鼻咽癌部分危及器官勾画差异对剂量分布的影响

目的 探讨全盲下危及器官（OARs）勾画指南的统一学习对鼻咽癌放疗时OARs勾画的体积及受照剂量分布差异的影响。方法 在全盲模式下,由4名医师对8例鼻咽癌患者在CT横断面上勾画双侧颞叶、内耳、中耳,后统一学习OARs勾画指南,比较学习前后上述OARs体积的差异及其受照射剂量的变化。结果 学习前后内耳及中耳绝对体积没有明显差异（P＞0.05）;学习后,左右两侧的颞叶的体积均较学习前有所缩小（P＜0.05）;上述3种OARs的一致性指数（AI）在学习后明显提高（P＜0.05）。从剂量学来看,与学习前相比,除了左侧内耳Dmean,右侧内耳的Dmean及两侧内耳的V50均有统计学差异（P＜0.05）;除了左侧中耳Dmean,右侧中耳的Dmean及两侧中耳的V50均无统计学差异（P＞0.05）;右侧颞叶的D2和左侧颞叶的Dmax、D1、D2在学习前后存在明显差异（P＜0.05）。指南的统一学习使双侧内耳Dmean和V50、左侧中耳的V50、左侧颞叶D1和右侧颞叶D2的Kappa值均明显提高（P＜0.05）。结论 不同观察者勾画的OARs体积和剂量之间存在明显差异,统一学习明显提高了颞叶、中耳及内耳勾画的准确性及一致性,从而使放疗计划的评价更加客观。     

Otoplast in the middle ear cleft — a rare complication of hearing aid fitting and its surgical management

Hearing aid fitment is a routine, usually safe procedure carried out by hearing aid technicians or even audiologists. Hearing aids are often considered the benign, non-surgical alternative to rehabilitate a patient who cannot be helped surgically. It is rare to have to resort surgery to manage a complication resulting from hearing aid fitting. We report here, a case of otoplast as a foreign body in the middle ear cleft (middle ear cavity and mastoid). The otoplast was used to prepare a mould for the hearing aid and the syringe carrying the otoplast burst splashing the material into the middle ear and the mastoid. This resulted in sudden excruciating pain, further loss of hearing and intractable otorrhea. After several attempts to remove the material elsewhere, our patient underwent a successful mastoid exploration to remove the otoplast resulting in a dry ear after seven years of persistent otorrhea. Patient’s professional requirement for good hearing necessitated a myringoplasty on the other (only hearing) ear. He is now happily rehabilitated with a dry but deaf left ear and a normally hearing right ear.     

豚鼠中耳黏膜早期辐射反应扫描电镜研究

目的：观察不同剂量放疗后中耳黏膜超微结构变化规律。方法：豚鼠左侧卧位，γ射线照射右侧耳，300cGy/F,5次／周；12只豚鼠分为照射15Gy组、30Gy组、45Gy后30天组，3只未照射作为对照，取中耳黏膜行扫描电镜观察。结果：中耳黏膜上皮在低剂量照射时无明显变化，随着剂量的增加，中耳腔内出现渗出，中耳黏膜纤毛、微纤毛可发生脱落、融合、倒伏、方向改变。结论：在实验观察时间内，放射后中耳黏膜纤毛结构随着剂量和时间的变化而改变，在放射早期、剂量较小时黏膜纤毛结构、功能可能正常，随剂量增加将会恶化。     

Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identified as the primary cause of BOR syndrome. We present a 3year old child with BOR syndrome, who came to us with bilateral low set, malformed ears & profound cochlear hearing loss along with bilateral branchial fistulae & unilateral renal agenesis. This child underwent successful cochlear implantation recently. The clinical presentation, pre-operative investigations, intra-operative findings & post-op habilitation status are presented with special highlights on the unique facial nerve course along with middle and inner ear anomalies which posed a surgical challenge during cochlear implantation.     

中耳癌诊治15例报告

 本文分析了15例中耳癌的临床资料。结果显示：中耳癌的预后差与其病期较晚治疗困难有关。早期诊断和手术加放射疗法的综合治疗可能提高生存率。慢性化脓性中耳炎可能是诱发中耳癌的原因之一，预防中耳炎可能减少中耳癌的发病率。     

Using Middle Ear Risk Index and ET Function as Parameters for Predicting the Outcome of Tympanoplasty

Otitis media is an important and a highly prevalent disease of the middle ear and poses serious health problem world wide especially in developing countries where large percentage of the population lack specialized medical care. With a large number of patients frequently undergoing tympanoplasty for tubotympanic type of CSOM, it’s important to assess the severity of the disease and predict the outcome of the surgical management whenever done. A normally functioning eustachian tube is an equally essential physiologic requirement for a healthy middle ear and normal hearing. In this study we have used the middle ear risk index (MERI) developed by Kartush which generates a numeric indicator of the severity of the middle ear disease to stratify patient groups according to the severity of the disease and to evaluate the efficiency of MERI score in predicting the outcome of tympanoplasty.     

Multi-Frequency Tympanometry: Clinical Applications for the Assessment of the Middle Ear Status

The aim of the present paper was to review the current knowledge on multi-frequency tympanometry and explore its role as a diagnostic tool in various otologic conditions. Literature review in Medline and other database sources. Prospective controlled, prospective comparative, and prospective cohort studies, animal studies, retrospective studies and systematic reviews. Multi-frequency tympanometry provides more accurate and detailed information about the middle ear dynamics than standard tympanometry. Otosclerosis and rheumatoid arthritis characteristically increase the resonant frequency (RF) of the middle ear. Ossicular chain discontinuity, atelectatic tympanic membrane, and otitis media with effusion typically decrease the RF of the middle ear. Multifrequency tympanometry can also assess the stage of rheumatoid arthritis in the presence of middle ear involvement. The RF can be affected by the mechanical impedance of the cochlea, and multi-frequency tympanometry can be helpful in the diagnostic workup of LVAS. Multi-frequency tympanometry can be a useful tool to predict the diagnosis of various middle ear pathologies preoperatively, due to the ensuing changes in the RF of the mechano-acoustic system of the middle ear, which can be accurately determined when this methodology is applied.     

电离辐射对中耳黏膜杯状细胞的影响

[目的]通过动物模型研究中耳黏膜辐射损伤产生放射性中耳炎的病理改变和机制.[方法]30只豚鼠作为实验对象,模仿临床放射治疗过程,以钴60产生的放射线照射中耳制作放射性中耳炎模型.γ射线照射右耳,300cGy/f,5次/周.实验动物分5组:0、15Gy、30Gy、45Gy、45Gy照射后30d,观察各组动物照射侧中耳黏膜杯状细胞的变化.[结果]中耳黏膜上皮在15Gy组无明显变化.30Gy组,咽鼓管及近咽鼓管鼓室口处黏膜水肿、增厚,杯状细胞明显增加.45Gy组和45Gy照射后30d组咽鼓管及近咽鼓管鼓室口处黏膜与正常组比较明显增厚,杯状细胞明显增加,而鼓岬部黏膜缺乏杯状细胞,放射前后未发现变化.[结论]在放射治疗早期,随着放射剂量的增加,咽鼓管及近咽鼓管鼓室口处黏膜杯状细胞明显增加,增加的杯状细胞将引起黏液的分泌增加,其与放射所致纤毛功能损害共同导致咽鼓管功能损害,引起渗出性中耳炎并使之加重.     

Anaplastic astrocytoma following radiation for a glomus jugular tumor.

Evaluation of radiation therapy for a given neoplasm includes consideration of possible treatment complications as well as potential benefit. A 43-year-old male with a glomus jugular tumor or the right middle ear had received 4480 rad to the right middle and inner ear and temporal bone. Eight years later, he developed an anaplastic astrocytoma of the right cerebellar hemisphere. At this time a third neoplasm, a left carotid body tumor, was demonstrated angiographically. Although radiation can be implicated in the genesis of the glial neoplasm, the presence of two neural crest derived tumors suggests that a lowered threshold for neoplastic transformation in neuroectodermal cells may have been an additional factor. Long-term follow-up of large numbers of patients with glomus jugulare tumors will be necessary to determine if multiple paragangliomas predispose to radiation-associated gliomas.     

50例鼻咽癌调强放疗中内耳受量评价

目的：分析鼻咽癌调强放疗对双侧内耳受量的影响。方法：选择2015年5月至12月就诊于成都军区总医院50例经病理确诊为鼻咽癌的患者,所有患者均接受30次调强放疗。再由一名主治医师在原始的CT图像上勾画出双侧的耳蜗、前庭及内耳道,并在计划的优化过程中对上述结构进行条件一致的剂量限定,最后通过DVH图对上述结构进行剂量分析,包括最大剂量点（Dmax ）、最小剂量点（Dmin ）及平均剂量（Dmean ）。结果：左侧耳蜗的最大剂量点、最小剂量点及平均剂量的均值分别为5366．3cGy、3981．2cGy、4550．1cGy;左侧前庭分别为4323．2cGy、3310．8cGy、3821．1cGy;左侧内耳道分别为5290．8cGy、3828．7cGy、4453．6cGy;右侧耳蜗的最大剂量点、最小剂量点及平均剂量的均值分别为5344．5cGy、3954．2cGy、4550．1cGy;右侧前庭分别为4368．3cGy、3262．9cGy、3796．0cGy;右侧内耳道分别为5165．0cGy、3765．2cGy、4375．2cGy。结论：在鼻咽癌的调强放疗计划设计过程中对内耳结构进行剂量限定,能在不减少靶区受量的同时有效的降低内耳结构的受照射剂量。     

Prospective study of inner ear radiation dose and hearing loss in head-and-neck cancer patients

PURPOSE: To determine the relationship between the radiation dose to the inner ear and long-term hearing loss. METHODS AND MATERIALS: Eligible patients included those receiving curative radiotherapy (RT) for head-and-neck cancer. After enrollment, patients underwent three-dimensional conformal RT planning and delivery (180-200 cGy/fraction) appropriate for their disease site and stage. The inner ear was contoured on axial CT planning images. Dose-volume histograms, as well as the mean and maximal dose for each structure, were calculated. Patients underwent pure tone audiometry at baseline (before treatment) and 1, 6, 12, 24, and 36 months after RT. The threshold level (the greater the value, the more hearing loss) in decibels was recorded for 250, 500, 1000, 2000, 4000, and 8000 Hz. For patients receiving predominantly unilateral RT, the contralateral ear served as the de facto control. The differences in threshold level between the ipsilateral and contralateral ears were calculated, and the temporal pattern and dose-response relation of hearing loss were analyzed using statistical methods that take into account the correlation between two ears in the same subject and repeated, sequential measurements of each subject. RESULTS: Of the 40 patients enrolled in this study, 35 qualified for analysis. Four patients who received concurrent chemotherapy and RT were analyzed separately. The 31 unilaterally treated patients received a median dose of 47.4 Gy (range, 14.1-68.8 Gy) to the ipsilateral inner ear and 4.2 Gy (range, 0.5-31.3 Gy) to the contralateral inner ear. Hearing loss was associated with the radiation dose received by the inner ear (loss of 210dB was observed in ears receiving >/=45 Gy) and was most appreciable in the higher frequencies (>/=2000 Hz). For a 60-year-old patient with no previous hearing loss in either ear, after receiving 45 Gy, the ipsilateral ear, according to our clinical model, would have a 19.3-dB (95% confidence interval [CI], 15.5-23.0) and 5.4-dB (95% CI, 3.5-7.5) hearing decrement compared with the contralateral ear for 8000 Hz and 1000 Hz, respectively. Age and an initial hearing difference within an ear pair also affected hearing loss. The baseline hearing threshold was inversely related to radiation-induced hearing loss. The degree of hearing loss was dependent on the frequency tested, age, baseline hearing, and baseline difference in hearing between a patient's two ears. CONCLUSION: High-frequency (>/=2000 Hz) hearing acuity worsens significantly after RT in a dose-dependent fashion. A larger number of patients needs to be studied to validate these results. This knowledge can be applied to create guidelines regarding future dose limits to the auditory apparatus for patients undergoing head-and-neck RT.     

原发性中耳炎癌复发一例报告及文献复习

目的原发性中耳类癌很少见,国内尚未见报道,现报告一例并结合文献复习进行讨论.方法患者女性,48岁,右中耳肿瘤切除术后19年复发,将活检标本作组织学(HE染色)、组织化学(奥辛蓝、PAS、嗜银染色)、免疫组化染色(SP法)检查.结果组织学,前后两次活检肿瘤形态一致,主要由实体小梁状及腺管状两种结构组成,瘤细胞异形性小,未见核分裂,但可见肿瘤呈浸润性生长.组织化学,奥辛蓝(AB)、过碘酸雪夫氏反应(PAS)染色阴性,嗜银染色胞浆内可见嗜银颗粒.免疫组化,突触素(Syn)、神经特异性烯醇化酶(NSE)、细胞角蛋白(CK)弥漫阳性,嗜铬蛋白(CgA)灶性阳性,溶菌酶(Lyz)阴性.结论原发性中耳类癌复发,同时与中耳腺瘤和中耳副节瘤等肿瘤鉴别.     

Contouring the middle and inner ear on radiotherapy planning scans

The purpose of this study was to establish guidelines that help radiation oncologists contour the inner and middle ear on treatment planning scans. The radiotherapy computed tomography (CT) scans of 15 previously treated patients were reviewed for the ability to identify 3 separate auditory structures. The middle ear, the cochlea, and the vestibular apparatus were identified and contoured on each scan using anatomic landmarks. The volume and maximum axial dimension of each contour were calculated. The middle ear, cochlea, and vestibular apparatus were identified on all scans. The middle ear was defined by the tympanic membrane laterally and by the interface between air and the temporal bone in all other directions. The plane of the internal auditory canal through the temporal bone was the landmark distinguishing the vestibular apparatus from the cochlea. The mean volume of the middle ear, vestibular apparatus, and cochlea were 0.58 cm3, 0.44 cm3, and 0.14 cm3, respectively. The maximum axial dimension across the contour averaged 1.57 cm for the middle ear, 1.10 cm for the vestibular apparatus, and 0.69 cm for the cochlea. A reference atlas was constructed that shows the contour of each structure on 5 consecutive CT images. Accurate identification of the middle ear and inner ear structures on radiotherapy planning scans is possible and is necessary if critical auditory organs are to be spared during radiotherapy of targets that are located near the base of the skull. The information generated in this study will help radiation oncologists contour auditory structures accurately.     

Histologic studies on otomycosis

Fungal infections of the ear are mostly described as mycoses of the auditory canal. The aim of our investigations was to find out how fungi colonize the ear in immunocompetent patients. In the years from 1993 to 2000, 128 patients suspected of having otomycosis were examined. Of these 115 patients suffered from chronic otitis media with persisting tympanum perforation and otorrhea. A further 13 patients had clinical signs of otitis externa only. In 54 out of 139 samples, fungi were found in the auditory canal, in five on the tympanic membrane, and in five in the middle ear. Two-thirds were isolated as moulds and one-third as yeasts. Dominating species were Aspergillus niger and Candida parapsilosis. Samples of 15 patients suspected of having mastoiditis or cholesteatoma were examined histologically. Fungal hyphae were observed in the middle ear cavity and/or between horny lamellae of cholesteatoma in 4 patients. In the middle ear of immunocompetent patients chronic-hyperplastic (polypous) inflammation was detected with increased production of mucus, which probably promotes the colonization with pathogenic fungi as in the middle ear just like in the auditory canal.     

鼻咽癌患者的中耳压力检测及其临床意义的探讨

目的：观察尚未接受放疗的鼻咽癌（ＮＰＣ）患者的耳部并发症情况,测量其中耳压力并探讨其临床意义。方法：调查１６０例ＮＰＣ患者放疗前一周内耳部并发症情况,并测量其中耳压力（ＭＥＰｓ）,另选择８０例鼻咽慢性炎症患者作对照组测量ＭＥＰｓ。结果：尚未放疗的ＮＰＣ患者有３７．５％（６０／１６０）并发分泌性中耳炎,ＮＰＣ组较对照组有一较高的中耳负压,不同ＴＮＭ分期及不同肿瘤部位的ＮＰＣ患者均普遍存在中耳高负压。     

Epidermal growth factor receptor as a novel molecular target for aggressive papillary tumors in the middle ear and temporal bone

Adenomatous tumors in the middle ear and temporal bone are rare but highly morbid because they are difficult to detect prior to the development of audiovestibular dysfunction. Complete resection is often disfiguring and difficult because of location and the late stage at diagnosis, so identification of molecular targets and effective therapies is needed. Here, we describe a new mouse model of aggressive papillary ear tumor that was serendipitously discovered during the generation of a mouse model for mutant EGFR-driven lung cancer. Although these mice did not develop lung tumors, 43% developed head tilt and circling behavior. Magnetic resonance imaging (MRI) scans showed bilateral ear tumors located in the tympanic cavity. These tumors expressed mutant EGFR as well as active downstream targets such as Akt, mTOR and ERK1/2. EGFR-directed therapies were highly effective in eradicating the tumors and correcting the vestibular defects, suggesting these tumors are addicted to EGFR. EGFR activation was also observed in human ear neoplasms, which provides clinical relevance for this mouse model and rationale to test EGFR-targeted therapies in these rare neoplasms.     

中耳癌14例治疗报告

对１４例中耳癌患者的临床资料进行了总结和分析。均采用综合治疗，即手术加术前、后放疗，其疗效与单纯放疗相比，发现综合治疗并无提高，而且手术并发症多。因此，建议对中耳癌患者首选放射治疗。手术适应于乳突根治术或放疗失败的晚期病例。颞骨切除后行面、副神经吻合，可大致保持面部肌肉对称，借助动肩以维持面肌运动，可有效的防止患侧面肌萎缩。对颈部淋巴结阴性患者无必要进行预防性清扫或放射治疗。     

Post operative sensorineural hearing loss after middle ear surgery

Mild Sensorineural hearing loss subsequent to middle ear surgery has till today been an important complication to middle ear surgery inspite of advances in surgical techniques, operative instruments, monitoring devices and better treatment options. Lack of proper knowledge about this problem is because of under reporting of exact magnitude of hearing loss on account of difficulty in measuring hearing threshold of patients in immediate postoperative period as it may lead to post operative infection and discomfort to the patient. In our study of 80 cases carried out at ENT department, Baroda Medical College and S.S.G Hospital, Baroda, we have utilized weber’s lateralisation principle and measured bone conduction thresholds of patients undergoing middle ear surgery for evaluation of postoperative Sensorineural loss as a result of middle ear surgery. Probable causes of post operative hearing loss in a patient undergoing middle ear surgery are, noise due to drills, continuous suction irrigation, vibrations, inner ear injury, manipulation of ossicles and a few unknown reasons.     

Radiation tolerance of normal temporal bone structures: implications for gamma knife stereotactic radiosurgery

Popular current thought states that hearing loss and facial weakness after radiosurgery of vestibular schwannomas is a function of cranial nerve damage. Although this may be true in some cases, the middle and inner ear contain rich networks of other sensitive structures that are at risk after radiotherapy and that may contribute to toxicity afterward. We reviewed the limited reported data regarding radiation tolerance of external, middle, and inner ear structures, and perspectives for therapy with gamma knife stereotactic radiosurgery are addressed.