To investigate genetic etiologies of conductive/mixed hearing loss (CHL/MHL) and guide management through genotype–phenotype correlations. Retrospective study of 98 patients (29 syndromic, 69 non-syndromic) with CHL/MHL. Clinical evaluations and next-generation sequencing were performed. Surgical outcomes and familial patterns were analyzed. Syndromic cases showed high genetic confirmation (96.6

BACKGROUND:Noise exposure (NE) disrupts blood-labyrinth barrier (BLB), damaging cell junctions and perivascular macrophage-like melanocytes (PVM/Ms), causing hair cell loss. OBJECTIVE:To determine vascular endothelial growth factor (VEGF)/pigment epithelium-derived factor (PEDF) signaling and PVM/Ms dysfunction contribute to BLB breakdown and repair following NE. MATERIAL AND METHODS:Proteomic profiling of the stria vascularis (SV) from control and NE mice was performed using Isobaric Tags for Relative and Absolute Quantitation (iTRAQ) and Parallel Reaction Monitoring (PRM). Immunofluorescence, qPCR, and ect. were used to assess changes in PVM/Ms distribution, VEGF/PEDF signaling, and junctional proteins. RESULTS:Nine altered proteins mapped to four functional modules: junctional stability, metabolic/vesicular regulation, ion homeostasis, and immune modulation. PPI-GO analysis identified 'regulation of cell junction assembly' as a pathway linking these proteins with VEGF/PEDF. NE induced an acute reduction in PVM/Ms coverage and early downregulation of VEGF, PEDF, and junctional proteins. VEGF and VEGFR2 peaked at 2 days after NE (NE2d), while PEDF and VEGFR1 peaked at 7 days after NE (NE7d), paralleling recovery of VE-Cadherin and ZO-1 and a compensatory rise in Occludin. CONCLUSIONS AND SIGNIFICANCE:NE triggers a coordinated, time-dependent disruption of the PVM/Ms-VEGF/PEDF-junctional protein axis, driving BLB instability and repair.

The extracellular matrix（ECM）is a complex network structure composed of collagen，glycoproteins，and proteoglycans.It not only provides structural support and viscoelasticity to tissues but also participates in cell signaling，responding to environmental forces and signals to mediate tissue remodeling in response to environmental cues. Due to the intricate and precise functions of the inner ear，the perception and transmission of sound rely on the complex interactions between cochlear cell structures and the ECM. In the inner ear，the ECM not only constitutes key structures such as the basilar membranes（BM）and tectorial membranes（TM），which are essential for sound perception，but also regulates cell shape，adhesion，and migration.Certain ECM components interact with cell surface receptors to activate signaling pathways that regulate gene expression.Additionally，the ECM modulates the storage and diffusion of ions and secreted factors, creating concentration gradients.These functions are critical for inner ear development，repair，and function.Thus，the ECM plays a vital role in auditory processes，and abnormalities in ECM are a cause of certain hereditary hearing loss.This review primarily summarizes the ECM genes that lead to hearing loss.

Active Facial nerve (FN) management, including decompression, end-to-end or rerouting anastomosis, and grafting consistently plays an important role in the surgical management of petrous bone cholesteatoma (PBC), and postoperative FN function is also a major concern for surgeons. The aim of this study is to analyze the long-term FN function outcomes in PBC patients with FN paralysis who have underwent different managements and to explore the prognostic factors. A retrospective analysis of 160 PBC patients with preoperative FN paralysis was conducted, and long-term FN function outcome was evaluated. Multivariate ordinal logistic regression models were used to determine the prognostic factors. 160 patients were collected. 102 males (63.75

Von Hippel–Lindau disease-associated endolymphatic sac tumors (VHL-associated ELSTs) present diagnostic challenges due to their rarity and nonspecific symptoms. This study describes clinical, pathological and genotypic features to guide treatment. We retrospectively analyzed seven patients with VHL-associated ELSTs. The mean age of otologic symptom [hearing loss (100%) and facial nerve paralysis (85.71%)] onset was 22.43 ± 8.68 years (range: 10–33). Surgical management included trans-labyrinthine and subtotal temporal bone resection approaches. Among three patients with severe preoperative facial nerve dysfunction, two underwent great auricular nerve grafting improved to House–Brackmann grade IV, while one receiving hypoglossal–facial nerve anastomosis reached grade V. Genetic testing identified pathogenic VHL gene missense mutations in three patients. Two female patients demonstrated disease progression during pregnancy. Literature analysis revealed exon-specific patterns: Exon 1 mutations correlated with cerebellar/spinal hemangioblastomas in female patients, while Exon 3 mutations were associated with multisystem tumors. These findings support that VHL-associated ELSTs manifest early with otologic symptoms and demonstrate exon-specific phenotypic patterns. Optimal management requires complete surgical resection, genetic diagnosis, and a multidisciplinary approach to address these complex tumors and achieve favorable outcomes.

Repeated low-intensity noise exposure is prevalent in industrialized societies. It has long been considered risk-free until recent evidence suggests that the temporary threshold shift (TTS) induced by such exposure might be a high-risk factor for hearing loss. This study was conducted to further investigate the manner in which repeated low-intensity noise exposure contributed to hearing damage. Two-month-old C57BL/6 J mice were exposed to white noise at 96 dB SPL for 8 h per day over 7 days to induce TTS. Auditory brainstem response (ABR) was monitored to assess changes in hearing thresholds, tracking the effects of noise exposure until the mice reached 12 months of age. Our results indicated that noise-exposed mice exhibited accelerated age-related hearing loss spanning from high to low frequencies. Proteomics analysis revealed an upregulation in the receptor for the advanced glycation end-products (RAGE) signaling pathway, which was associated with an activated inflammatory response, vascular injury, and mitochondrial and synaptic dysfunction. Further analysis confirmed increased levels of inflammatory cytokines in the cochlear lymph fluid and significant macrophages infiltration in the cochlear lateral wall, accompanied by hyperpermeability of the blood-labyrinth barrier. Additionally, degenerated mitochondria in the outer hair cells and decreased synaptic ribbons in the inner hair cells were also observed. These pathological changes indicated that noise exposure damages the cochlear cellular components, increasing the cochlear susceptibility to age-related stress. Our findings suggest that TTS caused by repeated low-intensity noise exposure correlates with a severe sensorineural hearing loss during aging; targeting the RAGE signaling pathway may be a promising strategy to mitigate damage from low-intensity noise and slow down the progression of age-related hearing loss.

Noise exposure is one of the most common causes of sensorineural hearing loss. Although many studies considered inflammation to be a major contributor to noise-induced hearing loss, the process of cochlear inflammation is still unclear. Studies have found that activation of the NF-κB signaling pathway results in the accumulation of macrophages in the inner ear plays an important role in hair cell damage. In this study, tandem mass tag (TMT) technique was used to analyze the changes in basilar membrane proteome expression before and after acoustic injury. After noise exposure, the nicotinamide adenine dinucleotide (NAD) metabolism level was decreased, and the NF-κB signaling pathway was activated. The expression of CD38, the main NAD hydrolase in mammals, may directly lead to inflammation onset. Then, anakinra, an IL-1 receptor blocker, and apigenin, a CD38 inhibitor, were administered to animals to protect against noise-induced hearing loss. Our results showed that anakinra had little influence on the hearing threshold shift, while apigenin significantly reduce the threshold shift of hearing by inhibiting the expression of NF-κB and CD38 can be a promising target for protecting against noise-induced hearing loss.

BACKGROUND:Adherens junction in the blood-labyrinth barrier is largely unexplored because it is traditionally thought to be less important than the tight junction. Since increasing evidence indicates that it actually functions upstream of tight junction adherens junction may potentially be a better target for ameliorating the leakage of the blood-labyrinth barrier under pathological conditions such as acoustic trauma. AIMS:This study was conducted to investigate the pathogenesis of the disruption of adherens junction after acoustic trauma and explore potential therapeutic targets. METHODS:Critical targets that regulated the disruption of adherens junction were investigated by techniques such as immunofluorescence and Western blotting in C57BL/6J mice. RESULTS:Upregulation of Vascular Endothelial Growth Factor (VEGF) and downregulation of Pigment Epithelium-derived Factor (PEDF) coactivated VEGF-PEDF/VEGF receptor 2 (VEGFR2) signaling pathway in the stria vascularis after noise exposure. Downstream effector Src kinase was then activated to degrade VE-cadherin and dissociate adherens junction, which led to the leakage of the blood-labyrinth barrier. By inhibiting VEGFR2 or Src kinase, VE-cadherin degradation and blood-labyrinth barrier leakage could be attenuated, but Src kinase represented a better target to ameliorate blood-labyrinth barrier leakage as inhibiting it would not interfere with vascular endothelium repair, neurotrophy and pericytes proliferation mediated by upstream VEGFR2. CONCLUSION:Src kinase may represent a promising target to relieve noise-induced disruption of adherens junction and hyperpermeability of the blood-labyrinth barrier.

Objectives This study aimed to establish the minimal clinically important difference (MCID) and assess the responsiveness of the Chinese version of Zurich Chronic Middle Ear Inventory (ZCMEI-21-Chn). Study Design Prospective multicenter study. Setting Four Chinese tertiary referral centers admitting patients nationwide. Patients 230 adult patients with chronic otitis media (COM) undergoing tympanoplasty. Intervention Patients were required to complete the ZCMEI-21-Chn to measure health-related quality of life both preoperatively and postoperatively. An anchor-based method was used to determine the MCID of the derivative cohort by including the Global Rating of Change Questionnaire as an anchor. The generalizability and consistency with functional outcomes of the MCID estimates were externally examined in a validation cohort using a receiver operating characteristic curve analysis. Results A total of 161 and 69 patients were included in the derivative and validation cohort. The mean preoperative and postoperative ZCMEI-21-Chn total scores were 28.4 (standard deviation [SD] 14.5) and 17.5 (SD 12.6). The mean change in ZCMEI-21-Chn score was 10.9 (SD 14.3, p < 0.001). The MCIDs of the ZCMEI-21-Chn for improvement and deterioration were estimated at 13 (SD 13.0) and −7 (SD 12.9), accordingly. For patients who have reported an improved health-related quality of life, a cutoff value of 15.6 dB HL for elevation of the air-conducted hearing threshold was noticed. However, change of clinical importance judged according to MCID and Japan Otological Society criteria disagreed with each other, notably with a Cohen's kappa (κ) of 0.14 (p = 0.21) in the validation cohort. Conclusion This study is the first to establish the MCID of a COM-specific questionnaire in Chinese. For the COM population undergoing surgical intervention, MCID values of 13 for improvement and −7 for deterioration are recommended. The results were externally validated to be generalizable to nationwide usage, yet distinguishable from the audiological criteria. The availability of the MCID greatly adds to the clinical utility of the ZCMEI-21-Chn by enabling a clinically meaningful interpretation of its score changes.

Objective:To investigate the characteristics and prognosis of two anastomosis techniques in repairing facial nerve defects. Methods:A retrospective analysis was conducted on 30 patients who underwent facial nerve anastomosis（direct or rerouting） for facial nerve defects in our department from January 2012 to December 2021. Among them, 21 were male and 9 were female, with an average age of（37.53±11.33） years, all with unilateral onset. Preoperative House-Brackmann（H-B） facial nerve function grades were Ⅳ in 2 cases, Ⅴ in 9 cases, and Ⅵin 19 cases. The duration of facial paralysis before surgery was within 6 months in 21 cases, 6-12 months in 6 cases, and over 1 year in 3 cases. The causes of facial paralysis included 14 cases of cholesteatoma, 6 cases of facial neurioma, 6 cases of trauma, and 4 cases of middle ear surgery injury. Surgical approaches included 9 cases of the middle cranial fossa approach, 8 cases of labyrinthine-otic approach, 7 cases of mastoid-epitympanum approach, and 6 cases of retroauricular lateral neck approach. Results:All patients were followed up for more than 2 years. The direct anastomosis was performed in 10 cases: 6 cases with defects located in the extratemporal segment and 4 cases in the tympanic segment. Rerouting anastomosis was performed in 20 cases: 11 cases with defects located in the labyrinthine-geniculate ganglion, 4 cases from the internal auditory canal to the geniculate ganglion, 3 cases in the internal auditory canal, and 2 cases in the horizontal-pyramid segment. Postoperative H-B facial nerve grades were Ⅱ in 2 cases, Ⅲ in 20 cases, and Ⅳ in 8 cases, with 73.3%（22/30） of patients achieving H-B grade Ⅲ or better. Conclusion:Both direct and rerouting anastomosis techniques can effectively repair facial nerve defects, with no significant difference in efficacy between the two techniques. Most patients can achieve H-B grade Ⅲ or better facial nerve function recovery. Preoperative facial nerve function and duration of facial paralysis are the main prognostic factors affecting the outcome of facial nerve anastomosis.

Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.

IntroductionSymptoms induced by arachnoid cysts in the fallopian canal are uncommon, and facial nerve paralysis without cerebrospinal fluid otorrhea is comparatively rarer.MethodsHerein, we present two cases of arachnoid cysts in the fallopian canal with acute severe facial nerve paralysis and review the relevant literature.ResultsThe symptoms and imaging findings of these two cases resembled those of facial nerve schwannomas. Cerebrospinal fluid otorrhea occurred upon removal of the arachnoid cyst, and the facial nerve was observed to be separated into multiple filaments or compressed and atrophied. Facial-hypoglossal nerve anastomosis and decompression were conducted after packing the dehiscence of cerebrospinal fluid otorrhea for the two cases.ConclusionArachnoid cysts of the fallopian canal rarely cause facial nerve paralysis. Enhanced magnetic resonance imaging is vital for differentiating schwannomas. Different treatment strategies should be adopted for patients with different degrees of facial nerve paralysis; however, concurrent repair of cerebrospinal fluid otorrhea and facial nerves during surgery can occasionally be challenging.

Purpose To review the resections of endolymphatic sac tumor (ELST) and describe our experience in the surgical management of ELST. Methods Retrospective investigation of consecutive patients who underwent resection of ELSTs at our hospital between 1999 and 2019. The symptoms, diagnosis, surgical findings, and outcomes were analyzed to develop a tumor staging system and corresponding surgical strategy. Results Retrospective review revealed the surgical treatment of 22 ELSTs. Based on intraoperative findings of tumor extent and size, ELSTs were classified into two types. Type-I ( n = 6) referred to the small tumors that were locally confined with limited invasion of semicircular canals and dura; type-II ( n = 16) referred to the large tumors that presented extensive erosion of at least one anatomic structure apart from the semicircular canals and the dura around endolymphatic sac. In this case series, Type-I ELST is amenable to resection through a transmastoidal approach, and subtotal petrosectomy is appropriate for the resection of type-II ELST. Sensorineural hearing loss (SNHL) is the most commonly preoperative symptom in both two types of cases. Five type-II ELSTs experienced recurrence and underwent reoperation, whereas all type-I ELSTs did not. Conclusion ELST usually results in SNHL (95%) at the time of diagnosis. The surgical strategy and prognosis of ELST resections are different between type-I and type-II: type-I ELST is amenable to transmastoidal approach with the preservation of facial nerve, whereas type-II ELST increase the surgical difficulty and the risk of recurrence, and subtotal petrosectomy is the basic requirement for the resection of type-II ELST.

目的 探讨经颅中窝入路颞骨岩部胆脂瘤切除的手术适应证、手术要点及面、听功能保护.方法 回顾性分析2006年1月至2021年1月我科经颅中窝入路手术治疗的颞骨岩部胆脂瘤病例的临床症状、体征、影像学特点、面神经修复方法、面、听功能保护、手术效果,总结分析颅中窝入路颞骨岩部胆脂瘤切除手术的适应证及手术要点.结果 接受颞骨岩部胆脂瘤外科切除的186例病例中,有56例患者采用颅中窝或颅中窝-乳突联合入路的手术方式.该组患者常见的临床症状分别为:听力下降(54/56,96.4%),面神经麻痹(42/56,75.0%)及耳漏(30/56,53.6%).依据Sanna分型:迷路上型39例(69.6%),广泛型11例(19.6%),岩尖型6例(10.7%).术前47例患者存在骨导听力,其中22例术后保留骨导听力,骨导听力保留率46.8%.手术涉及面神经的有36例(64.3%),其中20例行面神经减压术,1例行面神经端端吻合术,15例行面神经改道吻合术.术前面神经功能按House-Braekmann(H-B)法分级:Ⅰ级14例,Ⅱ级3例,Ⅲ级3例,Ⅳ级4例,Ⅴ级10例,Ⅵ级22例.术后面神经功能H-B分级:Ⅰ级15例,Ⅱ级11例,Ⅲ级11例,Ⅳ级9例,Ⅴ级1例,Ⅵ级9例.术后随访2-16年,1例患者术后5年复发,再次行手术切除.所有患者术后未发生硬膜外出血、颅内感染等并发症.结论 颅中窝入路是彻底切除迷路上、岩尖及部分广泛型岩部胆脂瘤、同时保留功能正常面神经和残留听力的最佳选择.面神经受损的患者可以依据损伤部位和程度的不同,采取减压、改道吻合或神经桥接移植以重建面神经功能.通过颅中窝入路切除岩部胆脂瘤,部分患者的听力有可能保留.

Background Iatrogenic facial nerve injury is one of the severest complications of middle ear surgery, this study aims to evaluate surgical management and prognosis in the era of improved surgical instruments. Methods Patients suffered from facial nerve paralysis after middle ear surgery between January 2000 and December 2019 were retrospectively collected. Demographic characters, primary disease and surgery, details of revision surgery were analyzed. Results Forty-five patients were collected, of whom 8 were injured at our center and 37 were transferred. For 8 patients injured at our center, seven (87.5%) ranked House-Brackmann (H-B) grade V and one (12.5%) ranked H-B VI before revision surgery; postoperatively, two (25.0%) patients recovered to H-B grade I, four (50.0%) recovered to H-B II, and the other two (25.0%) recovered to H-B III. For 37 patients transferred, thirteen (35.1%) ranked H-B grade V and 24 (64.9%) ranked H-B VI preoperatively, final postoperative grade ranked from H-B grade I to grade V, with H-B I 6 (16.2%) cases, H-B II 6 (16.2%) cases, H-B III 18 (48.6%) cases, H-B IV 5 (13.5%) cases and H-B V 2 (5.4%) cases. The most vulnerable site was tympanic segment (5, 62.5% and 27, 73.0% respectively). Twenty-one (46.7%) patients suffered from mild injury and 24 (53.3%) suffered from partial or complete nerve transection. For surgical management, twenty-one (46.7%) patients received decompression, nineteen (42.2%) received graft and 5 (11.1%) received anastomosis. Those decompressed within 2 months after paralysis had higher possibility of H-B grade I or II recovery ( P = 0.026), those received graft within 6 months were more likely to get H-B grade III recovery ( P = 0.041), and for patients underwent anastomosis within 6 months, all recovered to H-B grade III. Conclusions Tympanic segment is the vulnerable site. If facial nerve paralysis happens, high-resolution computed tomography could help identify the injured site. Timely treatment is important, decompression within 2 months after paralysis, graft and anastomosis within 6 months lead to better recovery.

Objective:To evaluate the effect of greater auricular nerve interposition grafting in the treatment of facial nerve defect, and explore the prognostic factors.Methods:The clinical data of patients who received greater auricular nerve interposition grafting in the Department of Otolaryngology Head and Neck Surgery of the Chinese PLA General Hospital from January 2010 to December 2021 were retrospectively collected, and efficacy of the surgery was evaluated. Univariate and multivariate ordinal logistic regression models were used to determine the prognostic factors.Results:Eighty-four patients were collected, of whom 39 (46.4%) were male and 45 (53.6%) were female, with an average age of (38.6±12.6) years. Facial nerve defect was caused by four kinds of primary diseases: benign facial nerve tumor (54 cases, 64.3%), other tumors (5 cases, 6.0%), iatrogenic facial nerve injury in middle ear surgery (19 cases, 22.6%), and cholesteatoma (6 cases, 7.1%). The median preoperative facial paralysis duration was 6 (1, 12) months. Preoperatively, there were 7 (8.3%) patients of House-Brackmann (HB) grade Ⅰ, 6 (7.1%) of grade Ⅱ, 11 (13.1%) of grade Ⅲ, 14 (16.7%) of grade Ⅳ, 19 (22.6%) of grade Ⅴ, and 27 (32.1%) of grade Ⅵ. Postoperatively, there were 46 (54.8%) patients recovered to grade Ⅲ, 26 (31.0%) to grade Ⅳ, and 12 (14.3%) to grade Ⅴ, and the overall postoperative HB grade declined compared with the preoperative HB grade ( Z=-4.264, P<0.001). For all kinds of primary diseases, higher preoperative HB grade ( OR=1.423, 95% CI: 1.031-1.966, P=0.032) and longer preoperative facial paralysis duration ( OR=1.020, 95% CI: 1.007-1.034, P=0.003) correlated with poorer prognosis. Gender, age, side, facial paralysis onset(sudden or progressive), facial electromyography result (fibrillation potential), surgical approach, location of proximal anastomosis, length of facial nerve defect, and anastomosis technique were not correlated with postoperative facial function grade (all P>0.05). Conclusions:The best outcome of great auricular nerve interposition grafting in the treatment of facial nerve defect is HB Ⅲ, which could be obtained by more than half of the patients. Higher preoperative HB grade and longer preoperative facial paralysis duration are risk factors to a poorer prognosis.

Objective:To elucidate the clinical manifestations, surgical management, nerve repair and outcomes of facial nerve hemangiomas.Methods:The data of patients with facial nerve hemangiomas in the Department of Otolaryngology-Head & Neck Surgery, Chinese PLA General Hospital from January 2010 to December 2021 were retrospectively analyzed. Appropriate surgical approach was selected based on the clinical manifestations, facial nerve function and imaging features of each patient. The facial nerve function was followed up after operation to analyze the therapeutic effect.Results:A total of 20 patients were in the final analysis, including 10 males and 10 females, with a mean age of (36.4±10.7) years (18-56 years). Facial nerve hemangioma successively manifested as facial paralysis (18 cases, 90.0%), hearing loss (11 cases, 55.0%), tinnitus (8 cases, 40.0%), and facial spasm (4 cases, 20.0%) according to the probability of onset. Intraoperative exploration showed that 65.0% (13/20) of the patients had multiple segments involvement, with the geniculate ganglion (16 cases, 80.0%), horizontal segment (11 cases, 55.0%) and labyrinthine segment (7 cases, 35.0%) of the facial nerve being most commonly involved. The tumor size and scope of involvement were determined according to the preoperative imaging examination, and the appropriate surgical approaches were selected based on hearing conditions, including the trans-mastoid approach in 5 cases, the trans-labyrinth approach in 5 cases, and the trans-middle fossa approach in 10 cases. Facial nerve repair was simultaneously performed in 19 patients, among which 2 patients underwent facial nerve re-route end-to-end anastomosis, 8 patients received great auricular nerve graft, 2 patients were subjected to facial nerve-hypoglossal nerve anastomosis, and 7 patients were subjected to facial nerve decompression. The House Brackmann (HB) grading scale was used to evaluate the facial nerve function. The longest follow-up time was 159 months, the shortest follow-up time was 17 months, and the average follow-up time was (81.9±39.3) months. There were 2 cases of grade Ⅰ (10.0%), 5 cases of grade Ⅳ (25.0%), 7 cases of grade Ⅴ (35.0%), and 6 cases of grade Ⅵ (30.0%) in the preoperative facial nerve function analysis. And 1 case of grade Ⅰ (5.0%), 10 cases of grade Ⅲ (50.0%), 8 cases of grade Ⅳ (40.0%), and 1 case of grade Ⅴ (5.0%) were in the postoperative facial nerve function analysis. The facial nerve function after operation was better than that before operation ( H=13.683, P<0.001). Conclusions:The possibility of facial nerve tumors should be taken into account under the condition of unknown facial paralysis, hearing loss and facial spasm. The characteristic CT images of the temporal bone are crucial for the diagnosis and differential diagnosis of facial nerve hemangioma. Surgical treatment and facial nerve repair are conducive to a favorable facial nerve function after surgery.

报告1例以分泌性中耳炎为首发表现的抗中性粒细胞胞质抗体（anti-neutrophil cytoplasmic antibodies，ANCA）相关肥厚性硬脑膜炎患者的诊断和治疗经过。患者青年女性，分泌性中耳炎后1个月出现头痛，头强化核磁提示弥漫性肥厚性硬脑膜炎，考虑免疫系统疾病，抗核抗体阴性、核周型抗中性粒细胞胞质抗体（P-ANCA）升高、脑脊液免疫球蛋白G升高，给予激素及环磷酰胺治疗有效，但在激素减量过程中出现病情反复，故给予生物制剂利妥昔单抗治疗，患者病情控制稳定。以分泌性中耳炎为首发表现的ANCA相关肥厚性硬脑膜炎病例临床少见，目前尚缺乏治疗指南或专家共识，在临床工作中，中耳炎后头痛的病例往往会首先想到颅内感染，从而忽略免疫系统疾病，故特此报道该病例以引起耳鼻喉科医师的关注。.

雷击是一种局部猛烈的自然灾害,雷击伤较少见,但破坏力强,能够损害人体多个器官组织,造成人身伤亡,其中听觉系统容易受到损伤[1,2].为提高临床工作中对雷击伤致聋的认识和诊治能力,本文回顾性总结了2019 年我院收治的2 例雷击伤致聋患者,结合国内外相关文献,对雷击伤致聋的临床诊治复习讨论如下: 1 临床资料 病例1:患者女,46 岁,因"雷击致伤后21 小时"入院.

PURPOSE:To demonstrate our experience in the treatment of petrous bone cholesteatoma (PBC).METHODS:Data of PBC patients in our hospital from January 2000 to December 2019 were collected. Surgical approaches and facial function were mainly discussed and compared with the literature. The management of 2 giant PBC cases affecting rhinopharynx has been demonstrated.RESULTS:The supralabyrinthine type was the most frequent type followed by the massive type. There were 5 cases with cholesteatoma extending into the clivus (2 cases), sphenoid (1 case) and rhinopharynx (2 cases). The translabyrinthine approach (40%) was our most frequently used approach followed by the middle fossa approach (36%) and the transmastoid approach (11%). There were 10 cases managed with the assistance of endoscope, including 3 cases with cholesteatoma extending into clivus, sphenoid and rhinopharynx separately. Obliteration of the cavity was performed in 70.3% (135/192) cases; 3 of them recurred. For the 2 giant PBC cases affecting rhinopharynx, traditional microscopic surgery assisted with transnasal endoscope was performed. The reduced exposure was beneficial for postoperative recovery, and the approach in the nasal cavity provided a permanent drainage for postoperative examination.CONCLUSION:Otologic endoscope combined with traditional microscopic surgery could reduce the exposure in surgery. For extremely extended cases of PBC, supplementary transnasal endoscopic approach deserves to be considered for the traditional temporal bone approach.