RESUMO
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.
Assuntos
Audiometria , Implante Auditivo de Tronco Encefálico , Humanos , Criança , Masculino , Feminino , Pré-Escolar , Adolescente , Estudos Retrospectivos , Lactente , Implante Auditivo de Tronco Encefálico/métodos , Resultado do Tratamento , Implantes Auditivos de Tronco Encefálico , Nervo Coclear/cirurgia , Nervo Coclear/anormalidades , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologiaRESUMO
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.
Assuntos
Implante Auditivo de Tronco Encefálico , Perda Auditiva , Neurofibromatose 2 , Percepção da Fala , Adulto , Humanos , Implante Auditivo de Tronco Encefálico/efeitos adversos , Implante Auditivo de Tronco Encefálico/métodos , Fala , Percepção da Fala/fisiologia , Neurofibromatose 2/complicações , Neurofibromatose 2/cirurgia , Perda Auditiva/etiologiaRESUMO
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.
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Implante Auditivo de Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatose 2 , Neuroma Acústico , Adulto , Humanos , Neurofibromatose 2/complicações , Neurofibromatose 2/cirurgia , Neuroma Acústico/complicações , Neuroma Acústico/cirurgia , Estudos Retrospectivos , Quebeque , Implante Auditivo de Tronco Encefálico/métodos , Resultado do Tratamento , AudiçãoRESUMO
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.
Assuntos
Implante Auditivo de Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Implante Coclear , Implantes Cocleares , Surdez , Percepção da Fala , Implante Auditivo de Tronco Encefálico/métodos , Criança , Surdez/cirurgia , Humanos , Resultado do TratamentoRESUMO
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.
Assuntos
Implante Auditivo de Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatose 2 , Implante Auditivo de Tronco Encefálico/efeitos adversos , Implante Auditivo de Tronco Encefálico/métodos , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Imãs , Neurofibromatose 2/complicações , Neurofibromatose 2/diagnóstico por imagem , Neurofibromatose 2/patologia , Estudos RetrospectivosRESUMO
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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Implante Auditivo de Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Neurofibromatose 2 , Percepção da Fala , Adulto , Implante Auditivo de Tronco Encefálico/métodos , Eletrodos , Humanos , Neurofibromatose 2/complicações , Neurofibromatose 2/diagnóstico por imagem , Neurofibromatose 2/cirurgia , Estudos Retrospectivos , Percepção da Fala/fisiologia , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Surdez/cirurgia , Orelha Interna/anormalidades , Neurofibromatose 2/complicações , Avaliação da Tecnologia Biomédica/métodos , Adulto , Surdez/etiologia , Orelha Interna/cirurgia , Humanos , Neurofibromatose 2/cirurgia , Ontário , Resultado do TratamentoRESUMO
OBJECTIVE: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility. STUDY DESIGN: Retrospective single cohort design. SETTING: Tertiary referral center. PATIENTS: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [nâ=â15], Late Group: ≥ 3 yr old [nâ=â15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (rangedâ±â6.5) months in the early group, 45.53 (rangedâ±â7.9) months in the late group. INTERVENTION(S): Retrosigmoid craniotomy and ABI placement. MAIN OUTCOME MEASURE(S): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room. RESULTS: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (Uâ=â12, pâ<â0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps. CONCLUSIONS: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Desenvolvimento da Linguagem , Inteligibilidade da Fala , Percepção da Fala , Resultado do Tratamento , Fatores Etários , Implantes Auditivos de Tronco Encefálico , Criança , Pré-Escolar , Estudos de Coortes , Surdez/cirurgia , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
Auditory brainstem implants (ABIs) stimulate the auditory system at the cochlear nucleus, bypassing the peripheral auditory system including the auditory nerve. They are used in patients who are not cochlear implant candidates. Current criteria for use in the United States are neurofibromatosis type 2 patients 12 years or older undergoing first- or second-side vestibular schwannoma removal. However, there are other nontumor conditions in which patients may benefit from an ABI, such as bilateral cochlear nerve aplasia and severe cochlear malformation not amendable to cochlear implantation. Recent experience with ABI in the pediatric population demonstrates good safety profile and encouraging results.
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Implante Auditivo de Tronco Encefálico/métodos , Implantes Auditivos de Tronco Encefálico , Neoplasias da Orelha/cirurgia , Perda Auditiva/cirurgia , Neurofibromatose 2/cirurgia , Criança , Nervo Coclear , Humanos , Seleção de Pacientes , Percepção da Fala , Resultado do TratamentoRESUMO
Auditory brainstem implant (ABI) is used to provide auditory sensations in patients with neurofibromatosis type 2 who lost their hearing due to a surgical removal of the tumor. ABI surgery, implant activation and follow-up sessions present unique challenges including the exact placement of the electrode pad in the lateral recess of the IVth ventricle, identification of electrodes that trigger non-auditory sensation and their deactivation which lowers the number of electrodes responsible for hearing, changes of T- and C-levels across follow-up sessions. We present a complete procedure using an example case starting from the surgical part with the detailed description of intraoperative eABR measurement as a guidance for pad placement to the ABI activation and first fitting sessions with auditory sensation assessment. Since the first ABI electrode pad position presented non-satisfactory intraoperative eABR results it was decided to move the pad slightly which resulted in better eABR (more electrodes with auditory responses). The discussed patient demonstrated great auditory and speech perception results after the first ABI fitting (which included three sessions over 2 consecutive days). Repositioning of the ABI electrode pad during the surgery was carried out taking into account the intraoperative eABR results and this led to an overall positive outcome for the patient. The placement of ABI electrode pad is crucial for later auditory results. This study provides detailed insight in this very specialized procedure that is not performed in every clinic and adds to the knowledge of intraoperative navigation using eABR measurements during ABI surgery.
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Implante Auditivo de Tronco Encefálico/métodos , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Perda Auditiva Unilateral/cirurgia , Monitorização Neurofisiológica Intraoperatória/métodos , Adulto , Feminino , HumanosRESUMO
INTRODUCTION: Paediatric Auditory Brainstem Implantation (ABI) is indicated for children with congenital cochlear aplasia, absent/hypoplastic vestibulocochlear nerve, for whom cochlear implantation is not possible. Knowledge of the anatomical landmarks and variants in anatomy of the brainstem is vital for ABI surgery. METHOD: Study was done at Auditory implant centre in Madras ENT research foundation, which includes 24 children who had undergone ABI surgery and are being followed up for 1 year, post operatively. Aims were to study the anatomical variants and the outcomes of ABI implantation. To determine if different anatomical variants effect placement of ABI electrode. To assess the patient outcomes by Categories of auditory Performance (CAP) scores and Speech Intelligibility Ratings (SIR) scores. RESULTS: All the candidates showed gradual improvement in audiological and verbal outcomes after the ABI. The mean CAP and SIR scores after 6 months of AVHT were 2.07 and 1.37 respectively. After 1 year of auditory verbal rehabilitation therapy CAP was 3.42 and SIR was 2.33. Flocculus of the cerebellum can be of different grades. Though, there was difficulty in insertion of the electrode in subjects with anatomical variants, the outcomes were comparable with other subjects. CONCLUSION: ABI surgery involves frequent anatomical variations surrounding the lateral recess which makes the positioning of the auditory prosthesis difficult. Variants during the surgery can make the placement of ABI electrodes difficult, but promising results were seen all the implantees.
Assuntos
Implante Auditivo de Tronco Encefálico/estatística & dados numéricos , Cóclea/anormalidades , Perda Auditiva Neurossensorial/reabilitação , Pontos de Referência Anatômicos , Implante Auditivo de Tronco Encefálico/métodos , Pré-Escolar , Nervo Coclear/anormalidades , Craniotomia/métodos , Seguimentos , Perda Auditiva Neurossensorial/etiologia , Humanos , Índia , Estudos Prospectivos , Estudos Retrospectivos , Inteligibilidade da Fala , Resultado do TratamentoRESUMO
Auditory brainstem implantation (ABI) is the only solution to restore hearing when cochlear nerves are disrupted together with the pathologies where bilateral cochleae do not provide a suitable location for cochlear implantation. We reported first two successful auditory brainstem implantation cases in patients with neurofibromatosis Type II (NF2) with bilateral acoustic neuroma causing bilateral profound sensorineural hearing loss in Malaysia. A good candidate selection, dedicated surgeons and rehabilitation team as well as strong family support are the crucial factors in achieving the best possible surgical, audiological and speech outcomes.
Assuntos
Implante Auditivo de Tronco Encefálico , Perda Auditiva Neurossensorial/cirurgia , Neurofibromatose 2/cirurgia , Neuroma Acústico/cirurgia , Adulto , Implante Auditivo de Tronco Encefálico/métodos , Implantes Auditivos de Tronco Encefálico , Perda Auditiva Neurossensorial/etiologia , Humanos , Masculino , Neurofibromatose 2/complicações , Neuroma Acústico/complicaçõesRESUMO
OBJECTIVES/HYPOTHESIS: To report a series of patients with neurofibromatosis type 2 (NF2), where each patient underwent both cochlear implantation and auditory brainstem implantation for hearing rehabilitation, and to discuss factors influencing respective implant success. STUDY DESIGN: Retrospective case series. METHODS: Ten NF2 patients with both cochlear implantations and auditory brainstem implantations were retrospectively reviewed. Speech testing for auditory brainstem implants (ABIs) and cochlear implants (CIs) was performed separately. Scores at last follow-up were obtained for Iowa vowels and consonants, Northwestern University Children's Perception of Speech (NU-CHIPS), and City University of New York (CUNY) sentences. RESULTS: Mean age at time of implant was 37 years for cochlear implantation and 40 years for auditory brainstem implantation (P = .790, t test). Nine of 10 patients had a CI and ABI on contralateral sides, and one had both devices on the same side. Mean duration of deafness in the implanted ear was 4.3 years for both cochlear implantation and auditory brainstem implantation (P = .491, t test). Follow-up range was 1 to 28 years. CI performance on NU-CHIPS was 32% to 100%, and sound + lip-reading CUNY was 56% to 100%. Four patients experienced an eventual decline in CI function to unusable levels. ABI performance on NU-CHIPS was 40% to 80%, and sound + lip-reading CUNY was 38% to 94%. There was no notable decline in ABI function over time. CONCLUSIONS: If the cochlear nerve is intact, cochlear implantation can be an effective strategy for hearing rehabilitation in NF2. However, a significant proportion experience a decline in CI performance related to growing vestibular schwannoma or tumor treatment. Auditory brainstem implantation remains the standard option for surgical hearing rehabilitation in NF2, but peak performance is generally lower than that achievable with cochlear implantation. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:2163-2169, 2018.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Implante Coclear/métodos , Correção de Deficiência Auditiva/métodos , Perda Auditiva/reabilitação , Neurofibromatose 2/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada , Feminino , Perda Auditiva/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: Auditory brainstem implants (ABIs) may be the only opportunity for patients with NF2 to regain some sense of hearing sensation. However, only a very small number of individuals achieved open-set speech understanding and high sentence scores. Suboptimal placement of the ABI electrode array over the cochlear nucleus may be one of main factors for poor auditory performance. In the current study, we present a method of awake craniotomy to assist with ABI placement. METHODS: Awake surgery and hearing test via the retrosigmoid approach were performed for vestibular schwannoma resections and auditory brainstem implantations in four patients with NF2. Auditory outcomes and complications were assessed postoperatively. RESULTS: Three of 4 patients who underwent awake craniotomy during ABI surgery received reproducible auditory sensations intraoperatively. Satisfactory numbers of effective electrodes, threshold levels and distinct pitches were achieved in the wake-up hearing test. In addition, relatively few electrodes produced non-auditory percepts. There was no serious complication attributable to the ABI or awake craniotomy. CONCLUSIONS: It is safe and well tolerated for neurofibromatosis type 2 (NF2) patients using awake craniotomy during auditory brainstem implantation. This method can potentially improve the localization accuracy of the cochlear nucleus during surgery.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Craniotomia/métodos , Neurofibromatose 2/cirurgia , Adulto , Feminino , Testes Auditivos , Humanos , Monitorização Neurofisiológica Intraoperatória , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To compare the intraoperative electrically evoked auditory brainstem response (EABR) morphologies between neurofibromatosis II (NF2) adult auditory brainstem implant (ABI) recipients who had auditory percepts post-operatively and those who did not and between NF2 adult ABI recipients and non-NF2 pediatric ABI recipients. METHODS: This was a retrospective case series at a single tertiary academic referral center examining all ABI recipients from 1994 to 2016, which included 34 NF2 adults and 11 non-NF2 children. The morphologies of intraoperative EABRs were evaluated for the number of waveforms showing a response, the number of positive peaks in those responses, and the latencies of each of these peaks. RESULTS: 27/34 adult NF2 patients and 9/10 children had EABR waveforms. 20/27 (74.0%) of the adult patients and all of the children had ABI devices that stimulated post-operatively. When comparing the waveforms between adults who stimulated and those who did not stimulate, the proportion of total number of intraoperative EABR peaks to total possible peaks was significantly higher for the adults who stimulated than for those who did not (p < 0.05). Children had a significantly higher proportion of total number of peaks to total possible peaks when compared to adults who stimulated (p < 0.02). Additionally, there were more likely to be EABR responses at the initial stimulation than intraoperatively in the pediatric ABI population (p = 0.065). CONCLUSIONS: The value of intraoperative EABR tracing may lie in its ability to predict post-operative auditory percepts based on the placement of the array providing the highest number of total peaks.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Neurofibromatose 2/fisiopatologia , Adolescente , Adulto , Implantes Auditivos de Tronco Encefálico , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVES: The auditory brainstem implant (ABI) provides sound awareness to patients who are ineligible for cochlear implantation. Auditory performance varies widely among similar ABI cohorts. We hypothesize that differences in electrode array position contribute to this variance. Herein, we classify ABI array position based on postoperative imaging and investigate the relationship between position and perception. DESIGN: Retrospective review of pediatric and adult ABI users with postoperative computed tomography. To standardize views across subjects, true axial reformatted series of scans were created using the McRae line. Using multiplanar reconstructions, basion and electrode array tip coordinates and array angles from vertical were measured. From a lateral view, array angles (V) were classified into types I to IV, and from posterior view, array angles (T) were classified into types A to D. Array position was further categorized by measuring distance vertical from basion (D1) and lateral from midline (D2). Differences between array classifications were compared with audiometric thresholds, number of active electrodes, and pitch ranking. RESULTS: Pediatric (n = 4, 2 with revisions) and adult (n = 7) ABI subjects were included in this study. Subjects had a wide variety of ABI array angles, but most were aimed superiorly and posteriorly (type II, n = 7) from lateral view and upright or medially tilted from posterior view (type A, n = 6). Mean pediatric distances were 8 to 42% smaller than adults for D1 and D2. In subjects with perceptual data, electrical thresholds and the number of active electrodes differed among classification types. CONCLUSIONS: In this first study to classify ABI electrode array orientation, array position varied widely. This variability may explain differences in auditory performance.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Implantes Auditivos de Tronco Encefálico , Percepção Auditiva , Tronco Encefálico/diagnóstico por imagem , Perda Auditiva Bilateral/reabilitação , Perda Auditiva Neurossensorial/reabilitação , Nervo Vestibulococlear/anormalidades , Adulto , Idoso , Audiometria , Pré-Escolar , Perda Auditiva Bilateral/etiologia , Perda Auditiva Neurossensorial/etiologia , Humanos , Imageamento Tridimensional , Lactente , Pessoa de Meia-Idade , Malformações do Sistema Nervoso/complicações , Neurofibromatose 2/complicações , Período Pós-Operatório , Estudos Retrospectivos , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
OBJECTIVES: The objectives of study was to 1) Describe relevant surgical anatomy in defining and accessing the lateral recess for placement of electrode, 2) Propose a working classification for grades of Flocculus; 3) To determine if different grades of cerebellar flocculus effects placement of ABI electrode and subjective outcomes in implantees. METHODS: Our study was a prospective study, and comprised of cohort of 12 patients who underwent ABI surgery via retrosigmoid approach between 1 Jan 2012 to 31 Dec 2014. All children with congenital profound sensorineural hearing loss with either absent cochlea or cochlear nerve were included in the study. Relevant anatomy was noted. We also noted down the difficulty encountered during the placement of ABI electrode. Auditory perception and speech intelligibility was scored post operatively for 1 year. RESULTS: Cerebellar flocculus was divided into 4 grades depending on the morphology of cerebellar flocculus. It was noted that Grade 3 & 4 flocculus (Group B) had difficult ABI electrode placement in comparison to Grade 1 & 2 flocculus (Group A). The subjective outcomes of Group A was better than Group B. However the p value was not statistically significant. CONCLUSION: Cerebellar flocculus can be graded depending on morphology and size. Flocculus of higher grades can make the placement of ABI electrodes difficult and adversely effects the postoperative subjective outcomes.
Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Percepção Auditiva/fisiologia , Cerebelo/fisiologia , Inteligibilidade da Fala/fisiologia , Percepção da Fala/fisiologia , Adolescente , Implantes Auditivos de Tronco Encefálico , Criança , Pré-Escolar , Nervo Coclear/anormalidades , Feminino , Humanos , Masculino , Estudos Prospectivos , Resultado do TratamentoRESUMO
OBJECTIVE: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime. METHOD: Retrospective review of 12 patients, managed over the period 2009-2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI). RESULTS: Four of the 12 patients achieved speech discrimination without lip-reading. In these individuals there is reason to think that the mechanism of their hearing loss was cochlear dysfunction. A further four patients received benefit to lip-reading and awareness of environmental sound. For such patients their hearing loss may have been due to both cochlear and neural dysfunction. Two patients gained access to environmental sound only from their CI. Two patients derived no benefit from their CIs, which were subsequently explanted. Both these latter patients had had prior ipsilateral tumour surgery, one just before the CI insertion. CONCLUSION: Cochlear implantation can lead to open set speech discrimination in patients with NF2 in the presence of a stable VS. Use of promontory stimulation and intraoperative electrically evoked auditory brainstem response testing, along with case history, can inform the decision whether to implant an ABI or CI.
Assuntos
Implante Coclear/métodos , Implantes Cocleares , Potenciais Evocados Auditivos do Tronco Encefálico , Perda Auditiva/cirurgia , Neurofibromatose 2/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Implante Auditivo de Tronco Encefálico/métodos , Feminino , Perda Auditiva/etiologia , Perda Auditiva/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Neurofibromatose 2/fisiopatologia , Seleção de Pacientes , Estudos Retrospectivos , Percepção da Fala , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To report the preliminary outcomes of auditory brainstem implantation (ABI) under a compassionate use protocol for two ABI devices that are not approved by the US Food and Drug Administration. METHODS: A retrospective review was performed of neurofibromatosis type 2 (NF2) patients who underwent microsurgery for vestibular schwannoma (VS) and placement of either the Cochlear ABI541 or Med-El Synchrony ABIs. Peri-operative and device- related complications were reviewed. Audiometric performance was also evaluated. RESULTS: Seven patients received either the Cochlear ABI541 (6) or the Med-El Synchrony ABI (1) after the resection of VS. No device or patient-related complications occurred to date. Surgical times and early audiological performance are similar to our previous experience with the Cochlear ABI24 device. CONCLUSIONS: Early experience with the Cochlear ABI541 and Med-El Synchrony ABI devices under a compassionate use protocol suggest that both devices are safe with comparable utility to the Cochlear ABI24 device.