Brazilian Society of Otology task force - Otosclerosis: evaluation and treatment.

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.

Estimated Costs Associated With Management of Otosclerosis With Hearing Aids vs Surgery in Europe.

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.

Management of Chronic Suppurative Otitis Media and Otosclerosis in Developing 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.

Use of Lasers in Otosclerosis Surgery.

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.

Advanced Otosclerosis: Stapes Surgery or Cochlear Implantation?

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.

Controversies in the Evaluation and Management of Otosclerosis.

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.

An Assessment of the Clinical Acceptability of Direct Acoustic Cochlear Implantation for Adults With Advanced Otosclerosis in the United Kingdom.

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: An update on diagnosis and treatment.

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.

[A retrospective analysis of 200-cases of otosclerosis].

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.

Evaluación del cambio en el umbral de la vía ósea en pacientes operados de estapedectomía / Study of the improvement in bone conduction threshold after stapedectomy

Introducción y objetivos: La alteración del umbral auditivo de la vía ósea no solo corresponde a enfermedad localizada en el oído interno y nervio coclear, sino que puede ser secundaria a enfermedad presente en el oído medio, como ocurre en la otosclerosis. El objetivo de este estudio es analizar el resultado audiológico posquirúrgico y evaluar el efecto de la estapedectomía en el umbral auditivo de la vía ósea en pacientes con otosclerosis. Material y métodos: Estudio retrospectivo de 95 pacientes (116 oídos) con hipoacusia de transmisión y mixta, diagnosticados de otosclerosis e intervenidos de forma consecutiva con la misma técnica de estapedectomía con platinectomía total. Se realizó audiometría tonal de las frecuencias 500, 1.000, 2.000 y 4.000Hz para la vía aérea y ósea en todos los casos de forma pre- y posquirúrgica (al mes y al año del procedimiento). Resultados: Se obtuvo un cierre del umbral diferencial de audición, con un valor residual<10dB en el 92,2% de los pacientes y<5dB en el 79,3%. La ganancia media obtenida en la vía aérea fue de 25dB. Los pacientes con afectación preoperatoria de la vía ósea mostraron una mejoría significativa en las frecuencias 1.000 (6dB) y 2.000 (12dB), con desaparición del escotoma de Carhart. Estos resultados se mantuvieron sin cambios al año de seguimiento. Conclusiones: Comprobamos una mejoría significativa de la vía ósea en las frecuencias 1.000 y 2.000Hz, con desaparición del escotoma de Carhart tras estapedectomía en pacientes diagnosticados de otosclerosis que presentan hipoacusia mixta (AU)

Introduction and objectives: Bone conduction threshold depression is not always a result of inner ear and cochlear nerve pathology. In fact, middle ear pathologies may be responsible for such threshold depression, as occurs in otosclerosis. The aims of this study were to evaluate the improvement of bone conduction threshold in patients with otosclerosis that underwent stapedectomy and to study the postoperative audiological results. Material and methods: This was a retrospective study on 95 patients (116 ears) diagnosed with otosclerosis having conductive or mixed hearing loss that received surgery (stapedectomy and complete removal of the footplate) consecutively. Audiometry was performed on all patients pre- and postoperatively (one month and one year after surgery). Bone and air conduction thresholds were measured at 4 frequencies (500, 1000, 2000 and 4000Hz). Results: The air-bone gap was closed, with a residual air-bone gap below 10dB in 92.2% of the patients and below 5dB in 79.3% of the cases. The air conduction threshold improved an average of 25dB. The patients that had an affected bone conduction threshold preoperatively improved bone conduction postoperatively at the frequencies of 1000 and 2000Hz (6 and 12dB, respectively). Consequently, the Carhart notch disappeared on the audiogram. These results were maintained at one year of follow up. Conclusions: We found a significant improvement in the bone conduction threshold at the frequencies of 1000 and 2000Hz and a disappearance of the Carhart notch in the audiogram after stapedectomy and total footplate removal in patients diagnosed with otosclerosis having mixed hearing loss (AU)

Differences in otosclerotic and normal human stapedial osteoblast properties are normalized by alendronate in vitro.

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.

The role of imaging in the diagnosis and management of otosclerosis.

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.

[Treatment results of otosclerosis regarding different types of prosthesis]. / Wyniki leczenia otosklerozy z uwzglednieniem róznych typów protez.

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 Gdansk 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.

[Otosclerosis- <> or the loss of the natural variant of inertness (<> of the labyrinth capsule)].

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.

High-fidelity, inexpensive surgical middle ear simulator.

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.

[The state of the stirrup prosthesis estimated from the results of functional multispiral computed tomography].

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.

[The use of functional multispiral computed tomography for differential diagnostics of otosclerosis and congenital malformation of the internal and middle ear (a case report)].

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.

Long-term results with the Rion E-type semi-implantable hearing aid.

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.