不完全分隔Ⅱ型内耳畸形患者影像学特征分析

目的 探讨不完全分隔Ⅱ型内耳畸形患者的影像学基本数据特征,为更好地认识该类畸形提供依据和临床指导。方法 将2020年1月—2021年12月新疆维吾尔自治区人民医院筛查的重度-极重度感音神经性耳聋患者根据Sennaroglu的分类方法,所有患者分为试验组（IP-Ⅱ）24例、对照1组（单纯前庭导水管扩大）15例、对照二组（内耳畸形）28例,对颞骨HRCT及内耳MRI进行图像后期处理,并完成各指标的测量,包括耳蜗高度、前庭长径、前庭宽径、前庭导水管外口宽度等,应用单因素方差分析比较3组间的各个指标的差异。结果 3组耳蜗高度、耳蜗底周长度、耳蜗底周管径、蜗孔宽度、前庭长径、前庭宽径组间差异有统计学意义（P<0.05）。不完全分隔试验组和对照一组的前庭导水管外口宽度、前庭导水管中段宽度、内淋巴囊长度、宽度比较,差异无统计学意义（P>0.05）。结论 不完全分隔IP-Ⅱ型影像学典型特征为耳蜗中周、顶周融合,伴前庭、前庭导水管扩大,以及内淋巴管、内淋巴囊扩大,本资料相关数据的测量对于认识IP-Ⅱ型内耳畸形具有重要的临床意义。     

Mondini内耳畸形患者的多通道人工耳蜗植入

目的探讨Mondini内耳畸形患者人工耳蜗植入方法及手术前后听力评估和术后言语康复效果。方法回顾性分析经乳突面隐窝进路行人工耳蜗植入的19例Mondini内耳畸形患者的临床资料，并将其中10例患者植入后听力情况与10例耳蜗发育正常的人工耳蜗植入者(对照组)进行比较。结果19例Mondini内耳畸形患者中，32个电极全部植入者12例，28个电极植入者2例，26个电极植入者2例，22个电极植入者2例，20个电极植入者l例；术中7例发生多量脑脊液流出(ozze)，5例发生脑脊液井喷(gusher)，7例无脑脊液外溢。两组患者术后听力接近，均在30～40dB HL左右，无严重并发症发生，17例Mondini内耳畸形患者术后听觉言语康复效果与耳蜗发育正常者接近，2例稍差。长期效果有待进一步观察。结论人工耳蜗植入适用于Mondini内耳畸形患者，但手术前应进行全面的听力学及影像学评估。     

不同内耳畸形人工耳蜗植入效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHODS: Among 410 patients who received multi-channel cochlear implantations from 1996 to 2004 in Beijing Tongren Hospital, 82 patients were diagnosed with inner ear malformations and implanted. A retrospective analysis was performed about the surgical characteristics and mapping characteristics after implantation. RESULTS: (1) All patients had auditory sensations. (2) Gusher was more common than the normal cochlear implantation. (3) The electrodes were inserted in the "cochleostomy" in full length of 80 Patients, but 2 pairs of electrodes remained outside of "cochleostomy" in 2 patients. (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, the T level and C level were similar with the normal cochlear implantation. The results had no significant difference in compare with normal cochlear group (P > 0. 05). (6) The abilities of speech discrimination and spoken language were improved through rehabilitation. CONCLUSIONS: The cochlear implantation can be performed safely in inner ear malformations. The outcome of hearing rehabilitation for patients with inner ear malformations are similar to those children with normal cochlear structure followed the multi-channel cochlear implantation.     

先天性内耳畸形的人工耳蜗植入

目的 探讨先天性内耳畸形引起重度感音神经性聋者人工耳蜗植入的有关问题。方法 2001年1月至2003年4月间对内耳畸形引起极重度感音神经性聋18例进行了人工耳蜗植入术。结果 18例中前庭水管11例，Waardenburg综合征3例，Mondini畸形3例，Usher综合征1例。全部病例采用Nucleus24型人工耳蜗，其中对前庭水管5例采用Contour植入体，其余病例采用直电极植入体。术中发现前庭水管11例开骨窗后仅有外淋巴搏动，但无井喷(脑脊液漏)，电极植入顺利。Waardenburg综合征3例和Mondini畸形3例中各有1例伴发圆窗骨性封闭畸形。结论 前庭水管者人工耳蜗植入手术顺利，术后效果与耳蜗发育正常者相同。如Mondini和Common Cavity等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形，要充分估计手术难度和避免术后可能出现的脑脊液耳鼻漏及其颅内感染。     

内耳畸形患儿的人工耳蜗植入

目的：探讨几种内耳畸形患儿的人工耳蜗植入效果。方法：对10例内耳结构异常的感音性耳聋患儿进行了人工耳蜗植入。结果：术后随访0．5～4．0年，10例基本达到了较满意的听觉言语恢复效果。结论：内耳畸形息儿行人工耳蜗植入，应严格进行术前听力学与影像学的评估，严格掌握手术的适应证，可以达到较满意效果。     

内耳畸形与耳蜗骨化患者的耳蜗植入

目的探讨先天性内耳畸形与耳蜗骨化患者耳蜗植入术的有关问题.方法对我院2002年10月～2004年2月间行耳蜗植入术的8例内耳畸形和2例耳蜗骨化的患者进行回顾性分析.结果4例大前庭水管综合征患者术中有外淋巴液搏动;3例Mondini畸形和1例共同腔畸形患者术中出现井喷;1例耳蜗部分骨化患者术中误将电极插入内听道后纠正;另一例耳蜗部分骨化患者植人短电极.1例Mondini畸形患者术后发生少量脑脊液耳鼻漏,保守治疗3月后痊愈,其余患者无并发症.所有患者均成功开机.结论对于内耳畸形或耳蜗骨化的患者,耳蜗植入术前详细的影像学评估,对术中困难的充分估计和正确、规范处理是手术成功的基本条件.     

先天性内耳畸形并中耳畸形患者多通道人工耳蜗植入术3例报告

目的：探讨先天性内耳畸形并中耳畸形患者行多通道人工耳蜗植入术的方法及效果。方法：1995年5月-2002年5月我院为3例罕见的先天性内耳畸形并中耳畸形患者经乳突进路行人工耳蜗植入术,分别植入27、28、32个电极,植入后3个月行声场测听。结果：3例患者均成功地行人工耳蜗植入,术中、术后无严重并发症发生,术后声场测听听阈达35-40dBHL。结论：罕见的先天性内耳畸形并中耳畸形患者也可行人工耳蜗植入术,术后效果满意。     

先天性内耳畸形患者多通道人工耳蜗植入的效果观察

目的探讨多通道人工耳蜗在先天性内耳畸形患者植入的效果.方法对16例先天性内耳畸形患者(畸形组,Mondini畸形12例,大前庭导水管综合征4例),2例经前庭窗植入电极,3例经鼓岬植入电极,11例经圆窗龛前上缘植入电极.2例术中发生镫井喷.以10例耳蜗发育正常的植入者作为对照组,进行术后效果对比.结果畸形组与对照组术中和术后并发症差异无显著性意义.术后听阈畸形组多数患者达到30～40 dB HL,与正常组相似,仅少数患者听阈稍高.经统计学分析差异无显著性意义(P>0.05).听觉语言康复效果畸形组多数与对照组接近.结论多通道人工耳蜗植入适用于绝大多数先天性内耳畸形的患者,术后听力及语言康复效果满意.     

复杂内耳畸形患儿人工耳蜗植入术后疗效分析

目的 探讨复杂内耳畸形患儿人工耳蜗植入术后的听觉言语发育效果.方法 回顾性分析2018年1月至2019年6月于西京医院行人工耳蜗植入术的17例复杂内耳畸形语前聋(内耳畸形组)患儿的临床资料,其中双侧植入1例,右侧植入8例,左侧植入8例,植入年龄1～10岁(平均3.49岁),男6例,女11例;IP-1畸形2例,IP-3畸...     

内耳结构异常患者的人工耳蜗植入术

目的探讨对内耳结构异常患者行人工耳蜗植入的方法并评估其术后效果。方法回顾性分析2000年1月~2010年5月行人工耳蜗植入术的24例内耳结构异常患者的临床资料,包括耳蜗骨化2例、Waarden-burg综合征Ⅱ型8例、Mondini畸形12例、共同腔畸形1例、半规管缺如1例;随机选择30例内耳结构正常的相同年龄段接受了人工耳蜗植入的极重度感音性聋患者作为对照组;分析并比较两组患者的术前听力和影像学资料以及手术植入方法和术后听觉言语康复情况。结果 1例共同腔畸形和2例Mondini畸形患者术中出现井喷,另有10例Mondini畸形、2例Waardenburg综合征Ⅱ型病例在耳蜗钻孔后见外淋巴液搏动性涌出,经抬高头位降低液压并以筋膜粒严密封堵开窗口等处理完成电极植入;2例耳蜗骨化者经扩大开窗范围并剔除骨化组织后分别植入8个和12个电极;其余病例均经常规方法完成电极植入。内耳结构异常组患者人工耳蜗植入术后均获得听觉,所有患者均未出现严重并发症。术后6个月内耳结构异常组啭音声场测听平均听阈35.4±4.9 dB HL,闭合式词表言语识别率平均为75.18%±3.1%,开放式词表言语识别率平均为70.32%±2.5%;对照组上述三项结果分别为31.4±5.5 dB HL、81.63%±3.7%、76.77%±5.4%,两组间比较差异无统计学意义(P>0.05)。结论对内耳结构异常的极重度感音性聋患者行人工耳蜗植入安全有效,但针对不同的内耳畸形需采取不同的处理方法。     

人工耳蜗植入对前庭功能影响的研究进展

人工耳蜗植入术(Cochlear implant,CI)已开展三十余年,是重度、极重度感音神经性耳聋患者重获听力的重要治疗手段。CI术后儿童中前庭症状鲜有发生,而成年人CI术后出现前庭功能症状较常见,特别是老年人风险更高。绝大多数CI术后前庭功能受损的患者在经康复训练后前庭功能得到了补偿,表明术后出现的前庭症状是可恢复的。CI术后前庭功能变化的原因和影响因素众多,涉及了个体、手术、检查方法、术后康复等多个方面。结合CI术前、术后评估及术后处理对策对术后前庭功能的保留提供了参考。     

Cochlear Function in Ears with Immunomediated Inner Ear Disorder

The aim of the present study was to evaluate the performance of ears with inner ear disorder, responsive to immunosuppressive drugs, in advanced tests designed to assess primary cochlear functions (temporal integration, frequency selectivity, cochlear mechanics). The results of this study suggest that immunomediated inner ear disease results, in the acute clinical stage, in the development of endolymphatic hydrops, which increases the stiffness of the vibrating structures within the inner ear and causes dysfunctions of the outer hair cells. Our patients presented with upsloping or flat sensorineural hearing loss, absence of evoked otoacoustic emissions and distortion-product otoacoustic evoked emissions and abnormal temporal integration, frequency selectivity and cochlear mechanics. Following immunosuppressive treatment, hydrops recovered, hearing subsequently returned to normal, the audiometric curve became flat at low-to-middle frequencies and primary cochlear function tended to normalize. This study seems to support the usefulness of testing primary cochlear functions in order to monitor the clinical course of immunomediated inner ear disorders.     

Cochlear Implantation Without Mastoidectomy: The Pericanal Electrode Insertion Technique

Objective - A new minimally invasive cochlear implantation method with direct electrode insertion through the external auditory canal (EAC) is presented. Material and methods - Surgery begins with a retroauricular skin incision. The bony surface of the mastoid plane behind the ear is dissected free and the skin of the EAC is elevated together with the posterior part of the tympanic membrane. Cochleostomy is performed through the EAC with a microdrill anterior to the round window. A rim is drilled into the postero-superior region of the bony EAC immediately above the incus towards the outer border of the EAC and connected to the retroauricular surface by a short tunnel. The implant device is placed in the usual retroauricular area. The electrode is inserted through the tunnel and rim into the tympanic cavity and pushed into the cochleostomy hole. The electrode is immobilized in the rim using glass ionomer cement and covered with bone dust. After placement of the ground electrode, the retroauricular incision is closed. The tympano-meatal flap is replaced and a dressing is put into the EAC. Results - The new method has been applied thus far in 15 adults and pre-adolescent deaf patients (8 females, 7 males). A MED-EL Combi 40+ device was used in 14 patients and a Nucleus CI24M in 1. Pericanal electrode insertion was easy, with insertion depths into the cochlea of &;#83 30 mm with the MED-EL and of 20 mm with the Nucleus device. There were no surgical complications, infections or electrode extrusions during postoperative observation periods ranging from 6 months to >2 years. The functional results were comparable to those obtained with cochlear implantation via mastoidectomy. The pericanal electrode insertion technique has several advantages, the most important being that the danger of facial nerve damage is minimized and that the operating time is reduced by up to 50%. Conclusion - Cochlear implantation with pericanal electrode insertion is a simple, fast and particularly safe option which may replace the classical transmastoidal cochlear implantation method in adults and older children.     

Dynamics of Inner Ear Pressure Change Caused by Intracranial Pressure Manipulation in the Guinea Pig

Previous studies have shown that pressure changes in the cerebrospinal fluid compartment are transmitted to the inner ear. The main route for pressure transfer is the cochlear aqueduct, about which little is known with regard to its dynamic properties. In the present study, sudden intracranial pressure changes (square waves and short pulses) were created in guinea pigs by means of an electronically controlled infusion system. Simultaneously with pressure manipulation, hydrostatic pressure was monitored in both the peridural space and the perilymphatic compartment of the inner ear. The onset of an inner ear pressure change following manipulation of intracranial pressure was immediate. Inner ear pressure increased or decreased without a measurable time lag, and equalized within a few seconds. During square wave intracranial pressure manipulation, inner ear pressure equalized somewhat more slowly after pressure increase than after pressure decrease. To a first approximation, the pressure equalization curves for the inner ear could be fitted with a single exponential function, rising or falling with a time constant in the range 1-3 s, and the system can be described as a low-pass filter composed of a constant compliance and a constant flow resistance. Detailed analysis, however, showed small deviations from a purely exponential recovery process. With a more complicated (non-linear) model, almost perfect fits to the inner ear pressure equalization curves could be obtained. This non-linearity may be a consequence of the dependence of the compliance and/or flow resistance on pressure.     

内耳结构异常的语前聋人工耳蜗植入儿童听觉语言康复效果评价

目的评价不同类型内耳结构异常的语前聋儿童人工耳蜗植入术后听觉语言康复效果。方法选取人工耳蜗植入儿童10例(12耳),其中内耳结构发育正常的3例,前庭导水管扩大3例,Mondini畸形1例,蜗孔狭窄1例,内听道狭窄听神经纤细2例,分别采用电刺激诱发听神经复合动作电位(electrically evoked compound action potential,eCAP)、助听听阈、听障儿童听觉能力、语言能力评估标准及方法,从听神经客观电生理水平、听察觉阈、听觉辨识与理解能力以及语言能力4方面评价患儿的康复效果。结果①客观电生理:单纯前庭导水管扩大患者听神经对电刺激敏感度与内耳发育正常者相似,Mondini畸形患者比内耳发育正常者略高,蜗孔狭窄及听神经纤细患者听神经敏感度较差;②听察觉阈:与内耳结构正常及轻度畸形(Mondini畸形)患者相比,蜗孔狭窄及听神经纤细患者听察觉阈偏高,高频比低频阈值低;③听觉辨识与理解能力:在安静环境近距离交谈情境下,单纯前庭导水管扩大、Mondini畸形及蜗孔狭窄患者对熟悉语词和短句的辨识与理解能力与内耳发育正常者无差别,听神经纤细患者得分较低;④语言能力:单纯前庭导水管扩大、Mondini畸形患者与内耳发育正常者无差别,蜗孔狭窄患者的语言交往与表达能力落后于内耳发育正常者,听神经纤细患者在语法、理解、交往、表达4个维度均落后于内耳发育正常者。结论语前聋儿童人工耳蜗植入术后听觉语言康复效果需要使用多种方法综合评价。Mondini畸形患者给予足够电刺激量后,其听觉语言能力可达到内耳发育正常者水平。蜗孔狭窄、听神经纤细等严重内耳畸形患者,人工耳蜗对其听觉语言能力发展有一定帮助,家长需要建立合理期望值并坚持长期康复。     

中耳感染性疾病Ⅰ期和分期人工耳蜗植入病例分析

目的总结中耳感染性疾病患者人工耳蜗植入(Cochlear Implant,CI)的临床经验。方法对2000年12月-2019年3月在解放军总医院行CI伴中耳感染性疾病患者的临床资料进行回顾性研究。对Ⅰ期或Ⅱ手术患者的中耳病变性质和范围、手术方式、术腔填塞物种类、手术疗效以及术后并发症等进行总结分析。结果术后随访9个月-10年,Ⅰ期植入11例,其中,慢性化脓性中耳炎静止期5例,活动期2例,中耳胆脂瘤4例(胆脂瘤范围较小,或有完整包膜)。Ⅱ期植入9例,其中,慢性化脓性中耳炎活动期6例,静止期1例;中耳胆脂瘤2例(胆脂瘤范围较大,或包绕正常组织,不易完整清除)。分期植入组中有1例耳蜗植入术后9年电极脱出。其余病例通过结合病例特点,采用不同手术方式均取得满意的治疗效果。结论对于中耳感染性疾病的患者,人工耳蜗分期植入相对于同期植入更加安全。Ⅰ期植入应尽可能选择病变较轻,病灶局限易完整清理的病例,术式选择应在彻底清除病灶的基础上尽量选择损伤范围较小的术式。