RESUMEN
BACKGROUND: NF2-schwannomatosis (NF2) is an autosomal dominant disorder prone to hearing loss. Auditory brainstem implants (ABIs) offer a promising solution for hearing rehabilitation in NF2. OBJECTIVE: To synthesize existing literature on ABI implantation in NF2, focusing on audiological outcomes and ABI-related complications. METHODS: The systematic review followed PRISMA guidelines and was registered in the PROSPERO database (CRD42022362155). Relevant studies were identified by searching PubMed, EMBASE, CENTRAL, CMB, and CNKI from inception to August 2023. Data on environmental sound discrimination, open-set discrimination, closed-set discrimination, and ABI-related complications were extracted and subjected to meta-analysis. Publication bias was evaluated using funnel plots and Egger's test. RESULTS: Thirty-three studies were included. The pooled estimate was 58% (95% CI 49-66%) for environmental sound discrimination and 55% (95% CI 40-69%) for closed-set discrimination. Regarding open-set discrimination, the pooled estimates were 30% (95% CI 19-42%) for sound only, 46% (95% CI 37-54%) for lip-reading only, and 63% (95% CI 55-70%) for sound plus lip-reading. The pooled occurrence of ABI-related complications was 33% (95% CI 15-52%). CONCLUSION: This meta-analysis underscores the effectiveness and safety of ABIs in NF2, providing valuable insights for evidence-based decision-making and hearing rehabilitation strategies.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurilemoma , Neurofibromatosis , Neurofibromatosis 2 , Neoplasias Cutáneas , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Resultado del Tratamiento , Audición , Estudios RetrospectivosRESUMEN
OBJECTIVE: Pediatric data regarding treatment via an auditory brainstem implant (ABI) remains sparse. The authors aimed to describe their experience at their institution and to delineate associated demographic data, audiometric outcomes, and surgical parameters. METHODS: An IRB-approved, retrospective chart review was conducted among the authors' pediatric patients who had undergone auditory brainstem implantation between 2012 and 2021. Demographic information including sex, age, race, coexisting syndrome(s), history of cochlear implant placement, average duration of implant use, and follow-up outcomes were collected. Surgical parameters collected included approach, intraoperative findings, number of electrodes activated, and complications. RESULTS: A total of 19 pediatric patients had an ABI placed at the authors' institution, with a mean age at surgery of 4.7 years (range 1.5-17.8 years). A total of 17 patients (89.5%) had bilateral cochlear nerve aplasia/dysplasia, 1 (5.3%) had unilateral cochlear nerve aplasia/dysplasia, and 1 (5.3%) had a hypoplastic cochlea with ossification. A total of 11 patients (57.9%) had a history of cochlear implants that were ineffective and required removal. The mean length of implant use was 5.31 years (0.25-10 years). Two patients (10.5%) experienced CSF-related complications requiring further surgical intervention. The most recent audiometric outcomes demonstrated that 15 patients (78.9%) showed improvement in their hearing ability: 5 with sound/speech awareness, 5 able to discriminate among speech and environmental sounds, and 5 able to understand common phrases/conversation without lip reading. Nine patients (47.4%) are in a school for the deaf and 7 (36.8%) are in a mainstream school with support. CONCLUSIONS: The authors' surgical experience with a multidisciplinary team demonstrates that the retrosigmoid approach for ABI placement in children with inner ear pathologies and severe sensorineural hearing loss is a safe and effective treatment modality. Audiometric outcome data showed that nearly 79% of these patients had an improvement in their environmental and speech awareness. Further multicenter collaborations are necessary to improve these outcomes and potentially standardize/enhance electrode placement.
Asunto(s)
Audiometría , Implantación Auditiva en el Tronco Encefálico , Humanos , Niño , Masculino , Femenino , Preescolar , Adolescente , Estudios Retrospectivos , Lactante , Implantación Auditiva en el Tronco Encefálico/métodos , Resultado del Tratamiento , Implantes Auditivos de Tronco Encefálico , Nervio Coclear/cirugía , Nervio Coclear/anomalías , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiologíaRESUMEN
OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. INTERVENTIONS: CI or ABI ipsilateral to vestibular schwannoma. MAIN OUTCOME MEASURES: Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. RESULTS: Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. CONCLUSION: Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.
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Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Implantación Coclear , Implantes Cocleares , Neurofibromatosis 2 , Neuroma Acústico , Percepción del Habla , Humanos , Neuroma Acústico/complicaciones , Neuroma Acústico/cirugía , Neuroma Acústico/patología , Implantes Cocleares/efectos adversos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Neurofibromatosis 2/patología , Resultado del Tratamiento , Estudios RetrospectivosRESUMEN
PURPOSE: Large individual differences and poor speech recognition outcomes are routinely observed in most patients who have received auditory brainstem implants (ABIs). A case report of an ABI recipient with exceptionally good speech recognition outcomes presents an opportunity to better understand the core information processing mechanisms that underlie variability and individual differences in outcomes. METHOD: A case study is reported of an adult ABI recipient (ID-006) with postlingually acquired, Neurofibromatosis Type 2 (NF2)-related hearing loss who displayed exceptional postoperative speech recognition scores. A novel battery of assessment measures was used to evaluate ID-006's auditory, cognitive, and linguistic information processing skills. RESULTS: Seventeen years following ABI activation, ID-006 scored 77.6% correct on the AzBio Sentences in quiet. On auditory processing tasks, ID-006 scored higher on tasks with meaningful sentences and much lower on tasks that relied exclusively on audibility. ID-006 also demonstrated exceptionally strong abilities on several cognitive and linguistic information processing tasks. CONCLUSIONS: Results from a novel battery of information processing tests suggest that ID-006 relies extensively on top-down predictive processing and cognitive control strategies to efficiently encode and process auditory information provided by his ABI. Results suggest that current measures of outcomes and benefits should be expanded beyond conventional speech recognition measures to include more sensitive and robust measures of speech recognition as well as neurocognitive measures such as executive function, working memory, and lexical access.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Pérdida Auditiva , Neurofibromatosis 2 , Percepción del Habla , Adulto , Humanos , Implantación Auditiva en el Tronco Encefálico/efectos adversos , Implantación Auditiva en el Tronco Encefálico/métodos , Habla , Percepción del Habla/fisiología , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Pérdida Auditiva/etiologíaRESUMEN
OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatosis 2 , Neuroma Acústico , Adulto , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Neuroma Acústico/complicaciones , Neuroma Acústico/cirugía , Estudios Retrospectivos , Quebec , Implantación Auditiva en el Tronco Encefálico/métodos , Resultado del Tratamiento , AudiciónRESUMEN
PURPOSE: Intraword variability designates the phenomenon that a particular target word is produced variably by a child at one point in the child's development. In this study, the amount of intraword variability is studied longitudinally in children with auditory brainstem implants (ABIs). Auditory brainstem implantation is a relative recent technique in pediatric hearing restoration. Therefore, little is known about the phonological development of these children's speech. METHOD: The intraword variability is investigated in three children with ABI, in comparison to children with cochlear implants, matched on lexical development. Intraword variability is measured using relative entropy in order to take into account the frequency distribution in children's productions. RESULT: Results showed considerable variation between the three children with ABI. Still, all children had higher levels of intraword variability in their spontaneous speech productions as compared to children with cochlear implants. CONCLUSION: It seems that children with ABI are lagging behind their phonological development in reference to children with cochlear implants.
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Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Implantación Coclear , Implantes Cocleares , Sordera , Percepción del Habla , Implantación Auditiva en el Tronco Encefálico/métodos , Niño , Sordera/cirugía , Humanos , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate the safety of 3 Tesla (T) magnetic resonance imaging (MRI) in patients with auditory brainstem implants (ABI) with the magnet removed at implantation and report incidence of complications. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary neurotology ambulatory practice. PATIENTS: Patients with diagnosis of Neurofibromatosis, type 2 (NF2) with functional ABIs. INTERVENTIONS: Observational recordings. MAIN OUTCOME MEASURES: Of the 89 patients meeting inclusion criteria, 7 patients underwent 3T MRI, with a total of 39 scans done. Three patients had 1 scan each, one patient had 4 scans, one patient had 5 scans, one patient had 6 scans, and one patient had 21 scans. The mean time between ABI placement and first 3âT scan was 118â±â73âmonths. The most common indication for imaging was surveillance of NF2 lesions. The most frequent scans were MRI brain (25.6%), followed by MRI of cervical (15%), thoracic (15%) and lumbar (15%) spine, and MRI IAC (8%). There were no reported complications for any of the scans. No scans were interrupted due to patient discomfort. There were no device malfunctions. CONCLUSIONS: 3âT MRIs are safe in patients with ABIs as long as the magnet is removed. It is recommended that the magnet be removed at the time of implantation in all NF2 patients, who require frequent surveillance.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatosis 2 , Implantación Auditiva en el Tronco Encefálico/efectos adversos , Implantación Auditiva en el Tronco Encefálico/métodos , Humanos , Imagen por Resonancia Magnética/efectos adversos , Imanes , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/diagnóstico por imagen , Neurofibromatosis 2/patología , Estudios RetrospectivosRESUMEN
PURPOSE: An auditory brainstem implant (ABI) represents an alternative for patients with profound hearing loss who are constrained from receiving a cochlear implant. The positioning of the ABI electrode influences the patient's auditory capacity and, therefore, quality of life and is challenging even with available intraoperative electrophysiological monitoring. This work aims to provide and assess the feasibility of visual-spatial assistance for ABI positioning. METHODS: The pose of the forceps instrument that grasps the electrode was electromagnetically navigated and interactively projected in the eyepieces of a surgical microscope with respect to a target point. Intraoperative navigation was established with an experimental technique for automated nasopharyngeal patient registration. Two ABI procedures were completed in a human specimen head. RESULTS: An intraoperative usability study demonstrated lower localization error when using the proposed visual display versus standard cross-sectional views. The postoperative evaluations of the preclinical study showed that the center of the electrode was misplaced to the planned position by 1.58 mm and 3.16 mm for the left and the right ear procedure, respectively. CONCLUSION: The results indicate the potential to enhance intraoperative feedback during ABI positioning with the presented system. Further improvements consider estimating the pose of the electrode itself to allow for better orientation during placement.
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Implantación Auditiva en el Tronco Encefálico , Estudios Transversales , Estudios de Factibilidad , Humanos , Calidad de Vida , Resultado del TratamientoRESUMEN
BACKGROUND: Cochlear nerve deficiency is one of the known causes of congenital sensorineural hearing loss. Management of hearing loss in children with cochlear nerve deficiency poses a multidimensional challenge. The absent or hypoplastic cochlear nerve may prevent electrical stimulation from reaching the brainstem and the auditory cortex. A deficient cochlear nerve can be associated with other inner ear malformations, which may diminish the success of cochlear implantation in those children. Promising results in adults after auditory brainstem implantation led to the expansion of candidacy to include the pediatric populations who were contraindicated for CIs. OBJECTIVE: To review the outcomes of cochlear implantation versus that of auditory brainstem implantation in children with various conditions of the auditory nerve. METHODS: This retrospective chart review study comprised two pediatric groups. The first group consisted of seven ABI recipients with cochlear nerve aplasia and the second group consisted of another seven children with cochlear nerve deficiencies who underwent CI surgery. The participants' auditory skills and speech outcomes were assessed using different tests selected from the Evaluation of Auditory Responses to Speech (EARS) test battery. RESULTS: There were some individual variations in outcomes depending on the status of the auditory nerve. The mean CAP score of the ABI group was 2.87, while the mean SIR score was 0.62. On the other hand, the mean CAP score of the CI group was 1.29, while the mean SIR score was 0.42. CONCLUSION: Our results are in good agreement with the reported auditory perception and speech and language development outcomes of pediatric auditory brainstem implantation. We added to the growing body of literature on the importance of verifying and identifying the status of the cochlear nerve in the decision-making process of the surgical management of those pediatric groups.
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Implantación Auditiva en el Tronco Encefálico , Implantación Coclear , Implantes Cocleares , Percepción del Habla , Adulto , Niño , Nervio Coclear/anomalías , Nervio Coclear/cirugía , Humanos , Estudios Retrospectivos , Percepción del Habla/fisiología , Resultado del TratamientoRESUMEN
RESUMO Objetivo mapear as evidências atuais em relação à percepção auditiva da fala e desenvolvimento de linguagem oral em usuários de implante auditivo de tronco encefálico (auditory brainstem implant - ABI), para responder à seguinte questão norteadora: "O que se sabe sobre a habilidade de percepção auditiva da fala e de linguagem oral em indivíduos usuários de implante auditivo de tronco encefálico?" Estratégia de pesquisa a busca foi realizada nas bases de dados BVSalud, PubMed e SciELO e, para literatura cinzenta, utilizou-se a fonte de informação Google Acadêmico, por meio dos descritores: implante auditivo de tronco encefálico (auditory brainstem implantation), linguagem (language), audição (hearing) e percepção auditiva (auditory perception). Critérios de seleção foram incluídos estudos nos quais foram aplicados testes para avalição da percepção auditiva ou para verificar desenvolvimento de linguagem oral em crianças e/ou adultos usuários de ABI. Foram incluídos artigos publicados nos últimos cinco anos e excluídos estudos secundários. Resultados Foram encontrados 1767 artigos nas bases de dados e fonte de informação, dos quais, 27 foram incluídos na revisão. Observou-se que a maioria dos usuários de ABI torna-se capaz de perceber alguns sons ambientais, alguns tornam-se capazes de reconhecer vocábulos, porém, poucos atingem o reconhecimento de frases. Conclusão a maioria dos usuários de ABI não avança para a habilidade de reconhecimento auditivo em conjunto aberto e há unanimidade na recomendação de métodos de comunicação visual para esses indivíduos.
ABSTRACT Purpose This scope review aims to map current evidence in relation to auditory perception of speech and oral language development in users of Auditory Brainstem Implant - ABI, to answer the following guiding question: "what do we know about the ability of auditory perception of speech and oral language in auditory brainstem implants users?" Research strategy The search was performed in the BVSalud, PubMed and SciELO databases and for gray literature the source of information Google Academic, using the descriptors: auditory brainstem implantation , language, hearing and auditory perception. Selection criteria Studies were included in which tests were applied to assess auditory perception or to verify oral language development in children and/or adults using ABI. Articles published in the last five years were included and secondary studies were excluded. Results 1767 articles were found in the databases and source of information, of which 27 studies were included. It was observed that most users of ABI become able to perceive some environmental sounds, some become able to recognize words, but few reach the recognition of sentences. Conclusion Most ABI users do not advance towards the open set auditory recognition skill and there is unanimity in recommending visual communication methods for these individuals.
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Humanos , Niño , Adulto , Pronóstico , Percepción Auditiva , Neurofibromatosis 2 , Implantación Auditiva en el Tronco Encefálico , Pérdida Auditiva/rehabilitación , Desarrollo del LenguajeRESUMEN
OBJECTIVES: Factors contributing to auditory brainstem implant (ABI) outcomes are poorly understood. The aims of this study are to (1) characterize ABI electrode array position on postoperative imaging and (2) determine if variability in position is related to perceptual outcomes. DESIGN: Retrospective cohort study. Subjects were selected from the adult ABI recipient population at Massachusetts Eye and Ear. Postoperative three-dimensional (3D) computed tomography (CT) reconstruction of the head was used to measure ABI array position in 20 adult ABI recipients (17 with Neurofibromatosis Type 2 (NF2) and three non-NF2 recipients). Three-dimensional electrode array position was determined based on angles from the horizontal using posterior and lateral views and on distances between the proximal array tip superiorly from the basion (D1), laterally (D2P) and posteriorly (D2L) from the midline. Array position was correlated with perceptual data (in 15 of the 20 recipients who used their ABI). Perceptual data included the number of electrodes that provided auditory sensation, location and type of side effects, level of speech perception (from no sound to open-set word recognition of monosyllables) and the amount of charge required for auditory perception. RESULTS: Although the 3D orientation of the ABI array exhibited a variety of angles, all arrays were posteriorly tilted from the lateral view and most were medially tilted from the posterior view. ABI position relative to the basion from posterior showed mean distances of 1.71 ± 0.42 and 1.1 ± 0.29 cm for D1 and D2, respectively, and a mean D2 of 1.30 ± 0.45 cm from the lateral view. A strong linear negative correlation was found between the number of active electrodes and the distance of the proximal array tip laterally from the basion (D2P; rs = -0.73, p = 0.006) when measured in the posterior view. Although side effects were experienced in all recipients and varied in type and location across the array, electrodes in the middle part of the array tended to elicit auditory sensations while the proximal and distal tips of the array tended to elicit nonauditory side effects. Arrays with and without low charge thresholds appeared to generally overlap in position. However, the two recipients with the best (open-set) speech perception had low charge thresholds and had arrays that were tilted superiorly in the posterior view. CONCLUSION: ABI recipients with better speech perception appear to share a profile of arrays that are tilted superiorly as compared to recipients with lower speech perception levels. These ABI recipients have a high number of active electrodes (10 or more) and require less electrical charge on individual electrodes to achieve optimal stimulation.
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Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatosis 2 , Percepción del Habla , Adulto , Implantación Auditiva en el Tronco Encefálico/métodos , Electrodos , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/diagnóstico por imagen , Neurofibromatosis 2/cirugía , Estudios Retrospectivos , Percepción del Habla/fisiología , Tomografía Computarizada por Rayos XRESUMEN
The Auditory Brainstem Implant (ABI) is based on the classic cochlear implant (CI) but uses a different stimulation electrode. At MED-EL, the early development activities on ABI started in the year 1994, with the suggestion coming from J. Helms and J. Müller from Würzburg, Germany in collaboration with the Univ. of Innsbruck Austria. The first ABI surgery in a neuro-fibromatosis (NF2) patient with the MED-EL device took place in the year 1997. Later, the indication of ABI was expanded to non-NF2 patients with severe inner-ear malformation, for whom a regular CI will not be beneficial. Key translational research activities at MED-EL in collaboration with numerous clinics investigating the factors that affect the hearing performance amongst ABI patients, importance of early ABI implantation in children, tools in pre-operative assessment of ABI candidates and new concepts that were pursued with the MED-EL ABI device. The CE-mark for the MED-EL ABI to be used in adults and children down to the age of 12 months without NF-2 was granted in 2017 mainly based on two long-term clinical studies in the pediatric population. This article covers the milestones of translational research from the first concept to the widespread clinical use of ABI in association with MED-EL.
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Implantación Auditiva en el Tronco Encefálico/tendencias , Implantes Auditivos de Tronco Encefálico/tendencias , Implantación Auditiva en el Tronco Encefálico/historia , Implantes Auditivos de Tronco Encefálico/historia , Neoplasias de los Nervios Craneales/cirugía , Sordera/cirugía , Historia del Siglo XX , Historia del Siglo XXI , Humanos , Neurofibromatosis/cirugía , Neuroma Acústico/cirugíaRESUMEN
OBJECTIVE: To determine if cochlear implantation without removal of inner ear schwannomas (IES) is an effective treatment option for Neurofibromatosis 2 (NF2) patients. To determine how the presence of an intracochlear schwannoma might impact cochlear implant (CI) outcomes and programming parameters. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary center for cochlear and auditory brainstem implantation. PATIENTS: Of 10 NF2 patients with IES, 8 are reported with no previous tumor removal on the implanted ear. INTERVENTIONS: Cochlear implant without tumor removal. MAIN OUTCOME MEASURES: Performance outcomes with CI at least 1-year post intervention. Programming parameters, including impedances, for patients with IES. RESULTS: All patients had full insertion of the electrode arrays with round window approaches. Performance outcomes ranged from 0 to 100% for Bamford-Kowal-Bench sentences. Impedance measurements for active electrodes for patients with IES were comparable to those measured in patients without vestibular schwannoma (VS). Only patients who had radiation treatment before receiving their implant had elevated threshold requirements for CI programming compared with CI recipients without VS. CONCLUSION: Cochlear implantation without tumor removal is an effective option for treating NF2 patients with IES. The presence of an intracochlear tumor did not have an impact on CI performance or programming requirements compared with patients without VS; however, previous treatment with radiation may be related to elevated current requirements in the CI settings.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantación Coclear , Implantes Cocleares , Neurofibromatosis 2 , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Estudios RetrospectivosRESUMEN
Auditory brainstem implantation is a hearing restoration technique on the central auditory pathway, which directly stimulates neural tissues of cochlear nucleus by electrode array to produce hearing, so it will not be restricted by abnormalities of the cochlea and cochlear nerve. In the past 30 years since the birth of multi-channel ABI, its indications have expanded from patients with neurofibromatosis type 2 (NF2) to congenital deafness patients with severe inner ear and/or cochlear nerve malformations, and the age of recipients decreases from adults to young children. This article summarizes the principle, indications, surgical techniques, complications and auditory outcomes of ABI.
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Implantación Auditiva en el Tronco Encefálico , Implantación Coclear , Sordera , Neurofibromatosis 2 , Adulto , Niño , Preescolar , Sordera/cirugía , Pruebas Auditivas , Humanos , Neurofibromatosis 2/cirugía , Resultado del TratamientoRESUMEN
In this review, we discuss the auditory brainstem implant (ABI), which is a neuroprosthetic device being an advanced hearing aid in cases of bilateral, profound or complete hearing impairment due to a non-functional or absent cochlear nerve, or an inner ear malformation precluding cochlear implantation. Originally indicated in patients with bilateral vestibular schwannomas caused by neurofibromatosis type 2, the ABI has in recent years seen an increase in the aural rehabilitation of congenitally deaf children. Outcomes for patients are mixed, but generally the device leads to a reasonable improvement in speech perception.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Implantación Coclear , Neurofibromatosis 2 , Niño , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Resultado del TratamientoRESUMEN
OBJECTIVE: To discuss indications for bilateral auditory brainstem implants (ABIs), compare audiometric outcomes of unilateral vs bilateral ABIs, and determine if patients have improved outcomes with addition of a second-side implant. STUDY DESIGN: Retrospective review of 24 patients with neurofibromatosis 2 (NF2) who underwent sequential placement of ABIs from 1989 to 2019. SETTING: Tertiary referral center. METHODS: Charts were reviewed for indication for second-side surgery, use of implants, and audiometric outcomes. Implants placed in the past 30 years were included in the study. Northwestern University Children's Perception of Speech (NU-CHIPS) and/or City University of New York (CUNY) sentence scores were compared in unilateral and bilateral conditions. RESULTS: Indications for a second-side implant included first-side implants with severe nonauditory symptoms (11), marginal audiometric results (9), outdated technology (2), or deterioration of first side (2). Seven patients are bilateral users and 1 patient discontinued bilateral use after a year due to no significant improvement over unilateral use. One patient with initial bilateral use was lost to follow-up. Thirteen patients are unilateral users due to nonaudiometric side effects or poor audiometric outcomes with the first side. Two patients are complete nonusers. Seventy-five percent had improved audiometric outcomes after the second-side implant, and 20% had stable findings. CONCLUSIONS: Second-side ABIs should be consider in patients with poor performance from a first-side implant. Most patients demonstrate subjective improvement with the second ABI. More research is needed for better objective assessments of improvements.
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Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Trastornos de la Audición/terapia , Neurofibromatosis 2/complicaciones , Adolescente , Audiometría , Niño , Preescolar , Femenino , Trastornos de la Audición/diagnóstico , Trastornos de la Audición/etiología , Humanos , Masculino , Neurofibromatosis 2/diagnóstico , Neurofibromatosis 2/terapia , Selección de Paciente , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
An Auditory Brainstem Implant (ABI) is a technique developed for patients with severe hearing loss. The ABI consists of a microphone and speech processor located on the scalp, which is connected to a transmitting and receiving coil and electrode on the brain stem placed in the skull. Eligible for an ABI are adults with cochlea and acoustic nerve damage due to neurofibromatosis type 2, and children with congenital malformation or aplasia, cochlear trauma or cochlear ossification after meningitis. An ABI can provide useful hearing. It has proven to be a safe procedure without serious complications. The entire ABI process is handled by a multidisciplinary team with extensive experience in cerebellopontine angle tumour surgery and cochlear implantation in adults and children. Concentration of this care in a specialized centre is important to maximize the chances of a successful outcome.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Pérdida Auditiva/cirugía , Adulto , Niño , Femenino , Pérdida Auditiva/etiología , Humanos , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVE: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label. MATERIAL AND METHODS: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded. RESULTS: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients. CONCLUSION: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid.
Asunto(s)
Implantación Auditiva en el Tronco Encefálico/estadística & datos numéricos , Implantación Coclear/estadística & datos numéricos , Implantes Cocleares/estadística & datos numéricos , Pérdida Auditiva/rehabilitación , Selección de Paciente , Sistema de Registros/estadística & datos numéricos , Adolescente , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Audiometría del Habla/métodos , Niño , Preescolar , Implantación Coclear/efectos adversos , Implantes Cocleares/efectos adversos , Femenino , Francia/epidemiología , Encuestas Epidemiológicas , Humanos , Masculino , Persona de Mediana Edad , Uso Fuera de lo Indicado/estadística & datos numéricos , Complicaciones Posoperatorias/epidemiología , Periodo Posoperatorio , Cuidados Preoperatorios , Estudios Prospectivos , Calidad de Vida , Adulto JovenRESUMEN
BACKGROUND: Neurofibromatosis 2 (NF2) is a rare genetic disorder that causes vestibular schwannomas to develop in both eighth cranial nerves. Almost all people with NF2 eventually become completely deaf as a result of progressive tumour enlargement or following surgical or radiotherapy treatment. Other rare abnormal conditions in the inner ears can also cause complete deafness. For people with either indication who are not candidates for cochlear implantation, auditory brainstem implantation is the only treatment option to restore some functional hearing. We conducted a health technology assessment of auditory brainstem implantation for adults with NF2 and severe inner ear abnormalities, which included an evaluation of effectiveness, safety, cost-effectiveness, the budget impact of publicly funding auditory brainstem implantation, and patient preferences and values. METHODS: We performed a systematic literature search of the clinical evidence. We assessed the risk of bias of each included study using the Risk of Bias in Non-randomized Studies-of Interventions (ROBINS-I) tool and the quality of the body of evidence according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) Working Group criteria. We performed a systematic economic literature search. We did not conduct a primary economic evaluation because the outcomes identified in our clinical evidence review were difficult to translate into measures appropriate for health economic modelling. We also analyzed the net budget impact of publicly funding auditory brainstem implantation over the next 5 years in Ontario, including the device, presurgical assessment, surgical procedure, and postsurgical rehabilitation. To contextualize the potential value of auditory brainstem implants, we spoke with six people with lived experience of NF2 and severe inner ear abnormalities. RESULTS: We included 22 publications (16 in NF2, five in severe inner ear abnormalities, and one in complications of auditory brainstem implantation) in the clinical evidence review. In adults with NF2, auditory brainstem implantation when compared with no intervention allows any degree of improvement in sound recognition (GRADE: High), allows any degree of improvement in speech perception when used in conjunction with lip-reading (GRADE: High), and provides subjective benefits of hearing (GRADE: High). It likely allows any degree of improvement in speech perception when using the implant alone (GRADE: Moderate) and may improve quality of life (GRADE: Low). In adults with severe inner ear abnormalities, auditory brainstem implantation when compared with no intervention likely allows any degree of improvement in sound recognition (GRADE: Moderate) and in any speech perception when using the implant alone (GRADE: Moderate). It may allow any degree of improvement in speech perception when used in conjunction with lip-reading (GRADE: Low), provide subjective benefits of hearing (GRADE: Low), and improve quality of life (GRADE: Low).We did not identify any economic studies on auditory brainstem implantation for adults with NF2 or adults with deafness due to severe inner ear abnormalities. We estimated that the annual net budget impact of publicly funding auditory brainstem implantation in Ontario over the next 5 years would range from about $130,000 in year 1 for two procedures to about $260,000 in year 5 for four procedures.People with whom we spoke who had received an auditory brainstem implant reported that it restored some hearing ability and improved their quality of life, though they also reported ongoing challenges in using the device or side effects from the procedure. CONCLUSIONS: When compared with no intervention, auditory brainstem implantation provides some benefit for completely deaf adults with NF2 or severe inner ear abnormalities who are not candidates for cochlear implantation. Based on evidence of moderate to high quality, auditory brainstem implants allow any degree of improvement in sound recognition and in speech perception when used in conjunction with lip-reading for people with NF2. The quality of evidence on these outcomes was low to moderate for people with severe inner ear abnormalities. These functional outcomes lead to subjective benefits of hearing which are consistently reported in the literature and in interviews with patients. We were unable to determine the cost-effectiveness of this treatment. We estimate that publicly funding auditory brainstem implantation in Ontario would result in additional costs of about $130,000 to $260,000 annually over the next 5 years.