Intratympanic steroid therapy for Bell's palsy with poor prognostic results.

In Bell's palsy, electrodiagnosis by electroneurography (ENoG) is widely used to predict a patient's prognosis. The therapeutic options for patients with poor prognostic results remain controversial. Here, we investigated whether early intervention with intratympanic steroid therapy (ITST) is an effective treatment for Bell's palsy patients with poor electrodiagnostic test results (≤ 10% electroneurography value). Patients in the concurrent ITST group (n = 8) received the standard systemic dose of prednisolone (410 mg total) and intratympanic dexamethasone (16.5 mg total) and those in the control group (n = 21) received systemic prednisolone at the standard dose or higher (average dose, 605 ± 27 mg). A year after onset, the recovery rate was higher in the ITST group than in the control group (88% vs 43%, P = 0.044). The average House-Brackmann grade was better in the concurrent ITST group (1.13 ± 0.13 vs 1.71 ± 0.16, P = 0.035). Concurrent ITST improves the facial nerve outcome in patients with poor electroneurography test results, regardless of whether equivalent or lower glucocorticoid doses were administered. This may be ascribed to a neuroprotective effect of ITST due to a higher dose of steroid reaching the lesion due to dexamethasone transfer in the facial nerve.

Calcium-Sensing Receptor Is Functionally Expressed in the Cochlear Perilymphatic Compartment and Essential for Hearing.

Maintaining Ca2+ homeostasis in lymphatic fluids is necessary for proper hearing. Despite its significance, the mechanisms that maintain the cochlear lymphatic Ca2+ concentrations within a certain range are not fully clarified. We investigated the functional expression of calcium-sensing receptor (CaSR), which plays a pivotal role in sensing extracellular Ca2+ concentrations for feedback regulations. Western blotting for CaSR revealed an approximately 130-kDa protein expression in cochlear tissue extracts and immunohistochemical analysis revealed its expression specifically in type I fibrocytes in the spiral ligament, fibrocytes in the supralimbal and limbal regions, the epithelium of the osseous spiral lamina, and the smooth muscle cells of the spiral modiolar arteries. Ca2+ imaging demonstrated that extracellular Ca2+ increased the levels of intracellular Ca2+ in CaSR-expressing fibrocytes in the spiral ligament, and that this was suppressed by the CaSR inhibitor, NPS2143. Furthermore, hearing thresholds were moderately elevated by intracochlear application of the CaSR inhibitors NPS2143 and Calhex231, across a range of frequencies (8-32 kHz). These results demonstrate the functional expression of CaSR in the cochlear perilymphatic compartment. In addition, the elevated hearing thresholds that are achieved by inhibiting CaSR suggest this is a required mechanism for normal hearing, presumably by sensing perilymphatic Ca2+ to stabilize Ca2+ concentrations within a certain range. These results provide novel insight into the mechanisms regulating Ca2+ homeostasis in the cochlea and provide a new perspective on cochlear physiology.

Contrast-enhanced magnetic resonance imaging of facial nerve swelling in patients with severe Ramsay Hunt syndrome.

OBJECTIVE: In Ramsay Hunt syndrome, contrast enhancement of magnetic resonance (MR) imaging seen in the affected facial nerve in the majority of cases, but its clinical significance has not been well investigated to date. The aim of this study was to elucidate the clinical significance of this imaging sign by quantitively investigating the correlation between the signal increase and swelling of the facial nerve. We also investigated the temporal change in this sign and its correlation with recovery. METHODS: We retrospectively evaluated swelling of the facial nerve in 16 patients with severe Ramsay Hunt syndrome who underwent both contrast-enhanced magnetic resonance imaging and facial nerve decompression surgery via a transmastoid approach alone or in combination with a middle cranial approach. All the patients had a Yanagihara score of ≤8 points and facial nerve degeneration of ≥90% confirmed by either a nerve excitability test or electroneurography. Swelling of the facial nerve was evaluated intraoperatively using a 4-point grading system. RESULTS: A significant correlation was observed between contrast enhancement on T1-weighted images and facial nerve swelling in the labyrinthine segment, geniculate ganglion, and pyramidal segment (P = 0.030, P = 0.018, and P = 0.037, respectively). Furthermore, the contrast enhancement increased significantly as more time elapsed after the onset of facial palsy (mean ± standard error, 14.7 ± 2.3 days, range, 5-42 days) in the geniculate ganglion and pyramidal segment (correlation coefficient, 0.546 and 0.689, P = 0.022 and P = 0.002, respectively). Patients with good recovery (Yanagihara score of ≥36) showed significantly lower contrast enhancement in the tympanic and mastoid segments (P = 0.021 and 0.020, respectively) than those who with poor recovery. CONCLUSION: In particular segments of the facial nerve, contrast enhancement on T1-weighted image correlated with facial nerve swelling and recovery. These observations underscore the clinical significance of contrast enhancement on T1-weighted images in patients with Ramsay Hunt syndrome.

Developmental delays assessed using the Enjoji Scale in children with cochlear implants who have intellectual disability with or without autism spectrum disorder.

OBJECTIVE: Intellectual disability (ID) and autism spectrum disorder (ASD) are common among children who are candidates for cochlear implants. However, the implications of these comorbidities for cochlear implant placement have been not fully established. This study sought to identify these implications by comparing developmental delays among children with these conditions. METHODS: Participants were children who were followed up at least every 6 months for 24 months after cochlear implant surgery. Developmental delays were assessed using the Enjoji Scale of Infant Analytical Development (Enjoji Scale) and compared in three groups with hearing loss: those with ID (ID group, n=4); those with ASD and ID (ASD+ID group, n=4); and those with typical development (control group, n=5). Developmental delay was evaluated longitudinally before and after cochlear implant placement for 18 months. RESULTS: Among the six subscales that make up the Enjoji Scale, language development and intelligence development were significantly delayed in all three groups and were exacerbated over time except for language development in the control group. Emotional development and social behavior were significantly delayed only in the ASD+ID group. Comparison of intergroup differences revealed delays in language development in the ID and ASD+ID groups compared with the control group. CONCLUSION: The Enjoji Scale successfully demonstrated developmental delays characteristic to the underlying comorbidities of ID with or without ASD in children with cochlear implants. The Enjoji Scale can be a useful diagnostic tool for screening children with cochlear implants for ID with or without ASD.

Remote Asymmetric Bromination Reaction with Vinylketene Silyl N,O-Acetal and Its Application to Total Synthesis of Pellasoren A.

Stereoselective bromination of the E,E-vinylketene silyl N,O-acetal possessing a chiral auxiliary has been achieved and applied to introduction of heteroatom at γ-position of α,ß-unsaturated imide. The reactions proceeded in high stereoselectivity. Total synthesis of pellasoren A, an antitumor propionate from the myxobacteriun Sorangium cellulosum, has been accomplished in short steps by this methodology and our method of reduced polypropionate synthesis.