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PubMedDate not supplied

[Obliterative otosclerosis].

In true (grade IV) obliterative otosclerosis, the limits of the oval windows are lost. The insertion of a prosthesis requires drilling and surgical ability. Thirty eight cases of the true obliterative otosclerosis operated in our Department between 1974 and 1992 have been reviewed. Only 6.2% of all the patients operated by us with the diagnosis of otosclerosis had true obliterative otosclerosis. The average preoperative gap in conversational frequencies was 39 dB. In general, the gap closure obtained (13 dB) was slightly lesser than that for otosclerosis. Reobliteration of the oval window may occur in this type of otosclerosis.

Audiometry↗
PubMed1996

[Pathogenesis of otosclerosis. "State of the art"].

Women suffer from otosclerosis 1.6 times more often than males. Histologically, otosclerotic foci can be found in temporal bones of females 1.9 times more often than in those of males. Characteristic topographic regions are the oval window, round window niche and promontory. Otosclerosis can also occur principally in any area of the enchondral/periosteal layer of the otic capsule. Evidence is presented that otosclerosis is an inflammatory tissue reaction associated with macrophages, T- and B-lymphocytes, HLA-DR positive cells and plasma cells. Dependent on the stage of the osteolytic bone disease present deposits of complement and immunoglobulins (IgG, IgA) can be found. These immunoglobulins have been identified as antibodies to measles virus proteins. Using the polymerase chain reaction we were successful in demonstrating RNA sequences of measles viruses in otosclerotic bone from footplates removed during stapes surgery. Since most of the otosclerotic lesions were in direct contact to the perilymphatic space, it may be expected that the endolymphatic sac--as the immune competent organ of the inner ear--specifically reacts to antigens delivered from the otosclerosis focus into the perilymph. Perilymph samples from patients were collected during stapes surgery and their antibody titers against measles were compared with that in corresponding blood serum. All samples revealed a significantly elevated-specific anti-measles IgG amount which was significantly higher than in the corresponding serum. In contrast, antibody titer in the perilymph against herpes simplex or cytomegalo viruses did not differ from that of the serum. These findings indicate that otosclerosis is a measles virus associated inflammatory osteolytic disease of the temporal bone. Since women suffer from severe measles virus infections more often than males, it can be hypothesized that females have a higher susceptibility of their cochleo-vestibular tissues to these infections (organotropism). In addition, estrogens are well-known stimulators of osteocytic activity and may play a dominant role during ossification of an otospongeotic bone lesion. This may explain the onset of a conductive hearing loss due to otosclerosis during pregnancy.

Adult↗
PubMed1996

Polymerase chain reaction amplification of a measles virus sequence from human temporal bone sections with active otosclerosis.

Investigation of a possible viral etiology for otosclerosis was initiated because of the clinical and histopathologic similarities between otosclerosis and Paget's disease of bone and the mounting evidence of a viral etiology in Paget's disease. Thus far, ultrastructural and immunohistochemical studies have revealed measles-like structures and antigens in active otosclerotic lesions. A method for isolation and identification of both DNA and RNA sequences in archival human temporal bone specimens using the polymerase chain reaction technique has been developed. With use of this technique, a 115-base pair sequence of the measles nucleocapsid gene has been identified in 8 of 11 different temporal bone specimens with histologic evidence of otosclerosis. Zero of nine control specimens without histologic evidence of otosclerosis were positive. The association between the presence of the measles nucleocapsid gene sequence and histologic otosclerosis was significant (p < 0.01). This study provides further evidence for a possible measles virus etiology in otosclerosis.

Base Sequence↗
PubMed1998

Association of COL1A1 and otosclerosis: evidence for a shared genetic etiology with mild osteogenesis imperfecta.

HYPOTHESIS: Otosclerosis is related to mild osteogenesis imperfecta with genetic defects in type I collagen. BACKGROUND: Otosclerosis is a common bone disease of the human otic capsule that has an underlying hereditary predisposition. The histopathology and clinical manifestations are strikingly similar to the milder forms of osteogenesis imperfecta in which mutations of type I collagen genes have been established as the underlying cause. METHODS: The authors investigated the genetic basis of otosclerosis by conducting an association study using polymorphic DNA markers from patients with clinical otosclerosis and random control subjects. RESULTS: This study showed a significant association between clinical otosclerosis and the type I collagen COL1A1 gene using three different polymorphic markers within the gene. CONCLUSIONS: Some cases of clinical otosclerosis may be related to mutations within the COL1A1 gene that are similar to those found in mild forms of osteogenesis imperfecta and result in null expression of the mutant allele.

Alleles↗
PubMed2006

Relationship between CT densitometry with a slice thickness of 0.5 mm and audiometry in otosclerosis.

The appropriate cutoff Hounsfield unit (HU) value for the diagnosis of otosclerosis was determined and the correlation between the bone conduction threshold and the findings of computed tomography (CT) densitometry investigated. CT images, 0.5-mm thick, were evaluated in 24 ears with otosclerosis and 19 control ears. Eight regions of interest were set around the otic capsule. The mean HU values in the area anterior to the oval window (A-OW) and anterior to the internal auditory canal (A-IAC) were significantly lower in otosclerosis than in controls. Based on receiver operating characteristic (ROC) analysis, the cutoff HU value in A-OW was determined to be 2,187.3 HU. The mean HU value in retrofenestral otosclerosis was significantly lower in the area A-OW, A-IAC and around the cochlea than in controls. Based on ROC analysis, the cutoff HU value in the latter was determined to be 2,045 HU. A statistically significant correlation was found between the density of the area A-OW and the hearing level at 500 and 1,000 Hz, and between the density of the area around the cochlea and the hearing level at most frequencies. These results suggest the semi-automated diagnosis of otosclerosis may be possible.

Absorptiometry, Photon↗
PubMed2002

[Evaluation of the morphology of stapedial reflex in otosclerosis. Provoked otosclerotic stapedial reflex].

Stapedial reflex is used, amongst other pathologies, for the study of otosclerosis. In this retrospective study we have collected 188 cases of patients with otosclerosis whose diagnosis has been confirmed surgically and their first line relatives. We have performed a descriptive analysis of audiometric tests, tympanometries and ipsilateral stapedial reflexes in relation with the evolutive phase of the disease. Transmission hypoacusis has been seen in 54%, mixed hypoacusis in 29% and sensorineural hypoacusis 8% of cases. On and OFF stapedial reflexes have been seen in 18%, inverted reflexes in 46% and absent reflexes in 27% of cases. The original drawings of the different types of reflexes during the evolution of otosclerosis can be seen in the four figures shown in the study: normal reflexes; ON and OFF a, b and c types; inverted a and b; and absent reflexes. The clinical evolutive phase and the audiometric and impedance tests have been correlated. Through Chi-square (p < 0.001), we have obtained a statistical significance in relation to the use of stapedial reflexes. Also it has been suggested the use of provoked stapedial reflexes in patients with sub clinical otosclerosis in order to diagnose the disease. We conclude that the knowledge of the evolutive morphology os stapedial reflexes in otosclerosis helps diagnostic capacity.

Acoustic Impedance Tests↗
PubMed2002

Incidence and characteristics of otosclerosis in the Japanese population.

OBJECTIVE: Otosclerosis is thought to be quite an uncommon disease in Japan. However, in recent years the incidence of this disease seems to have increased. Here we report about the characteristics and incidence of this disease in the Japanese population who underwent stapes surgery in our department. METHODS: A total of 80 patients (115 ears), who underwent stapes surgery by a single surgeon in 21 years and 8 months were included in this study. RESULTS: The incidence of clinical otosclerosis was found to be 0.22% among the outpatients with ear disease. During the first half of the period, the ratio of stapes surgery/tympanoplasty was less than 0.05. While, during the second half of this period, this ratio had increased to 0.08. The average number of stapes surgery performed in the first half period was 3.7 ears/year, and that in the second half was 6.8 ears/year. In contrast to otosclerosis in Caucasians, pure tone threshold in lower frequencies was much higher than that of higher frequencies (except for 8000 Hz). However, the improvement of the threshold after surgery was not so different from that reported in Caucasians. CONCLUSIONS: Taken together, the present results clearly demonstrate that the incidence of otosclerosis in Japan has been increasing. Moreover the rise in the slope of the conduction curve at higher frequencies may be a reflection of the pathological characteristics of otosclerosis in Japanese population, which is less invasive and has limited otosclerotic foci in the temporal bone.

Adult↗
PubMed2004

Late failure of cochlear implantation resulting from advanced cochlear otosclerosis: surgical and programming challenges.

OBJECTIVE: The objective of this study was to discuss cochlear implantation in the setting of severe cochlear otosclerosis and review programming challenges in a patient whose performance is deteriorating as a result of advancing disease. STUDY DESIGN: We conducted a case report and literature review. SETTING: Tertiary care medical center. PATIENTS, INTERVENTION, AND RESULTS: A case is presented of a 66-year-old man with otosclerosis who initially had good benefit from a cochlear implant but gradually lost benefit even with reimplantation. Imaging studies demonstrated severely distorted otic capsule anatomy from cochlear otosclerosis. CONCLUSIONS: Advancing cochlear otosclerosis can result in a severely thinned and distorted otic capsule. Although cochlear implantation is generally beneficial in cochlear otosclerosis, implantation in certain severe cases may be complicated as a result of difficulties with cerebrospinal fluid leak, programming challenges, and other potential hazards that can occur with a distorted anatomy.

Aged↗
PubMed2007

Vestibular-evoked myogenic potentials in patients with otosclerosis using air- and bone-conducted tone-burst stimulation.

OBJECTIVE: Otosclerosis is a progressive disease with a remodeling process causing ossicular malformation and conductive hearing loss. The aim of this study was to investigate whether vestibular-evoked myogenic potential (VEMP) correlates with the progression of otosclerosis. DESIGN: Fifteen patients with otosclerosis (21 ears) without operation and 10 healthy subjects (20 ears) underwent VEMP test using air-conducted (AC) and bone-conducted (BC) tone-burst stimulation. SETTING: Tertiary referral university hospital. RESULTS: In 21 unoperated otosclerotic ears, 5 ears (24%) showed present AC-VEMPs, and 16 ears had absent AC-VEMPs. Conversely, 16 ears (76%) displayed present BC-VEMPs and 5 ears with absent BC-VEMPs. In those with both AC- and BC-VEMPs, none of them showed air-bone gap greater than 30 dB; in those with absent AC-VEMPs but present BC-VEMPs, 27% of the ears had air-bone gap greater than 30 dB; and in those with absence of both AC- and BC-VEMPs, 80% of the ears revealed air-bone gap greater than 30 dB. Thus, a significant relationship existed among the presence of AC-VEMPs, BC-VEMPs, and magnitude of conductive hearing loss. CONCLUSION: The presence of an AC-VEMP may indicate an earlier stage of otosclerosis, although absent BC-VEMP infers a later stage. Restated, AC-VEMPs may complement the results obtained with BC-VEMPs to classify the stage of otosclerosis.

Acoustic Stimulation↗
PubMed2005

Quantification of angiogenesis in otosclerosis.

OBJECTIVES/HYPOTHESIS: The determinants of clinical versus histologic otosclerosis are unknown, but angiogenesis is associated with active disease. We hypothesized that quantification of angiogenesis in otosclerotic human temporal bones could reveal significant differences between clinical and histologic cases. STUDY DESIGN: We reviewed all otosclerosis specimens meeting criteria from the temporal bone collection of the Massachusetts Eye and Ear Infirmary and 10 normal controls. METHODS: Digital images were taken at predilection sites, followed by computer-assisted analysis. Canalicular area (CA), the aggregate of vascular spaces within bone, microvessel density (MVD), area, and depth were the main measures. Evidence of a direct connection between local vessels and the vasculature of the otosclerotic focus was also recorded for each specimen. RESULTS: The average area (mm) and depth (number of sections containing otosclerosis) of clinical lesions was significantly greater than histologic lesions. Total microvessel counts were significantly greater in clinical versus histologic lesions, and both clinical and histologic lesions contained significantly greater numbers of microvessels than the normal otic capsule. CA was also significantly higher in clinical lesions. MVD was slightly but not significantly higher in clinical lesions. Importantly, a direct connection between named vessels and the otosclerotic vasculature was significantly more frequent in clinical lesions. CONCLUSIONS: Computer-assisted quantification revealed significantly greater measures of angiogenesis in clinical versus histologic otosclerosis. Direct connection to adjacent vessels may support angiogenesis in this disease. Sustained angiogenesis may be an important determinant of clinical otosclerosis.

Aged↗
PubMed2006

Blood flow to the promontory in cochlear otosclerosis.

OBJECTIVE: To investigate Schwartze sign with measurements of blood flow to the promontory in patients with cochlear otosclerosis. DESIGN: Prospective clinical study. SETTING: Tertiary referral centre. PARTICIPANTS: Five patients with cochlear otosclerosis and five control subjects. Significant decalcification around the cochlea was observed by computed tomography (CT) in patients with cochlear otosclerosis. However, no recognizable lesion was observed at the oval window in two patients. One patient had mixed hearing loss and four patients had sensorineural hearing loss without an air-bone gap. MAIN OUTCOME MEASURES: The relationship between CT findings and the presence or absence of Schwartze sign was investigated. Blood flow to the promontory was measured through the tympanic membrane using laser speckle flowgraphy and laser Doppler flowmetry. RESULTS: The Schwartze sign correlated significantly with otosclerotic lesions invading the promontory. Patients with otosclerosis exhibited elevated and pulsating blood flow to the promontory with the Schwartze sign. CONCLUSIONS: Computed tomography demonstrated that cochlear otosclerosis can exist without the oval window lesion. Schwartze sign can be used as a sign of the otosclerotic invasion to the promontory. The reddening of the Schwartze sign is likely due to increased blood flow.

Adult↗
PubMed1989

Immunohistochemical evidence of measles virus antigens in active otosclerosis.

Despite intensive investigation, the cause of otosclerosis remains uncertain. Recent studies of Paget's disease of bone have revealed a possible viral origin. Because of similarities between otosclerosis and Paget's disease, we have pursued investigation of a possible viral cause of otosclerosis. Four temporal bone specimens from patients with otosclerosis, processed for immunohistochemistry, demonstrated positive specific reactivity with monoclonal antibodies to measles virus antigens using the indirect immunofluorescent and immunoperoxidase techniques. Reactivity was most intense in active foci. Reactivity in the peroxidase assay was also observed in areas of acting otosclerosis with application of primary antisera from patients with subacute sclerosing panencephalitis, a disorder of the central nervous system in which a defective measles virus has been isolated. Other related paramyxoviruses, including mumps and respiratory syncytial virus, were negative, as were negative controls.

Antibodies, Monoclonal↗
PubMed2004

Association between osteoporosis and otosclerosis in women.

Similarities between osteoporosis and otosclerosis have been noted, including a similar association with the COL1A1 gene. Herein, the authors explore the possible clinical relationship between these two common disorders of bone. In this retrospective study, the medical charts of 100 women aged 50 through 75 years who had undergone stapedectomy for otosclerosis were reviewed and the prevalence of osteoporosis in these women was noted. Similarly, the prevalence of osteoporosis was determined in a control group of 100 women aged 50 to 75 years with presbycusis. Fifteen of 100 women with otosclerosis had a concomitant diagnosis of osteoporosis as compared with four of 100 women with presbycusis, yielding a significant clinical association (p = 0.007) between otosclerosis and osteoporosis. This study suggests an association between otosclerosis and osteoporosis, providing impetus and justification for future prospective clinical studies and related research.

Age Distribution↗
PubMed1986

Coexisting otosclerosis and Ménière's disease: a diagnostic and therapeutic dilemma.

The coexistence of otosclerosis and Meniere's disease is well documented; however, otosclerosis may not be recognized in patients presenting mainly with Meniere's symptoms and, therefore, treatment may not be effective. In this paper, eight patients with otosclerosis and Meniere's disease are presented. The diagnosis of coexisting otosclerosis and Meniere's was initially missed in most of these patients. Medical and surgical treatment had been unsuccessful. Diagnostic and therapeutic guidelines for management of these patients are discussed. Our preliminary conclusions are: 1. otosclerosis should be diagnosed in patients with Meniere's disease, especially when bilateral or when suggested by clinical and/or laboratory findings, and 2. the addition of sodium fluoride to diet-diuretic management of such patients appears effective.

Adult↗
PubMed1995

Cochlear vascular pathology and hydrops in otosclerosis.

Three ears with otosclerosis were found incidentally in a series of human temporal bones examined to evaluate cochlear sensorineural degeneration. Otosclerosis was identified with microdissection, surface preparation technique and transmission electron microscopy. Vascular abnormalities were present in all ears, and otosclerosis involved the cochlear endosteum extensively, mainly in the scala tympani of the basal turn. In the scala tympani of the lower half of the basal turn, shunts had formed so that venules deviated abruptly from their normal radiating course towards the spiral vein, left the scala and entered into otosclerotic foci. There was a marked loss of radiating venules in areas where otosclerosis affected the endosteum of the scala. In the pair of bones capillaries in the stria vascularis were extremely dilated, the widest being 80 microns in diameter. The third single bone from a patient with Meniere's disease had severe cochleo-saccular hydrops. Ten serially sectioned temporal bones with known otosclerosis were reviewed. Two of the bones, one of which had cochleo-saccular hydrops, displayed vascular shunts in the scala tympani and enormously dilated strial capillaries with a maximum diameter of 139 microns.

Adult↗
PubMed1999

[Current aspects of the pathogenesis and clinical characteristics of otosclerosis: possibilities of drug therapy].

Otosclerosis is a multifactorial disease. A number of theories on the pathogenesis of this disease have been established in the last decades. It is important to review recent data on the pathogenesis of otosclerosis as it is a severe inner ear disease leading to deafness in the majority of cases. Surgical therapy is not always successful or feasible. In this review, authors describe the most relevant genetic, infective, immunological, inflammatory factors, as well as the impaired bone metabolism underlying the pathogenesis of otosclerosis. It is likely that genetic predisposition associated with morbilli infection may lead to bone resorption in the stapes and cochlea followed by spongiosis, fibrosis and sclerosis. It has been suggested that immunological mechanisms play a central role in the development of the disease. Some authors consider otosclerosis as autoimmune disorder based on the presence of several autoantibodies. Apart from classical diagnostic methods, such as audiometry and X-ray, novel radiological techniques including CT, MRI or radionuclide scan are helpful in the localization of otosclerosis. As surgery is sometimes contraindicated or unsuccessful, drug therapy including the use of anti-osteoporotic on non-steroidal antiinflammatory drugs may be administered, especially in the early phase of the disease.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2001

[Otosclerosis in populations living in northern Tunisia: epidemiology and etiology].

Otosclerosis is a hereditary disease frequently encountered in Caucasian populations (0.1 to 2% prevalence). In Tunisia, prevalence varies from 0.4% to 0.8%. The presence of a genetic factor associated with hormonal, biochemical or environmental factors, probably lead to variable expression of the otosclerosis according to age and sex. Mean age at onset of disease is 25 years (range 16-35 year) for 61.5% of affected women. In men this proportion is 50.2%. The incidence of otosclerosis is high in the 26-35 year age group. Our study showed that in northeastern areas of Tunisia, women in this age group were affected twice as often as men in this age group. This probably suggests that an endocrine mechanism is involved in disease etiology. However, in northwestern areas, there was no significant difference between the rates of otosclerosis between sexes. Geographical distribution of affected subjects according to the ethnic origin of their parents showed that the areas with the highest concentration of affected individuals were urban or seaside areas such as the gouvernorate of Nabeul. The frequency of otosclerosis was lower in rural areas and/or areas far from the seaside.

Adolescent↗
PubMed1975

The incidence of otosclerosis in the general population.

The incidence of clinical otosclerosis in the general population of Lithuania (USSR) was recorded from December 1961 to May 1970. A total of 974 otosclerotic individuals were found during this period, 27.2% of whom were males and 72.8%, females. In the majority of these patients, the clinical diagnosis of otosclerosis was confirmed at operation. The incidence of clinical otosclerosis in the population was found to be about 0.1%. The ratio of incidence of clinical:histological otosclerosis in the population investigated is assumed to be 1:4. The age period at risk for clinical otosclerosis is between 16 and 50 years, the greatest age period at risk being 20 to 40 years.

Adolescent↗