Tonsil microbiome in pediatric patients with post tonsillectomy hemorrhage for tonsillar hypertrophy.

OBJECTIVE: This study aimed to compare the tonsillar microbiota between post tonsillectomy patients with bleeding and without bleeding, and to investigate the potential role of tonsillar microbiota in the development of post-tonsillectomy hemorrhage (PTH). METHODS: Nineteen tonsillar tissues from PTH patients and 21 tissues from control patients were collected. Metagenomic sequencing was used to compare the microbiota in PTH and control groups. Alpha diversity indices were used to compare the richness and evenness of the microbiota between the two groups. PCoA and NMDS analyses were used to evaluate beta diversity. LDA analysis was conducted to identify significantly abundant genera. RESULTS: No significant difference in alpha diversity indices was found between PTH and control patients. The dominant bacteria in the tonsillar microbiota were Haemophilus, Streptococcus, and Fusobacterium. PCoA and NMDS analyses showed significant differences in beta diversity between PTH and control patients. PTH patients had a significantly higher relative abundance of Neisseria, Capnocytophaga, and Veillonella. Capnocytophaga was also identified as a significantly abundant genus by LDA analysis. CONCLUSION: This study demonstrates that there is a difference in the tonsillar microbiota between PTH and control patients. The results suggest that Neisseria, Capnocytophaga, and Veillonella may be associated with the development of PTH. These findings provide new insights into the potential role of the tonsillar microbiota in the development of PTH, and may help to develop new strategies for preventing and treating this potentially life-threatening complication.

[Diagnosis and treatment of disorders of laryngeal airway in children and prospect].

This paper focuses on the diagnosis and treatment of disorders of laryngeal airway in children, including congenital anomalies, infection, and tumor of the larynx to provide a new technology for managing these diseases. Based on the characteristic of them, the pediatric upper airway is dedicated to the clinical evaluation of airway obstruction and the assessment of the compromised pediatric airway, including clinical evaluation of symptoms, diagnostic endoscopy, and imaging examination. Information on endoscopic techniques used for dealing with different degrees of pediatric airway comprised is provided, also this techniques could diagnose what kinds of airway disorder. For example, determining the a particular laryngeal cleft at the initial otolaryngology encounter, flexible laryngoscopy should be performed. In order to define the extent of any identified cleft, the rigid bronchoscopy should be completed to evaluate for classification of laryngeal cleft including typeⅠ, Ⅱ, Ⅲa, Ⅲb, Ⅳa, Ⅳb under general anesthesia. The decision to pursue any therapy for the disorders of laryngeal airway in children should be based on the severity of the patient's symptoms, endoscopic and imaging examination. There are two medical treatments including nonsurgical therapy and surgical therapy. For example the laryngeal cleft, approximately half of type 1 and select type 2 patients can be managed entirely with nonsurgical therapy. Medical management is multifaceted. Under recommendations from the feeding team, a modified diet with thickened feeds and possibly altered feeding position should be initiated. Endoscopic surgical repair is the current gold standard for definitive repair of type 1, the majority of type 2, and selected type 3 clefts. Finally, information on new techniques used into the future for dealing with the diagnosis and treatment of disorders of laryngeal airway in children in this paper.

[Clinical analysis of 102 cases of pediatric vocal fold nodules].

Objective:To evaluate the impact of vocal fold nodules on the quality of life of children, and to compare the efficacy between conservative management（voice hygiene education, etc.） and laryngeal microsurgery. Methods:A retrospective study was performed on 102 children with vocal fold nodules, who received treatment in Children's Hospital of Fudan University during the period from January 2020 to December 2022. According to the regime, the patients were divided into conservative management group and surgical treatment group; Cases are divided into pre-school age group （2-5 years old） and school-age group （6-12 years old） based on age. The pediatric voice-related quality of life （pVRQOL） was used to evaluate the impact of vocal fold nodules on the quality of life of children and differences in efficacy between two treatment regimens. Results:The pVRQOL scores of vocal fold nodule grades 1, 2, and 3 were 91.58±8.17, 78.87±12.49, 72.50±12.08, respectively. There were statistical differences between grade 1 and grade 2, and between grade 2 and grade 3. There were statistical differences between grade 1 grade 2, grade 2 and grade 3 （P<0.001）, suggesting that the higher the rating of vocal cord nodules, the lower the pVRQOL score of patients was. The pVRQOL score of the school-age group was 69.83±11.11, which is lower than that of the preschool group（87.59±8.63）, and the difference was statistically significant （P=0.042）. Vocal fold nodules had a significant impact on the pVRQOL score of school-age children. In the conservative management group, the pVRQOL scores before and after treatment were 83.99±12.66 and 87.26±9.58, respectively, and there was significant difference between the two groups（P=0.046）. Indirect voice therapy such as voice hygiene education could improve children's pVRQOL scores and was more effective for school-age children（P<0.001）. The microsurgical treatment had a more significant improvement in the pVRQOL score of children. Conclusion:The higher the rating of vocal fold nodules, the greater the impact on the quality of life of patients. Vocal fold nodules had a greater impact on the quality of life of school-age children than that of preschool children. Voice hygiene education could improve the quality of life of children, which was more evident in the school-age group. Compared with voice hygiene therapy, laryngeal microsurgery had a more significant effect on improving the pVRQOL score of patients. It is recommended to undergo laryngeal microsurgery for the treatment of vocal fold nodules for the requirement of improving the quality of life related to voice. During the 6-month follow-up period after surgery in this study, the short-term efficacy of laryngeal microsurgery in treating vocal cord nodules in children is clear, but the long-term efficacy needs further research.

[New progress in the surgical treatment of laryngeal cyst in infants and children].

Laryngeal cyst is a cystic lesion occurring in the laryngeal cavity. Large laryngeal cyst in infants and young children can cause laryngeal wheezing and other upper airway obstruction symptoms. In severe cases, it can be even life-threatening and requires timely surgical treatment. Currently, there is a lack of unified clinical treatment strategy for this disease.This article summarizes the surgical methods, the advantages and disadvantages of various surgical methods for laryngeal cysts in recent years. It is recommended that needle aspiration, partial cyst wall resection, radical cyst dissection, transoral robotic surgery or external approach cyst resection should be selected through full communication and evaluation to clarify the extent of the lesion scope and the advantages and disadvantages of surgery.

Clinical Features and Outcomes of Omicron-Associated Croup in Children.

Objective: This study was aimed to describe the clinical features and outcomes of Omicron-associated croup in children. Methods: A total of 105 children aged 3-60 months (median age 11 months) with coronavirus disease 2019 (COVID-19) and cough were admitted to our hospital from December 16, 2022, to December 31, 2022. Their clinical features, treatment, and outcomes were reviewed. These children were followed up for 8 months. Results: Among the cases, 5 had complex medical histories, while the other children were generally healthy. Out of the cases, 41 (38.3%), 57 (54.4%), and 7 (6.7%) had mild, moderate, and severe laryngeal obstruction, respectively. They developed croup after 1-5 days of fever (median 2 days). The majority (90.5%) of COVID-19 children with croup did not have pneumonia, and most of them (64.8%) had decreased eosinophil counts. Additionally, most other blood routine indicators were normal. Five other viral infections (chlamydia pneumoniae, respiratory syncytial virus, adenovirus, coxsackie virus, and mycoplasma pneumoniae) were tested in 51 cases, and all results were negative. All cases recovered from croup after receiving prompt nebulization therapy and/or intravenous drip of hormone. The hospitalization rate was 6.7%. During the 8-month follow-up period, 71 cases experienced repeated fever due to various infections, but only 4 cases (4/105, 3.8%) had repeated croup. Conclusion: Omicron is a risk factor for croup, with most cases presenting mild to moderate laryngeal obstructions. Co-viral infection testing is unnecessary for such cases. The symptoms of Omicron-associated croup may be more severe than croup associated with other viruses; however, unnecessary hospitalizations can be avoided, leading to reduced healthcare expenses.

[The consensus among experts on the diagnosis and treatment of pediatric vocal cord paralysis].

Pediatric vocal ford paralysis is a vocal cord movement disorder caused by damage to the pediatric laryngeal motor nerves.It is mainly characterized by voice, breathing,and swallowing difficulties,and in severe cases,it can lead to choking in affected children. Currently, the diagnosis and treatment of this condition pose a significant challenge for pediatric otolaryngologists, as the goal is to minimize damage to the vocal folds and laryngeal framework.In order to standardize the diagnosis and treatment of pediatric vocal cord paralysis, the Pediatric Otolaryngology Committee of the Chinese Medical Association,in collaboration with multiple children's medical centers nationwide, have formulated this consensus document.

[Consensus recommendations on the evaluation and treatment of congenital laryngeal clefts].

Congenital laryngeal cleft is a rare airway malformation, mainly manifested as choking, feeding difficulties, which affects the growth and development of children. Patients with a severe laryngeal cleft may have recurrent aspiration, leading to cyanotic spells, or even death. Advances in development of endoscopic techniques have made early diagnosis possible. Depending on the degree of cleft, management may involve a variety of approaches ranging from medical management alone to open repair. Therefore, it is important for pediatric ENT doctors to diagnose and evaluate in clinical practice. This consensus statement, developed by the Pediatric otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance on diagnosis and management of laryngeal cleft, based on symptomatology, physical examinations, and laboratory tests.

An integrative analysis of the tumor suppressors and oncogenes from sexual dimorphism and gene expression alteration features in thyroid cancer.

BACKGROUND: The incidence of thyroid cancer has risen rapidly over the last decades. Although mortality rates are relatively low compared to other cancers, the rate of new cases started to increase in the early 2000s. While tumor suppressors and oncogenes were recently identified in thyroid cancer, the potential roles of these genes in thyroid cancer remain unclear. OBJECTIVE: Analyze the roles and functions of tumor suppressors and oncogenes in thyroid cancer. METHODS: Thyroid cancer data were collected from public databases, such as the UCSC Xena database of TCGA thyroid cancer, TISIDB, and UALCAN. The genes frequently associated with unfavorable thyroid cancer were examined and validated. The association of these target genes with thyroid tumorigenesis, stages, subtypes, and survival rates were analyzed. Additionally, the genes aberrantly expressed in thyroid cancer and significantly involved in thyroid tumorigenesis, stages, subtypes, and survival rates were identified. RESULTS: Female sex was identified as a risk factor for thyroid cancer. The expression of PAPSS2, PDLIM3, COPZ2, ALDH1B1, ANTXR1, GUF1, and SENP6 negatively correlated with thyroid cancer prognosis. CONCLUSION: Female sex was a risk factor for thyroid cancer. In addition, our analysis suggested that PAPSS2, PDLIM3, COPZ2, ALDH1B1, ANTXR1, GUF1, and SENP6 are negatively correlated with the prognosis of thyroid cancer. The expression of ANTXR1, GUF1, and PDLIM3 was weakly associated with thyroid cancer's immune and molecular subtypes.

Short-term outcomes of infants with hyperbilirubinemia-associated auditory neuropathy spectrum disorder in neonatal intensive care unit.

OBJECTIVES: Hyperbilirubinemia is a high-risk factor for auditory neuropathy spectrum disorder (ANSD) as well as hearing loss in general. This study described the outcomes of hyperbilirubinemia-associated ANSD infants diagnosed in hearing screening in the neonatal intensive care unit (NICU). METHODS: A total of 578 children with hyperbilirubinemia admitted to the NICU between October 2020 and October 2021 were included in this study. The distortion product otoacoustic emission (DPOAE) and automatic auditory brainstem response (AABR) were combined for hearing screening, and those who failed the DPOAE or/and AABR underwent an auditory brainstem response (ABR) test. Infants with ANSD were followed up for 12 months. RESULTS: Forty infants (40/578, 6.9%) failed the DPOAE or/and AABR tests, of which, 13 (13/578, 2.2%) were diagnosed as ANSD, and 27 (27/578, 4.7%) were diagnosed as having sensorineural hearing loss (SNHL). Of the 13 ANSD infants followed up for 12 months, 7 recovered, 3 improved, 3 did not recover, and 1 was lost, equating to improved or recovered hearing in 75% (9/12) of ANSD infants at 12 months of age. Moreover, the maximum bilirubin in recovered or improved ANSD infants was 408.6 ± 129.0 µmol/L, while the maximum bilirubin in unrecovered ANSD infants was 749.3 ± 323.0 µmol/L. Furthermore, poorly differentiated and absent ABR waveforms were observed in 6 and 14 ears at 1 month, 2 ears were lost, 6 (6/6, 100.0%) and 6 (6/12, 50.0%) ears were recovered or improved at 12 months of age. CONCLUSION: s: The incidence of hyperbilirubinemia associated-ANSD was 2.2% of infants screened in the NICU. ANSD caused by hyperbilirubinemia may be transient, with most infants improving or recovering hearing by 12 months of age. Infants with poorly differentiated ABR waveforms and low bilirubin concentration are more likely to recover and hearing aids are not recommended in hyperbilirubinemia-associated ANSD below 12 months of age.

[Consensus recommendations on the evaluation and treatment of laryngotracheal anomalies in infants and young children].

Infants with laryngotracheal anomalies are clinically manifested as stridor or noisy breathing, choking, hoarseness, feeding difficulties, and cyanotic spells, followed by developmental and growth retardation and other health issues; in severe cases, patients may present with severe dyspnea, which is associated with high mortality. A timely diagnosis as well as appropriate strategy for laryngotracheal anomalies is still challenging for pediatric otolaryngologists. This consensus statement, evolved from expert opinion by the members of the Pediatric Otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance for otolaryngologists who manage infants and young children with laryngotracheal anomalies in evaluation and treatment based on symptomatology, physical and laboratory examinations.

Deep learning for the screening of primary ciliary dyskinesia based on cranial computed tomography.

Objective: To analyze the cranial computed tomography (CT) imaging features of patients with primary ciliary dyskinesia (PCD) who have exudative otitis media (OME) and sinusitis using a deep learning model for early intervention in PCD. Methods: Thirty-two children with PCD diagnosed at the Children's Hospital of Fudan University, Shanghai, China, between January 2010 and January 2021 who had undergone cranial CT were retrospectively analyzed. Thirty-two children with OME and sinusitis diagnosed using cranial CT formed the control group. Multiple deep learning neural network training models based on PyTorch were built, and the optimal model was trained and selected to observe the differences between the cranial CT images of patients with PCD and those of general patients and to screen patients with PCD. Results: The Swin-Transformer, ConvNeXt, and GoogLeNet training models had optimal results, with an accuracy of approximately 0.94; VGG11, VGG16, VGG19, ResNet 34, and ResNet 50, which are neural network models with fewer layers, achieved relatively strong results; and Transformer and other neural networks with more layers or neural network models with larger receptive fields exhibited a relatively weak performance. A heat map revealed the differences in the sinus, middle ear mastoid, and fourth ventricle between the patients with PCD and the control group. Transfer learning can improve the modeling effect of neural networks. Conclusion: Deep learning-based CT imaging models can accurately screen for PCD and identify differences between the cranial CT images.

TNFSF15 and MIA Variant Associated with Immunotherapy and Prognostic Evaluation in Esophageal Cancer.

Background: Esophageal cancer (ESCA) is a common gastrointestinal tumor, and China is one of the regions with a high incidence. Tumor immune-related cells play important roles in the tumorigenesis and development of ESCA. However, the role of tumor immune-related genes in the development of ESCA has not been established. Methods: In this study, weighted gene coexpression network analysis (WGCNA) was used to analyze ESCA gene expression using data from The Cancer Genome Atlas (TCGA) database. Gene expression was associated with clinical traits, and modules related to CD8+T cells, dendritic cells, and regulatory T cells (Tregs) were obtained. Results: The GO analysis showed that inflammatory chemotaxis networks were activated by cell chemotaxis, chemokine activity, and chemokine binding receptor. Three hub genes (IL17C, TNFSF15, and MIA) related to tumor immunity and metastasis were identified by WGCNA, and the abnormal expression of each hub gene in ESCA has a poor prognosis, especially in patients with high expression (P < 0.05). The risk assessment analysis also showed that tumor stage was positively correlated with tumor risk in ESCA (P < 0.05). Therefore, more than 50 pairs of tumor tissues from the T1-T3 stages with different degrees of differentiation and paracancerous tissues were selected to confirm the expression of the three genes using RT-qPCR and immunofluorescence (IF). The infiltration of CD8+ T cells in tumor tissues was lower than that in normal tissues. According to the RT-qPCR, the expressions of IL17 C, TNFSF15, and MIA in moderately and poorly differentiated tissues were significantly higher than those in normal tissues (P < 0.05). In contrast, their expressions were decreased in high differentiated tissues (P < 0.05). Furthermore, IL17C, TNFSF15, and MIA were all positively correlated with immune checkpoint PD-1; TNFSF15 and MIA were also positively correlated with CTLA4, TIGIT, and CD96. Conclusion: In summary, IL17C, TNFSF15, and MIA may act as biomarkers for prognosis in moderately and poorly differentiated ESCAs, and they may be used as predictive genes of immunotherapy associated with CD8+ T cell and Tregs invasion in ESCAs.

COX-2/PGE2 upregulation contributes to the chromosome 17p-deleted lymphoma.

Deletions of chromosome 17p, where TP53 gene locates, are the most frequent chromosome alterations in human cancers and associated with poor outcomes in patients. Our previous work suggested that there were p53-independent mechanisms involved in chromosome 17p deletions-driven cancers. Here, we report that altered arachidonate metabolism, due to the deficiency of mouse Alox8 on chromosome 11B3 (homologous to human ALOX15B on chromosome 17p), contributes to the B cell malignancy. While the metabolites produced from lipoxygenase pathway reduced, chromosome 11B3 deletions or Alox8 loss, lead to upregulating its paralleling cyclooxygenase pathway, indicated by the increased levels of oncometabolite prostaglandin E2. Ectopic PGE2 prevented the apoptosis and differentiation of pre-B cells. Further studies revealed that Alox8 deficiency dramatically and specifically induced Cox-2(Ptgs2) gene expression. Repressing Cox-2 by its shRNAs impaired the tumorigenesis driven by Alox8 loss. And, in turn, tumor cells with Alox8 or 11B3 loss were sensitive to the COX-2 inhibitor celecoxib. This correlation between COX-2 upregulation and chromosome 17p deletions was consistent in human B-cell lymphomas. Hence, our studies reveal that the arachidonate metabolism abnormality with unbalanced ALOX and COX pathways underlies human cancers with 17p deletions and suggest new susceptibility for this disease.

Auditory and Speech Outcomes of Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Systematic Review and Meta-Analysis.

OBJECTIVE: The aim of this meta-analysis was to critically assess the effect of cochlear implantation on auditory and speech performance outcomes of children with auditory neuropathy spectrum disorder (ANSD). MATERIAL AND METHODS: A systematic literature search was conducted on PubMed, EMbase, and Web of Science. The outcomes included speech recognition score, Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR) score, and open-set speech perception. Results were expressed as standardized mean difference (SMD) or risk ratio (RR) with a 95% confidence interval (95% CI). RESULTS: A total of 15 studies was included in this meta-analysis. Pooled data showed that, there were no significant differences between ANSD and sensorineural hearing loss (SNHL) groups in terms of speech recognition score (SMD = 0.01, 95% CI: -0.45, 0.47; P = .959),CAP (SMD = 0.71, 95% CI: -0.13, 1.54; P = .098), SIR score (SMD = -0.09, 95% CI: -0.49, 0.32; P = .667), and open-set speech perception (RR = 0.85, 95% CI: 0.69, 1.05; P = .142). Sensitivity analysis by removing individual studies one at a time showed that the overall estimate and level of heterogeneity did not change substantially. CONCLUSION: The current evidence suggested that children with ANSD who underwent cochlear implants achieved comparable effects in auditory and speech performance as children with non-ANSD SNHL.

CT imaging features of paranasal sinuses in children with primary ciliary dyskinesia.

BACKGROUND: Primary ciliary dyskinesia (PCD) causes impaired mucociliary clearance and results in chronic pulmonary and sinonasal symptoms. OBJECTIVES: To study the CT imaging features of paranasal sinuses in children with PCD. MATERIALS AND METHODS: 17 PCD patients ranged from 4 to 13 years, a mean age of 7.9 ± 3.3 years, were included in the final analysis. Patients with non-PCD chronic rhinosinusitis (CRS) who accepted maxillary balloon catheter dilation were included in the control group. Paranasal sinuses CT scans were scored according to the Lund-Mackay staging system. The correlation between age and Lund-Mackay score was analyzed. RESULTS: 100% (17/17) had rhinosinusitis, 52.9% (9/17) had lung consolidation, 64.7% (11/17) had atelectasis, 35.3% (6/17) had bronchiectasis, and 47.1% (8/17) had a history of neonatal respiratory distress. The mean Lund-Mackay score of PCD patients was 14.2 ± 3.1, that of non-PCD CRS patients was 14.6 ± 5.5, the difference was not significant (p = .79). There was a significant inverse correlation between age and Lund-Mackay score in PCD patients (r = -0.530, p = .029) but not in non-PCD CRS patients (r = -0.168, p = .519). CONCLUSION: Radiographic severity of rhinosinusitis in PCD patients was similar to the control population but decreased with age. SIGNIFICANCE: First time to propose radiographic severity of rhinosinusitis in pediatric patients with PCD might decrease with age.

Ultrasound-guided microwave ablation in the treatment of early-stage tongue cancer.

Background: Tongue cancer is a common malignant tumor of the head and neck. Its treatment methods include surgery, radiotherapy, and chemotherapy. However, these treatments have serious side effects and poor cosmetic effect, so it is urgent to find new treatment methods. We pioneered the use of microwave ablation (MWA) in the treatment of early tongue cancer and achieved good results. Case Presentation: A 67-year-old woman (Han nationality) was admitted to the hospital because of progressive aggravation of tongue pain. She had a history of tongue pain of more than 1 year. Pathological biopsy showed squamous cell carcinoma; following this, radical operation of the tongue cancer was planned. The preoperative examination showed thyroid occupation in the upper mediastinum region compressing the airway; hence, the risk of general anesthesia was high. Consent was obtained from the patient and her family. Ultrasound-guided MWA was successfully performed under the lingual nerve block. The patient was followed for 1 year. She recovered well with no dysphagia and unclear articulation symptoms, and the cosmetic effect was excellent. Conclusion: To our knowledge, this is the first case of using MWA for the treatment of early-stage tongue cancer (ESTC). Ultrasound-guided MWA may be used for ESTC that can completely ablate the tumor and retain the function of the tongue, further improving the quality of life of the patient. However, it is only a case report and needs more research to verify the use of MWA in ESTC.

Temporal bone CT-based deep learning models for differential diagnosis of primary ciliary dyskinesia related otitis media and simple otitis media with effusion.

OBJECTIVE: To investigate the diagnostic value of deep learning (DL) in differentiating otitis media (OM) caused by otitis media with effusion (OME) and primary ciliary dyskinesia (PCD), so as to provide reference for early intervention. METHODS: From January 2010 to January 2021, 31 patients with PCD who had temporal bone computed tomography (TBCT) in the Children's Hospital of Fudan University were retrospectively analyzed. Another 30 age-matched cases of OME with TBCT were collected as the control group. The CT imaging signatures of children were observed. Besides, a variety of DL neural network training models were established based on PyTorch, and the optimal models were trained and selected for PCD screening. RESULTS: The google net-trained model worked best, with an accuracy of 0.99. Vgg16_bn, vgg19_bn, resnet18, and resnet34; having neural networks with fewer layers, better model effects, with an accuracy rate of 0.86, 0.9, 0.86, and 0.86, respectively. Resnet50 and other neural networks with more layers had relatively poor results. CONCLUSION: DL-based CT radiomics can accurately distinguish OM caused by OME from that induced by PCD, which can be used for screening the PCD.

An in-depth discussion of cholesteatoma, middle ear Inflammation, and langerhans cell histiocytosis of the temporal bone, based on diagnostic results.

Objective: This study aimed to conduct an in-depth investigation of the learning framework used for deriving diagnostic results of temporal bone diseases, including cholesteatoma and Langerhans cell histiocytosis (LCH). In addition, middle ear inflammation (MEI) was diagnosed by CT scanning of the temporal bone in pediatric patients. Design: A total of 119 patients were included in this retrospective study; among them, 40 patients had MEI, 38 patients had histology-proven cholesteatoma, and 41 patients had histology-proven LCH of the temporal bone. Each of the 119 patients was matched with one-third of the disease labels. The study included otologists and radiologists, and the reference criteria were histopathology results (70% of cases for training and 30% of cases for validation). A multilayer perceptron artificial neural network (VGG16_BN) was employed and classified, based on radiometrics. This framework structure was compared and analyzed by clinical experts according to CT images and performance. Results: The deep learning framework results vs. a physician's diagnosis, respectively, in multiclassification tasks, were as follows. Receiver operating characteristic (ROC) (cholesteatoma): (0.98 vs. 0.91), LCH (0.99 vs. 0.98), and MEI (0.99 vs. 0.85). Accuracy (cholesteatoma): (0.99 vs. 0.89), LCH (0.99 vs. 0.97), and MEI (0.99 vs. 0.89). Sensitivity (cholesteatoma): (0.96 vs. 0.97), LCH (0.99 vs. 0.98), and MEI (1 vs. 0.69). Specificity (cholesteatoma): (1 vs. 0.89), LCH (0.99 vs. 0.97), and MEI (0.99 vs. 0.89). Conclusion: This article presents a research and learning framework for the diagnosis of cholesteatoma, MEI, and temporal bone LCH in children, based on CT scans. The research framework performed better than the clinical experts.

Association Between Expanded Genomic Sequencing Combined With Hearing Screening and Detection of Hearing Loss Among Newborns in a Neonatal Intensive Care Unit.

Importance: Hearing loss is a global social burden. Early identification of hearing loss missed by newborn hearing screening tests in the neonatal intensive care unit is crucial. Objective: To assess the association between expanded genomic sequencing combined with hearing screening and detection of hearing loss as well as improvement in the neonatal intensive care unit. Design, Setting, and Participants: This cohort study was performed between August 8, 2016, and December 31, 2020, among 8078 newborns admitted to the neonatal intensive care unit of the Children's Hospital of Fudan University in Shanghai, China. Follow-up for hearing status was performed via telephone interviews between September 1 and November 30, 2021. Exposures: A hearing screening test and the expanded genomic sequencing targeting 2742 genes were administered to each patient. Those who failed the hearing screening test or had positive genetic findings were referred for diagnostic audiometry at a median of 3 months of age. Main Outcomes and Measures: The primary outcome was hearing loss missed by hearing screening test. Secondary outcomes were genetic findings and benefits associated with the expanded genomic sequencing for clinical management of patients in the neonatal intensive care unit. Results: Of 8078 patients (4666 boys [57.8%]; median age, 6.3 days [IQR, 3.0-12.0 days]), 52 of 240 (21.7%) received a diagnosis of hearing loss. Expanded genomic sequencing combined with hearing screening was associated with a 15.6% increase (7 of 45 patients) in cases of diagnosed hearing loss that were missed by hearing screening. Of the 52 patients with hearing loss, genetic factors were identified for 39 patients (75.0%); GJB2 and SLC26A4 were the most common genes identified. Patients with genetic findings experienced a more severe degree of hearing loss than those without genetic findings (21 profound, 4 severe, 7 moderate, and 7 mild vs 2 severe, 4 moderate, and 7 mild; P = .005), with more bilateral hearing loss (39 of 39 [100%] vs 9 of 13 [69.2%]; P = .003). Clinical management strategies were changed for patients who underwent genomic sequencing combined with hearing screening. Conclusions and Relevance: This study suggests that expanded genomic sequencing combined with hearing screening may be effective at detecting hearing loss among patients in the neonatal intensive care unit.

Imaging and audiological features of children with cochlear nerve deficiency.

Objective: Cochlear nerve deficiency (CND) accounts for 10-19% of hearing loss in children; this study investigated the imaging and audiological features of 25 CND children. Methods: A total of 563 children with an unpassed automatic auditory brainstem response were diagnosed with hearing loss in our department between December 2018 and December 2021, of which, the imaging and audiological features of 25 children (25/563, 4.4%) diagnosed with a CND were reviewed. Results: Twenty-one (21/25, 84.0%) CND children had unilateral deafness, and 4 cases of bilateral deafness. All deaf ears were diagnosed as severe hearing loss due to an auditory brainstem response. CM waves were recorded in 8 cases and DPOAE in 3 cases, suggesting the audiological characteristics of auditory neuropathy spectrum disorders (ANSD). MRI results indicated 23 cases had small cochlear nerves and 2 cases had absent cochlear nerves. No genetic mutations were identified in the 25 CND children. Conclusions: Most CND children had unilateral hearing loss; therefore, high-resolution MRI imaging of the internal auditory canal should be performed to detect the auditory nerve in children with severe hearing loss. Some CND children had characteristics of ANSD.