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1.
Ann Otol Rhinol Laryngol ; 130(1): 112-115, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32613850

RESUMO

OBJECTIVE: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature. CASE PRESENTATION AND MANAGEMENT: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. RESULTS: The surgery was without complications and postoperative course was uneventful. Two years after surgery, pure-tone and speech soundfield audiometry with left cochlear implant switched on showed a good improvement in pure-tone threshold and a word recognition score of 50% at 60 dB nHL. Literature review does not report any previous case of AIED EGPA-related. CONCLUSIONS: Cochlear implantation in AIED EGPA-related have been shown to be a viable treatment option in a stabilized phase of disease.


Assuntos
Síndrome de Churg-Strauss/complicações , Implante Coclear , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/etiologia , Adulto , Feminino , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Súbita/cirurgia , Humanos
2.
Otol Neurotol ; 42(1): e10-e14, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33301283

RESUMO

OBJECTIVE: To describe a case of bilateral sudden sensorineural hearing loss (SSNHL) and intralabyrinthine hemorrhage in a patient with COVID-19. STUDY DESIGN: Clinical capsule report. SETTING: Tertiary academic referral center. PATIENT: An adult woman with bilateral SSNHL, aural fullness, and vertigo with documented SARS-CoV-2 infection (IgG serology testing). INTERVENTIONS: High-dose oral prednisone with taper, intratympanic dexamethasone. MAIN OUTCOME MEASURES: Audiometric testing, MRI of the internal auditory canal with and without contrast. RESULTS: A patient presented with bilateral SSNHL, bilateral aural fullness, and vertigo. Serology testing performed several weeks after onset of symptoms was positive for IgG COVID-19 antibodies. MRI showed bilateral intralabyrinthine hemorrhage (left worse than right) and no tumor. The patient was treated with two courses of high-dose oral prednisone with taper and a left intratympanic dexamethasone injection, resulting in near-resolution of vestibular symptoms, a fluctuating sensorineural hearing loss in the right ear, and a severe to profound mixed hearing loss in the left ear. CONCLUSIONS: COVID-19 may have otologic manifestations including sudden SSNHL, aural fullness, vertigo, and intralabyrinthine hemorrhage.


Assuntos
/complicações , Perda Auditiva Neurossensorial/virologia , Hemorragia/virologia , Doenças do Labirinto/virologia , Adolescente , Anti-Inflamatórios/uso terapêutico , Dexametasona/administração & dosagem , Feminino , Perda Auditiva Bilateral/virologia , Perda Auditiva Súbita/virologia , Humanos , Injeção Intratimpânica , Prednisona/uso terapêutico
3.
Braz. j. otorhinolaryngol. (Impr.) ; 86(6): 767-773, Nov.-Dec. 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1142594

RESUMO

Abstract Introduction: Acute otitis media is a disease with high global prevalence, that can lead to several acute complications and auditory sequelae. Data regarding the auditory evaluation in the acute phase of acute otitis media are scarce. Objective: To evaluate the main audiometric changes (air and bone conduction thresholds) in the initial phase of an acute otitis media episode. Methods: A case-control study was performed. Patients diagnosed with acute otitis media with less than 7 days of evolution in relation to the complaint onset were selected, and healthy volunteers were selected as controls. The acute otitis media and control groups were submitted to pure tone and vocal audiometry. Results: The acute otitis media group included a total of 27 patients (30 ears). Hearing loss was present in 90.0% of the ears with acute otitis media, with conductive loss in 14 (46.67%) and mixed loss in 13 (43.33%). Both the air and bone conduction thresholds obtained with the tonal audiometry in the acute otitis media group were significantly worse than the controls at all tested frequencies (p< 0.05). In patients with acute otitis media, we observed that the thresholds for frequency >1 kHz (bone conduction) and 3 kHz (air conduction) were significantly worse in patients with tinnitus compared to patients without tinnitus. Conclusion: During the first 7 days of evolution after the onset of an isolated episode of acute otitis media, we observed significant increases in bone and air thresholds at all frequencies, especially >2 kHz, compared to healthy ears.


Resumo Introdução: A otite média aguda é uma doença de elevada incidência global, que pode levar a diversas complicações agudas e sequelas auditivas. Dados referentes à avaliação auditiva na fase aguda da otite média aguda são escassos. Objetivo: Avaliar as principais alterações audiométricas (limiares em via aérea e óssea) na fase inicial de um episódio de otite média aguda. Método: Realizou-se estudo de caso-controle. Selecionamos pacientes com diagnóstico de otite média aguda, com menos de sete dias de evolução em relação ao início das queixas, e voluntários saudáveis foram selecionados como controles. Os grupos otite média aguda e controle foram submetidos a audiometria tonal, vocal e audiometria. Resultados: O grupo otite média aguda incluiu 27 pacientes (30 orelhas). Observou-se presença de perda auditiva em 90% das orelhas com otite média aguda, condutiva em 14 (46,67%) e mista em 13 (43,33%). Tanto os limiares auditivos por via aérea quanto os limiares por via óssea obtidos com audiometria tonal do grupo otite média aguda eram significativamente piores em relação aos controles, em todas as frequências testadas (p < 0,05). Em pacientes com otite média aguda, observamos que os limiares das frequências acima de 1 kHz (via óssea) e 3 kHz (via aérea) eram significantemente piores entre pacientes com zumbido em comparação a pacientes sem zumbido. Conclusão: Nos primeiros sete dias de evolução do quadro inicial de um episódio isolado de otite média aguda, observamos aumentos significativos dos limiares ósseos e aéreos em todas as frequências, principalmente nas acima de 2 kHz, em comparação a orelhas sadias.


Assuntos
Humanos , Otite Média/complicações , Perda Auditiva Neurossensorial/etiologia , Audiometria de Tons Puros , Limiar Auditivo , Condução Óssea , Estudos de Casos e Controles
4.
Zhonghua Yi Xue Za Zhi ; 100(46): 3674-3679, 2020 Dec 15.
Artigo em Chinês | MEDLINE | ID: mdl-33342143

RESUMO

Objective: To explore the value of speculating etiology of the magnetic resonance imaging (MRI) T1 weighted imaging (T1WI) labyrinthine high signal ratio in patients with unilateral sudden deafness accompanied by vertigo and tinnitus and its relationship with hearing prognosis. Methods: Fifty-two patients with unilateral sudden deafness accompanied by vertigo and tinnitus who were admitted to Beijing Tongren Hospital Affiliated to Capital Medical University from January 2016 to July 2019 were collected, including 27 males and 25 females, aged (47.7±15.1) years. The inner ear MRI data of 52 patients (17 plain scan, 35 enhanced scan) with unilateral sudden deafness were retrospectively analyzed. Two radiologists independently measured the labyrinthine high signal intensity of the affected side and the contralateral normal side on T1WI and enhanced T1WI and calculated the signal ratio (the normal labyrinth signal was subtracted from the affected signal and then divided by the normal signal). The etiology of the enhanced group was judged based on two methods, including whether the abnormal high signal was enhanced or not (unenhancement indicated hemorrhage and enhancement indicated inflammation), and the locations of labyrinthine involvement on enhanced three-dimensional fluid attenuated inversion recovery (3D-FLAIR) (inflammation usually involved the perilymph spaces, while hemorrhage involved the perilymph and endolymph spaces). In the plain group, the locations of labyrinthine involvement on 3D-FLAIR was applied to infer the potential etiology. Results: The two methods presumed that 8 cases might be hemorrhage (22.9%, 8/35) and 27 be inflammation (77.1%, 27/35) in the enhanced group, which had a high consistency, while it was speculated that 7 patients might be hemorrhage (7/17) and 10 patients be inflammation (10/17) in the plain group. The measurement results of the two radiologists were highly consistent within and between the groups [the intraclass correlation coefficient (ICC) values were greater than 0.800]. The area under the receiver operating characteristic (ROC) curve (AUC) of the T1WI high signal ratio in the enhanced group for speculating etiology was 0.949 (P<0.01), when the predictive threshold value was 0.467, with a sensitivity of 96.3% and a specificity of 87.5%. It might be hemorrhage when the ratio was higher than the threshold value, otherwise it was inflammation. The T1WI labyrinthine high signal ratio was higher in the hemorrhage group than that of the inflammation group, and the hearing prognosis was worse (all P<0.05). The T1WI labyrinthine high signal ratio of the unrecovered group was higher than that of the recovered group (P=0.034). Conclusions: The etiology of labyrinthine high signal formation can be inferred by quantitative values combined with the involved sites. The high signal in the labyrinth indicates poor hearing prognosis, the higher the signal intensity, the greater the possibility of hemorrhage and the worse the hearing prognosis.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Zumbido , Adulto , Orelha Interna/diagnóstico por imagem , Feminino , Perda Auditiva Súbita/diagnóstico por imagem , Humanos , Imageamento Tridimensional , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Zumbido/diagnóstico por imagem , Vertigem
5.
Pan Afr Med J ; 35(Suppl 2): 139, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33193954

RESUMO

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 19-year-old man known for TRMA, who presented in the emergency department with bicytopenia (haemoglobin 5,4 g/dL, thrombocytes 38×109/L) revealed by dyspnea and chest pain. Investigations excluded bleeding, hemolysis, coagulopathy and iron deficiencies. A recent infection and an acute coronary syndrome have also been eliminated. We later found out that thiamine treatment had been discontinued three months before, due to general confinement in Tunisia during the COVID-19 pandemic. Parenteral administration of 100 mg of thiamine daily resulted in the recovery of haematopoiesis within three weeks.


Assuntos
Anemia Megaloblástica/sangue , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Diabetes Mellitus/sangue , Perda Auditiva Neurossensorial/sangue , Pandemias , Pneumonia Viral/epidemiologia , Deficiência de Tiamina/congênito , Trombocitopenia/etiologia , Síndrome Coronariana Aguda/diagnóstico , Anemia Megaloblástica/tratamento farmacológico , Anemia Megaloblástica/fisiopatologia , Dor no Peito/etiologia , Diabetes Mellitus/tratamento farmacológico , Diabetes Mellitus/fisiopatologia , Diagnóstico Diferencial , Hemoglobina A Glicada/análise , Acesso aos Serviços de Saúde , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/fisiopatologia , Hemoglobinas/análise , Humanos , Masculino , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Recidiva , Tiamina/provisão & distribução , Tiamina/uso terapêutico , Deficiência de Tiamina/sangue , Deficiência de Tiamina/tratamento farmacológico , Deficiência de Tiamina/fisiopatologia , Tunísia , Adulto Jovem
6.
Codas ; 32(5): e20190183, 2020.
Artigo em Português, Inglês | MEDLINE | ID: mdl-33174988

RESUMO

The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic and autoimmune disease. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis, however, the syndrome may also affect the melanocytes tissues, for example, the eyes, inner ear, meninges and skin. The syndrome origin mechanism is not yet completely known. Commonly, the specific ethnic groups that are affected by the VKHS are as follows: Hispanics, Asians, Indians, Native Americans and ethnic groups from the Middle East. The audiological characteristics of the syndrome and the possible audiologist interventions for a specific case will be reported. The patient was attended at the Clinic of Speech Therapy, Faculdade de Odontologia de Bauru (FOB). She is 53 years old and presented audiological complaints. She was diagnosed with VKHS by a specialist doctor. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, complaints about postural vertigo and acute tinnitus. The specific case reported presented sudden sensorineural hearing loss, vertigo, tinnitus and bilateral ocular disease. Even though drug treatment was performed, the hearing loss remained. Therefore, before the hearing aid (HA) fitting, the audiologist should perform the hearing management, investigate if the patient takes the drug treatment and the occurrence of suggestive symptoms of the syndrome. These are some points that help in the reference to the specialist doctor and the audiologist strongly participates in what concerns the hearing.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva , Zumbido , Síndrome Uveomeningoencefálica , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Humanos , Pessoa de Meia-Idade , Síndrome Uveomeningoencefálica/complicações , Síndrome Uveomeningoencefálica/diagnóstico , Vertigem
7.
Niger J Clin Pract ; 23(11): 1494-1499, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33221771

RESUMO

Background: Presbyacusis is a cause of hearing loss in adults worldwide. It is a type of auditory dysfunction thought to be due to a series of insults over time including agerelated degeneration, noise exposure, and diseases of the ear. It usually affects the high frequencies of hearing. Presbyacusis has a tremendous impact on the quality of life of millions of the aged and is fast becoming an increasingly prevalent disorder as the population ages. Nigerian population is becoming progressively older making it imperative to focus on this area of research. It is worth noting that the Nigerian public health system does not have an effective and sustainable hearing loss screening strategy for lateonset hearing loss in adults. Aim/Objective: This study evaluated the hearing threshold and pure tone audiometric pattern/s seen in participants with agerelated hearing loss (ARHL) in Gwagwalada Area Council of the Federal capital territoryAbuja. Subjects and Methods: The study was a prospective communitybased crosssectional study, and a multistage sampling technique was adopted for this study, conducted at Gwagwalada, Abuja. Individuals from 55 years and above were recruited into the study after signing an informed consent. After a thorough and detailed clinical assessment, tympanometry and diagnostic puretone audiometry were carried out in all the participants. Result: One hundred and fourteen participants were recruited into the study, out of which fourteen were excluded from the analysis on account of various exclusion criteria. The age range of the participants was 55 to 79 years, with a mean age of 65.8 ± 5.6 years.Twentyfive (25.0%) participants had normal hearing threshold, while seventyfive (75.0%) participants had sensorineural hearing loss (SNHL). Conclusion: The study showed that the prevalence of ARHL from age 55 years and above at Gwagwalada Area Council, of FCT, Abuja was 75.0%.


Assuntos
Audiometria de Tons Puros/métodos , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva/diagnóstico , Qualidade de Vida/psicologia , Testes de Impedância Acústica , Adolescente , Adulto , Idoso , Limiar Auditivo , Estudos Transversais , Feminino , Perda Auditiva/epidemiologia , Perda Auditiva/etiologia , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Prevalência , Estudos Prospectivos , Adulto Jovem
9.
Artigo em Chinês | MEDLINE | ID: mdl-33254313

RESUMO

Objective:The etiology and pathophysiologic mechanism of sudden sensorineural hearing loss are undefined. We will use artificial intelligence and big data methods to explore the correlation between sudden sensorineural hearing loss and serum indices. Method:A total of 1218 patients with sudden deafness admitted to Sun Yat-sen Memorial Hospital were selected as the experimental group, 95 861 healthy subjects were randomly selected as the control group at the same period. Serum biochemical indexes in two groups were collected and analyzed by TreeNet and CART machine learning algorithms, to screen out highly correlated indicators with sudden sensorineural hearing loss and dig out a set of clinical features for people with high risk of sudden sensorineural hearing loss. Result:It was found that high prevalence rate of sudden sensorineural hearing loss is related to eosinophils, reticulocyte and fibrinogen. The areas under the receiver operator characteristic curves(ROC-AUC) were exploited to evaluate the prediction performance of TreeNet model. Overall the TreeNet model has provided high predictive ability by ROC curve, achieving AUC of 0.99, both recall and accuracy rate of 99.90%. Conclusion:There is significant difference between sudden deadness and normal people in serum biochemical indexes. Eosinophil is the first important indicator to distinguish sudden sensorineural hearing loss. Treenet model has important referenced significance for the screening and diagnosis of sudden sensorineural hearing loss.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Inteligência Artificial , Big Data , Fibrinogênio , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/epidemiologia , Humanos
10.
Artigo em Chinês | MEDLINE | ID: mdl-33254314

RESUMO

Objective:To investigate the distribution of common inner ear and internal auditory canal malformations in children with single-sided deafness(SSD) ,and to explore the imaging etiology of SSD by comparing the quantitative parameters of key bone structures between deaf and normal ears in children with congenital SSD. Method:Forty children with SSD diagnosed in the Second Hospital of Lanzhou University from September 2016 to March 2019 were collected. All of them underwent HRCT examinations of temporal bone . The area of bone island, the width of vestibular, the width of internal auditory canal, the height of cochlear and the width of cochlear basal axis were measured. Paired t test was used to compare the difference between the hearing abnormality and normal hearing in children with SSD. Result:The rate of inner ear deformity was 62.5% in SSD group,the most common deformity was cochlear nerve canal deformity, 20 cases (50.0%) of cochlear canal stenosis and 3 cases (7.5%) of cochlear canal atresia.The second most common deformity was internal auditory canal deformity, including 5 cases (12.5%) of internal auditory canal stenosis and 1 case (2.5%) of internal auditory canal atresia. Other malformations included 1 case(2.5%) of RO, 2 cases (5.0%) of incomplete partition (IP) type II and 1 case (2.5%) of enlargement of vestibular aqueduct (EVA). There are no significant difference in the measured results of the key structures of the inner ear between two groups except the width of cochlear nerve canal, internal auditory canal and the area of bone island. Conclusion:The main inner ear deformities in children with SSD are cochlear nerve canal stenosis and inner auditory canal stenosis. HRCT of temporal bone has high diagnostic value for inner ear deformities in children with SSD.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Aqueduto Vestibular , Criança , Cóclea , Nervo Coclear , Surdez/diagnóstico por imagem , Humanos , Estudos Retrospectivos , Osso Temporal/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Aqueduto Vestibular/diagnóstico por imagem
11.
Artigo em Chinês | MEDLINE | ID: mdl-33254317

RESUMO

Objective:This study aimed to assess the clinical practice value of ocular vestibular evoked myogenic potential(oVEMP) and cervical vestibular evoked myogenic potential(cVEMP) in monitoring the rehabilitation of vestibular function in patients with suddensen sorineural hearing loss(SSHL). Method:Twenty-four patients with SSHL were retrospectively enrolled, showing no VEMP response on the affected side but exhibiting VEMP responses after therapies We analyzed the improvement and the restoration of hearing and the parameters of VEMP response. Result:After treatment, seven patients showed VEMP recovery, including three cases with both oVEMP and cVEMP recovery, three cases with oVEMP recovery, and one case with cVEMP recovery. Between VEMP recoved group and VEMP unrecoved group, before treatment, no significant difference was found in the thresholds of pure-tone audiometry(PTA). However, after treatment, VEMP recoved group exhibited lower PTA thresholds and better PTA shift (P<0.01). Multivariate analysis revealed that recovery of VEMP was the independent risk factor for the therapeutic effect of SSHL. Conclusion:The Combination of oVEMP and cVEMP is an objective tool for assessing vestibular otolithic end organ function during dynamic functional recovery in SSHL and the recovery of VEMP could predict the auditory improvement.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Potenciais Evocados Miogênicos Vestibulares , Audiometria de Tons Puros , Humanos , Membrana dos Otólitos , Estudos Retrospectivos
12.
Artigo em Chinês | MEDLINE | ID: mdl-33254356

RESUMO

Sensorineural hearing loss(SNHL) is a difficult and hot issue in the field of otology. The effect of medications targeting the mechanism of SNHL are often unsatisfactory for hearing improvement. Hearing aids and cochlear implants are the mainstream treatment methods at present, but neither of them can reverse the pathological changes of affected inner ear. In recent years, mesenchymal stem cells transplantation has been confirmed by several animal studies and clinical trials to have great potential for clinical applications in restoring the structure of the inner ear and hearing improvement to some extent. In this review, we review the characteristics of umbilical cord blood derived mesenchymal stem cells(UCBMSCs), the approaches of transplantation, the efficacy and mechanism of UCBMSCs in the treatment of SNHL, and the safety of clinical application, covering the existing problems and future prospects of this rising treatment.


Assuntos
Implante Coclear , Perda Auditiva Neurossensorial , Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais , Animais , Sangue Fetal , Perda Auditiva Neurossensorial/terapia
13.
Vestn Otorinolaringol ; 85(5): 18-23, 2020.
Artigo em Russo | MEDLINE | ID: mdl-33140928

RESUMO

Studies of the recent years are devoted to using the auditory steady-state responses (ASSR) as an evaluation method of amplification outcomes. In this case stimulation is performed in the sound field via loudspeaker with a hearing aid (HA) in patient's ear. GOAL: Estimation the optimal stimulation parameters of ASSR in free field, which provide maximum correlation with behavioral thresholds. MATERIALS AND METHODS: 26 subjects were included in the study: 10 with normal hearing (4 adults and 6 children) and 16 children (1-16 years old) with sensorineural hearing loss (SNHL). Aided and unaided ASSR thresholds were compared with corresponding behavioral thresholds (BT). All recordings were conducted in free sound field. We used mono-frequency stimulation method at 500-4000 Hz career frequencies. Modulation frequency was set as 40 Hz. Types of stimuli modulation: AM/FM modulation, exponential modulation (AM2), three carrier frequencies and modulated Chirp-tone (Chirp). RESULTS: In normal hearing subjects the difference between ASSR and BT varied from 0 dB to 35 dB, and the significant correlation between them was observed (p<0.05). The maximum difference between ASSR and BT in children with SNHL was at 500 Hz (15.6±4.3 dB), minimum - at 2 and 4 kHz (8±2.9 dB and 7±3 dB respectively). In aided condition maximum difference was detected at 500 Hz (10.2±3.8 dB), minimum - at 1 and 2 kHz (7.8±2.8 and 7.3±3.1 dB respectively). We got significantly less difference between ASSR and BT in all test conditions using following types of stimuli: at 500 Hz and 1000 Hz - AM2 (p<0.01); at 2 and 4 kHz - Chirp (p<0.05 and p<0.01 respectively). Thereby, free field ASSR might be used as an objective method of evaluation the amplification outcomes in children with SNHL.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Estimulação Acústica , Adolescente , Adulto , Limiar Auditivo , Criança , Pré-Escolar , Perda Auditiva Neurossensorial/diagnóstico , Testes Auditivos , Humanos , Lactente
14.
Vestn Otorinolaringol ; 85(5): 29-32, 2020.
Artigo em Russo | MEDLINE | ID: mdl-33140930

RESUMO

To determine the electrode impedance dynamics changes in the postoperative period after cochlear implantation as well as the time of their stabilization. MATERIAL AND METHODS: 75 patients with a bilateral sensorineural deafness aged from 1 to 4 years (average age - 1.5 years) were included in the study. 50 patients were implanted with Nucleus cochlear implants (Cochlear - Australia) and other 25 patients - with Advanced Bionics implants (Switzerland). RESULTS: The impedance dynamics analysis in two groups of patients implanted with «Cochlear¼ and «Advanced Bionics¼ cochlear implants demonstrated that statistically significant decrease in impedance (p<0.05) was observed after the beginning of electrical stimulation and the reduction of postoperative inflammatory process in the inner ear. The stabilization of the impedance levels in both groups of patients was obtained in 3-6 months after the switch-on of speech processor.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Neurossensorial , Criança , Pré-Escolar , Cóclea , Impedância Elétrica , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/cirurgia , Humanos , Lactente
15.
Vestn Otorinolaringol ; 85(5): 88-92, 2020.
Artigo em Russo | MEDLINE | ID: mdl-33140942

RESUMO

The article presents a review of the literature on the treatment of acute sensorineural hearing loss, acoustic trauma in people exposed to impulse noise. Groups of the most used drugs for the pharmacological correction of hearing impairment in this pathology are present, such as glucocorticosteroids, antioxidants, nootropics, antihypoxants, and others, are covered. Particular attention is paid to use of drugs taking into account the main links of pathogenesis.


Assuntos
Perda Auditiva Provocada por Ruído , Perda Auditiva Neurossensorial , Perda Auditiva Provocada por Ruído/diagnóstico , Perda Auditiva Provocada por Ruído/tratamento farmacológico , Perda Auditiva Provocada por Ruído/etiologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/terapia , Humanos , Estudos Prospectivos
16.
BMC Med Genet ; 21(1): 216, 2020 11 02.
Artigo em Inglês | MEDLINE | ID: mdl-33138774

RESUMO

BACKGROUND: To determine the carrier frequency and pathogenic variants of common genetic disorders in the north Indian population by using next generation sequencing (NGS). METHODS: After pre-test counselling, 200 unrelated individuals (including 88 couples) were screened for pathogenic variants in 88 genes by NGS technology. The variants were classified as per American College of Medical Genetics criteria. Pathogenic and likely pathogenic variants were subjected to thorough literature-based curation in addition to the regular filters. Variants of unknown significance were not reported. Individuals were counselled explaining the implications of the results, and cascade screening was advised when necessary. RESULTS: Of the 200 participants, 52 (26%) were found to be carrier of one or more disorders. Twelve individuals were identified to be carriers for congenital deafness, giving a carrier frequency of one in 17 for one of the four genes tested (SLC26A4, GJB2, TMPRSS3 and TMC1 in decreasing order). Nine individuals were observed to be carriers for cystic fibrosis, with a frequency of one in 22. Three individuals were detected to be carriers for Pompe disease (frequency one in 67). None of the 88 couples screened were found to be carriers for the same disorder. The pathogenic variants observed in many disorders (such as deafness, cystic fibrosis, Pompe disease, Canavan disease, primary hyperoxaluria, junctional epidermolysis bullosa, galactosemia, medium chain acyl CoA deficiency etc.) were different from those commonly observed in the West. CONCLUSION: A higher carrier frequency for genetic deafness, cystic fibrosis and Pompe disease was unexpected, and contrary to the generally held view about their prevalence in Asian Indians. In spite of the small sample size, this study would suggest that population-based carrier screening panels for India would differ from those in the West, and need to be selected with due care. Testing should comprise the study of all the coding exons with its boundaries in the genes through NGS, as all the variants are not well characterized. Only study of entire coding regions in the genes will detect carriers with adequate efficiency, in order to reduce the burden of genetic disorders in India and other resource poor countries.


Assuntos
Acil-CoA Desidrogenase/deficiência , Doença de Canavan/genética , Fibrose Cística/genética , Epidermólise Bolhosa Juncional/genética , Galactosemias/genética , Doença de Depósito de Glicogênio Tipo II/genética , Perda Auditiva Neurossensorial/genética , Hiperoxalúria Primária/genética , Erros Inatos do Metabolismo Lipídico/genética , Acil-CoA Desidrogenase/genética , Adulto , Doença de Canavan/epidemiologia , Conexinas/genética , Fibrose Cística/epidemiologia , Epidermólise Bolhosa Juncional/epidemiologia , Feminino , Galactosemias/epidemiologia , Expressão Gênica , Triagem de Portadores Genéticos/estatística & dados numéricos , Aconselhamento Genético , Doença de Depósito de Glicogênio Tipo II/epidemiologia , Perda Auditiva Neurossensorial/epidemiologia , Heterozigoto , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Hiperoxalúria Primária/epidemiologia , Índia/epidemiologia , Erros Inatos do Metabolismo Lipídico/epidemiologia , Masculino , Proteínas de Membrana/genética , Pessoa de Meia-Idade , Mutação , Proteínas de Neoplasias/genética , Serina Endopeptidases/genética , Transportadores de Sulfato/genética
17.
BMC Med Genet ; 21(1): 226, 2020 11 18.
Artigo em Inglês | MEDLINE | ID: mdl-33208113

RESUMO

BACKGROUND: Clinical genetic diagnosis of non-syndromic hearing loss (NSHL) is quite challenging. With regard to its high heterogeneity as well as large size of some genes, it is also really difficult to detect causative mutations using traditional approaches. One of the recent technologies called whole-exome sequencing (WES) has been thus developed in this domain to remove the limitations of conventional methods. METHODS: This study was a report on a research study of two unrelated pedigrees with multiple affected cases of hearing loss (HL). Accordingly, clinical evaluations and genetic analysis were performed in both families. RESULTS: The results of WES data analysis to uncover autosomal recessive non-syndromic hearing loss (ARNSHL) disease-causing variants was reported in the present study. Initial analysis identified two novel variants of MYO15A i.e. c.T6442A:p.W2148R and c.10504dupT:p.C3502Lfs*15 correspondingly which were later confirmed by Sanger validations and segregation analyses. According to online prediction tools, both identified variants seemed to have damaging effects. CONCLUSION: In this study, whole exome sequencing were used as a first approach strategy to identify the two novel variants in MYO15A in two Iranian families with ARNSHL.


Assuntos
Surdez/genética , Perda Auditiva Neurossensorial/genética , Mutação , Miosinas/genética , Adolescente , Adulto , Sequência de Bases , Consanguinidade , Surdez/diagnóstico , Surdez/patologia , Feminino , Expressão Gênica , Genes Recessivos , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/patologia , Humanos , Irã (Geográfico) , Masculino , Miosinas/deficiência , Linhagem , Sequenciamento Completo do Exoma
18.
Braz. j. otorhinolaryngol. (Impr.) ; 86(5): 558-567, Sept.-Oct. 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1132638

RESUMO

Abstract Introduction: Hearing aid users reject their own hearing aid because of annoyance with background noise. The reason for dissatisfaction is located anywhere from the hearing aid microphone to the integrity of neurons along the auditory pathway. In this preview, the output of hearing aid was recorded at the level of ear canal and at auditory cortex in good and poor hearing aid users, who were classified using acceptable noise level. Objective: To study the representation of amplified speech in good and poor hearing aid performers. Methods: A total of 60 participants (age ranged 15-65 years) with moderate bilateral sensorineural hearing impairment grouped into good (n = 35) and poor (n = 25) hearing aid performers. Gap detection test and aided SNR 50 were administered. In addition, ear canal acoustic measures and cortical auditory evoked potentials were recorded in unaided and aided conditions at 65 dB SPL. Results: Hearing aid minimally alters temporal contrast of speech reflected in envelope difference index. Although having similar temporal impairment, acoustic characteristics of amplified speech sounds and SNR 50 scores from both groups, the aided cortical auditory evoked potentials surprisingly showed significant earlier latencies and higher amplitudes in good performers than poor performers. In addition, good and poor performers classified based on annoyance level was predicted by latencies of 2N1 and 2P2 components of acoustic change complex. Further, a follow-up revealed hearing aid use has relation with acceptance towards noise. Conclusion: Participants who are willing to accept noise from those who are not willing to accept noise have subtle physiological changes evident at the auditory cortex, which supports the hearing aid usage.


Resumo Introdução: Usuários de aparelhos auditivos rejeitam seu próprio aparelho pelo incômodo relacionado ao ruído de fundo. O motivo da insatisfação varia desde o microfone do aparelho auditivo até a integridade dos neurônios ao longo da via auditiva. Nesta análise prévia, a saída da prótese auditiva foi registrada no nível do meato auditivo externo e no córtex auditivo em bons e maus usuários de prótese auditiva, que foram classificados com um nível de ruído aceitável. Objetivo: Estudar a representação da fala amplificada em bons e maus usuários de aparelhos auditivos. Método: Foram examinados 60 participantes (de 15 a 65 anos) com deficiência auditiva neurossensorial bilateral moderada divididos em bons (n = 35) e maus (n = 25) usuários de aparelhos auditivos. Teste de detecção de gap e SNR 50 assistido foi administrado. Além disso, as medidas acústicas do meato externo e os potenciais evocados auditivos corticais foram registrados em condições não assistida e assistida a 65 dB NPS. Resultados: O aparelho auditivo altera minimamente o contraste temporal da fala, refletido no envelope difference index. Embora tenham uma deficiência temporal similar, as características acústicas de sons de fala amplificados e escores do SNR 50 de ambos os grupos, os potenciais evocados auditivos corticais assistidos surpreendentemente demonstraram latências iniciais significativas e maiores amplitudes em bons do que em maus usuários. Além disso, os bons em comparação com os maus usuários, classificados com base no nível de incômodo, foram previstos pelas latências dos componentes 2N1 e 2P2 do complexo de alteração acústica. Além disso, os resultados do seguimento revelaram que o uso de aparelhos auditivos têm relação com a aceitação do ruído. Conclusão: Os participantes dispostos a aceitar o ruído, quando comparados com aqueles que não estão dispostos a aceitá-lo, apresentam evidência de sutis alterações fisiológicas no córtex auditivo, que apoiam o uso da prótese auditiva.


Assuntos
Humanos , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Percepção da Fala , Auxiliares de Audição , Fala , Perda Auditiva Neurossensorial , Ruído
19.
Rev. neurol. (Ed. impr.) ; 71(7): 253-256, 1 oct., 2020. ilus
Artigo em Espanhol | IBECS | ID: ibc-195710

RESUMO

INTRODUCCIÓN: La siderosis superficial del sistema nervioso central es una patología poco frecuente secundaria al sangrado crónico en el líquido cefalorraquídeo. Los tumores medulares son causa poco habitual de siderosis superficial, y el ependimoma es la etiología más común. CASO CLÍNICO: Mujer con pérdida auditiva neurosensorial y ataxia cerebelosa, diagnosticada de siderosis superficial en la resonancia magnética cerebral. No tenía antecedentes de dolor raquídeo axial, dolor radicular ni incontinencia esfinteriana. En la resonancia magnética de la columna se encontró una lesión intradural en S1. No se observaron signos de hemorragia intratumoral en las secuencias de resonancia magnética en eco de gradiente. En la cirugía, se apreció una masa blanda intradural con signos de sangrado crónico que se resecó. Basado en el examen microscópico e inmunohistoquímico de la muestra, se alcanzó el diagnóstico de paraganglioma de grado I de la Organización Mundial de la Salud. CONCLUSIONES. Dado que el único tratamiento probado capaz de prevenir un mayor deterioro por la siderosis superficial es detener el sangrado crónico en el espacio subaracnoideo, es importante establecer un diagnóstico temprano de la fuente de sangrado. Los casos no justificados de siderosis superficial del sistema nervioso central deben incluir una resonancia magnética de la columna rutinaria para descartar el sangrado de un tumor medular, incluso en pacientes asintomáticos. Debido a la gravedad del deterioro potencial causado por la siderosis superficial, cualquier lesión tumoral observada en una resonancia magnética del raquis, incluso sin presentar signos de sangrado, debería ser objeto de indicación quirúrgica


INTRODUCTION: Superficial siderosis of the central nervous system is an infrequent pathology secondary to chronic bleeding into the cerebrospinal fluid. Spinal tumors are infrequent cause of superficial siderosis being ependymoma the most common etiology. CASE REPORT: We report the case of a woman with sensorineural hearing loss and cerebellar ataxia, diagnosed of superficial siderosis on brain MRI. She had no previous history of axial back pain or radicular leg pain or bowel or bladder incontinence. On spine MRI an intradural lesion was found at the S1 level. No signs of intratumoral hemorrhage were observed on MRI gradient-echo images. At surgery, an intradural soft mass with signs of chronic bleeding was completely resected. Based on microscopic examination and immunohistochemistry of the specimen, a diagnosis of paraganglioma World Health Organization grade I was made. CONCLUSIONS: Since the only proven treatment able to prevent further deterioration from superficial siderosis is to stop chronic bleeding into subarachnoid space, is of paramount importance to establish an early diagnosis of the source of bleeding. Cases of unexplained superficial siderosis of central nervous system should include routine spinal MRI to rule out bleeding of spinal tumor even in asymptomatic patients. Due to severity of potential deterioration caused by superficial siderosis, any tumoral lesion observed on spinal MRI even without documented sings of bleeding should be considered for resection


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Siderose/complicações , Sistema Nervoso Central/patologia , Paraganglioma/diagnóstico , Perda Auditiva Neurossensorial/diagnóstico por imagem , Siderose/terapia , Siderose/diagnóstico por imagem , Paraganglioma/patologia , Espectroscopia de Ressonância Magnética , Paraganglioma/cirurgia , Hemorragia/etiologia , Hemorragia/fisiopatologia , Transtornos Cognitivos/complicações , Neoplasias da Medula Espinal/patologia
20.
Trends Hear ; 24: 2331216520960861, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33073727

RESUMO

Effective hearing aid (HA) rehabilitation requires personalization of the HA fitting parameters, but in current clinical practice only the gain prescription is typically individualized. To optimize the fitting process, advanced HA settings such as noise reduction and microphone directionality can also be tailored to individual hearing deficits. In two earlier studies, an auditory test battery and a data-driven approach that allow classifying hearing-impaired listeners into four auditory profiles were developed. Because these profiles were found to be characterized by markedly different hearing abilities, it was hypothesized that more tailored HA fittings would lead to better outcomes for such listeners. Here, we explored potential interactions between the four auditory profiles and HA outcome as assessed with three different measures (speech recognition, overall quality, and noise annoyance) and six HA processing strategies with various noise reduction, directionality, and compression settings. Using virtual acoustics, a realistic speech-in-noise environment was simulated. The stimuli were generated using a HA simulator and presented to 49 habitual HA users who had previously been profiled. The four auditory profiles differed clearly in terms of their mean aided speech reception thresholds, thereby implying different needs in terms of signal-to-noise ratio improvement. However, no clear interactions with the tested HA processing strategies were found. Overall, these findings suggest that the auditory profiles can capture some of the individual differences in HA processing needs and that further research is required to identify suitable HA solutions for them.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Percepção da Fala , Limiar Auditivo , Audição , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Ruído/efeitos adversos , Fala
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