Otosclerosis

Formation of spongy bone in the labyrinth capsule which can progress toward the STAPES (stapedial fixation) or anteriorly toward the COCHLEA leading to conductive, sensorineural, or mixed HEARING LOSS. Several genes are associated with familial otosclerosis with varied clinical signs.

Also Known As:

Networked: 1435 relevant articles (59 outcomes, 193 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hearing Loss (Hearing Impairment)
2.	Tinnitus
3.	Mixed Conductive-Sensorineural Hearing Loss
4.	Sensorineural Hearing Loss
5.	Otitis Media

Experts

1.	Karosi, Tamás: 30 articles (12/2019 - 07/2004)
2.	Sziklai, István: 26 articles (01/2016 - 07/2004)
3.	Grolman, Wilko: 22 articles (10/2018 - 01/2002)
4.	McKenna, Michael J: 19 articles (01/2022 - 02/2002)
5.	Van Camp, Guy: 15 articles (04/2022 - 04/2006)
6.	Wegner, Inge: 15 articles (12/2019 - 09/2013)
7.	Vincent, Robert: 15 articles (10/2018 - 12/2006)
8.	Schrauwen, Isabelle: 14 articles (04/2022 - 07/2007)
9.	Csomor, Péter: 13 articles (08/2014 - 09/2008)
10.	Sterkers, Olivier: 12 articles (11/2021 - 11/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Otosclerosis:

1.	Polytetrafluoroethylene (Teflon)FDA Link 01/01/1997 - "This study reports the evaluation of hearing results after implantation of a Teflon piston of a different diameter in cases of otosclerosis requiring stapedotomy. " 08/01/2006 - "It is suggested that small fenestra stapedotomy with Teflon piston artificial stapes is an ideal surgical treatment for otosclerosis." 12/01/1991 - "Three groups of patients were matched for age, sex, clinical type of otosclerosis, and type of 0.6-mm all-Teflon piston. " 10/01/1967 - "[Presentation of a film: "Teflon interposition in the surgery of otospongiosis"]." 05/01/1967 - "[Teflon interposition in the surgery of otospongiosis]."
2.	Diphosphonates (Bisphosphonates)IBA 02/01/2022 - "The efficacy of bisphosphonates for halting bone remodeling in otosclerosis remains unclear. " 06/01/1997 - "Prospective blinded efficacy studies of the bisphosphonates in the treatment of otosclerosis should be undertaken." 02/01/2022 - "Bisphosphonates have been used to decrease bone remolding in many diseases, including otosclerosis. " 01/01/2022 - "There is insufficient evidence to recommend the routine use of bisphosphonates in otosclerosis patients at present. " 01/01/2022 - "A Systematic Review of the Effectiveness of Bisphosphonates for Otosclerosis."
3.	TitaniumIBA 07/01/2013 - "The use of the titanium soft clip stapes piston gives good results in cases of stapedotomy for otosclerosis. " 07/01/2013 - "Case records of 20 patients of otosclerosis who had undergone stapedotomy using titanium soft clip stapes piston (Kurz, Germany) were retrospectively analysed. " 06/01/2008 - "An analysis of the air-bone gap closure obtained by a crimping and a non-crimping titanium stapes prosthesis in otosclerosis." 11/01/2003 - "The new low-weight, full-titanium stapes prosthesis with its slight rough surface and its good mechanical stability and biocompatibility can safely and successfully restore the function of the middle ear when implanted in patients with otosclerosis." 11/01/2003 - "To evaluate the early postoperative hearing results of a new titanium stapes prosthesis (K-Piston) implanted in patients with otosclerosis. "
4.	Sodium Fluoride (Ossin)FDA Link 06/01/2010 - "There has been only one double-blind, placebo-controlled trial of the use of sodium fluoride in otosclerosis patients, and this found a reduced incidence of deterioration in hearing after two years in the treatment group. " 01/01/1989 - "The effect of sodium fluoride treatment in patients with otospongiosis has been evaluated in a prospective clinical double-blind, placebo-controlled study of 95 patients. " 01/01/1989 - "A clinical double-blind, placebo-controlled study on sodium fluoride treatment in otospongiosis." 02/01/2023 - "Conservative Otosclerosis Treatment With Sodium Fluoride and Other Modern Formulations: A Systematic Review." 06/01/2010 - "There is low quality evidence suggesting that sodium fluoride may be of benefit to preserve hearing and reduce vestibular symptoms in patients with otosclerosis."
5.	Zoledronic Acid (Zometa)FDA Link 10/01/2017 - "Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. " 10/01/2012 - "Treatment with zoledronate or risedronate stabilized progressive SNHL related to otosclerosis in this small group of patients. " 10/01/2012 - "We hypothesized that newer generation bisphosphonates, such as risedronate and zoledronate, would be effective in slowing the progression of SNHL in patients with otosclerosis. " 07/01/2015 - "Nitrogen-containing bisphosphonates (e.g., zoledronate) are potent inhibitors of bone remodeling with proven efficacy in the treatment of metabolic bone diseases, including otosclerosis. " 03/01/2016 - "We have previously demonstrated that zoledronate, a nitrogen-containing bisphosphonate, can arrest the sensorineural hearing loss in cochlear otosclerosis. "
6.	Capsules (Microcapsules)IBA 09/01/2022 - "The aim of this study was to measure cone beam computed tomography (CBCT) derived bone density of 6 anatomic points around the otic capsule in preoperative assessment of patients with clinically suspected otosclerosis, and to compare these densities with control group. " 01/01/2022 - "This study aimed to describe the spatial distribution of osteocyte-depleted areas, so-called cellular voids, in the human otic capsule and compare it with that of otosclerosis. " 12/01/2018 - "This study assesses differences in otic capsule contour and thickness anterolateral to the anterior margin of the oval window in patients with otosclerosis compared with individuals with normal hearing. " 05/01/2011 - "To determine by histologic study of the developing otic capsule and temporal bones with otosclerosis the site, tissue of origin, and pathways of growth of the disease. " 10/01/2010 - "Recent studies have revealed that otosclerosis is a process of bone remodeling that is unique to the otic capsule only. "
7.	PlatinumIBA 01/01/2003 - "Evaluation of hearing results after implantation of a fluoroplastic-platinum piston (FP) and of a titanium piston (T) with a shaft diameter of respectively 0.6 mm and 0.4 mm, in cases of otosclerosis requiring stapedotomy. " 04/01/1999 - "Two hundred ears with otosclerosis have been operated upon using a 0.4-mm diameter Teflon platinum piston in 100 ears and a 0.6-mm diameter Teflon piston in the other 100 ears. " 09/01/1993 - "Otosclerosis is treated by a 'small-fenestra' stapedotomy with insertion of a teflon-platinum wire piston. " 02/01/1999 - "For many years good results in otosclerosis surgery have been achieved with a prosthesis made of platinum-wire and Teflon. " 08/01/2007 - "These data show that the nitinol prosthesis is equivalent to the platinum wire prosthesis in closing the ABG in patients with otosclerosis. "
8.	Transforming Growth Factor beta1 (TGF beta 1)IBA 09/01/2007 - "On the basis of very low P-values, replication in an independent population and a functional effect of the protective variant, we conclude that TGF-beta1 influences the susceptibility for otosclerosis, and that the I263 variant is protective against the disease." 12/01/2009 - "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." 09/01/2008 - "Otosclerosis and TGF-beta 1 gene in black South Africans." 09/01/2007 - "The coding polymorphism T263I in TGF-beta1 is associated with otosclerosis in two independent populations." 12/01/1995 - "The fact that the phenotype of the otosclerotic cells differed from that of the normal cells and could be modified by TGF beta 1 treatment, suggests that TGF beta 1 is implicated in the pathogenesis of otosclerosis."
9.	GoldIBA 09/01/1998 - "This study shows that the use of a pure gold piston ((K-piston) with a diameter of 0.4 mm gives good results in cases of stapedotomy for otosclerosis. " 09/01/1998 - "This study reports the evaluation of the hearing results after 62 implantations of a pure gold (99.9%) piston in cases of otosclerosis. " 09/01/1998 - "Experience with a new pure gold piston in stapedotomy for cases of otosclerosis." 01/01/2002 - "four hundred and seventy five patients (328 women, 147 men, average age: 45.2 years), who clinically and per-operatively had otosclerosis, underwent a stapedotomy using a pure gold piston prosthesis. " 01/01/2002 - "to determine the occurrence of the unusual side effect of a reparative granuloma after the implantation of a pure gold piston in cases of otosclerosis. "
10.	Etidronic Acid (HEDP)FDA LinkGeneric 06/01/1997 - "In this preliminary study etidronate appeared to be an effective treatment for the neurotologic symptoms of otosclerosis. " 06/01/1997 - "Etidronate for the the neurotologic symptoms of otosclerosis: preliminary study." 09/01/1993 - "A 2-year prospective double-blind study was performed to evaluate the role of etidronate disodium for the treatment of progressive hearing loss in patients with otosclerosis. " 09/01/1993 - "The effects of etidronate disodium on progressive hearing loss from otosclerosis." 06/01/1997 - "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. "

Therapies and Procedures

1.	Stapes Surgery (Stapedectomy) 03/01/2007 - "Stapedectomy is currently the treatment with which the best results are obtained among patients with otosclerosis, which is why it is the treatment of choice. " 02/01/1994 - "Stapedectomy is known to give good results for otosclerosis. " 09/01/2005 - "Stapes surgery is effective operation to cured otosclerosis, advanced otosclerosis or far-advanced otosclerosis had greater help to improved hearing." 02/01/2013 - "To study objective or subjective preoperative factors predicting improvement in listening capabilities and patient satisfaction after stapes surgery in otosclerosis. " 08/01/2021 - "These days, the gold standard procedure for otosclerosis treatment is stapes surgery. "
2.	Prostheses and Implants (Prosthesis) 01/01/2012 - "Developments in surgical technique and, more importantly, the use of increasingly sophisticated biocompatible prostheses have meant that good results can be achieved for otosclerosis sufferers in terms of restored hearing and very little postsurgical discomfort. " 01/01/2021 - "Our unique prosthesis yielded good outcomes for the treatment of otosclerosis even in the short term." 03/01/2016 - "The use of both the prosthesis gave good results in cases of otosclerosis. " 08/01/2022 - "To study spectrum of high resolution computed tomography (HRCT) imaging findings in otosclerosis, to predict approximate length of prosthesis required from pre-operative HRCT measurements and to correlate between oval window niche (OWN) height preoperatively and difficulty in introducing foot plate perforator during surgery. " 08/01/2022 - "Sensitivity of HRCT in detecting otosclerosis, correlation between pre-operative incudo-stapedial length (ISL) and intra operative prosthesis length, and correlation between OWN height and difficulty in introducing perforator were sought. "
3.	Cochlear Implantation 01/01/2023 - "The aim of the study was to measure the distance of the tympanic nerve to the oval window and round window niche in adult cadavers for evaluating its usability as an anatomical landmark during middle ear-related surgeries, including stapedotomy and cochleostomy, and for preventing its iatrogenic damage during surgical practices such as otosclerosis surgery and cochlear implantation. " 08/01/2022 - "Cochlear implantation outcomes in patients with otosclerosis: a single-centre study." 07/01/2022 - "An electronic literature search was performed using four main databases through February 2021 to identify original studies of cochlear implantation in patients with otosclerosis for inclusion in this systematic review. " 01/01/2022 - "This study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis. " 01/01/2017 - "An online survey of clinical professionals was constructed to characterize current standard of care for patients with advanced otosclerosis and to assess whether clinicians would be willing to refer patients into a trial to evaluate direct acoustic cochlear implantation. "
4.	Hearing Aids (Hearing Aid) 06/01/1999 - "In two patients with bilateral 'far-advanced otosclerosis', surgery was effective in enabling the patient to benefit from hearing-aids. " 01/01/2021 - "The objective of the study is to assess the long-term hearing results, addressing the bone conduction (BC) decay and the need for hearing aids in otosclerosis patients. " 01/01/2006 - "The aim of this study was to report a series of patients with far-advanced otosclerosis who were unable to benefit from hearing aids. " 02/24/2023 - "Treatment of Far-Advanced Otosclerosis: Stapedotomy Plus Hearing Aids to Maximize the Recovery of Auditory Function-A Retrospective Case Series." 06/01/2022 - "A validation study of the Swedish version of the Glasgow hearing aid benefit profile evaluated in otosclerosis subjects."
5.	Lasers (Laser) 04/01/2002 - "The high success rate and low incidence of serious complications support stapedotomy, without a laser but with resident involvement, as a highly effective treatment for otosclerosis." 12/01/2020 - "Conclusion:Laser-assisted stapedotomy is a safe and effective treatment of otosclerosis. " 12/01/2020 - "Objective:To assess the short-term efficacy of laser-assisted stapedotomy in patients with otosclerosis. " 10/01/2018 - "The retrospective review study was to investigate the role of endoscopic laser stapedotomy in treating patients with otosclerosis. " 01/01/2013 - "The objective of the present study was to compare the immediate result of laser-assisted piston stapedoplasty with the use of prosthetic stapes of different diameter in the patients with otosclerosis. "