人工耳蜗手术前后使用小儿主观测听法的意义

目的探讨主观听力学测试方法在低龄儿童人工耳蜗植入手术前后的作用。方法和结果103名5岁以下拟接受人工耳蜗植入低龄儿童,术前均完成非助听和助听主观听力测试。31名游戏测听(playaudiometry,PA)法、62名视觉强化测听(visualreinforcementaudiometry,VRA)法、10名行为观察测听(behavioralobservationaudiometry,BOA) VRA法获得左右侧单耳/或双耳裸耳听阈;30名PA法、63名VRA法、10名BOA VRA法分别在声场中获得每侧耳的助听听阈,并结合其他客观测试结果评估了听通路的功能。术后开机调试后103名患儿均有听觉反应并获得T、C值,其中48名由VRA、PA测试方法得到;27名经BOA法得到;28名经神经反应遥测(neuralresponsetelemetry,NRT)结合BOA法获得,患儿的听觉能力有明显改善。结论主观听力测试方法在人工耳蜗植入术前对评估听通路完整性非常重要,对预测低龄患儿术后听觉康复可能的疗效有重要意义。     

Preliminary results and technique for electrophysiological intra-operative monitoring of residual hearing during cochlear implantation

Abstract

Aims

To assess the feasibility of intra-operative monitoring of residual hearing using electrocochleography (ECOG) during cochlear implantation. We present our methods and preliminary results.

Methods

We attempted to monitor 21 consecutive paediatric and adult subjects during cochlear implantation. All subjects were implanted via a promontory cochleostomy with full electrode insertions of Cochlear^TM Nucleus® (Cochlear Nucleus, Cochlear Ltd, Australia) devices: 8 with straight electrodes and 13 with contoured electrodes. Baseline recordings were made after the completion of the posterior tympanotomy, and subsequently at stages of the cochleostomy and electrode insertion.

Results

We were unable to obtain recordings on five patients because the ear-insert tubing kinked, which disrupted transmission of the stimulus. Five had insufficient cochlear function to be able to record a baseline ECOG. No subjects in whom we were able to obtain a satisfactory baseline ECOG (n = 11) had a reduction in ECOG action potential (AP) amplitude, morphology or threshold up to completion of the cochleostomy. Seven maintained an unchanged ECOG throughout the procedure to full insertion. An implant had to be replaced because of a faulty straight electrode in one patient. The amplitude significantly reduced during insertion of the replacement array. Two had a reduction in amplitude, threshold, and shape of the wave related to inadvertent suction of the perilymph. Subsequently, one maintained this changed ECOG to the end of the procedure, but the other progressed to complete loss of the ECOG during insertion of the array. One other subject had a significant reduction in the amplitude during insertion of the electrode from a depth of approximately 18 mm to full insertion.

Conclusions

Intra-operative monitoring of residual hearing may be possible in most patients undergoing cochlear implantation. This pilot study suggests that cochleostomy is not associated with intra-operative loss of residual hearing; ECOG can be preserved during the procedure in most patients; intra-operative loss of hearing is most likely to occur when the tip of the array reaches the basal turn of the cochlea. This risk may increase if the array has to be removed and re-inserted. Suction of perilymph causes immediate changes, which may not recover.     

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。     

Molecular mechanisms and roles of inflammatory responses on low-frequency residual hearing after cochlear implantation

Preservation of low-frequency residual hearing is very important for combined electro-acoustic stimulation after cochlear implantation.However,in clinical practice,loss of low-frequency residual hearing often occurs after cochlear implantation and its mechanisms remain unclear.Factors affecting lowfrequency residual hearing after cochlear implantation are one of the hot spots in current research.Inflammation induced by injury associated with cochlear implantation is deemed to be significant,as it may give rise to low-frequency residual hearing loss by interfering with the blood labyrinth barrier and neural synapses.Pathological changes along the pathway for low-frequency auditory signals transmission may include latent factors such as damage to neuroepithelial structures,synapses,stria vascularis and other ultrastructures.In this review,current research on mechanisms of low-frequency residual hearing loss after cochlear implantation and possible roles of inflammatory responses are summarized.     

多频稳态诱发电位和听性脑干反应对感音神经性聋儿童客观听阈的评估

目的比较多频稳态诱发电位(MASSR)与短纯音听性脑干反应(Tb-ABR)对感音神经性聋儿童客观听阈的评估。方法对37名感音神经性聋儿童分别测试MASSR反应阈、Tb-ABR反应阈和行为听阈,参照行为听阈,比较MASSR反应阈和Tb ABR反应阈对行为听阈评估的准确性。结果MASSR反应阈、Tb-ABR反应阈和行为听阈之间均有较高的相关性。二者在频率为2、4kHz时,对行为听阈的评估具有相似的准确性;但在频率为0.5、1kHz时,MASSR的准确性较Tb ABR的准确性高。结论MASSR和Tb-ABR均可用作感音神经性聋儿童言语频率客观听阈的评估,但MASSR在低频(0.5、1kHz)时较Tb-ABR的准确性高。     

Improved hearing with cochlear implantation in Fabry's disease

Abstract

Objective

To describe our center's experience with cochlear implantation in patients suffering from Fabry's disease, an inherited mutation resulting in an alpha-galactosidase A enzyme deficiency.

Clinical Presentation

Case report of two patients aged 49 and 59 at implant, with genetically confirmed Fabry's disease and progressive hearing loss.

Intervention

Surgical implantation of Clarion (Advanced Bionics) and Nucleus Freedom (Cochlear) cochlear implants.

Conclusion

Cochlear implantation improves hearing discrimination by 60 points on the HINT scale. This suggests that cochlear implantation is a safe and effective intervention that improves hearing discrimination in patients suffering from Fabry's disease.     

人工耳蜗植入后对侧耳是否联合使用助听器声调识别的对比研究

目的 探讨人工耳蜗植入后对侧耳联合使用助听器对声调识别的影响;同时探讨人工耳蜗开机时间、康复训练时间、听障儿童的学习能力等因素对声调识别的影响。 方法 选择3~7岁的人工耳蜗植入儿童27例,采用《言语听觉反应评估》(EARS)中的“封闭式声调测试”作为测试材料,分别单独使用人工耳蜗(CI)和在人工耳蜗对侧耳联合使用助听器(CIHA)在安静和噪声两种环境下进行声调识别的测试,使用SPSS 17.0对测试结果进行统计学分析。 结果 无论在安静环境还是噪声环境下,CIHA的声调测试成绩均优于CI,此优势在噪声环境下更明显;人工耳蜗开机时间、康复训练时间较长者声调测试成绩均优于较短者;听障儿童的希-内学习能力测试结果学习能力DIQ≥100与DIQ<100者声调测试成绩差异无统计学意义。 结论 建议听障儿童人工耳蜗植入后对侧耳联合使用助听器,但助听器需要优化和调整。     

有低频残余听力感音神经聋的人工耳蜗植入术

目的介绍一种有低频残余听力感音神经聋的人工耳蜗植入技术,探讨人工耳蜗植入手术对有残余听力患者的治疗效果和价值。方法15例有残余听力的患者接受了保护残余听力的人工耳蜗植入手术。术中电极植入深度在19mm～24mm左右。术后分别检测单纯使用助听器、单纯使用人工耳蜗、人工耳蜗结合助听器三种不同状态下的听力。结果15例患者中,有13例术后残余听力保存良好,仅分别丢失5～20dB听力,但另2例术后残余听力全部丧失。术后在安静、信噪比15dB和10dB三种不同状态下的言语测试结果显示,人工耳蜗结合助听器使用者测试得分始终保持在很高水平;单纯使用人工耳蜗者也有较好的成绩,但在信噪比达10dB的条件下,测试成绩下降;而单纯使用助听器者,不仅在安静状态下听力成绩不甚理想,一旦加入竞争性噪声,听力测试成绩急剧下降。结论保护和利用残余听力的人工耳蜗植入技术,使人工耳蜗植入手术对象从重度或极重度聋扩大到高频为重度或极重度聋,低频（≤500Hz）为中、轻度聋的患者。接受这项技术患者的听力和言语识别能力均明显优于其单纯配戴助听器和单纯使用人工耳蜗时的听力和言语识别能力。     

Monitoring of cochlear function during cochlear implantation

OBJECTIVE: To report the feasibility of monitoring cochlear function during cochlear implantation. STUDY DESIGN: Case report. SETTING: Tertiary care referral center. METHODS: A child with audiologic features typical of bilateral auditory neuropathy underwent cochlear implantation. The scala tympani was entered inferior and slightly anterior to the round window membrane margin and smooth electrode insertion was achieved. Using single polarity click stimuli, the cochlear microphonic was measured at several steps during surgery. RESULTS: Cochlear microphonics were present at all stages during the implantation process and were clearly distinguished from neural responses by stimulus polarity inversion and constant latencies, despite changes in stimulus level. With the electrode in situ, amplitudes were smaller but persisted until the final measurement at 10 minutes after insertion. At follow-up 2 weeks after surgery, behavioral audiometry results indicated profound hearing loss in the operated ear. CONCLUSIONS: This paper demonstrates the feasibility of monitoring cochlear function during cochlear implantation. The routine surgical approach did not appear to adversely affect the functional measurements. Standard size, full electrode insertion did diminish the amplitude of the cochlear microphonics, possibly as a result of intracochlear mechanical impairment. Ultimately, profound hearing loss was documented, indicating that factors other than immediate changes induced by electrode insertion were likely responsible for the loss of cochlear function.     

Effect of preoperative residual hearing on speech perception after cochlear implantation

Objective: To assess the effect of substantial preoperative residual hearing on speech perception outcomes in adult cochlear implant recipients. Setting: Tertiary care academic referral center. Methods: Twenty‐nine patients with substantial preoperative residual hearing underwent cochlear implantation. Twenty‐one implant recipients matched for age and duration of hearing loss, but without preoperative residual hearing, served as controls. Postoperative speech perception was assessed using City University of New York sentence, consonant‐nucleus‐consonant, and hearing in noise test in quiet and in noise (+10 dB signal to noise ratio) tests at 1, 3, 6, and 12 months after fitting. Results: After implantation, there were no significant differences between groups for any of the tests administered. The mean change in speech perception abilities from baseline was significantly greater for the control patients than those with substantial preoperative residual hearing at a number of the test intervals across the various conditions. Moreover, at both 1 and 3 months, some patients in the residual hearing group had speech perception scores that were worse than their preoperative values. Ultimately, all of the patients with substantial residual hearing surpassed their preoperative performance. Discussion: Patients with substantial preoperative residual hearing can gain significant benefit from cochlear implantation. Although the degree of improvement in these individuals is somewhat more modest than for those patients without preoperative residual hearing, the outcomes are still excellent. That there were no significant differences between the patient groups suggests that having substantial residual hearing before implantation does not provide a measurable performance advantage for electrical stimulation. Patients with substantial residual hearing who are contemplating cochlear implantation should be counseled regarding a possible initial decline in speech perception performance.     

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.     

Preservation of residual hearing with cochlear implantation: How and why

Conclusions Hearing may be conserved in adults after implantation with the Nucleus Contour Advance perimodiolar electrode array. The degree of hearing preservation and the maximum insertion depth of the electrode array can vary considerably despite a defined surgical protocol. Residual hearing combined with electrical stimulation in the same ear can provide additional benefits even for conventional candidates for cochlear implantation.

Objectives We present preliminary results from a prospective multicentre study investigating the conservation of residual hearing after implantation with a standard-length Nucleus Contour Advance perimodiolar electrode array and the benefits of combined electrical and acoustic stimulation.

Material and methods The subjects were 12 adult candidates for cochlear implantation recruited according to national selection criteria. A “soft” surgery protocol was defined, as follows: 1–1.2-mm cochleostomy hole anterior and inferior to the round window; Nucleus Contour Advance electrode array inserted using the “Advance-off-stylet” technique; and insertion depth controlled by means of three square marker ribs left outside the cochleostomy hole. These procedures had been shown to reduce insertion forces in temporal bone preparations. Variations in surgical techniques were monitored using a questionnaire. Pure-tone thresholds were measured pre- and postoperatively. Patients who still retained thresholds <90 dB HL for frequencies up to 500 Hz were re-fitted with an in-the-ear (ITE) hearing aid. Word recognition was tested in quiet and sentence perception in noise for the cochlear implant alone and in combination with an ipsilateral hearing aid.

Results Hearing threshold level data were available for 12 patients recruited from 6 of the centres. Median increases in hearing threshold levels were 23, 27 and 33 dB for the frequencies 125, 250 and 500 Hz, respectively. These median increases include the data for two patients who had total loss of residual hearing due to difficulties encountered during surgery. “Cochlear view” X-ray images indicated that the depth of insertion varied between 300 and 430°, despite modest variations in the length of the electrode inserted (17–19 mm). The insertion angle had some influence on the preservation of residual hearing at frequencies of 250–500 Hz. Six of the 12 patients retained sufficient hearing for effective use of an ipsilateral ITE hearing aid (≤80 dB HL at 125 and 250 Hz; ≤90 dB HL at 500 Hz). Word recognition scores in quiet were improved from 10% to 30% with the cochlear implant plus ipsilateral hearing aid in 3 patients who had at least 3 months postoperative experience. Signal:noise ratio thresholds for sentence recognition were improved by up to 3 dB. Patients reported that they experienced greatly improved sound quality and preferred to use the two devices together.     

单侧人工耳蜗植入术前后双耳残余听力变化的初步研究

目的 通过观察单侧人工耳蜗植入术后儿童双耳残余听力情况,分析其变化趋势,探讨各种可能的影响因素。 方法 将29例单侧人工耳蜗植入的重度极重度感音神经性耳聋儿童纳入研究,根据术前颞骨CT分为A组(有大前庭导水管综合征)和B组(非大前庭导水管综合征),分别于术前、术后1周、开机时、术后半年定期进行裸耳纯音测听,比较植入耳残余听力保存情况及非植入耳残余听力的变化。 结果 患儿术前双耳均不同程度存在残余听力,术后均定期行纯音测听或行为测听,各频率仍部分存有残余听力,主要分布在低频区,且随频率升高其残余听力保留率逐渐下降,各频率残余听力保留率之间差异有统计学意义(Wald χ2=16.980, P=0.001);植入耳与非植入耳各频率残余听力保留率之间差异有统计学意义(Wald χ2=10.031, P=0.002);术前后各时间节点残余听力保留率之间差异无统计学意义(Wald χ2=3.384, P=0.336); AB两组之间残余听力保留率之间差异无统计学意义(Wald χ2=0.906, P=0.341)。 结论 人工耳蜗植入对植入耳残余听力的保留主要分布在低频区;随着植入时间的延长,残余听力可以基本保持;少部分患儿行单侧植入后对非植入耳残余听力有影响;前庭导水管扩大症患儿行单侧耳蜗植入术后,非植入耳残余听力在短期内可有波动。     

人工耳蜗植入后由植入体引发的局部反应

目的 探讨人工耳蜗植入后由植入体引发局部反应的发生率、症状、体征,分析其可能的病因、相关因素以及对患者的影响和主要的处理原则,为临床工作提供参考和借鉴.方法 回顾分析1995年9月至2007年7月植入多道人工耳蜗患者997例中由植入体引发局部反应的10例患儿的症状、体征、治疗及预后情况.结果 997例中有10例术后出现局部反应,发病率1.003%.10例中男6例,女4例.植入时年龄13个月至8岁,平均34个月.植入装置分别为Cochlear和Med-EL公司产品,各5例.首次发病时间为术后1个月至8年,平均为2年4个月.发病时年龄小于4岁的8例,大于5岁的2例.主要症状及体征为:植入侧局部肿胀、耳后不适、瘙痒感,触及有波动感,有的患儿局部有红点,声音清晰度下降等.发病次数为1～8次,平均2.4次.局部抽出液细菌培养阴性,2例经IgE检测示患者为中度过敏体质.有姐弟俩术后均有发病.除1例多次发病最后取出装置外,其余均经抗生素、抗组胺及激素等保守治疗有效.结论 人工耳蜗植入患者术后植入侧局部出现反应的发生率并不高,而反复出现相同症状的更少.主要病因除局部感染所致外,可能同植入体引发的局部变态反应有关.确诊该病目前虽有一定的难度,但术前过敏源筛查仍有必要.对于有过敏体质者,即使筛查阴性,术后仍需密切随访.对出现症状的患者除首先进行积极的保守治疗外,仍有必要进一步进行变态反应试验以确诊.必要时可给予抗免疫治疗.如继发严重细菌感染不能控制者,则应尽快取出体内装置,待病情稳定后选择无致敏原装置再次植入.发病年龄以4岁以下儿童多见.     

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。     

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.     

Evoked response audiometry in patients with peripheral hearing loss

The acoustically-evoked vertex response has been studied in normally-hearing subjects and those with peripheral hearing losses, including both sensorineural and conductive losses. Comparison of evoked response (ERA) thresholds with those determined by conventional manual (subjective) audiometry give results similar to those obtained by other workers. Of particular note was an ability to demonstrate an increase in amplitude and a reduction in latency of the evoked response when the ear was occluded in subjects with normal sound transmitting mechanisms, but not in cases of conductive hearing losses. Thus we are now able to perform the Bing test objectively.     

感音神经性聋儿多频稳态诱发电位及短纯音听性脑干反应与行为测听的相关性

目的 比较多频稳态诱发电位（MASSR）、短纯音听性脑干反应（Tb—ABR）与感音神经性聋儿行为测试听阈的差值．研究MASSR和Tb—ABR反应阈与行为听阈之间是否存在相关性以及在不同听力损失聋儿、不同的频率之间的差异。方法 对60名感音神经性聋儿分别测试MASSR和Tb—ABR反应阈和行为听阈，评价MASSR反应阈、Tb—ABR反应阈与行为听阈的相关性。结果 MASSR反应阈、Tb—ABR反应阈和行为听阈之间均有较高的相关性。二者在频率为2、4kHz时，对行为听阈的预测具有相似的、较高的准确性；但在频率为0．5、1kHz时，MASSR的准确性较Tb—ABR高。结论 MASSR和Tb—ABR均可用作感音神经性聋儿言语频率客观听阈的预测，为低龄儿童及难以检测行为听力的患儿提供诊断依据。