Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan.

(1) Background: The incidence of congenital cholesteatoma (CC) has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. (2) Methods: The participants were 100 patients (101 ears) who underwent initial surgical treatment at university hospitals in two rural prefectures between 2006 and 2022. A total of 68% of the patients were males and 32% were females, with a median age of 5 years. We reviewed the medical records for the date of birth, date of surgery, stage of disease, and first symptoms of the disease. (3) Results: The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. No significant difference was found between the incidences in the two prefectures. The number of surgeries performed was higher in the second half of the study period. No difference in the stage of progress was observed based on age. (4) Conclusions: The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence.

Electrical promontory stimulation test using a portable peripheral nerve stimulator with an ear canal electrode.

OBJECTIVE: An intact cochlear nerve is necessary for successful cochlear implantation (CI). Although the promontory stimulation test (PST) using a promontory stimulator (PS) and a transtympanic needle electrode is invasive, it is still commonly used to verify cochlear nerve function. PSs are currently unavailable because they are no longer manufactured; however, considering that PST continues to be beneficial in certain situations, alternative equipment is needed. The PNS-7000® (PNS) was developed as a neurologic instrument for stimulating the peripheral nerves. This study investigated the usefulness of the ear canal stimulation test (ECST) using PNS with a silver ball ear canal electrode, which is a new noninvasive alternative technique to the PST. METHODS: ECST was performed from November 2013 to December 2018 using PS and PNS for patients with severe to profound sensorineural hearing loss. The electrical threshold, most comfortable loudness level, uncomfortable loudness level, dynamic range, and gap detection were measured in the ECST. The results of these measured PNS items were compared with PS. RESULTS: ECST was performed in 61 ears of 35 patients (age, 59.9 ± 20.1 years) using PS and PNS. The sound sensation was elicited in 51 (83.6%) and 52 (85.2%) ears with PS and PNS, respectively. All items excluding GAP were measured in 46 (75%) and 43 (70%) ears at 50 and 100 Hz, respectively. GAP was measured in 33 ears by the ascending and descending methods using PS and PNS. Spearman's rank-order correlation coefficient revealed a significant positive linear correlation between the PS and PNS results in all measurements. No significant difference was found between the PS and PNS thresholds in all measured items. CONCLUSIONS: PNS is a useful instrument for performing ECST as a new alternative to PS. ECST using a silver ball electrode is a less invasive and easier test than PST.

Mitral regurgitation outcomes after transcatheter atrial septal defect closure.

BACKGROUND: Worsening mitral regurgitation (MR) is a complication of intervention for atrial septal defect (ASD). Little is known about mitral valve (MV) characteristics associated with worsening MR. We aimed to elucidate MR outcomes and predictors of worsening MR after transcatheter ASD closure. METHODS: We analyzed changes in MR from prior to transcatheter ASD closure to 6 months after the procedure and predictors of worsening MR via baseline transthoracic echocardiography in 238 patients (64.7% females; mean age, 53 ± 22 years). RESULTS: Worsening MR was defined as worsening to moderate in patients with less than or equal to mild MR at baseline or vena contracta width increasing of ≥2 mm by 6-month follow-up in patients with moderate MR. Worsening MR was observed in 29 patients (12.2%). The associated echocardiographic findings were pseudoprolapse, hamstringing, stiffness, and anteroposterior and intercommissural mitral annulus diameter in the univariable logistic regression analysis (all P < 0.05). Multivariable analysis after adjusting for age; long-standing persistent atrial fibrillation; and ASD size showed that models combining MV leaflet findings such as pseudoprolapse or hamstringing, or anterior leaflet stiffness with the ratio of the sum of anterior and posterior leaflet lengths to intercommissural mitral annulus diameter were statistically significant for predicting worsening MR (R2 = 0.393, P < 0.001 and R2 = 0.385, P < 0.001, respectively). CONCLUSIONS: Worsening MR after transcatheter ASD closure might depend on MV leaflet findings and annulus size in patients with long-standing persistent atrial fibrillation.

Reliability of Conventional Two-Dimensional Magnetic Resonance Imaging for Diagnosing Extraforaminal Stenosis in Lumbosacral Transition.

Introduction: Three-dimensional (3D) magnetic resonance imaging (MRI) is reportedly superior to two-dimensional (2D) MRI for diagnosing lumbar foraminal stenosis at L5-S1. In this study, we strictly distinguished the intra- and extraforaminal regions and compared the diagnostic reliability and accuracy of 2D and 3D MRI in each region. Methods: A total of 92 surgical cases of unilateral L5 radiculopathy were selected for imaging analysis, including 46 of foraminal stenosis at L5-S1 (Group F) and 46 of intraspinal canal stenosis at L4-5 (Group C) (48 men, 44 women; mean age, 66 years). The 2D and 3D MRI sets were assessed twice by two examiners. They were informed only of the laterality of the lesion in each case and asked to select among the following for each modality: "absence of foraminal stenosis," "intraforaminal stenosis," "extraforaminal stenosis," and "coincident intraforaminal and extraforaminal stenosis." The intra- and interobserver reliabilities were evaluated using kappa (κ) statistics for the intra- and extraforaminal regions and compared between 2D and 3D MRI. For each case, disagreements between examiners were resolved through discussion to obtain a diagnostic judgment for each modality. Subsequently, the final diagnosis of intra- and/or extraforaminal stenosis in Group F was made using multiple modalities and intraoperative findings. A comparison between 2D and 3D MRI in terms of diagnostic accuracy was performed for the intra- and extraforaminal regions. Results: No significant difference was observed in the κ statistics between 2D and 3D MRI for the intraforaminal region, whereas 3D MRI had significantly larger κ statistic than 2D MRI for the extraforaminal region. Ultimately, 3D MRI perfectly judged the extraforaminal region, whereas 2D MRI detected only 44.8% of the cases of extraforaminal stenosis. Conclusions: More than half of extraforaminal stenosis was overlooked by 2D MRI, suggesting that it is unreliable for diagnosing extraforaminal stenosis at L5-S1.

Survival in a Case of Emphysematous Cholecystitis With Sepsis Caused by Clostridium perfringens.

A 77-year-old man presented to the Department of Internal Medicine with a chief complaint of abdominal pain. During the outpatient examination, a computed tomography (CT) scan showed gallstones. The patient developed worsening abdominal pain and fever and was admitted to the emergency department. He was diagnosed with cholecystitis and hospitalized. Treatment with antimicrobial agents was initiated. On the second day of hospitalization, the patient developed a fever of 39°C, hypotension, and oliguria. An emergency CT scan was performed, which showed gas production in the gallbladder. He was diagnosed with emphysematous cholecystitis, and emergency percutaneous transhepatic gallbladder drainage was performed. The patient was transferred to the high-care unit, and intensive care was initiated. On the eighth day, a follow-up CT scan showed an abscess in the gallbladder bed, and drainage was performed percutaneously. His general condition gradually improved, and he was discharged from the hospital on day 24. The patient was readmitted for cholecystectomy three months after the initial admission. The prognosis of sepsis caused by Clostridium perfringens is extremely poor, with a mortality rate of 70%-100%. We present a case of emphysematous cholecystitis successfully treated with multimodal treatment despite the presence of sepsis due to Clostridium perfringens and discuss the possible prognostic factors by reviewing the literature.

Mitral valve leaflet stiffness predicts residual mitral regurgitation after transcatheter edge-to-edge mitral valve repair.

BACKGROUND: Residual regurgitation after transcatheter edge-to-edge mitral valve repair (TMVR) is a predictor of poor prognosis in patients with functional mitral regurgitation (FMR). This study sought to identify the mitral valve (MV) parameters measured by three-dimensional transesophageal echocardiography (3D-TEE) and MV leaflet features that predict residual mitral regurgitation (MR) after TMVR in patients with FMR. METHODS: Consecutive patients with FMR who underwent TMVR were classified into two groups based on the degree of residual MR just after TMVR: < 2+ in the optimal MR reduction group and ≥ 2+ in the suboptimal MR reduction group. The two groups were compared with respect to 3D-TEE parameters and the MV leaflet features, including the following parameters: stiffness, defined as a leaflet that remains at a fixed angle even during diastole, and thickness, measured in both clear and rough zones. RESULTS: Thirty-four of 46 patients (74%) were classified as the optimal MR reduction group. Multivariable analysis showed that anterior mitral leaflet + posterior mitral leaflet length/anteroposterior annulus diameter (p = .044) and MV leaflet stiffness (p = .007) were independent predictors of residual MR. CONCLUSION: MV leaflet stiffness and the ratio of MV leaflet lengths to the annulus diameter may be good predictors of residual MR after TMVR in patients with FMR.

White matter microstructural alterations in patients with neuropathic pain after spinal cord injury: a diffusion tensor imaging study.

Background: Through contrastive analysis, we aimed to identify the white matter brain regions that show microstructural changes in patients with neuropathic pain (NP) after spinal cord injury (SCI). Methods: We categorized patients with SCI into NP (n = 30) and non-NP (n = 15) groups. We extracted diffusion tensor maps of fractional anisotropy (FA) and mean (MD), axial (AD), and radial (RD) diffusivity. A randomization-based method in tract-based spatial statistics was used to perform voxel-wise group comparisons among the FA, MD, AD, and RD for nonparametric permutation tests. Results: Atlas-based analysis located significantly different regions (p < 0.05) in the appointed brain atlas. Compared to the non-NP group, the NP group showed higher FA in the posterior body and splenium of the corpus callosum and higher AD in the corpus callosum, internal capsule, corona radiata, posterior thalamic radiation, sagittal stratum, external capsule, cingulum, fornix/stria terminalis, superior longitudinal fasciculus, and uncinate fasciculus. Conclusion: The results demonstrated that compared with the non-NP group, NP pathogenesis after SCI was potentially related to higher values in FA that are associated with microstructural changes in the posterior body and splenium of the corpus callosum, which could be regarded as central sensitization or network hyperexcitability.

Association between sarcopenia and exercise capacity in patients with pulmonary hypertension without left heart disease.

BACKGROUND: Pulmonary hypertension (PH) has recently been described as a complex clinical syndrome affecting multiple organ systems, including the heart, lungs, and skeletal muscle, each of which plays an important role in exercise capacity. However, the relationship between exercise capacity and skeletal muscle abnormalities in patients with PH has not been fully elucidated. METHODS: We retrospectively analysed the exercise capacity and measures of skeletal muscle of 107 patients with PH without left heart disease (mean age 63 ± 15 years, 32.7% males, n = 30/6/66/5 in the clinical classification Group 1/3/4/5). RESULTS: Sarcopenia, low appendicular skeletal muscle mass index, low grip strength, and slow gait speed, determined by international criteria, were found in 15 (14.0%), 16 (15.0%), 62 (57.9%), and 41 (38.3%) patients, respectively. The mean 6-min walk distance of all patients was 436 ± 134 m and was independently associated with sarcopenia (standardised ß = -0.292, p < 0.001). All patients with sarcopenia showed reduced exercise capacity defined as 6-min walk distance < 440 m. Multivariable logistic regression analysis showed that each of the components of sarcopenia was associated with reduced exercise capacity (adjusted odds ratio and 95% confidence interval of appendicular skeletal muscle mass index: 0.39 [0.24-0.63] per 1 kg/m2, p = 0.006, grip strength: 0.83 [0.74-0.94] per 1 kg, p = 0.003, and gait speed: 0.31 [0.18-0.51] per 0.1 m/s, p < 0.001). CONCLUSIONS: Sarcopenia and its components are associated with reduced exercise capacity in patients with PH. A multifaceted evaluation may be important in the management of reduced exercise capacity in patients with PH.

Aging-Related Catatonia with Reversible Dopamine Transporter Dysfunction in Females with Depressive Symptoms: A Case Series.

OBJECTIVES: The authors describe five depressive patients with initially decreased striatal accumulation of dopamine transporter (DAT) single-photon emission computed tomography (SPECT), which improved in parallel with clinical symptoms. METHODS: Patients who exhibited decreased striatal accumulation and recovery of DATSPECT were identified among patients with the symptoms of depression. Their clinical and neuroimaging data were reviewed. RESULTS: Five patients were identified. All patients were presenile or senile women who presented with catatonia subsequent to symptoms of depression that remitted with treatment. DAT-SPECT showed a decreased striatal accumulation in all patients, which increased after treatment. Two patients had met the diagnostic criteria of probable dementia with Lewy bodies (DLB), but no longer did so after their symptoms improved. CONCLUSIONS: Reversible DAT dysfunction observed in this study suggests that reversible impairment of dopaminergic transmission in the striatum partly underlies catatonia. Careful consideration should be given to diagnosing DLB in patients with decreased DAT-SPECT accumulation, especially when catatonia is present.

Hearing Outcome and Predictors after Implanting Bone Conduction or Middle Ear Implants in Ears with Refractory Otitis Media.

Bone conduction implants (BCIs) and middle ear implants (MEIs) are promising options for individuals with persistent chronic inflammation of the middle or outer ear. However, the structure of the middle ear is often altered in patients who undergo mastoidectomy or posterior wall removal for refractory otitis media, leaving uncertainty regarding the efficacy of hearing devices. Only a few studies have examined auditory outcomes based on the etiology of hearing impairment. We investigated hearing outcomes, including speech audiometry, in patients who underwent implantation after surgery for refractory otitis media. Our findings indicated that patients who received BCIs or MEIs achieved favorable hearing outcomes. Furthermore, a correlation was observed between the preoperative bone-conduction threshold at 1 kHz in the better ear and the sound-field threshold at 1 kHz with BCIs, whereas no correlation was observed between the preoperative bone-conduction threshold and the sound-field threshold with MEIs. This study highlights the positive impact of BCIs and MEIs in patients who undergo implantation after surgery for refractory otitis media. Additionally, our study identified parameters that predict postoperative efficacy.

Doppler Underestimates Transvalvular Gradient Measured by Catheterization in Patients With Severe Aortic Stenosis.

We sought to clarify characteristics of patients with severe aortic stenosis (AS) in whom transvalvular mean pressure gradient (MPG) was underestimated with Doppler compared with catheterization. Study subjects included 127 patients with severe AS who underwent transcatheter aortic valve implantation. Between subjects with Doppler MPG underestimation ≥10 mm Hg (group U) and those without (group C), we retrospectively compared echocardiographic parameters and aortic valve calcification score using the Agatston method. Despite a strong correlation (rS = 0.88) and small absolute difference (2.1 ± 10.1 mm Hg) between Doppler and catheter MPG, 27 patients (21%) were in group U. Among 48 patients with catheter MPG ≥60 mm Hg, 10 patients (21%) revealed Doppler MPG of 40 to 59 mm Hg, suggesting they had been misclassified as having severe AS instead of very severe AS. According to the guidelines, indication of valve replacement for patients without symptoms should be considered for very severe AS but not for severe AS. Therefore, sole reliance on Doppler MPG could cause clinical misjudgments. Group U had larger relative wall thickness (median [interquartile range: 0.60 [0.50 to 0.69] vs 0.53 [0.46 to 0.60], p = 0.003) and higher calcification score (3,024 [2,066 to 3,555] vs 1,790 [1,293 to 2,501] arbitrary units, p <0.001). Both calcification score (per 100 arbitrary unit increment, odds ratio 1.10, 1.04 to 1.17, p = 0.002) and relative wall thickness (per 0.05 increment, odds ratio 1.29, 95% confidence interval 1.05 to 1.60, p = 0.02) were independently associated with Doppler underestimation. In conclusion, Doppler might underestimate transvalvular gradient compared with catheterization in patients with severe AS who have heavy valve calcification and prominent concentric remodeling left ventricular geometry.

Utility of biphasic calcium phosphate cement as a seal for root-end filling.

The recently developed biphasic calcium phosphate cement (BCPC) consists of α-tricalcium phosphate-tetracalcium phosphate as the solid phase and calcium phosphate solution as the liquid phase. BCPC powder is composed of a single solid solution with a monomodal size distribution. Here, we used a bacterial leakage model to examine the utility of BCPC as a seal for root-end filling. We prepared large (median particle size=9.96 µm; BCPC-L) and small (median particle size=4.84 µm; BCPC-S) BCPC powders. In total, 45 single-rooted teeth were instrumented, resected at the root-end, and retrofilled with experimental materials. Mineral trioxide aggregate (MTA) was used as the control. After visual confirmation of BCPC powder size and retrofilling quality by microscopy, bacterial leakage tests were conducted using Enterococcus faecalis. The bacterial leakage tests did not reveal any significant differences between BCPC-S and MTA. Our findings suggest that BCPC-S is useful for root-end filling.

Perforation of the Nasal Septum Identified During a Preoperative Otorhinolaryngology Consultation: A Case Report.

Nasal intubation is often required during oral surgery; however, nasal intubation can cause various complications including bleeding associated with nasal mucosal trauma during intubation and obstruction of the endotracheal tube. Two days before surgery, a nasal septal perforation was identified using computed tomography during a preoperative otorhinolaryngology consultation for a patient planned to undergo a nasally intubated general anesthetic. Subsequently, nasotracheal intubation was successfully performed after confirming the size and location of the nasal septal perforation. We used a flexible fiber optic bronchoscope to safely perform the nasal intubation while assessing for inadvertent migration of the endotracheal tube or soft-tissue damage around the perforation site. Careful preoperative planning in cooperation with the otorhinolaryngology department and use of computed tomography is recommended when a nasal abnormality is suspected.

Decoding cellular deformation from pseudo-simultaneously observed Rho GTPase activities.

Limitations in simultaneously observing the activity of multiple molecules in live cells prevent researchers from elucidating how these molecules coordinate the dynamic regulation of cellular functions. Here, we propose the motion-triggered average (MTA) algorithm to characterize pseudo-simultaneous dynamic changes in arbitrary cellular deformation and molecular activities. Using MTA, we successfully extract a pseudo-simultaneous time series from individually observed activities of three Rho GTPases: Cdc42, Rac1, and RhoA. To verify that this time series encoded information on cell-edge movement, we use a mathematical regression model to predict the edge velocity from the activities of the three molecules. The model accurately predicts the unknown edge velocity, providing numerical evidence that these Rho GTPases regulate edge movement. Data preprocessing using MTA combined with mathematical regression provides an effective strategy for reusing numerous individual observations of molecular activities.

Left ventricular hypertrophy as a predictor of cardiovascular outcomes after transcatheter aortic valve replacement.

AIMS: This study aimed to clarify the relationship between cardiovascular prognosis and left ventricular hypertrophy (LVH) in patients with severe aortic stenosis who underwent transcatheter aortic valve replacement (TAVR) and to investigate the relationship between cardiac sympathetic nerve (CSN) function and these factors using 123 I-metaiodobenzylguanidine scintigraphy. METHODS AND RESULTS: In this single-centre, retrospective observational study, 349 patients who underwent TAVR at our institution between July 2017 and May 2020 were divided into two groups: those with severe LVH pre-operatively [severe LVH (+) group] and those without LVH pre-operatively [severe LVH (-) group]. The rates of freedom from cardiovascular events (cardiovascular death and heart failure hospitalization) were compared. The relationship between changes in left ventricular mass index (LVMi) and changes in delay heart-mediastinum ratio (H/M) from before TAVR to 6 months after TAVR was also investigated. The event-free rate was significantly lower in the severe LVH (+) group (87.1% vs. 96.0%, log-rank P = 0.021). The severe LVH (+) group exhibited a significantly lower delay H/M value, scored by 123 I-metaiodobenzylguanidine scintigraphy, than the severe LVH (-) group (2.33 [1.92-2.67] vs. 2.67 [2.17-3.68], respectively, P < 0.001). Moreover, the event-free rate of post-operative cardiovascular events was lower among patients with a delay H/M value < 2.50 than that among other patients (87.7% vs. 97.2%, log-rank P = 0.012). LVMi was significantly higher (115 [99-130] vs. 90 [78-111] g/m2 , P < 0.001) and delay H/M value was significantly lower (2.53 [1.98-2.83] vs. 2.71 [2.25-3.19], P = 0.025) in the severe LVH (+) group than in the severe LVH (-) group at 6 months after TAVR. Patients with improved LVH at 6 months after TAVR also had increased delay H/M (from 2.51 [2.01-2.81] to 2.67 [2.26-3.02], P < 0.001), whereas those without improved LVH had no significant change in delay H/M (from 2.64 [2.23-3.06] to 2.53 [1.97-3.00], P = 0.829). CONCLUSIONS: Severe LVH before TAVR is a prognostic factor for poor post-operative cardiovascular outcomes. LVH associated with aortic stenosis and CSN function are correlated, suggesting their involvement in LVH prognosis.

Efficacy and safety of guide extension catheter in balloon pulmonary angioplasty for treatment of complex lesions in chronic thromboembolic pulmonary hypertension.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is used for treatment of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) and residual pulmonary hypertension after pulmonary endarterectomy (PEA) to improve hemodynamics, right ventricular function, and exercise capacity. However, the effectiveness and safety of guide extension catheters for BPA treatment in patients with CTEPH have not been demonstrated. METHODS: We retrospectively analyzed 91 lesions in 55 sessions of 28 patients with CTEPH who underwent BPA using a guide extension catheter. The purpose (backup, coaxial, and extension), efficacy, and safety of the guide extension catheters were explored. The efficacy of the guide extension catheter was assessed based on the success of the procedures and safety was evaluated based on procedure-related complications. RESULTS: Regarding the intended use, a guide extension catheter was used to strengthen the backup force of the guiding catheter in 52% of cases, extend the tip of the catheter in 38% of cases, and maintain the coaxiality of the guiding catheter in 10% of cases. Procedural success was achieved in 92.7% of 55 sessions and in 95.6% of 91 lesions. Complex lesions had a lower success rate than simple lesions (p = 0.04). Regarding safety concerns, complications were observed in 5 of 55 sessions (9.1%) and 6 of 91 lesions (6.6%). Only one case of pulmonary artery dissection using a guide extension catheter was reported. Except for this one case, extension catheter-related complications were not observed. CONCLUSIONS: A guide extension catheter can be used safely in BPA procedures with anatomically complex pulmonary artery branches and complex lesions by increasing backup support.

Biochemical characterization of mitochondria from adult worms and plerocercoid larvae of Spirometra mansoni shows mixed populations of anaerobic and aerobic mitochondria.

The mitochondria of adult and plerocercoid Spirometra mansoni were characterized in isolated mitochondria and in situ by electron microscopic histochemistry with special attention to the respiratory chain. Although the specific activities of the constituent enzyme complexes of succinate oxidase are fairly similar in adult and plerocercoid mitochondria, those of succinate oxidase and NADH-FRD are approximately 4- and 25-fold higher in adult mitochondria than in plerocercoid mitochondria, respectively. Quinone analysis by high performance liquid chromatography and mass spectrometry showed that adult and plerocercoid mitochondria contained both rhodoquinone-10 and ubiquinone-10 at concentrations of 4.98 and 0.106 nmol mg-1 for adult, and 0.677 and 0.137 nmol mg-1 for plerocercoid, respectively. Inhibition studies on the succinate-oxidase system of adult mitochondria showed that they possessed both cyanide-sensitive and -insensitive succinate oxidases, the latter of which produces hydrogen peroxide. Adult mitochondria, when NADH was used as a substrate, were shown to produce hydrogen peroxide, and the production of hydrogen peroxide decreased to undetectable levels in the presence of fumarate. The specific activities of NADH-fumarate reductase and cytochrome c oxidase were significantly higher in mature proglottids than in immature and gravid proglottids. Isopycnic density-gradient centrifugation analyses and in situ electron microscopic histochemistry revealed that both adult and plerocercoid mitochondria were heterogeneous in terms of respiratory function and physicochemical properties. The physiological significance of adult and plerocercoid mitochondria is discussed in relation to the oxygen tension of their parasitic habitats.