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JAAPA : Official Journal of the... Feb 2017Otosclerosis is a complex and progressive disease of pathological bone remodeling that affects the otic capsule of the temporal bone, resulting in hearing loss. Although...
Otosclerosis is a complex and progressive disease of pathological bone remodeling that affects the otic capsule of the temporal bone, resulting in hearing loss. Although traditional diagnostic methods are still used, improvements in technology and research have paved the way for additional diagnostic techniques and advancements. The traditional treatment of otosclerosis, stapes surgery, is now being augmented or replaced by innovations in hearing aid technology and cochlear implants. Earlier diagnosis of otosclerosis can occur through understanding of the cause, risk factors, and current diagnostic testing.
Topics: Acoustic Impedance Tests; Audiometry; Hearing Aids; Hearing Loss, Conductive; Humans; Otosclerosis; Stapes Surgery; Tomography, X-Ray Computed
PubMed: 28060022
DOI: 10.1097/01.JAA.0000511784.21936.1b -
Advances in Oto-rhino-laryngology 2011Otosclerosis is one of the more common forms of adult-onset hearing loss with a prevalence of 0.3-0.4% in Caucasians. It is typically characterized by a conductive... (Review)
Review
Otosclerosis is one of the more common forms of adult-onset hearing loss with a prevalence of 0.3-0.4% in Caucasians. It is typically characterized by a conductive hearing loss due to labyrinthine endochondral sclerosis that upon stapedio-vestibular joint invasion results in loss of free motion of the stapes. Its etiology remains poorly understood with both environmental factors and genetic causes implicated in its development. Several environmental influences have been studied and numerous genomic loci have been mapped in families segregating autosomal-dominant otosclerosis. Population-based studies have also identified associations with several genes. These advances are improving our understanding of this complex disease.
Topics: Gene Expression; Genetic Linkage; Genetic Predisposition to Disease; Genome-Wide Association Study; Humans; Otosclerosis; Prevalence; Risk Factors
PubMed: 21358194
DOI: 10.1159/000322488 -
Otolaryngologic Clinics of North America Apr 2018Otosclerosis is pathologically characterized by abnormal bony remodeling, which includes bone resorption, new bone deposition, and vascular proliferation in the temporal... (Review)
Review
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.
Topics: Hearing Loss, Conductive; Hearing Loss, Sensorineural; Humans; Otosclerosis; Stapes Surgery; Temporal Bone; Tomography, X-Ray Computed
PubMed: 29397947
DOI: 10.1016/j.otc.2017.11.001 -
Current Opinion in Otolaryngology &... Oct 2010The aim of this study is to summarize current advances in research and clinical aspects of cochlear otosclerosis. (Review)
Review
PURPOSE OF REVIEW
The aim of this study is to summarize current advances in research and clinical aspects of cochlear otosclerosis.
RECENT FINDINGS
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.
SUMMARY
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.
Topics: Bone Remodeling; Cochlea; Hearing Loss; Humans; Otosclerosis
PubMed: 20693902
DOI: 10.1097/MOO.0b013e32833d11d9 -
European Archives of... Sep 2010The objectives of our study was to review our current knowledge of the etiopathogenesis of otosclerotic bone remodeling including genetics, viral infection, autoimmunity... (Review)
Review
The objectives of our study was to review our current knowledge of the etiopathogenesis of otosclerotic bone remodeling including genetics, viral infection, autoimmunity and inflammation and to discuss disease pathogenesis with relevance to pharmacotherapy. Relevant publications on the etiopathogenesis, molecular biology, genetics and histopathology of otosclerosis from 1984 to 2009 were analyzed. Otosclerosis is a bone remodeling disorder of the human otic capsule; however, the etiopathogenesis remains unclear. Genetic predisposition, disturbed bone metabolism, persistent measles virus infection, autoimmunity, and hormonal and environmental factors also may play contributing roles in the pathogenesis of otosclerosis. Since diagnosis of otosclerosis is still based on histopathological examination of the removed stapes footplate, systemic prospective studies based on comprehensive histopathological and molecular biological analysis are necessary to obtain further information on the background of the disease.
Topics: Adult; Aged; Bone Remodeling; Causality; Cross-Sectional Studies; Hearing Loss, Bilateral; Humans; Middle Aged; Otosclerosis; Risk Factors; Stapes
PubMed: 20532905
DOI: 10.1007/s00405-010-1292-1 -
Otolaryngologic Clinics of North America Jun 1973
Review
Topics: Humans; Otosclerosis; Radiography; Tomography, X-Ray
PubMed: 4220299
DOI: No ID Found -
Otology & Neurotology : Official... Dec 2009Otosclerosis is a major cause of acquired hearing loss in adult life affecting exclusively the human temporal bone. Until recently, the etiopathogenesis of otosclerosis... (Review)
Review
OBJECTIVES
Otosclerosis is a major cause of acquired hearing loss in adult life affecting exclusively the human temporal bone. Until recently, the etiopathogenesis of otosclerosis was still a matter of debate. Genetic research, however, has evolved enormously the last years and unveiled important clues regarding the cause of otosclerosis. The objective of this article is to review the genetics of otosclerosis with special attention for the links to the bone homeostasis of the otic capsule.
DATA SOURCES
A detailed literature study was performed focusing on the recent genetic findings in otosclerosis and the special bone turnover of the otic capsule. A PubMed search and own research data were used to bring the relevant information for this review together.
CONCLUSION
Unlike all other bones in the human skeleton, the otic capsule undergoes very little remodeling after development, possibly due to local inner ear factors. Otosclerosis is a process of pathologic increased bone turnover in the otic capsule, which in most cases leads to stapes fixation, resulting in a conductive hearing loss. Although environmental factors such as estrogens, fluoride, and viral infection have been implicated, it is clear that genetic factors play a significant role in the manifestation of otosclerosis. From a genetic viewpoint, otosclerosis is considered to be a complex disease with rare autosomal dominant forms caused by a single gene. Already, 7 monogenic loci have been published, but none of the genes involved have been identified. For the complex form of otosclerosis, caused by an interaction between genetic and environmental factors, the first susceptibility genes were identified by case-control association studies. All 3 replicated genes, TGFB1, BMP2, and BMP4, are a part of the transforming growth factor-beta1 pathway. Data from both genetic association studies and gene expression analysis of otosclerotic bone showed that the TGF-beta1 pathway is most likely an important factor in the pathogenesis of otosclerosis.
Topics: Chondrogenesis; Ear, Inner; Gene Expression; Gene Frequency; Humans; Otosclerosis
PubMed: 19546831
DOI: 10.1097/MAO.0b013e3181a86509 -
B-ENT 2009To establish the origin of tinnitus in patients with otosclerosis. (Review)
Review
OBJECTIVE
To establish the origin of tinnitus in patients with otosclerosis.
METHODOLOGY
We performed a literature review to identify the characteristics of tinnitus in the general population and among patients with otosclerosis.
RESULTS
Characteristics of tinnitus among patients with otosclerosis differ from those among the general population, suggesting mechanisms other than those implicated in presbycusis. Considerable improvements in the prevalence of tinnitus following corrective surgery demonstrate the limited contribution of diffuse labyrinth lesions. Middle ear and possibly secondary inner ear dysfunction produced by stapes blockage appear to be major etiopathogenic factors in the development of tinnitus in patients with otosclerosis.
CONCLUSION
The conductive hearing deprivation produced by otosclerosis is associated with limited and reversible modifications in the central auditory pathway that are linked to tinnitus.
Topics: Auditory Pathways; Humans; Otosclerosis; Prevalence; Risk Factors; Tinnitus
PubMed: 20163050
DOI: No ID Found -
Lancet (London, England) Nov 1977
Topics: Humans; Otosclerosis; Sodium Fluoride
PubMed: 72906
DOI: No ID Found -
Autoimmunity Reviews Dec 2009To review our current knowledge of the etiopathogenesis of otosclerotic bone remodeling including genetics, viral infection, autoimmunity and inflammation and to discuss... (Review)
Review
OBJECTIVES
To review our current knowledge of the etiopathogenesis of otosclerotic bone remodeling including genetics, viral infection, autoimmunity and inflammation and to discuss disease pathogenesis with relevance for pharmacotherapy.
SYSTEMATIC REVIEW METHODOLOGY
Relevant publications on the etiopathogenesis, molecular biology, genetics and histopathology of otosclerosis from 1984 to 2009 were analyzed.
RESULTS AND CONCLUSIONS
Otosclerosis is a bone remodeling disorder of the human otic capsule, however, the etiopathogenesis remains unclear. Genetic predisposition, disturbed bone metabolism, persistent measles virus infection, autoimmunity, hormonal and environmental factors also may play contributing roles in the pathogenesis of otosclerosis. Since, diagnosis of otosclerosis is still based on histopathological examination of the removed stapes footplate, systemic prospective studies based on comprehensive histopathological and molecular biological analysis are necessary to get further information about the background of disease.
Topics: Animals; Anti-Inflammatory Agents; Autoimmune Diseases; Collagen Type I; Collagen Type I, alpha 1 Chain; Comorbidity; Genetic Predisposition to Disease; Humans; Measles; Measles virus; Otosclerosis; Polymorphism, Genetic; Vitamin D
PubMed: 19318139
DOI: 10.1016/j.autrev.2009.03.009