耳蜗性耳硬化症人工耳蜗植入的应用研究

分析耳蜗性耳硬化症患者的临床特点、人工耳蜗植入术中情况及人工耳蜗植入术后听觉言语康复效果,为该疾病的诊治提供参考。方法分析4例耳蜗性耳硬化症患者病史资料、听力学检查结果及影像学结果,观察人工耳蜗植入手术的术中所见,并定期对4例患者进行听力学及影像学随访。结果①所有患者人工耳蜗电极均经圆窗膜径路完全植入鼓阶。其中1例于术中发现镫骨完全固定,导致术中鼓阶开孔时外淋巴波动不明显;1例发现圆窗膜骨化,术中鼓阶开孔定位困难;其余2例患者镫骨活动好,圆窗结构清晰。所有患者术中电极阻抗检测均正常,且引出标准的神经反应遥测波形;②术后随访1~5年无术后并发症出现,声场测听示平均听阈为40.8 dBHL,平均言语识别率为77.3%,言语及交流能力较术前提高;颞骨高分辨率CT提示双侧内耳病变范围无明显进展。结论耳蜗性耳硬化症进展缓慢,严重时可导致重度/极重度感音神经性聋,当使用助听器无效时,人工耳蜗植入能帮助患者获得较满意的听觉康复效果。     

Cochlear implantation in cochlear otosclerosis

OBJECTIVES/HYPOTHESIS: The objective was to correlate implant performance in cochlear otosclerosis to 1) matched control samples, 2) severity of otic capsule involvement, 3) prior ipsilateral surgery, and 4) programming issues. STUDY DESIGN: Retrospective case controlled study. METHODS: Study cohort comprised 30 individuals. Diagnosis was based on prior ear surgery (stapedectomy [n = 18] or fenestration [n = 2]) and/or pathognomonic radiological findings. High-resolution computed tomography images of the temporal bones were assessed by two radiologists and graded (range, 0-3) for the extent of otosclerosis. Operative records were reviewed. Performance, programming visits, and the number of electrode deactivations at 6 months and at 1 year after implantation were determined for the individuals with otosclerosis and compared with a group of matched control subjects. A within-group comparison correlating severity of otosclerosis to the above was carried out. RESULTS: Implant performance in individuals with cochlear otosclerosis was not significantly different from those without. Previous surgery on the side of implantation did not alter performance. Programming difficulty as reflected in the number of visits and electrode deactivation for sound quality reasons were comparable. Deactivation for facial nerve stimulation occurred exclusively in otosclerotics with the most severe radiological disease (grade 3) and was only with non-modiolar hugging electrodes (n = 5). There was no observed difference between the radiological extent of otosclerosis and implant performance. CONCLUSION: Individuals with severe otosclerosis considering cochlear implantation can be counseled to expect similar benefit to those without, regardless of whether prior surgery occurred on the side of implantation or of severity of otic capsule involvement. There is a significant risk of facial nerve stimulation in otosclerotics with grade 3 disease.     

Evolution of otosclerosis to cochlear implantation

IntroductionOtosclerosis is an osteodystrophy of the labyrinthine capsule producing conductive hearing loss. If the process invades the cochlea, a sensorineural hearing loss usually takes place. The cochlear implant is a good alternative in these patients.ObjectiveTo ascertain the behaviour of cochlear implantation in otosclerosis.Material and methodsWe reviewed a database of 250 patients that underwent cochlear implantation, performing a retrospective study of 13 patients with clinical, audiological and/or imaging findings of bilateral otosclerosis. The 26 ears were studied as to their natural history, previous surgeries, evolution to profound hearing loss, computed tomography images, complications and functional results.ResultsOf the cases studied, 46% were female and 54% were men, with a mean age of 26 years at the onset of conductive hearing loss. Stapes surgery was performed in 19 ears (73%), with a mean patient age of 29 years, and 53% of them underwent cochlear implantation. Computed tomography results showed that there were signs of different degrees of radiological affectation in 54% of the ears. A total of 3 complications took place (23%): implant failure, overstimulation of the facial nerve and bilateral tinnitus were found. One year after implantation, the average percentages of correct 2-syllable words were 80% and 85% in open sentences.ConclusionsPatients having profound bilateral sensorineural hearing loss secondary to otosclerosis obtain great benefit from cochlear implantation.     

Revision cochlear implantation for facial nerve stimulation in otosclerosis

OBJECTIVE: To find if patients experiencing postsurgical facial nerve stimulation caused by underlying disease process (ie, otosclerosis) can improve their hearing performance with their cochlear implant by reimplantation and by an optimal programming strategy. DESIGN: Retrospective analysis. SETTING: Academic tertiary referral center. PATIENTS: Two cochlear otosclerosis patients with resistant facial nerve stimulation (FNS). Both patients were initially implanted with Nucleus 22 devices (Cochlear Corporation, Englewood, Colo) and they developed FNS after a period of use. Owing to the decreasing number of active electrodes, concurrent decreases in speech understanding occurred. INTERVENTIONS: Various programming approaches were used to address the FNS. Both subjects ultimately received Nucleus 24 devices. One was reimplanted in the same ear, and the other was implanted in the opposite ear. Both have been followed up for 8 months following the reimplantation. MAIN OUTCOME MEASURES: Cochlear implant programming levels, cochlear implant performance, and facial nerve stimulation. RESULTS: The FNS was managed for more than 3 years through optimized programming. However, the FNS progressed until performance dropped below acceptable levels. Reimplantation was believed to be the only option for improvement. After reimplantation and programming, both subjects showed immediate improvement in speech discrimination. One user increased his consonant-nucleus-consonant word score from 12% preoperatively to 42%, and the other's performance increased from 0% to 86%. CONCLUSIONS: Our results suggest that having more programming options with newer devices is critical in otosclerotic or ossified users who experience FNS. Also, reimplantation may be a useful tool to improve performance.     

Results with the Contour cochlear implant in patients with cochlear otosclerosis

BACKGROUND: Results after cochlear implant surgery may be complicated by postoperative facial nerve stimulation. Aim of the study presented was to evaluate postoperative results in implanting the straight Nucleus electrode array and the preformed Contour array in patients with deafness due to cochlear otosclerosis. METHODS: A retrospective analysis of intra- and postoperative reports of all patients with cochlear otosclerosis was carried out. Results with the Nucleus straight electrode array and the Contour array were compared with regard to postoperative facial nerve stimulation. RESULTS AND CONCLUSION: None of the Contour patients (n = 7) presented with postoperative facial nerve stimulation. This is in contrast to the majority (4 of 6) of patients being implanted with the straight electrode array. Our results indicate that the use of the Contour array is advantageous in patients being at risk for facial nerve stimulation. In addition intraoperative reports suggest a more reliable insertion of the Contour electrode array in cochlear otosclerosis with partial obliteration.     

Very far-advanced otosclerosis: stapedotomy or cochlear implantation

CONCLUSION: Every patient with severe or profound hearing loss must have a temporal bone high-resolution computed tomography (CT) scan. Stapedotomy is a simple, safe and low-cost procedure compared with cochlear implantation and can provide very good results. This can justify our decision to propose stapedotomy at the initial treatment in patients with very far-advanced otosclerosis. In cases of hearing failure after stapes surgery, cochlear implantation is an option. OBJECTIVE: This study aimed to find the best first intention treatment of very far-advanced otosclerosis. MATERIALS AND METHODS: This was a retrospective study and included 14 patients with non-measurable preoperative bone and air conduction thresholds and otosclerosis on temporal bone high-resolution CT scan. Stapes surgery followed by a well fitted hearing aid was the initial treatment in 11 patients and cochlear implantation in 7 patients, including 4 patients who had poor results after stapedotomy. Objective and subjective audiometric results were studied and compared between stapedotomy and cochlear implantation groups. RESULTS: Objective and subjective results were statistically better in the cochlear implant group than in the stapedotomy group. However, four patients in the stapedotomy group had comparable results to the patients with cochlear implants.     

Cochlear implantation of patients with far-advanced otosclerosis.

OBJECTIVE: This current single-subject, repeated-measures study was to describe our experience with 30 patients who had been diagnosed with "far-advanced otosclerosis" and who were included in our program of cochlear implants. We analyzed the history of the patients and their families before implantation, the surgical findings, and the performance over a follow up of 3 years. MATERIAL AND METHODS: All patients met one or more of the after criteria: 1) previous surgical intervention as a treatment of their otosclerosis; 2) signs of pericochlear hypodensities in high resolution computed tomography (HRCT) scans; and 3) family precedents of otosclerosis. All underwent standard surgical cochlear implantation. RESULTS: In 78% of the cases, a stapedectomy had previously been realized. Cochlear otosclerosis could be appreciated in HRCT in 78% of the patients. A family history of otosclerosis was found in 40%, and 33.3% of patients had familial precedents of nonfilial hypoacusis. The mean results in the two-syllable test were 20% preimplantation, 54% 6 months after implantation, and 52%, 62%, 54% at 1, 2, and 3 years after implantation. In the CID sentence test, they were in the order of 32% preimplantation and of 64% at 6 months, 66% after 1 year, of 68% after 2 years, and reaching 72% after 3 years. No complications related to the surgery were detected. CONCLUSION: Patients diagnosed with far-advanced otosclerosis have a good prognosis with cochlear implantation comparable to that of other patients in whom postlingual implants are performed.     

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。     

Cochlear implantation in patients with otosclerosis of the otic capsule

Objective

To evaluate outcomes of cochlear implantation of patients with otosclerosis of the otic capsule.

Late failure of cochlear implantation resulting from advanced cochlear otosclerosis: surgical and programming challenges.

OBJECTIVE: The objective of this study was to discuss cochlear implantation in the setting of severe cochlear otosclerosis and review programming challenges in a patient whose performance is deteriorating as a result of advancing disease. STUDY DESIGN: We conducted a case report and literature review. SETTING: Tertiary care medical center. PATIENTS, INTERVENTION, AND RESULTS: A case is presented of a 66-year-old man with otosclerosis who initially had good benefit from a cochlear implant but gradually lost benefit even with reimplantation. Imaging studies demonstrated severely distorted otic capsule anatomy from cochlear otosclerosis. CONCLUSIONS: Advancing cochlear otosclerosis can result in a severely thinned and distorted otic capsule. Although cochlear implantation is generally beneficial in cochlear otosclerosis, implantation in certain severe cases may be complicated as a result of difficulties with cerebrospinal fluid leak, programming challenges, and other potential hazards that can occur with a distorted anatomy.     

人工耳蜗在老年性聋患者中的应用

目的随着社会人口老龄化,听力障碍在老年人群中的发病率也逐年增加,老年性聋引起的沟通障碍会给老年人群带来生活及心理上的变化,严重影响了老年人的生活质量。目前老年性聋的干预措施较多,其中重度及极重度感音神经性老年性聋患者可选择人工耳蜗植入,本文就老年性聋人工耳蜗植入的选择标准、术前评估、术后并发症、术后康复及效果评估等进行综述。     

Outcome for bilateral cochlear implantation in patients with congenital Cytomegalovirus infection

ObjectivesTo analyze the impact of bilateral cochlear implantation (CI) on perceptual and linguistic development in hearing-impaired children with congenital Cytomegalovirus (CMV) infection.Patients and methodA retrospective study was performed for the period 1991-2016 in a pediatric CI reference center. Closed Set Word (CSW) recognition scores, Categories of Auditory Performance (CAP) and linguistic level on the MT Lenormand scale (MTL) were compared between bilateral (Bi) and unilateral (Uni) groups 12, 24 and 36 months after first CI (CI-1).Results84 patients with congenital CMV infection who underwent CI were included, in 2 groups: sequential or simultaneous bilateral CI (Bi) (N = 20), and unilateral CI (Uni) (N = 64). Twelve, 24 and 36 months after CI-1, CSW scores were 35.56%, 64.52% and 82.93% in Uni and 60.3%, 85% (P = 0.0084*), and 100% (P = 0.00085*) in Bi. CAP scores 12, 24 and 36 months after CI-1 were 2.57, 3.85 and 4.3 in Uni and 3.91 (P = 0.0068*), 5.00 (p = 0.029*) and 5.50 (P = 0.051*) in Bi. MTL linguistic level scores at 12, 24 and 36 months were 0.72, 1.25 and 1.65 in Uni, and 1.72, 3 (P = 0.033) and 3.11 (P = 0.045) in Bi. These significantly better scores in Bi at 24 and 36 months after CI-1 were also found on analysis of subgroups with no associated neurologic disorder (P = 0.046* and P = 0.032*), no associated psychiatric pathology (P = 0.0055* and P = 0.0073*), and no other associated disorder (P = 0.0018* and P = 0.035*), and for all subgroups together (P = 0.0036 and P = 0.037).ConclusionBilateral CI is a faster way than unilateral CI for patients with congenital CMV infection to achieve structured fluent oral language. 50% of the series showed cerebral abnormalities on MRI, without difference between groups. This was not in itself predictive of poor progression of oral communication, unless associated with major neurologic disorder. Some children made little or no use of their CI in the medium term.     

Management options for cochlear implantation in patients with chronic otitis media

ObjectivePatients with chronic otitis media with/without cholesteatoma present a significant challenge to safe cochlear implantation (CI). The aim of our study is to describe our experience and propose management options for CI in patients with chronic otitis media.Study designRetrospective case study.SettingTertiary academic center.Subject and methodsWe enrolled the 9 ears of 8 subjects who received CI in the ear with chronic otitis media from 2006 to 2013 by a single surgeon. CI was performed as a single-stage or staged operation with mastoid surgery according to the activity of ear infection.ResultsSix patients had bilateral chronic otitis media and 2 patients had long history of sensorineural hearing loss at contralateral ear. CI was performed with simultaneous radical mastoidectomy with closure of the EAC as a single-stage in 3 ears with a history of previous open cavity mastoidectomy and no active discharge. Staged CI was performed in 6 ears, after radical mastoidectomy with closure of the EAC in 3 ears and after intact canal wall mastoidectomy in 3 ears, due to active inflammation or complications related to otitis media. In one patient, wound infection had occurred, and implant was removed along with implantation at contralateral ear. Other subjects showed no evidence of recurrence.ConclusionDecision whether implantation as a single-stage or staged operation depends on the presence of active inflammation. Single-stage CI with proper mastoid surgery can be performed in patients without active inflammation. Staged procedure need to be done in ears with active inflammation. Proper application of mastoid surgery leads to safe CI for patients with chronic otitis media.     

多通道人工耳蜗植入在Common cavity畸形患者中的应用

目的：探讨Common cavity畸形患者人工耳蜗植入后的语言康复效果。方法：对6例Common cavity畸形患者采用经乳突外半规管进路行人工耳蜗植入,并与6例耳蜗发育正常组进行听力情况比较。结果：32个电极全部植入者3例,28个电极植入者1例,26个电极植入者1例,22个电极植入者1例。与正常组比较,术中术后并发症差异无显著性意义。术中脑脊液多量流出者2例,少量流出3例,无流出1例,两组术后听力接近,均在30-40dBHL左右,无严重并发症发生,大多数患者术后听觉语言康复效果与政治家耳蜗者接近,少数稍差。长期效果有待进一步观察。结论：人工耳蜗适用于Common cavity畸形患者,但手术前应进行全面的听力学及影像学评估。