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At least 19 recordsLinked to original sources
PubMedDate not supplied

Otosclerosis and cochlear otosclerosis: a post mortem study on temporal bones.

We have chosen among many temporal bones of donors deceased individuals with concomitant otosclerosis, three particular cases, one with classic otosclerosis, another with cochlear otosclerosis with concomitant oval window ankylosis and another with cochlear otosclerosis without stapes fixation. The different histopathologic features are discussed and clinical and therapeutical guidelines are proposed.

Cochlear Diseases↗
PubMed2001

Histologic studies of the posterior stapediovestibular joint in otosclerosis.

OBJECTIVE: To determine the prevalence of ankylosis or otosclerosis at the posterior stapediovestibular joint (SVJ) in temporal bones with otosclerosis, with special reference to stapes surgery. BACKGROUND: Long-term success of the laser stapedotomy minus prosthesis (STAMP) procedure, anterior crurotomy, and similar partial stapedectomy procedures depends on lack of ankylosis and lack of otosclerosis involving the posterior SVJ. Previous work has shown that the air-bone gap in otosclerosis correlates with narrowing and loss of the SVJ space. However, the prevalence and histologic features of otosclerotic involvement of the posterior SVJ space have not been well characterized. METHODS: Histologic assessment of serial sections through the oval window niche in 140 temporal bones with otosclerosis that had been sectioned in the axial plane (age range 20-95 years, mean 68). Bones with stapes mobilization or stapedectomy were excluded. RESULTS AND CONCLUSIONS: Two of 140 bones had otosclerosis exclusively at the posterior SVJ. Of the remaining 138 bones, all of which had otosclerosis at the anterior SVJ, 82 bones also had otosclerosis at the posterior joint. Of the 56 bones without otosclerosis of the posterior joint, there was bony ankylosis of the posterior joint in 3 bones. Thus, 53 bones (38%) had neither ankylosis nor otosclerosis involving the posterior joint, and they would be potentially suitable for a laser STAMP or a similar procedure. There was no correlation between otosclerosis at the posterior SVJ and age, sex, or duration of conductive hearing loss. Otosclerosis at the posterior joint in one ear was significantly associated with its presence at the posterior joint in the opposite ear (p = 0.01). The audiogram could not be used to reliably predict otosclerotic involvement of the posterior SVJ or the degree of footplate pathologic changes, such as ankylosis.

Adult↗
PubMed2001

Prevalence of otosclerosis in an unselected series of temporal bones.

BACKGROUND: Histologic otosclerosis is a disease process without clinical symptoms or manifestations that can be discovered only by sectioning of the temporal bone at autopsy. Clinical otosclerosis is otosclerosis at a site where it causes conductive hearing loss by interfering with the motion of the stapes or of the round window membrane. Various authors have studied the prevalence of histologic otosclerosis on laboratory collections of temporal bones. Some 12% to 15% of temporal bones with histologic otosclerosis have demonstrated stapedial fixation. Using these figures for calculating the prevalence of clinical otosclerosis gives an extrapolated clinical prevalence of 0.99% to 1.2%. This does not correlate well with the clinical data on otosclerotic families, from which a clinical prevalence of 0.3% has been estimated. OBJECTIVE: To study the prevalence of histologic otosclerosis in an unselected series of temporal bones. STUDY DESIGN: During a 1-year period, 118 consecutive pairs of temporal bones of deceased patients at a tertiary care center were collected to determine the prevalence of otosclerosis. Although histology remains the gold standard for evaluation of otosclerosis, the gross observation of temporal bone slices combined with microradiography was used to screen for otosclerotic lesions more rapidly and with a lower cost/benefit ratio. The temporal bones, which were suspected of having otosclerosis with these techniques, were further analyzed by conventional histology. RESULTS: 2.5% of the 236 temporal bones (or 3.4% of patients) studied demonstrated histologic otosclerosis. CONCLUSIONS: Although the prevalence of 2.5% is much lower than previously published figures on histologic otosclerosis, the extrapolated data (extrapolated clinical prevalence = 0.30% to 0.38%) correlate well with clinical studies of otosclerotic families. The previous studies based on laboratory collections were likely biased by hearing loss or other otologic diseases.

Adolescent↗
PubMed1991

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↗
PubMed1996

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↗
PubMed2001

Shift of the distribution of age in patients with otosclerosis.

Morphological and biochemical investigations have shown evidence of an association between measles virus and otosclerosis. Epidemiological analysis of age and gender distributions in the 1960s and 1970s revealed a higher incidence of otosclerosis in women, the average age of onset of clinical disturbances and need for surgery being between 15 and 40 years. In the late 1960s and early 1970s a campaign to vaccinate children against measles was started in Germany. The aim of our study was to evaluate whether this campaign has had any influence on the distributions of the age and gender of patients affected by otosclerosis over the past 20 years. The study included patients suffering from clinical otosclerosis who had undergone stapedectomy between 1978 and 1999 and whose clinical data were complete (n = 1351). Statistical analysis during the recruitment period indicated a significant increase in the average age of the otosclerosis patients (p = 0.012). With regard to the gender distribution it was found that the increase of otosclerosis in women compared to men was statistically insignificant (p = 0.418). These data strongly support the hypothesis of a measles virus involvement in otosclerosis and may reflect a decreased incidence of otosclerosis in the generation of patients vaccinated against measles virus.

Adolescent↗
PubMed2002

Similar COL1A1 expression in fibroblasts from some patients with clinical otosclerosis and those with type I osteogenesis imperfecta.

Because of the clinical and histopathologic similarities between otosclerosis and type I osteogenesis imperfecta, we examined COL1A1 messenger RNA (mRNA) expression in cultured fibroblasts from patients with clinical otosclerosis to determine whether abnormalities of expression of COL1A1 were present, as has been reported in type I osteogenesis imperfecta. Type I osteogenesis imperfecta has been found to result from mutations in the COL1A1 gene that result in null expression of the mutant allele. Patients with clinical otosclerosis were genotyped for the presence of an expressed 4 base-pair insertion polymorphism in the 3' region of the COL1A1 gene. Skin biopsies were performed, and cultured fibroblast cell lines were established from patients who were heterozygous for the polymorphism. Allelic expression was examined by reverse transcription-polymerase chain reaction and silver-stained polyacrylamide gel electrophoresis. Two of 9 patients with clinical otosclerosis demonstrated null or reduced expression of one COL1A1 allele. The differential expression of the two COL1A1 alleles in all subjects was also examined by a semiquantitative method using an ABI Prism 7700 Sequence Detection System (Taqman). We did this examination to determine whether milder abnormalities in COL1A1 expression might account for the development of otosclerosis in the 7 clinical cases that did not reveal evidence of null expression by the gel technique. Of the same 2 cases of otosclerosis that demonstrated evidence of null expression by gel electrophoresis, both were found to have significant differences in COL1A1 mRNA expression by the Taqman analysis. The remaining 7 cases revealed equal expression of the two COL1A1 alleles similar to that seen in controls. These results suggest that mutations in COL1A1 that are similar to those that occur in type I osteogenesis imperfecta may account for a small percentage of cases of otosclerosis, and that the majority of cases of clinical otosclerosis are related to other genetic abnormalities that have yet to be identified.

Alleles↗
PubMed1999

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↗
PubMedDate not supplied

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↗
PubMed2002

The anterior and superior malleal ligaments in otosclerosis: a histopathologic observation.

HYPOTHESIS: The aim of this study was to assess, in otosclerosis, whether the anterior and superior malleal ligaments show histologic changes that can lead to reduced malleal mobility and eventual fixation, and also to evaluate whether these changes are related to the degree of histologic otosclerosis. BACKGROUND: Fixation of the malleus seems to be one of the most controversial clinical entities in the acquired condition of otosclerosis. It has even been postulated that persistent conductive deafness, or progression of conductive deafness after initial improvement after stapedotomy, could be due to unsuspected malleus fixation. METHODS: Fifty eight temporal bones with known otosclerosis and 43 normal temporal bones were selected. In addition, 10 temporal bones of fetuses and children were also studied. Otosclerosis of the footplate and otic capsule was graded as none, mild, moderate, and severe. The histologic changes in the ligaments also were graded from none to severe. RESULTS: The median ages of patients in the otosclerotic and normal groups were 62 and 60 years, respectively. In the anterior malleal ligament of the otosclerotic bones, 10% mild (+), 60% moderate (++), and 30% severe (+++) degrees of hyalinization were observed. In the anterior malleal ligament of the nonotosclerotic bones, 14% showed no hyalinization, 24% had only a tinge of hyalinization (minimal), 51% had mild (+) hyalinization, and 11% had moderate (++) hyalinization. Superior ligament hyalinization appears to be related to the severity of anterior ligament hyalinization. The severity of otosclerosis in the footplate or the otic capsule did not appear to be related to the severity of hyalinization. CONCLUSION: From this study, it is apparent that otosclerotic bones have a significantly high incidence of hyalinization of the anterior malleal ligament. This seems to be related to the duration rather than the severity of otosclerosis. It is important to properly evaluate malleal mobility during all stapes surgery.

Adult↗
PubMed2003

Do the genes that cause otosclerosis reduce susceptibility to otitis media?

HYPOTHESIS: The genetic factors that cause otosclerosis reduce susceptibility to otitis media. BACKGROUND: Susceptibility to some infectious diseases is modulated by host genetic factors. Genes that reduce the morbidity and mortality of infectious diseases may confer a selective advantage and achieve high-frequency in at-risk populations. Acute otitis media in the pre-antibiotic era frequently led to complications with high morbidity and mortality. The long-term sequela of acute otitis media is chronic otitis media. Chronic otitis media has a prevalence of 0.5 to 2 percent in the Caucasian population. Clinical otosclerosis occurs in 1 percent of Caucasians. Histologic otosclerosis occurs in 10 percent of Caucasians. MATERIALS AND METHODS: Retrospective analysis of 2,362 subjects with surgically confirmed otosclerosis. Subjects' medical records were reviewed for evidence of chronic otitis media. The incidence of chronic otitis media in the general population was compared with the subject population. RESULTS: Of the 2,362 subjects with surgically confirmed otosclerosis, one subject with evidence of chronic otitis media was identified. The incidence of chronic otitis media in the subject population is 0.04 percent (p < 0.0001). CONCLUSIONS: The incidence of chronic otitis media in subjects with a surgically confirmed diagnosis of otosclerosis is significantly lower than expected. The genes that cause otosclerosis may confer resistance to the pathogens that cause chronic otitis media and/or acute otitis media. The mechanism of resistance is unknown.

Acute Disease↗
PubMed1998

[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↗