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PubMed1990

Small fenestra stapedotomy using a fiberoptic hand-held argon laser in obliterative otosclerosis.

Obliterative otosclerosis has been a challenge since the advent of stapes surgery. "Drill-out" procedures have had a generally poorer prognosis than conventional stapes surgery because of excessive bleeding, acoustic trauma from the burr, and reclosure of the oval window by otosclerosis. In this report, we describe our early experience using a hand-held fiberoptic argon laser for small fenestra stapedotomy in 10 cases of obliterative otosclerosis. Closure of the air-bone gap to within 10 dB was seen in 100% of the patients. There was no significant sensorineural hearing loss, vertigo, or facial weakness. Argon-laser stapedotomy using a hand-held fiberoptic system is a safe and effective alternative to drill-out stapedotomy in cases of obliterative otosclerosis.

Fenestration, Labyrinth↗

PubMedDate not supplied

Advantages of CO2 laser use in surgical management of otosclerosis.

BACKGROUND: Otosclerosis is a progressive osteo-destructive disorder of the bony labyrinth in which the fixation of the stapes causes the hearing loss. The aim of this study was the postoperative determination of parameters of the effect of surgical intervention on hearing and the incidence of complications and, on the basis of the differences in the examined parameters of the study, the estimation of the efficacy of the two mentioned surgical techniques in the treatment of otosclerosis. METHODS: In our research 40 patients with conductive hearing loss caused by otosclerosis underwent surgery with CO2 laser. Functional results were compared postoperatively with the results of 40 patients operated by the classical technique without the use of CO2 laser. The research was accomplished as a prospective comparative study. RESULTS: The air-bone interval (gap) as the difference between the rim of air and bone conductivity for separate frequencies did not significantly differ between the control and the experimental group. Both methods were effective in closing the air-bone gap with the rates of closure to within 10 dB in 82.6% and 75.3% for the laser and drill, respectively. The incidence of tinnitus was significantly lower in patients who underwent surgery with CO2 laser. The frequency of intraoperative and postoperative complications was significantly lower in the laser group. Differences were statistically significant for all parameters (p < 0.05). CONCLUSION: On the basis of the degree of postoperative hearing improvement, tinnitus and the incidence of complications it can be concluded that the use of CO2 laser during inverse stapedoplasty represents an effective and safe method, justifying the promotion of its use in the surgical management of otosclerosis.

Adult↗

PubMed2004

Far advanced otosclerosis: stapes surgery or cochlear implantation?

OBJECTIVE: Nowadays, two surgical alternatives are to be considered in patients with far advanced otosclerosis: stapedectomy and cochlear implantation. The aim of this study was to analyze and compare the hearing and communicative results obtained in two groups of patients affected with far advanced otosclerosis, who were treated with the two different surgical procedures, to assess the proper surgical approach for these patients. DESIGN: Retrospective study. SETTINGS: University hospital and referral audiologic centre. METHODS: We submitted 11 adult patients, affected by far advanced otosclerosis and exhibiting unsatisfactory communicative abilities with hearing aids, to two different procedures: 5 to cochlear implantation, and 6 to stapedotomy followed by hearing aid fitting. The benefits afforded by the two procedures were assessed by means of pure-tone audiometry and speech audiometry in an open field and by means of a speech perception test. MAIN OUTCOME MEASURES: Hearing and communicative outcomes, measured 1 year after intervention. RESULTS: The results achieved in the patients subjected to stapedotomy were unpredictable and variable: very good in some cases but unsatisfactory in others, whereas those who had cochlear implantation all achieved satisfactory results with regard to speech recognition. CONCLUSIONS: Patients who underwent cochlear implantation achieved better hearing and communicative results than those who underwent stapedotomy. Even if the number of patients reported in this study is too small to provide definitive results, and despite the reportedly better results afforded by cochlear implantation, in our opinion, correction of far advanced otosclerosis should first be attempted via stapedotomy, and cochlear implantation should instead be reserved for cases in which stapedotomy is unsuccessful or for which imaging techniques unequivocally reveal a fenestral or cochlear obstruction.

Acoustic Impedance Tests↗

PubMed2003

Otosclerosis: exclusion of linkage to the OTSC1 and OTSC2 loci in four Italian families.

Otosclerosis is the single most common cause of hearing impairment among adult caucasians. Little is known about its aetiology and its molecular aspects. Until now, genetic linkage in otosclerosis has been demonstrated in an Indian family and a Belgian family, showing the presence of two otosclerosis loci, OTSC1 and OTSC2, respectively. Linkage analysis has never been applied to Italian otosclerotic families. We have collected four multigenerational Italian otosclerotic families that show dominant transmission for the pathology. Here, we report a detailed audiological analysis of these families and a genetic linkage study on the OTSC1 and OTSC2 loci. Statistical analysis revealed the absence of linkage between the disease in our families and the OTSC1 and OTSC2 loci. These data strongly suggest the presence of one or more additional loci for otosclerosis, which still need to be defined.

Acoustic Impedance Tests↗

PubMed1992

[Predictive value of high frequency audiometry in otosclerosis].

A study was carried out to evaluate the value of high-frequency audiometry in stapes surgery in cases of otosclerosis. The hearing function was measured pre- and postoperatively by means of conventional and high-frequency audiometry (Demlar 20k). The operative findings of the gradation of otosclerosis were compared with the pre and postoperative audiometrical measurements. The results of this study point out that a clear relation exists between the preoperative high tone audiogram and the gradation of otosclerosis. In conclusion, we state that high-frequency audiometry can predict the state of stapes fixation in otosclerosis and that can be important in stapes surgery.

Audiometry↗

PubMed2005

Otosclerosis--do we have a viral aetiology?

The etiology of otosclerosis remains an enigma though there are evidences suggesting a viral involvement. This study aimed to find out the relationship between viral infections and otosclerosis. Twenty two patients with otosclerosis and 10 healthy controls were included in the study. IgM antibodies to varicella zoster virus (VZV), measles, rubella, human cytomegalovirus (HCMV) and herpes simplex virus (HSV) were detected using micro ELISA. Paul Bunnel Davidsohn test was performed to rule out Ebstein Barr virus (EBV) infection. Overall, 5(22.7%) patients showed antibodies to one or more viruses. IgM antibodies against measles and VZV could be demonstrated in 4(18.1%) and 1(4.5%) patients respectively. None of the samples were found to be positive for HSV, HCMV, rubella and EBV antibodies. Controls were negative for all the viruses tested. The difference in seropositivity between the patient and control group was not statistically significant (p>0.05). Thus, this study suggests that otosclerosis is not commonly associated with a systemic viral infection.

Adolescent↗

PubMed2006

[Effects of different surgical techniques for otosclerosis].

OBJECTIVE: To compare the effectiveness of three surgical techniques for otosclerosis. METHOD: Retrospective analysis was made of patients with otosclerosis who had received surgical treatment during 1993-2005. There were totally 70 patients (76 ears) in which 30 patients (33 ears) underwent total stapedectomy with original stapes, 24 patients (27 ears) underwent small fenestra stapedotomy with Teflon piston artificial stapes and 16 patients (16 ears) underwent stapes mobilization or elevation. Both the hearing results including closure of postoperative air-bone gap and change of postoperative bone conduction threshold at different frequencies and percentages of postoperative vertigo control are compared statistically among three general groups and among two subgroups(stapes mobilization group with 5 cases/ 5 ears and stapes elevation group with 11 cases/ 11 ears). RESULT: There were no statistically significant differences in postoperative speech pure tone average threshold in closing air-bone gap among three groups. Compared with ears treated by otal stapedectomy with original stapes, ears treated by small fenestra stapedotomy and stapes mobilization or elevation, more decreased air-bone gap and lower rates of post-operative sensorineural hearing loss (SNHL) and dizziness at high frequencies were found. CONCLUSION: Although the acceptable effects were found in 3 groups, the better hearing threshold at high frequencies and lower incidence of complications can be obtained in patients with otosclerosis underwent small fenestra stapedotomy and stapes mobilization or elevation. It is suggested that small fenestra stapedotomy with Teflon piston artificial stapes is an ideal surgical treatment for otosclerosis.

Hearing Loss, Sensorineural↗

PubMed1990

Ultrastructural and immunohistochemical evidence of measles virus in active otosclerosis.

Because of the similarity between otosclerosis and Paget's disease of bone, and the mounting evidence of a viral cause in Paget's disease, we have investigated a possible viral cause for otosclerosis. Transmission electron microscopy of stapes footplate fragments with active otosclerosis has revealed structures morphologically identical with measles virus nucleocapsid in osteoblasts and preosteoblasts. Immunofluorescence and immunoperoxidase studies have confirmed the presence of measles nucleocapsid antigen in active lesions. Application of sera from patients with subacute sclerosing panencephalitis, a defective measles virus infection of the central nervous system, resulted in positive immunoreaction in areas of active otosclerosis.

Antigens, Viral↗

PubMed1990

Menière's symptoms resulting from bilateral otosclerotic occlusion of the endolymphatic duct: an analysis of a causal relationship between otosclerosis and Menière's disease.

An association between otosclerosis and Menière's disease has been proposed on both a clinical and temporal bone histopathologic basis for well over three-quarters of a century. Controversy persists over a causal relationship between these two entities, however, and the underlying pathophysiologic mechanisms relating capsular otosclerosis with Menière's disease remain speculative. The first case of total, bilateral endolymphatic duct occlusion resulting from extensive capsular otosclerosis is presented in a woman manifesting Menière's symptoms. The severe endolymphatic hydrops resulting from otosclerotic endolymphatic duct occlusion is given as histopathologic proof of a causal relationship between these two entities. Analysis of 18 of our own temporal bone cases of extensive capsular otosclerosis without endolymphatic hydrops and review of the literature indicates the uniqueness of such a firm causal relationship in the present case.

Adult↗

PubMed1988

Otosclerosis masking coexistent acoustic neuroma.

The coexistence of an acoustic neuroma with otosclerosis can prove to be a great diagnostic dilemma. In such cases, the diagnosis of acoustic neuroma is usually delayed while more common causes of sensorineural hearing loss associated with otosclerosis or its surgery are considered. Any asymmetric progression of a sensorineural loss or shift in discrimination after stapes surgery, whether sudden or gradual, should provoke suspicion of a second pathologic process. These include perilymph fistula, labyrinthine otosclerosis or ischemia, and acoustic neuroma. The use of auditory brainstem response and acoustic reflex testing and various imaging techniques is essential for accurate diagnosis. This paper includes two cases that demonstrate this dual pathology, bringing the total to 15 such cases reported in the literature. The purposes of this paper are to alert the clinician to the diagnostic problem of acoustic neuroma coexisting with otosclerosis and to propose a means of evaluating these patients.

Adult↗

PubMed1987

Genetic and functional analysis of the otosclerosis-like condition of the LP/J mouse.

The LP/J mouse is the only available genetic model for otosclerosis. The otosclerosis-like condition of the LP/J mouse resembles the human condition in several ways, although there are differences in the two species in the most common locus of the dysplastic otic lesions. The mouse model would be more useful if its auditory dysfunction and genetic inheritance could also be compared with the human disease. Matings of the otosclerosis-like LP/J and the normal CBA/J inbred mice were used to generate F1 and backcross generations. The F1 showed neither functional (electrocochleographic) nor anatomical abnormalities, indicating a recessive nature of the mouse genetic disease. This is in contrast to some reports of dominance (with incomplete penetrance) of otosclerosis in humans. No evidence of X-chromosome-linked genetic influence was found in the mouse, in agreement with the human condition. These interpretations were confirmed in tests of the backcross of the F1 to the CBA/J parental genotype (BC). The anatomical and functional abnormalities were present in some of the backcrosses of the F1 to the LP/J parental genotype (BL). A comparison of bone-conducted and air-conducted electrocochleographic responses provided evidence for both conductive and sensorineural losses in the LP/J and BL mice.

Action Potentials↗

PubMed1986

Sodium fluoride: effectiveness of treatment for cochlear otosclerosis.

We examined the effectiveness of sodium fluoride in treatment of cochlear otospongiosis in ninety-four patients with cochlear otosclerosis and ninety-eight patients with stapedial otosclerosis and sensorineural hearing loss. The drug halted or slowed the progression of sensorineural hearing impairment in 63% of the patients with cochlear otosclerosis and 46% of the patients with stapedial otosclerosis. The single factor that best predicted which patients would respond most favorably to treatment was rate of progression before treatment. Sodium fluoride therapy was successful for 79% of the patients losing their hearing at a rate of 5 dB or more per year at one or more of the speech frequencies. The finding that patients with more rapid rates of progression responded most favorably to sodium fluoride therapy suggests that patients with the most active otospongiotic processes will be the most responsive to treatment.

Cochlea↗

PubMed1979

Long-term effect of otosclerosis on bone conduction.

There have not been many studies of long-term sensorineural function in otosclerosis. The reports of long-term follow-up of lateral canal fenestration cases show a surprising stability of bone conduction. Because the experience of the senior author in stapes surgery suggests that some patients with clinically proven otosclerosis have a tendency for progression of sensorineural hearing loss, a review of over 500 ears in patients with proven otosclerosis was undertaken. Data from the study indicate that patients with otosclerosis have more sensorineural hearing loss than the general population. Also some otosclerotic ears have a tendency for progression of sensorineural hearing loss.

Audiometry↗

PubMed1978

The genetics of Otosclerosis. I. Distorted sex ratio.

The offspring of 214 otosclerotic x normal couples were investigated, and within these sibships, the segregation of otosclerosis is compatible with autosomal dominant inheritance. However, the overall sex ratio is approximately 0.73, with otosclerosis being approximately 1.8 times more frequent in female offspring. These observations are interpreted as the consequence of selection against males carrying the otosclerosis gene. Elimination of such males occurs only in certain sibships. The remaining families demonstrate a sex ratio approximating unity with similar rates of otosclerosis in both sexes. This selection operates mainly, and possibly only, prenatally.

Female↗

PubMed1978

[Studies on the genetics of otosclerosis (author's transl)].

101 families with confirmed otosclerosis were investigated, and after correction for age an incidence was found of otosclerosis of 29.5% amongst the parents, 17.9% amongst the siblings and 8.8% amongst offspring. The statistical data for otosclerosis published to date were treated similarly giving together with our own as large a collection as possible. From this the risk of repeat occurrence of otosclerosis could be calculated to 10% in the brothers and 20% in the sisters of otosclerotic patients. The risk rates of occurrence for their children are the same. These data are intended for use in the genetic counseling of this disorder.

Adolescent↗

PubMed1993

Carbon dioxide lasers for otosclerosis.

Over the past eight years, the senior author (SGL) has sought the ideal microsurgical laser for otosclerosis surgery. This quest required learning the principles of laser physics and of the interactions of light with matter. Rather simple thermocouple experiments were performed in the laboratories of the Midwest Ear Foundation (MEF) to confirm some of what was predicted from these principles. More sophisticated transmission spectroscopy experiments were performed at the Massachusetts Institute of Technology (MIT) to evaluate which electromagnetic wavelengths are best absorbed by collagen and bone. MIT's spectroscopy studies paralleled MEF's thermocouple data. CO2 laser energy (10.6 microns) is nearly completely absorbed by collagen and bone, whereas visible argon and KTP laser energy (0.5 micron) is poorly absorbed. Despite the better optical precision of visible lasers, the author chose the CO2 laser for stapedectomy revision and stapedotomy, because long infrared electromagnetic energy has vastly superior tissue absorption characteristics for these procedures. With the development of I. L. Med's Unilase, the otologic surgeon now has a CO2 laser with optical precision and convenience approaching that of visible lasers. Clinical studies of 102 consecutive CO2 laser stapedectomy revisions show that in 92% of patients, hearing was significantly improved with the new CO2 laser revision techniques. Nearly half of these patients had undergone one or more previous, unsuccessful, nonlaser revisions. For primary otosclerosis, CO2 laser stapedotomy has simplified a technically difficult operation while significantly reducing mechanical trauma to the inner ear. Most gratifying to the senior author is that after approximately 450 consecutive CO2 laser operations for otosclerosis, no patient has experienced significant postoperative sensorineural hearing loss (greater than 15 dB; mean 500, 1000, 2000, 3000 Hz). This fact alone has justified our theoretical and laboratory efforts to find the safest laser for otosclerosis surgery.

Carbon Dioxide↗

PubMed1994

Facial nerve stimulation produced by cochlear implants in patients with cochlear otosclerosis.

Thirty-eight adult cochlear implants have been performed at the University of Minnesota. Facial nerve stimulation by the implant in response to sound has been noted in four of these cases. Three of the four were patients whose sensorineural hearing loss was caused by cochlear otosclerosis. In each case it was possible to place the electrodes, however multiple leads had to be deprogrammed in order to avoid facial nerve stimulation. In each case characteristic radiographic findings of cochlear otosclerosis could be identified on preoperative temporal bone computed tomography scans. Although facial nerve stimulation has been described as a complication of cochlear implantation, it has not been reported to be associated with cochlear otosclerosis. Postoperative programming of the implant may be limited by facial nerve stimulation. In some cases these limits may reduce the efficacy of the device. This possibility should be taken into account during preoperative counseling of patients with cochlear otosclerosis considering cochlear implantation.

Aged↗

PubMed1996

The subjective meaning of illness in severe otosclerosis: a descriptive study in three steps based on focus group interviews and written questionnaire.

The aim of this study of otosclerosis patients was to highlight the circumstances specific to an operable middle ear disease and to describe the psychologically unique aspects accompanying this disorder. In a first step five subjects participated in four focus group interviews, from which emanated a questionnaire answered in a second step by 28 randomly chosen subjects. Finally a second series of supplementary focus interviews were conducted with another group of subjects, after which the interview material was subjected to code-mapping, agreement and validation. The study results indicate that ear surgery was a very important occurrence in the lives of the patients and a unique aspect was that they were burdened by a sense of responsibility for deciding in favour of or against the ear surgery and in choosing the time for operation. An event as tangible as an operation impacts on the individual's psychological processing of the fact that one of his or her senses is damaged. The message to the person's surroundings is very clear: the operation makes others understand how serious the situation is, gives rise to a sense of sympathy. These factors together may promote adaptation to the handicap, or alternately, at least for some persons, may impede adaptation to the necessary hearing aids. To some individuals hearing aids were strongly associated with periods of deteriorating hearing and therefore had negative connotations. Thus the hearing aid was used while waiting for surgery or instead of surgery. The constant hope of regaining one's hearing through ear surgery, although not totally realistic in these cases of severe otosclerosis, always makes the hearing aid the second best solution. The overall conclusion from this study is that there are specific circumstances of an operable ear disease which have great impact upon quality of life, well-being and adaptation. The psychological situation is one of instability, feelings of responsibility and at times of anxiety. Patients with otosclerosis are sometimes told that they are better off than others with impaired hearing, because surgery can improve their hearing and because good amplification through hearing aids can be achieved in persons with conductive hearing losses. It is our hope that this study has helped to provide a fuller picture of the facts of life for persons with severe otosclerosis.

Adaptation, Psychological↗