[Surgical treatment of otosclerosis. Therapeutic results of employment of the Fisch prosthesis in 100 consecutive patients with otosclerosis].
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Adult↗
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Results for “Otosclerosis”
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Improvement of hearing in cases of otosclerosis. "Improvement of hearing in cases of otosclerosis. A new, one stage surgical technic" by Julius Lempert.
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Fenestration, Labyrinth↗
[Otosclerosis and otosclerosis surgery].
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Humans↗
Otosclerosis: the University of Minnesota temporal bone collection.
A study of 1452 human temporal bones revealed a previously unpublished material of 144 bones with otosclerosis. After exclusion of infants and individuals of races other than white, the incidence of otosclerosis was 12.75%. Of the bones with otosclerosis, 56.1% belonged to men and 43.9% to women. The incidence of clinical and histologic otosclerosis was practically the same for men (44.7% to 55.3%) as for women (47% to 53%). However, the incidence of bilateral otosclerosis was higher in women (88.9%) than in men (65.2%). Bilateral otosclerosis was present in 75.6%, whereas it was unilateral in 24.4%. Sixty-six (66) ears (45.8%) had clinical otosclerosis, whereas 78 (54.2%) had histologic otosclerosis--frequently unifocal lesions. The most common site was anterior to the oval window (117 ears, 81.25%), followed by round window niche (52 ears, 36.11%), apical and medial cochlear wall (31 ears, 21.52%), and anterior wall of the internal auditory canal (27 ears, 18.75%). The activity of lesions was directly related to their size. Smaller lesions were predominantly inactive, whereas medium and larger lesions were predominantly active. There was a positive correlation when the size of the lesions, activity, and degree of cochlear endosteal involvement were compared with bone conduction thresholds (37 cases). Correlations between size and activity, and between activity and associated sensorineural hearing loss did not necessarily follow the sequence of an initial active stage (spongiotic) to a final inactive one (sclerotic). Comparison of cases of otosclerosis with equivalent age groups of the normal population yielded worse bone conduction thresholds for the otosclerosis cases only in the age group 60 to 69 years and older. Comparison of average bone conduction thresholds between bones with one site of endosteal involvement (28.26 dB HL) revealed no significant differences. Bones with two or more sites of endosteal involvement had significant differences. Bones with two or more sites of endosteal involvement had significantly worse bone conduction thresholds (62 dB HL). The overall results are not suggestive of an association of sensorineural hearing loss with otosclerosis without stapedial fixation.
Adolescent↗
Vestibular symptoms in otosclerosis--correlation of otosclerotic involvement of vestibular apparatus and Scarpa's ganglion cell count.
Although several histopathologic studies have shown otosclerotic involvement of the vestibular apparatus in patients with otosclerosis, the pathogenesis of vestibular symptoms in otosclerosis remains unknown. A quantitative study of Scarpa's ganglion was performed in 217 temporal bones from 118 subjects with otosclerosis. Review of clinical records revealed an incidence of vestibular symptoms in 11.9% of these subjects. Scarpa's ganglion cell counts in temporal bones of subjects with otosclerosis and vestibular symptoms were lower than counts in temporal bones of subjects with otosclerosis but without vestibular symptoms and those of normal subjects. This difference in Scarpa's ganglion cell counts, adjusted for age, between the group with otosclerosis and vestibular symptoms and a group of normal subjects was highly significant (p = 0.0015), whereas the difference in Scarpa's ganglion cell count between a group with otosclerosis but without vestibular symptoms and a group of normal subjects was not significant (p = 0.53). There was also a significant correlation between elevation of the average bone-conduction threshold and the presence of vestibular symptoms in these subjects (p = 0.041). The endosteum of the perilymphatic space of the vestibule and the endosteum of the canal for the superior vestibular nerve or its cribrose area were the two most common sites of involvement by otosclerosis. However, there was no significant correlation between the presence of vestibular symptoms and otosclerotic involvement of any single site or the number of involved sites. Histologic examination of the vestibular nerve fibers and end organs subjacent to otosclerotic foci demonstrated no obvious degenerative changes. Thus our findings appear to suggest that the vestibular symptoms present in patients with otosclerosis are more common in patients with elevated bone conduction thresholds and are correlated with degeneration of the vestibular nerve, which appears to be independent of the severity of otosclerotic involvement of the vestibular end organs.
Aged↗
Does otosclerosis occur only in the temporal bone?
HYPOTHESIS: Otosclerosis does not occur outside the temporal bone. BACKGROUND: The widely accepted assumption that otosclerosis is confined to the temporal bone has never been tested. It is important to investigate this issue, particularly because of evidence that otosclerosis may be a systemic (genetic) disease that could affect other bones. METHODS: Biopsies from 9 to 11 skeletal sites were obtained from 2 patients with clinical otosclerosis. Two hundred forty-one nontemporal bone sections were examined by light microscopy. RESULTS: No nontemporal skeletal bone section showed histologic evidence of otosclerosis. The data indicate, with 95% confidence, that the true prevalence of otosclerosis in the extratemporal skeleton of the 2 patients examined was < 3%. CONCLUSIONS: These findings suggest that otosclerosis is unlikely to occur outside the temporal bone. Factors unique to the otic capsule that may predispose it to otosclerosis are lack of bone remodeling and the presence of globuli interossei.
Adult↗
The negative on/off effect in cochlear and early stapedial otosclerosis.
Acoustic reflex patterns were reviewed for 100 patients suspected of having cochlear otosclerosis (group 1) and 73 patients with confirmed stapedial otosclerosis and conductive hearing losses of less than 5 years duration (group 2). Three abnormal reflex patterns were exhibited by these patients--"negative on/off," "no response when the probe was in the affected ear," and "no response when the stimulus was in affected ear." Results showed that the negative on/off pattern was equally as common for the two subject groups. The "no response" patterns were more common for the early stapedial otosclerosis group. The presence of acoustic reflex patterns classically associated with stapedial otosclerosis in patients with cochlear otosclerosis leads us to predict that some degree of stapedial fixation is common in many cases of cochlear otosclerosis. Based upon these findings, the presence of the negative on/off pattern in patients with mild sensorineural hearing loss, excellent speech discrimination, and a family history of hearing loss, is now considered indicative of cochlear otosclerosis.
Adolescent↗
[Progressive sensorineural hearing loss in cochlear otosclerosis].
Otosclerosis is a bone dysplasia limited to the otic capsule causing abnormal resorption and redeposition of bone. The existence of the entity "pure labyrinthine otosclerosis" or "cochlear otosclerosis" is not accepted by all authors; however, there is clinical and histologic evidence to support the existence of a progressive sensorineural hearing loss due to otospongiotic-otosclerotic lesions of the labyrinthine capsule, although diagnosis of this condition may be difficult. The involvement of the inner ear is described as degenerative changes in the spiral ligament, stria vascularis, organ of Corti, and cochlear neurons. The most frequent audiometric configuration is a "bite-type" curve, but flat or rising shapes can also be observed; speech discrimination appears unusually good for a pure sensorineural hearing loss and recruitment is frequently absent. A cochlear otosclerosis should be suspected when there is a family history of otosclerosis, the onset of the hearing loss occurs from the third to fifth decade, and worsening of the hearing loss is observed during periods of intense hormonal and endocrine activity, a positive Schwartze sign is present and bilateral sensorineural loss is associated with signs of unilateral stapedial ankylosis. A definitive diagnosis of cochlear otosclerosis can be made only with computed tomography, which allows a quantitative assessment of the involvement of the labyrinthine capsule by spongiotic or sclerotic areas. The factors to be considered are: otosclerotic foci 1 mm or more in diameter and a density different from that of the normal otic capsule, partially or completely erased contour of the capsule, double ring effect, bony neoformation in the labyrinthine spaces, and increased thickness of the cochlear capsule. The medical management of cochlear otosclerosis is based on sodium fluoride, in association with calcium and vitamin D; some authors have also proposed diphosphonates as inhibitor agents of bone resorption. Surgery may be useful only in those patients presenting a hearing loss so severe that the bone threshold cannot be evaluated and a gap between air and bone conduction cannot be excluded; in these cases stapes operations can improve hearing to a level that may be useful in hearing aid application.
Cochlea↗
[Sensorineural deafness and cochlear otosclerosis (author's transl)].
The so-called labyrinthine otosclerosis is defined as a focal otosclerotic involvement of the labyrinthine capsule without stapes fixation. It produces a pure sensorineural hearing loss. The histology of this phenomenon is well known. The relative frequency of labyrinthine otosclerosis is between 4 and 40%, as referred in literature. On x-rays, an advanced demineralisation or complete obliteration of labyrinthic bone is suspicious of otosclerosis. There is no typical pattern of sensorineural hearing loss. But not seldom a fair discrimination index contrasts with a severe threshold evaluation in pure tone audiometry. 208 audiogramms of otosclerosic ears verified by stapedectomy were examined. After correction for normal presbyacusis the sensorineural hearing loss due to otosclerosis is twice that of presbyacusis. In otosclerosis tinnitus is often reported as of metallic type, but a characteristic tinnitus does not exist. There is no universal agreement about the relative frequency of vestibular disturbances in otosclerosis. One should diagnose labyrinthine otosclerosis only if several criteria found are not explanable otherwise, and these criteria are explained.
Audiometry↗
Evidence of abnormal stromelysin mRNA expression in suspected carriers of otosclerosis. A possible molecular marker.
OBJECTIVE: There is strong evidence that otosclerosis is a genetic disease affecting bone remodeling. We propose that, if otosclerosis is genetic, it will manifest itself universally, particularly at the mRNA transcription level. DESIGN: Skin biopsy specimens were taken in a single blind from human subjects who had been clinically and surgically identified as having otosclerosis. SETTING: Subjects were volunteers from the community, identified through hospital records. All procedures were carried out in a clinical research facility. PATIENTS: Twenty-one volunteers underwent a biopsy, including those positively identified as having otosclerosis (n = 4), their blood relatives (n = 8), or nonrelatives with normal hearing and no known history of otosclerosis (n = 9). INTERVENTION: Three connective tissue remodeling factors, procollagenase, prostromelysin, and tissue inhibitor of metalloprotease, were analyzed. The mRNA was extracted from each biopsy specimen, hybridized against radiolabeled cDNA, and quantitatively measured by radioautography. MAIN OUTCOME MEASURE: We expected to see significant differences in the pattern of mRNA expression for one or more of the three measured bone remodeling factors in otosclerotics, compared with age- and sex-matched negative controls. RESULTS: Two of the otosclerotic subjects had abnormally low levels of mRNA for prostromelysin and two had higher than normal levels. In three (75%) of the four, variability of mRNA expression among procollagenase, prostromelysin, and metalloprotease tissue inhibitor was higher than normal. Three (38%) of the eight relatives showed a similar pattern and two (22%) of the nine control subjects also tested as abnormal. CONCLUSION: This observed variability in otosclerotic subjects might be a manifestation of a genetic control defect, and abnormal stromelysin mRNA expression could serve as a genetic marker for otosclerosis.
Adolescent↗
Histological investigation of skin biopsies in otosclerosis and osteogenesis imperfecta.
Histological investigation of skin biopsies in four patients with osteogenesis imperfecta, nine patients with otosclerosis and 13 sex- and age-matched normal controls was carried out blindly. The dermal thickness was markedly reduced in osteogenesis imperfecta and slightly reduced in otosclerosis. Minor degenerative changes in the elastic fibres were seen in otosclerosis, while the elastic fibres were found to be more degenerated and more numerous in osteogenesis imperfecta. Our study does not support the hypothesis of otosclerosis being a localized form of osteogenesis imperfecta. The minor changes in the elastic fibres in otosclerosis indicate that further studies of the elastic fibres and of collagen will be necessary to determine whether otosclerosis is a localized disease or part of a general connective tissue disorder.
Adult↗
The doubtful value of tympanometry in the diagnosis of otosclerosis.
Many consider that the compliance of the middle ear as measured from the tympanogram can be helpful in diagnosing otosclerosis. To test this assertion, the compliance in 34 individuals with surgically proven otosclerosis was compared with the compliance in 34 age and sex matched, normal controls, randomly selected from the population. Though the mean compliance was different in the two groups, there was considerable overlap in the range of values which severely limits the practical usefulness of tympanometry. If the level of compliance is taken at which a false negative diagnosis would be made in 10 per cent of otosclerotic ears, a false positive diagnosis of otosclerosis would be made in 88 per cent of normal ears. If the level of compliance is taken at which a false positive diagnosis of otosclerosis would be made in 10 per cent of normal ears, 72 per cent of ears with otosclerosis would be considered normal. It is concluded that tympanometry will not help to arrive at a diagnosis of otosclerosis.
Acoustic Impedance Tests↗
Dynamics of otosclerosis-like lesions in LP/J mice.
The LP/J inbred mouse is the first known animal to spontaneously develop otosclerosis-like bone lesions in the middle and inner ear. These abnormal bone foci resemble human otosclerotic lesions, are inherited, and produce a progressive hearing loss. On the basis of histological evaluation alone, it was unclear whether these otosclerosis-like lesions in LP/J mice develop suddenly or gradually as in human otosclerosis. To study the dynamics of lesion formation, four different colored fluorochrome bone labels were given in sequence to postpubertal LP/J mice and CBA/J mice as normal controls. All of the otosclerosis-like lesions incorporated at least one fluorochrome, and half were labeled with two or more markers indicating continuous lesion growth over many weeks. It appears that otosclerosis-like lesions in LP/J mice develop during early adulthood and progress gradually as in human otosclerosis.
Animals↗
Paradoxical compliances in otosclerosis.
Results of impedancemetry have been compared with operative findings in 1 867 cases of otosclerosis, 1 583 of which were pure otosclerosis. This comparison indicated that: (1) with present techniques, the results of impedancemetry are only of a relative value in otosclerosis; it is thus necessary to asses the various connected anatomical elements in the middle ear with extreme rigor; (2) impedancemetry data have an absolute value in all disorders of the middle ear with closed eardrum associated with otosclerosis; (3) relative impedancemetry often gives informative results when it concurs with the other elements of clinical and audiometrical diagnosis; (4) impedancemetry data are a valuable element of early diagnosis of otosclerosis before the first audiometric expression, either by a lowering of compliance or, most of all, by appearance of an on-off effect (diphasic impedance change) or by disappearance of the stapedius reflex; (5) parallel or crossed paradoxical compliances are only apparently paradoxical and are usually the sign of either an early stapedo-vestibular fixation (before its audiometric expression) or an associated factor in the middle ear, both of which may have been unnoticed; (6) the problem of compliance value in otosclerosis will be solved only when the proper stapes frequency is found and when it is possible to test this frequency. This problem is the subject of our current research.
Adolescent↗
Histopathology of the stapes in otosclerosis.
A histopathological examination was made of 60 stapes obtained during surgery for clinical otosclerosis. The findings, correlated with the clinical history and macroscopic appearances of the stapes, led to the following observations. The histological extent of the focus of otosclerosis agrees well with the macroscopic appearance of the footplate lesion seen under the operating microscope. The earlier the age of onset of hearing loss the greater the probability of active, severe and diffuse otosclerotic involvement of the footplate. Two-thirds of the cases with diffuse and severe footplate otosclerosis had signs of active disease. A late age of onset of clinical otosclerosis tends to be associated with lesions that are limited to the anterior pole of the stapes footplate. Active otosclerosis is rare in these cases. The case for macroscopic increase in extent of the lesion with time could not be established by the data. The evidence supports the view that the severity and extent of otosclerotic disease in the footplate is determined more by age at onset than by duration of symptoms. Healing of the active focus of otosclerosis may not always occur despite a long duration of symptoms. A highly active focus in the stapes footplate can remain active for many years.
Ear Ossicles↗
Cochlear otosclerosis: acoustic reflex findings.
Progressive cochlear impairment develops in a small percentage of patients with clinical otosclerosis, apparently owing to involvement of the cochlea by the otosclerotic bone. In recent years it has been postulated that otosclerosis frequently produces a pure sensorineural hearing impairment without stapedial involvement. We investigated the acoustic reflex responses in a group of patients diagnosed as sensorineural otosclerosis and compared these reflex findings with the findings in patients with confirmed otosclerosis but with a minimal conductive impairment. In the minimal conductive hearing impairment group we substantiated the findings of others that the negative on-off reflex is common in otosclerotic patients whose conductive impairment is 10dB or less. In the group with cochlear otosclerosis we found abnormal reflexes in 58 percent of the patients. These findings indicate that stapedial involvement is common in patients thought to have pure sensorineural otosclerosis.
Adolescent↗
[Obliterative otosclerosis].
In true (grade IV) obliterative otosclerosis, the limits of the oval windows are lost. The insertion of a prosthesis requires drilling and surgical ability. Thirty eight cases of the true obliterative otosclerosis operated in our Department between 1974 and 1992 have been reviewed. Only 6.2% of all the patients operated by us with the diagnosis of otosclerosis had true obliterative otosclerosis. The average preoperative gap in conversational frequencies was 39 dB. In general, the gap closure obtained (13 dB) was slightly lesser than that for otosclerosis. Reobliteration of the oval window may occur in this type of otosclerosis.
Audiometry↗
[Pathogenesis of otosclerosis. "State of the art"].
Women suffer from otosclerosis 1.6 times more often than males. Histologically, otosclerotic foci can be found in temporal bones of females 1.9 times more often than in those of males. Characteristic topographic regions are the oval window, round window niche and promontory. Otosclerosis can also occur principally in any area of the enchondral/periosteal layer of the otic capsule. Evidence is presented that otosclerosis is an inflammatory tissue reaction associated with macrophages, T- and B-lymphocytes, HLA-DR positive cells and plasma cells. Dependent on the stage of the osteolytic bone disease present deposits of complement and immunoglobulins (IgG, IgA) can be found. These immunoglobulins have been identified as antibodies to measles virus proteins. Using the polymerase chain reaction we were successful in demonstrating RNA sequences of measles viruses in otosclerotic bone from footplates removed during stapes surgery. Since most of the otosclerotic lesions were in direct contact to the perilymphatic space, it may be expected that the endolymphatic sac--as the immune competent organ of the inner ear--specifically reacts to antigens delivered from the otosclerosis focus into the perilymph. Perilymph samples from patients were collected during stapes surgery and their antibody titers against measles were compared with that in corresponding blood serum. All samples revealed a significantly elevated-specific anti-measles IgG amount which was significantly higher than in the corresponding serum. In contrast, antibody titer in the perilymph against herpes simplex or cytomegalo viruses did not differ from that of the serum. These findings indicate that otosclerosis is a measles virus associated inflammatory osteolytic disease of the temporal bone. Since women suffer from severe measles virus infections more often than males, it can be hypothesized that females have a higher susceptibility of their cochleo-vestibular tissues to these infections (organotropism). In addition, estrogens are well-known stimulators of osteocytic activity and may play a dominant role during ossification of an otospongeotic bone lesion. This may explain the onset of a conductive hearing loss due to otosclerosis during pregnancy.
Adult↗