Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 2.231
Filtrar
1.
Heliyon ; 10(16): e35737, 2024 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-39224385

RESUMO

Purpose: Knowledge of the cochlear anatomy in individual patients is helpful for improving electrode selection and placement during cochlear implantation, as well as in surgical planning. The aim of this study was to develop a model-free automated segmentation algorithm to obtain 3D surfaces from clinical computed tomography (CT) scans that describe an individual's cochlear anatomy and can be used to quantitatively analyze the cochlea's vertical trajectory. Methods: Clinical CT scans were re-oriented and re-sliced to obtain mid-modiolar slices. Using these slices, we segmented the cross-section of the cochlea. Results: 3D surfaces were obtained for the first 1.5 turns of 648 cochleae. Validation of our algorithm against the manually segmented ground truth obtained from 8 micro-CT scans showed good agreement, with 90 % area overlap and an average distance of 0.11 mm between the segmentation contours. The average cochlear duct length for the basal turn was 16.1 mm along the central path and 22.4 mm along the outer wall. The use of an intrinsic, observer-independent coordinate system and principal component analysis enabled unambiguous quantitative evaluation of the vertical trajectory of the cochlea, revealing only a weak correlation between the symmetry of the commonly used basal turn diameters (B-ratio of A and B diameters) and the profile of the vertical trajectory. Conclusion: A model-free segmentation algorithm can achieve similar accuracy as previously published methods relying on statistical shapes. Quantitative analysis of the vertical trajectory can replace the categorization into rollercoaster, sloping, or intermediate vertical trajectory types.

2.
ACS Nano ; 2024 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-39259947

RESUMO

Sensorineural hearing loss (SNHL) represents a significant clinical challenge, predominantly attributed to oxidative stress-related mechanisms. In this work, we report an innovative antioxidant strategy for mitigating SNHL, utilizing synthetically engineered allomelanin nanoparticles (AMNPs). Empirical evidence elucidates AMNPs' profound capability in free radical neutralization, substantiated by a significant decrement in reactive oxygen species (ROS) levels within HEI-OC1 auditory cells exposure to cisplatin or hydrogen peroxide (H2O2). Comparative analyses reveal that AMNPs afford protection against cisplatin-induced and noise-induced auditory impairments, mirroring the effect of dexamethasone (DEX), a standard pharmacological treatment for acute SNHL. AMNPs exhibit notable cytoprotective properties for auditory hair cells (HCs), effectively preventing ototoxicity from cisplatin or H2O2 exposure, as confirmed by both in vitro assays and cultured organ of Corti studies. Further in vivo research corroborates AMNPs' ability to reverse auditory brainstem response (ABR) threshold shifts resulting from acoustic injury, concurrently reducing HCs loss, ribbon synapse depletion, and spiral ganglion neuron degeneration. The therapeutic benefits of AMNPs are attributed to mitigating oxidative stress and inflammation within the cochlea, with transcriptome analysis indicating downregulated gene expression related to these processes post-AMNPs treatment. The pronounced antioxidative and anti-inflammatory effects of AMNPs position them as a promising alternative to DEX for SNHL treatment.

3.
Int J Pediatr Otorhinolaryngol ; 186: 112082, 2024 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-39265223

RESUMO

OBJECTIVE: Genetic variants in the OTOF gene are responsible for non-syndromic hearing loss with an autosomal recessive inheritance pattern. The objective of our work was to evaluate the clinical characteristics of patients with biallelic pathogenic variants in OTOF and their evolution after treatment. METHODS: A cohort of 124 patients with prelingual hearing loss, studied from 1996 to 2023, was included in this study. A genetic analysis was conducted to identify the type and frequency of variants in the OTOF gene and their relation to the clinical characteristics of the patients. RESULTS: The homozygous p. Gln829* variant in the OTOF gene was detected in 3 probands (2.4 %) of a group 124 individuals with prelingual hearing loss. Another 6 family members to a total of 9 individuals were finally included. All presented with severe/profound bilateral sensorineural hearing loss of congenital onset. Three of these individuals were diagnosed with auditory neuropathy spectrum disorder. One individual passed the OAE test during the screening program, and since he did not have risk factors for hearing loss that would warrant ABR testing, this led to a delay in his hearing loss diagnosis. Four individuals underwent cochlear implants (three bilateral) with good functional outcomes. In three of them. However, in 17 familial cases with heterozygous variants, either no hearing loss was observed or it was within the expected range for their age. CONCLUSIONS: Hearing loss secondary to the p. Gln829* variant of the OTOF gene is relatively rare in our medical area. Its presence in homozygosity is the cause of severe/profound bilateral prelingual sensorineural hearing loss, responsible for auditory neuropathy with a good response to cochlear implantation.

4.
Heliyon ; 10(18): e37223, 2024 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-39309931

RESUMO

Radiotherapy is a crucial treatment for head and neck malignancies, but it can sometimes cause sensorineural hearing loss (SNHL). Changes in the immune microenvironment and sensory neuroepithelium of the inner ear after radiation exposure remain poorly understood. This study investigated cochlear morphology and macrophages in the inner ear after high-dose irradiation. The heads of heterozygous 8-week-old Cx3cr1GFP/+ male mice were irradiated with 30Gy of X-rays and biological samples were collected on days 1, 7, and 10 after irradiation. Auditory brainstem responses were used to assess auditory function in the mice. Changes in basilar membrane hair cells, spiral ganglion neurons (SGN), and inner ear macrophages were observed using hematoxylin-eosin (HE) staining and immunofluorescence staining. The expression of inflammatory mediators in the inner ear was detected by quantitative real-time reverse transcription-polymerase chain reaction (RT-qPCR) in cochlear tissue. The results showed no significant hair cell loss after a single high dose of radiation. However, the mice developed pantonal hearing loss on day 10 when HE staining revealed SGN atrophy and immunofluorescence showed decreased neurofilament expression. The number of macrophages in the inner ear reduced over time. RT-qPCR showed that cochlear inflammatory factors and chemokines were briefly upregulated on day 1st after irradiation and then decreased over time. In conclusion, high-dose irradiation causes acute SNHL that is not associated with hair cell loss and may be related to SGN changes. Radiation-induced SNHL is associated with a reduction in cochlear macrophages and changes in the immune microenvironment, but the relationship between the two remains to be investigated.

5.
Ear Nose Throat J ; : 1455613241284154, 2024 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-39315458

RESUMO

Objectives: Intratympanic steroid injection (ISI) for sudden sensorineural hearing loss (SSNHL) is a relatively popular and effective method, but there is no standardized method for intratympanic steroids for the treatment of SSNHL and no consensus on how to deliver steroids to the middle ear. The purpose of this study was to compare 2 means of intratympanic steroid delivery as therapy for SSNHL. Methods: A retrospective chart review was performed for the period from November 2018 to October 2022 at our Department of Otorhinolaryngology-Head and Neck Surgery. Sixty patients with profound SSNHL who have failed initial steroid therapy were divided into the continuously transtympanic steroid perfusion (TSP) therapy and the intermittent ISI therapy. Results: Posttreatment pure-tone average was 60.3 ± 18.2 dB in the TSP group and 67.5 ± 22.6 dB in the ISI group, 70.0% of subjects in the TSP group, and 46.7% of subjects in the ISI group had improved by 15 dB or more after the therapy. The increased hearing threshold of the TSP group (24.6 ± 14.1 dB) was better than the ISI group (16.6 ± 14.9 dB), and the hearing recovery was significantly different (P < .05) in the 2 groups. Besides the hearing improvement was most obvious in low-frequency areas in the TSP group, the most significant hearing improvement was at 250 Hz, reaching 30.8 ± 3.3 dB. Conclusions: In SSNHL patients who have failed initial steroid therapy, TSP therapy via a ventilation tube can achieve good hearing outcomes and serve as a salvage therapy for patients with SSNHL.

6.
J Neuroinflammation ; 21(1): 223, 2024 Sep 14.
Artigo em Inglês | MEDLINE | ID: mdl-39277762

RESUMO

The activation of the NLRP3 inflammasome has been linked to several inflammatory and autoinflammatory diseases. Despite cases of potential hearing improvement in immune-mediated diseases, direct evidence of the efficacy of targeting this mechanism in the inner ear is still lacking. Previously, we discovered that macrophages are associated with Sensorineural Hearing loss (SNHL) in Chronic Suppurative Otitis Media (CSOM), the leading cause of this permanent hearing loss in the developing world and incurring costs of $4 to $11 billion dollars in the United States. However, the underlying mechanism remained unknown. Here, we investigate how macrophages drive permanent hearing loss in CSOM. We first confirmed the occurrence of NLRP3 inflammasome activation in cochlear macrophages in CSOM. We then revealed that Outer Hair Cells (OHCs) were protected in CSOM by macrophage depletion and subsequently confirmed the same protection in the NLRP3 knockout condition. Furthermore, we showed that therapeutic inhibition of NLRP3 inflammasome activation and downstream inhibition of IL-1ß protects OHCs in CSOM. Collectively, our data demonstrates that the main driver for hearing loss in CSOM is NLRP3 inflammasome activation in cochlear macrophages and this is therapeutically targetable, leading the way for the development of interventions to prevent the leading cause of permanent hearing loss and a costly disease in the developed world.


Assuntos
Cóclea , Inflamassomos , Macrófagos , Proteína 3 que Contém Domínio de Pirina da Família NLR , Otite Média Supurativa , Animais , Feminino , Humanos , Masculino , Camundongos , Doença Crônica , Cóclea/metabolismo , Cóclea/patologia , Modelos Animais de Doenças , Perda Auditiva/etiologia , Perda Auditiva/prevenção & controle , Inflamassomos/metabolismo , Interleucina-1beta/metabolismo , Macrófagos/metabolismo , Camundongos Endogâmicos C57BL , Camundongos Knockout , Proteína 3 que Contém Domínio de Pirina da Família NLR/metabolismo , Proteína 3 que Contém Domínio de Pirina da Família NLR/antagonistas & inibidores
7.
Ear Nose Throat J ; : 1455613241285679, 2024 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-39316600

RESUMO

Preservation of residual hearing and vestibular function is a crucial factor in cochlear implantation (CI), especially in patients with residual low-frequency hearing thresholds. We report a case of a patient who underwent unilateral endoscope-assisted CI with a challenging surgical view following rigorous posterior tympanotomy. A 53-year-old male presented with left-sided intractable tinnitus due to sudden sensorineural hearing loss that had occurred 10 years prior. Due to the abnormal location of the round window (RW), which was far more posterior and inferior than usual and impeded insertion of the electrode using the conventional RW approach, endoscope-assisted CI was performed. Pure-tone audiometry at 3 months after CI revealed satisfactory hearing thresholds. Furthermore, there was alleviation of the left-sided tinnitus, which was indicated by a marked decrease in both the subjective visual analog scale loudness and Tinnitus Handicap Inventory scores. With proper indications, we strongly recommend applying the RW approach with endoscopic assistance over conventional bony cochleostomy for the preservation of low-frequency hearing thresholds in cases where RW visualization is insufficient following posterior tympanotomy.

8.
Laryngoscope ; 2024 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-39301868

RESUMO

OBJECTIVE: To study predictors of delayed presentation, diagnosis, and treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN: Retrospective medical chart review and patient telephone survey. SETTING: Tertiary medical center. SUBJECTS AND METHODS: All patients who initially presented or referred with unilateral ISSNHL between 2016 and 2020 were included. Associations between epidemiological, demographic, and socioeconomic profiles and delays in presentation, diagnosis and treatment were studied. RESULTS: A total of 518 patients were suitable for study inclusion. The total delay in the emergency department (ED) setup was a median (interquartile range, IQR) of 1 (0-1) day, 7 (6-12) days in a community otolaryngologist setup, and 15 (8-25) days in a general practitioner setup. Patients who presented to the ED first also had fewer diagnostic and treatment delays. Those who presented to a community otolaryngologist first had significantly longer presentation delay (5 [4-8] days p < 0.001) and significantly longer treatment delays (1 [1-3] days p < 0.001). Patients who presented to a general practitioner first had significantly longer presentation delays compared with ED presentation, and the longest diagnostic and treatment delays (3 [2-5], 8 [4-12] days, and 4 [2-7] days, p = < 0.01, p = <0.01, and p < 0.001, respectively). There was no association between socioeconomic status or demography and presentation, diagnostic, or treatment delays. CONCLUSION: Total delay in ISSNHL management is affected by the venue of the first medical encounter. General practitioners' level of awareness of the need for empiric steroidal treatment of ISSNHL without delay should be raised. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

9.
Acta Otolaryngol ; : 1-9, 2024 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-39305175

RESUMO

BACKGROUND: Kenny-Caffey Syndrome type 2 (KCS2) is a genetic disease affecting bone metabolism. However, cochlear implantation (CI) results have yet to be published in detail. OBJECTIVE: This study presents the gene, clinical characteristics, surgical outcomes, and literature review of 2 patients with sensorineural hearing loss related to KCS2. To enhance diagnostic detection and accuracy, we also compare the differential diagnosis between KCS2, otosclerosis, and Cogan's syndrome (CS). METHODS: Prior to CI, patients with KCS2 and CS underwent comprehensive audiological and radiological evaluations. Postoperative auditory speech outcomes and impedance values were recorded and analyzed statistically. A systematic search of the literature was conducted to summarize clinical characteristics. RESULTS: Patients diagnosed with KCS2 exhibit more pronounced changes in the inner ear. The impedance values in the KCS2 cohort were considerably higher (Mean = 12.13 kΩ) than those with CS (Mean = 8.8 kΩ) one year post-activation. The literature review exhibits the clinical manifestations associated with KCS2. CONCLUSION: CI is an effective treatment for KCS2 to restore hearing loss. More frequent programming and accurate adjustment of stimulation is of great necessity. A thorough examination, including temporal bone HRCT, 3D-MRI, audiological evaluations, and whole-exome sequencing, is essential for the diagnosis and treatment of KCS2.

10.
Neurohospitalist ; 14(4): 454-456, 2024 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-39308459

RESUMO

Superficial siderosis (SS) is an infrequent condition characterized by hemosiderin deposition in the central nervous system, resulting from chronic subarachnoid hemorrhage, often linked to dural mater diseases. Through a case report of a 50-year-old male with severe sensorineural hearing loss and newly diagnosed epilepsy, we explore SS triggered by a spinal ependymoma, diagnosed via resonance magnetic imaging (MRI). This case highlights the necessity of comprehensive neuroaxis imaging to identify treatable etiologies. The complexity of SS, with its varied clinical presentations, necessitates early detection and a multidisciplinary treatment approach. Despite limited treatment options and the uncertain efficacy of therapies like deferiprone, early intervention is crucial for mitigating irreversible neurological damage and enhancing patient prognosis.

11.
Am J Stem Cells ; 13(4): 212-221, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39308766

RESUMO

Sensorineural deafness mainly occurs due to damage to hair cells, and advances in stem cell technology, especially the application of induced pluripotent stem cells (iPSCs) and adult stem cells, provides new possibilities for hair cell regeneration. This review describes the basic knowledge of stem cells and their important applications in regenerative medicine, as well as recent progress in stem cell research in the field of hair cell regeneration, especially the induced differentiation of hair-like cells. At the same time, we also point out the challenges facing current research, including differentiation efficiency, cell stability issues, and treatment safety and long-term efficacy considerations. Finally, we look forward to the direction of future research, and emphasize the importance of the cell differentiation mechanism, simulation of the inner ear microenvironment, safety assessment, and personalized treatment strategies. In conclusion, despite many challenges, stem cell technology has shown great potential in the field of hearing research and is expected to bring revolutionary treatment options for patients with sensorineural hearing loss in the future.

12.
Ear Nose Throat J ; : 1455613241278755, 2024 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-39292925

RESUMO

Objective: Asymmetric sensorineural hearing loss (ASNHL) exhibits a higher prevalence among the elderly compared to younger individuals, yet optimal management remains subject to ongoing debate. We aimed to elucidate the clinical disparities among elderly patients with ASNHL, distinguishing between those with and without cerebellopontine angle (CPA) tumors. Methods: A retrospective analysis was conducted on elderly patients (aged ≥65 years) diagnosed with ASNHL who underwent magnetic resonance imaging (MRI) between January 2012 and December 2022 at our tertiary referral center. Results: A total of 119 patients were enrolled, with a median age of 71 years (range: 65-89 years). Among them, 11 patients (9.2%) exhibited abnormal MRI findings. In the CPA tumors group, vestibular schwannoma was the most prevalent abnormality (63.6%), with a mean growth rate of 0.53 mm/year (range: 0-1.33 mm/year). The prevalence of CPA tumors in patients with diabetes mellitus (DM) and ASNHL was significantly lower than in those without DM (P = .021). Vertigo emerged as a significant associated symptom in cases with CPA tumors (P = .011). However, there were no significant differences in mean hearing thresholds or asymmetry of hearing loss at individual frequencies between the 2 groups. Conclusions: Elderly patients with ASNHL and vertigo should undergo radiological assessment. Patients with DM exhibit a lower prevalence of CPA tumors than those without DM, warranting careful observation and follow-up due to the limited diagnostic yield of MRI. No discernible differences in audiometric patterns were detected between patients with and without CPA tumors.

13.
Cureus ; 16(8): e67760, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39323698

RESUMO

Biallelic missense mutations in the nuclear-encoded, cytosolic isoleucyl-tRNA synthetase (IARS) gene are associated with a rare and complex multisystemic phenotype, including growth retardation, intellectual disability, muscular hypotonia, diabetes mellitus, and deafness. These mutations impact the cytosolic isoform of IARS, which plays a crucial role in protein synthesis. The pathogenesis involves mitochondrial dysfunction, despite IARS being primarily a cytosolic enzyme, potentially linking it to the observed clinical manifestations. The efficacy of cochlear implantation for deafness due to IARS mutations and the safety of general anesthesia in such patients remain unclear. This report describes a rare case of progressive sensorineural hearing loss caused by IARS mutation-associated mitochondrial disease, which was successfully treated with cochlear implantation. Additionally, we discuss the safety of general anesthesia in this patient. A Japanese woman with IARS mutation-associated mitochondrial disease was diagnosed with severe bilateral sensorineural hearing loss at five years of age and immediately received hearing aids. Her hearing progressively deteriorated to profound impairment, necessitating cochlear implantation at 26 years of age, which resulted in satisfactory hearing. Furthermore, no perioperative general anesthesia-related adverse events were reported. Our case demonstrates that cochlear implantation can effectively restore hearing. This suggests that sensorineural hearing loss caused by IARS deficiency is primarily due to cochlear dysfunction. This case demonstrated that hearing loss is a crucial feature of IARS mutation-associated mitochondrial disease, which can be mitigated by cochlear implantation. While general anesthesia can be safely administered, careful consideration of anesthetic choices, such as avoiding depolarizing muscle relaxants and prolonged use of propofol, is essential to prevent complications. In this case, general anesthesia was well tolerated without perioperative events, providing valuable insight into the potential safety of such procedures in similar patients. Nevertheless, further studies are needed to confirm these findings across a broader population.

14.
Brain Commun ; 6(5): fcae317, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39318785

RESUMO

Sudden sensorineural hearing loss, a prevalent emergency in otolaryngology, is known to potentially precipitate cognitive and emotional disorders in affected individuals. Extensive research has documented the phenomenon of cortical functional reorganization in patients with sudden sensorineural hearing loss. However, the potential link between this neural functional remodelling and cognitive-emotional disorders remains unclear. To investigate this issue, 30 bilateral sudden sensorineural hearing loss patients and 30 healthy adults were recruited for this study. We collected clinical data and resting-state functional magnetic resonance imaging data from the participants. Gradient mapping analysis was employed to calculate the first three gradients for each subject. Subsequently, gradient changes in sudden sensorineural hearing loss patients were compared with healthy controls at global, regional and network levels. Finally, we explored the relationship between gradient values and clinical variables. The results revealed that at the global level, sudden sensorineural hearing loss did not exhibit significant differences in the primary gradient but showed a state of compression in the second and third gradients. At the regional level, sudden sensorineural hearing loss patients exhibited a significant reduction in the primary gradient values in the temporal pole and ventral prefrontal cortex, which were closely related to neuro-scale scores. Regarding the network level, sudden sensorineural hearing loss did not show significant differences in the primary gradient but instead displayed significant changes in the control network and default mode network in the second and third gradients. This study revealed disruptions in the functional hierarchy of sudden sensorineural hearing loss, and the alterations in functional connectivity gradients were closely associated with cognitive and emotional disturbances in patients. These findings provide new evidence for understanding the functional remodelling that occurs in sudden sensorineural hearing loss.

15.
Brain Sci ; 14(9)2024 Sep 17.
Artigo em Inglês | MEDLINE | ID: mdl-39335422

RESUMO

BACKGROUND/OBJECTIVES: Given the importance of emotion recognition for communication purposes, and the impairment for such skill in CI users despite impressive language performances, the aim of the present study was to investigate the neural correlates of emotion recognition skills, apart from language, in adult unilateral CI (UCI) users during a music in noise (happy/sad) recognition task. Furthermore, asymmetry was investigated through electroencephalographic (EEG) rhythm, given the traditional concept of hemispheric lateralization for emotional processing, and the intrinsic asymmetry due to the clinical UCI condition. METHODS: Twenty adult UCI users and eight normal hearing (NH) controls were recruited. EEG gamma and alpha band power was assessed as there is evidence of a relationship between gamma and emotional response and between alpha asymmetry and tendency to approach or withdraw from stimuli. The TAS-20 questionnaire (alexithymia) was completed by the participants. RESULTS: The results showed no effect of background noise, while supporting that gamma activity related to emotion processing shows alterations in the UCI group compared to the NH group, and that these alterations are also modulated by the etiology of deafness. In particular, relative higher gamma activity in the CI side corresponds to positive processes, correlated with higher emotion recognition abilities, whereas gamma activity in the non-CI side may be related to positive processes inversely correlated with alexithymia and also inversely correlated with age; a correlation between TAS-20 scores and age was found only in the NH group. CONCLUSIONS: EEG gamma activity appears to be fundamental to the processing of the emotional aspect of music and also to the psychocognitive emotion-related component in adults with CI.

16.
Adv Exp Med Biol ; 1457: 265-283, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39283432

RESUMO

Sudden sensorineural hearing loss (SSNHL) has emerged as a potential complication of COVID-19 infection and vaccination. Various mechanisms by which the SARS-CoV-2 virus can cause hearing loss have been reported, including direct viral invasion, neuroinflammation, blood flow disturbances, and immune-mediated response. However, the temporal relationship between COVID-19 infection and SSNHL remains unclear, with mixed findings and conflicting results reported in different studies. Similarly, while anecdotal reports have linked COVID-19 vaccination to SSNHL, evidence remains scarce. Establishing a correlation between COVID-19 vaccines and SSNHL implies a complex and multifactorial pathogenesis involving interactions between the immune system and the body's stress response. Nevertheless, it is important to consider the overwhelming evidence of the vaccines' safety and efficacy in limiting the spread of the disease and remains the primordial tool in reducing death.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Perda Auditiva Súbita , SARS-CoV-2 , Humanos , COVID-19/imunologia , COVID-19/prevenção & controle , COVID-19/complicações , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/imunologia , Perda Auditiva Súbita/virologia , Vacinas contra COVID-19/efeitos adversos , Vacinas contra COVID-19/imunologia , SARS-CoV-2/imunologia , Vacinação/efeitos adversos , Orelha Interna/imunologia , Perda Auditiva Neurossensorial/virologia , Perda Auditiva Neurossensorial/imunologia , Perda Auditiva Neurossensorial/etiologia
17.
Laryngoscope ; 2024 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-39323315

RESUMO

OBJECTIVE: Mutations in the MTRNR1 gene of mitochondrial DNA are associated with non-syndromic hearing loss and increased susceptibility to aminoglycoside ototoxicity. The aim of our study was to determine the clinical characteristics of sensorineural hearing loss caused by the m.1555A>G mutation in MTRNR1. METHODS: An observational retrospective study of the m.1555A>G mutation was conducted in patients with suspected hereditary bilateral sensorineural hearing loss in the Department of Otolaryngology of the Marqués de Valdecilla University Hospital (Cantabria, Spain) and in 100 controls with normal hearing. RESULTS: The m.1555A>G mutation was found in 82 individuals from 20 different families and in none of the controls. Variable degrees of hearing loss were observed, ranging from normal hearing to profound deafness. Patients with a history of streptomycin administration exhibited significantly more pronounced hearing loss. The onset of hearing loss occurred from childhood to adulthood, with progression or stability over the years. No associated vestibular alterations or other clinical manifestations outside the ear were found. Two cochlear implant recipients showed significant improvement in speech comprehension. CONCLUSIONS: Patients with the m.1555A>G mutation in the MTRNR1 gene often develop bilateral, symmetric sensorineural hearing loss, predominantly affecting high frequencies, worsened by streptomycin administration. This mutation does not affect the vestibular function. The variability in the severity of hearing loss, the heterogeneity of phenotypic expression, and the presence of carrier individuals with normal hearing may indicate the existence of modifying factors, both environmental and genetic. Cochlear implantees showed a good response in terms of speech intelligibility. Genetic testing for this mutation is recommended in patients with a family history of hearing loss to prevent the use of aminoglycosides if the mutation is found. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

18.
Front Neurol ; 15: 1448989, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39268064

RESUMO

Introduction: Video head-impulse tests (video-HITs) often fail to detect anterior inferior cerebellar artery (AICA) infarction due to peripheral and central vestibular system involvement. Anecdotal studies suggest that video-HITs may reveal bilateral impairment in AICA infarction. However, the diagnostic utility of video-HITs has not been established, particularly when compared to labyrinthitis, which accounts for the majority of acute audiovestibular syndrome (AAVS) cases. Methods: We reviewed the medical records of consecutive patients presenting with new-onset acute hearing loss and spontaneous vertigo (i.e., AAVS) between March 2018 and July 2023 at a tertiary hospital in South Korea. Video-HIT patterns were categorized as follows: (1) ipsilaterally positive, (2) contralaterally positive, (3) bilaterally normal, and (4) bilaterally positive. Results: Twenty-eight patients with AICA infarction (mean age ± standard deviation = 67 ± 15 years; 14 men) and 51 with labyrinthitis (63 ± 17 years, 26 men) were included in the analyses. Among the 28 patients with AICA infarction, 15 presented with AAVS in isolation, without other co-morbid neurologic deficits (15/28, 54%). The vestibulo-ocular reflex (VOR) gains of ipsilesional horizontal canals (HCs) ranged from 0.21 to 1.22 (median = 0.81, interquartile range [IQR] = 0.50-0.89). However, those for contralateral HC gain ranged from 0.57 to 1.19 (median = 0.89 [IQR = 0.73-0.97]). Collectively, HITs were bilaterally positive in 13 patients (including 12 patients with bilaterally positive HITs for the horizontal canal), normal in eight, ipsilesionally positive in six, and contralesionally positive in one patient with AICA infarction. The VOR gains were typically decreased ipsilaterally in 28 (28/51, 55%), normal in 17 (17/51, 33%), and decreased bilaterally in six patients with labyrinthitis (6/51, 12%). Logistic regression analysis revealed that bilaterally positive HITs (p = 0.004) and multiple vascular risk factors (p = 0.043) were more frequently associated with AICA infarction than labyrinthitis. Discussion: Among patients presenting with AAVS, bilaterally positive HITs can be indicative of AICA infarction in patients with multiple vascular risk factors.

19.
World J Otorhinolaryngol Head Neck Surg ; 10(3): 206-212, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39233854

RESUMO

Objective: To assess the effect of selective serotonin reuptake inhibitors (SSRIs) and tricyclic antidepressants (TCAs) in reducing vertigo, tinnitus, and hearing loss among patients with Meniere's disease (MD). Data Sources: The following databases were utilized in this scoping review: Ovid Medline, PubMed-NCBI, CINAHL, Cochrane Library, Web of Science, and Clinicaltrials.gov. Method: Studies were identified through the following search phrases: "serotonin specific reuptake inhibitors" OR "tricyclic antidepressants" AND "Meniere's disease." References from included manuscripts were examined for possible inclusion of additional studies. Results: The literature search yielded 23 results, which were screened by three independent reviewers. Seventeen studies and three duplicates were excluded. An examination of references from the included studies yielded two additional publications. A total of four published studies assessing SSRIs and TCAs among 147 patients with MD were ultimately included. Four studies described significant reductions in vertigo attack frequency among patients treated with either SSRIs or TCAs compared to their pretreatment baseline. Three studies assessed the drugs' effects on hearing, of which none found a significant difference among patients treated with SSRIs or TCAs. One study found a significant decrease in patient-reported tinnitus following treatment with TCAs or SSRIs compared to their pretreatment baseline. Conclusions: Data exploring SSRIs and TCAs among patients with MD suggests that these medications may reduce the frequency of tinnitus and vertigo, although there was significant heterogeneity in outcome reporting. There remains a need for larger-scale prospective studies that emphasize objective data to evaluate their effectiveness in reducing common MD symptoms.

20.
Cureus ; 16(8): e66120, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39229405

RESUMO

Musical hallucinations (MH) represent a rare and complex auditory phenomenon where individuals perceive music without external stimuli. This case study explores auditory Charles Bonnet syndrome (ACBS) in a 51-year-old male with a history of bilateral sensorineural hearing loss. The patient reported hearing recognizable prayer chants, initially perceived as external sounds from a nearby temple. Over time, these hallucinations persisted and interfered with his daily activities, prompting medical consultation. Despite the absence of psychiatric illness, the patient was diagnosed with ACBS and treated with risperidone, an atypical antipsychotic. The intervention led to a significant reduction in the frequency and intensity of the hallucinations, alongside improved sleep and concentration. The patient also experienced a recurrence of symptoms upon discontinuation of the medication, highlighting the importance of adherence to treatment. This case underscores the need for awareness and understanding of non-psychotic auditory hallucinations in individuals with hearing impairments. The pathophysiology of MH is not fully understood but is believed to involve abnormal activity in the auditory associative cortices due to sensory deprivation. Treatment approaches often include both pharmacological and non-pharmacological strategies, such as optimizing hearing with aids and providing psychoeducation. This study contributes to the limited literature on ACBS and emphasizes the efficacy of antipsychotics in managing MH. Further research is essential to explore the underlying mechanisms and to develop comprehensive management plans for patients experiencing these distressing auditory phenomena. The findings advocate for a multidisciplinary approach to treatment, integrating audiological and psychiatric care to improve patient outcomes.

SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...