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1.
Noise Health ; 26(120): 44-50, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38570310

RESUMO

OBJECTIVE: The aim of this study was to verify the association between the auditory handicap found in the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) questionnaire and hearing loss and the plasma levels of inflammatory biomarkers. MATERIALS AND METHODS: Cross-sectional study with 76 participants, 67 (88%) females and 9 (12%) males, with a mean age of 70 years. Tonal threshold audiometry and self-assessment with HHIE-S questionnaire were performed to measure the plasma levels of interleukin-2 (IL-2), IL-4, IL-6, and IL-10; tumor necrosis factor alpha; and interferon gamma (IFN-γ) flow cytometry method. For all data analyzed, the significance level adopted was P < 0.05 and 95% confidence interval. RESULTS: An inverse correlation was observed between the increase in plasma levels of IFN-γ and normal auditory handicap (P = 0.015; rs = -0.280). The severe handicap group showed an increase in the averages I (P = 0.005; rs = 0.350) and II (P = 0.016; rs = 0.368) in the right ear and the light/moderate handicap group increased the means I (P = 0.027; rs = 0.350) and II (P = 0.046; rs = 0.310) of the left ear. A statistically significant association was found between the speech recognition threshold (SRT) test results of the right ear and the severe handicap group (P = 0.002; rs = 0.271). CONCLUSIONS: There was an association between the increase in plasma levels of IFN-γ and normal auditory handicap. Additionally, statistically significant associations were observed between the mild/moderate and severe handicap groups with the increase in hearing means and an increase in SRT associated with the severe handicap group.


Assuntos
Perda Auditiva , Interferon gama , Masculino , Feminino , Humanos , Idoso , Estudos Transversais , Audiometria de Tons Puros , Perda Auditiva/diagnóstico , Inquéritos e Questionários , Sensação
2.
Artigo em Chinês | MEDLINE | ID: mdl-38563168

RESUMO

Objective:This study analyzed the pure tone audiometry results of the affected ear and the contralateral ear of unilateral Meniere's disease to investigate the correlation of the hearing threshold of the contralateral ear and the hearing prognosis of unilateral Meniere's disease. Methods:In this study, the follow-up data of 135 patients with unilateral Meniere's disease in Beijing Tongren Hospital were used to analyze the pure tone audiometry results of the affected and contralateral ears at the first visit and 1 year later. Results:①At the first visit, there was no statistically significant difference between the mean hearing thresholds of the affected ear in the normal hearing group and the high-frequency hearing loss group of the contralateral ear(P>0.05). ②The range of improvement of hearing thresholds in the affected ear was greater in the contralateral ear normal hearing group than in the contralateral ear high-frequency hearing loss group. In the normal hearing group of the contralateral ear, the hearing thresholds of the affected ear at 0.25 kHz(P<0.01), 0.50 kHz(P<0.01), 1.00 kHz(P<0.01), and 2.00 kHz(P<0.05) were significantly improved; and in the high-frequency hearing loss group of the contralateral ear, the hearing thresholds at 0.25 kHz(P<0.01) hearing thresholds improved significantly, and there was no significant difference between the rest of the frequencies before and after treatment(P>0.05). A consistent pattern was observed in both higher and lower age groups. ③After 1 year of follow-up, the low and mid-frequency hearing of the affected ear improved. 0.25 kHz(P<0.01), 0.50 kHz(P<0.01), 1.00 kHz(P<0.01) hearing thresholds improved significantly; 8.00 kHz hearing thresholds decreased slightly(P<0.05). Conclusion:After standardized treatment, the results of 1-year follow-up suggested that the low-frequency hearing of MD patients could be improved, but the high-frequency hearing was slightly decreased. The hearing prognosis of the affected ear with normal hearing threshold of the contralateral ear may be better.


Assuntos
Doença de Meniere , Humanos , Doença de Meniere/diagnóstico , Perda Auditiva de Alta Frequência , Orelha , Audição , Audiometria de Tons Puros , Prognóstico
3.
Sci Rep ; 14(1): 7359, 2024 03 28.
Artigo em Inglês | MEDLINE | ID: mdl-38548854

RESUMO

Pure-tone audiometry (PTA) is the gold standard for assessing hearing loss. However, traditional PTA tests require specialized equipment, trained personnel, and a soundproof environment. Recently, smartphone-based PTA tests have been developed as an alternative method for hearing assessment. The aim of this study was to validate the accuracy and reliability of a smartphone application-based audiometry test. This study was conducted to assess the performance of application-based audiometry from November 2021 to January 2022. Pure-tone thresholds were measured using a smartphone application-based PTA test and compared with results obtained using a traditional audiometer in a sound-treated booth. The smartphone application used in this study was the "Care4Ear (Care4ear, version 1.0.6, MIJ Co., Ltd.)". Hearing thresholds less than 35 dB HL were classified as group A, 35-64 dB HL as group B, and 65 dB HL or greater as group C for the classification of hearing levels. We evaluated the accuracy of smartphone audiometry for each group and compared the results of frequency-specific hearing tests. Additionally, we examined the results of smartphone audiometry in individuals (n = 27) with asymmetric hearing loss. Seventy subjects completed both conventional audiometry and smartphone application-based hearing tests. Among the ears assessed, 55.7% were classified as group A, while 25.7% and 18.6% were classified as group B and group C, respectively. The average hearing threshold obtained from conventional pure-tone audiometry was 37.7 ± 25.2 dB HL, whereas the application-based hearing test yielded thresholds of 21.0 ± 23.0 dB HL. A significant correlation (r = 0.69, p < 0.01) was found between the average hearing thresholds obtained from the application-based and conventional pure-tone audiometry tests. The application-based test achieved a 97.4% hit rate for classifying hearing thresholds as class A, but lower rates of 22.2% for class B and 38.5% for class C. Notably, a discrepancy was observed between the hearing threshold measured by the application and the conventional audiometry for the worse ear with asymmetric hearing. The smartphone-based audiometry is a feasible method for hearing evaluation especially in persons with normal hearing. In cases of hearing loss or asymmetric hearing loss, the results of the application-based audiometry may be inaccurate, limiting its diagnostic utility.


Assuntos
Surdez , Perda Auditiva , Humanos , Reprodutibilidade dos Testes , Limiar Auditivo , Perda Auditiva/diagnóstico , Audição , Audiometria de Tons Puros/métodos
4.
Nat Commun ; 15(1): 1896, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38429256

RESUMO

Inhibition of Notch signalling with a gamma-secretase inhibitor (GSI) induces mammalian hair cell regeneration and partial hearing restoration. In this proof-of-concept Phase I/IIa multiple-ascending dose open-label trial (ISRCTN59733689), adults with mild-moderate sensorineural hearing loss received 3 intratympanic injections of GSI LY3056480, in 1 ear over 2 weeks. Phase I primary outcome was safety and tolerability. Phase lla primary outcome was change from baseline to 12 weeks in average pure-tone air conduction threshold across 2,4,8 kHz. Secondary outcomes included this outcome at 6 weeks and change from baseline to 6 and 12 weeks in pure-tone thresholds at individual frequencies, speech reception thresholds (SRTs), Distortion Product Otoacoustic Emissions (DPOAE) amplitudes, Signal to Noise Ratios (SNRs) and distribution of categories normal, present-abnormal, absent and Hearing Handicap Inventory for Adults/Elderly (HHIA/E). In Phase I (N = 15, 1 site) there were no severe nor serious adverse events. In Phase IIa (N = 44, 3 sites) the average pure-tone threshold across 2,4,8 kHz did not change from baseline to 6 and 12 weeks (estimated change -0.87 dB; 95% CI -2.37 to 0.63; P = 0.252 and -0.46 dB; 95% CI -1.94 to 1.03; P = 0.545, respectively), nor did the means of secondary measures. DPOAE amplitudes, SNRs and distribution of categories did not change from baseline to 6 and 12 weeks, nor did SRTs and HHIA/E scores. Intratympanic delivery of LY3056480 is safe and well-tolerated; the trial's primary endpoint was not met.


Assuntos
Secretases da Proteína Precursora do Amiloide , Perda Auditiva Neurossensorial , Adulto , Idoso , Humanos , Audiometria de Tons Puros , Limiar Auditivo/fisiologia , Perda Auditiva Neurossensorial/tratamento farmacológico , Emissões Otoacústicas Espontâneas/fisiologia
5.
Fa Yi Xue Za Zhi ; 40(1): 15-19, 2024 Feb 25.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-38500456

RESUMO

OBJECTIVES: To study the application of CE-Chirp in the evaluation of hearing impairment in forensic medicine by testing the auditory brainstem response (ABR) in adults using CE-Chirp to analyze the relationship between the V-wave response threshold of CE-Chirp ABR test and the pure tone hearing threshold. METHODS: Subjects (aged 20-77 with a total of 100 ears) who underwent CE-Chirp ABR test in Changzhou De'an Hospital from January 2018 to June 2019 were selected to obtain the V-wave response threshold, and pure tone air conduction hearing threshold tests were conducted at 0.5, 1.0, 2.0 and 4.0 kHz, respectively, to obtain pure tone listening threshold. The differences and statistical differences between the average pure tone hearing threshold and V-wave response threshold were compared in different hearing levels and different age groups. The correlation, differences and statistical differences between the two tests at each frequency were analyzed for all subjects. The linear regression equation for estimating pure tone hearing threshold for all subjects CE-Chirp ABR V-wave response threshold was established, and the feasibility of the equation was tested. RESULTS: There was no statistical significance in the CE-Chirp ABR response threshold and pure tone hearing threshold difference between different hearing level groups and different age groups (P>0.05). There was a good correlation between adult CE-Chirp ABR V-wave response threshold and pure tone hearing threshold with statistical significance (P<0.05), and linear regression analysis showed a significant linear correlation between the two (P<0.05). CONCLUSIONS: The use of CE-Chirp ABR V-wave response threshold can be used to evaluate subjects' pure tone hearing threshold under certain conditions, and can be used as an audiological test method for forensic hearing impairment assessment.


Assuntos
Perda Auditiva , Audição , Adulto , Humanos , Estimulação Acústica/métodos , Limiar Auditivo/fisiologia , Audição/fisiologia , Perda Auditiva/diagnóstico , Audiometria de Tons Puros/métodos , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia
6.
J Int Adv Otol ; 20(1): 1-7, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38454281

RESUMO

BACKGROUND: The clinical outcomes of cochlear implantation vary for several reasons. It is necessary to study the different electrodes and variables for further development. The aim of this study is to report the clinical outcomes of a new slim lateral wall electrode (SlimJ). METHODS: Data of 25 cochlear implantations in 23 patients with the SlimJ electrode were retrospectively collected. The insertion results were assessed by image fusion of the preoperative computed tomography (CT), magnetic resonance imaging (MRI), and postoperative cone-beam CT. The hearing outcomes were evaluated by the improvement of speech recognition in noise, measured preoperatively and at follow-up. Postoperative pure-tone thresholds were obtained in cases with preoperative functional low frequency hearing [PTA (0.125-0.5 kHz) ≤ 80 dB HL]. RESULTS: The preoperative mean speech reception threshold (SRT) was +0.6 dB signal-to-noise ratio (SNR) (SD ± 4.2 dB) and the postoperative -3.5 dB SNR (SD ± 2.3 dB). The improvements between the preoperative and postoperative SRT levels ranged from 0.0 to 15.1 dB, with a mean improvement of 4.2 dB (SD ± 3.6 dB). Residual hearing in low frequencies (mean PTA(125-500 Hz)) was preserved within 30 dB HL in 70% and within 15 dB HL in 40% of patients who had preoperatively functional low frequency hearing. Mean insertion depth angle (IDA) was 401° (SD ± 41°). We observed scalar translocations from scala tympani to scala vestibuli in 2 ears (9%). CONCLUSION: The relatively atraumatic insertion characteristics make the SlimJ array feasible for hearing preservation cochlear implantation. The hearing outcomes are comparable to those reported for other electrodes and devices.


Assuntos
Implante Coclear , Implantes Cocleares , Percepção da Fala , Humanos , Estudos Retrospectivos , Audição , Implante Coclear/métodos , Audiometria de Tons Puros , Resultado do Tratamento
7.
J Int Adv Otol ; 20(1): 44-49, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38454288

RESUMO

BACKGROUND: Our study centers on various aspects of families who have 2 or more members with hearing loss (HL) and are living in Golestan province in Iran. We aimed to identify those families with the highest probability of hereditary HL and also to examine the impact of consanguinity among them. METHODS: The families included in the study underwent a comprehensive screening process that involved their prenatal and postnatal histories as well as family medical histories. Additionally, each patient received a thorough clinical ear examination. The evaluation also took into account factors such as patterns of inheritance, consanguinity, a 3-generation pedigree, and physical examination. Following this initial assessment, patients were referred for a complete hearing evaluation, which included pure-tone audiometry, speech recognition threshold, otoacoustic emission, and auditory brainstem response tests. RESULTS: We identified a total of 8553 individuals living in Golestan province who are hearing impaired. Among those, our records indicate that 320 families had at least 2 affected members. The rate of consanguinity marriage in non-syndromic families was 64.43%. Also, a significant number (88.12%, or n=282) of the families exhibited hereditary HL, among which a substantial proportion (89.72%, or n=253) presented with nonsyndromic forms of HL. Furthermore, bilateral, stable, and prelingual HL were the most frequently observed types, and a majority of the patients were diagnosed with sensorineural and profound HL. CONCLUSION: This study revealed a correlation between consanguinity and the incidence of familial HL, with more probability of bilateral, prelingual, sensorineural, and profound forms.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Humanos , Estudos Transversais , Irã (Geográfico)/epidemiologia , Perda Auditiva/epidemiologia , Perda Auditiva/genética , Perda Auditiva/complicações , Surdez/epidemiologia , Surdez/genética , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/genética , Audiometria de Tons Puros
8.
Artigo em Chinês | MEDLINE | ID: mdl-38433684

RESUMO

Objective:To analyze the clinical feature, diagnosis and treatment of Anca-associated vasculitis with ear symptoms. Methods:In this retrospective study, we summarized the clinical and laboratory examination, pure tone audiometry, aural immittance measurement, CT scan of temporal bone and treatment of 40 patients in the First Medical Center of the PLA General Hospital. Results:A total of 11 cases(27.5%) had the initial symptom in the ear. The most common symptoms were hearing loss, and the other symptoms included a sense of ear fullness, otorrhea and tinnitus. There were 35 cases with hearing loss: 19 cases with conductive hearing loss(47.5%), 9 cases with sensorineural hearing loss(22.5%), and 7 cases with mixed hearing loss(17.5%). 5 cases had a sense of ear fullness or tinnitus, and the results of the hearing test were normal(12.5%). All of the 40 patients had multi-system involvement, and respiratory system accounted for the most. All patients had a positive result of Anti-neutrophil cytoplasmic antibody(ANCA). Treatment included systemic hormonal, immunosuppressive, or biologic therapy. There were 3 cases recovered(7.5%), 22 cases with alleviated ear symptoms(55.0%), 6 cases with recurrent hearing loss(15%) and 9 cases had no significant improvement(22.5%). Conclusion:Conductive deafness(secretory otitis media) can be the first manifestation in the early stage of otitis media with AAV(OMAAV), later it may turn to binaural mixed deafness. Otolaryngologists need to consider OMAAV diagnosis when diagnosing and treating patients with recurrent secretory otitis media. Multi-system symptom consultation and ANCA examination can help identify. Early systemic medication and the application of immunosuppressants or biological agents can help relieve the ear symptoms.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Surdez , Otite Média com Derrame , Zumbido , Humanos , Anticorpos Anticitoplasma de Neutrófilos , Estudos Retrospectivos , Perda Auditiva Condutiva , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Audiometria de Tons Puros
9.
NEJM Evid ; 3(1): EVIDe2300296, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38320518

RESUMO

Hearing impairment is the most common sensory deficit. It affects 2 to 3 of 1000 newborns and nearly 50% of adults 75 years of age and older in the United States.1 Idiopathic sudden sensorineural hearing loss (ISSNHL) is characterized by an abrupt hearing loss requiring immediate diagnosis and treatment. Systemic glucocorticoids are widely used as the primary treatment for ISSNHL,2 but no head-to-head comparisons of the effectiveness and risk profiles of high doses over a more commonly used lower dose of glucocorticoids have been conducted to inform standard-of-care practice.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Recém-Nascido , Adulto , Humanos , Glucocorticoides/uso terapêutico , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Neurossensorial/diagnóstico , Audiometria de Tons Puros
10.
Otol Neurotol ; 45(4): 376-385, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38361325

RESUMO

OBJECTIVE: To investigate if pharmacological treatment with prednisone and L-N-acetylcysteine (STE + NAC) influence functional hearing preservation in cochlear implant (CI) surgery. STUDY DESIGNS: Preimplantation and postimplantation longitudinal case-control study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult recipients of CI with preimplantation functional hearing defined as an average of air-conducted thresholds at 125, 250, and 500 Hz (low-frequency pure-tone average [LFPTA]) <80 dB. INTERVENTIONS: Preimplantation and postimplantation audiometry. Weight-adjusted oral prednisone and L-N-acetylcysteine starting 2 days before surgery (Miami cocktail). Prednisone was continued for 3 days and L-N-acetylcysteine for 12 days after surgery, respectively. Cochlear implantation with conventional length electrodes. MAIN OUTCOME MEASURES: Proportion of patients with LFPTA <80 dB, and LFPTA change at 1-year postimplantation. RESULTS: All 61 patients received intratympanic and intravenous dexamethasone intraoperatively, with 41 patients receiving STE + NAC and 20 patients not receiving STE + NAC. At 1-year postimplantation, the proportion of functional hearing preservation was 83% in the STE + NAC group compared with 55% of subjects who did not receive STE + NAC ( p = 0.0302). The median LFPTA change for STE + NAC-treated and not treated subjects was 8.33 dB (mean, 13.82 ± 17.4 dB) and 18.34 dB (mean, 26.5 ± 23.4 dB), respectively ( p = 0.0401, Wilcoxon rank test). Perioperative STE + NAC treatment resulted in 10 dB of LFPTA better hearing than when not receiving this treatment. Better low-frequency preimplantation hearing thresholds were predictive of postimplantation functional hearing. No serious side effects were reported. CONCLUSION: Perioperative STE + NAC, "The Miami Cocktail," was safe and superior to intraoperative steroids alone in functional hearing preservation 1-year after cochlear implantation.


Assuntos
Implante Coclear , Implantes Cocleares , Adulto , Humanos , Criança , Implante Coclear/métodos , Estudos de Casos e Controles , Prednisona , Acetilcisteína , Estudos Retrospectivos , Limiar Auditivo , Audiometria de Tons Puros , Audição , Resultado do Tratamento
11.
PLoS One ; 19(2): e0290480, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38422002

RESUMO

Idiopathic sudden sensorineural hearing loss (ISSNHL) is the rapid onset of reduced hearing due to loss of function of the inner ear or hearing nerve of unknown aetiology. Evidence supports improved hearing recovery with early steroid treatment, via oral, intravenous, intratympanic or a combination of routes. The STARFISH trial aims to identify the most clinically and cost-effective route of administration of steroids as first-line treatment for ISSNHL. STARFISH is a pragmatic, multicentre, assessor-blinded, three-arm intervention, superiority randomised controlled trial (1:1:1) with an internal pilot (ISRCTN10535105, IRAS 1004878). 525 participants with ISSNHL will be recruited from approximately 75 UK Ear, Nose and Throat units. STARFISH will recruit adults with sensorineural hearing loss averaging 30dBHL or greater across three contiguous frequencies (confirmed via pure tone audiogram), with onset over a ≤3-day period, within four weeks of randomisation. Participants will be randomised to 1) oral prednisolone 1mg/Kg/day up to 60mg/day for 7 days; 2) intratympanic dexamethasone: three intratympanic injections 3.3mg/ml or 3.8mg/ml spaced 7±2 days apart; or 3) combined oral and intratympanic steroids. The primary outcome will be absolute improvement in pure tone audiogram average at 12-weeks following randomisation (0.5, 1.0, 2.0 and 4.0kHz). Secondary outcomes at 6 and 12 weeks will include: Speech, Spatial and Qualities of hearing scale, high frequency pure tone average thresholds (4.0, 6.0 and 8.0kHz), Arthur Boothroyd speech test, Vestibular Rehabilitation Benefit Questionnaire, Tinnitus Functional Index, adverse events and optional weekly online speech and pure tone hearing tests. A health economic assessment will be performed, and presented in terms of incremental cost effectiveness ratios, and cost per quality-adjusted life-year. Primary analyses will be by intention-to-treat. Oral prednisolone will be the reference. For the primary outcome, the difference between group means and 97.5% confidence intervals at each time-point will be estimated via a repeated measures mixed-effects linear regression model.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Adulto , Humanos , Audiometria de Tons Puros , Audição , Perda Auditiva Neurossensorial/tratamento farmacológico , Estudos Multicêntricos como Assunto , Prednisolona/uso terapêutico , Ensaios Clínicos Controlados Aleatórios como Assunto
12.
HNO ; 72(4): 291-302, 2024 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-38351342

RESUMO

Idiopathic sudden sensorineural hearing loss (ISSNHL) is one of the most common diseases in otolaryngology. Its etiology remains unknown. Furthermore, there is only a low level of evidence for the efficacy of established treatment modalities. In addition to systemic glucocorticoids, intratympanic corticosteroid treatment (ICT) has become increasingly important for treatment of ISSNHL. Different application strategies and treatment regimens have been described; however, uniform standards do not yet exist. ICT may be used for primary treatment as well as salvage therapy. Current data from meta-analyses show no benefit of intratympanic versus systemic primary therapy for sudden hearing loss (moderate evidence) but suggest a benefit of intratympanic secondary treatment over no treatment or placebo (high effect size, low evidence). Regarding combination of systemic and local glucocorticoid therapy in primary treatment of hearing loss, there may be a small benefit over systemic treatment alone (low effect size, low evidence).


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Glucocorticoides , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/tratamento farmacológico , Dexametasona/uso terapêutico , Resultado do Tratamento , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/etiologia , Corticosteroides/uso terapêutico , Injeção Intratimpânica , Audiometria de Tons Puros/efeitos adversos
13.
Artigo em Chinês | MEDLINE | ID: mdl-38297844

RESUMO

Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response(ASSR), auditory brainstem response(ABR) thresholds, and deformed partial otoacoustic emission(DPOAE). Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics(ACMG) Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing(WES) and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29(21.6%) had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66(49.3%) patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases(51.5%), STRC in 10 cases(15.1%), MPZL2 gene in six cases(9.1%), and USH2A in five cases(7.6%).The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases(47.1%) and c. 109G>A compound heterozygous mutation in 9 cases(26.5%). Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.


Assuntos
Perda Auditiva Neurossensorial , Síndromes de Usher , Humanos , Criança , Conexinas/genética , Conexina 26/genética , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/diagnóstico , Mutação , Perda Auditiva Bilateral , Audiometria de Tons Puros , Peptídeos e Proteínas de Sinalização Intercelular
14.
Artigo em Chinês | MEDLINE | ID: mdl-38297852

RESUMO

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Hiperlipidemias , Humanos , Audiometria de Tons Puros , Surdez/complicações , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Súbita/diagnóstico , Hiperlipidemias/complicações , Lipídeos
15.
Medicina (Kaunas) ; 60(2)2024 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-38399554

RESUMO

Background and Objectives: Granulomatosis with Polyangiitis (GPA) is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma that commonly affects the upper and lower respiratory tract and kidneys. Audiovestibular dysfunction in GPA diseases may have different clinical presentations. The aim of the present study was to evaluate hearing function in patients with GPA and to compare the results with a healthy control group. Materials and Methods: A total of 34 individuals participated in the study. The GPA group consisted of 14 participants, and the control group was composed of 20 healthy participants with no signs or symptoms of ear disease. The ages ranged from 18 to 65 years old, with a mean age of 43.8 years. The participants underwent a complete audiological evaluation using otoscopy, impedance audiometry, pure tone audiometry, speech audiometry-evaluation of speech thresholds, and speech recognition in quiet. Both ears were tested. All of the participants of the study were native Lithuanian speakers. Data were statistically analyzed using the Statistical Analysis System software SAS® Studio 3.8. A p value < 0.05 was regarded as statistically significant. Results: 92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. Conclusions: The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities.


Assuntos
Surdez , Granulomatose com Poliangiite , Perda Auditiva Neurossensorial , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Granulomatose com Poliangiite/complicações , Audiometria de Tons Puros/métodos
16.
Ann Otol Rhinol Laryngol ; 133(4): 400-405, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38197374

RESUMO

OBJECTIVE: Hyperbaric oxygen therapy (HBOT) is an accepted treatment option for sudden sensorineural hearing loss (SSNHL), but it is still recommended in combination with corticosteroids. We investigated the efficacy of salvage HBOT in refractory SSNHL that does not respond to corticosteroid combination therapy. METHODS: Eighty-four patients were included, who had unilateral SSNHL with an improvement of pure-tone average (PTA) less than 10 dB after using intratympanic plus systemic corticosteroids (combined therapy) as the initial therapy. The control group (n = 66) received no further treatment, and the HBOT group (n = 18) received additional treatment with HBOT (10 sessions in total with 2.5 atmospheres absolute for 1 hour). RESULTS: No differences in PTA or WDS were found between the 2 groups. However, the mean hearing gain in the HBOT group (16.8 ± 4.49 dB) was significantly higher than that in the control group (4.45 ± 1.03 dB) (P = .015). The proportion of patients with hearing recovery (hearing gain of 10 dB or more) after treatment was significantly higher in HBOT group (38.9%) than in the control group (10.6%). CONCLUSIONS: In patients with refractory SSNHL after steroid combined therapy, salvage HBOT showed a significant effect on hearing gain and recovery rate.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Oxigenoterapia Hiperbárica , Humanos , Perda Auditiva Súbita/terapia , Perda Auditiva Neurossensorial/terapia , Glucocorticoides/uso terapêutico , Dexametasona/uso terapêutico , Esteroides , Terapia de Salvação , Resultado do Tratamento , Audiometria de Tons Puros
17.
Sci Rep ; 14(1): 360, 2024 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-38172523

RESUMO

Idiopathic sudden sensorineural hearing loss (ISSNHL) is challenging for both nephrologists and otolaryngologists treating patients undergoing dialysis. This single-center, retrospective, observational study investigated the treatment outcomes of patients with ISSNHL undergoing dialysis, enrolling 700 patients (47 undergoing and 653 not undergoing dialysis) diagnosed with ISSNHL between January 2005 and December 2021 at Asan Medical Center, Republic of Korea. To balance pre-existing clinical characteristics, 1:5 propensity score matching (PSM) was performed with the patients who were not undergoing dialysis. Treatment included high-dose systemic steroid therapy or intra-tympanic steroid injections. The pure tone average of the groups was compared before and 2 weeks and 2 months after treatment. The hearing-improvement degree was evaluated using Siegel's criteria. Before PSM, age, prevalence of diabetes or hypertension, initial hearing threshold at each frequency level (0.5, 1, 2, and 4 kHz), and treatment strategies exhibited significant between-group differences. However, in the PS-matched cohort, none of the confounders showed significant between-group differences. Two months after steroid treatment, the non-dialysis patient group demonstrated significantly higher average improvement in pure tone audiometry (P = 0.029) and greater percentage of complete response according to Siegel's criteria. This study suggests that treatment outcomes for ISSNHL are significantly poorer for patients undergoing than for those not undergoing dialysis.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Diálise Renal , Resultado do Tratamento , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Súbita/tratamento farmacológico , Perda Auditiva Súbita/diagnóstico , Esteroides/uso terapêutico , Estudos Retrospectivos , Audiometria de Tons Puros , Glucocorticoides/uso terapêutico
18.
Trends Hear ; 28: 23312165231225545, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38192152

RESUMO

This study aimed to assess the validity of a high-frequency audiometry tool based on Bayesian learning to provide a reliable, repeatable, automatic, and fast test to clinics. The study involved 85 people (138 ears) who had their high-frequency thresholds measured with three tests: standard audiometry (SA), alternative forced choice (AFC)-based algorithm, and Bayesian active (BA) learning-based algorithm. The results showed median differences within ±5 dB up to 10 kHz when comparing the BA with the other two tests, and median differences within ±10 dB at higher frequencies. The variability increased from lower to higher frequencies. The BA showed lower thresholds compared to the SA at the majority of the frequencies. The results of the different tests were consistent across groups (age, hearing loss, and tinnitus). The data for the BA showed high test-retest reliability (>90%). The time required for the BA was shorter than for the AFC (4 min vs. 13 min). The data suggest that the BA test for high-frequency audiometry could be a good candidate for clinical screening. It would add reliable and significant information without adding too much time to the visit.


Assuntos
Surdez , Aprendizagem , Humanos , Teorema de Bayes , Reprodutibilidade dos Testes , Audiometria de Tons Puros
19.
JAMA Otolaryngol Head Neck Surg ; 150(2): 157-164, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38175662

RESUMO

Importance: Hearing loss has been suggested as a risk factor for dementia, but there is still a need for high-quality research to better understand the association between these 2 conditions and the underlying causal mechanisms and treatment benefits using larger cohorts and detailed data. Objective: To investigate the association between hearing loss and incident dementia, as well as how hearing aid use contributes to this association. Design, Setting, and Participants: This population-based cohort study was conducted in Southern Denmark between January 2003 and December 2017 and included all residents 50 years and older. We excluded all persons with dementia before baseline as well as those who did not live in the region 5 years before baseline, with incomplete address history, or who had missing covariate information. Exposures: Individual hearing status based on the Hearing Examinations in Southern Denmark database, which contains data on all pure-tone audiometry examinations performed at public hearing rehabilitation clinics in Southern Denmark. Main Outcomes and Measures: Incident cases of dementia and Alzheimer disease as identified from national registries. Results: The study population comprised 573 088 persons (298 006 women [52%]; mean [SD] age, 60.8 [11.3] years) with 23 023 cases of dementia and mean (SD) follow-up of 8.6 (4.3) years. Having a hearing loss was associated with an increased risk of dementia, with an adjusted hazard ratio (HR) of 1.07 (95% CI, 1.04-1.11) compared with having no hearing loss. Severe hearing loss in the better and worse ear was associated with a higher dementia risk, with an HR of 1.20 (95% CI, 1.09-1.32) and 1.13 (95% CI, 1.06-1.20), respectively, compared with having no hearing loss in the corresponding ear. Compared with people without hearing loss, the risk of dementia was higher among people with hearing loss who were not using hearing aids than those who had hearing loss and were using hearing aids, with HRs of 1.20 (95% CI, 1.13-1.27) and 1.06 (95% CI, 1.01-1.10), respectively. Conclusions and Relevance: The results of this cohort study suggest that hearing loss was associated with increased dementia risk, especially among people not using hearing aids, suggesting that hearing aids might prevent or delay the onset and progression of dementia. The risk estimates were lower than in previous studies, highlighting the need for more high-quality longitudinal studies.


Assuntos
Doença de Alzheimer , Surdez , Auxiliares de Audição , Perda Auditiva , Humanos , Feminino , Idoso , Pessoa de Meia-Idade , Estudos de Coortes , Perda Auditiva/complicações , Audiometria de Tons Puros , Fatores de Risco
20.
Acta Otolaryngol ; 144(1): 35-38, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38279924

RESUMO

BACKGROUND: Some studies have shown a positive effect of systemic corticosteroid on hearing results after stapedotomy, but its side effects can limit its routine administration. AIM: The aim of this study was to investigate the effect of local dexamethasone on the results of stapedotomy surgery. MATERIAL AND METHODS: Fifty two patients undergone stapedotomy surgery for otosclerosis involved. In the case group after stapedotomy we fulfilled the middle ear with dexamethasone and then the placement of the prosthesis was done. In the control group after stapedotomy we did not use dexamethasone in the middle ear. RESULTS: Gender, age, nausea, vomiting, postoperative vertigo and nystagmus did not significantly differ between the groups. A significant difference was observed in tinnitus rate between two groups. In the case group ABG decrease was higher and bone conduction thresholds improved at frequencies of 1000, 2000, and 4000 three months after surgery. CONCLUSION: Since local dexamethasone had a positive effect on the results of stapedotomy surgery, it can be used instead of systemic corticosteroids to reduce the side effects and increasing surgery's success rate. SIGNIFICANCE: If local dexamethasone had a positive influence on the results of stapedotomy surgery, it can be used instead of systemic corticosteroids to reduce the side effects and increasing surgery's success rate.


Assuntos
Otosclerose , Cirurgia do Estribo , Humanos , Otosclerose/cirurgia , Audiometria de Tons Puros , Cirurgia do Estribo/métodos , Condução Óssea , Orelha Média , Estudos Retrospectivos , Corticosteroides , Dexametasona/uso terapêutico , Resultado do Tratamento
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