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Introduction: The molecular basis and mechanisms of juvenile nasopharyngeal angiofibromas (JNA) pathogenesis are still unknown. Despite being a rare and benign neoplasm, JNA is a locally aggressive and potentially destructive head and neck neoplasm, typically found in young males. The advancement of genome technologies and analytical tools has provided an unparalleled opportunity to explore the intricacy of JNA. The present study provides the first evidence of the involvement of Y-chromosome genes in JNA., Methods: A total of 13 JNA patients at an advanced disease stage and five age-matched male controls were registered for this study. Whole-exome sequencing (WES) analysis was conducted followed by functional analysis to understand the molecular mechanism of the JNA., Results: WES analysis revealed a high prevalence of mutations in 14 genes within the protein-coding, male-specific region of the Y-chromosome of young males (mean age: 13.8 ± 2.4) with JNA. These mutations, occurring at 28 distinct positions, were characterized as moderate to high impact and were prevalent in nine JNA patients but not in the control group. The most frequently mutated genes were USP9Y and UTY, followed by KDM5D, DDX3Y, and TSPY4. The expression of USP9Y, UTY, and DDX3Y was found to be co-modulated, implying their coordinated regulation as a complex. Furthermore, somatic mutations were detected in genes previously linked to JNA., Conclusion: The wide array of genetic mutations in the Y-chromosome male-specific region, along with the somatic alterations identified in JNA, provides novel insights into JNA pathophysiology., (© 2024 Wiley Periodicals LLC.)

Objective: To assess the microvascular density (MVD) in juvenile nasopharyngeal angiofibroma (JNA) with CD34 immunostaining and evaluate its relationship with clinico-demographic features., Methods: This prospective study included patients with JNA undergoing endoscopic excision. The histopathological specimen was stained using CD-34 antibodies to calculate MVD. MVD and clinico-demographic features were correlated., Results: The study included 12 patients with a median age of 15.5 years. The mean MVD was 39 vessels/high power field (range 5 to 151 vessels). MVD was significantly associated only with the volume of tumour (r = 0.65, p = 0.02). The recurrence occurred in one patient with an MVD of 107. The median follow-up was 38 months., Conclusion: MVD is significantly associated with tumour volume in JNA, which implies a robust role of angiogenesis in the pathology of the tumour. Also, higher MVD may be a risk factor for recurrence., Competing Interests: Competing InterestsThe authors have no competing interests to declare that are relevant to the content of this article., (© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Significance Statement: Petrous cholesteatoma is rare but frequently leads to complications. A thorough radiological evaluation helps in identifying the pathology and the extent of disease. Sound anatomical knowledge is vital for the planning of surgical approach to get adequate access without damaging important structures. Presentations in congenital lesions are varied due to the uneven growth model of the temporal bone. To the best of our knowledge, the pattern of involvement in this case has never been reported earlier., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Currently, there is no consensus on estimating the malignant potential of Carotid Body Tumor (CBT) and the only way to predict a metastatic CBT is through DOTANOC Positron Emission Tomography (PET) scan. There is a well-established correlation between CBT and superoxide anions inside tumor cells. The purpose of this pilot study was to measure superoxide anions inside CBT cells and find if these can be used as marker to predict malignant potential of CBT. The results were also co-related with findings of DOTANOC PET scan retrospectively. The CBT tissue from 10 patients was stained using a fluorogenic dye and superoxide anions were measured by analysis of fluorescent image. The patients were divided into two groups - First group with four patients having potentially malignant CBT based upon clinico-surgical characteristics and second group with the rest of the six patients. It was seen that the superoxide anions were highest in the first group which included patients with metastatic carotid body tumor, patients with multiple paragangliomas and patient with positive family history (p = 0.011). The same patients also had metastasis and multiple tumors detected on DOTANOC PET scan. It was concluded that measuring superoxide anions in excised tumor tissue can be used to estimate malignant potential of CBT and can identify patients who truly require DOTANOC PET scan; without affecting the treatment, as it is an expensive investigation involving ionizing radiation and may not be available in all centres., Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03623-6., Competing Interests: Conflicts of interest/Competing InterestsThe authors declare that they have no competing interests., (© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

To describe clinico-demographical profile for post-lingual hearing loss in the adults undergoing cochlear implantation and their outcomes. A retrospective chart review was conducted including adult patients (> 18 years) with bilateral post-lingual severe to profound hearing loss who underwent cochlear implantation in a tertiary care hospital of north India. The clinico-demographical details were collected and the outcomes of the procedure were assessed in terms of speech intelligibility scores, usage and satisfaction scores. Twenty-one patients were included with a mean age of 38.6 years with 15 males and 6 females. The major cause of deafness was infections followed by ototoxicity. The complication rate was 4.8%. Preoperative SDS was not recordable in any of the patients. The mean postoperative SDS was 74%, with no issue of device malfunction in a mean follow-up of 44 months. Cochlear implantation is a safe surgery with good outcomes in post-lingually deafened adults with the major cause for deafness being infections., Competing Interests: Conflict of interestThe authors have no competing interests to declare that are relevant to the content of this article., (© Association of Otolaryngologists of India 2023, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Intra-labyrinthine schwannomas are rare. We present a case of a 50-year-old male with non-serviceable unilateral sensorineural hearing loss and tinnitus. CE-MRI revealed an enhancing signal in the basal turn of left cochlea suggestive of a schwannoma. A trans-mastoid standard facial recess approach was used for tumor excision. At one year follow up, patient is disease free., (© Association of Otolaryngologists of India 2021.)

Background: Tuberculosis (TB) of posterior pharyngeal wall (PPW), either primary or secondary, is extremely rare and can mimic malignancy in elderly patients., Case Report: A 36-year-old female patient presented with complains of nonhealing ulcer at the back of her throat for 4 months. On examination, there was an ulceroproliferative growth covered with slough over PPW. Positron emission tomography scan showed uptake in PPW. There was no uptake in any other part of body. A biopsy from PPW lesion showed epithelioid cell granulomas with Langhans type giant cells. Auramine-rhodamine staining showed few beaded bacilli consistent with TB., Conclusion: Tuberculosis of PPW is an extremely uncommon entity and can mimic malignancy. It should be kept as one of the differential diagnosis as it can be cured solely by giving anti-tubercular therapy.

Antibiotic prophylaxis is commonly given to all patients undergoing cochlear implant surgery. However, currently, there is no consensus if pro- phylactic usage of antibiotics in cochlear implantation accords any benefit and if the duration of such use varies according to the surgeon's experience or institutional preference. A systematic review was conducted to gather evidence on ideal duration for antibiotic prophylaxis rec- ommended for patients undergoing cochlear implantation. We registered the protocol in the International Prospective Register of Systematic Reviews (CRD42021235079) and reported the systematic review following the Preferred Reporting Items for Systematic Reviews and Meta- Analysis statement. Of the 278 screened articles, 6 full-text original articles satisfied the inclusion criteria and were included. There were a total of 2081 participants in these 6 retrospective studies and all studies except 1 included both adult and pediatric populations. Antibiotic therapy was given as intervention, either as single dose or multiple doses, and compared with other group(s) receiving either no antibiotic prophylaxis or a different duration of prophylaxis. Three studies did not find any significant difference between infection rates when a different duration of antibiotic prophylaxis was given, while 2 studies found a single dose to be more efficacious, and yet another study concluded that a longer dura- tion of antibiotic prophylaxis was more beneficial. Based on the available data, the ideal duration of post-operative antibiotic therapy to be given after cochlear implant surgery could not be defined. However, administrating a single dose of intraoperative antibiotic seems to be the most consistent practice so far.

Objectives: Sinonasal mucormycosis is a serious fungal infection. Early diagnosis and prompt antifungal therapy along with surgical intervention is the key to its management. Liposomal amphotericin B (LAmB) given intravenously is the antifungal agent of choice. However, the current literature is not clear on its optimum dosage. We did a retrospective study to find the optimum dose of LAmB in cases with sinonasal mucormycosis., Materials and Methods: Thirty patients diagnosed with mucormycosis involving sinonasal, rhino-orbital, or rhino-orbito-cerebral regions and receiving only LAmB as pharmacotherapy were included in our retrospective study from 2017 to 2020. A multiple logistic regression model was developed to correlate the total dose of LAmB and other parameters with the final outcome which was defined clinico-radiologically as improved, worsened, or death. The dose of LAmB which led to the first significant change in urea, creatinine, and potassium levels was also determined., Results: The model showed a good fit in goodness-to-fit analysis (Pearson = 0.999, deviance = 0.995), while the likelihood ratio was statistically significant (0.001). The overall model prediction was 83.3%. However, the correlation of outcome with any of the variables, including mean LAmB dose per kilogram (82.2 ± 13.02 mg/kg), was statistically not significant., Conclusion: Many patient-related factors (such as age, comorbidities, extent of the disease, and side effects from LAmB therapy), which vary on a case-to-case basis, contribute to the outcome in a mucormycosis patient. The optimum dose of LAmB for improved outcome still requires individualization guided by experience, till well-designed studies address the question., Competing Interests: None

Objectives: The lateralised vocal fold of unilateral vocal fold paralysis (ULVFP) is unphysiological for expiration wherein vocal folds normally adduct to increase expiratory resistance and prevent small airway collapse. ULVFP may therefore impair ventilatory function and ventilatory volume. This study seeks to test whether vocal fold medialisation improves forced vital capacity (FVC)., Design: Prospective inception cohort intervention study., Setting: Academic Tertiary Care Institution., Participants: Twenty-five patients of ULVFP with a phonatory gap ranging from 2 to 6 mm., Main Outcome Measures: Vocal fold medialisation was undertaken with autologous fat injection. Forced vital capacity (FVC) assessments by spirometry were undertaken pre-treatment and 1-month post-treatment., Results: Improvement in FVC was noted in all patients with the quantum of improvement ranging from 0.1 to 0.6 litres. Mean FVC improved from 3.10 L pre-injection to 3.45 L post-injection. (p < .001). A moderate correlation was noted between the degree of medialisation and improvement in FVC (r = .33, Pearson's correlation coefficient)., Conclusions: Objective improvement in FVC is consistently noted in post vocal fold medialisation for ULVFP and is probably mediated by increased glottic expiratory resistance and consequent improvement in intrinsic PEEP., (© 2021 John Wiley & Sons Ltd.)

Background: Juvenile nasopharyngeal angiofibroma is the vascular and locally aggressive tumor of the posterior nares and nasopharynx. Endoscopic excision is the preferred surgical modality in early Radkowski stages. The prime challenge is intraoperative bleeding. Literature is in favor of the use of plasma ablation to overcome surgical challenges., Objective: To evaluate the effectiveness of plasma ablation technique in the surgical management of juvenile nasopharyngeal angiofibroma., Materials and Methods: The current study recruited 36 patients of clinico-radiologically diagnosed cases of primary JNA of stage I and II (Modified Radkowski). In prospective arm, 18 consecutive patients were recruited, who underwent plasma ablation assisted endoscopic excision. In retrospective arm, 18 consecutive patients who underwent excision by traditional endoscopic instruments in the past two years, were recruited. Both the groups were compared for baseline characteristics, intraoperative blood loss, duration of surgery, length of hospital stay, and recurrence rates., Results: The use of plasma ablation decreased overall blood loss by 338 ml (mean difference) which was not statistically significant (p = 0.26). On subgroup analysis, the use of plasma ablation significantly decreased mean blood loss (648 ml, p = 0.046) and duration of surgery (83 min, p < 0.001) in patients who underwent embolization. No statistically significant difference was noted between two groups in length of hospital stay (p = 0.36) and recurrence rates (p = 0.64)., Conclusion: Plasma ablation is an effective technique available to decrease blood loss and duration of surgery during endoscopic excision of post embolized stage I and II patients of JNA., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Juvenile nasopharyngeal angiofibroma (JNA) is the most common benign tumor of the nasopharynx. For reasons unknown, this tumor is found almost always in male population. However, site of origin of JNA is still an enigma. Previously, JNA was considered to arise from the superior aspect of sphenopalatine foramen. Over last decade, the vidian canal was considered to be the more specific and likely site of origin. However, based on our observations, we believe this hypothesis to be anomalous as it does not explain major blood supply, pattern of skull base erosion in early stage of tumor and newer studies on electron microscopic and immunopathological findings. To explain these anomalies, we hypothesize the site of origin to be palato-vaginal canal., (Copyright © 2020. Published by Elsevier Ltd.)

Duplication anomalies of the internal auditory canal are rare, with only twenty-one cases reported in literature. These range from incomplete partition and complete partition to true duplication. We present three cases showing this entire spectrum of duplication abnormalities and discuss the role of imaging in the preoperative work up of such patients planned for cochlear implant., Competing Interests: Conflict of interestAll four authors declare that they have no conflict of interest.

Background: Aspiration pneumonia is an important cause of death in head and neck cancer patients. This study therefore aimed to evaluate the risk factors associated with aspiration pneumonia in head and neck cancer patients., Methods: Hospital death records from 12 years (2000-2012) were reviewed to obtain the number of deaths. Treatment details and cause of death were analysed. Statistical analysis was performed to identify the risk factors for aspiration pneumonia., Results: The records revealed that aspiration pneumonia was the cause of death in 51 out of 85 patients. Primary tumour site (oropharynx and hypopharynx, odds ratio 3.3; 95 per cent confidence interval 1.17-9.4, p = 0.02) and advanced tumour stage (odds ratio 4.2, 95 per cent confidence interval 1.16-15.61, p = 0.02) had significant negative impacts on aspiration pneumonia related mortality., Conclusion: Advanced pharyngeal cancer patients are at an increased risk of aspiration pneumonia related death. Investigations for the early detection of this condition are recommended in these high-risk patients.