SMARCA4 mutation causes human otosclerosis and a similar phenotype in mice.

BACKGROUND: Otosclerosis is a common cause of adult-onset progressive hearing loss, affecting 0.3%-0.4% of the population. It results from dysregulation of bone homeostasis in the otic capsule, most commonly leading to fixation of the stapes bone, impairing sound conduction through the middle ear. Otosclerosis has a well-known genetic predisposition including familial cases with apparent autosomal dominant mode of inheritance. While linkage analysis and genome-wide association studies suggested an association with several genomic loci and with genes encoding structural proteins involved in bone formation or metabolism, the molecular genetic pathophysiology of human otosclerosis is yet mostly unknown. METHODS: Whole-exome sequencing, linkage analysis, generation of CRISPR mutant mice, hearing tests and micro-CT. RESULTS: Through genetic studies of kindred with seven individuals affected by apparent autosomal dominant otosclerosis, we identified a disease-causing variant in SMARCA4, encoding a key component of the PBAF chromatin remodelling complex. We generated CRISPR-Cas9 transgenic mice carrying the human mutation in the mouse SMARCA4 orthologue. Mutant Smarca4+/E1548K mice exhibited marked hearing impairment demonstrated through acoustic startle response and auditory brainstem response tests. Isolated ossicles of the auditory bullae of mutant mice exhibited a highly irregular structure of the incus bone, and their in situ micro-CT studies demonstrated the anomalous structure of the incus bone, causing disruption in the ossicular chain. CONCLUSION: We demonstrate that otosclerosis can be caused by a variant in SMARCA4, with a similar phenotype of hearing impairment and abnormal bone formation in the auditory bullae in transgenic mice carrying the human mutation in the mouse SMARCA4 orthologue.

A comparison between endoscopic and microscopic approaches for stapes surgery: experience of a tertiary referral center.

PURPOSE: Otosclerosis is a common ear disease causing ankylosis of the stapedio-vestibular joint and conductive hearing loss. Stapedoplasty is the most advisable surgical solution. The restoration of hearing depends on the condition of the patient and the surgery itself. The aim of our work was to compare the surgical and audiological results of stapedoplasty performed with endoscopic versus microscopic technique. METHODS: This is a retrospective study of 254 patients treated with stapedoplasty with a microscopic approach (91/254) or with an endoscopic approach (163/254) between 2014 and 2021 at our tertiary referral center. Statistical significance of differences between the two methods was determined using the Mann-Whitney test for quantitative variables and the Wilcoxon matched-pairs signed-rank test for repeated measures. Categorical variables were assessed with Fisher's exact test. RESULTS: Both techniques improved the hearing status of patients, with no statistically significant difference between them. There was also no statistically significant difference in reported complications between the two techniques. There is a statistical difference (p < 0.001) in operating time between the two techniques: the endoscopic technique had a mean operating time of 39 min versus 45 min for the microscopic technique. CONCLUSIONS: The two techniques are comparable in terms of results and the choice depends on the surgeon's preferences and experience.

The effect of piston diameter in primary stapes surgery on surgical success.

PURPOSE: To evaluate the effect of piston diameter in patients undergoing primary stapes surgery on audiometric results and postoperative complications. METHODS: A retrospective single-center cohort study was performed. Adult patients who underwent primary stapes surgery between January 2013 and April 2022 and received a 0.4-mm-diameter piston or a 0.6-mm-diameter piston were included. The primary and secondary outcomes were pre- and postoperative pure-tone audiometry, pre- and postoperative speech audiometry, postoperative complications, intraoperative anatomical difficulties, and the need for revision stapes surgery. The pure-tone audiometry included air conduction, bone conduction, and air-bone gap averaged over 0.5, 1, 2 and 3 kHz. RESULTS: In total, 280 otosclerosis patients who underwent 321 primary stapes surgeries were included. The audiometric outcomes were significantly better in the 0.6 mm group compared to the 0.4 mm group in terms of gain in air conduction (median = 24 and 20 dB, respectively), postoperative air-bone gap (median = 7.5 and 9.4 dB, respectively), gain in air-bone gap (median = 20.0 and 18.1 dB, respectively), air-bone gap closure to 10 dB or less (75% and 59%, respectively) and 100% speech reception (median = 75 and 80 dB, respectively). We found no statistically significant difference in postoperative dizziness, postoperative complications and the need for revision stapes surgery between the 0.4 and 0.6 mm group. The incidence of anatomical difficulties was higher in the 0.4 mm group. CONCLUSION: The use of a 0.6-mm-diameter piston during stapes surgery seems to provide better audiometric results compared to a 0.4-mm-diameter piston, and should be the preferred piston size in otosclerosis surgery. We found no statistically significant difference in postoperative complications between the 0.4- and 0.6-mm-diameter piston. Based on the results, we recommend always using a 0.6-mm-diameter piston during primary stapes surgery unless anatomical difficulties do not allow it.

Surgical decision-making in superior canal dehiscence syndrome with concomitant otosclerosis.

OBJECTIVE: The diagnosis and management of Superior Canal Dehiscence Syndrome (SCDS) with concomitant otosclerosis can be a challenge. Otosclerosis can mask SCDS symptoms and stapes surgery may reveal or exacerbate vestibular symptoms. Our aim is to present four cases of SCDS with concomitant otosclerosis and thereby informing the reader about the possibility of this dual occurrence and its implications for treatment. CASES: Four patients with SCDS and concomitant otosclerosis are presented. Two patients underwent surgical treatment for both SCDS and otosclerosis and two patients opted for conservative management. OUTCOMES: The main differences between surgically and non-surgically treated cases are the presence of autophony and pressure-induced vertigo and a more severe experience of symptoms in surgically treated cases. Surgically treated cases achieved a sizeable reduction in postoperative air-bone gap and resolution of vestibular symptoms. CONCLUSION: The subjective severity of symptoms in combination with shared decision-making is key in determining the appropriate treatment plan for SCDS and concomitant otosclerosis.

Conventional vs. diode laser stapedotomy: audiological outcomes and clinical safety.

PURPOSE: To compare the hearing results and clinical safety of patients undergoing stapes surgery with conventional technique and diode laser. METHODS: Retrospective observational study, which included patients treated with primary stapes surgery performed between January 2009 and January 2020. Three audiometric measurements (PTA, GAP and SDS) were evaluated as main results, evaluated by analysis of covariance (controlling the preoperative value). Intraoperative and postoperative complications were also analyzed. Outcomes were measured 6 months (± 1 month) after surgery. RESULTS: 153 cases were included, 97 operated with conventional technique and 56 with laser technique. Postoperative GAP ≤ 10 dB was obtained in 85.6% of the total sample, 82.5% in the conventional technique and 91.1% in the laser technique. Analysis of covariance showed no significant differences in the three surgery outcomes between the two groups (PTA, p = 0.277; GAP, p = 0.509 and SDS, p = 0.530). Regarding surgical complications, sensorineural damage was higher in the conventional technique group (p = 0.05). On the other hand, there were four cases of facial paresis, all in the laser group, three of them with the 980 nm laser. CONCLUSIONS: Stapedotomy offered a high percentage of hearing success in the two groups studied. There were no significant differences in audiometric result, but there was a differential presentation of complications, being more frequent sensorineural hearing loss in the conventional technique group and facial paresis in the laser group.

Audiometric and Vestibular Function after Classic and Reverse Stapedotomy.

Background and Objectives: Besides classical stapedotomy, reverse stapedotomy has been used for many years in the management of otosclerosis. Our study aims to investigate whether reversing the surgical steps in stapedotomy impacts vestibular function and hearing improvement. Materials and Methods: A cohort of 123 patients underwent either classic or reverse stapedotomy procedures utilizing a fiber-optic argon laser. Audiological assessments, following the guidelines of the Committee on Hearing and Equilibrium, were conducted, including pure tone average, air-bone (AB) gap, overclosure, and AB gap closure. Vestibular evaluation involved pre- and postoperative comparison of rotatory test parameters, including frequency, amplitude, and slow phase velocity of nystagmus. Results: The study demonstrated an overall median overclosure of 3.3 (3.3, 5.0) dB and a mean AB gap closure of 20.3 ± 8.8 dB. Postoperative median AB gap was 7.5 (7.5, 11.3) dB in the reverse stapedotomy group and 10.0 (10.0, 12.5) dB in the classic stapedotomy group. While overclosure and AB gap closure were marginally superior in the reverse stapedotomy group, these differences did not reach statistical significance. No significant disparities were observed in the frequency, slow phase velocity, or amplitude of nystagmus in the rotational test. Conclusions: Although not always possible, reverse stapedotomy proved to be a safe surgical technique regarding postoperative outcomes. Its adoption may mitigate risks associated with floating footplate, sensorineural hearing loss, and incus luxation/subluxation, while facilitating the learning curve for less experienced ear surgeons.

The Impact of Surgical Expertise on the Cost-Effectiveness of Stapes Surgery.

INTRODUCTION: Otosclerosis is the primary cause of conductive hearing loss with normal otoscopy. As the condition worsens, certain patients may develop a sensorineural component. Patients with successful surgeries may still need hearing aids, which creates a dilemma for health professionals as there are insufficient data to make informed decisions. This study investigated the influence of the surgeon's proficiency level, individual patient factors (e.g., age at the time of intervention and survival rates), and surgery costs on the cost-effectiveness of stapes surgery. METHODS: We performed a cost-effectiveness analysis using an adapted Markov model incorporating annual all-cause mortalities. In addition, we introduced sensitivity analyses to address the effects of surgical expertise on adults with bilateral conductive hearing loss due to otosclerosis. A model was developed based on a decision tree with treatment options and complication scenarios for otosclerosis patients undergoing stapes surgery or receiving hearing aids. Annual all-cause mortality was considered. A sensitivity analysis was performed assigned to different training levels ("experts" and "less experienced") to simulate the effects of surgical experience on the cost-effectiveness of surgical outcomes. Successful surgery was defined as closing of the air-bone gap to 10 dB or less. Based on published data, "experts" were simulated with a 93.7% success rate, and "less experienced" were manufactured with a 68.9% success rate. RESULTS: Stapes surgery provides improved quality of life (QoL) compared to hearing aids with lower cumulative costs up to 87 years of age in the case of "expert" surgeons and up to 78 years of age, when performed by "less experienced" surgeons. CONCLUSIONS: Primary stapes surgery is highly cost-effective and delivers improved QoL compared to hearing aids with lower cumulative costs. Additionally, undergoing stapes surgical training remains highly cost-effective.

Diagnostic Value of the Air-Bone Threshold Gap in Stapes Fixation.

INTRODUCTION: The Carhart notch is a well-known sign of stapes fixation. However, previous studies have reported that the Carhart notch is not specific to stapes fixation and is also present in other middle ear diseases. Therefore, this study investigated the diagnostic value of threshold gap between air conduction and bone conduction (ABG) for stapes fixation, instead of the bone conduction dip representing the Carhart notch. METHODS: A total of 199 ears that underwent exploratory tympanotomy were enrolled in this retrospective study. They were categorized into three groups according to surgical findings: stapes fixation (SF), other ossicle fixation (OF), and chain disconnection (CD). Preoperative pure-tone audiograms and impedance audiograms were compared between the groups. RESULTS: The incidence of the Carhart notch did not differ between the groups. The ABG at 2,000 Hz showed a good diagnostic performance for distinguishing between the SF and CD groups (area under the curve, AUC = 0.816, p < 0.001), but poor performance for distinguishing between the SF and OF groups (AUC = 0.662, p = 0.003). Bone conduction at 2,000 Hz showed a moderate performance for distinguishing between the SF and CD groups (AUC = 0.707, p < 0.001) and did not show statistically significant results for distinguishing between the SF and OF groups (AUC = 0.594, p = 0.080). The tympanic membrane compliance was significantly higher in the CD group than in the SF group (p = 0.001). CONCLUSIONS: The Carhart notch was not a specific finding of SF. The sensitivity and specificity of ABG ≤15 dB at 2,000 Hz for distinguishing between SF and CD were 60.4% and 89.2%, respectively. To prepare for surgical interventions in patients with conductive hearing loss but a normal tympanic membrane, clinicians should comprehensively consider these results.

Impact of Otosclerosis on Auditory Ossicle Remodeling: A Scanning Electron Microscopy Analysis of Stapes Head Overloads.

BACKGROUND Otosclerosis is a pathology that interferes with the conduction of vibrations to the inner ear, triggering changes in the auditory ossicles and their associated joints due to mechanical overload. This study primarily aims to evaluate these overload-induced modifications in the stapes head resulting from the immobilization of the base of the third auditory ossicle in otosclerosis patients. MATERIAL AND METHODS We conducted a comparative analysis of patients undergoing their first surgery for otosclerosis. The test group consisted of 31 patients who underwent stapedotomy between 2020-2021. For comparison, we utilized a control group comprising stapes samples extracted during vestibular schwannoma surgeries via a transcochlear approach. A prospective analysis of bone tissue surface topography and chemical composition was executed using scanning electron microscopy (SEM). RESULTS SEM analysis of the stapes head in otosclerosis patients relative to the control group displayed no significant differences in chemical composition or the presence of otosclerotic foci. Nonetheless, various forms of bone tissue surface damage were noted on the stapes head in all otosclerosis patients. Mild changes were evident in 90% of the samples, while small linear bone tissue fractures were observed in 58% of the samples. Furthermore, minor osteophytic changes were detected in 16% of the samples. CONCLUSIONS The immobilization of the stapes base by otosclerotic foci instigates overloads in the incus-stapes joint, leading to the eventual remodeling of the stapes head articular surface.

Retrospective Study of 157 Patients with Otosclerosis to Evaluate Association Between Carhart Notch on the Preoperative Bone-Conduction Audiogram and Postoperative Hearing and Balance Evaluated by the Vestibular Disorders Activities of Daily Living Scale.

BACKGROUND A Carhart notch in the pure tone audiogram can be an indicator of stapes fixation in otosclerosis. This retrospective study of 157 patients with otosclerosis aimed to evaluate the association between the presence of a Carhart notch on the preoperative bone-conduction audiogram and postoperative hearing and balance evaluated by the Vestibular Disorders Activities of Daily Living scale. MATERIAL AND METHODS Patients with suspected otosclerosis based on medical history and audiometric tests were considered. The analysis included 157 consecutive patients who underwent surgery in the years 2016 to 2019, in whom the diagnosis of otosclerosis was confirmed during surgery. Carhart notch was defined as an impairment in the bone conduction threshold of ≥7.5 dB for 2000 Hz frequencies above the mean thresholds at higher and lower adjacent frequencies. The Vestibular Disorders Activities of Daily Living subjective scale was used in the preoperative period and 4 and 12 months after surgery. RESULTS The preoperative presence of Carhart notch and progressive sensorineural hearing loss were statistically significantly correlated with more common onset of tinnitus and then dizziness (P=0.006). Preoperative vertigo was observed in patients who had Carhart notch observed in the preoperative audiometric test. This vertigo more commonly coexisted with profound sensorineural hearing loss and minor or no improvement in average values of bone conduction after surgery (P=0.002). CONCLUSIONS Preoperative Carhart notch on audiogram and the severity of sensorineural hearing loss were associated with tinnitus and vertigo. However, preoperative Carhart notch was not associated with persistent postoperative tinnitus in patients with cochlear otosclerosis.

Comparison of endoscopic and microscopic methods in stapedotomy: a retrospective analysis.

PURPOSE: The goal of the present study was to compare the audiologic results, complications, and advantages/disadvantages of endoscopic and microscopic stapedotomy. METHODS: Patients who experienced stapedotomy surgery in the Ear Nose Throat Clinic (ENT) of Selcuk University Faculty of Medicine between September 2011 and January 2018 were included in the study. The data of all patients were analyzed and divided into two groups. Those who underwent endoscopic stapedotomy were included in group I, and patients who underwent microscopic stapedotomy formed group II. Surgical findings, complications, and operation times were compared for the two groups. Air and bone conduction thresholds were evaluated at the frequencies of 500, 1000, 2000, and 4000 Hz pre- and postoperatively at 1, 3, and 6 months, and the mean (± SD) air-bone gap value was recorded. RESULTS: While the mean pre- and postoperative air-bone gaps were 34.38 ± 7.47 dB (23-53 dB) and 9.69 ± 4.43 dB (0-19 dB), respectively, in group I, 34.32 ± 7.57 dB (23-6 dB) and 9.62 ± 4.25 dB (2-23 dB) were the respective means calculated in group II (p < 0.05). When the mean postoperative air-bone gap was compared, there was no statistically significant difference between the two groups (p = 0.774). The mean operative times for groups I and II were 57.22 ± 4.37 min and 63.70 ± 7.34 min, respectively (p < 0.001). The requirement for chorda tympani nerve manipulation and scutum curettage was significantly less in group I compared to group II (p = 0.003). Postoperative dysgeusia and postoperative pain were found to be higher in group II than group I, but they were not statistically significant (p > 0.05). CONCLUSION: Endoscopic stapedotomy displayed similar audiological results, shorter operation times, and similar complication rates, as well as being a less invasive surgery, compared to the microscopic approach.

Endoscopic stapedotomy: classic versus reversal technique.

OBJECTIVES: To compare hearing outcome and surgical complications between endoscopic classic and reversal stapedotomies. PATIENTS AND METHODS: A prospective single blinded randomized clinical study carried out on 60 patients with otosclerosis who were randomized into two groups; each containing 30 patients. Patients in group 1 underwent endoscopic classic stapedotomy. Patients in group 2 underwent endoscopic reversal stapedotomy. Both groups were compared as regards hearing outcome and surgical complications. RESULTS: The difference in the hearing outcome between the two groups was statistically non-significant. Post-operative closure of the air bone gap (ABG) within 10 dB was attained in 76.67% and 80% of patients in groups 1 and 2, respectively. The differences in the surgical complications between the two studied groups were statistically non-significant. CONCLUSION: Endoscopic classic and reversal stapedotomies are comparable to each other as regards hearing outcome and surgical complications. The authors recommend further studies with relatively larger sample size.

Comparison of the efficacy of cochlear implantation and stapes surgery in far advanced otosclerosis: a meta-analysis study.

OBJECTIVE: This study is to compare the hearing outcomes and complications of stapes surgery and cochlear implantation (CI) in patients with far-advanced otosclerosis (FAO). DATA SOURCES: A comprehensive electronic search of PubMed/MEDLINE, Scopus, Web of science and Cochrane Library was conducted in June 2021 for articles in the literature till this year. STUDY SELECTION: Studies are published in English language, conducted on human subjects, concerned with comparison of CI and stapes surgery in the management of FAO, not Laboratory study and not Opinion study. The current review followed the guidelines of preferred reporting items for systematic reviews and meta-analysis statement 2009 (PRISMA). DATA EXTRACTION: Twenty-six studies were included with 334 patients in CI group and 241 patients in stapes surgery group. Comparison between both groups was done in terms of postoperative complications, audiological outcomes, rete of revision surgery and patients' satisfaction rate. RESULTS: Postoperative complications rate was significantly lower in CI (13.6%) than stapes surgery (18.6%). CI had a significantly lower rate of revision surgery (8.1%) than stapes surgery (16.4%). CI had a better mean for pure tone average (29.1 dB) than stapedectomy (52.3 dB) while stapes surgery had a higher mean for recognition of monosyllables and disyllables than CI. CI had significantly higher satisfaction rate than stapes surgery. CONCLUSION: Both Stapes surgery and CI are reliable treatment options for FAO with close success rates. Statistics of CI are greater than stapes surgery and CI has a consistent improvement in audiometric outcomes in comparison to stapes surgery.

"Hot" vs "Cold" endoscopic stapes surgery: a matched case-control study.

PURPOSE: To compare hearing results and complication rates between two groups of patients operated on by endoscopic stapes surgery (ESS) for otosclerosis, either with CO2 fiber laser or microdrill. METHODS: A case-control study was performed. All consecutive cases of CO2 fiber laser ESS operated at a single center during the period 2017-2020 (case group) were matched to a control group of patients operated by traditional technique, according to year of surgery, preoperative mean air-bone gap, sex and age. Audiological data from preoperative and postoperative examinations and complication rates were compared. RESULTS: 46 cases were included. Mean operative time was significantly longer in the laser cohort (65 min) than in the drill one (45 min) (p = 0.003). Similar results were found in the two groups regarding the mean postoperative BC-PTA. The high-frequency bone conduction resulted significantly higher in the laser group (p = 0.002), suggesting an overclosure effect in the laser group. Consistently, a significant improvement of the BC-PTA threshold at 2000 Hz postoperatively was found in the laser group (p = 0.034). The postoperative AC-PTA significantly improved in both groups at all frequencies (p < 0.05), except for the AC threshold at 8 kHz. Similar rates of complications were found in the two groups. CONCLUSION: This study is the first to compare hearing results and complications between CO2 fiber laser and microdrill in ESS. Our results demonstrated similar functional outcomes between the two groups, confirming ESS as safe and effective, regardless of the technique used.

Audiological outcome after stapes surgery in relation to prosthesis type.

PURPOSE: Different techniques are used to fix crimp and CliP® Piston stapes prostheses to the long process of the incus (LPI). The CliP® Piston provides a stiff connection in contrast to the static bended loop of the crimp prosthesis, which imitates the physiological incudostapedial joint (ISJ) and thereby potentially leads to different hearing outcome. METHODS: In a retrospective single-center study of German-speaking one hundred and ninety patients who underwent stapes surgery CliP® Piston or crimp prostheses between the years of 2016 and 2019 by the same surgeon and in the same setting. Pre- and postoperative bone- (BC) and air-conduction (AC) pure-tone thresholds, pre- and postoperative air-bone gap (ABG) for 0.5, 1, 1.5, 2, 3, 4 kHz and the surgery time were examined. RESULTS: The postoperative bone conduction thresholds were significantly lower in the frequencies between 0.5 and 3 kHz and the mean ABG was < 10 dB in most cases independent of the prosthesis used. Crimp prosthesis showed a significantly better closure of the ABG at 0.5 kHz. CONCLUSIONS: The audiological outcome after stapes surgery is dependent on the type of prosthesis used, as reflected by the frequency-specific air-bone gap. The better ABG closure with the crimp prosthesis might be the result of the connection to the LPI imitating the physiological ISJ. The crimp prosthesis may be the better choice if use of hearing aids is expected postoperatively.

Assessment of the ability of the radiological incudo-stapedial angle to predict the stapedotomy technique type: a prospective case-series study.

PURPOSE: This study aimed to propose a radiological classification of the incudo-stapedial angle by preoperative high-resolution computed tomography (HRCT) images and to highlight its importance for predicting the use of reversal-steps stapedotomy (RSS) rather than the traditional non-reversal technique. METHODS: We included 83 candidates for stapedotomy operation. Two physicians measured the radiological incudo-stapedial joint angle in the preoperative HRCT. According to this measurement, the radiological incudo-stapedial joint was classified into three types: obtuse, right, and acute. In addition, this radiological classification was correlated with the intraoperative use of the stapedotomy technique, either reversal or non-reversal. RESULTS: The RSS technique was used in forty-two (97.7%) cases with an obtuse angle and twenty-six (89.7%) with a right angle. At the same time, the traditional non-reversal technique was used in all patients with an acute angle. The three groups differed significantly regarding the method used for stapedotomy (P value < 0.001). Moreover, Spearman's correlation coefficient revealed a significant correlation between the used technique and the radiological type of the incudo-stapedial angle (P value < 0.001). CONCLUSIONS: This prospective study proposed a preoperative radiological classification of the incudo-stapedial angle. This classification was significantly correlated with the type of stapedotomy technique. The RSS technique was feasible in most cases with an obtuse and right radiological incudo-stapedial angle. In contrast, the non-reversal method was used in all patients with an acute radiological incudo-stapedial angle. This radiological classification could predict the choice for the stapedotomy technique with an accuracy of 95.18%, a sensitivity of 73.33%, and a specificity of 100%.

Are there differences in revision stapes surgery outcomes between university and county clinics? A study from the quality register for otosclerosis surgery in Sweden.

PURPOSE: The aim of the study was to investigate hearing outcomes in stapes revision surgery with regard to the type of clinic (university clinic or county clinic). Furthermore, the aim was to investigate the risk of complications with a focus on tinnitus, hearing deterioration, and taste disturbance 1 year after surgery. METHODS: The study is based on data from the Swedish Quality Register for Otosclerosis Surgery (SQOS). Two study protocols were completed by the surgeon, and a questionnaire was distributed to the patients 1 year after surgery. A total of 156 revisions were available for analysis with both preoperative and postoperative audiometry data. RESULTS: Seventy-five percent of the patients reported better to much better hearing 1 year after revision surgery. An air bone gap ≤ 20 dB postoperatively was seen in 77% of the patients. Four percent had hearing deterioration ≥ 20 dB PTA4 AC. Eleven percent had worsened or newly developed tinnitus, 5% had taste disturbance, and 3% had dizziness 1 year after surgery. Preoperative and postoperative hearing did not differ between patients operated on in university vs. county clinics. CONCLUSIONS: Revision surgery in otosclerosis is a challenge for otologists, but no differences in hearing outcomes between university and county clinics were found in this nationwide study. The risk of hearing deterioration and deafness is higher than in primary stapes surgery, and revision surgery should be recommended primarily in cases with a large air-bone gap and moderate to severe preoperative hearing loss.

Audiometric and Self-Reported Outcomes in Patients with Otosclerosis and a Small Air-Bone Gap after Stapes Surgery.

INTRODUCTION: The main determinant in deciding on stapes surgery in patients with otosclerosis is the degree of hearing loss, specifically the size of the preoperative air-bone gap (ABG). The debate over the minimum ABG centers on the risk-to-benefit ratio of stapes surgery in patients with small ABG (sABG). The aim of this study was to measure the audiological outcomes and self-assessed satisfaction in a group of otosclerosis patients with an sABG who underwent stapedotomy. METHODS: There were 83 patients with preoperative sABG ≤25 dB HL (mean of 500, 1,000, 2,000, 4,000 Hz) included in this study. Audiometry was performed before surgery and 6 months and 12-36 months after surgery. Self-reported patient outcomes before and after surgery were collected using questionnaires. RESULTS: At the 6-month follow-up, the ABG was closed within 10 dB in 63 (78.8%) cases. Preoperatively, tinnitus was present in 70% of patients, of which 66% reported that tinnitus was a moderate or severe problem. Postoperatively, 71% of patients experienced a significant reduction in tinnitus severity and 34% of them reported complete disappearance. The self-report outcomes relating to quality of life and hearing reflected a good level of satisfaction in most patients. CONCLUSION: The possibility of reducing bothersome tinnitus after stapes surgery, and thus improving the patient's quality of life, should be taken into account when making a decision on stapes surgery in these patients.

[Role of computed tomography in the preoperative diagnosis of otosclerosis]. / Stellenwert der Computertomographie in der präoperativen Diagnostik der Otosklerose.

BACKGROUND: Otosclerosis is an osteodystrophy of the otic capsule and presents with progressive conductive hearing loss. Imaging studies, especially computed tomography (CT) and cone-beam CT, have gained increased relevance in the diagnosis of otosclerosis. OBJECTIVE: This study investigated whether there is a correlation between the extent of otosclerosis in high-resolution or cone-beam CT and hearing loss in pure-tone audiometry. MATERIALS AND METHODS: Based on an existing classification of otosclerotic foci, a classification was established. Preoperative CT scans of patients undergoing stapedotomy between 2015 and 2019 were evaluated and classified by two independent otorhinolaryngologists. The preoperative pure-tone audiograms were analysed and compared to the results of CT. RESULTS: A total of 168 CT studies (i.e., 168 ears) in 156 patients with intraoperatively confirmed otosclerosis were included in our study. A correlation between the extent of the otosclerotic focus or the calculated scores and hearing loss in pure-tone audiometry (air conduction, bone conduction and air-bone-gap) could not be proven. CONCLUSION: Preoperative CT is not obligatory. However, preoperative imaging using CT or cone-beam CT can be helpful to confirm the diagnosis and exclude other middle or inner ear pathologies as well as in planning of the surgical procedure in the overall context of otoscopy and audiometry. A correlation with the degree of hearing impairment could not be demonstrated and remains unclear.

Vertigo Associated with Otosclerosis and Stapes Surgery-A Narrative Review.

Otosclerosis is a pathological condition affecting the temporal bone, and is characterized by remodelling of the labyrinthine bone tissue through a dynamic process of osteolysis and osteogenesis. This condition progressively leads to hearing loss, tinnitus, and vertigo. Stapedotomy, a surgical procedure involving the removal of the stapes superstructure and its replacement with a prosthesis, is the treatment of choice to improve hearing in individuals with otosclerosis. However, vestibular dysfunction is a significant complication associated with this procedure, which can occur intraoperatively or postoperatively, ranging from the immediate postoperative period to weeks, months, or even years after surgery. This paper aims to provide a comprehensive review of the most important causes of vertigo associated with otosclerosis and stapes surgery with the goal of minimizing the incidence of this complication. Understanding the underlying factors contributing to vertigo in this context is crucial for the prevention and effective management of vertigo in patients undergoing stapedotomy.