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OBJECTIVES: The aim of the present study was to investigate whether prediction models built by de Graaff et al. (2020 ) can be used to improve speech recognition in experienced adult postlingual implanted Cochlear CI users. de Graaff et al. (2020 ) found relationships between elevated aided thresholds and a not optimal electrical dynamic range (<50 CL or >60 CL), and poorer speech recognition in quiet and in noise. The primary hypothesis of the present study was that speech recognition improves both in quiet and in noise when the sound processor is refitted to match targets derived from the prediction models from de Graaff et al. (2020 ). A second hypothesis was that subjectively, most of the CI users would find the new setting too loud because of an increase in C levels, and therefore, prefer the old settings. DESIGN: A within-participant repeated measures design with 18 adult Cochlear CI users was used. T- and C-levels were changed to "optimized settings," as predicted by the model of de Graaff et al. (2020 ). Aided thresholds, speech recognition in quiet, and speech recognition in noise were measured with the old settings and after a 4-week acclimatization period with the optimized settings. Subjective benefit was measured using the Device Oriented Subjective Outcome Scale questionnaire. RESULTS: The mean electrical dynamic range changed from 41.1 (SD = 6.6) CL to 48.6 (SD = 3.0) CL. No significant change in aided thresholds was measured. Speech recognition improved for 16 out of 18 participants and remained stable for 2 participants. Average speech recognition scores in quiet significantly improved by 4.9% (SD = 3.8%). No significant change for speech recognition in noise was found. A significant improvement in subjective benefit was found for one of the Device Oriented Subjective Outcome subscales (speech cues) between the old and optimized settings. All participants chose to keep the optimized settings at the end of the study. CONCLUSIONS: We were able to improve speech recognition in quiet by optimizing the electrical dynamic range of experienced adult CI users, according to the prediction models built by de Graaff et al. (2020 ). There was no significant change in aided thresholds nor in speech recognition in noise. The findings of the present study suggest that improved performance for speech recognition in quiet in adult Cochlear CI users can be achieved by setting the dynamic range as close as possible to values between 50 and 60 CL when the volume level is at 10.
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Implante Coclear , Implantes Cocleares , Ruído , Percepção da Fala , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Idoso , Adulto , Surdez/reabilitação , Limiar Auditivo , Ajuste de PróteseRESUMO
OBJECTIVE: The aim of the current study was to investigate the use of manually and automatically switching programs in everyday day life by adult cochlear implant (CI) users. DESIGN: Participants were fitted with an automatically switching sound processor setting and 2 manual programs for 3-week study periods. They received an extensive counselling session. Datalog information was used to analyse the listening environments identified by the sound processor, the program used and the number of program switches. STUDY SAMPLES: Fifteen adult Cochlear CI users. Average age 69 years (range: 57-85 years). RESULTS: Speech recognition in noise was significantly better with the "noise" program than with the "quiet" program. On average, participants correctly classified 4 out of 5 listening environments in a laboratory setting. Participants switched, on average, less than once a day between the 2 manual programs and the sound processor was in the intended program 60% of the time. CONCLUSION: Adult CI users switch rarely between two manual programs and leave the sound processor often in a program not intended for the specific listening environment. A program that switches automatically between settings, therefore, seems to be a more appropriate option to optimise speech recognition performance in daily listening environments.
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INTRODUCTION: There is an increased demand for well-validated PROMs in otology. This study will systematically assess the methodological quality of all published patient-reported outcome measures (PROMS) for patients with multiple ear complaints and to identify the best suitable PROM for use by clinicians treating patients with multiple ear complaints. METHODS: An extensive systematic mapping review of all otology questionnaires was performed to identify questionnaires measuring multiple ear complaints. The 'Consensus-based standards for the selection of health measurement Instruments' (COSMIN) checklists were used to evaluate the quality of the questionnaire by two researchers. The worst item score per aspect of the methodological assessment counted. RESULTS: Twelve multiple-complaint questionnaires were included in the study for quality assessment. Ten questionnaires were disease-specific (COMQ-12, CES, ZCMEI-21, MD-POSI, PAN-QOL, ETDQ-7, MDOQ, GYSSCDQ, COMOT-15 and DEU-MDDS). Two questionnaires were ear domain-specific (OQUA and COQOL). The majority of multiple complaint questionnaires lacked good design with concept elicitation and patient involvement. CONCLUSION: For the majority of questionnaires, the quality assessment was inadequate as only a few authors consulted with patients affected by the complaints in the development. Modifications of earlier versions of PROMS or combinations of multiple questionnaires lead to ongoing (cross-cultural) validation of these questionnaires albeit mediocre design and validation. The two domain-specific questionnaires are the COQOL and OQUA, both with adequate quality but different focus. COQOL to quantify the quality of life and OQUA to measure and evaluate the severity and impact of ear complaints. [Correction added on 26 January 2024, after first online publication: In the preceding sentence, the spelling of the abbreviation COQOL has been corrected in this version.].
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Otopatias , Medidas de Resultados Relatados pelo Paciente , Humanos , Otopatias/diagnóstico , Otopatias/terapia , Inquéritos e Questionários , Qualidade de VidaRESUMO
OBJECTIVES: Tinnitus is highly prevalent, but only a few risk factors for developing tinnitus are known and little is known about factors associated with the degree of annoyance of new-onset tinnitus. Longitudinal analysis can reveal risk factors associated with the development of tinnitus and might lead to targeted prevention. The aim of this study is twofold. (1) To identify risk factors that are longitudinally associated with the odds of developing tinnitus 5 years later. (2) To identify factors that are cross-sectionally associated with tinnitus annoyance in adults with new-onset tinnitus. METHODS: Baseline, 5-year, and 10-year follow-up data of participants in the Netherlands Longitudinal Study on Hearing (NL-SH) were used. The NL-SH is a web-based prospective cohort study, which started in 2006 and includes both normal hearing and hearing-impaired adults aged 18 to 70 years at baseline. The NL-SH uses an online digit-triplet speech-in-noise test to asses speech recognition ability in noise, and online questionnaires on various aspects of life. At follow-up, participants are asked (1) if they suffer from tinnitus and (2) to rate tinnitus annoyance on a 0 to 100 numeric rating scale. We investigated whether demographic (age, sex, living arrangement, educational level), lifestyle (history of tobacco smoking, alcohol use), health (asthma, severe heart disease, hypertension, history of stroke, osteoarthritis, rheumatoid arthritis, epilepsy, multiple sclerosis, and migraine), hearing (speech recognition ability in noise, hyperacusis, and occupational noise exposure), and psychological variables (distress, somatization, depression, and anxiety) were potential risk factors for new-onset tinnitus, or associated with annoyance caused by new-onset tinnitus. Generalized estimating equations were used to longitudinally analyze the association between potential risk factors and new-onset tinnitus measured 5 years later. A multivariable association model was constructed using a forward selection procedure with p < 0.05 for model entry. Linear regression analysis was used to cross-sectionally analyze the association between potential factors and tinnitus annoyance in new-onset tinnitus. For this purpose, a multivariable association model was constructed using a forward selection procedure with p <0.05 for model entry. RESULTS: In total, 734 participants without tinnitus at baseline were included, from which 137 participants reported to suffer from new-onset tinnitus 5 or 10 years later. Risk factors for new-onset tinnitus were history of smoking (odds ratio 1.5, 95% confidence interval [CI] 1.0 to 2.2, p = 0.027) and higher levels of somatization (odds ratio 2.0, 95% CI 1.2 to 3.3, overall p = 0.024). Factors associated with the degree of tinnitus annoyance were increased levels of anxiety (ß = 11.6, 95% CI 2.3-20.8, overall p = 0.035) and poor speech recognition ability in noise (ß = 13.5, 95% CI, 4.4 to 22.6, overall p = 0.014). CONCLUSIONS: Higher levels of somatization and a history of smoking were found to be risk factors for new-onset tinnitus 5 years later. Anxiety and poor speech recognition ability in noise were associated with higher degrees of tinnitus annoyance in new-onset tinnitus. Somatization deserves to be addressed in future research and clinical practice as it might provide part of a model for the development of chronic tinnitus.
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Perda Auditiva , Zumbido , Adulto , Humanos , Zumbido/epidemiologia , Estudos Longitudinais , Estudos Prospectivos , Hiperacusia/epidemiologia , Perda Auditiva/psicologiaRESUMO
INTRODUCTION: Risk factors for cardiovascular disease (CVD) are associated with sensorineural hearing loss. CVD risk factors are known to cluster and interact, thereby increasing the cumulative risk for CVD. Previously, using the database of the Netherlands Longitudinal Study on Hearing (NL-SH), an association was found between a history of smoking and an increased decline in speech recognition in noise over 10 years of follow-up. Prospectively limited data are available on the association between CVD risk factors, interactions of these risk factors, and hearing loss. In this study, data from the NL-SH were used to study the association between CVD risk factors and speech recognition in noise longitudinally. METHODS: Baseline, 5-year, and 10-year follow-up data of the NL-SH were included. The NL-SH is a web-based prospective cohort study which started in 2006. Participants were aged 18-70 years at baseline. Speech recognition in noise was determined with an online digit-triplet speech-in-noise test. In addition, participants completed online questionnaires on demographic, lifestyle, and health-related characteristics. The association of the ability to recognize speech in noise with CVD risk factors (i.e., obesity, rheumatoid arthritis [RA], hypertension, diabetes mellitus, and dyslipidemia) was analyzed longitudinally. We also analyzed the interaction between these risk factors (including age, sex, and history of smoking) and speech recognition in noise. RESULTS: None of the CVD risk factors or interactions of 2 CVD risk factors was significantly associated with a decline in SRT over time. Obesity (p = 0.016), RA (p = 0.027), and hypertension (p = 0.044) were associated with overall higher (more unfavorable) SRTs. No overall interactions between CVD risk factors were found. CONCLUSION: Obesity, RA, and hypertension were overall associated with a higher SRT, but no longitudinal associations between these or other CVD factors with SRTs were found. Also, no interactions between 2 CVD risk factors and SRTs were found. Although no longitudinal associations between CVD risk factors and decline in SRTs were found, clinicians should be alert about the concurrent association between CVD risk factors and hearing loss.
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Doenças Cardiovasculares , Percepção da Fala , Doenças Cardiovasculares/epidemiologia , Humanos , Estudos Longitudinais , Estudos Prospectivos , Fatores de Risco , FalaRESUMO
BACKGROUND: To compare cholesteatoma care internationally and to evaluate outcomes, ear surgeons must use the same terminology. However, a clear universal definition on how to describe the extension, destruction and accompanying morbidity caused by the cholesteatoma is lacking. The practical applicability by means of interrater agreement is assessed for the STAMCO and the ChOLE classification. METHODS: A total of 134 adult patients derived from the nationwide multicentre study in the Netherlands, entitled Dutch Cholesteatoma Data (DCD) were included. Retrospective analysis of 134 surgical reports according to the STAMCO and ChOLE classification for localisation/extension of the cholesteatoma, complication status and ossicular chain status. Both the percentage agreement and the interrater agreement were determined for each item of the classifications and interrater agreement was compared between the classifications as a whole. RESULTS: Differences in interrater agreement were found for both the localisation/extension of the cholesteatoma and ossicular chain status. STAMCO classification derived from the surgical report scored better on the localisation/extension of the cholesteatoma, whereas the ChOLE classification derived from the surgical report scored better on the status of the ossicular chain. In both classifications, complication status had a low agreement level but was also poorly registered in the surgical reports. CONCLUSION: Both STAMCO and ChOLE will be beneficial in uniform registration of cholesteatoma pathology in practice. Modifications proposed for both classifications may make them even more practical applicable in the future. A common denominator obtained from these two classifications may be incorporated in a standardised surgical report to facilitate evaluation which make outcomes transferable towards both classifications.
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Colesteatoma da Orelha Média , Colesteatoma , Adulto , Colesteatoma/diagnóstico , Colesteatoma/cirurgia , Colesteatoma da Orelha Média/cirurgia , Ossículos da Orelha , Humanos , Países Baixos , Estudos RetrospectivosRESUMO
PURPOSE: Differences in the definition and classification of cholesteatoma hinders comparing of surgical outcomes of cholesteatoma. Uniform registration is necessary to allow investigators to share and compare their findings. For many years surgical cholesteatoma procedures were divided into two main groups: canal wall up mastoidectomy (CWU) and canal wall down mastoidectomy (CWD). Recently, mastoid obliteration can be added to both procedures. Because of great variation within these main groups, the International Otology Outcome Group (IOOG) proposed the new SAMEO-ATO classification system to categorize tympanomastoid operations. The aim of our study was to correlate the mastoid bone extirpation (M-stage) with the contemporary (CWU, CWD with or without obliteration) system. METHODS: Demographic characteristics and type of performed surgery were registered for 135 cholesteatoma patients from sixteen hospitals, both secondary and tertiary care institutions, across the Netherlands. In addition, the surgical reports were collected, retrospectively classified according to the contemporary system and the new system and compared. Correlations of the outcomes were calculated. RESULTS: In total, there were 112 CWU and 14 CWD (both with or without obliteration) suitable for correlation analysis. Z test for correlation between the M-stage and CWU procedure was significant for M1a and M1b procedure and significant for M2c with the CWD procedure. CONCLUSION: The newly proposed SAMEO-ATO classification seems to be more detailed in the registration of surgical procedures than surgeons currently are used to. All M-stages of the SAMEO-ATO system are correlating well to the standard CWU and CWD except one 'in between' M-stage.
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Procedimentos Cirúrgicos Otológicos , Colesteatoma da Orelha Média/epidemiologia , Colesteatoma da Orelha Média/cirurgia , Humanos , Processo Mastoide/diagnóstico por imagem , Processo Mastoide/cirurgia , Países Baixos/epidemiologia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: According to the International Classification of Functioning, Disability and Health (ICF), functioning reflects the interplay between an individual's body structures and functions, activities, participation, environmental, and personal factors. To be useful in clinical practice, these concepts need to be operationalized into a practical and integral instrument. The Brief ICF Core Set for Hearing Loss (CSHL) provides a minimum standard for the assessment of functioning in adults with hearing loss. The objective of the present study was to operationalize the Brief CSHL into a digital intake tool that could be used in the otology-audiology practice for adults with ear and hearing problems as part of their intake assessment. DESIGN: A three-step approach was followed: (1) Selecting and formulating questionnaire items and response formats, using the 27 categories of the Brief CSHL as a basis. Additional categories were selected based on relevant literature and clinical expertise. Items were selected from existing, commonly used disease-specific questionnaires, generic questionnaires, or the WHO's official descriptions of ICF categories. The response format was based on the existing item's response categories or on the ICF qualifiers. (2) Carrying out an expert survey and a pilot study (using the three-step test interview. Relevant stakeholders and patients were asked to comment on the relevance, comprehensiveness, and comprehensibility of the items. Results were discussed in the project group, and items were modified based on consensus. (3) Integration of the intake tool into a computer-based system for use in clinical routine. RESULTS: The Brief CSHL was operationalized into 62 items, clustered into six domains: (1) general information, including reason for visit, sociodemographic, and medical background; (2) general body functions; (3) ear and hearing structures and functions; (4) activities and participation (A&P); (5) environmental factors (EF); and (6) personal factors (mastery and coping). Based on stakeholders' responses, the instructions of the items on A&P and EF were adapted. The three-step test interview showed that the tool had sufficient content validity but that some items on EF were redundant. Overall, the stakeholders and patients indicated that the intake tool was relevant and had a logical and clear structure. The tool was integrated in an online portal. CONCLUSIONS: In the current study, an ICF-based e-intake tool was developed that aims to screen self-reported functioning problems in adults with an ear/hearing problem. The relevance, comprehensiveness, and comprehensibility of the originally proposed item list was supported, although the stakeholder and patient feedback resulted into some changes of the tool on item-level. Ultimately, the functioning information obtained with the tool could be used to promote patient-centered ear and hearing care taking a biopsychosocial perspective into account.
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Audiologia , Perda Auditiva , Otolaringologia , Atividades Cotidianas , Adulto , Avaliação da Deficiência , Humanos , Classificação Internacional de Funcionalidade, Incapacidade e Saúde , Projetos PilotoRESUMO
OBJECTIVES: The objective of this study was to identify parameters which are related to speech recognition in quiet and in noise of cochlear implant (CI) users. These parameters may be important to improve current fitting practices. DESIGN: Adult CI users who visited the Amsterdam UMC, location VUmc, for their annual follow-up between January 2015 and December 2017 were retrospectively identified. After applying inclusion criteria, the final study population consisted of 138 postlingually deaf adult Cochlear CI users. Prediction models were built with speech recognition in quiet and in noise as the outcome measures, and aided sound field thresholds, and parameters related to fitting (i.e., T and C levels, dynamic range [DR]), evoked compound action potential thresholds and impedances as the independent variables. A total of 33 parameters were considered. Separate analyses were performed for postlingually deafened CI users with late onset (LO) and CI users with early onset (EO) of severe hearing impairment. RESULTS: Speech recognition in quiet was not significantly different between the LO and EO groups. Speech recognition in noise was better for the LO group compared with the EO group. For CI users in the LO group, mean aided thresholds, mean electrical DR, and measures to express the impedance profile across the electrode array were identified as predictors of speech recognition in quiet and in noise. For CI users in the EO group, the mean T level appeared to be a significant predictor in the models for speech recognition in quiet and in noise, such that CI users with elevated T levels had worse speech recognition in quiet and in noise. CONCLUSIONS: Significant parameters related to speech recognition in quiet and in noise were identified: aided thresholds, electrical DR, T levels, and impedance profiles. The results of this study are consistent with previous study findings and may guide audiologists in their fitting practices to improve the performance of CI users. The best performance was found for CI users with aided thresholds around the target level of 25 dB HL, and an electrical DR between 40 and 60 CL. However, adjustments of T and/or C levels to obtain aided thresholds around the target level and the preferred DR may not always be acceptable for individual CI users. Finally, clinicians should pay attention to profiles of impedances other than a flat profile with mild variations.
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Implante Coclear , Implantes Cocleares , Surdez , Percepção da Fala , Adulto , Impedância Elétrica , Humanos , Estudos RetrospectivosRESUMO
OBJECTIVES: Previous findings of longitudinal cohort studies indicate that acceleration in age-related hearing decline may occur. Five-year follow-up data of the Netherlands Longitudinal Study on Hearing (NL-SH) showed that around the age of 50 years, the decline in speech recognition in noise accelerates compared with the change in hearing in younger participants. Other longitudinal studies confirm an accelerated loss in speech recognition in noise but mostly use older age groups as a reference. In the present study, we determined the change in speech recognition in noise over a period of 10 years in participants aged 18 to 70 years at baseline. We additionally investigated the effects of age, sex, educational level, history of tobacco smoking, and alcohol use on the decline of speech recognition in noise. DESIGN: Baseline (T0), 5-year (T1), and 10-year (T2) follow-up data of the NL-SH collected until May 2017 were included. The NL-SH is a web-based prospective cohort study which started in 2006. Central to the NL-SH is the National Hearing test (NHT) which was administered to the participants at all three measurement rounds. The NHT uses three-digit sequences which are presented in a background of stationary noise. The listener is asked to enter the digits using the computer keyboard. The outcome of the NHT is the speech reception threshold in noise (SRT) (i.e., the signal to noise ratio where a listener recognizes 50% of the digit triplets correctly). In addition to the NHT, participants completed online questionnaires on demographic, lifestyle, and health-related characteristics at T0, T1, and T2. A linear mixed model was used for the analysis of longitudinal changes in SRT. RESULTS: Data of 1349 participants were included. At the start of the study, the mean age of the participants was 45 years (SD 13 years) and 61% of the participants were categorized as having good hearing ability in noise. SRTs significantly increased (worsened) over 10 years (p < 0.001). After adjustment for age, sex, and a history of tobacco smoking, the mean decline over 10 years was 0.89 dB signal to noise ratio. The decline in speech recognition in noise was significantly larger in groups aged 51 to 60 and 61 to 70 years compared with younger age groups (18 to 30, 31 to 40, and 41 to 50 years) (p < 0.001). Speech recognition in noise in participants with a history of smoking declined significantly faster during the 10-year follow-up interval (p = 0.003). Sex, educational level, and alcohol use did not appear to influence the decline of speech recognition in noise. CONCLUSIONS: This study indicated that speech recognition in noise declines significantly over a 10-year follow-up period in adults aged 18 to 70 years at baseline. It is the first longitudinal study with a 10-year follow-up to reveal that the increased rate of decline in speech recognition ability in noise already starts at the age of 50 years. Having a history of tobacco smoking increases the decline of speech recognition in noise. Hearing health care professionals should be aware of an accelerated decline of speech recognition in noise in adults aged 50 years and over.
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Audição , Percepção da Fala , Adolescente , Adulto , Idoso , Criança , Seguimentos , Testes Auditivos , Humanos , Estudos Longitudinais , Pessoa de Meia-Idade , Países Baixos , Ruído , Estudos Prospectivos , Fala , Teste do Limiar de Recepção da Fala , Adulto JovemRESUMO
Objective: To develop an intervention for the implementation of an ICF-based e-intake tool in clinical oto-audiology practice.Design: Intervention design study using the eight-stepped Behaviour Change Wheel. Hearing health professionals' (HHPs) and patients' barriers to and enablers of the use of the tool were identified in our previous study (steps 1-4). Following these steps, relevant intervention functions and policy categories were selected to address the reported barriers and enablers (steps 5-6); and behaviour change techniques and delivery modes were chosen for the selected intervention functions (steps 7-8).Results: For HHPs, the intervention functions education, training, enablement, modelling, persuasion and environmental restructuring were selected (step 5). Guidelines, service provision, and changes in the environment were identified as appropriate policy categories (step 6). These were linked to nine behaviour change techniques (e.g. information on health consequences), delivered through educational/training materials and workshops, and environmental factors (steps 7-8). For patients, the intervention functions education and enablement were selected, supported through service provision (steps 5-6). These were linked to three behaviour change techniques (e.g. environmental factors), delivered through their incorporation into the tool (steps 7-8).Conclusions: A multifaceted intervention was proposed to support the successful implementation of the intake tool.
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Audiologia/métodos , Avaliação da Deficiência , Implementação de Plano de Saúde/métodos , Otolaringologia/métodos , Telemedicina/métodos , Protocolos de Ensaio Clínico como Assunto , Humanos , Classificação Internacional de Funcionalidade, Incapacidade e SaúdeRESUMO
OBJECTIVE: To examine the construct validity, reliability and responsiveness of the Otology Questionnaire Amsterdam (OQUA). DESIGN: Multicentre, longitudinal study in 2 separate cohorts of patients visiting an ENT surgeon via an online survey programme. SETTING: Tertiary ENT clinics. PARTICIPANTS: Cohort 1 consisted of patients at their first visit at an ENT outpatient clinic with an ear complaint. Cohort 2 consisted of patients who underwent surgery, with a 3-month follow-up post-surgery. MAIN OUTCOME MEASURES: Construct validity: Hypothesis testing, internal consistency and inter-item correlation. Reliability: Test-retest reliability. The construct approach was used for assessing responsiveness. Hypotheses were formulated based on the association between the OQUA and Glasgow Health Status Inventory (GHSI) or Global Rating Scale (GRS). RESULTS: Construct validity: The correlation between the individual items in the impact domain ranged from 0.424 to 0.737. Confirmatory factor analysis showed a good fit. As expected, the OQUA impact showed strong relationships with GHSI total and general scale. Reliability: The test-retest reliability coefficient ranged from 0.541 to 0.838. Responsiveness: All hypotheses were conformed. As expected, the change score of the OQUA showed good correlation between OQUA impact and GHSI and moderate correlation between the GRS and OQUA complaints. CONCLUSION: The OQUA has 8 complaint domains (earache, pressure sensation, itching, tinnitus, hearing loss, ear discharge, loss of taste and dizziness) and 1 impact domain. Each domain results in one score of 0-100. The OQUA shows good results for construct validity, (test-retest) reliability and responsiveness, supporting the potential benefit for the patient with an ear complaint visiting the ENT surgeon. The extensive validation furthermore confirms a certified generic otology PROM with an impact and a complaints' part, to be used in different types of otologic interventions and patient groups.
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Otopatias/diagnóstico , Otolaringologia/métodos , Medidas de Resultados Relatados pelo Paciente , Feminino , Humanos , Estudos Longitudinais , Masculino , Países Baixos , Psicometria , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
ATP2B2 encodes the PMCA2 Ca2+ pump that plays an important role in maintaining ion homeostasis in hair cells among others by extrusion of Ca2+ from the stereocilia to the endolymph. Several mouse models have been described for this gene; mice heterozygous for loss-of-function defects display a rapidly progressive high-frequency hearing impairment. Up to now ATP2B2 has only been reported as a modifier, or in a digenic mechanism with CDH23 for hearing impairment in humans. Whole exome sequencing in hearing impaired index cases of Dutch and Polish origins revealed five novel heterozygous (predicted to be) loss-of-function variants of ATP2B2. Two variants, c.1963G>T (p.Glu655*) and c.955delG (p.Ala319fs), occurred de novo. Three variants c.397+1G>A (p.?), c.1998C>A (p.Cys666*), and c.2329C>T (p.Arg777*), were identified in families with an autosomal dominant inheritance pattern of hearing impairment. After normal newborn hearing screening, a rapidly progressive high-frequency hearing impairment was diagnosed at the age of about 3-6 years. Subjects had no balance complaints and vestibular testing did not yield abnormalities. There was no evidence for retrocochlear pathology or structural inner ear abnormalities. Although a digenic inheritance pattern of hearing impairment has been reported for heterozygous missense variants of ATP2B2 and CDH23, our findings indicate a monogenic cause of hearing impairment in cases with loss-of-function variants of ATP2B2.
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Biomarcadores/análise , Predisposição Genética para Doença , Perda Auditiva/genética , Mutação , ATPases Transportadoras de Cálcio da Membrana Plasmática/genética , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Seguimentos , Heterozigoto , Humanos , Masculino , Pessoa de Meia-Idade , Linhagem , Prognóstico , Adulto JovemRESUMO
Self speech recognition tests in quiet and noise at home are compared to the standard tests performed in the clinic. Potential effects of stimuli presentation modes (loudspeaker or audio cable) and assessment (clinician or self-assessment at home) on test results were investigated. Speech recognition in quiet was assessed using the standard Dutch test with monosyllabic words. Speech recognition in noise was assessed with the digits-in-noise test. Sixteen experienced CI users (aged between 44 and 83 years) participated. No significant difference was observed in speech recognition in quiet between and presentation modes. Speech recognition in noise was significantly better with the audio cable than with the loudspeaker. There was no significant difference in speech recognition in quiet at 65 dB and in speech recognition in noise between self-assessment at home and testing in the clinic. At 55 dB, speech recognition assessed at home was slightly but significantly better than that assessed in the clinic. The results demonstrate that it is feasible for experienced CI users to perform self-administered speech recognition tests at home. Self-assessment by CI users of speech recognition in quiet and noise within the home environment could serve as an alternative to the tests performed in the clinic.
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Audiometria da Fala/métodos , Implante Coclear/instrumentação , Implantes Cocleares , Ruído/efeitos adversos , Mascaramento Perceptivo , Pessoas com Deficiência Auditiva/reabilitação , Reconhecimento Psicológico , Autocuidado/métodos , Inteligibilidade da Fala , Percepção da Fala , Estimulação Acústica , Adulto , Idoso , Idoso de 80 Anos ou mais , Estimulação Elétrica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Pessoas com Deficiência Auditiva/psicologia , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos TestesRESUMO
OBJECTIVES: To review literature on the use of manual and automatically switching multimemory devices by hearing aid and CI recipients, and to investigate if recipients appreciate and adequately use the ability to switch between programmes in various listening environments. DESIGN: Literature was searched using PubMed, Embase and ISI/Web of Science. Additional studies were identified by screening reference and citation lists, and by contacting experts. STUDY SAMPLE: The search yielded 1109 records that were screened on title and abstract. This resulted in the full-text assessment of 37 articles. RESULTS: Sixteen articles reported on the use of multiple programmes for various listening environments, three articles reported on the use of an automatic switching mode. All studies reported on hearing aid recipients only, no study with CI recipients fulfilled the selection criteria. CONCLUSIONS: Despite the high number of manual and automatically switching multimemory devices sold each year, there are remarkably few studies about the use of multiple programmes or automatic switching modes for various listening environments. No studies were found that examined the accuracy of the use of programmes for specific listening environments. An automatic switching device might be a solution if recipients are not able, or willing, to switch manually between programmes.
Assuntos
Percepção Auditiva , Implante Coclear/instrumentação , Implantes Cocleares , Correção de Deficiência Auditiva/instrumentação , Auxiliares de Audição , Perda Auditiva/reabilitação , Audição , Pessoas com Deficiência Auditiva/reabilitação , Estimulação Acústica , Adulto , Idoso , Idoso de 80 Anos ou mais , Automação , Estimulação Elétrica , Meio Ambiente , Feminino , Perda Auditiva/diagnóstico , Perda Auditiva/fisiopatologia , Perda Auditiva/psicologia , Humanos , Masculino , Pessoa de Meia-Idade , Pessoas com Deficiência Auditiva/psicologia , Desenho de Prótese , Processamento de Sinais Assistido por Computador , Adulto JovemRESUMO
OBJECTIVES: The International Classification of Functioning Disability and Health (ICF) Core Sets for Hearing Loss (HL) were developed to serve as a standard for the assessment and reporting of the functioning and health of patients with HL. The aim of the present study was to compare the content of the intake documentation currently used in secondary and tertiary hearing care settings in the Netherlands with the content of the ICF Core Sets for HL. Research questions were (1) to what extent are the ICF Core Sets for HL represented in the Dutch Otology and Audiology intake documentation? (2) are there any extra ICF categories expressed in the intake documentation that are currently not part of the ICF Core Sets for HL, or constructs expressed that are not part of the ICF? DESIGN: Multicenter patient record study including 176 adult patients from two secondary, and two tertiary hearing care settings. The intake documentation was selected from anonymized patient records. The content was linked to the appropriate ICF category from the whole ICF classification using established linking rules. The extent to which the ICF Core Sets for HL were represented in the intake documentation was determined by assessing the overlap between the ICF categories in the Core Sets and the list of unique ICF categories extracted from the intake documentation. Any extra constructs that were expressed in the intake documentation but are not part of the Core Sets were described as well, differentiating between ICF categories that are not part of the Core Sets and constructs that are not part of the ICF classification. RESULTS: In total, otology and audiology intake documentation represented 24 of the 27 Brief ICF Core Set categories (i.e., 89%), and 60 of the 117 Comprehensive ICF Core Set categories (i.e., 51%). Various ICF Core Sets categories were not represented, including higher mental functions (Body Functions), civic life aspects (Activities and Participation), and support and attitudes of family (Environmental Factors). One extra ICF category emerged from the intake documentation that is currently not included in the Core Sets: sleep functions. Various Personal Factors emerged from the intake documentation that are currently not defined in the ICF classification. CONCLUSIONS: The results showed substantial overlap between the ICF Core Sets for HL and the intake documentation of otology and audiology, but also revealed areas of nonoverlap. These findings contribute to the evaluation of the content validity of the Core Sets. The overlap can be viewed as supportive of the Core Sets' content validity. The nonoverlap in Core Sets categories indicates that current Dutch intake procedures may not cover all aspects relevant to patients with ear/hearing problems. The identification of extra constructs suggests that the Core Sets may not include all areas of functioning that are relevant to Dutch Otology and Audiology patients. Consideration of incorporating both aspects into future intake practice deserves attention. Operationalization of the ICF Core Sets categories, including the extra constructs identified in this study, into a practical and integral intake instrument seems an important next step.
Assuntos
Audiologia , Documentação , Perda Auditiva/fisiopatologia , Classificação Internacional de Funcionalidade, Incapacidade e Saúde , Otolaringologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Perda Auditiva/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos , Adulto JovemRESUMO
The number of cochlear implant (CI) users is increasing annually, resulting in an increase in the workload of implant centers in ongoing patient management and evaluation. Remote testing of speech recognition could be time-saving for both the implant centers as well as the patient. This study addresses two methodological challenges we encountered in the development of a remote speech recognition tool for adult CI users. First, we examined whether speech recognition in noise performance differed when the steady-state masking noise was presented throughout the test (i.e. continuous) instead of the standard clinical use for evaluation where the masking noise stops after each stimulus (i.e. discontinuous). A direct coupling between the audio port of a tablet computer to the accessory input of the sound processor with a personal audio cable was used. The setup was calibrated to facilitate presentation of stimuli at a predefined sound level. Finally, differences in frequency response between the audio cable and microphones were investigated.
Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva/reabilitação , Percepção da Fala , Teste do Limiar de Recepção da Fala/métodos , Telemedicina/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Audiometria de Tons Puros , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ruído , Razão Sinal-Ruído , Adulto JovemRESUMO
The objective of this study was to determine whether hearing ability in adults is associated with medication use in general, the use of specific types of medication, or polypharmacy. In this exploratory study, data of the National Longitudinal Study on Hearing (NL-SH; n = 2,160) were used. In total, 62% of the participants reported using any medication in the past 28 days. Hearing ability in noise, as determined with an online digit-triplet speech-in-noise test, was significantly associated with (1) medication acting on the alimentary tract and metabolism (including diabetes and acid-related disorders), (2) use of calcium blockers, and (3) medication used for sensory organs.
Assuntos
Tratamento Farmacológico/estatística & dados numéricos , Perda Auditiva/epidemiologia , Adolescente , Adulto , Idoso , Antiácidos/uso terapêutico , Bloqueadores dos Canais de Cálcio/uso terapêutico , Estudos de Coortes , Estudos Transversais , Feminino , Fármacos Gastrointestinais/uso terapêutico , Humanos , Hipoglicemiantes/uso terapêutico , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Preparações Farmacêuticas , Estudos Prospectivos , Adulto JovemRESUMO
OBJECTIVE: The primary objective of the study was to investigate the feasibility, reliability, and validity of the Dutch digits in noise (DIN) test for measuring speech recognition in hearing aid and cochlear implant users and compare results to the standard sentences-in-noise (SIN) test. DESIGN: The relation between speech reception thresholds for DIN test and SIN test was analysed to determine the validity of the DIN test. As linguistic skills were expected to make different contributions in these tests, their influence was analysed. STUDY SAMPLE: Participants were 12 normal-hearing listeners, 24 hearing aid users, and 24 cochlear implant users. RESULTS: The DIN test was feasible for more participants than the SIN test. Intraclass correlation coefficients showed high reliability. The standard error of measurement was smaller for the DIN test than for the SIN test. DIN test and SIN test were highly correlated (r = 0.95 and r = 0.56 for NH+ HA and CI users respectively). In the regression analysis no significant contribution of basic linguistic skills or personal factors was found. CONCLUSION: In the assessment of speech recognition in noise of aided hearing-impaired listeners with hearing aids or cochlear implants, the DIN test is a feasible, reliable and valid test.
Assuntos
Implantes Cocleares , Auxiliares de Audição , Teste do Limiar de Recepção da Fala/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Limiar Auditivo , Estudos de Viabilidade , Feminino , Humanos , Linguística , Masculino , Pessoa de Meia-Idade , Ruído , Reprodutibilidade dos Testes , Razão Sinal-Ruído , Percepção da FalaRESUMO
The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.