Cochlear Implantation Via Extended Endaural Incision in a Patient With Congenital Ear Malformation.

Systematic Review and Meta-analysis of Surgical Complications following Cochlear Implantation in Canal Wall Down Mastoid Cavities.

To explore the congenital ear malformation (CEM)-specific quality of life (QOL) and examine the effects of total ear reconstruction surgery for QOL of CEM patients. A self-composed QOL scale was used for 129 patients with congenital external and middle ear malformation. All patients were requested to fill in the QOL scale before and 1 month after ear reconstruction surgery. The level of QOL varied according to the degree of ear malformation. The total QOL score of patients with unilateral and bilateral CEM was 28.5 ± 18.4 and 51.6 ± 23.6, respectively. The total QOL score of patients pre- and post-operation was 21.0 ± 14.0 and 14.2 ± 9.7 respectively. Physiological functions, psychological status and social interactions of the patients were of statistical significance after ear reconstruction surgery compared to that of at pre-operation. Congenital ear malformation-specific QOL scale can show sensitively the changes of QOL of CEM patients. And ear reconstruction surgery is beneficial for the patients.

This study focused on the intensities of cochlear implant (CI) stimulation in pediatric CI users with inner ear malformation or cochlear nerve deficiency (CND). In this population, CI programming is difficult because a large intensity of CI stimulation is required to achieve sufficient hearing, but the excess CI stimuli often induce facial nerve stimulation. We aimed to assess whether the results of intraoperative electrically evoked auditory brainstem responses (EABRs) testing predict maximum current levels of CI stimuli (cC levels) optimized by a behavioral-based method after long-term CI use. A retrospective case review. A tertiary referral CI center. A total of 116 ears with malformations (malformation group) and 63 control ears (control group) from patients younger than 18 years who received CI. The malformation group comprised 23 ears with a common cavity (CC), 26 with incomplete partition type 1 (IP-1), 26 with incomplete partition type 2 (IP-2), and 41 with CND. Diagnostic. Correlation between intraoperative EABR results and cC levels determined by the behavioral-based CI programming after long-term CI use. The CC, IP-1, and CND ears required significantly larger cC levels than the IP-2 ears and control groups. However, the cC levels increased to reach the plateau 1 year after surgery in all groups. Among the malformation group, 79 ears underwent intraoperative EABR testing. Greater than 80% of the CC, IP-1, and IP-2 ears and 54.8% of the CND ears exhibited evoked wave V (eV) and were included in the eV-positive category. Myogenic responses but no eV were observed in 18.2, 15.0, and 35.5% of the CC, IP-1, and CND ears, defined as the myogenic category. No eV or myogenic response was elicited in 9.7% of the CND ears. We focused on minimum current levels that elicited eV (eV levels) in the eV-positive category and maximum current levels that did not elicit any myogenic responses (myogenic levels) in the myogenic category. A significant relationship was detected between the eV levels and the cC levels. When analyzed in each malformation type, the eV levels significantly correlate with the cC levels in the CC and CND ears but not in the IP-1 and IP-2 ears, probably because of slight variation within the IP-1 group and the small number of the IP-2 group. The myogenic category did not show a significant relationship between the myogenic levels and cC levels, but the cC levels were similar to or smaller than the myogenic levels in most ears. This study confirmed that intraoperative EABR testing helps predict the optimal cC levels in malformation ears. EABR-based CI programming immediately after cochlear implantation, followed by behavioral-based CI programming, may allow us to achieve early postoperative optimization of CI maps even in young children with severe malformations.

Rationale:Cochlear implantation (CI) in CHARGE syndrome is technically challenging because of the anatomical anomalies. This case aims to report a successful case of CI in CHARGE syndrome by using the modified transcanal approach with external auditory canal (EAC) obliteration.Patient concerns:The 3-year-old girl presented at the outpatient department with bilateral hearing loss and nasal obstruction since birth.Diagnosis:The patient had bilateral profound sensorineural hearing loss, patent ductus arteriosus, atresia of the choanae, middle and inner ear anomalies, and growth retardation, fulfilling the criteria for typical CHARGE syndrome. High resolution temporal bone computed tomography scan revealed a poorly developed mastoid cavity, cochlear dysplasia, hypoplastic semicircular canals, ossicular chain malformation, and sigmoid sinus engorgement. Magnetic resonance imaging revealed a narrow internal auditory canal and a hypoplastic cochlear nerve.Interventions:Modified transcanal approach with external auditory canal obliterationOutcomes:CI was successfully done and there are no intraoperative or postoperative complications occurred after 1 year of follow up.Lessons:The modified transcanal approach is a reasonable and safer option for CI in CHARGE syndrome

A cochlear implant (CI) is a hearing device introduced in the 1980s for profoundly deaf subjects who gained little or no benefit from powerful hearing aids. This device comprises an electrode array inserted in the cochlea, connected to an internal receiver, and an externally worn speech processor. The CI transforms acoustic signals into electrical currents which directly stimulate the auditory nerve. Since the early 1990s, cochlear implantation in children has been developing rapidly. Although it is still difficult to predict how a child will perform with a cochlear implant, the success of cochlear implantation can no longer be denied. In this paper, some recent papers and reports, and the results of the various Nijmegen cochlear implant studies, are reviewed. Issues about selection, examinations, surgery and the outcome are discussed. Overall, our results were comparable with those of other authors. It can be concluded that cochlear implantation is an effective treatment for postlingually deaf as well as prelingually (congenital or acquired) deaf children with profound bilateral sensorineural deafness.

To discuss the method as well as effect of hearing restoration for patients with acoustic neuroma (AN) in the only hearing ear. A retrospective study was conducted in three patients with AN in the only hearing ear. Ipsilateral cochlear implantation (CI) was performed in case 1 and contralateral CI was performed in case 2 without tumor resection. In case 3, CI was performed 6 months after tumor removal by the retrosigmoid approach. Dynamic changes in the audiological tests was recorded regularly. In case 1 and case 2, no matter which side CI was performed, pure tone average (PTA) was improved clearly, and speech discrimination score (SDS) was slightly increased. In case 3, hearing loss was observed after tumor removal. Simultaneous bilateral CI after 12 months was performed in this case. PTA on both sides was largely lower than that before CI, and SDS was slightly improved. With bilateral CI, SDS was improved, especially in noise, compared with unilateral CI on either side. For patients with AN in the only hearing ear, CI can significantly improve PTA, and SDS can be slightly increased. Bilateral CI should improve the outcome of hearing restoration compared with unilateral CI.

Exclusively full texts, cited in PUBMED and MEDLINE search engines, that contain all the selected keywords “Charge syndrome”, “cochlear implant”, “cochlear implantation”, “bionic ear” and “ear malformation” have been selected to review the literature on surgical bionic hearing restoration in CHARGE syndrome. A total of 20 publications that specifically deal with the topic have been published between January 1999 and February 2017. At least ten main different otologic and oto-neurosurgical approaches have been suggested in bionic fitting. The otologic approaches were aided by a microscope or an endoscope or by a combined technique, with or without mastoidectomy. The review is conclude by proposing a strategy for the surgical bionic treatment of hearing in children with CHARGE syndrome.

Currently, there are limited large studies exploring cochlear implantation (CI) outcomes in patients with CHARGE syndrome. We aim to evaluate preoperative and postoperative speech and auditory outcomes in patients with CHARGE syndrome undergoing CI from a review of published single-center studies. PubMed, Scopus, and CINAHL. A total of 15 studies were included for analysis, encompassing 85 CHARGE patients with a total of 99 implanted ears. Extracted data included demographic information, study characteristics, and speech perception and auditory outcomes. Pooled analyses of scaled auditory outcome measures were performed using standardized mean differences (SMDs). Risk of bias was assessed using the Joanna Briggs Critical Appraisal Checklist to evaluate the quality and validity of included studies. This meta-analysis demonstrates significant global postoperative improvement in auditory outcomes for patients with CHARGE syndrome who underwent CI. Meta-analyzed data showed a significant increase in Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores following CI, with an estimated overall pre/post mean difference of 11.9 ( P = 0.036), and a significant increase in categories of auditory performance (CAP) scores following CI, with an estimated overall pre/post mean difference of 2.9 ( P = 0.004). Despite the complexity of inner ear malformations in this population, these findings contribute to the growing body of evidence supporting the use of CIs in CHARGE patients.

To evaluate (1) Audiological and surgical outcomes in patients with otosclerosis following cochlear implantation. (2) surgical difficulties and outcomes between both groups. (3) Audiological outcomes between both groups. Retrospective study conducted at Otology and Skull Base Surgery Center. Data were analyzed from 111 patients with otosclerosis (114 ears) who underwent cochlear implant surgery using the cochlear implant database. Demographic characteristics (age, sex, and operated ear), auditory outcomes, and operative details (extent of cochlear ossification, surgical approach [posterior tympanotomy or subtotal petrosectomy], electrode insertion [partial/complete, scala tympani or vestibuli], and complications) were analyzed Auditory outcomes were assessed over at least one year follow-up period using pure tone audiometry and speech discrimination scores. Patients were divided into two groups (with and without cochlear ossification) to compare auditory outcomes and surgical outcomes. The mean age of patients with ossified and non-ossified cochlea was 60.04 and 62.22years respectively. Sixty-five of 114 ears had cochlear ossification, with complete round window involvement in 75.4% of these patients, while the rest had partial or complete basal turn ossification. Subtotal petrosectomy was performed in 63.1% and 28.6% of ossified and non-ossified cochlea respectively while the rest underwent cochlear implantation through posterior tympanotomy. Only one case had scala vestibuli insertion and four had incomplete electrode insertion. Six patients underwent re-implantation due to infection, device failure, and erosion of the posterior canal wall. Auditory outcomes among patients with ossified otosclerosis were slightly better than those without ossification but this difference was not statistically significant. Cochlear implantation for otosclerosis yields excellent auditory outcomes with a low rate of surgical complications, despite the high incidence of cochlear ossification.

Cochlear hypoplasia is a congenital inner ear malformation (IEM) characterized by a reduced external cochlear dimension, usually accompanied by an abnormal internal architecture. Type IV cochlear hypoplasia is a cochlea with hypoplastic middle and apical turns. It may occur along with dislocation of the facial nerve, associated with semicircular abnormalities, less clearly marked promontory, or stapedial fixation. Such patients can present a broad spectrum of audiological test results, from sensorineural or mixed mild or profound hearing loss. The above anatomical changes may be responsible for intraoperative difficulties during cochlear implantation. In the studied case, a 6-month-old patient was diagnosed with an inner ear malformation - cochlear hypoplasia type IV on both sides. Computed tomography with multiplanar and 3D reconstruction was performed to analyze the middle and inner ear anatomy in detail. Both types of imaging reconstruction helped decide which cochlear implant electrode to choose. Perimodiolar-positioned cochlear implant electrode was found to be the most suitable choice. The patient underwent sequential bilateral cochlear implantation with expected incomplete electrode array insertion on both sides. First repeatable auditory responses were observed two months after the second implant activation. Good parental cooperation with therapists and adequately defined developmental goals in the presented patient allowed the multidisciplinary team to take advantage of the child's intellectual abilities and choose the suitable communication method; however, the patient's auditory responses were obtained slowly. The final auditory results cannot be predicted in inner ear malformations due to abnormal anatomical structure and, thus, heterogeneous innervation within the deformed cochlea. The programming of the sound processor must be individual in each case, based on the child's behavior observation and, if possible, objective test results. Patients with cochlear malformations usually require higher stimulation intensities to obtain sound sensations than patients with a typical cochlear structure.

Background: Congenital inner ear malformations (IEM) pose a challenge during cochlear implantation (CI) in terms of increased risk of surgical complications and uncertainty of outcomes. This case series presents the surgical complications encountered and outcomes of CI in those children with inner ear anomalies. Aims: (1) To examine the intraoperative findings and surgical complications in children with inner ear abnormalities undergoing CI and (2) to examine the reported speech perception outcomes in these children, postoperatively using the categories of auditory performance (CAP) scores, and speech intelligibility rating. Settings and Design: A retrospective study was conducted over a period of 2 years at a tertiary care center, involving three patients with IEMs who underwent CI. Subjects and Methods: Intraoperative complications encountered and their management alongside evaluation of surgical outcomes were analyzed. Results: Two children had incomplete partition type II, and one child was found to have a common cavity according to Sennaroglu classification. Intraoperative problems, such as facial nerve palsy and cerebrospinal fluid gusher, were few of the main difficulties faced. Nonetheless, following postoperative rehabilitation, all IEM patients demonstrated a notable increase in their verbal and auditory abilities. Conclusions: Children with IEMs may benefit from auditory rehabilitation with cochlear implants. A multidisciplinary approach aiding in definitive diagnosis, surgical planning, and postoperative rehabilitation is recommended for positive outcomes.

Progression of hearing loss and cochlear implantation in large vestibular aqueduct syndrome

A retrofacial approach of round window vibroplasty during Vibrant Soundbridge implantation for patients with congenital ear malformation

Compare outcomes among children with inner ear malformations and/or cochlear nerve deficiency (CND) who have received a cochlear implant (CI). Individual retrospective cohort study from 1993 to 2010. A select cohort of 76 children was identified. Imaging characteristics, operative findings, complications, mapping parameters, and performance were assessed. Comparisons among the different groups were undertaken. Surgery was mostly uncomplicated. Nearly all children demonstrated behavioral responses to CI stimulation irrespective of inner ear morphology or the presence of CND. Children with CND had higher pure tone averages (PTAs) and required greater charge for stimulation than other malformation types. Open-set speech perception was achieved in 100% of children with incomplete partition-enlarged vestibular aqueduct (IP-EVA), 50% of those with hypoplastic malformations, and 19% of CND cases. Robust responses on eighth nerve compound action potential (ECAP) testing through the implant was associated with higher levels of speech perception. Manually supplemented communication strategies were more common among children with hypoplastic malformations (69%) and CND (95%) than those with IP-EVA (18%). Children with IP-EVA malformations have an excellent prognosis for developing open-set speech perception and using oral communication modes following CI. On the contrary, children with severe malformations or CND may have elevated charge requirements for attaining sound detection alone. These children's prognosis for obtaining open-set speech understanding, using exclusive oral communication, and participating in mainstream education is more limited. These findings have important implications for considering alternative forms of intervention such as auditory brainstem implantation and/or supplementation with visually based communication strategies.

Cyclooxygenases (COXs) have important functions in various physiological and pathological processes. COX-2 expression is highly induced by a variety of stimuli and is observed during certain periods of embryonic development. In this report, the direct effect of COX-2 expression on embryonic development is examined in a novel COX-2 transgenic mouse model that ubiquitously expresses human COX-2 from the early stages of embryonic development. COX-2 transgenic fetuses exhibit severe skeletal malformations and die shortly after birth. Skeletal malformations are localized along the entire vertebral column and rib cage and are linked to defective formation of cartilage anlagen. The cartilage anlagen of axial skeleton fail to properly develop in transgenic embryos because of impaired precartilaginous sclerotomal condensation, which results from the reduction of cell number in the sclerotome. Despite the ubiquitous expression of COX-2, the number of apoptotic cells is highly increased in the sclerotome of transgenic embryos but not in other tissues, suggesting that it is a tissue-specific response. Therefore, the loss of sclerotomal cells due to an increased apoptosis is probably responsible for axial skeletal malformations in transgenic fetuses. In addition, the sclerotomal accumulation of p53 protein is observed in transgenic embryos, suggesting that COX-2 may induce apoptosis via the up-regulation of p53. Our results demonstrate that the aberrant COX-2 signaling during embryonic development is teratogenic and suggest a possible association of COX-2 with fetal malformations of unknown etiology.