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1.
Trends Hear ; 28: 23312165241264466, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39106413

RESUMO

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/-73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz-20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation.


Assuntos
Estimulação Acústica , Perda Auditiva Condutiva , Perda Auditiva Condutiva-Neurossensorial Mista , Prótese Ossicular , Localização de Som , Humanos , Localização de Som/fisiologia , Feminino , Masculino , Pessoa de Meia-Idade , Perda Auditiva Condutiva/fisiopatologia , Perda Auditiva Condutiva/cirurgia , Perda Auditiva Condutiva/diagnóstico , Perda Auditiva Condutiva/reabilitação , Adulto , Perda Auditiva Condutiva-Neurossensorial Mista/fisiopatologia , Perda Auditiva Condutiva-Neurossensorial Mista/reabilitação , Perda Auditiva Condutiva-Neurossensorial Mista/cirurgia , Perda Auditiva Condutiva-Neurossensorial Mista/diagnóstico , Idoso , Perda Auditiva Bilateral/fisiopatologia , Perda Auditiva Bilateral/reabilitação , Perda Auditiva Bilateral/diagnóstico , Perda Auditiva Bilateral/cirurgia , Resultado do Tratamento , Desenho de Prótese , Sinais (Psicologia) , Adulto Jovem , Limiar Auditivo , Condução Óssea/fisiologia
2.
Int J Pediatr Otorhinolaryngol ; 183: 112031, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39111074

RESUMO

OBJECTIVES: To assess the influence of three factors using retrospective chart review: age at which 2nd cochlear implant (CI) is implanted, prior hearing aid (HA) experience in the 2nd CI ear, and long-term experience with bilateral cochlear implants (BICIs) on sound localization in children with sequential BICIs. METHODS: Mean absolute error (MAE) in localizing speech noise of 60 children with sequential BICIs was compared across four age groups of the 2nd CI (1-5.0; 5.1-10.0; 10.1-14.0; & 14.1-19.0 years) and two extents of prior HA experience (more than and less than one year). MAE was also longitudinally analyzed after 4-6 years of experience with BICI involving 18 participants out of 60. RESULTS: Children who received 2nd CI before five years of age demonstrated significantly better localization than those who received it after ten years of age. More than one year of prior HA experience in the 2nd CI ear and extensive experience with sequential BICIs significantly enhanced localization performance. Inter-implant intervals and age at the 2nd CI showed a significant positive correlation with the MAE (poorer localization). CONCLUSION: The results indicate that age at 2nd CI is important in developing sound localization skills. Based on the results, obtaining 2nd CI within the first five years of life and no later than ten years old is recommended. The results also suggest that longer use of amplification before 2nd CI and prolonged BICI experience significantly fosters localization development.


Assuntos
Implante Coclear , Implantes Cocleares , Localização de Som , Humanos , Localização de Som/fisiologia , Criança , Estudos Retrospectivos , Pré-Escolar , Masculino , Feminino , Implante Coclear/métodos , Fatores Etários , Adolescente , Auxiliares de Audição , Lactente , Adulto Jovem , Percepção da Fala/fisiologia , Fatores de Tempo , Perda Auditiva Bilateral/cirurgia , Resultado do Tratamento
3.
Hear Res ; 450: 109068, 2024 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-38936172

RESUMO

BACKGROUND & RATIONALE: In prior work using non-speech stimuli, children with hearing loss show impaired perception of binaural cues and no significant change in cortical responses to bilateral versus unilateral stimulation. Aims of the present study were to: 1) identify bilateral responses to envelope and spectral components of a speech syllable using the frequency-following response (FFR), 2) determine if abnormalities in the bilateral FFR occur in children with hearing loss, and 3) assess functional consequences of abnormal bilateral FFR responses on perception of binaural timing cues. METHODS: A single-syllable speech stimulus (/dα/) was presented to each ear individually and bilaterally. Participants were 9 children with normal hearing (MAge = 12.1 ± 2.5 years) and 6 children with bilateral hearing loss who were experienced bilateral hearing aid users (MAge = 14.0 ± 2.6 years). FFR temporal and spectral peak amplitudes were compared between listening conditions and groups using linear mixed model regression analyses. Behavioral sensitivity to binaural cues were measured by lateralization responses as coming from the right or left side of the head. RESULTS: Both temporal and spectral peaks in FFR responses increased in amplitude in the bilateral compared to unilateral listening conditions in children with normal hearing. These measures of "bilateral advantage" were reduced in the group of children with bilateral hearing loss and associated with decreased sensitivity to interaural timing differences. CONCLUSION: This study is the first to show that bilateral responses in both temporal and spectral domains can be measured in children using the FFR and is altered in children with hearing loss with consequences to binaural hearing.


Assuntos
Estimulação Acústica , Sinais (Psicologia) , Percepção da Fala , Humanos , Criança , Masculino , Feminino , Adolescente , Estudos de Casos e Controles , Perda Auditiva Bilateral/fisiopatologia , Perda Auditiva Bilateral/psicologia , Perda Auditiva Bilateral/diagnóstico , Auxiliares de Audição , Fatores de Tempo , Pessoas com Deficiência Auditiva/psicologia , Fatores Etários , Localização de Som , Audição , Acústica da Fala , Potenciais Evocados Auditivos , Eletroencefalografia
4.
Otol Neurotol ; 45(7): 717-731, 2024 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-38896785

RESUMO

OBJECTIVE: To determine the cost-effectiveness or cost-utility of cochlear implants (CI) in adults with severe to profound bilateral hearing loss. DATABASES REVIEWED: PubMed (Medline), The Cochrane Library, Embase via Elsevier, EBSCOhost CINAHL, and Scopus. METHODS: The study included adult participants with severe to profound bilateral neurosensory hearing loss. The analysis encompassed quality of life improvements, costs, cost-effectiveness, cost-utility, cost-benefit, quality-adjusted life year (QALY), incremental cost-effectiveness ratio (ICER), and incremental cost-utility ratio (ICUR). Systematic reviews, meta-analyses, case series, and prospective or retrospective cohort studies published in English between 2010 and 2023 were retrieved. Exclusion criteria included incomplete studies, abstracts, clinical cases, editorials, letters, studies involving pediatric populations, single-side deafness, methodology research, noneconomic aspects of CI, mixed child and adult data, and studies published before 2010. The risk of bias was assessed following the criteria outlined in Appendix I of the economic evaluation's quality assessment as per the NICE Guideline Development Method. RESULTS: Ten articles met the criteria and were included in the qualitative synthesis. One study conducted a prospective cost-utility analyses, one carried out a cost-benefit analyses, one was a randomized controlled clinical trial focusing on cost-utility, and another was a clinical trial addressing cost-effectiveness. Six studies employed Markov models, and one study utilized uniquely the Monte Carlo method. None quantified the economic impact of improved hearing on cognitive function. CONCLUSIONS: The heterogeneity of sources impacted data quality. Unilateral and sequential bilateral CI appeared to be cost-effective when compared with bilateral hearing aids or nontechnological support. When properly indicated, simultaneous bilateral CIs are cost-effective compared to no interventions and to unilateral cochlear implantation through differential discounting or variations from the base cases, especially with a life expectancy of 5-10 years or longer.


Assuntos
Implante Coclear , Implantes Cocleares , Análise Custo-Benefício , Perda Auditiva Bilateral , Humanos , Implantes Cocleares/economia , Implante Coclear/economia , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Bilateral/economia , Adulto , Qualidade de Vida , Anos de Vida Ajustados por Qualidade de Vida
6.
JAMA Netw Open ; 7(5): e2412846, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38780940

RESUMO

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.


Assuntos
Doenças Vestibulares , Humanos , Feminino , Masculino , Pré-Escolar , Doenças Vestibulares/epidemiologia , Doenças Vestibulares/complicações , Criança , Perda Auditiva Bilateral/epidemiologia , Implante Coclear , Estudos de Coortes , Equilíbrio Postural/fisiologia , Testes de Função Vestibular , Prevalência , Paris/epidemiologia , Lactente
7.
Trends Hear ; 28: 23312165241256721, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38773778

RESUMO

This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3-5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.


Assuntos
Linguagem Infantil , Auxiliares de Audição , Perda Auditiva Bilateral , Desenvolvimento da Linguagem , Humanos , Masculino , Pré-Escolar , Feminino , Perda Auditiva Bilateral/reabilitação , Perda Auditiva Bilateral/diagnóstico , Perda Auditiva Bilateral/fisiopatologia , Perda Auditiva Bilateral/psicologia , Percepção da Fala , Estudos de Casos e Controles , Correção de Deficiência Auditiva/instrumentação , Resultado do Tratamento , Pessoas com Deficiência Auditiva/reabilitação , Pessoas com Deficiência Auditiva/psicologia , Índice de Gravidade de Doença , Compreensão , Audição , Audiometria de Tons Puros , Fatores Etários , Limiar Auditivo , Testes de Linguagem
9.
Neurol Sci ; 45(9): 4593-4596, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38676820

RESUMO

BACKGROUND: Wernicke's encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings. CASE REPORT: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution. CONCLUSION: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.


Assuntos
Corpo Caloso , Perda Auditiva Bilateral , Encefalopatia de Wernicke , Humanos , Masculino , Encefalopatia de Wernicke/diagnóstico por imagem , Encefalopatia de Wernicke/complicações , Encefalopatia de Wernicke/diagnóstico , Encefalopatia de Wernicke/tratamento farmacológico , Adulto , Corpo Caloso/diagnóstico por imagem , Corpo Caloso/patologia , Perda Auditiva Bilateral/etiologia , Tiamina/uso terapêutico , Tiamina/administração & dosagem , Complexo Vitamínico B/administração & dosagem , Complexo Vitamínico B/uso terapêutico , Imageamento por Ressonância Magnética , Imagem de Difusão por Ressonância Magnética
10.
Am J Otolaryngol ; 45(4): 104331, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38677147

RESUMO

OBJECTIVE: To compare changes in music appreciation after cochlear implant (CI) surgery for patients with bilateral and single-sided deafness (SSD). METHODS: A retrospective cohort study was performed on all adult CI unilateral or bilateral recipients from November 2019 to March 2023. Musical questionnaire subset data from the Cochlear Implant Quality of Life (CIQOL) - 35 Profile Instrument Score (maximum raw score of 15) was collected. Functional CI assessment was measured with CI-alone speech-in-quiet (SIQ) scores (AzBio and CNC). RESULTS: 22 adults underwent CI surgery for SSD and 21 adults for bilateral deafness (8 sequentially implanted). Every patient group had clinically significant improvements (p < 0.001) in mean SIQ scores in the most recently implanted ear (Azbio (% correct) SSD: 14.23 to 68.48, bilateral: 24.54 to 82.23, sequential: 6.25 to 82.57). SSD adults on average had higher music QOL scores at baseline (SSD: 11.05; bilateral: 7.86, p < 0.001). No group had significant increases in raw score at the first post-operative visit (SSD: 11.45, p = 0.86; bilateral: 8.15, p = 0.15). By the most recent post-implantation evaluation (median 12.8 months for SSD, 12.3 months for bilateral), SSD adults had a significant increase in raw score from baseline (11.05 to 12.45, p = 0.03), whereas bilaterally deafened (7.86 to 9.38, p = 0.12) adults had nonsignificant increases. CONCLUSIONS: SSD patients demonstrate higher baseline music appreciation than bilaterally deafened individuals regardless of unilateral or bilateral implantation and are more likely to demonstrate continued improvement in subjective music appreciation at last follow-up even when speech perception outcomes are similar.


Assuntos
Implante Coclear , Implantes Cocleares , Música , Qualidade de Vida , Humanos , Música/psicologia , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Implante Coclear/métodos , Idoso , Adulto , Inquéritos e Questionários , Surdez/cirurgia , Surdez/psicologia , Surdez/reabilitação , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Bilateral/reabilitação , Perda Auditiva Bilateral/psicologia , Resultado do Tratamento
11.
J Int Adv Otol ; 20(1): 89-93, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38454296

RESUMO

Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Pré-Escolar , Humanos , Masculino , Cóclea/cirurgia , Implante Coclear/efeitos adversos , Implante Coclear/métodos , Implantes Cocleares/efeitos adversos , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/cirurgia , Solução Salina , Telemetria/métodos
13.
Ear Nose Throat J ; 103(1_suppl): 130S-133S, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38488157

RESUMO

Viruses are often implicated as a cause of sensorineural hearing loss (SNHL), particularly sudden cases, including COVID-19. Determining the viral mechanism that leads to hearing loss is necessary for its future prevention and treatment. The 47-year-old woman who is the subject of this case study presented with sudden SNHL following multiple infections of COVID-19. Following a trial of a contralateral routing of sound device, she received a right cochlear implant (CI). Following a period of high performance, additional cases of COVID-19 infection and device failure issues resulted in the explant/reimplant of 1 ear and implantation of the contralateral ear. Despite extensive rehabilitation after these events, the patient continues to experience difficulties in speech understanding, not reaching her initial high levels of right ear performance. Further research is needed to determine the implications of COVID-19 as it relates to SNHL. This case study aimed to highlight the course of treatment and provide insight into the impact of COVID-19 on sudden hearing loss and its relationship to CI performance.


Assuntos
COVID-19 , Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Feminino , COVID-19/complicações , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/virologia , Pessoa de Meia-Idade , Implantes Cocleares/efeitos adversos , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/cirurgia , SARS-CoV-2 , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Bilateral/etiologia
14.
Laryngoscope ; 134(9): 3883-3891, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38506449

RESUMO

OBJECTIVES: To comprehensively examine the characteristics and prognosis of bilateral sudden sensorineural hearing loss (BSSHL) and its subtypes compared to unilateral sudden sensorineural hearing loss (USSHL). DATA SOURCES: PubMed, Scopus, and CINAHL. REVIEW METHODS: Databases were searched from inception to December 5, 2023, for studies reporting patient characteristics and audiometric outcomes for BSSHL and its simultaneous (Si-BSSHL) and sequential (Se-BSSHL) subtypes. Meta-analysis of continuous measures, proportions (%), mean differences (Δ), and odds ratio (OR) were performed. RESULTS: Eleven studies were included, consisting of 368 patients with BSSHL and 2,705 patients with USSHL. The pooled prevalence among all SSHL cases was 88.1% (95% CI: 81.2%-93.6%) for USSHL and 11.9% (95% CI: 6.4% to 18.8%) for BSSHL. PTA improvement following treatment with steroids was significantly worse in patients with BSSHL (Δ15.3 dB; 95% CI: 14.6 to 15.9; p < 0.0001) compared to patients with USSHL. There was no significant difference in post-treatment PTA improvement between the BSSHL subtypes. Patients with Si-BSSHL were significantly less likely to have an idiopathic etiology (OR: 0.4; 95% CI: 0.2 to 0.8; p = 0.01) and significantly more likely to have an autoimmune disease etiology (OR: 27.4; 95% CI: 2.2 to 336.1; p = 0.01), comorbid cardiovascular disease (OR: 2.3; 95% CI: 1.1 to 5.1; p = 0.03), and comorbid hypertension (OR: 2.5; 95% CI: 1.6 to 3.8; p < 0.0001) compared to patients with USSHL. CONCLUSIONS: BSSHL is a considerably rarer form of SSHL with worse prognosis compared to USSHL. BSSHL, and Si-BSSHL in particular, has significantly greater associations with systemic pathologies compared to USSHL. Laryngoscope, 134:3883-3891, 2024.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/diagnóstico , Prognóstico , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/epidemiologia , Perda Auditiva Bilateral/epidemiologia , Perda Auditiva Bilateral/diagnóstico , Masculino , Feminino , Prevalência
15.
Am J Audiol ; 33(2): 321-329, 2024 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-38466937

RESUMO

PURPOSE: The purpose of this study was to evaluate the potential contribution of limited English proficiency on daily hearing aid wear time for children with hearing loss. METHOD: A retrospective chart review was completed to evaluate hearing aid wear time based on data logging information available at the time of a follow-up visit following an initial hearing aid fitting. Children were included in the study if they had permanent bilateral hearing loss and were less than 60 months of age at the time of their first follow-up visit. Wear time was compared between children who attended an interpreter-mediated appointment and those who did not have an interpreter present. The presence of an interpreter at the appointment was the study indicator that the family had limited English proficiency. RESULTS: Children from families with limited English proficiency exhibited significantly shorter daily wear time (M = 1.3 hr) than their peers whose families were English-proficient speakers, thus, having a shared language with their audiologists (M = 5.2 hr). CONCLUSIONS: Results of this study suggest that family-clinician language discordance might put children at greater risk of shorter hearing aid wear time than children whose caregivers share a common language with their child's audiologist. There can be many linguistic, cultural, and educational factors that contribute to hearing aid wear time in children whose families have limited English proficiency as well as different approaches to improving that wear time. Efforts should ensure that hearing and hearing aid-related information is accessible to all families, especially those with clinician-family language discordance. Such efforts can include, among others, training that improves clinicians' cultural and linguistic responsiveness to the diverse families they serve.


Assuntos
Auxiliares de Audição , Humanos , Estudos Retrospectivos , Feminino , Masculino , Pré-Escolar , Lactente , Fatores de Tempo , Proficiência Limitada em Inglês , Perda Auditiva Bilateral/reabilitação
16.
J Int Adv Otol ; 20(1): 85-88, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38454295

RESUMO

The gene MED13 participates in transcription. The MED13L gene is a paralog of MED13 that is involved in developmental gene expression. Mutations in the gene have been shown to result in a heterogenous phenotype affecting several physiological systems. Hearing loss has been reported very rarely, and vestibular weakness has never been reported in the condition. In this report, we present a mutation of MED13L in c.1162A > T (p.Arg388Ter), where we detail and describe a cochleovestibular phenotype with objective vestibulometry for the first time. The child showed bilateral sloping sensorineural hearing loss, a bilateral vestibular weakness, and an inner ear vestibular structural abnormality on imaging. Early intervention with hearing aids and vestibular rehabilitation led to a favorable outcome in terms of speech, communication, and balance. We emphasize the importance of comprehensive audiovestibular assessment in children diagnosed with MED13L mutations for effective management of these children.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva , Vestíbulo do Labirinto , Criança , Humanos , Perda Auditiva Bilateral , Perda Auditiva Neurossensorial/genética , Complexo Mediador/genética , Mutação , Fenótipo
17.
Trends Hear ; 28: 23312165241229572, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38347733

RESUMO

Subjective reports indicate that hearing aids can disrupt sound externalization and/or reduce the perceived distance of sounds. Here we conducted an experiment to explore this phenomenon and to quantify how frequently it occurs for different hearing-aid styles. Of particular interest were the effects of microphone position (behind the ear vs. in the ear) and dome type (closed vs. open). Participants were young adults with normal hearing or with bilateral hearing loss, who were fitted with hearing aids that allowed variations in the microphone position and the dome type. They were seated in a large sound-treated booth and presented with monosyllabic words from loudspeakers at a distance of 1.5 m. Their task was to rate the perceived externalization of each word using a rating scale that ranged from 10 (at the loudspeaker in front) to 0 (in the head) to -10 (behind the listener). On average, compared to unaided listening, hearing aids tended to reduce perceived distance and lead to more in-the-head responses. This was especially true for closed domes in combination with behind-the-ear microphones. The behavioral data along with acoustical recordings made in the ear canals of a manikin suggest that increased low-frequency ear-canal levels (with closed domes) and ambiguous spatial cues (with behind-the-ear microphones) may both contribute to breakdowns of externalization.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Localização de Som , Percepção da Fala , Adulto Jovem , Humanos , Fala , Perda Auditiva Bilateral , Ruído , Percepção da Fala/fisiologia
18.
Cochlear Implants Int ; 25(2): 147-153, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38408736

RESUMO

OBJECTIVE AND METHOD: Skin breakdown over receiver/stimulator (RS) after cochlear implantation poses a serious challenge. We report our experience using a one-stage reconstruction and implant salvage approach. RESULTS: Between the years 2005 and 2017 five children, all females, with congenital- bilateral sensorineural hearing loss were identified. In all cases, a temporoparietal fascia flap (TPFF) and a large scalp flap were used to provide a two-layer coverage to the exposed RS. In the first three cases, a split-thickness skin graft was used to cover the donor site defect. In the latter two cases, a larger rotation flap was used, and a skin graft was not required. One case required revision due to the dehiscence of the wound and exposure of the RS. In another case, an accidental electrode array explantation occurred and the patient underwent a revision cochlear implantation. All patients had achieved complete healing and no change in hearing thresholds with the implants. CONCLUSIONS: We demonstrate our one-stage salvage technique with TPFF that saves the implant and prevents a two-stage procedure. The success rate can be improved with special care at reconstruction and with better protection of the implant during the procedure.


Assuntos
Implante Coclear , Implantes Cocleares , Terapia de Salvação , Retalhos Cirúrgicos , Humanos , Feminino , Implantes Cocleares/efeitos adversos , Implante Coclear/efeitos adversos , Pré-Escolar , Criança , Terapia de Salvação/métodos , Perda Auditiva Neurossensorial/cirurgia , Transplante de Pele/métodos , Reoperação/métodos , Deiscência da Ferida Operatória/cirurgia , Deiscência da Ferida Operatória/etiologia , Resultado do Tratamento , Lactente , Estudos Retrospectivos , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Bilateral/etiologia
19.
BMJ Case Rep ; 17(2)2024 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-38413140

RESUMO

Rare causes of stroke-like presentations can be difficult to diagnose. We report a case of a man in his 40s who first presented with stroke symptoms, but whose clinical course was not typical for a stroke. A detailed investigation of the patient's medical history revealed bilateral sensorineural hearing loss which prompted a wider diagnostic assessment.Furthermore, lack of vascular risk factors and a normal angiogram strengthened our suspicion of an unusual underlying condition. Raised lactic acid levels and genetic analysis confirmed a diagnosis of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome.


Assuntos
Acidose Láctica , Perda Auditiva Bilateral , Síndrome MELAS , Acidente Vascular Cerebral , Adulto , Humanos , Masculino , Acidose Láctica/diagnóstico , Ácido Láctico , Síndrome MELAS/complicações , Síndrome MELAS/diagnóstico , Síndrome MELAS/genética , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia
20.
Ear Hear ; 45(4): 905-914, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38351509

RESUMO

OBJECTIVE: The process of adapting to communicate with a cochlear implant (CI) is complex. The use of auditory training after cochlear implantation may help to facilitate improvements in postoperative speech recognition and quality-of-life outcomes in new adult CI recipients. However, the effectiveness of auditory training remains uncertain and long-term effects have not been examined in a large sample of new adult CI users. As such, the objective of this study was to examine the influence of common forms of auditory training on speech recognition and CI-related quality-of-life (CI-related QOL) outcomes at 1 year after cochlear implantation. We hypothesized that patients who reported use of computer-based auditory training (CBAT) would show improved speech and CIQOL-35 Profile scores at 1 year after activation of their implant, compared with their peers. DESIGN: This study was designed as a prospective study and was undertaken at a tertiary academic CI center. Participants included 114 adults undergoing cochlear implantation for bilateral hearing loss. Patients serially self-reported use of the following types of post-CI auditory training over their first-year postactivation: (1) face-to-face training (e.g., speech-language pathologist), (2) passive home-based training (e.g., listening to audiobooks), and (3) CBAT (e.g., self-directed software). Outcomes measures for this study included change in Consonant-Nucleus-Consonant phoneme (CNCp), CNC word (CNCw), AzBio sentences in quiet, and CIQOL-35 Profile global and domain scores from pre-CI to 12-mo post-CI. RESULTS: Of 114 patients, 94 (82.5%) used one or more auditory training resources. Of these, 19.3% used face-to-face training, 67.5% passive home-based training, and 46.5% CBAT. Of 114 patients, 73 had complete CIQOL data. At 12 mo, only CBAT use was associated with significantly greater improvements in global and all domain-specific CIQOL scores ( d -range = 0.72-0.87), compared with those not using CBAT. Controlling for demographics and use of multiple training resources, CBAT remained the strongest positive predictor of CIQOL improvement, with significant associations with global score (ß = 12.019[4.127,19.9]) and all domain scores at 12-mo post-CI: communication (ß = 11.937[2.456,21.318), emotional (ß = 12.293[1.827,22.759), entertainment (ß = 17.014[5.434,28.774), environment (ß = 13.771[1.814,25.727]), listening effort (ß = 12.523[2.798,22.248]), and social (ß = 18.114[7.403,28.826]). No significant benefits were noted with use of CBAT or any other form of auditory training and speech recognition scores at 12-mo post-CI ( d -range = -0.12-0.22). CONCLUSIONS: Auditory training with CBAT was associated with improved CI-related QOL outcomes at 12-mo post-CI. Given its availability and low cost, this study provides evidence to support using CBAT to improve real-world functional abilities in new adult CI recipients.


Assuntos
Implante Coclear , Implantes Cocleares , Qualidade de Vida , Percepção da Fala , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Adulto , Estudos Prospectivos , Perda Auditiva Bilateral/reabilitação , Idoso de 80 Anos ou mais , Terapia Assistida por Computador
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