Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “OTOSCLEROSIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 91 records · Page 5Linked to original sources
PubMed1992

Aberration of the tissue collagenase system in association with otosclerosis.

Studies of aural and other body tissues suggest that otosclerosis represents the local manifestation of a general disorder of connective tissue. In particular, collagen abnormalities have been described. We have undertaken a pilot study of the in vivo messenger RNA (mRNA) transcription for procollagenase (precursor of collagenase), as well as for stromelysin and tissue inhibitor of metalloprotease (TIMP), an activator and a specific inhibitor of tissue collagenase activity, respectively. Human skin from individuals with surgically confirmed otosclerosis was compared to skin from their family members (clinically positive and clinically negative) and from unrelated normal controls. Preliminary data indicate that on average there are significantly lower levels of mRNA production for stromelysin among individuals with otosclerosis as compared to all others tested. Similar trends were demonstrated for TIMP and procollagenase, although these did not achieve statistical significance. In addition to suggesting a pathogenetic mechanism for the development of the disease, these data could serve as the basis of possible confirmatory tests for early diagnosis of otosclerosis and as a method for evaluating the genotype of offspring of affected individuals prior to their age of clinical manifestation. This could translate into the application of prophylactic treatment regimens in the future. The proposed abnormalities also suggest candidate genes for otosclerosis.

Audiometry↗
PubMedDate not supplied

[IgA, IgM and IgG levels and antibodies to native DNA and collagen type II in the perilymph of patients with otosclerosis].

Investigation of IgA, IgM and IgG levels in the serum of 20 and perilymph of 10 otosclerosis patients discovered a significant rise in the IgG concentrations versus control values. Five otosclerosis patients demonstrated perilymph and serum antibodies to collagen type II. Perilymph of 5 and sera of 10 otosclerosis patients exhibited a high titer of antibodies to native DNA specific for autoimmune process. The findings evidence immune disturbances in otosclerosis proving participation of autoimmune mechanisms in the disease pathogenesis. The results may be helpful in design of the conservative treatments of otosclerosis hypoacusis.

Adult↗
PubMed1991

Effect of sodium fluoride on early stages of otosclerosis.

The present investigation evaluates the effectiveness of NaF treatment in modifying the natural course of subclinical otosclerosis, as monitored by the stapedius reflex. The study was carried out on 128 relatives of patients suffering from surgically confirmed footplate otosclerosis. The diagnosis of subclinical otosclerosis was made on the basis of presence of the on-off effect. One group of subjects was treated with NaF in doses ranging from 6 to 16 mg according to age. The treatment lasted 2 years. A second group served as a control. Changes in stapedius reflex morphology were evaluated at 1, 2, and 5 years from the onset of treatment. The investigation demonstrated that NaF has a stabilizing effect on early otosclerosis. This drug, in fact, arrests the disease process in more than 60 percent of ears at the 2-year follow-up and in more than 50 percent at 5 years. A program of secondary prevention of otosclerosis by NaF is suggested.

Adolescent↗
PubMed1993

A genetic study of otosclerosis in a population living in the north of Tunisia.

The frequency of otosclerosis has been estimated to be 0.6 per 100 inhabitants in a population living in the North of Tunisia. The sex ratio in probands is 0.73 with clinical otosclerosis being approximately twice as frequent in females than in males, an observation which could be due to hormonal factors. The main risk period for otosclerosis is between 25 and 35 years of age in both sexes. Segregation analysis was performed in 193 nuclear families belonging to 65 pedigrees of otosclerosis. The pattern of the disease is due to a rare dominant major gene with a high polygenic component. This finding was unexpected since otosclerosis is usually considered to be a disease with simple dominant inheritance and incomplete penetrance. The authors have estimated that only 13% of affected patients are carriers of the rare dominant gene. This gene has strong penetrance which, however, varies according to age and sex.

Adolescent↗
PubMedDate not supplied

[The presence of antibodies directed against specific cartilagenous collagens in patients with otosclerosis].

The etiology of otosclerosis is unknown. Some investigators showed the presence of high levels of antibodies titers against type II collagen, the major cartilage collagen, proposing that the pathogenesis of otosclerosis is related to cartilaginous remnants contained in the otic capsule. Other collagens like type IX and XI, namely "Minor cartilage collagens", appear to be restricted to cartilaginous tissue. The aim of this study was to test otosclerosis patients for the presence of antibodies to these minor collagens using enzyme-linked-immunosorbent assays. Levels of antibodies to collagens type II, IX and XI were higher in these patients as compared to sex- and age-matched control subjects, while no differences were found between the levels of antibodies to collagens type I, III, VI and XI. These observations for the first time document the presence of autoantibodies against a minor collagens in patients with otosclerosis and support a possible role for collagen autoimmunity in the etiopathogenesis of otosclerosis.

Adult↗
PubMed1993

[Cartilage-specific autoimmunity in otosclerosis].

The otic capsule of patients with otosclerosis contains premature bone with numerous cartilaginous remnants. Some investigators have proposed that the pathogenesis of otosclerosis is related to these cartilaginous rests in the otic capsule. In this study we investigated the presence of a humoral immune reaction against cartilage-specific antigens using ELISA-methods in patients with otosclerosis. Concomitantly, 8 age- and sex-matched healthy blood donors, free of any symptoms of autoimmune disease, served as controls. The following antigen substrates were used: collagen (I, II, III, VI, IX and XI), chondrocytes and chondrocyte membranes. Findings then showed that the levels of antibodies to collagens type II and IX as well as to chondrocytes were higher in the otosclerosis patients than in the control subjects. The high titer of antibodies against chondrocytes was not accompanied by an increase in antibodies against the chondrocyte membranes. To our knowledge these observations represent the first evidence for the existence of autoantibodies against minor collagens and chondrocyte-specific antigens and support a possible role for a cartilage-specific autoimmunity in the etiopathogenesis of otosclerosis.

Adult↗
PubMed1993

Hearing aids and otosclerosis.

The purpose of this article on the use of hearing aids in patients with otosclerosis is to emphasize that all otosclerosis patients cannot be assumed to be good users of hearing aid amplification. Patients with losses of 60 dB or less and with purely conductive lesions may be able to use aids well. Individuals with mixed-type impairments due to otosclerosis may have significant problems utilizing amplification. These cases demonstrate the use of hearing aid amplification in patients with mixed-type hearing impairment due to otosclerosis. This is a very different group of patients from the group with a pure conductive lesion, in which there is little problem in fitting a hearing aid, provided that the instrument has sufficient power and gain to override the loss. A special subgroup of patients who have far-advanced otosclerosis and have successful stapes surgery to correct the conductive component of their hearing loss present a unique problem. They may initially suffer from severe recruitment requiring patience, special training and compression-type hearing aids.

Aged↗
PubMed1994

Endolymphatic hydrops associated with otosclerosis.

Endolymphatic hydrops (EH) associated with otosclerosis has been noted for many years. However, the causal relationship of these two entities remains controversial. Having reviewed the records of patients with otosclerosis describing fluctuant hearing loss and vertiginous symptoms, the authors found the EH may coexist with otosclerosis preoperatively; they may be two separate diseases that exist coincidentally; or EH may be caused by the otosclerotic process. Secondly, EH may occur with a fistula after surgery. Occurring after stapedectomy, EH may be caused by fistulization of the bony labyrinth, which is effectively treated by surgery to seal the fistula, which may cause EH to subside and hearing to improve. Thirdly, delayed EH may occur months or years after stapedectomy, possibly as a result of otosclerotic foci or surgical insult to the labyrinth. Dexamethasone, diuretics, and a room air rebreather can be used in the treatment of delayed EH. Hearing may be maintained or may deteriorate, but there usually is no dizziness. The clinical manifestations of EH associated with otosclerosis include a conductive or mixed type of hearing loss; the presence of fullness, tinnitus, fluctuation of hearing, episodic vertigo, an elevated negative summating potential (SP), and an increased summating potential:action potential (SP:AP) ratio shown by ECoG. This report presents five cases of EH associated with otosclerosis.

Adult↗
PubMed1997

Etidronate for the the neurotologic symptoms of otosclerosis: preliminary study.

The efficacy of etidronate, a bisphosphonate, was assessed as a treatment for the inner ear symptoms of otosclerosis in a retrospective case review of 896 patients diagnosed with otosclerosis, with primary complaints of dizziness, hearing loss, tinnitus or Meniere's syndrome. The diagnosis of otosclerosis was based on small-pixel computed tomography of the temporal bones. Of the 896 patients placed on an etidronate protocol, 545 were followed for more than six months and were analyzed. The symptomatic response to etidronate, as well as audiologic and computerized rotary chair results were used in the assessment. Patients who were previously on sodium fluoride were separately analyzed. In this preliminary study etidronate appeared to be an effective treatment for the neurotologic symptoms of otosclerosis. Prospective blinded efficacy studies of the bisphosphonates in the treatment of otosclerosis should be undertaken.

Adolescent↗
PubMed1996

HLA associations in otosclerosis in Japanese patients.

Otosclerosis is a disease of the otic capsule that is caused by abnormal resorption and redeposition of bony tissue. Sixty-two unrelated Japanese patients exhibiting clinical otosclerosis were typed for HLA-A, -B, -C antigens. Twenty-one of the patients were also typed for DR antigens. The frequency of HLA-Aw33 was significantly higher in otosclerosis patients than in the control group (24.2% vs 9.5%). This finding suggests that the presence of HLA-Aw33 antigens may be related to an increased susceptibility to otosclerosis or to its clinical outcome.

Adolescent↗
PubMed2006

Effect of cochlear reserve on postoperative outcome in otosclerosis.

Patients with elevated bone conduction (BC) thresholds are not considered a good candidate for otosclerosis surgery. Sometimes, it might be difficult to decide to operate these patients considering relatively poor cochlear function. However, viewpoints may vary among otologists. This study was undertaken to compare hearing outcome following otosclerosis surgery in patients who had bone conduction (BC) thresholds >or= 30 dB, and to investigate whether BC thresholds >30 dB has a negative impact on hearing outcome. Medical records of 111 patients who had undergone otosclerosis surgery were reviewed. Of 111 patients, 83 had undergone stapedotomy, and 28 stapedectomy. The patients were grouped based on preoperative four-tone BC threshold. Eighty-seven patients had average BC threshold 30 dB, and constituted poor-cochlear reserve group. Pre- and postoperative air conduction (AC) and BC thresholds, air-bone (AB) gap, vocal audiometry results and amount of deterioration in BC were determined. Mean postoperative AB gap was almost the same in both groups (14 and 15 dB) (P > 0.05). Percentage of AB gap = 10 dB favored good-cochlear reserve group (41 vs 29%)(P > 0.05). Analysis of mean hearing gain was slightly in favor of good-cochlear reserve group (19 vs 15 dB) (P > 0.05). Better BC thresholds were obtained postoperatively in good-cochlear reserve group (P < 0.001). Deterioration > 10 dB in BC was observed in 5.7 and 12.5% of the patients with good- and poor-cochlear reserve, respectively (P > 0.05). Based on the results of this small sample-size study, even though BC threshold of 30 dB was not considered a negative factor for hearing gain, otosclerosis surgery might have detrimental effects on postoperative BC thresholds in patients who had BC thresholds >30 dB.

Adult↗
PubMed2006

Case report: Meniere's disease and otosclerosis--different outcomes of the same disease?

The etiologies of Meniere's disease and otosclerosis are largely unknown. An association between these two diseases has been proposed on both a clinical and histopathologic basis but the causal relationship is controversial. In this paper we report two families in which both otosclerosis and Meniere's disease are inherited as independent phenotypes suggesting that the two diseases represent different outcomes of the same mutation. Thus the Meniere's disease occasionally seen in otosclerotic patients might not be caused by otosclerosis, but rather by a molecular defect leading to endolymphatic hydrops and/or clinical otosclerosis.

Aged↗
PubMed2006

Histologic changes in the anterior mallear ligament and the head of the malleus in otosclerosis.

OBJECTIVE: To determine ossicular and anterior mallear ligament (AML) changes in otosclerosis. STUDY DESIGN AND SETTING: Hyalinization of AML was graded as follows: none, patchy, or diffuse in 95 temporal bones (TBs) with otosclerosis: 52 with stapedial fixation (SF); 43 without fixation (NSF); and 52 age-matched controls. Fixation of the head of the malleus was noted. RESULTS: Hyalinization with SF was 17 none, 23 patchy, and 12 diffuse; with NSF it was 16 none, 20 patchy, and 7 diffuse; and in controls, 23 none, 24 patchy, and 5 diffuse. There was no significant difference in hyalinization among groups and no correlation between degree of hyalinization and age. The malleus head was fixed in 4 TBs with SF. CONCLUSION: Based on our findings, we do not believe that there is a relationship between hyalinization of the AML and otosclerosis; however, otosclerosis with SF seems to be a predisposing factor for fixation of the head of the malleus.

Adolescent↗
PubMed2004

[Cochlear otosclerosis and pseudo fourth turn of the cochlea].

OBJECTIVE: To describe different sequences on magnetic resonance imaging (MRI) in otosclerosis with peri-cochlear involvement. METHOD: MRI T1 and T2 sequences with T1 gadolinium injection and computed tomography (CT) scans with millimetric slices on axial and coronal views were obtained. The diagnosis of bilateral otosclerosis was confirmed by surgical exploration. RESULTS: On the CT scan, there was a fourth turn of the cochlea which appeared on the MRI T1 sequence with an intermediate signal and on the T2 sequences with a high intensity signal. After gadolinium injection, there was signal enhancement, suggestive of active otospongiosis. On the CT scan, there was another lesion in front of the cochlea with endosteal involvement. This was no however visible on the MRI, even after gadolinium infusion, in accordance with inactive otospongiosis. DISCUSSION: We reviewed the literature concerning MRI and results in otosclerosis. CONCLUSION: MRI of the labyrinth with T1 sequences and gadolinium injection can be contributive to the diagnosis of otosclerosis to differentiate inactive from inactive otospongiosis. However, prospective studies must be conducted to confirm this hypothesis.

Audiometry, Pure-Tone↗
PubMed2002

Otosclerosis: a genetically heterogeneous disease involving at least three different genes.

Otosclerosis is caused by abnormal bone homeostasis of the otic capsule, resulting in hearing impairment in 0.3%-0.4% of the white population. The etiology of the disease remains unclear and environmental as well as genetic factors have been implicated. We localized the first autosomal-dominant locus to chromosome 15 in 1998 (OTSC1) in an Indian family and, recently, we reported the localization of a second gene for otosclerosis to a 16 cM interval on chromosome 7q (OTSC2). In this study, we recruited and analyzed nine additional families (seven Belgian and two Dutch families with 53 affected and 20 unaffected subjects) to investigate the importance of these loci in autosomal-dominant otosclerosis. We completed linkage analysis with three microsatellite markers of chromosome 15 (D15S652, D15S1004, D15S657) and five microsatellite markers of chromosome 7 (D7S495, D7S2560, D7S684, D7S2513, D7S2426). In two families, results compatible with linkage to OTSC2 were found, but in the seven remaining families OTSC1 and OTSC2 were excluded. Heterogeneity testing provided significant evidence for genetic heterogeneity, with an estimated 25% of families linked to OTSC2. These results indicate that, besides OTSC1 and OTSC2, there must be at least one additional otosclerosis locus.

Chromosomes, Human, Pair 15↗
PubMed1999

Obliterative otosclerosis.

From January 1990 to December 1996, 293 primary stapedectomies for otosclerosis were performed, among which 14 had obliterative otosclerosis (4.7 per cent). Probability of bilateral obliterative disease was 50 per cent. With this particular condition, a drill-out procedure was used to perform either a stapedectomy or a stapedotomy. In two patients with bilateral 'far-advanced otosclerosis', surgery was effective in enabling the patient to benefit from hearing-aids. In patients with a measurable hearing-loss, an air-bone gap closure to within 10 dB was achieved in 62.5 per cent of the cases and to within 10-20 dB in 37.5 per cent of the cases, with no deterioration of air-conduction thresholds at 8 kHz. A mild sensorineural hearing loss at 4 kHz was observed in 25 per cent of the cases. There was no statistical difference between stapedectomy and stapedotomy. According to these results, the drill-out technique is a safe and effective procedure in cases of obliterative otosclerosis.

Adolescent↗
PubMed1992

[Stapes surgery in otosclerosis and small middle ear abnormality].

Stapes surgery in otosclerosis (first and revision operation) and in minor middle ear malformation follows the same principles, but the surgical problems are rather different. When analyzing three groups of patients, which had been operated on the same technique, we could show that the risks of the operation in malformation and revision surgery are not greater than they are in primary otosclerosis surgery. The audiologic results are more satisfying in the latter; malformation surgery and revision surgery have almost the same results which are lying somewhat below those of primary otosclerosis surgery. In the hands of an experienced surgeon, who is able to adopt his surgical technique at the individual situation, stapes surgery is nearly without risk in all these indications; an improvement of the air-conducting level of 18-23 dB can be expected and a social hearing above 35 dB can be provided in 83% of otosclerosis patients, in 79% of malformation patients and in 57% of revision patients.

Audiometry, Pure-Tone↗
PubMed2004

Temporal bone density measurements using CT in otosclerosis.

OBJECTIVE: To assess the bone density around the bony labyrinth in otosclerosis patients and to compare it to that of a control population. MATERIAL AND METHODS: This was a prospective case-control study. Ten patients with otosclerosis (mean age 42 years; range 24-55 years) and 33 control patients with vestibular schwannoma (mean age 46 years; range 20-71 years) were included. All patients underwent a clinical examination, audiometry and a CT scan comprising axial and coronal views of both temporal bones. In the otosclerosis group, audiometry showed unilateral involvement in six patients and bilateral hearing loss in four. The bone density was measured at the fissula ante fenestram (FAF) and at five other anatomical points on the bony labyrinth. RESULTS: In the control group, the bone density was similar at the six anatomical points. In the otosclerosis patients, the mean bone density at the FAF was lower than that in control patients (1649+/-99.1 vs 2049+/-13.4 HU; p < 0.01). For patients with FAF bone densities < 2000 HU, a correlation was observed between hearing threshold and FAF bone density. CONCLUSION: FAF bone density appears to be a good indicator of disease progression, and could serve as a follow-up and prognostic parameter.

Adult↗