Auditory brainstem implantation to rehabilitate profound hearing loss with totally ossified cochleae induced by pneumococcal meningitis

Hearing rehabilitation by cochlear implantation is not always possible in case of total ossification after pneumococcal meningitis. We report 3 cases of postmeningitis profound hearing loss with total cochlear ossification in adults who underwent auditory brainstem implantation (Nucleus 22, Cochlear Inc., Lane Cove, Australia) between 1999 and 2004. The postoperative follow-up period ranged from 1 to 6 years. Eleven to 15 out of 22 electrodes were activated. All patients had significant speech discrimination in the sound-only mode and an enhanced lip-reading performance with the implant. Auditory brainstem implants are an efficient means of auditory rehabilitation and may be considered in selected cases of bilateral profound hearing loss with the impossibility of cochlear implantation.     

耳蜗骨化患者的人工耳蜗植入术

目的：探讨耳蜗骨化患者手术的技巧及术后效果。方法：回顾我科1997—2011年期间共29例耳蜗骨化的患者行人工耳蜗植入术。术前影像学及植入前骨化状况及电生理评估患者耳蜗功能。术后对患者进行言语评估及听觉行为分级标准和言语可懂度分级标准评估术后效果。结果：在29例耳蜗骨化患者中骨化程度为Ⅱ级的患者19例,骨化程度为Ⅰ级的患者4例,骨化程度Ⅲ级的患者4例。其中耳蜗骨化Ⅰ、Ⅱ级的患者中17例电极完全植入耳蜗,其余6例为部分植入电极。耳蜗骨化Ⅲ级的患者全部为部分植入。耳蜗植入术后大部分患者取得了较好的听力及语言的能力。结论：耳蜗骨化患者在完善术前评估的基础上可以完成人工耳蜗植入术并且部分患者可以取得较好的术后效果。术中电刺激听觉诱发电位的检测为评估残存螺旋神经的功能提供了一个很好的方法。     

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.     

Polyarteritis nodosa and cochlear implantation

Polyarteritis nodosa is a systemic disease which affects the small to medium-sized muscular arteries. Sudden or progressive, bilateral hearing loss is a presenting otologic manifestation. To date, no case of cochlear implantation in patients with polyarteritis nodosa has been reported. The authors present a case of polyarteritis nodosa (confirmed by biopsy) in a 71-year-old man with progressive, bilateral sensorineural hearing loss who underwent cochlear implantation. A successful full insertion of the Nucleus 3G electrode array was achieved without surgical or post-operative complications. The patient immediately showed a positive subjective response and, at three month post-operative evaluation, had gained useful open-set speech perception. A review of five temporal bone cases with hearing loss and polyarteritis nodosa revealed the possibility of fibrosis and ossification in the basal turn of the cochlea, of which the surgeon should be aware prior to cochlear implantation.     

耳蜗骨化患者人工耳蜗植入术

目的介绍耳蜗骨化患者人工耳蜗植入术中情况与术后效果，探讨耳蜗骨化时人工耳蜗植入术的可行性。方法回顾性分析31例耳蜗骨化患者人工耳蜗植入术中和术后的临床资料。结果1995年5月至2005年7月因重度和极重度感音神经性聋接受人工耳蜗植入术的患者720例，术中诊断耳蜗骨化31例（4．3％），其中男14例，女17例；手术时患者年龄1．4～59．0岁，平均13．2岁。轻度骨化27例，严重骨化4例。全部患者均经乳突一面隐窝进路完成人工耳蜗植入术。术中和术后均未出现并发症。患者术后声场听阈均达30～40dB，与无耳蜗骨化的患者相比差异无统计学意义（P〉0．05）。结论尽管耳蜗骨化时人工耳蜗植入手术难度很大，但耳蜗骨化时经常规乳突一面隐窝进路仍能完成电极植入，且轻度耳蜗骨化时能将电极全部植入蜗内，并且对电极损伤极小，术后听力效果好，因此人工耳蜗植入术对于耳蜗轻度骨化具有可行性。     

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。     

Cochlear histopathology in the labyrinthectomized ear: implications for cochlear implantation

Limited damage to the cochlea and preservation of hearing after labyrinthectomy have been the subject of many case reports. One might hypothesize that, even when hearing is lost, there may be less damage to the cochlea than anticipated, and some neural elements that can be electrically stimulated may be preserved. Four labyrinthectomized temporal bones on file at the House Ear Institute were evaluated histopathologically. All had some remaining spiral ganglion cell population, the neural element that we think is stimulated by the intracochlear electrode. We also examined the population of hair cells and dendrites and the presence and extent of cochlear ossification, factors that may influence the performance of a cochlear implant. This is the first study of its type. Results indicate that cochlear implantation in the labyrinthectomized ear may be feasible.     

人工耳蜗再植入病例分析

目的 报告人工耳蜗再植入的常见原因、手术方法及再植入术后患者听力语言康复情况.方法 2007年5月～2010年11月北京友谊医院采用面隐窝进路为23例患者行人工耳蜗再植入,分析其再植入原因及手术方法.结果 23例患者均顺利完成人工耳蜗再植入,再植入术后患者听力言语康复效果满意或正在康复机构接受康复.再次植入的原因分别为:电极植入下鼓室1例;植入体部分破碎1例;电极部分脱出1例;电极从外耳道后壁穿出1例,磁铁移位1例;耳蜗底转骨化致植入失败1例;皮瓣或切口感染4例;耳蜗植入体故障7例;原因不明者3例;电极阻抗无限大2例;左侧耳蜗植入后无反应再植入右侧1例.结论 人工耳蜗再植入的原因多样且较复杂,再植入手术需要注意蜗内纤维化和骨化导致电极植入困难的问题.术前准确评估及患者术后避免头部剧烈碰撞是避免人工耳蜗再植入的关键.     

Auditory brainstem implant in a child with severely ossified cochlea

OBJECTIVE: The hearing outcome after implanting a severely ossified cochlea has always been less satisfactory than implanting a patent one. The aim of our study is to present a case where brainstem implantation was successfully performed as an alternative to cochlear implantation in a child with bilateral severe ossification of the cochlea. STUDY DESIGN: Case presentation. This study was conducted at Gruppo Otologico, Rome, Italy, a private referral center for neurotology and skull base surgery. METHODS: The subject of our study was a 12-year-old female child with postmeningitic deafness and bilaterally ossified cochleae. This case is the first brainstem implantation performed at our center with the indication of severe ossification of the cochlea. RESULTS: Successful brainstem implantation of a device was carried out, and the hearing of the patient was restored to the degree that she can freely use the telephone after 8 months of implantation. CONCLUSION: Although more cases are needed before establishing the exact outcome of brainstem implantation in cases of deafness in the presence of severe bilateral cochlear ossification, preliminary results show the superiority of brainstem implants to conventional or even customized cochlear implants.     

Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up

OBJECTIVE: To evaluate the long-term hearing outcomes of neurofibromatosis type 2 (NF2) patients with cochlear implants. METHODS: Retrospective analysis of cochlear implant performance in NF2 patients using open- and closed-set speech perception testing. RESULTS: Patients with NF2-associated bilateral vestibular schwannomas frequently become profoundly deaf. The aim of surgical resection should be to preserve serviceable hearing in at least one ear; however, this goal can be difficult to achieve. Frequently, tumor size or poor preoperative hearing status can require a surgical approach that leaves the patient with a profound, bilateral sensorineural hearing loss. If the cochlear nerve is preserved anatomically after vestibular schwannoma surgery, and if promontory stimulation confirms the functionality of the cochlear nerve, then cochlear implantation is an excellent option to restore hearing. We present six cochlear implant patients with NF2 who attained a significant improvement in open- and closed-set speech understanding with a mean follow-up of 7.9 (range: 5-13) years after surgery. In all but one case, the hearing results did not deteriorate over the follow-up period. CONCLUSION: Early surgical intervention for vestibular schwannomas in NF2 patients when the cochlear nerve can be spared is an important consideration to allow for possible cochlear implantation. A 6- to 8-week recovery period for the anatomically intact cochlear nerve may be necessary to obtain a positive promontory stimulation response following tumor resection and should be performed prior to cochlear implantation.     

Bilateral cochlear implantation in a patient with bilateral temporal bone fractures

With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation.     

Acoustic Analysis of Voice in Cochlear Implant Recipients with Post-Meningitic Hearing Loss

The objective of this study was to evaluate the critical time period between the onset of sensorineural hearing loss and cochlear implantation with respect to normal voice production in children with post-meningitic hearing loss. Acoustic measures of voice production were obtained from ten paediatric cochlear implant recipients with post-meningitic hearing loss. Acoustic measures were obtained utilising the Multi-Dimensional Voice Program and Computerized Speech Laboratory (Kay Elemetrics Corp.). Measures were based on sustained phonation of the vowel /a/. Acoustic parameters included fundamental frequency, short- and long-term frequency perturbation, and short- and long-term amplitude perturbation. Measures of fundamental frequency and short-term frequency and amplitude perturbation were comparable to values of children with normal hearing. Long-term control of frequency was within normal limits for subjects with a period of auditory deprivation of less than four months. Measures of long-term amplitude perturbation were normal for all patients except those with cochlear ossification. Early restoration of auditory feedback with cochlear implantation, the absence of cochlear ossification, residual aided hearing following meningitis, and auditory-verbal therapy were identified as factors in preserving the long-term control of frequency and amplitude in the setting of post-meningitic hearing loss.     

Cochlear implants in systemic autoimmune vasculitis syndromes

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Be?het's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.     

Cochlear implantation as a successful rehabilitation for radiation-induced deafness

Radiotherapy of the head and neck can be associated with conductive and/or sensori-neural hearing loss. We report the case of a 67-year-old man who developed complete bilateral deafness caused by labyrinthitis and radiation-induced neuritis of the acoustic nerve after postoperative radiotherapy of a nasopharyngeal carcinoma. Two years postoperatively extensive clinical workup including computed tomography and magnetic resonance imaging showed no recurrence or secondary brain tumors. To facilitate sound perception a Combi 40 cochlear implant was implanted. Because of fibrosis the insertion depth of the stimulating electrode into the scala tympani was limited and therefore a “short electrode version” was used. Six months after implantation the patient had achieved an excellent enviromental sound recognition and moderate speech intelligibility. Present experiences has shown that although radiotherapy can cause damage to the labyrinth and acoustic nerves and central hearing pathways, there may still be surviving auditory nerve fibers that can be stimulated successfully by a cochlear implant. Received: 30 July 1997 / Accepted: 28 October 1997     

Temporal bone imaging for cochlear implantation

Computerized tomography (CT) imaging of the inner ear structures is helpful when evaluating a patient for cochlear implantation. The accuracy of CT scan imaging in determining the presence of cochlear ossification was assessed. A retrospective study of 104 implant patient charts, operative reports, and CT scans was performed. The CT scan was in agreement with the operative findings in 78% of patients included in the study. Twenty-two percent were found to have ossification at surgery which was not detected radiographically. One patient with extensive otospongiosis and an obstructed cochlea on CT scan had a patent cochlea demonstrated by magnetic resonance imaging (MRI) which was corroborated at surgery. In 32 children with postmeningitic hearing loss, the CT scan had only a 53% accuracy in assessing cochlear ossification. Since 69% of these patients were found to have some degree of cochlear ossification, otologists should expect to encounter some degree of bony obstruction within the basal turn of the cochlea even when the CT scan is normal. Nevertheless, cochlear implantation can successfully be performed in these cases.     

人工耳蜗植入术中迷路骨化与内耳畸形的处理

目的：对迷路骨化与内耳畸形患者进行听力康复。方法：人工耳蜗植入术。结果：术中发现内耳迷路骨化与先天性内耳畸形各１例。其中，迷路骨化继发于分娩后未定病因，术中见单侧前庭和耳蜗广泛骨化，后将耳蜗电有植入对侧未骨化的耳蜗；先天天性内耳畸形呈鼓岬骨质增厚，圆窗和卵圆窗骨性封闭并增厚，基底转起始部鼓阶扩张，予成功植入耳蜗电极。结论：人工耳蜗植入术中迷路骨化耳畸形时，应根据具体情况采取适当的措施，以保证手术取     

Cochlear Implants in Systemic Autoimmune Vasculitis Syndromes

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Beçhet's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.     

人工耳蜗植入术295例语言康复临床分析

目的 研究耳蜗植入术后听觉言语康复效果及其影响因素。 方法 采用听觉行为分级(CAP)、言语可懂度分级(SIR)及视频和录音言语清晰度分析法分别对295例人工耳蜗植入术后患者进行听觉言语康复效果评估。 结果 2例蜗神经细小合并内耳畸形患者开机听阈为45 dB,其余听阈为25~40 dB。单因素分析结果显示:术前助听器的佩戴、植入年龄、植入后时间、术前残余听力、内耳畸形程度、脑白质病变对CAP及SIR差异有统计学意义;手术方法、中耳炎、Waardenburg综合征对CAP、SIR差异无统计学意义。多因素Logistic回归分析结果显示:植入后时间、脑白质病变与CAP、SIR显著相关。 结论 大部分患者可从人工耳蜗植入术中获得满意效果,其听觉言语康复程度受多种因素的影响。     

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.