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Statistical analysis of otosclerosis surgery performed by Jean Marquet.

The early and long-term hearing results of 1,681 primary otosclerosis operations performed by the same surgeon, Jean Marquet, were reviewed retrospectively and analyzed with very strict statistical standards. Significantly better short- and long-term results were achieved with the stapedotomy technique compared to total stapedectomy, mainly at the higher frequencies (4 and 8 kHz) important for speech discrimination. Whatever technique was used to open the footplate (micropick, microdrill, or laser), no statistical audiometric difference could be found. The results were equal whether or not the stapedial tendon was preserved. Perioperative problems like pronounced oozing, difficult anatomic relationships, and accidental perilymph aspiration could affect hearing at higher frequencies. The calibrated hole technique was equally as good as conventional oval window sealing in sealing of the fenestra to prevent fistula. The stapedotomy technique was found the safest, having fewer complications.

Adolescent↗

High-resolution computed tomographic evaluation of the cochlear capsule in otosclerosis: relationship between densitometry and sensorineural hearing loss.

Otosclerotic cochlear involvement is a rather frequent disease that has not been clearly understood in terms of diagnosis and management. Objective evaluation methods are needed to confirm the clinical diagnosis, investigate the relationship with hearing impairment, and validate the results of treatment. In this study two ear groups with bone conduction hearing loss (BCHL) were investigated with audiometry and high-resolution computed tomography (HRCT). In the first group (n = 22) the diagnosis of fenestral otosclerosis was confirmed at operation; the second group (n = 9) was composed of ears clinically suspicious for purely cochlear involvement. Additionally, a control group (n = 14) of otologically normal ears was also studied. Foci of demineralization were demonstrated in 58% of the ears in the two groups; the sensorineural hearing loss (SNHL) in those ears was significantly worse than in those with normal radiologic findings. Three methods of HRCT densitometry were used to determine the abnormal regions in the cochlear capsule; the results suggested that hypodense regions were consistent with a greater degree of SNHL, in contrast to the hyperdense ones in ears with better cochlear reserves. Agreement was found between the location of the density change and the frequency topography of the SNHL; densitometric values were correlated with the bone conduction thresholds for certain frequencies. It is concluded that the spongiotic foci are responsible for the SNHL, since there was a correlation between their location and the SNHL frequency. The determination of better hearing in those ears with sclerotic foci supports the hypothesis that the sclerotic phase may not be a healing process following the spongiotic phase, and that it can be the first stage of the disease.

Cochlea↗

Changing patterns of otosclerosis surgery in teaching institutions.

The rapidly dwindling number of patients with otosclerosis suitable for surgery has made a severe impact on this aspect of training and experience in our medical centers. Results secured in the past and expected in the future are now difficult to achieve. This trend has been analyzed with particular reference to the experience in the last 3 years at the University of Miami--Jackson Memorial Hospital--Veterans Administration Hospital Medical Center. The analysis of results and complications also compared patients operated on by residents vs. faculty. Hearing loss as a consequence of improperly performed stapes surgery or complications thereof can seldom be salvaged. Stapes surgery should be performed in medical centers and community hospitals only by designated "stapes surgeons."

Adolescent↗

The fate of the non-operated ear in otosclerosis.

Records of 300 consecutive patients who had only one ear operated on by stapedectomy and who received long-term followup were studied. These cases came from 3036 stapedectomy operations performed between January 1961 and April 1969. In general, the two ears behaved the same: if a "flat" sensorineural loss occurred in one ear, it was likely to develop in the other. Similarly, if one ear developed a high-tone loss, the other would do likewise. With the exception of acute fistula, there is no suggestion that the operation of stapedectomy predisposes an ear to late sensorineural problems. Patients with bone-conduction thresholds that are depressed at all frequencies when first examined should be advised that progressive sensorineural hearing loss may occur later in both ears. Accordingly, the benefit gained by stapedectomy may ultimately need to be supplemented by hearing aids. This study also revealed that a patient with clinical conductive otosclerosis in only one ear at first presentation had only a 50% chance of long-term benefit from stapedectomy.

Female↗

The effects of etidronate disodium on progressive hearing loss from otosclerosis.

A 2-year prospective double-blind study was performed to evaluate the role of etidronate disodium for the treatment of progressive hearing loss in patients with otosclerosis. A pulsed dosage regimen was used during the 2-year period and the patients were followed up with otologic and audiometric examinations. Although statistically significant differences were not achieved between the study and control groups, the study did reveal a trend toward stabilization or improvement in air conduction thresholds in some frequencies (1000 and 4000 Hz) and in bone conduction thresholds at other frequencies (500, 1000, and 2000 Hz). The incidence of adverse side effects was similar in the treatment and control groups. Although no definite conclusions can be drawn from this pilot study, the findings provide encouragement for performing a larger and longer-term study.

Adult↗

Superior semicircular canal dehiscence simulating otosclerosis.

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

Aged↗

Far advanced otosclerosis and intractable benign paroxysmal positional vertigo treated with combined cochlear implantation and posterior semicircular canal occlusion.

This paper presents a combined procedure for the management of intractable benign paroxysmal positional vertigo (BPPV) and profound hearing loss in a patient with far advanced otosclerosis. The procedure comprised of a posterior semicircular canal occlusion and cochlear implantation as one combined procedure in the same ear. The combined approach added little to the operative morbidity and proved effective in this patient's management. A search of the literature reveals this to be a unique case.

Cochlear Implantation↗

MRI of cochlear otosclerosis.

Cochlear otosclerosis is an uncommon cause of mixed and sensorineural hearing loss. This has a characteristic appearance on CT, producing a distinctive pericochlear hypodense double ring. However, its appearance on MRI is not as readily appreciated, producing a ring of intermediate signal in the pericochlear and perilabyrinthine regions on T(1) weighted images, demonstrating mild to moderate enhancement after gadolinium administration. Increased signal on T(2) weighted images may also be seen. Recognition of these MRI features is important as MRI may be the first modality of investigation, especially when patients present with symptoms indicative of sensorineural hearing loss. We review four patients who presented with sensoineural hearing loss, and who were imaged with MRI as the first line of investigation.

Cochlea↗

Laser stepedotomy for otosclerosis.

The argon laser microscope recently developed by the author is used to vaporize the stapes tendon, the posterior crus and a rosette of holes in the stapes footplate in the surgical treatment of otosclerosis. An autogenous vein--stainless steel piston assembly is used to reconstruct the stapes portion of the ossicular chain. The surgical technique and results in a preliminary series of 11 patients are reported. Rationale and advantages over conventional stapedectomy are discussed.

Audiometry↗

Failures in surgery for stapedial otosclerosis.

The causes for failure in surgery for stapedial otosclerosis are many. Most of these occur in the early postoperative period. Failures occurring later are due to either a breakdown of the reconstructed transformer system or to a labyrinthitis due to failure of the vestibular seal. The role of cochlear otospongiosis in producing late failures has not been sufficiently emphasized. This clinical review of 105 surgical failures examined consecutively during the years of 1975 through 1979 will demonstrate the importance of cochlear otospongiosis in this consideration. The fate of the unoperated-on ear in unilateral surgical failures will be demonstrated. Control of the progressive sensorineural hearing loss in these instances can be gained by the use of sodium fluoride therapy.

Adolescent↗

Complications after surgery for otosclerosis.

This study was based on a detailed review of 503 cases of otosclerosis that were operated upon by me from 1961 to 1968. The vicissitudes of anesthesia, disequilibrium, dead ears and unexpected emotional abberations as well as legal and technical problems are discussed. Stapedectomy has been one of the most rewarding and technically challenging procedures that has come along in my career so far in medicine.

Adult↗

The argon laser in surgery for chronic ear disease and otosclerosis.

The use of the argon laser in chronic ear disease and otosclerosis surgery is reviewed. Its advantages and limitations are discussed, and safe parameters for use established. The argon laser is an effective tool to remove, cut, and coagulate tissue. It was used in over 500 microsurgical otologic procedures without deleterious effects. One hundred primary laser stapedotomies were performed, and the audiological results were compared to those obtained using conventional small fenestra stapedectomy techniques. No significant statistical difference was present in the audiologic results after 1 year of observation. The clinical course of the laser stapedotomy patients suggested this technique was less traumatic than conventional techniques.

Chronic Disease↗

The nystagmus threshold in turning test in different age groups and in patients suffering from otosclerosis.

The threshold of nystagmus during angular acceleration and deceleration was investigated in two series of healthy persons with an average age of 20 and 42 years and in series of otosclerotic patients with an average age of 42 years. It was shown that age as such does not influence the thresholds, but a lesion in the inner ear, such as otosclerosis, causes higher thresholds even in younger subjects.

Acceleration↗

Otosclerosis surgery.

Results of otosclerosis surgery using the stapes footplate removal with various types of reconstruction are reported in 456 ears (360 patients). Primary success was obtained in around 95% while 83% retained their improvement over the years. The posterior crus on fascia has become the preferred method because of its stability and relative lack of inner ear complications. Inner ear damage is mostly due to perilymph fistulae, which should be promptly recognized and repaired, since revision after the ear has become deaf does not restore hearing. Some late inner ear losses may be due to reactions to foreign material introduced with the graft. Bilateral operations should not be done if the first operation was difficult or caused vertigo for several days.

Acoustic Stimulation↗

The pre- and postoperative ENG findings in clinical otosclerosis and the late hearing results.

125 patients suffering from otosclerosis underwent oto-neurological investigations preoperatively and again daily from the second to sixth day postoperatively. An abnormal ENG in the form of a spontaneous or positional nystagmus, directional preponderance or a diminished caloric reaction could be found preoperatively in 30% of the patients. No statistically significant difference between patients with or without abnormal ENG findings could be shown in the late postoperative hearing results of 3 years in a series of different types of operations. Nearly half of the patients had postoperative nystagmus. On the second day nystagmus beat towards the operated ear in 22.3% of the patients. On the sixth day there was nystagmus only in one-third of the patients and it then beat towards towards the operated ear in only 12.1% of the patients and away from the operated ear in 12.6%. No statistically significant correlation could be found between the nystagmus findings and the late hearing results after 3 years' observation in this series of different types of operation.

Adolescent↗

Revision surgery for otosclerosis.

The outcome of surgery was analyzed in 76 otosclerosis patients (82 operations) undergoing revisions during the period 1986-89. The ears were divided into 3 groups based on preoperative A-B gaps 1) larger than 25 dB, 2) between 10 to 25 dB, and 3) less than 10 dB. A final hearing gain of more than 11 dB was recorded in 76%, 40% and 10% of the ears in groups 1, 2 and 3, respectively. In the groups with conductive component none of the ears deteriorated. In the sensorineural group 2 patients suffered further loss of 10 and 29 dB, respectively, and one ear became deaf. At revision the most common causes of conductive impairment were found to be dislocation of the prosthesis, remnants of footplate or new bone growth. Fistula was suspected in 10 ears and verified in 5. Several reconstruction methods must be mastered.

Cochlear Implants↗

GC serum groups and otosclerosis.

Five genetic serum protein marker systems (HP, TF, GC, BF and PI) were studied in patients with otosclerosis and in controls. The distributions of GC phenotypes and alleles showed significant differences between patients and controls with an excess of the IF-allele and the IF-variant among the patients.

Alleles↗

Hearing recovery following large and small fenestra stapes surgery for otosclerosis.

Fifty-five ear of 49 patients with otosclerosis were operated on with either large fenestra stapes (stapedectomy; SDE) or small fenestra stapes (stapedectomy; STO) surgery. Pre-operative average hearing levels for the speech frequency ranges (0.5, 1, 2 kHz) were 56.1 dBHL for SDE and 61.0 dBHL for STO. These improved to 39.0 dBHL and 35.0 dBHL. Statistically, significant hearing recovery was obtained for a frequency range from 0.125 to 2 kHz for SDE and from 0.125 to 4 kHz for STO (p < 0.05, Dunnett's multiple variance test). The degree of post-operative hearing improvement for STO was significantly higher at 0.5, 2 and 8 kHz than that for SDE (p < 0.05, Student's t-test). A pre-operative speech discrimination score of 80% was obtained at 68.9 dB for SDE and at 76.0 dB for STO. These values improved to 56.1 dB and 49.5 dB post-operatively. Thus, it was proved that STO results in better hearing than SDE does.

Adolescent↗