[Otosclerosis : clinical presentation and management]. / Otosclérose : présentation clinique et prise en charge.

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.

L'otosclérose est caractérisée par un remodelage pathologique de l'os de la capsule otique. Classiquement, un foyer d'os patho­logique entraîne une fixation progressive de l'étrier qui résulte en une atteinte auditive de transmission. L'atteinte peut être uni ou bilatérale. L'otoscopie est normale. L'hypoacousie apparaît généralement entre 30 et 50 ans, régulièrement avec un acouphène. Parfois, l'oreille interne est aussi affectée, avec une atteinte auditive de perception ou encore vestibulaire (vertige). La réhabilitation auditive apporte d'excellents résultats, par un appareillage auditif ou une chirurgie de l'étrier, la stapédotomie. En cas d'atteinte auditive de perception marquée, une implantation cochléaire est considérée. Un CT-scan ou un Cone Beam CT en coupes fines permet de confirmer le diagnostic et de planifier la chirurgie.

[The application of endoscope and microscope in the stapes surgeries].

Objective:To compare the application of endoscope and microscope in all kinds of stapes surgeries. Methods:Fifty-nine stapes surgeries have been collected from April 2020 to May 2023 in Senior Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School. Hearing level, hospital stay post-operation, times of hospital visit post-operation, etc. have been compared between the endoscopic group and microscopic group. Patients who were failed to place the stapes prosthesis because of the poor exposure of the oval window have been analyzed. Results:Otosclerosis was the most common diagnosis in both groups. There was 1（1/23） middle ear malformation in the endoscopic group and 5（5/36） middle ear malformations in the microscopic group. There were 2 Van Der Hover syndromes and 4 Treacher Collins syndromes in the microscopic group. In the endoscopic group ABG of 10 ears（43.5%） ≤ 10 dB, and ABG of 21 ears（91.3%） ≤20 dB.In the microscopic group ABG of 13 ears（41.9%） ≤ 10 dB, and ABG of 28 ears（90.3%） ≤ 20 dB. There was no statistic difference between 2 groups. Times of hospital visit post-operation in the endoscopic group was less than in the microscopic group（P<0.01）. There was no facial palsy, tympanic perforation or profound sensorineural hearing loss in both groups. Conclusion:Endoscope is more suitable for patients who are evaluated with no severe stapes malformation, or less manipulation of drilling the bone. It could also reduce the hospital visit post-operation. Patients with narrow ear canal or severe middle ear malformation are recommended to perform the surgery with microscope, because it provides the chance of manipulation with 2-hands of surgeons.

Otosclerosis: audiometric results and quality of life after stapedectomy with interposition or CO2 laser-calibrated stapedotomy without interposition.

BACKGROUND: The surgical treatment of otosclerosis consists of the replacement of the stapes using a piston. AIMS/OBJECTIVES: The objective of this study is to assess the audiometric results and quality of life (QOL) of patients who underwent surgery for otosclerosis by stapedectomy with fascia interposition (STIP) or by stapedotomy using CO2 laser (SLaser) without fascia interposition. MATERIAL AND METHODS: Ninety-one patients operated on for otosclerosis by STIP (40 patients) and by SLaser (51 patients) were included. Audiometric results were graphically displayed using the Amsterdam Hearing Evaluation Plot and were compared to the Common Otology Database (COD) comprising 660 patients. The patients' QOL was assessed with an otological validated survey. The significance level was p < .05. RESULTS: There was no significant difference in hearing improvement between SLaser and STIP-operated patients. There was no significant difference between our results and that of COD. QOL was significantly improved (+3.5/10) postoperatively for both STIP and SLaser in both social and professional fields. CONCLUSIONS AND SIGNIFICANCE: By comparing QOL and hearing gain of STIP and SLaser, our results suggest that both surgical techniques are a safe and successful treatment for otosclerotic stapes fixation. Further studies aiming at comparing larger series may confirm and refine these results.

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.

High-resolution computed tomography and pure-tone audiometry in patients with otosclerosis in the spongiotic phase.

OBJECTIVE: There is no consensus in the literature regarding the relationship between high-resolution computed tomography findings and hearing thresholds in pure-tone audiometry in otosclerosis. This study evaluated the association between high-resolution computed tomography findings and pure-tone audiometry in otosclerosis in the spongiotic phase. METHODS: A cross-sectional study was conducted of 57 ears with surgically confirmed stapes fixation and tomographic findings. Air conduction and bone conduction thresholds on audiometry, and air-bone gap, were analysed. RESULTS: There were no correlations between sites affected by otospongiosis and air conduction threshold, bone conduction threshold or air-bone gap in the analysed tomographic images, but the diameter of the otospongiotic focus was greater in the presence of extension of the otospongiotic foci to the cochlear endosteum. CONCLUSION: There were no relevant associations between high-resolution computed tomography findings and pure-tone audiometric measurements. However, the diameter of the otospongiotic focus was greater in the presence of extension of the otospongiotic foci to the cochlear endosteum.

Preoperative Evaluation of Otosclerosis: A National Survey of Otologists.

OBJECTIVE: Describe practice patterns in preoperative assessment for stapedectomy. STUDY DESIGN: Survey. SETTING: Tertiary referral center. SUBJECTS: Active members of the American Neurotologic Society and American Otologic Society. INTERVENTION: Survey. MAIN OUTCOME MEASURES: Percent of respondents performing preoperative testing with acoustic reflexes (ARs), electrocochleography, vestibular evoked myogenic potentials, and computed tomography (CT). Further analysis of those not ordering routine CT to determine whether imaging would be ordered for previous ear surgery, vestibular complaints, childhood hearing loss, AR inconsistent with otosclerosis, possible advanced otosclerosis, or atypical complaints, including autophony. Further subgroup analysis based on years in practice and practice setting (private versus academic). RESULTS: Most respondents (56.5%) had practiced more than 15 years and worked in academic settings (69.4%). Rates of routine use of preoperative AR, vestibular evoked myogenic potential, and electrocochleography were 80, 4.7, and 0%, respectively. There were no significant differences based on time in practice or practice settings. For CT, 35.3% reported routine use with a statistically significant difference between academic and private practice respondents (42.4% versus 19.2%, p = 0.040). For CT contingent on specific clinical factors, only AR inconsistent with otosclerosis showed a statistically significant difference between academic and private practice providers (85.3% versus 57.1%, p = 0.020). CONCLUSION: Most otologists routinely obtain AR before stapedectomy. Academic providers more commonly order CT routinely and for AR inconsistent with otosclerosis. Most respondents not ordering routine CT ordered imaging in specific clinical scenarios. Overall, there is a high level of consistency in preoperative testing regardless of practice setting or time in practice.

Inner ear impairment after stapedotomy: do the cervical vestibular evoked myogenic potentials play a diagnostic role?

BACKGROUND: Otosclerosis is characterized by a bony remodeling process that ends up with stapes fixation. The hearing impairment can be recovered by surgery by replacing the stapes superstructure. Due to the surgical management of the vestibule, the vestibular examination could provide an insight into the correlation between this kind of surgery and vestibular changes. OBJECTIVES: To evaluate the impact of the stapedotomy on the inner ear. METHODS: We evaluated pure tone audiometry and the presence of vestibular evoked myogenic potentials (VEMPs) in 41 patients with otosclerosis before and after the stapedotomy operation. RESULTS: Air conduction (Ac)-VEMPs were present in 18 cases preoperatively and 31 cases postoperatively. Bone conduction (Bc)-VEMPs were present in 23 cases preoperatively and 33 cases postoperatively. ABG was closed to less than 20 dB in all cases after the operation. CONCLUSIONS: The preoperative Bc-VEMPS had an outstanding capability to predict the type of hearing loss. The postoperative absence of VEMPS despite the closure of ABG indicated the impact of otosclerosis on the saccular cells. The use of Thulium Laser in stapedotomy didn't affect significantly the saccular cells. SIGNIFICANCE: Integrated use of audiometry and VEMPs was effective to evaluate the changes associated with otosclerosis and the stapedotomy operation.

Thulium laser in stapedotomy surgery.

BACKGROUND: Thulium laser use in stapedotomy surgery is usually associated with many doubts regarding the thermal effect on the vestibular area and the production of acoustic shock waves, which may result in permanent complications. OBJECTIVES: We aimed to evaluate the efficacy and safety of the Thulium laser use in stapedotomy and its long-term effects on the clinical and audiological results. MATERIAL AND METHODS: It was a retrospective randomized case-series study. We included 148 otosclerosis patients. They were divided randomly into two groups; group A (multiple shots) included 62 patients and group B (one shot with a manual perforator) included 86 patients. RESULTS: There was a statistically significant improvement of the ABG in both groups. ABG closure (<10 dB) occurred in 87.1% of patients in group A, and 89.5% of patients in group B. Intraoperative complications were more in group B (p-value = 0.038). The postoperative complications were more in group A (p-value = .017). CONCLUSIONS: According to our experience on a relatively large number of cases, the Thulium laser is a safe tool to be used in stapedotomy either in multiple shots or one shot with a manual perforator. It improved functional hearing efficiently without showing signs of inner ear impairment.

Classification of Wideband Tympanometry by Deep Transfer Learning With Data Augmentation for Automatic Diagnosis of Otosclerosis.

Otosclerosis is a common disease of the middle ear leading to stapedial fixation. Its rapid and non-invasive diagnosis could be achieved through wideband tympanometry (WBT), but the interpretation of the raw data provided by this tool is complex and time-consuming. Convolutional neural networks (CNN) could potentially be applied to this situation to help the clinicians categorize WBT data. A dataset containing 135 samples from 80 patients with otosclerosis and 55 controls was obtained. We designed a lightweight CNN to categorize samples into the otosclerosis and control. Receiver operating characteristic (ROC) analysis showed an area under the curve (AUC) of 0.95 ±0.011, and the F1-score was 0.89 ±0.031 ( r=10). The performance was further improved by data augmentation schemes and transfer learning strategies (AUC: 0.97 ±0.010, F1-score: 0.94 ±0.016, , ANOVA). Finally, the most relevant diagnostic features employed by the CNN were assessed via the activation pattern heatmaps. These results are crucial for the visual interpretation of WBT graphic outputs which clinicians use in routine, and for a better understanding of the WBT signal in relation to the ossicular mechanics.

[Tendoplasty and its effectiveness in the rehabilitation of hearing loss in patients with otosclerosis]. / Tendoplastika i ee effektivnost' pri khirurgicheskoi reabilitatsii patsientov s tugoukhost'yu, obuslovlennoi otosklerozom.

The article briefly presents the physiology of the stapes muscle tendon (SMT), and features in its defeat. Its length was studied using high-resolution multispiral computed tomography of the temporal bones. We also studied the possibility of its restoration using the author's method of tendoplasty using metallized stapes prostheses. Tendoplasty with stapedoplasty was performed in 74 patients with otosclerosis (OS), and 48 patients had stapedoplasty without tendoplasty. As a result of research, the average length of the SMT was 2.38±0.02 mm, which explains the need to use a 3 mm long venous autograft for tendoplasty. The author's method of tendoplasty for stapedoplasty allows restoring the acoustic reflex in 54.1% of OS patients using artificial stapes prostheses. The preservation of the vascular bed in the thickness of the restored tendon can improve the trophy of the long incus process and reduce the risk of its dystrophic changes in the postoperative period. In addition, this fact confirms the importance of the stapes muscle in the acoustic reflex.

Gray-Scale Inversion on High Resolution Computed Tomography of the Temporal Bone: An Observational Study.

OBJECTIVES: This is a qualitative study to explore the utility of gray-scale inversion or the "invert" function of high-resolution computed tomography (HRCT) scans in the diagnosis of temporal bone anatomy and pathology. METHODS: This is a case series describing an innovative application of an existing image processing tool to visualize temporal bone anatomy and pathology. Illustrative patients at a tertiary referral center with otologic symptoms and findings leading to HRCT scans of the temporal bone were included. Diagnostic HRCT scans were evaluated utilizing the gray-scale inversion function (invert function). RESULTS: Nine illustrative cases which demonstrate conditions such as persistent stapedial artery, membranous stapes footplate, total ossicular prosthesis migration into the vestibule, third window syndrome such as superior semicircular canal dehiscence (SSCD) and cochlea-facial nerve dehiscence, otosclerosis, and ossicular chain discontinuity are included. The enhanced visualization was confirmed surgically in 3 cases, and 1 had physiological confirmation using cervical vestibular evoked myogenic potentials (cVEMP). CONCLUSIONS: Gray-scale inversion can be used to improve visualization of temporal bone anatomy and pathologic changes when diagnoses are in doubt. The invert function is a useful adjunct in the armamentarium of both radiologists and otologists when evaluating HRCT of the temporal bone.

[Use of the Carina active middle ear implant in otosclerosis patients]. / Einsatz des aktiven Mittelohrimplantats Carina bei Otosklerose.

Patients with otosclerosis can suffer from different grades of combined hearing loss. In addition to surgery (stapedectomy), conventional hearing aids can be used in the treatment of otosclerosis. In cases of severe conductive components in addition to sensorineural hearing loss, treatment with normal hearing aids can be difficult or impossible. In these patients, implantable hearing systems such as cochlear implants represent a possible alternative. The totally implantable Carina middle ear system can be used in patients with even high-grade sensorineural hearing loss. Based on two exemplary cases, the option of using the Carina system in otosclerosis patients and post-implantation results are reported.

Wideband absorbance tympanometry: a novel method in identifying otosclerosis.

PURPOSE: The purpose of the study was to know whether the wideband absorbance measurements can be a useful tool to identify ears with otosclerosis. The present study analyzed WBA measurements and highlighted its effectiveness in identifying ears with otosclerosis and differentiating from healthy normal ears. METHODS: The study included 42 ears with otosclerosis which were compared with an equal sample size of healthy normal ears. WBA across frequencies and wideband average absorbance (375-2000 Hz) at the peak and ambient pressure, and resonance frequency were measured and analyzed. RESULTS: Results showed that WBA levels increased with an increase in frequencies up to 2000 Hz and decreased thereafter, both in the otosclerosis and healthy normal ears. The mean WBA in the otosclerosis group was significantly lower in the 250-2000 Hz frequency range than in the healthy normal ear group. The WBA values at ambient pressure reduced significantly up to 500 Hz for the healthy normal ear group and 1500 Hz for otosclerosis group, compared with peak pressure. Further, the analysis of wideband average absorbance at ambient pressure showed reduced absorbance (0.35) and higher resonance frequency (1350.33 Hz) in the otosclerosis group compared with the healthy normal ear group (0.60 and 930.14 Hz, respectively). ROC analysis indicated that WBA is suitable for identifying otosclerotic ears and also in differentiating from healthy normal ears based on WBA values from 250 to 1500 Hz. High diagnostic values of WBA (> 90% sensitivity and specificity) were observed at a frequency of 1000 Hz. CONCLUSIONS: The inclusion of WBA into clinical routine test procedures could be a useful tool for detecting otosclerosis. Further research is required to validate its clinical use in combination with other middle ear measures.

Wideband absorbance pattern in adults with otosclerosis and ossicular chain discontinuity.

OBJECTIVES: Evidence from previous literature had shown that the use of a single frequency probe tone is not sensitive enough to detect middle ear pathologies, especially related to the ossicles, which hinders accurate diagnosis. The goal of the present study was to compare the outcome of wideband absorbance (WBA) tympanometry and to determine the difference in WBA pattern in adults with otosclerosis and ossicular chain discontinuity. MATERIALS AND METHODS: Estimated adult cases of otosclerosis (10 ears) and ossicular chain discontinuity (06 ears) along with healthy individuals (10 ears) in the age range of 24 to 48 years (mean age: 38.6 years) were considered for the study. WBA was measured at peak and ambient pressure along with resonance frequency and compared with the data obtained from the healthy individuals to determine the WBA pattern. RESULTS: Data analysis revealed a distinct WBA pattern showing high absorbance at 750 Hz for ossicular chain discontinuity compared to healthy individuals, whereas the otosclerosis group showed reduced absorbance (p < 0.05) at low frequencies (250 Hz to 1500 Hz). WBA measured at the peak and ambient pressure did not elicit any significant difference across the frequencies. Also, the average WBA tympanogram measured between 375 Hz and 2000 Hz showed a significant difference in ambient pressure only in the otosclerosis group. In comparison to healthy individuals (901 Hz), ossicular chain discontinuity showed a significant reduction in resonance frequency (674 Hz), whereas in cases with otosclerosis had higher resonance frequency (1445 Hz). CONCLUSIONS AND SIGNIFICANCE: The present study showed different WBA patterns between the groups and the absorbance values were significantly different at the low frequencies. This suggests that WBA has the potential to differentiate ossicles related pathologies from normal and also between the ear with otosclerosis and ossicular chain discontinuity.

The usefulness of wideband absorbance in the diagnosis of otosclerosis.

Objective: To compare wideband absorbance (WBA) patterns between ears with otosclerosis and normal hearing ears and to investigate if WBA findings could be useful in the diagnosis of otosclerosis.Design: WBA was obtained at 107 frequency samples ranging from 0.226 to 8 kHz (24 per octave). A T-test was performed to compare between WBA in ears with otosclerosis and in normal hearing ears. The ability of WBA to discriminate between the patients with otosclerosis from the normal hearing participants was tested with a receiver operating characteristics (ROC) curve analysis.Study sample: Thirty-five patients with otosclerosis (age 31-64) and thirty-five normal hearing volunteers (age 32-64).Results: In frequency range 0.432-1.059 kHz, mean WBA in otosclerosis was significantly lower than mean WBA in normal hearing ears and in frequency range 4.238-8 kHz mean WBA in otosclerosis was significantly higher than mean WBA in normal hearing ears. The ROC analysis revealed that ears with otosclerosis and normal hearing ears could be distinguished based on mean WBA in frequency range >0.5 ≤ 1 kHz (AUC = 0.673) and based on mean WBA in frequency range >4 ≤ 8 kHz (AUC = 0.769).Conclusion: Our results suggest that WBA findings in ears with otosclerosis differ from WBA findings in normal hearing ears.

Osteoprotegerin gene polymorphisms and otosclerosis: an additional genetic association study, multilocus interaction and meta-analysis.

BACKGROUND: Otosclerosis (OTSC) is among the most common causes of a late-onset hearing loss in adults and is characterized by an abnormal bone growth in the otic capsule. Alteration in the osteoprotegerin (OPG) expression has been suggested in the implication of OTSC pathogenesis. METHODS: A case-control association study of rs2228568, rs7844539, rs3102734 and rs2073618 single nucleotide polymorphisms (SNPs) in the OPG gene was performed in a Tunisian-North African population composed of 183 unrelated OTSC patients and 177 healthy subjects. In addition, a multilocus association and a meta-analysis of existing studies were conducted. RESULTS: Rs3102734 (p = 0.013) and rs2073618 (p = 0.007) were significantly associated with OTSC, which were predominantly detected in females after multiple corrections. Among the OPG studied SNPs, the haplotypes A-A-C-G (p = 0.0001) and A-A-C-C (p = 0.0004) were significantly associated with OTSC in females. Multilocus association revealed that the SNPs: rs2073618 in OPG, rs1800472 in TGFß1, rs39335, rs39350 and rs39374 in RELN, and rs494252 in chromosome 11 showed significant OTSC-associated alleles in Tunisian individuals. In addition, meta-analysis of the rs2073618 SNP in Tunisian, Indian and Italian populations revealed evidence of an association with OTSC (OR of 0.826, 95% CI [0.691-0.987], p = 0.035). CONCLUSIONS: Our findings suggest that rs3102734 and rs2073618 variants are associated with OTSC in North African ethnic Tunisian population. Meta-analysis of the rs2073618 in three different ethnic population groups indicated an association with OTSC.

Availability of audiological evaluation for the differential diagnosis of clinical otosclerosis.

OBJECTIVE: The availability of an audiological evaluation for the differential diagnosis of clinical otosclerosis (OS) was examined. METHODS: Included were 98 patients (107 ears) with OS and 19 patients (20 ears) with incudostapedial disconnection (ISD) diagnosed by surgery between 2009 and 2017 at Aichi University Hospital. Results of preoperative pure-tone audiometry and impedance audiometry were analyzed. The most reliable index for distinguishing the two diseases was evaluated, and the cut-off value, sensitivity, and specificity were calculated. RESULTS: In the univariate analysis, age, static compliance, reversed ipsilateral acoustic reflex (reversed IAR) at 0.5 kHz and 2 kHz, negative contralateral acoustic reflex (negative CAR), difference between the air-bone gap at 0.25 and 2 kHz (0.25-2 kHzABG) and that at 0.25 and 4 kHz (0.25-4 kHzABG) showed statistical significance between the two diseases, whereas the Carhart notch did not. Multivariate logistic regression analysis by the variable selection method showed that reversed IAR (0.5 kHz), negative CAR, and 0.25-4 kHzABG were statistically significantly better factors for the differential diagnosis of the two diseases. The receiver operating characteristic curve for diagnosing OS using the difference between air-bone gap at 0.25 kHz and 4 kHz showed moderate accuracy. If the cut-off value of 0.25-4 kHzABG was 10 dB, then the sensitivity and specificity were 81.1% and 55.0%, respectively. In other words, if the cut-off value of 0.25-4 kHzABG was less than 10 dB, then the possibility of diagnosing ISD becomes higher, and if greater than 10 dB, then the possibility of diagnosing OS becomes higher. CONCLUSION: The differences in the air-bone gap at low and high frequency in pure-tone audiometry, static compliance, and acoustic reflex in impedance audiometry are useful for distinguishing OS from ISD. However, combinations of several examinations including computed tomography imaging are needed for the differential diagnosis of other ear diseases and specific preoperative planning.

Clinical aspects of otosclerotic ossicular chain fixations. / Az otoscleroticus eredetu hallócsontláncolati fixatiók klinikuma.

Otosclerosis is a human-specific ear disease characterised by complex bone-remodelling with multifactorial aetiology. It affects the bony labyrinth capsule and consequently fixates the stapes to the oval window rim. The fixation of the ossicular chain leads to a decrease in the middle ear's acoustic impedance-fitting and amplifier function that leads to conductive hearing loss. The process is progressive and when it involves the inner ear structures, it deteriorates the sensorineural function as well. The course of the illness can be prevented or delayed if hearing reconstructive surgery is performed on time. The development of stapes surgery spans the 20th century, and despite the major surgical steps - laid down by Shea and Marquet in the 1960s - are quite conservative, fine adjustments are still being made mainly due to technical progress. Several studies confirm that stapedotomy remained the first-to-offer therapeutic option in otosclerosis. With an adequate surgical technique, significant improvement can be achieved in the air conduction threshold, the air-bone gap may be minimalized or ceased over the speech frequencies, which significantly improves the quality of life of the patients. In this quest, we reviewed the Hungarian and the international literature as well in context with otosclerosis, with special attention to the newest methods in diagnostics and treatment management. Orv Hetil. 2020; 161(19): 780-788.

The Sensitivity of the cVEMP Test in Detecting A Superior Semicircular Canal Dehiscence and the Influence of a Coexisting Incudal Lysis: A Case Report.

In this case report, the air-conducted cervical vestibular evoked myogenic potentials (AC cVEMP) test was only sensitive for the left superior semicircular canal dehiscence (SCD), even though the contralateral SCD was of equal length (2.5 mm). Furthermore, a lysis of the processus lenticularis incudis caused a real conductive hearing loss in the left ear. A diminished left AC cVEMP was thus expected, but the opposite was shown (increased corrected amplitude, lowered detection threshold). The patient only experienced hearing loss, so middle ear surgery was performed to repair the lysis. The postoperative AC cVEMP showed a further "uncovering" of the SCD with increased corrected amplitude on the left but no vestibular symptoms. The significance of an SCD should be interpreted with caution, even when the AC cVEMP and the imaging are significant. Furthermore, AC cVEMPs should not be considered as evidence for the absence or presence of conductive hearing loss.

GPER-1 and sex-hormone levels in patients with otosclerosis.

OBJECTIVE: Otosclerosis is a widespread disease but the etiopathogenesis is still not fully understood. Hormonal factors especially estrogens are accused in recent years. The study aimed to evaluate the levels of G-protein associated membrane estrogen receptor-1 (GPER-1) and sex-hormones in patients with otosclerosis. SUBJECT AND METHODS: The study included 60 people (30 otosclerosis patients, 30 control group). Serum sex-hormone (estradiol, progesterone, prolactin and total testosterone) and GPER-1 levels were measured in otosclerosis patients and compared with the normal population. For the otosclerosis group, air conduction and bone conduction thresholds and air-bone gaps were viewed from audiograms and the relationships between hearing and GPER-1 or sex-hormone levels were also investigated. RESULTS: Sex-hormone levels were not different between the groups. GPER-1 level was significantly lower in the otosclerosis group [3.1353 (0.76-8.21) ng/mL] than the control group [5.4773 (0.96-20.31) ng/mL] (p =0.017). Differential diagnosis with ROC analysis for the GPER-1 level was also significant (p=0.017). GPER-1 level was significantly lower for the females than the males in the otosclerosis group (p=0.043). Serum estradiol, progesterone, and prolactin levels were significantly higher (p=0.02, p =0.029 and p=0.019 respectively) and the GPER-1 level was significantly lower (p= 0.04) in the female patients compared to the female controls. There was no statistically significant relationship between GPER-1 or sex-hormone levels and hearing parameters. CONCLUSION: GPER-1 level was lower in the otosclerosis patients compared to healthy volunteers and also lower in females than males in the patient group. Female sex-hormone levels were higher and GPER-1 level was lower in the female patient group than the female control group. Neither GPER-1 nor sex-hormone levels were not predictive of hearing levels. These findings indicate that sex-hormones especially estrogen and GPER-1 might have a potential role in the etiopathogenesis of otosclerosis. This is the first study in the literature that investigates the GPER-1 values in otosclerosis.