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Outcome of stapes surgery for otosclerosis.

Otosclerosis is an autosomal dominant disease affecting the otic capsule. It is believed to be rare in Asian countries including Malaysia. We analyzed the results of 29 patients (35 ears) who had undergone stapes surgery for suspected otosclerosis from January 1996 to June 2002. The demographic data was analyzed. The procedure most commonly performed was stapedotomy. The closure of air bone gap after surgery was good at 2 kHz and 4 kHz compared to frequency below 2 kHz. Closure of air bone gap to within 10 dB was achieved in 74.3%. About 7.5% of our patients did not gain in average air conduction. The outcome of the procedure is comparable to those reported by authors who used the same evaluation criteria. No serious complications occurred in our patients.

Adult↗

[Natural history of otosclerosis--from tinnitus to deafness].

Authors review the most important data about natural history, signs and symptoms and clinical stages of otosclerosis. Development of otosclerosis is long-lasting and involves several decades of lives. First symptoms already appear in teenagers in about 20% of cases. In advanced cases there are irreversible sensorineural hearing loss. When total deafness occurs cochlear implantation is nowadays the treatment of choice.

Adolescent↗

[Importance of laser in the surgical technique of otosclerosis and its results].

The authors bring up the matter of the usefulness of laser in otosclerosis surgery. They set out some advantages of laser utilization. A bibliografic review is done and some important authors are found explaining the advantages of this instrument. Some new tendencies on stapes surgery are presented. Teaching on otosclerosis surgery problems are stated, with some different criteria. The idea that improvement of audiologic results in stapes surgery is not easy is strengthened, because the results obtained with the classic techniques are excellent, but some of these methods increase the surgical comfort and reduce the potential risks to the patient.

Humans↗

[Surgical treatment of otosclerosis].

A significant step forward in otosclerosis surgery was made in 1956 with the advent of stapedectomy. This led to a significant reduction in surgical complications and to a high level of patient satisfaction. Hearing aids are the alternative to surgery, and have themselves undergone considerable technical improvements. In advanced otosclerosis, cochlear implants can improve hearing when stapes surgery and a conventional hearing aid are inadequate. These advances are modifying the surgical indications.

Adult↗

[Detection of measles and rubella-specific antigens in the endochondral ossification zone in otosclerosis].

Paramyxoviruses have been described in the osteoclasts in Paget's disease and it has been suggested that the disease was caused by this virus. There is considerable similarity of the histological features of active otosclerosis and Paget's disease and the probable presence of viral antigens in the otosclerotic footplate of the stapes of 15 patients has been investigated by means of immunohistochemical methods. The active otosclerotic lesions of all 15 patients studied displayed a high concentration of antibodies of the IgG-class which was less pronounced in the cytoplasm of the cells of the adjacent enchondral layer. Using polyclonal antibodies against measles and rubella viruses, both antigens could be shown expressed by the chondrocytes of the enchondral layer and by occasional osteocytes and osteoclasts of the otosclerotic lesion. IgG antibodies or viral antigens were absent in the normal bone of the stapes specimens. The interpretation and significance of these findings is discussed, with particular reference to the pathogenesis of otosclerosis.

Adult↗

[Otosclerosis. A paramyxovirus-induced inflammatory reaction].

Despite intensive investigation the cause of otosclerosis remains unknown. Recent immunohistochemical and electron microscopic studies of otosclerotic stapes have revealed a possible viral aetiology. Therefore we investigated the histology, ultrastructure and immunohistochemistry of fragments of otosclerotic footplates. Immunoglobulins G (IgG) and A (IgA) were found in plasma cells, oseocytes, chondrocytes and connective tissue of the active lesions. Polyclonal and monoclonal antibodies against paramyxovirus and rubella virus antigens produced reproducible reactions with these antigens at different sites of otosclerotic stapes, especially in the epithelial cells of the overlying middle ear mucosa. Using specific T-lymphocyte and B-lymphocyte markers about 80% of the lymphocytes present in the otosclerotic footplate proved to be T-lymphocytes. There was neither deposition of immunoglobulins nor any expression of viral antigens in non-otosclerotic footplates which were investigated as controls. Our results indicate that otosclerosis is an inflammatory reaction of the otic capsule initiated or caused by paramyxovirus and/or rubella virus. The silent progress of the disease and its onset under certain conditions, e.g. endocrine upsets, show similarity to slow viral infections.

Adult↗

[Surgical treatment of otosclerosis using stapedotomy].

Results achieved by the operative technique "small window" in the treatment of 140 patients with otosclerosis are presented. Success of surgery was estimated on the basis of differences in air-bone gap (GAP) for frequencies from 0.5 kHz to 3 kHz before and after surgery. The complete closure of GAP in this region or its decrease below 10 dB was achieved in 79.2% of the operated patients. Postoperative GAP values ranged from 10 to 15 dB in 12.2% of cases. Hearing was maintained at the preoperative level in 3.6% while postoperative conductive hearing impairment was found in 5% of cases. In 18 (12.8%) ears with postoperative good functional success in speech region it has come to damages of high frequency perception from 20 to 60 dB. The authors have concluded that the operative technique "small window" provides valuable possibilities in hearing reparation in otosclerosis under condition that highly educated and skillful surgeon is available.

Adolescent↗

Otosclerosis and mucopolysaccharidosis.

By means of a literature review, clinical observations and temporal bone histopathology in the Hunter syndrome, we could show a reason for otosclerosis in childhood, a very rare observation. Therefore we do not believe that the presence of otosclerotic foci in temporal bones is mere coincidence in MPS II. It seems there is a causal connection between the generalized metabolic disorder and otosclerosis on the basis of enzyme deficiency.

Child↗

Stapedius reflex in the monitoring of NaF treatment of subclinical otosclerosis.

Stapedius reflex testing was utilized to monitor the progression of subclinical otosclerosis and evaluate the efficacy of NaF treatment in stabilizing the disease process. The investigation was performed on 93 relatives of patients suffering with surgically confirmed otosclerosis. Subjects were divided into two groups. The first group was treated with NaF in doses ranging from 6 to 16 mg in relation to age. The treatment lasted two years. The second group served as control subjects. Before entering the research, all subjects presented reflex abnormalities represented by a partial or complete on-off effect in one or both ears. Changes in stapedius reflex morphology (from partial to complete on-off and from complete on-off to absence of the reflex) were evaluated at 1 and 2 years. The groups had not developed significant differences at 1 year. At 2 years, the incidence of stable stapedius reflex findings was 91.5% in the treated ears and 77% in the control group. The difference is significant (p less than 0.05) and indicates a stabilizing effect of NaF on the disease process.

Acoustic Impedance Tests↗

Cochlear otosclerosis: a review of audiometric findings in 150 cases.

Audiometric findings and histories were reviewed for 150 patients diagnosed by polytomography as probable cochlear otosclerosis cases. Most patients were under 65 and had a relatively short duration of hearing impairment. Most had a mild to moderate loss with excellent speech discrimination. Seventy-five percent of the ears had acoustic reflex findings indicative of minimal stapes fixation. We recommend the acoustic reflex test for identification of patients with probable cochlear otosclerosis.

Adult↗

Densitometry of the cochlear capsule and correlation between bone density loss and bone conduction hearing loss in otosclerosis.

Radiodensity of the bony cochlear capsule was investigated by CT-scanning in 134 ears with otosclerosis and 35 normal control ears. A technique of densitometry of the cochlea was developed comprising the measurement of maximum and minimum density together with mapping of the cochlea and measurement of the density at six predefined points in the cochlear capsule. Bone density in the normal cochlea showed very little interindividual variation. In the otosclerosis patients, minimum density was decreased in 58% of the ears, indicating the presence of a focus. At visual examination, foci were only detectable when density loss exceeded 200 Hounsfield. A positive correlation was found between bone density loss and bone conduction hearing loss with a maximum at 2,000 Hz and the medioventral point of the cochlear wall. The method appears to be useful for the follow-up during fluoride treatment, as is demonstrated by a case report.

Cochlea↗

Two cases of otosclerosis in Kumasi, Ghana, case report.

The incidence of otosclerosis in relationship to race has been established in North America; however, there are no reliable data about its occurrence among negro Africans. Over a period of three years 650 consecutive cases of hearing loss excluding those with acute and chronic otitis media, Eustachian tube dysfunction, wax, foreign bodies, and traumatic perforations, were studied in Kumasi, Ghana. Only two cases of otosclerosis were detected. It is postulated that the rarity among people of the black race may be related to the flat occipital protuberance of the skull among African people.

Adult↗

[Computerized tomography in cochlear otosclerosis].

After a review on osseous labyrinthine modifications in cochlear otosclerosis, the authors report their experience, based on four patients, in CT evaluation of the disease. CT high resolution programs detect demineralization centres, which are distinctive of cochlear otosclerosis, at an early stage. CT must be considered a more informative investigation than multidirectional tomography.

Adult↗

CT densitometry of the cochlear capsule in otosclerosis.

Cochlear otosclerosis can be recognized in most cases by high-definition axial and coronal CT images. However, routine technique does not allow a quantitative measurement of the changes. Reported here is a study of cochlear CT densitometry. The densitometric profile of the cochlear capsule was obtained in 10 ears with normal hearing and 50 ears in 27 patients with known clinical otosclerosis and progressive mixed-type hearing loss. Patients with hearing loss had decreased absorption of as much as 60% in comparison to the normal range. In 42 ears with abnormal densitometric profiles, the changes were visibly apparent on the CT images, whereas in eight others, the densitometric profile alone demonstrated decreased absorption.

Adult↗

Fenestral and cochlear otosclerosis: computed tomographic evaluation.

Computed tomography has replaced conventional multidirectional tomography in the imaging of patients with the presumed diagnosis of otosclerosis. In addition to obliterative disease, which was easily diagnosed with multidirectional tomography, anterior and posterior foci of varying sizes can now also be visualized preoperatively. Active cochlear otosclerosis (otospongiosis) is diagnosed in the presence of foci of demineralization in the otic capsule. Diagnosis of these entities has been of great interest to our referring otologic surgeons.

Adult↗

Differential diagnostic value of tympanometry in adhesive processes and otosclerosis.

280 tympanograms were recorded in sound-conductive hearing loss to assess their value in the differential diagnosis of otosclerosis and adhesive process. A normal tympanogram can be recorded in otosclerosis, In 48% of adhesive processes a more or less normal impedance pattern can be demonstrated. Therefore, in evaluating tympanograms in adhesive processes, one must take into consideration the microscopic picture of the eardrum and its mobility.

Acoustic Impedance Tests↗

Otosclerosis and Meniere's syndrome: diagnosis and treatment.

Occasionally a patient with otosclerosis and a conductive hearing loss will develop typical findings of Meniere's syndrome in the involved ear, years later. A review of clinical and pathological studies in the literature and in our laboratory and clinic indicates a likely cause-and-effect relationship for these cases. The pathology and pathogenesis of the syndrome of otosclerosis and Meniere's syndrome is discussed. A stapedectomy/sacculotomy was used to treat 17 patients, 13 of whom acquired a satisfactory result in terms of improvement of hearing and control of vertigo. This technique and findings are described and discussed.

Adult↗

Cochlear and otoconial abnormalities in capsular otosclerosis with hydrops.

Temporal bones from four patients with capsular otosclerosis were examined by microdissection. Otoconia and abnormal crystalline deposits were studied by scanning electron microscopy and x-ray analytical methods. One patient showed more or less symmetrical invasion of the basilar membrane and osseous lamina by connective tissue from thickened endosteum adjacent to the larger anterior foci. In one ear sensorineural degeneration was circumscribed; in the other it was extensive and associated with cochleosaccular hydrops. Two other patients were deaf, with severe sensorineural degeneration; one had multiple active foci and evidence of cochleosaccular hydrops. In the fourth patient, who had small anterior foci, no specific inner ear pathology was found related to otosclerosis. In the hydropic labyrinths, apatite was present as abnormal deposits in the cochlear duct and as rigid crusts replacing the otoconia. Apparently these changes had been associated with abnormal labyrinthine fluid dynamics rather than with the otospongiotic process per se.

Aged↗