Development of auditory skills after cochlear implantation in children with inner ear malformations

Conclusions: CI improves hearing thresholds and auditory skills in children with most types of inner ear malformations. However, the development of sound detection skills is not as good as it is in children without inner ear malformations. Objectives: To investigate the influence of inner ear malformations on development of auditory skills after cochlear implantation (CI). Methods: Records of 20 children with inner ear malformations who underwent cochlear implantation before 4 years of age and followed up for more than 2 years were retrospectively reviewed. Hearing thresholds, the Meaningful Auditory Integration Scale (MAIS), and Meaningful Use of Speech Scale (MUSS) scores before and after CI were analyzed and compared with 20 age-matched deaf children who underwent CI. Results: The children with inner ear malformations showed significant improvements in hearing thresholds and the MAIS and MUSS scores 1 year after CI (p?p?p?相似文献   

The new classification system for inner ear malformations: the INCAV system

Conclusions: The proposed INCAV system standardizes reporting of inner ear malformations; gives adequate information about the structures of inner ear; defines the ears which could not be classified before; and helps in the selection of the ear as the cochlear implant candidate. Also it is easy-to-use for radiologists, and useful to the referring otolaryngologists.

Objective: This study was conducted to explore a more specific, definitive classification system which was based on radiological criteria for inner ear malformations.

Method: This study found 43 patients who had inner ear malformations, magnetic resonance (MR), and computed tomography (CT) imaging, together with the retrospective evaluation of the medical records between August 2010 and February 2015. It analyzed inner ear structures by dividing five sub-groups and each sub-group was given a letter: internal acoustic canal (I), cochlear nerve (N), cochlea (C), vestibular aqueduct (A) and vestibule (V). Based on their malformations, these anatomical structures have been assigned grades and have been classified by using increasing numbers which were dependent to increasing order of severity of the malformation.

Results: Among these 43 patients, there were six normal (I₀N₀C₀A₀V₀) and 80 inner ear malformations. All of the ears were defined successfully by the INCAV system.     

A new cochlear implant electrode with a “cork”-type stopper for inner ear malformations

Objective

Gusher in inner ear malformations is common in patients with incomplete partition type I and type III. It is also common in less severe form as oozing in incomplete partition type II and large vestibular aqueduct. It is important to prevent cerebrospinal fluid (CSF) escape around the electrode to prevent meningitis.

Methods

The custom-made device was produced by Med-El Company. It has a “cork”-like stopper instead of the usual silicon ring to prevent gusher. There are two types of electrodes of different lengths. The standard one is 25 mm (contact space 1.7 mm) and the short one is 20 mm (contact space 1.3 mm). It was used in 50 patients with different inner ear malformations.

Results

Thirteen patients had gusher, and 11 patients oozing during cochleostomy. One patient with initial prototype of the cork electrode had to be revised because of persistent oozing around the electrode. Another patient had slow extrusion of the electrode most probably due to CSF pulsation and had to be revised. Both patients had no more CSF fistula.

Conclusion

CSF fistula in inner ear malformations is a serious situation which may lead to recurrent meningitis. The new electrode with “cork” stopper looks promising in preventing the postoperative CSF leak around the electrode.     

Wideband tympanometry findings in inner ear malformations

ObjectiveThe deficits in the cochlea which is at the one end of the ear sound transfer system, may effect middle ear functions. Wideband typanometry (WBT) is frequently used to evaluate these transfer functions which play a crucial role in setting the impedance matching between the external ear and the cochlea. To this end, the aim of this study was to investigate the ear transfer functions in inner ear malformations via WBT, and to question whether these functions change depending on the types of inner ear malformation.MethodsThis prospective case-control study was conducted in a university hospital. One hundered-fifty-seven ears (aged 3–37 years) under the groups of cochlear hypoplasia, incomplete partition I, incomplete partition II, cochlear aplasia and complete labyrinthine aplasia were evaluated. In the control group, 30 ears with normal hearing were enrolled and WBT was carried out. Tympanometric peak pressure, equivalent middle ear volume, static admittance, tympanogram width, resonance frequency, average wideband tympanometry and absorbance measurements were analyzed.ResultsThe inner ear malformation groups demonstrated statistically significant differences than the control group and from each other in terms of traditional tympanometric parameters and WBT test parameters (p < 0.05). The most remarkable difference was between the group of complete labyrinthine aplasia and the control group, most probably because of complete labyrinthine aplasia’s structural effects. However, on some parameters, incomplete partition II and the control group showed similarities. In absorbance measurements, there was significant difference between all patient groups and the control group, especially at high frequencies (p < 0.05). The largest difference was between the control group and the group of complete labyrinthine aplasia which has revealed the lowest absorbance values (p < 0.05). In averaged-wideband tympanogram analysis, all patient groups obtained a lower amplitude peak than the control group; complete labyrinthine aplasia group had the flattest peaked amplitude, while the incomplete partition II group had a near-normal curve.ConclusionThe results of the study revealed the distinctive effects of inner ear malformations in middle ear transfer functions. It is concluded that the absence of inner ear structures causes negative effects on energy absorbance and the other transfer functions of the middle ear. WBT may provide additional information on diagnosis of patients with inner ear malformations.     

Inner ear malformations with oto-liquorrhea. Tomographic findings in three cases with a mixed hearing impairment

Summary The paper deals with three boys suffering from a mixed hearing loss. They were all referred to tomography of the temporal bones in order to find some middle ear malformation amenable to surgery. The tomograms revealed identical malformations of the inner ear; the lumen of the labyrinth are found irregularly dilated and the internal acoustic meatus is widened. Its course in the lateral end is abnormal with a downwards turn towards the basal coil of the cochlea. Explorative tympanotomy in one of the patients revealed a severe otoliquorrhea during an attempt to perform a stapedectomy, and this may serve as a warning against operation in patients presenting the mentioned tomographic changes which, once seen, are easily recognized.

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Zusammenfassung Die Arbeit berichtet über drei Jungen, bei denen eine kombinierte Schwerhörigkeit bestand. Bei allen wurde die Tomographie der Ossa Temporalia durchgeführt, um Mißbildungen des mittleren Ohres mit der Möglichkeit der chirurgischen Intervention aufzuzeigen. Die Tomogramme zeigten identische Mißbildungen des inneren Ohres: Die Lumina des Labyrinthes waren unregelmäßig diktiert und der Meatus acusticus internus erweitert. Er verlief am lateralen Ende in Richtung der Basalwindung der Cochlea. Bei Tympanotomie und Versuch der Stapedektomie bei einem der Patienten kam es zu einer schweren Otoliquorrhoe. Dieser Befund soll als Wagung dienen, bei Patienten die solche tomographische Befunde zeigen, zu operieren; Befunde, die, einmal gesehen, leicht erkannt werden können.

     

内耳发育畸形患者人工耳蜗植入术后的疗效分析

目的分析内耳发育畸形患者行人工耳蜗植入术后的疗效。方法回顾性分析2011年1月~2017年7月在西南医科大学附属医院行人工耳蜗植入术的30例内耳发育畸形患者的临床资料,其中大前庭水管综合征(large vestibular aqueduct syndrome,LVAS) 8例,Mondini畸形（不完全分隔Ⅱ型）7例,同时伴有LVAS和Mondini畸形10例,内听道狭窄5例。随机选取同时期临床资料相匹配的30例耳蜗结构正常的人工耳蜗植入患者作为对照组。采用听觉行为分级标准和言语可懂度分级标准进行评估。比较两组患者的术前听力、术中情况以及术后听觉言语康复情况。结果3例LVAS患者在术中耳蜗钻孔后出现外淋巴液不同程度的外涌,1例Mondini畸形患者术中出现脑脊液井喷现象。两组患者术后均获得有意义的听觉反应,术后的听觉言语能力相比较,差异无统计学意义（P>0.05）。同组患者,术后3,6,9,12个月的听觉言语能力均较各自术前均有明显提高（P<0.05）。结论内耳发育畸形患者不是人工耳蜗植入术的绝对禁忌证,其术后言语康复效果与内耳结构正常者无明显差异,但手术难度比内耳结构正常者要大,术前应根据不同的畸形类型制定个性化的手术方案,以减少并发症的发生。     

A novel combination of drug therapy to protect residual hearing post cochlear implant surgery

Conclusions A cocktail combining NAC, Mannitol, and Dexamethasone may be used to prevent loss of residual hearing post-implantation. There is a window of opportunity to treat the cochlea before the onset of cell death in HCs. Objective Inner ear trauma caused by cochlear implant electrode insertion trauma (EIT) initiates multiple molecular mechanisms in hair cells (HCs) or support cells (SCs), resulting in initiation of programmed cell death within the damaged tissues of the cochlea, which leads to loss of residual hearing. In earlier studies L-N-acetylcysteine (L-NAC), Mannitol, and dexamethasone have been shown independently to protect the HCs loss against different types of inner ear trauma. These three molecules have different otoprotective effects. The goal of this preliminary study is to test the efficacy of a combination of these molecules to enhance the otoprotection of HCs against EIT. Methods OC explants were dissected from P-3 rats and placed in serum-free media. Explants were divided into control and experimental groups. Control group: (1) untreated controls; (2) EIT. Experimental group: (1) EIT?+?L-NAC (5, 2, or 1?mM); (2) EIT?+?Mannitol (100, 50, or 10?mM); (3) EIT?+?Dex (20, 10, or 5?μg/mL); (4) EIT?+?L-NAC?+?Mannitol?+?Dex. After EIT was caused in an in-vitro model of CI, explants were cultured in media containing L-NAC alone, Mannitol alone, or Dex alone at decreasing concentrations. Concentrations of L-NAC, Mannitol, and Dex that showed 50% protection of hair cell loss individually were used as a combination in experimental group 4. Results There was an increase of total hair cell (THC) loss in the EIT OC explants when compared with control group HC counts or the tri-therapy cochlea. This study defined the dosage of L-NAC, Mannitol, and Dex for the survival of 50% protection of hair cells in vitro. Their combination provided close to 96% protection, demonstrating an additive effect.     

感音神经性聋患儿中先天性内耳畸形的构成、影像学及临床听力学特征

目的:分析婴幼儿、儿童先天性感音神经性聋(SNHL)中先天性内耳畸形的构成、影像学及临床听力学特征。方法:回顾性分析2005-02-2010-01上海交通大学医学院附属上海儿童医学中心耳鼻咽喉科诊治的860例先天性SNHL患儿中,经颞骨高分辨率CT及MRI发现有先天性内耳畸形的125例(225耳)患儿的听力学及影像学资料。结果:860例先天性SNHL患儿中有先天性内耳畸形者占14.5%;累及双侧98例(78.4%),单侧27例(21.6%)。225耳中167耳(74.2%)为极重度聋,36耳(16%)为重度聋,22耳(9.8%)为中度聋。该组内耳畸形中,前庭水管扩大最多见(75.6%),其次为前庭畸形(32%),再次为耳蜗前庭畸形(23.1%)。耳蜗前庭畸形中以Mondini畸形最常见(55.8%),其次为共同腔畸形(28.9%)。累及耳蜗的内耳畸形中极重度聋者明显多于未累及耳蜗的内耳畸形中极重度聋者。结论:对了解中国婴幼儿、儿童先天性SNHL中先天性内耳畸形的构成,对先天性SNHL的病因诊断以及对包括助听器、耳蜗植入等在内的干预策略的制订及其预后有一定意义。     

The Erasmus atelectasis classification: proposal of a new classification for atelectasis of the middle ear in children

OBJECTIVES: Atelectasis presents a challenging, often progressive, problem in children. Because of the lack of a clinically practical classification, we introduce a new classification, which in our opinion is more useful in the pediatric age group. This alternative classification enables a more clinically relevant correlation between stage of disease and clinical sequelae and technical difficulty at surgery. STUDY DESIGN: Observational study of patients seen and operated at the Sophia Children's Hospital in Rotterdam, The Netherlands between 1989 and 2005. METHODS: Based on clinical appearance, each ear was placed into one of the five groups of the proposed classification and into one of the four stages of Sadé's classification. Preoperative air and bone conduction thresholds and air-bone gaps (ABG) were calculated using the four-tone pure-tone (500, 1,000, 2,000, and 4,000 Hz) averages for bone and air conduction. RESULTS:: Of the 248 ears in the study group, 72 were in stage I, with an ABG of 18.2 +/- 12.3 dB. Twenty-two were in stage II, with an ABG of 12.9 +/- 9.5 dB. In stage III, there were 32 ears, with an ABG of 11.6 +/- 10.0 dB. Thirty-one ears were in stage IV, with an ABG of 16.1 +/- 11.5 dB. Eighty-five ears were in stage V, with an ABG of 26.1 +/- 13.3 dB. When grouped according to Sadé's classification, 92 ears could not be classified. CONCLUSIONS: We found the currently proposed classification more useful in that it follows the natural progression of the disease and is more practical in determining operative procedures at each stage.     

内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例,对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170,18.8%)明显高于其他文献报道;②32例内耳畸形中,大前庭导水管23例(占全部畸形数71.3%),大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例,并发外半规管未发育1例),Mondini畸形2例,Mondini畸形并发外半规管未发育前庭腔扩大1例,耳蜗CT影像疑似为“三叉”无法分类1例;③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例,及大前庭导水管并发Mondini畸形1例);④耳蜗影像疑似为“三叉”患者,术中发生严重井喷,电极植入困难,4个电极不能植入,术后听力未改善,半年后行对侧耳植入成功;⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作,术后1年行手术探查,后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括,大前庭导水管综合征及其相伴发或单发的各类内耳畸形;②内耳畸形非人工耳蜗植入术的绝对禁忌证,但术中严重井喷多见,电极植入不完全多见,术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗,术前详细的影像学检查可以对各类畸形进行详细分类,并在术前对手术难度有充分的准备,可以减少相关并发症的发生。     

Optical coherence tomography of the cochlea in the porcine model

Objectives/Hypothesis: To demonstrate the feasibility of optical coherence tomography in microstructural imaging of the porcine cochlea. Study Design: Ex vivo, porcine model. Methods: Optical coherence tomographic images of the porcine cochlea were obtained by thinning the bone from the basal turn of the cochlea leaving the endosteum intact. The images were compared with the corresponding histological sections. Results: In the areas of thinned bone, images were obtained of the stria vascularis, Reissner's membrane, basilar membrane, tectorial membrane, scala media, scala tympani, and scala vestibuli. The bone was too thick for adequate light penetration in the areas where it was not thinned. Good histological correlation was obtained. Conclusions: Cochlear and vestibular microanatomic structures of the pig cochlea were clearly identified with histological confirmation, suggesting the potential application of this noninvasive imaging modality for in vivo imaging of the human cochlea.     

感音神经性聋患者内耳CT表型与SLC26A4基因型关系探讨

目的 分析感音神经性聋患者内耳高分辨率螺旋CT(下简称CT)表型与SLC26A4基因致病性突变类型之间的关系,探讨前庭导水管扩大相关内耳畸形患者SLC26 A4基因检测辅助CT诊断的可行性.方法 以Sennaroglu2010分类为标准分析2 705例感音神经性聋患者内耳CT表型,并采用DNA测序的方法检测全部患者SLC26A4基因致病性突变检出率及突变类型,统计该基因突变在患者各类内耳CT表型中的分布特点,分析SLC26A4基因突变类型与CT表型之间的关系.结果 ①2 705例患者中CT诊断为内耳畸形826例(30.54％,826/2 705),其中耳蜗畸形446例:Michel畸形5例、耳蜗未发育12例、共同腔畸形6例、耳蜗发育不全畸形34例(CH-Ⅰ 9例、CH-Ⅱ 8例、CH-Ⅲ 17例)、耳蜗分隔不全畸形389例(IP-Ⅰ 22例、IP-Ⅱ 352例、IP-Ⅲ 15例);非耳蜗畸形380例(前庭导水管扩大340例,单纯前庭、半规管、内听道畸形40例);内耳CT正常1 879例.②共检出SLC26A4基因双等位基因致病性突变517例,其中纯合突变164例、复合杂合突变353例,均在前庭导水管扩大相关内耳畸形患者(IP-Ⅱ 264例,前庭导水管扩大253例)中检出,在本组前庭导水管扩大相关内耳畸形患者中检出率为74.71％ (517/692).结论 SLC26A4基因致病性突变与前庭导水管扩大相关内耳畸形密切相关,SLC26A4基因检测在前庭导水管扩大相关内耳畸形患者中可辅助CT成为诊断工具.     

Vestibulo-ocular reflex (VOR) preserved in bilateral severe vestibular malformations with internal auditory canal stenosis

A 60-year-old woman, who has suffered from bilateral deafness throughout her life, visited our outpatient clinic. Computed tomography (CT) revealed inner ear malformations, which comprise cochlear aplasia with hypoplastic vestibule in the right ear and a common cavity in the left ear, and narrow internal auditory canals. We performed electronystagmography with caloric stimulation and stimulation of earth-vertical axis rotation (EVAR) or off-vertical axis rotation (OVAR), and studied vestibular evoked myogenic potentials (VEMPs) and vestibular ocular reflex (VOR). Slight horizontal nystagmus was induced by the stimulation of EVAR but not by caloric stimulation. Slight vertical nystagmus was observed during OVAR, whereas the VEMP test elicited no response. The result of horizontal or vertical VOR performed in the dark was almost normal. These findings suggest that VOR can be acquired even with severe malformation of the inner ear whose labyrinthine functions markedly reduce bilaterally until nystagmus is slightly induced by rotation stimulation.     

Middle ear malformations in identical twins

The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium.     

Bilateral implantation in children with cochleovestibular nerve hypoplasia

To report on the outcomes of sequential bilateral cochlear implantation (CI) in children with inner ear malformation. The study design is a retrospective case study. The setting is a tertiary reference center. Two children presenting a profound bilateral congenital hearing loss with bilateral hypoplasia of the cochleovestibular nerves and hypoplasic external semicircular canal had a cochlear implant at respectively 16 months and 33 months. A second implant was proposed at respectively 17 and 20 months after the first implant. The main outcome measures are audiometry, perceptive results in closed and open set words (CSW and OSW) and oral production at follow-up. The first cochlear implant gave respectively mean thresholds at 60 dB and 70 dB. Bilateral CI showed mean threshold at respectively 40 dB and 55 dB. In case 1, perceptive assessment was 83% and 70% in respectively CSW and OSW with oral production and comprehension of sentences after 1 year follow-up. In case 2, the perceptive assessment showed no perceptive or linguistic evolution at 6 months follow-up. In cochleovestibular nerve hypoplasia, bilateral implantation could be discussed in cases of limited result after unilateral implant.     

内耳畸形伴脑脊液耳漏的高分辨率CT特征

目的:探讨先天性内耳畸形伴脑脊液耳漏的高分辨率CT(HRCT) 特征.方法:回顾性分析11例先天性内耳畸形伴脑脊液耳漏患者的HRCT表现.结果:共11例患者,耳蜗未发育4例,共腔畸形2例,耳蜗未未分隔型3例,耳蜗不全分隔型2例.内耳道底骨质缺损8例.所有患者患侧乳突气化和发育程度与对侧一致,前庭窗处都有软组织影.结论:内耳畸形伴脑脊液耳漏患者常存在内耳道底骨质缺损.前庭窗是脑脊液由内耳进入中耳腔的好发部位.HRCT能为先天性内耳畸形伴脑脊液耳漏患者的诊断及治疗提供可靠依据.