OBJECTIVE: To determine differences in failure rate and hearing outcomes of a completely encircling heat-activated crimping prosthesis (SMart 360°) compared to partially encircling prosthesis (SMart).
METHODS: Retrospective chart review.
METHODS: Private neurotology tertiary referral center.
METHODS: Patients who underwent stapedotomies performed by the senior authors from 2008 to 2019 using the SMart prosthesis and SMart 360° prothesis.
METHODS: Stapedotomy operations with placement of a SMart or SMart 360° prosthesis.
METHODS: Incidence of early failure requiring revision surgery. Differences in preoperative air-bone gap (ABG) compared to postoperative ABG at 3 months, 1 year, and 2 years after surgery.
RESULTS: A total of 228 stapedotomies were performed (SMart n = 48 and SMart 360° n = 180). Mean preoperative ABG for SMart and SMart 360° were 26.15 and 29 dB, respectively. The mean difference in ABG for the SMart at 3 months, 1 year, and 2 years were 17, 18, and 11 dB, respectively. The mean difference in ABG for the SMart 360° at 3 months, 1 year, and 2 years were 20, 20, and 19 dB. ABG differences at 3 months (p = 0.10) and 1 year (p = 0.36) were not statistically different. The failure rate for the SMart prosthesis was 12.5% and for the SMart 360° 2.2% (p = 0.002).
CONCLUSIONS: There were no statistically significant differences in ABG changes for SMart compared to SMart 360°. The Smart 360 corrects the problem with early failure seen with the Smart prosthesis.
UNASSIGNED: Determination of most efficacious stapes prosthesis.
OBJECTIVE: Which stapes prosthesis produces better hearing results with fewer failures.
UNASSIGNED: To disseminate information necessary to choose the best stapes prosthesis for patients.
METHODS: Level III.
UNASSIGNED: 2022-029-agh.

Otosclerosis is a complex skeletal condition that originates from both hereditary and environmental factors. Gradual conductive hearing loss is the main character. Aim to contrast and assess the postoperative results, such as hearing improvement, following the use of bone cement to fix the Titanium versus Teflon stapes piston. 50 patients who had endoscopic stapedotomy with different prostheses insertions were included in this prospective, randomized research. They were splitted into two groups in the: group A consisted of 25 ears in which titanium prostheses with bone cement were applied, while group B consisted of 25 ears Teflon prostheses with bone cement were used. A statistically significant difference was observed in average air conduction (better hearing) between groups A and B at 3, 6, and 12 months postoperatively based on audiometric results. When comparing groups B and A after 3, 6, and 12 months after surgery, the average air bone gap (ABG) was greater in group B, and this difference was statistically significant. Between the two groups, there was no discernible variation in any of the preoperative metrics. 96% of groups A and B were successful. Using bone cement in primary stapedotomy may help fix the procedure and reduce the chance of persistent hearing loss in patients with otosclerosis. This is especially true when titanium is used, as titanium has the ideal mass and stiffness to support acoustic transmission with a low rate of adverse effects and better average ABG.

Az otosclerosis egy máig tisztázatlan eredetű csontanyagcsere-betegség, amely csakis humán temporalis csontban alakít ki apró csontátépüléssel járó gócokat. Ezek az elváltozások a leggyakrabban az ovális ablakfülke elülső részén és a stapestalpban alakulnak ki, és mechanikailag fixálják a hallócsontláncolatot, következményes vezetéses halláscsökkenést okozva ezzel. Az otosclerosis kezelésével kapcsolatban természetes igény, hogy különféle gyógyszeres terápiás lehetőségekkel próbáljuk visszafordítani a betegséget, ez idáig azonban csupán a csontátépülést sikerült lelassítani. A nátrium-fluorid- és a biszfoszfonátkezelés igen ígéretesnek mutatkozott, súlyos mellékhatásaik miatt azonban alkalmazásuk csak kivételes esetekben jöhet szóba. A célzott (biológiai) terápiákhoz nagy reményt fűznek, ezeket azonban Magyarországon még nem engedélyezték. Az otosclerosis terápiájának „gold standard” eljárása a sebészi megoldás, mely nem a betegséget gyógyítja meg, pusztán a halláscsökkenés vezetéses komponensét küszöböli ki. A régebbi műtéti technikát, a stapedectomiát egyre inkább felváltja a technikailag egyszerűbb stapedotomia, ám mindmáig szinte ekvivalens eljárásnak tekinti őket a szakirodalom. A szokványos mikroszkópos stapessebészet mellett külön bemutatjuk a legmodernebb endoszkópos stapedotomiát is. Orv Hetil. 2024; 165(32): 1237–1241.

UNASSIGNED: HE4 and CA 125 are identified as a potential biomarker for the detection of some diseases with fibrosis.
UNASSIGNED: The purpose of this pilot study was to evaluate the value of human epididymis protein 4 (HE4) and cancer antigen-125 (CA-125) in otosclerosis patients.
UNASSIGNED: The study population consisted of 60 people (30 otosclerosis patients, 30 control group). We collected blood samples for HE4 and CA-125 levels. Serum HE4 and CA-125 levels were measured by enzyme-linked immunosorbent assay (ELISA). We compared the results between otosclerosis patients and the normal subject. The area under the curve (AUC) of the receiver operating characteristic (ROC) curve was performed to evaluate the diagnostic value.
UNASSIGNED: There was no differences in CA-125 level between the otosclerosis (20.3 U/mL [10.4-42.1] and control group (19.3 U/mL [15.3-49.8]) (p > 0.05). HE4 level was significantly higher in the otosclerosis group (60.9 pmol/L [32.1-101.8])] than the control group (25.3 pmol/L [12.4-91.9]) (p < 0.001). The AUC in ROC analysis of HE4 was 0.768 (p < 0.001).
UNASSIGNED: Serum HE4 level may be a useful biomarker in otosclerosis. Further studies with a larger number of patients are required to confirm our pilot results.

OBJECTIVE: Obliteration of the round window (RW) in cases of otosclerosis presents a significant clinical challenge due to its association with more severe hearing loss and a poorer prognosis for functional recovery after stapes surgery. The objective is to assess and characterize the occurrence of RW involvement in otosclerosis cases and to identify patterns of disease progression that may indicate a potential for RW obliteration.
METHODS: We selected archival temporal bones from donors with otosclerosis. We evaluated the degree of RW obliteration using a semi-quantitative scale and the location of the foci within the temporal bone, and whether the foci were continuous or isolated.
RESULTS: Most of the foci were located anteriorly to the oval window (89.2%), while RW area involvement was seen in 26.9% of the ears. In cases with fenestral foci, 68.1% directly involved and/or fixed the footplate. Among donors with bilateral otosclerosis, foci affected both ears in a similar pattern in 64.2%. Among donors with RW involvement, ones with continuous, large lesions that extended from the oval window associated with complete RW obliteration, while ones with smaller degrees of obliteration had solitary foci scattered within the otic capsule.
CONCLUSIONS: Our results demonstrate a high rate of RW involvement in cases of otosclerosis. Ears with continuous lesions extending from the oval window region to the RW area were more likely to present with complete RW obliteration. These results provide insights that could lead to better prognostic assessment of patients with otosclerosis in the future.
METHODS: N/A Laryngoscope, 2024.

BACKGROUND: Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air-bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors\' studies and seems to be related to a third mobile window (TMW) phenomenon. This can lead to misdiagnosis and inappropriate treatment. Given that it would be inappropriate and harmful to perform CT scans in all patients with a clinical diagnosis of otosclerosis, this study aims to highlight some clinical features useful for the differential diagnosis between otosclerosis and these rare cases of EVA presenting with an ABG, thus enabling the identification of suspected cases to be tested with CT scans.
METHODS: Between April and May 2024, a narrative review was conducted focusing on the differential diagnosis between some rare cases of EVA and otosclerosis. Clinical, audiological, and radiologic features of both conditions were investigated.
RESULTS: This review demonstrates the diagnostic challenge in differentiating atypical cases of EVA from otosclerosis in a subset of patients. Clinical and audiological features are important for differential diagnosis, but may not always be sufficient. Therefore, high-resolution computed tomography (HRCT) of the temporal bone plays a pivotal role in definitive diagnosis.
CONCLUSIONS: In some specific cases, pre-operative imaging assessment using HRCT emerges as an essential tool for differentiating these two conditions and avoiding unnecessary stapes surgery.

UNASSIGNED: Otosclerosis (OTSC) is one of the most common causes of progressive adult-onset hearing loss in the Caucasian population, with a female preponderance. The etiology of OTSC is complex and there are a number of genetic variants reported to be associated with OTSC susceptibility, but no data on the genetic background of OTSC in patients originating from the central-eastern part of Europe have been available. The purpose of our study was to investigate in Polish patients the frequency of genetic variants previously reported to be most strongly associated with OTSC.
UNASSIGNED: Genomic DNA was isolated from blood samples or buccal swabs. Variants in TGFB1 (rs1800472) and RELN (rs39335, rs39350, rs39374) were genotyped in surgically confirmed OTSC patients (n = 94) and a control group (n = 198) using custom TaqMan SNP genotyping assays and real-time PCR. Allele and genotype frequencies were compared between the groups in statistical analysis and the odds ratios with 95% confidence intervals were calculated to estimate the risk.
UNASSIGNED: For all of the tested variants the distributions of alleles and genotypes were not statistically significantly different between OTCS patients and the control group. There were also no statistically significant differences in relation to gender of the tested subjects.
UNASSIGNED: Despite multiple confirmatory studies on TGFB1 and RELN association with OTSC development in some populations, no significant association between the studied variants and OTSC was found in Polish patients. Our results indicate the presence of inter-population differences in OTSC susceptibility factors and confirm the large genetic heterogeneity of this disorder.

OBJECTIVE: This study aimed to conduct a comparative analysis of audiological and postoperative clinical outcomes between the endoscopic and microscopic stapedotomy approaches.
METHODS: This study employed a randomized, controlled design. Twenty-seven patients with bilateral otosclerosis underwent stapedotomy in both ears, with randomized allocation of the surgical technique (endoscopic vs. microscopic) for the first operated ear. Air-bone gap (ABG) and ABG gain were measured at least 12 months postoperatively. Postoperative outcomes including pain (Visual Analog Scale-VAS), dizziness, early-term (day 1) and late-term (6 months) dysgeusia were evaluated. The Glasgow Benefit Inventory (GBI) assessed health-related quality of life at one month postoperatively, and operative time was measured.
RESULTS: This study compared endoscopic (n = 27 ears) and microscopic (n = 27 ears) stapedotomy for otosclerosis. Both groups achieved similar hearing improvement with no significant differences in pre-operative and post-operative bone/air conduction thresholds, ABG, and ABG gain (all p > 0.05). The endoscopic stapedotomy group demonstrated reduced postoperative pain (lower VAS scores, p < 0.001), lower early dysgeusia (3.7% vs. 33.3%, p = 0.005), shorter operative time (47.3 vs. 75.4 min, p < 0.001) and improved patient-reported outcomes (higher GBI score, p = 0.014) when compared to microscopic stapedotomy group. No significant differences were observed in postoperative dizziness or late-term dysgeusia between groups.
CONCLUSIONS: This study found similar hearing improvement with both endoscopic and microscopic stapedotomy for otosclerosis. However, the endoscopic approach showed advantages in reduced postoperative pain, early dysgeusia, and operative time, with improved patient-reported quality of life. These findings suggest endoscopic stapedotomy as a valuable alternative to the conventional microscopic approach.

The gold standard for otosclerosis diagnosis, aside from surgery, is high-resolution temporal bone computed tomography (TBCT), but it can be compromised by the small size of the lesions. Many artificial intelligence (AI) algorithms exist, but they are not yet used in daily practice for otosclerosis diagnosis. The aim was to evaluate the diagnostic performance of AI in the detection of otosclerosis. This case-control study included patients with otosclerosis surgically confirmed (2010-2020) and control patients who underwent TBCT and for whom radiological data were available. The AI algorithm interpreted the TBCT to assign a positive or negative diagnosis of otosclerosis. A double-blind reading was then performed by two trained radiologists, and the diagnostic performances were compared according to the best combination of sensitivity and specificity (Youden index). A total of 274 TBCT were included (174 TBCT cases and 100 TBCT controls). For the AI algorithm, the best combination of sensitivity and specificity was 79% and 98%, with an ideal diagnostic probability value estimated by the Youden index at 59%. For radiological analysis, sensitivity was 84% and specificity 98%. The diagnostic performance of the AI algorithm was comparable to that of a trained radiologist, although the sensitivity at the estimated ideal threshold was lower.

Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.