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This article discusses the use of the endoscopic endonasal approach (EEA) in combination with extended approaches for the surgical treatment of tumors in the middle cranial fossa. The case study presented involves a 55-year-old woman with a schwannoma in the right pterygopalatine fossa, infratemporal fossa, middle cranial fossa, and posterior cranial fossa. The authors describe the surgical techniques used, including the prelacrimal approach and the modified endoscopic Denker's approach, which allowed for gross total tumor removal. The article emphasizes the importance of a multidisciplinary skull base team and careful consideration of various factors in achieving successful outcomes with minimal complications. [Extracted from the article]

Abstract Background Wolfram syndrome gene 1 (WFS1) accounts for most of the familial nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) which is characterized by sensorineural hearing losses equal to and below 2000 Hz. The current study aimed to contribute to our understanding of the molecular basis of LFSNHL in an affected Taiwanese family. Methods The Taiwanese family with LFSNHL was phenotypically characterized using audiologic examination and pedigree analysis. Genetic characterization was performed by direct sequencing of WFS1 and mutation analysis. Results Pure tone audiometry confirmed that the family members affected with LFSNHL had a bilateral sensorineural hearing loss equal to or below 2000 Hz. The hearing loss threshold of the affected members showed no progression, a characteristic that was consistent with a mutation in the WFS1 gene located in the DFNA6/14/38 locus. Pedigree analysis showed a hereditarily autosomal dominant pattern characterized by a full penetrance. Among several polymorphisms, a missense mutation Y669H (2005T>C) in exon 8 of WFS1 was identified in members of a Taiwanese family diagnosed with LFSNHL but not in any of the control subjects. Conclusion We discovered a novel heterozygous missense mutation in exon 8 of WFS1 (i.e., Y669H) which is likely responsible for the LFSNHL phenotype in this particular Taiwanese family.

Keywords: adult; humans; language; paranasal sinuses; speech; voice EN adult humans language paranasal sinuses speech voice 1022 1024 3 05/28/21 20210601 NES 210601 Chronic rhinosinusitis (CRS) is a prevalent inflammatory disease of the paranasal sinuses. The final polyp grading score, which was obtained by adding the results of Meltzer polyp grading of both sides, ranged from 0 to 8 (no polyp: 0, mild: 1 to 2, moderate 3 to 5, and severe 6 to 8). Effect of CRS severity on nasalance scores The correlation between the polyp grading scores and nasalance scores were statistically significant for nasal sentences ( I p i < 0.001) and the mixed passage ( I p i = 0.03) but was nonsignificant for the oral passage ( I p i = 0.206). [Extracted from the article]

Study Objectives: We investigated the therapeutic effects of exercise in patients with obstructive sleep apnea (OSA), aiming to identify the subgroups that benefit the most and determine the optimal exercise protocol., Methods: Major databases were searched for randomized controlled trials involving patients with OSA performing aerobic exercise and/or resistance training. The investigated outcomes included apnea-hypopnea index (AHI), Epworth Sleepiness Scale (ESS), body mass index (BMI), and peak oxygen consumption during exercise (VO 2 peak). The pre- and postintervention unstandardized mean difference (USMD) of these parameters was compared between the exercise and control groups., Results: Twelve studies involving 526 patients were included. Exercise training significantly reduced AHI (USMD = -7.08 events/hour, 95% confidence interval [CI]: -9.98 to -4.17, p < 0.00001), ESS (USMD = -2.37, CI: -3.21 to -1.54, p < 0.00001), and BMI (USMD = -0.72 kg/m 2 , CI: -1.22 to -0.22, p = 0.005) and enhanced VO 2 peak (USMD = 3.46 mL/kg/min, CI: 1.20 to 5.71, p = 0.003). Subgroup analyses revealed that in continuous positive airway pressure (CPAP)-adherent patients, exercise significantly improved VO 2 peak but did not reduce AHI and ESS. A trend was observed that combining resistance training with aerobic exercise resulted in greater AHI reduction and VO 2 peak enhancement. Notably, exercise improved AHI, ESS, BMI, and VO 2 peak regardless of the baseline AHI or BMI., Conclusions: Exercise, including resistance and aerobic training, should be part of treatment for patients with OSA of all severities, regardless of obesity status, and even for those who are already under CPAP., Systematic Review Registration: The study protocol was registered with the PROSPERO database (#CRD42023423527)., (© 2024 American Academy of Sleep Medicine.)

Background: Inhibitors of apoptosis proteins (IAPs), which counteract apoptosis by potently inhibiting caspase activation, are promising targets of new anti-tumor therapy. However, their roles in the pathogenesis of nasopharyngeal carcinoma (NPC), an Epstein-Barr virus (EBV)-associated carcinoma, are not fully understood. Herein, we investigated the expression and regulation of IAPs in NPC., Methods and Results: Using real-time quantitative polymerase chain reaction (PCR) analysis, we found that among the IAPs family only the transcription of survivin, HIAP-1, and HIAP-2 was consistently up-regulated in NPC and metastatic NPC tissues. Immunohistochemical staining showed that their proteins were more predominantly expressed in tumor cells nests. Noteworthy, these IAPs were upregulated by interleukin-1 alpha stimulation or EBV infection, and subsequently resulted in triggering rapid proliferation of NPC verified by strong Ki-67 staining., Conclusion: Survivin, HIAP-1, and HIAP-2 were distinctly upregulated in NPC, suggesting they may play significant roles in NPC tumorigenesis and serve as tumor markers with prognostic and therapeutic implications., ((c) 2008 Wiley Periodicals, Inc.)

Background: Nasopharyngeal carcinoma (NPC) is associated with Epstein-Barr virus (EBV) and has high metastatic potential. Discoidin domain receptors (DDR1, DDR2) are receptor-type tyrosine kinases activated by collagen. Their ability to induce expression of matrix metalloproteinase is related with tumor invasion. Therefore, we aim to investigate DDRs gene expression and its regulation in NPC., Methods and Results: By use of real-time quantitative polymerase chain reaction (Q-PCR), DDR2 gene expression but not DDR1 was significantly higher in primary and metastatic NPC. DDR2 was predominantly distributed in NPC tumor cells rather than in infiltrating lymphocytes. EBV Z-transactivator (Zta) transfection may distinctly elevate DDR2 level. Furthermore, data from reporter assay indicate that Zta could transactivate DDR2 promoter activity, suggesting the possible upregulation mechanism., Conclusion: DDR2 was differentially upregulated in NPC and modulated by EBV Zta protein. DDR2 may play a role in NPC invasion and serve as a diagnostic and therapeutic target.

Background: Wolfram syndrome gene 1 (WFS1) accounts for most of the familial nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) which is characterized by sensorineural hearing losses equal to and below 2000 Hz. The current study aimed to contribute to our understanding of the molecular basis of LFSNHL in an affected Taiwanese family., Methods: The Taiwanese family with LFSNHL was phenotypically characterized using audiologic examination and pedigree analysis. Genetic characterization was performed by direct sequencing of WFS1 and mutation analysis., Results: Pure tone audiometry confirmed that the family members affected with LFSNHL had a bilateral sensorineural hearing loss equal to or below 2000 Hz. The hearing loss threshold of the affected members showed no progression, a characteristic that was consistent with a mutation in the WFS1 gene located in the DFNA6/14/38 locus. Pedigree analysis showed a hereditarily autosomal dominant pattern characterized by a full penetrance. Among several polymorphisms, a missense mutation Y669H (2005T>C) in exon 8 of WFS1 was identified in members of a Taiwanese family diagnosed with LFSNHL but not in any of the control subjects., Conclusion: We discovered a novel heterozygous missense mutation in exon 8 of WFS1 (i.e., Y669H) which is likely responsible for the LFSNHL phenotype in this particular Taiwanese family.