脊索瘤是罕见的骨恶性肿瘤,起源于胚胎残留的脊索组织,过去认为脊索瘤相对于颅底更多见于骶骨,然而,现有证据表明,其在颅底( 32％)、脊柱(32.8％)和骶骨(29.2％)的分布几乎是均等的[1].虽然脊索瘤为低度恶性肿瘤,但易局部侵犯且预后不良.对于发生于斜坡的脊索瘤,目前公认的治疗原则是最大限度地安全地切除肿瘤,术中强调对神经及血管的保护,术后辅助放疗[2].本文报道1例侵犯海绵窦包绕颈内动脉的斜坡脊索瘤综合治疗结果.

目的 探讨经口入路低温等离子切除早期声门型喉癌的效果.方法 2011年9月～2016年1月对105例早期声门型喉癌(T1期87例,T2期18例)全麻下行经口入路低温等离子喉肿瘤切除术,术后定期随访,观察肿瘤复发、转移及生存情况.术后第1年喉镜每3个月复查1次,第2～5年每6个月复查1次,5年以后每年复查1次;颈部超声为6个月复查1次;胸部CT为每年复查1次.结果 105例均在内镜下行低温等离子肿瘤切除术,未发生气道爆燃或气道黏膜灼伤等医疗不良事件.2例术后发热及肺部感染,抗生素治疗5～7d后复查胸片,肺部感染控制后出院.6例术后出现声带粘连,主要表现为声音嘶哑,无明显喉梗阻症状,不影响患者生活质量.2例术后出现颈前皮下气肿,3 cm×3 cm以内,术后3 d皮下气肿逐步消退.术后病理均为喉部鳞状细胞癌.98例中位随访时间61.8月(14～100个月),11例发生喉腔局部肿瘤复发,1、2、3年累积复发率分别为0％、9.2％、11.2％;5例发生颈部淋巴结转移,1、2、3年累积转移率分别为0％、3.1％、5.1％;17例死亡,其中10例死于喉癌及肿瘤复发,5例死于肺癌(考虑为多重癌),2例死于心脑血管疾病,1、3、5年累积生存率分别为100.0％、91.4％、83.8％.结论 经口入路低温等离子切除早期声门型喉癌总体疗效满意.

神经纤维瘤病( neurofibromatosis,NF)是一种不完全外显的常染色体显性遗传病,表现为全身多器官、多系统的神经纤维结节性病变,可引起皮肤、骨骼、内脏等损伤和功能障碍,也有表现为颅内肿瘤、皮质畸形,有癫痫、学习障碍、发育异常、精神异常等症状[1,2].美国国立卫生研究院( National Institutes of Health,NIH)在基因研究的基础上于1987年提出将神经纤维瘤病分为Ⅰ型和Ⅱ型.Ⅱ型以中枢神经系统受累为主,表现为听神经瘤(双侧)、脑膜瘤、胶质瘤等,较少见.Ⅰ型即von Recklinghausen病,占90％,以多发性皮肤神经纤维瘤和皮肤色素斑、虹膜色素结节为特征,少数可恶变,头颈部症状表现为三叉神经、面神经和颈部皮神经分布区域的神经纤维瘤,此外,眶颞区、舌亦好发[3] ,咽侧、食管、鼻 -鼻窦等部位也有报道,而累及喉部的报道罕见.2018年11 月,我科收治喉部神经纤维瘤病1 例,报道如下.

Objective structured clinical examination (OSCE) is an important assessment method for examining clinical thinking and clinical skills of medical students. OSCE has been applied in the quarterly phase examination in our department. According to the characteristics of otolaryngology, three stations of common items and five stations for personalized tests were set in the assessment. According to the different training that residents received during their stay in the previous department , different contents were assessed in the station for personalized examination. During the OSCE, residents took turns entering into each examine station and teachers gave scores according to the marking criteria of different station. After the examinations, two-way assessment and feedback were conducted between students and teachers. Through the practice in standardized training of residents in recent years, we found that OSCE was more comprehensive and flexible than others, which gained good application effects.

茎突综合征( styloid process syndrome)是因为茎突过长、方位或形态异常等因素,致使与其临近的肌肉、血管、神经及黏膜等结构相抵触,产生咽部异物感、咽部疼痛感、反射性耳痛、舌咽神经痛、头颈部疼痛和涎腺分泌增多等症状[1].由于临床表现差异较大,缺乏典型症状,故常误诊误治[2].本例反复于外院就诊7年均未确诊,2017年10月就诊我院后予以相应的诊断和治疗,于内镜引导下截断茎突,报道如下.

面神经鞘瘤属上皮源性良性肿瘤,可发生于从桥小脑角至腮腺区的面神经任何节段和分支,以乳突段、迷路段多见[1].对肿瘤大、侵犯广的患者常采用侧颅底手术,术后主要并发症有脑脊液漏、脑水肿、后组脑神经麻痹失代偿、急性肺水肿等.肺动脉栓塞并非术后常见并发症,如未经及时治疗,病死率可达30%以上[2].

OBJECTIVE:To observe clinical characteristics of 15 cases of laryngeal nonsquamous malignancies.METHOD:The clinical data of 15 patients with laryngeal nonsquamous malignancies were collected. Details about pathology, CT, treatment and follow-up results were carefully taken into account.RESULT:There were more male patients than females while the sex ratio was 4:1. Supraglottic malignancies accounting for 53.33% seemed to be the most common type. In all the 15 patients, 1 patient gave up other treatment except tracheotomy to relieve laryngeal obstruction. Three patients took radiotherapy and chemotherapy. The rest patients underwent surgical procedure after strict endoscopic, radiologic staging. Radiotherapy and (or) chemotherapy were chosen after operation. The follow-up results show that 1 year survival rate was 79.4% and the 3 year survival rate was 57.8%, 5 year survival rate was 27.5% which was not optimistic.CONCLUSION:Being a kind of rare type laryngeal nonsquamous malignancies have difference clinical characteristics with laryngeal squamous cell carcinoma. Laryngeal nonsquamous cell carcinoma have much lower 5 year survival rate and are easier to suffer recurrence and metastasis compared with laryngeal squamous cell carcinoma.

OBJECTIVE:To describe our experience in the clinical manifestation and treatment of malignant tumors of the external and middle ear.METHOD:The study reviewed 39 patients between 1994-2011 in our hospital, including 15 pinna tumors, 18 external canal tumors and 6 middle ear tumors. 23 males and 16 females were enrolled in this study. The mean age of patients at the time of surgery was 59. Radiotherapy or radiotherapy and chemotherapy were the only possible treatment in 6 cases. Thirty-three patients were treated surgically, and 9 patients also received radiotherapy after surgery.RESULT:All of the patients had been followed up over 3 years, except for 1 case of external canal and 1 case of middle ear tumor. The 3-year survival of pinna, external canal and middle ear tumors were 86.7%, 82.4% and 60.0% respectively. At the last follow up, the pinna tumors showed that the survival rate was 100% in T1, T2 and Tx stage, and 0% in T4 stage; the external canal tumors showed that the survival rate was 90% in T1 stage, and 66.7% in T2, T3 stage; the middle ear tumors showed that the survival rate was 100% in T1 and T2 stage, 0% in T3 stage.CONCLUSION:The T staging system is for an important prognostic factor, and it is important for an early diagnosis and radical surgery to achieve a better therapeutical result.

Objective To investigate the clinical values of laryngeal electromyography (LEMG) for evaluating the amyotrophic lateral sclerosis(ALS) patients with swallowing disorder .Methods A total of 25 ALS patients with or without complaining of swallowing disorder were recruited for the study .Their right cricothyroid muscles were taken unilaterally with concentric needle electrodes by anterior cervical approach .Each patient was tested with Kub-ota drinking test too .Results Of all the 25 patients ,11 were found abnormal in the right cricothyroid muscle which presented with typical degeneration patterns ,15 cases were found abnormal by Kubota drinking test .The coinci-dence rate of the two methods was 60% ,the poisitive coincidence rate was 32% .The McNemar test showed there was some consistency between the drinking water test and LEMG (P=0 .344) ,and the Kappa coefficient was 0 .219 . Conclusion The cricothyroid muscle electromyography may also be a potential method to evaluate ALS patients since it seemed to offer an evidence of dysphagia related with abnormal sensory function of laryngeal mucosa .But there may be some limitations .

OBJECTIVE:To observe the characteristics of acoustic parameters of 21 patients with amyotrophic lateral sclerosis (ALS) complaining of dysarthria and to explore the possibility and clinical value of Multi-Dimensional Voice Program (MDVP) on aseessing the patient's voice.METHODS:The clinical data and speech intelligibility of each of the 21 patients with ALS complaining of dysarthria were collected. All the 21 patients with ALS and 44 normal subjects were suggested to pronounce the vowel [a:]. The voice samples were collected and analyzed by Multi Dimensional Voice Program. The data were compared to detect the difference between the two groups.RESULTS:Speech intelligibility of 10 in the 21 patients was above 97% including 5 patients whose scores were 100%. It seemed that the speech intelligibility score was not related with the duration of the disease and the on-set part. The values of noise to harmonic ratio (NHR) of both the light and the severe damage to the speech group were higher than those of the control group with significant difference (P<0.05).Significant difference was noticed between the severe damage to the speech group and control group in Jitter.CONCLUSION:Multi Dimensional Voice Program could be used to analyze the characteristics of acoustic parameters of patients with ALS patients complaining of dysarthria. There was more noise composition in the voice of the patients with dysarthria. NHR seemed to be the sensitive and stable parameter. Especially in patients whose speech intelligibility were affected severely, more acoustic parameters were detected with significant difference.

Objective To detect the laryngeal electromyography(LEMG)features of the patients with amyotrophic lateral sclerosis(ALS).Methods Twenty three ALS patients with bulbar symptom and/or hoarse were included for standard LEMG of cricothyroid muscle,thyroarytenoid muscle and posterior cricoarytenoid.Results Of 23patients with 69muscles included,10cases with 11muscles presented classic denervation characteristics,such as fibrillation potential,positive sharp wave,and polyphasic motor unit action potential.Ten of 23cricothyroid muscles were found abnormal,with only 1thyroarytenoid muscle being abnormal,and no thyroarytenoid muscle being abnormal.Six old of 8(75%)bulbar onset ALS patients presented abnormal LEMG,and 4of 15(26.67%)for limb onset ALS patients.The difference was significant(P<0.05).Other factors such as sex,bulbar symptom,and bucking made no difference to the abnormal LEMG rate.Conclusion The LEMG of patients with ALS did show abnormal.More laryngeal muscles were affected in patients with bulbar onset than limb onset.Cricothyroid muscle had more denervation changes amrg all intrinsic laryngeal muscles.

1临床资料患儿,女,5岁3个月,主因发作性眩晕1h入我院儿科。患儿入院前1h无诱因突发眩晕、恶心、非喷射性呕吐,不能站立及行走,不伴抽搐、意识障碍、发热、耳鸣、听力改变、耳闷,既往无类似发作。足月顺产,生长发育良好。儿科医师查体见双眼自发性眼震（方向不详）,余未见明显异常。入院后给予能量合剂等对症治疗,同时完善血常

鼻中隔穿孔临床上并不少见,尽管大多患者症状并不明显,但有症状者生活质量明显下降.目前其治疗效果尚不能令人十分满意.本文就其病因、病理生理以及治疗等方面的进展进行综述,希望对临床处理有所帮助.

OBJECTIVE:To explore the quality and quantity of glutamate(Glu) on the auditory pathway of inferior colliculus when exposed to conditioning noise or traumatic sound to find the relationship between the change of neurotransmitters and the protection of hearing, and the mechanism in the phenomena.METHODS:Twenty Sprague-Dawley rats were divided into four groups: control group, conditioning group, intensive noise group and combining group. The hearing function was measured by ABR. Glu was detected by microdialysis and HPLC.RESULTS:The ABR levels after exposure were (47.0+/-2.7) dB SPL (intensive noise group ),(31.0+/-2.2) dB SPL (conditioning group ) and (36.0+/-2.2) dB SPL (combining group ).The levels of Glu were (310.5+/-78.5)x 10(-7)mol/L (intense noise group ),(162.9+/-64.1)x10(-7)mol/L (conditioning group ); (113.6+/-38.1)x10(-7)mol/L (combining group),(56.3+/-23.9)x10(-7)mol/L (control group).CONCLUSION:The level of Glu on the auditory pathway of inferior colliculus was higher when exposed to noise . Glu was exhausted when given conditioning sound, and the level of Glu was lower when given following intense noise. High level of Glu can damage hearing, so exhaustion of Glu in sound condition may contribute to the protection.

条件性噪声(sound conditoning)的保护现象是指预先给动物低水平的长期的声音刺激可以对抗由高强度噪声引起的永久性阈移,已证实大多哺乳动物普遍存在该现象.研究多集中于内耳的形态、生理的变化,也有代谢活性、中耳肌反射、热休克蛋白等因素可能参与保护作用,但其确切的机制还不是十分清楚.本文从刺激声音频率、刺激时间等方面对条件性噪声的特点进行总结,同时探讨可能机理.

目的对应用HA-BMP复合人工听小骨的听骨链重建术后听力效果进行评价,寻找一种生物相容性好并且能被机体骨组织替代的听骨链重建材料.多孔羟基磷灰石(Hydroxyapatites,HA)人工骨具有良好的生物相容性及骨传导功能,无毒、无免疫反应,因此在临床上有很大的应用前景.骨形态发生蛋白(Bone Morphogenetic Protein,BMP)是一种存在于骨组织中的酸性糖蛋白,其主要生物学作用是诱导未分化的间充质细胞分化形成软骨与骨.方法用骨形态发生蛋白(BMP)复合多孔羟基磷灰石(HA)制成复合型人工听小骨(BMP-HA),在动物实验成功的基础上,试用于临床,共计14例,其中部分听骨链赝复物(partial ossicular replacement prosthesis PORP)9例,全听骨链赝复物(total ossicular replacement prosthesis,TORP)5例.结果术后均获一期干耳,随访至少3年,无脱出现象,术后听力稳定,平均气导较术前提高29.5dB.结论BMP-HA人工听小骨是一种具有较强骨诱导能力、生物相容性良好的人工听小骨,作为中耳传音结构重建材料,优于单纯羟基磷灰石人工听小骨.