BACKGROUND: Otosclerosis is a bone disorder affecting the labyrinthine capsule that leads to conductive and occasionally sensorineural hearing loss. The etiology of otosclerosis remains unknown; factors such as infection, hormones, inflammation, genetics, and autoimmunity have been discussed. Treatment consists primarily of surgical stapes replacement and cochlear implantation. High-resolution computed tomography is routinely used to visualize bone pathology. In the present study, we used synchrotron radiation phase-contrast imaging (SR-PCI) to examine otosclerosis plaques in a temporal bone for the first time. The primary aim was to study their three-dimensional (3D) outline, vascular interrelationships, and connections to the middle ear.
METHODS: A donated ear from a patient with otosclerosis who had undergone partial stapedectomy with the insertion of a stapes wire prosthesis was investigated using SR-PCI and compared with a control ear. Otosclerotic lesions were 3D rendered using the composite with shading technique. Scalar opacity and color mapping were adjusted to display volume properties with the removal of bones to enhance surfaces. Vascular bone channels were segmented, and the communications between lesions and the middle ear were established.
RESULTS: Fenestral, cochlear, meatal, and vestibular lesions were outlined three-dimensionally. Vascular bone channels were found to be frequently connected to the middle ear mucosa, perilabyrinthine air spaces, and facial nerve vessels. Round window lesions partly embedded the cochlear aqueduct which was pathologically narrowed, while the inferior cochlear vein was significantly dilated in its proximal part.
CONCLUSIONS: Otosclerotic/otospongiotic lesions were imaged for the first time using SR-PCI and 3D rendering. The presence of shunts and abnormal vascular connections to the labyrinth appeared to result in hyper-vascularization, overloading the venous system, and leading to sensorineural hearing loss. We speculate about possible local treatments to alleviate the impact of such critical lesions on the labyrinthine microcirculation.

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UNASSIGNED: To investigate the effect of day-case stapes surgery on hearing results, quality of life, patient satisfaction, and complications rates, compared with inpatient stapes surgery.
UNASSIGNED: A single-center, nonblinded, randomized controlled trial in a tertiary referral center.
UNASSIGNED: One hundred twelve adult patients planned for primary or revision stapes surgery were randomly assigned to either the day-case or inpatient treatment group. The effect on hearing outcomes (primarily), hearing benefits, quality of life, patient satisfaction, postoperative complications, and causes of crossover or readmission (secondarily) were assessed using auditory evaluations, questionnaires, and patients\' charts over a follow-up period of 1 year.
UNASSIGNED: Audiometric measurements and postoperative success rates were not different between the inpatient and day-case group. There were no statistically significant differences between both groups regarding the overall quality of life (QoL) (HUI3), disease-specific QoL (GHSI), change in postoperative health status (GBI), and postoperative complications rate. We found a high patient satisfaction toward the day-case approach. Six patients allocated to the inpatient group requested same-day discharge. Of the day-case patients, there was a crossover rate to inpatient care of 38% (20 patients), mainly due to postoperative nausea and vomiting (25%), vertigo (20%), or dizziness (40%).
UNASSIGNED: We found no significant differences in outcomes of audiometric measurements, QoL, patient satisfaction and postoperative complications following day-case, and inpatient stapes surgery. Therefore, stapes surgery in a day-case setting seems to be a feasible approach in terms of postoperative outcome, safety, and desirability when taking patient selection and surgical planning into account. Besides this, the familiarity with a day-case approach of both patient and the surgical team, will increase the acceptance and feasibility of day-case stapes surgery.

Objectives: To investigate the prevalence of cochlear-facial dehiscence (CFD) and other radiographical pathologies in ears with facial nerve stimulation (FNS) from a cochlear implant (CI). Methods: Retrospective case-control study of 27 patients with CI and FNS on either ear (study group) and 27 patients without FNS, matched for age, sex and type of electrode array (control group). Preoperative CT scans of all 108 ears were re-evaluated. Subanalyses included comparisons between the study and control groups and associations between FNS and radiographic pathologies. Results: CFDs were detected in 20 of 54 ears (37%) in the study group and in 3 of 54 ears (6%) in the control group (P < 0.001). The corresponding numbers of otosclerosis were 10 (18%) and 0 (P = 0.011) and of developmental anomalies 16 (30%) and 8 (15%) (not significant). FNS was present in 33 ears in the study group, of which 14 (42%) had a CFD. FNS was absent in six ears with CFD and CI, four of which contralateral to an ear with FNS. Eight of 14 ears with FNS and CFD had a lateral electrode array and six had a perimodiolar electrode array. We found no association between the presence of CFD and stimulation thresholds for FNS. The adjusted odds ratio for developing FNS in the presence of a CFD was 9.9 (95% CI 2.7-36.0). Conclusions: CFD was the most common radiographic pathology in ears with FNS, with a 10-fold increased risk of FNS. To avoid CI-related FNS, preoperative CT scan and awareness of typical dehiscence symptoms are strongly recommended.

Conductive hearing loss with normal otoscopic findings has many differential diagnosis, but the diagnosis of otosclerosis is retrospective after exploratory tympanotomy. Congenital ossicular anomalies in isolation are rare and often have a delayed diagnosis, particularly if unilateral. Herein we present a rare case of stapes abnormality that was encountered as a surprise during exploratory tympanotomy in a case of conductive hearing loss mimicking clinical otosclerosis and was managed accordingly.

OBJECTIVE: 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%.

We report resolution of right-sided pulsatile tinnitus in a 44-year-old male who underwent stapedectomy for fenestral otosclerosis. Initial workup revealed a mixed hearing loss and absent stapedial reflexes consistent with ossicular fixation. CT angiography demonstrated near complete stenosis of the left-sided transverse and sigmoid sinuses and dominant contralateral venous outflow. We hypothesized that the dominant right cerebral venous outflow tract created turbulent flow that was conducted to cochlea. Successful stapedectomy was performed, and the pulsatile tinnitus resolved. This case report demonstrates evidence that the sound of turbulent blood flow can be conducted through bone and an instance where the perception of vascular tinnitus was eliminated with stapedectomy.

Otosclerosis is an early adult-onset disease that is associated with 5-9% and 18-22% of all cases of hearing and conductive hearing loss, respectively, and it is suspected to have a viral etiology. However, the role of viral infection in otosclerosis is still inconclusive. This study aimed to investigate whether rubella infection was associated with otosclerosis risk. We conducted a nationwide case-control study in Taiwan. Data were retrospectively analyzed from the Taiwan National health Insurance Research Database. Cases consisted of all patients who were aged ≥6 years and had a first-time diagnosis of otosclerosis for the period between 2001 and 2012. The controls were exact matched to cases in a 4:1 ratio by birth year, sex, and must survive in the index year of their matched cases. Adjusted odds ratio (OR) and 95% confidence interval (CI) were estimated by using conditional logistic regression. We examined 647 otosclerosis cases and 2588 controls without otosclerosis. Among the 647 patients with otosclerosis, 241 (37.2%) were male and 406 (62.8%) were female, with most aged between 40 and 59 years, with a mean age of 44.9 years. After adjusting for age and sex, conditional logistic regression revealed that exposure to rubella was not associated with a significant increase in otosclerosis risk (adjusted OR, 2.0; 95% CI, 0.18-22.06, p = 0.57). In conclusion, this study did not show that rubella infection was associated with the risk of otosclerosis in Taiwan.

Far-advanced otosclerosis (FAO) refers to severe otosclerosis with scarce auditory functions. The identification of the best method to correctly listen to sound and speech has a large impact on patients\' quality of life. We retrospectively analyzed the auditory function of 15 patients affected by FAO who were treated with stapedectomy plus hearing aids independent of the severity of their auditory deficit before surgery. The combination of surgery and hearing aids allowed excellent recovery of the perception of pure tone sounds and speech. Four patients, because of poor auditory thresholds, needed a cochlear implant after stapedectomy. Despite being based on a small sample of patients, our results suggest that stapedotomy plus hearing aids could improve the auditory capacities of patients with FAO independent of their auditory thresholds at T0. The careful selection of patients is fundamental to obtain the best outcomes.

Objectives: Several studies reported low detection rates of otosclerosis in high-resolution computed tomography (HRCT), especially when the scans were reviewed by non-specialized general radiologists. In the present study, we conducted a retrospective review of the detection of otosclerosis in HRCT by general radiologists and the impact of inadequately filled radiological request forms on the detection rate. Methods: Retrospective analysis of hospital records, HRCT reports, and radiological referral notes of 40 patients who underwent stapedotomy surgery for otosclerosis. HRCT imaging data sets were retrospectively reviewed by a blinded experienced neuroradiologist, whose reading served as the gold standard. Results: General radiologists reading HRCT scans had an overall detection rate of otosclerosis of 36.1% in this cohort (13 of 36 available HRCT reports). The neuroradiologist had a much higher detection rate of 82.5% (33 of 40 cases). Interobserver agreement between the general radiologists and the subspecialist neuroradiologist was poor (Cohen’s kappa κ = 0.26). General radiologists missed the diagnosis in 15 of the 33 CT-positive scans, corresponding to a missed diagnosis rate of 45.4%. There was a highly significant association between a missed diagnosis and the lack of an explicitly mentioned clinical suspicion of otosclerosis in the request forms (Pearson’s chi-squared test, p < 0.005). Conclusion: The diagnosis of otosclerosis is frequently missed by radiologists on HRCT scans of the temporal bone in a clinical setting. Possible reasons include a relative lack of experience of general radiologists with temporal bone imaging as well as the failure of clinicians to unambiguously communicate their suspicion of otosclerosis.