Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations

Conclusion. We have demonstrated differences in cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. The differences in cortical processing patterns between these two cases may have been influenced by the differing clinical courses and pathogenesis of hearing loss due to genetic mutations. Our results suggest the importance of hearing during early childhood for the development of a normal cortical language network. Objectives. To investigate the cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. Methods: The cortical activity of two adult patients with known genetic mutations (GJB2, SLC26A4) was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task and compared with that of normal-hearing controls. Results: A patient with a GJB2 mutation showed activation in the right auditory association area [BA21, BA22], and the left auditory association area [BA42] even with visual language task; in contrast, a patient with an SLC26A4 mutation showed no significant activation in the corresponding area.ArticleACTA OTO-LARYNGOLOGICA. 131(11):1232-1236 (2011)journal articl

Impact of upgraded radiotherapy system on outcomes in postoperative head and neck squamous cell carcinoma patients

Background: This study was performed to evaluate the impact of upgrade of radiotherapy system, including launch of intensity-modulated radiation therapy (IMRT), on the therapeutic outcomes. Materials and methods: Patients with head and neck (H&N) squamous cell carcinoma (SCC) who underwent postoperative radiotherapy at our hospital between June 2009 and July 2019 were retrospectively reviewed. In July 2014, we converted the radiotherapy technique for these patients from a 3-dimensional conformal radiotherapy (3D-CRT) to IMRT, along with the adoption of a meticulous planning policy and a few advanced procedures, including online imaging guidance. Results: A total of 136 patients (57 treated with the previous system and 79 treated with the upgraded system) were reviewed. There were significantly more patients with extracapsular extension in the upgraded-system group than the previous-system group (p = 0.0021). There were significantly fewer patients with ≥ Grade 2 acute and late adverse events in the upgraded-system group than the previous-system group. The differences in progression-free survival (PFS), distant metastasis-free survival (DFFS), locoregional progression-free survival (LRPFS), and overall survival (OS) between the two groups were not statistically significant (p = 0.8962, 0.9926, 0.6244, and 0.4827, respectively). Multivariate analysis revealed that the upgrade had neither positive nor negative impact on survival outcomes. Extracapsular extension was independently associated with decreased LRPFS and OS (p = 0.0499 and 0.0392, respectively). Conclusions: The IMRT-centered upgrade was beneficial for the postoperative patients with H&N SCC, because survival outcomes were sustained with less toxicities.

Differences between acoustic trauma and other types of acute noise-induced hearing loss in terms of treatment and hearing prognosis

Objectives: To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews.Methods: The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group.Results: Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p = .030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant.Conclusions: Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects

Epidemiological survey of acute low-tone sensorineural hearing loss

Objectives: A nationwide epidemiological survey involving 23 hospitals in Japan was conducted and the predictive values of demographic data were examined statistically.Methods: A total of 642 patients from 23 hospitals, including 20 university hospitals, in Japan were enrolled in the study. Age ranged from 8 to 87 years, and all were diagnosed with acute low-tone sensorineural hearing loss (ALHL) between 1994 and 2016. Demographic data for the patients, such as symptoms, gender, mean age, and distribution of ALHL grading, were collected and analyzed in relation to prognosis using Student’s t-test, χ2 test and logistic regression.Results: Female gender (p < .013), younger age (p < .001), low-grade hearing loss (p < .001), and shorter interval between onset and initial visit (p < .004) were significantly predictive of a good prognosis. The prognosis for definite ALHL was significantly better than that for probable ALHL (p < .007).Conclusions: The severity of initial hearing loss, interval between onset and initial visit and age were important prognostic indicators for ALHL, while female gender was an important prognostic indicator peculiar to ALHL

The effect of initial treatment on hearing prognosis in idiopathic sudden sensorineural hearing loss : a nationwide survey in Japan

Objective: To investigate the hearing prognosis of idiopathic sudden sensorineural hearing loss (SSNHL) treated with different initial therapies.Methods: Subjects consisted of patients diagnosed with idiopathic SSNHL within 7 days from onset and showing severe hearing loss (≥60 dB), who were registered in a Japanese multicenter database between April 2014 and March 2016. Subjects were divided into four groups according to initial therapy: (1) steroids, (2) steroids + Prostaglandins (PGs), (3) intratympanic steroids (ITS), and (4) no steroids. Hearing outcomes were compared among the groups.Results: In total, 1305 patients were enrolled. The final hearing level and hearing gain of patients treated with steroids + PGs were significantly higher than those of patients treated with steroids alone or no steroids. The ratio of good prognosis (complete recovery or marked improvement) in patients treated with steroids + PGs was higher than that in patients treated with steroids alone or no steroids. There was no difference in the prognosis of patients treated with steroids alone or no steroids.Conclusion: A large number of patients with idiopathic SSNHL were registered in a multicenter database. PG use in combination with steroid administration was associated with a good hearing prognosis in patients with severe hearing loss

Idiopathic sudden sensorineural hearing loss and acute low-tone sensorineural hearing loss: a comparison of the results of a nationwide epidemiological survey in Japan

Objectives: The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis.Methods: Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis.Results: Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent.Conclusion: The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease

A Case of Epidermolysis Bullosa Acquisita Associated with Laryngeal Stenosis

ArticleACTA DERMATO-VENEREOLOGICA.92(1):93-94(2012)journal articl

Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2

CONCLUSION. We have demonstrated differences in cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. The differences in cortical processing patterns between these two cases may have been influenced by the differing clinical courses and pathogenesis of hearing loss due to genetic mutations. Our results suggest the importance of hearing during early childhood for the development of a normal cortical language network. OBJECTIVES. To investigate the cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. METHODS: The cortical activity of two adult patients with known genetic mutations (GJB2, SLC26A4) was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task and compared with that of normal-hearing controls. RESULTS: A patient with a GJB2 mutation showed activation in the right auditory association area [BA21, BA22], and the left auditory association area [BA42] even with visual language task; in contrast, a patient with an SLC26A4 mutation showed no significant activation in the corresponding area