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1.
Int J Pediatr Otorhinolaryngol ; 182: 112024, 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38972250

RESUMO

OBJECTIVE: Self-report instruments are commonly used in tinnitus clinics, but they are presently available only for adults. There is a lack of a validated multi-item instrument to capture tinnitus-related problems in children and their impact on everyday life. This study has developed and validated a specifically child-centered questionnaire to assess the impact of tinnitus. METHODS: Development of the tool consisted of several stages. Following a pilot study on 12 children with tinnitus, a validation study was done on a further 192 children with tinnitus aged between 11 and 14 years. The children had an audiological examination, completed a Visual Analogue Scale (VAS) and the newly framed questionnaire. RESULTS: The development and validation process resulted in the new 11-item Children's Tinnitus Questionnaire (CTQ). It includes items concerning the impact of tinnitus on functional, cognitive, emotional and social domains. The validity of the new tool has been established by finding significant correlations between it and VAS loudness (r = 0.42), VAS annoyance (r = 0.67), and VAS coping (r = -0.41). Validity has also been confirmed by measuring differences in CTQ scores and 4 groups of children having graded incidences of tinnitus. The internal consistency assessed with Cronbach's alpha was high (α = 0.82). CONCLUSION: The Children's Tinnitus Questionnaire (CTQ) is the first fully validated multi-item instrument designed specifically for children. The tool has the potential to become a valuable new instrument for use in clinical practice and research; it might be useful for assessing the impact of tinnitus on those children who find that the condition creates problems in their everyday life.

2.
Ear Hear ; 2024 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-38915137

RESUMO

OBJECTIVES: A wide variety of intraoperative tests are available in cochlear implantation. However, no consensus exists on which tests constitute the minimum necessary battery. We assembled an international panel of clinical experts to develop, refine, and vote upon a set of core consensus statements. DESIGN: A literature review was used to identify intraoperative tests currently used in the field and draft a set of provisional statements. For statement evaluation and refinement, we used a modified Delphi consensus panel structure. Multiple interactive rounds of voting, evaluation, and feedback were conducted to achieve convergence. RESULTS: Twenty-nine provisional statements were included in the original draft. In the first voting round, consensus was reached on 15 statements. Of the 14 statements that did not reach consensus, 12 were revised based on feedback provided by the expert practitioners, and 2 were eliminated. In the second voting round, 10 of the 12 revised statements reached a consensus. The two statements which did not achieve consensus were further revised and subjected to a third voting round. However, both statements failed to achieve consensus in the third round. In addition, during the final revision, one more statement was decided to be deleted due to overlap with another modified statement. CONCLUSIONS: A final core set of 24 consensus statements was generated, covering wide areas of intraoperative testing during CI surgery. These statements may provide utility as evidence-based guidelines to improve quality and achieve uniformity of surgical practice.

3.
Med Sci Monit ; 30: e944090, 2024 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-38859565

RESUMO

BACKGROUND The dichotic digit test (DDT) is one of the tests for the behavioral assessment of central auditory processing. Dichotic listening tests are sensitive ways of assessing cortical structures, the corpus callossum, and binaural integration mechanisms, showing strong correlations with learning difficulties. The DDT is presently available in a number of languages, each appropriate for the subject's native language. However, there is presently no test in the Italian language. The goal of this study was to develop an Italian version of the one-pair dichotic digit test (DDT-IT) and analyze results in 39 normal-hearing Italian children 11 to 13 years old. We used 2 conditions of presentation: free recall and directed attention (left or right ear), and looked at possible effects of sex and ear side. MATERIAL AND METHODS This study involved 3 steps: creation of the stimuli, checking their quality with Italian speakers, and assessment of the DDT-IT in our subject pool. The study involved 39 children (26 girls and 13 boys), aged 11-13 years. All participants underwent basic audiological assessment, auditory brainstem response, and then DDT-IT. RESULTS Results under free recall and directed attention conditions were similar for right and left ears, and there were no sex or age effects. CONCLUSIONS The Italian version of DDT (DDT-IT) has been developed and its performance on 39 normal-hearing Italian children was assessed. We found there were no age or sex effects for either the free recall condition or the directed attention condition.


Assuntos
Testes com Listas de Dissílabos , Humanos , Feminino , Masculino , Criança , Adolescente , Testes com Listas de Dissílabos/métodos , Itália , Idioma , Audição/fisiologia , Percepção Auditiva/fisiologia , Atenção/fisiologia
4.
Brain Sci ; 14(6)2024 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-38928598

RESUMO

Palatine and pharyngeal tonsil hypertrophy may lead to dysfunction of the auditory tube due to a propensity for infection, potentially giving rise to otitis media. This is a quantitative and longitudinal study, developed from 2019 to 2021, at the State University of Campinas (UNICAMP). The studied sample comprised 15 participants aged 5 to 12 years (mean 7.9 years), 12 male and 3 female, arranged into two groups: children diagnosed with pharyngeal and/or palatine tonsil hypertrophy who were candidates for surgery (G1), and children who were later evaluated after surgery (G2). As part of the test, an otoscopy and measurements of logoaudiometry, pure-tone threshold audiometry, wideband tympanometry (ambient and peak pressure), and otoacoustic emissions (TEOAEs and DPOAEs, both at ambient and peak pressure) were all performed. There were statistically significant differences between phases in pure-tone audiometry, in terms of 226 Hz tympanometry, wideband tympanometry in peak pressure conditions, in the amplitude measurement TEOAEs in both pressure conditions, in DPOAEs in ambient pressure conditions, and in the signal/noise measurement in both pressures in DPOAEs. Overall, it was found that hearing tests were different for subjects with palatine and pharyngeal tonsil hypertrophy compared to the post-surgical group.

5.
Brain Sci ; 14(5)2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38790444

RESUMO

Testing of ChatGPT has recently been performed over a diverse range of topics. However, most of these assessments have been based on broad domains of knowledge. Here, we test ChatGPT's knowledge of tinnitus, an important but specialized aspect of audiology and otolaryngology. Testing involved evaluating ChatGPT's answers to a defined set of 10 questions on tinnitus. Furthermore, given the technology is advancing quickly, we re-evaluated the responses to the same 10 questions 3 and 6 months later. The accuracy of the responses was rated by 6 experts (the authors) using a Likert scale ranging from 1 to 5. Most of ChatGPT's responses were rated as satisfactory or better. However, we did detect a few instances where the responses were not accurate and might be considered somewhat misleading. Over the first 3 months, the ratings generally improved, but there was no more significant improvement at 6 months. In our judgment, ChatGPT provided unexpectedly good responses, given that the questions were quite specific. Although no potentially harmful errors were identified, some mistakes could be seen as somewhat misleading. ChatGPT shows great potential if further developed by experts in specific areas, but for now, it is not yet ready for serious application.

6.
J Commun Disord ; 109: 106426, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38692192

RESUMO

INTRODUCTION: Central auditory processing disorders (CAPD) can significantly affect the daily functioning of a child, and the first step in determining whether rehabilitation procedures are required is a proper diagnosis. Different guidelines for making diagnoses have been published in the literature, and in various centers normative values for psychoacoustic tests of CAPD have been used internally. The material presented in this paper is based on more than 1000 children and is the largest collection so far published. The aim of this study is to present normative values for tests assessing CAPD in children aged 6 to 12 years, divided by age at last birthday. METHOD: We tested 1037 children aged 6 to 12 years who were attending primary schools and kindergartens. The criteria for inclusion were a normal audiogram, intellectually normal, no developmental problems, and no difficulties in auditory processing. To evaluate auditory processing all children were given three tests on the Senses Examination Platform: the Frequency Pattern Test (FPT), Duration Pattern Test (DPT), and Dichotic Digit Test (DDT). RESULTS: The results from 1,037 children allowed us to determine normative values for FPT, DPT, and DDT in seven different age groups (6 through to 12 years). We developed a newapproach, based on quantile-based norms, to determine normative values in each group. Three categories - average, below-average, and above-average - allow for a broader but more realistic interpretation than those used previously. We compare our results with published standards. CONCLUSIONS: Our study is the largest normative database published to date for CAPD testing, setting a standard for each child by age in years. We used the Senses Examination Platform, a universal tool, to unify standards for the classification of CAPD. Our study can serve as a basis for the development of a Polish model for the diagnosis of CAPD.


Assuntos
Transtornos da Percepção Auditiva , Humanos , Criança , Feminino , Valores de Referência , Masculino , Transtornos da Percepção Auditiva/diagnóstico
7.
Artigo em Inglês | MEDLINE | ID: mdl-38573516

RESUMO

PURPOSE: To study outcome after cochlear implantation using the Cochlear Implant (CI) outcome assessment protocol based on the International Classification of Functioning, Disability and Health (ICF) model (CI-ICF). METHODS: Raw data of a prospective, longitudinal, multicenter study was analyzed. Seventy-two CI candidates were assessed preoperatively and six months postoperatively using the CI-ICF protocol. Following tools were used: (1) Work Rehabilitation Questionnaire (WORQ), (2) Abbreviated Profile of Hearing Aid Benefit (APHAB), (3) Audio Processor Satisfaction Questionnaire (APSQ), (4) Speech, Spatial, and Qualities of Hearing Scale (SSQ12), (5) Hearing Implant Sound Quality Index (HISQUI19), (6) Nijmegen CI Questionnaire (NCIQ) (7) pure tone audiometry, (8) speech audiometry, (9) sound localization. RESULTS: There was a significant improvement of speech discrimination in quiet (p = 0.015; p < 0.001) and in noise (p = 0.041; p < 0.001), sound detection (p < 0.001), tinnitus (p = 0.026), listening (p < 0.001), communicating with-receiving-spoken messages (p < 0.001), conversation (p < 0.001), family relationships (p < 0.001), community life (p = 0.019), NCIQ total score and all subdomain scores (p < 0.001). Subjective sound localization significantly improved (p < 0.001), while psychometric sound localization did not. There was no significant subjective deterioration of vestibular functioning and no substantial change in sound aversiveness. CI users reported a high level of implant satisfaction postoperatively. CONCLUSION: This study highlights the positive impact of cochlear implantation on auditory performance, communication, and subjective well-being. The CI-ICF protocol provides a holistic and comprehensive view of the evolution of CI outcomes.

8.
Life (Basel) ; 14(3)2024 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-38541643

RESUMO

(1) Background: One of the possible symptoms of COVID-19 is a sudden loss of smell and taste. The main aim of the study was to evaluate the severity of post-COVID-19 olfactory dysfunction (OD). A secondary aim was to assess the relationship between OD and gustatory (taste) dysfunction (GD). Margins: 2.5 cm (1 inch) at top, bottom, right, and left. (2) Methods: The study group consisted of 81 subjects (16 men and 65 women) aged between 12 and 73 years. All of the patients presented to a center for subjective OD associated with COVID-19. They were tested with a Sniffin' Sticks test (SST) for OD and a Taste Strip test (TS) for GD. (3) Anosmia was present in 18 participants (22%), hyposmia in 52 (64%), and normosmia in 11 (14%). Some 36% of the patients reported imaginary smells (phantosmia), but it did not correlate with olfactory sensitivity. Comparing the different parts of the SST showed that subjects scored lowest on the threshold part of the test. The results of the discrimination and identification parts of the test were better, implying that if the stimulus is intense enough, incorrect discrimination and identification of odors is less frequent. A sweet taste was the easiest to recognize (78% could do so), while the most difficult to recognize was salty (68%). There were weak and statistically non-significant correlations between olfactory and taste dysfunction. (4) Conclusions: The results suggest that post-COVID-19 olfactory dysfunction was more peripheral than central. Testing patients for the severity of post-COVID-19 OD may help clinicians treat the condition. Because there is no fully effective treatment, research on post-COVID-19 OD is needed.

9.
Int Arch Otorhinolaryngol ; 28(1): e122-e128, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38322437

RESUMO

Introduction Peripheral hearing loss, besides causing inadequate auditory input, can lead to distortions in the tonotopic auditory map and reorganization of neural networks. Therefore, the processing of temporal aspects of a sound stimulus and, consequently, the effectiveness of human communication can be negatively impacted. Objective To test the temporal ordering and auditory resolution of people with mild and moderate sensorineural hearing loss and to compare them with the those of people with normal hearing. Methods A total of 19 right-handed individuals aged 16 to 59 years with mild to moderate postlingually acquired symmetric bilateral sensorineural hearing loss participated in the study. They were submitted to frequency and duration pattern tests and a random gap detection test. Results The mean correct response rate in the frequency pattern test was of 66.3%, and, in the duration pattern test, 71.7%. The mean threshold in the random gap detection test was of 14.1 ms. A comparison with the criteria established for normal subjects without peripheral hearing loss revealed that more than half the subjects had abnormal results in the temporal ordering test, while a smaller fraction had reduced temporal resolution. Conclusions The performance of the subjects with acquired sensorineural hearing loss was poorer than that of the participants without peripheral hearing loss. Their results on the temporal ordering test were also poorer than in the temporal resolution test, demonstrating the importance of analyzing both these auditory skills in people with peripheral hearing loss.

10.
Brain Sci ; 14(2)2024 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-38391748

RESUMO

This study looked at the possible effect of the COVID-19 pandemic on individuals who came to our clinic seeking relief from tinnitus. The performance of the subjects during the COVID-19 pandemic was compared with similar individuals who came to our clinic before the pandemic began. The study involved 50 adults with chronic tinnitus, made up of a study group (24 subjects tested during the COVID-19 pandemic of 2020-2021) and a control group before the pandemic began (26 subjects tested from 2013 to 2017). None of the 24 reported having contracted COVID-19. Data collection involved the Tinnitus Handicap Inventory (THI) questionnaire, audiological tests, and quantitative electroencephalography (qEEG). In terms of THI scores, there were no statistically significant differences between the two groups. However, with regard to qEEG, some changes were observed, with significant decreases in alpha and beta band activity in the study group compared to the control group, particularly over the auditory cortex. We conclude that COVID-19 did not have a discernible impact on the general well-being of individuals with tinnitus. However, it did appear to alter brain activity, specifically in the alpha and beta bands over the auditory cortex, and these reults warrant further investigation.

11.
Int J Audiol ; 63(4): 250-259, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36799648

RESUMO

OBJECTIVES: The aim of this interventional non-randomised prospective controlled study was to assess the effectiveness of transcutaneous vagus nerve stimulation (tVNS) in human subjects with tinnitus. DESIGN: The ParasymTM tVNS device was paired with an auditory stimulation. Treatment and observations were conducted over 12 weeks. Audiological evaluation was performed. Responses from a set of questionnaires and quantitative electroencephalography (qEEG) before and after treatment were collected. Voice measurements were done to assess possible side-effects of tVNS. STUDY SAMPLE: The study involved 29 adults who had chronic tinnitus (15 patients who underwent tVNS paired with sounds and a control group of 14 patients who did not). RESULTS: In general, subjective and objective measurements of tinnitus showed no improvement in the study group compared to the controls, although certain parameters as gauged by the questionnaires did statistically improve. The loudness and frequency of tinnitus remained the same in both groups. For the qEEG, activity in the theta band increased significantly in the study group compared to the control group. CONCLUSIONS: The tVNS was not effective in reducing tinnitus symptoms in our study group. However, changes in the theta band suggest there might be cortical effects that might, with sustained treatment, lead to improvements.


Assuntos
Zumbido , Estimulação Elétrica Nervosa Transcutânea , Estimulação do Nervo Vago , Adulto , Humanos , Estudos Prospectivos , Projetos de Pesquisa , Zumbido/diagnóstico , Zumbido/terapia , Estimulação do Nervo Vago/efeitos adversos
12.
Rev. CEFAC ; 26(1): e3223, 2024. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1529401

RESUMO

ABSTRACT Purpose: to investigate the frequency-following response (FFR) for sustained neural activity. Methods: 39 individuals, aged between 20 to 47 months old were divided into 2 groups: (i) 20 individuals without prenatal exposure to the congenital Zika syndrome (CZS) or hydrocephaly, normal development, no risk factors for hearing loss or syndromic hearing impairment and (ii) 19 individuals diagnosed with CZS and microcephaly - based on imaging studies linked to the clinical presentation of the condition. All participants exhibited normal click-ABR tests. FFR waveforms were documented using the /da/ syllable employing the Navigator Pro. The statistical analysis used was ANOVA (p-value <0.05). Results: no distinctions were observed concerning the variables of group, age, or gender with respect to FFR latency values, except for an interaction between gender and group for latency values associated with waves V and F. Children with CZS and microcephaly showed a difference for latency values in wave V for both males and females, when compared to the control group. Conclusion: children presented with CZS and microcephaly showed higher average latencies for waves V, A, C, D and F (male) compared to the control group, whereas, in waves E, F (female) and O they showed higher values in the control group.

13.
Int. arch. otorhinolaryngol. (Impr.) ; 28(1): 122-128, 2024. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1557995

RESUMO

Abstract Introduction Peripheral hearing loss, besides causing inadequate auditory input, can lead to distortions in the tonotopic auditory map and reorganization of neural networks. Therefore, the processing of temporal aspects of a sound stimulus and, consequently, the effectiveness of human communication can be negatively impacted. Objective To test the temporal ordering and auditory resolution of people with mild and moderate sensorineural hearing loss and to compare them with the those of people with normal hearing. Methods A total of 19 right-handed individuals aged 16 to 59 years with mild to moderate postlingually acquired symmetric bilateral sensorineural hearing loss participated in the study. They were submitted to frequency and duration pattern tests and a random gap detection test. Results The mean correct response rate in the frequency pattern test was of 66.3%, and, in the duration pattern test, 71.7%. The mean threshold in the random gap detection test was of 14.1 ms. A comparison with the criteria established for normal subjects without peripheral hearing loss revealed that more than half the subjects had abnormal results in the temporal ordering test, while a smaller fraction had reduced temporal resolution. Conclusions The performance of the subjects with acquired sensorineural hearing loss was poorer than that of the participants without peripheral hearing loss. Their results on the temporal ordering test were also poorer than in the temporal resolution test, demonstrating the importance of analyzing both these auditory skills in people with peripheral hearing loss.

14.
Eur Arch Otorhinolaryngol ; 281(5): 2353-2363, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38133806

RESUMO

PURPOSE: This multicentric, retrospective study aimed to analyze the short-term safety and effectiveness of the mCLIP Partial Prosthesis. METHODS: Patients underwent tympanoplasty with implantation of a mCLIP Partial Prosthesis. Follow-up examination included ear microscopy and pure-tone audiometry to determine the post-operative pure tone average of the frequencies 0.5, 1, 2 and 3 kHz (PTA4). The post-operative PTA4 air bone gap (ABG) was used to evaluate the audiological outcome. A post-operative minimum and maximum follow-up period was not defined. Thus, the follow-up times of each study center were different, which resulted in different follow-up times for the audiological analysis and for adverse events (AE). RESULTS: 72 (66 adults, 6 children) patients were implanted with the mCLIP Partial Prosthesis. 68 (62 adults, 6 children) patients underwent audiological examination; all 72 patients were examined for adverse events. All patients (N = 68): 72.1% of the patients showed a PTA4 ABG of ≤ 20 dB. Individual post-operative bone conduction (BC) PTA4 thresholds were stable in 67 patients. The mean post-operative follow-up time was 78 ± 46 days. Children (N = 6): 5 out of 6 children showed a PTA4 ABG of ≤ 20 dB. None of the children reported a BC PTA4 deterioration of > 10 dB HL after the implantation. The mean post-operative follow-up time was 101 ± 45 days. Adverse events (all patients, N = 72): 15 (14 adults, 1 child) patients had AEs (27 AEs and 2 Follow-Ups). The mean post-operative follow-up time was 375 days. CONCLUSION: Clinical data show satisfactory audiological parameters after implantation of the mCLIP Partial Prosthesis. The prosthesis is safe and effective for implantation in children and adults. TRIAL REGISTRATION NUMBER: NCT05565339, 09 September 2022, retrospectively registered.


Assuntos
Prótese Ossicular , Adulto , Criança , Humanos , Resultado do Tratamento , Estudos Retrospectivos , Seguimentos , Implantação de Prótese , Condução Óssea , Audiometria de Tons Puros
15.
Cureus ; 15(11): e49733, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38046713

RESUMO

This paper provides a step-by-step guide for organizing the scientific program (OSP) of international conferences. Through informal discussions, a panel of experts organizing international conferences came up with this guide, which includes a flowchart, checklist, and detailed discussions of each step. Subsequently, additional specialists were invited to evaluate this synopsis and provide their input. All of the participants approved the final version after the outline was improved. This guide proposes the following six steps: 1) preparation, 2) recruitment, 3) building the agenda, 4) cross-checking the program, 5) reviewing and finalizing, and 6) in-conference refining. Thirteen items are specified across the six main steps in a detailed checklist. This OSP guide includes a flowchart and a checklist for providing a comprehensive manual for establishing, conducting, and organizing international scientific conferences. Understanding the procedures that are expected to be followed when holding a scientific conference enables the involved parties to organize and assign tasks to one another as well as create a schedule that allows them to finish their work on time. This guide can be used at any kind of scientific conference to describe an organized process, resulting in a professional and distinguished scientific program.

16.
Artigo em Inglês | MEDLINE | ID: mdl-37887644

RESUMO

This study aimed to report quality of life (QoL) scores in unilateral cochlear implant (CI) users and to generate guidance for clinicians on using QoL measures to individualize CI counselling and rehabilitation and to increase access to CIs as a mode of rehabilitation. Participants (n = 101) were unilateral CI users with single-sided deafness (SSD; n = 17), asymmetrical hearing loss (AHL; n = 26), or bilateral hearing loss (Uni; n = 58). Generic QoL was assessed via the Health Utilities Index (HUI-3), and disease-specific QoL was assessed via the Speech, Spatial, and Qualities of Hearing scale (SSQ12) and Nijmegen CI Questionnaire (NCIQ) at preimplantation and at 6 and 12 months of CI use. All groups had significantly increased HUI-3 scores at both intervals. The SSD group showed significant benefit on the SSQ12 at visit 3, the AHL group showed significant benefit on the SSQ12 and most NCIQ subdomains at both intervals, and the Uni group showed significant benefit with both tests at both intervals. Unilateral CI recipients demonstrate improved QoL within the first 12 months of device use. Regular assessment with generic and disease-specific questionnaires has the potential to play an important role in personalizing treatment and possibly in increasing access to CI provision.


Assuntos
Implantes Cocleares , Surdez , Percepção da Fala , Humanos , Adulto , Qualidade de Vida , Surdez/reabilitação , Anos de Vida Ajustados por Qualidade de Vida , Resultado do Tratamento
17.
Am J Case Rep ; 24: e940439, 2023 Oct 03.
Artigo em Inglês | MEDLINE | ID: mdl-37786244

RESUMO

BACKGROUND Brown-Vialetto-Van Laere (BVVL) syndrome is a rare autosomal recessive disorder caused by mutations in intestinal riboflavin transporter genes, resulting in a motor neuron disorder of childhood, which can be associated with sensorineural deafness. This report describes a 4-year-old Polish girl with progressive hearing loss and delayed speech development diagnosed with Brown-Vialetto-Van Laere syndrome who was treated with riboflavin (vitamin B2) and cochlear implants. CASE REPORT The case report concerns a girl from Poland who, at the age of 2 years 10 months, developed progressive atypical neurological symptoms of unknown etiology: ataxia of the upper and lower limbs, gait abnormalities, generalized muscle weakness, visual and hearing problems, and regression of speech development. A karyotype study (whole-exome sequencing) revealed alterations within SLC52A2, leading to the diagnosis of Brown-Vialetto-Van Laere syndrome and initiation of high-dose riboflavin treatment. As a 4-year-old child, she presented to the Institute of Physiology and Pathology of Hearing - World Hearing Center in Poland with progressive hearing loss and speech regression. Hearing tests revealed bilateral profound sensorineural hearing loss with auditory neuropathy. Surgical treatment was applied in the form of bilateral cochlear implantation. CONCLUSIONS This report shows the importance of genetic testing in infants who present with atypical symptoms or signs. In this case, the diagnosis of Brown-Vialetto-Van Laere syndrome resulted in timely correction of the genetic riboflavin (vitamin B2) deficiency and improved hearing following the use of cochlear implants.


Assuntos
Paralisia Bulbar Progressiva , Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Neurossensorial , Feminino , Lactente , Humanos , Pré-Escolar , Fala , Perda Auditiva Neurossensorial/etiologia , Paralisia Bulbar Progressiva/complicações , Paralisia Bulbar Progressiva/diagnóstico , Paralisia Bulbar Progressiva/genética , Riboflavina/uso terapêutico , Surdez/complicações , Surdez/tratamento farmacológico
18.
Sci Rep ; 13(1): 18417, 2023 10 27.
Artigo em Inglês | MEDLINE | ID: mdl-37891242

RESUMO

Previous studies indicate changes in neurotransmission along the auditory pathway in subjective tinnitus. Most authors, however, investigated brain regions including the primary auditory cortex, whose physiology can be affected by concurrent hearing deficits. In the present MR spectroscopy study we assumed increased levels of glutamate and glutamine (Glx), and other Central Nervous System metabolites in the temporal lobe outside the primary auditory cortex, in a region involved in conscious auditory perception and memory. We studied 52 participants with unilateral (n = 24) and bilateral (n = 28) tinnitus, and a control group without tinnitus (n = 25), all with no severe hearing losses and a similar hearing profile. None of the metabolite levels in the temporal regions of interest were found related to tinnitus status or laterality. Unexpectedly, we found a tendency of increased concentration of Glx in the control left medial frontal region in bilateral vs unilateral tinnitus. Slightly elevated depressive and anxiety symptoms were also shown in participants with tinnitus, as compared to healthy individuals, with the bilateral tinnitus group marginally more affected. We discuss no apparent effect in the temporal lobes, as well as the role of frontal brain areas, with respect to hearing loss, attention and psychological well-being in chronic tinnitus. We furthermore elaborate on the design-related and technical obstacles of MR spectroscopy.


Assuntos
Córtex Auditivo , Perda Auditiva , Zumbido , Humanos , Zumbido/diagnóstico , Córtex Auditivo/diagnóstico por imagem , Córtex Auditivo/metabolismo , Audição , Espectroscopia de Ressonância Magnética , Perda Auditiva/metabolismo
19.
J Clin Med ; 12(20)2023 Oct 19.
Artigo em Inglês | MEDLINE | ID: mdl-37892760

RESUMO

Hyperacusis, a kind of decreased sound tolerance, is difficult to measure objectively. It often co-occurs with tinnitus. There is a need for valid and reliable patient-reported outcome measures to capture this subjective phenomenon. The aim of the study was to create a questionnaire capturing hyperacusis in terms of loudness, fear, and pain and to evaluate its psychometric properties. The study sample consisted of 106 adult patients with hyperacusis and tinnitus with a mean age of 45.2 years. A medical interview, an audiological examination, and several questionnaires (the Tinnitus Handicap Inventory, the Hyperacusis Questionnaire, the State-Trait Anxiety Inventory, and Visual Analog Scales) were applied. The final 14-item Hyperacusis Assessment Questionnaire showed an appropriate three-factor structure with 70.5% of the variance explained. Convergent and divergent validity were confirmed by correlations with other measures of hyperacusis, anxiety, tinnitus severity, misophonia, and hearing thresholds. The internal consistency assessed with Cronbach's alpha was excellent (α = 0.91), as was reproducibility (intraclass correlation, ICC = 0.96). The new Hyperacusis Assessment Questionnaire is a psychometrically sound and brief tool assessing the severity of hyperacusis in terms of loudness, fear, and pain. It can be used in clinical practice and scientific research for patients with hyperacusis and tinnitus.

20.
Life (Basel) ; 13(10)2023 Oct 21.
Artigo em Inglês | MEDLINE | ID: mdl-37895473

RESUMO

BACKGROUND: While gender differences of several diseases have been already described in the literature, studies in the area of hyperacusis are still scant. Despite the fact that hyperacusis is a condition that severely affects the patient's quality of life, it is not well investigated; a comprehensive understanding of its features, eventually including gender differences, could be a valuable asset in developing clinical intervention strategies. AIM: To evaluate gender differences among subjects affected by hyperacusis. METHODS: A literature search was conducted focused on adult patients presenting hyperacusis, using the MedLine bibliographic database. Relevant peer-reviewed studies, published in the last 20 years, were sought. A total of 259 papers have been identified, but only 4 met the inclusion criteria. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. RESULTS: The four selected papers included data from 604 patients; of these, 282 subjects resulted as affected by hyperacusis (125 females and 157 males). Questionnaires for analyzing factors affecting the attentional, social and emotional variance of hyperacusis (such as VAS, THI, TSCH, MASH) were administered to all included subjects. The data suggest that there are no hyperacusis gender-specific differences in the assessed population samples. CONCLUSIONS: The literature data suggest that males and females exhibit a similar level of hyperacusis. However, in light of the subjective nature of this condition, the eventual set up of further tests to assess hyperacusis features could be very helpful in the near future.

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