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1.
Sci Rep ; 14(1): 14203, 2024 06 20.
Article in English | MEDLINE | ID: mdl-38902305

ABSTRACT

Hearing problems are commonly diagnosed with the use of tonal audiometry, which measures a patient's hearing threshold in both air and bone conduction at various frequencies. Results of audiometry tests, usually represented graphically in the form of an audiogram, need to be interpreted by a professional audiologist in order to determine the exact type of hearing loss and administer proper treatment. However, the small number of professionals in the field can severely delay proper diagnosis. The presented work proposes a neural network solution for classification of tonal audiometry data. The solution, based on the Bidirectional Long Short-Term Memory architecture, has been devised and evaluated for classifying audiometry results into four classes, representing normal hearing, conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. The network was trained using 15,046 test results analysed and categorised by professional audiologists. The proposed model achieves 99.33% classification accuracy on datasets outside of training. In clinical application, the model allows general practitioners to independently classify tonal audiometry results for patient referral. In addition, the proposed solution provides audiologists and otolaryngologists with access to an AI decision support system that has the potential to reduce their burden, improve diagnostic accuracy, and minimise human error.


Subject(s)
Audiometry, Pure-Tone , Neural Networks, Computer , Humans , Audiometry, Pure-Tone/methods , Female , Male , Hearing Loss/diagnosis , Hearing Loss/classification , Adult , Middle Aged , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Conductive/diagnosis , Hearing Loss, Conductive/classification
2.
Otolaryngol Head Neck Surg ; 162(3): 337-342, 2020 Mar.
Article in English | MEDLINE | ID: mdl-31986973

ABSTRACT

OBJECTIVES: Idiopathic sudden sensorineural hearing loss (ISSNHL) is a distressing condition that can significantly affect quality of life. Unilateral ISSNHL, occurring first in 1 ear and then the contralateral ear at a separate and discrete time, is a rare presentation that we refer to as metachronous ISSNHL. Our objective was to characterize the presentation of metachronous ISSNHL and report on management and hearing outcomes. STUDY DESIGN: Retrospective case series. SETTING: Otology clinic at an academic tertiary referral center. SUBJECTS AND METHODS: Patients ≥18 years old presenting with metachronous ISSNHL between April 2008 to November 2017 were identified through review of the clinic electronic medical record. Metachronous ISSNHL was defined as unilateral ISSNHL occurring in temporally discrete episodes (>6 months apart) affecting both ears. Patients with identifiable causes for sudden hearing loss were excluded. Patient demographics, comorbidities, management, and audiologic outcomes were recorded. RESULTS: Eleven patients with metachronous ISSNHL were identified out of 558 patients with ISSNHL. In patients with metachronous ISSNHL, the mean ± standard deviation age at the time of ISSNHL in the second ear was 58.6 ± 15.2 years (range, 31-77 years). The mean interval between episodes was 9.6 ± 7.5 years (range, 1-22 years). Patients were treated with systemic and intratympanic steroids with variable hearing recovery; 5 patients with resultant bilateral severe to profound hearing loss underwent successful cochlear implantation. CONCLUSION: Metachronous ISSNHL is uncommon. Treatment is similar to ISSNHL, and cochlear implantation can successfully restore hearing in individuals who do not experience recovery.


Subject(s)
Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/therapy , Hearing Loss, Sudden/classification , Hearing Loss, Sudden/therapy , Comorbidity , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Quality of Life , Recovery of Function , Retrospective Studies
3.
Eur Arch Otorhinolaryngol ; 277(2): 393-400, 2020 Feb.
Article in English | MEDLINE | ID: mdl-31691017

ABSTRACT

PURPOSE: To determine the efficacy and safety of the intratympanic infiltration of infliximab at the hearing threshold of patients in follow-up for refractory immune-mediated hearing loss. METHODS: 17 patients were collected with relapses, despite maintenance treatment with oral azathioprine associated or not with oral prednisone at low doses (between 5 and 7.5 ml/day) or refractory relapses to previous intratympanic corticoid treatment being 19 affected ears infiltrated. We measured the hearing threshold by Pure-Tone Average (PTA) 500-3000 Hz, 125-8000 Hz and 250-8000 Hz in pre-infiltration (baseline) and follow-up 3 weeks post-infiltration with auditory threshold at frequencies 125-8000 Hz. RESULTS: The average age was 50.68 years (±15.23 years). After the administration of intratympanic infliximab, an improvement of the hearing threshold was showed in the Pure-Tone Average (PTA) calculated at 500-3000 Hz (p = 0.004), 125-8000 Hz (p = 0.001) and 250-8000 Hz (p = 0.006). An immediate improvement in low frequencies also was observed: 125, 250 and 500 Hz (p = 0.009, p = 0.002 and p < 0.001 respectively) also at 1000 Hz (p = 0.004) and a persistence of the effect at 3 months in the low frequencies: 125 Hz (p = 0.020), 250 Hz (p = 0.006) and 500 Hz (p = 0.002). CONCLUSIONS: Infliximab intratympanic infiltration improves the hearing threshold in patients with immune-mediated hearing loss. The effect of improving the hearing threshold is higher in low frequencies and persists within 3 months of the infiltration. The administration of intratympanic infliximab is an effective and safe technique.


Subject(s)
Hearing Loss, Sensorineural/drug therapy , Immunosuppressive Agents/administration & dosage , Infliximab/administration & dosage , Administration, Oral , Adult , Aged , Auditory Threshold , Azathioprine/administration & dosage , Chronic Disease , Female , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/immunology , Humans , Injection, Intratympanic , Longitudinal Studies , Male , Middle Aged , Prednisone/administration & dosage , Prospective Studies , Recurrence , Treatment Outcome
4.
Article in Chinese | MEDLINE | ID: mdl-31163550

ABSTRACT

Objective: To investigate the correlation between plasma fibrinogen level and the incidence of sudden hearing loss. Method: A total of 225 patients (age ranging from 18 to 70 years) with sudden sensorineural hearing loss(SSNHL) were selected. The disease onset was within 2 weeks. No patient received previous medical intervention. Depending on the types of auditory threshold curve, SSNHL was divided into low frequency descending type, high frequency descending type, flat desending type and total deafness type. Two hundred and twenty-nine cases with normal hearing and no inflammatory diseases in the same period was selected as control group. Fibrinogen level and Blood Routine Indexes were detected for the purpose of retrospective cohort study.Result: The levels of fibrinogen in SSNHL group(2.98±0.59) g/L were significantly higher than that in control group(2.66±0.36) g/L (P<0.01). According to the types of auditory threshold curve, SSNHL groups were divided into group A(55 cases, 24.44%), Group B(36 cases, 16.00%), Group C(43 cases,19.11%) and Group D(91 cases, 40.44%). The average fibrinogen levels before treatment were at(2.75±0.46)g/L, (3.16±0.61) g/L, (3.02±0.63) g/L and(3.03±0.63) g/L respectively. There was no significant difference in fibrinogen level(P=0.286) between group A and the control group. Fibrinogen levels of group B, group C and group D were significantly elevated compared to control group (P<0.01). Conclusion: The level of fibrinogen is not significantly correlated with development of sudden deafness of low frequency descending type. The incidence of high frequency descending type, flat descending type and profound deafness type are all correlated with the level of high fibrinogen at the onset of sudden deafness. The classification of sudden deafness based on audiogram curves could be of great significance for analysis of possible causes and selection of treatment options.


Subject(s)
Fibrinogen/analysis , Hearing Loss, Sensorineural/blood , Hearing Loss, Sudden/blood , Adolescent , Adult , Aged , Case-Control Studies , Hearing Loss, Sensorineural/classification , Hearing Loss, Sudden/classification , Hearing Tests , Humans , Middle Aged , Retrospective Studies , Young Adult
5.
Article in Spanish | LILACS | ID: biblio-1000311

ABSTRACT

INTRODUCCIÓN: El peso al nacer menor a 1.500 gramos es considerado un factor de riesgo auditivo. Analizamos una muestra de 137 niños nacidos con muy bajo peso, atendidos en un hospital pediátrico de alta complejidad. OBJETIVOS: Determinar la frecuencia de hipoacusia neurosensorial; analizar las diferencias entre los subgrupos: screening auditivo neonatal y pacientes de consultorio externo; valorar si existen diferencias entre los subgrupos de peso menor a 1.000 gramos y mayor a 1.000 gramos; investigar el grado de hipoacusia y determinar si la misma es unilateral o bilateral; estimar el porcentaje de niños de screening auditivo que regresaron para seguimiento; describir los factores de riesgo auditivo que presenta la muestra.


INTRODUCTION: Birth weight less than 1,500 grams is considered an auditory risk factor. We analyzed a sample of 137 children born with very low weight and treated in a high complexity pediatric hospital. OBJECTIVE: Determine frequency of sensorineural hearing loss; to analyze differences between the subgroups: neonatal auditory screening and patients from the outpatient clinic; assess if there are differences between subgroups of less and than 1,000 grams; investigate the degree of hearing loss and determine whether it is unilateral or bilateral; estimate the percentage of hearing screening children who returned for follow-up; describe the auditory risk factors presented by the sample.


INTRODUÇÃO: O peso do bebe ao nascer menor do que 1500 gramas é considerado um fator de risco auditivo. Neste trabalho, foi analisada uma amostra de 137 crianças nascidas com muito baixo peso, atendidos em um hospital pediátrico de alta complexidade. Os objetivos do trabalho foram: determinar a frequência de perda auditiva neurossensorial; analisar diferenças entre os subgrupos: screening auditivo neonatal e pacientes de consultório externo; avaliar se existem diferenças entre os subgrupos de peso menor do que 1000 gramas; investigar o grau de perda auditiva e determinar se a mesma foi unilateral ou bilateral; estimar a percentagem de crianças de screening auditivo que regressaram para acompanhamento; descrever os fatores de risco auditivo que apresentou a amostra. MATERIAL E MÉTODO: Foram excluídos pacientes oncológicos, com traumatismo cranioencefálico, transplantados ou com cirurgias do ouvido prévias ao diagnóstico da perda auditiva.


Subject(s)
Humans , Male , Adolescent , Infant, Very Low Birth Weight , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/epidemiology , Cross-Sectional Studies , Retrospective Studies , Risk Factors , Observational Study , Hearing Loss, Sensorineural/classification
7.
Codas ; 28(6): 687-696, 2016.
Article in Portuguese, English | MEDLINE | ID: mdl-27982251

ABSTRACT

PURPOSE: To establish the relationship between speech intelligibility index (SII) values generated at the verification of hearing aids programmed according to DSLm [i/o] v5 prescription rule and a proposed individual classification that considers the combination of hearing loss degree and configuration. METHODS: Forty-one children aged between 4 and 80 months were selected, totaling 78 ears for analysis. We considered hearing thresholds at the frequencies of 250, 500, 1000, 2000 and 4000 Hz; and analyzed values of the Speech Intelligibility Index (SII) for the input signal of 65 dB SPL obtained during the verification of hearing aids using the equipment Verifit®Audioscan. RESULTS: Hearing losses were classified into five homogeneous groups regarding audiometric degree and configuration. The groups were heterogeneous when compared to each other. From the groups, three ranges of SII values were determined. Equations were developed for classification of hearing loss according to groups and for determination of the adjusted SII values. CONCLUSION: The SII value is a useful indicator of audibility for speech sounds in different characteristics of hearing losses, and can guide observations of auditory skills. The SII has stronger relationship with the association of the audiometric degree and configuration when compared with degree of hearing loss alone.


Subject(s)
Auditory Threshold/physiology , Hearing Aids , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Speech Intelligibility/physiology , Audiometry , Child , Child, Preschool , Hearing Loss, Sensorineural/rehabilitation , Humans , Infant , Severity of Illness Index , Speech Perception
8.
CoDAS ; 28(6): 687-696, nov.-dez. 2016. tab, graf
Article in Portuguese | LILACS | ID: biblio-828594

ABSTRACT

RESUMO Objetivo estabelecer relações entre valores do índice de inteligibilidade de fala - SII gerados na verificação dos aparelhos de amplificação sonora programados conforme regra prescritiva DSLm[i/o]v5 e uma proposta de classificação de indivíduos que considere a associação de grau e configuração de perdas auditivas. Método foram selecionadas 41 crianças com idades entre 4 e 80 meses, totalizando 78 orelhas para análise. Foram considerados os limiares auditivos nas frequências 250, 500, 1000, 2000 e 4000 Hz e analisados valores de SII para os sinais de entrada 65 dB NPS, obtidos na verificação dos AASI no equipamento Verifit®Audioscan. Resultados as perdas auditivas foram classificadas em cinco grupos homogêneos quanto às características audiológicas (grau e configuração audiométrica) e heterogêneos entre si. A partir dos grupos, determinaram-se três intervalos de valores de SII. Foram determinadas equações para classificação da perda auditiva conforme grupos e equações para determinação de valores de SII ajustado. Conclusão o valor de SII pode ser considerado um indicador da audibilidade para sons de fala para diferentes características de perdas auditivas e nortear avalições de comportamento auditivo. O SII tem relação mais forte com a associação das variáveis grau e configuração audiométrica, quando comparado com sua relação com o grau da perda auditiva isoladamente.


ABSTRACT Purpose To establish the relationship between speech intelligibility index (SII) values generated at the verification of hearing aids programmed according to DSLm [i/o] v5 prescription rule and a proposed individual classification that considers the combination of hearing loss degree and configuration. Methods Forty-one children aged between 4 and 80 months were selected, totaling 78 ears for analysis. We considered hearing thresholds at the frequencies of 250, 500, 1000, 2000 and 4000 Hz; and analyzed values of the Speech Intelligibility Index (SII) for the input signal of 65 dB SPL obtained during the verification of hearing aids using the equipment Verifit®Audioscan. Results Hearing losses were classified into five homogeneous groups regarding audiometric degree and configuration. The groups were heterogeneous when compared to each other. From the groups, three ranges of SII values were determined. Equations were developed for classification of hearing loss according to groups and for determination of the adjusted SII values. Conclusion The SII value is a useful indicator of audibility for speech sounds in different characteristics of hearing losses, and can guide observations of auditory skills. The SII has stronger relationship with the association of the audiometric degree and configuration when compared with degree of hearing loss alone.


Subject(s)
Humans , Infant , Child, Preschool , Child , Auditory Threshold/physiology , Speech Intelligibility/physiology , Hearing Aids , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Audiometry , Speech Perception , Severity of Illness Index , Hearing Loss, Sensorineural/rehabilitation
9.
Int J Audiol ; 55(4): 224-31, 2016.
Article in English | MEDLINE | ID: mdl-26750766

ABSTRACT

OBJECTIVE: Determine the sensitivity, specificity, and accuracy of pediatric hearing loss classification using behavioral evidence of early prelingual auditory development (EPLAD). Validate behavioral measures of EPLAD. DESIGN: EPLAD was assessed in a prospective sample of hearing-impaired children using the infant-toddler meaningful auditory integration scale (ITMAIS/MAIS). Hearing losses were classified using tone-burst auditory brainstem response (ABR) and ITMAIS/MAIS scores. This process was repeated in a second retrospective sample. STUDY SAMPLE: The prospective sample was comprised of 139 hearing-impaired children under five years of age. Approximately equal proportions of mild-moderate, severe, and profound losses were included. The second retrospective sample was comprised of case records for 144 hearing-impaired children meeting the same selection criteria. This sample contained more than 80% profound losses. RESULTS: EPLAD trajectories reached different asymptotes after two years of age, depending on the severity of hearing loss, allowing children over this age to be classified. The sensitivity of EPLAD classifications was over 90%; specificity was over 82%; and accuracy was over 88%. CONCLUSIONS: Behavioral evidence of EPLAD provides an initial means of classifying pediatric hearing losses which can facilitate initial treatment options prior to diagnostic evaluation with tone-burst ABR.


Subject(s)
Auditory Perception , Child Behavior , Child Development , Hearing Loss, Sensorineural/diagnosis , Acoustic Stimulation , Age Factors , Audiometry, Pure-Tone , Auditory Threshold , Child, Preschool , Early Diagnosis , Evoked Potentials, Auditory, Brain Stem , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Sensorineural/psychology , Humans , Infant , Infant Behavior , Predictive Value of Tests , Reproducibility of Results , Retrospective Studies , Severity of Illness Index
10.
HNO ; 64 Suppl 1: S1-6, 2016 Mar.
Article in English | MEDLINE | ID: mdl-26607156

ABSTRACT

OBJECTIVE: Selecting subjects for clinical trials on hearing loss therapies relies on the patient meeting the audiological inclusion criteria. In studies on the treatment of idiopathic sudden sensorineural hearing loss, the patient's acute audiogram is usually compared with a previous audiogram, the audiogram of the non-affected ear, or a normal audiogram according to an ISO standard. Generally, many more patients are screened than actually fulfill the particular inclusion criteria. The inclusion criteria often require a calculation of pure-tone averages, selection of the most affected frequencies, and calculation of hearing loss differences. MATERIALS AND METHODS: A software tool was developed to simplify and accelerate this inclusion procedure for investigators to estimate the possible recruitment rate during the planning phase of a clinical trial and during the actual study. This tool is Microsoft Excel-based and easy to modify to meet the particular inclusion criteria of a specific clinical trial. The tool was retrospectively evaluated on 100 patients with acute hearing loss comparing the times for classifying automatically and manually. The study sample comprised 100 patients with idiopathic sudden sensorineural hearing loss. RESULTS AND CONCLUSION: The age- and sex-related normative audiogram was calculated automatically by the tool and the hearing impairment was graded. The estimated recruitment rate of our sample was quickly calculated. Information about meeting the inclusion criteria was provided instantaneously. A significant reduction of 30 % in the time required for classifying (30 s per patient) was observed.


Subject(s)
Audiometry, Pure-Tone/standards , Clinical Trials as Topic/standards , Diagnosis, Computer-Assisted/methods , Eligibility Determination/methods , Hearing Loss, Sensorineural/diagnosis , Software/standards , Algorithms , Diagnosis, Computer-Assisted/standards , Eligibility Determination/classification , Eligibility Determination/standards , Female , Germany , Hearing Loss, Sensorineural/classification , Humans , Machine Learning , Male , Middle Aged , Patient Selection , Pattern Recognition, Automated , Practice Guidelines as Topic , Programming Languages , Reproducibility of Results , Sensitivity and Specificity , Translating
11.
Pediatr Dermatol ; 32(4): 437-46, 2015.
Article in English | MEDLINE | ID: mdl-25727235

ABSTRACT

Neutrophilic dermatoses (NDs) are inflammatory skin conditions that are not associated with infection. The classification and clinical approach to these conditions in children is poorly described. This review classifies these conditions into five nosological subtypes: Sweet's syndrome, pyoderma gangrenosum, aseptic pustules, neutrophilic urticarial dermatoses, and Marshall's syndrome. In addition, we review the various secondary diseases that need to be excluded in the clinical management of the NDs of childhood, with a focus on the autoinflammatory conditions that the reader may not be familiar with. We propose a practical clinical approach to these disorders.


Subject(s)
Neutrophil Infiltration , Skin Diseases/classification , Abscess/classification , Abscess/diagnosis , Abscess/drug therapy , Cataract/classification , Cataract/diagnosis , Cataract/drug therapy , Child , Collagen Type XI/classification , Collagen Type XI/deficiency , Craniofacial Abnormalities/classification , Craniofacial Abnormalities/diagnosis , Craniofacial Abnormalities/drug therapy , Diagnosis, Differential , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/drug therapy , Humans , Osteochondrodysplasias/classification , Osteochondrodysplasias/diagnosis , Osteochondrodysplasias/drug therapy , Pyoderma Gangrenosum/classification , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/drug therapy , Skin Diseases/diagnosis , Skin Diseases/drug therapy , Sweet Syndrome/classification , Sweet Syndrome/diagnosis , Sweet Syndrome/drug therapy , Urticaria/classification , Urticaria/diagnosis , Urticaria/drug therapy
12.
Clin Otolaryngol ; 40(4): 355-62, 2015 Aug.
Article in English | MEDLINE | ID: mdl-25639297

ABSTRACT

OBJECTIVE: Recent reports have identified hypercholesterolaemia as a significant risk factor for idiopathic sudden sensorineural hearing loss (ISSNHL). Therefore, we investigated whether lipid profiles and lipoprotein ratios are correlated with the prognosis of hearing recovery in ISSNHL patients. DESIGN: A retrospective cohort study. MAIN OUTCOME MEASURES: Patients with ISSNHL were classified into four groups (complete, partial, slight and no recovery) according to their degree of hearing recovery using Siegel's criteria and the Sudden Deafness Research Group (SDRG) criteria developed by the Japanese Ministry of Welfare. All patients' lipid profiles were analysed, including total cholesterol (TC), high-density lipoprotein (HDL) and low-density lipoprotein (LDL) cholesterol and triglycerides. We calculated the ratios of TC/HDL-C and LDL-C/HDL-C and used statistical methods to evaluate correlations between lipid profiles and lipoprotein ratios and ISSNHL prognosis. RESULTS: Hearing recovery was observed in 103 (62.0%) of 166 cases using Siegel's criteria and in 114 (68.7%) of 166 cases using SDRG's criteria. Among the three recovery groups (i.e. excluding the no recovery group), the ratio of LDL-C/HDL-C was found to be associated with recovery outcome by showing the ratio on an upward trend from complete recovery to slight recovery group, and the difference is statistically significant (P = 0.016 by Siegel's criteria, P = 0.041 by SDRG's criteria). Multiple linear regression analysis further revealed a significantly higher LDL-C/HDL-C ratio in slight hearing recovery group compared with complete recovery group (P = 0.007 by Siegel's criteria, P = 0.031 by SDRG's criteria). CONCLUSION: We suggested that lipoprotein ratio of LDL-C/HDL-C may be a prognostic factor for hearing recovery in ISSNHL patients. Further studies should be conducted to determine whether hearing outcomes in ISSNHL can be improved by changing patients' lipid profiles via antilipidemic treatment.


Subject(s)
Hearing Loss, Sensorineural/etiology , Hearing Loss, Sudden/etiology , Hyperlipidemias/complications , Female , Hearing Loss, Sensorineural/classification , Hearing Loss, Sudden/classification , Humans , Lipoproteins, HDL/blood , Lipoproteins, LDL/blood , Male , Middle Aged , Prognosis , Retrospective Studies , Risk Factors , Treatment Outcome
13.
Blood ; 124(6): e4-e10, 2014 Aug 07.
Article in English | MEDLINE | ID: mdl-24990887

ABSTRACT

Abnormalities of platelet size are one of the distinguishing features of inherited thrombocytopenias (ITs), and evaluation of blood films is recommended as an essential step for differential diagnosis of these disorders. Nevertheless, what we presently know about this subject is derived mainly from anecdotal evidence. To improve knowledge in this field, we evaluated platelet size on blood films obtained from 376 patients with all 19 forms of IT identified so far and found that these conditions differ not only in mean platelet diameter, but also in platelet diameter distribution width and the percentage of platelets with increased or reduced diameters. On the basis of these findings, we propose a new classification of ITs according to platelet size. It distinguishes forms with giant platelets, with large platelets, with normal or slightly increased platelet size, and with normal or slightly decreased platelet size. We also measured platelet diameters in 87 patients with immune thrombocytopenia and identified cutoff values for mean platelet diameter and the percentage of platelets with increased or reduced size that have good diagnostic accuracy in differentiating ITs with giant platelets and with normal or slightly decreased platelet size from immune thrombocytopenia and all other forms of IT.


Subject(s)
Blood Platelets/pathology , Thrombocytopenia/blood , Thrombocytopenia/genetics , Adolescent , Adult , Case-Control Studies , Cell Size , Child , Child, Preschool , Diagnosis, Differential , Female , Hearing Loss, Sensorineural/blood , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/genetics , Humans , Infant , Male , Middle Aged , Molecular Motor Proteins/genetics , Mutation , Myosin Heavy Chains/genetics , Purpura, Thrombocytopenic, Idiopathic/blood , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Thrombocytopenia/classification , Thrombocytopenia/congenital , Young Adult
14.
Vestn Otorinolaringol ; (2): 37-43, 2014.
Article in Russian | MEDLINE | ID: mdl-24781170

ABSTRACT

The objective of the present work was to study specific features of the audiological phenotype and the prevalence of GJB2-related sensorineural hearing loss (SNHL) in the infants suffering acoustic disturbances. The study included 264 children with bilateral non-syndromic sensorineural loss of hearing diagnosed during the first year of life by means of detailed audiological examination that included tympanometry, registration of short-latency auditory action potentials (SLAAP), delayed evoked otoacoustic emission (DEOAE), distortion product-frequency otoacoustic emission (DPFOAE), and auditory brain-stem response (ABR). In addition, stationary acoustically evoked responses (SAER) were recorded in 38 children presenting with hearing impairment associated with GJB2-related sensorineural loss of hearing. The follow-up dynamic study involved 113 children subjected to repeated audiological examination. The study revealed the genotype with pathological mutations in 182 (69.0%) children including 171 (64.8%) ones with biallelic mutations and 11 (4.2%) with a single mutation (heterozygous genotype). Eighty two (31.0%) children had genotype without mutations. A total of 21 different mutations and 30 different genotypes were identified. Analysis of the family histories of the children showed that neither the absence of relatives suffering from hearing impairment nor the presence of risk factors of acquired hearing impairment excludes the possibility of GJB2-related sensorineural loss of hearing in the infants. Otoacoustic emission fails to be registered in the majority of the children with the altered genotype (87%) during the stay in the maternity house. Mutations in the GJB2 gene are most frequently diagnosed in the patients with the moderate, moderately severe, and severe loss of hearing. At the same time, almost half of the infants presenting with the mild loss of hearing were found to exhibit changes in the GJB2 gene. The thresholds of registration of short-latency auditory action potentials remain stable in 90.0% of the children presenting with GJB2-related sensorineural loss of hearing which makes it possible to choose the strategy of their rehabilitative treatment (the use of hearing aids or cochlear implantation) during the very first months of life and predict the favourable outcome of cochlear implantation and hearing aid measures. The results of the present work illustrate the importance and practical significance of genetic studies (GJB2 gene tresting) of the infants suffering sensorineural loss of hearing and other acoustic disturbances for the elucidation of etiology of these conditions, prognosis of the disease, and the choice of the strategy for its treatment.


Subject(s)
Connexins/genetics , Hearing Loss, Sensorineural/genetics , Child, Preschool , Connexin 26 , Female , Genotype , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/epidemiology , Hearing Tests , Humans , Infant , Male , Mutation/genetics , Phenotype , Prevalence , Severity of Illness Index
16.
HNO ; 61(1): 14-24, 2013 Jan.
Article in German | MEDLINE | ID: mdl-23223925

ABSTRACT

BACKGROUND: Previous studies comparing the Freiburg speech tests (FST) currently used in Germany to assess the severity of hearing loss with two modern speech intelligibility tests [the Göttingen sentence test (GöSa) and the monosyllabic rhyme test devised by von Wallenberg and Kollmeier (WaKo)] have indicated that a replacement of the old procedure would be possible. The current study investigates the consequences of the modern test procedures for the estimation of reduction in earning capacity resulting from hearing loss, and considers the optimal presentation levels for the WaKo test. METHODS: The FST, GöSa, and WaKo speech intelligibility tests were performed on 29 volunteers with a hearing impairment. All tests were conducted in silence. The presentation levels for the Freiburg monosyllabic test were 60, 80, and for some participants also 100 dB SPL. The WaKo test was carried out once at 45, 65, and 85 dB SPL (a reduction of 15 dB relative to the FST) and again at 40, 60 and 80 dB SPL (reduction of 20 dB relative to the FST). RESULTS: A consideration across all presentation levels revealed that the best correlation match between the two monosyllabic tests was achieved at a 20-dB reduction in presentation level for the WaKo test relative to the FST. On average, the application of modern procedures and the different options for level reduction had only a minor effect on the quantitative assessment of reduction in earning capacity.


Subject(s)
Diagnosis, Computer-Assisted/methods , Diagnosis, Computer-Assisted/statistics & numerical data , Disability Evaluation , Expert Testimony/legislation & jurisprudence , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/diagnosis , Speech Discrimination Tests/methods , Speech Discrimination Tests/statistics & numerical data , Adult , Aged , Audiometry, Pure-Tone/statistics & numerical data , Auditory Threshold , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Statistics as Topic
17.
Med Tr Prom Ekol ; (3): 25-30, 2012.
Article in Russian | MEDLINE | ID: mdl-22702132

ABSTRACT

The article covers data on wide prevalence of occupational neuro-sensory deafness in Russian Federation where nowadays three major deafness classifications are applied, international, for noise-related occupations and for medical and social examination. The authors analyzed present classifications of deafness, emphasized positive and negative aspects of each classification from occupational medicine viewpoint. Several classifications applied at the same time complicate professional activity of specialists solving diagnostic, occupational and social problems of patients with occupational deafness and result in conflicts. Advantage of the classification for noise-related occupations is that it enables to evaluate premorbid deafness, since early signs of ear alteration due to noise, and even slight negative changes in hearing could be addressed by specialists (as stages and degrees cover 10 dB differences), however review of audiometric assessment is necessary for noise-related occupations. To increase efficiency of ENT-specialists work in diagnosis and prevention of occupational neuro-sensory deafness, legal and methodic basis should be developed.


Subject(s)
Hearing Loss, Sensorineural/classification , Noise, Occupational/adverse effects , Occupational Diseases/classification , Audiometry , Hearing Loss, Sensorineural/etiology , Humans , Occupational Diseases/etiology , Russia , Severity of Illness Index
18.
Laryngorhinootologie ; 91(12): 782-8, 2012 Dec.
Article in German | MEDLINE | ID: mdl-22234848

ABSTRACT

BACKGROUND: In addition to the standardized Freiburg sentence test in silence validated speech tests in noise are available. They are mainly used to test the benefit of hearing systems in everyday-life situations. In diagnostics tests in noise can also give additional information of the patients' hearing impairment. The determined speech reception threshold (SRT) depends on the degree of hearing loss and the used speech and noise test material. Aim of this study was to analyze the results of the Göttingen sentence test in noise in relation to different audiogram classes. MATERIAL AND METHODS: The Göttingen sentence test in stationary noise (65 dB) was performed for 135 patients with different degrees of hearing losses. Based on the air-conducted threshold all ears were categorized to an audiogram class by an automated procedure. RESULTS: For the mild hearing losses the results of the Göttingen sentence test increased with increasing pure-tone-average (PTA) and the values are smaller than 0 dB SNR with a small standard deviation (± 1-2 dB SNR). For the moderate hearing losses values of 5 dB SNR with a standard deviation of 6 dB SNR were achieved on average. Patients with a high hearing loss achieved values higher than 20 dB SNR. CONCLUSION: For the mild hearing losses the results were located in a small range. However, they can give important information about the patient's hearing impairment mainly appears in a noisy environment. With increasing hearing loss also the reduced hearing in silence affects the results achieved with the Göttingen sentence test in noise. Therefore the hearing in silence has to be taken into account at the interpretation of the results.


Subject(s)
Audiometry, Pure-Tone/methods , Audiometry, Speech/methods , Hearing Loss, High-Frequency/diagnosis , Hearing Loss, Noise-Induced/diagnosis , Hearing Loss, Sensorineural/diagnosis , Speech Reception Threshold Test , Adult , Aged , Auditory Threshold , Expert Testimony , Female , Germany , Hearing Aids , Hearing Loss, High-Frequency/classification , Hearing Loss, Noise-Induced/classification , Hearing Loss, Sensorineural/classification , Humans , Male , Middle Aged , Occupational Diseases/classification , Occupational Diseases/diagnosis , Reference Values
19.
HNO ; 59(9): 908-14, 2011 Sep.
Article in German | MEDLINE | ID: mdl-21732149

ABSTRACT

BACKGROUND: The present study analyzes the best combination of frequencies for the calculation of mean hearing loss in pure tone threshold audiometry for correlation with hearing loss for numbers in speech audiometry, since the literature describes different calculation variations for plausibility checking in expertise. Three calculation variations, A (250, 500 and 1000 Hz), B (500 and 1000 Hz) and C (500, 1000 and 2000 Hz), were compared. METHODS: Audiograms in 80 patients with normal hearing, 106 patients with hearing loss and 135 expertise patients were analyzed in a retrospective manner. Differences between mean pure tone audiometry thresholds and hearing loss for numbers were calculated and statistically compared separately for the right and the left ear in the three patient collectives. RESULTS: We found the calculation variation A to be the best combination of frequencies, since it yielded the smallest standard deviations while being statistically different to calculation variations B and C. The 1- and 2.58-fold standard deviation (representing 68.3% and 99.0% of all values) was ±4.6 and ±11.8 dB for calculation variation A in patients with hearing loss, respectively. CONCLUSIONS: For plausibility checking in expertise, the mean threshold from the frequencies 250, 500 and 1000 Hz should be compared to the hearing loss for numbers. The common recommendation reported by the literature to doubt plausibility when the difference of these values exceeds ±5 dB is too strict as shown by this study.


Subject(s)
Audiometry, Pure-Tone/methods , Audiometry, Pure-Tone/statistics & numerical data , Audiometry, Speech/methods , Audiometry, Speech/statistics & numerical data , Auditory Threshold , Expert Testimony/legislation & jurisprudence , Hearing Loss, High-Frequency/diagnosis , Hearing Loss, Sensorineural/diagnosis , Adolescent , Adult , Aged , Audiometry, Pure-Tone/instrumentation , Audiometry, Speech/instrumentation , Child , Female , Hearing Loss, Sensorineural/classification , Humans , Male , Middle Aged , Reference Values , Reproducibility of Results , Retrospective Studies , Statistics as Topic , Young Adult
20.
Folia Phoniatr Logop ; 63(1): 43-8, 2011.
Article in English | MEDLINE | ID: mdl-20699618

ABSTRACT

OBJECTIVE: To develop a clear and meaningful set of definitions of types of hearing impairment. METHOD: A critical analysis was made of previous definitions together with a consideration of current knowledge of functions and dysfunctions of the auditory system. RESULTS AND CONCLUSIONS: A coherent set of definitions compatible with the contemporary understanding of auditory disorders and their perceptual effects is proposed.


Subject(s)
Hearing Loss/classification , Audiometry , Auditory Pathways/physiopathology , Cochlea/physiopathology , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Hearing Loss, Conductive/classification , Hearing Loss, Conductive/physiopathology , Hearing Loss, Mixed Conductive-Sensorineural/classification , Hearing Loss, Mixed Conductive-Sensorineural/physiopathology , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Humans
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