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PubMed1984

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↗
PubMed2005

[Is imaging possible for otosclerosis? Characteristics of the disease and a survey of the clinical relevance of imaging].

Otosclerosis is a localized progressive disease of bone remodeling within the otic capsule of the human temporal bone. Histomorphologically, active cochlea otosclerosis (otospongiosis) is diagnosed in the presence of foci of demineralization in the otic capsule. The clinical symptoms of otosclerosis are associated with mixed and sensorineural hearing loss. With recent technological advances, diagnostic imaging of the inner ear is becoming more and more important in the evaluation of diseases affecting the cochlea. Studies could demonstrate that otosclerosis has a characteristic appearance on high-resolution computed tomography (HRCT), producing a distinctive pericochlear hypodense double ring. Its appearance on MRI is not as readily appreciated, producing a ring of intermediate signals in the pericochlear and perilabyrinthine regions on T1-weighted images, demonstrating mild to moderate enhancement after gadolinium administration. The ethiology and pathophysiology of otosclerosis are described and a review of the literature is given to illustrate the clinical relevance of imaging in otosclerosis.

Diagnosis, Differential↗
PubMed1985

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↗
PubMed1999

Clinical otosclerosis, prevalence estimates and spontaneous progress.

This retrospective study was performed in order to estimate the prevalence of clinical otosclerosis as a function of age and gender, characterize the hearing level in otosclerosis and describe the spontaneous progress of the disease as a function of age. Clinically based samples were obtained from an audiological department, including 556 subjects: n = 166 (30%) males and n = 390 (70%) females with a median age of 75 years (range 22-95 years) at the time of examination. A subdivision of the sample into 3 age-bands, 20-39 years (n = 39), 40-59 years (n = 78) and > or = 60 years (n = 439), was performed. The overall prevalence estimate of clinical otosclerosis in the area in question was 1.41/1,000 [95% confidence interval (CI) = 1.3-1.5/1,000] with an estimate of 0.9/1,000 (95% CI = 0.8-1.0/1,000) in males and 1.85/1,000 (95% CI = 1.7-2.1/1,000) in females, with an increase in the prevalence as a function of age from 0.22/1,000 (95% CI = 0.15-0.29/1,000) to 3.53/1,000 (95% CI = 3.2-3.86/1,000) in the elderly. The estimates should be considered underestimates, as not all clinical otosclerosis in the area was included. No significant differences in the better and worse ear hearing levels averaged across 0.5-4 kHz (BEHL/WEHL 0.5-4 kHz) were found as a function of gender, and in general the impairment in the BEHL 0.5-4 kHz was fairly moderate until the age of 60 years. In ears previously subjected to surgery a significantly better hearing level of median 63 dB (range 24-119 dB) was found than in the no-surgery ears, with a median hearing level of 71 dB (range 5-120 dB). An estimate of the progress showed an increment in the hearing level in the elderly > or = 60 years of 30 dB over 30 years in non-operated ears, fairly similar to the 40 dB progress in operated ears. It was concluded that a significantly higher prevalence of clinical otosclerosis is present in females than in males, that the overall hearing level in otosclerosis is fairly moderate until the age of 60 years, and that previously operated ears have significantly better hearing than non-operated ears.

Age Factors↗
PubMed2002

Middle ear dynamic characteristics in patients with otosclerosis.

OBJECTIVE: To investigate the middle ear dynamic characteristics in patients with otosclerosis using the sweep frequency impedance meter (SFI test) and conventional tympanometry, and also to evaluate the diagnostic efficiency of the SFI test for otosclerosis. DESIGN: The study was designed to collect a total of 25 (36 ears) consecutive patients with otosclerosis. All subjects followed a clinical protocol, which consisted of a hearing problem questionnaire, otoscopic examination, and audiometric measurements. These included pure tone audiometry, conventional tympanometry, and SFI test. RESULTS: In the SFI test, the middle ear dynamic characteristics were measured in terms of the resonance frequency and middle ear mobility. Three distinct categories of middle ear dynamic characteristics were found in patients with otosclerosis, i.e., high stiffness, normal stiffness, and low stiffness middle ear status. On comparison of the results of SFI with conventional tympanometry, a significantly higher percentage of abnormal stiffness was found when using the SFI test than that when using conventional tympanometry. CONCLUSIONS: The present findings confirm the advantage of the SFI test over conventional tympanometry in detecting middle ear status and mechanics in patients with otosclerosis. Moreover, different middle ear dynamic characteristics in patients with otosclerosis are most likely to be related to the different stages of the pathological changes.

Acoustic Impedance Tests↗
PubMed2002

Single photon emission computed tomography in otosclerosis: diagnostic accuracy and correlation with age, sex, and sensorineural involvement.

OBJECTIVE: To evaluate the accuracy of single photon emission computed tomography (SPECT) in detecting otospongiotic foci of the labyrinthine capsule in otosclerotic patients and to correlate the metabolic patterns detected by SPECT with age, sex, and sensorineural hearing loss. PATIENTS AND METHODS: Thirty-six patients with surgically confirmed otosclerosis and a control group of 12 subjects with normal hearing; each subject underwent SPECT study of the skull with (99m)technetium-diphosphonate ((99m)Tc-medronate). Statistical analysis of the results was performed by use of the Mann-Whitney U test. RESULTS: SPECT seemed to be very sensitive in differentiating otospongiotic bone from normal bone; in the affected group, only 2 of 72 ears yielded false-negative results (sensitivity 97.2%). Moreover, in the otosclerotic patients, the mean petrosa uptake value was higher than in the control group, a difference that was statistically significant (p < 0.0001). MAIN OUTCOME MEASURES: Regarding uptake in relation to patient age, an inverse relationship was observed between increased metabolic activity and age: the mean uptake index in the younger patients was significantly higher than that in the older patients (p < 0.0001). By contrast, no statistically significant differences were found between men and women (p = 0.1519). Comparison of SPECT alterations with bone conduction thresholds revealed a statistically significant correlation (p = 0.0002 and p = 0.0010) between increased metabolic bone activity and sensorineural involvement only in the younger group of otosclerotic patients. CONCLUSIONS: Diphosphonate bone SPECT is the only functional method that allows in vivo evaluation of disease activity, and it was highly sensitive in detecting otosclerosis. SPECT can be considered useful in the diagnosis of otosclerosis that is difficult to recognize, such as cochlear otosclerosis and far-advanced otosclerosis, and it could also find a place in evaluating the efficacy of medical therapy for otosclerosis.

Adult↗
PubMed2004

Association of otosclerosis with Sp1 binding site polymorphism in COL1A1 gene: evidence for a shared genetic etiology with osteoporosis.

HYPOTHESIS: There is an association between otosclerosis and osteoporosis. BACKGROUND: Both osteoporosis and otosclerosis are common bone diseases to which relatively large portions of the population are genetically predisposed. Recently, a strong association has been described between osteoporosis and an Sp1 binding site of putative functional significance in the first intron of the COL1A1 gene. METHODS: We applied polymerase chain reaction-based restriction enzyme analysis to determine the polymorphic distribution of the Sp1 site in 100 patients with otosclerosis and 108 control subjects. RESULTS: This study showed a significant association between otosclerosis and the COL1A1 first intron Sp1 site. The allelic frequency of the Sp1 site is very similar between otosclerosis and osteoporosis. CONCLUSION: Some cases of otosclerosis and osteoporosis could share a functionally significant polymorphism in the Sp1 transcription factor binding site in the first intron of the COL1A1 gene.

Aged↗
PubMed2006

Cochlear implantation of patients with far-advanced otosclerosis.

OBJECTIVE: This current single-subject, repeated-measures study was to describe our experience with 30 patients who had been diagnosed with "far-advanced otosclerosis" and who were included in our program of cochlear implants. We analyzed the history of the patients and their families before implantation, the surgical findings, and the performance over a follow up of 3 years. MATERIAL AND METHODS: All patients met one or more of the after criteria: 1) previous surgical intervention as a treatment of their otosclerosis; 2) signs of pericochlear hypodensities in high resolution computed tomography (HRCT) scans; and 3) family precedents of otosclerosis. All underwent standard surgical cochlear implantation. RESULTS: In 78% of the cases, a stapedectomy had previously been realized. Cochlear otosclerosis could be appreciated in HRCT in 78% of the patients. A family history of otosclerosis was found in 40%, and 33.3% of patients had familial precedents of nonfilial hypoacusis. The mean results in the two-syllable test were 20% preimplantation, 54% 6 months after implantation, and 52%, 62%, 54% at 1, 2, and 3 years after implantation. In the CID sentence test, they were in the order of 32% preimplantation and of 64% at 6 months, 66% after 1 year, of 68% after 2 years, and reaching 72% after 3 years. No complications related to the surgery were detected. CONCLUSION: Patients diagnosed with far-advanced otosclerosis have a good prognosis with cochlear implantation comparable to that of other patients in whom postlingual implants are performed.

Adult↗
PubMedDate not supplied

Etiopathogenesis of otosclerosis.

Otosclerosis is a frequent cause of nonsyndromic hearing loss which affects exclusively the human temporal bone. Various etiopathogenetic hypotheses have been proposed. The major hypotheses considered are genetic factors, immunologic factors and viral infection. Since the familial incidence of otosclerosis is known a recent genetic analysis has given evidence of three otosclerosis genes (OTSC1-3). Mutations in the collagen gene COL1A1 have been found in one large family with several cases of otosclerosis. Concerning an immunologic etiopathogenetic process, the presence of serum antibodies against collagen II and IX in patients with otosclerosis confirms the hypothesis of a collagen autoimmune mechanism. Finally as a possible cause of this chronic inflammatory disease morphologic and biochemical investigations have revealed a measles virus association. In conclusion, various etiopathogenetic factors may contribute to the genesis of otosclerosis.

Bone Resorption↗
PubMedDate not supplied

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↗
PubMedDate not supplied

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↗
PubMed1984

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↗
PubMed1999

Stapedectomy for far-advanced otosclerosis.

OBJECTIVE: This study aimed to describe far-advanced otosclerosis and to present the authors' results with stapedectomy in 78 ears with far-advanced otosclerosis. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at an Otology/Neurotology tertiary referral center. PATIENTS: Stapedectomy was performed on 78 ears of 60 patients with far-advanced otosclerosis, and the results followed from 1 to 21 years with a mean of 5 years. INTERVENTION: Stapedectomy was performed on all ears with far-advanced otosclerosis. MAIN OUTCOME MEASURE: Hearing for air conduction (AC) and bone conduction (BC), speech discrimination, and impedance were tested on all patients before and after operation. The Rinne test was performed on all ears with a 256-cycle magnesium tuning fork. The pure-tone average for AC and BC was computed for 500, 1,000, and 2,000 Hz. Hearing improvement was defined as air-bone gap closure to 10 dB or less and/or AC improvement of 20 dB or more, with no decline in speech discrimination score of more than 10%. RESULTS: Hearing improvement was achieved in 52 (66.7%) of 78 ears of all operations. In group 1, AC was greater than 90 dB, BC was greater than 60 dB, and hearing improved in 26 (81.2%) of 32 ears of operations. In group 2, AC was greater than 90 dB and no measurable BC and hearing improved in 11 (68.8%) of 16 ears of operations. In group 3, there was no measurable AC and BC greater than 60 dB and hearing improved in two (50%) of four ears of operations. In group 4, there was no measurable AC and BC and hearing improved in 11 (42.3%) of 26 ears of operations. Nonmeasurable BC became measurable in 42.9% of ears, nonmeasurable AC became measurable in 73.3% of ears, and all of these became aidable after operation. CONCLUSIONS: A negative Rinne test result with a 256-Hz magnesium tuning fork proved to be the best test to separate far-advanced otosclerosis from sensorineural hearing loss of other causes. Stapedectomy is of benefit in most ears with profound hearing loss of far-advanced otosclerosis, especially in those ears with some measurable hearing by AC.

Adolescent↗
PubMedDate not supplied

[Otosclerosis: experience at the Hotel-Dieu in France. 71 surgical cases].

Otosclerosis is one of the common causes of hearing loss. The incidence varies between 0.1% and 2%. In Lebanon otosclerosis is a common entity that has not been well evaluated. To the best of our knowledge there is no epidemiologic analysis of the incidence or outcomes of otosclerosis in Lebanon. We collected the number of stapedectomies performed for otosclerosis in different hospitals between Jan. 1994 and Dec. 1995. We also retrospectively reviewed the charts of 71 cases who underwent stapedectomy at Hôtel-Dieu de France-St Joseph University Hospital Medical Center, Beirut, Lebanon. Between 1992 and 1996 the incidence of otosclerosis in Lebanon as revealed through stapedectomy is 5/100,000. We report also on the pathology, technique, complications and outcomes of stapedectomy surgery for otosclerosis in Hôtel-Dieu Hospital. Further epidemiologic studies and screening is required to reveal the exact incidence of this common entity that could be underdiagnosed or untreated in our country.

Adult↗
PubMed2005

[Results of surgical treatment for advanced otosclerosis].

OBJECTIVE: To assess the effect of stapes surgery on advanced otosclerosis (AO). METHODS: In 300 cases randomly collected of otosclerosis in 1970 to 1999, 68 cases (77ears) were selected for retrospective analysis, which met the criteria of advanced otosclerosis (mixed deafness, with bone-conduction levels exceeding 40 dB and air-conduction levels exceeding 70 dB in 500 - 2000 Hz). RESULTS: The air-conduction of sixty-eight cases (77 ears) were from 77.32 dB to 53.7 dB (500-2000 Hz) after operation, mean decreased 23.62 dB. Of 71 ears (92.21%) obtained air-conduction improved over 10 dB, of 46 ears (59.74%) gained A-B Gap closure in 10 dB. Air-conduction were from 79.01 dB to 58.23 dB (500 - 4000 Hz) after operation and mean decreased 20.78 dB. Of 68 ears (88.31%) obtained air-conduction improved over 10 dB, of 32 ears (41.56%) gained A-B gap closure in 10 dB. After followed up 5-25 years, of 67 ears were retained stabilization, of 28 ears obtained more improve than postoperation. But 4 ears had drop (all was stapes-elevation and re-improvement after them was reviewed). CONCLUSION: Stapes surgery is effective operation to cured otosclerosis, advanced otosclerosis or far-advanced otosclerosis had greater help to improved hearing.

Adult↗
PubMed2006

[CT findings in otosclerosis. A series of 11 cases].

INTRODUCTION: Our study's aim is to evaluate the reliability of computed tomography in diagnosis, extension and post-surgical follow-up of otosclerosis. PATIENTS AND METHODS: A group of 11 patients was examined during a period of 25 months from 2002 to 2004. All of them had clinically and audiometrically suspected otosclerosis with bilateral conductive hear loss in 7 cases (3 had surgical management), unilateral in 2, and mixed earing loss in 2. High resolution CT was performed once in 8 patients and twice in the 3 operated. RESULTS: CT showed otosclerosis foci in all our study's patients, which was bilateral in 9 and unilateral in 2. The extension of the lesions varied from type IA to type 4B according to the otosclerosis staging. CONCLUSION: Computed tomography is, so far, the most efficient imaging modality in otosclerosis, showing otosclerosis foci when the clinical features are doubtful, allowing an accurate extension of the lesions and detecting complications during post-surgical follow-up.

Adult↗
PubMed2006

[Far-advanced otosclerosis].

OBJECTIVES: The aim of this study was to report a series of patients with far-advanced otosclerosis who were unable to benefit from hearing aids. Among patients with profound hearing loss, it is particularly useful to diagnose far-advanced otosclerosis even if relatively rare, because stapes surgery can improve hearing to a level allowing conventional hearing aid use (sometime the only choice before cochlear implant). MATERIAL AND METHODS: We retrospectively reviewed the charts of 7 patients (9 operated ears) in order to highlight diagnostic criteria, surgical indications and results of stapes surgery (stapedectomy or stapedotomy). RESULTS: Diagnosis of far-advanced otosclerosis was based on clinical presentation, course of hearing loss, positive family history of otosclerosis, and results of CT scan which was helpful in all the cases. Obliterative otosclerosis was found in 55% of the cases. Success of stapes surgery was observed in 89%, with no significant difference between stapedectomy or stapedotomy. CONCLUSION: Although rare, far-advanced otosclerosis must be diagnosed because patients can benefit from stapes surgery (and subsequently fitting of appropriate hearing aids).

Aged↗
PubMed1981

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↗