Stapes Fixation Research Articles

Comparison of Uncomfortable Loudness Level in Stapedotomy with or without Stapedial Tendon Preservation

Background: Otosclerosis is one of the commonest diseases of the ear mostly involves the otic capsule. Most often otosclerotic foci appear in stapes region leading to stapes fixation, predominantly affect the adolescence female. The most common presenting symptom of clinical otosclerosis is conductive deafness. The mainstay of treatment for otosclerosis is surgery. Surgical options include stapedectomy, stapedotomy with or without stapedial tendon preservation. The latter being is the procedure of choice. Aim: The aim of this study is to compare the outcome of uncomfortable loudness level in stapedotomy with or without stapedial tendon preservation. Methods: A prospective observational study was conducted in the Department of Otolaryngology-Head & Neck Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka for 18 months in patients with otosclerosis. Total 30 subjects were selected based on the inclusion and exclusion criteria. All patients were assessed pre-operatively by clinical examination, otoscopy and microscopic examination. Hearing was assessed by pure tone audiometry. Uncomfortable level and stapedial reflex threshold were tested in all cases. The selected cases were placed into two groups. Stapedial tendon resection in Group-I and stapedial tendon preservation in Group-II. Post-operative follow up was done at 3 months and 6 months. Hearing and uncomfortable loudness level were evaluated with PTA during follow up by calculating the average of 500Hz, 1000Hz, 2000Hz and 4000HZ. The data were calculated manually. The statistical significance was set to P< 0.05. Results of the study were expressed as mean, standard deviation (± SD), frequency and percentages. Means and standard deviations were reported for continuous variables. Frequencies and percentages were reported for categorical variables. Unpaired Student’s t test was done to compare the continuous variables and Chi Square test was done to compare the categorical variables. Results: In this study preoperative average ABG for group I and group II were 35 ± 4.57 dB and 34 ± 4.17 dB respectively. In group I, post operative average ABG after 3 months and 6 months were 14 ± 3.7 dB and 13±3.3 dB respectively. Post operative average ABG after 3 months was 13 ± 5.7 dB and was 12 ± 4.4 dB for group II. But the hearing improvement between two groups was not statistically significant. In case of preoperative mean UCL was 95 ± 1.8 dB and 96 ± 2.5 dB for group I and group II respectively. Postoperative mean UCL after 3 months was 96 ± 3.57 dB and after 6 months was 99 ± 6.28 dB in group I. For group II, postoperative mean UCL after 3 months and 6 months was 107±4.2 dB and 113 ± 3.2 dB respectively. Here mean UCL was on average 11 dB higher for group II in 3 months and additional 6 dB improvement noted after 6 months, but show minimal change in group I. This finding was statistically significant. Conclusion: Preservation of the stapedial tendon is the choice in the surgical treatment of otosclerosis which helps to improve functional outcomes as well as to provide the more physiologic protection of middle ear. Postoperative discomfort threshold levels were increased in patients who had their stapedial tendon restored. Bangladesh J Otorhinolaryngol 2021; 27(2): 117-123

Radiological and Audiological Prediction for Ossicular Fixation in Chronic Otitis Media and Tympanic Membrane Perforation.

Recurrent middle-ear infection can lead to ossicular fixation, adversely affecting post-tympanoplasty hearing outcomes. Preoperative prediction of ossicular fixation remains challenging. We aimed to investigate potential predictors of ossicular fixation in patients with chronic otitis media. Retrospective. Tertiary academic medical center. Patients with noncholesteatomatous chronic otitis media and tympanic membrane perforation, without ossicular discontinuities. Diagnostic. The fixation of each ossicle was assessed during tympanoplasty. The impact of preoperative otoscopic findings, computed tomography (CT) features, and hearing levels on the prediction of ossicular fixation was evaluated using uni- and multivariable logistic regression analyses. One hundred thirty-five patients were included. Soft-tissue density between the malleus head and the anterior wall (odds ratio, 3.789 [95% confidence interval, 1.177-12.196]; p = 0.0255) and poor development of mastoid cells (16.826 [2.015-134.520]; p = 0.0078) were independent predictors of malleus fixation. In addition, ≥50% tympanic membrane perforation (5.412 [1.908-15.353]; p = 0.0015), poor development of mastoid cells (3.386 [1.039-11.034]; p = 0.0431), and a ≥40-dB preoperative air-bone gap (ABG) at 500 Hz (4.970 [1.732-14.261]; p = 0.0029) were independent predictors of incus fixation. Soft-tissue density surrounding the stapes (18.833 [1.856-191.104]; p = 0.0119) and a ≥40-dB preoperative ABG at 500 Hz (13.452 [1.640-∞]; p = 0.0138) were correlated with stapes fixation. The accurate prediction of ossicular fixation in patients with chronic otitis media based on CT features and the ABG may facilitate decision-making regarding the need for ossiculoplasty, possibly avoiding unnecessary manipulation or overlooking of fixation.

고식적 등골수술이 불가능한 등골고정 환자에서 등골절제 후 이소골성형술을 시행한 2예

Stapedial fixation result in conductive hearing loss, and the disrupted sound conduction mechanism could be restored with stapes surgery. Conventional stapes surgery cannot be performed in patients with disrupted ossicular chain, especially absence of incus and malleus. In this study, we reported two cases of stapedectomy with ossiculoplasty in patients with stapedial fixation and disrupted ossicular chain. Stapedectomy was performed in two patients and the oval window was covered with perichondrium or temporalis fascia. Total ossicular replacement prosthesis was inserted between tympanic membrane and oval window. All of patients showed improvement of hearing and subjective satisfaction for outcome. The postoperative air-bone gap was 8.3 and 26.7 dB. Postoperatively, no complication such as sensorineural hearing loss or dizziness was reported. Therefore, stapedectomy with ossiculoplasty is safe and effective treatment option for patients with conductive hearing loss due to stapedial fixation and disruption of ossicular chain. (J Clinical Otolaryngol 2021;32:143-148)

Effect of malleostapedotomy procedure on 17 cases of congenital middle ear malformation

Objective: To conclude the clinical features and the postoperative efficacy of congenital middle ear malformation treated with Malleostapedotomy (MS), and to explore the security and effectiveness of MS surgery. Methods: The clinical data of 17 patients (18 ears) with congenital middle ear malformation undergoing MS procedure were analyzed. There were 10 males (11 ears) and 7 females (7 ears), aged from 7 to 48 years. The imaging examination, pure-tone audiometry, intraoperative findings and postoperative hearing improvement of these patients were analyzed and summarized, and software SPSS23.0 was used for statistical analysis. Rusults All the 17 patients (18 ears) presented with hearing loss since childhood on the affected sides. Preoperative high resolution CT (HRCT) of the temporal bone revealed definite malformations in 9 ears (6 ears with incus long process dysplasia and 3 ears with anterior and posterior crus dysplasia). Before surgery, the mean bone conductive hearing threshold at 500, 1 000, 2 000 and 4 000 Hz was (15.6±10.2) dB HL, the mean air conductive hearing threshold was (60.6±9.7) dB HL, and the mean air-bone gap was (45.0±8.9) dB. During the surgery, all 18 ears were found to be accompanied by absence or hypoplasia of incus long process. 12 ears had stapes fixation, 6 ears had oval window atresia. All patients were treated with MS procedure by using Piston. The patients were followed up for 3 months to 1 year. The mean bone conductive hearing threshold was (14.7±8.8) dB HL. The mean air conductive hearing threshold was (37.7±11.6) dB HL, and the mean air-bone gap was (23.0±8.0) dB. There were statistically significant differences in the mean air conductive hearing threshold and mean air-bone gap before and after surgery (P<0.05). While there were no statistically significant differences in the mean bone conductive hearing threshold before and after surgery (P=0.550). Conclusions: MS procedure is safe and reliable in patients with congenital middle ear malformation of incus long process dysplasia, stapes fixation or oval window atresia. HRCT is useful in evaluating the major deformity of ossicular chain and facial nerve deformity. However, it is not enough to evaluate the joint of incus-stapes and oval window atresia. MS surgery in middle ear malformation requires advanced surgical experience and skills. The hearing improvement can be significant, even though some air-bone gap after surgery exist.

Stapedotomy in Congenital Fixation with Cochlear Hypoplasia: A New Concept Among the Treatment Options for Cochlear Hypoplasia.

Objectives:To introduce the concept of stapedotomy as a new treatment alternative in cochlear hypoplasia (CH) and propose a new guideline for its management.Methods:Forty-two primary cases out of 355 presented with congenital stapes fixation between January 2003 and September 2015 were included in the study. Computed tomography scans of all cases with congenital stapes fixation were reviewed, and cases with inner ear anomalies were taken into account. Eleven cases had various inner ear anomalies, and 9 cases had various types of CH. In the present paper, only the CH cases with stapes fixation, all of whom underwent stapedotomy, are reviewed regarding preoperative audiological and radiological characteristics as well as surgical findings and postoperative audiological results.Results:The patients were aged between 4 and 22. There were 2 males (3 ears) and 4 females (6 ears). Three cases had bilateral stapedotomy. The remaining 3 cases had unilateral surgery. The average preoperative air–bone gap (ABG) was 50.3 dB. Postoperative hearing: preoperative ABG was 50.3 dB. Postoperative ABG was calculated as 20.1 dB hearing.Conclusion: Hearing loss (HL) in hypoplastic cochlea demonstrates the full spectrum of HL types. CH is a unique inner ear anomaly that can be treated with all of the available rehabilitation modalities. As a result of current findings, a new treatment algorithm for CH is proposed.KEYWORDS: Congenital stapes fixation, cochlear hypoplasia, congenital hearing loss, inner ear malformations, stapedotomy

Reliability of high-resolution CT scan in diagnosis of ossicular tympanosclerosis.

ObjectivesTo evaluate the reliability of high‐resolution CT scans (HRCT scans) in the diagnosis of tympanosclerosis and to determine its benefit to predict the post‐surgical hearing outcome based on comparing radiological and surgical findings.MethodsA retrospective study at a tertiary institute included 940 ears that underwent tyampanoplasty for chronic suppurative otitis media (CSOM) between January 2013 and March 2017. Preoperative temporal bone HRCT scans were analyzed to check for the prediction of tympanosclerosis and ossicular fixation. Intraoperatively, ossicular chain integrity was checked. Preoperative and postoperative audiometric evaluations using air‐bone gap (ABG) were compared. A postoperative pure‐tone ABG of 20 dB or less was considered as a successful hearing result. Results are compared with historical control groups, the study has been reviewed and approved by the IRB at the medical research center in Hamad Medical Corporation; however, it is a retrospective study so no informed consent was obtained from the patients.ResultsThe study included 940 ears that underwent tympanoplasties due to CSOM, where 238 out of 940 (25.3%) of ears showed tympanosclerosis during tympanoplasty, intraoperatively, tympanosclerosis was localized to the eardrum in 174 of the 238 involved ears (73.1%), A 64 out of 238 (26.9%) of the ears with tympanosclerosis showed ossicular fixation, divided as 45 ears with Incudo‐malleal fixation, 14 ears with stapes fixation, and 5 ears with triple ossicular fixation. HRCT scan of the temporal bone was suggestive of ossicular chain fixation in 79 cases distributed as 55 incudo‐malleal fixations, 19 stapes fixation, and 5 complete ossicular fixations, with a sensitivity of 96.8%, and specificity of 98%. The audiological results were analyzed, with a patient follow‐up after 6 months.ConclusionsOur study showed that CT scans when combined with the clinical findings can be an informative guide to otolaryngologists for preoperative evaluation and counseling of tympanosclerosis surgeries.

Comparison of hearing reconstruction techniques in tympanosclerosis with stapes fixation

Background/Aim: Surgery for tympanosclerosis with stapes fixation for hearing reconstruction is controversial, with many reports in the relevant literature advocate opposing suggestions. The primary aims were to evaluate hearing improvement with varied hearing reconstruction techniques performed for tympanosclerosis with stapes fixation. Methods: Patients with tympanosclerosis and stapes fixation, whose hearing impairment was re-constructed surgically and who were followed for 1-5 years were reviewed in this retrospective cohort study. The audiological outcomes of reconstruction methods, including mobilization, partial ossicular replacement prosthesis (PORP), total ossicular replacement prosthesis (TORP), and teflon pistons (TP) were documented. Results: The study included 76 ears of 76 patients; 29 ears in the mobilization technique group, 28 ears in the PORP technique group, 10 ears in the TORP technique group, and 9 ears in the TP technique group. When postoperative and preoperative hearing were evaluated, the mobilization and PORP techniques showed statistically significant improvement in both air conduction threshold gain and air-bone gap closure (P0.05). Bone conduction threshold showed no significant deterioration in any techniques. Conclusion: For hearing gain, mobilization and PORP were effective in tympanosclerosis patients with stapes fixation after removal of sclerotic plaques, whereas the TORP and TP techniques did not show significant improvement. It can be concluded that, if a functionally mobile stapes cannot be achieved, it is not advisable to perform TORP replacement or a stapedotomy. However, these methods may still be used in carefully selected patients and be successful in some.

Hearing Outcomes of Stapes Surgery in Children With Stapes Fixation and Ossicular Anomalies.

To investigate hearing outcomes after stapes surgery in children with stapes fixation. Retrospective study. Tertiary referral center. Forty-nine patients (66 ears) aged less than 15 years who received stapes surgery. Stapes surgery. Preoperative symptoms, bilateral involvement, pure-tone hearing levels, and perioperative complications were analyzed using paired t test and Mann-Whitney U test. The chief complaint of all patients was hearing disturbance. Sixteen ears were diagnosed with stapes fixation and an additional congenital ossicular anomaly and 50 ears had only stapes fixation. Preoperative mean bone conduction and air conduction thresholds were 12.0 ± 5.8 dB and 60.9 ± 10.9 dB, respectively. The mean air-bone gap (ABG) was 48.9 ± 12.0 dB in patients with stapes fixation and an ossicular anomaly. The postoperative mean ABG was 23.6 ± 14.5 dB, and the ABG closure was 25.3 ± 18.2 dB. In patients with stapes fixation only, the preoperative mean bone conduction and air conduction thresholds were 14.3 ± 7.5 dB and 49.6 ± 9.5 dB, respectively, and the mean ABG was 35.5 ± 9.6 dB. The postoperative mean ABG was 14.4 ± 10.3 dB, and the ABG closure was 16.2 ± 16.1 dB. The successful results (ABG <20 dB) were 75.8% overall, 56.3% for fixation and an ossicular anomaly, and 82.0% for fixation only. In children with stapes fixation, hearing loss was worse when the fixation was combined with an ossicular anomaly. Ossicular continuity, especially of the incus, is the most important factor for successful stapes surgery. Appropriate diagnosis and surgical intervention can lead to good results for children with stapes fixation.

Numerical study on the effect of middle ear malformations on energy absorbance

In order to study the effect of middle ear malformations on energy absorbance, we constructed a mechanical model that can simulate the energy absorbance of the human ear based on our previous human ear finite element model. The validation of this model was confirmed by two sets of experimental data. Based on this model, three common types of middle ear malformations, i. e. incudostapedial joint defect, incus fixation and malleus fixation, and stapes fixation, were simulated by changing the structure and material properties of the corresponding tissue. Then, the effect of these three common types of middle ear malformations on energy absorbance was investigated by comparing the corresponding energy absorbance. The results showed that the incudostapedial joint defect significantly increased the energy absorbance near 1 000 Hz. The incus fixation and malleus fixation dramatically reduced the energy absorbance in the low frequency, which made the energy absorbance less than 10% at frequencies lower than 1 000 Hz. At the same time, the peak of energy absorbance shifted to the higher frequency. These two kinds of middle ear malformations had obvious characteristics in the wideband acoustic immittance test. In contrast, the stapes fixation only reduced the energy absorbance in the low frequency and increased energy absorbance in the middle frequency slightly, which had no obvious characteristic in the wideband acoustic immittance test. These results provide a theoretical reference for the wideband acoustic immittance diagnosis of middle ear malformations in clinic.

Systematic Review and Meta-analysis of Endoscopic vs Microscopic Stapes Surgery for Stapes Fixation.

To systematically review the current literature regarding the operative outcomes of stapes surgery for stapes fixation via the endoscopic and microscopic approaches. PubMed, Embase, and Web of Science. An electronic search was conducted with the keywords "endoscop* or microscop*" and "stapes surgery or stapedectomy or stapedotomy or otosclerosis or stapes fixation." Studies were included if they compared endoscopy with microscopy for stapes surgery performed for stapes fixation and evaluated hearing outcomes and postoperative complications. Articles focusing on stapes surgery other than for stapes fixation were excluded. The database search yielded 1317 studies; 12 remained after dual-investigator screening for quantitative analysis. The mean MINORS score was 18 of 24, indicating a low risk of bias. A meta-analysis demonstrated no statistically significant difference between the groups with regard to operative time, chorda tympani nerve manipulation or sacrifice, or postoperative vertigo. There was a 2.6-dB mean improvement in the change in air-bone gap in favor of endoscopic stapes surgery and a 15.2% increased incidence in postoperative dysgeusia in the microscopic group, but the studies are heterogeneous. Endoscopic stapes surgery appears to be a reasonable alternative to microscopic stapes surgery, with similar operative times, complications, and hearing outcomes. Superior visibility with the endoscope was consistently reported in all the studies. Future studies should have standardized methods of reporting visibility, hearing outcomes, and postoperative complications to truly establish if endoscopic stapes surgery is equivalent or superior to microscopic stapes surgery.

Stapes surgery in osteogenesis imperfecta: retrospective analysis of 18 operated ears.

To evaluate short and long term results of stapes surgery in patients with osteogenesis imperfecta (OI), METHODS: Retrospective case series of 18 primary stapes surgeries performed on 11 hearing-impaired OI patients with evidence of stapes fixation, in a Tertiary referral center. We analysed pre-operative and post-operative hearing results at 1month and at least 1year RESULTS: The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10dB in 46% of the cases and to within 15dB in 92% of the cases at 1-year follow-up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4kHz) was 18.4dB at 1month and 22.4dB at 1year. Stapes surgery in OI gives good results with few complications in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease.

When to Say When-Reasons to Abort Surgery for Congenital Aural Atresia.

To determine reasons for premature termination in surgery for congenital aural atresia (CAA). Retrospective chart review. High-volume tertiary care center. Patients undergoing primary surgical repair of CAA whose surgeries were aborted for various indications. Patients with CAA who underwent elective aural atresia surgery at the University of Virginia from July 2004 to January 2020 were identified from the medical record and an institutional database of patients with CAA. Patients undergoing revision surgery or surgery not for hearing correction were excluded. All included candidates were noted to be good surgical candidates for atresia repair based on preoperative Jahrsdoerfer score of 6 or greater. Reasons for terminating surgery prematurely were categorized by anatomical constraints (inability to identify middle ear space, position of the facial nerve, or other anatomic abnormality), stapes fixation, and middle ear and/or mastoid inflammatory disease. Reasons for premature termination in CAA surgery. Over the interval, 319 patients underwent primary surgery for CAA. Surgery was terminated prematurely without completing the primary objective in 14 patients (4.4%); surgery was terminated in 8 (57%) due to anatomic constraints, 4 (29%) due to stapes fixation, and 2 (14%) due to middle ear space and/or mastoid inflammatory disease. Premature termination in surgery for CAA is a rare occurrence with careful study of the preoperative high-resolution computed tomography of the temporal bone. Of the reasons for premature termination, anatomic constraints were the most common. Knowing limitations in surgery-both technical and anatomic-is critical to optimize outcomes and prevent complications.

Prognostic factors for duration of vertigo after stapes surgery via a time-to-event analysis

Background Vertigo or dizziness after stapes surgery occurs sometimes, and it is generally temporary. However, while rare, it can be prolonged. Aims/Objectives To investigate the prognostic factors for duration of vertigo following stapedotomy using a time-to-event analysis. Materials and methods The present study included a total of 35 primary ears (26 with otosclerosis and nine with congenital stapes fixation) from 31 patients. We assessed residual rates of nystagmus and complaints of subjective vestibular symptoms using Kaplan–Meier time-to-event methods. Results Postoperative spontaneous nystagmus was observed in 23 (65.7%) ears, and postoperative subjective vestibular symptoms were confirmed in 27 (77.1%) ears. The total mean duration of postoperative spontaneous nystagmus and subjective vestibular symptoms was 9.8 (range: 0–158) and 33.9 days (0–732), respectively. A history of stapes surgery in the opposite ear was a significant predictive factor for prolonged nystagmus and subjective vestibular symptoms (p = .0059 and p = .0146). Conclusions and significance For individuals with a history of stapes surgery in the opposite ear, spontaneous nystagmus and vertigo/dizziness sensations following stapedotomy may persist for a longer duration than in those without a history of stapes surgery in the opposite ear.

Numerical analysis of the effects of ossicular chain malformations on bone conduction stimulation

To assess the effects of ossicular chain malformations on the performance of bone conduction hearing aids, a human ear finite-element model that includes an ear canal, a middle ear, and a spiral cochlea incorporating the third windows was established. This finite element model was built based on micro-computed tomography scanning and reverse modelling techniques, and the reliability of the finite element model was verified by comparison with reported experimental data. Based on this model, two main types of ossicular chain malformations, i.e., the incudostapedial disconnection and the ossicles fixation, were simulated, and their influences on bone conduction were analyzed by comparing the trans-cochlear-partition differential pressures. The results indicate that the incudostapedial disconnection mainly deteriorates the bone conduction response at mid frequencies. The stapes fixation has the largest effect among the ossicles fixation with the bone conduction stimulation, which also mainly decreases the mid-frequency response of the bone conduction, especially at 2 kHz. As the speech intelligibility has the most important frequency range at the range between 1 kHz and 2.5 kHz, the mid-frequency deterioration caused by ossicular chain malformations should be compensated in optimizing the design of the bone conduction hearing aids. For treating patients with the ossicular chain malformations, especially for the patients who suffer from the stapes fixation, the output of bone conduction hearing aids’ actuator in the middle frequency band should be improved.

Fifteen-Year Follow-Up of Stapedotomy Patients: Audiological Outcomes and Associated Factors in a Middle Income Country

Objective: To evaluate the short-term (postoperative), medium-term (5 years), and long-term (10 and 15 years) audiometric results of patients who underwent stapedotomy and to determine specific factors associated with better postoperative outcomes. Methods: This study is a retrospective case review of 486 ears with surgically confirmed stapes fixation who underwent microscopic small fenestra stapedotomy. Preoperative, postoperative, and medium- and long-term air conduction (AC), bone conduction (BC), and air-bone gap (ABG) were assessed. Postoperative factors associated with better postoperative outcomes were evaluated. Results: At 10- and 15-year follow-ups, ABG, AC, and BC were significantly deteriorated but clinically preserved in comparison with postoperative results. According to a multiple quantile regression, younger age was associated with better postoperative results at 0.25 kHz (p = 0.003) and 4 kHz (p = 0.028) and a smaller preoperative ABG was associated with better audiometric results at 0.25 kHz (p = 0.048), 0.5 kHz (p = 0.001), and 4 kHz (p = 0.001). In addition, younger age (p = 0.001 for AC and p < 0.001 for BC) and preoperative AC PTA (p < 0.001 for AC) were significantly associated with better postoperative AC and BC PTA. Conclusions: Stapedotomy surgery provides short-, medium-, and long-term hearing benefits in our studied cohort. ABG, AC, and BC thresholds obtained after the surgery are clinically preserved in 5-, 10-, and 15-year follow-ups, with an age-expected BC deterioration. Smaller preoperative ABG and younger age were positive predictors for better postoperative ABG. Future research should address long-term subjective and quality of life outcomes.

Development and Validation of a Clinical Score for Predicting the Risk of Tympanosclerosis Before Surgery.

To develop and validate a clinical score to predict the risk of tympanosclerosis before surgery. A sample of 404 patients who underwent middle ear microsurgery for otitis media was enrolled. These patients were randomly divided into 2 cohorts: the training cohort (n = 243, 60%) and the validation cohort (n = 161, 40%). The preoperative predictors of tympanosclerosis were determined by multivariate logistic regression analysis and implemented using a clinical score tool. The predictive accuracy and discriminative ability of the clinical score were determined by the area under the curve (AUC) and the calibration curve. The multivariate analysis in the training cohort (n = 243, 60%) identified independent factors for tympanosclerosis as the female sex (odds ratio [OR]: 3.83; 95% CI: 1.66-9.37), the frequency-specific air-bone gap at 250 Hz ≥ 45 dB HL (OR: 3.68; 95% CI: 1.68-8.57), aditus ad antrum blockage (OR: 3.29; 95% CI: 1.38-8.43), type I eardrum calcification (OR: 25.37; 95% CI: 8.41-88.91) or type II eardrum calcification (OR: 18.86; 95% CI: 6.89-58.77), and a history of otitis media ≥ 10 years (OR: 4.10; 95% CI: 1.58-11.83), which were all included in the clinical score tool. The AUC of the clinical score for predicting tympanosclerosis was 0.89 (95% CI: 0.85-0.93) in the training cohort and 0.89 (95% CI: 0.84-0.95) in the validation cohort. The calibration curve also showed good agreement between the predicted and observed probability. The clinical score achieved an optimal prediction of tympanosclerosis before surgery. The presence of calcification pearls on the promontorium tympani is a strong predictor of tympanosclerosis with stapes fixation.

Isolated congenital middle ear malformations: comparison of preoperative HRCT and surgical findings

Objective:To compare preoperative temporal bone HRCT readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations（CMEMs） and summarize the malformations that can be diagnosed with HRCT. Method:A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. Result:One hundred and fifty-two patients and 168 ears were reviewed. One hundred and seven cases（63.7%） could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Sixty-one cases（36.3%） were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 51.2% of the cases.The stapes and incudostapedial complex are the most commonly involved structures. Conclusion:HRCT provides helpful preoperative clinical information in CMEM.Most cases of aplasia or dysplasia of the ossicular chain, e.g., aplasia/dysplasia of the long process of the incus, aplasia of the stapes'superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis.

Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss

Introduction&#x0D; Otosclerosis presents as conductive or mixed hearing loss depending upon the stage of the disease. Isolated sensorineural hearing loss though known to occur has been rarely reported. Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with progressive hearing loss. The aim of this article is to note the prevalence of cochlear otosclerosis in young patients presenting with progressive sensorineural hearing loss. &#x0D; Materials and Methods&#x0D; Retrospective chart analysis of 19 patients who presented with progressive SNHL with unknown etiology was done.&#x0D; Results&#x0D; Otosclerosis involving the pericochlear region was diagnosed in 27 ears which was identified by high resolution CT scan of temporal bone. In four ears, in addition, there was demineralization just anterior to oval window without clinical evidence of stapes fixation.&#x0D; Conclusion&#x0D; In young patients with progressive SNHL, high resolution CT scan of temporal bone should be done to identify progressive cochlear otosclerosis. Medical management should be initiated in these patients to halt the progression of disease.

The effect of one-stage tympanoplasty for stapes fixation with tympanosclerosis

Objective:To evaluate hearing outcome and complications of one-stage tympanoplasty in patients with stapes fixation due to tympanosclerosis. Method:59 patients（sixty-one ears） underwent one-stage tympanoplasty for stapes fixation due to tympanosclerosis were retrospectively analyzed. Stapes fixation due to tympanosclerosis were proved during the surgery in these patients diagnosed with chronic otitis media. For all the patients, tympanosclerotic plaques around stapes were removed for stapes mobilization. Then the ossicular chain was rebuilt by autogenous incus or PORP. The pre-and post-operative audiometric results（500 Hz, 1 kHz, 2 kHz and 4 kHz） were evaluated for each patient. Improvement of pure-tone average more than 10 dB postoperatively were accepted as success criteria. Result:Complications included temporary facial paralysis（1/61）, temporary vertigo（2/61）, mild elevation in bone conduction thresholds（2/61） and delayed healing of tympanic membrane（1/61）. Postoperative（1 and 3 months） bone conduction thresholds improved at frequencies of 1 and 2 kHz（P<0.01）. Postoperative（1 and 2 years） air conduction thresholds improved at all frequencies（P<0.01 or P<0.05）. A gain ≥10 dB in pure-tone average was found in 44（72.13%） patients at 1 year after surgery. The air conduction levels were significantly improved in both autogenous incus and PORP groups（P<0.01）. There was no difference about success rate between these two groups（P>0.05）. Conclusion:For patients with stapes fixation due to tympanosclerosis, one-stage tympanoplasty can improve hearing threshold though ossicular chain reconstruction and stapes release. The major complications such as facial paralysis and sensorineural hearing loss should be avoided by delicate surgical operation.

A Case Series of X-Linked Deafness-2 with Sensorineural Hearing Loss, Stapes Fixation, and Perilymphatic Gusher: MR Imaging and Clinical Features of Hypothalamic Malformations.

X-linked deafness-2 (DFNX2) is an X-linked recessive disorder characterized by profound sensorineural hearing loss and a pathognomonic temporal bone deformity. Because hypothalamic malformations associated with DFNX2 have been rarely described, we aimed to further describe these lesions and compare them with features of a nonaffected population. All patients diagnosed with DFNX2 between 2006 and 2019 were included and compared with age-matched patients with normal MR imaging findings and without hypothalamic dysfunction. MR imaging features differing between groups were selected to help identify DFNX2. Sensitivity and specificity were calculated for these features. Agreement among 3 radiologists was quantified using the index κ. Information on the presence or absence of gelastic seizures, precocious puberty, or delayed puberty was also gathered. We selected distinctive MR imaging features of hypothalamic malformations in DFNX2. The feature selected on axial T2 images was the folded appearance of the ventromedial hypothalamus (sensitivity, 100%; specificity, 95.8%) characterized by an abnormal internal/external cleft (sensitivity, 100%; specificity, 95.7%). On coronal T2, the first distinctive feature was a concave morphology of the medial eminence (sensitivity, 100%; specificity, 97.1%), the second feature was at least 1 hypothalamic-septum angle ≥90° (sensitivity, 90%; specificity, 72.5%), and the third feature was a forebrain-hypothalamic craniocaudal length of ≥6 mm (sensitivity, 70%; specificity, 79.7%). Clinical features were also distinctive because 9 patients with DFNX2 did not present with gelastic seizures or precocious puberty. One patient had delayed puberty. The κ index and intraclass correlation coefficient ranged between 0.78 and 0.95. Imaging and clinical features of the hypothalamus suggest that there is a hypothalamic malformation associated with DFNX2. Early assessment for pubertal delay is proposed.