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Otosclerosis is characterized by pathological remodeling of the bone of the otic capsule. Classically, foci of pathological bone leads to progressive fixation of the stapes, resulting in conductive hearing loss. Involvement can be uni- or bilateral. Otoscopy is normal. -Hypoacusis generally appears between the age of 30 to 50, regularly with tinnitus. Sometimes, the inner ear is also affected, with sensori-neural hearing loss or vestibular impairment (vertigo). Successful hearing rehabilitation can be achieved with hearing aids or stapes surgery (stapedotomy). Cochlear implantation is considered in cases of severe or profound sensorineural hearing loss. High resolution CT-scan or Cone Beam CT confirm the diagnosis and optimal planning of surgical treatment., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Objectives: To review and provide evidence-based recommendations for the diagnosis and treatment of otosclerosis., Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on otosclerosis were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions., Results: The topics were divided into 2 parts: 1) Diagnosis - audiologic and radiologic; 2) Treatment - hearing AIDS, pharmacological therapy, stapes surgery, and implantable devices - bone-anchored devices, active middle ear implants, and Cochlear Implants (CI)., Conclusions: The pathophysiology of otosclerosis has not yet been fully elucidated, but environmental factors and unidentified genes are likely to play a significant role in it. Women with otosclerosis are not at increased risk of worsening clinical condition due to the use of contraceptives or during pregnancy. Drug treatment has shown little benefit. If the patient does not want to undergo stapedotomy, the use of hearing aids is well indicated. Implantable systems should be indicated only in rare cases, and the CI should be indicated in cases of profound deafness., (Copyright © 2023 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier España S.L.U. All rights reserved.)

Importance: Surgery and hearing aids have similar outcomes in terms of hearing acuity but differ in terms of cost, aesthetics, and patient quality of life. The cost-effectiveness and budget impact of otosclerosis treatments have never been studied in Europe., Objectives: To compare the estimated mean costs per patient over 10 years of surgery vs hearing aids for the treatment of otosclerosis and to estimate the budget impact of an increase in the proportion of patients receiving surgical treatment., Design, Setting, and Participants: This economic evaluation analyzed French and European epidemiological data on the surgical management of symptomatic otosclerosis and compared them with data from the literature to build economic models. The analysis was conducted in January 2021., Exposures: Two care pathways were considered in the treatment of otosclerosis, either hearing aid or surgery., Main Outcomes and Measures: Costs were studied over 10 years using Markov models of the 2 care pathways (hearing aid vs surgery). The budget impact analysis was performed over 5 and 10 years, assuming a 1-percentage point yearly increase in the proportion of patients receiving surgical treatment., Results: Over 10 years, the estimated mean cost per patient was significantly lower in the surgery group compared with the hearing aid group (€3446.9 vs €6088.4; mean difference, -€2641.5; 95% CI -€4064.8 to -€1379.4 [US $3913.4 vs US $6912.4; mean difference, -US $2999.0; 95% CI, -US $4614.9 to -US $1566.1]). Increasing surgical treatment by 1 percentage point per year for 10 years would lead to overall savings of €1 762 304 (US $2 000 798) in France, with an increase of €1 322 920 (US $1 501 952) at 10 years for the public health insurance system and a decrease of €3 085 224 (US $3 502 750) at 10 years for patients and private health insurers. Sensitivity analyses showed that these results were robust., Conclusions and Relevance: These results suggest that in France, treating otosclerosis surgically is slightly less expensive over 10 years than using hearing aids, when considering all payers. The proposed models developed in this study could be adjusted to perform the same analysis in other countries.

Chronic ear disease is a major cause of acquired hearing loss in the developing world. It is prevalent on every continent, but occurs more commonly in poorer nations owing to a lack of preventative measures. This article outlines the particular challenges in treating this disease in the developing world, including a discussion of surgical management and special situations. Otosclerosis is another surgically treatable cause of hearing loss that is found throughout the developing world. Surgeons working in these environments should be prepared to deal with advanced otosclerotic disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Controversies have been associated with the etiology, diagnosis, evaluation, and management of otosclerosis since Valsalva first described stapes fixation as a cause of hearing loss. Although the exact mechanism of the bone remodeling associated with otosclerosis remains uncertain, stapedotomy has been accepted as the surgical treatment of most patients with stapedial otosclerosis. There remains a disparity of opinion, however, regarding the role of preoperative imaging, surgical technique, implant selection, and medical therapy for cochlear otosclerosis. In addition, opinions vary regarding the optimal postoperative care of patients undergoing stapedotomy and a patient's ability to participate in activities that may result in barotrauma., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Lasers were introduced as an atraumatic modality for accomplishing several of the crucial steps in otosclerosis surgery. Advances in laser technology have spurred coevolution of refinements in the technique of the operation. Several varieties of laser systems are available to suit individual preference and to augment a surgeon's armamentarium; however, a clear advantage in terms of surgical outcome or patient safety remains to be demonstrated., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Diagnosis and treatment of advanced otosclerosis can be controversial. In 1961, House and Sheehy defined advanced otosclerosis as hearing loss in air conduction threshold by 85 dB with nonmeasurable bone conduction. Recently, the definition of advanced otosclerosis is mostly based on the decrease of speech recognition. There are some treatment modalities: stapes surgery and hearing aids, cochlear implantation, or direct acoustic cochlear implant. The authors propose a new algorithm for treatment. If the patient is treated with cochlear implantation, the surgeon should be cautious for facial nerve stimulation after surgery because it is the most prevalent complication., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To identify the audiological improvement postoperatively and improve the understand of otosclerosis for a better performance of personalized surgical treatment. Method: We retrospectively reviewed a series of 200 cases after surgery for ototsclerosis.The clinical characteristics,pre-and postoperative audiometric results,signs of Carhart notch, Gelle test and the characteristics of high-resolution computed tomography of temporal bone were analyzed retrospectively. Result: 73% of patients had tinnitus, while 4% had sense of ear fullness,80.79% had Carhart notch,92.09% had negative result in Gelle test and 45.66% had positive signs on computed tomography. 68% of the patients revealed a type A tympanogram with only 22% type As.Fifty-six cases with laser stapedotomy achieved a the air bone gap at 250 Hz,500 Hz,1 kHz,2 kHz and 4 kHz of 25.54,16.25,13.75,6.34,15.96 dB,respectively. The bone conduction thresholds at 250 Hz,500 Hz,1 kHz,2 kHz improved 2.05,1.51,3.75 and 3.93 dB,respectively. At 4 kHz, bone conduction threshold increased by 1.34 dB.The improvement of bone conduction threshold at 250 Hz,1 kHz,2 kHz was significantly but for the revisions at 500 Hz and 4 kHz. Conclusion: The diagnosis of otosclerosis should be based on the combination of medical history, pure tone audiometry, tympanometry, Carhart notch, Gelle test and high resolution computed tomography of temporal bone.Surgical technique of stapedotomy with Piston artificial auditory ossicle implantation could improve not only the air conduction threshold, but also the bone conduction threshold at 250 Hz,1 kHz,2 kHz., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Hypothesis: Assess the clinical acceptability of direct acoustic cochlear implantation for patients with advanced otosclerosis and the support for conducting a controlled trial of its effectiveness in the United Kingdom., Background: Emerging evidence supports the efficacy of direct acoustic cochlear implantation in patients with advanced otosclerosis whose needs cannot be managed using the combination of stapes surgery and hearing aids. A controlled trial would provide evidence for its effectiveness and cost-effectiveness to healthcare commissioners., Methods: 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. A consensus process was conducted to define inclusion criteria for the future trial., Results: No survey respondent considered direct acoustic cochlear implantation to be inappropriate with a majority indicating that they would refer patients into a future trial. The consensus was that there is a lack of available treatment options for those patients with bone conduction thresholds worse than 55 dB HL and who did not meet current criteria for cochlear implantation., Conclusion: The present study confirms that a controlled trial to evaluate the effectiveness of direct acoustic cochlear implantation would have the support of clinicians in the United Kingdom. A feasibility study would be required to determine whether patients who meet the inclusion criteria could be recruited in a timely manner and in sufficient numbers to conduct a formal evaluation of effectiveness.

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.

Objective: Identify and compare phenotypic properties of osteoblasts from patients with otosclerosis (OSO), normal bones (HOB), and normal stapes (NSO) to determine a possible cause for OSO hypermineralization and assess any effects of the bisphosphonate, alendronate., Study Design: OSO (n = 11), NSO (n = 4), and HOB (n = 13) cultures were assayed for proliferation, adhesion, mineralization, and gene expression with and without 10(-10)M-10(-8)M alendronate., Setting: Academic hospital., Methods: Cultures were matched for age, sex, and passage number. Cell attachment and proliferation + alendronate were determined by Coulter counting cells and assaying tritiated thymidine uptake, respectively. At 7, 14, and 21 days of culture + alendronate, calcium content and gene expression by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) were determined., Results: OSO had significantly more cells adhere but less proliferation than NSO or HOB. Calcification was significantly increased in OSO compared to HOB and NSO. NSO and HOB had similar cell adhesion and proliferation rates. A dose-dependent effect of alendronate on OSO adhesion, proliferation, and mineralization was found, resulting in levels equal to NSO and HOB. All cultures expressed osteoblast-specific genes such as RUNX2, alkaline phosphatase, type I collagen, and osteocalcin. However, osteopontin was dramatically reduced, 9.4-fold at 14 days, in OSO compared to NSO. Receptor activator of nuclear factor κB ligand/osteoprotegerin (RANKL/OPG), important in bone resorption, was elevated in OSO with decreased levels of OPG levels. Alendronate had little effect on gene expression in HOB but in OSO increased osteopontin levels and decreased RANKL/OPG., Conclusions: OSO cultures displayed properties of hypermineralization due to decreased osteopontin (OPN) and also had increased RANKL/OPG, which were normalized by alendronate., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.)

Objective: To examine the evidence for the role of radiologic imaging in the diagnosis and management of otosclerosis., Data Sources: A review of contemporary (1990 to present) English medical literature via MedLine using the terms imaging, otosclerosis, otospongiosis, stapes surgery, computed tomography, magnetic resonance, CT, and MRI was performed., Study Selection: Abstracts were reviewed independently by 2 authors and relevant articles were then evaluated. Exclusion criteria included editorials, non-English language, comments, and letters., Data Extraction: Level of evidence was assigned in accordance with the Oxford Centre for Evidence-based Medicine guidance (Levels I-V)., Results: Thirty-seven articles met the inclusion criteria, of which, 11 were of Level III, 22 of Level IV, and 4 of level V evidence. High-resolution computed tomography (CT) of the temporal bones is the imaging technique of choice in the diagnosis of otosclerosis with newer multidetector scanners demonstrating a sensitivity and specificity in excess of 90%. There is Level III evidence that CT densitometry and extent of disease on CT correlates with hearing thresholds. Extensive and multifocal disease on CT has a poorer prognosis (Level III/IV). The potential use of CT in staging classifications, surgical planning, predicting surgical outcomes and risk of complications has also been described and evaluated., Conclusion: This systematic review indicates that imaging has a useful role in both the diagnosis and management of otosclerosis, supported principally by Level III/IV evidence.

Hypothesis: A high-fidelity, inexpensive middle ear simulator could be created to enhance surgical training that would be rated as having high face validity by experts., Background: With rapid prototyping using additive manufacturing technology (AMT), one can create high-resolution 3-dimensional replicas of the middle ear at low cost and high fidelity. Such a simulator could be of great benefit for surgical training, particularly in light of new resident training guidelines., Methods: AMT was used to create surgical middle ear simulator (SMS) with 2 different materials simulating bone and soft tissue. The simulator is composed of an outer box with dimensions of an average adult external auditory canal without scutum and an inner cartridge based on an otosclerosis model. The simulator was then rated by otology experts in terms of face validity and fidelity as well as their opinion on the usefulness of such a device., Results: Eighteen otologists from 6 tertiary academic centers rated the simulator; 83.3% agreed or highly agreed that SMS has accurate dimensions and 66.6% that it has accurate tactile feedback. When asked if performance of stapedotomy with the SMS improves with practice, 46% agreed. As to whether practicing stapedotomy with the SMS translates to improvement with live surgery, 78% agreed with this statement. Experts' average rating of the components of SMS (of possible 5) was as follows: middle ear dimensions, 3.9; malleus, 3.7; incus, 3.6; stapes, 3.6; chorda tympani, 3.7; tensor tympani, 4.1; stapedius, 3.8; facial nerve, 3.7; and promontory, 3.5. Overall, 83% found SMS to be at least "very useful" in training of novices, particularly for junior and senior residents., Conclusion: Most experts found the SMS to be accurate, but there was a large discrepancy in rating of individual components. Most found it to be very useful for training of novice surgeons. With these results, we are encouraged to proceed with further refinements that will strengthen the SMS as a training tool for otologic surgery.

Unlabelled: Surgery is the most effective method of improving hearing in patients with otosclerosis. The level of improvement depends on the stage of the disease and the chosen surgical method., The Aim of This Study: is to present hearing results in patients treated surgically by means of different types of prostheses and methods of vestibule sealing., Material and Methods: 230 cases (160 men, 70 women) between the age of 21 and 64 (median 36 years) treated for the first time in the Department of Otolaryngology of Medical University in Gdańsk underwent epidemiologic and clinical analysis. The researches took into account the level of hearing improvement based on the reduced air-bone reserve for frequencies 0.5, 1.0, 2.0 i 4.0 kHz in groups with where prostheses type 1 and 2 were used., Results: 230 stapedotomies were performed. In 110 patients prosthesis type 1 was used (PTFE) and in 120 type 2 (piston PTFE with platinum tape). Each vestibule was sealed with homogenic fat tissue or fibrin sponge. After 6 weeks from the operation the air-bone reserve was reduced by 5 to 30 dB, the reserve existed after one year from the operation in some patients. 35% of the patients presented with vertigo after the operation and 5% with tinnitus., Conclusions: Stapedotomy is a method of hearing improvement in patients with otosclerosis. Best results are achieved when prosthesis type 2 is used and homogenic fat tissue serves to seal the vestibule. Broad opening of the vestibule may be the cause for vertigo and lack of hearing improvement occurrence., (Copyright © 2012 Polish Otolaryngology Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.)

The author suggests an original hypothesis of otosclerosis based on the analyses of the literature publications for many years and his personal clinical observations. The normal labyrinth capsule is considered to be bradytrophic, i.e. inert and showing an extremely low level of metabolic processes. The disturbance of bradytrophicity under the action of individual factors and/or especially their combination make it involved in the maintenance of calcium homeostasis in the body. The validity of this conjecture is confirmed by the results of histological investigations, viz. the appearance of diquide or xplasma-like, bone in the labyrinth of the patients suffering otosclerosis. Such bone resorption is known to occur in other parts of the bony skeletontoo and should be regarded as a normal physiological process contributing to the replenishment of blood calcium deficiency.The subsequent reorganization (remodeling) of any part of the bony skeleton is physiologically neutral. In the labyrinth capsule,with its small size and delicate structure, such reorganization induces the otosclerotic process responsible for dysfunction of the membranaceous labyrinth. The surgical treatment of the patients presenting with otosclerosis should be supplemented by conservative treatment intended to slow down the otosclerotic reorganization and to restore bradytrophicity of the labyrinth capsule.

This paper reports a rare clinical observation of congenital malformation of the internal and middle ear diagnosed with the help of functional multispiral computed tomography (fMSCT). The patient presenting with conductive hearing loss was suspected to have otosclerosis. However, the use of fMSCT made it possible to arrive at a more precise diagnosis, that is congenital malformation of the internal and middle ear that clinically manifested itself as the conductive loss of hearing. It was decided to refrain from the surgical intervention: first, because its efficacy in this case was considered to be doubtful and second, it was fraught with the high risk of development of postoperative complications.

The objective of the present study was to estimate the state of the stirrup prosthesis based on the results of functional multispiral computed tomography (fMSCT). A total of 30 patients suffering otosclerosis and treated by stapedoplasty were examined using the modified fMSCT technique developed by the authors. The data obtained were compared with the results of the audiologic study. It was shown that modified fMSCT makes it possible to objectively and visually estimate the state of the stirrup prosthesis, elucidate the causes of poor outcome of the surgical treatment and identify the indications for the second operation. The study has demonstrated that a more pronounced decrease of the air-bone gap (ABG) is associated with a rise in the amplitude of prosthesis movements whereas the degree of reduction of bone conduction does not significantly correlate with the depth to which the prosthesis reaches into the vestibulim.

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.

Objective: The Rion implantable hearing aid (IHA) Ehime (E)-type was developed for ears with middle ear diseases. This study focused on the current status of the patients, device problems, postoperative difficulties, and preventive measures against them., Study Design: Case series with chart review., Setting: Tertiary referral hospital., Subjects and Methods: Subjects were 30 patients who were implanted with the IHA E-type between 1984 and 1997 and followed up for more than 10 years. Current status of IHA implantees, incidents of device problems, and postoperative troubles and hearing outcomes were reviewed., Results: Eleven patients (36.7%) still use the original device. The average period of use was 16.6 +/- 3.3 years (21 years at most). The incidence of problems was lower with the second version of the device compared to the first version. Frequencies of the troubles were related to the types of original ear diseases: seven of 17 cases with chronic otitis media (41.2%), two of seven cases with cholesteatoma (28.6%), and two of six cases with tympanosclerosis (33.3%). No cholesteatoma occurred after surgical procedures (i.e., external ear canal closure and tympanic membrane lateralized) (P = 0.06). The device was exposed through a retroauricular skin fistula where the internal coil had been implanted. Significantly fewer infections were observed when the two-stage operation was used (P < 0.01)., Conclusion: For long-term success in implantation of the IHA, careful control of middle ear inflammation and measures against eustachian tube dysfunction are required in addition to technological advancements., (Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.)

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.

Objective: The aim of this study was to investigate the clinical aspects of balance disorders and to assess the audiovestibular functions of patients with otosclerosis., Materials and Methods: Among 13,800 patients examined for balance disorders between 2002 and 2006, 98 (0.7%) presented with otosclerosis. Seventy-three patients (28 treated nonoperatively, 27 operated on unilaterally, and 18 operated on bilaterally for otosclerosis) with an audiovestibular assessment were included in this retrospective study., Results: Complaints were dizziness in 42 patients (56%), benign paroxysmal positional vertigo in 37 patients (51%), and rotatory vertigo in 21 patients (29%), with no difference between patients operated on and those treated nonoperatively. In patients treated nonoperatively, despite the absence of correlation for hearing loss between the right and left ears, a strong correlation was observed for the bithermic caloric response (BCR; Y = 2.54 + 0.73X, Y = left, X = right, R2 = 0.71, n = 26, p < 0.0001, analysis of variance). In contrast, the correlation between the ipsilateral and contralateral ears in BCR was weak in patients operated on 1 side (Y = 15.1 + 0.66X, Y = contralateral, X = ipsilateral, n = 27, R2 = 0.20, p < 0.05), and this was associated to a caloric paresis suggesting a surgery-induced deficit. There was no correlation between hearing thresholds and the BCR., Conclusion: The proportion of patients with otosclerosis among those with balance disorders was similar to the estimated prevalence of otosclerosis in the general population. Despite the asymmetric hearing loss, BCR was symmetrical in patients treated nonoperatively. In contrast, the loss of vestibular function symmetry in patients suggested a surgery-induced vestibular trauma.

The aim of this study was to audit the introduction of the use of the CO2 laser into our department and to compare hearing outcomes and complication rates in patients who underwent either laser or mechanical stapedectomy. We found that the use of laser is at least as safe as the traditional approach with regards the rate of post-operative complications. One patient in the laser group suffered prolonged post-operative tinnitus, whilst one patient in the traditional group suffered prolonged post-operative vertigo. There was no evidence, however, of improved Air-Bone Gap closure compared to the traditional approach (Pre- and Post-Op Air Bone Gaps of 34 +/- 3 and 9 +/- 2 for laser stapedectomy versus 35 +/- 4 and 13 +/- 2 for traditional stapedectomy (mean +/- SEM)). In summary, therefore, CO2 laser surgery for otosclerosis is a safe surgical procedure resulting in similar hearing outcomes to that obtained following mechanical stapes surgery.

Presented here is a first-person account of the evolution of the practice of surgical neurootology to that of medical neurootology shaped mainly by results of treatment directed at underlying otosclerosis-like lesions of the otic capsule and metabolic factors. With new technologies and rapidly evolving concepts, the changing treatment algorithms did not remain constant to provide the usual evidence-based outcome analyses. However, the majority of the patients presenting with neurootological symptoms had undergone previous medical or surgical treatment before undergoing the medical management herein described. The underlying ongoing basic science findings over this period were linked to the clinical observations. On the basis of the more effective results of treating neurootological disorders, recommendations are made for future areas of investigation-mostly basic science-into developing an investigative foundation for future effective management of patients with a variety of neurootological disorders.

Objective: To study the hearing results and complications in otosclerotic patients who underwent Carbon dioxide (CO2) laser stapedotomy using Teflon piston prosthesis and autologous blood seal., Material and Method: The charts of all primary CO2 laser stapedotomy procedures performed at Bangkok Metropolitan Administration Medical College and Vajira Hospital between 1997 and 2005 were reviewed. Patients who used Teflon piston prosthesis and autologous blood seal and had follow-up data of more than one year were selected for inclusion in the study. Any patients who underwent CO2 laser stapedotomy for diseases other than otosclerosis or those with inadequate postoperative data were excluded. The hearing results from preoperative and postoperative (most recent follow-up) periods were analyzed using closure of air-bone gaps and postoperative sensorineural hearing loss (SNHL) at pure-tone average (PTA) and different frequencies. Major complications were recorded., Results: The present review yielded 73 primary CO2 laser stapedotomy procedures performed between 1997 and 2005, and 36 patients who underwent 40 CO2 laser stapedotomies met the criteria for analysis. The hearing outcomes were followed for an average of 22 months. The average preoperative and postoperative PTA air-bone gaps were 32.7 and 7.5 dB, respectively. The rate of patients who had postoperative PTA air-bone gaps within 10 dB was 77.5% and within 15 dB was 95%. There was a significant closure of air-bone gaps at PTA and at all frequencies (the frequencies from 0.5 to 4 kHz) and the closures of air-bone gaps at 0.5, 1, and 2 kHz were statistically better than at 4 kHz. The overall rate of postoperative SNHL was 7.5% at high pure-tone bone conduction average. There were no major postoperative complications., Conclusion: CO2 laser stapedotomy with Teflon piston prosthesis and autologous blood seal is a safe and effective treatment for otosclerosis. The procedure provides acceptable hearing results and gives the more air-bone gap closure at the low and mid frequency ranges without significant postoperative SNHL and other serious complications.

As part of the GENDEAF consortium, a European multi-centre otosclerotic database is under construction to collect the clinical data of as many otosclerotic patients as possible. Otosclerosis represents a heterogeneous group of heritable diseases in which different genes may be involved regulating the bone homeostasis of the otic capsule. The purpose of the GENDEAF otosclerosis database is to explore the otosclerotic phenotype more in depth. Subtle phenotypic differences otherwise not visible, may become statistically relevant in a large number of patients. Their identification can lead towards the discovery of new genes involved in the pathway of abnormal bone metabolism in the human labyrinth. As soon as one of the otosclerotic genes is identified, it would allow us to identify genotype-phenotype correlations. From other deafness genes, it is know that different mutations in the same gene may cause similar phenotypes of varying severity. Also the variability in treatment outcomes after surgery or fluoride therapy may result not only from differences in practice or surgical skill among physicians, but also on the nature of the underlying disorder. Screening large numbers of patients would make it possible to undertake clinical trials comparing different treatments. Identifying a genetic susceptibility would allow us to dissect out possible environmental factors that prevent the expression of clinical otosclerosis in those that carry the mutated gene and yet retain normal hearing.

Otosdcerosis is an autosomal dominant condition affecting the temporal bone. It presents predominantly with deafness in a young population. This review looks at the aetiological theories, present treatment strategies and surgical outcomes of this condition.

Otosclerosis is a primary osteodystrophy which affects a localized area within the human temporal bone. Hearing loss is the most functional deficit caused by otosclerosis. However, tinnitus and vestibular disorders are frequently reported by otosclerotic patients, especially in those patients with inner ear involvement. The best therapy in achieving a significant improvement is surgery (stapedoplasty). In most patients if the operation is not carried out for pure middle ear type, the hearing impairment can progress to high-degree hearing loss. Recently, guidelines for the treatment of otosclerosis reported the 20 dbHL threshold as the minimum air-bone gap in performing stapedoplasty. We believe that stapedoplasty is an effective procedure for selected patients affected by otosclerosis with an air-bone gap which is smaller than 20 dbHL. An operation in the earlier phases of the disease can arrest the progression of otosclerosis, preserve inner ear structures and provide a complete auditory recovery, with increased satisfaction of the patient., (Copyright 2003 Elsevier Science Ltd.)

Because of varying origin different therapy of tinnitus methods are applied (surgical, pharmacological, psychotherapy). Treatment of objective tinnitus of vascular origin is usually surgical. It is similar as in the case of tinnitus of mechanical origin. The procedure of operating. Treatment of subjective tinnitus is much more complicated because of difficulties with determining of its occurence. We can separate morbus Ménière, otosclerosis, which require precise treatment. Apart from these disease treatment of subjective tinnitus is multidirectional. It may include pharmacological treatment, psychotherapy, surgical treatment and other methods.

Objective: Otosclerosis is a disease of hereditary liability. It might be related to an autosomal dominant inheritance. The genetic penetrance is determined by multifactorial influences. This paper was designed to study the reasonable strategies for prevention and treatment of this disease., Methods: A retrospective review derived from data of the management and follow-up of 14 cases in 6 families. According to the special pathological features of active phase of the otospongiosis, the suitable strategies for prevention and treatment of this disease were suggested., Results: Diagnosis of 14 cases were confirmed by audiological, operative and/or pathological examinations. Eleven of fourteen cases underwent stapedectomy, in which 10 cases had the surgery on the ear with severer hearing loss, and one case on both sides. In all of the surgical cases, hearing levels improved significantly. Three cases have not yet received the stapedectomy, in which two cases scheduled for the surgery are currently treated by chondroitin sulfate and one case refused the surgical treatment due to financial shortage., Conclusion: Examination of the ear function at regular intervals (6-12 months) is recommended for the adults among the family members with high-incidence of otosclerosis. Audiological and radiological examination play an important role in early diagnosis and treatment. Attention should be paid to the medicine for management and prevention of this disease. Stapedectomy is an optimal choice for clinical otosclerosis not only due to its capacity to improve the hearing level but also to prevent the advance of hearing loss. Hearing aid is the suitable choice if stapedectomy is contraindicated.

Otosclerosis is a multifactorial disease. A number of theories on the pathogenesis of this disease have been established in the last decades. It is important to review recent data on the pathogenesis of otosclerosis as it is a severe inner ear disease leading to deafness in the majority of cases. Surgical therapy is not always successful or feasible. In this review, authors describe the most relevant genetic, infective, immunological, inflammatory factors, as well as the impaired bone metabolism underlying the pathogenesis of otosclerosis. It is likely that genetic predisposition associated with morbilli infection may lead to bone resorption in the stapes and cochlea followed by spongiosis, fibrosis and sclerosis. It has been suggested that immunological mechanisms play a central role in the development of the disease. Some authors consider otosclerosis as autoimmune disorder based on the presence of several autoantibodies. Apart from classical diagnostic methods, such as audiometry and X-ray, novel radiological techniques including CT, MRI or radionuclide scan are helpful in the localization of otosclerosis. As surgery is sometimes contraindicated or unsuccessful, drug therapy including the use of anti-osteoporotic on non-steroidal antiinflammatory drugs may be administered, especially in the early phase of the disease.

Fluorine content in bone samples taken from the middle ears of otosclerotic patients was determined. Otosclerotic stapes footplate was found to have a significantly higher content of fluorine than skeletal bone from the meatus. Fluorine contents in footplate and meatal wall samples of otosclerotic patients drinking fluoridated water were slightly higher than those of patients drinking low-fluoride water. In the clinical part of the study, hearing levels of 280 patients with otosclerosis living in an area with low-fluoride water were assessed. In 344 operated ears, the preoperative and long-term postoperative air conduction and bone conduction thresholds of patients drinking fluoridated water did not differ significantly from those of patients drinking low-fluoride water. After a mean follow-up period of 9.6 years, air conduction thresholds of non-operated ears in patients drinking fluoride-poor water were found to be significantly worse than those of patients drinking fluoridated tap water, likewise there were significant differences in bone conduction thresholds at 2 and 4 kHz. Thus, fluoridation of drinking water has a beneficial effect on non-operated otosclerotic ears but has no significant effect on hearing levels of operated ears.

When a patient presents with bilateral progressive hearing loss and has a family history of hearing loss, otosclerosis should be placed high on the list of possible causes. Primary care physicians should determined whether the hearing loss is conductive or sensorineural with otologic examination and tuning-fork evaluation. Unless there is an obvious reversible cause for the progressive hearing loss, the patient should be referred to an otolaryngologist for more intensive otologic and audiologic examination. The patient's principal concern is the ability to hear and comprehend the spoken word in normal circumstances. Fortunately, most patients can be rehabilitated with surgery or a hearing aid. Fluoride treatment may have a significant role in preventing further hearing loss from otosclerosis.

For many diseases the etiology of disordered function is unknown and therapy is either symptomatic or empirical. The empirical approach to therapy is acceptable to many physicians, particularly if a treatment can be based on some attractive, although unproven, concept of pathogenesis. A hypothetical explanation of disordered function can become widely popular in spite of a serious lack of scientific support. When evidence emerges that refutes the logic of a concept of pathogenesis, then that concept becomes a myth. Human temporal bone studies have identified several myths in neurotology that deserve our attention. Among these myths are the following: (1) cochlear otosclerosis is a common cause for sensorineural hearing loss, (2) idiopathic sudden sensorineural hearing loss is a vascular disorder that should be treated as a medical emergency, (3) the conductive component of hearing loss in Paget's disease is caused by ossicular fixation and therefore should be surgically correctable, (4) vascular loops should be considered as a possible cause for otherwise unexplained otologic symptoms, (5) surgical manipulations on the endolymphatic sac are scientifically sound therapies for selected cases of Meniere's disease, and (6) perilymph fistulas are a common cause for audiovestibular symptoms that are not related to a stressful incident.

The spiral ligament of the stria vascularis is nearly always damaged by otosclerotic foci when the patient is nearly totally deaf because of otosclerosis. Vascular drugs are to be prescribed in such a case, besides the Sodium Fluoride treatment. The Sodium Fluoride is, according to us, the best treatment to arrest the progression of the otosclerotic disease. Sodium fluoride will destroy the proteolytic enzymes damaging the inner ear membranes and the hair of the Corti hair cells.

Otosclerosis classically describes an osteodystrophic change in the bony labyrinth and stapes footplate, of autosomal dominant inheritance, reported rare under the age of 5, extremely "rare" in the Oriental and Black race, "non-existent" in the American Indian, and with a clinical incidence of 5 per 1000 Caucasians. The differential diagnosis of a non-effusion conductive hearing loss in a child should include otosclerosis, congenital malleus or footplate fixation, tympanosclerotic fixation, congenital cholesteatoma, lysis of the incus long process, Paget's disease, osteogenesis imperfecta, and fibromuscular hyperplasia of the renal artery. Presented is a case report of a 14-year-old black male with bilateral clinical otosclerosis and a persistent stapedial artery. Preoperative multiple-frequency tympanometry and Zwislocki acoustic reactance and resistance analysis demonstrated absence of the "W" resonance pattern on high-frequency tympanometry and the classic friction and stiffness patterns of otosclerotic fixation. Repeat multiple-frequency tympanometry testing post-stapedectomy demonstrated prosthesis articulation. Prosthesis position can be monitored postoperatively by these acoustic impedance studies.

Studies of the type presented here have rarely been undertaken and should be useful in surgery and pathology of the inner and middle ear. Samples of perilymph were collected during stapedectomy in patients with otosclerosis. In view of the very small volume of perilymph obtainable from each patient's vestibule (2 to 4 microliter), the only assay method that could be used to measure drug levels was thin layer chromatography on silica gel. Despite pooling of the perilymphs of 5 patients, trimethoprim (TMP) levels could not be measured but the authors were able to demonstrate that sulfamethoxazole (SMZ) does penetrate into the perilymph. Since the TMP-SMZ combination (co-trimoxazole) is active against the pathogens usually encountered in middle ear fluids, it is concluded that the drug could be of benefit in the treatment of middle and inner ear infections or after surgical operations on this area.

In summary, the available published information on otospongiosis and metabolic factors in the production of endolymphatic hydrops viz. Meniere's disease was reviewed. By addressing these factors in a therapeutic approach, the effective management of this disorder reveals results as good as the reported surgical results. In addition, it was possible to salvage patients who had previously undergone successful surgery only to deteriorate several years later. At this time, the role of surgery on the endolymphatic sac is reserved for those patients who are failures in this form of management.