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On the basis of data obtained from different sources, the incidence of clinical otosclerosis in Sweden in the year 1981 was estimated to be 6.1 cases per 100,000 population. This figure is lower than others reported previously. The annual incidence of stapedectomies is 8.3 per 100,000 population.
A total of 2,985 stapedectomies were done during the 25-year period 1959 to 1984 in a busy urban otologic practice. One hundred twenty-three of these cases were found to have obliterative otosclerosis which resulted in a drill-out stapedectomy. Postoperatively, a minimum of 2 years follow-up yielded 98 of the original 123 drill-out cases constituting the study group. Several parameters will be evaluated: 1. Initial hearing gain (measured 3 months after surgery), 2. long-term hearing results (measured 2 years and beyond), 3. additional sensorineural hearing loss, and 4. probability of bilateral obliterative disease.
A prospective computed tomographic and complex-motion tomographic evaluation was performed in 18 suspected or proven cases of otosclerosis. Although there was good agreement between both methods, there was no greater diagnostic accuracy with computed tomography. However, computed tomography demonstrated the changes better than complex-motion tomography.
Serum biochemistry was monitored in 50 patients with otosclerosis confirmed surgically. Age distribution was 25 to 50 years (mean:30 years), and the group included 8 men. Constants determined were serum sodium, potassium, chlorine, iron, phosphorus, aluminium, magnesium, copper and alkaline phosphatase. All patients were free from systemic or metabolic disease. Results failed to demonstrate variations in sodium, potassium, iron, and chlorine concentrations when compared with controls, with non-significant differences in copper, magnesium, phosphorus, aluminium and alkaline phosphatases. These findings were observed both in young patients in whom the otosclerotic process is in the active phase, and in older patients, this excluding correlations between duration of the disease and variations in concentrations of specific ions. The presence of aluminium in the active otosclerotic process is therefore due to local factors of which the mechanism is unknown at the present time.
Forty-two patients (84 ears) with surgically confirmed otosclerosis were examined with a computerized tomographic scanner (Philips Tomoscan 310) equipped with a table swivel mechanism. All ears were scanned in the horizontal and a new semilongitudinal plane. In patients with an unimpaired bone conduction threshold, a normal labyrinthine capsule was found in all ears except two. Normal bone conduction does not exclude extensive labyrinthine otospongiosis. In cases with bone conduction impairment, the bony labyrinth appeared normal in about half the ears. In the other half, areas of bone resorption were present and a positive correlation was found between the degree of bone loss and the amount of bone conduction threshold loss.
The basis of the early detection of otosclerosis is dual: first, a better knowledge of the mechanism of the disease itself, which is of enzymatic origin, and the starting point of which is an antigen-antibody conflict between the otic capsule and the embryonic cartilaginous remnants; - second, the fact that the otospongiotic disease appears to be a genetic deafness with an autosomal inheritance and about 40% of genes penetrance. Consequently an early detection is absolutely essential to combat the disease at its beginning and to assure its prevention. The early diagnosis of the otospongiotic/otosclerotic disease is twofold: first, the early detection of stapedial fixation is given by systematic impedance-audiometry screening on school-children by means of stapedius reflex elicitation in order to detect a possible diphasic impedance change (on-off effect); - second, the early diagnosis of progressive cochlear deterioration is yielded through systematic bone-conducted audiometry testing in young children in families with an otospongiotic/otosclerotic history.
The patient with far advanced otosclerosis (a nonmeasurable bone-air gap) may appear superficially to be suffering from profound or total sensorineural impairment. Only by a careful history and examination can a proper diagnosis be made. Stapedectomy has produced satisfactory results in 46 per cent of these cases in our hands.
High-resolution CT is the method of choice in determining the extent of demineralization in otospongiosis. By comparing the maximum and minimum densities, this examination has become of clinical importance in the diagnostic approach to otosclerosis and in the follow-up of fluorine therapy.
The tinnitus on moist tubotympanical catarrh and otosclerosis had been registered before and after the operation in relation to the frequence and the audibility. 222 children ears and 55 adult with a moist tubotympanical catarrh had been studied. Of these 28 children 26 adult ears had a tinnitus. After the operation and after using a "Donaldson silicone Drain Tube" without relation of the tinnitus there had been reached a very good win of hearing and excepted for 6 adults, whose tinnitus only had been lower, and elimination of the tinnitus. In 65 patients from 71 patients with stapedectomy it was able to look out for the tinnitus before and after the operation. Before the operation 44 patients had no tinnitus and 21 patients had a very disagreeable tinnitus; 17 patients from these had after the stapedectomy no longer a tinnitus. The audibility of the tinnitus from 4 patients had been reduced very much. The result of hearing the management of the inner ear after the operation haven't had any relation to the tinnitus before the operation.
A case of operation for presumed otosclerosis. Discovery of a neurinoma between the branches of the stapes probably originating from the geniculate. Facial paralysis was not present either before or after the operation which, in fact, was only partial (a more extensive operation was refused later on). A curious F.P. "to cold", 30 years beforehand, recovered without sequelae.
This study of a possible relationship between the ABG and the remodeling of the niche in otosclerosis in a significant number of patients operated on by stapedectomy allows the authors to define six types of ABG shape, corresponding to various types of stapedial fixation and remodeling of the niche. But the difficulties in finding the real bone conduction threshold levels, which ABG shapes depend on, lead us first to explain the mechanism of the various dips and notches altering bone conduction curves; and second, to try to find a safe and sure method for precise evaluation of the real cochlear reserve in otospongiosis/otosclerosis. We conclude that the best method is the bone conduction speech audiometry, mainly the bone conduction speech Weber tes, which automatically shows the true cochlear reserve of the ear to be operated on.
Cochlear otosclerosis, in the histopathological sense, is not uncommon but remains a clinical enigma. Similarly the aetiology of otospongiosis has remained obscure. Nevertheless the enzymatic concept of the initiation and mechanism of the underlying process has been recognized and confirmed together with the essential function of the matrix vesicles forming the site of primary enzymatic calcification. It may be concluded that otospongiosis is a primary lytic process initiated by lysosomal enzymes of matrix vesicles activated in the cartilaginous cell rests of the area. The cause of the process is still unknown.
A retrospective study of 613 operations on 495 patients with otosclerosis was made in the Department of Otorhinolaryngology of Hospital General Universitario of Alicante between 1974 to 1992. The clinical-pathological characteristics of this series were studied. Statistical analysis of the results was made. The improvement in air-bone gap for conversational frequencies was 27 dB. Closure of the postoperative air-bone gap for conversational frequencies was 7.6 dB and the overall percentage of closure of this gap was 80.5%. Better auditory results were obtained with partial platinectomy than with stapedectomy. Nothing was inserted between the prosthesis and the vestibule, permitting a shorter, easier operation with no occurrence of perilympathic fistule.
OBJECTIVE: To assess the success of surgical treatment of otosclerosis by stapedectomy and replacement with prostheses and to compare the results with other series. DESIGN: 115 operations done in 109 patients at one of the ENT units of the General Hospital Kandy. RESULTS AND CONCLUSIONS: After post-operative periods varying from 1 to 6 years the patients were tested by audiometry. The treatment was successful in 86 (74.4%), partially successful in 21 (18.2%) and unsuccessful in 8 (6.9%). These results compare well with other published series.
Implant-generated surface potentials, or averaged electrode voltages (AEVs), were collected by means of the electrode-by-electrode (E-E) mapping variable mode strategy. Three topics were investigated. 1) Eighteen children under the age of 7 were tested and the E-E map of 4 of them was found deviant; all 4 children were deaf owing to meningitis. Some electrodes marked as failing by E-E mapping did not cause problems during device fitting, and electrodes not usable in device fitting showed normal AEVs in 1 child. Overall, the AEVs agreed well with abnormalities in the behavioral threshold (T) and comfort (C) levels. The E-E maps provided useful clues for the audiologist in most cases. 2) Repeated E-E mapping in 2 children who displayed large fluctuations over time of T levels suggested a fluctuation in (neural) responsiveness in 1 child and new bone formation in the other. 3) Although massive phase reversals of AEVs in 2 patients deafened by otosclerosis seemed to indicate a very permeable cochlear bone, stimulation in the pseudomonopolar mode across the basal turn did not affect T levels, and affected pitch perception in only 1 patient. Deviant AEVs from abnormal cochleas should, therefore, not be interpreted too easily as an indication of an electrode failure, faulty electrode placement, or inadequate tonotopy.
The authors report 1279 surgical operations of otosclerosis performed between 1980 and 1992 in 959 operated patients. They chose the autegenous vein or perichondrium interposition TeflonR piston; however this technique has improved over the past few years. The size of the stapedectomy has been diminishing; from total, then partial stapedectomy, at last to 0.8 mm across stapedotomy. The graft has become exclusively from venous origin and the diameter of the piston has been reduced from 0.8 to 0.6 then to 0.4 mm. Seven groups of patients have been examined according to the size of the incision for stapedotomy or stapedectomy and the size of the piston. The audiometric study was realized after one month, one year, three years, fine or even ten years after surgery. Comparative tests were made considering the sex and the age of the patient, the thinness of the graft, and surgical revisions. The audiometric study lied not only upon the audiometric Rinne's closing but also upon the bone conduction variation (postoperative bc-preoperative bc) in the course of time. The evolution of tinnitus, of vertigo has been, as well, the subject of a careful study in time according to the surgical techniques. The study shows that the audiometric results (Rinne's closing, bc evolution) are statically much better with total stapedectomy, then with partial stapedectomy, at last with stapedotomy in the first three postoperative years. The best audiometric results are obtained with wider pistons (0.8 mm diameter) and venous approach. The results regarding tinnitus and vertigos are dissimilar especially during the first operative year. After three years of evolution, the significant audiometric differences tend to reduce and the audiometric results become the same (no significant difference) whatever the surgical technique may be. After three years, simple, calibrated stapedotomy without interposition statically gives similar results in literature. However, each surgical technique may, though rather infrequently, produce some incidents or complications that undoubtedly influence the operator as for the choice of the surgical technique to use.
Computer-aided tomography (CAT) is the most informative method for imaging the morphologic substrate of an otosclerotic process. In otospongiosis the tomograms show compact bone demineralization sites to the front of the vestibule fenestra (the fenestral form) or round the cochlear osseous capsule (the cochlear form); this latter form is better detectable by densitometry. During the stage of "mature" inactive otosclerosis the labyrynth fenestra are exposed to partial or complete obliteration. Combinations of active and inactive foci in the temporal bone pyramid are seen in 38% of cases. CAT helps accurately define the cause of stapedoplasty failure: anvil necrosis and the prosthesis subdislocation. CAT should be an obligatory diagnostic component in examinations of patients with sound conduction abnormalities.
Imaging studies play an important role in the diagnosis of otosclerosis and in the clinical management of this disease. CT scanning at present is the tool of choice for the assessment of the labyrinthine windows and cochlear capsules. MR imaging thus far has had limited applications in the examination of the cochlear capsules but has been more useful in the assessment of the cochlear lumen prior to cochlear implant in patients with profound bilateral hearing loss.