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2.
Cochlear Implants Int ; 22(6): 353-357, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34151741

RESUMO

INTRODUCTION: Understanding the clinical manifestations of the coronavirus disease (COVID-19) has become an urgent objective in the research community. Amongst the emerging neurologic complications is sensorineural hearing loss. While several cases of hearing loss amongst COVID-19 patients have been reported, the management of these patients has yet to be discussed and determined. Herein we present cochlear implant outcomes for a patient who suffered from unilateral sensorineural hearing loss after a prolonged hospital course for COVID-19. METHODS: A patient who was hospitalized for COVID-19 for one month and subsequently developed unilateral profound sensorineural hearing loss underwent cochlear implantation for single-sided deafness. His COVID-19 hospital course was reviewed in detail. Speech perception and audiometric assessments were used to evaluate cochlear implant outcomes. RESULTS: After cochlear implantation, the patient demonstrated improved speech perception on the implanted side and decreased tinnitus within 1 month of activation. CONCLUSIONS: Cochlear implantation may be an appropriate intervention for patients who suffer from severe sensorineural hearing loss following infection with COVID-19.


Assuntos
COVID-19 , Implante Coclear , Surdez , Perda Auditiva Unilateral , Surdez/etiologia , Surdez/cirurgia , Perda Auditiva Unilateral/etiologia , Perda Auditiva Unilateral/cirurgia , Hospitalização , Humanos , SARS-CoV-2
3.
World Neurosurg ; 151: e94-e99, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33819711

RESUMO

OBJECTIVE: Microvascular decompression (MVD) is the most effective treatment for hemifacial spasm (HFS). However, surgical difficulties due to complex anatomy or revision surgery can endanger the functional integrity of the brainstem. We describe surgically challenging cases and provide operative guidance that may be helpful for neurosurgeons who perform MVDs. METHODS: Of 3028 patients with HFS who underwent MVDs consecutively by a single neurosurgeon, complex or unusual cases associated with surgical difficulty were selected. Medical charts and images were reviewed, with the primary focus being intraoperative findings, operative techniques, and clinical outcomes. All MVDs were performed using the interposition method. RESULTS: Surgically difficult cases were categorized into six types: tandem, perforator, atypical location, encircling, revision, and penetrating types. During the follow-up period (11.5-42.7 months; median 24.9 months), the spasm-free rate was 88.4%. Intraoperative changes in brainstem auditory evoked potentials were observed in 31.5% of patients. Immediate postoperative facial palsy and deafness were observed in 6.0% and 1.5% of patients, respectively. Revision surgery showed the highest surgical morbidity among the unusual HFS types. Detailed illustrations and descriptions of MVD in patients with surgically challenging HFS are provided. CONCLUSIONS: Complex or unusual HFS types carry higher surgical risks in MVD. Neurosurgeons performing MVDs need to be prepared to manage complex HFS cases in order to achieve favorable clinical outcomes.


Assuntos
Espasmo Hemifacial/diagnóstico por imagem , Cirurgia de Descompressão Microvascular/métodos , Adulto , Idoso , Competência Clínica , Surdez/epidemiologia , Surdez/etiologia , Potenciais Evocados Auditivos do Tronco Encefálico , Paralisia Facial/epidemiologia , Paralisia Facial/etiologia , Seguimentos , Humanos , Monitorização Neurofisiológica Intraoperatória , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Resultado do Tratamento
4.
Complement Ther Med ; 56: 102581, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33197677

RESUMO

INTRODUCTION: Neurofibromatosis type 2 (NF2) is a rare, progressive and incurable genetic disorder associated with progressive hearing loss and eventual deafness. As a group, patients with NF report high levels of stress and depressive symptoms. However, no studies have explored improvement in these symptoms after psychosocial interventions. We have previously shown that a mind-body program tailored to adults with NF2 who are deaf (the Relaxation Response and Resiliency Program for Deaf NF2, d3RP-NF2) improves quality of life and resiliency over and above a Health Enhancement program when both are delivered via live-video and assisted by Communication Access Realtime Translation (CART). Here we tested the effects of the programs on depression and perceived stress. METHODS: Forty-five patients with NF2 and significant hearing loss were randomized to the d3RP-NF2 or Health-Enhancement program and completed measures of depression (PHQ-9) and perceived stress (PSS-10) at baseline, post-intervention, and six-month follow-up. RESULTS: Patients randomized to the d3RP-NF2 program, but not to the control condition, experienced significant decreases on both measures from baseline to post-test, which were maintained at follow-up (within group tests). However, improvements following the d3RP-NF2 program was not significantly higher than those observed in the control group (between group tests). CONCLUSION: Results provide the first evidence of improvement in symptoms of depression and perceived stress among deaf patients living with NF2 who participate in a virtual mind-body program.


Assuntos
Surdez , Depressão , Terapias Mente-Corpo/métodos , Neurofibromatose 2 , Estresse Psicológico , Adulto , Surdez/etiologia , Surdez/psicologia , Surdez/terapia , Atenção à Saúde , Depressão/etiologia , Depressão/psicologia , Depressão/terapia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neurofibromatose 2/complicações , Neurofibromatose 2/psicologia , Qualidade de Vida , Estresse Psicológico/etiologia , Estresse Psicológico/psicologia , Estresse Psicológico/terapia , Realidade Virtual
5.
Otol Neurotol ; 42(3): 408-413, 2021 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33351564

RESUMO

OBJECTIVE: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure. STUDY DESIGN: Retrospective chart review and systematic review of the literature using PubMed and Embase. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Four hundred ninety-eight devices in 439 distinct adult patients. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Implant failure rate and etiology. RESULTS: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9-77.1 vs mean 47.6 yr, CI: 39.9-55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful.In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%). CONCLUSIONS: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Adulto , Criança , Surdez/epidemiologia , Surdez/etiologia , Surdez/cirurgia , Humanos , Falha de Prótese , Reoperação , Estudos Retrospectivos
6.
Niger Postgrad Med J ; 27(4): 371-376, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33154292

RESUMO

Background: Children with cerebral palsy (CP) suffer from multiple problems and potential disabilities. These range from musculoskeletal problems, mental retardation, epilepsy, ophthalmologic and hearing impairment among others. Consequences of hearing loss include problems with speech and language development. Early detection in this difficult-to-test population may prevent these consequences of hearing loss. An otoacoustic emission assessment is useful in this regard. This study assessed transient-evoked otoacoustic emissions (TEOAEs) in children with CP. Materials and Methods: The study population were children with CP who presented at the paediatric neurology clinic during the study period. An equal number of control population matched for age and sex were also recruited using simple random sampling. An interviewer-administered questionnaire was used to obtain relevant clinical information. All participants selected underwent a detailed ear, nose and throat examination and TEOAE testing. Results: There were 330 participants in this study, categorised into CP cases (165) and non-CP controls (165). The age range of the participants was 1-12 years, with a mean age of 4.44 ± 2.92 among CP patients and 4.47 ± 2.90 among the controls. The male-to-female ratio was 2:1. TEOAEs were 'failed' in 83.6% of the CP patients and in 28.5% of the controls. This study found a statistically significant difference in 'failed' TEOAE result between the CP patients and the controls (P = 0.0001). Conclusion: This study found a high prevalence of 'failed' TEOAEs in children with CP in Kano.


Assuntos
Paralisia Cerebral , Surdez , Paralisia Cerebral/complicações , Paralisia Cerebral/diagnóstico , Paralisia Cerebral/epidemiologia , Criança , Pré-Escolar , Surdez/etiologia , Feminino , Humanos , Lactente , Masculino , Nigéria/epidemiologia , Emissões Otoacústicas Espontâneas
7.
PLoS One ; 15(10): e0240832, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33119665

RESUMO

Hypnosis is a powerful tool to affect the processing and perception of stimuli. Here, we investigated the effects of hypnosis on the processing of auditory stimuli, the time course of event-related-potentials (ERP; N1 and P3b amplitudes) and the activity of cortical sources of the P3b component. Forty-eight participants completed an auditory oddball paradigm composed of standard, distractor, and target stimuli during a hypnosis (HYP), a simulation of hypnosis (SIM), a distraction (DIS), and a control (CON) condition. During HYP, participants were suggested that an earplug would obstruct the perception of tones and during SIM they should pretend being hypnotized and obstructed to hear the tones. During DIS, participants' attention was withdrawn from the tones by focusing participants' attention onto a film. In each condition, subjects were asked to press a key whenever a target stimulus was presented. Behavioral data show that target hit rates and response time became significantly reduced during HYP and SIM and loudness ratings of tones were only reduced during HYP. Distraction from stimuli by the film was less effective in reducing target hit rate and tone loudness. Although, the N1 amplitude was not affected by the experimental conditions, the P3b amplitude was significantly reduced in HYP and SIM compared to CON and DIS. In addition, source localization results indicate that only a small number of neural sources organize the differences of tone processing between the control condition and the distraction, hypnosis, and simulation of hypnosis conditions. These sources belong to brain areas that control the focus of attention, the discrimination of auditory stimuli, and the organization of behavioral responses to targets. Our data confirm that deafness suggestions significantly change auditory processing and perception but complete deafness is hard to achieve during HYP. Therefore, the term 'deafness' may be misleading and should better be replaced by 'hypoacusis'.


Assuntos
Encéfalo/diagnóstico por imagem , Cognição/fisiologia , Surdez/fisiopatologia , Hipnose/métodos , Estimulação Acústica , Adolescente , Adulto , Atenção/fisiologia , Percepção Auditiva , Comportamento/fisiologia , Encéfalo/fisiologia , Encéfalo/fisiopatologia , Surdez/diagnóstico por imagem , Surdez/etiologia , Eletroencefalografia/métodos , Potenciais Evocados/fisiologia , Potenciais Evocados Auditivos/fisiologia , Feminino , Audição/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Percepção da Fala/fisiologia , Adulto Jovem
8.
Eur Ann Otorhinolaryngol Head Neck Dis ; 137 Suppl 1: S11-S18, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32863156

RESUMO

OBJECTIVE: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016. PATIENTS AND METHODS: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared. RESULTS: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling. CONCLUSION: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling.


Assuntos
Percepção Auditiva , Linguagem Infantil , Implante Coclear/estatística & dados numéricos , Implantes Cocleares/estatística & dados numéricos , Surdez/reabilitação , Sistema de Registros/estatística & dados numéricos , Fatores Etários , Implante Coclear/efeitos adversos , Implante Coclear/métodos , Implantes Cocleares/efeitos adversos , Comunicação , Correção de Deficiência Auditiva/instrumentação , Correção de Deficiência Auditiva/estatística & dados numéricos , Surdez/etiologia , Remoção de Dispositivo/estatística & dados numéricos , Educação de Pessoas com Deficiência Auditiva/métodos , Educação de Pessoas com Deficiência Auditiva/estatística & dados numéricos , Seguimentos , França , Humanos , Lactente , Recém-Nascido , Inclusão Escolar/estatística & dados numéricos , Reoperação/estatística & dados numéricos , Instituições Acadêmicas , Inteligibilidade da Fala , Fonoterapia/estatística & dados numéricos , Fatores de Tempo
9.
Eur Ann Otorhinolaryngol Head Neck Dis ; 137 Suppl 1: S51-S56, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32739279

RESUMO

OBJECTIVES: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France. MATERIALS AND METHODS: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations. RESULTS: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02). CONCLUSION: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise.


Assuntos
Implante Coclear/métodos , Implantes Cocleares/estatística & dados numéricos , Surdez/reabilitação , Sistema de Registros/estatística & dados numéricos , Adulto , Audiometria da Fala/métodos , Percepção Auditiva , Criança , Implante Coclear/efeitos adversos , Implante Coclear/estatística & dados numéricos , Surdez/etiologia , Feminino , França , Humanos , Masculino , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Testes de Discriminação da Fala/estatística & dados numéricos
12.
J Laryngol Otol ; 134(6): 509-518, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32508296

RESUMO

OBJECTIVE: To determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management. METHODS: A retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears. RESULTS: Out of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent). CONCLUSION: Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.


Assuntos
Cóclea/patologia , Surdez/etiologia , Orelha Interna/anormalidades , Doenças do Labirinto/congênito , Adolescente , Criança , Pré-Escolar , Cóclea/anormalidades , Cóclea/inervação , Nervo Coclear/anormalidades , Nervo Coclear/fisiopatologia , Constrição Patológica/patologia , Surdez/diagnóstico , Orelha Interna/diagnóstico por imagem , Orelha Interna/patologia , Feminino , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Humanos , Lactente , Doenças do Labirinto/epidemiologia , Imageamento por Ressonância Magnética/métodos , Masculino , Prevalência , Prognóstico , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Doenças do Nervo Vestibulococlear/congênito , Doenças do Nervo Vestibulococlear/epidemiologia
13.
Int J Pediatr Otorhinolaryngol ; 134: 110043, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32305661

RESUMO

INTRODUCTION: Congenital sensorineural hearing loss is a heterogeneous disorder; its etiological profile varies between populations. Pathogenic variants of GJB2 gene are the major cause of non-syndromic hearing loss. Congenital cytomegalovirus infection (cCMV) is the most important prenatal etiological factor causing hearing loss and other disorders. Perinatal events, syndromes, postnatal infections or traumas are less common. Causes of the remaining one third of hearing loss cases are unknown. OBJECTIVES: To determine the etiological profile of hearing loss in pediatric cochlear implant users in Lithuanian population. METHODS: The data of 122 children (70 male/52 female; aged 7.6 ± 3.3 years) cochlear implant users were analysed. Medical records of all children recruited in Santaros Clinics (Vilnius, Lithuania) were analysed to identify prenatal, perinatal, or postnatal risk factors based on the adapted list proposed by the Joint Committee of Infant Hearing. Genetic counselling and testing according to the scheme were performed to 101 children. DNA of 117 children was extracted from the DBS on Guthrie cards and CMV DNA detected using real time PCR. RESULTS: Non-syndromic hearing loss was diagnosed in 65 cases (53.3%), 58 of which were GJB2 gene-associated; syndromic hearing loss was diagnosed to 8 children (6.6%). Perinatal (prematurity, low birth weight, hypoxia, hyperbilirubinemia, sepsis, ototoxicity, and meningitis) and postnatal (meningitis) risk factors were associated with hearing loss in 16 (13.1%) and 4 (3.3%) study participants respectively. CMV DNA was detected in 12 samples (9.8%). The cause of hearing loss remained unknown only for 17 (13.9%) children. CONCLUSIONS: The major cause of HL in the current study was GJB2 gene alterations. Only 14% of the cohort had congenital hearing loss of unknown origin.


Assuntos
Implante Coclear , Conexinas/genética , Infecções por Citomegalovirus/complicações , Surdez/etiologia , Perda Auditiva Neurossensorial/etiologia , Adolescente , Criança , Pré-Escolar , Implantes Cocleares , Estudos de Coortes , Infecções por Citomegalovirus/congênito , Surdez/genética , Surdez/reabilitação , Feminino , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/reabilitação , Humanos , Hiperbilirrubinemia Neonatal/complicações , Hipóxia/complicações , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido Prematuro , Lituânia , Masculino , Meningite/complicações , Sepse Neonatal/complicações
14.
Ont Health Technol Assess Ser ; 20(4): 1-85, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32190165

RESUMO

Background: Neurofibromatosis 2 (NF2) is a rare genetic disorder that causes vestibular schwannomas to develop in both eighth cranial nerves. Almost all people with NF2 eventually become completely deaf as a result of progressive tumour enlargement or following surgical or radiotherapy treatment. Other rare abnormal conditions in the inner ears can also cause complete deafness. For people with either indication who are not candidates for cochlear implantation, auditory brainstem implantation is the only treatment option to restore some functional hearing. We conducted a health technology assessment of auditory brainstem implantation for adults with NF2 and severe inner ear abnormalities, which included an evaluation of effectiveness, safety, cost-effectiveness, the budget impact of publicly funding auditory brainstem implantation, and patient preferences and values. Methods: We performed a systematic literature search of the clinical evidence. We assessed the risk of bias of each included study using the Risk of Bias in Non-randomized Studies-of Interventions (ROBINS-I) tool and the quality of the body of evidence according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) Working Group criteria. We performed a systematic economic literature search. We did not conduct a primary economic evaluation because the outcomes identified in our clinical evidence review were difficult to translate into measures appropriate for health economic modelling. We also analyzed the net budget impact of publicly funding auditory brainstem implantation over the next 5 years in Ontario, including the device, presurgical assessment, surgical procedure, and postsurgical rehabilitation. To contextualize the potential value of auditory brainstem implants, we spoke with six people with lived experience of NF2 and severe inner ear abnormalities. Results: We included 22 publications (16 in NF2, five in severe inner ear abnormalities, and one in complications of auditory brainstem implantation) in the clinical evidence review. In adults with NF2, auditory brainstem implantation when compared with no intervention allows any degree of improvement in sound recognition (GRADE: High), allows any degree of improvement in speech perception when used in conjunction with lip-reading (GRADE: High), and provides subjective benefits of hearing (GRADE: High). It likely allows any degree of improvement in speech perception when using the implant alone (GRADE: Moderate) and may improve quality of life (GRADE: Low). In adults with severe inner ear abnormalities, auditory brainstem implantation when compared with no intervention likely allows any degree of improvement in sound recognition (GRADE: Moderate) and in any speech perception when using the implant alone (GRADE: Moderate). It may allow any degree of improvement in speech perception when used in conjunction with lip-reading (GRADE: Low), provide subjective benefits of hearing (GRADE: Low), and improve quality of life (GRADE: Low).We did not identify any economic studies on auditory brainstem implantation for adults with NF2 or adults with deafness due to severe inner ear abnormalities. We estimated that the annual net budget impact of publicly funding auditory brainstem implantation in Ontario over the next 5 years would range from about $130,000 in year 1 for two procedures to about $260,000 in year 5 for four procedures.People with whom we spoke who had received an auditory brainstem implant reported that it restored some hearing ability and improved their quality of life, though they also reported ongoing challenges in using the device or side effects from the procedure. Conclusions: When compared with no intervention, auditory brainstem implantation provides some benefit for completely deaf adults with NF2 or severe inner ear abnormalities who are not candidates for cochlear implantation. Based on evidence of moderate to high quality, auditory brainstem implants allow any degree of improvement in sound recognition and in speech perception when used in conjunction with lip-reading for people with NF2. The quality of evidence on these outcomes was low to moderate for people with severe inner ear abnormalities. These functional outcomes lead to subjective benefits of hearing which are consistently reported in the literature and in interviews with patients. We were unable to determine the cost-effectiveness of this treatment. We estimate that publicly funding auditory brainstem implantation in Ontario would result in additional costs of about $130,000 to $260,000 annually over the next 5 years.


Assuntos
Implante Auditivo de Tronco Encefálico/métodos , Surdez/cirurgia , Orelha Interna/anormalidades , Neurofibromatose 2/complicações , Avaliação da Tecnologia Biomédica/métodos , Adulto , Surdez/etiologia , Orelha Interna/cirurgia , Humanos , Neurofibromatose 2/cirurgia , Ontário , Resultado do Tratamento
15.
Ann Otol Rhinol Laryngol ; 129(8): 833-837, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32019320

RESUMO

OBJECTIVES: Too little is known about hearing loss rehabilitation in patients with Alström syndrome (AS). Benefits of hearing aids (HA) have not been fully documented and only one case treated with a Cochlear Implant (CI) has been described in the proceedings of a conference. Furthermore, comorbidities and risk of complications following surgical intervention may contraindicate Cochlear Implant procedures in these patients.The present case report concerns the first AS patient with CI in the literature. METHODS: After reporting a concise description of the audiological profile of patients with AS described in the literature, the case of a 22-year-old woman with genetically confirmed Alström syndrome who underwent a sequential bilateral CI (Bi-CI) rehabilitation is reported. Audiological results before and after cochlear implantation are described. RESULTS: The patient showed an excellent functional outcome with CIs, which enabled her to achieve communicative, social and academic results comparable with her peers, and no complications occurred. CONCLUSIONS: AS is not necessarily an absolute contraindication to CI. For many AS patients, a good cognitive function and adequate life expectancy represent a clear indication to prompt and adequate hearing rehabilitation with CIs. The description of this type of clinical cases could in the future also generate indications for a tailored audiological treatment of patients with very specific needs, such as patients with Alström Syndrome.


Assuntos
Síndrome de Alstrom/complicações , Implantes Cocleares , Surdez/cirurgia , Percepção da Fala/fisiologia , Audiometria , Surdez/etiologia , Surdez/fisiopatologia , Feminino , Humanos , Adulto Jovem
16.
Codas ; 32(1): e20180278, 2020.
Artigo em Português, Inglês | MEDLINE | ID: mdl-32049152

RESUMO

PURPOSE: To compare the frequency of risk indicators in preterm and full-term babies; to analyze the possible relationships among the presence of risk for hearing loss with language acquisition and socioeconomic, demographic and obstetric variables. METHODS: This is a longitudinal cohort study, with a sample of 87 babies. Gestational, obstetric and sociodemographic data were collected from mothers and babies. The socioeconomic classification status of the families were classified using the Brazilian Criteria for Economic Classification. The risk for language was assessed using the Language Acquisition Enunciation Signs and the Denver II test. The data were analyzed using the STATISTICA 9.1 software, using the chi-square and the Mann-Whitney U tests and simple and multiple linear regression models. RESULTS: Permanence in a neonatal intensive care (65.52%), ototoxic (48.28%), mechanical ventilation (39.66%) and hyperbilirubinemia (46.55%) were the more frequent risk indicators in the sample. Regarding socioeconomic, demographic and obstetric factors, there was a correlation among prenatal care, gestational age, birth weight, feeding with hearing risk. Acquisition and development of language showed statistical significance with varicella, HIV, Apgar score and birth weight >1500 grams. CONCLUSION: Preterm babies showed higher frequency of risk indicators compared to full-term babies. Among environmental factors, prenatal care, which interferes in the outcome of gestational age, birth weight, Apgar score and presence of infectious diseases, as well as feeding, emerged as significant factors related to hearing and language acquisition. Prematurity was the relevant biological factor related to hearing and language risk.


Assuntos
Surdez/etiologia , Perda Auditiva/etiologia , Estudos de Coortes , Surdez/prevenção & controle , Feminino , Perda Auditiva/prevenção & controle , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Unidades de Terapia Intensiva Neonatal , Desenvolvimento da Linguagem , Estudos Longitudinais , Masculino , Nascimento Prematuro , Cuidado Pré-Natal , Fatores de Risco , Fatores Socioeconômicos
17.
Cell Mol Life Sci ; 77(4): 619-635, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31522250

RESUMO

Estrogen is the major female hormone involved in reproductive functions, but it also exerts a variety of additional roles in non-reproductive organs. In this review, we highlight the preclinical and clinical studies that have pointed out sex differences and estrogenic influence on audition. We also describe the experimental evidences supporting a protective role of estrogen towards acquired forms of hearing loss. Although a high level of endogenous estrogen is associated with a better hearing function, hormonal treatments at menopause have provided contradictory outcomes. The various factors that are likely to explain these discrepancies include the treatment regimen as well as the hormonal status and responsiveness of the patients. The complexity of estrogen signaling is being untangled and many downstream effectors of its genomic and non-genomic actions have been identified in other systems. Based on these advances and on the common physio-pathological events that underlie age-related, drug or noise-induced hearing loss, we discuss potential mechanisms for their protective actions in the cochlea.


Assuntos
Estrogênios/metabolismo , Audição , Animais , Cóclea/metabolismo , Cóclea/patologia , Surdez/etiologia , Surdez/metabolismo , Surdez/patologia , Feminino , Humanos , Masculino , Receptores de Estrogênio/metabolismo , Caracteres Sexuais , Fatores Sexuais , Transdução de Sinais
18.
Laryngoscope ; 130(6): E407-E415, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31643090

RESUMO

OBJECTIVES/HYPOTHESIS: To outline a possible decision-making process for sporadic vestibular schwannoma (VS) with contralateral nonserviceable hearing at diagnosis. STUDY DESIGN: Retrospective case series. METHODS: Diagnosed VS was studied in a tertiary referral center from 1995 to 2013. RESULTS: Twenty-eight patients were included, with a mean follow-up of 6.9 years (range = 0.5-20 years). Ten were stage 1, 10 were stage 2, five were stage 3, and three were stage 4. Ipsilateral hearing levels were A (n = 3), B (n = 10), C (n = 7) and D (n = 8) American Academy of Otolaryngology-Head and Neck Surgery classification. Contralateral hearing levels were C (n = 11) and D (n = 17). Initial VS management included surveillance (n = 12) or surgery (n = 16), and four patients were later treated with surgery (n = 3) or stereotactic irradiation (n = 1). Hearing was improved by hearing aids and/or etiological treatment (n = 8), restored by contralateral (n = 15) or ipsilateral (n = 4) cochlear implants (CIs), or ipsilateral auditory brainstem implants (ABIs) (n = 3). Finally, 18 CIs were active daily; 14 of them presented high or moderate benefit with mean open-set dissyllabic word scores (WRS) of 58.1% and sentence recognition scores (SRS) of 69.7%, but only one ABI was still active (WRS of 70% and SRS of 87% with lip reading). CONCLUSIONS: When early removal of VS was not necessary, contralateral CI or etiological treatment for hearing loss might be recommended initially. Ipsilateral CI is proposed, whereas VS should be operated on if previous hearing restoration was not successful. ABI should be reserved for the rare cases where a contralateral CI could not be implanted or the cochlear nerve was sectioned during VS removal. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:E407-E415, 2020.


Assuntos
Tomada de Decisão Clínica/métodos , Implante Coclear/estatística & dados numéricos , Surdez/terapia , Neuroma Acústico/terapia , Radiocirurgia/estatística & dados numéricos , Adolescente , Implantes Auditivos de Tronco Encefálico , Criança , Pré-Escolar , Implantes Cocleares , Surdez/etiologia , Surdez/fisiopatologia , Feminino , Audição/fisiologia , Humanos , Lactente , Masculino , Neuroma Acústico/complicações , Neuroma Acústico/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
Eur Arch Otorhinolaryngol ; 277(3): 695-704, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31776734

RESUMO

PURPOSE: To determine the 2-year outcome of health-related quality of life (HRQoL) in adults who received a cochlear implant (CI) for single-sided deafness (SSD). METHODS: Twenty adults (mean age at implantation: 47 ± 11 years) with SSD (PTA worse ear: 113 dB HL, PTA better ear: 14 dB HL) were administered the Nijmegen Cochlear Implant Questionnaire (NCIQ), and the Health Utility Index 3 (HUI 3). Questionnaire administration occurred before cochlear implantation and 3, 6, 12, and 24 months after implant activation. RESULTS: Of the 20 patients, 2 discontinued CI use within the observation period due to poor benefit. The NCIQ total score of the sample increased significantly over time (p = 0.003). The largest increase occurred within the first 3 months of CI use. Also, the HUI 3 multi-attribute utility score increased significantly (p = 0.03). The post-treatment increase of this score (+ 0.11 points) indicated that the gain in HRQoL was clinically relevant. Patients with a duration of deafness > 10 years had in all measures an equal HRQoL improvement than had patients with a duration of deafness < 10 years. CONCLUSION: Cochlear implantation led to significant improvement of hearing-specific and generic HRQoL in our patients. The improvement was seen after 3 or 6 months but did not increase further at later intervals. Patients with long-lasting SSD may be at higher risk of discontinuing CI use. However, if they adapt to the CI, they can experience an equal increase of HRQoL as patients with a short duration of SSD.


Assuntos
Implante Coclear , Surdez , Perda Auditiva Unilateral , Qualidade de Vida , Adulto , Implantes Cocleares , Surdez/etiologia , Surdez/cirurgia , Feminino , Pesquisas sobre Serviços de Saúde , Perda Auditiva Unilateral/etiologia , Perda Auditiva Unilateral/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Percepção da Fala , Fatores de Tempo , Resultado do Tratamento
20.
CoDAS ; 32(1): e20180278, 2020. tab
Artigo em Português | LILACS | ID: biblio-1055889

RESUMO

RESUMO Objetivo Comparar a frequência de indicadores de risco em bebês nascidos pré-termo e a termo; analisar as possíveis relações entre a presença de risco para perda auditiva com variáveis socioeconômicas, demográficas, obstétricas e risco à linguagem. Método Trata-se de um estudo de coorte longitudinal com amostra de 87 bebês. Foram coletados dados gestacionais, obstétricos e sociodemográficos das mães e dos bebês. A classificação socioeconômica das famílias foi analisada por meio do Critério de Classificação Econômica Brasil. O risco à linguagem foi avaliado por meio dos Sinais Enunciativos de Aquisição da Linguagem e Teste Denver II. Os dados foram analisados utilizando o programa STATISTICA 9.1, por meio dos Testes Quiquadrado e U de Mann-Whitney, Modelo de regressão linear simples e múltiplo. Resultados A permanência em UTI neonatal (65,52%), ototóxico (48,28%), ventilação mecânica (39,66%) e hiperbilirrubinemia (46,55%) foram os indicadores de risco mais frequentes na amostra. Considerando fatores socioeconômicos, demográficos e obstétricos, houve correlação entre pré-natal, idade gestacional, peso ao nascer e alimentação com o risco auditivo. A aquisição e desenvolvimento de linguagem mostrou significância estatística com a varicela, HIV, Apgar e peso >1500 gramas. Conclusão Os prematuros apresentaram maior frequência de indicadores de risco, comparados aos bebês a termo. Dos fatores ambientais, o pré-natal que interfere no desfecho da idade gestacional, peso ao nascer, apgar e presença de doenças infecciosas, além da alimentação, despontaram como significativos relacionados com o desenvolvimento da audição e a aquisição da linguagem. A prematuridade foi o fator biológico relevante relacionado ao risco auditivo e linguístico.


ABSTRACT Purpose To compare the frequency of risk indicators in preterm and full-term babies; to analyze the possible relationships among the presence of risk for hearing loss with language acquisition and socioeconomic, demographic and obstetric variables. Methods This is a longitudinal cohort study, with a sample of 87 babies. Gestational, obstetric and sociodemographic data were collected from mothers and babies. The socioeconomic classification status of the families were classified using the Brazilian Criteria for Economic Classification. The risk for language was assessed using the Language Acquisition Enunciation Signs and the Denver II test. The data were analyzed using the STATISTICA 9.1 software, using the chi-square and the Mann-Whitney U tests and simple and multiple linear regression models. Results Permanence in a neonatal intensive care (65.52%), ototoxic (48.28%), mechanical ventilation (39.66%) and hyperbilirubinemia (46.55%) were the more frequent risk indicators in the sample. Regarding socioeconomic, demographic and obstetric factors, there was a correlation among prenatal care, gestational age, birth weight, feeding with hearing risk. Acquisition and development of language showed statistical significance with varicella, HIV, Apgar score and birth weight >1500 grams. Conclusion Preterm babies showed higher frequency of risk indicators compared to full-term babies. Among environmental factors, prenatal care, which interferes in the outcome of gestational age, birth weight, Apgar score and presence of infectious diseases, as well as feeding, emerged as significant factors related to hearing and language acquisition. Prematurity was the relevant biological factor related to hearing and language risk.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Surdez/etiologia , Perda Auditiva/etiologia , Cuidado Pré-Natal , Fatores Socioeconômicos , Recém-Nascido Prematuro , Unidades de Terapia Intensiva Neonatal , Fatores de Risco , Estudos de Coortes , Estudos Longitudinais , Surdez/prevenção & controle , Nascimento Prematuro , Perda Auditiva/prevenção & controle , Desenvolvimento da Linguagem
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