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PubMed2003

Otosclerosis and chronic tinnitus.

Chronic subjective tinnitus is a common feature of clinical otosclerosis. Analysis of the records of 1,014 consecutive cases of clinical otosclerosis, all confirmed by stapes surgery in South Australia between 1960 and 1972, gives a preoperative prevalence of this symptom of 65%. The association of tinnitus with various predictors is considered, and a statistical analysis is presented. Tinnitus has an association with gender (p < .0001), mean preoperative bone conduction (BC) level (p = .0012), mean air conduction (AC) level (p = .0192), and mean air-bone gap (p = .0075). The associations between tinnitus and the age of the patient, the duration of deafness, the presence of Schwartze's sign, and the severity of footplate pathological involvement were all nonsignificant. The association of tinnitus with the AC and BC thresholds is unexpectedly paradoxical. An economic predictive model for tinnitus in otosclerosis has been constructed from the 2 strongly significant variables, gender and mean BC hearing level, by logistic regression. In this large series of cases, the log odds in favor of finding tinnitus are about 0.810 for male subjects and 1.394 for female subjects when the BC level is zero. The log odds fall by 0.014 for each decibel of mean BC rise.

Adolescent↗
PubMedDate not supplied

Sensorineural impairment in unilateral otosclerosis.

Audiometric findings were evaluated in a group of patients with clinical unilateral otosclerosis. A comparison was made between bone conduction threshold in the better hearing ear and the ear with the mixed hearing loss. Over 50% of the patients had a significant elevation in the bone conduction thresholds in the ear with clinical otosclerosis as compared to the uninvolved ear. A pair of temporal bones from a patient with unilateral otosclerosis and unilateral mixed hearing loss is presented.

Adult↗
PubMedDate not supplied

Polygenic inheritance of otosclerosis.

A large family has been studied and its pedigree traced for six generations. Fifteen relatives are known to have had otosclerosis. Of these, the only six individuals who developed this disease before the age of twenty were offspring of second-cousin marriages. Other children in the extended family developed the disease later and may have had somewhat less severe symptoms. The original hypothesis that the severe, early onset cases occurred among those homozygous for a monogenic trait became improbable on mathematical analysis. We conclude that the inheritance of otosclerosis in this family is polygenic and probably multifactorial. Individuals marrying within the family have a greatly increased risk of giving birth to children who will develop otosclerosis early, and perhaps severly. Those who marry outside the family have a greatly decreased risk. They do, of course have a higher risk than the general population of having children who will at some point experience a conductive hearing loss.

Adult↗
PubMedDate not supplied

Sensorineural hearing loss in stapedial otosclerosis.

Controversy exists as to whether individuals with stapedial otosclerosis have elevated bone conduction thresholds due to cochlear involvement by the focus. A comparison was made between the Carhart-corrected bone conduction thresholds in 45 unoperated, non-noise-exposed individuals with stapedial otosclerosis and the thresholds in a matched random sample of the population with no conductive hearing loss. No consistent, significant difference was discerned. It is considered that individuals with stapedial otosclerosis do not in general have elevated bone conduction thresholds compared with control subjects. However, there was a suggestion that there may be a small, progressive elevation of bone conduction thresholds in older patients.

Adult↗
PubMedDate not supplied

1984 Santa Barbara state-of-the-art symposium on otosclerosis. Results, conclusions, consensus.

A state-of-the-art symposium on otosclerosis convened in Santa Barbara, California on Jan 6, 1984. The panel included some of the most distinguished and experienced otosclerosis surgeons in the world, with a collective experience of over 100,000 stapes operations. The purpose of the symposium was to develop guidelines for safe management of otosclerosis and to identify the at-risk patient for surgery. Topics included techniques developed by these surgeons through avoidance, recognition, and correction of technical problems; complications; and errors that lead to poor results. Discussed in order were patient evaluation, performance of surgery, and postoperative care.

Anesthesia, Local↗
PubMedDate not supplied

Osteogenesis imperfecta of the temporal bone and its relation to otosclerosis.

Osteogenesis imperfecta (OI) designates a heterogeneous group of heritable disorders of connective tissue that in addition to bone may affect tendons, ligaments, fascia, skin, sclerae, blood vessels, teeth, and hearing. The current classification identifies at least four major syndrome groups or types. It also recognizes a considerable number of additional syndromes that may represent supplementary types or subgroups. Loss of hearing is the least constant of the prominent features of OI. Its incidence varies between 26% and 60%. In OI, formation and remodelling of bone are variously affected. In the temporal bone the development of the inner ear capsule may be involved severely. In the stapes the disturbance in lamellar bone formation can lead to extreme thinness, dehiscence, and nonunion of the stapedial superstructure with the footplate. Osteogenesis imperfecta can be associated with otosclerosis, another bone dysplasia with a different morphology. Otosclerosis, in turn, may interfere with sound conduction and perception. Thus, the hearing loss encountered in OI may be the result of OI, otosclerosis, or a combination of both.

Adolescent↗
PubMed1979

Otosclerosis surgery: reassessment of its value in 1978.

Today, surgical correction is the treatment of choice for the hearing loss associated with footplate otosclerosis. Experience with stapes surgery reveals this to be functionally and economically superior to the long-term use of hearing aids. There is, however, a predictable rate of surgical complications. Sensorineural hearing loss, dizziness and/or facial nerve palsy have an immense social and economic impact upon the patient, the family and the surgeon. Otosclerosis surgery will be presented in the perspective of benefits, untoward results, and alternate forms of treatment. A cost analysis of the foregoing will shed light on the future of otosclerosis surgery.

Adult↗
PubMed1983

Otosclerosis: relationship of spiral ligament hyalinization to sensorineural hearing loss.

The sensorineural component of a mixed hearing loss due to otosclerosis is generally accepted as due to the otosclerotic lesion. The existence of pure cochlear otosclerosis without stapes fixation has been questioned. However, we are documenting 7 such cases in a separate publication. Results of this study, which evaluates 46 temporal bones with clinical or cochlear otosclerosis, demonstrate that the degree of sensorineural loss is directly related to the amount of hyalinization of the spiral ligament. The hyalinization occurs adjacent to active otospongiotic lesions but not next to inactive otosclerotic lesions. Both types of lesions may involve the cochlear endosteum. Small channels through the endosteal bone from the lesion to the spiral ligament have been found. The hyalinization spreads laterally from these channels. The hyalinization is presumably a result of the passage of toxic substances (proteolytic enzymes) from the lesion to the ligament. Strial atrophy is most pronounced on ligaments with the greatest degree of hyalinization. Hyalinization in only one ear produces decreased hearing compared to the other ear.

Cochlea↗
PubMed1995

The role and significance of chondroitin sulfate in the development of otosclerosis.

Chondroitin sulfate is a sulfated glycosaminoglycan that predominates in the ground substance of cartilage. Using monoclonal antichondroitin sulfate in 61 specimens of human otosclerotic lesions, we studied the distribution of this glucosaminoglycan in various stages of otosclerosis. Our findings show that chondroitin sulfate plays an important role in the development of otosclerosis. In addition, the distribution of chondroitin sulfate clearly delineates the stage of otosclerosis referred to as active into two distinct histologic stages. Dividing the active stage into "osteolytic" and "sponge-chondroid" would be reasonable based on our findings.

Cartilage↗
PubMed2002

Small fenestra stapedotomy for otosclerosis in a Canadian teaching centre.

OBJECTIVE: This study reports the results of 112 primary stapedotomies and 13 revision stapedotomies performed by the senior author. STUDY DESIGN: Retrospective case review of all primary and revision stapedotomies performed at University Hospital between 1994 and 1999. All patients in this series had otosclerosis and underwent stapedotomy using a 0.6-mm diameter platinum wire/Teflon piston prosthesis. The air-bone gap was calculated as the difference between the preoperative boneconduction and the postoperative air-conduction thresholds. The average follow-up time post-stapedotomy to audiometric testing was approximately 2.5 months. OUTCOME MEASURES: An average air-bone gap closure at 500, 1000, and 2000 Hz to 10 dB or less was used as the criterion for success. The effects of stapedotomy on speech reception thresholds (SRTs), speech discrimination scores (SDSs), and airconduction thresholds are also reported. RESULTS: In primary stapedotomy, an air-bone gap closure of 10 dB or less was achieved in 85.7% of patients. A significant hearing gain was achieved at all frequencies (250-8000 Hz), with the greatest benefit being achieved at the lower frequencies. The SRT was significantly improved post-stapedotomy by an average of 26.7 dB, and no significant change was found in the SDS. In revision stapedotomy, 38.5% of patients had a significant hearing gain at 250 to 4000 Hz. The SRT was significantly improved postoperatively by an average of 12.7 dB, and no significant change was found in SDS. Overall complication rates were similar to other series with two cases of partial hearing loss (1.6%), one incus fracture (0.8%), one large tympanic membrane perforation (0.8%), and one perilymphatic fistula (0.8%), which was successfully repaired. No patients in this series experienced complete sensorineural hearing loss, facial nerve injury, worsened tinnitus, or reparative granuloma. CONCLUSIONS: The results of this study are comparable to other similar studies examining the use of stapedotomy in patients with otosclerosis. The high success rate and low incidence of serious complications support stapedotomy, without a laser but with resident involvement, as a highly effective treatment for otosclerosis.

Adolescent↗
PubMedDate not supplied

The role of type II collagen autoimmunity in otosclerosis revisited.

A recent theory, suggesting that otosclerosis results from autoreactivity to type II collagen present in the fetal cartilaginous remnants of the human bony labyrinth, is based on two observations. Otosclerotic patients have increased concentrations of circulating antibody to type II collagen, and immunization of rodents with cartilage collagen induces 'otosclerosis-like' lesions. Independent researchers have been unable to confirm the first promising results. No significant abnormalities could be found in immunized animals. We report the result of type II collagen antibody recordings in a well described group of otosclerotic patients and controls, and the histological findings in temporal bones of MRL/1-mice with spontaneous type II collagen autoreactivity. Our results cannot support the view of autoreactivity to type II collagen as an etiopathogenetic factor in otosclerosis.

Adolescent↗
PubMed1994

Group specific component in serum and otosclerosis: no association.

An earlier Swedish study suggested a positive association between otosclerosis and the group-specific component GC*1F marker. We investigated the distribution of GC subtypes in 101 Danish patients with otosclerosis who all had surgery performed in the county of Funen. Compared to 1674 Danish controls we found no evidence of any association between markers from the GC serum protein system and otosclerosis.

Female↗
PubMed1995

Otosclerosis: a measles virus associated inflammatory disease.

The etiology of otosclerosis is still unknown. Immunohistochemical studies have revealed the characteristics of chronic inflammation in the otospongiotic area. Paramyxoviral structures have been identified by electron microscopy and the expression of measles virus antigen has been observed by immunohistochemistry in active otosclerotic tissue. By use of the polymerase chain reaction, measles virus related sequences have been detected in otosclerotic bone tissue but not in control specimens. The aim of our study was i) to detect measles virus genome in affected patients using a more sensitive PCR system and ii) to search for anti-measles virus IgG in the perilymph. In 13 out of 14 specimens of bone fragments from surgically removed stapedes of patients suffering from otosclerosis, measles virus RNA sequences could be detected while other tissues from the same patients and from a negative control group lacked such sequences. Furthermore, IgG anti-measles virus antibodies were detected in the perilymph of 6 patients. Our results support our previously stated hypothesis that otosclerosis is a measles virus associated disease which provokes a local immune response within the inner ear.

Adult↗
PubMed1996

Measles virus in otosclerosis and the specific immune response of the inner ear.

Histologic and immunohistochemical studies of otosclerotic lesions have shown that there is a chronic inflammatory reaction of the otic capsule with bone resorption resulting from vascular invasion accompanied by inflammatory cells. During the active lytic stage of otosclerosis, paramyxoviral structures have been identified by electron microscopy and measles virus antigen expression by immunohistochemistry. Recently, measles virus related sequences have been detected in tissue of otosclerotic lesions. Because the otosclerotic focus has a close relation to the perilymphatic space, the expression of measles virus antigens within it should represent an immunologic challenge to the immune system of the endolymphatic sac. In this study, measles virus specific antibodies were detected in all of the perilymph samples from 19 patients suffering from otosclerosis, and the relative amount of these IgG antibodies was much higher than in serum samples of the same patients or in perilymph of control patients. These findings support the hypothesis that measles viruses play an crucial role in the pathogenesis of otosclerosis.

Antibodies, Viral↗
PubMed2001

[Pathogenesis mechanism in ear fullness in otosclerosis].

To clarify the mechanism underlying ear fullness in otosclerosis, we studied the relationship between clinical features and examinations. Subjects were 116 otosclerosis patients (140 ears). The presence or absence of ear fullness was judged from a questionnaire in initial diagnosis or a chart description. Ear fullness was observed in 44 ears (31%) and absent in 96. The averaged air and bone conduction hearing levels (500 Hz-4 k Hz) in initial diagnosis were significantly lower in the group with ear fullness. The difference in averaged hearing was mainly apparent at 2 k Hz and 4 k Hz. The difference in hearing at lower frequencies (125 Hz, 250 Hz, and 500 Hz) and higher frequencies (2 k Hz, 4 k Hz, and 8 k Hz) was significantly larger in the group of ear fullness. From these results, we postulated that ear fullness in otosclerosis is caused by fixation of the stapes. The psychoacoustic abnormal sensation caused by lower input of lower-frequency sound or incomplete fixation of the stapes may cause ear fullness. Further study is needed to clarify the pathogenesis of ear fullness in different ear pathologies.

Adult↗
PubMedDate not supplied

[Otosclerosis surgery with CO2 laser].

Since Shea described the stapedectorny, many physicians have developed modifications of the technique, prosthesis or instrumentation. We have adopted one of the most recent advances to the treatment of otosclerosis, CO2 laser. Interventions were carried out in 20 consecutive ears using CO2 laser. Otosclerosis with type I, II and III foci were diagnosed and stapedectomies were reviewed. Stapedectomy was performed with a Causse prosthesis. We describe the technique and the laser power and application time used. The preoperative and auditory results at 1 month are reported. The advantages of opening otosclerosis with CO2 laser as the first or review procedure are analyzed.

Adult↗
PubMed1976

The use of electro-acoustic impedance measurements in detecting early clinical otosclerosis.

The first evidence that sodium fluoride (NaFl) can stop the otosclerotic process was recently presented. This development has placed new emphasis on the early detection of clinical otosclerosis. Electro-acoustic impedance measurements often detect minute changes in absolute impedance and compliance of the ossicular chain. The most valuable diagnostic information, however, is a negative on-off (biphasic) type of acoustic reflex. These results are often evident prior to the detection of positive clinical signs of otosclerosis. The negative on-off acoustic reflex is reviewed in this paper along with case discussions involving medical/surgical management of early otosclerosis.

Acoustic Impedance Tests↗
PubMed2000

Pure sensorineural hearing loss and otosclerosis. An imaging case report.

Pure sensorineural hearing loss is not a rare finding in otological practice. Numerous aetiologies could be at the origin of such a deficit. However, otosclerosis is very rarely cited as a cause of pure sensorineural hearing loss. We present one such case of pure sensorineural hearing loss linked to otosclerosis in a 30-year old caucasian male and underline the high contribution of computed tomography to confirm such a diagnosis. Pure sensorineural hearing loss due to otosclerosis is a rare event and can be misdiagnosed. The clinical diagnosis of such a disease may be difficult. In these cases, CT-Scan is the exam of choice to confirm the diagnosis.

Adult↗