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PubMed2006

Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence.

In 2003, it was reported that superior semicircular canal dehiscence can mimic otosclerosis because of low-frequency bone conduction hearing gain and dissipation of air-conducted acoustic energy through the dehiscence. We report the case of a 17-year-old girl with left-sided combined hearing loss thought to be due to otosclerosis. Bone conduction thresholds were -10 dB at 250 and 500 Hz and she had a 40 dB air-bone gap at 250 Hz. When a tuning fork was placed at her ankle she heard it in her left ear. Acoustic reflexes and vestibular evoked myogenic potentials could be elicited bilaterally. Imaging of the temporal bones showed no otosclerosis, superior semicircular canal dehiscence or large vestibular aqueduct, but a left-sided, Mondini-like dysplasia of the cochlea with a modiolar deficiency could be seen. Mondini-like cochlear dysplasia should be added to the causes of inner-ear conductive hearing loss.

Adolescent↗
PubMed2007

A seventh locus for otosclerosis, OTSC7, maps to chromosome 6q13-16.1.

Otosclerosis is a common form of hearing impairment among white adults with a prevalence of 0.3-0.4%. It is caused by abnormal bone homeostasis of the otic capsule that compromises free motion of the stapes in the oval window. Otosclerosis is in most patients a multifactorial disease, caused by both genetic and environmental factors. In some cases, the disease is inherited as a monogenic autosomal dominant trait, sometimes with reduced penetrance. However, families large enough for genetic linkage studies are extremely rare. To date, five loci (OTSC1-5) have been reported, but none of the responsible genes have been cloned yet. An additional locus, OTSC6, has been reported to the HUGO nomenclature committee but the relevant linkage study has not been published. In this study, a genome-wide linkage study was performed in a large Greek pedigree segregating autosomal dominant otosclerosis. A seventh locus, OTSC7, was localized on chromosome 6q13-16.1 with a multipoint LOD score of 7.5 in the 13.47 cM region defined by markers D6S1036 (centromeric) and D6S300 (telomeric). Linkage analysis of this new locus in 13 smaller Belgian and Dutch families has identified one family from The Netherlands in which allele segregation suggests linkage to this region. The overlap between the critical regions of these two families is a 1.06 Mb interval between the genetic markers D6S1036 (centromeric) and D6S406 (telomeric) on chromosome 6q13.

Chromosome Mapping↗
PubMed2003

The aetiology of otosclerosis: a review of the literature.

During the last century, hundreds of studies have been performed to identify factors that are involved in the aetiology of otosclerosis. These studies include a variety of aetiological factors and, although many theories have been postulated, the process of the development of the disease remains unclear. A historical overview and analysis of the literature dealing with the aetiology of otosclerosis is presented. The role of collagen disorders, genetic linkage studies, associations with the HLA system and measles virus infection as causal factors is discussed. From an epidemiological point of view, the disease has an autosomal dominant mode of inheritance. Therefore, since the introduction of new genetic research techniques over the last decades, more studies have been performed to find evidence of a genetic factor that initiates the development of otosclerosis. In this review, we tried to categorize the most prominent studies in sections according to their subjects within the diversity of aetiological factors that have been studied.

Adolescent↗
PubMed2004

[Results with the Contour cochlear implant in patients with cochlear otosclerosis].

BACKGROUND: Results after cochlear implant surgery may be complicated by postoperative facial nerve stimulation. Aim of the study presented was to evaluate postoperative results in implanting the straight Nucleus electrode array and the preformed Contour array in patients with deafness due to cochlear otosclerosis. METHODS: A retrospective analysis of intra- and postoperative reports of all patients with cochlear otosclerosis was carried out. Results with the Nucleus straight electrode array and the Contour array were compared with regard to postoperative facial nerve stimulation. RESULTS AND CONCLUSION: None of the Contour patients (n = 7) presented with postoperative facial nerve stimulation. This is in contrast to the majority (4 of 6) of patients being implanted with the straight electrode array. Our results indicate that the use of the Contour array is advantageous in patients being at risk for facial nerve stimulation. In addition intraoperative reports suggest a more reliable insertion of the Contour electrode array in cochlear otosclerosis with partial obliteration.

Cochlear Implants↗
PubMed2001

Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases.

The aim of this study was to determine if a relationship exists between bone level thresholds and the extension of otosclerotic foci within the otic capsule. The study consisted of a retrospective case review in a university hospital. We included patients who underwent surgery for otosclerosis in our department and who had a CT scan prior to surgery. We analyzed the data charts and CT scans of 437 cases (386 patients). On CT scan, we distinguished patients with fenestral otosclerosis and/or with a pericochlear focus. A pericochlear focus could be extended (Group 2) or not (Group 1) to the cochlear endosteum. Data for Groups 1 and 2 were compared with those for the control group of all patients for whom CT scan showed no cochlear focus (Group 3). Of the 437 CT scans, 399 were positive (91.3%). An anterior focus was reported in 305 cases (69.8%), a footplate thickening in 21 cases (4.8%) and both anomalies were encountered in 60 cases (13.7%). A pericochlear focus was reported in 53 examinations. This focus was extended to the endosteum in 14 cases (26.4% of the pericochlear foci). In Group 1, preoperative air conduction (AC) thresholds were significantly lower than in the control group (p < 0.05). The air--bone gap was also significantly larger in Group 1 (p < 0.05). Bone conduction (BC) thresholds were lower in Group 1 than in the control group but the difference was not significant. In Group 2, preoperative AC thresholds were significantly lower than in the control group (p < 0.05). BC thresholds were also lower in Group 2 than in the control group and the difference was significant (p < 0.05). As a result of this study, we assume that there may be a relationship between bone level thresholds and the radiological extension of otosclerosis within the otic capsule.

Adult↗
PubMed2001

A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36.

Otosclerosis due to abnormal bone homeostasis of the otic capsule is a frequent cause of hearing loss in adults. Usually, the hearing loss is conductive, resulting from fixation of the stapedial footplate, which prevents normal ossicular vibration in response to sound. An additional type of sensorineural hearing loss may be caused by otosclerotic damage to the cochlea. The etiology of the disease is unknown, and both environmental and genetic factors have been implicated. Autosomal dominant inheritance with reduced penetrance has been proposed, but large families are extremely rare. To elucidate the pathogenesis of the disease, identification of the responsible genes is essential. In this study, we completed linkage analysis in a Belgian family in which otosclerosis segregates as an autosomal dominant disease. After excluding linkage to a known locus on chromosome 15 (OTSC1), we found linkage on chromosome 7q, with a multipoint LOD score of 3.54. Analysis of key recombinant individuals maps this otosclerosis locus (OTSC2) to a 16-cM interval on chromosome 7q34-36 between markers D7S495 and D7S2426.

Alleles↗
PubMed2001

Management of far advanced otosclerosis in the era of cochlear implantation.

OBJECTIVE: To evaluate issues pertaining to cochlear implantation in patients with far advanced cochlear otosclerosis. STUDY DESIGN: Prospective cohort. SETTING: Tertiary care referral center. PATIENTS: Eight adult patients (18 years of age or older) referred for management of profound hearing loss, the cause of which was determined to be otosclerosis. INTERVENTION: Cochlear implantation with multichannel cochlear implant device. MAIN OUTCOME MEASURES: Benefit from cochlear implant as measured by CID sentence scores, incidence and management of facial nerve stimulation, and technical issues pertaining to cochlear implantation in this patient population. RESULTS: All patients demonstrated significant improvement in auditory function as measured by performance on CID sentence scores and ability to engage in telephone conversation. Facial nerve stimulation was present in two of eight patients and was managed with deactivation of the stimulating electrodes. Ossification in the basal turn of the cochlea, detected on preoperative computed tomography, necessitated placement of the electrode into the scala vestibuli in two patients and use of a thinner electrode (Nucleus 24) in a third patient. CONCLUSION: Patients with profound hearing loss secondary to otosclerosis derive excellent benefits from cochlear implantation. Surgical implantation may be complicated by ossification of the cochlea, which can be detected on preoperative computed tomography. Electrode activation may be complicated by facial nerve stimulation, which can be addressed with programming strategies.

Adolescent↗
PubMed2003

Histologic changes on stapedial footplate in otosclerosis. Correlations between histologic activity and clinical findings.

OBJECTIVE: To quantify the relationship between the stage of histologic changes of the stapedial footplate in otosclerosis and the magnitude of preoperative hearing loss, tinnitus, vestibular disorder, and postoperative improvement of hearing. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: The study included 97 patients (ears) (69 female and 28 male patients), with conductive or mixed hearing loss who were operated on for otosclerosis. The criterion for including a patient in the study was otosclerosis established by tympanoscopy and confirmed by histologic examination of a piece of the stapedial footplate. MAIN OUTCOME MEASURES: By the histologic features of the stapedial footplate fragments, the stage of the otosclerotic lesion was classified as spongiotic, fibrotic, or sclerotic. The patients were carefully matched for sex, age, duration of hearing impairment, presence of tinnitus, and vestibular symptoms. Preoperative and postoperative air-conduction and bone-conduction thresholds were calculated as an average of four frequencies (0.5, 1, 2, and 4 kHz). Analysis was subsequently carried out on the preoperative and postoperative air-bone gap and bone-conduction threshold improvement. RESULTS: With regard to the histologic stage of otosclerotic lesions, tinnitus and vestibular disorders were present more frequently in patients with the sclerotic type of lesion. The type of otosclerotic lesion had no significant influence on the mean preoperative air-conduction threshold, bone-conduction threshold, and air-bone gap or on postoperative air-conduction threshold and bone-conduction threshold, but the postoperative air-bone gap was higher in patients with the fibrotic type of otosclerotic lesion and was highest in patients with the spongiotic type of otosclerotic lesion (p < 0.01). CONCLUSIONS: Tinnitus, vestibular disorders, and better postoperative closure of the air-bone gap are present more frequently in patients with a sclerotic type of otosclerotic lesion on the stapedial footplate.

Adult↗
PubMed2003

Increased activity of the diastrophic dysplasia sulfate transporter in otosclerosis and its inhibition by sodium fluoride.

HYPOTHESIS: This study investigates the function of the diastrophic dysplasia sulfate transporter (DTDST) in otosclerotic bone and the effect on it of sodium fluoride (NaF). BACKGROUND: Otosclerosis is a localized bone dystrophy with increased bone turnover. DTDST is implicated in the regulation of the bone turnover. MATERIALS AND METHODS: Primary cultures of cells were obtained from the stapes and external auditory canal (EAC) of 26 patients with otosclerosis and from nine control patients. Sulfate uptake was quantified under basal conditions and with NaF. The NaF signaling pathways were investigated using forskolin and verapamil. RESULTS: The relative initial rates of sulfate uptake and the apparent Vmax values were: otosclerotic stapes > EAC > control stapes = control EAC. The sulfate uptake by the otosclerotic stapes was correlated with the loss of sensorineural hearing. The amounts of DTDST mRNA (RNase protection assay) in the four subgroups did not differ. NaF (10(-6)M, 1 hr) inhibited sulfate uptake by the otosclerotic stapes and EAC cells but not by control samples. CONCLUSION: The authors believe that whether the increased DTDST activity is a cause or an effect of otosclerosis, it appears to be a specific target for NaF treatment.

Adult↗
PubMed2004

Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications.

OBJECTIVES: To collect data from a large number of cochlear implant recipients with otosclerosis and to make an assessment of these patients' clinical characteristics, computed tomographic scans, surgical findings, and complications, and to quantify the occurrence of postoperative facial nerve stimulation. STUDY DESIGN: Retrospective multicenter study. PATIENTS: Fifty-three patients with otosclerosis from four cochlear implant centers in the United Kingdom and The Netherlands were reviewed. Sixty surgical procedures were performed in these patients: 57 devices were placed in 56 ears. RESULTS: The computed tomographic imaging demonstrated retrofenestral (cochlear) otosclerotic lesions in the majority of patients. Although not statistically significant, the extent of otosclerotic lesions on the computed tomographic scan as categorized in three types tends to be greater in patients with rapidly progressive hearing loss, in patients in whom there is surgically problematic insertion of the electrode array, and in patients with facial nerve stimulation. In four patients, revision surgery had to be performed. Twenty of 53 (38%) patients experienced facial nerve stimulation at various periods postoperatively. CONCLUSION: Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.

Adult↗
PubMed2006

Adam Politzer (1835-1920) and the description of otosclerosis.

In 1893, Adam Politzer was the first to describe otosclerosis as a specific disease fixating the stapes. The aim of this study is to follow Politzer's research to understand how he finally explained the mechanism responsible for the fixation of the stapes. Politzer conducted his preliminary research from 1862 to 1893. From the concept of a dry catarrh of the middle ear, the fixation of the stapes became progressively associated with a specific ossification in and around the footplate. Politzer presented his first results in 1893. He completed his research by concluding in 1901 that otosclerosis had become an independent disease and should have the right to its own chapter in otologic books. He selected the word otosclerosis to describe this new pathologic entity.

Ear, Inner↗
PubMed2006

Perioperative recordings of vestibular-evoked myogenic potentials in otosclerosis.

OBJECTIVES: The aim of the present study was to investigate saccular function in patients with otosclerosis. Furthermore, the influence of stapedotomy on the vestibular-evoked myogenic potentials (VEMPs) should be demonstrated. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. METHODS: Bone-conducted tone-burst-evoked VEMPs were measured in 23 patients (25 ears) with unilateral or bilateral otosclerosis preoperatively and postoperatively. RESULTS: Preoperatively, VEMPs could be recorded in 11 ears (44%). There was no statistically significant correlation among the extent of preoperative sensorineural hearing loss, age, and VEMP measurements. Postoperatively, VEMPs were found in 14 ears (56%). In three cases (12%), VEMPs reappeared after surgery. The rare cases of preoperative vertigo could not be correlated to the nonappearance of VEMPs. CONCLUSION: Stapedotomy surgery does not influence VEMPs, implying that the saccular receptors are not injured by surgery. Moreover, in some cases, the elicitability of VEMPs was improved by stapedotomy surgery. Seemingly, otosclerosis can influence the generation of VEMPs most probably due to an involvement of the otolith organ's saccular receptors. No correlation was found between the clinical occurrence of vertigo and the elicitability of VEMPs.

Adult↗
PubMed2006

Glucocorticoids inhibit diastrophic dysplasia sulfate transporter activity in otosclerosis by interleukin-6.

HYPOTHESIS/OBJECTIVE: Otosclerosis is a bone remodeling disorder localized to the otic capsule and associated with inflammation. In vitro, increased activity of the diastrophic dysplasia sulfate transporter (DTDST), which is implicated in bone metabolism, has been reported. Because glucocorticoids modulate the bone turnover and inhibit inflammatory processes, we investigated the effect of dexamethasone (Dex) on interleukin-6 and DTDST in otosclerosis. STUDY DESIGN: The authors conducted a prospective, case-control study. MATERIALS AND METHODS: Primary cell cultures were obtained from stapes and external auditory canals in otosclerosis (n = 21) and control patients (n = 18). Assays with [H]Dex evaluated specific binding sites in otosclerotic and control stapes. The effects of Dex (10 to 10 M) and RU486 (10 M), a glucocorticoid antagonist, were studied on DTDST activity by sulfate uptake. IL-6 secretion was measured in culture media before and after Dex (10 M, 24 hours). The effect of IL-6 (10 M, 24 hours) was assessed on DTDST activity in control stapes. RESULTS: : The number of specific Dex-binding sites was similar in all stapedial cultures. Dex inhibited DTDST activity (19.4 +/- 1.02 vs. 29.4 +/- 3.94 pmol/microg prot/5 minutes) only in otosclerotic stapes. This effect was dose-dependent, antagonized by RU 486 and only observed 24 hours after Dex exposure. Interleukin (IL)-6 stimulated DTDST activity in normal stapes, whereas Dex inhibited IL-6 production only in otosclerotic stapes. CONCLUSION: Dex inhibits the DTDST activity, at least in part, through a reduction of IL-6 secretion only in otosclerotic cells. This effect is mediated through the glucocorticoid receptors and may lead to the reduction of bone turnover.

Analysis of Variance↗
PubMed1991

Unilateral otosclerosis and noise-induced occupational hearing loss.

The interaction between noise-induced hearing loss and otosclerosis remains unclear and controversial. In the present study, the effect of chronic noise exposure was examined in 31 patients with unilateral otosclerosis. Consideration was given to hearing loss at specific frequencies, the influence of age, and the effect of the size of the air-bone gap. A small, though statistically significant, protective effect of otosclerosis was demonstrated at 4 kHz. This effect was maintained despite advancing age. An apparent inverse relationship was found between the amount of hearing loss and the size of the air-bone gap at 4 kHz, but multiple linear regression analysis suggested that this finding was contaminated by age effects.

Adult↗
PubMed1985

Use of CT in the evaluation of cochlear otosclerosis.

Otosclerosis (otospongiosis) occurs when the hard endochondral bone of the otic capsule is replaced by spongy vascular foci of haversian bone. Using computed tomography (CT), we studied the ears of 32 selected patients with mixed or sensorineural hearing loss (one patient had normal hearing); 24 were suspected of having otosclerosis. CT proved valuable in detecting cochlear otosclerosis, foci of demineralization, and changes in bony texture and enables the easy recognition of subtle radiographic findings. Our paper also reports the CT findings of temporal bones in osteogenesis imperfecta and Paget disease.

Adolescent↗
PubMedDate not supplied

Dehiscences of the horizontal segment of the facial canal in otosclerosis.

Dehiscences of the bony horizontal segment of the facial canal are rather common in human adults. These facial canal dehiscences occur most often in the region of the oval window. This study presents the observations of the facial canal in the oval window region in 427 operations for otosclerosis. The incidence of dehiscences in the facial canal to the middle ear space in otosclerosis was studied. Dehiscences were observed in 14 of the 427 patients (3.27%) who had a stapedotomy. This incidence is very low compared to the anatomical studies of the facial nerve in human cadaver temporal bones. Dehiscences of the facial canal are a variation of the normal anatomy of the facial nerve and these dehiscences occur sporadically in otosclerosis.

Adult↗
PubMedDate not supplied

Far-advanced otosclerosis.

Patients with 'far-advanced' or 'very far-advanced' otosclerosis have a profound bilateral hearing loss, with apparently no cochlear reserve measured by standard clinical audiometers. These patients have no benefit from a powerful hearing aid, have severe feedback problems and the cochlear implant may be the only solution. A total of 12 stapedotomies were performed in 8 patients with far-advanced or very far-advanced otosclerosis, with satisfactory results, improved air conduction thresholds and improved cochlear function and speech discrimination. Stapedotomy is a useful procedure for patients with far-advanced otosclerosis which improves patient's hearing to levels where they can benefit from less powerful hearing aids with fewer feedback problems, compared to no measurable preoperative hearing aid benefit. It also spares those patients being submitted to cochlear implantation.

Adult↗
PubMed2001

Persistent measles virus infection and otosclerosis.

The cause of otosclerosis is still unknown. Recently, measles virus involvement has been implicated. The aim of this study was to analyze the presence of measles virus RNA within the otosclerotic focus and to evaluate the perilymphatic antibody pattern. Bone and perilymph specimens from 40 patients with the spontaneous form of otosclerosis and from control patients were investigated by reverse transcription polymerase chain reaction (RT-PCR), Western blot techniques, and cell culture. By the use of RT-PCR, measles virus RNA could be detected in 32 patients, but not in controls. Analysis of perilymph revealed the presence of antibodies to N, F1, and M measles virus proteins in all cases, and antibodies against H protein in 2 additional cases. In preosteoblasts cultured from otosclerotic bone chips, no measles virus RNA could be amplified. We conclude that the spontaneous form of otosclerosis is, in the vast majority of cases, a measles virus-associated disease of the otic capsule.

Adult↗