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PubMed2005

Effectiveness assessment of otosclerosis surgery.

CONCLUSIONS: The epidemiological characteristics of otosclerosis and its treatment in Andalusia resemble those of other populations with similar socioeconomic levels. Two complementary approaches, such as questionnaires and pure-tone audiometry, are required to assess the effectiveness of otosclerosis surgery (OS) reliably and precisely. OBJECTIVES: We describe a new method to assess effectiveness in OS. It is based on the results of pure-tone audiometry and a specially designed quality of hearing questionnaire (QHQ). The objectives of the study are: (i) to report the general epidemiologic profile of otosclerosis in Andalusia; (ii) to study the effectiveness of OS in our community using conventional methods; and (iii) to study the outcomes of OS using the QHQ and to compare them to those obtained using conventional methods. MATERIAL AND METHODS: All 31 hospitals in the public healthcare system of Andalusia were studied. They were graded into four groups using a specially designed grouping system. The data were obtained from the minimum basic dataset. The prevalence of otosclerosis in Andalusia was calculated from the incidence data, the duration of the disease and life expectancy. To assess the effectiveness of OS, 475 clinical records from 15 hospitals representing all 4 groups were analysed. Effectiveness was assessed by conventional methods, using data obtained from pure-tone audiometry, and by using version 1.02 p of the QHQ. RESULTS: The incidence of clinical otosclerosis was 5.67 patients/100,000 inhabitants/year. The calculated prevalence was 0.287%. The number of cases increased progressively during the study period (p<0.001). The 15-45-year age group was the largest (62.2%) and 68.4% of patients were females. The most frequent type of otosclerosis was estapediovestibularis (fenestral), non-obliterative (91.8%). Only 48 cases (2.3%) of cochlear and 45 (2.2%) of obliterative otosclerosis were reported. The most frequently employed therapeutic procedures were stapedectomy and stapedotomy (75.70%). The average total and preoperative lengths of stay were 3.59 and 1.04 days, respectively. There were significant differences between the different types of otosclerosis. Improvement in the air-bone gap was 15.37+/-1.19 dB (n=164) and the overclosure or operative damage was 0.49+/-0.85 dB (n=164). A gap improvement of 10-40 dB was observed in 61.4% of patients. The > 65 years age group showed the best gap improvement but the largest variability. The quality of hearing measured by the QHQ showed that, in general, a better gap improvement was associated with a higher quality of hearing (Pearson correlation r=0.183; p<0.05). The 15-45-year age group had the worst gap improvement but, in contrast, the better quality of hearing.

Adolescent↗
PubMed2006

Audiometric analyses confirm a cochlear component, disproportional to age, in stapedial otosclerosis.

OBJECTIVE: To report the preoperative audiometric profile of surgically confirmed otosclerosis. STUDY DESIGN: Retrospective, multicenter study. SETTING: Four tertiary referral centers. PATIENTS: One thousand sixty-four surgically confirmed patients with otosclerosis. INTERVENTIONS: Therapeutic ear surgery for hearing improvement. MAIN OUTCOME MEASURES: Preoperative audiometric air conduction (AC) and bone conduction (BC) hearing thresholds were obtained retrospectively for 1064 patients with otosclerosis. A cross-sectional multiple linear regression analysis was performed on audiometric data of affected ears. Influences of age and sex were analyzed and age-related typical audiograms were created. Bone conduction thresholds were corrected for Carhart effect and presbyacusis; in addition, we tested to see if separate cochlear otosclerosis component existed. Corrected thresholds were than analyzed separately for progression of cochlear otosclerosis. RESULTS: The study population consisted of 35% men and 65% women (mean age, 44 yr). The mean pure-tone average at 0.5, 1, and 2 kHz was 57 dB hearing level. Multiple linear regression analysis showed significant progression for all measured AC and BC thresholds. The average annual threshold deterioration for AC was 0.45 dB/yr and the annual threshold deterioration for BC was 0.37 dB/yr. The average annual gap expansion was 0.08 dB/year. The corrected BC thresholds for Carhart effect and presbyacusis remained significantly different from zero, but only showed progression at 2 kHz. CONCLUSION: The preoperative audiological profile of otosclerosis is described. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself. Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis.

Adult↗
PubMed2006

Surgical findings and long-term hearing results in 3,050 stapedotomies for primary otosclerosis: a prospective study with the otology-neurotology database.

OBJECTIVE: To evaluate with a new otologic database the results of primary stapes surgery for otosclerosis with up to 14 years of follow-up in a consecutive series of 2,525 patients operated on by the same surgeon with the same technique (stapedotomy and vein graft interposition) and to provide online access to the complete data of this study for the reviewers. To study the effect of specific operative findings (obliterative otosclerosis and simultaneous malleus ankylosis) and age at the time of surgery on the long-term outcome. STUDY DESIGN: Prospective clinical study using a new computerized otologic database. SETTING: : Tertiary referral center. PATIENTS: Two thousand five hundred twenty-five patients who underwent 3,050 stapedotomies for otosclerotic stapes fixation were enrolled in this study from January 1991 to December 2004. Separate analyses were made for two unique pathologies (92 cases of obliterative otosclerosis and 19 cases of simultaneous malleus ankylosis) diagnosed during surgery and for patients in two age brackets ( or=65 yr [302 patients]). INTERVENTION: Stapedotomy with vein graft interposition and reconstruction with either a Teflon piston, a bucket handle prosthesis, or a total prosthesis. MAIN OUTCOME MEASURES: Preoperative and postoperative audiometric evaluation using conventional audiometry. Air-bone gap (ABG), bone-conduction thresholds, and air-conduction thresholds were all assessed. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months and then annually for 14 years. RESULTS: Overall, the postoperative ABG was closed to 10 dB in 94.2% of cases. The mean four-frequency postoperative ABG was 1.7 dB compared with 25.6 dB preoperatively. The mean four-frequency bone-conduction thresholds were unchanged postoperatively. A significant postoperative sensorineural hearing loss (SNHL; >15 dB) was seen in 0.5% of cases in this series. Postoperative ABG was achieved to within 10 dB in 95% of cases of obliterative otosclerosis and in 64.7% of cases of simultaneous malleus ankylosis. A significant postoperative SNHL (>15 dB) was seen in 4.8% of cases of obliterative otosclerosis and was not observed in any cases of simultaneous malleus ankylosis. Postoperative ABG was achieved to within 10 dB in 93.5% of cases in the pediatric series and in 94.5% of cases in the senior series. A significant postoperative SNHL (>15 dB) was seen in 0.7% of cases in the senior group but was not observed in the children. CONCLUSION: Using a new otologic database, our series confirms that stapedotomy with vein graft interposition for otosclerotic stapes fixation is a safe and successful treatment for long-term hearing improvement. The deterioration in hearing with time after stapedotomy did not exceed the rate of hearing loss because of presbyacusis. Therefore, argon laser stapedotomy with vein graft interposition is our preferred surgical technique in the treatment of otosclerosis. Obliterative otosclerosis and simultaneous malleus ankylosis may be encountered during stapedotomy. Our study shows that reasonable success rates can still be expected in these situations. Stapedotomy results in the elderly and in children are comparable to those obtained in patients of other groups of age undergoing surgery for otosclerosis without an increased risk for complications.

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↗
PubMed2005

Otosclerosis among South African indigenous blacks.

OBJECTIVE: To report cases of clinical otosclerosis histologically confirmed among indigenous South African blacks. DESIGN: A retrospective study. SETTING: Referral tertiary center, MEDUNSA, Garankuwa Hospital, South Africa. SUBJECTS: All fifteen indigenous South African blacks diagnosed with clinical otosclerosis at Garankuwa Hospital between January 1993 and January 2003 in the Ear, Nose and Throat Department. INTERVENTIONS: A retrospective study of the records of all patients seen at the Ear, Nose and Throat (ENT)outpatient clinic of GaRankuwa Hospital between January 1993 and January 2003 was undertaken. In addition, data was obtained from operating theatre records and histology reports. The files of these patients with a diagnosis of otosclerosis were reviewed for details on demographics, symptoms, audiometric test results, radiology, surgical and pathology reports. All of these patients were included in the study. RESULTS: From a weekly outpatient attendance of about 300 patients over a ten-year period, a total number of fifteen patients were identified with a definite diagnosis of clinical otosclerosis. Nine of them were confirmed surgically and histologically as having otosclerosis. These nine cases consisted of five females and four males, the mean age for females was thirty-five years and for males forty-seven years. They all presented with a progressive painless hearing loss with no ear discharge or previous surgery with negative family history. Audiometry confirmed a mixed hearing loss with a Carrhart notch at 2kHz typical for otosclerosis and all had normal tympanic membrane. All of these patients had a unilateral stapedectomy done. Schucknet wire prosthesis was used for surgical reconstruction. The outcome of surgery in all of these patients was satisfactory. The other six patients are still awaiting surgery. CONCLUSION: This study confirms the existence of otosclerosis among indigenous South African blacks. Otosclerosis should be included in the differentials of a conductive hearing loss in blacks.

Adult↗
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↗
PubMed2006

Association between cupular deposits and otosclerosis.

OBJECTIVE: To evaluate whether otosclerosis is an underlying mechanism for the production of cupular deposits and to study the association between cupular deposits and dysequilibrium in otosclerosis. DESIGN: Retrospective human temporal bone (TB) study. The incidence of cupular deposits in these 70 TBs was analyzed. Correlations between cupular deposits and vestibular symptoms, endosteal involvement of the otosclerotic focus, stapedial fixation, and clinical history of stapes surgery were evaluated. SETTING: Otolaryngology laboratory in a tertiary academic medical center. PATIENTS: The study material consisted of 35 human TBs with otosclerosis and 35 age-matched controls. MAIN OUTCOME MEASURES: Morphometric evaluations of the incidence of cupular deposits, endosteal involvement of the otosclerotic focus, and stapedial fixation were made by light microscopy. Clinical records were reviewed retrospectively for clinical history of stapes surgery and prevalence of vestibular symptoms. The incidence of cupular deposits was compared between the otosclerotic and control groups. Correlations between cupular deposits and vestibular symptoms, endosteal involvement of the otosclerotic focus, stapedial fixation, and clinical history of stapes surgery were evaluated in the subjects with otosclerosis. RESULTS: The incidence of cupular deposits in TBs with otosclerosis was significantly higher than in those without whereas there was no correlation between the incidence of the deposits and dysequilibrium in cases of otosclerosis. An increase in deposits did not correlate with stapedial fixation, stapes surgery, or endosteal involvement. CONCLUSIONS: Our results suggest otosclerosis as an underlying mechanism for the production of cupular deposits; however, we did not find an association between these deposits and vestibular symptoms.

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↗
PubMed2005

Cochlear implantation in otosclerosis.

OBJECTIVE: The purpose of this study was to evaluate and compare the results obtained in a group of implanted otosclerotic patients with a group of cochlear implant (CI) patients not affected by otosclerosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Nine patients affected by profound sensorineural hearing loss caused by otosclerosis and nine patients affected by profound sensorineural hearing loss not caused by otosclerosis were evaluated. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Preoperative imaging, intraoperative findings, postoperative facial nerve stimulation, and speech perception performance measures were performed and the data analyzed. RESULTS: Otosclerosis patients showed signs of cochlear ossification both on high-resolution computed tomography scans and intraoperatively. The incidence of facial nerve stimulation was higher in the otosclerosis group, three out of nine, and was generally related to the use of electrical stimulation from the Nucleus 22 cochlear implant. Psychophysical and speech perception measures did not show significant differences between the two groups, despite some otosclerosis patients showing increased electrical thresholds and comfort levels and slightly poorer speech perception performance scores. CONCLUSIONS: Patients with otosclerosis who have progressed to profound hearing loss derive significant benefit from cochlear implants; however, an increased risk of cochlear ossification and facial nerve stimulation has to be taken in account during preoperative counseling. The advance in imaging techniques, CI technology and the possibility to stimulate precise regions of the cochlea with lower intensities make it possible for the surgeons and audiologists to readily and successfully manage these complications as they arise.

Adolescent↗
PubMed2005

Cochlear implantation in cochlear otosclerosis.

OBJECTIVES/HYPOTHESIS: The objective was to correlate implant performance in cochlear otosclerosis to 1) matched control samples, 2) severity of otic capsule involvement, 3) prior ipsilateral surgery, and 4) programming issues. STUDY DESIGN: Retrospective case controlled study. METHODS: Study cohort comprised 30 individuals. Diagnosis was based on prior ear surgery (stapedectomy [n = 18] or fenestration [n = 2]) and/or pathognomonic radiological findings. High-resolution computed tomography images of the temporal bones were assessed by two radiologists and graded (range, 0-3) for the extent of otosclerosis. Operative records were reviewed. Performance, programming visits, and the number of electrode deactivations at 6 months and at 1 year after implantation were determined for the individuals with otosclerosis and compared with a group of matched control subjects. A within-group comparison correlating severity of otosclerosis to the above was carried out. RESULTS: Implant performance in individuals with cochlear otosclerosis was not significantly different from those without. Previous surgery on the side of implantation did not alter performance. Programming difficulty as reflected in the number of visits and electrode deactivation for sound quality reasons were comparable. Deactivation for facial nerve stimulation occurred exclusively in otosclerotics with the most severe radiological disease (grade 3) and was only with non-modiolar hugging electrodes (n = 5). There was no observed difference between the radiological extent of otosclerosis and implant performance. CONCLUSION: Individuals with severe otosclerosis considering cochlear implantation can be counseled to expect similar benefit to those without, regardless of whether prior surgery occurred on the side of implantation or of severity of otic capsule involvement. There is a significant risk of facial nerve stimulation in otosclerotics with grade 3 disease.

Case-Control Studies↗
PubMed2006

Activated osteoclasts with CD51/61 expression in otosclerosis.

HYPOTHESIS: Stapes ankylosis is supposed to be a disease with variable histopathology caused by otosclerosis or pseudo-otosclerosis. Persistent measles virus infection of the otic capsule could induce reactivation of quiescent embryonic osteoclasts in otosclerosis. BACKGROUND: Presence of measles virus RNA was demonstrated in the footplates of otosclerotic patients by reverse-transcription polymerase chain reaction (RT-PCR). Histology of active otosclerosis is featured by the presence of numerous osteoclasts with unknown phenotype. METHODS: Nucleic acid was extracted from stapes footplates of clinically otosclerotic patients (n = 261). Genomic RNA of measles virus was amplified by RT-PCR. Amplification results were correlated to postoperative histologic and CD51/61 specific immunohistologic findings. A parallel alcalic phosphatase activity assessment was performed to evaluate the metabolic activity of osteoclasts in each section. RESULTS: Among 261 stapes fixation cases, 175 otosclerotic stapes contained measles virus RNA. Histology for virus negative stapes (n = 86) represented nonotosclerotic, degenerative disorders. Histologically confirmed otosclerosis was featured by the presence of osteoclasts with renewed, embryonic phenotype. In otosclerosis, alcalic phosphatase activity was significantly higher compared with nonotosclerotic stapes ankylosis (P < .001). CONCLUSION: The presence of CD51/61 positive osteoclasts in otosclerotic bone containing viral sequences provides the basis for an inflammatory bone remodeling disorder. Otosclerosis is a disease caused by persistent measles virus infection and reactivation of resting embryonic osteoclasts in the otic capsule.

Adult↗
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↗
PubMed2000

Persistent measles virus infection as a possible cause of otosclerosis: state of the art.

The etiopathogenesis of otosclerosis is still largely unexplained and remains controversial. Morphologic examinations have shown the presence of a chronic inflammation in otosclerotic tissue. Among the proposed explanations for this inflammation are an immunologic reaction against collagen, mutations of collagen gene 1A1, and a viral infection. In this paper, we focus on the role of measles virus in otosclerosis, and we review the current literature, devoting particular attention to a suspected paramyxoviral etiopathogenesis in Paget's disease. Our examination of footplate fragments by reverse transcription polymerase chain reaction testing in 95 patients with otosclerosis revealed the presence of measles virus RNA in 83% of cases. Quantification of measles virus immunoglobulin G (IgG) in otosclerosis patients indicated that the ratio of antimeasles virus IgG in total IgG was higher in perilymph than in serum. Furthermore, an almost identical incidence of otosclerosis and measles virus-caused mortality in women suggests that women are more susceptible to measles virus infection. Finally, since the introduction of the measles virus vaccination program in Europe, there has been a decline in the incidence of otosclerosis. Moreover, the average age of patients at diagnosis and surgery at our hospital has increased to 54 years. Our findings, when they are considered along with findings regarding the presence of paramyxoviral RNA in Paget's disease, support the hypothesis that measles virus is involved in the etiopathogenesis of otosclerosis.

Adolescent↗
PubMed1977

[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↗
PubMed2006

[Analysis of results of stapedotomy in patients with obliterative otosclerosis].

INTRODUCTION: In obliterative type of otosclerosis the stapes footplate is greatly thickened and diffusely replaced by a massive otosclerotic masses that fill in the oval window niche, often annular ligament cannot be visualized. In the case of obliterative otosclerosis the only effective way of treatment is surgery. MATERIAL AND METHODS: The analysed group consisted of 53 patients with primary obliterative otosclerosis. In the control group there were 30 consecutive patients treated for otosclerosis. The auditory organ was assessed on the ground of the following criteria for the frequencies 0.5, 1, 2 and 4 kHz.: 1. Change of pure tone thresholds of air conduction (AC) 2. Change of pure tone thresholds of bone conduction (BC) 3. Change of pure tone thresholds of air-bone gap (ABG) as the difference between the mean AC and BC thresholds before the operation (AC0 and BC0) and 12 months after the operation (AC1 and BC1). RESULTS: Postoperatively mean value of change of AC thresholds in the analysed group was about 24 +/- 2 dBHL and in the control group about 25 +/- 2 dBHL (no significant difference). In the analysed group the mean value of BC thresholds varied about 8 dBHL and in the control group about 11 dBHL (no significant difference). The average values of ABG after the operation varied about 14 +/- 1 dBHL in both--the analysed and the control group. Postoperatively the mean value ABG was amounted to 20 +/- 1 dBHL in the analysed group and in the control group to 18 +/- 1 dBHL (no significant difference). On the basis of the subjective assessment completely subsidence of aural tinnitus reported 70% patients in the analysed group and 80% patients in the control group. CONCLUSION: 1. The incidence of obliterative otosclerosis represents 9,6% of all otosclerotic patients treated surgically. 2. Stapedotomy is the treatment of choice in patients with obliterative otosclerosis, because significant improvement of AC, BC and ABG 12 months after the operation was reported, mean values did not differ between the above and control group. 3. Complete subsidence of aural tinnitus was reported in 70% patients with obliterative otosclerosis.

Adult↗
PubMed1993

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↗