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Michael J. Mckenna - One of the best experts on this subject based on the ideXlab platform.
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Otosclerosis temporal bone pathology
Otolaryngologic Clinics of North America, 2018Co-Authors: Reuven Ishai, Alicia M. Quesnel, Michael J. MckennaAbstract:: Otosclerosis is pathologically characterized by abnormal bony remodeling, which includes bone resorption, new bone deposition, and vascular proliferation in the temporal bone. Sensorineural hearing loss in Otosclerosis is associated with extension of Otosclerosis to the cochlear endosteum and deposition of collagen throughout the spiral ligament. Persistent or recurrent conductive hearing loss after stapedectomy has been associated with incomplete footplate fenestration, poor incus-prosthesis connection, and incus resorption in temporal bone specimens. Human temporal bone pathology has helped to define the role of computed tomography imaging for Otosclerosis, confirming that computed tomography is highly sensitive for diagnosis, yet limited in assessing cochlear endosteal involvement.
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lack of evidence for nonotosclerotic stapes fixation in human temporal bone histopathology
Otology & Neurotology, 2016Co-Authors: Alicia M. Quesnel, Reuven Ishai, Joseph B. Nadol, Fred H. Linthicum, Sebahattin Cureoglu, Ivan A Lopez, Michael J. MckennaAbstract:HYPOTHESIS Nonotosclerotic stapes fixation does not represent a significant cause of stapes ankylosis in patients undergoing stapedectomy; the vast majority have Otosclerosis. BACKGROUND Nonotosclerotic stapes fixation has been proposed as the diagnosis in 30 to 40% of patients undergoing stapedectomy (after excluding rare congenital, systemic, and syndromic causes of stapes fixation and tympanosclerosis). This finding was based on the histopathologic evaluation of total stapedectomy surgical specimens. Since these specimens do not include the surrounding otic capsule, the histopathologic evidence of Otosclerosis may be missed. METHODS Human temporal bone specimens from patients who underwent stapes mobilization, stapedotomy, or stapedectomy during life were evaluated for histologic evidence of Otosclerosis. Patients with a history of temporal bone trauma, tympanosclerosis, and congenital, systemic, or syndromic causes of stapes fixation were excluded. Therefore, most temporal bone donors carried a clinical diagnosis of Otosclerosis. RESULTS Two hundred ten specimens from three temporal bone collections were independently evaluated. Otosclerosis was found on histology in 99% (207/210). Therefore, the incidence of nonotosclerotic stapes fixation was 1% (3/210). In two of the three patients who did not have Otosclerosis, the contralateral temporal bone had Otosclerosis on histopathologic evaluation. These patients may have had Otosclerosis in the footplate only (which was removed at the time of surgery and not available for review). CONCLUSION Nonotosclerotic stapes fixation is not likely a distinct pathologic classification from Otosclerosis. Most patients diagnosed with nonotosclerotic stapes fixation likely have Otosclerosis, but do not have otosclerotic foci in the stapes itself.
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single nucleotide polymorphisms in the col1a1 regulatory regions are associated with Otosclerosis
Clinical Genetics, 2007Co-Authors: Wenjie Chen, Michael J. Mckenna, Guy Van Camp, Nicole C Meyer, Markus Pfister, D J Mcbride, K Fukushima, Melissa Thys, Richard J H SmithAbstract:Otosclerosis (MIM 166800) has a prevalence of 0.2-1% among white adults, making it the single most common cause of hearing impairment in this ethnic group. Although measles virus, hormones, human leukocyte antigen alleles and genetic factors have been implicated in the development of Otosclerosis, its etiology remains unknown. In a focused effort to identify genetic factors in Otosclerosis, we have mapped four disease loci (MIM 166800/605727/608244/608787); however, cloning the disease-causing genes in these intervals has not been successful. Here, we used a case-control study design to investigate the association between collagen type I genes and Otosclerosis. We identified susceptibility and protective haplotypes in COL1A1 that are significantly associated with Otosclerosis in the Caucasian population. These haplotypes alter reporter gene activity in an osteoblast cell line by affecting binding of transcription factors to cis-acting elements. Our data suggest that increased amounts of collagen alpha1(I) homotrimers are causally related to the development of Otosclerosis. Consistent with this hypothesis, mouse mutants homozygous for a Col1a2 frameshift mutation on a C57BL/6J background that deposit only homotrimeric type I collagen have hearing loss.
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Current research in Otosclerosis.
Current opinion in otolaryngology & head and neck surgery, 2006Co-Authors: Konstantina M. Stankovic, Michael J. MckennaAbstract:Purpose of review The aim of this article is to summarize and put into historical perspective current advances in research in Otosclerosis, a disorder of the human temporal bone with a hereditary predisposition that is among the most common causes of acquired hearing loss. Recent findings Genetic studies have revealed that Otosclerosis is heterogeneous, with evidence for defects in at least seven genes associated with six distinct chromosomal loci. Measurements of high levels of osteoprotegerin expression in the normal otic capsule and soft tissues of the cochlea provide the first molecular insight as to why the normal otic capsule remodels minimally, if at all. Osteoprotegerin knockout mice provide the best available animal model to date to study abnormal otic capsule remodeling that closely resembles Otosclerosis. There is mounting evidence that the measles virus plays an important role in pathogenesis of Otosclerosis although the mechanisms by which the virus results in Otosclerosis remain unknown. Quantitative measures of angiogenesis can reliably distinguish between clinical and histological Otosclerosis. Advances in the emerging field of osteoimmunology will likely impact and benefit from the research in Otosclerosis. Summary Insights into molecular mechanisms that inhibit extensive remodeling in the normal otic capsule, and understanding of how these mechanisms are dysregulated in Otosclerosis will allow future design of rational treatment strategies for Otosclerosis.
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association of Otosclerosis with sp1 binding site polymorphism in col1a1 gene evidence for a shared genetic etiology with osteoporosis
Otology & Neurotology, 2004Co-Authors: Michael J. Mckenna, Anh Nguyenhuynh, Arthur G. KristiansenAbstract:HYPOTHESIS There is an association between Otosclerosis and osteoporosis. BACKGROUND Both osteoporosis and Otosclerosis are common bone diseases to which relatively large portions of the population are genetically predisposed. Recently, a strong association has been described between osteoporosis and an Sp1 binding site of putative functional significance in the first intron of the COL1A1 gene. METHODS We applied polymerase chain reaction-based restriction enzyme analysis to determine the polymorphic distribution of the Sp1 site in 100 patients with Otosclerosis and 108 control subjects. RESULTS This study showed a significant association between Otosclerosis and the COL1A1 first intron Sp1 site. The allelic frequency of the Sp1 site is very similar between Otosclerosis and osteoporosis. CONCLUSION Some cases of Otosclerosis and osteoporosis could share a functionally significant polymorphism in the Sp1 transcription factor binding site in the first intron of the COL1A1 gene.
Isabelle Schrauwen - One of the best experts on this subject based on the ideXlab platform.
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Insufficient evidence for a role of SERPINF1 in Otosclerosis.
Molecular genetics and genomics : MGG, 2019Co-Authors: Hanne Valgaeren, Manou Sommen, Matthias Beyens, Geert Vandeweyer, Isabelle Schrauwen, Anne Schepers, I. Schatteman, Vedat Topsakal, Ingeborg Dhooge, Henricus P. M. KunstAbstract:Otosclerosis is a common form of hearing loss (HL) due to abnormal remodeling of the otic capsule. The genetic causes of Otosclerosis remain largely unidentified. Only mutations in a single gene, SERPINF1, were previously published in patients with familial Otosclerosis. To unravel the contribution of genetic variation in this gene to Otosclerosis, this gene was re-sequenced in a large population of Otosclerosis patients and controls. Resequencing of the 5′ and 3′ UTRs, coding regions, and exon–intron boundaries of SERPINF1 was performed in 1604 unrelated Otosclerosis patients and 1538 unscreened controls, and in 62 large Otosclerosis families. Our study showed no enrichment of rare variants, stratified by type, in SERPINF1 in patients versus controls. Furthermore, the c.392C > A (p.Ala131Asp) variant, previously reported as pathogenic, was identified in three patients and four controls, not replicating its pathogenic nature. We could also not find evidence for a pathogenic role in Otosclerosis for 5′ UTR variants in the SERPINF1-012 transcript (ENST00000573763), described as the major transcript in human stapes. Furthermore, no rare variants were identified in the Otosclerosis families. This study does not support a pathogenic role for variants in SERPINF1 as a cause of Otosclerosis. Therefore, the etiology of the disease remains largely unknown and will undoubtedly be the focus of future studies.
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COL1A1 association and Otosclerosis: a meta-analysis.
American Journal of Medical Genetics Part A, 2012Co-Authors: Isabelle Schrauwen, Ayda Khalfallah, Richard J H Smith, Megan Ealy, Erik Fransen, Charlotte Claes, Alexander M. Huber, Laura Rodriguez Murillo, Saber Masmoudi, Guy Van CampAbstract:Otosclerosis is a disease of abnormal bone remodeling in the human otic capsule that can lead to progressive hearing loss. Little of the underlying disease etiology has been elucidated thus far, although several studies have suggested that COL1A1 may play a role based on its importance in bone metabolism and other diseases like osteoporosis and osteogenesis imperfecta. Genetic association studies between COL1A1 and Otosclerosis, however, have been contradictory. To resolve this issue, we studied a large Belgian-Dutch and a Swiss population for a genetic association between COL1A1 and Otosclerosis and additionally performed a meta-analysis to investigate the overall genetic effect of COL1A1 on all Otosclerosis populations studied to date. We found a significant association both in the Belgian-Dutch population and in the meta-analysis. In aggregate, our analysis supports evidence for an association between COL1A1 and Otosclerosis although effect sizes of the variants reported in the initial studies are likely to be an overestimate of true effect sizes.
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Association of COL1A1 and TGFB1 polymorphisms with Otosclerosis in a Tunisian population.
Annals of human genetics, 2011Co-Authors: Ayda Khalfallah, Isabelle Schrauwen, Malek Mnejja, Hassen Hadj-kacem, Leila Dhouib, Mohamed Ali Mosrati, Bochra Hakim, Imed Lahmar, Ilhem Charfeddine, Nabil DrissAbstract:Otosclerosis is a condition characterized by an abnormal bone metabolism in the otic capsule, resulting in conductive and/or sensorineural hearing loss. Otosclerosis is a common disorder in which genes play an important role. Case-control association studies have implicated several genes in the abnormal bone metabolism associated with Otosclerosis: COL1A1, TGFB1, BMP2, and BMP4. To investigate the association of these genes with Otosclerosis in the Tunisian population, we examined nine single nucleotide polymorphisms (SNPs) in 159 unrelated Otosclerosis patients and 155 unrelated controls. We found an association of rs11327935 in COL1A1 with Otosclerosis that was shown to be sex specific. The coding polymorphism T263I in TGFB1 was also associated with Otosclerosis in the Tunisian population. The effect sizes of both the associations were consistent with previous studies, as the same effect was found in all cases. The association of BMP2 and BMP4 was not significant. However, a trend towards association was found for the BMP4 gene that was consistent with earlier reports. In conclusion, this study replicates and strengthens the evidence for association between polymorphisms of COL1A1 and TGFB1 in the genetic aetiology of Otosclerosis.
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The etiology of Otosclerosis: a combination of genes and environment.
The Laryngoscope, 2010Co-Authors: Isabelle Schrauwen, Guy Van CampAbstract:Otosclerosis is a common form of hearing loss characterized by abnormal bone remodeling in the otic capsule. It is a complex genetic disease, caused by a combination of genetic and environmental factors. During the past decade, several attempts have been made to identify factors for Otosclerosis. This review provides an overview of the current understanding of the etiology of Otosclerosis and describes the genetic and environmental factors that have been implicated in the disease. Environmental factors include fluoride and viral factors, particularly measles. Genetic association studies for Otosclerosis have reported several associations of genetic variants that influence the risk of disease, mainly involving bone remodeling pathways, although their individual risk contributions are small. Rare monogenic forms of Otosclerosis also exist, which are caused by a mutation in a single gene leading to a clear familial segregation of the disease. Linkage analysis of large Otosclerosis families has led to the identification of seven loci, and recently evidence was found that T cell receptor beta is a gene responsible for familial Otosclerosis, suggesting an underlying immunological pathway. However, this might also represent an autoimmune process, a hypothesis that is supported by other data as well. In conclusion, a variety of pathways have been identified to be involved in the development of Otosclerosis, showing that distinct mechanisms involving both genetic and environmental risk factors can influence and contribute to a similar disease outcome.
István Sziklai - One of the best experts on this subject based on the ideXlab platform.
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diagnostic value of cone beam ct in histologically confirmed Otosclerosis
European Archives of Oto-rhino-laryngology, 2014Co-Authors: Balázs Liktor, István Sziklai, Péter Csomor, Peter Revesz, Imre Gerlinger, Tamás KarosiAbstract:This retrospective case review was performed with the aim to asses the value of cone-beam computed tomography (CBCT) in the preoperative diagnosis of Otosclerosis. A total of 32 patients with histologically confirmed stapedial Otosclerosis, who underwent unilateral stapedectomies were analyzed. Preoperative temporal bone CBCT scans were performed in all cases. CBCT imaging was characterized by a slice thickness of 0.3 mm and multiplanar image reconstruction. Histopathologic examination of the removed stapes footplates was performed in all cases. Findings of CBCT were categorized according to Marshall’s grading system (from grade 0 to grade 3). Histopathologic results were correlated to multiplanar reconstructed CBCT scans, respectively. Histologically active foci of Otosclerosis (n = 21) were identified by CBCT in all cases with a sensitivity of 100 %. However, CBCT was unable to detect histologically inactive Otosclerosis (n = 11, sensitivity = 0 %). According to CBCT scans, no retrofenestral lesions were found and all positive cases were recruited into the grade 1 group indicating solely fenestral lesions at the anterior pole of stapes footplates. In conclusion, CBCT is a reliable imaging method with considerably lower radiation dose than high-resolution CT (HRCT) in the preoperative diagnosis of Otosclerosis. These results indicate that CBCT has high sensitivity and specificity in the detection of hypodense lesions due to histologically active Otosclerosis.
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Two Subgroups of Stapes Fixation: Otosclerosis and Pseudo‐Otosclerosis
The Laryngoscope, 2005Co-Authors: Tamás Karosi, József Kónya, Mihály Petkó, László Z. Szabó, József Pytel, József Jóri, István SziklaiAbstract:Hypothesis: Stapes ankylosis is a disease with variable histopathology and can be caused by Otosclerosis or pseudo-Otosclerosis. Viral pathogenesis of Otosclerosis could be established only by correlative analysis: histologic examination of the stapes footplate and reverse-transcriptase polymerase chain reaction (RT-PCR) amplification of the viral RNA. Background: Presence of the RNA genome of measles virus was demonstrated in the footplates of clinically otosclerotic patients by RT-PCR, and also viral proteins were detected by immunohistochemistry. Methods: Nucleic acids were extracted from ankylotic stapes footplates of clinically stapes fixation patients (n = 104). Measles virus genomic nucleoprotein (NP) RNA was amplified by seminested RT-PCR. Amplification results were correlated to postoperative histologic and audiologic findings. Results: Measles virus RNA was detectable only in histologically otosclerotic stapes footplates (n = 67). Histology for virus negative footplates (n = 37) excluded Otosclerosis. Virus negative stapes footplates showed nonotosclerotic, degenerative disorders. Conclusions: Stapes ankylosis is a heterogeneous disease causing conductive hearing loss with different etiologies. Nonotosclerotic stapes fixations could be established as pseudo-Otosclerosis and may belong to nonspecific, degenerative disorders with variable and noncharacteristic histopathology. Otosclerosis is an inflammatory disease caused by persisting measles virus infection of the otic cap-sule.
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Mechanisms of inner ear damage in Otosclerosis
2002Co-Authors: István SziklaiAbstract:It is observed for a long time, that the deterioration of hearing in Otosclerosis is faster than in an age-matched normal population. Also known is the so called cochlear Otosclerosis. The frequency of this type of Otosclerosis is estimated to be in between 1-2% of the Otosclerosis cases.
Michael M Paparella - One of the best experts on this subject based on the ideXlab platform.
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Histopathological incidence of facial canal dehiscence in Otosclerosis
European Archives of Oto-Rhino-Laryngology, 2011Co-Authors: Shigenobu Nomiya, Sebahattin Cureoglu, Norimasa Morita, Shin Kariya, Kazunori Nishizaki, Rie Nomiya, Michael M PaparellaAbstract:The objective of this study was to evaluate the histopathological incidence of facial canal dehiscence in Otosclerosis cases compared with non-otosclerotic controls. 133 temporal bones from 84 Otosclerosis (35 unilateral Otosclerosis, 49 bilateral Otosclerosis) cases were compared to 102 age-matched normal temporal bones from 70 subjects (38 unilateral normal cases, 32 bilateral normal cases). Temporal bones were serially sectioned in the horizontal plane at a thickness of 20 μm, and were stained with hematoxylin and eosin. We evaluated the location and the invasion of Otosclerosis to the facial canal and incidence of facial canal dehiscence under light microscopy. Facial canal was subdivided into four portions: (1) the geniculate ganglion, (2) the tensor tympani muscle, (3) the oval window, and (4) mastoid. The incidence of facial canal dehiscence in Otosclerosis [66 temporal bones (49.6%)] was significantly lower than normal controls [67 control temporal bones (65.7%)] in the oval window area ( P = 0.019). Temporal bones with otosclerotic invasion to the thin bone of the canal were significantly less likely to have dehiscence [10 temporal bones (31.3%)] compared to the otosclerotic bones without invasion [56 temporal bones (55.5%)] ( P = 0.025). There was no significant difference in the incidence of facial canal dehiscence between temporal bones with and without Otosclerosis in the entire segment of facial nerve. Our findings in this study suggest that otosclerotic lesions have the potential to close dehiscence of the facial canal in the oval window area.
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cochlear Otosclerosis adjacent to round window and oval window a histopathological temporal bone study
Otology & Neurotology, 2010Co-Authors: Sebahattin Cureoglu, Norimasa Morita, Teruyuki Sato, Kyoichi Terao, Shruti Joglekar, Armin Farajzadeh Deroee, Kazuo Ishikawa, Michael M PaparellaAbstract:HYPOTHESIS: The purpose of this histopathological study is to examine temporal bones of patients with cochlear Otosclerosis adjacent to the round window or adjacent to the oval window as compared with healthy controls. BACKGROUND: It is unclear if the extent and site of Otosclerosis affects the extent of damage to cochlear structures and hearing loss. METHODS: Twelve temporal bones from 10 patients with cochlear Otosclerosis adjacent to the round window, 11 temporal bones from 8 patients with cochlear Otosclerosis adjacent to the oval window, and 12 bones of healthy age-matched controls were selected for study. We calculated the number of spiral ganglion cells, changes in cochlear structures, the extent and site of cochlear Otosclerosis, and audiometric data. RESULTS: The loss of spiral ganglion cells and the absence of outer hair cells in patients with cochlear Otosclerosis adjacent to the round window were significantly higher than those in patients with cochlear Otosclerosis adjacent to the oval window and healthy controls. The area of the spiral ligament in patients with cochlear Otosclerosis adjacent to the oval window was significantly smaller than that in healthy controls. However, no significant difference was found in the spiral ligament of patients with cochlear Otosclerosis adjacent to the round window and healthy controls. There was no significant difference between patients with cochlear Otosclerosis and age-matched controls in audiometric data. CONCLUSION: Cochlear Otosclerosis adjacent to the round window caused significantly more damage to spiral ganglion cells and outer hair cells than cochlear Otosclerosis adjacent to the oval window without loss of spiral ligament.
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Cochlear Otosclerosis.
Current opinion in otolaryngology & head and neck surgery, 2010Co-Authors: Sebahattin Cureoglu, Muzeyyen Y Baylan, Michael M PaparellaAbstract:The aim of this study is to summarize current advances in research and clinical aspects of cochlear Otosclerosis. Recent studies have revealed that Otosclerosis is a process of bone remodeling that is unique to the otic capsule only. Even though no obvious bone remodeling is seen in the otic capsule under normal conditions, remodeling starts when some molecular factors trigger the capsule in certain patients who have genetic and/or environmental tendencies. Cochlear Otosclerosis is defined as Otosclerosis located in the otic capsule involving the cochlear endosteum and causing sensorineural hearing loss or mixed-type hearing loss. It has been clearly shown that, when Otosclerosis is sufficiently severe to involve the cochlear endosteum, it usually fixes the stapes as well.
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R436 – Association Between Chronic Otitis Media and Otosclerosis
Otolaryngology–Head and Neck Surgery, 2008Co-Authors: Sebahattin Cureoglu, Nomiya Rie, Nomiya Shigenobu, Schachern Patricia, Norimasa Morita, Shin Kariya, Kazunori Nishizaki, Michael M PaparellaAbstract:Problem In a previous clinical study, the incidence of chronic otitis media in cases of Otosclerosis was reported to be less than that observed in patients without Otosclerosis. Histopathologically, we can detect minimal changes such as histological Otosclerosis or silent otitis media which are not detected clinically. The purpose of this study is to reveal the association of Otosclerosis and chronic otitis media by evaluating human temporal bones, histopathologically. Methods 1235 human temporal bones were reviewed for this study. In order to match patients with Otosclerosis, patients with chronic otitis media were limited to 16 to 92 years of age. The incidence of Otosclerosis (clinical Otosclerosis, histological Otosclerosis) and chronic otitis media, either clinical (tympanic membrane perforation) or silent (without perforation) were analyzed. Results There was no statistically difference between the incidence of chronic otitis media in temporal bones with and without Otosclerosis. Conclusion The association of chronic otitis media and Otosclerosis appears to be a coincidental. Significance The incidence of chronic otitis media in cases of Otosclerosis is not less than that observed in cases of chronic otitis media in cases without Otosclerosis. Support International Hearing Foundation, Hubbard Foundation, Starkey Foundation.
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Association between cupular deposits and Otosclerosis.
Archives of otolaryngology--head & neck surgery, 2006Co-Authors: Hideo Hayashi, Sebahattin Cureoglu, Patricia A. Schachern, Mehmet Faruk Oktay, Hisaki Fukushima, Michihiko Sone, Michael M PaparellaAbstract:Objective: To evaluate whether Otosclerosis is an underlying mechanism for the production of cupular deposits and to study the association between cupular deposits and dysequilibrium in Otosclerosis. Design:Retrospectivehumantemporalbone(TB)study. The incidence of cupular deposits in these 70 TBs was analyzed.Correlationsbetweencupulardepositsandvestibular symptoms, endosteal involvement of the otosclerotic focus, stapedial fixation, and clinical history of stapes surgery were evaluated. Setting: Otolaryngology laboratory in a tertiary academic medical center. Patients:Thestudymaterialconsistedof35humanTBs with Otosclerosis and 35 age-matched controls. Main Outcome Measures: Morphometric evaluations of the incidence of cupular deposits, endosteal involvement of the otosclerotic focus, and stapedial fixationweremadebylightmicroscopy.Clinicalrecordswere reviewedretrospectivelyforclinicalhistoryofstapessurgery and prevalence of vestibular symptoms. The incidenceofcupulardepositswascomparedbetweentheotoscleroticandcontrolgroups.Correlationsbetweencupular deposits and vestibular symptoms, endosteal involvementoftheotoscleroticfocus,stapedialfixation,andclinical history of stapes surgery were evaluated in the subjects with Otosclerosis. Results: The incidence of cupular deposits in TBs with Otosclerosis was significantly higher than in those without whereas there was no correlation between the incidence of the deposits and dysequilibrium in cases of Otosclerosis. An increase in deposits did not correlate with stapedial fixation, stapes surgery, or endosteal involvement. Conclusions:Our results suggest Otosclerosis as an underlying mechanism for the production of cupular deposits; however, we did not find an association between these deposits and vestibular symptoms. Arch Otolaryngol Head Neck Surg. 2006;132:1331-1334
Sebahattin Cureoglu - One of the best experts on this subject based on the ideXlab platform.
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lack of evidence for nonotosclerotic stapes fixation in human temporal bone histopathology
Otology & Neurotology, 2016Co-Authors: Alicia M. Quesnel, Reuven Ishai, Joseph B. Nadol, Fred H. Linthicum, Sebahattin Cureoglu, Ivan A Lopez, Michael J. MckennaAbstract:HYPOTHESIS Nonotosclerotic stapes fixation does not represent a significant cause of stapes ankylosis in patients undergoing stapedectomy; the vast majority have Otosclerosis. BACKGROUND Nonotosclerotic stapes fixation has been proposed as the diagnosis in 30 to 40% of patients undergoing stapedectomy (after excluding rare congenital, systemic, and syndromic causes of stapes fixation and tympanosclerosis). This finding was based on the histopathologic evaluation of total stapedectomy surgical specimens. Since these specimens do not include the surrounding otic capsule, the histopathologic evidence of Otosclerosis may be missed. METHODS Human temporal bone specimens from patients who underwent stapes mobilization, stapedotomy, or stapedectomy during life were evaluated for histologic evidence of Otosclerosis. Patients with a history of temporal bone trauma, tympanosclerosis, and congenital, systemic, or syndromic causes of stapes fixation were excluded. Therefore, most temporal bone donors carried a clinical diagnosis of Otosclerosis. RESULTS Two hundred ten specimens from three temporal bone collections were independently evaluated. Otosclerosis was found on histology in 99% (207/210). Therefore, the incidence of nonotosclerotic stapes fixation was 1% (3/210). In two of the three patients who did not have Otosclerosis, the contralateral temporal bone had Otosclerosis on histopathologic evaluation. These patients may have had Otosclerosis in the footplate only (which was removed at the time of surgery and not available for review). CONCLUSION Nonotosclerotic stapes fixation is not likely a distinct pathologic classification from Otosclerosis. Most patients diagnosed with nonotosclerotic stapes fixation likely have Otosclerosis, but do not have otosclerotic foci in the stapes itself.
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Histopathological incidence of facial canal dehiscence in Otosclerosis
European Archives of Oto-Rhino-Laryngology, 2011Co-Authors: Shigenobu Nomiya, Sebahattin Cureoglu, Norimasa Morita, Shin Kariya, Kazunori Nishizaki, Rie Nomiya, Michael M PaparellaAbstract:The objective of this study was to evaluate the histopathological incidence of facial canal dehiscence in Otosclerosis cases compared with non-otosclerotic controls. 133 temporal bones from 84 Otosclerosis (35 unilateral Otosclerosis, 49 bilateral Otosclerosis) cases were compared to 102 age-matched normal temporal bones from 70 subjects (38 unilateral normal cases, 32 bilateral normal cases). Temporal bones were serially sectioned in the horizontal plane at a thickness of 20 μm, and were stained with hematoxylin and eosin. We evaluated the location and the invasion of Otosclerosis to the facial canal and incidence of facial canal dehiscence under light microscopy. Facial canal was subdivided into four portions: (1) the geniculate ganglion, (2) the tensor tympani muscle, (3) the oval window, and (4) mastoid. The incidence of facial canal dehiscence in Otosclerosis [66 temporal bones (49.6%)] was significantly lower than normal controls [67 control temporal bones (65.7%)] in the oval window area ( P = 0.019). Temporal bones with otosclerotic invasion to the thin bone of the canal were significantly less likely to have dehiscence [10 temporal bones (31.3%)] compared to the otosclerotic bones without invasion [56 temporal bones (55.5%)] ( P = 0.025). There was no significant difference in the incidence of facial canal dehiscence between temporal bones with and without Otosclerosis in the entire segment of facial nerve. Our findings in this study suggest that otosclerotic lesions have the potential to close dehiscence of the facial canal in the oval window area.
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cochlear Otosclerosis adjacent to round window and oval window a histopathological temporal bone study
Otology & Neurotology, 2010Co-Authors: Sebahattin Cureoglu, Norimasa Morita, Teruyuki Sato, Kyoichi Terao, Shruti Joglekar, Armin Farajzadeh Deroee, Kazuo Ishikawa, Michael M PaparellaAbstract:HYPOTHESIS: The purpose of this histopathological study is to examine temporal bones of patients with cochlear Otosclerosis adjacent to the round window or adjacent to the oval window as compared with healthy controls. BACKGROUND: It is unclear if the extent and site of Otosclerosis affects the extent of damage to cochlear structures and hearing loss. METHODS: Twelve temporal bones from 10 patients with cochlear Otosclerosis adjacent to the round window, 11 temporal bones from 8 patients with cochlear Otosclerosis adjacent to the oval window, and 12 bones of healthy age-matched controls were selected for study. We calculated the number of spiral ganglion cells, changes in cochlear structures, the extent and site of cochlear Otosclerosis, and audiometric data. RESULTS: The loss of spiral ganglion cells and the absence of outer hair cells in patients with cochlear Otosclerosis adjacent to the round window were significantly higher than those in patients with cochlear Otosclerosis adjacent to the oval window and healthy controls. The area of the spiral ligament in patients with cochlear Otosclerosis adjacent to the oval window was significantly smaller than that in healthy controls. However, no significant difference was found in the spiral ligament of patients with cochlear Otosclerosis adjacent to the round window and healthy controls. There was no significant difference between patients with cochlear Otosclerosis and age-matched controls in audiometric data. CONCLUSION: Cochlear Otosclerosis adjacent to the round window caused significantly more damage to spiral ganglion cells and outer hair cells than cochlear Otosclerosis adjacent to the oval window without loss of spiral ligament.
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Cochlear Otosclerosis.
Current opinion in otolaryngology & head and neck surgery, 2010Co-Authors: Sebahattin Cureoglu, Muzeyyen Y Baylan, Michael M PaparellaAbstract:The aim of this study is to summarize current advances in research and clinical aspects of cochlear Otosclerosis. Recent studies have revealed that Otosclerosis is a process of bone remodeling that is unique to the otic capsule only. Even though no obvious bone remodeling is seen in the otic capsule under normal conditions, remodeling starts when some molecular factors trigger the capsule in certain patients who have genetic and/or environmental tendencies. Cochlear Otosclerosis is defined as Otosclerosis located in the otic capsule involving the cochlear endosteum and causing sensorineural hearing loss or mixed-type hearing loss. It has been clearly shown that, when Otosclerosis is sufficiently severe to involve the cochlear endosteum, it usually fixes the stapes as well.
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R436 – Association Between Chronic Otitis Media and Otosclerosis
Otolaryngology–Head and Neck Surgery, 2008Co-Authors: Sebahattin Cureoglu, Nomiya Rie, Nomiya Shigenobu, Schachern Patricia, Norimasa Morita, Shin Kariya, Kazunori Nishizaki, Michael M PaparellaAbstract:Problem In a previous clinical study, the incidence of chronic otitis media in cases of Otosclerosis was reported to be less than that observed in patients without Otosclerosis. Histopathologically, we can detect minimal changes such as histological Otosclerosis or silent otitis media which are not detected clinically. The purpose of this study is to reveal the association of Otosclerosis and chronic otitis media by evaluating human temporal bones, histopathologically. Methods 1235 human temporal bones were reviewed for this study. In order to match patients with Otosclerosis, patients with chronic otitis media were limited to 16 to 92 years of age. The incidence of Otosclerosis (clinical Otosclerosis, histological Otosclerosis) and chronic otitis media, either clinical (tympanic membrane perforation) or silent (without perforation) were analyzed. Results There was no statistically difference between the incidence of chronic otitis media in temporal bones with and without Otosclerosis. Conclusion The association of chronic otitis media and Otosclerosis appears to be a coincidental. Significance The incidence of chronic otitis media in cases of Otosclerosis is not less than that observed in cases of chronic otitis media in cases without Otosclerosis. Support International Hearing Foundation, Hubbard Foundation, Starkey Foundation.
