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1.
Ear Hear ; 45(2): 378-389, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37759357

RESUMO

OBJECTIVES: This study aimed to: (1) determine the interaction between cognitive load and balance in children and young adults with bilateral cochleovestibular dysfunction who use bilateral cochlear implants (CIs) and (2) determine the effect of an auditory balance prosthesis (the BalanCI) on this interaction. Many (20 to 70%) children with sensorineural hearing loss experience some degree of vestibular loss, leading to poorer balance. Poor balance could have effects on cognitive resource allocation which might be alleviated by the BalanCI as it translates head-referenced cues into electrical pulses delivered through the CI. It is hypothesized that children and young adults with cochleovestibular dysfunction will demonstrate greater dual-task costs than typically-developing children during dual balance-cognition tasks, and that BalanCI use will improve performance on these tasks. DESIGN: Study participants were 15 typically-developing children (control group: mean age ± SD = 13.6 ± 2.75 years, 6 females) and 10 children and young adults who use bilateral CIs and have vestibular dysfunction (CI-V group: mean age ± SD=20.6 ± 5.36 years, 7 females). Participants completed two working memory tasks (backward auditory verbal digit span task and backward visuospatial dot matrix task) during three balance conditions: seated, standing in tandem stance with the BalanCI off, and standing in tandem stance with the BalanCI on. Working memory performance was quantified as total number of correct trials achieved. Postural stability was quantified as translational and rotational path length of motion capture markers worn on the head, upper body, pelvis, and feet, normalized by trial time. RESULTS: Relative to the control group, children and young adults in the CI-V group exhibited poorer overall working memory across all balance conditions ( p = 0.03), poorer translational postural stability (larger translational path length) during both verbal and visuospatial working memory tasks ( p < 0.001), and poorer rotational stability (larger rotational path length) during the verbal working memory task ( p = 0.026). The CI-V group also exhibited poorer translational ( p = 0.004) and rotational ( p < 0.001) postural stability during the backward verbal digit span task than backward visuospatial dot matrix task; BalanCI use reduced this stability difference between verbal and visuospatial working memory tasks for translational stability overall ( p > 0.9), as well as for rotational stability during the maximum working memory span (highest load) participants achieved in each task ( p = 0.91). CONCLUSIONS: Balance and working memory were impaired in the CI-V group compared with the control group. The BalanCI offered subtle improvements in stability in the CI-V group during a backward verbal working memory task, without producing a negative effect on working memory outcomes. This study supports the feasibility of the BalanCI as a balance prosthesis for individuals with cochleovestibular impairments.


Assuntos
Implante Coclear , Implantes Cocleares , Criança , Feminino , Humanos , Adulto Jovem , Memória de Curto Prazo , Cognição , Sinais (Psicologia) , Equilíbrio Postural
2.
Am J Otolaryngol ; 45(1): 104087, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37839178

RESUMO

BACKGROUND: Functional hearing loss can be due to an auditory manifestation of functional neurological disorder, previously known as conversion disorder. METHODS: This is a case series of 3 pediatric patients with a diagnosis of idiopathic SSNHL who ultimately were found to have functional neurological disorder. RESULTS: Average age was 12.7 years at presentation (range 10-14 years). All three patients underwent invasive interventions prior to their initial clinic visit. All patients demonstrated profound SNHL on behavioural audiogram, but normal otoacoustic emissions (OAE) and auditory brainstem response testing. With counselling, both patients demonstrated significant hearing improvement. CONCLUSIONS: Early use of OAE's in the workup of SSNHL can avoid unnecessary and potentially harmful therapies and expedite access to counselling services which may help lead to symptom resolution.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Criança , Adolescente , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/terapia , Potenciais Evocados Auditivos do Tronco Encefálico , Testes Auditivos , Emissões Otoacústicas Espontâneas
3.
Hum Brain Mapp ; 43(12): 3662-3679, 2022 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-35429083

RESUMO

Unilateral auditory deprivation in early childhood can lead to cortical strengthening of inputs from the stimulated side, yet the impact of this on bilateral processing when inputs are later restored beyond an early sensitive period is unknown. To address this, we conducted a longitudinal study with 13 bilaterally profoundly deaf adolescents who received unilateral access to sound via a cochlear implant (CI) in their right ear in early childhood before receiving bilateral access to sound a decade later via a second CI in their left ear. Auditory-evoked cortical responses to unilateral and bilateral stimulation were measured repeatedly using electroencephalogram from 1 week to 14 months after activation of their second CI. Early cortical responses from the newly implanted ear and bilateral stimulation were atypically lateralized to the left ipsilateral auditory cortex. Duration of unilateral deafness predicted an unexpectedly stronger representation of inputs from the newly implanted, compared to the first implanted ear, in left auditory cortex. Significant initial reductions in responses were observed, yet a left-hemisphere bias and unequal weighting of inputs favoring the long-term deaf ear did not converge to a balanced state observed in the binaurally developed system. Bilateral response enhancement was significantly reduced in left auditory cortex suggesting deficits in ipsilateral response inhibition of new, dominant, inputs during bilateral processing. These findings paradoxically demonstrate the adaptive capacity of the adolescent auditory system beyond an early sensitive period for bilateral input, as well as restrictions on its potential to fully reverse cortical imbalances driven by long-term unilateral deafness.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Unilateral , Percepção da Fala , Estimulação Acústica , Adolescente , Pré-Escolar , Audição , Humanos , Estudos Longitudinais
4.
Ear Hear ; 43(2): 420-435, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34534156

RESUMO

OBJECTIVES: Older adults with age-related hearing loss (ARHL) are at greater risk of falling and have greater mobility problems than older adults with normal hearing (NH). The underlying cause of these associations remains unclear. One possible reason is that age-related declines in the vestibular system could parallel those observed in the auditory system within the same individuals. Here, we compare the sensitivity of vestibular perceptual abilities (psychophysics), vestibular end-organ functioning (vestibular evoked myogenic potentials and video head impulse tests), and standing balance (posturography) in healthy older adults with and without ARHL. DESIGN: A total of 46 community-dwelling older adults, 23 with ARHL and 23 with NH, were passively translated in heave (up and down) and rotated in pitch (tilted forward and backward) in the dark using a motion platform. Using an adaptive staircase psychophysical procedure, participants' heave and pitch detection and discrimination thresholds were determined. In a posturography task, participants' center of pressure (COP) path length was measured as they stood on a forceplate with eyes open and closed, on firm and compliant surfaces, with and without sound suppression. Baseline motor, cognitive, and sensory functioning, including vestibular end-organ function, were measured. RESULTS: Individuals with ARHL were less sensitive at discriminating pitch movements compared to older adults with NH. Poorer self-reported hearing abilities were also associated with poorer pitch discrimination. In addition to pitch discrimination thresholds, lower pitch detection thresholds were significantly associated with hearing loss in the low-frequency range. Less stable standing balance was significantly associated with poorer vestibular perceptual sensitivity. DISCUSSION: These findings provide evidence for an association between ARHL and reduced vestibular perceptual sensitivity.


Assuntos
Presbiacusia , Potenciais Evocados Miogênicos Vestibulares , Vestíbulo do Labirinto , Idoso , Audição , Humanos , Equilíbrio Postural/fisiologia , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Vestíbulo do Labirinto/fisiologia
5.
Audiol Neurootol ; 25(1-2): 6-24, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31533097

RESUMO

BACKGROUND: In patients with bilateral vestibulopathy, the regular treatment options, such as medication, surgery, and/or vestibular rehabilitation, do not always suffice. Therefore, the focus in this field of vestibular research shifted to electrical vestibular stimulation (EVS) and the development of a system capable of artificially restoring the vestibular function. Key Message: Currently, three approaches are being investigated: vestibular co-stimulation with a cochlear implant (CI), EVS with a vestibular implant (VI), and galvanic vestibular stimulation (GVS). All three applications show promising results but due to conceptual differences and the experimental state, a consensus on which application is the most ideal for which type of patient is still missing. SUMMARY: Vestibular co-stimulation with a CI is based on "spread of excitation," which is a phenomenon that occurs when the currents from the CI spread to the surrounding structures and stimulate them. It has been shown that CI activation can indeed result in stimulation of the vestibular structures. Therefore, the question was raised whether vestibular co-stimulation can be functionally used in patients with bilateral vestibulopathy. A more direct vestibular stimulation method can be accomplished by implantation and activation of a VI. The concept of the VI is based on the technology and principles of the CI. Different VI prototypes are currently being evaluated regarding feasibility and functionality. So far, all of them were capable of activating different types of vestibular reflexes. A third stimulation method is GVS, which requires the use of surface electrodes instead of an implanted electrode array. However, as the currents are sent through the skull from one mastoid to the other, GVS is rather unspecific. It should be mentioned though, that the reported spread of excitation in both CI and VI use also seems to induce a more unspecific stimulation. Although all three applications of EVS were shown to be effective, it has yet to be defined which option is more desirable based on applicability and efficiency. It is possible and even likely that there is a place for all three approaches, given the diversity of the patient population who serves to gain from such technologies.


Assuntos
Terapia por Estimulação Elétrica , Doenças Vestibulares/terapia , Vestíbulo do Labirinto/fisiopatologia , Implante Coclear , Eletrodos Implantados , Humanos , Reflexo Vestíbulo-Ocular/fisiologia , Doenças Vestibulares/fisiopatologia
6.
Audiol Neurootol ; 25(1-2): 60-71, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31678979

RESUMO

INTRODUCTION: To determine the impact of a head-referenced cochlear implant (CI) stimulation system, BalanCI, on balance and postural control in children with bilateral cochleovestibular loss (BCVL) who use bilateral CI. METHODS: Prospective, blinded case-control study. Balance and postural control testing occurred in two settings: (1) quiet clinical setting and (2) immersive realistic virtual environment (Challenging Environment Assessment Laboratory [CEAL], Toronto Rehabilitation Institute). Postural control was assessed in 16 and balance in 10 children with BCVL who use bilateral CI, along with 10 typically developing children. Children with neuromotor, cognitive, or visual deficits that would prevent them from performing the tests were excluded. Children wore the BalanCI, which is a head-mounted device that couples with their CIs through the audio port and provides head-referenced spatial information delivered via the intracochlear electrode array. Postural control was measured by center of pressure (COP) and time to fall using the WiiTM (Nintendo, WA, USA) Balance Board for feet and the BalanCI for head, during the administration of the Modified Clinical Test of Sensory Interaction in Balance (CTSIB-M). The COP of the head and feet were assessed for change by deviation, measured as root mean square around the COP (COP-RMS), rate of deviation (COP-RMS/duration), and rate of path length change from center (COP-velocity). Balance was assessed by the Bruininks-Oseretsky Test of Motor Proficiency 2, balance subtest (BOT-2), specifically, BOT-2 score as well as time to fall/fault. RESULTS: In the virtual environment, children demonstrated more stable balance when using BalanCI as measured by an improvement in BOT-2 scores. In a quiet clinical setting, the use of BalanCI led to improved postural control as demonstrated by significant reductions in COP-RMS and COP-velocity. With the use of BalanCI, the number of falls/faults was significantly reduced and time to fall increased. CONCLUSIONS: BalanCI is a simple and effective means of improving postural control and balance in children with BCVL who use bilateral CI. BalanCI could potentially improve the safety of these children, reduce the effort they expend maintaining balance and allow them to take part in more complex balance tasks where sensory information may be limited and/or noisy.


Assuntos
Implante Coclear , Implantes Cocleares , Equilíbrio Postural/fisiologia , Doenças Vestibulares/cirurgia , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Estudos Prospectivos , Resultado do Tratamento , Doenças Vestibulares/fisiopatologia
7.
Ear Hear ; 41(3): 678-685, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31567563

RESUMO

OBJECTIVES: To measure the acceptance of a cochlear implant by children with single-sided deafness (SSD) using datalogging technology in the cochlear implant processor. DESIGN: Datalogs from follow-up clinical audiology appointments for 23 children with SSD were extracted from their cochlear implant processors ranging from 1 to 8 visits (M = 3.74, SD = 1.79). The number of hours the cochlear implant was in use per day, the number of times the coil disconnected from the internal device, and the percentage of daily cochlear implant use in different auditory environments were collected from the datalogs. Linear mixed-effects regressions were used to analyze the relationship between age, hearing experience, cochlear implant use, and coil-offs per day. Nonlinear regressions were conducted to evaluate cochlear implant use in different environments. RESULTS: Children with SSD wore their cochlear implants for 6.22 (SD = 2.81; range = 0.0004 to 14.74) hours per day on average. No significant change in cochlear implant use was seen as the children grew older or gained more hearing experience. As hearing experience increased, the number of coil-offs per day was reduced. Preschoolers spent more time in "music" and "speech" and less time in "noise" and "quiet" than older and younger children while older children spent more time in "speech-in-noise." CONCLUSIONS: Children with SSD consistently wear their cochlear implants. However, the auditory environments to which they are exposed vary over time. Regular cochlear implant use by this population suggests that it does not detract from a normal-hearing ear and that children with SSD appreciate access to bilateral input.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Unilateral , Percepção da Fala , Adolescente , Criança , Surdez/cirurgia , Perda Auditiva Unilateral/cirurgia , Humanos
8.
J Acoust Soc Am ; 146(6): 4352, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31893744

RESUMO

This paper asked whether children fitted with bilateral hearing aids (BHA) develop normal perception of binaural cues which are the basis of spatial hearing. Data from children with BHA (n = 26, age = 12.6 ± 2.84 years) were compared to data from a control group (n = 12, age = 12.36 ± 2.83 years). Stimuli were 250 Hz click-trains of 36 ms and a 40 ms consonant-vowel /da/ at 1 Hz presented through ER3A insert-earphones unilaterally or bilaterally. Bilateral stimuli were presented at different interaural level difference (ILD) and interaural timing difference (ITD) conditions. Participants indicated whether the sound came from the left or right side (lateralization) or whether one sound or two could be heard (binaural fusion). BHA children lateralized ILDs similarly to the control group but had impaired lateralization of ITDs. Longer response times relative to controls suggest that lateralization of ITDs was challenging for children with BHA. Most, but not all, of the BHA group were able to fuse click and speech sounds similarly to controls. Those unable to fuse showed particularly poor ITD lateralization. Results suggest that ITD perception is abnormal in children using BHAs, suggesting persistent effects of hearing loss that are not remediated by present clinical rehabilitation protocols.


Assuntos
Surdez/reabilitação , Auxiliares de Audição , Perda Auditiva/reabilitação , Pessoas com Deficiência Auditiva/reabilitação , Estimulação Acústica/métodos , Adolescente , Criança , Implante Coclear/métodos , Implantes Cocleares/efeitos adversos , Feminino , Audição/fisiologia , Humanos , Masculino , Localização de Som/fisiologia , Percepção do Tempo
9.
Eur J Neurosci ; 45(9): 1212-1223, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28177166

RESUMO

The cervical vestibular evoked myogenic potential (cVEMP) is a common and simple test of vestibulospinal reflex patency. In the clinic, cVEMPs are measured in response to loud sounds from the sternocleidomastoid (SCM) on the ventral neck, as subjects maintain an uncomfortable head posture needed to recruit SCM. Here we characterize the cVEMP in a dorsal neck turner (splenius capitis; SPL), and compare it with the SCM cVEMP. cVEMPs were recorded simultaneously via surface electromyography from SCM and SPL from 17 healthy subjects in a variety of postures, including head-turned postures adopted while either seated or standing, and the clinical posture. Like the SCM cVEMP recorded ipsilateral to the side of sound stimulation, the cVEMP on the contralateral SPL (synergistic with ipsilateral SCM) was characterized by a biphasic wave of muscle activity that began at ~ 13 ms. cVEMP reliability was higher on SPL vs. SCM in standing postures (chi-squared; P < 0.05), and equivalent results were obtained from SPL in a standing or seated posture. In 9 of the 17 subjects, we also obtained bilateral intramuscular (IM) recordings from SPL at the same time as the surface recordings. In these subjects, the initial surface response in SPL was associated with a consistent decrease in multi-unit IM SPL activity. Overall, these results demonstrate that SPL recordings offer a complimentary target for cVEMP assessments. The expression of SPL cVEMPs in simple head-turned postures may also improve the utility of cVEMP testing for vestibular assessment in children, the elderly, or non-compliant.


Assuntos
Músculos do Pescoço/fisiologia , Músculos Paraespinais/fisiologia , Postura/fisiologia , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Estimulação Acústica/métodos , Adulto , Eletromiografia/métodos , Feminino , Humanos , Masculino , Reflexo/fisiologia , Reprodutibilidade dos Testes , Vestíbulo do Labirinto/fisiologia , Adulto Jovem
10.
Anesthesiology ; 125(5): 889-903, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27541315

RESUMO

BACKGROUND: Opioid analgesia is an essential component of perioperative care, but effective analgesia can be limited by excessive sedation and respiratory depression. The cortical signatures associated with sedation by opioids and the relationship between changes in cortical activity and respiratory function are not well understood. The objectives of this study were to identify the electroencephalogram signatures of sedation and respiratory changes induced by morphine in a pediatric population after elective surgery. METHODS: After otologic surgery, patients (14.8 ± 2.8 yr, n = 10) stayed overnight for pain relief with morphine (3 to 10 mg), hydration, and clinical observation. Electroencephalogram activity and polysomnography were performed before and after morphine, and electroencephalogram spectral properties and cardiorespiratory activities were analyzed. RESULTS: Compared to wakefulness and non-rapid eye movement sleep, morphine reduced high-frequency ß1 (13.5 to 20 Hz) and ß2 (20 to 30Hz) electroencephalogram powers (n = 10) and decreased coherence between frontal and occipital ß2 electroencephalogram activities (n = 9), therefore indicating that morphine induced a deep sedative state. Morphine also reduced respiratory rate by 8.3% (n = 10). Interestingly, there was a significant correlation between the reduction in ß1 electroencephalogram activity and the depression in respiratory rate induced by morphine (R = 0.715, n = 10). With significant reduction in ß1 power, respiratory rate was decreased by more than 25%, suggesting that reduction in cortical arousal is associated with the severity of respiratory rate depression. CONCLUSIONS: Analgesic doses of morphine are associated with reduction in respiratory rate when accompanied by reduction in ß1 electroencephalogram power, indicating a powerful effect of cortical arousal state per se in respiratory rate depression by morphine.


Assuntos
Analgésicos Opioides/farmacologia , Encéfalo/efeitos dos fármacos , Estado de Consciência/efeitos dos fármacos , Morfina/farmacologia , Insuficiência Respiratória/fisiopatologia , Taxa Respiratória/efeitos dos fármacos , Adolescente , Criança , Pré-Escolar , Eletroencefalografia/efeitos dos fármacos , Feminino , Humanos , Masculino , Polissonografia/efeitos dos fármacos
12.
Otol Neurotol ; 45(8): 913-918, 2024 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-39142312

RESUMO

INTRODUCTION: This study reviews the feasibility of implanting active osseointegrated bone conduction devices in young children, below the prior age for FDA indication (<12 years), which has recently been reduced to 5 years. Outcomes included differences in adverse event rates and operative time between two groups (<12 and 12 years or older). MATERIALS AND METHODS: This study is a retrospective review of children receiving active osseointegrated bone conduction devices at a tertiary referral center academic hospital. One hundred and twenty-four children received 135 active osseointegrated bone conduction devices (May 2018-March 2024). RESULTS: Of 135 devices, 77 (57%) were in children <12 years (mean age (SD) = 7.9 (2.0) years, range = 4.9-11.9 years) and 58 (43%) were in 12 years or older (mean age (SD) = 15.1 (1.7) years, range = 12-18 years). Adverse events were significantly higher in the older group, occurring in 8 (10%) of 77 devices in children <12 years and 15 (26%) of 58 devices in children 12 years and older (26%) (Fisher's exact test = 0.0217 at p < 0.05). Major adverse events occurred in 5/124 (4%) patients, with 2 in patients <12 years (2/73, 3%) and 3 in children 12 and older (3/51, 6%). The proportion of major events between groups was not significantly different (Fisher's exact test = 0.4, p < 0.05). Mean surgical time was significantly less (t = -2.8799, df = 120.26, p = 0.005) in the children <12 years (mean (SD) = 66.5 (22.4) min) compared to those 12 and over (mean (SD) = 78.32 (23.1) min). CONCLUSIONS: Implantation of active osseointegrated bone conduction devices is feasible in children as young as 5 years and demonstrates low rates of complication. Further miniaturization may allow even earlier safe intervention.


Assuntos
Condução Óssea , Estudos de Viabilidade , Osseointegração , Humanos , Criança , Condução Óssea/fisiologia , Estudos Retrospectivos , Masculino , Feminino , Pré-Escolar , Osseointegração/fisiologia , Adolescente , Resultado do Tratamento , Perda Auditiva Condutiva/cirurgia , Auxiliares de Audição , Prótese Ancorada no Osso , Implantação de Prótese/métodos
13.
Trends Hear ; 28: 23312165241261480, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38887094

RESUMO

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.


Assuntos
Implante Coclear , Implantes Cocleares , Humanos , Lactente , Implante Coclear/instrumentação , Implante Coclear/efeitos adversos , Masculino , Feminino , Implantes Cocleares/efeitos adversos , Resultado do Tratamento , Canadá , Estados Unidos , Fatores de Tempo , Estudos Retrospectivos , Limiar Auditivo , Complicações Pós-Operatórias
14.
Laryngoscope ; 134(8): 3832-3838, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38426810

RESUMO

OBJECTIVE: The objective of this study was to assess the prevalence of genetic variants associated with hearing loss in a large cohort of children in Canada using high throughput next generation sequencing (NGS). METHODS: A total of 485 children with hearing loss underwent NGS testing with an 80 gene panel of syndromic and non-syndromic variants known to be associated with hearing loss. Genetic variants were classified as pathogenic, likely pathogenic, likely benign, benign, or variants of uncertain significance (VUS), according to the American College of Medical Genetics and Genomics guidelines. RESULTS: Across the 80 genes tested, 923 variants, predominantly in 28 genes, were identified in 324 children. Pathogenic variants occurred in 19/80 (23.8%) of the hearing loss related genes tested and confirmed the etiology of hearing loss in 73/485 (15.1%) of children. GJB2 was the most prevalent gene, affecting 28/73 (38.4%) children with confirmed genetic hearing loss in our cohort. Most identified variants (748/923, 81.0%, in 76/80 genes) were of uncertain significance. CONCLUSION: Genetic testing using NGS identified the etiology in approximately 15% of childhood hearing loss in a Canadian cohort which is lower than what is typically reported. GJB2 was the most common genetic cause of hearing loss. VUS are commonly identified, presenting clinical challenges for counseling. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3832-3838, 2024.


Assuntos
Testes Genéticos , Perda Auditiva , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Canadá/epidemiologia , Criança , Masculino , Feminino , Pré-Escolar , Prevalência , Perda Auditiva/genética , Perda Auditiva/epidemiologia , Lactente , Adolescente , Estudos de Coortes , Variação Genética/genética , Conexina 26/genética , Predisposição Genética para Doença
15.
Laryngoscope ; 2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38899833

RESUMO

OBJECTIVE: The Hearing Utility Measure (HUM) is a replacement hearing attribute for the Health Utilities Index, Mark 3 (HUI-3) designed to improve the responsiveness of utility estimates to changes in hearing-related quality of life. The final development step is to derive the instrument's utility scoring function. METHODS: Residents of Ontario, Canada, aged ≥18 years participated in standard gamble and visual analogue scale exercises. Valuations for levels (response options) within each domain, and for each domain relative to the other domains were elicited and used to generate a hearing utility function. The function outputs hearing utility ranging from 0 = 'unable to hear at all' to 1 = 'perfect hearing' for each of the 25,920 hearing states classifiable by the HUM. Performance was assessed relative to the criterion standard: directly elicited standard gamble utility. Distributions of HUM-derived hearing utility were compared with legacy HUI-3 derived estimates. RESULTS: A total of 126 respondents participated (mean age 39.2, range 18-85 years, 53% female [67/126]). The utility function performed well in the estimation of directly elicited utilities (mean difference 0.03, RMSE 0.06). Using the legacy HUI-3, estimated hearing utility was 1.0 for 118/126 respondents (93.6%) compared with just 66/126 (52.4%) using the HUM. CONCLUSION: The new hearing attribute is capable of measuring variations in hearing utility not captured by the legacy HUI-3, especially near the ceiling of hearing function. These findings justify its application and further work to study its measurement properties in hearing loss populations. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

16.
Pediatr Blood Cancer ; 60(2): 287-92, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23002030

RESUMO

BACKGROUND: Cisplatin (CDDP) ototoxicity is a significant side effect of the current treatment of medulloblastoma (MB). Cumulative dose of CDDP and age are recognized risk factors for hearing loss, but inter-individual susceptibility limits our ability to identify patients at risk for hearing loss. We describe the kinetics of early audiometric changes during therapy and identify profiles associated with a higher risk of needing hearing aids. PROCEDURE: Serial audiometric evaluations were performed during and after completion of therapy in children with average risk (AR) and high-risk (HR) MB. Each audiogram was scored according to five grading systems. Variations of pure tone thresholds were analyzed at each frequency for each consecutive audiogram. CDDP dose modifications and hearing outcome were recorded. RESULTS: A total of 258 audiograms from 35 patients (22 AR, 13 HR) were analyzed. Eighteen AR patients (81.3%) required dose reduction and the median cumulative dose of CDDP administered was 412.5 mg/m(2) (150-600), corresponding to 68% of the intended dose. Three HR patients (23.0%) required dose reduction. At a median follow-up of 67 months (11-117), nine patients (25.7%) required hearing support: After two cycles of CDDP (150 mg/m(2) ), the average hearing loss at 8,000 Hz was twice higher in the group that eventually required hearing support. CONCLUSION: Early alteration of high-frequency thresholds may help identify individuals who will require hearing support. In the MB population, alternative strategies should be developed to limit the cumulative dose of CDDP to prevent significant ototoxicity.


Assuntos
Antineoplásicos/efeitos adversos , Neoplasias Cerebelares/tratamento farmacológico , Cisplatino/efeitos adversos , Perda Auditiva/induzido quimicamente , Meduloblastoma/tratamento farmacológico , Adolescente , Audiometria , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Fatores de Risco
17.
Proc Natl Acad Sci U S A ; 107(13): 6070-5, 2010 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-20231461

RESUMO

In addition to its role in shifting the line of sight, the oculomotor system is also involved in the covert orienting of visuospatial attention. Causal evidence supporting this premotor theory of attention, or oculomotor readiness hypothesis, comes from the effect of subsaccadic threshold stimulation of the oculomotor system on behavior and neural activity in the absence of evoked saccades, which parallels the effects of covert attention. Here, by recording neck-muscle activity from monkeys and systematically titrating the level of stimulation current delivered to the frontal eye fields (FEF), we show that such subsaccadic stimulation is not divorced from immediate motor output but instead evokes neck-muscle responses at latencies that approach the minimal conduction time to the motor periphery. On average, neck-muscle thresholds were approximately 25% lower than saccade thresholds, and this difference is larger for FEF sites associated with progressively larger saccades. Importantly, we commonly observed lower neck-muscle thresholds even at sites evoking saccades

Assuntos
Movimentos Sacádicos/fisiologia , Campos Visuais/fisiologia , Animais , Atenção/fisiologia , Eletromiografia , Potenciais Evocados Visuais , Movimentos Oculares/fisiologia , Movimentos da Cabeça/fisiologia , Macaca mulatta/fisiologia , Masculino , Neurônios Motores/fisiologia , Músculos do Pescoço/fisiologia , Nervo Oculomotor/fisiologia , Estimulação Luminosa , Limiar Sensorial/fisiologia , Vias Visuais/fisiologia
18.
Otol Neurotol ; 44(3): 233-240, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-36728258

RESUMO

OBJECTIVE: Children with single-sided deafness (SSD) show reduced language and academic development and report hearing challenges. We aim to improve outcomes in children with SSD by providing bilateral hearing through cochlear implantation of the deaf ear with minimal delay. STUDY DESIGN: Prospective cohort study of 57 children with SSD provided with cochlear implant (CI) between May 13, 2013, and June 25, 2021. SETTING: Tertiary children's hospital. PARTICIPANTS: Children with early onset (n = 40) or later onset of SSD (n = 17) received CIs at ages 2.47 ± 1.58 years (early onset group) and 11.67 ± 3.91 years (late onset group) (mean ± SD). Duration of unilateral deafness was limited (mean ± SD = 1.93 ± 1.56 yr). INTERVENTION: Cochlear implantation of the deaf ear. MAIN OUTCOMES/MEASURES: Evaluations of device use (data logging) and hearing (speech perception, effects of spatial release from masking on speech detection, localization of stationary and moving sound, self-reported hearing questionnaires). RESULTS: Results indicated that daily device use is variable (mean ± SD = 5.60 ± 2.97, range = 0.0-14.7 h/d) with particular challenges during extended COVID-19 lockdowns, including school closures (daily use reduced by mean 1.73 h). Speech perception with the CI alone improved (mean ± SD = 65.7 ± 26.4 RAU) but, in the late onset group, remained poorer than in the normal hearing ear. Measures of spatial release from masking also showed asymmetric hearing in the late onset group ( t13 = 5.14, p = 0.001). Localization of both stationary and moving sound was poor (mean ± SD error = 34.6° ± 16.7°) but slightly improved on the deaf side with CI use ( F1,36 = 3.95, p = 0.05). Decreased sound localization significantly correlated with poorer self-reported hearing. CONCLUSIONS AND RELEVANCE: Benefits of CI in children with limited durations of SSD may be more restricted for older children/adolescents. Spatial hearing challenges remain. Efforts to increase CI acceptance and consistent use are needed.


Assuntos
COVID-19 , Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Unilateral , Localização de Som , Percepção da Fala , Adolescente , Humanos , Criança , Lactente , Pré-Escolar , Implante Coclear/métodos , Estudos Prospectivos , Ruído , Controle de Doenças Transmissíveis , Perda Auditiva Unilateral/cirurgia , Fatores de Tempo , Surdez/cirurgia
19.
Sci Rep ; 13(1): 9721, 2023 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-37322114

RESUMO

Study objectives were to: (1) quantify stability in children and young adults using cochlear implants with concurrent cochleovestibular dysfunction (CI-V) during balance perturbations and (2) to assess effects of an auditory head-referencing device (BalanCI) on their stability. The BalanCI provides auditory feedback via cochlear implants to cue posture and potentially avoid falling in children with CI-V. It was hypothesized that children and young adults with CI-V respond with larger movements to floor perturbations than typically-developing peers (controls) and that BalanCI use decreases these movements. Motion in response to treadmill perturbations was captured by markers on the head, torso, and feet in eight CI-V and 15 control participants. Stability (area under the curve of motion displacement) and peak displacement latencies were measured. The CI-V group demonstrated less stability and slower responses than the control group during medium and large backwards perturbations (p's < 0.01). In the CI-V group, BalanCI use improved stability during large backwards perturbations (p < 0.001), but worsened stability during large sideways perturbations (p's < 0.001). Children and young adults with CI-V move more to remain upright during perturbations than typically-developing peers. The BalanCI has potential to aid physical/vestibular therapy in children with CIs who have poor balance.


Assuntos
Implante Coclear , Implantes Cocleares , Humanos , Criança , Adulto Jovem , Postura/fisiologia , Movimento , Posição Ortostática , Equilíbrio Postural/fisiologia
20.
JAMA Netw Open ; 6(10): e2339042, 2023 10 02.
Artigo em Inglês | MEDLINE | ID: mdl-37889489

RESUMO

Importance: School closures and other COVID-19-related restrictions could decrease children's exposure to speech during important stages of development. Objective: To assess whether significant decreases in exposure to spoken communication found during the initial phase of the COVID-19 pandemic among children using cochlear implants are confirmed for a larger cohort of children and were sustained over the first years of the COVID-19 pandemic. Design, Setting, and Participants: This cohort study used datalogs collected from children with cochlear implants during clinical visits to a tertiary pediatric hospital in Toronto, Ontario, Canada, from January 1, 2018, to November 11, 2021. Children with severe to profound hearing loss using cochlear implants were studied because their devices monitored and cataloged levels and types of sounds during hourly use per day (datalogs) and because their hearing and spoken language development was particularly vulnerable to reduced sound exposure. Statistical analyses were conducted between January 2022 and August 2023. Main Outcomes and Measures: Daily hours of sound were captured by the cochlear implant datalogging system and categorized into 6 auditory scene categories, including speech and speech-in-noise. Time exposed to speech was calculated as the sum of daily hours in speech and daily hours in speech-in-noise. Residual hearing in the ear without an implant of children with unilateral cochlear implants was measured by pure tone audiometry. Mixed-model regression analyses revealed main effects with post hoc adjustment of 95% CIs using the Satterthwaite method. Results: Datalogs (n = 2746) from 262 children (137 with simultaneous bilateral cochlear implants [74 boys (54.0%); mean (SD) age, 5.8 (3.5 years)], 38 with sequential bilateral cochlear implants [24 boys (63.2%); mean (SD) age, 9.1 (4.2) years], and 87 with unilateral cochlear implants [40 boys (46.0%); mean (SD) age, 7.9 (4.6) years]) who were preschool aged (n = 103) and school aged (n = 159) before the COVID-19 pandemic were included in analyses. There was a slight increase in use among preschool-aged bilateral cochlear implant users through the pandemic (early pandemic, 1.4 h/d [95% CI, 0.3-2.5 h/d]; late pandemic, 2.3 h/d [95% CI, 0.6-4.0 h/d]) and little change in use among school-aged bilateral cochlear implant users (early pandemic, -0.6 h/d [95% CI, -1.1 to -0.05 h/d]; late pandemic, -0.3 h/d [95% CI, -0.9 to 0.4 h/d]). However, use decreased during the late pandemic period among school-aged children with unilateral cochlear implants (-1.8 h/d [95% CI,-3.0 to -0.6 h/d]), particularly among children with good residual hearing in the ear without an implant. Prior to the pandemic, children were exposed to speech for approximately 50% of the time they used their cochlear implants (preschool-aged children: bilateral cochlear implants, 46.6% [95% CI, 46.5%-47.2%] and unilateral cochlear implants, 52.1% [95% CI, 50.7%-53.5%]; school-aged children: bilateral cochlear implants, 47.6% [95% CI, 46.8%-48.4%] and unilateral cochlear implants, 51.0% [95% CI, 49.4%-52.6%]). School-aged children in both groups experienced significantly decreased speech exposure in the early pandemic period (bilateral cochlear implants, -12.1% [-14.6% to -9.4%]; unilateral cochlear implants, -15.5% [-20.4% to -10.7%]) and late pandemic periods (bilateral cochlear implants, -5.3% [-8.0% to -2.6%]; unilateral cochlear implants, -11.2% [-15.3% to -7.1%]) compared with the prepandemic baseline. Conclusions and Relevance: This cohort study using datalogs from children using cochlear implants suggests that a sustained reduction in children's access to spoken communication was found during more than 2 years of COVID-19 pandemic-related lockdowns and school closures.


Assuntos
COVID-19 , Implantes Cocleares , Surdez , Percepção da Fala , Masculino , Criança , Humanos , Pré-Escolar , Pandemias , Surdez/epidemiologia , Surdez/cirurgia , Estudos de Coortes , COVID-19/epidemiologia , Controle de Doenças Transmissíveis , Fala , Ontário/epidemiologia
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