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At least 19 recordsLinked to original sources
PubMed2004

Questioning the relationship between cochlear otosclerosis and sensorineural hearing loss: a quantitative evaluation of cochlear structures in cases of otosclerosis and review of the literature.

BACKGROUND: The literature provides conflicting information regarding the prevalence and cause of sensorineural hearing loss (HL) in individuals with otosclerosis. OBJECTIVE: The purpose of this study was to further evaluate the association between involvement of the cochlear endosteal layer with otosclerosis and sensorineural HL. STUDY DESIGN: Retrospective case review. METHODS: Temporal bones and audiograms from five individuals with otosclerosis were evaluated. The cochlear elements were quantified. The location and extent of the cochlear element loss was correlated with the location and extent of endosteal involvement with otosclerosis. RESULTS: A reduction in the population of cochlear elements was observed in most individuals; however, the reduction was not proportional to the extent of endosteal involvement with otosclerosis. The cochlear elements remained normal adjacent to some areas of endosteal involvement with otosclerosis. One individual with extensive cochlear otosclerosis had normal hearing and predominantly normal cochlear elements. CONCLUSION: This study demonstrates a variable amount of degeneration of the cochlear elements in individuals with otosclerotic involvement of the endosteum. The reduction in the population of cochlear elements was not related to the extent and location of endosteal involvement with otosclerosis. These findings suggest that factors that limit the effect of otosclerotic endosteal involvement on the cochlear elements or processes that effect the cochlear elements directly and are independent of bone involvement may be present.

Aged↗
PubMed2005

Two subgroups of stapes fixation: otosclerosis and pseudo-otosclerosis.

HYPOTHESIS: Stapes ankylosis is a disease with variable histopathology and can be caused by otosclerosis or pseudo-otosclerosis. Viral pathogenesis of otosclerosis could be established only by correlative analysis: histologic examination of the stapes footplate and reverse-transcriptase polymerase chain reaction (RT-PCR) amplification of the viral RNA. BACKGROUND: Presence of the RNA genome of measles virus was demonstrated in the footplates of clinically otosclerotic patients by RT-PCR, and also viral proteins were detected by immunohistochemistry. METHODS: Nucleic acids were extracted from ankylotic stapes footplates of clinically stapes fixation patients (n = 104). Measles virus genomic nucleoprotein (NP) RNA was amplified by seminested RT-PCR. Amplification results were correlated to postoperative histologic and audiologic findings. RESULTS: Measles virus RNA was detectable only in histologically otosclerotic stapes footplates (n = 67). Histology for virus negative footplates (n = 37) excluded otosclerosis. Virus negative stapes footplates showed nonotosclerotic, degenerative disorders. CONCLUSIONS: Stapes ankylosis is a heterogeneous disease causing conductive hearing loss with different etiologies. Nonotosclerotic stapes fixations could be established as pseudo-otosclerosis and may belong to nonspecific, degenerative disorders with variable and noncharacteristic histopathology. Otosclerosis is an inflammatory disease caused by persisting measles virus infection of the otic capsule.

Adult↗
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↗
PubMed2006

Current research in otosclerosis.

PURPOSE OF REVIEW: The aim of this article is to summarize and put into historical perspective current advances in research in otosclerosis, a disorder of the human temporal bone with a hereditary predisposition that is among the most common causes of acquired hearing loss. RECENT FINDINGS: Genetic studies have revealed that otosclerosis is heterogeneous, with evidence for defects in at least seven genes associated with six distinct chromosomal loci. Measurements of high levels of osteoprotegerin expression in the normal otic capsule and soft tissues of the cochlea provide the first molecular insight as to why the normal otic capsule remodels minimally, if at all. Osteoprotegerin knockout mice provide the best available animal model to date to study abnormal otic capsule remodeling that closely resembles otosclerosis. There is mounting evidence that the measles virus plays an important role in pathogenesis of otosclerosis although the mechanisms by which the virus results in otosclerosis remain unknown. Quantitative measures of angiogenesis can reliably distinguish between clinical and histological otosclerosis. Advances in the emerging field of osteoimmunology will likely impact and benefit from the research in otosclerosis. SUMMARY: Insights into molecular mechanisms that inhibit extensive remodeling in the normal otic capsule, and understanding of how these mechanisms are dysregulated in otosclerosis will allow future design of rational treatment strategies for otosclerosis.

Angiogenesis Inducing Agents↗
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↗
PubMed2006

Chromosomal mapping and phenotypic characterization of hereditary otosclerosis linked to the OTSC4 locus.

OBJECTIVE: To perform chromosomal mapping and clinical analysis of hereditary otosclerosis linked to the fourth locus for otosclerosis (OTSC4) in an Israeli family. DESIGN: Pedigree study. SETTING: A genetics of hearing loss research laboratory, a clinical genetics laboratory, a center for speech and hearing, and an otolaryngology department at a university and medical centers in Israel. SUBJECTS: An Israeli family of which 24 members were ascertained and a pedigree was constructed; 12 members had otosclerosis. INTERVENTIONS: Confirmation of otosclerosis by surgery (3 subjects) and by audiologic evaluation, medical history, and family history (9 subjects), and whole-genome scanning to identify the chromosomal region of the mutant locus. MAIN OUTCOME MEASURES: Chromosomal location of the otosclerosis locus. RESULTS: Linkage to the 16q21-23.2 interval was identified and confirmed with a logarithm of odds (LOD) score of 3.97 at theta = 0. The new locus for otosclerosis was designated OTSC4. The OTSC4 interval of 9 to 10 megabase includes several genes involved in the immune system and bone homeostasis that may be good candidates for genes otosclerosis. CONCLUSION: The elucidation of the OTSC4 gene may disclose the etiology of the disorder, and the functional and structural analysis of the protein may open new options for diagnosis, treatment, and prevention of otosclerosis.

Adult↗
PubMed2005

Monogenic nonsyndromic otosclerosis: audiological and linkage analysis in a large Greek pedigree.

OBJECTIVE: The aim of our study was to characterize the hearing impairment in a large multigenerational Greek family with autosomal dominant nonsyndromic otosclerosis and to perform genetic linkage analysis to known otosclerosis loci and collagen genes. In addition, we looked for mutations in the NOG gene to rule out congenital stapes ankylosis syndrome. METHODS: Audiological analysis of the affected persons was based on multiple linear regression (MLR) analysis and construction of age-related typical audiograms (ARTA). Genotyping of microsatellite DNA polymorphisms for known otosclerosis (OTSC) loci or collagen genes and linkage analysis using the MLINK computer program were performed. The coding region of the NOG gene was screened for mutations by direct DNA sequencing. RESULTS: The hearing loss in this family appears in childhood as conductive, but soon becomes mixed. Because the additional sensorineural component is progressive, this finally has lead to a pure sensorineural hearing loss in some family members, as the conductive component is masked. Audiological analysis showed an age-independent conductive component and a progressive frequency-specific sensorineural component. Linkage analysis excluded linkage to the four known otosclerosis loci (OTSC1, OTSC2, OTSC3, and OTSC5), as well as to the COL1A1 and COL1A2 genes. Mutation analysis of the coding region of the NOG gene did not reveal any disease causing mutation. CONCLUSIONS: This study represents the first description of a detailed audiological analysis in a large pedigree segregating otosclerosis as a monogenic autosomal dominant trait. Exclusion of the four known otosclerosis loci in this family shows that monogenic otosclerosis is a genetically heterogeneous disease involving at least five different genes. A mutation in the NOG gene is not the underlying molecular mechanism of the early onset otosclerosis segregating in this family.

Ankylosis↗
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↗