Characterization of the Sheep Round Window Membrane

xlomafota13 shared this article with you from Inoreader Characterization of the Sheep Round Window Membrane Via Journal of the Association for Research in Otolaryngology Abstract Intratympanic injection is a clinically used approach to locally deliver therapeutic molecules to the inner ear. Drug diffusion, at least in part, is presumed to occur through the round window membrane (RWM), one of the two openings to the inner ear. Previous studies in human temporal bones have identified a three-layered structure of the RWM with a thickness of 70–100 μm. This is considerably thicker than the RWM in rodents, which are mostly used to model RWM permeability and assess drug uptake. The sheep has been suggested as a large animal model for inner ear research given the similarities in structure and frequency range for hearing. Here, we report the structure of the sheep RWM. The RWM is anchored within the round window niche (average vertical diameter of 2.1 ± 0.3 mm and horizontal diameter of 2.3 ± 0.4 mm) and has a curvature that leans towards the scala tympani. The centre of the RWM is the thinnest (55–71 μm), with increasing thickness towards the edges (< 171 μm), where the RWM forms tight attachments to the surrounding bony niche. The layered RWM structure, including an outer epithelial layer, middle connective tissue and inner epithelial layer, was identified with cellular features such as wavy fibre bundles, melanocytes and blood vessels. An attached "meshwork structure" which extends over the cochlear aqueduct was seen, as in humans. The striking anatomical similarities between sheep and human RWM suggest that sheep may be evaluated as a more appropriate system to predict RWM permeability and drug delivery in humans than rodent models. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Identification of a novel CYP26A1 mutation in a Chinese family with congenital microtia.

xloma.fota.13 shared this article with you from Inoreader Identification of a novel CYP26A1 mutation in a Chinese family with congenital microtia. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Identification of a novel CYP26A1 mutation in a Chinese family with congenital microtia. Int J Pediatr Otorhinolaryngol. 2020 Nov 07;139:110488 Authors: Guo P, Ji Z, Jiang H, Huang X, Wang C, Pan B Abstract OBJECTIVES: Microtia is defined as a congenital malformation characterized by a small, abnormally shaped auricle, with atresia or stenosis of the auditory canal. This study investigated a mutation of the cytochrome P450, family 26, subfamily A, polypeptide 1(CYP26A1) gene, which is considered important in craniofacial development, in a family affected with microtia. METHODS: Whole-exome sequencing (WES) was performed on the proband and his family members to identify disease-associated variants. Computational predictions of the altered protein were analyzed using several bioinformatics tools. The wild-type (WT) and mutant forms of CYP26A1 cDNA were transfected into human embryonic kidney cells, and the mRNA and protein levels were compared using quantitative polymerase chain reaction (qPCR) and Western blot analyses. RESULTS: In this two-generation family, the proband and his mother were diagnosed with unilateral microtia. Unilateral microtia and ipsilateral accessory ear were observed in one of the twins, who were sisters of the proband. The father and the other twin showed no abnormal clinical features. A heterozygous mutation of a C to T in the CYP26A1 gene, which leads to truncation of the CYP26A1 protein, was identified in this family. The nonsense mutation cosegregated with patients and was absent in normal members of the family. The prediction software indicated that it was a possibly pathogenic mutation. The structure of the protein varied significantly between the WT and mutant proteins. Functional analysis showed that this mutation caused a significant decrease in both the mRNA and protein levels. CONCLUSIONS: Our findings suggest that this mutation of CYP26A1 may be a pathogenic factor leading to the phenotypes of microtia and accessory ear in this family. Further studies are needed to prove the function of this mutation and to explore the possible mechanism by which this variant is involved in the occurrence of microtia. PMID: 33197841 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Clinical heterogeneity of the SLC26A4 gene in UAE patients with hearing loss and bioinformatics investigation of DFNB4/Pendred syndrome missense mutations.

xloma.fota.13 shared this article with you from Inoreader Clinical heterogeneity of the SLC26A4 gene in UAE patients with hearing loss and bioinformatics investigation of DFNB4/Pendred syndrome missense mutations. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Clinical heterogeneity of the SLC26A4 gene in UAE patients with hearing loss and bioinformatics investigation of DFNB4/Pendred syndrome missense mutations. Int J Pediatr Otorhinolaryngol. 2020 Nov 09;:110467 Authors: Chouchen J, Mahfood M, Alobathani M, Eldin Mohamed WK, Tlili A Abstract BACKGROUND: The development of next generation sequencing-based techniques showed an important progress in the identification of pathogenic variants related to monogenetic diseases with genetic and phenotypic heterogeneities. Hereditary hearing loss is considered as one of these heterogeneous diseases, given the large number of deafness causing genes, the different modes of inheritance and the phenotypic variabilities associated to the severity, age of onset and/or presence or absence of other clinical manifestations. MATERIAL AND METHODS: In this study, we performed next-generation sequencing (NGS) in 51 UAE patients with hearing loss and no GJB2 mutations. In addition, we reviewed all reported SLC26A4 missense mutations with a confirmed DFNB4/Pendred syndrome phenotype and tried to find a genotype/phenotype correlation using different criteria. RESULTS: By analyzing the NGS data, we identified one new SLC26A4 variant c.1150G > C (p.Glu384Gln) and one known SLC26A4 mutation c.716T > A (p.Val239Asp) in two different patients. Direct Sanger sequencing and segregation analyses confirmed the implication of both DNA variants in the deafness phenotype. Moreover, the clinical examination of both patients showed that one patient has syndromic deafness (Pendred syndrome) and the second one has non-syndromic deafness. The analysis of all confirmed missense mutations didn't reveal a complete genotype/phenotype correlation. CONCLUSION: To the best of our knowledge, this is the first report of mutations associated with DFNB4/Pendred deafness in the GCC region. PMID: 33199029 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Adenoidectomy: Anatomical variables as predictive factors of intraoperative adenoid residues.

xloma.fota.13 shared this article with you from Inoreader Adenoidectomy: Anatomical variables as predictive factors of intraoperative adenoid residues. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Adenoidectomy: Anatomical variables as predictive factors of intraoperative adenoid residues. Int J Pediatr Otorhinolaryngol. 2020 Nov 11;:110493 Authors: Pagella F, Lizzio R, Pusateri A, Ugolini S, Maiorano E, Mirabella R, De Silvestri A, Tinelli G, Matti E Abstract OBJECTIVES: Adenoid hypertrophy is a common cause of upper airway obstruction in children. However, after adenoidectomy, nasal obstructive symptoms may persist or recur, requiring surgical revision. The aim of this study is to evaluate if individual patient features can influence the efficacy of the traditional technique. METHODS: A retrospective observational study was conducted by recruiting patients from candidates for adenoidectomy. All children underwent conventional transoral curettage adenoidectomy with endoscopic control at the end of procedure, and in presence of adenoid residues, a concomitant revision adenoidectomy was performed. For each patient, the following data were collected: age, sex, weight, height, length of the soft palate and surgical technique used. RESULTS: In 18% of patients (113/612), the most critical areas of the nasopharynx were not reached by standard surgery, making a complete adenoidectomy difficult. In this group, the average length of the soft palate was 3.1 cm, 5 mm more than the average of the sample, and 6 mm more than the average length of patients undergoing standard surgery alone (p < 0.001). CONCLUSION: Our study confirms the hypothesis that a greater length of the soft palate conditions the results of the intervention. The length of the soft palate can be considered an intraoperative criterion to select the cases in which perform endoscopic control after the standard procedure. PMID: 33199030 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The 'Frankentower:' A clinician-driven approach to enhancing endoscopic data technology.

xloma.fota.13 shared this article with you from Inoreader The "Frankentower:" A clinician-driven approach to enhancing endoscopic data technology. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles The "Frankentower:" A clinician-driven approach to enhancing endoscopic data technology. Int J Pediatr Otorhinolaryngol. 2020 Nov 04;:110486 Authors: Ngombu S, Payne LM, Cecil JM, Grischkan JM Abstract OBJECTIVE: To develop a more efficient endoscopy tower and evaluate its capability to improve retrieval and storage of videos across 6 clinical sites. METHODS: Descriptive study that took place at tertiary care Pediatric Otolaryngology Department over a 2 year period. In collaboration with our Information Services Department and Otolaryngology endoscopy vendors, we assembled our "Frankentower" endoscopy cart using components from 3 medical device manufacturers. RESULTS: It is feasible to create a functional composite endoscopic tower. Implementation of "Frankentower" improved overall management and retrieval of endoscopy video and images in our department which lead to better patient care and facilitated education for medical students, residents and faculty. CONCLUSIONS: The "Frankentower" is a feasible, cost-effective solution to streamline the capture, access and storage of patient endoscopy exams across our operating rooms, inpatient consult service, Emergency Department and outpatient clinics. PMID: 33213960 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Leveraging telemedicine to preserve pediatric global health missions in the era of COVID-19.

xloma.fota.13 shared this article with you from Inoreader Leveraging telemedicine to preserve pediatric global health missions in the era of COVID-19. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Leveraging telemedicine to preserve pediatric global health missions in the era of COVID-19. Int J Pediatr Otorhinolaryngol. 2020 Nov 12;:110494 Authors: Patel KR, Zablah E, Yager PH, Hartnick CJ Abstract This paper outlines the use of a global telehealth program to leverage the potential of telehealth to not only 1) preserve the previous progress of our pediatric surgical airway global teaching mission, but also: 2) to provide rapid, international dissemination of information related to care of pediatric COVID-19 patients; 3) to virtually support the attainment of self-sufficiency of our host countries in relation to our teaching mission; and 4) to inspire host countries to be local champions for each other during the COVID-19 crisis. PMID: 33213961 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A new training package (3Cs: Connect, Communicate and Collaborate) for improving family responsive service delivery in early intervention for children with hearing loss: A proof of concept study.

xloma.fota.13 shared this article with you from Inoreader A new training package (3Cs: Connect, Communicate and Collaborate) for improving family responsive service delivery in early intervention for children with hearing loss: A proof of concept study. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles A new training package (3Cs: Connect, Communicate and Collaborate) for improving family responsive service delivery in early intervention for children with hearing loss: A proof of concept study. Int J Pediatr Otorhinolaryngol. 2020 Nov 04;:110484 Authors: Alduhaim A, Purcell A, Cumming S, Doble M Abstract BACKGROUND: An objective of early intervention for children with hearing loss is to enhance family engagement in therapy to maximise a child's speech and language potential. However, requiring a service provider to work collaboratively with a child's family can create problems in developing and underdeveloped countries, where skilled service providers and services for children with hearing loss are lacking and where an expert model of intervention prevails. OBJECTIVES: To determine the preliminary effectiveness of a new training package Connect, Communicate and Collaborate (3Cs), in improving the knowledge and confidence of service providers in the delivery of family responsive services in an early intervention program for children with hearing loss. METHODS: Five learning modules were developed based on service provider experience working with children with hearing loss, and parents of children with hearing loss. Six participants completed the training package comprising five training modules and an introductory session over a 6-week period. Participants' confidence and knowledge in providing family responsive practice was measured pre and post training using visual analogue scales, and participants were also invited to provide their reflections on the program. RESULTS: Pre- and post-training confidence ratings revealed significant improvements in the perceptions of participants in the implementation of responsive family practice across four of five of programme learning modules (p < 0.002). Participant reflection statements indicate they became more considerated in their family responsive practice. Despite positive experiences working with parents, participants stated they had ongoing difficulties guiding families through the decision-making processes of habilitation. The reflection process assisted learning and improved practice by supporting participants to build on their strengths. CONCLUSION: The 3Cs package improved the confidence in and knowledge of delivery of responsive family services for six participants in Kuwait. It also improved the participant's self-evaluation skills. The 3Cs provides professional development that meets the needs of service providers working with children with hearing loss to improve inclusion of families in the therapy process. PMID: 33213962 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: Radiological findings similar to Hirayama disease.

xloma.fota.13 shared this article with you from Inoreader A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: Radiological findings similar to Hirayama disease. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: Radiological findings similar to Hirayama disease. Int J Pediatr Otorhinolaryngol. 2020 Nov 11;:110491 Authors: Sekioka A, Fukumoto K, Kawasaki T, Koyama M, Fujimoto Y, Miyake H, Nomura A, Yamada S, Kanai R, Makino A, Urushihara N Abstract Constant neck flexion has been considered crucial to reducing anastomotic tension after tracheal resection. However, in rare cases, anteflexion can cause cervical cord damage, leading to acute neurological disorders such as tetraplegia. Here, we report a case of 5-year-old boy presenting with acute neurological disorder triggered by a chin-to-chest position over 4 days of deep sedation after cricotracheal resection. The radiological findings would suggest a mechanism similar to Hirayama disease, in which a shift of the dura leads to chronic muscular weakness and atrophy in young populations. PMID: 33213963 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Iatrogenic orbital cerebrospinal fluid leak in a young pediatric patient: Factors to consider.

xloma.fota.13 shared this article with you from Inoreader Iatrogenic orbital cerebrospinal fluid leak in a young pediatric patient: Factors to consider. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Iatrogenic orbital cerebrospinal fluid leak in a young pediatric patient: Factors to consider. Int J Pediatr Otorhinolaryngol. 2020 Nov 17;:110500 Authors: Niermeyer WL, Allen DZ, Kern C, Elmaraghy C Abstract Orbital abscess and subperiosteal abscess are pathologies which may require surgical treatment in the pediatric patient. Though rare, orbital cerebrospinal fluid (CSF) leak is a serious complication of abscess drainage. This paper presents a unique 5-month-old male with transorbital abscess drainage complicated by CSF leak. An endoscope was used to repair the defect through the orbitotomy incision. There was no evidence of persistent leak at follow-up. The surgical approach likely contributed to the complication, and the otolaryngologist played a key role in the leak repair. This case should serve to raise the awareness regarding considerations to avoid orbital CSF leak. PMID: 33218688 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Ethosuximide induced macroglossia and oropharyngeal edema.

xloma.fota.13 shared this article with you from Inoreader Ethosuximide induced macroglossia and oropharyngeal edema. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Ethosuximide induced macroglossia and oropharyngeal edema. Int J Pediatr Otorhinolaryngol. 2020 Nov 15;:110498 Authors: Shang H, Glaun M, Ongkasuwan J Abstract Acute macroglossia and laryngeal edema are rare adverse side effects that can cause life-threatening airway obstruction. We report a case of acute macroglossia that began after initiation of ethosuximide in a 15-year-old female with severe medically refractory epilepsy. Macroglossia worsened over the next two weeks of ethosuximide administration, preventing extubation. Macroglossia and laryngeal edema improved upon ethosuximide wean, and completely resolved after discontinuation. The patient was extubated successfully, with precautionary nasal trumpet placement and dexamethasone administration prior to extubation. In medically complex patients on multiple pharmacologic agents, anti-epileptic drugs should be suspected as a possible cause of acute macroglossia. PMID: 33218689 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Effects of allergic rhinitis on the progression and recovery of acute otitis media in a mouse model.

xloma.fota.13 shared this article with you from Inoreader Effects of allergic rhinitis on the progression and recovery of acute otitis media in a mouse model. Via International journal of pediatric otorhinolaryngology,http://goo.gl/4YdRhz Related Articles Effects of allergic rhinitis on the progression and recovery of acute otitis media in a mouse model. Int J Pediatr Otorhinolaryngol. 2020 Nov 13;:110497 Authors: Cho CG, Kim HB, Kim CK, Kim BH, Lim YS, Park SW, Park JH Abstract OBJECTIVES: The objective of this study was to evaluate the effects of allergic rhinitis (AR) on the development, progression, and recovery of acute otitis media (OM) in an animal model and investigate the secondary effects of bacterial infection. METHODS: BALB/c mice were divided into four groups: AR + OM, AR, OM, and control groups. AR + OM and AR groups were sensitized with ovalbumin (OVA) and alum and then challenged intranasally with OVA. Phosphate-buffered saline (PBS) was administered to the OM and control groups the same number of times. After AR induction, OM was induced by surgical inoculation of non-typeable Haemophilus influenza (NTHi) into the middle ear (ME) cavity of the mice in the AR + OM and OM groups. PBS was injected into the bulla in the AR and control groups. Each group was subdivided into sets of six mice, one for each of the four time points (0, 2, 7, and 10 days post-bacterial inoculation), at which point the mice were euthanized and ME and nasal cavity mucosa were obtained and evaluated. The occurrence of OM and the ME mucosa thickness were evaluated and compared among the four groups. Tissue expression of interleukin (IL)-1β, IL-6, and tumor necrosis factor-α ( TNF-α) in infected ME mucosa was assessed by immunohistochemical staining. We also investigated IgE, IL-4, and IL-5 in the nasal mucosa. RESULTS: Most of the ears showed OM on post-inoculation day 2 in both AR + OM and OM groups. In the AR + OM group, 58.3% of ears still had OM on post-inoculation day 10, while only 16.7% of the OM group had OM. The ME mucosa of all groups increased, and the AR + OM group exhibited the thickest mucosa. The OM group showed peak thickness on post-inoculation day 2 and then decreased, whereas the ME mucosa thickness of the AR + OM group continued to increase to day 7. In the OM group, the expression of IL-1β, IL-6, and TNF-α in the ME also increased significantly, peaking on post-inoculation day 2, and then gradually decreased. In the AR + OM group, the expression of these proteins increased until day 7 and then decreased. The IgE and Th2 response (IL-4 and IL-5) cytokines were expressed at higher levels in the AR + OM and AR groups than in the OM and control groups. CONCLUSION: The inflammatory reaction to NTHi was more intense and lasted longer in the allergic group, which indicates that AR affects the progression and subsequent recovery of acute bacterial OM. PMID: 33218690 [PubMed - as supplied by publisher] View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.