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1.
J Laryngol Otol ; 134(5): 419-423, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32425141

RESUMO

OBJECTIVE: To assess the Framingham risk score as a prognostic tool for idiopathic sudden sensorineural hearing loss patients. METHODS: Medical records were reviewed for unilateral idiopathic sudden sensorineural hearing loss patients between January 2010 and October 2017. The 10-year risk of developing cardiovascular disease was calculated. Patients were subdivided into groups: group 1 - Framingham risk score of less than 10 per cent (n = 28); group 2 - score of 10 to less than 20 per cent (n = 6); and group 3 - score of 20 per cent or higher (n = 5). RESULTS: Initial pure tone average and Framingham risk score were not significantly associated (p = 0.32). Thirteen patients in group 1 recovered completely (46.4 per cent), but none in groups 2 and 3 showed complete recovery. Initial pure tone average and Framingham risk score were significantly associated in multivariable linear regression analysis (R2 = 0.36). The regression coefficient was 0.33 (p = 0.003) for initial pure tone average and -0.67 (p = 0.005) for Framingham risk score. CONCLUSION: Framingham risk score may be useful in predicting outcomes for idiopathic sudden sensorineural hearing loss patients, as those with a higher score showed poorer hearing recovery.


Assuntos
Perda Auditiva Neurossensorial/diagnóstico , Doença Aguda , Audiometria de Tons Puros , Doenças Cardiovasculares/diagnóstico , Feminino , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recuperação de Função Fisiológica/fisiologia , Medição de Risco/métodos
2.
Ann Otol Rhinol Laryngol ; 129(8): 806-812, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32249593

RESUMO

OBJECTIVE: The aim of this study was to investigate the serum renin levels of patients with idiopathic sudden sensorineural hearing loss (ISSNHL). MATERIAL AND METHODS: Twenty-four patients with ISSNHL and 24 asymptomatic healthy volunteers were included in the study. Subjects underwent pure-tone audiometry and serum renin levels were measured. RESULTS: There were 14 women (mean age:42.35 ± 9.53) and 10 men (mean age:43.8 ± 6.87) in the patient group. There were 14 women (mean age:42.4 ± 4.7) and 10 men (mean age:41.4 ± 4.59) in the control group. ISSNHL was detected on the right side in 13 patients and on the left side in 11 patients. Serum renin levels of the patients and controls were 788.01 ± 327.8 and 282.37 ± 107.73 pg/mL, respectively. The serum renin levels were found to be significantly higher in the patient group compared to the control group (P ≤ .001). There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss (r = 0.77; P = .001). CONCLUSION: Serum renin levels of patients with ISSNHL were higher than controls. There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss.


Assuntos
Perda Auditiva Neurossensorial/sangue , Perda Auditiva Súbita/sangue , Audição/fisiologia , Renina/sangue , Adulto , Audiometria de Tons Puros/métodos , Biomarcadores/sangue , Ensaio de Imunoadsorção Enzimática , Feminino , Seguimentos , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/fisiopatologia , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
3.
J Comput Assist Tomogr ; 44(3): 386-388, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32168082

RESUMO

PURPOSE: Incomplete partition III (IP-III) characterized by congenital mixed or sensorineural hearing loss is a rare genetic disease transmitted through X-linked inheritance. Incomplete partition III can be easily achieved based on pathognomonic computed tomography findings. The aims of this study were to investigate the otic capsule abnormalities in IP-III and to report irregular contour of membranous labyrinth and hypomineralized areas at otic capsule, which have not previously been described. MATERIALS AND METHODS: The otic capsule features of 10 subjects (8 affected patients, 1 of whom is a female; 2 carrier mothers), who were diagnosed on clinical and typical radiologic findings, were analyzed. RESULTS: All patients had typical IP-III as described in the literature. Seven of 10 patients had irregular contour. Seven of 10 patients demonstrated hypomineralized areas, which were very hypodense to normally develop otic capsule areas. One affected patients and 2 carrier mothers had a normal-looking membranous labyrinth contour and normal mineralization at otic capsule. CONCLUSIONS: We report for the first time the irregular contour of inner ear structures and hypodense otic capsule areas in patients with IP-III. We think that though speculative, abnormal development of the inner endosteal layer results in irregular contour of inner ear structures. Hypomineralized areas at otic capsule could be explained by abnormal development of middle enchondral layer due to reduced or absent vascular supply from middle ear mucosa during fetal life. These findings may be accepted as additional criteria of IP-III.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Idoso , Desmineralização Patológica Óssea/diagnóstico por imagem , Desmineralização Patológica Óssea/patologia , Criança , Pré-Escolar , Orelha Interna/anormalidades , Orelha Interna/diagnóstico por imagem , Feminino , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Masculino , Adulto Jovem
4.
Int. arch. otorhinolaryngol. (Impr.) ; 24(1): 5-10, Jan.-Mar. 2020. tab
Artigo em Inglês | LILACS | ID: biblio-1090555

RESUMO

Abstract Introduction The cochlea and the vestibular receptors are closely related in terms of anatomy and phylogeny. Patients with moderate to profound sensorineural hearing loss (MPSHL) should have their vestibular organ functions tested. Objective To evaluate the incidence of vestibular abnormalities in patients with MPSHL and to study the correlation between the etiology of hearing loss (HL) and a possible damage to the labyrinth. Methods A case-control retrospective study was performed. In the case group, 20 adults with MPSHL of known etiology were included. The control group was composed of 15 adults with normal hearing. The case group was divided into 4 subgroups based on the etiology (bacterial meningitis, virus, vascular disease, congenital). Cervical vestibular-evoked myogenic potentials (cVEMPs) were used to rate the saccular function and lower vestibular nerve. Results The study was performed in 70 ears, and it highlighted the presence of early biphasic P1-N1 complex in 29 (71.5%) out of 40 ears in the study group, and in all of the 30 ears in the control group (p = 0.001). Regarding the presence or absence of cVEMPs among the four subgroups of patients with MPSHL, the data were statistically significant (p < 0.001). The comparison between the latencies and amplitude of P1-N1 in case and control groups from other studies and in the four subgroups of cases in the present study did not detect statistically significant differences. Conclusion The present study demonstrates that patients with MPSHL have a high incidence of damage to the labyrinthine organs, and it increases the current knowledge about the etiopathogenesis of sensorineural HL, which is often of unknown nature.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Potenciais Evocados Miogênicos Vestibulares , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia , Audiometria de Tons Puros , Doenças Vasculares/complicações , Viroses/complicações , Estudos de Casos e Controles , Doenças Vestibulares/diagnóstico , Doenças Vestibulares/epidemiologia , Incidência , Estudos Retrospectivos , Meningites Bacterianas/complicações , Perda Auditiva Neurossensorial/congênito , Doenças do Labirinto/diagnóstico , Doenças do Labirinto/fisiopatologia , Doenças do Labirinto/epidemiologia
5.
BMC Med Genet ; 21(1): 1, 2020 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-31898538

RESUMO

BACKGROUND: Hearing loss (HL) represents the most common congenital sensory impairment with an incidence of 1-5 per 1000 live births. Non-syndromic hearing loss (NSHL) is an isolated finding that is not part of any other disorder accounting for 70% of all genetic hearing loss cases. METHODS: In the current study, we reported a polygenic mode of inheritance in an NSHL consanguineous family using exome sequencing technology and we evaluated the possible effect of the detected single nucleotide variants (SNVs) using in silico methods. RESULTS: Two bi-allelic SNVs were detected in the affected patients; a MYO15A (. p.V485A) variant, and a novel MITF (p.P338L) variant. Along with these homozygous mutations, we detected two heterozygous variants in well described hearing loss genes (MYO7A and MYH14). The novel MITF p. Pro338Leu missense mutation was predicted to change the protein structure and function. CONCLUSION: A novel MITF mutation along with a previously described MYO15A mutation segregate with an autosomal recessive non-syndromic HL case with a post-lingual onset. The findings highlight the importance of carrying whole exome sequencing for a comprehensive assessment of HL genetic heterogeneity.


Assuntos
Heterogeneidade Genética , Perda Auditiva Neurossensorial/genética , Fator de Transcrição Associado à Microftalmia/genética , Miosinas/genética , Idade de Início , Alelos , Criança , Feminino , Predisposição Genética para Doença , Perda Auditiva Neurossensorial/fisiopatologia , Heterozigoto , Homozigoto , Humanos , Masculino , Herança Multifatorial/genética , Linhagem , Fenótipo , Polimorfismo de Nucleotídeo Único/genética , Sequenciamento Completo do Exoma
6.
Otolaryngol Head Neck Surg ; 162(1): 114-120, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31593522

RESUMO

OBJECTIVE: To describe the progression of sensorineural hearing loss (SNHL) in the better- and poorer-hearing ears in children with asymptomatic congenital cytomegalovirus (CMV) infection with isolated SNHL. STUDY DESIGN: Longitudinal prospective cohort study. SETTING: Tertiary medical center. SUBJECTS AND METHODS: We analyzed hearing thresholds of the better- and poorer-hearing ears of 16 CMV-infected patients with isolated congenital/early-onset or delayed-onset SNHL identified through hospital-based CMV screening of >30,000 newborns from 1982 to 1992. RESULTS: By 12 months of age, 4 of 7 patients with congenital/early-onset SNHL developed worsening thresholds in the poorer-hearing ear, and 1 had an improvement in the better-hearing ear. By 18 years of age, all 7 patients had worsening thresholds in the poorer-hearing ear and 3 patients had worsening thresholds in the better-hearing ear. Hearing loss first worsened at a mean age of 2 and 6 years in the poorer- and better-hearing ears, respectively. Nine patients were diagnosed with delayed-onset SNHL (mean age of 9 years vs 12 years for the poorer- and better-hearing ears), 6 of whom had worsening thresholds in the poorer-hearing ear and 1 in both ears. CONCLUSION: In most children with congenital CMV infection and isolated SNHL, the poorer-hearing ear worsened earlier and more precipitously than the better-hearing ear. This study suggests that monitoring individual hearing thresholds in both ears is important for appropriate interventions and future evaluation of efficacy of antiviral treatment.


Assuntos
Doenças Assintomáticas , Infecções por Citomegalovirus/congênito , Progressão da Doença , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Fatores Etários , Idade de Início , Limiar Auditivo , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Perda Auditiva Neurossensorial/fisiopatologia , Testes Auditivos , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Monitorização Fisiológica/métodos , Triagem Neonatal/métodos , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Estados Unidos
7.
Probl Radiac Med Radiobiol ; 24: 522-536, 2019 Dec.
Artigo em Inglês, Ucraniano | MEDLINE | ID: mdl-31841493

RESUMO

OBJECTIVE: to study the hirudotherapy efficacy in presbyacuzis praecox in clean-up workers (CUWs) of the Chornobyl disaster (ChD) during the post-accident years. MATERIALS AND METHODS: From archive data of previously examined 8,136 males' CUWs we selected among them 129 persons with the determined presbyacuzis praecox during the post-accident years. According to the physical dosime- try data the individual radiation of received by CUWs during the work on a rotational scheme in the Chornobyl exclu- sion zone from the end of 1986 to 1992-1994 amounted to 0.21-0.50 Gy. The examinations were carried out using a modern standard set of audiometric, vestibulometric and electrophysiological methods. Two forms of sensory and neural hearing loss in the elderly were distinguished, namely the presbyacuzis and presbyacuzis praecox. Prior to work in the exclusion zone, patients' auditory and vestibular functions were within normal range. Among 129 patients, 68 ones with presbyacuzis praecox were included in two main age groups (aged 40-49 and 50-59 yrs) and were treated using hirudotherapy, taking into consideration their coagulation hemostasis. Other 61 patients of analogical age groups were treated by allopathotherapy. For the analysis of results obtained, techniques of variational statistics were used. RESULTS: Direct correlation (r = 0.71) between inhibitory processes in central areas of the auditory analyzer in pres- byacuzis praecox in CUWs was established by electrophysiology and by speech audiometry data obtained before the treatment. Hyperacusis signs were detected in CUWs of two main and two control groups. Following the use of two treatment schemas, a significant improving of auditory functions was found (p < 0.05) according to tone and speech audiometries. The positive hirudotherapy effect concerning hearing functions was registered in 88% cases (in 59 CUWs among 68 ones); if allopathotherapy had been used, such effect was found in 65% cases (in 45 control patients among 61 ones of control group). The duration of allopathotherapy effect reached 6-9 months comparing to 12-18 months of hiruditherapy one, being twice longer. Improving the patients' coagulation hemostasis, hirudotherapy activated cardiovascular activity favoring the increase of social adequacy in CUWs with presbyacuzis praecox. CONCLUSIONS: It has been shown that hirudotherapy as a kind of naturopathy has significant advantages over alopa- totherapy by the absence of side effects, 23.0% higher and twice as long as improvement of auditory functions. Hirudotherapy, as an effective therapeutic and recreational measures, should be more widely implemented in clini- cal practice in order to minimize the development and progression of diseases in the special population of people who have been exposed to ionizing radiation due to the Chornobyl catastrophe to continue their vitality.


Assuntos
Acidente Nuclear de Chernobyl , Socorristas , Perda Auditiva Neurossensorial/terapia , Aplicação de Sanguessugas/métodos , Lesões por Radiação/terapia , Adulto , Audiometria da Fala , Coagulação Sanguínea/fisiologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Reconhecimento Fisiológico de Modelo/fisiologia , Equilíbrio Postural/fisiologia , Doses de Radiação , Exposição à Radiação/efeitos adversos , Lesões por Radiação/diagnóstico , Lesões por Radiação/etiologia , Lesões por Radiação/fisiopatologia , Sobreviventes , Resultado do Tratamento , Ucrânia
8.
J Int Adv Otol ; 15(3): 352-357, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31846911

RESUMO

OBJECTIVES: Jervell and Lange-Nielsen syndrome is a rare autosomal recessive disease characterized by congenital sensorineural deafness and significant QT interval prolongation. Aims were to study the prevalence of long QT in congenital hearing loss, complications encountered, outcomes by Categories of auditory Performance (CAP) scores and Speech Intelligibility Rating (SIR) scores and to create an algorithm with precautions to be followed in Long QT children. MATERIALS AND METHODS: Study was done at Auditory implant center at a tertiary referral care ENT hospital which includes 41 paediatric patients who were diagnosed to have Long QT during preoperative assessment and underwent cochlear implantation. A standard Protocol was followed in all candidates which includes comprehensive targeted history and investigations, preoperative and intraoperative precautions, and the findings were recorded. RESULTS: Preoperative prophylactic Beta blockers, avoiding sympathetic stimulation and drugs prolonging QT interval with rational use of Magnesium Sulphate and standby of defibrillator were the standard precautions practised. Fatal Arrhythmias were encountered intra-operatively in five patients which was treated with cardiac pacing. Cardiac monitoring was done intraoperatively and during switch-on. Significant improvement in CAP and SIR scores were observed at 3 and 6 months when compared to their base line values. CONCLUSION: With special attention to preoperative evaluation, appropriate intraoperative precautions and monitoring, judicious surgical planning and post surgical follow-up cochlear implantation may be performed safely in patients with JLNS with good postoperative results allowing for improved audition.


Assuntos
Implante Coclear/métodos , Surdez/cirurgia , Perda Auditiva Neurossensorial/cirurgia , Síndrome de Jervell-Lange Nielsen/cirurgia , Percepção Auditiva , Pré-Escolar , Surdez/congênito , Surdez/fisiopatologia , Feminino , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Síndrome de Jervell-Lange Nielsen/complicações , Síndrome de Jervell-Lange Nielsen/fisiopatologia , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Inteligibilidade da Fala , Resultado do Tratamento
9.
J Int Adv Otol ; 15(3): 454-458, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31846928

RESUMO

Muckle-Wells syndrome (MWS), a subclass of cryopyrin-associated periodic syndrome (CAPS), sometimes includes complications of bilateral progressive sensorineural hearing loss. A 48-year-old woman had been diagnosed with pediatric rheumatic arthritis at aged 6 years; however, systematic therapy with prednisolone and methotrexate showed limited efficacy for her general fatigue and arthritic pain, and it never improved the hearing level. She underwent a cochlear implant surgery for progressive profound bilateral hearing loss. After 7 years of cochlear implant surgery, she was diagnosed with MWS by genetic tests. Interleukin (IL)-1ß monoclonal antibody therapy (canakinumab) improved general fatigue and arthritic pain but showed no effect on cochlear symptoms. Owing to successful cochlear implant surgery, she reacquired the hearing and communication function while being able to understand over 90% of monosyllables and words in the sound field of her daily life at 65 dB SPL for the next 13 years of her life. This suggests that peripheral cochlear damage induced by chronic inflammation contributes to the sensorineural hearing loss in cases with MWS, and that cochlear implantation can provide long-term hearing efficacy for patients with MWS with irreversible profound hearing loss.


Assuntos
Implante Coclear , Síndromes Periódicas Associadas à Criopirina/complicações , Perda Auditiva Neurossensorial/cirurgia , Cóclea/fisiopatologia , Feminino , Audição , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Pessoa de Meia-Idade , Período Pós-Operatório , Fatores de Tempo , Resultado do Tratamento
10.
Int. arch. otorhinolaryngol. (Impr.) ; 23(4): 433-439, Out.-Dez. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1024413

RESUMO

Introduction: Studies have reported that although speech perception in noise was unaltered with and without digital noise reduction (DNR), the annoyance toward noise measured by acceptable noise level (ANL) was significantly improved by DNR with the range between 2.5 and 4.5 dB. It is unclear whether a similar improvement would be observed in those individuals who have an ANL ≥ 14 dB (predictive of poor hearing aid user) often rejects their aid because of annoyance toward noise. Objectives: (a) To determine the effect of activation of DNR on the improvement in the aided ANL from low- and high-ANL groups; and (b) to predict the change in ANL when DNR was activated. Method: Ten bilateral mild to severe sloping sensorineural hearing loss (SNHL) participants in each of the low- and high-ANL groups were involved. These participants were bilaterally fitted with receiver in canal (RIC) hearing aids (Oticon, Smorum, Egedal, Denmark) with a DNR processor. Both SNR-50% (Signal to noise ratio (in dB) required to achieve 50 % speech recognition) and ANL were assessed in DNR-on and DNR-off listening conditions. Results: Digital noise reduction has no effect on SNR-50 in each group. The annoyance level was significantly reduced in the DNR-on than DNR-off condition in the low-ANL group. In the high-ANL group, a strong negative correlation was observed between the ANL in DNR off and a change in ANL after DNR was employed in the hearing aid (benefit). The benefit of DNR on annoyance can be effectively predicted by baseline-aided ANL by linear regression. Conclusion: Digital noise reduction reduced the annoyance level in the high-ANL group, and the amount of improvement was related to the baseline-aided ANL value (AU)


Assuntos
Pessoa de Meia-Idade , Idoso , Limiar Auditivo/fisiologia , Percepção da Fala/fisiologia , Efeitos do Ruído , Auxiliares de Audição , Método Simples-Cego , Perda Auditiva Neurossensorial/fisiopatologia
11.
Audiol Neurootol ; 24(6): 299-308, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31846976

RESUMO

INTRODUCTION: Precurved cochlear implant (CI) electrode arrays were developed in an attempt to improve the auditory outcome of cochlear implantation, which varies greatly. The recent CI532 (Cochlear Corp., Sydney, Australia) may offer further advantages as its electrode array is thinner than previous precurved CI electrode arrays. The aims here were to investigate 1-year postoperative speech recognition, intraoperative electrically evoked compound action potentials (ECAPs), and their possible relation in patients implanted with a CI532 or its predecessor CI512. METHODS: A retrospective analysis of data from 63 patients subjected to cochlear implantation at the Karolinska University Hospital, Sweden, was performed. Speech recognition of the implanted ear was evaluated using phonemically balanced monosyllabic Swedish words at 65 dB SPL. ECAPs were evaluated using the intraoperative ECAP threshold across ≥8 electrodes generated by the automated neural response telemetry of the CI. RESULTS: The median aided speech recognition score (SRS) 1 year after implantation was 52% (quartile 1 = 40%, quartile 3 = 60%, n = 63) and did not differ statistically significantly between patients with CI512 (n = 38) and CI532 (n = 25). The mean ECAP threshold was 188 CL (current level; SD = 15 CL, n = 54) intraoperatively and did not differ statistically significantly between patients with CI512 (n = 32) and CI532 (n = 22), but the threshold for each electrode varied more between patients with a CI512 (p < 0.0001). A higher mean ECAP threshold was associated with a worse SRS (Spearman's ρ = -0.46, p = 0.0004, n = 54). The association remained among those with a CI512 (Spearman's ρ = -0.62, p = 0.0001, n = 32) when stratified by CI electrode array. CONCLUSION: No statistically significant difference in speech recognition 1 year after cochlear implantation or in mean threshold of ECAP intraoperatively was found between patients with a CI512 and the more recent, slim CI532, but the ECAP thresholds varied more between those with a CI512. A statistically significant association between SRS and mean ECAP threshold was found, but stratified analysis suggests that the association may be true only for patients with a CI512.


Assuntos
Potenciais de Ação/fisiologia , Implante Coclear , Implantes Cocleares , Potenciais Evocados Auditivos/fisiologia , Perda Auditiva Neurossensorial/reabilitação , Percepção da Fala , Adulto , Idoso , Idoso de 80 Anos ou mais , Limiar Auditivo/fisiologia , Eletrodos Implantados , Feminino , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Período Intraoperatório , Masculino , Pessoa de Meia-Idade , Período Pós-Operatório , Estudos Retrospectivos , Índice de Gravidade de Doença , Telemetria
12.
Am J Audiol ; 28(4): 843-856, 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31647880

RESUMO

Purpose The aim of this study was to quantify the portion of variance in several measures suggested to be indicative of peripheral noise-induced cochlear synaptopathy and hidden hearing disorder that can be attributed to individual cognitive capacity. Method Regression and relative importance analysis was used to model several behavioral and physiological measures of hearing in 32 adults ranging in age from 20 to 74 years. Predictors for the model were hearing sensitivity and performance on a number of cognitive tasks. Results There was a significant influence of cognitive capacity on several measures of cochlear synaptopathy and hidden hearing disorder. These measures include frequency modulation detection threshold, time-compressed word recognition in quiet and reverberation, and the strength of the frequency-following response of the speech-evoked auditory brainstem response. Conclusions Measures of hearing that involve temporal processing are significantly influenced by cognitive abilities, specifically, short-term and working memory capacity, executive function, and attention. Research using measures of temporal processing to diagnose peripheral disorders, such as noise-induced synaptopathy, need to consider cognitive influence even in a young, healthy population.


Assuntos
Cóclea/lesões , Cognição/fisiologia , Perda Auditiva Provocada por Ruído/fisiopatologia , Perda Auditiva/fisiopatologia , Testes de Impedância Acústica , Adulto , Idoso , Audiometria , Limiar Auditivo , Cóclea/fisiopatologia , Feminino , Perda Auditiva/psicologia , Perda Auditiva Provocada por Ruído/psicologia , Perda Auditiva Neurossensorial/fisiopatologia , Perda Auditiva Neurossensorial/psicologia , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Percepção da Fala , Adulto Jovem
13.
Exp Brain Res ; 237(11): 2983-2993, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31515588

RESUMO

Acoustic trauma, aging, genetic defects or ototoxic drugs are causes for sensorineural hearing loss involving sensory hair cell death and secondary degeneration of spiral ganglion neurons. Auditory implants are the only available therapy for severe to profound sensorineural hearing loss when hearing aids do not provide a sufficient speech discrimination anymore. Neurotrophic factors represent potential therapeutic candidates to improve the performance of cochlear implants (CIs) by the support of spiral ganglion neurons (SGNs). Here, we investigated the effect of pleiotrophin (PTN), a well-described neurotrophic factor for different types of neurons that is expressed in the postnatal mouse cochlea. PTN knockout mice exhibit severe deficits in auditory brainstem responses, which indicates the importance of PTN in inner ear development and function and makes it a promising candidate to support SGNs. Using organotypic explants and dissociated SGN cultures, we investigated the influence of PTN on the number of neurons, neurite number and neurite length. PTN significantly increased the number and neurite length of dissociated SGNs. We further verified the expression of important PTN-associated receptors in the SG. mRNA of anaplastic lymphoma kinase, αv integrin, ß3 integrin, receptor protein tyrosine phosphatase ß/ζ, neuroglycan C, low-density lipoprotein receptor-related protein 1 and syndecan 3 was detected in the inner ear. These results suggest that PTN may be a novel candidate to improve sensorineural hearing loss treatment in the future.


Assuntos
Proteínas de Transporte/fisiologia , Citocinas/fisiologia , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Neurônios/fisiologia , Gânglio Espiral da Cóclea/fisiologia , Animais , Citocinas/deficiência , Feminino , Células HEK293 , Perda Auditiva Neurossensorial/patologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Masculino , Camundongos , Camundongos Knockout , Neuritos/fisiologia
14.
Audiol Neurootol ; 24(5): 231-236, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31553979

RESUMO

BACKGROUND: Wide-band tympanometry (WBT) was introduced as a beneficial diagnostic test for Ménière's disease (MD) almost 15 years ago. However, an acute episode of MD has not been evaluated by using WBT yet. OBJECTIVE: To investigate WBT findings in patients with MD during acute attacks. METHOD: Thirty definite MD patients with unilateral acute low-tone sensorineural hearing loss and aural fullness, and thirty age- and sex-matched control subjects were enrolled prospectively in a tertiary referral center. Ears were divided into three groups as follows: (1) affected ears of MD patients, (2) contralateral ears of MD patients, (3) control ears. Individuals underwent WBT. The resonance frequency (RF), mean absorbance value, mean low- and high-frequency absorbance values (LF-A and HF-A), and double peak width at 2 kHz of conductance tympanometry (2-kHz PW) were assessed. RESULTS: Seventy percent in group 1, 66.7% in group 2, and 78.3% in group 3 demonstrated double peaks at 2 kHz. The mean 2-kHz PW values were 157.52 ± 79.19, 177.40 ± 79.14, and 139.64 ± 87.501 daPa for groups 1, 2, and 3, respectively. There were no significant differences between groups with respect to 2-kHz PW, RF, absorbance, LF-A, and HF-A. CONCLUSION: This was the first study that evaluated the effects of acute Ménière attacks on WBT findings. An acute Ménière attack was found to have no significant effect on the 2-kHz PW and other variables measured using WBT.


Assuntos
Testes de Impedância Acústica , Perda Auditiva Neurossensorial/diagnóstico , Doença de Meniere/diagnóstico , Adulto , Idoso , Feminino , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Masculino , Doença de Meniere/fisiopatologia , Pessoa de Meia-Idade , Estudos Prospectivos , Vibração
15.
J Int Adv Otol ; 15(2): 257-262, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31418715

RESUMO

OBJECTIVES: A surge of literature indicated that temporal integration and temporal envelope perception contribute largely to the perception of speech. A review of literature showed that the perception of speech with temporal integration and temporal envelope perception in noise might be affected due to sensorineural hearing loss but to a varying degree. Because the temporal integration and temporal envelope share similar physiological processing at the cochlear level, the present study was aimed to identify the relationship between temporal integration and temporal envelope perception in noise by individuals with mild sensorineural hearing loss. MATERIALS AND METHODS: Thirty adult males with mild sensorineural hearing loss and thirty age- and gender-matched normal-hearing individuals volunteered for being the participants of the study. The temporal integration was measured using synthetic consonant-vowel-consonant syllables, varied for onset, offset, and onset-offset of second and third formant frequencies of the vowel following and preceding consonants in six equal steps, thus forming a six-step onset, offset, and onset-offset continuum, each. The duration of the transition was kept short (40 ms) in one set of continua and long (80 ms) in another. Temporal integration scores were calculated as the differences in the identification of the categorical boundary between short- and long-transition continua. Temporal envelope perception was measured using sentences processed in quiet, 0 dB, and -5 dB signal-to-noise ratios at 4, 8, 16, and 32 contemporary frequency channels, and the temporal envelope was extracted for each sentence using the Hilbert transformation. RESULTS: A significant effect of hearing loss was observed on temporal integration, but not on temporal envelope perception. However, when the temporal integration abilities were controlled, the variable effect of hearing loss on temporal envelope perception was noted. CONCLUSION: It was important to measure the temporal integration to accurately account for the envelope perception by individuals with normal hearing and those with hearing loss.


Assuntos
Perda Auditiva Neurossensorial/fisiopatologia , Ruído , Percepção da Fala/fisiologia , Estimulação Acústica , Adulto , Análise de Variância , Limiar Auditivo/fisiologia , Humanos , Masculino , Psicoacústica , Razão Sinal-Ruído
16.
Orphanet J Rare Dis ; 14(1): 202, 2019 08 19.
Artigo em Inglês | MEDLINE | ID: mdl-31426838

RESUMO

BACKGROUND: Idiopathic bilateral vestibulopathy (IBV) is an acquired bilateral peripheral vestibular dysfunction of unknown etiology, with persistent unsteadiness but without sensorineural hearing loss (SNHL) other than age-related hearing loss (ARHL). MAIN TEXT: The prevalence of IBV is unknown. The most common symptom is persistent unsteadiness, particularly in darkness and/or on uneven ground. The other main symptom is oscillopsia during head and body movements. IBV is neither associated with SNHL, except for ARHL, nor any other neurological dysfunction that causes balance disorders. The clinical time course of IBV can generally be divided into two main types: progressive type and sequential type. The progressive type involves gradually progressive persistent unsteadiness without episodes of vertigo. The sequential type involves recurrent vertigo attacks accompanied by persistent unsteadiness. Originally, IBV was found to exhibit bilateral dysfunction in the lateral semicircular canals (LSCCs) and the superior vestibular nerve (SVN) system. However, recently, with the development of more sophisticated vestibular function tests of the otolith organs and vertical semicircular canals, it has been revealed that IBV involves peripheral vestibular lesions other than those already identified in the LSCC and the SVN system. Furthermore, novel subtypes of IBV that do not involve bilateral dysfunction of the LSCC and/or the SVN system have been proposed. Therapeutically, exercise-based vestibular rehabilitation in adult bilateral vestibulopathy (BVP) patients has resulted in improved gaze and postural stability moderately. There are several technical approaches for the treatment of BVP such as vestibular implants, sensory substitution devices and noisy galvanic vestibular stimulation. CONCLUSIONS: Combined use of various vestibular function tests, including recently developed tests, revealed the diversity of lesion sites in IBV. Further studies are required to determine the therapeutic effects of the technical approaches on IBV.


Assuntos
Vestibulopatia Bilateral/fisiopatologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Percepção de Movimento/fisiologia , Vertigem/fisiopatologia
17.
Eur Arch Otorhinolaryngol ; 276(11): 3089-3094, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31463602

RESUMO

PURPOSE: To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. METHODS: All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. RESULTS: No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale). CONCLUSIONS: A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.


Assuntos
Audiometria de Tons Puros/métodos , Audiometria da Fala/métodos , Implante Coclear , Perda Auditiva Neurossensorial , Perda Auditiva , Idoso , Implante Coclear/efeitos adversos , Implante Coclear/métodos , Implante Coclear/estatística & dados numéricos , Feminino , Perda Auditiva/classificação , Perda Auditiva/diagnóstico , Perda Auditiva/fisiopatologia , Perda Auditiva/cirurgia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/fisiopatologia , Perda Auditiva Neurossensorial/cirurgia , Humanos , Masculino , Avaliação de Processos e Resultados em Cuidados de Saúde , Valor Preditivo dos Testes , Prognóstico , Percepção da Fala
18.
Trends Hear ; 23: 2331216519864499, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31455167

RESUMO

Interaural phase difference (IPD) discrimination upper frequency limits and just-noticeable differences (JNDs), interaural level difference (ILD) JNDs, and diotic intensity JNDs were measured for 20 older hearing-impaired listeners with matched moderate sloping to severe sensorineural hearing losses. The JNDs were measured using tone stimuli at 500 Hz. In addition to these auditory tests, the participants completed a cognitive test (Trail Making Test). Significant performance improvements in IPD discrimination were observed across test sessions. Strong correlations were found between IPD and ILD discrimination performance. Very strong correlations were observed between IPD discrimination and Trail Making performance as well as strong correlations between ILD discrimination and Trail Making performance. These relationships indicate that interindividual variability in IPD discrimination performance did not exclusively reflect deficits specific to any auditory processing, including early auditory processing of temporal information. The observed relationships between spatial audition and cognition may instead be attributable to a modality-general spatial processing deficit and/or individual differences in global processing speed.


Assuntos
Percepção Auditiva/fisiologia , Perda Auditiva Neurossensorial/fisiopatologia , Adulto , Fatores Etários , Idoso , Limiar Auditivo , Cognição/fisiologia , Feminino , Audição , Perda Auditiva , Testes Auditivos , Humanos , Masculino
19.
Int J Pediatr Otorhinolaryngol ; 125: 201-205, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31401454

RESUMO

Many studies showed that children with sensorineural hearing loss (SNHL) have possible vestibular affection which is related to the severity of cochlear pathology. OBJECTIVES: this work is designed to evaluate vestibular function in children with congenital severe to profound SNHL and correlate the degree of hearing loss with the results of vestibular tests. METHODS: this work included 52 children divided into two groups; control group consisted of 20 normal hearing children with no vestibular complaints, and study group consisted of 32 children with congenital severe to profound SNHL. All children were submitted to basic audiologic evaluation, combined vestibular evoked myogenic potentials, sinusoidal harmonic acceleration test (SHA) of rotatory chair test. RESULTS: all children in the control group had normal oVEMP and cVEMPs results while abnormal cVEMPs and oVEMPs results were found in 89% and 96.9% of the tested ears of the study group respectively. Sinusoidal harmonic acceleration test results were normal in control group with significant reduction in gain, phase lead and higher level of asymmetry in 50% of the children in study group. There was a significant relation between the degree of hearing loss and the cVEMPs, oVEMPs abnormalities, while SHA test results showed no such relationship. CONCLUSION: There is an evident vestibular abnormalities in children with severe to profound sensorineural hearing loss as revealed by the rotatory chair testing and VEMPs recordings. Vestibular assessment is very important in such group as it has an impact on their rehabilitation plan.


Assuntos
Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/fisiopatologia , Doenças Vestibulares/complicações , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Masculino , Doenças Vestibulares/diagnóstico , Doenças Vestibulares/fisiopatologia , Vestíbulo do Labirinto/fisiopatologia
20.
Am J Audiol ; 28(2S): 437-449, 2019 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-31461328

RESUMO

Purpose This study was carried out to understand the neural intricacies of auditory spatial processing in listeners with sensorineural hearing impairment (SNHI) and compare it with normal hearing (NH) listeners using both local and global measures of waveform analyses. Method A standard group comparison research design was adopted in this study. Participants were assigned to 2 groups. Group I consisted of 13 participants with mild-moderate flat or sloping SNHI, while Group II consisted of 13 participants with NH sensitivity. Electroencephalographic data using virtual acoustic stimuli (spatially loaded stimuli played in center, right, and left hemifields) were recorded from 64 electrode sites in passive oddball paradigm. Both local (electrode-wise waveform analysis) and global (dissimilarity index, electric field strength, and topographic pattern analyses) measures were performed on the electroencephalographic data. Results Results of local waveform analyses marked the appearance of mismatch negativity in an earlier time window, relative to those reported conventionally in both the groups. The global measures of electric field strength and topographic modulations (dissimilarity index) revealed differences between the 2 groups in different time periods, indicating multiphases (integration and consolidation) of spatial processing. Further, the topographic pattern analysis showed the emergence of different scalp maps for SNHI and NH in the time window corresponding to mismatch negativity (78-150 ms), suggestive of differential spatial processing between the groups at the cortical level. Conclusions The findings of this study highlights the differential allotment of neural generators, denoting variations in spatial processing between SNHI and NH individuals.


Assuntos
Encéfalo/fisiopatologia , Potenciais Evocados Auditivos/fisiologia , Perda Auditiva Neurossensorial/fisiopatologia , Processamento Espacial/fisiologia , Estimulação Acústica , Adulto , Idoso , Atenção , Estudos de Casos e Controles , Eletroencefalografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
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