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1.
Medicine (Baltimore) ; 99(10): e19419, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32150093

RESUMO

Although early detection and intervention may improve the outcome of the congenital cytomegalovirus (cCMV) infection, few studies assessed the real-world clinical practice for cCMV patients. We analyzed medical claims data to assess the patterns of diagnoses and medical care for cCMV patients.We used a subset of medical claims database (JMDC Claims Database) in Japan, covering 207,547 newborns between April 2010 and March 2017 and observed for at least 6 months. The diagnosis of cCMV and related symptoms and sequelae and medical care, including essential examinations and antiviral treatment, were identified using standardized codes.Overall, we identified 53 (25.5 per 100,000 newborns) cCMV patients diagnosed within 6 months after birth; of these, 83% were diagnosed within 1 month and 68% had at least 1 cCMV-related symptom at birth. Objective hearing tests and fundus examinations were performed within 6 months in 60% and 30% of patients, respectively. Antivirals were prescribed in 26% of patients. During the observation period (median = 33 months), sensorineural hearing loss (49%) and developmental problems (28%) were commonly identified as cCMV-related sequelae. The proportions of the patients continuously followed up with objective hearing tests up to 36 months were 30% in total and 56% in antiviral-treated patients, respectively.The cCMV patients did not necessarily receive a timely diagnosis nor continuous follow-ups in usual clinical practice. Although the universal screening for cCMV may, if implemented, facilitate early diagnosis, it should be accompanied by strategic follow-up plans to support timely interventions.


Assuntos
Infecções por Citomegalovirus/diagnóstico , Triagem Neonatal , Antivirais/uso terapêutico , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/congênito , Infecções por Citomegalovirus/tratamento farmacológico , Bases de Dados Factuais , Diagnóstico Precoce , Feminino , Perda Auditiva Neurossensorial/complicações , Humanos , Recém-Nascido , Revisão da Utilização de Seguros/estatística & dados numéricos , Japão , Masculino
2.
BMC Med Genet ; 21(1): 35, 2020 02 17.
Artigo em Inglês | MEDLINE | ID: mdl-32066420

RESUMO

BACKGROUND: We report a large family with four successive generations, presenting with a complex phenotype of severe congenital neutropenia (SCN), partially penetrant monocytosis, and hearing loss of varying severity. METHODS: We performed whole exome sequencing to identify the causative variants. Sanger sequencing was used to perform segregation analyses on remaining family members. RESULTS: We identified and classified a pathogenic GFI1 variant and a likely pathogenic variant in MYO6 which together explain the complex phenotypes seen in this family. CONCLUSIONS: We present a case illustrating the benefits of a broad screening approach that allows identification of oligogenic determinants of complex human phenotypes which may have been missed if the screening was limited to a targeted gene panel with the assumption of a syndromic disorder. This is important for correct genetic diagnosis of families and disentangling the range and severity of phenotypes associated with high impact variants.


Assuntos
Síndrome Congênita de Insuficiência da Medula Óssea/genética , Proteínas de Ligação a DNA/genética , Perda Auditiva Neurossensorial/genética , Cadeias Pesadas de Miosina/genética , Neutropenia/congênito , Fatores de Transcrição/genética , Adulto , Idoso , Síndrome Congênita de Insuficiência da Medula Óssea/complicações , Síndrome Congênita de Insuficiência da Medula Óssea/diagnóstico , Síndrome Congênita de Insuficiência da Medula Óssea/fisiopatologia , Exoma/genética , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/diagnóstico , Doenças Genéticas Inatas/genética , Doenças Genéticas Inatas/fisiopatologia , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Mutação/genética , Neutropenia/complicações , Neutropenia/diagnóstico , Neutropenia/genética , Neutropenia/fisiopatologia , Linhagem , Fenótipo , Sequenciamento Completo do Exoma
3.
PLoS One ; 15(2): e0228498, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32017804

RESUMO

OBJECTIVES: Tinnitus is a common symptom among patients with hearing loss, and many studies have reported successful tinnitus suppression with hearing devices. Active middle ear implantation of the Vibrant Soundbridge (VSB) is a good alternative to existing hearing devices. This study evaluated the effects of VSB implantation on tinnitus and sought to identify the main audiological factor that affects tinnitus suppression. METHODS: The study participants were 16 adults who had tinnitus with sensorineural hearing loss, and who underwent VSB implantations. Pure-tone audiometry; word recognition test; tinnitus handicap inventory (THI); and visual analog scale (VAS) assessment of loudness, awareness, and annoyance were performed before and 12 months after surgery. Changes in hearing threshold, word recognition scores (WRS), THI scores, and VAS scores were analyzed. RESULTS: VAS scores for loudness (mean difference: 1.9, 95% CI: 0.6, 3.1), awareness (mean difference: 1.6, 95% CI: 0.4, 2.8), and annoyance (mean difference: 1.7, 95% CI: 0.7, 2.8) showed significant improvements from baseline to 12 months after surgery. In addition, THI scores showed a significant decrease (mean difference: 13.8, 95% CI: 2.9, 24.9). The average hearing threshold level, WRS, and most comfortable level (MCL) also showed significant improvements at 12 months after surgery (mean difference: 17.3, 95% CI: 13.3, 21.3; mean difference: -7.6, 95% CI: -15.1, -0.1; mean difference: 26.3, 95% CI: 22.9, 29.6, respectively). Among the aforementioned factors, changes in MCL were best correlated with those in THI scores (mean difference: 2.55, 95% CI: 0.90, 4.21). CONCLUSION: A VSB implant is beneficial to subjects with tinnitus accompanied by sensorineural hearing loss. The changes in THI scores best correlated with those in MCL. This improvement may represent a masking effect that contributes to tinnitus suppression in patients with VSB implants.


Assuntos
Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/cirurgia , Zumbido/diagnóstico , Idoso , Audiometria de Tons Puros , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prótese Ossicular , Índice de Gravidade de Doença , Zumbido/etiologia , Resultado do Tratamento , Escala Visual Analógica
4.
Artigo em Chinês | MEDLINE | ID: mdl-32086912

RESUMO

Objective:The aim of this study was to explore the clinical features, hearing prognosis and differential diagnosis of recurrent low frequency sensorineural hearing loss (RLFD) . Method:The clinical characteristics, clinical manifestations, audiological examination and auxiliary examination of RLFD patients were retrospectively analyzed. We summarized clinical features, draw the pure tone audiometry curves, and analyze the diagnosis of RLFD. Result:Forty-seven patients (53 ears) with RLFD had a hearing review time of 1-124 months. The course of disease ranged from 3 to 320 months with an average course of 29 months. ①The incidence of tinnitus in the accompanying symptoms was 93.6%(44 cases), and the ear suffocation was 83.0%(39 cases), 38.3% (18 cases) of the patients developed vestibular symptoms during the course of the disease. ②During the observation period, 27 cases(57.4%) were diagnosed with related diseases: 7 cases(14.9%) Meniere's disease, 6 cases(12.8%) vestibular migraine, 2 cases(4.3%) with Meniere's disease and migraine, and 1 case(2.1%) with idiopathic intracranial hypotension 11 cases(23.4%) were possible cochlear migraine; ③Migraine-related RLFD had a younger onset age, more common in women; ④83.0%(44 ears)of the affected ears had stable or improved low-frequency hearing during the observation period, 17.0%(9 ears)of the affected ears experienced low-frequency hearing; ⑤18.9%(10 ears)of the affected ears had high-frequency hearing loss; ⑥RLFD had 6 types of audiological outcomes: low-frequency improvement combined with high-frequency stability; low-frequency stability combined with high-frequency stability; low frequency progress combined with high frequency stability type; low frequency improvement combined with high frequency progress type; low frequency stability combined with high frequency progress type; low frequency progress combined with high frequency progress type; ⑦Rising type hearing curve low frequency hearing prognosis is good, mountain type and descending low frequency hearing prognosis is poor. Conclusion:Tinnitus and ear stuffiness are the early symptoms and the most disturbing symptoms in patients with RLFD. The mechanism of Migraine may play an important role in the pathogenesis of RLFD. The rare causes such as intracranial hypotension syndrome should not be ignored. Most of the patients with RLFD had stable or improved hearing after long-term fluctuation, but some patients with low or high frequency hearing might decline. The type of initial hearing curve was a prognostic factor. Long-term hearing follow-up is helpful for prognosis evaluation.


Assuntos
Perda Auditiva Neurossensorial/diagnóstico , Audiometria de Tons Puros , Diagnóstico Diferencial , Feminino , Perda Auditiva Neurossensorial/complicações , Humanos , Masculino , Doença de Meniere/complicações , Transtornos de Enxaqueca/complicações , Prognóstico , Estudos Retrospectivos , Zumbido/complicações
5.
Int J Pediatr Otorhinolaryngol ; 131: 109862, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31927148

RESUMO

OBJECTIVES: To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). METHODS: An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss. RESULTS: Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA. CONCLUSION: Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.


Assuntos
Perda Auditiva Neurossensorial/complicações , Perda Auditiva/congênito , Torcicolo/complicações , Aqueduto Vestibular/anormalidades , Estudos de Casos e Controles , Conexina 26 , Conexinas/genética , Feminino , Perda Auditiva/complicações , Humanos , Lactente , Masculino , Mutação , Estudos Retrospectivos , Vertigem/etiologia
6.
Int J Pediatr Otorhinolaryngol ; 131: 109842, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31927149

RESUMO

OBJECTIVES: Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current literature reports that Stickler syndrome is the most common syndrome associated with PRS, and 22q11 deletion syndrome (22q11 DS) as the second most common. This study identifies associations between PRS and genetic syndromes. METHODS: A retrospective chart review was performed to identify patients diagnosed with PRS over a 10-year period from 4/1/2007 to 4/1/2017 at a tertiary children's hospital. RESULTS: 4,052 consecutive charts were reviewed and 234 patients had a diagnosis of PRS confirmed with the triad of micrognathia, glossoptosis, and airway obstruction. Of note, all of these patients had cleft palate. Of the 234 patients with PRS, 65 patients had syndromic diagnoses (28%). One patient had 22q11 DS (0.43%), and 31 patients had Stickler syndrome (13.2%). Additionally, 3 patients had central hypoventilation syndrome, 3 patients had Duane syndrome, 2 patients had Cornelia de Lange syndrome, 2 patients had Emanuel syndrome, 2 patients had Gordon syndrome, 2 patients had Mobius syndrome, 2 patients had Nager syndrome. Multiple other syndromes were identified, but occurred in isolated cases. CONCLUSION: This study supports literature that PRS is most commonly associated with Stickler Syndrome but rarely associated with 22q11 DS given that only 1 patient had both PRS and 22q11 DS.


Assuntos
Artrite/complicações , Doenças do Tecido Conjuntivo/complicações , Perda Auditiva Neurossensorial/complicações , Síndrome de Pierre Robin/complicações , Descolamento Retiniano/complicações , Síndrome da Deleção 22q11/complicações , Adolescente , Artrogripose/complicações , Criança , Pré-Escolar , Transtornos Cromossômicos/complicações , Fissura Palatina/complicações , Pé Torto Equinovaro/complicações , Síndrome de Cornélia de Lange/complicações , Síndrome da Retração Ocular/complicações , Feminino , Deformidades Congênitas da Mão/complicações , Cardiopatias Congênitas/complicações , Humanos , Hipoventilação/complicações , Hipoventilação/congênito , Lactente , Recém-Nascido , Deficiência Intelectual/complicações , Masculino , Disostose Mandibulofacial/complicações , Síndrome de Möbius/complicações , Hipotonia Muscular/complicações , Estudos Retrospectivos , Apneia do Sono Tipo Central/complicações
7.
BMC Infect Dis ; 19(1): 1046, 2019 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-31822287

RESUMO

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is the most common congenital infection and a leading cause of long-term neurological and sensory sequelae, the most common being sensorineural hearing loss (SNHL). Despite extensive research, clinical or laboratory markers to identify CMV infected children with increased risk for disease have not been identified. This study utilizes viral whole-genome next generation-sequencing (NGS) of specimens from congenitally infected infants to explore viral diversity and specific viral variants that may be associated with symptomatic infection and SNHL. METHODS: CMV DNA from urine specimens of 30 infants (17 asymptomatic, 13 symptomatic) was target enriched and next generation sequenced resulting in 93% coverage of the CMV genome allowing analysis of viral diversity. RESULTS: Variant frequency distribution was compared between children with symptomatic and asymptomatic cCMV and those with (n = 13) and without (n = 17) hearing loss. The CMV genes UL48A, UL88, US19 and US22 were found to have an increase in nucleotide diversity in symptomatic children; while UL57, UL20, UL104, US14, UL115, and UL35 had an increase in diversity in children with hearing loss. An analysis of single variant differences between symptomatic and asymptomatic children found UL55 to have the highest number, while the most variants associated with SNHL were in the RL11 gene family. In asymptomatic infants with SNHL, mutations were observed more frequently in UL33 and UL20. CONCLUSION: CMV genomes from infected newborns can be mapped to 93% of the genome at a depth allowing accurate and reproducible analysis of polymorphisms for variant and gene discovery that may be linked to symptomatic and hearing loss outcomes.


Assuntos
Infecções por Citomegalovirus/diagnóstico , Citomegalovirus/genética , DNA Viral/genética , Perda Auditiva Neurossensorial/diagnóstico , Criança , Citomegalovirus/classificação , Citomegalovirus/isolamento & purificação , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/virologia , DNA Viral/metabolismo , DNA Viral/urina , Feminino , Perda Auditiva Neurossensorial/complicações , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Recém-Nascido , Masculino , Mutação , Filogenia , Análise de Componente Principal
8.
BMC Neurol ; 19(1): 337, 2019 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-31864340

RESUMO

BACKGROUND: This study aims to investigate the clinical value of two kinds of vestibular-evoked myogenic potentials in patients with sudden sensorineural hearing loss (SSNHL). METHODS: A total of 82 patients were divided into two groups: vertigo group and non-vertigo group. All patients underwent examinations for pure tone hearing thresholds, middle ear analysis, the videonystagmography, caloric tests, and vestibular-evoked myogenic potentials elicited from the sternocleidomastoid and extraocular muscle. In addition, 30 healthy subjects were selected as the control group. RESULTS: For the 30 healthy subjects, the average latency of p13 and n23 of the cervical vestibular evoked myogenic potentials (cVEMPs) were 13.13 ± 2.89 ms and 23.51 ± 3.25 ms, respectively, and the bilateral amplitude asymmetry rate ranged within 0.05-0.31. The average latency of n10 of the ocular vestibular evoked myogenic potentials (oVEMPs) was 10.13 ± 0.48 ms. The average amplitude of the n10-p15-wave was 5.58 ± 0.65 µV. Among the 35 vertigo patients with SSNHL, 27 patients had normal cVEMP and oVEMP examination results, five patients had abnormal oVEMP examination results, and five patients had abnormal cVEMP examination results. The latency and amplifier of oVEMPs and cVEMPs were within the normal range in 47 SSNHL patients without vertigo. The chi-square value was 5.647, the P-value was equal to 0.017, and the difference was statistically significant at a confidence interval of 95%. CONCLUSIONS: OVEMPs and cVEMPs can be used evaluate the vestibular nerve function of SSNHL patients with vertigo.


Assuntos
Perda Auditiva Neurossensorial/diagnóstico , Testes Auditivos/métodos , Potenciais Evocados Miogênicos Vestibulares , Adulto , Feminino , Perda Auditiva Neurossensorial/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Vertigem/complicações
9.
Pediatr Ann ; 48(11): e441-e447, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31710363

RESUMO

Exocrine pancreatic insufficiency in children can lead to lifelong complications related to malnutrition and poor growth. The clinical presentation can be subtle in the early stages of insufficiency as the large functional capacity of the pancreas is gradually lost. The pediatrician plays a crucial role in the early identification of these children to ensure a timely referral so that a diagnosis can be made and therapy initiated. Early nutritional therapy allows for prevention and correction of deficiencies, which leads to improved outcomes and survival. When insufficiency is suspected, the workup should start with an indirect test of exocrine pancreatic function, such as fecal elastase, to establish the diagnosis. Once a diagnosis is established, further testing to delineate the etiology should be pursued, with cystic fibrosis being high on the differential list and assessed for with a sweat test. Assessment of anthropometry at every visit is key, as is monitoring of laboratory parameters and physical examination findings that are suggestive of malabsorption and malnutrition. The mainstay of management is administration of exogenous pancreatic enzymes to facilitate digestion and absorption. [Pediatr Ann. 2019;48(11):e441-e447.].


Assuntos
Transtornos da Nutrição Infantil/etiologia , Insuficiência Pancreática Exócrina/diagnóstico , Acil-CoA Desidrogenase de Cadeia Longa/deficiência , Anus Imperfurado/complicações , Criança , Transtornos da Nutrição Infantil/diagnóstico , Transtornos da Nutrição Infantil/terapia , Quimotripsina/metabolismo , Síndrome Congênita de Insuficiência da Medula Óssea/complicações , Fibrose Cística/complicações , Gorduras na Dieta/metabolismo , Displasia Ectodérmica/complicações , Terapia de Reposição de Enzimas , Insuficiência Pancreática Exócrina/etiologia , Insuficiência Pancreática Exócrina/terapia , Fezes/enzimologia , Transtornos do Crescimento/complicações , Perda Auditiva Neurossensorial/complicações , Humanos , Hipotireoidismo/complicações , Deficiência Intelectual/complicações , Erros Inatos do Metabolismo Lipídico/complicações , Doenças Mitocondriais/complicações , Doenças Musculares/complicações , Nariz/anormalidades , Avaliação Nutricional , Pâncreas/diagnóstico por imagem , Pâncreas/fisiologia , Pancreatopatias/complicações , Elastase Pancreática/metabolismo , Testes de Função Pancreática , Pancreatite Crônica/complicações , Pancreatite Crônica/etiologia , Síndrome de Shwachman-Diamond/complicações , Esteatorreia/etiologia , Tripsinogênio/sangue
10.
Subst Abuse Treat Prev Policy ; 14(1): 48, 2019 11 06.
Artigo em Inglês | MEDLINE | ID: mdl-31694678

RESUMO

BACKGROUND: Methadone is a long-acting opioid receptor agonist. Reported adverse effects of methadone include constipation, respiratory depression, dizziness, nausea, vomiting, itching, sweating, rhabdomyolysis, QT prolongation, and orthostatic hypotension. Hearing loss has been rarely reported following methadone use, and when reported, long term follow-up is rare. Herein we report a case of methadone poisoning with rhabdomyolysis, acute kidney injury, and persistent hearing loss documented by a 2 year follow up. CASE PRESENTATION: The patient was a 34 years old male who presented with a reduced level of consciousness and acute hearing loss after suicidal ingestion of 40 mg of methadone while experiencing family-related stresses. He had no prior history of methadone use, abuse, or addiction. Initial laboratory testing was significant for a serum creatinine concentration of 4.1 mg/dl, a mixed metabolic and respiratory acidosis, thrombocytopenia, abnormal hepatic transaminases, and coagulation tests. The patient then developed severe rhabdomyolysis. Also, audiometry showed a bilateral sensorineural hearing loss. The patient required hemodialysis for 11 days while his metabolic abnormalities gradually resolved. However, his hearing loss was persistent, as demonstrated by 2 years of follow up. CONCLUSION: Our patient simultaneously had kidney failure, rhabdomyolysis, and permanent hearing loss following methadone poisoning. Although rare, ototoxicity and permanent hearing loss may happen in cases of methadone poisoning. While opioid-induced hearing loss is uncommon, methadone toxicity should be taken into account for any previously healthy patient presenting with acute hearing loss with or without rhabdomyolysis.


Assuntos
Perda Auditiva Neurossensorial/induzido quimicamente , Metadona/envenenamento , Insuficiência Renal/induzido quimicamente , Rabdomiólise/induzido quimicamente , Adulto , Analgésicos Opioides/efeitos adversos , Perda Auditiva Neurossensorial/complicações , Humanos , Masculino , Insuficiência Renal/complicações , Rabdomiólise/complicações
11.
Elife ; 82019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31570117

RESUMO

Auditory deprivation in the form of deafness during development leads to lasting changes in central auditory system function. However, less is known about the effects of mild-to-moderate sensorineural hearing loss (MMHL) during development. Here, we used a longitudinal design to examine late auditory evoked responses and mismatch responses to nonspeech and speech sounds for children with MMHL. At Time 1, younger children with MMHL (8-12 years; n = 23) showed age-appropriate mismatch negativities (MMNs) to sounds, but older children (12-16 years; n = 23) did not. Six years later, we re-tested a subset of the younger (now older) children with MMHL (n = 13). Children who had shown significant MMNs at Time 1 showed MMNs that were reduced and, for nonspeech, absent at Time 2. Our findings demonstrate that even a mild-to-moderate hearing loss during early-to-mid childhood can lead to changes in the neural processing of sounds in late childhood/adolescence.


Assuntos
Percepção Auditiva , Encefalopatias/fisiopatologia , Perda Auditiva Neurossensorial/complicações , Adolescente , Criança , Desenvolvimento Infantil , Pré-Escolar , Potenciais Evocados Auditivos , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino
13.
Acta Otolaryngol ; 139(11): 1004-1007, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31486695

RESUMO

Background: Patients with end-stage renal disease (ESRD) have a higher risk of suffering sudden sensorineural hearing loss (SSNHL), but little is known about this population. Aims and objectives: To investigate the clinical characteristics, treatment and recovery of SSNHL patients with ESRD. Materials and methods: Records of 32 SSNHL patients with ESRD were reviewed, including clinical characteristics and hearing recovery. Patients were divided into intratympanic steroid (ITS) group and oral steroid (OS) group, and hearing recovery was compared between two groups. Results: Twenty-six patients (81.3%) exhibited tinnitus, and 18 patients (56.3%) suffered vertigo. Mean pure-tone threshold at the initial presentation was 73.2 ± 19.4 dB, and the audiogram configuration was ascending in 9.4%, descending in 9.4%, flat in 34.4% and profound in 46.9% cases. At 3-month follow-up, percentages of patients in complete recovery, partial recovery, slight recovery, and no improvement were 18.8%, 31.3%, 21.9% and 28.1%, respectively. Furthermore, the overall recovery rate, complete recovery rate and hearing improvement were significantly higher in the ITS group than those in the OS group. Conclusion: SSNHL patient with ESRD often suffered a severe hearing loss with a high rate of accompanying tinnitus and vertigo. ITS may provide better audiological results for SSNHL with ESRD than OS.


Assuntos
Perda Auditiva Neurossensorial/complicações , Perda Auditiva Súbita/complicações , Falência Renal Crônica/complicações , Adulto , Dexametasona/administração & dosagem , Feminino , Glucocorticoides/administração & dosagem , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Súbita/tratamento farmacológico , Humanos , Injeção Intratimpânica , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
14.
PLoS One ; 14(9): e0222041, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31490980

RESUMO

Tinnitus and hearing impairment are prevalent among headache patients. This study aims to investigate the risk of tinnitus, sensorineural hearing impairment, and sudden deafness in patients with non-migraine headache. Participants included 43 294 patients with non-migraine headache (non-migraine headache cohort) and 173 176 patients with no headache of any type (control cohort) frequency-matched with respect to 10-year age interval and sex from the Longitudinal Health Insurance Database 2005 of the Taiwan National Health Insurance Research Database. The mean age of the non-migraine headache cohort was 28.4 ± 14.9 years, and 58.5% of this cohort was male. The incidence rates of tinnitus, sensorineural hearing impairment, and sudden deafness were compared between cohorts using the Kaplan-Meier method with the log-rank test. A Cox proportional hazard model was used to examine the association of tinnitus, sensorineural hearing impairment, and sudden deafness with non-migraine headache, with adjustment for all covariates. The combined risk of either tinnitus, sensorineural hearing impairment, or sudden deafness was higher in the non-migraine headache cohort than in the control cohort (adjusted odds ratio [aHR], 2.73; 95% confidence interval [95% CI], 2.62-2.84; p < 0.0001). Subgroup analysis showed that patients in the non-migraine headache cohort were at significantly higher risk of developing tinnitus (aHR, 3.05; 95% CI, 2.91-3.19; p < 0.0001), sensorineural hearing impairment (aHR, 1.89; 95% CI, 1.74-2.05; p < 0.0001), and sudden deafness (aHR, 2.14; 95% CI, 1.77-2.59; p < 0.0001) than were controls. In this population-based study, the risks of tinnitus, sensorineural hearing impairment, and sudden deafness were found to be significantly higher in patients with non-migraine headache than in those without headache.


Assuntos
Cefaleia/complicações , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Súbita/complicações , Zumbido/complicações , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Estudos Retrospectivos , Risco
15.
Am J Audiol ; 28(3): 548-552, 2019 Sep 13.
Artigo em Inglês | MEDLINE | ID: mdl-31430172

RESUMO

Objective Current recommendations for cochlear hydrops treatment include systemic glucocorticoids and diuretics. Cochlear cells express dopamine receptors, although their role is unknown in the pathophysiology of cochlear hydrops. Case Description We report the case of remission of recurrent right-sided cochlear hydrops in a young male patient treated with bromocriptine due to pituitary macroprolactinoma. Transient improvement was observed after oral steroid and diuretic treatment, but cochlear hydrops recurred until the dose of bromocriptine was increased to 10 mg daily. Conclusion Bromocriptine may stimulate dopamine receptors in cochlear cells with potential therapeutic role in patients with cochlear hydrops. There are no widely accepted and effective treatments for endolymphatic hydrops, and identifying potential new and efficacious therapeutics is of high relevance.


Assuntos
Bromocriptina/uso terapêutico , Doenças Cocleares/tratamento farmacológico , Perda Auditiva Neurossensorial/tratamento farmacológico , Antagonistas de Hormônios/uso terapêutico , Neoplasias Hipofisárias/tratamento farmacológico , Prolactinoma/tratamento farmacológico , Adulto , Audiometria de Tons Puros , Doenças Cocleares/complicações , Diuréticos/uso terapêutico , Furosemida/uso terapêutico , Glucocorticoides/uso terapêutico , Perda Auditiva Neurossensorial/complicações , Humanos , Imagem por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/patologia , Prolactinoma/complicações , Prolactinoma/diagnóstico por imagem , Prolactinoma/patologia , Recidiva
16.
Acta Otolaryngol ; 139(10): 866-869, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31452421

RESUMO

Objective: This study aimed to explore clinical characteristics and prognosis of elderly cases with sudden sensorineural hearing loss. Methods: Fifty-five elderly cases with sudden sensorineural hearing loss and 55 younger cases with sudden sensorineural hearing loss were enrolled in the study. Recovery rates of hearing between elderly group and younger group were compared. In elderly group, the recovery rate of hearing in the patients with normal hearing of the contralateral ear was compared to those with hearing loss of the contralateral ear. Results: The overall recovery rate in the elderly group was 50.9%, significantly lower than 74.5% in the younger group (p < .01). Among the elderly group, the patients with normal hearing of the contralateral ear recovered better than those with hearing loss of the contralateral ear (recovery rates, 76.7% versus 20.0%). Among the elderly group, the recovery rate of cases with treatment onset ≤14 days was 64.7%, while the recovery rate of those with treatment onset longer than 14 days was only 28.6%, with significant difference (p < .01). Conclusion: Elderly cases with sudden sensorineural hearing loss had poor prognosis. Prognosis of elderly cases with sudden sensorineural hearing loss was associated with hearing of the contralateral ear and treatment onset.


Assuntos
Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Súbita/complicações , Perda Auditiva Súbita/diagnóstico , Adolescente , Adulto , Fatores Etários , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
17.
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
18.
Int J Pediatr Otorhinolaryngol ; 127: 109650, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31466025

RESUMO

BACKGROUND: Several studies have demonstrated that children with sensorineural hearing loss (SNHL) exhibit postural instabilities, as well as balance and gait disorders, due to the vestibular dysfunction that they are prone to display as a consequence of inner ear injury. Thus, some experiments have proposed vestibular rehabilitation exercises programs as a treatment to improve these motor skills in children with SNHL. OBJECTIVE: Assess the evidence quality of the trials that used vestibular rehabilitation exercises programs to improve the postural control, balance and gait of children with SNHL. METHODS: This is a systematic review that surveyed articles in nine databases, published up to July 4, 2019, in any language, using the following inclusion criteria: (1) Randomized or quasi-randomized controlled trials. (2) Participants of both groups with clinical diagnosis of SNHL, aged up to 12 years old, with no physical problems, cognitive or neurological impairments, except the vestibular dysfunction. (3) Using vestibular rehabilitation exercises programs to improve the following outcomes: postural control, balance and/or gait. RESULTS: Six experiments, including 153 children, met the inclusion criteria of this systematic review. Two randomized controlled trials (45 children) on the postural control exhibited low evidence quality and four others; three randomized and controlled trials (90 children) on the balance and one quasi-randomized (18 children) on the gait demonstrated very low evidence quality, respectively. CONCLUSION: There is promising evidence that vestibular rehabilitation exercises programs improve the postural control, balance and gait of children with SNHL. However, due to the methodological limitations of the trials and low quality of current evidence on this topic, the trials results analyzed by this systematic review should be interpreted with caution. Due to the low quality of evidence observed in this review, we suggest that new trials be proposed on this topic, with better methodological quality, to prove the effectiveness of vestibular rehabilitation exercises programs to improve the postural control, balance and gait of children with SNHL.


Assuntos
Terapia por Exercício , Marcha , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/reabilitação , Equilíbrio Postural , Doenças Vestibulares/reabilitação , Criança , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Doenças Vestibulares/etiologia
19.
Neuroradiol J ; 32(6): 420-425, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31337256

RESUMO

Cryopyrin-associated periodic syndrome is a hereditary inflammatory disorder encompassing a wide spectrum of clinical phenotypes. This disorder has been associated with mutation of the NLRP3 gene coding for cryopyrin, which leads to overproduction of interleukin-1. The condition causes relapsing bouts of inflammation involving multiple organ systems, including the central nervous system, and, if untreated, can lead to long-term debilitating effects. A literature review revealed only a few brief descriptions of magnetic resonance imaging findings in cryopyrin-associated periodic syndrome patients. We describe serial magnetic resonance imaging findings in an 11-year-old female with clinically diagnosed cryopyrin-associated periodic syndrome who presented with intermittent headaches, progressive sensorineural hearing loss, fevers, and abdominal pain. The magnetic resonance imaging showed progressively worsening low T2 signal in the cochlea, cochlear enhancement, and leptomeningeal enhancement. We also describe some previously unreported findings in this syndrome, including cranial nerve and cauda equina enhancement.


Assuntos
Cauda Equina/diagnóstico por imagem , Sistema Nervoso Central/diagnóstico por imagem , Cóclea/diagnóstico por imagem , Nervos Cranianos/diagnóstico por imagem , Síndromes Periódicas Associadas à Criopirina/diagnóstico por imagem , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Criança , Síndromes Periódicas Associadas à Criopirina/complicações , Progressão da Doença , Feminino , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Meninges/diagnóstico por imagem , Medula Espinal/diagnóstico por imagem
20.
Curr Opin Ophthalmol ; 30(5): 306-313, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31313752

RESUMO

PURPOSE OF REVIEW: The literature regarding prophylactic treatment of rhegmatogenous retinal detachment in Stickler syndrome remains controversial. We review major published clinical studies and offer a critical analysis of this subject. SUMMARY: Stickler syndrome is a systemic collagenopathy affecting multiple organ systems including the eye, ear, and skeleton. Stickler syndrome is probably the most common cause of genetically determined pediatric rhegmatogenous retinal detachment. Congenital developmental anomalies constitute over half rhegmatogenous detachments (RRD) in patients less than 10 years. The majority are caused by hereditary vitreoretinopathies associated with Stickler syndrome. Sixty percent of patients with Stickler syndrome develop RRD's over their lifetime with possible severe visual loss and subsequent lifelong morbidity. In view of these complications, some have emphasized the importance of prophylactic laser treatment to the retina of patients with Stickler syndrome to reduce the occurrence of and/or prevent future rhegmatogenous retinal detachment, but there appears to be insufficient data to support the absolute benefit of such prophylactic treatment. Guidelines regarding the age at prophylactic treatment as well as type and frequency of intervention are scarce and would benefit from additional clinical investigations.


Assuntos
Artrite/complicações , Doenças do Tecido Conjuntivo/complicações , Perda Auditiva Neurossensorial/complicações , Descolamento Retiniano/prevenção & controle , Criança , Feminino , Humanos , Masculino , Descolamento Retiniano/complicações , Descolamento Retiniano/etiologia
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