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1.
Artigo em Chinês | MEDLINE | ID: mdl-34979615

RESUMO

Objective:To identify the deaf-causing mutation by the genetic analysis in a family with non-syndromic hereditary deafness. Methods:Medical history collection, hearing, vision, and genome whole-exome sequencing were performed on the members of the deaf family. Results:Two mutation sites were identified in the MYO7A gene, namely c.1183C>T and 1496T>C, of which c.1183C>T has a small number of foreign literature reports, and 1496T>C is a newly discovered mutation site. According to ACMG mutation guideline showed that these two mutations were pathogenic mutations of the proband. Sanger sequencing verified that c.1183C>T was derived from the father, and 1496T>C was derived from the mother. These two mutation sites were not found in the healthy population in the Exome Sequencing Project(ESP6500) database, 1000 Genomes Project database, and the Gnomad database. Moreover, the second child in this family included a heterozygous mutation of c.1183C>T and 1496T>C and was confirmed to become severe sensorineural deaf. Conclusion:A new pathogenic compound heterozygous mutation in the MYO7A gene has been discovered, which provides more diagnostic evidence for the autosomal recessive non-syndromic deafness caused by the MYO7A gene mutation and improves the prenatal gene diagnosis in high-risk families for mutation carriers to reduce congenital disabilities.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Criança , Surdez/genética , Perda Auditiva Neurossensorial/genética , Heterozigoto , Humanos , Mutação , Linhagem , Fenótipo
2.
Ann R Coll Surg Engl ; 104(1): e12-e13, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34972493

RESUMO

Round window rupture following barotrauma such as diving and air travel is well documented. However, we describe a case of round window rupture following a slap to the ear, which has not been described previously. A 12-year-old boy was slapped by his teacher in rural Nepal over his left ear and immediately noted hearing loss. There was no dizziness or tinnitus. He presented to our unit 21 days after the trauma and examination showed a perforation of the tympanic membrane in the anterior inferior quadrant with an otherwise normal ear examination and no nystagmus seen. Audiogram showed a left profound hearing loss, which was supported by auditory brainstem response test. Exploratory tympanotomy showed active perilymph leakage from the round window niche as demonstrated in the video attached. The round window was packed with cartilage and fascia to address the perilymph leakage. The patient was discharged the following day without any complications. This is an unusual cause for round window membrane rupture where the patient presented with hearing loss as his only symptom. Tympanotomy is recommended for patients with hearing loss following trauma with normal computed tomography imaging to exclude perilymph leak secondary to inner ear barotrauma.


Assuntos
Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/etiologia , Janela da Cóclea/lesões , Ferimentos não Penetrantes/complicações , Criança , Humanos , Masculino , Ventilação da Orelha Média , Ruptura/etiologia
3.
BMC Infect Dis ; 22(1): 16, 2022 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-34983409

RESUMO

BACKGROUND: Enterovirus has been described as a cause of aseptic meningitis in humorally immunosuppressed patients. CASE PRESENTATION: A 67-year-old female with a history of mantle cell lymphoma on rituximab therapy presented with subacute hepatitis, myalgias, and sensorineural hearing loss several months after an initial febrile illness. She was diagnosed with enterovirus infection by CSF PCR as a unifying etiology of her presentation, representing an unusual presentation of disease. DISCUSSION AND CONCLUSIONS: This patient's unique presentation and clinical course presents important implications in the care of similarly immunosuppressed patients with cryptic complaints.


Assuntos
Infecções por Enterovirus , Enterovirus , Perda Auditiva Neurossensorial , Meningite Asséptica , Meningite , Idoso , Infecções por Enterovirus/complicações , Infecções por Enterovirus/diagnóstico , Feminino , Humanos , Meningite Asséptica/diagnóstico
4.
Gene ; 808: 146000, 2022 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-34626719

RESUMO

Hearing loss is a common disease, of which genetic factors are the main cause. The incidence of mild or moderate postlingual deafness in children is not high, and the impact on life and learning is not as severe as that of prelingual deafness. This leads to insufficient attention to the disorder in the clinic. To date, only a few disease-causing genes have been reported. This report describe a case of novel heterozygous mutations in OTOGL that causes nonsyndromic mild sensorineural hearing loss. Basic information, imaging examinations, audiological examination, and vestibular function tests of the proband were collected. Blood samples of the proband's family were collected and analyzed by whole exome sequencing and Sanger sequencing. A pedigree diagram was drawn and the genetic patterns were analyzed. The proband is a 16-year-old female student with mild sensorineural hearing loss. High-resolution CT of the inner ear and vestibular function tests showed no abnormalities. The age of onset was approximately 4 years old. Except for hearing loss, no lesions were seen in other organs. The parents of the proband were not close relatives and had normal hearing. Two novel heterozygous mutations were found in the OTOGL gene. The c.5038del (p.D1680Ifs*6) variant was inherited from the father, and the c.2770C > T (p.R924X) variant from the mother. They enriched the mutation spectrum of OTOGL, which provides the basis for gene function research and genetic consultation.


Assuntos
Perda Auditiva Neurossensorial/genética , Proteínas de Membrana/genética , Adolescente , Adulto , China , Família , Feminino , Genótipo , Heterozigoto , Humanos , Masculino , Proteínas de Membrana/metabolismo , Mutação , Linhagem , Fenótipo , Sequenciamento Completo do Exoma
6.
Cells ; 10(12)2021 12 20.
Artigo em Inglês | MEDLINE | ID: mdl-34944105

RESUMO

Emerging evidence indicates that perinatal infection and inflammation can influence the developing immune system and may ultimately affect long-term health and disease outcomes in offspring by perturbing tissue and immune homeostasis. We posit that perinatal inflammation influences immune outcomes in offspring by perturbing (1) the development and function of fetal-derived immune cells that regulate tissue development and homeostasis, and (2) the establishment and function of developing hematopoietic stem cells (HSCs) that continually generate immune cells across the lifespan. To disentangle the complexities of these interlinked systems, we propose the cochlea as an ideal model tissue to investigate how perinatal infection affects immune, tissue, and stem cell development. The cochlea contains complex tissue architecture and a rich immune milieu that is established during early life. A wide range of congenital infections cause cochlea dysfunction and sensorineural hearing loss (SNHL), likely attributable to early life inflammation. Furthermore, we show that both immune cells and bone marrow hematopoietic progenitors can be simultaneously analyzed within neonatal cochlear samples. Future work investigating the pathogenesis of SNHL in the context of congenital infection will therefore provide critical information on how perinatal inflammation drives disease susceptibility in offspring.


Assuntos
Cóclea/patologia , Hematopoese , Sistema Imunitário/crescimento & desenvolvimento , Inflamação/patologia , Animais , Feto/imunologia , Perda Auditiva Neurossensorial/imunologia , Humanos
7.
Biomed Res Int ; 2021: 3548706, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34970625

RESUMO

Purpose: Radiotherapy-induced sensorineural hearing loss (RISNHL) is a common adverse effect in patients with head and neck cancer. Given that there are few studies on the pathogenesis of RISNHL at present, we summarized the possible pathogenesis of RISNHL and possible protective measures found at present by referring to relevant literatures. Methods: We performed a comprehensive literature search in the PubMed database, using keywords "sensorineural hearing loss," "radiotherapy," and "cancer," among others. The literature was examined for the possible mechanism and preventive measures of sensorineural hearing loss induced by radiotherapy. Results: We found that the incidence of RISNHL was closely related to the damage directly caused by ionizing radiation and the radiation-induced bystander effect. It also depends on the dose of radiation and the timing of chemotherapy. Studies confirmed that RISNHL is mainly involved in post-RT inflammatory response and changes in reactive oxygen species, mitogen-activated protein kinase, and p53 signaling pathways, leading to specific manners of cell death. We expect to reduce the incidence of hearing loss through advanced radiotherapy techniques, dose limitation of organs at risk, application of cell signaling inhibitors, use of antioxidants, induction of cochlear hair cell regeneration, and cochlear implantation. Conclusion: RISNHL is associated with radiation damage to DNA, oxidative stress, and inflammation of cochlear cells, stria vascularis endothelial cells, vascular endothelial cells, spiral ganglion neurons, and other supporting cells. At present, the occurrence mechanism of RISNHL has not been clearly illustrated, and further studies are needed to better understand the underlying mechanism, which is crucial to promote the formulation of better strategies and prevent the occurrence of RISNHL.


Assuntos
Neoplasias de Cabeça e Pescoço/radioterapia , Perda Auditiva Neurossensorial/etiologia , Radioterapia (Especialidade)/métodos , Animais , Humanos , Dosagem Radioterapêutica
8.
Orv Hetil ; 162(51): 2055-2060, 2021 12 19.
Artigo em Húngaro | MEDLINE | ID: mdl-34898470

RESUMO

Összefoglaló. A hirtelen halláscsökkenés patofiziológiája még nagyrészt tisztázatlan, így oki terápia nem lehetséges. Az elsodleges kezelést a helyileg vagy szisztémásan adott kortikoszteroid jelenti, egységes protokoll azonban nem áll rendelkezésre. Nagy vagy súlyos fokú hirtelen halláscsökkenés esetén kóroki tényezoként felmerül a perilymphafistula lehetosége még azoknál a betegeknél is, akiknél nem szerepel trauma az anamnézisben. A kórkép mutéti kezelése a dobüreg feltárását követoen a belso fül ablakainak obliterálása. Amennyiben ez a megoldás nem eredményez megfelelo hallásjavulást, hagyományos vagy implantálható hallókészülékek alkalmazása javasolt. A közleményben részletezett esetünkben teljes siketséggel járó, jobb oldali hirtelen halláscsökkenés alakult ki, melynek hátterében egyértelmu okot azonosítani nem sikerült. Az eredménytelen kombinált, intratympanalis és szisztémás szteroidkezelést követoen exploratív tympanotomiát végeztünk, melynek során a belso fül ablakait obliteráltuk. Hallásjavulást ezt követoen sem sikerült kimutatni, így cochlearis implantáció elvégzése mellett döntöttünk. Az implantációt a kerek ablakon keresztül végeztük, mely alapján kijelenthetjük, hogy az elozetes kerekablak-obliteráció nem zárja ki a késobbi cochlearis implantációt. Orv Hetil. 2021; 162(51): 2055-2060. Summary. The pathophysiology of sudden sensorineural hearing loss is mainly unknown, therefore no causative treatment exists. Systemic and local administration of corticosteroids serves as first line therapy although protocols vary. In cases of severe or profound hearing loss with no improvement for medical therapy, perilymphatic fistulae can be assumed even without any history of trauma. Therefore, inner ear window obliteration as a primary surgical option in the early stage can be considered. For patients without complete recovery, conventional hearing aids or implantable hearing devices can be offered. In our case report, we present a patient with right sided idiopathic sudden deafness. After failure of conservative combined intratympanic and systemic steroid therapy, explorative tympanotomy and obliteration of the inner ear windows were performed. As no hearing improvement was witnessed, successful cochlear implantation via round window insertion was performed. Our case justifies that obliterating the round window membrane does not rule out further successful cochlear implantation. Orv Hetil. 2021; 162(51): 2055-2060.


Assuntos
Implante Coclear , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Audição , Humanos , Janela da Cóclea
9.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi ; 56(12): 1283-1291, 2021 Dec 07.
Artigo em Chinês | MEDLINE | ID: mdl-34963216

RESUMO

Objective: To analyze the temporal bone CT and inner ear magnetic resonance imaging characteristics of cochlear implant patients with no cochlear nerve display in the inner auditory canal under MRI. To retrospectively analyze the long-term hearing and speech rehabilitation effects of such patients after cochlear implant. And to analyze the correlation between the results of imaging examinations and the postoperative effects of cochlear implant patients with this type of cochlear nerve deficiency. Methods: A total of 88 children with cochlear nerve deficiency, who underwent cochlear implantation in Shandong Provincial ENT Hospital from May 2014 to October 2018, were enrolled. Patients with cochlear malformations were excluded,only the patients with cochlear nerve deficiency whose cochlear structure was normal and no cochlear nerve displayed in inner auditory canal under MRI were enrolled. There were 64 patients, including 4 bilaterally implanted, 68 ears in total, with an average age of (2.8±1.7) years (range 1-6 years) at the time of implantation. The implanted product was Cochlear, including 24RECA and 512 models. All patients underwent inner ear magnetic resonance imaging and temporal bone CT scan before operation. Auditory speech function assessments were performed at 12 months, 24 months, and 36 months after surgery, including categories of auditory performance (CAP), speech intelligibility rating (SIR) and hearing aid threshold test. The imaging evaluation content included the width of the cochlear nerve canal of temporal bone CT, the width of the internal auditory canal, the width of the auditory nerve at the cerebellopontine angle of the inner ear MRI, and the ratio of the facial nerve to the width of the auditory nerve at the cerebellopontine angle. The correlations between the results of postoperative hearing aid hearing threshold, CAP, SIR and imaging results were analyzed. Results: Among the 64 cases of cochlear nerve not shown under MRI, 56 ears with CT data showed that the width of the cochlear nerve canal in temporal bone CT was (0.72±0.30) mm (mean±standard deviation, the same below), and the width of the internal auditory canal was (4.07±1.10) mm; 66 ears with MRI data showed that the diameter of the auditory nerve at the cerebellopontine angle of the inner ear MRI was (1.58±0.27) mm, the diameter of the facial nerve was (1.57±0.27) mm, and the ratio of the diameter of the facial nerve to the auditory nerve was (1.02±0.23). The average hearing thresholds at 12, 24, and 36 months after surgery were (46.8±2.5) dB HL, (40.7±0.8) dB HL, and (36.8±1.5) dB HL, respectively. The preoperative and postoperative CAP scores at 12, 24 and 36 months were (1.0±1.0), (3.8±1.4), (4.5±1.4) and (5.1±0.7) points, respectively. The preoperative and postoperative SIR scores at 12, 24, and 36 months were (1.1±0.3), (1.9±0.9), (2.5±0.9), and (2.9±0.6) points, respectively. The hearing threshold at 24 months after surgery was negatively correlated with the width of the internal auditory canal of temporal bone CT (r=-0.349, P=0.037), and the hearing threshold at 36 months after surgery was positively correlated with the ratio of the diameter of the facial nerve to the auditory nerve at the cerebellopontine angle of the inner ear MRI (r=0.740, P=0.001). Conclusions: Children with cochlear implants whose cochlear nerves are not shown on MRI can benefit from cochlear implantation, and their speech and auditory functions can improve significantly after surgery. The width of the internal auditory canal in the temporal bone CT and the ratio of the diameter of the facial nerve to the auditory nerve at the cerebellopontine angle of the inner ear MRI may be related to the long-term hearing threshold after surgery.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Criança , Pré-Escolar , Nervo Coclear/diagnóstico por imagem , Perda Auditiva Neurossensorial/cirurgia , Humanos , Lactente , Imageamento por Ressonância Magnética , Prognóstico , Estudos Retrospectivos , Inteligibilidade da Fala
10.
Vestn Otorinolaringol ; 86(6): 17-21, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34964323

RESUMO

OBJECTIVE: To study the effectiveness of auditory training using virtual reality technologies in people with chronic sensorineural hearing loss in dynamics. MATERIAL AND METHODS: The study included 68 patients aged from 24 to 97 years (mean age 57.63±15.87 years) with latent hearing loss (hearing loss at high frequencies), I degree of hearing loss and its variations. Auditory training was carried out on the basis of the Diagnostic and Rehabilitation System for spatial and speech hearing based on virtual reality using various audiovisual scenarios ReviAudio and control of speech perception, spatial hearing and hearing quality using the SSQRus questionnaire. RESULTS: A comparative analysis of the training results using the ReviAudio software revealed positive trends in the patient's response time and the patient's accuracy in determining the direction of the target signal, regardless of the symmetry and asymmetry of hearing loss. Patients with asymmetric hearing impairment observe an improvement in spatial hearing parameters, patients with symmetric hearing loss - speech perception and hearing quality, patients with latent hearing loss report an improvement in the quality of hearing. The auditory training is effective in individuals with mild chronic sensorineural hearing loss and in individuals with latent hearing loss as an adjunctive treatment.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Perda Auditiva , Percepção da Fala , Realidade Virtual , Adulto , Idoso , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/terapia , Humanos , Pessoa de Meia-Idade
11.
Otolaryngol Clin North Am ; 54(6): 1181-1191, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34774230

RESUMO

In 2020, the lower age limit for pediatric cochlear implant candidacy was lowered from 12 to 9 months of age. However, audiometric-based criteria for pediatric cochlear implant candidacy have remained unchanged for more than 20 years, requiring bilateral profound sensorineural hearing loss for children less than 2 years and bilateral severe-to-profound sensorineural hearing loss for children 2 years and older. Despite the static audiometric criteria, there is an increasing literature base demonstrating significant benefits for pediatric cochlear implant recipients who exceed current Food and Drug Administration-labeled indications for cochlear implantation.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Percepção da Fala , Criança , Perda Auditiva Neurossensorial/cirurgia , Humanos
12.
Otolaryngol Clin North Am ; 54(6): 1241-1251, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34774232

RESUMO

Vestibular dysfunction is the most common comorbidity associated with childhood sensorineural hearing loss. Early identification of vestibular dysfunction enables early intervention to mitigate its impact of motor, behavioral, and neurocognitive deficits of developing children. Screening for vestibular impairment can be achieved in the busy clinical setting.


Assuntos
Perda Auditiva Neurossensorial , Doenças Vestibulares , Criança , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/terapia , Humanos , Doenças Vestibulares/diagnóstico , Doenças Vestibulares/terapia
13.
Audiol Neurootol ; 26(6): 479-486, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34814132

RESUMO

OBJECTIVES: This study was conducted to explore the effectiveness of hyperbaric oxygen (HBO) in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL) and recommend the appropriate course of treatment. METHODS: 102 patients (105 diseased ears) with ISSNHL were recruited from the Department of Neurology and Otorhinolaryngology, West China Fourth Hospital, Sichuan University, between January 2018 and September 2020. Of them, 45 patients (group A) received intravenous steroid (IVS), and the remaining patients (group B) received IVS and HBO therapy (HBOT). Pure-tone audiometry (PTA) was performed twice at baseline and 10 days after treatment. Patients in group B were subdivided into group 1 (≤10 sessions) and group 2 (>11 sessions) to verify the correlation between the efficacy and course of HBOT, at the follow-up endpoint, the PTA was performed again. The multivariate logistical regression model was used to analyze the related factors of prognosis. RESULTS: Compared with the control group, significantly larger hearing gains and better hearing recovery rate were observed in the IVS + HBOT group (p < 0.05). The time of treatment and course of HBOT were significantly correlated with the hearing threshold after treatment (p < 0.05) and had no significant relationship with tinnitus and age (p > 0.05). CONCLUSION: HBOT + IVS is an effective method for ISSNHL, especially for the recovery of low-frequency hearing and initial hearing levels of severe and profound. Tinnitus is the most common concomitant symptom of ISSNHL, and prolonging the course of HBOT did not significantly improve it. Initiating HBOT within 7 days for 10-25 sessions of treatment was more beneficial.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Oxigenação Hiperbárica , Audiometria de Tons Puros , Glucocorticoides , Perda Auditiva Neurossensorial/terapia , Perda Auditiva Súbita/terapia , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
14.
Orphanet J Rare Dis ; 16(1): 468, 2021 11 04.
Artigo em Inglês | MEDLINE | ID: mdl-34736503

RESUMO

BACKGROUND: Achondroplasia is the most common form of disproportionate skeletal dysplasia. The condition is caused by a mutation in the FGFR3 gene, affecting endochondral bone growth, including the craniofacial anatomy. Recurrent otitis media infections, chronic middle ear effusion, and hearing loss are common in children with achondroplasia, but few studies have investigated hearing loss in adults with this condition. OBJECTIVES: This population-based study investigated the prevalence, severity, and type of hearing loss in Norwegian adults with achondroplasia. METHODS: We collected data on 45 adults with genetically confirmed achondroplasia: 23 men and 22 women, aged 16-70 years. All participants underwent a comprehensive audiologic assessment, including medical history, pure-tone audiometry, speech audiometry, and impedance audiometry. According to the Global Burden of Disease classification, pure-tone average ≥ 20 decibel hearing level (dB HL) was considered clinically significant hearing loss. RESULTS: Insertion of ventilation tubes had been performed in 44% (20/45) of the participants, 49% (22/45) had a history of adenoidectomy, while 20% (9/45) used hearing aids. Hearing loss in at least one ear was found in 53% (24/45) of the participants; in 57% (13/23) of the men and 50% (11/22) of the women. In the youngest age group (age 16-44 years), 50% (14/28) had hearing loss, although predominantly mild (20-34 dB HL). An abnormal tympanometry (Type B or C) was found in 71% (32/45) of the participants. The majority (15/24) had conductive hearing loss, or a combination of conductive and sensorineural hearing loss (8/24). CONCLUSIONS: Adults with achondroplasia are at increased risk of early hearing loss. Our findings underline the importance of a regular hearing assessment being part of standard care in achondroplasia, including adolescents and young adults. In adult patients diagnosed with hearing loss, an evaluation by an otolaryngologist should be considered, and the need for hearing aids, assistive listening devices, and workplace and educational accommodations should be discussed. Clinical trial registration ClinicalTrials.gov identifier NCT03780153.


Assuntos
Acondroplasia , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Acondroplasia/complicações , Acondroplasia/genética , Testes de Impedância Acústica , Adolescente , Adulto , Idoso , Audiometria de Tons Puros , Estudos Transversais , Feminino , Perda Auditiva/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Noruega , Adulto Jovem
15.
J Transl Med ; 19(1): 424, 2021 10 12.
Artigo em Inglês | MEDLINE | ID: mdl-34641888

RESUMO

BACKGROUND: The association between exposure to air pollution and sudden sensorineural hearing loss (SSNHL) has not been extensively discussed in the literature. Therefore, we conducted this nationwide study to evaluate the risk of SSNHL in Taiwanese residents with exposure to air pollution. METHODS: We enrolled subjects aged older than 20 years with no history of SSNHL from 1998 to 2010, and followed up until developing SSNHL, withdrawn from the National Health Insurance program, and the end of the database (2011/12/31). The air quality data are managed by Taiwan Environmental Protection Administration. The annual concentrations of PM2.5, SO2, CO, NO, and NO2 from 1998 to 2010 were classified into the three levels according to tertiles. We calculated the annual average of pollutants from baseline until the end of the study, and classified into tertiles. The adjusted hazard ratio (aHR) was estimated by using the multivariate Cox proportional hazard model. RESULTS: When considered continuous air pollutants concentration, subjects who exposed with higher concentration of CO (aHR = 2.16, 95% CI 1.50-3.11), NO (aHR = 1.02, 95% CI 1.01-1.03), and NO2 (aHR = 1.02, 95% CI 1.01-1.04) developing significant higher risk of SSNHL. When classified air pollutants concentration into low, moderate and high level by tertiles, and selected low level as reference, patients exposed with moderate (aHR = 1.56, 95% CI 1.20-2.04) or high level (aHR = 1.33, 95% CI 1.01-1.75) of PM2.5 showed significant higher risk of developing SSNHL. CONCLUSION: This study indicated an increased risk of SSNHL in residents with long-term exposure to air pollution. Nevertheless, further experimental, and clinical studies are needed to validate the study findings.


Assuntos
Poluentes Atmosféricos , Poluição do Ar , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Idoso , Poluentes Atmosféricos/toxicidade , Poluição do Ar/estatística & dados numéricos , Perda Auditiva Neurossensorial/induzido quimicamente , Perda Auditiva Neurossensorial/epidemiologia , Humanos , Modelos de Riscos Proporcionais , Fatores de Risco
17.
Trends Hear ; 25: 23312165211041475, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34606381

RESUMO

A deep recurrent neural network (RNN) for reducing transient sounds was developed and its effects on subjective speech intelligibility and listening comfort were investigated. The RNN was trained using sentences spoken with different accents and corrupted by transient sounds, using the clean speech as the target. It was tested using sentences spoken by unseen talkers and corrupted by unseen transient sounds. A paired-comparison procedure was used to compare all possible combinations of three conditions for subjective speech intelligibility and listening comfort for two relative levels of the transients. The conditions were: no processing (NP); processing using the RNN; and processing using a multi-channel transient reduction method (MCTR). Ten participants with normal hearing and ten with mild-to-moderate hearing loss participated. For the latter, frequency-dependent linear amplification was applied to all stimuli to compensate for individual audibility losses. For the normal-hearing participants, processing using the RNN was significantly preferred over that for NP for subjective intelligibility and comfort, processing using the RNN was significantly preferred over that for MCTR for subjective intelligibility, and processing using the MCTR was significantly preferred over that for NP for comfort for the higher transient level only. For the hearing-impaired participants, processing using the RNN was significantly preferred over that for NP for both subjective intelligibility and comfort, processing using the RNN was significantly preferred over that for MCTR for comfort, and processing using the MCTR was significantly preferred over that for NP for comfort.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Percepção da Fala , Humanos , Redes Neurais de Computação , Ruído/efeitos adversos , Inteligibilidade da Fala
18.
J Int Adv Otol ; 17(5): 387-392, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34617887

RESUMO

OBJECTIVE: To evaluate the relationship between the third window abnormalities and congenital inner ear malformations in pediatric patients with different types of hearing loss. If such a relationship should exist, it would be important to take it into account, in order to diagnose and treat pediatric hearing loss cases more accurately. METHODS: Two hundred twenty-one children with hearing loss who had temporal bone computed tomography (CT) examination and were identified from 2013 to 2018 were retrospectively evaluated. The types of hearing loss were grouped as sensorineural hearing loss (SNHL), conductive hearing loss (CHL), and mixed hearing loss (MHL). Third window abnormalities included superior semicircular canal (SC) dehiscence, posterior SC dehiscence, enlarged vestibular aqueduct (EVA), X-linked stapes gusher, perilymph fistula, and bone dyscrasias. Congenital inner ear malformations included cochleovestibular, SC, and internal acoustic canal malformations. The relationships were analyzed with chi-square and Fisher's exact tests. RESULTS: In the study, 40 patients had unilateral hearing loss and 181 had bilateral hearing loss. In 402 ears, the rates of SNHL, CHL, and MHL were 88.5%, 6.9%, and 4.4%, respectively. EVA was the most common third window abnormality (41/402; 9.7%), and SC malformations were the most common inner ear malformations (53/402; 13.2%). In the SNHL group, superior and posterior SC dehiscence were associated with cochleovestibular malformations (P = .035 and.020, respectively). In the CHL group, there was a relationship between EVA and SC malformations (P = .041). No relationships were found in the MHL group. CONCLUSION: Third window abnormalities and congenital inner ear malformations may be encountered simultaneously in children with SNHL and CHL.


Assuntos
Perda Auditiva Neurossensorial , Aqueduto Vestibular , Criança , Perda Auditiva Condutiva/etiologia , Humanos , Estudos Retrospectivos , Osso Temporal/diagnóstico por imagem , Aqueduto Vestibular/diagnóstico por imagem
19.
J Int Adv Otol ; 17(5): 452-460, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34617898

RESUMO

BACKGROUND AND OBJECTIVES: Vestibular schwannomas (VS) frequently lead to ipsilateral sensorineural hearing loss (HL) as part of its natural history or as a result of treatment. Cochlear implantation represents a well-documented treatment of profound HL that cannot be treated adequately with a conventional hearing aid, thus being offered to selected VS patients. A functional cochlea and cochlear nerve are prerequisites for sound perception with a cochlear implant (CI). The potential impact of radiotherapy on these structures is thus an important issue for subsequent CI hearing outcomes. The objective of this article is to present a case and to review the existing literature on the outcomes of cochlear implantation in irradiated VS patients systematically. METHODS: A systematic literature review using preferred reporting items for systematic reviews and meta-analyses was conducted. Medline was searched systematically. Papers reporting ipsilateral CI outcomes after radiotherapy of VS were included. Additionally, results of CI after stereotactic radiotherapy in a 54-year-old male with neurofibromatosis type 2 are presented. RESULTS: A total of 14 papers (33 patients) fulfilled inclusion criteria. Moderate preoperative HL was found in 11 patients. Six had moderate to severe HL, whereas 16 had severe HL or total deafness. Postoperative hearing outcomes varied from poor in 27% of patients to excellent in 19%, with remaining cases lying in between (mean follow-up of 19 months). Most patients achieved improvement in hearing and quality of life. CONCLUSION: Despite variation in the degree of hearing outcome, CI after radiotherapy of VS appears to be effective in the majority of cases, as more than 70% of patients have good or excellent outcomes within 1-2 years post-implantation. Subjective benefits are considerable, even in cases with relatively poor objective outcome.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Neuroma Acústico , Percepção da Fala , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/radioterapia , Neuroma Acústico/cirurgia , Qualidade de Vida , Resultado do Tratamento
20.
J Int Adv Otol ; 17(5): 465-467, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34617900

RESUMO

Past studies have found an association between cochlear hydrops or early Meniere's disease and acute low-frequency hearing loss (ALHL) without vertigo. However, its mechanism remains unclear in some ALHL cases. This report presents a case of ALHL associated with iron-deficiency anemia (IDA). The patient was a 49-year-old female who had previously been treated with betamethasone for sudden hearing loss in the right ear. Eight months later, the symptoms recurred and cochlear hydrops was diagnosed. Isosorbide and betamethasone were administered orally, and intravenous hydrocortisone tapering was added 1 week later, but these treatments were ineffective. At the same time and subsequently, iron sucrose was intravenously administered for IDA, and the patient's hearing loss gradually resolved within 2 months. In view of the increase in serum hemoglobin levels after iron therapy, this might have been the most effective treatment in this case. The hearing loss could therefore be associated with the patient's IDA.


Assuntos
Anemia Ferropriva , Hidropisia Endolinfática , Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Anemia Ferropriva/complicações , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/tratamento farmacológico , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/tratamento farmacológico , Humanos , Pessoa de Meia-Idade , Vertigem
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