X-linked deafness/incomplete partition type 3: Radiological evaluation of temporal bone and intracranial findings

PURPOSE:X-linked deafness (XLD) is a rare disease, characterized by typical cochlear incomplete partition type 3 anomaly (IP-III). Accompanying hypothalamic anomalies were also recently described. The purpose of this study was to document the temporal bone and intracranial imaging findings in a series of patients with XLD with a review of the literature, to better understand this anomaly.METHODS:The CT and MRI studied of 13 XLD patients were retrospectively evaluated. All structures of the otic capsule (OC) were subjectively and retrospectively assessed. The OC thickness and the size of the cochlea were measured and compared to the age-matched control group. Intracranial structures were also evaluated with specific attention to the hypothalamic region.RESULTS:All cases had bilateral IP-III anomaly, bulbous internal auditory canals (IACs), absent bony modiolus with preserved interscalar septa, intact cochleovestibular, and facial nerves. OC thickness was decreased in all cases compared to the control group (p<0.001). In XLD patients, the cochlea had decreased transverse dimension and increased height compared to the control group (p< 0.001). Five patients (38.4%) had bilateral cystic structures adjacent to the vestibule and/or semicircular canals (SCCs). Hypothalamus was thickened or had a lobular appearance in all cases (subtle in one). Additionally, hamartoma-like appearance of the hypothalamus was present in half.CONCLUSION:XLD is a rare inner ear anomaly that is frequently associated with hypothalamic malformations. The OC thickness of IP-III patients appears to be decreased with accompanying decreased transverse dimension of the cochlea which could have implications in electrode selection during cochlear implantation. Cystic /diverticular lesions surrounding the vestibule and semicircular canals are also frequently seen but a rarely reported finding

CT dose management for neurologic events in patients with cardiac devices: Radiation exposure variation in patients with cardiac devices

PURPOSE:To compare the inter-center cranial computed tomography (CT) acquisition rates, CT findings, CT related radiation dose, and variability of CT acquisition parameters for neurologic events among patients with implantable cardioverter-defibrillator (ICD) or left ventricular assist device (LVAD).METHODS:A total of 224 patients [ICD group (n = 155) and LVAD group (n = 69)] who had at least one cranial CT scan were enrolled from three medical centers. The variability and effect of the number, indication, and findings of cranial CT scans as well as CT acquisition parameters including tube potential, tube current, tube rotation time (TI), slice collimation (cSL), and spiral or sequential scanning techniques on CT dose index volume (CTDIvol), total dose length product (DLP) were analyzed.RESULTS:The mean DLP value of Center A and mean CTDIvol values of Center A and C were significantly lower than Center B (p < 0.001). The mean CTDIvol and DLP values in the ICD group were substantially lower than the LVAD group (p<0.001). The most potent parameters causing the changes in CTDIvol and DLP were kV, mAs values, and CT scanning technique as sequential or spiral according to multivariate linear regression analysis.CONCLUSION:Cranial CT acquisition parameters and radiation doses vary significantly between centers, which necessitates optimization of cranial CT protocols to overcome the cumulative radiation dose burden in patients with neurologic events

Quality of life and related factors among chronic hepatitis B-infected patients: a multi-center study, Turkey

Background: The aim of this study was to assess health-related quality of life (HRQOL) among chronic hepatitis B (CHB) patients in Turkey and to study related factors

Quality of life and related factors among chronic hepatitis B-infected patients: a multi-center study, Turkey

balkan, ilker inanc/0000-0002-8977-5931; Altindis, Mustafa/0000-0003-0411-9669; Sahin, Ahmet Ziyaettin/0000-0003-1060-6746; atilla, aynur/0000-0001-8027-1991WOS: 000386954300001PubMed: 27809934Background: The aim of this study was to assess health-related quality of life (HRQOL) among chronic hepatitis B (CHB) patients in Turkey and to study related factors. Methods: This multicenter study was carried out between January 01 and April 15, 2015 in Turkey in 57 centers. Adults were enrolled and studied in three groups. Group 1: Inactive HBsAg carriers, Group 2: CHB patients receiving antiviral therapy, Group 3: CHB patients who were neither receiving antiviral therapy nor were inactive HBsAg carriers. Study data was collected by face-to-face interviews using a standardized questionnaire, Short Form-36 (SF-36) and Hepatitis B Quality of Life (HBQOL). Values equivalent to p < 0.05 in analyses were accepted as statistically significant. Results: Four thousand two hundred fifty-seven patients with CHB were included in the study. Two thousand five hundred fifty-nine (60.1 %) of the patients were males. Groups 1, 2 and 3, consisted of 1529 (35.9 %), 1721 (40.4 %) and 1007 (23.7 %) patients, respectively. The highest value of HRQOL was found in inactive HBsAg carriers. We found that total HBQOL score increased when antiviral treatment was used. However, HRQOL of CHB patients varied according to their socio-demographic properties. Regarding total HBQOL score, a higher significant level of HRQOL was determined in inactive HBV patients when matched controls with the associated factors were provided. Conclusions: The HRQOL score of CHB patients was higher than expected and it can be worsen when the disease becomes active. Use of an antiviral therapy can contribute to increasing HRQOL of patients