 --> 19:47.530 Some years ago suggested on the basis of some 19:47.540 --> 19:50.380 epidemiologic studies that 19:50.390 --> 19:53.290 sodium fluoride would prevent the progression 19:53.290 --> 19:56.240 of the sensory neural hearing loss by changing 19:56.240 --> 19:57.270 the auto spongy, 19:57.270 --> 19:59.750 idiotic or immature Phase two, 19:59.750 --> 20:02.680 The Auto Sclerotic or Mature Phase. 20:03.500 --> 20:06.420 They based their judgment of the change of 20:06.420 --> 20:08.500 the lesion on polly tomography. 20:09.520 --> 20:10.040 I felt, 20:10.040 --> 20:10.660 however, 20:10.660 --> 20:13.570 that this was a nebulous way of making 20:13.570 --> 20:16.410 this determination as it's very difficult to make 20:16.410 --> 20:19.010 exactly the same exposure months 20:19.010 --> 20:21.050 apart on each patient, 20:22.230 --> 20:24.650 We therefore decided to use strontium 20:24.660 --> 20:27.540 85 a radioactive isotope with a 20:27.540 --> 20:29.890 very short half life to try and 20:29.890 --> 20:32.120 determine the effectiveness of 20:33.340 --> 20:34.560 sodium fluoride. 20:35.340 --> 20:37.580 six of our patients had had drill out 20:37.590 --> 20:40.560 procedures at the time of statehood ectomy 20:41.300 --> 20:42.780 improved their hearing, 20:42.780 --> 20:45.120 but subsequently there was a loss 20:45.540 --> 20:46.460 of the hearing. 20:47.340 --> 20:50.280 We had known for years that a subsequent drill 20:50.280 --> 20:53.060 out would result in temporary hearing 20:53.060 --> 20:54.040 improvement again, 20:54.040 --> 20:55.760 but it would again be lost. 20:56.720 --> 20:59.050 We contacted these patients and stated that 20:59.060 --> 21:01.720 possibly by doing a third 21:01.720 --> 21:04.500 operation and putting them on sodium fluoride, 21:04.730 --> 21:07.690 we might prevent the regrowth regrowth of the bone. 21:09.190 --> 21:12.170 We would give them strontium 85 a week prior 21:12.170 --> 21:14.720 to the surgery to determine the activity 21:14.910 --> 21:17.170 of the growing auto sclerotic bone 21:17.540 --> 21:20.270 Strontium 85 is picked up by 21:21.150 --> 21:24.060 osteoblasts and bone but not normal bone. 21:24.630 --> 21:26.960 This slide shows the results of 21:27.440 --> 21:30.230 our analysis at the time of surgery. 21:30.910 --> 21:33.600 The foot plate was removed and dry. 21:33.980 --> 21:34.710 These patients, 21:34.710 --> 21:35.500 as I stated, 21:35.500 --> 21:37.520 had been given strontium 85. 21:37.520 --> 21:38.880 A week prior to surgery. 21:39.440 --> 21:40.620 At the same time, 21:40.620 --> 21:43.360 a piece of bone from the canal wall was removed. 21:43.940 --> 21:46.670 We found that There was as 21:46.670 --> 21:49.170 much as 34 times as much radio 21:49.170 --> 21:52.070 activity in the foot plate as in 21:52.070 --> 21:53.220 the canal bone. 21:53.610 --> 21:55.460 The mean being 3-1, 21:56.230 --> 21:58.870 none of these patients had a regression of their 21:58.870 --> 21:59.380 hearing, 21:59.380 --> 22:01.600 and so at a second operation, 22:02.370 --> 22:05.270 the same procedure was carried out at this 22:05.270 --> 22:05.640 time, 22:05.640 --> 22:08.560 we found out that the radio activity in the foot plate 22:10.040 --> 22:12.540 varied from 5 to 1 to 1 to 22:12.540 --> 22:13.160 0, 22:13.540 --> 22:16.420 with a mean of 1.5 to one. 22:17.040 --> 22:20.040 This indicated to us that the sodium fluoride had 22:20.040 --> 22:22.620 indeed decreased the radio activity 22:22.970 --> 22:23.960 in the foot play. 22:25.440 --> 22:27.990 A microscopic analysis of this 22:28.000 --> 22:30.880 show that in at least half of the 22:30.880 --> 22:32.860 cases the auto spongy, 22:32.860 --> 22:35.860 chaotic foot plate in the first year 22:36.940 --> 22:39.720 showed a change in the second year to an auto 22:39.720 --> 22:41.070 sclerotic foot plate, 22:41.070 --> 22:43.880 thereby indicating the sodium fluoride had an 22:43.880 --> 22:46.430 effect on the photo sclerotic 22:46.440 --> 22:49.400 focus the soft tissue detail is lost 22:49.400 --> 22:51.700 here because it was necessary to drive these 22:51.700 --> 22:54.660 specimens in order to weigh them and determine the 22:54.660 --> 22:56.820 amount of radio activity. 22:59.300 --> 23:02.250 This is another graph showing this change 23:02.250 --> 23:04.650 in that the percent decrease in 23:04.650 --> 23:06.820 activity between the 1st and 2nd 23:06.820 --> 23:09.400 surgeries showed a 23:09.400 --> 23:10.870 maximum change of 23:10.870 --> 23:13.270 150 23:13.840 --> 23:16.770 and a minimum change of 24 with a medium 23:17.440 --> 23:18.850 of 82%. 23:22.540 --> 23:25.320 What we're trying to do here is to convert the auto 23:25.320 --> 23:27.830 spongy idiotic focus into the auto 23:27.830 --> 23:29.140 sclerotic focus. 23:29.150 --> 23:31.760 Every auto sclerotic focus has 23:31.760 --> 23:33.100 both types of photos. 23:33.100 --> 23:35.860 I this shows 23:35.870 --> 23:38.430 the active Oto spongy chaotic 23:38.440 --> 23:38.920 phase. 23:38.920 --> 23:40.350 It's very cellular. 23:40.360 --> 23:43.360 The marrow spaces are occupy more 23:43.360 --> 23:46.180 area than the bony areas. 23:46.490 --> 23:49.400 We can see an active osteoclasts here. 23:50.740 --> 23:53.230 This is the auto sclerotic phase. 23:53.250 --> 23:56.200 The barrel spaces are much less cellular. 23:56.480 --> 23:59.280 The bone occupies the largest area of the 23:59.280 --> 23:59.980 specimen, 24:00.280 --> 24:03.120 and this is what we hope the sodium fluoride will 24:03.120 --> 24:06.020 do is change the Oto spongy idiotic lesion 24:06.020 --> 24:07.880 to the auto sclerotic lesion. 24:08.390 --> 24:11.020 This graph represents 34 patients 24:11.440 --> 24:14.130 who had been followed over a period of two years and 24:14.130 --> 24:16.970 gradually showed an increase in their bone conduction 24:16.970 --> 24:19.910 threshold at this point were placed on sodium 24:19.910 --> 24:22.820 fluoride and it slowed down the progression of 24:22.820 --> 24:25.250 the sensory neural hearing loss but did not 24:25.250 --> 24:26.460 necessarily halted. 24:26.580 --> 24:29.120 A certain group of these patients who had the 24:29.120 --> 24:31.010 strontium 85 study, 24:31.790 --> 24:34.740 in spite of the fact that there was less radio activity 24:34.740 --> 24:35.970 in the second ear, 24:36.170 --> 24:38.910 both foot plates appeared auto sclerotic. 24:38.920 --> 24:41.160 At the first and the second surgery, 24:43.040 --> 24:45.610 there were two cases in which the 24:45.610 --> 24:48.160 lesion was auto spongy chaotic at the first 24:48.160 --> 24:48.680 surgery. 24:48.680 --> 24:51.670 And at the second surgery and yet at the second surgery, 24:51.670 --> 24:54.160 the radio activity was decreased. 24:54.540 --> 24:54.770 Yeah, 24:55.240 --> 24:58.070 there was one case in which the first foot plate 24:58.070 --> 25:00.920 was auto sclerotic and the second auto spongy, 25:00.920 --> 25:01.380 chaotic. 25:01.660 --> 25:04.020 And this was one of the few patients that showed an 25:04.030 --> 25:06.760 increase in the radio activity in the 25:06.760 --> 25:07.660 second tier. 25:09.540 --> 25:12.100 One patient did not receive her sodium 25:12.100 --> 25:15.050 fluoride as she misunderstood that she was to 25:15.050 --> 25:17.760 continue taking it and her first foot plate 25:17.760 --> 25:18.610 was neurotic, 25:18.610 --> 25:19.860 as was the second. 25:23.340 --> 25:26.130 These are the composite audio g of two 25:26.130 --> 25:26.910 patients. 25:26.990 --> 25:29.580 This was one of the failures with sodium 25:29.580 --> 25:30.160 fluoride. 25:30.160 --> 25:32.880 This shows the patient's hearing loss over a period of 25:32.890 --> 25:35.760 10 years and at this point was placed on 25:35.760 --> 25:38.630 sodium fluoride but continued to lose the hearing. 25:39.330 --> 25:42.310 This patient was placed on sodium fluoride at this 25:42.310 --> 25:44.870 point for a period of six months 25:45.180 --> 25:47.760 and the hearing stabilized and then the fluoride was 25:47.760 --> 25:48.710 discontinued. 25:48.720 --> 25:51.640 Her hearing dropped precipitously and at this point 25:51.640 --> 25:54.420 she was placed back on fluoride and her hearing 25:54.420 --> 25:55.450 improved somewhat. 25:55.940 --> 25:58.150 We have not seen many patients Improved, 25:58.150 --> 26:00.490 but we've had one show as much as 25 26:00.490 --> 26:01.850 decibels improvements. 26:03.640 --> 26:06.560 This is a composite of those patients who were in 26:06.560 --> 26:09.270 the strontium 85 study showing the 26:09.270 --> 26:12.270 amount of decrease of radio activity between 26:12.270 --> 26:13.870 the 1st and 2nd gear. 26:14.080 --> 26:16.270 Only one of them showing an increase. 26:16.940 --> 26:19.170 This is the group that had the auto spongy, 26:19.170 --> 26:21.520 chaotic foot plate at the first surgery and auto 26:21.520 --> 26:22.900 sclerotic at the second. 26:23.440 --> 26:26.320 This is the group that had the auto sclerotic foot plate 26:26.320 --> 26:29.230 at both the 1st and 2nd surgeries and in 26:29.230 --> 26:29.920 spite of that, 26:29.920 --> 26:32.690 we see there was considerable decrease in the radio 26:32.690 --> 26:33.460 activity. 26:34.400 --> 26:37.220 This was one case that showed 26:37.220 --> 26:39.920 an auto spongy idiotic lesion and then an auto 26:39.920 --> 26:40.970 sclerotic lesion. 26:40.970 --> 26:43.740 And in spite of that there was a decrease in the 26:43.740 --> 26:44.410 activity. 26:44.800 --> 26:47.660 This is the patient who did not receive the 26:47.660 --> 26:50.570 sodium fluoride and we see there was an increase in the 26:50.570 --> 26:51.670 radio activity. 26:55.050 --> 26:57.490 This is the dosage that we 26:57.500 --> 27:00.400 use for the treatment of our patients. 27:00.400 --> 27:02.770 It's been pretty well nationally accepted. 27:04.940 --> 27:07.910 I keep my patients on this dosage for a period 27:07.910 --> 27:10.070 of six months at the end of this time, 27:10.070 --> 27:11.560 the hearing is retested. 27:12.240 --> 27:14.560 If the hearing level is stable, 27:14.570 --> 27:17.360 I reduced the dosage to three times weekly 27:18.220 --> 27:21.140 in another six months to twice a week and finally to 27:21.140 --> 27:21.860 once a week. 27:22.740 --> 27:25.730 Occasionally one will find a patient in which the hearing 27:25.820 --> 27:28.560 loss increases when the dose is reduced. 27:28.940 --> 27:29.700 In this case, 27:29.700 --> 27:32.390 the patient is put back on the dose that he was 27:32.390 --> 27:35.370 receiving before and maintained on that 27:35.380 --> 27:36.240 for a year, 27:36.600 --> 27:39.590 the hearing is rechecked and if there is no change again, 27:39.590 --> 27:42.510 an attempt is made to reduce the amount of 27:42.510 --> 27:43.570 sodium fluoride. 27:44.540 --> 27:45.310 In summary, 27:45.310 --> 27:48.240 I'm convinced that otis sclerosis can produce 27:48.240 --> 27:49.850 a sensory neural hearing loss, 27:50.840 --> 27:53.760 the exact mechanism of the cause 27:53.760 --> 27:56.170 of the hearing loss is as yet undetermined. 27:56.930 --> 27:59.870 We can make the diagnosis of cochlear otis 27:59.870 --> 28:02.720 sclerosis by polly tomography If 28:02.720 --> 28:04.670 such equipment is not available, 28:04.680 --> 28:07.390 a family history in a patient 28:07.400 --> 28:10.030 with a progressive sensory neural hearing 28:10.030 --> 28:11.770 loss and preferably a Schwartz, 28:11.770 --> 28:12.430 he sign, 28:12.690 --> 28:15.450 is a good indication that that patients flat 28:15.840 --> 28:18.650 progressive sensory neural hearing loss may be due 28:18.650 --> 28:19.860 to otis sclerosis. 28:20.670 --> 28:23.430 I feel that the treatment with sodium 28:23.430 --> 28:26.040 fluoride and calcium is innocuous. 28:26.170 --> 28:28.690 I have yet to see a serious complication, 28:29.180 --> 28:31.820 and I treat all patients 28:32.100 --> 28:34.690 in whom I've made the diagnosis of cochlear otis 28:34.690 --> 28:36.770 sclerosis as I have just outlined.