BACKGROUND:This study aims to identify a pathogenic SDHD mutation associated with hereditary head and neck paraganglioma (HNPGL) in a Chinese family and to explore its implications for genetic counseling. METHODS:The study involved a family with 15 members spanning three generations. A 31-year-old patient (II-4) was diagnosed with a left parotid gland tumor and a right carotid body tumor, while both the father and elder sister had right carotid body tumors, and the third sister had bilateral carotid body tumors. Whole exome sequencing and Sanger sequencing were employed to identify candidate pathogenic variants. Genetic counseling was conducted for third-generation descendants to assess the likelihood of carrying the mutation and to guide future diagnosis and treatment. RESULTS:A nonsense mutation in the SDHD gene (NM_001276503:exon2:c.C64T: p.R22X) was identified in the patient and three other affected family members. Genetic counseling for the third generation revealed that only one child (III-4) carried the pathogenic mutation inherited from the patient's third sister. CONCLUSION:We identified a pathogenic mutation in SDHD in a Chinese HNPGL family, which is the second reported case of its kind. Our genetic counseling analysis for the third generation provided important information for the family and guidance for future diagnosis and treatment.

The present study aimed to describe the clinical features and survival of patients with temporal bone malignant tumors. A total of 20 consecutive cases treated at Beijing Tiantan Hospital (Beijing, China) between March 2014 and March 2022 were retrospectively reviewed. Of the 20 patients, 16 were men and 4 were women, with an age range of 9-90 years old. The pathological types included squamous cell carcinoma (SCC; n=7), adenoid cystic carcinoma (ACC; n=7), chondrosarcoma (n=2), fibrosarcoma (n=1), endolymphatic cystic papillary adenocarcinoma (n=1), rhabdomyosarcoma (n=1) and low-grade malignant peripheral nerve sheath tumor (n=1). Patients initially diagnosed with SCC and ACC were staged according to the revised Pittsburgh classification system (T1, n=1; T3, n=5; T4, n=8). All patients received surgery, followed by postoperative radiotherapy (RT) with/without chemotherapy in 10 patients. Kaplan-Meier analysis was used to calculate the survival rate, and the log-rank test was applied to compare the differences in survival. The duration of follow-up ranged from 1 to 140 months. Notably, the 3- and 5-year disease-specific survival (DSS) rates were 79 and 73%, and the 3- and 5-year overall survival (OS) rates were 74 and 68%. Pathological type and posterior cranial fossa meningeal involvement were significantly associated with both OS and DSS. Furthermore, patients with a positive surgical margin had a significantly worse DSS rate. In conclusion, the incidence of temporal bone malignancies is low with an insidious onset. Radical resection is the main treatment option, and postoperative RT with/without chemotherapy may be supplemented in cases of an advanced stage or when tumors have a positive surgical margin.

Objective:To summarize and analyze the effect of facial nerve decompression surgery for the treatment of Bell's palsy and Hunt syndrome. Methods:The clinical data of 65 patients with facial nerve palsy who underwent facial nerve decompression in our center from October 2015 to October 2022 were retrospectively analyzed, including 54 patients with Bell's palsy and 11 patients with Hunter syndrome. The degree of facial paralysis（HB grade） was evaluated before surgery, and ENoG, pure tone audiometry, temporal bone CT and other examinations were completed. All patients had facial palsy with HB grade V or above after conservative treatment for at least 1 month, and ENoG decreased by more than 90%. All patients underwent facial nerve decompression surgery through the transmastoid approach within 3 months after onset of symptoms. The recovery effect of facial nerve function after surgery in patients with Bell's palsy and Hunter syndrome was summarized and analyzed. In addition, 15 cases in group A（operated within 30-60 days after onset） and 50 cases in group B（operated within 61-90 days after onset） were grouped according to the course of the disease（the interval between onset of symptoms and surgery） to explore the effect of surgical timing on postoperative effect. Results:There was no significant difference between the two groups of patients with Chi-square test（P=0.54） in 42 patients（77.8%, 42/54） with Bell's palsy and 7 patients（63.6%, 7/11） in patients with Hunter syndrome who recovered to grade Ⅰ-Ⅱ. According to the course of the disease, 10 cases（66.7%, 10/15） in group A recovered to grade Ⅰ-Ⅱ after surgery. In group B, 39 patients（78.0%, 39/50） recovered to grade Ⅰ-Ⅱ after surgery, and there was no statistically significant difference between the two groups by Chi-square test（P=0.58）. Conclusion:Patients with Bell's palsy and Hunter syndrome can achieve good results after facial nerve decompression within 3 months of onset, and there is no significant difference in the surgical effect between the two types of patients.

BACKGROUND:Spatial hearing is a critical feature in daily life. However, there is quite a range in hearing loss patients regarding the effect of bone conduction device on localization performance. OBJECTIVES:To analyze the localization performance in patients with bilateral conductive or mixed hearing loss fitted with one Baha® Attract system. MATERIALS AND METHODS:This prospective study included 12 patients who had followed up for more than one year. The parameters analyzed included (1) audiological results: sound field threshold, speech discrimination scores (SDSs), and sound localization test, and (2) functional results: scores for the Speech, Spatial and Qualities of Hearing Scale (SSQ) and the Chinese translation of the Spatial Hearing Questionnaire (C-SHQ). RESULTS:The audiological assessments showed a reduction of 28.5 dB in the mean sound field thresholds and improvements of 61.7% in the SDSs for disyllabic words. The root mean square error improved slightly with the Baha® Attract system. Patients showed promising results in the functional questionnaire assessments, with significant improvements in the SSQ and C-SHQ scores. CONCLUSIONS:Although most patients were not able to localize sound accurately after surgery, the change in the scores of the SSQ and C-SHQ indicated that the Baha® Attract system could improve spatial hearing.

目的 探讨岩骨次全切除术应用于多种侧颅底病变的治疗效果.方法 回顾性分析2007年7月—2019年9月行岩骨次全切除术的78例患者的临床资料,男40例,女38例;平均年龄(37.3±14.1)岁.其中岩骨胆脂瘤28例,慢性中耳炎术后感染20例,颞骨良性肿瘤19例,脑脊液耳漏11例.术前患者均行纯音测听、颞骨CT、内耳MRI,面神经功能评价House-Brackmann分级:Ⅰ级25例,Ⅱ级19例,Ⅲ级15例,Ⅳ级10例,V级5例,Ⅵ级4例;全聋21例,余者平均听力52.2 dB.术后复查颞骨CT、内耳MRI并评价面神经功能.结果 平均随访5.51年,术后无死亡、颅内出血、脑脊液耳漏等严重并发症.岩骨胆脂瘤、慢性中耳炎术后感染患者术后复发各2例,经再次手术治愈;颞骨良性肿瘤均完全切除肿瘤,其中14例面神经鞘瘤患者中9例行面神经-舌下神经吻合术.术后面神经功能评价:Ⅰ级28例,Ⅱ级20例,Ⅲ级17例,Ⅳ级7例,V级2例,Ⅵ级4例;所有患者均无实用听力.结论 岩骨次全切除术适用于广泛的岩骨胆脂瘤、慢性中耳炎术后感染及颞骨良性肿瘤的切除,也可用于防治脑脊液耳漏;此术式无法保留或重建听力,故仅适用于无听力重建条件者.

目的 分析突发性聋患者的脑白质病变程度,探讨脑白质高信号对突发性聋预后的影响.方法 回顾性收集2021年1月至2021年12月就诊于首都医科大学附属北京天坛医院的143名单侧突发性聋患者的临床资料,根据Fazekas量表评分进行分组,比较各组治疗效果,分析影响突发性聋预后的危险因素.结果 0分组72例(50.35％),1分组63例(44.01％),2分组8例(5.59％).各组患者的患侧初始听阈差异无统计学意义(P＞0.05).0分组与1分组、0分组与2分组患者年龄差异有统计学意义(P＜0.01).各组治疗前及治疗后听力阈值比较,差异均有统计学意义(P＜0.01).0分组、1分组、2分组的痊愈率差异有统计学意义(P＜0.01).年龄、患侧初始听阈是影响痊愈的危险因素.结论 脑白质高信号及伴随的心脑血管危险因素可能影响突发性聋对治疗的反应.控制突发性聋患者的心脑血管异常,有助于获得较好的预后.

目的 探讨颞骨次全切除术在外耳道腺样囊性癌的临床应用效果.方法 回顾性分析2015-2019年收治的8例外耳道腺样囊性癌患者的临床资料,其中男2例,女6例;年龄33～76岁,平均年龄55.6岁.耳痛7例,外耳道肿块、耳溢液、听力下降各6例,面瘫1例;从最初出现症状到确诊平均为2.75年,5例曾被误诊,3例曾被误治手术;6例行颞骨次全切除+腮腺浅叶切除术,2例行颞骨次全切除+全腮腺切除术.结果 围手术期内1例患者出现局部切口感染,1例出现脑脊液漏.全部患者平均随访4.34年;6例患者无瘤存活;1例复发患者于我院二次手术后3年再次出现局部复发伴肺转移,接受放射治疗,目前带瘤生存;1例患者术后随访3.5年死于其他疾病.结论 外耳道腺样囊性癌早期症状不典型,容易误诊;确诊后应实施颞骨次全切除术以达到彻底切除肿瘤目的,术中应同时处理腮腺;术后放疗也是一种可选择的补充治疗手段.

Context Bell's palsy is a form of idiopathic, facial nerve palsy. Initial treatment includes the use of oral corticosteroids and/or antiviral agents, but facial paralysis may persist. Some surgeons suggest that surgical decompression of the facial nerve can be a beneficial, but the optimal surgical approach, extent of nerve decompression, and timing of surgery remain unclear. Objective This study intended to evaluate the efficacy of delayed, facial nerve decompression for severe Bell's palsy (BP) and to explore the relationship of opportunity timing for operations, with postoperative recovery for facial nerve function. Design The research team performed a retrospective study. Setting The study took place at Beijing Tiantan Hospital of Capital Medical University in Beijing, China. Participants Participants were 45 patients who had been diagnosed with BP between 2015 and 2021 and who had undergone facial nerve decompression using the transmastoid approach, between 30 and 180 days after the onset of BP. According to the operation's timing, the research team divided the participants into three groups, consisting of participants who underwent surgery: (1) at 30-60-days after BP onset-19 participants, (2) at 61-90 days after BP onset-18 participants, and (3) at more than 90 days after BP onset-8 participants. Outcome Measures The research team: (1) analyzed participants' demographic and preoperative and postoperative clinical characteristics, (2) compared the surgical outcomes with participants' House-Brackmann (HB) scales, and (3) analyzed the factors affecting the recovery of facial nerve function using logistic regression. Results Decompression surgery was effective for 29 participants (64.4%), with similar rates for participants who underwent surgery after 30-60 days (73.7%) and 61-90 days (77.8%), but the surgery' success was significantly higher for those groups than for participants who underwent surgery after >90 days (12.5%), with P = .008 and P = .003, respectively. Multivariate logistic regression analysis showed that disease duration was the only factor significantly associated with the effectiveness of surgery (odds ratio = 120.337; 95% confidence interval 2.997-4832.267, P = .011). Conclusions For patients with severe Bell's palsy with ineffective conservative treatment, surgery performed 30 to 90 days after the onset of paralysis can have therapeutic benefits, whereas surgery performed after 3 months is relatively ineffective.

ObjectiveTo investigate the relationship between the thresholds of intraoperative facial nerve response and postoperative facial nerve function recovery in patients with Bell's palsy.MethodsClinical data from Bell's palsy patients who underwent facial nerve decompression surgery at Beijing Tiantan Hospital from October 2015 to October 2022 were collected. The patients selected for analysis had intraoperative facial nerve monitoring with accurate recording of the facial nerve direct stimulation threshold and at least 1 year of detailed follow-up. The patients with postoperative facial nerve function recovery to grade Ⅰ-Ⅱ (HB grading) were defined as having good recovery, while those recovering to grade Ⅲ or higher were defined as having poor recovery. The patients were divided into two groups (A and B) according to facial nerve reaction excitability, with group A having a direct stimulation threshold of ≤1.5 mA and group B having a direct stimulation threshold of > 1.5 mA or a stimulation volume up to 3 mA without being able to record neural response waveform. The relationship between postoperative facial nerve function recovery and facial nerve direct stimulation threshold was analyzed.ResultsA total of 36 Bell's palsy patients were included in this study, who underwent facial nerve decompression surgery through the transmastoid-epitympanum approach within 1-3 months after onset. Of the 36 patients, 24 (66.7%) had good recovery and 12 (33.3%) had poor recovery. Only 20 (55.6%, 20/36) patients had facial nerve direct stimulation threshold recorded during the operation, and all were ≤1.5 mA(0.1-1.5 mA). The reaction waveforms of 16 (44.4%, 16/36) patients could not be recorded even when facial nerve stimulation increased to 3 mA. There was no significant difference in age, gender, lateral discourse, timing of surgery, and preoperative electroneurography (ENoG) between the two groups(all P > 0.05), but the good recovery rate of the patients in group A was significantly higher than that in group B, and the difference was statistically significant ( P=0.009). ConclusionFor patients with Bell's palsy undergoing decompression surgery of the facial nerve, the intraoperative direct stimulation threshold of the facial nerve may have some predictive value for postoperative facial nerve function recovery.

Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.

目的 评估基于回顾性研究的突发性感音神经性耳聋(简称突聋)合并糖尿病患者激素治疗后血糖分级管理方案的临床效果,为此类患者激素治疗后的血糖管理提供依据.方法 选择2020年2月至2020年12月在首都医科大学附属北京天坛医院行激素治疗的突聋合并糖尿病患者60例,采用随机数字表法分为对照组和观察组,每组30例.对照组进行常规的血糖宣教指导,观察组实施血糖分级管理方案.比较干预前后两组患者空腹血糖、餐后2h血糖水平及突聋的治疗有效率.结果 干预后两组空腹血糖水平与干预前比较差异无显著性(P>0.05).但观察组餐后2h血糖水平明显低于干预前,对照组餐后2h血糖水平明显高于干预前,且干预后观察组餐后2h血糖水平明显低于对照组,差异均有显著性(P<0.05).观察组突聋的治疗有效率为86.7％,与对照组(73.3％)比较差异无显著性(P>0.05).结论 血糖分级管理方案可以较好地控制突聋合并糖尿病患者激素治疗后的血糖升高,减少血糖波动,值得临床应用.

目的 评估经皮骨锚式助听器(transcutaneous bone-anchored hearing aid,tBCHD)Baha Attract对传导性或混合性聋患者的助听效果及安全性.方法 采用前瞻性自身对照研究方法收集13例行Baha Attract植入术的传导性或混合性聋患者临床资料,其中先天性外中耳畸形8例,慢性化脓性中耳炎或中耳胆脂瘤术后4例,慢性中耳炎1例.术前进行纯音测听、言语识别率测试、颞骨CT等评估,术后1个月开机时测试助听后的声场纯音听阈和言语识别率,同时比较术前、术后3个月及1年的言语、空间和音质听觉量表(the speech,spatial,and qualities of hearing scale,SSQ)和健康效用指数量表3(health utilities index mark 3,HUI 3).结果 术后随访42～49个月,发生皮下血肿1例,植入体周围血清肿2例,均治愈.13例患者术前平均气导听阈为66.6±8.0 dB HL,术后声场下助听听阈为24.0±7.1 dB HL;手术前后安静环境下单音节言语识别率分别为24.2％±20.1％、87.7％±7.3％,双音节言语识别率分别为38.9％±24.1％、92.3％±4.8％,手术前后噪声下言语识别率分别为28.9％±22.0％、85.0％±14.4％,与术前比较均有显著差异.SSQ量表结果显示Baha Attract术后患者在言语识别、声源定位及音质评价3个维度得分均明显提高.HUI 3量表结果示Baha Attract术后患者在听力、言语、情绪、疼痛方面显著改善,总体健康状态评分显著提高.结论 Baha Attract是一种安全、有效的人工听觉植入装置,可显著提高传导性或混合性聋患者的听力并改善其生活质量,配戴舒适,术后并发症较少.

Objective:To analyze the clinical characteristics of unilateral acoustic neuroma（AN） with normal hearing, so as to provide evidence for early identification AN. Methods:Clinical datas from 73 patients of unilateral AN with normal hearing of Otorhinolaryngology Head and Neck Surgery of Beijing Tiantan Hospital affiliated of Capital Medical University from August 2019 to April 2022 admitted to department were retrospectively analyzed. All patients underwent pure tone audiometry（PTA）, speech discrimination score（SDS）, auditory brainstem response（ABR）, distortion product otoacoustic emission（DPOAE） and head enhanced MRI. Results:The incidence of normal hearing among patients with AN was 10.7%. Male∶female=1∶2.2; the mean age of the patients was（37.3±9.4） years; the mean tumor size was（24.2±11.2） mm. Tinnitus was the most common reason for visit; the patients who had headache and dizziness had larger tumors. Surgery was the main treatment, and the patients who underwent surgery had larger tumors than those of follow-up. Heterogeneous tumors were the most common type of MRI, homogeneous tumors were smaller than heterogeneous and cystic tumors. The sensitivity of ABR in the diagnosis of AN with normal hearing was 95.9%, and that of ≥20 mm tumors was 100%; prolonged Ⅴ-waves were the most common, patients with Ⅴ-wave deletion had larger tumors than those with normal or prolonged Ⅴ-waves. Patients who had the longer the Ⅴ-wave and the longer difference between Ⅰ-Ⅴ wave had larger tumors. DPOAE was not elicited at full frequency in 11 patients. There was no statistically significant difference in age among patients with different symptoms, treatments, types of MRI, ABR and DPOAE. Conclusion:AN of normal hearing was most common in 30-39 years old women. Patients had different symptoms, phenotypes of MRI and ABR. Patients with normal hearing who had tinnitus, dizziness, headache, facial paraesthesia, and recovery after sudden haring loss can be further examination of ABR and DPOAE for early identification AN. The sensitivity of ABR in diagnosis of hearing normal AN was 95.9%, and the abnormal type of Ⅴ-wave is related to tumor size.

目的 分析听神经瘤患者临床听力学特征,为听神经瘤的诊断提供参考依据.方法 回顾性分析首都医科大学附属北京天坛医院耳鼻咽喉头颈外科接诊的394例单侧听神经瘤患者临床资料,所有患者均行纯音测听、言语识别率、听性脑干诱发电位和颅脑增强MRI.结果 患侧听力正常者54例,轻度听力损失58例,中度63例,中重度45例,重度45例,极重度31例,全聋者98例;高频听力损失最多见.24.7％患者言语识别率和纯音测听下降不成比例.听性脑干诱发电位波形正常者8例,波形缺失者228例和其他波形异常者158例;听性脑干诱发电位诊断听神经瘤的敏感度:内听道内肿瘤85.7％,内听道外98.5％.结论 听神经瘤的听力学表型多样,听力正常者不能排除听神经瘤;听力损失程度不能预判肿瘤大小;纯音测听与言语识别率不一致应警惕蜗后病变;听性脑干诱发电位诊断听神经瘤敏感度随肿瘤增大而增加.

目的 探讨短期进展为急性后循环脑梗死(posterior circulation cerebral infarction,PCI)的突发感音神经性聋(sudden sensorineural hearing loss,SSNHL)患者的临床及影像学特征.方法 选取2017年1月至2021年12月首都医科大学附属北京天坛医院以突发性聋首诊住院,且以短期进展为急性PCI患者21例为病例组,按照1∶4匹配性别相同、年龄±3岁、听力损失类型相同的无急性PCI患者84例为对照组.比较两组的一般资料、临床及影像学特征.应用多因素logistic回归模型分析短期进展为PCI的SSNHL患者的危险因素.结果 病例组21例,男17例,女4例,平均年龄(54.3±12.8)岁;对照组84例,男68例,女16例,平均年龄(53.8±12.2)岁.两组患者听力均为平坦下降型及全聋型.病例组高血压、糖尿病、高脂血症、心脑血管疾病史、饮酒、高同型半胱氨酸血症、既往有突发性聋病史、发病时伴头晕或眩晕、平均听力、双侧突发性聋、进行性听力下降、双耳重度极重度感音神经性聋以及椎-基底动脉重度狭窄/闭塞的比例均高于对照组[90.5％比 45.2％,47.6％比 19.0％,85.7％比 17.9％,57.1％比 15.5％,33.3％比 10.7％,42.9％比 9.5％,28.6％比 1.2％,85.7％比33.3％,(89.35±22.40)dB HL 比(77.12±21.88)dB HL,33.3％比2.4％,23.8％比0.0％,28.6％比 1.2％,66.7％比20.2％],差异有统计学意义(P＜0.05).多因素logistic回归模型分析显示,发病时伴头晕或眩晕(OR=24.888,95％CI:3.761～164.671,P=0.001)、合并≥3个动脉粥样硬化危险因素(OR=21.228,95％CI:4.459～101.072,P=0.000)及椎-基底动脉重度狭窄或闭塞(OR=11.960,95％CI:2.356～60.708,P=0.003)的患者在短期更有可能进展为急性PCI.结论 突发性聋患者合并多个动脉粥样硬化危险因素,发病时伴头晕或眩晕,尤其是双侧或进行性听力下降时,应尽快完善影像学评估,以便早期识别可能进展为急性PCI的高危患者.

Objective:To investigate the relationship between the severity and curative effect of hearing loss and clinical indicators in patients with sudden hearing loss （SHL）. Methods:The Spearman correlation coefficient was used to analyze the correlation between the efficacy of SHL and clinical indicators.A total of two hundred and seventy-three patients with SHL were selected and divided into three quantile groups according to the average hearing threshold of the the involved ear frequency of the first pure tone audiometry at admission. Univariate and multivariate ordered logistic regression were used to evaluate the relationship between initial hearing level and clinical indicators of SHL patients. The Spearman correlation coefficient was used to analyze the correlation between efficacy of SHL and clinical indicators. Results:Compared with patients with lower hearing loss （≤50 dB HL），patients with higher hearing loss （>50 dB HL） had higher Neutrophil, Monocyte, Triglycerides, Hemoglobin, Fibrinogen, Glucose, Neutrophil/high-density lipoprotein cholesterol ratio （NHR）, Monocyte/high-density lipoprotein cholesterol ratio, Monocyte/lymphocyte cell ratio, age, dizziness, and lower Platelet/ lymphocyte cell ratio and High-density lipoprotein cholesterol, and poor final hearing threshold.Multivariate logistic regression showed that NHR and age were independent risk factors for initial hearing loss in SHL patients.And the NHR, Neutrophil/lymphocyte cell ratio （NLR）, course of disease, type of hearing curve, and final hearing threshold were significantly negatively correlated with curative effect. Conclusion:SHL patients with higher NHR and NLR values, the longer time from onset to visit, and the more severe hearing loss had worse efficacy.However, SHL patients with higher NHR and age values had greater initial hearing loss,the degree of hearing loss and curative effect are different in SHL patients with different types of hearing threshold curve and age.

目的 探讨非药物干预措施对慢性鼻窦炎鼻内镜术后疼痛及睡眠质量的影响.方法 选择2020年5月至2021年2月在首都医科大学附属北京天坛医院进行慢性鼻窦炎鼻内镜手术治疗的60例患者,采用随机数字表法分为观察组和对照组,每组30例.对照组术后实施常规护理,观察组术后在常规护理的基础上联合应用认知教育、心理疏导、冷敷、耳穴贴压、缩唇呼吸等非药物干预措施.采用疼痛数字评价量表、匹兹堡睡眠质量指数评估患者的疼痛程度和睡眠质量.结果 观察组患者术后24h和48h的疼痛程度低于对照组,手术当天和术后第1天的睡眠质量优于对照组,差异均有显著性(P<0.05).结论 慢性鼻窦炎鼻内镜术后应用非药物干预方案可有效缓解疼痛,改善睡眠质量,值得临床应用.

Head and neck paragangliomas(HNPGL) are rare neurogenic tumors, in which genetic factors play a certain role. So far, 10 genes related to HNPGL(SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, HIF2A, RET, NF1 and TMEM127) have been found. The clinical phenotypes of different gene mutations are different. SDHD, SDHC and SDHAF2 gene mutations are almost 100% related to HNPGL. SDHD and SDHAF2 gene mutations tend to form multiple paragangliomas, and its genetic pattern has obviously maternal imprinting. SDHB mutations have sympathetic paraganglioma and malignant tendency. Understanding the molecular mechanism and genotype-phenotypic relationship of paraganglioma can provide significant help for genetic counseling, diagnosis and treatment, and development of follow-up strategies in patients with HNPGL.

Objective To analyze the clinical effects of modified infratemporal fossa type A approach for glomus jugulare tumors. Methods The clinical data of patients with glomus jugulare tumors who received modified infratemporal fossa approach type A from May 2014 to December 2019 were retrospectively collected in the Beijing Tiantan Hospital, Capital Medical University. Modified infratemporal fossa approach type A was performed and improved on two aspects: anterior transfer of facial nerve and management of sigmoid sinus. CT was performed 6 hours after surgery to exclude intracranial hemorrhage. MRI was reviewed 2 weeks after surgery to define residual tumor, and every year to determine recurrence. Follow-up was conducted to assess the facial nerve function. Results A total of 39 patients were included in this study. Patients' grades of preoperative facial nerve function according to House-Brackmann (HB) were as following: grade Ⅰ in 12 patients, grade Ⅱ in 6 cases, grade Ⅲ in 8 cases, grade Ⅳ in 9 cases, grade Ⅴ in 3 cases, and grade Ⅵ in 1 case. All 39 patients successfully completed surgery, and there were no deaths and no severe complications such as hemiplegia, or intracranial infections. Cerebrospinal fluid leakage occurred in 1 case and drainage was performed. There was no intracranial hemorrhage in the CT exam 6 hours after surgery. Total resection was performed in 37 cases (94.9%) and nearly total resection in 2 cases (5.1%). The postoperative facial function after 1 year was HB grade Ⅰ in 8 patients, grade Ⅱ in 16 cases, grade Ⅲ in 9 cases, grade Ⅳ in 4 cases, grade Ⅴ in 2 cases. The facial function was improved after the surgery(P < 0.05). One year after surgery, tumor recurrence occurred in 1 patient and radiotherapy was performed. There was no significant tumor enlargement during follow-up. Conclusions The modified infratemporal fossa approach type A keeps the advantage of classical approach. By adjusting the anterior transfer of the facial nerve, the preservation of facial nerve function was improved. By compressing the sigmoid sinus instead of ligation, the occurrence of brain injury and cerebrospinal fluid leakage were reduced.

本文报道一例外耳圆柱瘤病例，老年女性，主诉发现右外耳肿物12年，初步诊断为右外耳肿物，全身麻醉下行右侧外耳肿物切除术+游离全厚皮片移植术。术后病理报告为圆柱瘤，随访观察1年无复发。