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1.
Otol Neurotol ; 45(5): e406-e410, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38728556

RESUMO

OBJECTIVE: To describe the rare process of osteolytic labyrinthitis, previously referred to as labyrinthine sequestrum, which involves progressive obliteration of the bony and membranous labyrinth with eventual supplantation with soft tissue and, in some cases, bony sequestrum. PATIENTS: Three patients with diverse presentations of osteolytic labyrinthitis from two tertiary care academic medical centers. INTERVENTIONS: Case series report analyzing the relevant clinical, radiologic, pathologic, and surgical data on our patients with osteolytic labyrinthitis and comparing these index cases to the existing literature. MAIN OUTCOME MEASURES: We describe the varying image findings seen in osteolytic labyrinthitis on computed tomography and magnetic resonance imaging. Also, we report successful surgical intervention and hearing rehabilitation with cochlear implantation in patients with osteolytic labyrinthitis. RESULTS: Our three patients presented with profound sudden sensorineural hearing loss and vertigo consistent with labyrinthitis. None of the three patients had a history of chronic otitis media. Imaging workup revealed varying degrees of erosion to the otic capsule bone demonstrating the spectrum of disease seen in osteolytic labyrinthitis. Although two cases showed osteolytic changes to the semicircular canals and vestibule, the first case revealed frank bony sequestrum within the obliterated labyrinth. The three cases were taken for surgical debridement and cochlear implantation. CONCLUSIONS: We propose the new term, osteolytic labyrinthitis-previously referred to as labyrinthine sequestrum-to describe the rare spectrum of disease characterized by destruction of the osseous and membranous labyrinth and potential supplantation with bony sequestrum. Cochlear implantation is a viable option in selected patients with osteolytic labyrinthitis.


Assuntos
Implante Coclear , Labirintite , Humanos , Implante Coclear/métodos , Labirintite/cirurgia , Labirintite/complicações , Labirintite/diagnóstico por imagem , Masculino , Feminino , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/etiologia , Adulto , Resultado do Tratamento , Osteólise/diagnóstico por imagem , Osteólise/cirurgia , Osteólise/complicações , Idoso , Vertigem/cirurgia , Vertigem/etiologia , Vertigem/diagnóstico por imagem
2.
PLoS One ; 19(5): e0302447, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38713651

RESUMO

OBJECTIVE: Vascular disease like small-vessel disease (SVD) is the most likely cause among the potential causes of Sudden sensorineural hearing loss (SSNHL). Understanding the relationship between SVD and SSNHL is crucial for developing effective prevention and treatment strategies. To confirm the relationship between SVD and SSNHL, the effect of SVD is confirmed by focusing on the duration and recurrence of SSNHL. METHODS: This article reports a retrospective observational study that investigated the relationship between SVD and SSNHL using the South Korea Health Insurance Review and Assessment Service (HIRA) database from 2010 to 2020. This retrospective observational study included 319,569 SSNHL patients between 2010 and 2020. RESULTS: Participant demographics were controlled using Propensity Score Matching. The hazard ratios (HR) for the effect of SVD on the duration of SSNHL were 1.045 for the group with SVD before the onset of SSNHL and 1.234 for the group with SVD after the onset of SSNHL. SVD was statistically significant for the recurrence of SSNHL, with an odds ratio of 1.312 in the group with SVD compared to the group without SVD. The HR for the period until a recurrence in the group with SVD was 1.062. CONCLUSIONS: The study identified SVD as a possible cause of SSNHL and found that the duration of SSNHL increased only in the presence of SVD. SVD also affected the recurrence of SSNHL, with the recurrence rate being 1.312 times higher in the group with SVD.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Masculino , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/complicações , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , República da Coreia/epidemiologia , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/epidemiologia , Adulto , Idoso , Fatores de Risco , Recidiva , Adulto Jovem , Pontuação de Propensão
4.
BMJ Case Rep ; 17(4)2024 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-38663901

RESUMO

Heimler Syndrome 2 (HS-2) is a rare, autosomal recessive mild form of a peroxisomal biogenesis disorder. Though knowledge regarding the disorder is limited, emerging research has found that sensorineural hearing loss, occasional or late onset pigmentation, amelogenesis imperfecta and nail abnormalities are clinical characteristics representative of HS-2.A school-aged male presented to the dental department with a chief complaint of a lack of enamel on multiple teeth. The patient's medical history was significant for patent ductus arteriosus, bilateral sensorineural hearing loss and biallelic mutation of the PEX6 gene. The clinical exam revealed dental crowding, hypoplasia, hypo-calcification of multiple teeth and enlarged pulp chambers of maxillary molars. This case report details the clinical findings associated with HS-2, the comprehensive dental treatment to be rendered to the patient, and critical information to paediatric dentists and general dentists so that they can make proper referrals to medical specialties.


Assuntos
Perda Auditiva Neurossensorial , Humanos , Masculino , Criança , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia
5.
J Pak Med Assoc ; 74(3): 476-479, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38591281

RESUMO

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Meningite , Criança , Humanos , Masculino , Feminino , Implantes Cocleares/efeitos adversos , Implante Coclear/efeitos adversos , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/etiologia , Estudos Transversais , Perda Auditiva/epidemiologia , Perda Auditiva/complicações , Surdez/epidemiologia , Surdez/cirurgia , Meningite/complicações , Demografia
6.
Acta otorrinolaringol. esp ; 75(2): 83-93, Mar-Abr. 2024. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-231380

RESUMO

Introducción: La hipoacusia neurosensorial (HNS) congénita o de inicio precoz es una de las enfermedades hereditarias más frecuentes en nuestro medio y es la deficiencia sensorial más frecuente. Es importante realizar un estudio etiológico de la hipoacusia y el estudio genético mediante la secuenciación de nueva generación (NGS) es la prueba con mayor rendimiento diagnóstico. Nuestro estudio muestra los resultados genéticos obtenidos en una serie de pacientes con HNS congénita/de inicio precoz bilateral. Material y método: Se incluyeron 105 niños diagnosticados de HNS bilateral a los que se les realizó un estudio genético entre los años 2019 y 2022. El estudio genético consistió en una secuenciación masiva del exoma completo, filtrando el análisis para los genes incluidos en un panel virtual de hipoacusia con 244 genes. Resultados: Se obtuvo un diagnóstico genético en 48% (50/105) de los pacientes. Se detectaron variantes patogénicas y probablemente patogénicas en 26 genes diferentes, siendo los genes más frecuentemente afectados el gen GJB2, USH2A y STRC. De las variantes detectadas 52% (26/50) se asociaron a una hipoacusia no sindrómica, 40% (20/50) una hipoacusia sindrómica y 8% restante (4/50) se podían asociar tanto a una hipoacusia sindrómica como no sindrómica. Conclusiones: El estudio genético constituye una parte fundamental del diagnóstico etiológico de la HNS bilateral. Nuestra serie muestra que el estudio genético de la hipoacusia mediante NGS tiene un alto rendimiento diagnóstico y nos proporciona información de gran utilidad en la práctica clínica.(AU)


Introduction: Congenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. Materials and methods: We included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). Results: 48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. Conclusions: Genetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.(AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/etiologia , Diagnóstico Pré-Implantação , Otolaringologia , Sequenciamento de Nucleotídeos em Larga Escala
7.
Br J Haematol ; 204(5): 2016-2024, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38500389

RESUMO

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.


Assuntos
Talassemia beta , Humanos , Talassemia beta/complicações , Talassemia beta/terapia , Masculino , Feminino , Adulto , Estudos de Casos e Controles , Pessoa de Meia-Idade , Itália/epidemiologia , Adulto Jovem , Terapia por Quelação , Perda Auditiva/epidemiologia , Perda Auditiva/etiologia , Adolescente , Audiometria de Tons Puros , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Prevalência
8.
Vestn Otorinolaringol ; 89(1): 21-27, 2024.
Artigo em Russo | MEDLINE | ID: mdl-38506021

RESUMO

Chronic sensorineural hearing loss (SNHL) is a common disease that leads to disability of the population. Despite the many reports devoted to SNHL, the question of the pathogenesis of the disease is still open. Many researchers consider the development of SNHL as a manifestation of microangiopathy. The mechanism of development of microangiopathy in SNHL is multifactorial, but most researchers agree that endothelial dysfunction (ED) triggers a complex of pathological changes in the vessels of the inner ear. OBJECTIVE: Review of the results of scientific research in recent years on the problem of etiopathogenesis of sensorineural hearing loss from the perspective of endothelial dysfunction in the formation of auditory disorders.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Humanos , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/epidemiologia
9.
Int J Mol Sci ; 25(5)2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38473883

RESUMO

Congenital cytomegalovirus (CMV) infection is the main cause of non-hereditary sensorineural hearing loss (SNHL). In order to shed light on SNHL pathophysiology, we examined the auditory pathway in CMV-infected fetuses; the temporal lobe, in particular the auditory cortex, and the inner ear. We investigated both inner ears and temporal lobes of 20 human CMV-infected fetuses at 21 weeks of gestation. As a negative group, five fetuses from spontaneous miscarriages without CMV infection were studied. Inner ears and temporal lobes were histologically examined, immunohistochemistry for CMV and CMV-PCR were performed. On the auditory cortex, we evaluated the local microglial reaction to the infection. CMV-positive cells were found in 14/20 brains and the damage was classified as severe, moderate, or mild, according to histological features. Fetuses with severe brain damage had a statistically higher temporal lobe viral load and a higher number of activated microglial cells in the auditory cortex compared to fetuses with mild brain damage (p: 0.01; p: 0.01). In the inner ears, the marginal cells of the stria vascularis were the most CMV positive. In our study, CMV affected the auditory pathway, suggesting a tropism for this route. In addition, in the auditory cortex, microglial activation may favor further tissue damage contributing to hearing loss.


Assuntos
Infecções por Citomegalovirus , Perda Auditiva Neurossensorial , Humanos , Citomegalovirus , Vias Auditivas/patologia , Perda Auditiva Neurossensorial/etiologia , Feto/patologia
10.
HNO ; 72(4): 291-302, 2024 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-38351342

RESUMO

Idiopathic sudden sensorineural hearing loss (ISSNHL) is one of the most common diseases in otolaryngology. Its etiology remains unknown. Furthermore, there is only a low level of evidence for the efficacy of established treatment modalities. In addition to systemic glucocorticoids, intratympanic corticosteroid treatment (ICT) has become increasingly important for treatment of ISSNHL. Different application strategies and treatment regimens have been described; however, uniform standards do not yet exist. ICT may be used for primary treatment as well as salvage therapy. Current data from meta-analyses show no benefit of intratympanic versus systemic primary therapy for sudden hearing loss (moderate evidence) but suggest a benefit of intratympanic secondary treatment over no treatment or placebo (high effect size, low evidence). Regarding combination of systemic and local glucocorticoid therapy in primary treatment of hearing loss, there may be a small benefit over systemic treatment alone (low effect size, low evidence).


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Glucocorticoides , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/tratamento farmacológico , Dexametasona/uso terapêutico , Resultado do Tratamento , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/etiologia , Corticosteroides/uso terapêutico , Injeção Intratimpânica , Audiometria de Tons Puros/efeitos adversos
11.
Trop Doct ; 54(2): 189-190, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38321775

RESUMO

Typhoid fever, also known as enteric fever, is a multisystemic infection primarily caused by Salmonella enterica serotype Typhi, and less commonly by Salmonella enterica serotypes Paratyphi A, B, and C. The classic presentation includes fever, malaise, diffuse abdominal pain, and constipation. If left untreated, typhoid fever can progress to delirium, obtundation, intestinal haemorrhage, bowel perforation, and death within a month of onset. However, the clinical course can deviate from the classic stepladder fever pattern, which now occurs in as few as 12% of cases.1 In this report, we describe an atypical presentation as sensorineural hearing loss in an otherwise healthy young male.


Assuntos
Perda Auditiva Neurossensorial , Perfuração Intestinal , Febre Tifoide , Humanos , Masculino , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Salmonella paratyphi A , Salmonella typhi , Febre Tifoide/complicações , Febre Tifoide/diagnóstico , Febre Tifoide/tratamento farmacológico , Adulto Jovem
12.
J Pediatr ; 268: 113934, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38309519

RESUMO

OBJECTIVE: The objective of this study was to determine if valganciclovir initiated after 1 month of age improves congenital cytomegalovirus-associated sensorineural hearing loss. STUDY DESIGN: We conducted a randomized, double-blind, placebo-controlled phase 2 trial of 6 weeks of oral valganciclovir at US (n = 12) and UK (n = 9) sites. Patients of ages 1 month through 3 years with baseline sensorineural hearing loss were enrolled. The primary outcome was change in total ear hearing between baseline and study month 6. Secondary outcome measures included change in best ear hearing and reduction in cytomegalovirus viral load in blood, saliva, and urine. RESULTS: Of 54 participants enrolled, 35 were documented to have congenital cytomegalovirus infection and were randomized (active group: 17; placebo group: 18). Mean age at enrollment was 17.8 ± 15.8 months (valganciclovir) vs 19.5 ± 13.1 months (placebo). Twenty (76.9%) of the 26 ears from subjects in the active treatment group did not have worsening of hearing, compared with 27 (96.4%) of 28 ears from subjects in the placebo group (P = .09). All other comparisons of total ear or best ear hearing outcomes were also not statistically significant. Saliva and urine viral loads decreased significantly in the valganciclovir group but did not correlate with change in hearing outcome. CONCLUSIONS: In this randomized controlled trial, initiation of antiviral therapy beyond the first month of age did not improve hearing outcomes in children with congenital cytomegalovirus-associated sensorineural hearing loss. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT01649869.


Assuntos
Antivirais , Infecções por Citomegalovirus , Ganciclovir , Perda Auditiva Neurossensorial , Valganciclovir , Humanos , Infecções por Citomegalovirus/tratamento farmacológico , Infecções por Citomegalovirus/congênito , Infecções por Citomegalovirus/complicações , Valganciclovir/uso terapêutico , Valganciclovir/administração & dosagem , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/virologia , Perda Auditiva Neurossensorial/etiologia , Antivirais/uso terapêutico , Antivirais/administração & dosagem , Masculino , Feminino , Método Duplo-Cego , Lactente , Administração Oral , Ganciclovir/análogos & derivados , Ganciclovir/uso terapêutico , Ganciclovir/administração & dosagem , Pré-Escolar , Resultado do Tratamento , Carga Viral , Recém-Nascido
13.
Eur Arch Otorhinolaryngol ; 281(6): 3265-3268, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38409582

RESUMO

BACKGROUND: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a maternally inherited mitochondrial disease that affects various systems in the body, particularly the brain, nervous system, and muscles. Among these systems, sensorineural hearing loss is a common additional symptom. METHODS: A 42-year-old female patient with MELAS who experienced bilateral profound deafness and underwent bilateral sequential cochlear implantation (CIs). Speech recognition and subjective outcomes were evaluated. RESULTS: Following the first CI follow-up, the patient exhibited improved speech recognition ability and decided to undergo the implantation of the second ear just two months after the initial CI surgery. The second CI also demonstrated enhanced speech recognition ability. Subjective outcomes were satisfactory for bilateral CIs. CONCLUSIONS: MELAS patients receiving bilateral CIs can attain satisfactory post-CI speech recognition, spatial hearing, and sound qualities.


Assuntos
Implante Coclear , Implantes Cocleares , Síndrome MELAS , Humanos , Feminino , Adulto , Síndrome MELAS/complicações , Implante Coclear/métodos , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/etiologia , Percepção da Fala
14.
World Neurosurg ; 185: 91-94, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38340797

RESUMO

Nervus intermedius (NI) arises from the superior salivary nucleus, solitary nucleus, and trigeminal tract. It leaves the pons as 1 to 5 roots and travels between the facial and vestibulocochlear nerves before merging with the facial nerve within the internal auditory canal. The mastoid segment of the facial nerve then gives rise to a sensory branch that supplies the posteroinferior wall of the external auditory meatus and inferior pina. This complex pathway renders the nerve susceptible to various pathologies, leading to NI neuralgia. Here, the authors present an unusual intraoperative finding of an atrophic NI in a patient with refractory NI neuralgia and a history of ipsilateral sudden-onset central facial palsy and microvascular decompression for trigeminal neuralgia. The patient underwent NI sectioning via the previous retrosigmoid window and achieved partial ear pain improvement. The gross size of the NI is compared with a cadaveric specimen through stepwise dissection. This case highlights the potential significance of subtle central ischemic events and subsequent atrophy of NI in the pathogenesis of NI neuralgia, as well as the ongoing need to investigate the therapeutic efficacy of nerve sectioning.


Assuntos
Paralisia Facial , Perda Auditiva Neurossensorial , Humanos , Paralisia Facial/cirurgia , Paralisia Facial/etiologia , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/etiologia , Nervo Facial/cirurgia , Cadáver , Neuralgia do Trigêmeo/cirurgia , Neuralgia do Trigêmeo/diagnóstico por imagem , Neuralgia do Trigêmeo/etiologia , Atrofia , Cirurgia de Descompressão Microvascular/métodos , Masculino , Pessoa de Meia-Idade , Feminino
15.
Sci Rep ; 14(1): 2910, 2024 02 05.
Artigo em Inglês | MEDLINE | ID: mdl-38316838

RESUMO

Whether cervical spondylosis (CS) is a risk factor for sudden sensorineural hearing loss (SSNHL) remains unclear. This study used national population-based data to investigate the risk of SSNHL in patients with CS in Taiwan of different ages and sexes. This study used data covering 2 million people in Taiwan, which were obtained from the National Health Insurance Research Database. The data that support the findings of this study are available from National Health Insurance Research Database but restrictions apply to the availability of these data, which were used under license for the current study, and so are not publicly available. Data are however available from the corresponding authors upon reasonable request and with permission of National Health Insurance Research Database. This retrospective cohort study enrolled 91,587 patients with a newly diagnosed CS between January 2000 and December 2018. Case and control cohorts were matched 1:1 according to age, sex, and comorbidities. SSNHL incidence rate and risk were compared between the groups. Cox regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). The mean follow-up period was 8.80 (SD = 4.12) and 8.24 (SD = 4.09) years in the CS and control cohorts, respectively. The incidence rate of SSNHL in the CS cohort (85.28 per 100 000 person-years) was 1.49-fold significantly higher than that in the non-CS cohort (57.13 per 100,000 person-years) (95% CI 1.32-1.68, P < .001). After age, sex, and selected comorbidities were adjusted for, CS exhibited an independent risk factor for SSNHL (adjusted HR = 1.52; 95% CI 1.34-1.71, P < .001). An age-stratified analysis in this study demonstrated a strong and highly significant association between CS and SSNHL in patients aged < 35 years (IRR = 2.28, 95% CI 1.18-4.39, P = .013). This large-scale Taiwanese-population-based retrospective study found that CS was associated with an increased risk of SSNHL. Acute hearing loss in patients with CS, particularly at a young age, should be carefully evaluated, and prompt treatment for SSNHL should be initiated.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Estudos Retrospectivos , Comorbidade , Fatores de Risco , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/complicações
16.
Nat Rev Nephrol ; 20(5): 295-312, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38287134

RESUMO

Hearing loss affects nearly 1.6 billion people and is the third-leading cause of disability worldwide. Chronic kidney disease (CKD) is also a common condition that is associated with adverse clinical outcomes and high health-care costs. From a developmental perspective, the structures responsible for hearing have a common morphogenetic origin with the kidney, and genetic abnormalities that cause familial forms of hearing loss can also lead to kidney disease. On a cellular level, normal kidney and cochlea function both depend on cilial activities at the apical surface, and kidney tubular cells and sensory epithelial cells of the inner ear use similar transport mechanisms to modify luminal fluid. The two organs also share the same collagen IV basement membrane network. Thus, strong developmental and physiological links exist between hearing and kidney function. These theoretical considerations are supported by epidemiological data demonstrating that CKD is associated with a graded and independent excess risk of sensorineural hearing loss. In addition to developmental and physiological links between kidney and cochlear function, hearing loss in patients with CKD may be driven by specific medications or treatments, including haemodialysis. The associations between these two common conditions are not commonly appreciated, yet have important implications for research and clinical practice.


Assuntos
Insuficiência Renal Crônica , Humanos , Insuficiência Renal Crônica/fisiopatologia , Insuficiência Renal Crônica/complicações , Perda Auditiva/etiologia , Perda Auditiva/fisiopatologia , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia
17.
J Chin Med Assoc ; 87(3): 328-333, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38289277

RESUMO

BACKGROUND: Idiopathic sudden sensorineural hearing loss (ISSNHL) is an emergency that causes permanent hearing loss if timely treatment is not provided. However, the evidence supporting the effect of intratympanic steroid injection (ITSI) starting time on hearing outcome is limited. METHODS: We retrospectively enrolled 582 patients with ISSNHL who were treated with ITSIs and reviewed their clinical and audiological variables. The relationship between ITSI starting time and hearing recovery was analyzed. RESULTS: The mean starting time of ITSI was 13.17 ± 16.53 days. The overall hearing recovery rate was 55.15% (recovery = mean hearing level gain of ≥10 dB). The recovery rates were 79.2%, 67.4%, 50%, 36.6%, and 17.8% for the ITSI starting times of 1 to 3, 4 to 7, 8 to 14, 15 to 28, and ≥29 days, respectively. A multivariate analysis revealed that ITST starting time (odds ratio [OR] = 0.94, 95% CI, 0.92-0.96, p < 0.001) and salvage therapy (OR = 0.55, 95% CI, 0.35-0.86, p = 0.009) were independent poor prognostic factors for patients with ISSNHL. CONCLUSION: Earlier ITSI treatment is associated with a higher hearing recovery rate. Comorbidities and post-ITSI complications were nonsignificant independent risk factors.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Glucocorticoides/uso terapêutico , Dexametasona , Estudos Retrospectivos , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/tratamento farmacológico , Resultado do Tratamento , Esteroides/uso terapêutico
18.
Ann Otol Rhinol Laryngol ; 133(4): 390-399, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38197255

RESUMO

OBJECTIVE: To investigate the role and distribution of various molecular markers using immunohistochemistry and immunofluorescence to further elucidate and understand the pathogenesis of otosclerosis. METHODS: Archival celloidin formalin-fixed 20-micron thick histologic sections from 7 patients diagnosed with otosclerosis were studied and compared to controls. Sections in the mid-modiolar region were immunoreacted with rabbit polyclonal antibodies against nidogen-1, ß2-laminin, collagen-IX, BSP, and monoclonal antibodies against TGF ß-1 and ubiquitin. Digital images were acquired using a high-resolution light and laser confocal microscope. RESULTS: Nidogen-1, BSP, and collagen-IX were expressed in the otospongiotic regions, and to lesser extent, in the otosclerotic regions, the latter previously believed to be inactive. ß2-laminin and ubiquitin were uniformly expressed in both otospongiotic and otosclerotic regions. There was a basal level of expression of all of these markers in the normal hearing and sensorineural hearing loss specimens utilized as control. TGF ß -1, however, though present in the otosclerosis bones, was absent in the normal hearing and sensorineural hearing loss controls. CONCLUSIONS: Our results propose that the activity and function of TGF-1 may play a key role in the development and pathogenesis of otosclerosis. Further studies utilizing a higher number of temporal bone specimens will be helpful for future analysis and to help decipher its role as a potential target in therapeutic interventions.


Assuntos
Perda Auditiva Neurossensorial , Otosclerose , Humanos , Coelhos , Animais , Otosclerose/patologia , Cóclea/patologia , Perda Auditiva Neurossensorial/etiologia , Colágeno , Laminina/metabolismo , Ubiquitinas/metabolismo
19.
Pract Radiat Oncol ; 14(3): 212-215, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38211694

RESUMO

The cochlear apparatus is one of the major organs at risk when considering radiation therapy (RT) for brain, head, and neck tumors. Radiation oncologists currently consider mean dose constraints of <35 Gy for conventionally fractioned radiation therapy (RT), <4 Gy for single fraction stereotactic radiosurgery, and <17.1 or 25 Gy for 3- or 5-fraction stereotactic radiosurgery, respectively, as the standard of care. Indeed, dose adjustments are made in the setting of concurrent platinum-based chemotherapy or when prioritizing tumor coverage during treatment planning. Despite guidelines, in many patients, RT to the cochlea may still cause sensorineural hearing loss through progressive degeneration and ossification of the inner ear. There are several audiologic and otolaryngologic interventions for incident RT-induced hearing loss, including hearing aids, cochlear implants, or, in the context of vestibular schwannoma due to neurofibromatosis type 2, auditory brain stem implantation. Cochlear implants are the most effective at restoring hearing and improving quality of life for those with an intact cochlear nerve. An early multidisciplinary approach is essential to optimally manage RT-induced hearing loss, and this topic discussion serves as a guide for radiation oncologists on cochlear dosimetric considerations as well as how to address potential RT-induced adverse effects.


Assuntos
Perda Auditiva Neurossensorial , Humanos , Perda Auditiva Neurossensorial/etiologia , Lesões por Radiação/etiologia , Lesões por Radiação/terapia , Radioterapia/efeitos adversos , Radioterapia/métodos
20.
Ann Otol Rhinol Laryngol ; 133(4): 411-417, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38186353

RESUMO

OBJECTIVES: To investigate the role of normal weight central obesity (NWCO) in the prognosis of sudden sensorineural hearing loss (SSNHL). METHODS: We retrospectively investigated 807 cases of SSNHL from January of 2008 to August of 2019 from the Department of Otorhinolaryngology at Kaohsiung Medical University Hospital in southern Taiwan. We analyzed the association between overweight and obesity, NWCO, and the prognosis of SSNHL. The demographic and clinical characteristics, audiometry results, and outcomes were also reviewed. RESULTS: The nonobese (body mass index [BMI] < 24 kg/m2) and overweight and obese groups (BMI ≥ 24 kg/m2) comprised 343 (42.50%) and 464 (57.50%) patients, respectively. The favorable prognosis rates in the nonobese and the overweight and obese groups were 45.48% and 45.91%, respectively, without a significant difference (P = .9048). Multivariate logistic regression revealed that BMI (adjusted odds ratio [aOR] = 1.00, 95% CI = 0.948-1.062, P = .9165) was not significantly associated with SSNHL recovery. The normal weight noncentral obesity (NWNCO) and NWCO groups comprised 266 (77.55%) and 77 (22.45%) patients, respectively, and had favorable prognosis rates of 48.50% and 35.06%, respectively. The difference between the groups was significant (P = .0371). Multivariate logistic regression analysis revealed that NWCO (aOR = 2.51, 95% CI = 1.292-5.019, P = .0075) was significantly associated with SSNHL recovery. CONCLUSIONS: NWCO may significantly affect the prognosis of SSNHL.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Humanos , Obesidade Abdominal/complicações , Obesidade Abdominal/epidemiologia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Sobrepeso , Obesidade/complicações , Obesidade/epidemiologia , Perda Auditiva Súbita/diagnóstico , Perda Auditiva Súbita/etiologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia
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