Hearing preservation after translabyrinthine approach performed to remove a large vestibular schwannoma

We report the case of a 27-year-old woman from whom a left vestibular schwannoma (grade IV) was removed by a translabyrinthine approach. The initial period was uneventful except for a few days of vertigo. This patient had no facial paresis (House-Brackman grade I). She was seen 1, 3, 6 and 12 months after operation and reported a sensation of hearing in the left ear. The audiological studies showed a left medium hearing loss. At the 1-year follow-up visit, her hearing status was unchanged. A hearing aid was fitted and functioned satisfactory. At the 5-year follow-up visit, audiometry studies were unchanged. Our case demonstrated that some hearing preservation could be obtained even if the vestibular schwannoma was large and excised by a translabyrinthine approach. Hearing with the help of a good hearing aid allowed a satisfactory function in this case. We believe that it is possible to preserve some hearing after translabyrinthine approach even if no care was taken to preserve the membraneous labyrinth and the loss of endolymph and even if the vestibular schwannoma was large. However, further experimental studies are needed to determine and understand the mechanisms of hearing preservation in this case.     

Impact of cochlear implantation on peripheral vestibular function in adults

ObjectiveCochlear implantation may have a detrimental effect on vestibular function and residual hearing. Our goal was to investigate the impact of cochlear implantation on peripheral vestibular function and the symptomatology that ensues.Material and methodsA prospective observational study included all adults undergoing cochlear implantation by the same operator between July 2014 and December 2015, with pre- and postoperative (4 months) neurovestibular balance examination comprising a questionnaire and clinical tests [head impulse test (HIT), head-shaking test (HST), skull vibration test (SVT)] and instrumental tests [caloric test of the lateral semicircular canal and cervical vestibular-evoked myogenic potentials (cVEMP)].ResultsTwenty-two patients were included, with a mean age of 62 years and sex-ratio of 1.2. Before implantation, 50% of subjects (n = 11) reported at least one episode of vertigo associated with balance disorder during their life. After implantation, there were 11 cases of vertigo but only one patient described persistent discomfort related to vertigo 4 months after surgery. Patients with impaired vestibular function after 4 months, taking all symptoms together, were all aged more than 75 years. HIT was abnormal in 18% of cases before implantation and in 59% after (P = NS). HST showed nystagmus in one patient both before and after surgery. Only 18% of patients showed nystagmus induced by SVT before surgery, increasing to one-third after surgery (P = NS). Caloric test of the lateral canal showed hypofunction in 50% of cases before surgery, including 10% of cases with areflexia. This rate increased after surgery to 58%, with 18% areflexia (P = NS). cVEMPs were not detected in 68% of cases before implantation and this rate increased to 86% after surgery (P = NS). There were no significant associations (P > 0.05) between test results and symptoms.ConclusionsIn the medium term, although older subjects more frequently presented vestibular disorder, cochlear implantation induced little vertigo or balance disorder, sometimes even improving vestibular function. However, vestibular disorders were frequent preoperatively and increased postoperatively. We tested vestibular function on different stimulation frequencies and yet found no correlation between postoperative test results and postoperative vertigo.     

听神经瘤术后单侧耳聋听力重建问题初探

摘要：目的探讨听神经瘤术后单侧耳聋患者人工听觉重建的效果。方法回顾性分析2016年7~12月上海交通大学医学院附属第九人民医院耳鼻咽喉头颈外科收治的听神经瘤术后单侧耳聋行人工听觉植入患者8例,其中同侧一期骨桥植入患者6例,二期同侧骨桥植入2例。比较术前及术后1年噪声下言语识别能力以及生活质量改变。结果所有病例均成功手术,肿瘤均完全切除。至随访1年,8例患者均正常使用听觉植入装置。骨桥植入患者噪声下言语识别能力明显提高,生活质量明显改善。结论骨桥可以帮助听神经瘤术后单侧耳聋患者有效地进行听觉功能重建,需要根据患者情况制订个体化的治疗方案。

     

Pneumolabyrinth,intracochlear and vestibular fluid loss after cochlear implantation

The present case was a 38-year-old male who presented with progressive hearing loss, resulting in profound bilateral hearing loss. He had a past history of childhood medulloblastoma, which was treated with posterior fossa craniotomy and radiotherapy. A ventriculoperitoneal (VP) shunt was put in place to manage the hydrocephalus. Cochlear implantation (CI) was carried out on his right ear by a standard procedure. At CI activation, the electric impedance of the electrode was very high, and computed tomography revealed that there was no area of liquid density, suggesting depletion of the perilymph in the cochlea and vestibule. Eight months later, the impedance improved gradually, and the cochlea was filled with perilymph. Consequently, one of the causes of the pneumolabyrinth in the present case was that a scarred stenotic cochlear canaliculus secondary to surgery or radiation therapy might have prevented the CSF from filling the scala. In addition, it is also possible that the VP shunt might have altered the CSF pressure, leading to depletion of the perilymph.     

Outcomes of cochlear implantation in patients with neurofibromatosis type 2

In neurofibromatosis type 2 (NF2) bilateral vestibular schwannomas (VS) or their treatment usually results in bilateral hearing loss. Cochlear implantation (CI) was traditionally not used in these patients due to concern that retrocochlear disease would render the implant ineffective. This paper describes the auditory outcomes of CI in 13 patients with NF2 and includes patients with untreated VS and patients undergoing VS removal with cochlear nerve preservation. The non-user rate was 7.7%. Of the active users, median CUNY score was 98%, median BKB score in quiet was 90% and median BKB score in noise was 68%. CI is a viable option in selected patients with NF2.     

Double challenge: cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

ObjectiveVestibular dysfunction associated with cochlear implantation is rare. It is usually seen in patients with otosclerosis due to spread of electrical activity throughout the demineralized bone. A 17-year old female with progressive hearing loss 2 years after meningitis and vestibular dysfunction in the implanted ear is presented in this study.FindingsThe patient had mild hearing loss in the right ear and total hearing loss on the left side because of complete ossification of the cochlea following meningitis. She had to have cochlear implantation in the right ear because of progression of hearing loss. She had successful implantation but she experienced vestibular dysfunction following activation of cochlear electrodes. Closure of two electrodes caused disruption of auditory programming. Then the patient was subjected to long term vestibular rehabilitation program.ConclusionTiming for implantation before the completion of cochlear ossification is crucial not to miss the chance for hearing restoration. However, difficulties in hearing rehabilitation due to extensive ossification can be doubled by vestibular problems triggered by stimulation of the vestibular nerve by cochlear electrodes. Attempts to reduce the balance problem will complicate auditory programming. Vestibular rehabilitation for long term helps to carry on hearing progress.     

The impact of vestibular status prior to cochlear implantation on postural control: A multiple case study

Objective: The goal of the present study was to investigate the impact of unilateral cochlear implantation on postural control in relation to the vestibular status before CI surgery.

Methods: We recruited 17 participants (four CI candidates and 13 hearing controls) and performed complete vestibular evaluation (cVEMP, oVEMP, vHIT) and postural evaluation using a force platform, prior and following unilateral cochlear implant surgery.

Results: Our study suggests that an increase in postural sway following cochlear implant was present only for the participants that received the implant in the ear with the better vestibular function. cVEMP and oVEMP measures in the implanted ear prior to unilateral cochlear implantation may help to predict postural control performance following surgery.

Discussion: A thorough evaluation of the vestibular function, as described in the present study, could not only be helpful to make a more accurate prognosis of the risks of fall following cochlear implantation, but also to provide proper vestibular rehabilitation for at-risk patients.     

人工耳蜗及相关技术的进展

人工耳蜗植入(cochlear implantation, CI)是重度-极重度感音神经性聋患者的听觉和言语康复的主要方法, 随着人们对听觉系统和中枢可塑性的深入研究,手术技术及辅助工具的开发,以及人工耳蜗植入相关电子科技和材料科学的发展,人工耳蜗及相关技术有了很大的进步。本文分析人工耳蜗植入体,尤其是电极设计和言语处理器的升级、人工耳蜗植入适应证的拓宽、术前评估手段的完善及精准微创人工耳蜗植入和残余听力保留的开展,并对人工耳蜗植入相关技术进行展望。     

前庭水管扩大综合征患者的人工耳蜗植入术

目的　评价前庭水管扩大综合征患者人工耳蜗植入术的安全性和可行性。方法　 1995年 5月 1日～ 2 0 0 2年 6月 1日因双耳重 极重度感音神经性聋在北京协和医院接受人工耳蜗植入术的患者 312例中诊断为双耳前庭水管扩大者 10例 (3 2 % ) ,其中语前聋 7例 ,语后聋 3例。回顾性分析这 10例患者的临床资料。结果　 10例患者人工耳蜗电极植入顺利 ,8例耳蜗底回开窗时发生轻度井喷 ,迅速用颞肌筋膜牢固封闭圆窗制止井喷。全部患者术后未出现脑脊液漏、颅内感染、面瘫、中耳炎等并发症。开机 6个月时 8例患者具有开放性言语识别力 ,已进入普通学校 (幼儿园、小学、大学 )就读。另 2例语前聋的幼儿视觉强化测听听阈达 4 0dBHL ,与其他无内耳畸形的全聋儿童术后效果差异无显著性。全聋前语言能力较好的 5例患者术后语言能力明显好于语前聋的患者 ,语言交流基本听不出聋人特有的语音特征。另 5例患者语言均有不同程度的进步。结论　尽管前庭水管扩大患者在人工耳蜗植入术中可能出现井喷 ,但术后未出现并发症且听力 言语康复效果好 ,因此重 极重度聋的前庭水管扩大综合征患者行人工耳蜗植入术是安全可行的     

共同腔畸形人工耳蜗手术适应证及手术方法的探讨

目的：探讨共同腔畸形人工耳蜗手术适应证以及人工耳蜗电极植入人路的选择。方法：在对重度或全聋患者进行人工耳蜗植入术前影像掌检查中,发现了6例耳蜗、前庭、外半规管呈共同腔畸形,其中5例有残留听力,1例未查到残留听力。结果：6例影像学检查呈共同腔畸形患者中,对5例有残留听力患者进行了人工耳蜗植入,其中3例选择了常规入路植入电极,2例选择了经乳突侧入路植入电极,术后均建立了人工耳蜗的听觉反应。1例因未查到残留听力,放弃了人工耳蜗手术治疗。结论：有残留听力的共同腔畸形患者,如果能够接受术后听觉言语识别效果差的事实,可以进行人工耳蜗手术。无残留听力或无法了解到有听觉反应的共同腔畸形患者,在现有技术条件下应放弃人工耳蜗植入手术。     

95例大前庭水管综合征的临床分析

目的　调查大前庭水管综合征 (largevestibularaqueductsyndrome,LVAS)的发病、遗传和听觉损失情况 ,探讨LVAS之前庭水管外口宽度与听力损失的关系。方法　回溯性搜集 1 992～ 2 0 0 2年 ,95例 (1 90耳 )双侧LVAS的病史 ,听力和前庭功能检查 ,用每例LVAS颞骨CT片的标尺 ,测量前庭水管外口的宽度。结果　 95例 (1 90耳 )LVAS中 ,男 65例 ,女 30例 ,男女比为 2 2∶1。有家族史者 1 2例(1 2 6 % )。平均就诊年龄 7 6岁 ,年龄范围从 1 2～ 39岁。本组绝大多数误诊 ,因轻度外伤而致重度感音神经性聋 2 1例 (2 2 % ) ;上呼吸道感染致聋 1 3例 (1 3 6 % ) ,误诊为突发性聋 9例 (9 5 % ) ;拟诊氨基糖甙类中毒 5例 (5 3 % )。重度感音神经性聋 1 72耳 ,占 92 4%。 77例 (1 54耳 )前庭水管外口宽度( x±s)为 (7 5± 1 2 )mm ,听力损失与外口宽度不成比例。听力损害可能与富含蛋白的内淋巴从内淋巴囊倒流于耳蜗和前庭有关。结论　LVAS是独立的疾病 ,具有波动性及进行性感音神经性聋。诊断依靠颞骨CT扫描或磁共振成像 (magneticresonanceimaging ,MRI)。若听力波动后能改善可保守治疗 ,若明显听力下降 ,影响学习 ,可考虑采用耳蜗植入术     

17例人工耳蜗再植入原因分析

目的总结17例人工耳蜗患者耳蜗再植入的原因,探讨降低耳蜗再植入风险的方法,提高患者手术治疗满意度及术后听力言语康复水平。方法回顾性分析2012年11月—2018年7月解放军总医院海南医院17例采用面隐窝入路圆窗再植入人工耳蜗患者的临床资料,分析其再植入原因。结果17例患者均顺利完成耳蜗再植入手术,再植入术后随访观察患者满意度高,人工耳蜗助听听阈大幅改善,言语分辨能力明显提高,患者听力言语康复效果提升。17例人工耳蜗再植入原因分别为:植入体故障6例;植入体不工作2例;植入体受撞击后损坏2例;植入体接收刺激器移位1例; 植入体接收刺激器部位破裂1例;皮瓣感染3例,其中1例为右侧感染后原植入体同侧再植入,皮瓣感染切口无法愈合后右侧植入体取出后行左侧人工耳蜗植入;电极未完全植入1例;应患者要求取出旧植入体同侧再植入新型植入体1例。结论人工耳蜗再植入原因复杂多样,选择合适材料的植入体、避免剧烈撞击、执行严格的无菌操作以及进行准确的人工耳蜗植入术前评估是避免人工耳蜗再植入的关键。     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

听神经瘤显微手术后听力保存及影响因素

目的探讨听神经瘤显微手术后听力保存结果并分析其影响因素。方法回顾性研究1998年1月～2008年1月显微外科手术治疗听神经瘤32例,影像学检查（MRI）确定肿瘤大小及生长部位,听力学检查确定术前、术后听力水平,分析术前听力情况、肿瘤的大小及生长部位与术后听力保存之间的关系。结果32例听神经瘤均实现肿瘤完全切除。术后实用听力保存率为15．6％（5／32）。术前听力水平A级6例,B级8例,术后听力保存率分别为50％（3／6）和25％（2／8）。内听道型肿瘤（IAC）9例,直径1-19mm小肿瘤4例。听力保存率分别为44．4％4／9）;和25％（1／4）。20例哑铃型肿瘤,1例术后保存了实用听力。在IAC型肿瘤中,3例中心型肿瘤术后均实现了听力保存,近内耳门型肿瘤2例,1例术后具有实用听力。结论术前听力情况、肿瘤大小及生长部位是预测术后听力的重要因素。     

Knowledge about cochlear implantation: A parental perspective

Objectives: Cochlear implantation (CI) is used for children with severe to profound hearing loss who show little or no improvement using hearing aids. This study explored parental knowledge of their children’s CI.

Methods: A cross-sectional study involving the parents of 115 pediatric CI patients was conducted at King Abdullah Specialized Children’s Hospital in Riyadh, Saudi Arabia. Parents were interviewed by telephone using a 50-question validated questionnaire.

Results: Most parents of children with CI reported being comfortable in using the internet (68.7%) and social media (40.9%) to obtain information regarding CI. Although most parents of children with CI relied on health professionals and websites as their main sources of information, they were also able to obtain necessary information at meetings for CI patients and health professionals. Parents of children with CI felt they had sufficient information regarding the impact of hearing loss (78%) and CI (71%) on speech understanding and language development; however, they had insufficient information regarding criteria for CI candidacy, available brands of CI devices, and the advantages and disadvantages of each.

Conclusion: Parents reported that health professionals were the ideal source of information regarding hearing loss and CI. Moreover, our study showed that parents should learn more about cochlear implant devices, the post-implantation process, and candidacy criteria.