脑膜炎后耳蜗骨化患者人工耳蜗植入的临床分析

目的总结因脑膜炎而致严重感音神经性听力损失的人工耳蜗植入经验,探讨其诊疗策略。方法回顾性分析2010年9月—2020年9月于首都医科大学附属北京同仁医院耳鼻咽喉头颈外科因脑膜炎致重度及极重度感音神经性听力损失进行人工耳蜗植入的22例患者资料,其中成人13例,儿童9例。对其术前影像学、手术所见进行分析。结果颞骨HRCT检查中16例(72.7%)诊断耳蜗骨化;6例(27.3%)未诊断,但经手术探查证实存在耳蜗骨化。18例内耳MRI检查中,3例(16.7%)未发现耳蜗信号改变,而经手术探查存在耳蜗骨化。两者结合后检出率为90.9%(20/22),20例(90.9%)可见合并半规管等其他迷路病变。术中未见耳蜗骨化者1例(4.5%),圆窗骨化7例(31.8%),耳蜗底转骨化14例(63.6%)。电极完全植入者18例(81.8%),部分植入者4例（18.2%）。结论脑膜炎后耳蜗骨化可致严重感音神经性听力损失,人工耳蜗是理想的治疗策略,建议尽早植入。术前颞骨HRCT和MRI对确定是否适合耳蜗植入和术前计划至关重要,诊断存在假阴性,但联合检查可大大提高耳蜗骨化的术前诊断阳性率。     

重度-极重度感音性聋老年人人工耳蜗植入的初步探讨

耳聋是困扰老年人的最常见的疾病之一。目前,人工耳蜗植入(CI)是重度-极重度感音性聋且助听器效果不佳老年人的听觉和言语康复的最有效方法,但老年耳聋患者CI并不如婴幼儿和儿童听障人群开展的普及,并且老年人本身耳聋发生、听力学和听觉康复有其特殊性。从老年耳聋的发生率、耳聋常见原因和听力学特点,老年人CI的适应证,目前老年CI的开展情况,老年人CI的疗效特点等方面进行分析和讨论,并对老年耳聋患者CI的未来发展做展望。     

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.     

老年及老年前期人工耳蜗植入的临床初步研究

目的 探索人工耳蜗在双耳重度或极重度聋和全聋的老年患者中的临床应用安全性和效果.方法 2008年11月至2009年11月解放军总医院先后对8例50岁以上患者行人工耳蜗植入术,其中男5例,女3例;年龄52～76岁,中位数58岁.所有患者术前均为双侧极重度感音神经性听力损失,佩戴助听器无效或效果不佳.观察患者手术耐受及并发症情况.开机后3个月进行助听听阈评估和言语评估.结果 8例患者手术顺利,术中神经反应遥测均引出反应.术后康复顺利,无任何并发症.开机3个月后声场内言语频率平均助听听阈啭音(听力级)为35～50 dB,但言语测听结果个体差异较大.部分患者单音节、双音节、安静及噪声环境下的句子可实现较好识别率.结论 老年前期及老年患者可耐受全身麻醉状态下的人工耳蜗植入,只要术前准备充分则为安全的手术,但部分病例为中耳炎(胆脂瘤)或颞骨外伤等因素导致,需要特别注意中耳情况.老年重度以上听力损失患者人工耳蜗植入可明显改善言语识别能力.     

43例人工耳蜗植入患者基因检测及术后康复效果分析

目的总结川南地区43例人工耳蜗植入患者的基因突变类型,分析其人工耳蜗术后康复效果,了解两者的相关性。方法对43例耳聋患者行GJB2、SLC26A4、mtDNA 12S rRNA基因检测,有基因位点发生致病突变者为耳聋基因检测阳性,归为A组;其余为基因检测阴性,归为B组。两组患者均行单侧人工耳蜗植入术,于术前、术后3、6、12个月行听觉行为分级（categories of auditory performance,CAP）、言语可懂度分级（speech intelligibility rating,SIR）评估。结果43例患者中基因位点发生致病突变者14例（A组）;耳聋基因筛查阴性29例（B组）。A组中9例患者GJB2检测阳性,6例患者SLC26A4检测阳性,其中1例患者GJB2、SLC26A4检测结果均为阳性,两组患者术后1年内不同时间点 CAP 和SIR 评分均较术前明显提高,且评分随着术后时间的延长逐渐提高(P<0.05),但两组患者术前及术后不同时间点CAP和SIR评分比较均无差异(P>0.05)。结论43例患者中常见致病耳聋基因为GJB2、SLC26A4,人工耳蜗植入术能有效提高患者听力及言语能力,但耳聋基因突变与人工耳蜗植入术后康复效果无明显相关性。     

Magnetic resonance imaging in patients with meningitis induced hearing loss

The aim of this multicentre study was to compare T1 with T2 weighted MRI scans of the labyrinth after meningitis and to investigate whether waiting with scanning improved the reliability of diagnosing an ongoing process such as cochlear osteogenesis. Forty-five patients were included who suffered from meningitis induced hearing loss (radiological imaging <1 year after meningitis). Twenty-one gadolinium enhanced T1 and 45 T2 weighted MRI scans were scored by two radiologists regarding the condition of the labyrinth. These radiological observations were compared with the condition of the cochlea as described during cochlear implantation. A higher percentage of agreement with surgery was found for T2 (both radiologists 73%) than for T1 weighted MRI scans (radiologist 1: 62%, radiologist 2: 67%), but this difference is not significant. There was no significant difference between early (0–3 months) and late (>3 months) scanning, showing that radiological imaging soon after meningitis allows early diagnosis without suffering from a lower agreement with surgical findings.     

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.     

Translabyrinthine vestibular schwannoma resection with simultaneous cochlear implantation

Objectives: (1) Report a rare case of translabyrinthine resection of a sporadic vestibular schwannoma (VS) and concurrent cochlear implantation (CI). (2) Discuss pre-, intra-, and post-operative considerations in this unique patient population. (3) Describe surgical and audiologic outcomes reported in this population.

Methods: Case report and review of the literature. PubMed search ‘Cochlear Implantation’[Mesh] AND (‘Neuroma, Acoustic’[Mesh] OR ‘VESTIBULAR SCHWANNOMA’[All Fields] OR ‘SCHWANNOMA’[All Fields]) limited to humans and English language. Returned 64 search results, abstracts and references of relevant papers reviewed.

Results: A 75-year-old male with longstanding history of slowly progressive severe hearing loss and tinnitus presented for evaluation of worsening imbalance, vertigo, and nausea. Workup revealed a 7?mm right intracanalicular mass on MRI concerning for vestibular schwannoma. Audiogram showed bilateral, symmetric, severe-to-profound sensorineural hearing loss, with poor open-set speech comprehension while bilaterally aided. He underwent successful concurrent right translabyrinthine resection of his VS with complete preservation of the cochlear nerve and uncomplicated cochlear implantation.

Discussion: Literature review revealed few previous reports of simultaneous VS and CI. The vast majority of these were in patients with neurofibromatosis Type 2 in whom auditory outcomes were poor. This patient represents one of the few cases of concurrent translabyrinthine tumor removal and CI for a spontaneous VS.

Conclusion: Single-stage cochlear implantation and translabyrinthine tumor resection is a feasible and safe option to consider for auditory rehabilitation in rare situations.     

人工耳蜗植入术残余听力保留现状和研究进展

随着手术方法和电极设计等技术的创新,人工耳蜗手术取得了一定的进步,使患者残余听力得到一定的保留。所有手术方法的共同目标即是：保护耳蜗内结构,保留残余听力,以提高听觉言语能力。人工耳蜗术后残余听力的丧失被认为是多种因素的结果,近年来,为获得更好的残余听力保留,人们在电极设计与选择、新型电极涂层、围手术期药物治疗、机器人辅助、图像和电生理引导等方面的研究有了新的突破,对于残余听力的保护有了很大程度的提高。本文将对人工耳蜗术后残余听力保护相关因素的进展作一综述。     

Speech perception with a cochlear implant used in conjunction with a hearing aid in the opposite ear

The aim of this study was to determine the improvement in speech recognition provided by a cochlear implant (CI) in conjunction with a hearing aid (HA) in the opposite ear. The study was a retrospective cohort study in the context of a university teaching hospital CI programme. Seven CI patients who still use their HA in the opposite ear were tested. The scores with the CI alone and the CI in conjunction with an HA were evaluated by using three speech perception tests in quiet (Freiburger Numbers, Freiburger Monosyllables, and Innsbrucker Sentence Test). In the majority of tests and subjects, the CI alone performed better than the HA alone, and the bimodal (CI+HA) condition was superior to the CI alone. On the sentence test, the patients as a group improved from 47-96% (mean: 79%; CI alone) to 50-100% (mean: 88.1%; CI+HA, pv<0.05). With the more difficult monosyllable test, the scores improved from 15-52% (mean: 37.2%; CI alone) to 15-82% (mean: 48.7%; CI+ HA, p<0.05). On the numbers test, scores increased from 65-98% (mean: 83%; CI alone) to 75-98% (mean: 88.7%; CI+ HA, p<0.05). All patients in this study were implanted in the poorer ear. The results of the present study suggest the advantage of CI usage in conjunction with an HA in the opposite ear.     

老年性聋患者人工耳蜗植入效果分析

目的 探究老年性聋患者人工耳蜗植入术后听觉言语康复效果及生活质量的变化。方法 对31例行人工耳蜗植入的老年性聋患者进行听力学、言语功能及生活质量评估并比较差异。听力学评估采用助听听阈,言语能力评估采用词表识别率,评估时间为术前、开机后6个月及开机后12个月。生活质量评估采用Nijmegen 人工耳蜗植入量表(NCIQ),评估时间为术前及开机后12个月。结果 共纳入31例患者,31例患者术前、开机后6个月及开机后12个月助听听阈分别为(62.55±3.69)、(46.58±5.14)、(38.68±4.26)dBHL,差异具有统计学意义(P＜0.05)。术前、开机后6个月及开机后12个月单音节词识别率分别为(9.55±5.81)%、(54.77±8.90)%、(68.52±7.21)%,差异具有统计学意义(P＜0.05);术前、开机后6个月及开机后12个月双音节词识别率分别为(19.87±9.72)%、(64.00±6.53)%、(74.26±6.79)%,差异具有统计学意义(P＜0.05);术前、开机后6个月及开机后12个月短句识别率分别为(28.00±10.58)%、(68.52±7.78)%、(77.61±8.59)%,差异具有统计学意义(P＜0.05)。术前及开机12个月NCIQ总量表得分分别为(35.90±5.80)、(65.16±8.18)分,差异具有统计学意义(P＜0.05)。结论 人工耳蜗植入可以改善老年性聋患者的听觉言语能力及生活质量,对于重度以上听力损失且助听器效果不佳的老年性聋患者可以选择人工耳蜗植入。     

电诱发听性脑干反应在人工耳蜗植入中的应用

目的分析人工耳蜗植入患者耳蜗植入前电诱发听性脑干反应（electrical auditory brainstem response,EABR）的测试结果并初步探讨其意义。方法对14例不同病因、不同年龄的人工耳蜗植入患者耳蜗植入前进行测试,采用EMG外置电刺激器连接面神经探针作为刺激电极,采用Neuro Soft公司生产的Neruo Audio听觉诱发电位仪及调试软件进行EABR测试,测量并分析EABR波形,各波潜伏期和波间期,Ⅴ波波幅、阈值。结果EABR的Ⅱ、Ⅲ、Ⅳ、Ⅴ波与声诱发ABR相似,Ⅲ、Ⅴ波的潜伏期较声诱发ABR缩短,但Ⅲ~Ⅴ波间期与声诱发ABR相同。14例耳聋患者均记录到EABR的Ⅲ波/Ⅴ波,引出率为100％。4例极重度感音神经性聋未合并其他异常患者术中2.5、2.0 mA刺激Ⅲ、Ⅴ波典型,潜伏期和波间期均正常,术中神经反应遥测技术（neural response telemetru,NRT）引出,提示其听神经脑干传导通路无异常,预后效果良好。其他部分患者存在潜伏期延长或波形分化不良或刺激阈值较高等表现,提示可能由于不同因素所导致听觉传导通路异常。结论EABR是通过电刺激耳蜗来了解听觉系统对电刺激的生理反应,最接近人工耳蜗的工作状态,可以对患者听力康复的效果提供更准确的预测;特别是对于一些特殊患者,具有评估人工耳蜗植入效果的优势。而对各种特殊类型耳聋的人工耳蜗植入术前检测、术后评估数据仍需深入探索。     

Combined local anesthesia and monitored anesthesia care for cochlear implantation

IntroductionCochlear implantation has become a routine procedure for patients with hearing loss. In some patients, general anesthesia might be contraindicated due to multiple co-morbidities. We describe a successful protocol for cochlear implantation under local anesthesia with light sedation.Case reportAn 81-year-old patient presented with profound sensorineural hearing loss. Her past medical history revealed ischemic coronaropathy, managed by stenting. After multidisciplinary evaluation and clear adapted information to the patient, surgery was performed under local anesthesia with light sedation and monitored anesthesia care. The procedure lasted 70 min, and was without incident and under good conditions for the surgeon. During the intervention, the patient was comfortable. No nausea or vomiting was noted. The postoperative period was smooth and uneventful.ConclusionWe find local anesthesia with light sedation a good alternative to general anesthesia for patients where general anesthesia is contraindicated. An experienced surgical and anesthesiology team is essential to shorten the duration of the procedure.     

Outcomes with bilateral cochlear implantation following sudden dual sensory loss after ethylene glycol poisoning

Abstract

Acute loss of vision accompanied by profound loss of hearing is fortunately rare, but has a catastrophic effect on both the patient and their family. Re-establishing communication and spatial awareness are high priorities. We describe the case of a 45 year-old man who presented as a result of poisoning by ethylene glycol. Following assessment by clinicians who learned the deaf-blind alphabet in order to communicate, he had his hearing successfully rehabilitated with simultaneous bilateral cochlear implants. The patient recovered the ability to understand speech near perfectly in quiet, to attend to the ear giving the clearer signal in noise, and to localise sources of sound. The patient reported that the latter skill facilitated mobility. This is the first reported case of a patient with acute dual sensory loss due to ethylene glycol poisoning benefiting from bilateral cochlear implants.     

A case of improved hearing with cochlear implantation in Gaucher disease type 1

Gaucher disease is a lysosomal storage disorder that is caused by congenital defective function of the enzyme glucocerebrosidase. Glucocerebroside that is not hydrolyzed by glucocerebrosidase mainly accumulates in the reticular tissue. We describe a Japanese boy with Gaucher disease type 1 who developed bilateral profound sensorineural hearing loss within approximately 4 years. We performed cochlear implantation initially on his right ear and again on his left ear 5 months later. The cochlear implants were successfully utilized with a speech discrimination score of 95% on a Japanese sentence recognition test. There are many reports of central hearing loss in Gaucher disease type 2 or 3. However, to the best of our knowledge, this is the first report of profound inner ear hearing loss with Gaucher disease. It also appears to be the first record of cochlear implantation for Gaucher disease. Cochlear implants may be useful for sensorineural hearing loss in patients with Gaucher disease without neurological symptoms other than hearing loss.     

Predicting speech perception outcomes following cochlear implantation in adults with unilateral deafness or highly asymmetric hearing loss

Objective: Unilateral deafness and highly asymmetric hearing loss can impair listening abilities in everyday situations, create substantial audiological handicap, and reduce overall quality of life. Preliminary evidence suggests that cochlear implantation may be effective in reversing some of these detrimental effects. Patient-level data from existing studies were re-analysed to explore potential factors that may be predictive of improved speech perception scores following implantation.

Methods: Logistic regression modelling examined whether improved speech perception following implantation under various listening conditions was related to the duration of deafness of the severe-to-profoundly deaf ear and/or the level of hearing in the better ear.

Results: Patients with a shorter duration of deafness were more likely to improve in listening conditions that created a less favourable SNR at the implanted ear than the non-implanted ear. Those with more residual hearing in the better ear were more likely to improve in the listening condition that created a less favourable SNR at that ear.

Discussion: The analysis suggests that characteristics of both ears may be relevant when seeking to identify those candidates who are likely to obtain benefit to speech perception following cochlear implantation.     

Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation

IntroductionAuditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals.ObjectiveTo verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy.MethodsA prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation.ResultsTwo participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications.ConclusionThe use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills.     

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。     

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.