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PubMed2006

Role of stapes surgery in improving hearing loss caused by otosclerosis.

The aim of our study was to evaluate the functional results of stapes surgery and to compare the effectiveness of small fenestra stapedotomy with that of total stapedectomy in improving hearing in patients affected by otosclerosis. Three hundred and fifty-seven consecutive ears, in 265 patients affected by otosclerosis, underwent surgery. All cases underwent either primary small fenestra stapedotomy (group A, 196/357, 54.91 per cent) or stapedectomy (group B, 161/357, 45.09 per cent). After surgery, 256/357 (71.71 per cent) cases showed a 0-20 dB gap. There were no significant differences in hearing results between the two groups at either early or late post-operative assessment. The mean post-operative pure tone average and air-bone gap results were slightly greater for group B than for group A, at both early and late post-operative assessments, but these differences were not statistically significant. Therefore, in group A, the mean pure tone average at 4 kHz significantly improved, from 56.60 to 47.66 dB at early post-operative assessment and to 52.98 dB at late post-operative assessment. Our study suggests that the technique of microtomy of the oval window is able to improve hearing results especially at high frequencies.

Adult↗
PubMed1975

Tympanometric curves and otosclerosis.

Patients with clinical otosclerosis (28 ears) were examined audiometrically and with an electroacoustic impedance bridge. The results were compared to corresponding findings for normal ears. The tympanometric curves of surgically proven otosclerosis were found to differ characteristically from those of normal ears. A curve typical of footplate fixation shows a rapid increase in acoustic impedance as external auditory canal pressure is reduced from ambient air pressure.

Air Pressure↗
PubMed2000

Oral vaccine in otosclerosis.

Otosclerosis is a genetically based disease in which the development of autoimmune mechanisms can lead to its clinical expression. Anti-type II collagen antibodies have been found in otosclerotic patient serum. The first clinically unexpressive phase of the disease can be diagnosed from a family history and ON-OFF stapedial reflexes with evidence of an autoimmune disorder. The use of antigenic oral hyposensitization is accepted, based on autoimmune pathogenesis. Hyposensitization should be initiated during this subclinical period of otosclerosis with a type II collagen oral vaccine.

Administration, Oral↗
PubMed1999

[Clinical aspects of the osteolytic (inflammatory) phase of cochlear otosclerosis].

During the last years it has been demonstrated that the active stage of otosclerosis (French: otospongieuse) is caused by an osteolytic inflammation associated with a measles-virus infection. Under influences that are not yet well understood (e.g. estrogens), the osteolytic process is arrested and changed to osteoblast activity accompanied by new bone formation. This latter process can be understood as a scar formation. If the process takes place within the area of the oval window, it results in fixation of the stapes and conductive hearing loss. When the otosclerotic process is restricted to the cochlea, clinical signs are not well defined. We report on exemplary cases of progressive bilateral sensorineural hearing loss caused by otosclerosis, restricted to the cochlea. The characteristics of the clinical course include: 1. Bilateral asymmetric sensorineural hearing loss. 2. Roaring tinnitus. 3. Episodes of sudden hearing loss. 4. Excellent response to prednisolone therapy. 5. In the late stage of the disease a small but transitory conductive hearing loss. High resolution computed tomography in such cases reveals diffuse osteolytic foci within the cochlear walls. In severe cases of such progressive deafness we discuss an antiproliferative (immunosuppressive) therapy with cyclosporine and/or a radionuclide therapy with Sn-117 m, an isotope with a very small irradiation radius.

Adult↗
PubMed1999

Hearing in patients operated unilaterally for otosclerosis. Self-assessment of hearing and audiometric results.

The objective of this study was to examine the outcome of unilateral stapes surgery in one patient group with bilateral hearing loss and one group with unilateral hearing loss. The patients' own estimations of improvement in hearing ability and the occurrence of other ear-related symptoms were examined retrospectively and in a follow-up study. Ninety-five of 123 patients operated for otosclerosis in only one ear between 1987 and 1992 responded to a follow-up examination. Observed audiometric findings and changes thereof, along with the patients' own estimations of their hearing handicap pre- and postoperatively, and the occurrence of other ear-related symptoms were studied. Despite good surgical results (closure of air-bone gap within 20 dB in 94%), 33% of the patients had severe hearing disabilities postoperatively, and many of these patients needed further amplification with a hearing aid. Mild dizziness occurred in 33% of the patients postoperatively and did not decrease over time. Discomfort in the operated ear due to strong sounds was reported in 20%. Change in sound quality occurred in 80% of the operated ears, but tended to disappear over time. From the results of this study it may be concluded that surgery in one ear only, leaving the other ear with poor hearing, is not an optimal hearing rehabilitation of patients with otosclerosis. It is important endevour to achieve bilateral hearing in order to give the patient good social hearing. Postoperative dizziness and unpleasant hearing quality do occur frequently, and the patients need to be informed about these problems preoperatively.

Audiometry, Pure-Tone↗
PubMed1992

Otosclerosis. Treating progressive hearing loss in young adults.

When a patient presents with bilateral progressive hearing loss and has a family history of hearing loss, otosclerosis should be placed high on the list of possible causes. Primary care physicians should determined whether the hearing loss is conductive or sensorineural with otologic examination and tuning-fork evaluation. Unless there is an obvious reversible cause for the progressive hearing loss, the patient should be referred to an otolaryngologist for more intensive otologic and audiologic examination. The patient's principal concern is the ability to hear and comprehend the spoken word in normal circumstances. Fortunately, most patients can be rehabilitated with surgery or a hearing aid. Fluoride treatment may have a significant role in preventing further hearing loss from otosclerosis.

Adolescent↗
PubMed1985

Familial combined hyperlipidemia and otosclerosis--the occurrence in a large kindred.

The occurrence of otosclerosis and hyperlipidemia in four generations of a single family is described. The lipid abnormality fulfilled the characteristics of combined familial hyperlipidemia. Whereas a genetic linkage between combined hyperlipidemia and otosclerosis is feasible, our study indicates that both conditions are inherited through autosomal but unlinked genes.

Adolescent↗
PubMed1999

Otosclerosis in the 1960s, 1970s, 1980s, and 1990s.

EDUCATIONAL OBJECTIVE: The focus of this paper is on the changing patterns of presentation of otosclerosis over the past 37 years. STUDY DESIGN: Retrospective chart review. SETTING: Private otology practice. PATIENTS: Randomized selection of 400 patients (100 per decade) from 15,372 who underwent stapedectomy over the past four decades. OUTCOME MEASURES: Extent of cochlear involvement, bilaterality of disease, length of history, degree of hearing loss, and pathological findings are noted. RESULTS: The decreasing incidence of footplates necessitating drillouts is discussed. Changing audiometric patterns at presentation such as decreased pure-tone average hearing thresholds and smaller air-bone gaps are also reviewed. CONCLUSION: This review demonstrates the changing patterns of presentation of otosclerosis over the past 37 years and will help guide the stapes surgeon into the year 2000.

Audiometry, Pure-Tone↗
PubMed2001

Pathophysiology of otosclerosis.

OBJECTIVE: To review current knowledge of the pathophysiology of otosclerosis and to review hypotheses for the amelioration of this disease. DATA SOURCES: Review of the literature and experimental observations by the authors. CONCLUSIONS: Otosclerosis is a localized disease of bone remodeling within the otic capsule of the human temporal bone. Unlike other similar bone diseases, it does not occur outside of the temporal bone. These lesions seem to begin by resorption of stable otic capsule bone in adults, followed by a reparative phase with bone deposition. There are clearly genetic factors that lead to this disease, but measles virus infection and autoimmunity also may play contributing roles. Surgical correction of the conductive hearing loss is highly effective, but nonsurgical intervention has not yet been shown to prevent or slow the disease. Of the factors that may inhibit this process, fluorides, cytokine inhibitors, and bisphosphonates, third-generation bisphosphonates appear to hold the most promise.

Bone Resorption↗
PubMed2001

Antimeasles immunoglobulin G in sera of patients with otosclerosis is lower than that in healthy people.

BACKGROUND: There is some evidence for an inflammatory process as a driving force in otosclerosis. Two popular hypotheses for the induction of this chronic inflammation have been proposed: an autoimmune phenomenon induced by an otic capsule specific antigen and measles virus infection. METHODS: Antibodies against measles virus hemagglutinin, polymerase, nucleocapsid, and matrix proteins were evaluated in sera from otosclerotic patients and in sera from healthy age-and sex-matched controls by use of the Western blot analyses. RESULTS: Significant differences were not detected between healthy men and women or between otosclerotic men and women. There were significantly stronger reactions against all viral proteins in the group of healthy women as compared with otosclerotic women despite a high standard deviation. The group of healthy male blood donors demonstrated significantly stronger reactions against polymerase and nucleocapsid proteins. Healthy blood donors again demonstrated stronger reaction compared with respective otosclerotic patients in a separate reaction for viral matrix protein. CONCLUSION: Our observation is consistent with viral participation in otosclerotic pathogenesis, but it is difficult to say if the diminished antimeasles humoral response is a consequence or the cause for a local measles infection. In light of the present data, we can discuss autoantibodies in otosclerosis as a sign of autoimmunity triggered by measles virus.

Adult↗
PubMed2002

Safety of the erbium:yttrium-aluminum-garnet laser in stapes surgery in otosclerosis.

OBJECTIVE: The purpose of this study was to present early and late bone-conduction hearing thresholds and data about cochlear and vestibular disturbances in patients after erbium:yttrium-aluminum-garnet (Er:YAG) laser stapedotomy in otosclerosis. STUDY DESIGN: The study design was a retrospective study. SETTING: The study was conducted at an academic tertiary referral center. PATIENTS: In this study, audiologic data of 117 patients undergoing Er:YAG laser-assisted stapedotomy for otosclerosis between 1993 and 1999 were included. MAIN OUTCOME MEASURES: The preoperative minus 2 postoperative (early, 1-3 days; late, at least 6 weeks) average pure-tone bone-conduction thresholds at 1, 2, and 4 kHz and 0.5, 1, 2, and 3 kHz were calculated. The postoperative appearance of nystagmus, vertigo, and tinnitus was analyzed. RESULTS: A total of 91 of 117 patients showed unchanged preoperative minus postoperative pure-tone bone-conduction averages at 1, 2, and 4 kHz in the late postoperative measurement. A slight deterioration was observed in 8 of 117 patients. Regarding the frequencies 0.5, 1, 2, and 3 kHz, 97 of 117 patients showed unchanged preoperative minus postoperative pure-tone bone-conduction averages. A new transient tinnitus appeared in 37 of 117 patients, and a new persistent tinnitus was found in 3 of 117 patients. Most of the patients had no postoperative dizziness (63/117 patients) and no postoperative nystagmus (109/117 patients). CONCLUSION: The study did not show significant sensorineural hearing loss at or below 3 kHz. Vestibular and cochlear function has no clinically relevant suppression after Er:YAG laser stapedotomy.

Adolescent↗
PubMed2005

Video-oculography findings in patients with otosclerosis.

OBJECTIVE: To evaluate the existence of vestibular irritation with video-oculography before and after stapes surgery and to examine whether there would be signs of specific end-organ irritation. STUDY DESIGN: A prospective study of preoperative and postoperative nystagmus, vertigo, and hearing thresholds. SETTING: University hospital, tertiary referral center. PATIENTS: Thirty-three patients (mean age, 47 yr) with otosclerosis. INTERVENTION: Stapedotomy/stapedectomy with laser or microdrill. MAIN OUTCOME MEASURES: Spontaneous, gaze-evoked, and head-shaking nystagmus was measured preoperatively and approximately 1 week, 1 month, and 3 months after the operation. Three dimensions of nystagmus were identified and their slow-phase velocities were calculated. RESULTS: Spontaneous horizontal nystagmus was found preoperatively in 18% (slow-phase velocities, 1.3-3.3 deg/s) and postoperatively in 11 to 19% of the patients (slow-phase velocities, 1.3-3.8 deg/s). Head-shaking nystagmus was not detected preoperatively. After the operation, 11 to 15% of the patients had head-shaking nystagmus (slow-phase velocities, 6.6-17.8 deg/s), but this prevalence did not differ statistically significantly from the preoperative level (p = 0.18). Vertical nystagmus was found equally pre- and postoperatively. Torsional nystagmus was not found. One week after the operation, nine patients (27%) had some sensation of vertigo, but it lasted over 1 month in only one patient. We found no significant correlation with vertigo and the types of nystagmus. CONCLUSION: Nystagmus with a low slow-phase velocity can occur in patients with otosclerosis. However, according to the video-oculographic findings and subjective symptoms, significant vestibular dysfunction seems to be rare and temporary after stapes surgery.

Adult↗
PubMed2006

Exploratory tympanotomy revealing incus discontinuity and stapedial otosclerosis as a cause of conductive hearing loss.

OBJECTIVE: To describe a unilateral progressive conductive hearing loss caused by incus discontinuity (without erosion of the long process of the incus), and otosclerosis with fixation of the stapedial footplate. STUDY DESIGN: Case report. SETTING: Department of Otolaryngology, Head and Neck Surgery of the University of Rochester Medical Center, which is a regional tertiary referral center. PATIENT: A 54-year-old woman with multiple otologic complaints including tympanic membrane perforations, otalgia, tinnitus, and hearing loss. Audiography demonstrated 100% speech discrimination bilaterally and a significant conductive right-sided hearing loss. INTERVENTION: The patient underwent a stapedectomy, during which a discontinuity between the long process of the incus and the stapes with no bony erosion was identified. The stapedectomy was completed and an ossicular piston prosthesis was inserted to reestablish ossicular continuity with the tympanic membrane. MAIN OUTCOME MEASURE: Improved subjective hearing confirmed objectively by audiography. CONCLUSION: This is the third reported case of an unusual combination of otosclerosis and ossicular discontinuity, and the first such case report in a patient without head trauma. In addition, it adds a unique item to the differential diagnosis of the pathologic features implicated in an ear with a conductive deficit and normal tympanogram.

Ear, Middle↗
PubMed2005

Does pregnancy affect otosclerosis?

OBJECTIVE: To evaluate the effect of pregnancy on the hearing of women with otosclerosis. STUDY DESIGN: A retrospective study of women who had undergone stapedectomy. The women were equally divided into two groups: one group with children and a control group without children. Air and bone conduction, as well as discrimination, were measured before and after stapedectomy in both groups. PATIENTS: Ninety-four women (47 with children and 47 without) were evaluated. Because many of the women had bilateral otosclerosis, the total number of ears studied was 128. RESULTS: Mean pure tone air and bone conduction thresholds were not worse in women with children versus those women without children. In fact, mean pre- and postoperative pure tone air and bone conduction thresholds from 500 Hz through 4,000 Hz in women with children were slightly but significantly better than women without children. There was no difference in discrimination scores between groups. Within the group with children, no significant correlation was found between number of children and hearing loss. Also, no correlation was found between breastfeeding and the amount of hearing loss. CONCLUSION: We found no adverse effect on hearing in otosclerotic women who had children compared with women without children. Even with increasing numbers of pregnancies, no deleterious impact was noted. Air conduction, bone conduction, and discrimination were not worse in women with children versus childless women. No significant correlation was found between the number of children and hearing loss, and neither did breastfeeding affect the amount of hearing loss.

Adult↗
PubMed1978

Combination of fibromuscular hyperplasia, renal aplasia, hypoplasia or dysplasia and otosclerosis occurring in the same individual or the same family.

We have found 3 reports of fibromuscular hyperplasia (FMH) of the renal arteries with hypertension occurring in 2 siblings as well as a few instances of renal agenesis and unilateral renal aplasia occurring in the same family. In this paper we report on FMH of the renal arteries in 2 women, one giving birth to a child with renal agenesis, the other to a child with a focally dysplastic hypoplasia of the kidneys. A third family is reported, heavily loaded with hypertension and otosclerosis, in which 2 siblings with FMH and otosclerosis were found. Another 4 patients without known heredity for hypertension, but with FMH combined with renal or urinary tract anomalies, are also reported. The findings are discussed, particularly in relation to the findings in a large material of chronic non-obstructive pyelonephritis, where in those with well maintained renal function, hypertension below the age of 40 was found predominantly in females with a positive family history of hypertension and signs highly suggestive of infected hypoplasia or dysplasia of the kidney.

Abnormalities, Multiple↗
PubMed1979

Mineral content of skeletal bones in otosclerosis.

In 63 patients with otosclerosis confirmed by operation, the bone mineral content was determined by photon absorptiometry. The bone mineral content and bone mineral concentration were found to be normal, which lends support to the assumption that otosclerosis is a localized disease and not a manifestation of a generalized disorder of the skeletal system.

Absorption↗
PubMed1984

Otosclerosis in South Australia.

In this paper we report on the prevalence of clinical otosclerosis in South Australia, and discuss some aspects of its distribution and character. Our data comes from an exhaustive search for stapedectomy patients in a relatively closed population within a fixed time span, and hence our figures represent a lower limit for the true prevalence and not an unbiased estimate. Nevertheless, even with no allowance for this downward bias it would seem that South Australia has a high prevalence of otosclerosis by world standards, particularly of obliterative cases. The highest prevalence is about 5 per 1 000 of population for men and 10 per 1 000 of population for women, which occurs in the 50-59 year old age group. In common with many other studies, our data shows a prevalence for females approximately twice that for males, over most age groups.

Adolescent↗
PubMed1993

Value of computed tomography (CT) in the diagnosis of cochlear otosclerosis.

The temporal bones of 40 patients with surgically confirmed otosclerosis were studied with high-resolution CT. The hearing of these patients was re-examined 5 years later. Foci of demineralization in the cochlear capsule were detected in 36% of operated ears and in 44% of otosclerotic ears not operated on. Mean bone conduction (BC) thresholds of ears with positive radiological findings did not differ significantly from those of otosclerotic ears with normal CT, either at the time of CT examination or 5 years later. Over 5 years, elevation of BC thresholds by 10 dB or more was observed significantly more often in operated ears with abnormal CT than in operated ears with normal CT, but in otosclerotic ears not operated on the corresponding difference was non-significant. It was concluded that CT examination is of little value in predicting sensorineural hearing loss in patients with otosclerosis.

Adult↗