Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges,surgical results,and complications

Objective: To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA).

Background: Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications.

Study design: Retrospective review of patients’ medical records and CT/MRI.

Setting: Tertiary referral center.

Materials and methods: The medical records and CT/MRI images of 55?PS/NSEVA patients receiving 80 cochlear implantations from 1982–2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved.

Results: Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo.

Conclusion: Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.     

Systematic review of the literature on the clinical effectiveness of the cochlear implant procedure in paediatric patients

The aim of this systematic review of the literature was to summarize the results of scientific publications on the clinical effectiveness of the cochlear implant (CI) procedure in children. The members of the Working Group first examined existing national and international literature and the principal international guidelines on the procedure. They considered as universally-accepted the usefulness/effectiveness of unilateral cochlear implantation in severely-profoundly deaf children. Accordingly, they focused attention on systematic reviews addressing clinical effectiveness and cost/efficacy of the CI procedure, with particular regard to the most controversial issues for which international consensus is lacking. The following aspects were evaluated: post-CI outcomes linked to precocity of CI; bilateral (simultaneous/ sequential) CI vs. unilateral CI and vs. bimodal stimulation; benefits derived from CI in deaf children with associated disabilities. With regard to the outcomes after implantation linked to precocity of intervention, there are few studies comparing post-CI outcomes in children implanted within the first year of life with those of children implanted in the second year. The selected studies suggest that children implanted within the first year of life present hearing and communicative outcomes that are better than those of children implanted after 12 months of age. Concerning children implanted after the first year of life, all studies confirm an advantage with respect to implant precocity, and many document an advantage in children who received cochlear implants under 18 months of age compared to those implanted at a later stage. With regard to bilateral CI, the studies demonstrate that compared to unilateral CI, bilateral CI offers advantages in terms of hearing in noise, sound localization and during hearing in a silent environment. There is, however, a wide range of variability. The studies also document the advantages after sequential bilateral CI. In these cases, a short interval between interventions, precocity of the first CI and precocity of the second CI are considered positive prognostic factors. In deaf children with associated disabilities, the studies analyzed evidence that the CI procedure is also suitable for children with disabilities associated with deafness, and that even these children may benefit from the procedure, even if these may be slower and inferior to those in children with isolated deafness, especially in terms of high communicative and perceptive skills.     

Cochlear implantation in children with cochlear aplasia

Abstract Conclusion: Children with cochlear aplasia can achieve favorable speech perception abilities from cochlear implantation (CI) by inserting an electrode array into the vestibule if an audiological response is reliably demonstrated before surgery by behavioral audiometry and objective measures. Objectives: The purpose of this study was to evaluate the surgical outcome of CI in children with cochlear aplasia. Methods: The medical records of 454 children who underwent CI at the Dong-A University Hospital between January 1994 and September 2011 were reviewed. The prevalence of cochlear aplasia and the preoperative evaluations, operative findings, and postoperative speech perception abilities of implanted children with cochlear aplasia were evaluated. Results: The implanted children with cochlear aplasia accounted for 2% of those with inner ear malformations and 0.4% of all pediatric cochlear implantees. They showed audiological responses in preoperative electrophysiologic tests, and the eighth nerve was present in magnetic resonance imaging. The electrode array was inserted into the vestibule through an oval window after removing the stapes or through a transmastoid labyrinthotomy site. Electrical compound action potential and electrical auditory brainstem response tests showed clear neural responses after implantation. The hearing thresholds of the implanted ear improved to 25 dB HL and the children achieved significant open set speech perception abilities.     

Contemporary surgical issues in paediatric cochlear implantation

Abstract

Objective: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. Design: Narrative literature review. Results: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. Conclusion: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.     

259例人工耳蜗植入患者的并发症分析及处理

目的分析259例人工耳蜗植入(cochlear implantation,CI)患者的并发症,探讨并发症产生的原因、处理方法和预后。方法对1997年3月～2006年12月在我科接受人工耳蜗植入的259例患者的并发症及其处理和预后情况进行回顾性分析。结果259例人工耳蜗植入患者平均年龄8.4岁,最小11个月,最大52岁。随访时间平均2.9年。有4例患者发生严重的手术并发症(1例鼓膜穿孔行二期修补术、2例面神经麻痹行面神经减压术、1例脑脊液耳漏合并脑膜炎)。有39例患者出现轻度手术并发症,包括鼓膜穿孔、血肿、切口感染、面神经部分暴露等,经保守治疗或术中简单处理均得到有效控制。与人工耳蜗装置相关的并发症10例(3例人工耳蜗电极未植入或仅部分植入耳蜗内、1例术后电极脱出、5例人工耳蜗装置故障、1例耳蜗内植入后无反应)。所有手术并发症经过保守治疗或手术干预,预后良好。10例人工耳蜗装置故障经调整或再植入后,9例得到圆满解决,1例植入体取出。结论人工耳蜗植入是相对安全的手术。大部分手术并发症为轻度并发症,通过保守治疗或小的手术干预预后良好。人工耳蜗植入病例的选择要注意适应症。     

Temporalis myofascial flap coverage for extrusion of internal device after cochlear implantation

Two pediatric patients with internal device exposure of cochlear implant (CI) were treated successfully using vascularized temporalis myofascial flaps. The visible scarring was minimal, and the CI function was excellent. Although we used temporalis myofascial flaps for late complications of an implanted ear, it can also be used in a primary case to provide flap reinforcement. In conclusion, the temporalis myofascial flap technique is an ultimate surgical option that offers advantages for CI patients with flap-related problems.     

Pediatric cochlear implant revision surgery and reimplantation: An analysis of 957 cases

Objective

In this study causes, risk factors, prevention strategies, diffuculties encountered during revision cochlear implant surgery, reimplantation in pediatric age group were evaluated.

Methods

Charts of 957 CI patients younger than 18 years of age implanted at Izmir Bozyaka Teaching and Research Hospital between 1998 and July 2012 and 18 referred CI complications at same age group were retrospectively evaluated. Revision and reimplantation surgeries were systematically reviewed.

Results

We encountered 26 surgical complications in 24 patients and 36 device related problems in 36 patients. Eighteen referred cases including 7 surgical complications and 11 device related problems were also evaluated. A total number of 80 complications were evaluated. In 11 cases conservative management was successful, 19 revision surgery was performed but we failed in 4 cases. We reimplanted 48 cases and 4 additional cases were implanted after failed revision surgery. Implant was extracted in one case. In one case we could implant the other side. Forty-six of reimplantations were done in one stage surgery, 5 cases required second stage surgery.

Conclusions

Surgical complications and device related problems of cochlear implantation may be different in children and majority of them require revision surgery or reimplantation. Although surgical problems leading to revision surgery and reimplantation are expected to diminish by experience every center has to deal with device failures. Both revision surgery and reimplantation require extra care and it should be better carried out by experienced surgeons. Implant performances are expected to be comparable with primary implantations.     

Cochlear implantation in atelectasis and chronic otitis media: long-term follow-up.

OBJECTIVE: To report the long-term results of cochlear implantation in cases with chronic otitis media or atelectasis using a single surgical technique performed in a single cochlear implant center. PATIENTS: Nine patients who were implanted using the blind-pit closure of the external ear canal technique (4 patients with adhesive otitis media and 5 with radical mastoid cavities). Follow-up ranged from 18 months to 12 years (mean, 7.05 yr). INTERVENTION: The surgical procedure was performed in 2 stages. The first stage included canal wall down or lowering any high facial ridge in previous mastoidectomies, removal of all skin, and blind-pit closure of the external ear canal without mastoid cavity obliteration or eustachian tube obliteration. Cochlear implantation was performed 6 months after the first surgical procedure. RESULTS: All operations were uneventful, and during cochlear implantation, as a second stage, no epithelia or other problems were encountered. No serious complications were encountered during the follow-up period. One case had a minor disruption of the external canal closure that was reclosed successfully under local anesthesia. All patients were using the device at the last follow-up interval with no device problems. CONCLUSION: Blind-sac closure of the external ear canal without obliteration is a rather safe surgical procedure in cases with chronic otitis media or atelectasis. Meticulous surgical technique and proper patient selection are of paramount importance. However, a 2-stage procedure may not always be necessary and might best be confined to those patients who have active inflammatory disease at the primary procedure.     

Labyrinthine calcification in ears with otitis media and antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV): A report of two cases

Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) has been proposed as a new type of otitis media. The hearing loss caused by OMAAV can be expected to improve with early detection and intervention, but if it continues to worsen and leads to deafness, it is challenging to recover the patient's hearing. When bilateral deafness occurs, cochlear implant (CI) surgery is the only way to improve hearing. Here, Case 1 showed unilateral cochlear calcification, and Case 2 showed bilateral cochlear calcification. In Case 1, CI surgery was performed on the ear lacking calcification, and in Case 2 it was performed on the ear with milder calcification. In Case 2, granulation was present from the tympanic space to the mastoid, the round window was closed, and the basal turn of the cochlea was narrowed. Such calcification of the cochlea caused by OMAAV has not been reported so far. It is essential to detect these changes by computed tomography scans at an early stage and to perform CI surgery at an appropriate time, because hearing improvements are not expected in patients who become deaf because of OMAAV.     

中耳乳突炎性病变的人工耳蜗植入术

目的探索双侧重度感音神经性聋合并中耳乳突炎性病变人工耳蜗植入手术方法.方法 29例不同类型中耳乳突炎患者行人工耳蜗植入手术,根据炎症病变程度采用Ⅰ期手术26例,分期手术3例.非化脓性及慢性中耳炎静止期者,行完壁式乳突根治术,清除病灶,保留骨性外耳道,同期行人工耳蜗植入;化脓性中耳炎者,行完壁式或开放式乳突根治术,清除病灶保留骨性外耳道或重建外耳道后上壁,获得干耳后,Ⅱ期行人工耳蜗植入.结果 29例中耳乳突不同炎症病变患者经Ⅰ期或分期手术,人工耳蜗电极均顺利植入.随访2～8年,无炎症病变复发,开机后人工耳蜗工作正常,无电极裸露、脱出.结论中耳乳突炎性病变经正确的手术措施干预后,可行人工耳蜗植入.强调依据病变程度和范围个性化处理.     

人工耳蜗植入术后发生面瘫的预防及处理

目的分析人工耳蜗植入术(CI)后的面瘫并发症病例,探讨并发症的预防和处理方法.方法对1998年3月~2012年1月在解放军总医院耳鼻咽喉-头颈外科接受人工耳蜗植入的1299例患者中发生的面瘫并发症及其处理和预后情况进行回顾性分析.结果1299例患者中,4例患者行人工耳蜗植入术后发生面神经麻痹,其中3例为迟发性面瘫,2例行面神经减压术,预后良好.结论人工耳蜗植入是相对安全的手术,面神经麻痹的发生率较低,为0.31%,经保守治疗或手术干预预后良好.     

Late skin flap failure following cochlear implantation

The objective of the study was to determine risk factors for late skin flap failure following cochlear implantation. The study design was a retrospective case series. Data were collated from 371 implanted patients over a ten-year period. Of these patients, 1.3 per cent (n = 5) developed late skin flap failure. It is concluded that skin flap failure is a significant, though relatively uncommon, complication of cochlear implant surgery. What causes flap failure and why it should occur in such a variable fashion are still largely unknown, though infection undoubtedly plays an important role. The aetiological factors associated with flap failure and possible prevention strategies are discussed.     

蜗窗膜入路人工耳蜗植入术

目的　探讨以持续性眩晕为表现的良性阵发性位置性眩晕（benign paroxysmal positional vertigo,BPPV）患者的诊治。方法　回顾性分析2例以持续性眩晕为表现的BPPV患者的临床资料。结果　此2例患者在坐位及平躺位见方向向患侧的水平自发持续性眼震,甩头试验健侧阳性,平卧侧头试验双侧均诱发出水平离地性眼震,平躺后头部向患侧连续转360°时分别出现2个眼震消失点和2个眼震最强点,给予手法复位后患者眩晕症状缓解。结论　水平半规管BPPV患者偶可表现为持续性眩晕发作,其病因为壶腹嵴帽耳石症,临床表现与耳石重力因素和水平半规管空间位置相关。     

Cochlear implantation in deaf infants

OBJECTIVES: With the application of universal newborn hearing screening programs, a large pool of newly identified deaf infants has been identified. The benefits of early intervention with cochlear implants (CI) is being explored. Mounting evidence suggests that age at implantation is a strong predictor of language outcomes. However, new behavioral procedures are needed to measure speech and language skills during infancy. Also, procedures are needed to analyze the speech input to young CI recipients. STUDY DESIGN: Cohort-sequential. METHODS: Thirteen infants with profound hearing loss who were implanted between the ages of 6 to 12 months of age participated in this study. Eight participated in two new behavioral methodologies: 1) the visual habituation procedure to assess their discrimination of speech sounds; 2) the preferential looking paradigm to assess their ability to learn associations between speech sounds and objects. Older implanted infants and normal-hearing infants were also tested for comparison. The pitch of mothers' speech to infants was analyzed. RESULTS: Patterns of looking times for the very early implanted infants were similar to those of normal hearing infants. Mothers' speech to infants with CIs was similar in pitch to normal-hearing infants who had the same duration of experience with sounds. CONCLUSIONS: No surgical or anesthetic complications occurred in this group of infants, and the pattern of listening skill development mirrors that seen in normal-hearing infants. Mothers adjust their speech to suit the listening experience of their infants.     

Kochleaimplantat bei Erwachsenen: Indikation und Durchführung

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery. Modern advanced CI devices offer technical reliability and multiple speech encoding strategies and recently, even small processors which are worn behind the ear. Further technical advances are continuously available, even for implanted patients. Upgrading is usually easy because the outer parts of the implant are exchangeable and software upgrades can be transmitted transcutaneously to the implant. Assuming that medical and rehabilitation requirements are available, there are only very few indications against CI in adults. Candidate patients should therefore be transferred to CI-centres where a decision can be taken depending on presurgical evaluation results.     

Cochlear implants in adults: indications and realizations. Part I: Diagnosis,operation techniques and results

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery. Modern advanced CI devices offer technical reliability and multiple speech encoding strategies and recently, even small processors which are worn behind the ear. Further technical advances are continuously available, even for implanted patients. Upgrading is usually easy because the outer parts of the implant are exchangeable and software upgrades can be transmitted transcutaneously to the implant. Assuming that medical and rehabilitation requirements are available, there are only very few indications against CI in adults. Candidate patients should therefore be transferred to CI-centres where a decision can be taken depending on presurgical evaluation results.     

Paroxysmal positional vertigo after cochlear implantation

CONCLUSIONS: We observed 4 cases of paroxysmal positional vertigo (PPV) among 62 cochlear implant (CI) recipients. They occurred in the implanted ear without chronological relation with the surgical procedure or the implant's activation. All of them relapsed within 3 months after an initially successful repositioning maneuver, and finally recovered after the second one. None of the patients showed labyrinthine weakness in the implanted ear. The outbreak of PPV did not affect the patients' speech perception performances. OBJECTIVES: To report and discuss the occurrence of PPV after cochlear implantation. PATIENTS AND METHODS: Among 32 adult patients who received a Nucleus CI, 4 suffered from PPV on the basis of Dix-Hallpike's maneuvers. After a Semont's repositioning maneuver, recurrences were similarly addressed. An electro-nystagmography (ENG) recording of caloric irrigation tests was obtained once the symptoms subsided. RESULTS: The observed incidence of 12.5% exceeds the figures reported in the literature. No anatomic abnormalities were identified in these patients, nor were any intraoperative or postoperative complications reported. PPV developed 1-12 months after CI surgery in the posterior canal and relapsed within 3 months. ENG showed a normal reflectivity in two patients, while the other two had a significant prevalence of the implanted side. The outcomes of the speech perception tests after CI in all the four patients matched those of their corresponding categories.     

The use of subtotal petrosectomy in cochlear implant candidates with chronic otitis media

Cochlear implants (CI) candidates with chronic otitis media require special attention and management. The need of opening of the inner ear creates potential routes of spread of infection to subarachnoid spaces and lead to meningitis. The aim of the study was to analyse the technique and complications of subtotal petrosectomy (SP) in cochlear implant candidates with chronic otitis media at three different CI centres. A retrospective study was carried out in three Territory Referral Hospitals. The centres follow Fisch’s philosophy and surgical techniques of SP. The study group consisted of 19 patients, 4 men and 15 women, aged 12–82 years. All patients underwent SP with either primary or staged CI implantation. Indications for single or a staged management, difficulties during surgery and complications were analysed. Skin and muscle flap design in primary and revision cases as well as imaging follow-up strategy are discussed. In 14 patients implantation was performed in a single stage and in 5 cases in two stages. Follow-up ranged from 8 months to 10 years. All the patients use their implants and there were no major nor minor complications. The use of subtotal petrosectomy with cochlear implants is a safe and efficient technique when strict surgical steps and rules are applied. Closure of the external ear canal after previous meatoplasty can be challenging and extreme care dissecting the skin flaps is required. In patients with extensive cholesteatoma, active discharge from the ear with resistant bacteria or an “unstable” situation, the procedure can be staged.     

Cochlear implantation in obstructed cochleas: the effect of the degree of obstruction on the number of activated electrodes and the amount of postoperative speech perception

Objective: With cochlear implantation, insertion of the electrodes can be hampered by cochlear obstructions. The aim of this study was to investigate the effects of these obstructions on the number of activated electrodes and postoperative speech perception. Study design and setting: Retrospective analysis of the operation reports and CT‐scans of patients who underwent cochlear implantation, at the Department of Otorhinolaryngology of the University Medical Center of Utrecht (n = 295). Patients and interventions: Fifty patients with a certain degree of cochlear obstruction were included. Based on the surgical intervention to overcome these obstructions they were subdivided into first‐, second‐ and third degree obstructions. The number of implanted and activated electrodes was determined. Main outcome measures: Three different speech perception tests after 12 months of cochlear implant use. Results: The number of activated electrodes significantly diminished with increasing obstruction degree (P < 0.01). This was caused by a lower amount of implanted electrodes combined with a higher amount of switched off electrodes. With a higher degree of obstruction the amount of postoperative speech perception decreased significantly (P < 0.01). This is partly explained by the lower number of activated electrodes and partly explained by the fact that a higher obstructions degree correlates with a higher degree of (retro‐) cochlear pathology. Significantly more electrodes can be inserted into the severely obstructed cochlea using a Double Array Cochlear Implant. Conclusion: Cochlear obstructions pose a surgical threat and negatively influence the postoperative speech perception results. Fortunately, due to surgical techniques, first and second degree obstructed cochleas can often be bypassed. Implanting of a Double Array implant brightens the prospects of patients with third (severe) degree obstructed cochleas.     

34例人工耳蜗植入术后再次手术患者的原因和处理方法分析

目的分析人工耳蜗植入术后再次手术患者的原因及处理方法。方法回顾性分析2009年1月~2017年12月1 693例接受人工耳蜗植入术后再次手术的34例(2.01%,34/1 693)患者的临床资料,分析导致再次手术的原因及处理方法。结果 34例中,皮下积液、感染12例(0.71%,12/1 693),皮瓣感染坏死4例(0.24%,4/1 693),植入体移位7例(0.41%,17/1 693),人工耳蜗损坏、故障5例(0.30%,5/1 693),外耳道后壁穿孔2例(0.12%,2/1 693),脑脊液耳漏2例(0.12%,2/1 693),异位植入1例(0.06%,1/1 693),排异反应1例(0.06%,1/1 693)。12例皮下积液、感染的患者均行切开引流、清理病变后保留人工耳蜗;4例皮瓣感染坏死患者均行皮瓣转移修复术,3例取出人工耳蜗并于对侧植入新人工耳蜗,1例保留人工耳蜗;7例植入体移位的患者5例手术复位,2例取出人工耳蜗,其中1例同侧再次植入人工耳蜗;5例人工耳蜗损坏、故障的患者均取出损坏的人工耳蜗,同侧重新植入同型号新人工耳蜗;2例外耳道后壁穿孔的患者均行外耳道后壁修补术,1例取出人工耳蜗,1例保留,均于对侧植入新人工耳蜗;2例脑脊液耳漏的患者堵塞耳蜗造瘘口后均取出人工耳蜗;1例异位植入的患者重新植入新人工耳蜗;1例排异反应的患者清理病灶后保留人工耳蜗。结论人工耳蜗植入术后再次手术的原因有患者自身原因、植入体原因及人工耳蜗植入手术原因,需根据不同原因进行相应的处理。