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Sample records for acute sensorineural impairment

  1. Idiopathic Sudden Sensorineural Hearing Loss With Minimal Hearing Impairment

    PubMed Central

    Cho, Chin Saeng

    2015-01-01

    Objectives The aim of the study was to determine the characteristics of patients who did not match the audiometric criteria of idiopathic sudden sensorineural hearing loss (SSNHL) but complained of acute hearing loss. Methods By thorough medical chart reviews, historical cohort study was performed with consecutive data of 589 patients complaining of acute unilateral sensorineural hearing loss without identifiable causes between 2005 and 2013. Those patients demonstrating a hearing loss of at least 30 dB at three consecutive frequencies based on pure tone audiometry were classified as group I; the others were classified as group II. Patients' characteristics, final hearing, and hearing improvement rate (HIR) between the two groups were compared. Results Group II exhibited distinctive characteristics, including an early age of onset of the hearing loss (P<0.01), an absence of accompanying diabetes (P<0.01) and hypertension (P<0.01), and better unaffected hearing and final hearing compared with group I (P<0.001). However, the HIR of the patients in the two groups was not significantly different (P>0.05). Conclusion Patients who did not meet the audiological criteria of SSNHL exhibited distinctive characteristics compared to SSNHL patients. PMID:26622953

  2. Acute Wernicke encephalopathy and sensorineural hearing loss complicating bariatric surgery.

    PubMed

    Jethava, Ashif; Dasanu, Constantin A

    2012-01-01

    Health-care professionals must be aware of the mandatory vitamin supplementation in patients status post bariatric surgery. A recent increase in the number of gastric bypass surgeries in US has been associated with a proportional increase in Wernicke encephalopathy reports. Subtle or atypical neurologic features are not uncommon. Our report is of a female patient with acute Wernicke encephalopathy accompanied by sensorineural hearing loss six weeks after bariatric surgery. The patient had only a partial recovery of her neurologic symptoms eightweeks after vigorous therapy for this condition. Symptomatic thiamine (vitamin B1) and vitamin B12 deficiencies are particularly concerning effects of bariatric procedures, as neurologic and cognitive deficits may be long lasting or even permanent despite aggressive replacement therapy.

  3. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2

    SciTech Connect

    Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R.

    1994-10-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at {theta} = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3{prime} to exon 50 of the dystrophin gene) and at DXS1068 (5{prime} to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5{prime}DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. 57 refs., 6 figs., 1 tab.

  4. Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population.

    PubMed

    Riga, M; Psarommatis, I; Lyra, Ch; Douniadakis, D; Tsakanikos, M; Neou, P; Apostolopoulos, N

    2005-04-01

    Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.

  5. [The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Skryabina, L Yu; Barkhatova, O A

    2016-01-01

    The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

  6. AICA syndrome with facial palsy following vertigo and acute sensorineural hearing loss.

    PubMed

    Ikegami-Takada, Tomoko; Izumikawa, Masahiko; Doi, Tadashi; Takada, Yohei; Tomoda, Koichi

    2012-04-01

    We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease.

  7. Acupuncture as a primary and independent treatment in the acute phases of sudden sensorineural hearing loss

    PubMed Central

    Jin, Yuanyuan; Lu, Ming

    2016-01-01

    Abstract Sudden sensorineural hearing loss (SSHL) is an otological emergency defined as a rapid hearing loss, seriously affects patient's social life. To data, no study has reported the treatment by acupuncture alone in the acute phase. In this report, Acupuncture and Moxibustion therapy of excitation-focus transfer is outlined. The patient was a 26-year-old young woman who had an SSHL coupled with ear fullness. The patient had no past medical history, but she had undergone variable emotions and had a history of excessive noise exposure. The patient refused to receive any medicine especially steroids and hyperbaric oxygen therapy. She just only received acupuncture treatment. Her symptoms and outcome measurements were improved every week and completely recovered after the last week. Even though the article presents a single case and is based on self-reports, there are very clear trends on how patients with SSHL responded to acupuncture treatments. PMID:27368045

  8. [The expertise of health status dynamics in the members of locomotive crews presenting with occupational sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Bulatskaia, T V; Merkulova, E P

    2013-01-01

    The objective of the present study was to estimate the diagnostic value of the characteristics of somatic pathology included in the algorithm for early diagnostics of occupational sensorineural impairment of hearing (OSNIH). The relationship between the development of the main nosological forms of somatic pathology as possible markers of internal ear diseases and professional sensorineural impairment of hearing was elucidated among the members of locomotive crews (n=224). The parameters of interest were evaluated 4 years before, within 1 year, and 4 years after the establishment of diagnosis of OSNIH. It was shown that the members of locomotive crews most frequently suffer atherosclerosis (20.0%), gastrointestinal disorders (34.8%), arterial hypertension (25.0%), osteochondrosis (largely vertebrogenic lumbodynia (21.3%)), and microcirculatory disturbances in the form of vascular spasm in the fundus of the eye (32.9%). 97.6% of the subjects above 35 years of age presented with hypercholesterolemia. Comparative analysis of the occurrence of these nosological forms among the members of locomotive crews with hearing problems on the one hand and the normally functioning organ of hearing on the other hand has demonstrated that none of these pathologies can be regarded as a marker for early diagnosis of OSNIH. At the same time, overweight even in the subjects under 35 years of age should be taken into account when planning and implementing preventive measures.

  9. [Hereditary sensorineural hearing impairment and macrothrombocytopenia: a rare MYH9 gene mutation].

    PubMed

    Böttcher, A; Knecht, R; Busch, C-J; Lörincz, B B; Dalchow, C V

    2013-02-01

    We report on a rare case of an exon 16 mutation of the MYH9 gene in a 23-year-old woman. This gene encodes for non-muscular myosin IIA, which acts as a cytoskeletal contractile protein in diverse cell types. This disorder led to sensorineural hearing loss, macrothrombocytopenia, and proteinuria. MYH9 gene mutation can lead to diverse organ manifestation like pre-senile cataract or renal failure which are progressive in course. Due to the current lack of causal treatment, diagnostic steps, advice for follow-up examinations and symptomatic therapy approaches are presented.

  10. Multicenter audiometric results with the Vibrant Soundbridge, a semi-implantable hearing device for sensorineural hearing impairment.

    PubMed

    Snik, A F; Mylanus, E A; Cremers, C W; Dillier, N; Fisch, U; Gnadeberg, D; Lenarz, T; Mazolli, M; Babighian, G; Uziel, A S; Cooper, H R; O'Connor, A F; Fraysse, B; Charachon, R; Shehata-Dieler, W E

    2001-04-01

    The Vibrant Soundbridge, a semi-implantable hearing device for subjects with moderate to severe sensorineural hearing impairment was introduced commercially. First audiologic results are presented on 63 patients from 10 European implant centers. Hearing loss was at 0.5, 1, 2, and 4 kHz varying between 43 and 81 dB HL. The patients used the analogue audio processor, type 302. Measured sound-field gain was compared with NAL-R target values. For most patients an acceptable agreement was found. There was a subgroup of patients, however, with relatively low gain. The results suggest that this was related to the suboptimal positioning and fixation of the transducer to the incus.

  11. Sensorineural hearing loss: a complication of acute otitis media in adults.

    PubMed

    Park, Joo Hyun; Park, Sung Joon; Kim, Young Ho; Park, Min-Hyun

    2014-07-01

    We aim to evaluate the incidence and clinical manifestations of sensorineural hearing loss (SNHL) in adult patients with acute otitis media (AOM). Seventy-five patients (age > 18 years; 83 ears) diagnosed with AOM between January 2008 and March 2011 at our clinic were enroled and retrospectively reviewed. We detected audiometrically confirmed SNHL during the course of AOM in eight patients. The clinical course, treatment, and audiometric final outcome of each case were reviewed. SNHL was associated with AOM in 8 out of 83 ears (9.3%). The mean age of patients was 57.5 years, and the mean follow-up period was 21.1 months (range 0.6-46.3 months). The most common symptom was tinnitus. Mean bone conduction hearing threshold was 39.5 dB in pure tone audiometry. All patients showed high-frequency HL, and three showed pan-frequency HL. All patients were treated with oral antibiotics at the initial visit. Seven ears were treated with a combination of oral steroids. Myringotomy was also performed. Seven of eight patients showed improvement; however, 8 kHz thresholds were not improved. This suggested that the inflammation spread through the round window. The mean duration of recovery was 18.6 days. SNHL associated with AOM in adult patients occurs during the early phases of the disease course. High-frequency hearing was commonly affected and was well treated with oral antibiotics, myringotomy, and steroid therapy. Audiometry can be helpful for treating adult patients with AOM. Active treatment, including myringotomy, should be performed during the early phase, if SNHL is suspected.

  12. Short-Term Outcomes of Acute Low-Tone Sensorineural Hearing Loss According to Treatment Modality

    PubMed Central

    Chang, Jinkyung; Yum, Gunhwee; Im, Ha-Young; Jung, Jong Yoon; Rah, Yoon Chan

    2016-01-01

    Background and Objectives We compared improvements in hearing thresholds in acute low-tone sensorineural hearing loss (ALHL) patients after two different treatments: steroid alone and steroid and diuretic combined. We analyzed how the duration between the onset of symptoms and the initiation of treatment affected hearing loss improvement and investigated the relation between presence of vertigo in ALHL patients and ALHL progression to Ménière's disease (MD). Subjects and Methods We retrospectively analyzed the medical records of 47 ALHL patients aged 21 to 76 years. Patients received either orally administered steroid alone (n=12) or steroid and diuretic combined (n=35). We compared improvements in the two groups' hearing thresholds at three lower frequencies (125, 250, and 500 Hz) after participants had received one month of each respective treatment. Results Our two treatments did not show any statistical difference in hearing loss improvement after one month. Forty percent of ALHL patients with vertigo developed MD, which was a significantly higher rate than the 12.5% of ALHL patients without vertigo who developed MD. The shorter duration between the onset of symptoms and the initiation of treatment significantly increased improvement in the sum of lower frequency hearing threshold after one month. Conclusions The current study suggests that steroid and diuretic administered together and steroid alone similarly improve the hearing threshold in ALHL patients after one month. We concluded that patients should initiate ALHL treatment as soon as they experience symptoms. ALHL patients should also be notified of their higher risk of developing MD. PMID:27144234

  13. The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

    PubMed

    Kletke, S; Batmanabane, V; Dai, T; Vincent, A; Li, S; Gordon, K A; Papsin, B C; Cushing, S L; Héon, E

    2016-10-15

    The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL.

  14. The Prevalence and Characteristics of Tinnitus with Profound Sensori-Neural Hearing Impairment.

    ERIC Educational Resources Information Center

    Drukier, Gale S.

    1989-01-01

    Of 331 children (aged 6-18) with profound hearing impairment, 96 were found to have tinnitus. More females than males reported tinnitus. Most of the children with tinnitus were bothered to some degree by it and indicated that the noises adversely affected their ability to hear voices. (JDD)

  15. Altered Contralateral Auditory Cortical Morphology in Unilateral Sudden Sensorineural Hearing Loss

    PubMed Central

    Fan, Wenliang; Zhang, Wenjuan; Li, Jing; Zhao, Xueyan; Mella, Grace; Lei, Ping; Liu, Yuan; Wang, Haha; Cheng, Huamao; Shi, Hong; Xu, Haibo

    2015-01-01

    Objective: To investigate the cerebral gray matter volume alterations in unilateral sudden sensorineural hearing loss patients within the acute period by the voxel-based morphometry method, and to determine if hearing impairment is associated with regional gray matter alterations in unilateral sudden sensorineural hearing loss patients. Study Design: Prospective case study. Setting: Tertiary class A teaching hospital. Patients: Thirty-nine patients with left-side unilateral sudden sensorineural hearing loss and 47 patients with right-side unilateral sudden sensorineural hearing loss. Intervention: Diagnostic. Main Outcome Measure: To compare the regional gray matter of unilateral sudden sensorineural hearing loss patients and healthy control participants. Results: Compared with control groups, patients with left side unilateral sudden sensorineural hearing loss had significant gray matter reductions in the right middle temporal gyrus and right superior temporal gyrus, whereas patients with right side unilateral sudden sensorineural hearing loss showed gray matter decreases in the left superior temporal gyrus and left middle temporal gyrus. A significant negative correlation with the duration of the sudden sensorineural hearing loss (R = −0.427, p = 0.012 for left-side unilateral SSNHL and R = −0.412, p = 0.013 for right-side unilateral SSNHL) was also found in these brain areas. There was no region with increased gray matter found in both groups of unilateral sudden sensorineural hearing loss patients. Conclusions: This study confirms that detectable decreased contralateral auditory cortical morphological changes have occurred in unilateral SSNHL patients within the acute period by voxel-based morphometry methods. The gray matter volumes of these brain areas also perform a negative correlation with the duration of the disease, which suggests a gradual brain structural impairment after the progression of the disease. PMID:26595717

  16. A novel mutation in the mitochondrial tRNA(Ser(UCN)) gene in a family with non-syndromic sensorineural hearing impairment.

    PubMed

    Hutchin, T P; Parker, M J; Young, I D; Davis, A C; Pulleyn, L J; Deeble, J; Lench, N J; Markham, A F; Mueller, R F

    2000-09-01

    We describe a family with non-syndromic sensorineural hearing impairment inherited in a manner consistent with maternal transmission. Affected members were found to have a novel heteroplasmic mtDNA mutation, T7510C, in the tRNA(Ser(UCN)) gene. This mutation was not found in 661 controls, is well conserved between species, and disrupts base pairing in the acceptor stem of the tRNA, making it the probable cause of hearing impairment in this family. Sequencing of the other mitochondrial tRNA genes did not show any other pathogenic mutations. Four other mutations causing hearing impairment have been reported in the tRNA(Ser(UCN)) gene, two having been shown to affect tRNA(Ser(UCN)) levels. With increasing numbers of reports of mtDNA mutations causing hearing impairment, screening for such mutations should be considered in all cases unless mitochondrial inheritance can be excluded for certain.

  17. Mitochondrial DNA variant m.15218A > G in Finnish epilepsy patients who have maternal relatives with epilepsy, sensorineural hearing impairment or diabetes mellitus

    PubMed Central

    2013-01-01

    Background Mitochondrial diseases caused by mutations in mitochondrial DNA (mtDNA) affect tissues with high energy demand. Epilepsy is one of the manifestations of mitochondrial dysfunction when the brain is affected. We have studied here 79 Finnish patients with epilepsy and who have maternal first- or second-degree relatives with epilepsy, sensorineural hearing impairment or diabetes mellitus. Methods The entire mtDNA was studied by using conformation sensitive gel electrophoresis and PCR fragments that differed in mobility were directly sequenced. Results We found a common nonsynonymous variant m.15218A > G (p.T158A, MTCYB) that occurs in haplogroup U5a1 to be more frequent in patients with epilepsy. The m.15218A > G variant was present in five patients with epilepsy and in four out of 403 population controls (p = 0.0077). This variant was present in two branches in the phylogenetic network constructed on the basis of mtDNA variation among the patients. Three algorithms predicted that m.15218A > G is damaging in effect. Conclusions We suggest that the m.15218A > G variant is mildly deleterious and that mtDNA involvement should be considered in patients with epilepsy and who have a maternal history of epilepsy, sensorineural hearing impairment or diabetes mellitus. PMID:23870133

  18. Sensorineural Organs Dysfunction and Cognitive Decline: A Review Article

    PubMed Central

    Wongrakpanich, Supakanya; Petchlorlian, Aisawan; Rosenzweig, Andrew

    2016-01-01

    Vision, hearing, olfaction, and cognitive function are essential components of healthy and successful aging. Multiple studies demonstrate relationship between these conditions with cognitive function. The present article focuses on hearing loss, visual impairment, olfactory loss, and dual sensory impairments in relation to cognitive declination and neurodegenerative disorders. Sensorineural organ impairment is a predictive factor for mild cognitive impairment and neurodegenerative disorders in the elderly. We recommend early detection of sensorineural dysfunction by history, physical examination, and screening tests. Assisted device and early cognitive rehabilitation may be beneficial. Future research is warranted in order to explore advanced treatment options and method to slow progression for cognitive declination and sensorineural organ impairment. PMID:28053826

  19. Homozygosity for the V37I GJB2 mutation in fifteen probands with mild to moderate sensorineural hearing impairment: further confirmation of pathogenicity and haplotype analysis in Asian populations.

    PubMed

    Gallant, Emily; Francey, Lauren; Tsai, Ellen A; Berman, Micah; Zhao, Yaru; Fetting, Heather; Kaur, Maninder; Deardorff, Matthew A; Wilkens, Alisha; Clark, Dinah; Hakonarson, Hakon; Rehm, Heidi L; Krantz, Ian D

    2013-09-01

    Hearing impairment affects 1 in 650 newborns, making it the most common congenital sensory impairment. Autosomal recessive nonsyndromic sensorineural hearing impairment (ARNSHI) comprises 80% of familial hearing impairment cases. Mutations in GJB2 account for a significant number of ARNSHI (and up to 50% of documented recessive (e.g., more than 1 affected sibling) hearing impairment in some populations). Mutations in the GJB2 gene are amongst the most common causes of hearing impairment in populations of various ethnic backgrounds. Two mutations of this gene, 35delG and 167delT, account for the majority of reported mutations in Caucasian populations, especially those of Mediterranean and Ashkenazi Jewish background. The 235delC mutation is most prevalent in East Asian populations. Some mutations are of less well-characterized significance. The V37I missense mutation, common in Asian populations, was initially described as a polymorphism and later as a potentially pathogenic mutation. We report here on 15 unrelated individuals with ARNSHI and homozygosity for the V37I GJB2 missense mutation. Nine individuals are of Chinese ancestry, two are of unspecified Asian descent, one is of Japanese descent, one individual is of Vietnamese ancestry, one of Philippine background and one of Italian and Cuban/Caucasian background. Homozygosity for the V37I GJB2 mutation may be a more common pathogenic missense mutation in Asian populations, resulting in mild to moderate sensorineural hearing impairment. We report a presumed haplotype block specific to East Asian individuals with the V37I mutation encompassing the GJB2 gene that may account for the high prevalence in East Asian populations.

  20. Acute stress impairs set-shifting but not reversal learning.

    PubMed

    Butts, K A; Floresco, S B; Phillips, A G

    2013-09-01

    The ability to update and modify previously learned behavioral responses in a changing environment is essential for successful utilization of promising opportunities and for coping with adverse events. Valid models of cognitive flexibility that contribute to behavioral flexibility include set-shifting and reversal learning. One immediate effect of acute stress is the selective impairment of performance on higher-order cognitive control tasks mediated by the medial prefrontal cortex (mPFC) but not the hippocampus. Previous studies show that the mPFC is required for set-shifting but not for reversal learning, therefore the aim of the present experiment is to assess whether exposure to acute stress (15 min of mild tail-pinch stress) given immediately before testing on either a set-shifting or reversal learning tasks would impair performance selectively on the set-shifting task. An automated operant chamber-based task, confirmed that exposure to acute stress significantly disrupts set-shifting but has no effect on reversal learning. Rats exposed to an acute stressor require significantly more trials to reach criterion and make significantly more perseverative errors. Thus, these data reveal that an immediate effect of acute stress is to impair mPFC-dependent cognition selectively by disrupting the ability to inhibit the use of a previously relevant cognitive strategy.

  1. Acute stress selectively impairs learning to act.

    PubMed

    de Berker, Archy O; Tirole, Margot; Rutledge, Robb B; Cross, Gemma F; Dolan, Raymond J; Bestmann, Sven

    2016-07-20

    Stress interferes with instrumental learning. However, choice is also influenced by non-instrumental factors, most strikingly by biases arising from Pavlovian associations that facilitate action in pursuit of rewards and inaction in the face of punishment. Whether stress impacts on instrumental learning via these Pavlovian associations is unknown. Here, in a task where valence (reward or punishment) and action (go or no-go) were orthogonalised, we asked whether the impact of stress on learning was action or valence specific. We exposed 60 human participants either to stress (socially-evaluated cold pressor test) or a control condition (room temperature water). We contrasted two hypotheses: that stress would lead to a non-selective increase in the expression of Pavlovian biases; or that stress, as an aversive state, might specifically impact action production due to the Pavlovian linkage between inaction and aversive states. We found support for the second of these hypotheses. Stress specifically impaired learning to produce an action, irrespective of the valence of the outcome, an effect consistent with a Pavlovian linkage between punishment and inaction. This deficit in action-learning was also reflected in pupillary responses; stressed individuals showed attenuated pupillary responses to action, hinting at a noradrenergic contribution to impaired action-learning under stress.

  2. Acute stress selectively impairs learning to act

    PubMed Central

    de Berker, Archy O.; Tirole, Margot; Rutledge, Robb B.; Cross, Gemma F.; Dolan, Raymond J.; Bestmann, Sven

    2016-01-01

    Stress interferes with instrumental learning. However, choice is also influenced by non-instrumental factors, most strikingly by biases arising from Pavlovian associations that facilitate action in pursuit of rewards and inaction in the face of punishment. Whether stress impacts on instrumental learning via these Pavlovian associations is unknown. Here, in a task where valence (reward or punishment) and action (go or no-go) were orthogonalised, we asked whether the impact of stress on learning was action or valence specific. We exposed 60 human participants either to stress (socially-evaluated cold pressor test) or a control condition (room temperature water). We contrasted two hypotheses: that stress would lead to a non-selective increase in the expression of Pavlovian biases; or that stress, as an aversive state, might specifically impact action production due to the Pavlovian linkage between inaction and aversive states. We found support for the second of these hypotheses. Stress specifically impaired learning to produce an action, irrespective of the valence of the outcome, an effect consistent with a Pavlovian linkage between punishment and inaction. This deficit in action-learning was also reflected in pupillary responses; stressed individuals showed attenuated pupillary responses to action, hinting at a noradrenergic contribution to impaired action-learning under stress. PMID:27436299

  3. Dietary phosphorus acutely impairs endothelial function.

    PubMed

    Shuto, Emi; Taketani, Yutaka; Tanaka, Rieko; Harada, Nagakatsu; Isshiki, Masashi; Sato, Minako; Nashiki, Kunitaka; Amo, Kikuko; Yamamoto, Hironori; Higashi, Yukihito; Nakaya, Yutaka; Takeda, Eiji

    2009-07-01

    Excessive dietary phosphorus may increase cardiovascular risk in healthy individuals as well as in patients with chronic kidney disease, but the mechanisms underlying this risk are not completely understood. To determine whether postprandial hyperphosphatemia may promote endothelial dysfunction, we investigated the acute effect of phosphorus loading on endothelial function in vitro and in vivo. Exposing bovine aortic endothelial cells to a phosphorus load increased production of reactive oxygen species, which depended on phosphorus influx via sodium-dependent phosphate transporters, and decreased nitric oxide production via inhibitory phosphorylation of endothelial nitric oxide synthase. Phosphorus loading inhibited endothelium-dependent vasodilation of rat aortic rings. In 11 healthy men, we alternately served meals containing 400 mg or 1200 mg of phosphorus in a double-blind crossover study and measured flow-mediated dilation of the brachial artery before and 2 h after the meals. The high dietary phosphorus load increased serum phosphorus at 2 h and significantly decreased flow-mediated dilation. Flow-mediated dilation correlated inversely with serum phosphorus. Taken together, these findings suggest that endothelial dysfunction mediated by acute postprandial hyperphosphatemia may contribute to the relationship between serum phosphorus level and the risk for cardiovascular morbidity and mortality.

  4. Depressive Symptoms and Impaired Physical Function after Acute Lung Injury

    PubMed Central

    Colantuoni, Elizabeth; Mendez-Tellez, Pedro A.; Dinglas, Victor D.; Shanholtz, Carl; Husain, Nadia; Dennison, Cheryl R.; Herridge, Margaret S.; Pronovost, Peter J.; Needham, Dale M.

    2012-01-01

    Rationale: Survivors of acute lung injury (ALI) frequently have substantial depressive symptoms and physical impairment, but the longitudinal epidemiology of these conditions remains unclear. Objectives: To evaluate the 2-year incidence and duration of depressive symptoms and physical impairment after ALI, as well as risk factors for these conditions. Methods: This prospective, longitudinal cohort study recruited patients from 13 intensive care units (ICUs) in four hospitals, with follow-up 3, 6, 12, and 24 months after ALI. The outcomes were Hospital Anxiety and Depression Scale depression score greater than or equal to 8 (“depressive symptoms”) in patients without a history of depression before ALI, and two or more dependencies in instrumental activities of daily living (“impaired physical function”) in patients without baseline impairment. Measurements and Main Results: During 2-year follow-up of 186 ALI survivors, the cumulative incidences of depressive symptoms and impaired physical function were 40 and 66%, respectively, with greatest incidence by 3-month follow-up; modal durations were greater than 21 months for each outcome. Risk factors for incident depressive symptoms were education 12 years or less, baseline disability or unemployment, higher baseline medical comorbidity, and lower blood glucose in the ICU. Risk factors for incident impaired physical function were longer ICU stay and prior depressive symptoms. Conclusions: Incident depressive symptoms and impaired physical function are common and long-lasting during the first 2 years after ALI. Interventions targeting potentially modifiable risk factors (e.g., substantial depressive symptoms in early recovery) should be evaluated to improve ALI survivors’ long-term outcomes. PMID:22161158

  5. Acute stress impairs cognitive flexibility in men, not women.

    PubMed

    Shields, Grant S; Trainor, Brian C; Lam, Jovian C W; Yonelinas, Andrew P

    2016-09-01

    Psychosocial stress influences cognitive abilities, such as long-term memory retrieval. However, less is known about the effects of stress on cognitive flexibility, which is mediated by different neurobiological circuits and could thus be regulated by different neuroendocrine pathways. In this study, we randomly assigned healthy adults to an acute stress induction or control condition and subsequently assessed participants' cognitive flexibility using an open-source version of the Wisconsin Card Sort task. Drawing on work in rodents, we hypothesized that stress would have stronger impairing effects on cognitive flexibility in men than women. As predicted, we found that stress impaired cognitive flexibility in men but did not significantly affect women. Our results thus indicate that stress exerts sex-specific effects on cognitive flexibility in humans and add to the growing body of research highlighting the need to consider sex differences in effects of stress.

  6. Congenital sensorineural hearing loss

    SciTech Connect

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  7. Sudden sensorineural hearing loss.

    PubMed

    Stew, B T; Fishpool, S J C; Williams, H

    2012-02-01

    Sudden onset sensorineural hearing loss is a medical emergency that continues to be poorly understood despite being recognized in the literature since 1944 (De Kleyn, 1944). A commonly used criterion to qualify for this diagnosis is a sensorineural hearing loss over three contiguous pure-tone frequencies of 30 dB or more that develops within 72 hours. The vast majority of cases are unilateral and the estimated annual incidence is 20 per 100 000 persons (Nosrati-Zarenoe et al, 2007). A cause for the hearing loss is only identified in up to 10% of cases but 50% of patients will improve spontaneously (Penido et al, 2009).

  8. [Genotype--phenotype correlation limits in sensorineural hearing loss: case report of a three-year-old child with a bilateral cochleovestibular impairment and a molecular variant of the COCH gene].

    PubMed

    Montava, M; Roman, S; Sigaudy, S; Marlin, S; Nicollas, R; Triglia, J M

    2012-01-01

    Mutations of the COCH gene inherited in an autosomal dominant mode are responsible for late-onset cochleovestibular impairment on both sides. Our objective is to report the youngest patient (3 years) associating a molecular variant of the COCH gene and a cochleovestibular impairment on both sides. The clinical sequence has started with a vestibular dysfunction in a two-year-old child: recurrent rotatory dizziness during 12 months. At the age of 3, a sensorineural hearing loss on both sides has occured associated with spontaneous variation during 6 months. The lack of mutation of the connexin 26, connexin 30 and pendrin genes has reorientated the genetic investigation. A molecular variant of the COCH gene was found in the vWFA2 domain. It was an in-frame deletion predicting the synthesis of an abnormal protein in which 21 aminoacid were missing. Others family members with mutation were asymptomatics. In this isolated case report, the study was in favor of a non pathogenic molecular variant of the COCH gene. For all that, mutations of the COCH gene could be searched in progressive cochleovestibular dysfunctions on both sides in children, even without family affect.

  9. Disrupted functional brain connectome in unilateral sudden sensorineural hearing loss.

    PubMed

    Xu, Haibo; Fan, Wenliang; Zhao, Xueyan; Li, Jing; Zhang, Wenjuan; Lei, Ping; Liu, Yuan; Wang, Haha; Cheng, Huamao; Shi, Hong

    2016-05-01

    Sudden sensorineural hearing loss (SSNHL) is generally defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies and within a three-day period. This hearing loss is usually unilateral and can be associated with tinnitus and vertigo. The pathogenesis of unilateral sudden sensorineural hearing loss is still unknown, and the alterations in the functional connectivity are suspected to involve one possible pathogenesis. Despite scarce findings with respect to alterations in brain functional networks in unilateral sudden sensorineural hearing loss, the alterations of the whole brain functional connectome and whether these alterations were already in existence in the acute period remains unknown. The aim of this study was to investigate the alterations of brain functional connectome in two large samples of unilateral sudden sensorineural hearing loss patients and to investigate the correlation between unilateral sudden sensorineural hearing loss characteristics and changes in the functional network properties. Pure tone audiometry was performed to assess hearing ability. Abnormal changes in the peripheral auditory system were examined using conventional magnetic resonance imaging. The graph theoretical network analysis method was used to detect brain connectome alterations in unilateral sudden sensorineural hearing loss. Compared with the control groups, both groups of unilateral SSNHL patients exhibited a significantly increased clustering coefficient, global efficiency, and local efficiency but a significantly decreased characteristic path length. In addition, the primary increased nodal strength (e.g., nodal betweenness, hubs) was observed in several regions primarily, including the limbic and paralimbic systems, and in the auditory network brain areas. These findings suggest that the alteration of network organization already exists in unilateral sudden sensorineural hearing loss patients within the acute period

  10. Sudden sensorineural hearing loss.

    PubMed

    Schreiber, Benjamin E; Agrup, Charlotte; Haskard, Dorian O; Luxon, Linda M

    2010-04-03

    Sudden sensorineural hearing loss is usually unilateral and can be associated with tinnitus and vertigo. In most cases the cause is not identified, although various infective, vascular, and immune causes have been proposed. A careful examination is needed to exclude life threatening or treatable causes such as vascular events and malignant diseases, and patients should be referred urgently for further assessment. About half of patients completely recover, usually in about 2 weeks. Many treatments are used, including corticosteroids, antiviral drugs, and vasoactive and oxygen-based treatments. Although no treatment is proven, we recommend a short course of oral high-dose corticosteroids. There is much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are needed to establish evidence-based management.

  11. Sudden Sensorineural Hearing Loss

    PubMed Central

    Kuhn, Maggie; Heman-Ackah, Selena E.; Shaikh, Jamil A.

    2011-01-01

    Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram. PMID:21606048

  12. Acute stress impairs the retrieval of extinction memory in humans

    PubMed Central

    Raio, Candace M.; Brignoni-Perez, Edith; Goldman, Rachel; Phelps, Elizabeth A.

    2014-01-01

    Extinction training is a form of inhibitory learning that allows an organism to associate a previously aversive cue with a new, safe outcome. Extinction does not erase a fear association, but instead creates a competing association that may or may not be retrieved when a cue is subsequently encountered. Characterizing the conditions under which extinction learning is expressed is important to enhancing the treatment of anxiety disorders that rely on extinction-based exposure therapy as a primary treatment technique. The ventromedial prefrontal cortex, which plays an important role in the expression of extinction memory, has been shown to be functionally impaired after stress exposure. Further, recent research in rodents found that exposure to stress led to deficits in extinction retrieval, although this has yet to be tested in humans. To explore how stress might influence extinction retrieval in humans, participants underwent a differential aversive learning paradigm, in which one image was probabilistically paired with an aversive shock while the other image denoted safety. Extinction training directly followed, at which point reinforcement was omitted. A day later, participants returned to the lab and either completed an acute stress manipulation (i.e., cold pressor), or a control task, before undergoing an extinction retrieval test. Skin conductance responses and salivary cortisol concentrations were measured throughout each session as indices of fear arousal and neuroendocrine stress responses, respectively. The efficacy of our stress induction was established by observing significant increases in cortisol for the stress condition only. We examined extinction retrieval by comparing conditioned responses during the last trial of extinction (day 1) with that of the first trial of re-extinction (day 2). Groups did not differ on initial fear acquisition or extinction, however, one day later participants in the stress group (n = 27) demonstrated significantly less

  13. Acute stress impairs the retrieval of extinction memory in humans.

    PubMed

    Raio, Candace M; Brignoni-Perez, Edith; Goldman, Rachel; Phelps, Elizabeth A

    2014-07-01

    Extinction training is a form of inhibitory learning that allows an organism to associate a previously aversive cue with a new, safe outcome. Extinction does not erase a fear association, but instead creates a competing association that may or may not be retrieved when a cue is subsequently encountered. Characterizing the conditions under which extinction learning is expressed is important to enhancing the treatment of anxiety disorders that rely on extinction-based exposure therapy as a primary treatment technique. The ventromedial prefrontal cortex, which plays a critical role in the expression of extinction memory, has been shown to be functionally impaired after stress exposure. Further, recent work in rodents has demonstrated that exposure to stress leads to deficits in extinction retrieval, although this has yet to be tested in humans. To explore how stress might influence extinction retrieval in humans, participants underwent a differential aversive learning paradigm, in which one image was probabilistically paired with an aversive shock while the other image denoted safety. Extinction training directly followed, at which point reinforcement was omitted. A day later, participants returned to the lab and either completed an acute stress manipulation (i.e., cold pressor), or a control task, before undergoing an extinction retrieval test. Skin conductance responses and salivary cortisol concentrations were measured throughout each session as indices of fear arousal and neuroendocrine stress response, respectively. The efficacy of our stress induction was established by observing significant increases in cortisol for the stress condition only. We examined extinction retrieval by comparing conditioned responses during the last trial of extinction (day 1) with that of the first trial of re-extinction (day 2). Groups did not differ on initial fear acquisition or extinction, however, a day later participants in the stress group (n=27) demonstrated significantly

  14. Prospective mutation screening of three common deafness genes in a large Taiwanese Cohort with idiopathic bilateral sensorineural hearing impairment reveals a difference in the results between families from hospitals and those from rehabilitation facilities.

    PubMed

    Wu, Chen-Chi; Chen, Pei-Jer; Chiu, Yu-Hsun; Lu, Ying-Chang; Wu, Ming-Chueh; Hsu, Chuan-Jen

    2008-01-01

    Accurate epidemiological data on common deafness genes are essential to improve the efficiency and to reduce the cost of molecular diagnosis. They may depend on several factors, including a clear delineation of the source of patients being studied. In the present study, we hypothesize that patients with idiopathic sensorineural hearing loss recruited from different sources might reveal discrepancies in the epidemiological results of genetic screening, because patients from different sources might demonstrate distinct clinical or audiologic features and thus result in biased selection of subjects. To elucidate the relative importance of common deafness genes in Taiwanese and to verify our hypothesis, we conducted a prospective project screening mutations in GJB2, SLC26A4 and mitochondrial 12S rRNA gene in a total of 420 Taiwanese families with idiopathic bilateral sensorineural hearing loss, of which 325 families were recruited from hospitals and 95 from hearing rehabilitation facilities. Allele frequencies of common mutations in these three genes and distributions of the corresponding genotypes were then compared between the two groups. The allele frequencies of mutations in SLC26A4, GJB2 and mitochondrial 12S rRNA in the probands of the 420 families were 14.4, 21.7 and 3.8%, respectively. The allele frequency of SLC26A4 mutations in the hospital group was significantly higher than that in the rehabilitation facility group (16.2 vs. 8.4%, chi(2)-test, p < 0.05), whereas no difference in the frequencies of GJB2 mutations and mitochondrial 12S rRNA mutations was found between the two groups. Distributions of probands classified by SLC26A4 genotypes were also different between the two groups (chi(2)-test, p < 0.05). Accordingly, a discrepancy in the genetic screening results might exist between different sources of idiopathic hearing-impaired patients. Further analysis of audiological results and construction of a logistic regression model showed that different

  15. Spautin-1 Ameliorates Acute Pancreatitis via Inhibiting Impaired Autophagy and Alleviating Calcium Overload

    PubMed Central

    Xiao, Juan; Feng, Xueping; Huang, Xiao-Ying; Huang, Zhongshi; Huang, Yanqiang; Li, Chaogan; Li, Genliang; Nong, Song; Wu, Ruoshi; Huang, Yongzhi; Long, Xi-Dai

    2016-01-01

    Acute pancreatitis is characterized by zymogen preactivation. Severe inflammation caused by zymogen activation can eventually lead to multiple organ dysfunctions which contribute to the high mortality rate of severe acute pancreatitis. However, there is no specific treatment available for acute pancreatitis therapy. Here, we show that spautin-1, which effectively inhibits autophagy flux, ameliorated the pathogenesis of acute pancreatitis induced by cerulein or L-arginine. CaMKII phosphorylation due to cytosolic calcium overload was revealed in this paper. It was also demonstrated that autophagic protein aggregates degradation blockade accompanied by impaired autophagy correlated positively with intra-acinar cell digestive aymogen activation stimulated by cerulein or L-arginine. The role of spautin-1 in ameliorating acute pancreatitis was shown here to be associated with impaired autophagy inhibition and Ca2+ overload alleviation. We provide a promising therapy for acute pancreatitis through targeting both impaired autophagy and increased cytosolic calcium. PMID:27579473

  16. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss.

    PubMed

    Sekiya, Kenichi; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kakigi, Ryusuke; Pantev, Christo; Okamoto, Hidehiko

    2016-01-25

    Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL.

  17. Impairment of striatal mitochondrial function by acute paraquat poisoning.

    PubMed

    Czerniczyniec, Analía; Lanza, E M; Karadayian, A G; Bustamante, J; Lores-Arnaiz, S

    2015-10-01

    Mitochondria are essential for survival. Their primary function is to support aerobic respiration and to provide energy for intracellular metabolic pathways. Paraquat is a redox cycling agent capable of generating reactive oxygen species. The aim of the present study was to evaluate changes in cortical and striatal mitochondrial function in an experimental model of acute paraquat toxicity and to compare if the brain areas and the molecular mechanisms involved were similar to those observed after chronic exposure. Sprague-Dawley rats received paraquat (25 mg/Kg i.p.) or saline and were sacrificed after 24 h. Paraquat treatment decreased complex I and IV activity by 37 and 21 % respectively in striatal mitochondria. Paraquat inhibited striatal state 4 and state 3 KCN-sensitive respiration by 80 % and 62 % respectively, indicating a direct effect on respiratory chain. An increase of 2.2 fold in state 4 and 2.3 fold in state 3 in KCN-insensitive respiration was observed in striatal mitochondria from paraquat animals, suggesting that paraquat redox cycling also consumed oxygen. Paraquat treatment increased hydrogen peroxide production (150 %), TBARS production (42 %) and cardiolipin oxidation/depletion (12 %) in striatal mitochondria. Also, changes in mitochondrial polarization was induced after paraquat treatment. However, no changes were observed in any of these parameters in cortical mitochondria from paraquat treated-animals. These results suggest that paraquat treatment induced a clear striatal mitochondrial dysfunction due to both paraquat redox cycling reactions and impairment of the mitochondrial electron transport, causing oxidative damage. As a consequence, mitochondrial dysfunction could probably lead to alterations in cellular bioenergetics.

  18. Impairments of spatial working memory and attention following acute psychosocial stress.

    PubMed

    Olver, James S; Pinney, Myra; Maruff, Paul; Norman, Trevor R

    2015-04-01

    Few studies have investigated the effect of an acute psychosocial stress paradigm on impaired attention and working memory in humans. Further, the duration of any stress-related cognitive impairment remains unclear. The aim of this study was to examine the effect of an acute psychosocial stress paradigm, the Trier Social Stress, on cognitive function in healthy volunteers. Twenty-three healthy male and female subjects were exposed to an acute psychosocial stress task. Physiological measures (salivary cortisol, heart rate and blood pressure) and subjective stress ratings were measured at baseline, in anticipation of stress, immediately post-stress and after a period of rest. A neuropsychological test battery including spatial working memory and verbal memory was administered at each time point. Acute psychosocial stress produced significant increases in cardiovascular and subjective measures in the anticipatory and post-stress period, which recovered to baseline after rest. Salivary cortisol steadily declined over the testing period. Acute psychosocial stress impaired delayed verbal recall, attention and spatial working memory. Attention remained impaired, and delayed verbal recall continued to decline after rest. Acute psychosocial stress is associated with an impairment of a broad range of cognitive functions in humans and with prolonged abnormalities in attention and memory.

  19. Acute stress does not affect the impairing effect of chronic stress on memory retrieval

    PubMed Central

    Ozbaki, Jamile; Goudarzi, Iran; Salmani, Mahmoud Elahdadi; Rashidy-Pour, Ali

    2016-01-01

    Objective(s): Due to the prevalence and pervasiveness of stress in modern life and exposure to both chronic and acute stresses, it is not clear whether prior exposure to chronic stress can influence the impairing effects of acute stress on memory retrieval. This issue was tested in this study. Materials and Methods: Adult male Wistar rats were randomly assigned to the following groups: control, acute, chronic, and chronic + acute stress groups. The rats were trained with six trials per day for 6 consecutive days in the water maze. Following training, the rats were either kept in control conditions or exposed to chronic stress in a restrainer 6 hr/day for 21 days. On day 22, a probe test was done to measure memory retention. Time spent in target and opposite areas, platform location latency, and proximity were used as indices of memory retention. To induce acute stress, 30 min before the probe test, animals received a mild footshock. Results: Stressed animals spent significantly less time in the target quadrant and more time in the opposite quadrant than control animals. Moreover, the stressed animals showed significantly increased platform location latency and proximity as compared with control animals. No significant differences were found in these measures among stress exposure groups. Finally, both chronic and acute stress significantly increased corticosterone levels. Conclusion: Our results indicate that both chronic and acute stress impair memory retrieval similarly. Additionally, the impairing effects of chronic stress on memory retrieval were not influenced by acute stress. PMID:27635201

  20. Isolated contralateral sudden sensorineural hearing loss: an unusual manifestation of pontine infarct.

    PubMed

    Muttikkal, Thomas Jose Eluvathingal; Kesser, Bradley W; Mukherjee, Sugoto

    2014-09-01

    Unilateral, acute onset sensorineural hearing loss ("sudden sensorineural hearing loss" [SSNHL]) as an isolated event without other associated neurological deficits usually results from a lesion of the cochlea. Lesions in the ascending central auditory pathways cranial to the cochlear nucleus seldom result in unilateral hearing loss due to decussation of the central auditory pathways at multiple levels. We describe a patient with a tiny acute infarct in the right pons resulting in isolated acute onset left-sided SSHNL, without any other associated acute neurological deficits.

  1. Acute pentobarbital treatment impairs spatial learning and memory and hippocampal long-term potentiation in rats.

    PubMed

    Wang, Wei; Tan, Tao; Tu, Man; He, Wenting; Dong, Zhifang; Han, Huili

    2015-10-01

    Reports of the effects of pentobarbital on learning and memory are contradictory. Some studies have not shown any interference with learning and memory, whereas others have shown that pentobarbital impairs memory and that these impairments can last for long periods. However, it is unclear whether acute local microinjections of pentobarbital affect learning and memory, and if so, the potential mechanisms are also unclear. Here, we reported that the intra-hippocampal infusion of pentobarbital (8.0mM, 1μl per side) significantly impaired hippocampus-dependent spatial learning and memory retrieval. Moreover, in vitro electrophysiological recordings revealed that these behavioral changes were accompanied by impaired hippocampal CA1 long-term potentiation (LTP) and suppressed neuronal excitability as reflected by a decrease in the number of action potentials (APs). These results suggest that acute pentobarbital application causes spatial learning and memory deficits that might be attributable to the suppression of synaptic plasticity and neuronal excitability.

  2. [Acute bilateral impaired vision with central scotoma in an 11-year-old boy].

    PubMed

    Pollithy, S; Ach, T; Schaal, K B; Dithmar, S

    2012-09-01

    This article presents a case of acute bilateral impaired vision and central scotoma in an 11-year-old boy. Looking directly into a laser beam of a laser pointer for only a few seconds can cause retinal damage in the form of lesions of the retinal pigment epithelium and the photoreceptor layer, up to retinal hemorrhage. Patients often complain about impaired vision and a central scotoma of the affected eye.

  3. Reduced Acute Recovery from Alcohol Impairment in Adults with ADHD

    PubMed Central

    Roberts, Walter; Milich, Richard; Fillmore, Mark T.

    2013-01-01

    Rationale Prior research has found that adults with attention-deficit/hyperactivity disorder (ADHD) show increased sensitivity to the impairing effects of alcohol (Weafer et al. 2009). However, these studies have focused exclusively on the ascending limb of the blood alcohol concentration (BAC) curve, and it is unclear whether these adults continue to show increased sensitivity during the later phase of the dose as BAC is declining. Objective This study tested the hypothesis that those with ADHD would display increased response to alcohol during the ascending limb of the BAC curve and less recovery from the impairing effects during the descending limb. Methods Adult social drinkers with ADHD and control adults completed measures of motor coordination, reaction time, and subjective intoxication twice following 0.64 g/kg alcohol and placebo. The measures were administered during the ascending limb of the BAC curve and again during the descending limb. Results During the ascending limb, alcohol reduced motor coordination, slowed reaction time (RT), and increased self-reports of subjective intoxication. Those with ADHD displayed greater impairment of motor coordination compared with controls. During the descending limb, controls reported diminished subjective intoxication and showed recovery from the impairing effects of alcohol on both their motor coordination and their RT. Those with ADHD showed reduced subjective intoxication and faster RT during this time, but they did not recover motor control. Conclusions The protracted time course of motor impairment in adults with ADHD despite reductions in subjective intoxication may contribute to poor decision making and diminished behavioral control in this group. PMID:23430161

  4. Pediatric Sudden Sensorineural Hearing Loss.

    PubMed

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

    Sudden sensorineural hearing loss is defined as sudden unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is rare among children. The mechanism of the process and prognosis of the disorder remains unclear. The current incidence of sudden sensorineural hearing loss among pediatric population is unknown. The authors carried out a retrospective chart analysis of patients under 15 years of age from 2004 to 2015, who consulted to the Otolaryngology Head and Neck Surgery Department of Inonu University Medical Faculty. Age, sex, number of affected ear and side, audiometric evaluations, medical follow-up, treatment method, duration of treatment recovery, associated complaints; tinnitus and/or vertigo, presence of mumps disease were recorded for each patient. A 4-frequency pure-tone average (500, 1000, 2000, and 4000 Hz) was calculated for each ear. Complete recovery, defined as some hearing level compared with the nonaffected ear, was observed in 3 patients (21.4 %) and there was no partial hearing recovery. The hearing loss of 11 patient remained unchanged after prednisolone treatment. Two of the 11 patients had bilaterally total sensorineural hearing loss and evaluated as appropriate for cochlear implantation. Sex of patient and laterality of hearing loss were not correlated with hearing recovery. Sensorineural hearing loss among pediatrics has been the issue of otolaryngologists. The incidence, etiology, and treatment methods should be more studied.

  5. Acute nicotine treatment prevents REM sleep deprivation-induced learning and memory impairment in rat.

    PubMed

    Aleisa, A M; Helal, G; Alhaider, I A; Alzoubi, K H; Srivareerat, M; Tran, T T; Al-Rejaie, S S; Alkadhi, K A

    2011-08-01

    Rapid eye movement (REM) sleep deprivation (SD) is implicated in impairment of spatial learning and memory and hippocampal long-term potentiation (LTP). An increase in nicotine consumption among habitual smokers and initiation of tobacco use by nonsmokers was observed during SD. Although nicotine treatment was reported to attenuate the impairment of learning and memory and LTP associated with several mental disorders, the effect of nicotine on SD-induced learning and memory impairment has not been studied. Modified multiple platform paradigm was used to induce SD for 24 or 48 h during which rats were injected with saline or nicotine (1 mg kg(-1) s.c.) twice a day. In the radial arm water maze (RAWM) task, 24- or 48-h SD significantly impaired learning and short-term memory. In addition, extracellular recordings from CA1 and dentate gyrus (DG) regions of the hippocampus in urethane anesthetized rats showed a significant impairment of LTP after 24- and 48-h SD. Treatment of normal rats with nicotine for 24 or 48 h did not enhance spatial learning and memory or affect magnitude of LTP in the CA1 and DG regions. However, concurrent, acute treatment of rats with nicotine significantly attenuated SD-induced impairment of learning and STM and prevented SD-induced impairment of LTP in the CA1 and DG regions. These results show that acute nicotine treatment prevented the deleterious effect of sleep loss on cognitive abilities and synaptic plasticity.

  6. Detecting impairment: sensitive cognitive measures of dose-related acute alcohol intoxication.

    PubMed

    Cash, Catherine; Peacock, Amy; Barrington, Helen; Sinnett, Nicholas; Bruno, Raimondo

    2015-04-01

    The cognitive impairment that results from acute alcohol intoxication is associated with considerable safety risks. Other psychoactive substances, such as medications, pose a similar risk to road and workplace safety. However, there is currently no legal limit for operating vehicles or working while experiencing drug-related impairment. The current study sought to identify a brief cognitive task sensitive to a meaningful degree of impairment from acute alcohol intoxication to potentially stand as a reference from which to quantify impairment from other similar substances. A placebo-controlled single-blind crossover design was employed to determine the relative sensitivity of four commonly-administered cognitive tasks (Compensatory Tracking Task, Digit Symbol Substitution Test, Brief Stop Signal Task and Inspection Time Task) to alcohol-related impairment in male social drinkers at ~0.05% ascending breath alcohol concentration (BrAC), ~0.08% peak BrAC and 0.05% descending BrAC. The Inspection Time Task was identified as the most sensitive task, detecting a medium to large magnitude increase in impairment (g ≈ 0.60) at 0.05% ascending and descending BrAC, and a large magnitude effect size (g = 0.80) at 0.08% peak BrAC. The remaining tasks failed to demonstrate sensitivity to dose-dependent and limb-dependent changes in alcohol-induced impairment. The Inspection Time Task was deemed the most sensitive task for screening alcohol-related impairment based on the present results. Confirmation of equivalence with other drug-related impairment and sensitivity to alcohol-induced impairment in real-world settings should be established in future research.

  7. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

    PubMed Central

    Sekiya, Kenichi; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kakigi, Ryusuke; Pantev, Christo; Okamoto, Hidehiko

    2016-01-01

    Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL. PMID:26863274

  8. Cognitive Impairment and Whole Brain Diffusion in Patients with Neuromyelitis Optica after Acute Relapse

    ERIC Educational Resources Information Center

    He, Diane; Wu, Qizhu; Chen, Xiuying; Zhao, Daidi; Gong, Qiyong; Zhou, Hongyu

    2011-01-01

    The objective of this study investigated cognitive impairments and their correlations with fractional anisotropy (FA) and mean diffusivity (MD) in patients with neuromyelitis optica (NMO) without visible lesions on conventional brain MRI during acute relapse. Twenty one patients with NMO and 21 normal control subjects received several cognitive…

  9. [Sudden unilateral sensorineural hearing loss after carbon monoxide intoxication].

    PubMed

    Michalska-Piechowiak, Teresa; Miarzyńska, Maria; Perlik-Gattner, Irena

    2004-01-01

    A case of unilateral sensorineural hearing loss of the left ear after carbon monoxide intoxication was presented. The diagnosis was based upon an interview, medical examinations and audiometric investigations. Results of diagnostic evaluations, clinical presentation and treatment were discussed. Hearing improvement was obtained after 6 days of treatment and normal hearing returned after 14 days. Patients who suffer from CO intoxication are at risk of hearing impairment, therefore, there is a need for audiometric follow up in these patients.

  10. Impaired lipid clearance in patients with previous acute pancreatitis.

    PubMed Central

    Guzmán, S; Nervi, F; Llanos, O; León, P; Valdivieso, V

    1985-01-01

    Fasting serum triglycerides were measured in 52 patients who had sustained an attack of pancreatitis (gall stone related 33, alcoholism six) at least six months earlier. Several patients (23%) had raised fasting serum triglycerides, with a type IV phenotype in all but one patient. The 40 patients with normal fasting serum triglycerides received an oral load of 100 g sunflower oil to compare their clearance of dietary triglycerides with that of a control group of 54 subjects. The clearance of ingested triglycerides was significantly impaired in the patients - irrespective of the presumed aetiological factor, or clinical condition associated with pancreatitis - compared with the clearance in controls. A triglyceride tolerance test is the only way to detect those patients in whom a future attack of pancreatitis may be precipitated by a diet rich in fat, or endogenous over production of triglycerides as after an alcoholic debauch. PMID:4029716

  11. Does acute exposure to aldehydes impair pulmonary function and structure?

    PubMed

    Abreu, Mariana de; Neto, Alcendino Cândido; Carvalho, Giovanna; Casquillo, Natalia Vasconcelos; Carvalho, Niedja; Okuro, Renata; Ribeiro, Gabriel C Motta; Machado, Mariana; Cardozo, Aléxia; Silva, Aline Santos E; Barboza, Thiago; Vasconcellos, Luiz Ricardo; Rodrigues, Danielle Araujo; Camilo, Luciana; Carneiro, Leticia de A M; Jandre, Frederico; Pino, Alexandre V; Giannella-Neto, Antonio; Zin, Walter A; Corrêa, Leonardo Holanda Travassos; Souza, Marcio Nogueira de; Carvalho, Alysson R

    2016-07-15

    Mixtures of anhydrous ethyl alcohol and gasoline substituted for pure gasoline as a fuel in many Brazilian vehicles. Consequently, the concentrations of volatile organic compounds (VOCs) such as ketones, other organic compounds, and particularly aldehydes increased in many Brazilian cities. The current study aims to investigate whether formaldehyde, acetaldehyde, or mixtures of both impair lung function, morphology, inflammatory and redox responses at environmentally relevant concentrations. For such purpose, C57BL/6 mice were exposed to either medical compressed air or to 4 different mixtures of formaldehyde and acetaldehyde. Eight hours later animals were anesthetized, paralyzed and lung mechanics and morphology, inflammatory cells and IL-1β, KC, TNF-α, IL-6, CCL2, MCP-1 contents, superoxide dismutase and catalalase activities were determined. The extra pulmonary respiratory tract was also analyzed. No differences could be detected between any exposed and control groups. In conclusion, no morpho-functional alterations were detected in exposed mice in relation to the control group.

  12. Elevated Plasma Homocysteine Level Increased the Risk of Early Renal Impairment in Acute Ischemic Stroke Patients.

    PubMed

    Chen, Jingjuan; Li, Guode; Xu, Zuohang; Zhang, Chengguo; Wang, Yukai; Xie, Haiqun; Shao, Yan; Peng, Lingmei; Lu, Jiancong; Yuan, Dahua

    2017-03-08

    Renal insufficiency is associated with the prognosis of acute ischemic stroke (AIS) and homocysteine (Hcy) levels. This study investigated the association between plasma Hcy levels and renal insufficiency in patients with AIS. A total of 987 patients with AIS who had been treated at the First People's Hospital of Foshan between 2011 and 2014 were retrospectively studied. Based on their cystatin C (Cys C) levels, the patients were divided into the normal renal function group (Cys C ≤ 1.25 mg/L) or the renal impairment group (Cys C > 1.25 mg/L). Multivariate regression analysis was applied to reveal the association between hyperhomocysteinemia (HHcy) and renal impairment. The renal impairment group showed more advanced age of onset, higher percentage of prior stroke and hypertension, higher baseline National Institute of Health Stroke Scale score, lower high-density lipoprotein cholesterol levels, and higher Hcy levels compared with the normal renal function group. A multivariate analysis revealed a relationship between early renal impairment and Hcy levels: an increase of Hcy by 1 μmol/L was associated with an increase of 12-18% of the risk of renal impairment among patients with AIS and HHcy. Patients with AIS and HHcy had a 2.42-3.51 fold increase of the risk of renal impairment compared with patients with normal Hcy level (P < 0.001). In conclusion, patients with stroke and HHcy could be more prone to renal impairment.

  13. Effects of Acute Alcohol Consumption in Older and Younger Adults: Perceived Impairment Versus Psychomotor Performance*

    PubMed Central

    Gilbertson, Rebecca; Ceballos, Natalie A.; Prather, Robert; Nixon, Sara Jo

    2009-01-01

    Objective: Perceived impairment and psychomotor performance following acute alcohol administration in older (ages 50-74, n = 42; 22 male) and younger (ages 25-35, n = 26; 12 male) adults were investigated in this study. Method: Double-blind, placebo-controlled alcohol administration techniques were designed to produce peak levels of breath alcohol concentration consistent with an episode of social drinking (40 mg/100 ml). Behavioral measures (Trail Making Test, Forms A and B), as well as measures of self-reported perceived intoxication and impairment, were administered on the ascending and descending limbs at common time points after beverage ingestion. Results: Results indicated that psychomotor performance differences did not parallel self-reported levels of perceived impairment. Relative to younger adults, older adults exhibited performance deficits on the ascending limb while simultaneously reporting less perceived impairment. Conversely, on the descending limb, older adults who received alcohol reported more perceived impairment than did those who received placebo, although psychomotor performance between these two groups of older drinkers did not differ. For younger participants, a moderate dose of alcohol facilitated performance on the ascending limb; however, these differences were not reflected on the descending limb. Conclusions: These results reinforce the common knowledge that self-reported measures may not provide an accurate reflection of performance outcomes and, importantly, that older adults may be impaired even under a moderate dose of alcohol, although they may not be aware (i.e., report) of this impairment. PMID:19261236

  14. Studying nursing interventions in acutely ill, cognitively impaired older adults

    PubMed Central

    McCauley, Kathleen; Bradway, Christine; Hirschman, Karen B; Naylor, Mary D

    2015-01-01

    Background Between one and two of every five hospitalized older adults have cognitive deficits, often not accurately assessed or well managed. Cognitive impairment adds substantially to the complexity of these patients’ care, places them at high risk for poor outcomes and increases the cost of health care. Methods We describe three evidence-based interventions, each capitalizing on the unique contributions of nurses and designed to improve outcomes of hospitalized older adults who have cognitive deficits. Interventions of varying intensity were compared across three hospitals (Phase I) and subsequently within the same hospitals (Phase II). All enrolled patients were screened during their index hospitalizations and cognitive deficits were communicated to relevant health care team members (Augmented Standard Care-ASC, lowest intensity). At one hospital, ASC was the only intervention. Patients at a second hospital also had care influenced by specially prepared registered nurses (Resource Nurse Care-RNC, medium intensity). Finally, patients at third hospital also received advanced practice nurse coordinated care (Transitional Care Model-TCM, higher intensity). In Phase II, newly enrolled patients at these same hospitals all received the TCM. We summarize major themes from review of multiple data sources and researcher recollections related to facilitators and barriers to implementing a complex research study. Findings Effective implementation of the three intervention strategies depended on clinician engagement and communication; degree of participation by nurses in the educational program with subsequent practice improvement; and success of advanced practice nurses in implementing the TCM with both with patients, family caregivers and clinicians. Implications Based on lessons learned in implementing complex research studies within the “real world” of clinical practice settings, recommendations focus on strengthening facilitators, minimizing barriers and gaining

  15. Impaired sympathetic vascular regulation in humans after acute dynamic exercise

    NASA Technical Reports Server (NTRS)

    Halliwill, J. R.; Taylor, J. A.; Eckberg, D. L.

    1996-01-01

    1. The reduction in vascular resistance which accompanies acute dynamic exercise does not subside immediately during recovery, resulting in a post-exercise hypotension. This sustained vasodilatation suggests that sympathetic vascular regulation is altered after exercise. 2. Therefore, we assessed the baroreflex control of sympathetic outflow in response to arterial pressure changes, and transduction of sympathetic activity into vascular resistance during a sympatho-excitatory stimulus (isometric handgrip exercise) after either exercise (60 min cycling at 60% peak aerobic power (VO2,peak)) or sham treatment (60 min seated rest) in nine healthy subjects. 3. Both muscle sympathetic nerve activity and calf vascular resistance were reduced after exercise (-29.7 +/- 8.8 and -25.3 +/- 9.1%, both P < 0.05). The baroreflex relation between diastolic pressure and sympathetic outflow was shifted downward after exercise (post-exercise intercept, 218 +/- 38 total integrated activity (heartbeat)-1; post-sham intercept, 318 +/- 51 total integrated activity (heartbeat)-1, P < 0.05), indicating less sympathetic outflow across all diastolic pressures. Further, the relation between sympathetic activity and vascular resistance was attenuated after exercise (post-exercise slope, 0.0031 +/- 0.0007 units (total integrated activity)-1 min; post-sham slope, 0.0100 +/- 0.0033 units (total integrated activity)-1 min, P < 0.05), indicating less vasoconstriction with any increase in sympathetic activity. 4. Thus, both baroreflex control of sympathetic outflow and the transduction of sympathetic activity into vascular resistance are altered after dynamic exercise. We conclude that the vasodilation which underlies post-exercise hypotension results from both neural and vascular phenomena.

  16. Impaired sympathetic vascular regulation in humans after acute dynamic exercise.

    PubMed Central

    Halliwill, J R; Taylor, J A; Eckberg, D L

    1996-01-01

    1. The reduction in vascular resistance which accompanies acute dynamic exercise does not subside immediately during recovery, resulting in a post-exercise hypotension. This sustained vasodilatation suggests that sympathetic vascular regulation is altered after exercise. 2. Therefore, we assessed the baroreflex control of sympathetic outflow in response to arterial pressure changes, and transduction of sympathetic activity into vascular resistance during a sympatho-excitatory stimulus (isometric handgrip exercise) after either exercise (60 min cycling at 60% peak aerobic power (VO2,peak)) or sham treatment (60 min seated rest) in nine healthy subjects. 3. Both muscle sympathetic nerve activity and calf vascular resistance were reduced after exercise (-29.7 +/- 8.8 and -25.3 +/- 9.1%, both P < 0.05). The baroreflex relation between diastolic pressure and sympathetic outflow was shifted downward after exercise (post-exercise intercept, 218 +/- 38 total integrated activity (heartbeat)-1; post-sham intercept, 318 +/- 51 total integrated activity (heartbeat)-1, P < 0.05), indicating less sympathetic outflow across all diastolic pressures. Further, the relation between sympathetic activity and vascular resistance was attenuated after exercise (post-exercise slope, 0.0031 +/- 0.0007 units (total integrated activity)-1 min; post-sham slope, 0.0100 +/- 0.0033 units (total integrated activity)-1 min, P < 0.05), indicating less vasoconstriction with any increase in sympathetic activity. 4. Thus, both baroreflex control of sympathetic outflow and the transduction of sympathetic activity into vascular resistance are altered after dynamic exercise. We conclude that the vasodilation which underlies post-exercise hypotension results from both neural and vascular phenomena. Images Figure 7 PMID:8866370

  17. Impaired Bile Acid Homeostasis in Children with Severe Acute Malnutrition

    PubMed Central

    Zhang, Ling; Voskuijl, Wieger; Mouzaki, Marialena; Groen, Albert K.; Alexander, Jennifer; Bourdon, Celine; Wang, Alice; Versloot, Christian J.; Di Giovanni, Valeria; Wanders, Ronald J. A.; Bandsma, Robert

    2016-01-01

    Objective Severe acute malnutrition (SAM) is a major cause of mortality in children under 5 years and is associated with hepatic steatosis. Bile acids are synthesized in the liver and participate in dietary fat digestion, regulation of energy expenditure, and immune responses. The aim of this work was to investigate whether SAM is associated with clinically relevant changes in bile acid homeostasis. Design An initial discovery cohort with 5 healthy controls and 22 SAM-patients was used to identify altered bile acid homeostasis. A follow up cohort of 40 SAM-patients were then studied on admission and 3 days after clinical stabilization to assess recovery in bile acid metabolism. Recruited children were 6–60 months old and admitted for SAM in Malawi. Clinical characteristics, feces and blood were collected on admission and prior to discharge. Bile acids, 7α-hydroxy-4-cholesten-3-one (C4) and FGF-19 were quantified. Results On admission, total serum bile acids were higher in children with SAM than in healthy controls and glycine-conjugates accounted for most of this accumulation with median and interquartile range (IQR) of 24.6 μmol/L [8.6–47.7] compared to 1.9 μmol/L [1.7–3.3] (p = 0.01) in controls. Total serum bile acid concentrations did not decrease prior to discharge. On admission, fecal conjugated bile acids were lower and secondary bile acids higher at admission compared to pre- discharge, suggesting increased bacterial conversion. FGF19 (Fibroblast growth factor 19), a marker of intestinal bile acid signaling, was higher on admission and was associated with decreased C4 concentrations as a marker of bile acid synthesis. Upon recovery, fecal calprotectin, a marker of intestinal inflammation, was lower. Conclusion SAM is associated with increased serum bile acid levels despite reduced synthesis rates. In SAM, there tends to be increased deconjugation of bile acids and conversion from primary to secondary bile acids, which may contribute to the

  18. Impaired baroreflex control of vascular resistance and heart rate in acute myocardial infarction.

    PubMed Central

    Imaizumi, T; Takeshita, A; Makino, N; Ashihara, T; Yamamoto, K; Nakamura, M

    1984-01-01

    The baroreflex control of vascular resistance and heart rate was studied in 11 patients to determine whether it is impaired in patients with acute myocardial infarction. Reflex forearm vasoconstriction in response to lower body negative pressure at 40 mm Hg was less in the early convalescent phase (mean seven days) than in the late convalescent phase (mean 41 days). Pressor as well as vasoconstricting responses to the cold pressor test did not differ between the early and late convalescent phases. The slope of the regression line relating systolic blood pressure and the RR interval during a transient rise in blood pressure produced by intravenous phenylephrine was appreciably reduced in the early convalescent phase of myocardial infarction. These results suggest that baroreflex control of vascular resistance and heart rate is impaired in patients with acute myocardial infarction. PMID:6477780

  19. Pediatric vestibular evaluation: two children with sensorineural hearing loss.

    PubMed

    Valente, L Maureen; Goebel, Joel A; Sinks, Belinda

    2012-04-01

    These two cases illustrate several important areas of vestibular evaluation with children. The two case reports represent two children who display very different vestibular findings despite having significant sensorineural hearing loss. These case reports highlight that pediatric findings can differ significantly from adult findings, stressing the importance of comparing pediatric results with pediatric normative data. These two cases also highlight that vestibular techniques may successfully be adapted for use with hearing-impaired children. That is, rotary chair, computerized dynamic posturography, and vestibular evoked myogenic potentials can be adapted to use with children, including those who demonstrate significant sensorineural hearing loss. Although there is a paucity of research and clinical work in this area, some investigators (Eviatar and Eviatar, 1977; Buchman et al, 2004; Jacot et al, 2009) have reported very rapid recovery from pediatric vestibular deficits. However, it is important for audiologists to be aware that techniques may successfully be adapted for children and that many children should undergo thorough vestibular evaluation.

  20. Acute and subacute exposure to malathion impairs aversive but not non-associative memory in rats.

    PubMed

    Valvassori, Samira S; Fortunato, Jucélia J; Gomes, Karin M; Réus, Gislaine Z; Martins, Márcio R; Gavioli, Elaine C; Schetinger, Maria Rosa C; Dal-Pizzol, Felipe; Quevedo, João

    2007-07-01

    Malathion [S-(1,2-dicarbethoxy) ethyl-0,0-dimethyl-phosphorodithioate] is an organophosphorus compound that is widely used as pesticide especially in developing countries. This pesticide affects the central nervous system by inhibiting acetylcholinesterase, leading to an increase of acetylcholine in the synaptic cleft, and subsequent activation of cholinergic muscarinic and nicotinic receptors. In humans, intoxication with organophosphates causes a wide range of neurological symptoms, including memory deficits. The present study was aimed to investigate the effects of the acute (1 h prior the test) and subacute (once a day for 28 days) exposure to malathion at doses of 25, 50, 100 and 150 mg/kg in rats tested in the step-down inhibitory avoidance task, open-field habituation and elevated plus-maze tests. Interestingly, the acute and subacute treatment with malathion impaired aversive-memory in the step-down inhibitory avoidance task, but did not alter the animal performance in the elevated plus-maze and in the habituation to the open-field tests, and neither modified spontaneous locomotion. The activity of acetylcholinesterase enzyme was significantly reduced after subacute, but not acute, treatment with malathion (25, 100 and 150 mg/kg). Our results suggest that malathion impairs aversive-memory retention but not non-associative memory, without affecting anxiety-related behaviors. These findings support the view that the inhibition of acetylcholinesterase enzyme is not correlated with cognitive deficits observed in acute and subacute malathion-treated rats.

  1. Converging, Synergistic Actions of Multiple Stress Hormones Mediate Enduring Memory Impairments after Acute Simultaneous Stresses.

    PubMed

    Chen, Yuncai; Molet, Jenny; Lauterborn, Julie C; Trieu, Brian H; Bolton, Jessica L; Patterson, Katelin P; Gall, Christine M; Lynch, Gary; Baram, Tallie Z

    2016-11-02

    Stress influences memory, an adaptive process crucial for survival. During stress, hippocampal synapses are bathed in a mixture of stress-released molecules, yet it is unknown whether or how these interact to mediate the effects of stress on memory. Here, we demonstrate novel synergistic actions of corticosterone and corticotropin-releasing hormone (CRH) on synaptic physiology and dendritic spine structure that mediate the profound effects of acute concurrent stresses on memory. Spatial memory in mice was impaired enduringly after acute concurrent stresses resulting from loss of synaptic potentiation associated with disrupted structure of synapse-bearing dendritic spines. Combined application of the stress hormones corticosterone and CRH recapitulated the physiological and structural defects provoked by acute stresses. Mechanistically, corticosterone and CRH, via their cognate receptors, acted synergistically on the spine-actin regulator RhoA, promoting its deactivation and degradation, respectively, and destabilizing spines. Accordingly, blocking the receptors of both hormones, but not each alone, rescued memory. Therefore, the synergistic actions of corticosterone and CRH at hippocampal synapses underlie memory impairments after concurrent and perhaps also single, severe acute stresses, with potential implications to spatial memory dysfunction in, for example, posttraumatic stress disorder.

  2. Evidence-based practice: management of adult sensorineural hearing loss.

    PubMed

    Chau, Justin K; Cho, John J W; Fritz, Dieter K

    2012-10-01

    Sensorineural hearing loss is a complex disease state influenced by genetics, age, noise, and many other factors. This article reviews our current knowledge regarding the causes of sensorineural hearing loss and reviews the more challenging clinical presentations of sensorineural hearing loss. We have reviewed the latest medical literature in an attempt to provide an evidence-based strategy for the assessment and management of sudden sensorineural hearing loss, rapidly progressive sensorineural hearing loss, and asymmetric/unilateral sensorineural hearing loss.

  3. Inhibition of MDMA-induced increase in cortisol does not prevent acute impairment of verbal memory

    PubMed Central

    Kuypers, KPC; Torre, R; Farre, M; Pujadas, M; Ramaekers, JG

    2013-01-01

    Background Ecstasy use is commonly linked with memory deficits in abstinent ecstasy users. Similar impairments are being found during ecstasy intoxication after single doses of ± 3,4 metylenedioxymethamphetamine (MDMA). The concordance of memory impairments during intoxication and abstinence suggests a similar neuropharmacological mechanism underlying acute and chronic memory impairments. The mechanism underlying this impairment is to date not known. We hypothesized that cortisol might play an important role in this mechanism as cortisol, implicated in the regulation of memory performance, can be brought out of balance by stressors like MDMA. Methods In the present study, we aimed to block the MDMA-induced acute memory defect by giving participants a cortisol synthesis inhibitor (metyrapone) together with a single dose of MDMA. Seventeen polydrug MDMA users entered this placebo-controlled within subject study with four treatment conditions. The treatments consisted of MDMA (75 mg) and metyrapone (750 mg), alone and in combination, and double placebo. Pre-treatment with metyrapone or Placebo occurred 1 h prior to MDMA or Placebo administration. Memory performance was tested at peak drug concentrations by means of several memory tests. Cortisol levels were determined in blood and oral fluid; this served as a control measure to see whether manipulations were effective. Results Main findings indicated that whereas treatment with metyrapone blocked the expected MDMA-induced increase in cortisol levels in blood, it did not prevent the MDMA-induced memory deficit from happening. Conclusion We therefore conclude that MDMA-induced increments in cortisol concentrations are not related to MDMA-induced memory impairments. PMID:22946487

  4. Animal Model of Sensorineural Hearing Loss Associated with Lassa Virus Infection

    PubMed Central

    Yun, Nadezhda E.; Ronca, Shannon; Tamura, Atsushi; Koma, Takaaki; Seregin, Alexey V.; Dineley, Kelly T.; Miller, Milagros; Cook, Rebecca; Shimizu, Naoki; Walker, Aida G.; Smith, Jeanon N.; Fair, Joseph N.; Wauquier, Nadia; Bockarie, Bayon; Khan, Sheik Humarr

    2015-01-01

    ABSTRACT Approximately one-third of Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of acute disease or in early convalescence. With 500,000 annual cases of Lassa fever (LF), LASV is a major cause of hearing loss in regions of West Africa where LF is endemic. To date, no animal models exist that depict the human pathology of LF with associated hearing loss. Here, we aimed to develop an animal model to study LASV-induced hearing loss using human isolates from a 2012 Sierra Leone outbreak. We have recently established a murine model for LF that closely mimics many features of human disease. In this model, LASV isolated from a lethal human case was highly virulent, while the virus isolated from a nonlethal case elicited mostly mild disease with moderate mortality. More importantly, both viruses were able to induce SNHL in surviving animals. However, utilization of the nonlethal, human LASV isolate allowed us to consistently produce large numbers of survivors with hearing loss. Surviving mice developed permanent hearing loss associated with mild damage to the cochlear hair cells and, strikingly, significant degeneration of the spiral ganglion cells of the auditory nerve. Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic loss of hearing and provided further insights into the mechanistic cause of LF-associated hearing loss. IMPORTANCE Sensorineural hearing loss is a major complication for LF survivors. The development of a small-animal model of LASV infection that replicates hearing loss and the clinical and pathological features of LF will significantly increase knowledge of pathogenesis and vaccine studies. In addition, such a model will permit detailed characterization of the hearing loss mechanism and allow for the development of appropriate diagnostic approaches and medical care for LF patients with hearing impairment. PMID:26719273

  5. Sudden sensorineural hearing loss after heroin injection.

    PubMed

    Schrock, Andreas; Jakob, Mark; Wirz, Stefan; Bootz, Friedrich

    2008-05-01

    Sudden sensorineural hearing loss is a symptom of cochlear injury. Potential aetiologies are vascular diseases, viral infections, allergic reactions, autoimmune disorders, and traumatic rupture of the intralabyrinthe membrane. Unlike in unilateral cases bilateral sensorineural hearing loss is often associated with specific disease entities. We report a case of sudden bilateral deafness after intravenous heroin abuse. The putative pathophysiological mechanisms are discussed.

  6. Profile and Stability of Sensorineural Hearing Loss in Persistent Pulmonary Hypertension of the Newborn.

    ERIC Educational Resources Information Center

    Walton, Joseph P.; Hendricks-Munoz, Karen

    1991-01-01

    This study found that 19 of 51 infants with persistent pulmonary hypertension of the newborn were diagnosed with sensorineural hearing loss, an incidence 25 times greater than that of intensive care unit infants in general. Treatment durations with mechanical ventilation were significantly longer for the hearing-impaired group compared to the…

  7. Adipose triglyceride lipase deletion from adipocytes, but not skeletal myocytes, impairs acute exercise performance in mice

    PubMed Central

    Dubé, John J.; Sitnick, Mitch T.; Schoiswohl, Gabriele; Wills, Rachel C.; Basantani, Mahesh K.; Cai, Lingzhi; Pulinilkunnil, Thomas

    2015-01-01

    Adipose triglyceride lipase (ATGL) is the rate-limiting enzyme mediating triacylglycerol hydrolysis in virtually all cells, including adipocytes and skeletal myocytes, and hence, plays a critical role in mobilizing fatty acids. Global ATGL deficiency promotes skeletal myopathy and exercise intolerance in mice and humans, and yet the tissue-specific contributions to these phenotypes remain unknown. The goal of this study was to determine the relative contribution of ATGL-mediated triacylglycerol hydrolysis in adipocytes vs. skeletal myocytes to acute exercise performance. To achieve this goal, we generated murine models with adipocyte- and skeletal myocyte-specific targeted deletion of ATGL. We then subjected untrained mice to acute peak and submaximal exercise interventions and assessed exercise performance and energy substrate metabolism. Impaired ATGL-mediated lipolysis within adipocytes reduced peak and submaximal exercise performance, reduced peripheral energy substrate availability, shifted energy substrate preference toward carbohydrate oxidation, and decreased HSL Ser660 phosphorylation and mitochondrial respiration within skeletal muscle. In contrast, impaired ATGL-mediated lipolysis within skeletal myocytes was not sufficient to reduce peak and submaximal exercise performance or peripheral energy substrate availability and instead tended to enhance metabolic flexibility during peak exercise. Furthermore, the expanded intramyocellular triacylglycerol pool in these mice was reduced following exercise in association with preserved HSL phosphorylation, suggesting that HSL may compensate for impaired ATGL action in skeletal muscle during exercise. These data suggest that adipocyte rather than skeletal myocyte ATGL-mediated lipolysis plays a greater role during acute exercise in part because of compensatory mechanisms that maintain lipolysis in muscle, but not adipose tissue, when ATGL is absent. PMID:25783895

  8. Impaired gas exchange: accuracy of defining characteristics in children with acute respiratory infection1

    PubMed Central

    Pascoal, Lívia Maia; Lopes, Marcos Venícios de Oliveira; Chaves, Daniel Bruno Resende; Beltrão, Beatriz Amorim; da Silva, Viviane Martins; Monteiro, Flávia Paula Magalhães

    2015-01-01

    OBJECTIVE: to analyze the accuracy of the defining characteristics of the Impaired gas exchange nursing diagnosis in children with acute respiratory infection. METHOD: open prospective cohort study conducted with 136 children monitored for a consecutive period of at least six days and not more than ten days. An instrument based on the defining characteristics of the Impaired gas exchange diagnosis and on literature addressing pulmonary assessment was used to collect data. The accuracy means of all the defining characteristics under study were computed. RESULTS: the Impaired gas exchange diagnosis was present in 42.6% of the children in the first assessment. Hypoxemia was the characteristic that presented the best measures of accuracy. Abnormal breathing presented high sensitivity, while restlessness, cyanosis, and abnormal skin color showed high specificity. All the characteristics presented negative predictive values of 70% and cyanosis stood out by its high positive predictive value. CONCLUSION: hypoxemia was the defining characteristic that presented the best predictive ability to determine Impaired gas exchange. Studies of this nature enable nurses to minimize variability in clinical situations presented by the patient and to identify more precisely the nursing diagnosis that represents the patient's true clinical condition. PMID:26155010

  9. Post-learning REM sleep deprivation impairs long-term memory: reversal by acute nicotine treatment.

    PubMed

    Aleisa, A M; Alzoubi, K H; Alkadhi, K A

    2011-07-15

    Rapid eye movement sleep deprivation (REM-SD) is associated with spatial learning and memory impairment. During REM-SD, an increase in nicotine consumption among habitual smokers and initiation of tobacco use by non-smokers have been reported. We have shown recently that nicotine treatment prevented learning and memory impairments associated with REM-SD. We now report the interactive effects of post-learning REM-SD and/or nicotine. The animals were first trained on the radial arm water maze (RAWM) task, then they were REM-sleep deprived using the modified multiple platform paradigm for 24h. During REM-SD period, the rats were injected with saline or nicotine (1mg/kg s.c. every 12h: a total of 3 injections). The animals were tested for long-term memory in the RAWM at the end of the REM-SD period. The 24h post-learning REM-SD significantly impaired long-term memory. However, nicotine treatment reversed the post-learning REM-SD-induced impairment of long-term memory. On the other hand, post-learning treatment of normal rats with nicotine for 24h enhanced long-term memory. These results indicate that post-learning acute nicotine treatment prevented the deleterious effect of REM-SD on cognitive abilities.

  10. Impairment in functional status and survival in patients with acute myeloid leukaemia.

    PubMed

    Wedding, Ulrich; Röhrig, Bernd; Klippstein, Almuth; Fricke, Hans-Joerg; Sayer, Herbert G; Höffken, Klaus

    2006-10-01

    Acute myeloid leukaemia (AML) is mainly affecting elderly patients. Elderly patients are increasingly affected by impairment of functional status (FS). FS is of prognostic relevance for survival in different tumours. Data for patients with AML are rare. Within a prospective trial we recruited patients with newly diagnosed AML and measured FS by two different methods: Karnofsky performance status (KPS) and instrumental activities of daily living (IADL). Sixty-three patients aged 19-85 years (median 61.1) were included. Twenty-three had prior myelodisplastic syndrome (MDS), 7 favourable, 17 unfavourable karyotype. Fifty received induction chemotherapy, 13 palliative chemotherapy. Median survival was 15.2 months (95% CI, 10.8-22.3) in all patients. Age, cytogenetic risk group, and impaired KPS and IADL significantly influenced median survival in univariate analysis. Impairment of IADL was the single most predictive variable. In multivariate analysis, impairment of IADL Score (HR:4.3, 95% CI 1.7-10.5, P = 0.001) and of KPS (HR:4.8, 95% CI 1.9-12.3, P = 0.001), and unfavourable cytogenetic risk group (HR:6.0, 95% CI 2.5-14.3, P < 0.001) significantly predicted median survival. In patients with AML, FS and not age is a major predictor of survival. The influence of FS is independent from cytogenetic risk group. IADL measurement adds information to KPS. The results have to be confirmed in a large sample of patients.

  11. Cannabis and tolerance: acute drug impairment as a function of cannabis use history

    PubMed Central

    Ramaekers, J. G.; van Wel, J. H.; Spronk, D. B.; Toennes, S. W.; Kuypers, K. P. C.; Theunissen, E. L.; Verkes, R. J.

    2016-01-01

    Cannabis use history as predictor of neurocognitive response to cannabis intoxication remains subject to scientific and policy debates. The present study assessed the influence of cannabis on neurocognition in cannabis users whose cannabis use history ranged from infrequent to daily use. Drug users (N = 122) received acute doses of cannabis (300 μg/kg THC), cocaine HCl (300 mg) and placebo. Cocaine served as active control for demonstrating neurocognitive test sensitivity. Executive function, impulse control, attention, psychomotor function and subjective intoxication were significantly worse after cannabis administration relative to placebo. Cocaine improved psychomotor function and attention, impaired impulse control and increased feelings of intoxication. Acute effects of cannabis and cocaine on neurocognitive performance were similar across cannabis users irrespective of their cannabis use history. Absence of tolerance implies that that frequent cannabis use and intoxication can be expected to interfere with neurocognitive performance in many daily environments such as school, work or traffic. PMID:27225696

  12. Cannabis and tolerance: acute drug impairment as a function of cannabis use history.

    PubMed

    Ramaekers, J G; van Wel, J H; Spronk, D B; Toennes, S W; Kuypers, K P C; Theunissen, E L; Verkes, R J

    2016-05-26

    Cannabis use history as predictor of neurocognitive response to cannabis intoxication remains subject to scientific and policy debates. The present study assessed the influence of cannabis on neurocognition in cannabis users whose cannabis use history ranged from infrequent to daily use. Drug users (N = 122) received acute doses of cannabis (300 μg/kg THC), cocaine HCl (300 mg) and placebo. Cocaine served as active control for demonstrating neurocognitive test sensitivity. Executive function, impulse control, attention, psychomotor function and subjective intoxication were significantly worse after cannabis administration relative to placebo. Cocaine improved psychomotor function and attention, impaired impulse control and increased feelings of intoxication. Acute effects of cannabis and cocaine on neurocognitive performance were similar across cannabis users irrespective of their cannabis use history. Absence of tolerance implies that that frequent cannabis use and intoxication can be expected to interfere with neurocognitive performance in many daily environments such as school, work or traffic.

  13. The effects of acute alcohol on motor impairments in adolescent, adult, and aged rats.

    PubMed

    Ornelas, Laura C; Novier, Adelle; Van Skike, Candice E; Diaz-Granados, Jaime L; Matthews, Douglas B

    2015-03-01

    Acute alcohol exposure has been shown to produce differential motor impairments between aged and adult rats and between adolescent and adult rats. However, the effects of acute alcohol exposure among adolescent, adult, and aged rats have yet to be systematically investigated within the same project using a dose-dependent analysis. We sought to determine the age- and dose-dependent effects of acute alcohol exposure on gross and coordinated motor performance across the rodent lifespan. Adolescent (PD 30), adult (PD 70), and aged (approximately 18 months) male Sprague-Dawley rats were tested on 3 separate motor tasks: aerial righting reflex (ARR), accelerating rotarod (RR), and loss of righting reflex (LORR). In a separate group of animals, blood ethanol concentrations (BEC) were determined at multiple time points following a 3.0 g/kg ethanol injection. Behavioral tests were conducted with a Latin square repeated-measures design in which all animals received the following doses: 1.0 g/kg or 2.0 g/kg alcohol or saline over 3 separate sessions via intraperitoneal (i.p.) injection. During testing, motor impairments were assessed on the RR 10 min post-injection and on ARR 20 min post-injection. Aged animals spent significantly less time on the RR when administered 1.0 g/kg alcohol compared to adult rats. In addition, motor performance impairments significantly increased with age after 2.0 g/kg alcohol administration. On the ARR test, aged rats were more sensitive to the effects of 1.0 g/kg and 2.0 g/kg alcohol compared to adolescents and adults. Seven days after the last testing session, animals were given 3.0 g/kg alcohol and LORR was examined. During LORR, aged animals slept longer compared to adult and adolescent rats. This effect cannot be explained solely by BEC levels in aged rats. The present study suggests that acute alcohol exposure produces greater motor impairments in older rats when compared to adolescent and adult rats and begins to establish a

  14. High-affinity α4β2 nicotinic receptors mediate the impairing effects of acute nicotine on contextual fear extinction.

    PubMed

    Kutlu, Munir Gunes; Holliday, Erica; Gould, Thomas J

    2016-02-01

    Previously, studies from our lab have shown that while acute nicotine administered prior to training and testing enhances contextual fear conditioning, acute nicotine injections prior to extinction sessions impair extinction of contextual fear. Although there is also strong evidence showing that the acute nicotine's enhancing effects on contextual fear conditioning require high-affinity α4β2 nicotinic acetylcholine receptors (nAChRs), it is unknown which nAChR subtypes are involved in the acute nicotine-induced impairment of contextual fear extinction. In this study, we investigated the effects of acute nicotine administration on contextual fear extinction in knock-out (KO) mice lacking α4, β2 or α7 subtypes of nAChRs and their wild-type (WT) littermates. Both KO and WT mice were first trained and tested for contextual fear conditioning and received a daily contextual extinction session for 4 days. Subjects received intraperitoneal injections of nicotine (0.18 mg/kg) or saline 2-4 min prior to each extinction session. Our results showed that the mice that lack α4 and β2 subtypes of nAChRs showed normal contextual fear extinction but not the acute nicotine-induced impairment while the mice that lack the α7 subtype showed both normal contextual extinction and nicotine-induced impairment of contextual extinction. In addition, control experiments showed that acute nicotine-induced impairment of contextual fear extinction persisted when nicotine administration was ceased and repeated acute nicotine administrations alone did not induce freezing behavior in the absence of context-shock learning. These results clearly demonstrate that high-affinity α4β2 nAChRs are necessary for the effects of acute nicotine on contextual fear extinction.

  15. Atypical Structural Connectome Organization and Cognitive Impairment in Young Survivors of Acute Lymphoblastic Leukemia.

    PubMed

    Kesler, Shelli R; Gugel, Meike; Huston-Warren, Emily; Watson, Christa

    2016-05-01

    Survivors of pediatric acute lymphoblastic leukemia (ALL) are at increased risk for cognitive impairments that disrupt everyday functioning and decrease quality of life. The specific biological mechanisms underlying cognitive impairment following ALL remain largely unclear, but previous studies consistently demonstrate significant white matter pathology. We aimed to extend this literature by examining the organization of the white matter connectome in young patients with a history of ALL treated with chemotherapy only. We applied graph theoretical analysis to diffusion tensor imaging obtained from 31 survivors of ALL age 5-19 years and 39 matched healthy controls. Results indicated significantly lower small-worldness (p = 0.007) and network clustering coefficient (p = 0.019), as well as greater cognitive impairment (p = 0.027) in the ALL group. Regional analysis indicated that clustered connectivity in parietal, frontal, hippocampal, amygdalar, thalamic, and occipital regions was altered in the ALL group. Random forest analysis revealed a model of connectome and demographic variables that could automatically classify survivors of ALL as having cognitive impairment or not (accuracy = 0.89, p < 0.0001). These findings provide further evidence of brain injury in young survivors of ALL, even those without a history of central nervous system (CNS) disease or cranial radiation. Efficiency of local information processing, reorganization of hub connectivity, and cognitive reserve may contribute to cognitive outcome in these children. Certain connectome properties showed U-shaped relationships with cognitive impairment suggesting an optimal range of regional connectivity.

  16. Impairment of electron transfer chain induced by acute carnosine administration in skeletal muscle of young rats.

    PubMed

    Macarini, José Roberto; Maravai, Soliany Grassi; Cararo, José Henrique; Dimer, Nádia Webber; Gonçalves, Cinara Ludvig; Kist, Luiza Wilges; Bogo, Mauricio Reis; Schuck, Patrícia Fernanda; Streck, Emilio Luiz; Ferreira, Gustavo Costa

    2014-01-01

    Serum carnosinase deficiency is an inherited disorder that leads to an accumulation of carnosine in the brain tissue, cerebrospinal fluid, skeletal muscle, and other tissues of affected patients. Considering that high levels of carnosine are associated with neurological dysfunction and that the pathophysiological mechanisms involved in serum carnosinase deficiency remain poorly understood, we investigated the in vivo effects of carnosine on bioenergetics parameters, namely, respiratory chain complexes (I-III, II, and II-III), malate dehydrogenase, succinate dehydrogenase, and creatine kinase activities and the expression of mitochondrial-specific transcription factors (NRF-1, PGC-1α , and TFAM) in skeletal muscle of young Wistar rats. We observed a significant decrease of complexes I-III and II activities in animals receiving carnosine acutely, as compared to control group. However, no significant alterations in respiratory chain complexes, citric acid cycle enzymes, and creatine kinase activities were found between rats receiving carnosine chronically and control group animals. As compared to control group, mRNA levels of NRF-1, PGC-1α , and TFAM were unchanged. The present findings indicate that electron transfer through the respiratory chain is impaired in skeletal muscle of rats receiving carnosine acutely. In case these findings are confirmed by further studies and ATP depletion is also observed, impairment of bioenergetics could be considered a putative mechanism responsible for the muscle damage observed in serum carnosinase-deficient patients.

  17. Impairment of Electron Transfer Chain Induced by Acute Carnosine Administration in Skeletal Muscle of Young Rats

    PubMed Central

    Macarini, José Roberto; Maravai, Soliany Grassi; Cararo, José Henrique; Dimer, Nádia Webber; Gonçalves, Cinara Ludvig; Kist, Luiza Wilges; Bogo, Mauricio Reis; Schuck, Patrícia Fernanda; Streck, Emilio Luiz; Ferreira, Gustavo Costa

    2014-01-01

    Serum carnosinase deficiency is an inherited disorder that leads to an accumulation of carnosine in the brain tissue, cerebrospinal fluid, skeletal muscle, and other tissues of affected patients. Considering that high levels of carnosine are associated with neurological dysfunction and that the pathophysiological mechanisms involved in serum carnosinase deficiency remain poorly understood, we investigated the in vivo effects of carnosine on bioenergetics parameters, namely, respiratory chain complexes (I–III, II, and II-III), malate dehydrogenase, succinate dehydrogenase, and creatine kinase activities and the expression of mitochondrial-specific transcription factors (NRF-1, PGC-1α, and TFAM) in skeletal muscle of young Wistar rats. We observed a significant decrease of complexes I–III and II activities in animals receiving carnosine acutely, as compared to control group. However, no significant alterations in respiratory chain complexes, citric acid cycle enzymes, and creatine kinase activities were found between rats receiving carnosine chronically and control group animals. As compared to control group, mRNA levels of NRF-1, PGC-1α, and TFAM were unchanged. The present findings indicate that electron transfer through the respiratory chain is impaired in skeletal muscle of rats receiving carnosine acutely. In case these findings are confirmed by further studies and ATP depletion is also observed, impairment of bioenergetics could be considered a putative mechanism responsible for the muscle damage observed in serum carnosinase-deficient patients. PMID:24877122

  18. Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus

    PubMed Central

    Chawki, Sylvain; Aouizerate, Jessie; Trad, Selim; Prinseau, Jacques; Hanslik, Thomas

    2016-01-01

    Abstract Introduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus. Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered. Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered. PMID:27603334

  19. Protective Effects of Myricetin on Acute Hypoxia-Induced Exercise Intolerance and Mitochondrial Impairments in Rats

    PubMed Central

    Zou, Dan; Liu, Peng; Chen, Ka; Xie, Qi; Liang, Xinyu; Bai, Qian; Zhou, Qicheng; Liu, Kai; Zhang, Ting; Zhu, Jundong; Mi, Mantian

    2015-01-01

    Purpose Exercise tolerance is impaired in hypoxia. The aim of this study was to evaluate the effects of myricetin, a dietary flavonoid compound widely found in fruits and vegetables, on acute hypoxia-induced exercise intolerance in vivo and in vitro. Methods Male rats were administered myricetin or vehicle for 7 days and subsequently spent 24 hours at a barometric pressure equivalent to 5000 m. Exercise capacity was then assessed through the run-to-fatigue procedure, and mitochondrial morphology in skeletal muscle cells was observed by transmission electron microscopy (TEM). The enzymatic activities of electron transfer complexes were analyzed using an enzyme-linked immuno-sorbent assay (ELISA). mtDNA was quantified by real-time-PCR. Mitochondrial membrane potential was measured by JC-1 staining. Protein expression was detected through western blotting, immunohistochemistry, and immunofluorescence. Results Myricetin supplementation significantly prevented the decline of run-to-fatigue time of rats in hypoxia, and attenuated acute hypoxia-induced mitochondrial impairment in skeletal muscle cells in vivo and in vitro by maintaining mitochondrial structure, mtDNA content, mitochondrial membrane potential, and activities of the respiratory chain complexes. Further studies showed that myricetin maintained mitochondrial biogenesis in skeletal muscle cells under hypoxic conditions by up-regulating the expressions of mitochondrial biogenesis-related regluators, in addition, AMP-activated protein kinase(AMPK) plays a crucial role in this process. Conclusions Myricetin may have important applications for improving physical performance under hypoxic environment, which may be attributed to the protective effect against mitochondrial impairment by maintaining mitochondrial biogenesis. PMID:25919288

  20. Disordered macrophage cytokine secretion underlies impaired acute inflammation and bacterial clearance in Crohn's disease.

    PubMed

    Smith, Andrew M; Rahman, Farooq Z; Hayee, Bu'Hussain; Graham, Simon J; Marks, Daniel J B; Sewell, Gavin W; Palmer, Christine D; Wilde, Jonathan; Foxwell, Brian M J; Gloger, Israel S; Sweeting, Trevor; Marsh, Mark; Walker, Ann P; Bloom, Stuart L; Segal, Anthony W

    2009-08-31

    The cause of Crohn's disease (CD) remains poorly understood. Counterintuitively, these patients possess an impaired acute inflammatory response, which could result in delayed clearance of bacteria penetrating the lining of the bowel and predispose to granuloma formation and chronicity. We tested this hypothesis in human subjects by monitoring responses to killed Escherichia coli injected subcutaneously into the forearm. Accumulation of (111)In-labeled neutrophils at these sites and clearance of (32)P-labeled bacteria from them were markedly impaired in CD. Locally increased blood flow and bacterial clearance were dependent on the numbers of bacteria injected. Secretion of proinflammatory cytokines by CD macrophages was grossly impaired in response to E. coli or specific Toll-like receptor agonists. Despite normal levels and stability of cytokine messenger RNA, intracellular levels of tumor necrosis factor (TNF) were abnormally low in CD macrophages. Coupled with reduced secretion, these findings indicate accelerated intracellular breakdown. Differential transcription profiles identified disease-specific genes, notably including those encoding proteins involved in vesicle trafficking. Intracellular destruction of TNF was decreased by inhibitors of lysosomal function. Together, our findings suggest that in CD macrophages, an abnormal proportion of cytokines are routed to lysosomes and degraded rather than being released through the normal secretory pathway.

  1. Impaired mental rotation in benign paroxysmal positional vertigo and acute vestibular neuritis

    PubMed Central

    Candidi, Matteo; Micarelli, Alessandro; Viziano, Andrea; Aglioti, Salvatore M.; Minio-Paluello, Ilaria; Alessandrini, Marco

    2013-01-01

    Vestibular processing is fundamental to our sense of orientation in space which is a core aspect of the representation of the self. Vestibular information is processed in a large subcortical–cortical neural network. Tasks requiring mental rotations of human bodies in space are known to activate neural regions within this network suggesting that vestibular processing is involved in the control of mental rotation. We studied whether mental rotation is impaired in patients suffering from two different forms of unilateral vestibular disorders (vestibular neuritis – VN – and Benign Paroxysmal positional Vertigo – BPPV) with respect to healthy matched controls (C). We used two mental rotation tasks in which participants were required to: (i) mentally rotate their own body in space (egocentric rotation) thus using vestibular processing to a large extent and (ii) mentally rotate human figures (allocentric rotation) thus using own body representations to a smaller degree. Reaction times and accuracy of responses showed that VN and BPPV patients were impaired in both tasks with respect to C. Significantly, the pattern of results was similar in the three groups suggesting that patients were actually performing the mental rotation without using a different strategy from the control individuals. These results show that dysfunctional vestibular inflow impairs mental rotation of both own body and human figures suggesting that unilateral acute disorders of the peripheral vestibular input massively affect the cerebral processes underlying mental rotations. PMID:24324422

  2. Disordered macrophage cytokine secretion underlies impaired acute inflammation and bacterial clearance in Crohn's disease

    PubMed Central

    Smith, Andrew M.; Rahman, Farooq Z.; Hayee, Bu'Hussain; Graham, Simon J.; Marks, Daniel J.B.; Sewell, Gavin W.; Palmer, Christine D.; Wilde, Jonathan; Foxwell, Brian M.J.; Gloger, Israel S.; Sweeting, Trevor; Marsh, Mark; Walker, Ann P.; Bloom, Stuart L.

    2009-01-01

    The cause of Crohn's disease (CD) remains poorly understood. Counterintuitively, these patients possess an impaired acute inflammatory response, which could result in delayed clearance of bacteria penetrating the lining of the bowel and predispose to granuloma formation and chronicity. We tested this hypothesis in human subjects by monitoring responses to killed Escherichia coli injected subcutaneously into the forearm. Accumulation of 111In-labeled neutrophils at these sites and clearance of 32P-labeled bacteria from them were markedly impaired in CD. Locally increased blood flow and bacterial clearance were dependent on the numbers of bacteria injected. Secretion of proinflammatory cytokines by CD macrophages was grossly impaired in response to E. coli or specific Toll-like receptor agonists. Despite normal levels and stability of cytokine messenger RNA, intracellular levels of tumor necrosis factor (TNF) were abnormally low in CD macrophages. Coupled with reduced secretion, these findings indicate accelerated intracellular breakdown. Differential transcription profiles identified disease-specific genes, notably including those encoding proteins involved in vesicle trafficking. Intracellular destruction of TNF was decreased by inhibitors of lysosomal function. Together, our findings suggest that in CD macrophages, an abnormal proportion of cytokines are routed to lysosomes and degraded rather than being released through the normal secretory pathway. PMID:19652016

  3. Fractal Dimension of EEG Activity Senses Neuronal Impairment in Acute Stroke

    PubMed Central

    Zappasodi, Filippo; Olejarczyk, Elzbieta; Marzetti, Laura; Assenza, Giovanni; Pizzella, Vittorio; Tecchio, Franca

    2014-01-01

    The brain is a self-organizing system which displays self-similarities at different spatial and temporal scales. Thus, the complexity of its dynamics, associated to efficient processing and functional advantages, is expected to be captured by a measure of its scale-free (fractal) properties. Under the hypothesis that the fractal dimension (FD) of the electroencephalographic signal (EEG) is optimally sensitive to the neuronal dysfunction secondary to a brain lesion, we tested the FD’s ability in assessing two key processes in acute stroke: the clinical impairment and the recovery prognosis. Resting EEG was collected in 36 patients 4–10 days after a unilateral ischemic stroke in the middle cerebral artery territory and 19 healthy controls. National Health Institute Stroke Scale (NIHss) was collected at T0 and 6 months later. Highuchi FD, its inter-hemispheric asymmetry (FDasy) and spectral band powers were calculated for EEG signals. FD was smaller in patients than in controls (1.447±0.092 vs 1.525±0.105) and its reduction was paired to a worse acute clinical status. FD decrease was associated to alpha increase and beta decrease of oscillatory activity power. Larger FDasy in acute phase was paired to a worse clinical recovery at six months. FD in our patients captured the loss of complexity reflecting the global system dysfunction resulting from the structural damage. This decrease seems to reveal the intimate nature of structure-function unity, where the regional neural multi-scale self-similar activity is impaired by the anatomical lesion. This picture is coherent with neuronal activity complexity decrease paired to a reduced repertoire of functional abilities. FDasy result highlights the functional relevance of the balance between homologous brain structures’ activities in stroke recovery. PMID:24967904

  4. Fractal dimension of EEG activity senses neuronal impairment in acute stroke.

    PubMed

    Zappasodi, Filippo; Olejarczyk, Elzbieta; Marzetti, Laura; Assenza, Giovanni; Pizzella, Vittorio; Tecchio, Franca

    2014-01-01

    The brain is a self-organizing system which displays self-similarities at different spatial and temporal scales. Thus, the complexity of its dynamics, associated to efficient processing and functional advantages, is expected to be captured by a measure of its scale-free (fractal) properties. Under the hypothesis that the fractal dimension (FD) of the electroencephalographic signal (EEG) is optimally sensitive to the neuronal dysfunction secondary to a brain lesion, we tested the FD's ability in assessing two key processes in acute stroke: the clinical impairment and the recovery prognosis. Resting EEG was collected in 36 patients 4-10 days after a unilateral ischemic stroke in the middle cerebral artery territory and 19 healthy controls. National Health Institute Stroke Scale (NIHss) was collected at T0 and 6 months later. Highuchi FD, its inter-hemispheric asymmetry (FDasy) and spectral band powers were calculated for EEG signals. FD was smaller in patients than in controls (1.447±0.092 vs 1.525±0.105) and its reduction was paired to a worse acute clinical status. FD decrease was associated to alpha increase and beta decrease of oscillatory activity power. Larger FDasy in acute phase was paired to a worse clinical recovery at six months. FD in our patients captured the loss of complexity reflecting the global system dysfunction resulting from the structural damage. This decrease seems to reveal the intimate nature of structure-function unity, where the regional neural multi-scale self-similar activity is impaired by the anatomical lesion. This picture is coherent with neuronal activity complexity decrease paired to a reduced repertoire of functional abilities. FDasy result highlights the functional relevance of the balance between homologous brain structures' activities in stroke recovery.

  5. Constraint-induced sound therapy for sudden sensorineural hearing loss--behavioral and neurophysiological outcomes.

    PubMed

    Okamoto, Hidehiko; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kitahara, Tadashi; Inohara, Hidenori; Kakigi, Ryusuke; Pantev, Christo

    2014-01-29

    Sudden sensorineural hearing loss is characterized by acute, idiopathic hearing deterioration. We report here the development and evaluation of "constraint-induced sound therapy", which is based on a well-established neuro-rehabilitation approach, and which is characterized by the plugging of the intact ear ("constraint") and the simultaneous, extensive stimulation of the affected ear with music. The sudden sensorineural hearing loss patients who received the constraint-induced sound therapy in addition to the standard corticosteroid therapy showed significantly better recovery of hearing function compared to those who had only received corticosteroid treatments. Additionally, the brain activity obtained in a subgroup of patients suggested that the constraint-induced sound therapy could have prevented maladaptive auditory cortex reorganization. Constraint-induced sound therapy thus appears to be an effective, practical, and safe treatment option for sudden sensorineural hearing loss.

  6. Acute stress does not impair long-term memory retrieval in older people.

    PubMed

    Pulopulos, Matias M; Almela, Mercedes; Hidalgo, Vanesa; Villada, Carolina; Puig-Perez, Sara; Salvador, Alicia

    2013-09-01

    Previous studies have shown that stress-induced cortisol increases impair memory retrieval in young people. This effect has not been studied in older people; however, some findings suggest that age-related changes in the brain can affect the relationships between acute stress, cortisol and memory in older people. Our aim was to investigate the effects of acute stress on long-term memory retrieval in healthy older people. To this end, 76 participants from 56 to 76 years old (38 men and 38 women) were exposed to an acute psychosocial stressor or a control task. After the stress/control task, the recall of pictures, words and stories learned the previous day was assessed. There were no differences in memory retrieval between the stress and control groups on any of the memory tasks. In addition, stress-induced cortisol response was not associated with memory retrieval. An age-related decrease in cortisol receptors and functional changes in the amygdala and hippocampus could underlie the differences observed between the results from this study and those found in studies performed with young people.

  7. Sensorineural hearing loss in hemodialysis patients.

    PubMed

    Jakić, Marko; Mihaljević, Dubravka; Zibar, Lada; Jakić, Marijana; Kotromanović, Zeljko; Roguljić, Hrvoje

    2010-03-01

    Chronic renal failure affects all organ systems. Senses are not exception and hearing impairment is common, particularly sensorineural hearing loss (SNHL). The term SNOS of unknown origin or uremic deafness is related to only a smaller part of the cases with unclear etiology of the impairment. The study searched for SNOS in 66 chronic hemodialysis (HD) patients, mean age 51.50 +/- 12.70 years. They were treated by HD for 69.70 +/- 53.80 months. The relation between the severity of the impairment and the patients' age, duration of HD treatment (months) and a set of laboratory parameters typical for chronic HD patients was examined. The aim of the study was to detect potential causes of the impairment. The increased hearing threshold (HT) of above 20 dB for all frequencies was found in 42 patients (mean HT 26 +/- 10.50 dB), for speaking area frequencies in 22 patients (mean HT 19.70 +/- 8.80 dB), and in 56 patients for high frequencies (mean HT 41.70 +/- 19.70 dB). The significant positive correlation of HT was found only with the patients' age (r = 0.49, p < 0.01). The patients older than 45 years had higher mean HT than those younger, and those older than 65 also had higher HT than the younger ones. Patients with pathological value of HT were significantly more common among the older subgroup of patients, when divided according to the age at both cutoff values of 45 and of 60 years. Mean HT did not differ significantly according to the duration of HD treatment (subgroups A- no longer than 60 months, B- from 61 to 120 months, and C- longer than 120 months). The patients with pathological HT did not differ significantly in frequency among those subgroups, and the subgroups were not different according to the mean age (A--50.30 +/- 13.20 years; B--51.40 +/- 12.75 years; C--55.80 +/- 10.55 years). In conclusion, our results along with other authors'published data report on SNHL as very frequent finding among chronic HD patients and suggest multifactorial etiology

  8. [Sensorineural hearing loss due to neonatal hyperbilirubinemia].

    PubMed

    Clarós, P; Turcanu, D; Caballero, M; Costa, C; Clavería, M A; Clarós, A; Clarós, A

    2003-01-01

    In this article, the sensorineural hearing loss is presented as a possible sequelae of neonatal hyperbilirubinemia. In our program of early hipoacusia detection, 241 babies were examined from January 1996 until November 1999; 7 cases had a history of hyperbilirubinemia in the neonatal period and 2 of them were diagnosed of sensorineural hearing loss. We discuss how the bilirubin or any other associated factor might have been the cause and this could explain the selective affectation of some children.

  9. Acute exhaustive aerobic exercise training impair cardiomyocyte function and calcium handling in Sprague-Dawley rats

    PubMed Central

    Ljones, Kristine; Ness, Henning Ofstad; Solvang-Garten, Karin; Gaustad, Svein Erik; Andre Høydal, Morten

    2017-01-01

    Introduction Recent data from long-distance endurance participants suggest that cardiac function is impaired after completion. Existing data further indicate that right ventricular function is more affected than left ventricular function. The cellular mechanisms underpinning cardiac deterioration are limited and therefore the aim of this study was to examine cardiomyocyte and molecular responses of the right and left ventricle to an acute bout of exhaustive endurance exercise. Materials and methods Male Sprague-Dawley rats were assigned to sedentary controls or acute exhaustive endurance exercise consisting of a 120 minutes long forced treadmill run. The contractile function and Ca2+ handling properties in isolated cardiomyocytes, protein expression levels of sarcoplasmic reticulum Ca2+-ATPase and phospholamban including two of its phosphorylated states (serine 16 and threonine 17), and the mitochondrial respiration in permeabilized cardiac muscle fibers were analyzed. Results The exercise group showed a significant reduction in cardiomyocyte fractional shortening (right ventricle 1 Hz and 3 Hz p<0.001; left ventricle 1 Hz p<0.05), intracellular Ca2+ amplitude (right ventricle 1 and 3 Hz p<0.001; left ventricle 1 Hz p<0.01 and 3 Hz p<0.05) and rate of diastolic Ca2+ decay (right ventricle 1 Hz p<0.001 and 3 Hz p<0.01; left ventricle 1 and 3 Hz p<0.01). Cardiomyocyte relaxation during diastole was only significantly prolonged at 3 Hz in the right ventricle (p<0.05) compared to sedentary controls. We found an increase in phosphorylation of phospholamban at serine 16 and threonine 17 in the left (p<0.05), but not the right, ventricle from exhaustively exercised animals. The protein expression levels of sarcoplasmic reticulum Ca2+-ATPase and phospholamban was not changed. Furthermore, we found a reduction in maximal oxidative phosphorylation and electron transport system capacities of mitochondrial respiration in the right (p<0.01 and p<0.05, respectively), but not the

  10. Sudden sensorineural hearing loss associated with internal carotid artery pseudoaneurysm: causal or incidental?

    PubMed

    Palma, Silvia; Soloperto, Davide; Casoni, Federica; Rovati, Raffaella; Galassi, Giuliana

    2013-03-01

    Sudden deafness is acute onset of impaired hearing which develops within hours to few days. The commonly accepted audiometric criterion is a decrease in hearing of ≥ 30 dB, affecting at least three consecutive frequencies. Hearing loss is thought to involve several causative factors, including internal ear circulatory disturbances. We report the case of a female with an internal carotid artery (ICA) pseudoaneurysm in the distal cervical tract and unilateral sudden sensorineural hearing loss (SSNHL). As putative risk vascular factor, the patient had history of migraine since youth. Extensive screenings for autoimmune, rheumatic diseases, virological, and microbiological infections were negative. The patient denied recent cervical trauma. Furosemide and oral prednisone were given with initial benefit and withdrawn in 3 weeks. The patient experienced short-lasting episodes of headache, tinnitus, vertigo. Five weeks after first onset, she underwent magnetic resonance imaging (MRI) angiogram which revealed fusiform dilatation of left ICA in the cervical tract. It can be proposed, but it remains to be proved, that the pseudoaneurysm of the cervical ICA plays a role in the patient SSNHL in relation to turbulent flow or thromboembolism of branches to the inner ear.

  11. Pressor recovery after acute stress is impaired in high fructose-fed Lean Zucker rats.

    PubMed

    Thompson, Jennifer A; D'Angelo, Gerard; Mintz, James D; Fulton, David J; Stepp, David W

    2016-06-01

    Insulin resistance is a powerful predictor of cardiovascular disease; however, the mechanistic link remains unclear. This study aims to determine if early cardiovascular changes associated with short-term fructose feeding in the absence of obesity manifest as abnormal blood pressure control. Metabolic dysfunction was induced in Lean Zucker rats by short-term high-fructose feeding. Rats were implanted with telemetry devices for the measurement of mean arterial blood pressure (MAP) and subjected to air jet stress at 5 and 8 weeks after feeding. Additional animals were catheterized under anesthesia for the determination of MAP and blood flow responses in the hind limb and mesenteric vascular beds to intravenous injection of isoproterenol (0.001-0.5 μm), a β-adrenergic agonist. Metabolic dysfunction in high-fructose rats was not accompanied by changes in 24-h MAP Yet, animals fed a high-fructose diet for 8 weeks exhibited a marked impairment in blood pressure recovery after air-jet stress. Dose-dependent decreases in MAP and peripheral blood flow in response to isoproterenol treatment were significantly attenuated in high-fructose rats. These data suggest that impaired blood pressure recovery to acute mental stress precedes the onset of hypertension in the early stages of insulin resistance. Further, blunted responses to isoproterenol implicate β2-adrenergic sensitivity as a possible mechanism responsible for altered blood pressure control after short-term high-fructose feeding.

  12. Which type of repetitive muscle contractions induces a greater acute impairment of position sense?

    PubMed

    Fortier, Sylvie; Basset, Fabien A; Billaut, François; Behm, David; Teasdale, Normand

    2010-04-01

    The objective of this study was to determine which type of repetitive muscle contractions induces a greater acute impairment of elbow position sense. Eleven male subjects participating in the study underwent (i) an exercise task (ET) consisting of 9 sets of 10 voluntary isometric, concentric, or eccentric contractions randomly performed on three separate sessions, and (ii) a pre- and post-exercise maximal voluntary isometric contraction (iMVC). Prior to and between sets of ET, a proprioception task (PT) consisting of matching the right arm to the left reference arm was performed at three different target angular positions (70 degrees , 110 degrees and 150 degrees). Each ET was immediately followed by 3 PT and 1 min rest. The statistical analysis revealed that post-exercise iMVCs were significantly decreased compared to pre-exercise iMVC in all conditions with a greater drop following the eccentric task. Despite this greater drop, position sense was significantly affected by the concentric exercise task. In addition, the spectral EMG signals significantly shifted towards lower frequencies from the initial values, regardless of exercise task. The results showed that concentric muscle contractions impaired position sense to a greater extent compared to isometric and eccentric contractions.

  13. Cardiac Function Remains Impaired Despite Reversible Cardiac Remodeling after Acute Experimental Viral Myocarditis

    PubMed Central

    Gotzhein, Frauke; Escher, Felicitas; Blankenberg, Stefan; Westermann, Dirk

    2017-01-01

    Background. Infection with Coxsackievirus B3 induces myocarditis. We aimed to compare the acute and chronic phases of viral myocarditis to identify the immediate effects of cardiac inflammation as well as the long-term effects after resolved inflammation on cardiac fibrosis and consequently on cardiac function. Material and Methods. We infected C57BL/6J mice with Coxsackievirus B3 and determined the hemodynamic function 7 as well as 28 days after infection. Subsequently, we analyzed viral burden and viral replication in the cardiac tissue as well as the expression of cytokines and matrix proteins. Furthermore, cardiac fibroblasts were infected with virus to investigate if viral infection alone induces profibrotic signaling. Results. Severe cardiac inflammation was determined and cardiac fibrosis was consistently colocalized with inflammation during the acute phase of myocarditis. Declined cardiac inflammation but no significantly improved hemodynamic function was observed 28 days after infection. Interestingly, cardiac fibrosis declined to basal levels as well. Both cardiac inflammation and fibrosis were reversible, whereas the hemodynamic function remains impaired after healed viral myocarditis in C57BL/6J mice. PMID:28352641

  14. Acute tolerance to alcohol impairment of behavioral and cognitive mechanisms related to driving: drinking and driving on the descending limb

    PubMed Central

    Weafer, Jessica

    2015-01-01

    Rationale Alcohol effects on behavioral and cognitive mechanisms influence impaired driving performance and decisions to drive after drinking (Barry 1973; Moskowitz and Robinson 1987). To date, research has focused on the ascending limb of the blood alcohol curve, and there is little understanding of how acute tolerance to impairment of these mechanisms might influence driving behavior on the descending limb. Objectives To provide an integrated examination of the degree to which alcohol impairment of motor coordination and inhibitory control contributes to driving impairment and decisions to drive on the ascending and descending limbs of the blood alcohol curve. Methods Social-drinking adults (N=20) performed a testing battery that measured simulated driving performance and willingness to drive, as well as mechanisms related to driving: motor coordination (grooved pegboard), inhibitory control (cued go/no-go task), and subjective intoxication. Performance was tested in response to placebo and a moderate dose of alcohol (0.65 g/kg) twice at comparable blood alcohol concentrations: once on the ascending limb and again on the descending limb. Results Impaired motor coordination and subjective intoxication showed acute tolerance, whereas driving performance and inhibitory control showed no recovery from impairment. Greater motor impairment was associated with poorer driving performance under alcohol, and poorer inhibitory control was associated with more willingness to drive. Conclusions Findings suggest that acute tolerance to impairment of motor coordination is insufficient to promote recovery of driving performance and that the persistence of alcohol-induced disinhibition might contribute to risky decisions to drive on the descending limb. PMID:21960182

  15. Dynamic cerebral autoregulation and beat to beat blood pressure control are impaired in acute ischaemic stroke

    PubMed Central

    Eames, P; Blake, M; Dawson, S; Panerai, R; Potter, J

    2002-01-01

    Objectives: Hypertension and chronic cerebrovascular disease are known to alter static cerebral autoregulation (CA) but the effects of acute stroke on dynamic CA (dCA) have not been studied in detail. Those studies to date measuring dCA have used sympathetically induced blood pressure (BP) changes, which may themselves directly affect dCA. This study assessed whether dCA is compromised after acute stroke using spontaneous blood pressure (BP) changes as the stimulus for the dCA response. Methods: 56 patients with ischaemic stroke (aged 70 (SD 9) years), studied within 72 hours of ictus were compared with 56 age, sex, and BP matched normal controls. Cerebral blood flow velocity was measured using transcranial Doppler ultrasound (TCD) with non-invasive beat to beat arterial BP levels, surface ECG, and transcutaneous CO2 levels and a dynamic autoregulatory index (dARI) calculated. Results: Beat to beat BP, but not pulse interval variability was significantly increased and cardiac baroreceptor sensitivity (BRS) decreased in the patients with stroke. Dynamic CA was significantly reduced in patients with stroke compared with controls (strokes: ARI 3.8 (SD 2.2) and 3.2 (SD 2.0) for pressor and depressor stimuli respectively v controls: ARI 4.7 (SD 2.2) and 4.5 (SD 2.0) respectively (p<0.05 in all cases)). There was no difference between stroke and non-stroke hemispheres in ARI, which was also independent of severity of stroke, BP, BP variability, BRS, sex, and age. Conclusion: Dynamic cerebral autoregulation, as assessed using spontaneous transient pressor and depressor BP stimuli, is globally impaired after acute ischaemic stroke and may prove to be an important factor in predicting outcome. PMID:11909905

  16. Acute auditory agnosia as the presenting hearing disorder in MELAS.

    PubMed

    Miceli, Gabriele; Conti, Guido; Cianfoni, Alessandro; Di Giacopo, Raffaella; Zampetti, Patrizia; Servidei, Serenella

    2008-12-01

    MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.

  17. Genetics Home Reference: sensorineural deafness and male infertility

    MedlinePlus

    ... deafness and male infertility sensorineural deafness and male infertility Enable Javascript to view the expand/collapse boxes. ... All Close All Description Sensorineural deafness and male infertility is a condition characterized by hearing loss and ...

  18. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  19. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  20. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  1. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  2. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  3. Mephedrone in adolescent rats: residual memory impairment and acute but not lasting 5-HT depletion.

    PubMed

    Motbey, Craig P; Karanges, Emily; Li, Kong M; Wilkinson, Shane; Winstock, Adam R; Ramsay, John; Hicks, Callum; Kendig, Michael D; Wyatt, Naomi; Callaghan, Paul D; McGregor, Iain S

    2012-01-01

    Mephedrone (4-methylmethcathinone, MMC) is a popular recreational drug, yet its potential harms are yet to be fully established. The current study examined the impact of single or repeated MMC exposure on various neurochemical and behavioral measures in rats. In Experiment 1 male adolescent Wistar rats received single or repeated (once a day for 10 days) injections of MMC (30 mg/kg) or the comparator drug methamphetamine (METH, 2.5 mg/kg). Both MMC and METH caused robust hyperactivity in the 1 h following injection although this effect did not tend to sensitize with repeated treatment. Striatal dopamine (DA) levels were increased 1 h following either METH or MMC while striatal and hippocampal serotonin (5-HT) levels were decreased 1 h following MMC but not METH. MMC caused greater increases in 5-HT metabolism and greater reductions in DA metabolism in rats that had been previously exposed to MMC. Autoradiographic analysis showed no signs of neuroinflammation ([(125)I]CLINDE ligand used as a marker for translocator protein (TSPO) expression) with repeated exposure to either MMC or METH. In Experiment 2, rats received repeated MMC (7.5, 15 or 30 mg/kg once a day for 10 days) and were examined for residual behavioral effects following treatment. Repeated high (30 mg/kg) dose MMC produced impaired novel object recognition 5 weeks after drug treatment. However, no residual changes in 5-HT or DA tissue levels were observed at 7 weeks post-treatment. Overall these results show that MMC causes acute but not lasting changes in DA and 5-HT tissue concentrations. MMC can also cause long-term memory impairment. Future studies of cognitive function in MMC users are clearly warranted.

  4. Neuropsychological impairment in acute HIV and the effect of immediate antiretroviral therapy

    PubMed Central

    Kore, Idil; Ananworanich, Jintanat; Valcour, Victor; Fletcher, James LK; Chalermchai, Thep; Paul, Robert; Reynolds, Jesse; Tipsuk, Somporn; Ubolyam, Sasiwimol; Rattanamanee, Somprartthana; Jagodzinski, Linda; Kim, Jerome; Spudich, Serena

    2015-01-01

    OBJECTIVE To investigate neuropsychological performance (NP) during acute HIV infection (AHI) before and after combination antiretroviral therapy (cART). DESIGN Prospective study of Thai AHI participants examined at 3 and 6 months following initiation of cART. METHODS 36 AHI participants were evaluated pre-cART at median 19 days since HIV exposure and 3 and 6 months after cART with the Grooved Pegboard test (GP), Color Trails 1 & 2 (CT1, CT2), and Trail Making Test A (TM). Raw scores were standardized to 251 age-and-education-matched HIV-uninfected Thais. To account for learning effects, change in NP performance was compared to that of controls at 6 months. Analyses included multivariable regression, non-parametric repeated measures ANOVA, and Mann-Whitney U test. RESULTS Baseline NP scores for the AHI group were within normal range (Z scores range: −0.26 to −0.13). NP performance improved on CT1, CT2, and TM in the initial 3 months (ps <0.01) with no significant change during the last 3 months. Only improvement in CT1 was greater than that seen in controls at 6 months (p=0.018). Participants that performed >1 standard deviation below normative means on >2 tests (n=8) exhibited higher baseline cerebrospinal fluid (CSF) HIV RNA (p=0.047) and had no improvement after cART. CONCLUSIONS Most AHI individuals had normal NP performance and early cART slightly improved their psychomotor function. However, approximately 25% had impaired NP performance which correlated with higher CSF HIV RNA, and these abnormalities were not reversed by early cART possibly indicating limited reversibility of cognitive impairment in a subset of AHI individuals. PMID:26509933

  5. Slight impairment of psychomotor skills in children after treatment of acute lymphoblastic leukemia.

    PubMed

    Harten, G; Stephani, U; Henze, G; Langermann, H J; Riehm, H; Hanefeld, F

    1984-08-01

    Several studies have reported a decline in intelligence and cognitive functions in survivors of childhood acute lymphoblastic leukemia (ALL). Other investigators, however, have found no intellectual impairment in these children. Fifty-one long-term survivors of ALL, having been treated according to the protocols of the BFM Study Group from 1970 to 1979, were assessed retrospectively using neurophysical methods. The results were compared with those obtained from 30 patients with other malignancies, who had received neither radiation therapy to the central nervous system (CRT) nor any methotrexate during chemotherapy. Additionally, neurological examinations and cranial computed tomography (CCT) were performed. neuropsychological examinations included verbal functions, intelligence (performance), psychomotor speed, motor skills and sensory integration. The results of verbal tests and the IQs, tested by nonspeed-related measures, were within normal limits in both groups. About one-third of all patients showed mild disturbances of psychomotor speed and motor skills. Children with leukemia had lower scores than those with solid tumors for nearly all tasks, but only tests for sensory integration revealed significant differences between former ALL patients and tumor patients. Furthermore, the following results were obtained related to different therapeutic modalities: The higher total radiation doses had been during CRT (maximum 24 GY), the more neuropsychological functions were impaired, particularly motor accuracy and sensory integration. These disturbances improved with the length of survival. Widening of subarachnoidal space was found in 33% of the CCT obtained. There was no correlation between the intellectual functions of the survivors and the CCT abnormalities. Neurological findings mainly consisted of slight fine motor disturbances.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. Mephedrone in Adolescent Rats: Residual Memory Impairment and Acute but Not Lasting 5-HT Depletion

    PubMed Central

    Motbey, Craig P.; Karanges, Emily; Li, Kong M.; Wilkinson, Shane; Winstock, Adam R.; Ramsay, John; Hicks, Callum; Kendig, Michael D.; Wyatt, Naomi; Callaghan, Paul D.; McGregor, Iain S.

    2012-01-01

    Mephedrone (4-methylmethcathinone, MMC) is a popular recreational drug, yet its potential harms are yet to be fully established. The current study examined the impact of single or repeated MMC exposure on various neurochemical and behavioral measures in rats. In Experiment 1 male adolescent Wistar rats received single or repeated (once a day for 10 days) injections of MMC (30 mg/kg) or the comparator drug methamphetamine (METH, 2.5 mg/kg). Both MMC and METH caused robust hyperactivity in the 1 h following injection although this effect did not tend to sensitize with repeated treatment. Striatal dopamine (DA) levels were increased 1 h following either METH or MMC while striatal and hippocampal serotonin (5-HT) levels were decreased 1 h following MMC but not METH. MMC caused greater increases in 5-HT metabolism and greater reductions in DA metabolism in rats that had been previously exposed to MMC. Autoradiographic analysis showed no signs of neuroinflammation ([125I]CLINDE ligand used as a marker for translocator protein (TSPO) expression) with repeated exposure to either MMC or METH. In Experiment 2, rats received repeated MMC (7.5, 15 or 30 mg/kg once a day for 10 days) and were examined for residual behavioral effects following treatment. Repeated high (30 mg/kg) dose MMC produced impaired novel object recognition 5 weeks after drug treatment. However, no residual changes in 5-HT or DA tissue levels were observed at 7 weeks post-treatment. Overall these results show that MMC causes acute but not lasting changes in DA and 5-HT tissue concentrations. MMC can also cause long-term memory impairment. Future studies of cognitive function in MMC users are clearly warranted. PMID:23029034

  7. Acute caffeine administration effect on brain activation patterns in mild cognitive impairment.

    PubMed

    Haller, Sven; Montandon, Marie-Louise; Rodriguez, Cristelle; Moser, Dominik; Toma, Simona; Hofmeister, Jeremy; Sinanaj, Indrit; Lovblad, Karl-Olof; Giannakopoulos, Panteleimon

    2014-01-01

    Previous studies showed that acute caffeine administration enhances task-related brain activation in elderly individuals with preserved cognition. To explore the effects of this widely used agent on cognition and brain activation in early phases of cognitive decline, we performed a double-blinded, placebo-controlled functional magnetic resonance imaging (fMRI) study during an n-back working memory task in 17 individuals with mild cognitive impairment (MCI) compared to 17 age-matched healthy controls (HC). All individuals were regular caffeine consumers with an overnight abstinence and given 200 mg caffeine versus placebo tablets 30 minutes before testing. Analyses included assessment of task-related activation (general linear model), functional connectivity (tensorial-independent component analysis, TICA), baseline perfusion (arterial spin labeling, ASL), grey matter density (voxel-based morphometry, VBM), and white matter microstructure (tract-based spatial statistics, TBSS). Acute caffeine administration induced a focal activation of the prefrontal areas in HC with a more diffuse and posteromedial activation pattern in MCI individuals. In MCI, TICA documented a significant caffeine-related enhancement in the prefrontal cortex, supplementary motor area, ventral premotor and parietal cortex as well as the basal ganglia and cerebellum. The absence of significant group differences in baseline ASL perfusion patterns supports a neuronal rather than a purely vascular origin of these differences. The VBM and TBSS analyses excluded potentially confounding differences in grey matter density and white matter microstructure between MCI and HC. The present findings suggest a posterior displacement of working memory-related brain activation patterns after caffeine administration in MCI that may represent a compensatory mechanism to counterbalance a frontal lobe dysfunction.

  8. Chikungunya virus induced sudden sensorineural hearing loss.

    PubMed

    Bhavana, Kranti; Tyagi, Isha; Kapila, Rajeev Kumar

    2008-02-01

    The aim of this study is to demonstrate the association of Chikungunya virus and sudden sensorineural hearing loss. In the case report described we had a case which developed sudden unilateral sensorineural hearing loss following chikungunya fever. A 15-year-old female presented to us with the complains of unilateral sudden onset of hearing loss following an episode of fever, arthralgia and rashes 1 month ago. At the time of these symptoms there were many cases of chikungunya fever in the city, three being in her locality. Clinically Chikungunya fever was suspected and a positive serological test further confirmed our diagnosis. The hearing loss could thus be attributed to Chikungunya virus. Viruses have always been implicated in causing sudden sensorineural hearing loss but Chikungunya virus as a cause has not been documented earlier making this case report a unique one.

  9. Sustained impairment of respiratory function and swim performance following acute oil exposure in a coastal marine fish.

    PubMed

    Johansen, J L; Esbaugh, A J

    2017-04-06

    Acute exposure to crude oil polycyclic aromatic hydrocarbons (PAH) can severely impair cardiorespiratory function and swim performance of larval fish; however, the effects of acute oil exposure on later life stages and the capacity for subsequent recovery is less clear. Red drum (Sciaenops ocellatus) is an economically important apex predator native to the Gulf of Mexico, which was directly exposed to the 2010 Deep Water Horizon (DWH) oil spill. Here we examine impact and recovery of young adult red drum from exposure to concentrations of 0, 4.1, and 12.1μgL(-1) ΣPAH50 naturally weathered oil-water accommodated fractions (geometric mean), which are well within the range of concentrations measured during the DWH incident. We focused on aerobic scope (ASc), burst- and critical swimming speeds (Uburst and Ucrit), cost of transport (COT), as well as the capacity to repay oxygen debt following exhaustive exercise (EPOC), which are critical parameters for success of all life stages of fishes. A 24h acute exposure to 4.1μgL(-1) ΣPAH caused a significant 9.7 and 12.6% reduction of Uburst and Ucrit respectively, but no change in ASc, COT or EPOC, highlighting a decoupled effect on the respiratory and swimming systems. A higher exposure concentration, 12.1μgL(-1) ΣPAH, caused an 8.6 and 8.4% impairment of Uburst and Ucrit, as well as an 18.4% reduction in ASc. These impairments persisted six weeks post-exposure, suggesting that recorded impacts are entrenched. Large predatory fishes are critically dependent on the cardiorespiratory and swimming systems for ecological fitness, and long-term impairment of performance due to acute oil exposure suggests that even acute exposure events may have long lasting impacts on the ecological fitness of affected populations.

  10. Clinical Features and Prognosis of Sudden Sensorineural Hearing Loss Secondary to Intralabyrinthine Hemorrhage

    PubMed Central

    Lee, Jae Woo; Park, Yoon Ah; Park, Sang Man; Kong, Tae Hoon; Park, Sang Yoo; Bong, Jeong Pyo; Park, Dong-Joon

    2016-01-01

    Background and Objectives A number of etiologies of idiopathic sudden sensorineural hearing loss (ISSNHL) have been proposed, including viral infection, vascular disturbance, and immune-mediated mechanisms. Intralabyrinthine hemorrhage (ILH) as a cause of SSNHL is extremely rare, and there have been no studies defining the characteristics of hearing impairment and prognosis in patients with ISSNHL due to ILH. This study aimed to investigate the difference in impaired hearing patterns and prognosis for hearing recovery between patients with ISSNHL due to ILH confirmed by magnetic resonance imaging (MRI) and sex- and age-matched patients with ISSNHL due to causes other than ILH. Subjects and Methods We compared the results of audiometry and MRI in 12 patients who had ILH on MRI (hemorrhage group) and in 23 sex- and age-matched controls without abnormal findings related to their hearing loss on MRI (non-hemorrhage group). Initial hearing impairment, progression, and recovery of hearing loss were compared between the two groups. Results A majority of patients (92%) in the hemorrhage group complained of dizziness. Initial hearing impairment was more frequent in the hemorrhage group than in the non-hemorrhage group (94.09±35.9 vs. 66.66±30.1, p-value=0.036). The final recovery threshold in the hemorrhage group was worse (78.19±46.26 vs. 37.17±31.96, p-value=0.014) than that in the non-hemorrhage group. In the hemorrhage group, hearing recovery seemed to occur less often at high frequencies (2,000, 4,000, and 8,000 Hz) than at low frequencies (250, 500, and 1,000 Hz). Conclusions The presence of ILH was associated with poor hearing prognosis and the occurrence of vertigo. The abrupt onset of hearing loss associated with vertigo and the presence of hyperresonance on fat-suppressed T1-weighted MRI images of labyrinthic fluid strongly suggests acute intralabyrinthine hemorrhage, and is predictive of considerable hearing impairment and poor prognosis. PMID:27144231

  11. Acute stress impairs recall after interference in older people, but not in young people.

    PubMed

    Hidalgo, Vanesa; Almela, Mercedes; Villada, Carolina; Salvador, Alicia

    2014-03-01

    Stress has been associated with negative changes observed during the aging process. However, very little research has been carried out on the role of age in acute stress effects on memory. We aimed to explore the role of age and sex in the relationship between hypothalamus-pituitary-adrenal axis (HPA-axis) and sympathetic nervous system (SNS) reactivity to psychosocial stress and short-term declarative memory performance. To do so, sixty-seven participants divided into two age groups (each group with a similar number of men and women) were exposed to the Trier Social Stress Test (TSST) and a control condition in a crossover design. Memory performance was assessed by the Rey Auditory Verbal Learning Test (RAVLT). As expected, worse memory performance was associated with age; but more interestingly, the stressor impaired recall after interference only in the older group. In addition, this effect was negatively correlated with the alpha-amylase over cortisol ratio, which has recently been suggested as a good marker of stress system dysregulation. However, we failed to find sex differences in memory performance. These results show that age moderates stress-induced effects on declarative memory, and they point out the importance of studying both of the physiological systems involved in the stress response together.

  12. LSD Acutely Impairs Fear Recognition and Enhances Emotional Empathy and Sociality

    PubMed Central

    Dolder, Patrick C; Schmid, Yasmin; Müller, Felix; Borgwardt, Stefan; Liechti, Matthias E

    2016-01-01

    Lysergic acid diethylamide (LSD) is used recreationally and has been evaluated as an adjunct to psychotherapy to treat anxiety in patients with life-threatening illness. LSD is well-known to induce perceptual alterations, but unknown is whether LSD alters emotional processing in ways that can support psychotherapy. We investigated the acute effects of LSD on emotional processing using the Face Emotion Recognition Task (FERT) and Multifaceted Empathy Test (MET). The effects of LSD on social behavior were tested using the Social Value Orientation (SVO) test. Two similar placebo-controlled, double-blind, random-order, crossover studies were conducted using 100 μg LSD in 24 subjects and 200 μg LSD in 16 subjects. All of the subjects were healthy and mostly hallucinogen-naive 25- to 65-year-old volunteers (20 men, 20 women). LSD produced feelings of happiness, trust, closeness to others, enhanced explicit and implicit emotional empathy on the MET, and impaired the recognition of sad and fearful faces on the FERT. LSD enhanced the participants' desire to be with other people and increased their prosocial behavior on the SVO test. These effects of LSD on emotion processing and sociality may be useful for LSD-assisted psychotherapy. PMID:27249781

  13. Skeletal, neuromuscular and fitness impairments among children with newly diagnosed acute lymphoblastic leukemia

    PubMed Central

    Ness, Kirsten K.; Kaste, Sue C.; Zhu, Liang; Pui, Ching-Hon; Jeha, Sima; Nathan, Paul C.; Inaba, Hiroto; Wasilewski-Masker, Karen; Shah, Durga; Wells, Robert J.; Karlage, Robyn E.; Robison, Leslie L.; Cox, Cheryl L.

    2014-01-01

    This study describes skeletal, neuromuscular and fitness impairments among 109 children (median age 10 (range 4–18) years, 65.1% male, 63.3% white) with acute lymphoblastic leukemia (ALL), enrolled on a physical activity trial from 2009 to 2013. Outcomes were measured 7-10 days after diagnosis and compared to age- and sex-specific expected values. Associations between function and HRQL were evaluated with logistic regression. Children low values for BMD z-scores/height (mean±standard error: −0.53±0.16 vs. 0.00±0.14, p <0.01), body mass index percentile (57.6±3.15 vs. 50.0±3.27%, p=0.02), quadriceps strength (201.9±8.3 vs. 236.1±5.4 Newtons, p<0.01), six minute walk distance (385.0±13.1 vs. 628.2±7.1 meters, p < 0.001), and Bruininks-Oseretsky Test of Motor Proficiency (23±2.5 vs. 50±3.4%, p < 0.001). Quadriceps weakness was associated with a 20.9-fold (95% CI 2.5–173.3) increase in poor physical HRQL. Children with newly diagnosed ALL have weakness and poor endurance and may benefit from early rehabilitation that includes strengthening and aerobic conditioning. PMID:25030039

  14. LSD Acutely Impairs Fear Recognition and Enhances Emotional Empathy and Sociality.

    PubMed

    Dolder, Patrick C; Schmid, Yasmin; Müller, Felix; Borgwardt, Stefan; Liechti, Matthias E

    2016-10-01

    Lysergic acid diethylamide (LSD) is used recreationally and has been evaluated as an adjunct to psychotherapy to treat anxiety in patients with life-threatening illness. LSD is well-known to induce perceptual alterations, but unknown is whether LSD alters emotional processing in ways that can support psychotherapy. We investigated the acute effects of LSD on emotional processing using the Face Emotion Recognition Task (FERT) and Multifaceted Empathy Test (MET). The effects of LSD on social behavior were tested using the Social Value Orientation (SVO) test. Two similar placebo-controlled, double-blind, random-order, crossover studies were conducted using 100 μg LSD in 24 subjects and 200 μg LSD in 16 subjects. All of the subjects were healthy and mostly hallucinogen-naive 25- to 65-year-old volunteers (20 men, 20 women). LSD produced feelings of happiness, trust, closeness to others, enhanced explicit and implicit emotional empathy on the MET, and impaired the recognition of sad and fearful faces on the FERT. LSD enhanced the participants' desire to be with other people and increased their prosocial behavior on the SVO test. These effects of LSD on emotion processing and sociality may be useful for LSD-assisted psychotherapy.

  15. XIAP inhibitors induce differentiation and impair clonogenic capacity of acute myeloid leukemia stem cells

    PubMed Central

    Moreno-Martínez, Daniel; Nomdedeu, Meritxell; Lara-Castillo, María Carmen; Etxabe, Amaia; Pratcorona, Marta; Tesi, Niccolò; Díaz-Beyá, Marina; Rozman, María; Montserrat, Emili; Urbano-Ispizua, Álvaro; Esteve, Jordi; Risueño, Ruth M.

    2014-01-01

    Acute myeloid leukemia (AML) is a neoplasia characterized by the rapid expansion of immature myeloid blasts in the bone marrow, and marked by poor prognosis and frequent relapse. As such, new therapeutic approaches are required for remission induction and prevention of relapse. Due to the higher chemotherapy sensitivity and limited life span of more differentiated AML blasts, differentiation-based therapies are a promising therapeutic approach. Based on public available gene expression profiles, a myeloid-specific differentiation-associated gene expression pattern was defined as the therapeutic target. A XIAP inhibitor (Dequalinium chloride, DQA) was identified in an in silico screening searching for small molecules that induce similar gene expression regulation. Treatment with DQA, similarly to Embelin (another XIAP inhibitor), induced cytotoxicity and differentiation in AML. XIAP inhibition differentially impaired cell viability of the most primitive AML blasts and reduced clonogenic capacity of AML cells, sparing healthy mature blood and hematopoietic stem cells. Taken together, these results suggest that XIAP constitutes a potential target for AML treatment and support the evaluation of XIAP inhibitors in clinical trials. PMID:24952669

  16. Medulloblastoma manifesting as sudden sensorineural hearing loss.

    PubMed

    Terakawa, Yuzo; Tsuyuguchi, Naohiro; Takami, Toshihiro; Ohata, Kenji

    2011-07-01

    We present a rare case of medulloblastoma which presented with unilateral sudden sensorineural hearing loss as an initial symptom. A 19-year-old man was admitted to our hospital with a chief complaint of dizziness and facial numbness on the right side. His illness had begun two years previously with sudden hearing loss on the right side, for which he had been treated as an idiopathic sudden hearing loss. Magnetic resonance imaging demonstrated abnormal signals located mainly in the right middle cerebellar peduncle. We performed partial resection of the tumor by suboccipital craniotomy. The histopathological diagnosis was medulloblastoma. Intrinsic brain tumor is an extremely rare cause of sudden sensorineural hearing loss and is therefore easily overlooked as was in the present case. The present case highlights not only the need to evaluate patients with sudden sensorineural hearing loss by magnetic resonance imaging but also the importance of paying attention to intrinsic lesions involving the brainstem. Although this condition like the presented case might be rare, intrinsic brain tumor should be considered as a potential cause of sudden sensorineural hearing loss, as it may be easily missed leading to a delay in appropriate treatment.

  17. Acute Free-Iron Exposure Does Not Explain the Impaired Haemorheology Associated with Haemochromatosis

    PubMed Central

    McNamee, Antony P.; Sabapathy, Surendran; Singh, Indu; Horobin, Jarod; Guerrero, Janelle; Simmonds, Michael J.

    2016-01-01

    . Acute exposure to elevated iron levels does not appear (in isolation) to account for these differences. Further consideration is required prior to utilising routine venesection blood for harvesting RBC concentrates due to the potential risk of microvascular disorders arising from impaired haemorheology. PMID:26741993

  18. Interferon-α acutely impairs whole-brain functional connectivity network architecture - A preliminary study.

    PubMed

    Dipasquale, Ottavia; Cooper, Ella A; Tibble, Jeremy; Voon, Valerie; Baglio, Francesca; Baselli, Giuseppe; Cercignani, Mara; Harrison, Neil A

    2016-11-01

    Interferon-alpha (IFN-α) is a key mediator of antiviral immune responses used to treat Hepatitis C infection. Though clinically effective, IFN-α rapidly impairs mood, motivation and cognition, effects that can appear indistinguishable from major depression and provide powerful empirical support for the inflammation theory of depression. Though inflammation has been shown to modulate activity within discrete brain regions, how it affects distributed information processing and the architecture of whole brain functional connectivity networks have not previously been investigated. Here we use a graph theoretic analysis of resting state functional magnetic resonance imaging (rfMRI) to investigate acute effects of systemic interferon-alpha (IFN-α) on whole brain functional connectivity architecture and its relationship to IFN-α-induced mood change. Twenty-two patients with Hepatitis-C infection, initiating IFN-α-based therapy were scanned at baseline and 4h after their first IFN-α dose. The whole brain network was parcellated into 110 cortical and sub-cortical nodes based on the Oxford-Harvard Atlas and effects assessed on higher-level graph metrics, including node degree, betweenness centrality, global and local efficiency. IFN-α was associated with a significant reduction in global network connectivity (node degree) (p=0.033) and efficiency (p=0.013), indicating a global reduction of information transfer among the nodes forming the whole brain network. Effects were similar for highly connected (hub) and non-hub nodes, with no effect on betweenness centrality (p>0.1). At a local level, we identified regions with reduced efficiency of information exchange and a sub-network with decreased functional connectivity after IFN-α. Changes in local and particularly global functional connectivity correlated with associated changes in mood measured on the Profile of Mood States (POMS) questionnaire. IFN-α rapidly induced a profound shift in whole brain network structure

  19. The acute inhibition of rapid eye movement sleep by citalopram may impair spatial learning and passive avoidance in mice.

    PubMed

    Bridoux, A; Laloux, C; Derambure, P; Bordet, R; Monaca Charley, C

    2013-03-01

    Rapid eye movement (REM) sleep is known to be essential for memory. Hence, REM sleep deprivation impairs memory processes. The frequently prescribed selective serotonin reuptake inhibitors (SSRIs) are known to cause REM sleep deprivation and to impair cognitive performance in humans and rodents. We suggested that impaired memory processes by citalopram in C57/BL6 mice could be explained by the acute inhibition of REM sleep. We hypothesized that those acute citalopram 5 and 10 mg/kg injections induced REM sleep deprivation, altered cognitive performance in passive avoidance, impaired spatial memory compared to controls. Three experiments have been realized: (1) mice received successively physiological saline, injection of citalopram 5 and 10 mg/kg and were recorded by polysomnographic recording after each injection. (2) Cognitive performance was evaluated in the passive avoidance with two groups of mice. One group received citalopram before training and one, after training. (3) Spatial learning was evaluated with another group of animals in the Y-maze test. At 5 and 10 mg/kg, citalopram delayed REM sleep onset and decreased REM sleep amounts (vs. controls). The same doses were administrated in the passive avoidance test and have significantly shortened latency to enter the dark compartment. In the Y-maze, citalopram-treated mice showed a decreased percentage of time spent in the novel arm in contrast to the two other arms compared with controls. We showed that citalopram impaired cognitive performance in behavioral tasks. Those impairments could be linked to REM sleep deprivation induced by citalopram although causal relationship needs to be investigated in further studies.

  20. [Clinical diagnose and significance of congenital sensorineural hearing loss combined with BPES].

    PubMed

    Ao, Liying; Liu, Yongzhi

    2015-09-01

    To analyze congenital sensorineural hearing loss combined with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). For the case of cochlear implantation to child with congenital sensorineural deafness combined BPES, accomplish routine examination and assessment, combining with literature to analyze the clinical diagnosis of this disease and its significance. Sensorineural hearing loss is a common congenital diseases with neonatal incidence of 1 per thousand - 3 per thousand, 50%-70% of deafness is associated with genetic factors, the incidence of congenital sensorineural hearing loss combined with eye disease is about 40%-60%, mainly reflected in ametropia and retinopathy. BPES's main clinical manifestations is blepharophimosis, ptosis, epicanthus inversus, and telecanthus. BPES is a rare autosomal dominant disease caused by FOXL 2 gene mutation, sometimes associated with retarded growth, delayed development, congenital heart disease, and microcephaly. Suffering from both sensorineural hearing loss and BPES is rare in reported literature. This case is diagnosed by clinical examination, without visual impairment. Facial nerve dysplasia has been found during the surgery. For congenital deafness patients with eye disease or other diseases, timely and correct diagnosis has important clinical significance, which can improve the diagnostic rate and make it coming true to early intervention, and then, effectively improve the quality of the patients. There are few literature reports, of patients with two kinds of genetic diseases. Our inference is that the cases are rare or the patients has visited different departments and ignored the other systems' signs. Therefore, in such doubtful cases, we should do the professional comprehensive examination in daily clinical work in order to avoid missed diagnosis or delayed treatment and intervention. By analyzing this case, the patient may also suffer from facial nerve dysplasia. Preoperatively viewing CT scan and

  1. Impaired mitochondrial function is abrogated by dexrazoxane in doxorubicin-treated childhood acute lymphoblastic leukemia survivors

    PubMed Central

    Lipshultz, Steven E.; Anderson, Lynn M.; Miller, Tracie L.; Gerschenson, Mariana; Stevenson, Kristen E.; Neuberg, Donna S.; Franco, Vivian I.; LiButti, Daniel E.; Silverman, Lewis B.; Vrooman, Lynda M.; Sallan, Stephen E.

    2015-01-01

    BACKGROUND Impaired cardiac function in doxorubicin-treated childhood cancer survivors is partly mediated by disruption of mitochondrial energy production. Doxorubicin intercalates into mitochondrial DNA (mtDNA) disrupting genes encoding for polypeptides that make ATP. METHODS This cross-sectional study examined mtDNA copy numbers/cell and oxidative phosphorylation (OXPHOS) in peripheral blood mononuclear cells (PBMCs) in 64 childhood survivors of high-risk acute lymphoblastic leukemia (ALL) treated on Dana-Farber Cancer Institute Childhood ALL protocols who had received doxorubicin alone (42%) or with dexrazoxane (58%), a cardioprotectant. Mitochondrial DNA copies per cell and OXPHOS enzyme activities of nicotinamide adenine dinucleotide (NADH) dehydrogenase (Complex I, CI) and cytochrome c oxidase (Complex IV, CIV) were measured by quantitative real time-polymerase chain reaction (qRT-PCR) immunoassay and thin layer chromatography, respectively. RESULTS At a median follow-up of 7.8 years after treatment, the median number of mtDNA copies per cell for patients treated with doxorubicin alone was significantly higher than for those who also received dexrazoxane (medians, 1106.3 and 310.5; P=0.001). No significant differences were detected between groups for CI or CIV activities. CONCLUSIONS Doxorubicin-treated survivors had increased PBMC mtDNA copies/cell and concomitant use of dexrazoxane was associated with lower mtDNA copies/cell. Due to a possible compensatory increase in mtDNA copies/cell to maintain mitochondrial function in the setting of mitochondrial dysfunction, overall OXPHOS activity was not different between groups. The long-term sustainability of this compensatory response in these survivors at risk for cardiac dysfunction over their lifespan is concerning. PMID:26762648

  2. Acute Acrolein Exposure Induces Impairment of Vocal Fold Epithelial Barrier Function

    PubMed Central

    Zheng, Wei; Sivasankar, M. Preeti

    2016-01-01

    Acrolein is a ubiquitous pollutant abundant in cigarette smoke, mobile exhaust, and industrial waste. There is limited literature on the effects of acrolein on vocal fold tissue, although there are clinical reports of voice changes after pollutant exposures. Vocal folds are responsible for voice production. The overall objective of this study was to investigate the effects of acrolein exposure on viable, excised vocal fold epithelial tissue and to characterize the mechanism underlying acrolein toxicity. Vocal fold epithelia were studied because they form the outermost layer of the vocal folds and are a primary recipient of inhaled pollutants. Porcine vocal fold epithelia were exposed to 0, 50, 100, 500, 900 or 1300 μM of acrolein for 3 hours; the metabolic activity, epithelial resistance, epithelial permeability, tight junction protein (occludin and claudin 3) expression, cell membrane integrity and lipid peroxidation were investigated. The data demonstrated that acrolein exposure at 500 μM significantly reduced vocal fold epithelial metabolic activity by 27.2% (p≤0.001). Incubation with 100 μM acrolein caused a marked increase in epithelial permeability by 130.5% (p<0.05) and a reduction in transepithelial electrical resistance (TEER) by 180.0% (p<0.001). While the expression of tight junctional protein did not change in acrolein-treated samples, the cell membrane integrity was significantly damaged with a 45.6% increase of lipid peroxidation as compared to controls (p<0.05). Taken together, these data provide evidence that acute acrolein exposure impairs vocal fold epithelial barrier integrity. Lipid peroxidation-induced cell membrane damage may play an important role in reducing the barrier function of the epithelium. PMID:27643990

  3. Impaired cognitive ability and anxiety-like behavior following acute seizures in the Theiler's virus model of temporal lobe epilepsy.

    PubMed

    Umpierre, Anthony D; Remigio, Gregory J; Dahle, E Jill; Bradford, Kate; Alex, Anitha B; Smith, Misty D; West, Peter J; White, H Steve; Wilcox, Karen S

    2014-04-01

    Viral infection of the CNS can result in encephalitis and acute seizures, increasing the risk for later-life epilepsy. We have previously characterized a novel animal model of temporal lobe epilepsy that recapitulates key sequela in the development of epilepsy following viral infection. C57BL/6J mice inoculated with the Daniel's strain of Theiler's Murine Encephalomyelitis Virus (TMEV; 3×10(5) PFU, i.c.) display acute limbic seizures that secondarily generalize. A majority of acutely seized animals develop spontaneous seizures weeks to months later. As part of our investigation, we sought to assess behavioral comorbidity following TMEV inoculation. Anxiety, depression, cognitive impairment, and certain psychoses are diagnosed in persons with epilepsy at rates far more frequent than in the general population. We used a battery of behavioral tests to assess anxiety, depression, cognitive impairment, and general health in acutely seized animals inoculated with TMEV and compared behavioral outcomes against age-matched controls receiving a sham injection. We determined that TMEV-seized animals are less likely to move through the exposed center of an open field and are less likely to enter into the lighted half of a light/dark box; both behaviors may be indicative of anxiety-like behavior. TMEV-seized animals also display early and persistent reductions in novel object exploration during novel object place tasks and do not improve in their ability to find a hidden escape platform in Morris water maze testing, indicative of impairment in episodic and spatial memory, respectively. Cresyl violet staining at 35 and 250 days after injection reveals bilateral reductions in hippocampal area, with extensive sclerosis of CA1 evident bilaterally along the rostral-caudal axis. Early and persistent behavioral changes in the TMEV model provide surrogate markers for assessing disease progression as well as endpoints in screening for the efficacy of novel compounds to manage both

  4. Acute Cardiac Impairment Associated With Concurrent Chemoradiotherapy for Esophageal Cancer: Magnetic Resonance Evaluation

    SciTech Connect

    Hatakenaka, Masamitsu; Yonezawa, Masato; Nonoshita, Takeshi; Nakamura, Katsumasa; Yabuuchi, Hidetake; Shioyama, Yoshiyuki; Nagao, Michinobu; Matsuo, Yoshio; Kamitani, Takeshi; Higo, Taiki; Nishikawa, Kei; Setoguchi, Taro; Honda, Hiroshi

    2012-05-01

    Purpose: To evaluate acute cardiac effects of concurrent chemoradiotherapy (CCRT) for esophageal cancer. Methods and Materials: This prospective study was approved by the institutional review board, and written informed consent was obtained from all participants. The left ventricular function (LVF) of 31 patients with esophageal cancer who received cisplatin and 5-fluorouracil-based CCRT was evaluated using cardiac cine magnetic resonance imaging. The patients were classified into two groups according to mean LV dose. The parameters related to LVF were compared between before and during (40 Gy) or between before and after CCRT using a Wilcoxon matched-pairs single rank test, and parameter ratios (during/before CCRT, after/before CCRT) were also compared between the groups with a t test. Data were expressed as mean {+-} SE. Results: In the low LV-dose group (n = 10; mean LV dose <0.6 Gy), LV ejection fraction decreased significantly (before vs. during vs. after CCRT; 62.7% {+-} 2.98% vs. 59.8% {+-} 2.56% vs. 60.6% {+-} 3.89%; p < 0.05). In the high LV-dose group (n = 21; mean LV dose of 3.6-41.2 Gy), LV end-diastolic volume index (before vs. after CCRT; 69.1 {+-} 2.93 vs. 57.0 {+-} 3.23 mL/m{sup 2}), LV stroke volume index (38.6 {+-} 1.56 vs. 29.9 {+-} 1.60 mL/m{sup 2}), and LV ejection fraction (56.9% {+-} 1.79% vs. 52.8% {+-} 1.15%) decreased significantly (p < 0.05) after CCRT. Heart rate increased significantly (before vs. during vs. after CCRT; 66.8 {+-} 3.05 vs. 72.4 {+-} 4.04 vs. 85.4 {+-} 3.75 beats per minute, p < 0.01). Left ventricle wall motion decreased significantly (p < 0.05) in segments 8 (before vs. during vs. after CCRT; 6.64 {+-} 0.54 vs. 4.78 {+-} 0.43 vs. 4.79 {+-} 0.50 mm), 9 (6.88 {+-} 0.45 vs. 5.04 {+-} 0.38 vs. 5.27 {+-} 0.47 mm), and 10 (9.22 {+-} 0.48 vs. 8.08 {+-} 0.34 vs. 8.19 {+-} 0.56 mm). The parameter ratios of LV end-diastolic volume index, stroke volume index, wall motion in segment 9, and heart rate showed significant difference

  5. Acute impairment of saccadic eye movements is associated with delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage.

    PubMed

    Rowland, Matthew J; Garry, Payashi; Westbrook, Jon; Corkill, Rufus; Antoniades, Chrystalina A; Pattinson, Kyle T S

    2016-12-09

    OBJECTIVE Delayed cerebral ischemia (DCI) causing cerebral infarction remains a significant cause of morbidity and mortality following aneurysmal subarachnoid hemorrhage (aSAH). Early brain injury in the first 72 hours following rupture is likely to play a key role in the pathophysiology underlying DCI but remains difficult to quantify objectively. Current diagnostic modalities are based on the concept of vasoconstriction causing cerebral ischemia and infarction and are either invasive or have a steep learning curve and user variability. The authors sought to determine whether saccadic eye movements are impaired following aSAH and whether this measurement in the acute period is associated with the likelihood of developing DCI. METHODS As part of a prospective, observational cohort study, 24 male and female patients (mean age 53 years old, range 31-70 years old) were recruited. Inclusion criteria included presentation with World Federation of Neurosurgical Societies (WFNS) Grades 1 or 2 ("good grade") aSAH on admission and endovascular treatment within 72 hours of aneurysmal rupture. DCI and DCI-related cerebral infarction were defined according to consensus guidelines. Saccadometry data were collected at 3 time points in patients: in the first 72 hours, between Days 5 and 10, and at 3 months after aSAH. Data from 10 healthy controls was collected on 1 occasion for comparison. RESULTS Age-adjusted saccadic latency in patients was significantly prolonged in the first 72 hours following aSAH when compared with controls (188.7 msec [95% CI 176.9-202.2 msec] vs 160.7 msec [95% CI 145.6-179.4 msec], respectively; p = 0.0054, t-test). By 3 months after aSAH, there was no significant difference in median saccadic latency compared with controls (188.7 msec [95% CI 176.9-202.2 msec] vs 180.0 msec [95% CI 165.1-197.8 msec], respectively; p = 0.4175, t-test). Patients diagnosed with cerebral infarction due to DCI had a significantly higher age-adjusted saccadic latency in the

  6. Prevalence of mild cognitive impairment in employable patients after acute coronary event in cardiac rehabilitation

    PubMed Central

    Salzwedel, Annett; Heidler, Maria-Dorothea; Haubold, Kathrin; Schikora, Martin; Reibis, Rona; Wegscheider, Karl; Jöbges, Michael; Völler, Heinz

    2017-01-01

    Introduction Adequate cognitive function in patients is a prerequisite for successful implementation of patient education and lifestyle coping in comprehensive cardiac rehabilitation (CR) programs. Although the association between cardiovascular diseases and cognitive impairments (CIs) is well known, the prevalence particularly of mild CI in CR and the characteristics of affected patients have been insufficiently investigated so far. Methods In this prospective observational study, 496 patients (54.5 ± 6.2 years, 79.8% men) with coronary artery disease following an acute coronary event (ACE) were analyzed. Patients were enrolled within 14 days of discharge from the hospital in a 3-week inpatient CR program. Patients were tested for CI using the Montreal Cognitive Assessment (MoCA) upon admission to and discharge from CR. Additionally, sociodemographic, clinical, and physiological variables were documented. The data were analyzed descriptively and in a multivariate stepwise backward elimination regression model with respect to CI. Results At admission to CR, the CI (MoCA score < 26) was determined in 182 patients (36.7%). Significant differences between CI and no CI groups were identified, and CI group was associated with high prevalence of smoking (65.9 vs 56.7%, P = 0.046), heavy (physically demanding) workloads (26.4 vs 17.8%, P < 0.001), sick leave longer than 1 month prior to CR (28.6 vs 18.5%, P = 0.026), reduced exercise capacity (102.5 vs 118.8 W, P = 0.006), and a shorter 6-min walking distance (401.7 vs 421.3 m, P = 0.021) compared to no CI group. The age- and education-adjusted model showed positive associations with CI only for sick leave more than 1 month prior to ACE (odds ratio [OR] 1.673, 95% confidence interval 1.07–2.79; P = 0.03) and heavy workloads (OR 2.18, 95% confidence interval 1.42–3.36; P < 0.01). Conclusion The prevalence of CI in CR was considerably high, affecting more than one-third of cardiac patients. Besides age and education

  7. Early-onset sensorineural hearing loss in Lassa fever.

    PubMed

    Ibekwe, T S; Okokhere, P O; Asogun, D; Blackie, F F; Nwegbu, M M; Wahab, K W; Omilabu, S A; Akpede, G O

    2011-02-01

    Lassa fever (LF) is a viral hemorrhagic disease which affects one-fourth to two million people annually with the fatality rate of about 10,000. It is associated with sensorineural hearing loss (SNHL) usually at the convalescent stage. Recently, cases of SNHL at the acute phase have been reported. This study was done to further investigate the incidence and features of SNHL in acute phase of LF. It is a prospective case-control study of LF patients seen with acute SNHL conducted between July 2007 and April 2009 at Irrua Specialist Teaching Hospital Nigeria. The diagnosis of acute LF was based on the clinical features and detection of IgM antibodies and/or positive Lassa virus-specific reverse transcriptase-polymerase chain reaction using primers S36+ and LVS 339 while SNHL was diagnosed clinically and confirmed with PTA and speech discrimination tests. Patients with other acute febrile illnesses were used as control. Statistical analysis was done using SPSS version 11 and Fisher's exact test while level of significance was set at p < 0.05. Out of the 37 confirmed cases of LF, 5 (13.5%) and none (0%) of the control developed early-onset SNHL (p = 0.03). Forty percent of the cases studied had negative IgM. The audiograms showed involvement at all frequency groups with pure tone average 65-85 dB and the speech discrimination 20-40%. The overall case fatality rate was 27.0%, and for early SNHL cases 60.0% (p > 0.05). The incidence of SNHL in LF infection is about 13.5% and could be a reflection of a worse disease process. There is possibility of direct viral invasion aside immunological reaction as a causative mechanism.

  8. Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?

    PubMed

    Ishibashi, S; Yokota, T; Shiojiri, T; Matunaga, T; Tanaka, H; Nishina, K; Hirota, H; Inaba, A; Yamada, M; Kanda, T; Mizusawa, H

    2003-05-01

    Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.

  9. Reconciling conceptualizations of relationships and person-centred care for older people with cognitive impairment in acute care settings.

    PubMed

    Rushton, Carole; Edvardsson, David

    2017-02-10

    Relationships are central to enacting person-centred care of the older person with cognitive impairment. A fuller understanding of relationships and the role they play facilitating wellness and preserving personhood is critical if we are to unleash the productive potential of nursing research and person-centred care. In this article, we target the acute care setting because much of the work about relationships and older people with cognitive impairment has tended to focus on relationships in long-term care. The acute care setting is characterized by archetypal constraints which differentiate it from long-term care, in terms of acuity and haste, task-orientated work patterns and influence from "the rule of medicine," all of which can privilege particular types of relating. In this article, we drew on existing conceptualizations of relationships from theory and practice by tapping in to the intellectual resources provided by nurse researchers, the philosophy of Martin Buber and ANT scholars. This involved recounting two examples of dyadic and networked relationships which were re-interpreted using two complementary theoretical approaches to provide deeper and more comprehensive conceptualizations of these relationships. By re-presenting key tenets from the work of key scholars on the topic relationships, we hope to hasten socialization of these ideas into nursing into the acute care setting. First, by enabling nurses to reflect on how they might work toward cultivating relationships that are more salutogenic and consistent with the preservation of personhood. Second, by stimulating two distinct but related lines of research enquiry which focus on dyadic and networked relationships with the older person with cognitive impairment in the acute care setting. We also hope to reconcile the schism that has emerged in the literature between preferred approaches to care of the older person with cognitive impairment, that is person-centred care versus relationship-centred care

  10. Fish Oil Diet Associated with Acute Reperfusion Related Hemorrhage, and with Reduced Stroke-Related Sickness Behaviors and Motor Impairment

    PubMed Central

    Pascoe, Michaela C.; Howells, David W.; Crewther, David P.; Constantinou, Nicki; Carey, Leeanne M.; Rewell, Sarah S.; Turchini, Giovanni M.; Kaur, Gunveen; Crewther, Sheila G.

    2014-01-01

    Ischemic stroke is associated with motor impairment and increased incidence of affective disorders such as anxiety/clinical depression. In non-stroke populations, successful management of such disorders and symptoms has been reported following diet supplementation with long chain omega-3-polyunsaturated-fatty-acids (PUFAs). However, the potential protective effects of PUFA supplementation on affective behaviors after experimentally induced stroke and sham surgery have not been examined previously. This study investigated the behavioral effects of PUFA supplementation over a 6-week period following either middle cerebral artery occlusion or sham surgery in the hooded-Wistar rat. The PUFA diet supplied during the acclimation period prior to surgery was found to be associated with an increased risk of acute hemorrhage following the reperfusion component of the surgery. In surviving animals, PUFA supplementation did not influence infarct size as determined 6 weeks after surgery, but did decrease omega-6-fatty-acid levels, moderate sickness behaviors, acute motor impairment, and longer-term locomotor hyperactivity and depression/anxiety-like behavior. PMID:24567728

  11. Fish oil diet associated with acute reperfusion related hemorrhage, and with reduced stroke-related sickness behaviors and motor impairment.

    PubMed

    Pascoe, Michaela C; Howells, David W; Crewther, David P; Constantinou, Nicki; Carey, Leeanne M; Rewell, Sarah S; Turchini, Giovanni M; Kaur, Gunveen; Crewther, Sheila G

    2014-01-01

    Ischemic stroke is associated with motor impairment and increased incidence of affective disorders such as anxiety/clinical depression. In non-stroke populations, successful management of such disorders and symptoms has been reported following diet supplementation with long chain omega-3-polyunsaturated-fatty-acids (PUFAs). However, the potential protective effects of PUFA supplementation on affective behaviors after experimentally induced stroke and sham surgery have not been examined previously. This study investigated the behavioral effects of PUFA supplementation over a 6-week period following either middle cerebral artery occlusion or sham surgery in the hooded-Wistar rat. The PUFA diet supplied during the acclimation period prior to surgery was found to be associated with an increased risk of acute hemorrhage following the reperfusion component of the surgery. In surviving animals, PUFA supplementation did not influence infarct size as determined 6 weeks after surgery, but did decrease omega-6-fatty-acid levels, moderate sickness behaviors, acute motor impairment, and longer-term locomotor hyperactivity and depression/anxiety-like behavior.

  12. KCNQ/Kv7 channel activator flupirtine protects against acute stress-induced impairments of spatial memory retrieval and hippocampal LTP in rats.

    PubMed

    Li, C; Huang, P; Lu, Q; Zhou, M; Guo, L; Xu, X

    2014-11-07

    Spatial memory retrieval and hippocampal long-term potentiation (LTP) are impaired by stress. KCNQ/Kv7 channels are closely associated with memory and the KCNQ/Kv7 channel activator flupirtine represents neuroprotective effects. This study aims to test whether KCNQ/Kv7 channel activation prevents acute stress-induced impairments of spatial memory retrieval and hippocampal LTP. Rats were placed on an elevated platform in the middle of a bright room for 30 min to evoke acute stress. The expression of KCNQ/Kv7 subunits was analyzed at 1, 3 and 12 h after stress by Western blotting. Spatial memory was examined by the Morris water maze (MWM) and the field excitatory postsynaptic potential (fEPSP) in the hippocampal CA1 area was recorded in vivo. Acute stress transiently decreased the expression of KCNQ2 and KCNQ3 in the hippocampus. Acute stress impaired the spatial memory retrieval and hippocampal LTP, the KCNQ/Kv7 channel activator flupirtine prevented the impairments, and the protective effects of flupirtine were blocked by XE-991 (10,10-bis(4-Pyridinylmethyl)-9(10H)-anthracenone), a selective KCNQ channel blocker. Furthermore, acute stress decreased the phosphorylation of glycogen synthase kinase-3β (GSK-3β) at Ser9 in the hippocampus, and flupirtine inhibited the reduction. These results suggest that the KCNQ/Kv7 channels may be a potential target for protecting both hippocampal synaptic plasticity and spatial memory retrieval from acute stress influences.

  13. Association between Metabolic Syndrome and Cognitive Impairment after Acute Ischemic Stroke: A Cross-Sectional Study in a Chinese Population

    PubMed Central

    Li, Pan; Quan, Wei; Lu, Da; Wang, Yan; Zhang, Hui-Hong; Liu, Shuai; Jiang, Rong-Cai; Zhou, Yu-Ying

    2016-01-01

    Background and Objectives Metabolic syndrome (MetS), a risk factor for many vascular conditions, is associated with vascular cognitive disorders. The objective of the present study was to explore the associations of MetS and its individual components with the risks of cognitive impairment and neurological dysfunction in patients after acute stroke. Methods This cross-sectional study enrolled 840 patients ranging in age from 53 to 89 years from the Tianjin area of North China. Cognitive function was evaluated using the Montreal Cognitive Assessment (MoCA) and Mini-Mental State Examination. Neuropsychiatric behavior was assessed using the Neuropsychiatric Inventory Questionnaire. Emotional state was examined according to the Hamilton Depression Rating Scale, and neuromotor function was evaluated using the National Institutes of Health Stroke Scale, Barthel index, and the Activity of Daily Living test. After overnight fasting, blood samples were obtained to measure biochemistry indicators. Results MetS and its individual components were closely correlated with MoCA score. MetS patients had high levels of inflammation and a 3.542-fold increased odds ratio (OR) for cognitive impairment [95% confidence interval (CI): 1.972–6.361]. Of the individual MetS components, central obesity (OR 3.039; 95% CI: 1.839–5.023), high fasting plasma glucose (OR 1.915; 95% CI: 1.016–3.607), and type 2 diabetes (OR 2.241; 95% CI: 1.630–3.081) were associated with an increased incidence of cognitive impairment. Consistent and significant worsening in different neurological domains was observed with greater numbers of MetS components. Conclusions MetS was associated with worse cognitive function, neuromotor dysfunction, and neuropsychological symptoms among Chinese acute stroke patients. PMID:27936074

  14. Impaired neuroendocrine and immune response to acute stress in medication-naive patients with a first episode of psychosis.

    PubMed

    van Venrooij, Janine A E M; Fluitman, Sjoerd B A H A; Lijmer, Jeroen G; Kavelaars, Annemieke; Heijnen, Cobi J; Westenberg, Herman G M; Kahn, René S; Gispen-de Wied, Christine C

    2012-03-01

    Little is known about how the biological stress response systems--the autonomic nervous system (ANS), the hypothalamic-pituitary-adrenal (HPA) axis, and the immune system--function during psychosis. Results of studies on the effect of stress on the immune and autonomic system in patients with schizophrenia are inconsistent. The present study investigates whether the stress response is impaired in medication-naive patients with a first episode of psychosis. Ten male patients with a first episode of psychosis and 15 controls were exposed to the stress of public speaking. Parameters of the ANS (heart rate and catecholamines), the HPA axis (plasma adrenocorticotropic hormone [ACTH] and cortisol), and the immune system (number and activity of natural killer [NK] cells) were measured. Peak responses were calculated to examine the relationship between stress-induced activation of the different systems. Subjective stress and anxiety before and during the task were assessed. Patients and controls displayed similar autonomic responses to acute stress. However, there was an impaired HPA axis response, slow onset and return of ACTH, and flattened cortisol response and a reduced increase in number NK cells and NK cell activity in patients with a first episode of psychosis. Furthermore, in patients, the relationship between the different stress response systems was weaker or absent compared with controls. These findings indicate that impairments in stress processing are associated with the endophenotype of psychosis and are not a result of illness progression or antipsychotic medication.

  15. Acute Stress Dysregulates the LPP ERP Response to Emotional Pictures and Impairs Sustained Attention: Time-Sensitive Effects

    PubMed Central

    Alomari, Rima A.; Fernandez, Mercedes; Banks, Jonathan B.; Acosta, Juliana; Tartar, Jaime L.

    2015-01-01

    Stress can increase emotional vigilance at the cost of a decrease in attention towards non-emotional stimuli. However, the time-dependent effects of acute stress on emotion processing are uncertain. We tested the effects of acute stress on subsequent emotion processing up to 40 min following an acute stressor. Our measure of emotion processing was the late positive potential (LPP) component of the visual event-related potential (ERP), and our measure of non-emotional attention was the sustained attention to response task (SART). We also measured cortisol levels before and after the socially evaluated cold pressor test (SECPT) induction. We found that the effects of stress on the LPP ERP emotion measure were time sensitive. Specifically, the LPP ERP was only altered in the late time-point (30–40 min post-stress) when cortisol was at its highest level. Here, the LPP no longer discriminated between the emotional and non-emotional picture categories, most likely because neutral pictures were perceived as emotional. Moreover, compared to the non-stress condition, the stress-condition showed impaired performance on the SART. Our results support the idea that a limit in attention resources after an emotional stressor is associated with the brain incorrectly processing non-emotional stimuli as emotional and interferes with sustained attention. PMID:26010485

  16. Contribution of High Frequencies to Speech Recognition in Quiet and Noise in Listeners with Varying Degrees of High-Frequency Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Amos, Nathan E.; Humes, Larry E.

    2007-01-01

    Purpose: The contribution of audible high-frequency information to speech-understanding performance in listeners with varying degrees of high-frequency sensorineural hearing loss was examined. Method: Thirty-six elderly hearing-impaired (EHI) and 24 young normal-hearing (YNH) listeners were tested in quiet (+20 dB speech-to-noise ratio [SNR]) and…

  17. Vibrant Soundbridge rehabilitation of sensorineural hearing loss.

    PubMed

    Khan, Andleeb; Hillman, Todd; Chen, Douglas

    2014-12-01

    The Vibrant Soundbridge is a means to rehabilitate patients with sensorineural hearing loss. It differs from hearing aids in that it uses mechanical energy rather than acoustic sound to deliver better sound quality to the inner ear. The implant's crucial component is a floating mass transducer that is directly fixed to the incus to drive it, which is introduced into the middle ear through a facial recess approach. Although this is a newer technology, studies thus far have demonstrated better hearing results compared with hearing aids in terms of functional gain and speech intelligibility, and better outcomes on subjective assessments.

  18. Laterality of sudden sensorineural hearing loss.

    PubMed

    Reiss, Michael; Reiss, Gilfe

    2014-08-01

    It is known that sudden sensorineural hearing loss and other otoneurologic diseases, such as tinnitus or Ménière disease, occur more frequently in the left ear than in the right. We studied lateralization of sudden deafness in 489 patients treated at Radebeul Hospital from January 2004 to December 2009. The male-to-female ratio was 1:1.24; we found a predominance of the left side only in female patients. The cause for this predominance is unclear. The slight asymmetry might indicate a greater vulnerability of the left inner ear in women, suggesting hormonal factors in the genesis of sudden deafness.

  19. Sudden Sensorineural Hearing Loss: Primary Care Update

    PubMed Central

    Leung, Marcia A; Flaherty, Anna; Zhang, Julia A; Hara, Jared; Barber, Wayne

    2016-01-01

    The primary care physician's role in recognizing sudden sensorineural hearing (SSNHL) loss and delivering initial treatment is critical in the management of the syndrome. This role involves recognizing its clinical symptoms, distinguishing it from conductive hearing loss with the Weber tuning fork or the Rauch hum test, and urgent administration of high dose oral corticosteroids. Diagnosis and treatment should not be delayed for audiometric testing or referral to otolaryngology. This paper provides an update on the initial evaluation and treatment of this syndrome based on the literature and clinical guideline recommendations. PMID:27413627

  20. Sudden sensorineural hearing loss associated with vardenafil.

    PubMed

    Snodgrass, Amanda J; Campbell, Heather M; Mace, David L; Faria, Victoria L; Swanson, Kathleen M; Holodniy, Mark

    2010-01-01

    The phosphodiesterase type 5 (PDE-5) inhibitors-sildenafil, vardenafil, and tadalafil-are used primarily in erectile dysfunction, but sildenafil is also indicated for pulmonary hypertension. Common adverse effects of vardenafil include headache, flushing, nasal congestion, dyspepsia, and nausea. Recently, PDE-5 inhibitors have been associated with adverse vision effects, and emerging evidence now indicates that they may also be responsible for hearing changes and hearing loss. We describe a patient who developed unilateral sudden sensorineural hearing loss possibly related to the use of vardenafil for erectile dysfunction. To our knowledge, only one other case of hearing loss related to this drug class has been published. Our patient was a 57-year-old man who came to the emergency department with right-sided mild-to-moderate hearing loss in the 500-3000-Hz range, confirmed by audiogram, that occurred after ingestion of vardenafil. The patient was hospitalized 2 days later for administration of intravenous dexamethasone, followed by oral prednisone. He reported that his hearing had improved on the fourth hospital day and was discharged 3 days later, continuing to taper the prednisone on an outpatient basis. A repeat audiogram after 10 days of corticosteroid therapy confirmed that his hearing in the 500-3000-Hz range was within normal limits. Use of the Naranjo adverse drug reaction probability scale indicated a possible (score of 3) adverse reaction of sudden sensorineural hearing loss associated with vardenafil consumption. We also performed an analysis of hearing loss cases related to PDE-5 inhibitors in the United States Food and Drug Administration's Adverse Event Reporting System database to compare the characteristics of our patient with those of other reported adverse event cases. Based on the temporal relation of the sudden sensorineural hearing loss to this patient's drug consumption, we propose that the vardenafil is a likely cause of the hearing loss

  1. [Review of functional impairment associated with acute illness in the elderly].

    PubMed

    Osuna-Pozo, Carmen María; Ortiz-Alonso, Javier; Vidán, Maite; Ferreira, Guillermo; Serra-Rexach, José Antonio

    2014-01-01

    Hospitalization is a risk for elderly population, with a high probability of having adverse events. The most important one is functional impairment, due to its high prevalence and the serious impact it has on the quality of life. The main risk factors for functional decline associated with hospitalization are, age, immobility, cognitive impairment, and functional status prior to admission. It is necessary to detect patients at risk in order to implement the necessary actions to prevent this deterioration, with physical exercise and multidisciplinary geriatric care being the most important.

  2. Enhanced muscle shortening and impaired Ca2+ channel function in an acute septic myopathy model.

    PubMed

    Friedrich, Oliver; Hund, Ernst; von Wegner, Frederic

    2010-04-01

    Myopathies in critically ill patients are increasingly documented. Various animal models of chronic sepsis have been employed to investigate reduced membrane excitability or altered isometric contractility of skeletal muscle. In contrast, immediate changes occurring during acute sepsis are significantly under-characterised; L-type Ca(2+) channel function or isotonic shortening are examples. We recorded slowly activating L-type Ca(2+) currents (I (Ca)) in voltage-clamped single intact mouse skeletal muscle fibres and tested the effects of acute challenge with serum fractions from critical illness myopathy patients (CIM). Using a high-speed camera system, we simultaneously recorded unloaded fibre shortening during isotonic contractions with unprecedented temporal resolution (approximately 1,600 frames/s). Time courses of fibre lengths and shortening velocity were determined from automated imaging algorithms. CIM fractions acutely induced depression of I (Ca) amplitudes with no shifts in I (Ca)-V-relations. Voltage-dependent inactivation was unaltered and I (Ca) activation and inactivation kinetics were prolonged compared to controls. Unexpectedly, maximum unloaded speed of shortening was slightly faster following CIM serum applications, suggesting a direct action of CIM serum on weak-binding-state cross-bridges. Our results are compatible with a model where CIM serum might acutely reduce a fraction of functional L-type Ca(2+) channels and could account for reduced SR Ca(2+) release and force production in CIM patients. Acute increase in isotonic shortening velocity might be an early diagnostic feature suitable for testing in clinical studies. The acute challenge model is also robust against atrophy or fibre type changes that ordinarily would have to be considered in chronic sepsis models.

  3. Exogenous hydrogen sulfide eliminates spatial memory retrieval impairment and hippocampal CA1 LTD enhancement caused by acute stress via promoting glutamate uptake.

    PubMed

    He, Jin; Guo, Ruixian; Qiu, Pengxin; Su, Xingwen; Yan, Guangmei; Feng, Jianqiang

    2017-03-20

    Acute stress impairs the hippocampus-dependent spatial memory retrieval, and its synaptic mechanisms are associated with hippocampal CA1 long-term depression (LTD) enhancement in the adult rats. Endogenous hydrogen sulfide (H2S) is recognized as a novel gasotransmitter and has the neural protective roles. However, very little attention has been paid to understanding the effects of H2S on spatial memory retrieval impairment. We observed the protective effects of NaHS (a donor of H2S) against spatial memory retrieval impairment caused by acute stress and its synaptic mechanisms. Our results showed that NaHS abolished spatial memory retrieval impairment and hippocampal CA1 LTD enhancement caused by acute stress, but not by glutamate transporter inhibitor l-trans-pyrrolidine-2,4-dicarboxylic (tPDC), indicating that the activation of glutamate transporters is necessary for exogenous H2S to exert its roles. Moreover, NaHS restored the decreased glutamate uptake in the hippocampal CA1 synaptosomal fraction caused by acute stress. Dithiothreitol (DTT, a disulfide reducing agent) abolished a decrease in the glutamate uptake caused by acute stress, and NaHS eradicated the decreased glutamate uptake caused by 5,5'-dithio-bis(2-nitrobenzoic)acid (DTNB, a thiol oxidizing agent), collectively, revealing that exogenous H2S increases glutamate uptake by reducing disulfide bonds of the glutamate transporters. Additionally, NaHS inhibited the increased expression level of phosphorylated c-Jun-N-terminal kinase (JNK) in the hippocampal CA1 region caused by acute stress. The JNK inhibitor SP600125 eliminated spatial memory retrieval impairment, hippocampal CA1 LTD enhancement and the decreased glutamate uptake caused by acute stress, indicating that exogenous H2S exerts these roles by inhibiting the activation of JNK signaling pathway.

  4. Acute ozone (O3) -induced impairment of glucose regulation: Age-related and temporal changes

    EPA Science Inventory

    O3 is associated with adverse cardiopulmonary health effects in humans and is thought to produce metabolic effects, such as insulin resistance. Recently, we showed that episodic O3 exposure increased insulin levels in aged rats. We hypothesized that O3 exposure could impair gluc...

  5. Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome.

    PubMed

    Lee, Eun Jung; Yoon, Yong Joo

    2012-01-01

    This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment.

  6. Auditory Word Comprehension Impairment in Acute Stroke: Relative Contribution of Phonemic versus Semantic Factors

    ERIC Educational Resources Information Center

    Rogalsky, Corianne; Pitz, Eleanor; Hillis, Argye E.; Hickok, Gregory

    2008-01-01

    Auditory word comprehension was assessed in a series of 289 acute left hemisphere stroke patients. Participants decided whether an auditorily presented word matched a picture. On different trials, words were presented with a matching picture, a semantic foil, or a phonemic foil. Participants had significantly more trouble with semantic foils…

  7. Sensorineural hearing loss and auditory perceptual organization

    NASA Astrophysics Data System (ADS)

    Hall, Joseph W.; Grose, John H.; Buss, Emily

    2004-05-01

    This talk will consider the implications of sensorineural hearing loss for auditory perceptual organization. In everyday environments, the listener is often faced with the difficulty of processing a target sound that intermingles acoustically with one or more extraneous sounds. Under such circumstances, several auditory processes enable the complex waveforms reaching the two ears to be interpreted in terms of putative auditory objects giving rise to the target and extraneous sounds. Such processes of perceptual organization depend upon the central analysis of cues that allow distributed spectral information to be either linked together or split apart on the basis of details related to such variables as synchrony of onset/modulation, harmonic relation, rhythm, and interaural differences. Efficient perceptual organization must depend not only upon such central auditory analyses but also upon the fidelity with which the peripheral auditory system encodes the spectral and temporal characteristics of sound. We will consider the implications of sensorineural hearing loss for perceptual organization in terms of both peripheral and central auditory processes.

  8. Hyperbaric oxygen therapy in sudden sensorineural hearing loss following spinal anesthesia: case reports.

    PubMed

    Carneiro, Sandra N; Guerreiro, Ditza V; Cunha, Anita M; Camacho, Óscar F; Aguiar, Isabel C

    2016-01-01

    The management of sudden sensorineural hearing loss following spinal anesthesia is currently an open problem. Several strategies have been used with variable results and, to the best of our knowledge, there are no prior accounts in the literature on the use of hyperbaric oxygen therapy in the treatment of this complication. We report two cases of acute onset of unilateral hearing loss after spinal anesthesia, with significantly improved results after hyperbaric oxygen therapy. A hypothesis on the possible mechanism behind this complication is discussed. A relation is established between hyperbaric oxygen therapy and this hypothetical mechanism, in order to explain successful results in the reported cases.

  9. Dissonance perception by listeners with sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Tufts, Jennifer B.; Leek, Marjorie R.; Molis, Michelle R.

    2004-05-01

    The perceived dissonance of two simultaneous tones (a dyad) depends upon their frequency separation (in the case of two pure tones) or their fundamental frequency ratio (in the case of two harmonic complex tones). The purpose of this study was to determine whether the perceived dissonance of puretone and harmonic complex dyads is altered in the presence of sensorineural hearing (SNHL), and, if so, whether this can be explained by the reduced frequency selectivity typically associated with SNHL. Four normal-hearing and four hearing-impaired listeners evaluated the dissonance of puretone and harmonic complex dyads centered at 500 and 2000 Hz. Frequency selectivity was estimated at 500 and 2000 Hz for each listener. People with SNHL rated the dissonance of puretone dyads similarly to the normal-hearing listeners, although auditory sensitivity and frequency selectivity differed considerably between the groups. However, their ratings of harmonic complex dyads did not show the pronounced differences in dissonance as a function of fundamental frequency ratio that were observed in the normal-hearing group. The poorer frequency selectivity of these listeners may have allowed more extensive interactions to occur among harmonic components, resulting in a less clear separation of dissonance and consonance. [Work supported by NIH-NIDCD.

  10. Sensorineural hearing loss after otitis media with effusion and subacute mastoiditis after viral infections of the upper respiratory tract: A comparative study of conservative and surgical treatment.

    PubMed

    Wilhelm, Thomas; Stelzer, Tim; Hagen, Rudolf

    2016-09-01

    Involvement of the middle ear after viral infections of the upper airways may lead to serous otitis media with effusion in the absence of bacterial infection. This can be accompanied by a concomitant shading of the mastoid air cells, which could manifest as a reduced opacity on computed tomography (CT) in the absence of a history of chronic mastoiditis or acute inflammatory signs. This can lead to a subsequent impairment of inner ear function. CT scans reveal an extended pneumatization of the temporal bones in affected patients. Inner ear hearing impairment can probably be attributed to a concomitant labyrinthine reaction-the so-called toxic inner ear lesion. If no remission occurs within 5 days after initial conservative treatment (paracentesis or hemorrheologic infusions), surgical treatment with a mastoidectomy can accelerate hearing restoration. We conducted a retrospective, nonrandomized study of short- and long-term hearing outcomes in patients with a toxic inner ear lesion who had been treated with conservative measures alone (CONS group) or with surgery (SURG group) in a tertiary care referral center. Our study group was made up of 52 consecutively presenting patients (57 ears) who had been seen over a 10-year period; there were 20 patients (21 ears) in the CONS group and 32 patients (36 ears) in the SURG group. Initially, 15 CONS patients (75%) and 18 SURG patients (56%) complained of dizziness or a balance disorder. The initial averaged sensorineural hearing loss (over 0.5, 1.0, 2.0, and 3.0 kHz) was 32.4 ± 15.6 dB in the CONS group and 35.4 ± 12.0 dB in the SURG group. At follow-up (mean: 31.7 mo), the SURG group experienced a significantly greater improvement in hearing (p = 0.025). We conclude that patients with viral otitis media and concomitant noninflammatory mastoiditis with impairment of inner ear function (sensorineural hearing loss) experience a better hearing outcome when a mastoidectomy is performed during primary treatment.

  11. Acute alcohol consumption impairs controlled but not automatic processes in a psychophysical pointing paradigm.

    PubMed

    Johnston, Kevin; Timney, Brian; Goodale, Melvyn A

    2013-01-01

    Numerous studies have investigated the effects of alcohol consumption on controlled and automatic cognitive processes. Such studies have shown that alcohol impairs performance on tasks requiring conscious, intentional control, while leaving automatic performance relatively intact. Here, we sought to extend these findings to aspects of visuomotor control by investigating the effects of alcohol in a visuomotor pointing paradigm that allowed us to separate the influence of controlled and automatic processes. Six male participants were assigned to an experimental "correction" condition in which they were instructed to point at a visual target as quickly and accurately as possible. On a small percentage of trials, the target "jumped" to a new location. On these trials, the participants' task was to amend their movement such that they pointed to the new target location. A second group of 6 participants were assigned to a "countermanding" condition, in which they were instructed to terminate their movements upon detection of target "jumps". In both the correction and countermanding conditions, participants served as their own controls, taking part in alcohol and no-alcohol conditions on separate days. Alcohol had no effect on participants' ability to correct movements "in flight", but impaired the ability to withhold such automatic corrections. Our data support the notion that alcohol selectively impairs controlled processes in the visuomotor domain.

  12. Use of the Cognitive Performance Scale (CPS) to detect cognitive impairment in the acute care setting: concurrent and predictive validity.

    PubMed

    Büla, Christophe J; Wietlisbach, Vincent

    2009-10-28

    The Cognitive Performance Scale (CPS) was initially designed to assess cognition in long term care residents. Subsequently, the CPS has also been used among in-home, post-acute, and acute care populations even though CPS' clinimetric performance has not been studied in these settings. This study aimed to determine CPS agreement with the Mini Mental Status Exam (MMSE) and its predictive validity for institutionalization and death in a cohort (N=401) of elderly medical inpatients aged 75 years and over. Medical, physical and mental status were assessed upon admission. The same day, the patient's nurse completed the CPS by interview. Follow-up data were gathered from the central billing system (nursing home stay) and proxies (death). Cognitive impairment was present in 92 (23%) patients according to CPS (score >or= 2). Agreement with MMSE was moderate (kappa 0.52, P<.001). Analysis of discordant results suggested that cognitive impairment was overestimated by the CPS in dependent patients with comorbidities and depressive symptoms, and underestimated in older ones. During follow-up, subjects with abnormal CPS had increased risks of death (adjusted hazard ratio (adjHR) 1.7, 95% CI 1.0-2.8, P=.035) and institutionalization (adjHR 2.7, 95% CI 1.3-5.3, P=.006), independent of demographic, health and functional status. Interestingly, subjects with abnormal CPS were at increased risk of death only if they also had abnormal MMSE. The CPS predicted death and institutionalization during follow-up, but correlated moderately well with the MMSE. Combining CPS and MMSE provided additional predictive information, suggesting that domains other than cognition are assessed by professionals when using the CPS in elderly medical inpatients.

  13. [The audiological phenotype and the prevalence of GJB2-related sensorineural loss of hearing in the infants suffering acoustic disturbances].

    PubMed

    Lalaiants, M R; Markova, T G; Bakhshinian, V V; Bliznets, E A; Poliakov, A V; Tavartikiladze, G A

    2014-01-01

    The objective of the present work was to study specific features of the audiological phenotype and the prevalence of GJB2-related sensorineural hearing loss (SNHL) in the infants suffering acoustic disturbances. The study included 264 children with bilateral non-syndromic sensorineural loss of hearing diagnosed during the first year of life by means of detailed audiological examination that included tympanometry, registration of short-latency auditory action potentials (SLAAP), delayed evoked otoacoustic emission (DEOAE), distortion product-frequency otoacoustic emission (DPFOAE), and auditory brain-stem response (ABR). In addition, stationary acoustically evoked responses (SAER) were recorded in 38 children presenting with hearing impairment associated with GJB2-related sensorineural loss of hearing. The follow-up dynamic study involved 113 children subjected to repeated audiological examination. The study revealed the genotype with pathological mutations in 182 (69.0%) children including 171 (64.8%) ones with biallelic mutations and 11 (4.2%) with a single mutation (heterozygous genotype). Eighty two (31.0%) children had genotype without mutations. A total of 21 different mutations and 30 different genotypes were identified. Analysis of the family histories of the children showed that neither the absence of relatives suffering from hearing impairment nor the presence of risk factors of acquired hearing impairment excludes the possibility of GJB2-related sensorineural loss of hearing in the infants. Otoacoustic emission fails to be registered in the majority of the children with the altered genotype (87%) during the stay in the maternity house. Mutations in the GJB2 gene are most frequently diagnosed in the patients with the moderate, moderately severe, and severe loss of hearing. At the same time, almost half of the infants presenting with the mild loss of hearing were found to exhibit changes in the GJB2 gene. The thresholds of registration of short

  14. Acute-onset unilateral psychogenic hearing loss in adults: report of six cases and diagnostic pitfalls.

    PubMed

    Oishi, Naoki; Kanzaki, Sho; Kataoka, Chinatsu; Tazoe, Mami; Takei, Yasuhiko; Nagai, Keiichi; Kohno, Naoyuki; Ogawa, Kaoru

    2009-01-01

    We encountered 6 rare cases of acute-onset unilateral psychogenic hearing loss in adults. All were women in their 20s and 30s. Three cases had severe hearing impairment characterized by hearing loss at every frequency; 2 cases had profound hearing impairment, and 1 case had low-frequency hearing impairment. Of the 6 cases, 3 had a history of hearing loss, and 1 had a history of psychogenic visual disturbance. All 6 cases were initially diagnosed as having idiopathic sudden sensorineural hearing loss; all subsequently received steroid therapy. Three cases were not diagnosed as being psychogenic in origin until otoacoustic emissions and auditory brain responses were performed. Although the presence of distinctive clinical features (age, gender, and past history) is important for suspecting psychogenic hearing loss, objective audiological tests such as otoacoustic emissions are essential for diagnosing some cases. Compared to the existing reports of similar cases, our cases had a poorer prognosis (only 2 cases were cured).

  15. Acute paraquat exposure impairs colonic motility by selectively attenuating nitrergic signalling in the mouse.

    PubMed

    Diss, Lucy; Dyball, Sarah; Ghela, Tina; Golding, Jonathan; Morris, Rachel; Robinson, Stephen; Tucker, Rosemary; Walter, Talia; Young, Paul; Allen, Marcus; Fidalgo, Sara; Gard, Paul; Mabley, Jon; Patel, Bhavik; Chatterjee, Prabal; Yeoman, Mark

    2016-02-01

    Paraquat, a common herbicide, is responsible for large numbers of deaths worldwide through both deliberate and accidental ingestion. Previous studies have eluded that the bioavailability of paraquat increases substantially with increasing dose and that these changes may in part be due to the effects that these high concentrations have on the gastrointestinal tract (GI tract). To date, the actions of acute, high concentrations (20mM for 60 min) of paraquat on the GI tract, particularly the colon which is a major site of paraquat absorption, are unknown. This study examined the effects of acute paraquat administration on colonic motility in the C57BL/6 mouse. Acute paraquat exposure decreased colonic motility and the amplitude of colonic migrating motor complexes (CMMCs), which are major motor patterns involved in faecal pellet propulsion. In isolated segments of distal colon, paraquat increased resting tension and markedly attenuated electrical field stimulation-evoked relaxations. Pharmacological dissection of paraquat's mechanism of action on both the CMMCs and field stimulated tissue using the nitric oxide synthase inhibitor NG-nitro-L-arginine and direct measurement of NO release from the myenteric plexus, demonstrated that paraquat selectively attenuates nitrergic signalling pathways. These changes did not appear to be due to alterations in colonic oxidative stress, inflammation or complex 1 activity, but were most likely caused by paraquat's ability to act as a redox couple. In summary, these data demonstrate that acute paraquat exposure attenuates colonic transit. These changes may facilitate the absorption of paraquat into the circulation and so facilitate its toxicity.

  16. Renal impairment and worsening of renal function in acute heart failure: can new therapies help? The potential role of serelaxin.

    PubMed

    Schmieder, Roland E; Mitrovic, Veselin; Hengstenberg, Christian

    2015-08-01

    Renal dysfunction is a frequent finding in patients with acute heart failure (AHF) and an important prognostic factor for adverse outcomes. Worsening of renal function occurs in 30-50% of patients hospitalised for AHF, and is associated with increased mortality, prolonged hospital stay and increased risk of readmission. Likely mechanisms involved in the decrease in renal function include impaired haemodynamics and activation of neurohormonal factors, such as the renin-angiotensin-aldosterone system, the sympathetic nervous system and the arginine-vasopressin system. Additionally, many drugs currently used to treat AHF have a detrimental effect on renal function. Therefore, pharmacotherapy for AHF should carefully take into account any potential complications related to renal function. Serelaxin, currently in clinical development for the treatment of AHF is a recombinant form of human relaxin-2, identical in structure to the naturally occurring human relaxin-2 peptide hormone that mediates cardiac and renal adaptations during pregnancy. Data from both pre-clinical and clinical studies indicate a potentially beneficial effect of serelaxin on kidney function. In this review, we discuss the mechanisms and impact of impairment of renal function in AHF, and the potential benefits of new therapies, such as serelaxin, in this context.

  17. Zinc causes acute impairment of glutathione metabolism followed by coordinated antioxidant defenses amplification in gills of brown mussels Perna perna.

    PubMed

    Trevisan, Rafael; Flesch, Samira; Mattos, Jacó Joaquim; Milani, Márcio Raimundo; Bainy, Afonso Celso Dias; Dafre, Alcir Luiz

    2014-01-01

    Zinc demonstrates protective and antioxidant properties at physiological levels, although these characteristics are not attributed at moderate or high concentrations. Zinc toxicity has been related to a number of factors, including interference with antioxidant defenses. In particular, the inhibition of glutathione reductase (GR) has been suggested as a possible mechanism for acute zinc toxicity in bivalves. The present work investigates the biochemical effects of a non-lethal zinc concentration on antioxidant-related parameters in gills of brown mussels Perna perna exposed for 21 days to 2.6 μM zinc chloride. After 2 days of exposure, zinc caused impairment of the antioxidant system, decreasing GR activity and glutathione levels. An increase in antioxidant defenses became evident at 7 and 21 days of exposure, as an increase in superoxide dismutase and glutathione peroxidase activity along with restoration of glutathione levels and GR activity. After 7 and 21 days, an increase in cellular peroxides and lipid peroxidation end products were also detected, which are indicative of oxidative damage. Changes in GR activity contrasts with protein immunoblotting data, suggesting that zinc produces a long lasting inhibition of GR. Contrary to the general trend in antioxidants, levels of peroxiredoxin 6 decreased after 21 days of exposure. The data presented here support the hypothesis that zinc can impair thiol homeostasis, causes an increase in lipid peroxidation and inhibits GR, imposing a pro-oxidant status, which seems to trigger homeostatic mechanisms leading to a subsequent increase on antioxidant-related defenses.

  18. Clinical and audiologic characteristics of patients with sensorineural tinnitus and its association with psychological aspects: an analytic retrospective study.

    PubMed

    Al-Swiahb, Jamil Nasser; Hwang, Eul Seung; Kong, Ji Sun; Kim, Woo Jin; Yeo, Sang Won; Park, Shi Nae

    2016-12-01

    This study was performed to analyze clinical and audiologic characteristics of sensorineural tinnitus and to investigate the associating factors reflecting psychological aspects of stress and depression of the patients. This is a retrospective analytical study conducted in a tinnitus clinic of a tertiary referral center of a university hospital. The medical records of 216 patients suffering from sensorineural tinnitus were thoroughly evaluated to determine correlations between clinical and audiological characteristics, including age, sex, predisposing or etiologic factors, hearing levels up to extended high frequencies, and tinnitus severity. Psychological aspects of stress and depression were also evaluated and analyzed to seek the associations with tinnitus severity. All data were stored in our database bank and were statistically analyzed. Our study subjects showed a slight male predominance. The highest percentage of tinnitus was found in patients of 60-80 years old. Only 32.5 % of tinnitus patients were subjectively aware of their hearing loss, whereas 73 % of subjects had hearing deficits in some frequencies in their audiogram. Hearing impairments were of the low-frequency sensorineural type in 18.2 % of patients and were limited to the high frequencies in 77.9 % of patients. Tinnitus was unilateral in 51 % of patients and had a tonal nature in 45 % of patients. In total, 45.8 % of patients with high-frequency sensorineural hearing loss had high-pitched tinnitus. There were significant correlations between tinnitus severity, loudness and annoyance. Correlations with THI (Tinnitus Handicap Inventory) and Beck depression index scores were also found. Sensorineural tinnitus was related with hearing loss in some frequencies nevertheless of patients' own awareness of hearing loss. Loudness and annoyance of tinnitus seems to be two important factors reflecting psychological problems of patients' stress and depression.

  19. The prefrontal cortex communicates with the amygdala to impair learning after acute stress in females but not in males.

    PubMed

    Maeng, Lisa Y; Waddell, Jaylyn; Shors, Tracey J

    2010-12-01

    Acute stress exposure enhances classical eyeblink conditioning in male rats, whereas exposure to the same event dramatically impairs performance in females (Wood and Shors, 1998; Wood et al., 2001). We hypothesized that stress affects learning differently in males and females because different brain regions and circuits are being activated. In the first experiment, we determined that neuronal activity within the medial prefrontal cortex (mPFC) during the stressful event is necessary to disrupt learning in females. In both males and females, the mPFC was bilaterally inactivated with GABA agonist muscimol before the stressor. Inactivation prevented only the impaired performance in females; it had no consequence for performance in males. However, in the second experiment, excitation of the mPFC alone with GABA antagonist picrotoxin was insufficient to elicit the stress effect that was prevented through the inactivation of this region in females. Therefore, we hypothesized that the mPFC communicates with the basolateral amygdala to disrupt learning in females after the stressor. To test this hypothesis, these structures were disconnected from each other with unilateral excitotoxic (NMDA) lesions on either the same or opposite sides of the brain. Females with contralateral lesions, which disrupt the connections on both sides of the brain, were able to learn after the stressful event, whereas those with ipsilateral lesions, which disrupt only one connection, did not learn after the stressor. Together, these data indicate that the mPFC is critically involved in females during stress to impair subsequent learning and does so via communication with the amygdala.

  20. Sensorineural hearing loss and celiac disease: A coincidental finding

    PubMed Central

    Volta, Umberto; Ferri, Gian Gaetano; De Giorgio, Roberto; Fabbri, Angela; Parisi, Claudia; Sciajno, Laura; Castellari, Alessandra; Fiorini, Erica; Piscaglia, Maria; Barbara, Giovanni; Granito, Alessandro; Pirodda, Antonio

    2009-01-01

    BACKGROUND Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). OBJECTIVE To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. METHODS A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. RESULTS SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. CONCLUSIONS SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency. PMID:19668795

  1. Impaired mitochondrial respiration and protein nitration in the rat hippocampus after acute inhalation of combustion smoke.

    PubMed

    Lee, Heung M; Reed, Jason; Greeley, George H; Englander, Ella W

    2009-03-01

    Survivors of massive inhalation of combustion smoke endure critical injuries, including lasting neurological complications. We have previously reported that acute inhalation of combustion smoke disrupts the nitric oxide homeostasis in the rat brain. In this study, we extend our findings and report that a 30-minute exposure of awake rats to ambient wood combustion smoke induces protein nitration in the rat hippocampus and that mitochondrial proteins are a sensitive nitration target in this setting. Mitochondria are central to energy metabolism and cellular signaling and are critical to proper cell function. Here, analyses of the mitochondrial proteome showed elevated protein nitration in the course of a 24-hour recovery following exposure to smoke. Mass spectrometry identification of several significantly nitrated mitochondrial proteins revealed diverse functions and involvement in central aspects of mitochondrial physiology. The nitrated proteins include the ubiquitous mitochondrial creatine kinase, F1-ATP synthase alpha subunit, dihydrolipoamide dehydrogenase (E3), succinate dehydrogenase Fp subunit, and voltage-dependent anion channel (VDAC1) protein. Furthermore, acute exposure to combustion smoke significantly compromised the respiratory capacity of hippocampal mitochondria. Importantly, elevated protein nitration and reduced mitochondrial respiration in the hippocampus persisted beyond the time required for restoration of normal oxygen and carboxyhemoglobin blood levels after the cessation of exposure to smoke. Thus, the time frame for intensification of the various smoke-induced effects differs between blood and brain tissues. Taken together, our findings suggest that nitration of essential mitochondrial proteins may contribute to the reduction in mitochondrial respiratory capacity and underlie, in part, the brain pathophysiology after acute inhalation of combustion smoke.

  2. Impaired mitochondrial respiration and protein nitration in the rat hippocampus after acute inhalation of combustion smoke

    SciTech Connect

    Lee, Heung M.; Reed, Jason; Greeley, George H.; Englander, Ella W.

    2009-03-01

    Survivors of massive inhalation of combustion smoke endure critical injuries, including lasting neurological complications. We have previously reported that acute inhalation of combustion smoke disrupts the nitric oxide homeostasis in the rat brain. In this study, we extend our findings and report that a 30-minute exposure of awake rats to ambient wood combustion smoke induces protein nitration in the rat hippocampus and that mitochondrial proteins are a sensitive nitration target in this setting. Mitochondria are central to energy metabolism and cellular signaling and are critical to proper cell function. Here, analyses of the mitochondrial proteome showed elevated protein nitration in the course of a 24-hour recovery following exposure to smoke. Mass spectrometry identification of several significantly nitrated mitochondrial proteins revealed diverse functions and involvement in central aspects of mitochondrial physiology. The nitrated proteins include the ubiquitous mitochondrial creatine kinase, F1-ATP synthase {alpha} subunit, dihydrolipoamide dehydrogenase (E3), succinate dehydrogenase Fp subunit, and voltage-dependent anion channel (VDAC1) protein. Furthermore, acute exposure to combustion smoke significantly compromised the respiratory capacity of hippocampal mitochondria. Importantly, elevated protein nitration and reduced mitochondrial respiration in the hippocampus persisted beyond the time required for restoration of normal oxygen and carboxyhemoglobin blood levels after the cessation of exposure to smoke. Thus, the time frame for intensification of the various smoke-induced effects differs between blood and brain tissues. Taken together, our findings suggest that nitration of essential mitochondrial proteins may contribute to the reduction in mitochondrial respiratory capacity and underlie, in part, the brain pathophysiology after acute inhalation of combustion smoke.

  3. Impairment of T cell development and acute inflammatory response in HIV-1 Tat transgenic mice

    PubMed Central

    Fiume, Giuseppe; Scialdone, Annarita; Albano, Francesco; Rossi, Annalisa; Maria Tuccillo, Franca; Rea, Domenica; Palmieri, Camillo; Caiazzo, Elisabetta; Cicala, Carla; Bellevicine, Claudio; Falcone, Cristina; Vecchio, Eleonora; Pisano, Antonio; Ceglia, Simona; Mimmi, Selena; Iaccino, Enrico; Laurentiis, Annamaria de; Pontoriero, Marilena; Agosti, Valter; Troncone, Giancarlo; Mignogna, Chiara; Palma, Giuseppe; Arra, Claudio; Mallardo, Massimo; Maria Buonaguro, Franco; Scala, Giuseppe; Quinto, Ileana

    2015-01-01

    Immune activation and chronic inflammation are hallmark features of HIV infection causing T-cell depletion and cellular immune dysfunction in AIDS. Here, we addressed the issue whether HIV-1 Tat could affect T cell development and acute inflammatory response by generating a transgenic mouse expressing Tat in lymphoid tissue. Tat-Tg mice showed thymus atrophy and the maturation block from DN4 to DP thymic subpopulations, resulting in CD4+ and CD8+ T cells depletion in peripheral blood. In Tat-positive thymus, we observed the increased p65/NF-κB activity and deregulated expression of cytokines/chemokines and microRNA-181a-1, which are involved in T-lymphopoiesis. Upon LPS intraperitoneal injection, Tat-Tg mice developed an abnormal acute inflammatory response, which was characterized by enhanced lethality and production of inflammatory cytokines. Based on these findings, Tat-Tg mouse could represent an animal model for testing adjunctive therapies of HIV-1-associated inflammation and immune deregulation. PMID:26343909

  4. Acute ethanol ingestion impairs appetitive olfactory learning and odor discrimination in the honey bee

    PubMed Central

    Mustard, Julie A; Wright, Geraldine A; Edgar, Elaina A; Mazade, Reece E.; Wu, Chen; Lillvis, Joshua L

    2008-01-01

    Invertebrates are valuable models for increasing our understanding of the effects of ethanol on the nervous system, but most studies on invertebrates and ethanol have focused on the effects of ethanol on locomotor behavior. In this work we investigate the influence of an acute dose of ethanol on appetitive olfactory learning in the honey bee (Apis mellifera), a model system for learning and memory. Adult worker honey bees were fed a range of doses (2.5, 5, 10 or 25%) of ethanol and then conditioned to associate an odor with a sucrose reward using either a simple or differential conditioning paradigm. Consumption of ethanol before conditioning significantly reduced both the rate of acquisition and the asymptotic strength of the association. Honey bees also exhibited a dose dependent reduction in arousal/attention during conditioning. Consumption of ethanol after conditioning did not affect recall 24 h later. The observed deficits in acquisition were not due to the affect of ethanol on gustatory sensitivity or motor function. However, honey bees given higher doses of ethanol had difficulty discriminating amongst different odors suggesting that ethanol consumption influences olfactory processing. Taken together, these results demonstrate that an acute dose of ethanol affects appetitive learning and olfactory perception in the honey bee. PMID:18723103

  5. Functional MRI for characterization of renal perfusion impairment and edema formation due to acute kidney injury in different mouse strains

    PubMed Central

    Chen, Rongjun; Gutberlet, Marcel; Jang, Mi-Sun; Meier, Martin; Mengel, Michael; Hartung, Dagmar; Wacker, Frank; Rong, Song; Hueper, Katja

    2017-01-01

    Purpose The purpose was to characterize acute kidney injury (AKI) in C57BL/6 (B6)- and 129/Sv (Sv)-mice by noninvasive measurement of renal perfusion and tissue edema using functional MRI. Methods Different severities of AKI were induced in B6- and Sv-mice by renal ischemia reperfusion injury (IRI). Unilateral clamping of the renal pedicle for 35 min (moderate AKI) or 45 min (severe AKI) was done. MRI (7-Tesla) was performed 1, 7 and 28 days after surgery using a flow alternating inversion recovery (FAIR) arterial spin labeling (ASL) sequence. Maps of perfusion and T1-relaxation time were calculated. Relative MRI-parameters of the IRI kidney compared to the contralateral not-clipped kidney were compared between AKI severities and between mouse strains using unpaired t-tests. In addition, fibrosis was assessed by Masson Trichrome and collagen IV staining. Results After moderate AKI relative perfusion impairment was significantly higher in B6- than in Sv-mice at d7 (55±7% vs. 82±8%, p<0.05) and d28 (76±7% vs. 102±3%, p<0.01). T1-values increased in the early phase after AKI in both mouse strains. T1-increase was more severe after prolonged ischemia times of 45 min compared to 35 min in both mouse strains, measured in the renal cortex and outer stripe of outer medulla. Kidney volume loss (compared to the contralateral kidney) occurred already after 7 days but proceeded markedly towards 4 weeks in severe AKI. Early renal perfusion impairment was predictive for later kidney volume loss. The progression to chronic kidney disease (CKD) in the severe AKI model was similar in both mouse strains as revealed by histology. Conclusion Quantification of renal perfusion and tissue edema by functional MRI allows characterization of strain differences upon AKI. Renal perfusion impairment was stronger in B6- compared to Sv-animals following moderate AKI. Prolonged ischemia times were associated with more severe perfusion impairment and edema formation in the early phase and

  6. [Bouveret's syndrome: biliary ileus manifested by acute upper gastrointestinal hemorrhage and impaired gastric emptying].

    PubMed

    Simonek, J; Lischke, R; Drábek, J; Pafko, P

    2002-05-01

    The authors present a very rare case of impaired gastric evacuation, known as Bouveret's syndrome, caused by a large biliary concrement wedged in the duodenum as a result of the development of a cholecystoduodenal fistula in a 77-year-old man. The condition was manifested clinically by developed high ileus and subsequent haemorrhage into the upper GIT. The diagnosis was established on the background of the clinical picture, passage through the upper GIT and endoscopy. As the attempt to remove the concrement endoscopically failed, laparotomy had to be used. In the conclusion of this case-record the authors discuss the method of assessment of the correct diagnosis endoscopically and possibilities of therapeutic strategy.

  7. Exercise capacity is not impaired after acute alcohol ingestion: a pilot study.

    PubMed

    Popovic, Dejana; Damjanovic, Svetozar S; Plecas-Solarovic, Bosiljka; Pešić, Vesna; Stojiljkovic, Stanimir; Banovic, Marko; Ristic, Arsen; Mantegazza, Valentina; Agostoni, Piergiuseppe

    2016-12-01

    The usage of alcohol is widespread, but the effects of acute alcohol ingestion on exercise performance and the stress hormone axis are not fully elucidated.We studied 10 healthy white men, nonhabitual drinkers, by Doppler echocardiography at rest, spirometry, and maximal cardiopulmonary exercise test (CPET) in two visits (2-4 days in between), one after administration of 1.5 g/kg ethanol (whisky) diluted at 15% in water, and the other after administration of an equivalent volume of water. Plasma levels of NT-pro-BNP, cortisol, and adrenocorticotropic hormone (ACTH) were also measured 10 min before the test, at maximal effort and at the third minute of recovery. Ethanol concentration was measured from resting blood samples by gas chromatography and it increased from 0.00 ± 0.00 to 1.25 ± 0.54‰ (P < 0.001). Basal echocardiographic and spirometric parameters were normal and remained so after acute alcohol intake, whereas ACTH, cortisol, and NT-pro-BNP nonsignificantly increased in all phases of the test. CPET data suggested a trend toward a slight reduction of exercise performance (peak VO2 = 3008 ± 638 vs. 2900 ± 543 ml/min, ns; peak workload = 269 ± 53 vs. 249 ± 40 W, ns; test duration 13.7 ± 2.2 vs. 13.3 ± 1.7 min, ns; VE/VCO2 22.1 ± 1.4 vs. 23.3 ± 2.9, ns). Ventilatory equivalent for carbon dioxide at rest was higher after alcohol intake (28 ± 2.5 vs. 30.4 ± 3.2, P = 0.039) and maximal respiratory exchange ratio was lower after alcohol intake (1.17 ± 0.02 vs. 1.14 ± 0.04, P = 0.04). In conclusion, we showed that acute alcohol intake in healthy white men is associated with a nonsignificant exercise performance reduction and stress hormone stimulation, with an unchanged exercise metabolism.

  8. Acute and chronic methyl mercury poisoning impairs rat adrenal and testicular function

    SciTech Connect

    Burton, G.V.; Meikle, A.W.

    1980-05-01

    Animals poisoned with methyl mercury (CH/sub 3/Hg) exhibit stress intolerance and decreased sexual activity, which suggest both adrenal and testicular dysfunction. Adrenal and testicular function was studied in male rats after treatment with CH/sub 3/Hg. In animals treated chronically, the adrenal glands were markedly hyperplastic with enlargement of the zona fasciculata. The mean basal serum levels of corticosterone were similar in experimental (17.8 ..mu..g/dl) and control (16.8 ..mu..g/dl) groups. However, with ether stress, experimental animals had a subnormal response, and the mean serum levels of corticosterone increased to only 23.9 ..mu../dl compared to 40.6 ..mu..g/dl in the controls. Exogenous ACTH stimulation produced a mean level of 19.0 ..mu..g/dl in the CH/sub 3/Hg-treated animals and 49.7 ..mu..g/dl in the controls. In vitro studies demonstrated a defect in the conversion of cholesterol to pregnenolone. A profound impairment in swimming was partially reversed with glucocorticoid therapy. In animals treated with CH/sub 3/Hg, serum testosterone was lower than normal in the basal state. Human chorionic gonadotropin stimulation increased the mean serum concentration of testosterone to 23.4 ng/ml in controls, but it was only 4.50 ng/ml in experimental animals. The data indicate that CH/sub 3/Hg poisoning impairs adrenal and testicular steroid hormone secretion, which accounts in part for the diminished stress tolerance and decreased sexual activity observed in CH/sub 3/Hg-intoxicated animals.

  9. Review of hepatocellular cancer, hypertension and renal impairment as late complications of acute porphyria and recommendations for patient follow-up.

    PubMed

    Stewart, Mary Felicity

    2012-11-01

    This review critically appraises the data emerging from small retrospective and prospective cohort studies suggesting that patients with the autosomal dominant acute porphyrias may be at increased risk of hepatocellular cancer (HCC), hypertension (HT) and renal impairment. The most striking finding is a marked excess risk of HCC in Swedish patients with acute intermittent porphyria (AIP). As Sweden has a relatively high prevalence of AIP due to a founder effect, it is uncertain to what extent the finding is generalisable to other populations or other acute porphyrias and whether early intervention through screening can improve outcomes. As yet there is no evidence for the cost-effectiveness of systematic surveillance for HCC in acute porphyria outside Sweden. Data from several populations also suggest a high prevalence of chronic sustained HT and renal impairment in AIP, but it is uncertain if this represents a true excess risk, in particular for asymptomatic patients. As these long-term complications are important and potentially treatable, a pragmatic recommendation is that symptomatic patients with acute porphyria should be offered specialist long-term follow-up and, for those aged >50 years, annual liver ultrasound may be considered following discussion of the likely risks and benefits. Opportunistic cardiovascular risk assessment can readily be incorporated into a structured annual review so that appropriate drugs safe for use in acute porphyria are prescribed promptly. As these diseases are rare, collaborative international epidemiological studies such as those being coordinated through the European Porphyria Network are essential to inform best clinical practice.

  10. Copper acutely impairs behavioral function and muscle acetylcholinesterase activity in zebrafish (Danio rerio).

    PubMed

    Haverroth, Gabriela M B; Welang, Chariane; Mocelin, Riciéri N; Postay, Daniela; Bertoncello, Kanandra T; Franscescon, Francini; Rosemberg, Denis B; Dal Magro, Jacir; Dalla Corte, Cristiane L

    2015-12-01

    Copper is a heavy metal found at relatively high concentrations in surface waters around the world. Copper is a micronutrient at low concentrations and is essential to several organisms. At higher concentrations copper can become toxic, which reveal the importance of studying the toxic effects of this metal on the aquatic life. Thus, the objective of this study was to evaluate the toxic effects of copper on the behavior and biochemical parameters of zebrafish (Danio rerio). Zebrafish were exposed for 24h at a concentration of 0.006 mg/L Cu. After the exposure period, behavioral profile of animals was recorded through 6 min using two different apparatuses tests: the Novel Tank and the Light-Dark test. After behavioral testing, animals were euthanized with a solution of 250 mg/L of tricaine (MS-222). Brain, muscle, liver and gills were extracted for analysis of parameters related to oxidative stress and accumulation of copper in these tissues. Acetylcholinesterase (AChE) activity was determined in brain and muscle. Results showed acute exposure to copper induces significant changes in behavioral profile of zebrafish by changing locomotion and natural tendency to avoid brightly lit area. On the other hand, there were no significant effects on parameters related to oxidative stress. AChE activity decreased significantly in zebrafish muscle, but there were no significant changes in cerebral AChE activity. Copper levels in tissues did not increase significantly compared to the controls. Taken together, these results indicate that a low concentration of copper can acutely affect behavioral profile of adult zebrafish which could be partially related to an inhibition on muscle AChE activity. These results reinforce the need of additional tests to establishment of safe copper concentrations to aquatic organisms and the importance of behavioral parameters in ecotoxicological studies.

  11. Cholinesterase inhibitors, donepezil and rivastigmine, attenuate spatial memory and cognitive flexibility impairment induced by acute ethanol in the Barnes maze task in rats.

    PubMed

    Gawel, Kinga; Labuz, Krzysztof; Gibula-Bruzda, Ewa; Jenda, Malgorzata; Marszalek-Grabska, Marta; Filarowska, Joanna; Silberring, Jerzy; Kotlinska, Jolanta H

    2016-10-01

    Central cholinergic dysfunction contributes to acute spatial memory deficits produced by ethanol administration. Donepezil and rivastigmine elevate acetylcholine levels in the synaptic cleft through the inhibition of cholinesterases-enzymes involved in acetylcholine degradation. The aim of our study was to reveal whether donepezil (acetylcholinesterase inhibitor) and rivastigmine (also butyrylcholinesterase inhibitor) attenuate spatial memory impairment as induced by acute ethanol administration in the Barnes maze task (primary latency and number of errors in finding the escape box) in rats. Additionally, we compared the influence of these drugs on ethanol-disturbed memory. In the first experiment, the dose of ethanol (1.75 g/kg, i.p.) was selected that impaired spatial memory, but did not induce motor impairment. Next, we studied the influence of donepezil (1 and 3 mg/kg, i.p.), as well as rivastigmine (0.5 and 1 mg/kg, i.p.), given either before the probe trial or the reversal learning on ethanol-induced memory impairment. Our study demonstrated that these drugs, when given before the probe trial, were equally effective in attenuating ethanol-induced impairment in both test situations, whereas rivastigmine, at both doses (0.5 and 1 mg/kg, i.p.), and donepezil only at a higher dose (3 mg/kg, i.p.) given prior the reversal learning, attenuated the ethanol-induced impairment in cognitive flexibility. Thus, rivastigmine appears to exert more beneficial effect than donepezil in reversing ethanol-induced cognitive impairments-probably due to its wider spectrum of activity. In conclusion, the ethanol-induced spatial memory impairment may be attenuated by pharmacological manipulation of central cholinergic neurotransmission.

  12. Acute Thermotherapy Prevents Impairments in Cutaneous Microvascular Function Induced by a High Fat Meal

    PubMed Central

    Harvey, Jennifer C.; Roseguini, Bruno T.; Goerger, Benjamin M.; Fallon, Elizabeth A.

    2016-01-01

    We tested the hypothesis that a high fat meal (HFM) would impair cutaneous vasodilation, while thermotherapy (TT) would reverse the detrimental effects. Eight participants were instrumented with skin heaters and laser-Doppler (LD) probes and tested in three trials: control, HFM, and HFM + TT. Participants wore a water-perfused suit perfused with 33°C (control and HFM) or 50°C (HFM + TT) water. Participants consumed 1 g fat/kg body weight. Blood samples were taken at baseline and two hours post-HFM. Blood pressure was measured every 5–10 minutes. Microvascular function was assessed via skin local heating from 33°C to 39°C two hours after HFM. Cutaneous vascular conductance (CVC) was calculated and normalized to maximal vasodilation (%CVCmax). HFM had no effect on initial peak (48 ± 4 %CVCmax) compared to control (49 ± 4 %CVCmax) but attenuated the plateau (51 ± 4 %CVCmax) compared to control (63 ± 4 %CVCmax, P < 0.001). Initial peak was augmented in HFM + TT (66 ± 4 %CVCmax) compared to control and HFM (P < 0.05), while plateau (73 ± 3 % CVCmax) was augmented only compared to the HFM trial (P < 0.001). These data suggest that HFM negatively affects cutaneous vasodilation but can be minimized by TT. PMID:27595112

  13. Diabetes insipidus as a rare cause of acute cognitive impairment in multiple sclerosis.

    PubMed

    Tiedje, V; Schlamann, M; Führer, D; Moeller, L C

    2013-10-01

    Multiple sclerosis (MS) is a complex neurodegenerative disease presenting with a diversity of clinical symptoms including palsy and cognitive impairment. We present a 59-year-old woman with a history of secondary progressive MS since 1987, who was referred to our department because of recent onset of confusion and polydipsia. Initial lab tests showed mildly elevated serum sodium levels and low urine osmolality. Under water deprivation, diuresis and low urine osmolality persisted and serum sodium levels rose above 150 mmol/l. Oral desmopressin resulted in normalisation of serum sodium as well as urine osmolarity, confirming a diagnosis of central diabetes insipidus. As drug-induced diabetes could be excluded, pituitary magnetic resonance imaging (MRI) was performed. A demyelinating lesion was detected in the hypothalamus. The patient was started on oral desmopressin treatment (0.2 mg/day). Fluid intake and serum sodium levels have since remained normal. In summary, we report the rare case of a patient presenting with diabetes insipidus due to progressive MS. Diabetes insipidus should be considered in MS patients who develop new onset of polydipsia.

  14. Analysis of Phase 3 telavancin nosocomial pneumonia data excluding patients with severe renal impairment and acute renal failure

    PubMed Central

    Torres, A.; Rubinstein, E.; Corey, G. R.; Stryjewski, M. E.; Barriere, S. L.

    2014-01-01

    Objectives Telavancin is approved in Europe for the treatment of nosocomial pneumonia caused by methicillin-resistant Staphylococcus aureus when other alternatives are not suitable. The approved European prescribing information contraindicates the use of telavancin in patients with severe renal impairment (creatinine clearance <30 mL/min, including patients on haemodialysis) and pre-existing acute renal failure owing to the higher observed mortality in these patients. Data from the ATTAIN studies were reanalysed, excluding patients with these contraindicating conditions at baseline. (At the time of submission of this article, the European marketing authorization of telavancin for the treatment of nosocomial pneumonia was suspended pending evidence of a new European Medicines Agency-approved supplier. Clinigen Healthcare Ltd, Theravance's commercialization partner for telavancin in Europe, is in the process of seeking approval of a new manufacturing source.) Methods A post hoc analysis of data from two Phase 3 ATTAIN trials of telavancin for the treatment of Gram-positive nosocomial pneumonia assessing clinical outcomes and safety. Results The all-treated population for this analysis represented 84.2% (1266/1503) of the ATTAIN all-treated population. The cure rates in the clinically evaluable population were similar in the telavancin (82.5%, 231/280) and vancomycin (81.3%, 243/299) groups [treatment difference (95% CI): 1.3% (−5.0% to 7.6%)], and were consistent with the overall ATTAIN study results. The cure rate was higher in the telavancin than the vancomycin treatment group in microbiologically evaluable patients with only Gram-positive pathogens isolated at baseline [85.0% (130/153) versus 75.2% (109/145), respectively; treatment difference (95% CI): 9.7% (0.6%–18.8%)]. The incidences of adverse events were similar between treatment groups and consistent with the overall findings of the ATTAIN study. Conclusions This analysis demonstrated that in the subset

  15. Impaired glucose tolerance in pediatric burn patients at discharge from the acute hospital stay.

    PubMed

    Fram, Ricki Y; Cree, Melanie G; Wolfe, Robert R; Barr, David; Herndon, David N

    2010-01-01

    Hyperglycemia, secondary to the hypermetabolic stress response, is a common occurrence after thermal injury. This stress response has been documented to persist up to 9 months postburn. The purpose of this study was to measure insulin sensitivity in severely burned children before discharge when wounds are 95% healed. Twenty-four children, aged 4 to 17 years, with burns > or = 40% TBSA underwent a 2-hour oral glucose tolerance test before discharge from the acute pediatric burn unit. Plasma glucose and insulin levels as well as the Homeostasis Model Assessment for Insulin Resistance (HOMAIR) were compared with published oral glucose tolerance test data from healthy, nonburned children. There was a significant difference between severely burned children and nonburned, healthy children with respect to the HOMAIR. Severely burned children had a HOMAIR of 3.53 +/- 1.62 compared with the value in nonburned, healthy children of 1.28 +/- 0.16 (P < .05). Insulin resistance secondary to the hypermetabolic stress response persists in severely burned children when burn wounds are at least 95% healed. The results of this study warrant future investigations into therapeutic options for the burned child during the rehabilitative phase of their care after injury.

  16. Impaired glucose tolerance in pediatric burn patients at discharge from the acute hospital stay

    PubMed Central

    Fram, Ricki Y.; Cree, Melanie G.; Wolfe, Robert R.; Barr, David; Herndon, David N.

    2013-01-01

    Objective Hyperglycemia, secondary to the hypermetabolic stress response, is a common occurrence after thermal injury. This stress response has been documented to persist up to 9 months post burn. The purpose of this study was to measure insulin sensitivity in severely burned children prior to discharge when wounds are 95% healed. Methods Twenty-four children, aged 4–17 years, with burns ≥ 40% total body surface area (TBSA) underwent a 2 hour oral glucose tolerance test (OGTT) prior to discharge from the acute pediatric burn unit. Plasma glucose and insulin levels, as well as the Homeostasis Model Assessment for Insulin Resistance (HOMAIR) were compared to published OGTT data from healthy, non-burned children. Results There was a significant difference between severely burned children and non-burned, healthy children with respect to the HOMAIR. Severely burned children had a HOMAIR of 3.53±1.62 compared to the value in non-burned healthy children was 1.28±0.16 (p<0.05). Conclusion Insulin resistance secondary to the hypermetabolic stress response persists in severely burned children when burn wounds are at least 95% healed. The results of this study warrant future investigations into therapeutic options for the burned child during the rehabilitative phase of their care after injury. PMID:20634704

  17. Epidemiological and clinical aspects of ear nose and throat sensorineural emergencies in the Yaoundé reference hospital

    PubMed Central

    Djomou, François; Nkouo, Yves Christian Andjock; Mindja, Eko David; Nchinda, Choffor; Meka, Luc; Mbamyah-Lyonga, Emilia; Ndjolo, Alexis

    2016-01-01

    Introduction Sensorineural emergencies (SNE) are rare clinical situations. Few patients consult early explaining subsequent difficulty in having accurate data and management. Three clinical conditions are considered SNE in otolaryngology; they include sudden sensorineural hearing loss (SSHL), Bell's palsy and acute vertigo. There is very little data available on sensorineural emergencies in our setting. The aim of this study was to provide preliminary data on the management of Ear Nose and Throat (ENT) sensorineural emergency cases in Yaoundé Reference Hospital. Methods A descriptive retrospective study was carried out based on data collected over a period of 5 years, January 2010 to July 2014 at the Yaoundé Reference Hospital. Information was obtained from patients’ files collected from the archives of the institution. Patients presenting with SSHL, Bell's palsy, acute vertigo who consulted during the study period were included in the study. Results A total of 22 patients were included in the study out of 6406 patients who consulted at the ENT Unit. The prevalence of SNE in ENT consultations was 0.003, distributed as follows; 13 patients (59.1%) of SNE had Bell's palsy, seven (31.8%) had vestibular neuritis and two (9.1%) had SSHL. Conclusion The prevalence of SNE was low with idiopathic Bell's palsy being the most frequent. There was a general delay in arrival of patients hence delay in diagnosis. This delay could equally be a factor for treatment failure and poor prognosis. More effort should be made in terms of population sensitization about the necessity of getting early medical attention. PMID:27800104

  18. Molecular and hereditary mechanisms of sensorineural hearing loss with focus on selected endocrinopathies.

    PubMed

    Masindova, I; Varga, L; Stanik, J; Valentinova, L; Profant, M; Klimes, I; Gasperikova, D

    2012-07-01

    Hearing loss is one of the most widespread sensory disorders. The incidence of deafness in general population is 1:1000 newborns. About one half of the cases of the congenital sensorineural hearing loss (SNHL) is inherited. Recessive mutations in the gap junction beta 2 (GJB2) gene are the most common genetic causes of the nonsyndromic SNHL. The GJB2 encodes a protein connexin 26 which forms a subunit of gap junction essential for the correct function of the inner ear. The syndromic SNHL is associated with a wide range of other symptoms, which encompass also dysfunctions of endocrine organs. The Pendred syndrome associated with the hearing impairment is characterized by a prelingual, bilateral sever to profound SNHL, goiter, and iodine organification defect. It is an autosomal recessive disorder, which develops due to mutations in pendrin, an anion channel encoded by SLC26A4 gene. Another important type of syndromic hearing loss is the Maternally Inherited Diabetes and Deafness syndrome, which is caused by several mitochondrial DNA mutations. These mutations are clinically manifested by a hearing impairment with development of the diabetes in the adult age. Hearing impairment occurs during puberty when sensation of high frequency tones is affected following with further progress to profound bilateral sensorineural hearing impairment in the whole frequency range. This review deals with the molecular mechanisms of common genetic causes of the hereditary SNHL along with the selected endocrinopathies emphasizing that the DNA analyses along with the functional studies significantly contribute to the early SNHL diagnosis followed by personalized therapy and genetic counseling.

  19. Frontline Science: ATF3 is responsible for the inhibition of TNF-α release and the impaired migration of acute ethanol-exposed monocytes and macrophages.

    PubMed

    Hu, Chaojie; Meng, Xiaoming; Huang, Cheng; Shen, Chenlin; Li, Jun

    2017-03-01

    Binge drinking represses host innate immunity and leads to a high risk of infection. Acute EtOH-pretreated macrophages exhibit a decreased production of proinflammatory mediators in response to LPS. ATF3 is induced and counter-regulates the LPS/TLR4 inflammatory cascade. Here, we investigated the potential role of ATF3 in LPS tolerance in acute ethanol-pretreated macrophages. We found that there was an inverse correlation between ATF3 and LPS-induced TNF-α production in acute ethanol-pretreated murine monocytes and macrophages. The knockdown of ATF3 attenuated the inhibitory effects of acute ethanol treatment on LPS-induced TNF-α production. Furthermore, ChIP assays and co-IP demonstrated that ATF3, together with HDAC1, negatively modulated the transcription of TNF-α. In binge-drinking mice challenged with LPS, an up-regulation of ATF3 and HDAC1 and a concomitant decrease in TNF-α were observed. Given that HDAC1 was concomitantly induced in acute ethanol-exposed monocytes and macrophages, we used the HDACi TSA or silenced HDAC1 to explore the role of HDAC1 in acute ethanol-treated macrophages. Our results revealed that TSA treatment and HDAC1 knockdown prevented acute ethanol-induced ATF3 expression and the inhibition of TNF-α transcription. These data indicated a dual role for HDAC1 in acute ethanol-induced LPS tolerance. Furthermore, we showed that the induction of ATF3 led to the impaired migration of BM monocytes and macrophages. Overall, we present a novel role for ATF3 in the inhibition of LPS-induced TNF-α and in the impairment of monocyte and macrophage migration.

  20. Senescence marker protein-30/gluconolactonase deletion worsens glucose tolerance through impairment of acute insulin secretion.

    PubMed

    Hasegawa, Goji; Yamasaki, Masahiro; Kadono, Mayuko; Tanaka, Muhei; Asano, Mai; Senmaru, Takafumi; Kondo, Yoshitaka; Fukui, Michiaki; Obayashi, Hiroshi; Maruyama, Naoki; Nakamura, Naoto; Ishigami, Akihito

    2010-02-01

    Senescence marker protein-30 (SMP30) is an androgen-independent factor that decreases with age. We recently identified SMP30 as the lactone-hydrolyzing enzyme gluconolactonase (GNL), which is involved in vitamin C biosynthesis in animal species. To examine whether the age-related decrease in SMP30/GNL has effects on glucose homeostasis, we used SMP30/GNL knockout (KO) mice treated with L-ascorbic acid. In an ip glucose tolerance test at 15 wk of age, blood glucose levels in SMP30/GNL KO mice were significantly increased by 25% at 30 min after glucose administration compared with wild-type (WT) mice. Insulin levels in SMP30/GNL KO mice were significantly decreased by 37% at 30 min after glucose compared with WT mice. Interestingly, an insulin tolerance test showed a greater glucose-lowering effect in SMP30/GNL KO mice. High-fat diet feeding severely worsened glucose tolerance in both WT and SMP30/GNL KO mice. Morphometric analysis revealed no differences in the degree of high-fat diet-induced compensatory increase in beta-cell mass and proliferation. In the static incubation study of islets, insulin secretion in response to 20 mm glucose or KCl was significantly decreased in SMP30/GNL KO mice. On the other hand, islet ATP content at 20 mm in SMP30/GNL KO mice was similar to that in WT mice. Collectively, these data indicate that impairment of the early phase of insulin secretion due to dysfunction of the distal portion of the secretion pathway underlies glucose intolerance in SMP30/GNL KO mice. Decreased SMP30/GNL may contribute to the worsening of glucose tolerance that occurs in normal aging.

  1. Acute fatigue impairs neuromuscular activity of anterior cruciate ligament-agonist muscles in female team handball players.

    PubMed

    Zebis, M K; Bencke, J; Andersen, L L; Alkjaer, T; Suetta, C; Mortensen, P; Kjaer, M; Aagaard, P

    2011-12-01

    In sports, like team handball, fatigue has been associated with an increased risk of anterior cruciate ligament (ACL) injury. While effects of fatigue on muscle function are commonly assessed during maximal isometric voluntary contraction (MVC), such measurements may not relate to the muscle function during match play. The purpose of this study was to investigate the effect of muscle fatigue induced by a simulated handball match on neuromuscular strategy during a functional sidecutting movement, associated with the incidence of ACL injury. Fourteen female team handball players were tested for neuromuscular activity [electromyography (EMG)] during a sidecutting maneuver on a force plate, pre and post a simulated handball match. MVC was obtained during maximal isometric quadriceps and hamstring contraction. The simulated handball match consisted of exercises mimicking handball match activity. Whereas the simulated handball match induced a decrease in MVC strength for both the quadriceps and hamstring muscles (P<0.05), a selective decrease in hamstring neuromuscular activity was seen during sidecutting (P<0.05). This study shows impaired ACL-agonist muscle (i.e. hamstring) activity during sidecutting in response to acute fatigue induced by handball match play. Thus, screening procedures should involve functional movements to reveal specific fatigue-induced deficits in ACL-agonist muscle activation during high-risk phases of match play.

  2. 5-Fluorouracil catabolism to 5-fluoro-5,6-dihydrouracil is reduced by acute liver impairment in mice

    SciTech Connect

    Innocenti, Federico; Danesi, Romano; Bocci, Guido; Natale, Gianfranco . E-mail: gianfranco.natale@anist.med.unipi.it; Del Tacca, Mario

    2005-03-01

    This study investigated the effect of acute liver damage on the inactivation of 5-fluorouracil (5-FU) to its main catabolite 5-fluoro-5,6-dihydrouracil (5-FUH{sub 2}) in mice. Plasma pharmacokinetics of 5-FU and 5-FUH{sub 2} in mice receiving 5-FU (10, 30, and 90 mg/kg) were compared to those in mice pretreated with carbon tetrachloride and receiving the same 5-FU doses. Carbon tetrachloride-induced hepatic damage was histopathologically examined under light microscopy and serum transaminases and dihydropyrimidine dehydrogenase activities were also measured. Liver histopathology and elevated aminotransferase activity levels confirmed the presence of liver damage. 5-FU C{sub max} and AUC both increased up to 71% in mice with liver damage. This was reflected by decreased 5-FUH{sub 2} production, since 5-FUH{sub 2} C{sub max} and AUC levels decreased up to 47% and 61%, respectively. Metabolic ratios between 5-FUH{sub 2} and 5-FU AUCs were considerably decreased as well, further suggesting that liver damage caused a reduction in 5-FU catabolism. DPD activity was not altered in damaged livers. The present results indicate that 5-FU disposition in mice could be profoundly altered in the presence of severe liver impairment, potentially leading to enhanced anabolic activation of 5-FU. This effect seems to be ascribed to a reduction of viable hepatocytes, rather than to an inactivation of DPD activity.

  3. Extrapancreatic organ impairment during acute pancreatitis induced by bile-pancreatic duct obstruction. Effect of N-acetylcysteine

    PubMed Central

    Manso, Manuel A; Ramudo, Laura; De Dios, Isabel

    2007-01-01

    Summary Multiple organ failure is frequently associated with acute pancreatitis (AP). Our aim was to study pulmonary, hepatic and renal complications developed in the course of AP experimentally induced in rats by bile-pancreatic duct obstruction (BPDO), differentiating the complications caused by AP itself, from those directly caused by bile duct obstruction (BDO), after ligating the choledocus. N-acetylcysteine (NAC) was administered as a therapeutic approach. Myeloperoxidase activity revealed neutrophil infiltration in lungs from 12 h after BDO, even if AP was not triggered. Lactate dehydrogenase (LDH) activity indicated hepatocyte death from 48 h after BDO, and from 24 h following BPDO-induced AP onwards, an effect delayed until 48 h by NAC treatment. Rats with single cholestasis (BDO) and rats with BPDO-induced AP showed a significant increase in plasma aspartate aminotransferase (AST), alanine aminotransferase (ALT) and bilirubin concentration from 12 h onwards, whose values were reduced by NAC treatment at early BPDO. No renal failure was found during 120 h of bile-pancreatic obstruction. Our results showed lung and liver impairment as a result of BDO, even if AP does not develop. Pancreatic damage and extrapancreatic complications during AP induced by BPDO were palliated by NAC treatment. PMID:17877536

  4. Acute and chronic wound fluids inversely influence adipose-derived stem cell function: molecular insights into impaired wound healing.

    PubMed

    Koenen, Paola; Spanholtz, Timo A; Maegele, Marc; Stürmer, Ewa; Brockamp, Thomas; Neugebauer, Edmund; Thamm, Oliver C

    2015-02-01

    Wound healing is a complex biological process that requires a well-orchestrated interaction of mediators as well as resident and infiltrating cells. In this context, mesenchymal stem cells play a crucial role as they are attracted to the wound site and influence tissue regeneration by various mechanisms. In chronic wounds, these processes are disturbed. In a comparative approach, adipose-derived stem cells (ASC) were treated with acute and chronic wound fluids (AWF and CWF, respectively). Proliferation and migration were investigated using 3-(4,5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) test and transwell migration assay. Gene expression changes were analysed using quantitative real time-polymerase chain reaction. AWF had a significantly stronger chemotactic impact on ASC than CWF (77·5% versus 59·8% migrated cells). While proliferation was stimulated by AWF up to 136·3%, CWF had a negative effect on proliferation over time (80·3%). Expression of b-FGF, vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 was strongly induced by CWF compared with a mild induction by AWF. These results give an insight into impaired ASC function in chronic wounds. The detected effect of CWF on proliferation and migration of ASC might be one reason for an insufficient healing process in chronic wounds.

  5. Acute tubulointerstitial nephritis with severe renal impairment associated with multisystem IgG4-related disease.

    PubMed

    Beltrame, Rafael Coimbra Ferreira; Friderichs, Maurício; Fior, Bárbara Rayanne; Schaefer, Pedro Guilherme; Thomé, Gustavo Gomes; Silva, Dirceu Reis da; Barros, Elvino José Guardão; Seligman, Renato; Veronese, Francisco Veríssimo

    2016-01-01

    The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%. The patient was treated with prednisone at a dose of 60 mg/day, followed by mycophenolate mofetil, and showed clinical and renal function improvement at 6 months of follow-up. The high index of suspicion of IgG4-related disease with multisystem involvement and the early treatment of this condition are essential to improve the prognosis of affected patients. Resumo A doença relacionada à IgG4 tem um espectro clínico amplo em que múltiplos órgãos podem ser afetados, e o diagnóstico depende de achados histopatológicos típicos e elevada expressão de IgG4 em plasmócitos no tecido afetado. Descrevemos o quadro clínico e a evolução de um paciente com nefrite túbulo-intersticial aguda, insuficiência renal grave e manifestações sistêmicas como linfoadenomegalias e pancreatite crônica. O diagnóstico foi confirmado pelas características clínicas e pela histopatologia renal e de linfonodo, na qual a imunohistoquímica mostrou tecido linfoide com policlonalidade e expressão aumentada de IgG4, com uma relação IgG4/IgG total > 80%. O paciente foi tratado com prednisona na dose de 60 mg/dia, seguido de micofenolato mofetil, e apresentou melhora clínica e da função renal depois de 6 meses de tratamento. O alto índice de suspeição da doença relacionada ao IgG4 com comprometimento multissist

  6. Sensorineural hearing loss in Kawasaki disease.

    PubMed

    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  7. [Auto-immune sensorineural deafness: physiopathology and therapeutic approach].

    PubMed

    Hervier, B; Bordure, P; Masseau, A; Calais, C; Agard, C; Hamidou, M

    2010-03-01

    Sensorineural hearing loss may be due to an autoimmune mechanism. The mechanisms that could induce autoimmune inner ear damage are now better understood, but are not exclusive. Moreover, there is no specific immunologic test available for the diagnosis of autoimmune sensorineural hearing loss, which could also complicate the disease course of other autoimmune systemic diseases. Thus, the incidence of sensorineural autoimmune hearing loss is probably underestimated. The aim of this study was to review the experimental immunologic data in favour of an autoimmune mechanism in this subgroup of sensorineural hearing loss: humoral specific response against inner ear (autoantibodies against a transmembrane transporter) and also cellular response (against cochlin: one of the major proteins expressed in the inner ear). The aim of this review was also to focus on clinical and epidemiological human data that provide evidence for an autoimmune etiopathogeny of some sensorineural hearing loss. Therapeutic options such as immunosuppressive treatments (oral corticosteroids and other immunosuppressive drugs, such as methotrexate and anti-TNFalpha) are also discussed.

  8. Sensori-neural hearing loss in patients treated with irradiation for nasopharyngeal carcinoma

    SciTech Connect

    Grau, C.; Moller, K.; Overgaard, M.; Overgaard, J.; Elbrond, O. )

    1991-08-01

    The present investigation has been carried out to evaluate the sensitivity of the inner ear to irradiation. Cochlear function was tested in a cohort of 22 patients before and 7-84 months after receiving external irradiation for nasopharyngeal carcinoma. The pre-irradiation sensori-neural hearing threshold at 500, 1000, 2000, and 4000 Hz was used as a baseline for the individual patient, and the observed sensori-neural hearing loss (SNHL) was calculated as the difference between pre- and post-irradiation values. The pre-irradiation hearing level or patient age was not correlated with the actual SNHL. In contrast, there was a significant correlation between the total radiation dose to the inner ear and the observed hearing impairment. SNHL was most pronounced in the high frequencies, with values up to 35 dB (4000 Hz) and 25 dB (2000 Hz) in some patients. The latent period for the complication appeared to be 12 months or more. The deleterious effect of irradiation on the hearing should be kept in mind both in treatment planning and in the follow-up after radiotherapy.

  9. Systemic lipopolysaccharide administration impairs retrieval of context-object discrimination, but not spatial, memory: Evidence for selective disruption of specific hippocampus-dependent memory functions during acute neuroinflammation.

    PubMed

    Czerniawski, Jennifer; Miyashita, Teiko; Lewandowski, Gail; Guzowski, John F

    2015-02-01

    Neuroinflammation is implicated in impairments in neuronal function and cognition that arise with aging, trauma, and/or disease. Therefore, understanding the underlying basis of the effect of immune system activation on neural function could lead to therapies for treating cognitive decline. Although neuroinflammation is widely thought to preferentially impair hippocampus-dependent memory, data on the effects of cytokines on cognition are mixed. One possible explanation for these inconsistent results is that cytokines may disrupt specific neural processes underlying some forms of memory but not others. In an earlier study, we tested the effect of systemic administration of bacterial lipopolysaccharide (LPS) on retrieval of hippocampus-dependent context memory and neural circuit function in CA3 and CA1 (Czerniawski and Guzowski, 2014). Paralleling impairment in context discrimination memory, we observed changes in neural circuit function consistent with disrupted pattern separation function. In the current study we tested the hypothesis that acute neuroinflammation selectively disrupts memory retrieval in tasks requiring hippocampal pattern separation processes. Male Sprague-Dawley rats given LPS systemically prior to testing exhibited intact performance in tasks that do not require hippocampal pattern separation processes: novel object recognition and spatial memory in the water maze. By contrast, memory retrieval in a task thought to require hippocampal pattern separation, context-object discrimination, was strongly impaired in LPS-treated rats in the absence of any gross effects on exploratory activity or motivation. These data show that LPS administration does not impair memory retrieval in all hippocampus-dependent tasks, and support the hypothesis that acute neuroinflammation impairs context discrimination memory via disruption of pattern separation processes in hippocampus.

  10. Sudden sensorineural hearing loss in a multiple sclerosis case.

    PubMed

    Tekin, Muhammet; Acar, Gul Ozbilen; Cam, Osman Halit; Hanege, Fatih Mehmet

    2014-01-01

    Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. MS involves different regions of the central nervous system in different periods, and causes demyelination. MS is a neuromotor disorder which progresses with remissions and relapses. Symptoms of MS may regress completely or heal after the relapses leaving sequelae. Sudden sensorinerural hearing loss (SSHL) is hearing loss of 30 dB or more over at least three contiguous audiometric frequencies that develops over a period of a few hours to 3 days. In 4-10 % of the MS patients, sensorineural hearing loss occurs between relapses or remissions. In this case, audiotory brainstem response (ABR) test is the most appropriate test for the diagnosis of sensorineural hearing loss in MS patients. In this article, we will discuss a patient diagnosed as MS who presented with sudden sensorineural hearing loss during the remission of the disease.

  11. The PDE4 inhibitor rolipram reverses object memory impairment induced by acute tryptophan depletion in the rat

    PubMed Central

    Lieben, C.; Smits, L.; Blokland, A.

    2007-01-01

    Rationale The selective type IV phosphodiesterase inhibitor, rolipram, has been shown to improve long-term memory and can reverse the cholinergic deficit caused by scopolamine. However, the underlying mechanisms of action of rolipram remain obscure. Objectives The present study investigates the effect of rolipram in a serotonergic-deficit model of acute tryptophan depletion (ATD). In addition, the levels of plasma tryptophan (TRP) were compared to object recognition performance. Materials and methods The experiments were conducted using male Wistar rats. The time-dependent effect of ATD treatment (a gelatin-based protein mixture) on plasma TRP levels (0, 1, 3, and 6 h after injection) and object recognition task (ORT) performance (0.5, 1, 3, and 6 h after ATD treatment) was examined. The effect of rolipram (0, 0.01, 0.03, and 0.1 mg/kg, i.p.) was tested in the condition in which ATD induced a clear memory deficit. Results ATD significantly lowered the plasma TRP ratio (TRP/Σlarge neutral amino acid) with a maximum of 48%, approximately 1 h after administration. Furthermore, ATD impairs ORT performance when administered 3 h before testing. Rolipram (0.1 mg/kg) reversed the memory deficit induced by ATD in a dose-dependent manner. Conclusions On the basis of previous studies and the ability to reverse a serotonergic deficit, we suggest that rolipram may act through elevation of cyclic adenosine monophosphate levels and subsequent increase in neurotransmitter release. PMID:17265077

  12. Deep Sequencing Reveals Novel Genetic Variants in Children with Acute Liver Failure and Tissue Evidence of Impaired Energy Metabolism

    PubMed Central

    Valencia, C. Alexander; Wang, Xinjian; Wang, Jin; Peters, Anna; Simmons, Julia R.; Moran, Molly C.; Mathur, Abhinav; Husami, Ammar; Qian, Yaping; Sheridan, Rachel; Bove, Kevin E.; Witte, David; Huang, Taosheng; Miethke, Alexander G.

    2016-01-01

    Background & Aims The etiology of acute liver failure (ALF) remains elusive in almost half of affected children. We hypothesized that inherited mitochondrial and fatty acid oxidation disorders were occult etiological factors in patients with idiopathic ALF and impaired energy metabolism. Methods Twelve patients with elevated blood molar lactate/pyruvate ratio and indeterminate etiology were selected from a retrospective cohort of 74 subjects with ALF because their fixed and frozen liver samples were available for histological, ultrastructural, molecular and biochemical analysis. Results A customized next-generation sequencing panel for 26 genes associated with mitochondrial and fatty acid oxidation defects revealed mutations and sequence variants in five subjects. Variants involved the genes ACAD9, POLG, POLG2, DGUOK, and RRM2B; the latter not previously reported in subjects with ALF. The explanted livers of the patients with heterozygous, truncating insertion mutations in RRM2B showed patchy micro- and macrovesicular steatosis, decreased mitochondrial DNA (mtDNA) content <30% of controls, and reduced respiratory chain complex activity; both patients had good post-transplant outcome. One infant with severe lactic acidosis was found to carry two heterozygous variants in ACAD9, which was associated with isolated complex I deficiency and diffuse hypergranular hepatocytes. The two subjects with heterozygous variants of unknown clinical significance in POLG and DGUOK developed ALF following drug exposure. Their hepatocytes displayed abnormal mitochondria by electron microscopy. Conclusion Targeted next generation sequencing and correlation with histological, ultrastructural and functional studies on liver tissue in children with elevated lactate/pyruvate ratio expand the spectrum of genes associated with pediatric ALF. PMID:27483465

  13. Concussive Brain Trauma in the Mouse Results in Acute Cognitive Deficits and Sustained Impairment of Axonal Function

    PubMed Central

    Creed, Jennifer A.; DiLeonardi, Ann Mae; Fox, Douglas P.; Tessler, Alan R.

    2011-01-01

    Abstract Concussive brain injury (CBI) accounts for approximately 75% of all brain-injured people in the United States each year and is particularly prevalent in contact sports. Concussion is the mildest form of diffuse traumatic brain injury (TBI) and results in transient cognitive dysfunction, the neuropathologic basis for which is traumatic axonal injury (TAI). To evaluate the structural and functional changes associated with concussion-induced cognitive deficits, adult mice were subjected to an impact on the intact skull over the midline suture that resulted in a brief apneic period and loss of the righting reflex. Closed head injury also resulted in an increase in the wet weight:dry weight ratio in the cortex suggestive of edema in the first 24 h, and the appearance of Fluoro-Jade-B-labeled degenerating neurons in the cortex and dentate gyrus of the hippocampus within the first 3 days post-injury. Compared to sham-injured mice, brain-injured mice exhibited significant deficits in spatial acquisition and working memory as measured using the Morris water maze over the first 3 days (p<0.001), but not after the fourth day post-injury. At 1 and 3 days post-injury, intra-axonal accumulation of amyloid precursor protein in the corpus callosum and cingulum was accompanied by neurofilament dephosphorylation, impaired transport of Fluoro-Gold and synaptophysin, and deficits in axonal conductance. Importantly, deficits in retrograde transport and in action potential of myelinated axons continued to be observed until 14 days post-injury, at which time axonal degeneration was apparent. These data suggest that despite recovery from acute cognitive deficits, concussive brain trauma leads to axonal degeneration and a sustained perturbation of axonal function. PMID:21299360

  14. Genetic Effects on Sensorineural Hearing Loss and Evidence-based Treatment for Sensorineural Hearing Loss.

    PubMed

    Yu, Yong-qiang; Yang, Huai-an; Xiao, Ming; Wang, Jing-wei; Huang, Dong-yan; Bhambhani, Yagesh; Sonnenberg, Lyn; Clark, Brenda; Jin, Yuan-zhe; Fu, Wei-neng; Zhang, Jie; Yu, Qian; Liang, Xue-ting; Zhang, Ming

    2015-09-01

    In this article, the mechanism of inheritance behind inherited hearing loss and genetic susceptibility in noise-induced hearing loss are reviewed. Conventional treatments for sensorineural hearing loss (SNHL), i.e. hearing aid and cochlear implant, are effective for some cases, but not without limitations. For example, they provide little benefit for patients of profound SNHL or neural hearing loss, especially when the hearing loss is in poor dynamic range and with low frequency resolution. We emphasize the most recent evidence-based treatment in this field, which includes gene therapy and allotransplantation of stem cells. Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss. Although some treatments are still at the experimental stage, it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.

  15. The relationship between hearing aid frequency response and acceptable noise level in patients with sensorineural hearing loss

    PubMed Central

    Jalilvand, Hamid; Pourbakht, Akram; Jalaee, Shohreh

    2015-01-01

    Background: When fitting hearing aid as a compensatory device for an impaired cochlea in a patient with sensorineural hearing loss (HL), it is needed to the effective and efficient frequency response would be selected regarding providing the patient's perfect speech perception. There is not any research about the effects of frequency modifications on speech perception in patients with HL regarding the cochlear desensitization. The effect (s) of modifications in frequency response of hearing aid amplification on the results of acceptable noise level (ANL) test is the main aim of this study. Materials and Methods: The amounts of ANL in two conditions of linear amplification (high frequency emphasis [HFE] and mid frequency emphasis [MFE]) were measured. Thirty-two male subjects who participated in this study had the moderate to severe sensorineural HL. Results: There was not any significant difference between ANL in linear amplification of hearing aid with HFE frequency response and ANL in linear amplification of hearing aid with MFE frequency response. Conclusion: The gain modification of frequency response not only does not affect the patient's performance of speech intelligibility in ANL test. This indicates that we need to note to the cochlear desensitization phenomenon when fitting hearing aid as a compensatory device for an impaired cochlea in a patient. The cochlear desensitization has not been considered properly in hearing aid fitting formula which is needed to be explored more about the bio-mechanisms of impaired cochlea. PMID:26918238

  16. Long-term follow-up in distal renal tubular acidosis with sensorineural deafness.

    PubMed

    Peces, R

    2000-11-01

    A 20-year-old man presented with failure to thrive and bilateral genu valgum. On the basis of growth failure, skeletal deformity, hyperchloremic metabolic acidosis with alkaline urine and hypokalemia, nephrocalcinosis, and hearing loss, a diagnosis of distal renal tubular acidosis (DRTA) with sensorineural deafness was made. The genu valgum was treated by corrective osteotomy. Skeletal deformity was corrected and impaired growth improved after sustained therapy of metabolic acidosis with alkali supplementation. During an 8-year follow-up period the patient's glomerular filtration rate remained stable, the nephrocalcinosis did not progress, and his height increased 10 cm. Although nephrolithiasis led to atrophy of the right kidney, at last follow-up, when the patient was 44 years old, his creatinine clearance was 50 ml/min per 1.73 m2 body surface.

  17. Prognostic Factors in Sudden Sensorineural Hearing Loss

    PubMed Central

    Atay, Gamze; Kayahan, Bahar; çınar, Betül çiçek; Saraç, Sarp; Sennaroğlu, Levent

    2016-01-01

    Background: Sudden sensorineural hearing loss (SSNHL) is still a complex and challenging process which requires clinical evidence regarding its etiology, treatment and prognostic factors. Therefore, determination of prognostic factors might aid in the selection of proper treatment modality. Aims: The aim of this study is to analyze whether there is correlation between SSNHL outcomes and (1) systemic steroid therapy, (2) time gap between onset of symptoms and initiation of therapy and (3) audiological pattern of hearing loss. Study Design: Retrospective chart review. Methods: Patients diagnosed at our clinic with SSNHL between May 2005 and December 2011 were reviewed. A detailed history of demographic features, side of hearing loss, previous SSNHL and/or ear surgery, recent upper respiratory tract infection, season of admission, duration of symptoms before admission and the presence of co-morbid diseases was obtained. Radiological and audiological evaluations were recorded and treatment protocol was assessed to determine whether systemic steroids were administered or not. Treatment started ≤5 days was regarded as “early” and >5 days as “delayed”. Initial audiological configurations were grouped as “upward sloping”, “downward sloping”, “flat” and “profound” hearing loss. Significant recovery was defined as thresholds improved to the same level with the unaffected ear or improved ≥30 dB on average. Slight recovery was hearing improvement between 10–30dB on average. Hearing recovery less than 10 dB was accepted as unchanged. Results: Among the 181 patients who met the inclusion criteria, systemic steroid was administered to 122 patients (67.4%), whereas 59 (32.6%) patients did not have steroids. It was found that steroid administration did not have any statistically significant effect in either recovered or unchanged hearing groups. Early treatment was achieved in 105 patients (58%) and 76 patients (42%) had delayed treatment. Recovery

  18. Persistent Positional Vertigo in a Patient with Sudden Sensorineural Hearing Loss: A Case Report

    PubMed Central

    Kim, Yong Won; Shin, Jung Eun; Lee, Yong-Sik

    2015-01-01

    Because inner ear organs are interconnected through the endolymph and surrounding endolymphatic membrane, the patients with sudden sensorineural hearing loss (SSNHL) often complain of vertigo. In this study, we report a patient with SSNHL accompanied by persistent positional vertigo, and serial findings of head-roll tests are described. At acute stage, head-roll test showed persistent geotropic direction-changing positional nystagmus (DCPN), which led to a diagnosis of SSNHL and ipsilateral light cupula. Although vertigo symptom gradually improved, positional vertigo lasted for more than 3 weeks. At this chronic stage, persistent apogeotropic DCPN was observed in a head roll test, which led to a diagnosis of the heavy cupula. Although the mechanism for the conversion of nystagmus direction from geotropic to apogeotropic persistent DCPN is unclear, the change of specific gravity of the endolymph might be one of the plausible hypothetical explanations. PMID:26413578

  19. NAP prevents acute cerebral oxidative stress and protects against long-term brain injury and cognitive impairment in a model of neonatal hypoxia-ischemia.

    PubMed

    Greggio, Samuel; de Paula, Simone; de Oliveira, Iuri M; Trindade, Cristiano; Rosa, Renato M; Henriques, João A P; DaCosta, Jaderson C

    2011-10-01

    Hypoxia-ischemia (HI) is a common cause of neonatal brain damage with lifelong morbidities in which current therapies are limited. In this study, we investigated the effect of neuropeptide NAP (NAPVSIPQ) on early cerebral oxidative stress, long-term neurological function and brain injury after neonatal HI. Seven-day-old rat pups were subjected to an HI model by applying a unilateral carotid artery occlusion and systemic hypoxia. The animals were randomly assigned to groups receiving an intraperitoneal injection of NAP (3 μg/g) or vehicle immediately (0 h) and 24 h after HI. Brain DNA damage, lipid peroxidation and reduced glutathione (GSH) content were determined 24 h after the last NAP injection. Cognitive impairment was assessed on postnatal day 60 using the spatial version of the Morris water maze learning task. Next, the animals were euthanized to assess the cerebral hemispheric volume using the Cavalieri principle associated with the counting point method. We observed that NAP prevented the acute HI-induced DNA and lipid membrane damage and also recovered the GSH levels in the injured hemisphere of the HI rat pups. Further, NAP was able to prevent impairments in learning and long-term spatial memory and to significantly reduce brain damage up to 7 weeks following the neonatal HI injury. Our findings demonstrate that NAP confers potent neuroprotection from acute brain oxidative stress, long-term cognitive impairment and brain lesions induced by neonatal HI through, at least in part, the modulation of the glutathione-mediated antioxidant system.

  20. Screening for cognitive impairment with the Montreal Cognitive Assessment in Chinese patients with acute mild stroke and transient ischaemic attack: a validation study

    PubMed Central

    Zuo, Lijun; Dong, Yanhong; Zhu, Rongyan; Jin, Zhao; Li, Zixiao; Wang, Yilong; Zhao, Xingquan; Sachdev, Perminder; Zhang, Wei; Wang, Yongjun

    2016-01-01

    Objective We aimed to establish the cut-off point of the Montreal Cognitive Assessment (MoCA-Beijing) in screening for cognitive impairment (CI) within 2 weeks of mild stroke or transient ischaemic attack (TIA). Methods A total of 80 acute mild ischaemic stroke patients and 22 TIA patients were recruited. They received the MoCA-Beijing and a formal neuropsychological test battery. CI was defined by 1.5 SD below the established norms on a formal neuropsychological test battery. Results Most stroke and TIA patients were in their 50s (53.95±11.43 years old), with greater than primary school level of education. The optimal cut-off point for MoCA-Beijing in discriminating patients with CI from those with no cognitive impairment (NCI) was 22/23 (sensitivity 85%, specificity 88%, positive predictive value=91%, negative predictive value=80%, classification accuracy=86%). The predominant cognitive deficits were characteristic of frontal-subcortical impairment, such as visuomotor speed (46.08%), attention/executive function (42.16%) and visuospatial ability (40.20%). Conclusions A MoCA-Beijing cut-off score of 22/23 is optimally sensitive and specific for detecting CI after mild stroke, and TIA in the acute stroke phase, and is recommended for routine clinical practice. PMID:27406642

  1. Prognostic factors of profound idiopathic sudden sensorineural hearing loss.

    PubMed

    Wen, Yu-Hsuan; Chen, Peir-Rong; Wu, Hung-Pin

    2014-06-01

    Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1%) and tinnitus (77.4%). At the 2-month follow-up, 172 (29.8%) patients showed some degree of hearing recovery, but only 21 (3.6%) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.

  2. Acute genistein treatment mimics the effects of estradiol by enhancing place learning and impairing response learning in young adult female rats.

    PubMed

    Pisani, Samantha L; Neese, Steven L; Doerge, Daniel R; Helferich, William G; Schantz, Susan L; Korol, Donna L

    2012-09-01

    Endogenous estrogens have bidirectional effects on learning and memory, enhancing or impairing cognition depending on many variables, including the task and the memory systems that are engaged. Moderate increases in estradiol enhance hippocampus-sensitive place learning, yet impair response learning that taps dorsal striatal function. This memory modulation likely occurs via activation of estrogen receptors, resulting in altered neural function. Supplements containing estrogenic compounds from plants are widely consumed despite limited information about their effects on brain function, including learning and memory. Phytoestrogens can enter the brain and signal through estrogen receptors to affect cognition. Enhancements in spatial memory and impairments in executive function have been found following treatment with soy phytoestrogens, but no tests of actions on striatum-sensitive tasks have been made to date. The present study compared the effects of acute exposure to the isoflavone genistein with the effects of estradiol on performance in place and response learning tasks. Long-Evans rats were ovariectomized, treated with 17β-estradiol benzoate, genistein-containing sucrose pellets, or vehicle (oil or plain sucrose pellets) for 2 days prior to behavioral training. Compared to vehicle controls, estradiol treatment enhanced place learning at a low (4.5 μg/kg) but not high dose (45 μg/kg), indicating an inverted pattern of spatial memory facilitation. Treatment with 4.4 mg of genistein over 2 days also significantly enhanced place learning over vehicle controls. For the response task, treatment with estradiol impaired learning at both low and high doses; likewise, genistein treatment impaired response learning compared to rats receiving vehicle. Overall, genistein was found to mimic estradiol-induced shifts in place and response learning, facilitating hippocampus-sensitive learning and slowing striatum-sensitive learning. These results suggest signaling through

  3. Impaired Fasting Glucose and Recurrent Cardiovascular Disease among Survivors of a First Acute Myocardial Infarction: Evidence of a Sex Difference ? The Western New York Experience

    PubMed Central

    Donahue, Richard P; Dorn, Joan M; Stranges, Saverio; Swanson, Mya; Hovey, Kathleen; Trevisan, Maurizio

    2009-01-01

    Background and aims There is little epidemiological evidence regarding the association of impaired glucose metabolism with recurrent cardiovascular events. We therefore examined potential sex differences in the effect of impaired fasting glucose (IFG) on recurrent cardiovascular disease (CVD) in a community-based study of survivors of a first acute myocardial infarction (MI). Methods and results This report focuses on 1,226 incident MI cases (28.4% women) discharged alive from area hospitals in the Western New York Acute MI Study (1996–2004). Deaths and underlying cause of death were determined via query of the National Death Index (Plus) Retrieval Program with follow-up through December 31, 2004. Outcomes reported included fatal or nonfatal coronary heart disease (CHD) or coronary revascularization surgery and total stroke. Traditional CHD risk factors and other explanatory variables were determined by clinical examination after the first acute event. Impaired fasting glucose was defined as fasting blood glucose between 100 and 125 mg/dl. During a mean follow-up of 4.5 years, there were 91 recurrent events (26.1%) in women and 173 recurrent events (19.7%) in men. After multivariable adjustment, the hazard ratios for recurrent cardiovascular events were 1.96 (95% CI: 1.15–3.16) and 2.59 (1.56–4.30) in women with IFG and with diabetes, respectively, compared to normoglycemic women. Among men, neither IFG nor diabetes was independently related to risk of recurrence. Conclusions In this study, IFG was a strong risk factor for recurrent cardiovascular events only among women. These results suggest that increased cardiovascular risk in MI survivors begins at lower glucose levels in women than men. PMID:20227262

  4. Acute and chronic interference with BDNF/TrkB-signaling impair LTP selectively at mossy fiber synapses in the CA3 region of mouse hippocampus.

    PubMed

    Schildt, Sandra; Endres, Thomas; Lessmann, Volkmar; Edelmann, Elke

    2013-08-01

    Brain-derived neurotrophic factor (BDNF) signaling via TrkB crucially regulates synaptic plasticity in the brain. Although BDNF is abundant at hippocampal mossy fiber (MF) synapses, which critically contribute to hippocampus dependent memory, its role in MF synaptic plasticity (long-term potentiation, LTP) remained largely unclear. Using field potential recordings in CA3 of adult heterozygous BDNF knockout (ko, BDNF+/-) mice we observed impaired (∼50%) NMDAR-independent MF-LTP. In contrast to MF synapses, LTP at neighboring associative/commissural (A/C) fiber synapses remained unaffected. To exclude that impaired MF-LTP in BDNF+/- mice was due to developmental changes in response to chronically reduced BDNF levels, and to prove the importance of acute availability of BDNF in MF-LTP, we also tested effects of acute interference with BDNF/TrkB signaling. Inhibition of TrkB tyrosine kinase signaling with k252a, or with the selective BDNF scavenger TrkB-Fc, both inhibited MF-LTP to the same extent as observed in BDNF+/- mice. Basal synaptic transmission, short-term plasticity, and synaptic fatigue during LTP induction were not significantly altered by treatment with k252a or TrkB-Fc, or by chronic BDNF reduction in BDNF+/- mice. Since the acute interference with BDNF-signaling did not completely block MF-LTP, our results provide evidence that an additional mechanism besides BDNF induced TrkB signaling contributes to this type of LTP. Our results prove for the first time a mechanistic action of acute BDNF/TrkB signaling in presynaptic expression of MF-LTP in adult hippocampus.

  5. A novel locus for non-syndromic sensorineural deafness (DFN6) maps to chromosome Xp22.

    PubMed

    del Castillo, I; Villamar, M; Sarduy, M; Romero, L; Herraiz, C; Hernández, F J; Rodríguez, M; Borrás, I; Montero, A; Bellón, J; Tapia, M C; Moreno, F

    1996-09-01

    Non-syndromic X-linked deafness is highly heterogeneous. At least five different clinical forms have been described, but only two loci have been mapped. Here we report a Spanish family affected by a previously undescribed X-linked form of hearing impairment. Deafness is non-syndromic, sensorineural, and progressive. In affected males, the auditory impairment is first detected at school age, affecting mainly the high frequencies. Later it evolves to become severe to profound, involving all frequencies for adulthood. Carrier females manifest a moderate hearing impairment in the high frequencies, with the onset delayed to the fourth decade of life. Deafness was assumed to be X-linked dominant, with incomplete penetrance and variable expressivity in carrier females. The family was genotyped for a set of microsatellite markers evenly spaced at intervals of about 10 cM. We found evidence of linkage to markers in the Xp22 region (maximum lod score of 5.30 at theta = 0.000 for DXS8036 and for DXS8022). The position of the novel deafness locus (DFN6) was refined by haplotype analysis. Mapping of the breakpoints in two critical recombinants allowed us to define an interval for DFN6, delimited by DXS7108 on the distal side and by DXS7105 on the proximal side, and spanning a genetic distance of about 15 cM.

  6. Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy.

    PubMed

    Maeda, Yukihide; Kataoka, Yuko; Sugaya, Akiko; Kariya, Shin; Kobayashi, Katsuhiro; Nishizaki, Kazunori

    2015-06-01

    Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss.

  7. Acute tryptophan depletion induced by a gelatin-based mixture impairs object memory but not affective behavior and spatial learning in the rat.

    PubMed

    Lieben, Cindy K J; van Oorsouw, Kim; Deutz, Nicolaas E P; Blokland, Arjan

    2004-05-05

    One manner to study the role of serotonin (5-HT) in behavioral functions is through nutritional manipulation of its precursor tryptophan (TRP). By means of the method of acute TRP depletion, plasma TRP levels can be reduced in a reversible way in both humans and rats. In the present study a TRP-free protein-carbohydrate mixture was used to investigate the behavioral effects of lowering TRP and 5-HT concentrations in adult male rats. These animals were tested in models of anxiety (open field, home cage emergence test), depression (forced swimming test) and cognition (object recognition test and Morris water escape test). The TRP-free protein-carbohydrate mixture substantially reduced the ratio TRP/SigmaLNAA within 2 and 4 h by 75 and 60%, respectively. It was found that 4 h after administration, the treatment did not affect anxiety-related behavior nor did it cause depressive-like behavior. Also, no treatment effect was found on spatial learning performance in a Morris water escape test. On the other hand, performance in an object recognition test was clearly impaired after TRP depletion. Taken together, these data suggest that acute lowered central 5-HT levels are not associated with changes in affective behavior (i.e. anxiety and depression), but do impair object memory in adult rats.

  8. Myeloperoxidase and serum amyloid A contribute to impaired in vivo reverse cholesterol transport during the acute phase response but not group IIA secretory phospholipase A2[S

    PubMed Central

    Annema, Wijtske; Nijstad, Niels; Tölle, Markus; de Boer, Jan Freark; Buijs, Ruben V. C.; Heeringa, Peter; van der Giet, Markus; Tietge, Uwe J. F.

    2010-01-01

    Atherosclerosis is linked to inflammation. HDL protects against atherosclerotic cardiovascular disease, mainly by mediating cholesterol efflux and reverse cholesterol transport (RCT). The present study aimed to test the impact of acute inflammation as well as selected acute phase proteins on RCT with a macrophage-to-feces in vivo RCT assay using intraperitoneal administration of [3H]cholesterol-labeled macrophage foam cells. In patients with acute sepsis, cholesterol efflux toward plasma and HDL were significantly decreased (P < 0.001). In mice, acute inflammation (75 µg/mouse lipopolysaccharide) decreased [3H]cholesterol appearance in plasma (P < 0.05) and tracer excretion into feces both within bile acids (−84%) and neutral sterols (−79%, each P < 0.001). In the absence of systemic inflammation, overexpression of serum amyloid A (SAA, adenovirus) reduced overall RCT (P < 0.05), whereas secretory phospholipase A2 (sPLA2, transgenic mice) had no effect. Myeloperoxidase injection reduced tracer appearance in plasma (P < 0.05) as well as RCT (−36%, P < 0.05). Hepatic expression of bile acid synthesis genes (P < 0.01) and transporters mediating biliary sterol excretion (P < 0.01) was decreased by inflammation. In conclusion, our data demonstrate that acute inflammation impairs cholesterol efflux in patients and macrophage-to-feces RCT in vivo in mice. Myeloperoxidase and SAA contribute to a certain extent to reduced RCT during inflammation but not sPLA2. However, reduced bile acid formation and decreased biliary sterol excretion might represent major contributing factors to decreased RCT in inflammation. PMID:20061576

  9. Reconciling concepts of space and person-centred care of the older person with cognitive impairment in the acute care setting.

    PubMed

    Rushton, Carole; Edvardsson, David

    2016-07-26

    Although a large body of literature exists propounding the importance of space in aged care and care of the older person with dementia, there is, however, only limited exploration of the 'acute care space' as a particular type of space with archetypal constraints that maybe unfavourable to older people with cognitive impairment and nurses wanting to provide care that is person-centred. In this article, we explore concepts of space and examine the implications of these for the delivery of care to older people who are cognitively impaired. Our exploration is grounded in theorisations of space offered by key geographers and phenomenologists, but also draws on how space has been constructed within the nursing literature that refers specifically to acute care. We argue that space, once created, can be created and that nursing has a significant role to play in the process of its recreation in the pursuit of care that is person-centred. We conclude by introducing an alternative logic of space aimed at promoting the creation of more salutogenic spaces that invokes a sense of sanctuary, safeness, and inclusion, all of which are essential if the care provided to the older person with cognitive impairment is apposite to their needs. The concept of 'person-centred space' helps to crystallize the relationship between space and person-centred care and implies more intentional manipulation of space that is more conducive to caring and healing. Significantly, it marks a return to Nightingale's wisdom, that is, to put the person in the best possible conditions for nature to act upon them.

  10. Chronic Stress Induces a Hyporeactivity of the Autonomic Nervous System in Response to Acute Mental Stressor and Impairs Cognitive Performance in Business Executives

    PubMed Central

    Teixeira, Renata Roland; Díaz, Miguel Mauricio; Santos, Tatiane Vanessa da Silva; Bernardes, Jean Tofoles Martins; Peixoto, Leonardo Gomes; Bocanegra, Olga Lucia; Neto, Morun Bernardino; Espindola, Foued Salmen

    2015-01-01

    The present study examined the incidence of chronic stress in business executives (109 subjects: 75 male and 34 female) and its relationship with cortisol levels, cognitive performance, and autonomic nervous system (ANS) reactivity after an acute mental stressor. Blood samples were collected from the subjects to measure cortisol concentration. After the sample collection, the subjects completed the Lipp Inventory of Stress Symptoms for Adults and the Stroop Color-Word Test to evaluate stress and cognitive performance levels, respectively. Saliva samples were collected prior to, immediately after, and five minutes after the test. The results revealed that 90.1% of the stressed subjects experienced stress phases that are considered chronic stress. At rest, the subjects with chronic stress showed higher cortisol levels, and no gender differences were observed. No differences were found between the stressed and non-stressed subjects regarding salivary amylase activity prior to test. Chronic stress also impaired performance on the Stroop test, which revealed higher rates of error and longer reaction times in the incongruent stimulus task independently of gender. For the congruent stimulus task of the Stroop test, the stressed males presented a higher rate of errors than the non-stressed males and a longer reaction time than the stressed females. After the acute mental stressor, the non-stressed male group showed an increase in salivary alpha-amylase activity, which returned to the initial values five minutes after the test; this ANS reactivity was not observed in the chronically stressed male subjects. The ANS responses of the non-stressed vs stressed female groups were not different prior to or after the Stroop test. This study is the first to demonstrate a blunted reactivity of the ANS when male subjects with chronic psychological stress were subjected to an acute mental stressor, and this change could contribute to impairments in cognitive performance. PMID:25807003

  11. Chronic stress induces a hyporeactivity of the autonomic nervous system in response to acute mental stressor and impairs cognitive performance in business executives.

    PubMed

    Teixeira, Renata Roland; Díaz, Miguel Mauricio; Santos, Tatiane Vanessa da Silva; Bernardes, Jean Tofoles Martins; Peixoto, Leonardo Gomes; Bocanegra, Olga Lucia; Neto, Morun Bernardino; Espindola, Foued Salmen

    2015-01-01

    The present study examined the incidence of chronic stress in business executives (109 subjects: 75 male and 34 female) and its relationship with cortisol levels, cognitive performance, and autonomic nervous system (ANS) reactivity after an acute mental stressor. Blood samples were collected from the subjects to measure cortisol concentration. After the sample collection, the subjects completed the Lipp Inventory of Stress Symptoms for Adults and the Stroop Color-Word Test to evaluate stress and cognitive performance levels, respectively. Saliva samples were collected prior to, immediately after, and five minutes after the test. The results revealed that 90.1% of the stressed subjects experienced stress phases that are considered chronic stress. At rest, the subjects with chronic stress showed higher cortisol levels, and no gender differences were observed. No differences were found between the stressed and non-stressed subjects regarding salivary amylase activity prior to test. Chronic stress also impaired performance on the Stroop test, which revealed higher rates of error and longer reaction times in the incongruent stimulus task independently of gender. For the congruent stimulus task of the Stroop test, the stressed males presented a higher rate of errors than the non-stressed males and a longer reaction time than the stressed females. After the acute mental stressor, the non-stressed male group showed an increase in salivary alpha-amylase activity, which returned to the initial values five minutes after the test; this ANS reactivity was not observed in the chronically stressed male subjects. The ANS responses of the non-stressed vs stressed female groups were not different prior to or after the Stroop test. This study is the first to demonstrate a blunted reactivity of the ANS when male subjects with chronic psychological stress were subjected to an acute mental stressor, and this change could contribute to impairments in cognitive performance.

  12. Bilateral sudden sensorineural hearing loss following unilateral temporal bone fracture.

    PubMed

    Hunchaisri, Niran

    2009-06-01

    Temporal bone fractures usually cause unilateral sensorineural hearing loss (SNHL) by fracture that violated otic capsule of that side. Bilateral SNHL from unilateral temporal bone fracture were rarely seen. Labyrinthine concussion was considered to be the pathogenesis in these cases. This article reports an additional case of bilateral SNHL from unilateral temporal bone fracture but in a different pattern of SNHL which may result from an occlusion of the internal auditory artery.

  13. Lipid Profile among Patients with Sudden Sensorineural Hearing Loss.

    PubMed

    Mohammed, Ali A Muttalib

    2014-12-01

    Associations between hearing and blood lipids have been the focus of scientific inquiry for more than 50 years. The aim of the present study is to evaluate the association between hyperlipidaemia among patients presented with sudden sensorineural hearing loss compared to normal controls. A case control study concerned with 22 patients presented with sudden sensorineural hearing loss who underwent lipid profile evaluation. The lipid profile of these patients was compared with corresponding results of 55 age matched persons (volunteers) with normal hearing. These patients were collected from the Out Patient Department of ENT at Al-Jamhory Teaching Hospital, Mosul/Iraq and private clinic of the author for the period from February 2011 to July 2013. The average age of patients was 44.7 years with a range of 26-65 years. The peak age incidence was in the 5(th) decade of life. The study included 11 male patients (50 %) and 11 females (50 %). Meanwhile, the average age of the control group was 41.7 years with 25 (45.5 %) males and 30 (54.5 %) females. Statistical analysis showed that there was significant difference between the means of lipid profile and blood sugar of the patients and the control group apart from HDL where there was no significant difference. In conclusion, hyperlipidemia seems to be significantly associated with the occurrence of sudden sensorineural hearing loss according to this study.

  14. Cannabinoid HU210 protects isolated rat stomach against impairment caused by serum of rats with experimental acute pancreatitis.

    PubMed

    Cao, Ming-hua; Li, Yong-yu; Xu, Jing; Feng, Ya-jing; Lin, Xu-hong; Li, Kun; Han, Tong; Chen, Chang-jie

    2012-01-01

    Acute pancreatitis (AP), especially severe acute pancreatitis often causes extra-pancreatic complications, such as acute gastrointestinal mucosal lesion (AGML) which is accompanied by a considerably high mortality, yet the pathogenesis of AP-induced AGML is still not fully understood. In this report, we investigated the alterations of serum components and gastric endocrine and exocrine functions in rats with experimental acute pancreatitis, and studied the possible contributions of these alterations in the pathogenesis of AGML. In addition, we explored the intervention effects of cannabinoid receptor agonist HU210 and antagonist AM251 on isolated and serum-perfused rat stomach. Our results showed that the AGML occurred after 5 h of AP replication, and the body homeostasis was disturbed in AP rat, with increased levels of pancreatic enzymes, lipopolysaccharide (LPS), proinflammtory cytokines and chemokines in the blood, and an imbalance of the gastric secretion function. Perfusing the isolated rat stomach with the AP rat serum caused morphological changes in the stomach, accompanied with a significant increment of pepsin and [H+] release, and increased gastrin and decreased somatostatin secretion. HU210 reversed the AP-serum-induced rat pathological alterations, including the reversal of transformation of the gastric morphology to certain degree. The results from this study prove that the inflammatory responses and the imbalance of the gastric secretion during the development of AP are responsible for the pathogenesis of AGML, and suggest the therapeutic potential of HU210 for AGML associated with acute pancreatitis.

  15. The clinical analysis of bilateral successive sudden sensorineural hearing loss.

    PubMed

    Wang, Yaowen; Zhang, Litao; Zhang, Jianhua; Zhang, Xuqun; Zhang, Weimin; Chen, Xing; Tang, Shixiong

    2016-11-01

    The objective of this study is to investigate the clinical characteristics of successive bilateral sudden sensorineural hearing loss (SSNHL) with an interval of more than 1 year to aid the evaluation and management of successive bilateral SSNHL (BSSNHL). 14 successive BSSNHL patients and 118 unilateral SSNHL patients with severe and profound hearing loss were reviewed retrospectively. Information about successive BSSNHL was collected included demographics, the intervals between the attacks of bilateral ears, the past medical history, inducing factors, accompanying symptoms, pure-tone tests, blood tests, b-ultrasound examinations of vertebral artery and carotid artery, and medical interventions. And the comparison of improvement rate was made between successive BSSNHL and unilateral SSNHL. SPSS 15.0 was used to analyze the data. In successive BSSNHL, there were six males and eight females; the average aged was 49.86 ± 15.45 years (20-73 years). The interval of the two attacks was 11.43 ± 12.07 years (1-50 years) on average. The onset of treatment was 18.86 ± 12.71 days. Tinnitus was seen in 100 % of the patients, followed by vertigo in 42.85 %, and ear fullness in 21.43 %. 3 of 14 patients described obvious inducing factor: tiredness. Five patients (35.71 %) had hypertension histories, three (21.43 %) had diabetes histories, two (14.29 %) had surgery histories, one (7.14 %) was with depression history, one (7.14 %) was with coronary heart disease history. 30 % (3/10) patients were with atherosclerotic plaque in carotid artery. 4 (28.57 %) patients were with high blood sugar. 8 patients (57.14 %) were with high blood lipids. Thyroid function tests were positive in 27.27 % (3/11) patients. No abnormality was found in antinuclear antibodies titer. The percentage of profound and severe hearing loss were 71.43 and 78.57 % in the recently affected ear and contralateral ear separately. The PTAs of the recently affected ear were 77.14 ± 27.12

  16. Elaboration of a clinical and paraclinical score to estimate the probability of herpes simplex virus encephalitis in patients with febrile, acute neurologic impairment.

    PubMed

    Gennai, S; Rallo, A; Keil, D; Seigneurin, A; Germi, R; Epaulard, O

    2016-06-01

    Herpes simplex virus (HSV) encephalitis is associated with a high risk of mortality and sequelae, and early diagnosis and treatment in the emergency department are necessary. However, most patients present with non-specific febrile, acute neurologic impairment; this may lead clinicians to overlook the diagnosis of HSV encephalitis. We aimed to identify which data collected in the first hours in a medical setting were associated with the diagnosis of HSV encephalitis. We conducted a multicenter retrospective case-control study in four French public hospitals from 2007 to 2013. The cases were the adult patients who received a confirmed diagnosis of HSV encephalitis. The controls were all the patients who attended the emergency department of Grenoble hospital with a febrile acute neurologic impairment, without HSV detection by polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF), in 2012 and 2013. A multivariable logistic model was elaborated to estimate factors significantly associated with HSV encephalitis. Finally, an HSV probability score was derived from the logistic model. We identified 36 cases and 103 controls. Factors independently associated with HSV encephalitis were the absence of past neurological history (odds ratio [OR] 6.25 [95 % confidence interval (CI): 2.22-16.7]), the occurrence of seizure (OR 8.09 [95 % CI: 2.73-23.94]), a systolic blood pressure ≥140 mmHg (OR 5.11 [95 % CI: 1.77-14.77]), and a C-reactive protein <10 mg/L (OR 9.27 [95 % CI: 2.98-28.88]). An HSV probability score was calculated summing the value attributed to each independent factor. HSV encephalitis diagnosis may benefit from the use of this score based upon some easily accessible data. However, diagnostic evocation and probabilistic treatment must remain the rule.

  17. A comparison of toxicities in acute myeloid leukemia patients with and without renal impairment treated with decitabine.

    PubMed

    Levine, Lauren B; Roddy, Julianna Vf; Kim, Miryoung; Li, Junan; Phillips, Gary; Walker, Alison R

    2017-01-01

    Purpose There are limited data regarding the clinical use of decitabine for the treatment of acute myeloid leukemia in patients with a serum creatinine of 2 mg/dL or greater. Methods We retrospectively evaluated 111 patients with acute myeloid leukemia who had been treated with decitabine and compared the development of toxicities during cycle 1 in those with normal renal function (creatinine clearance greater than or equal to 60 mL/min) to those with renal dysfunction (creatinine clearance less than 60 mL/min). Results Notable differences in the incidence of grade ≥3 cardiotoxicity (33% of renal dysfunction patients vs. 16% of normal renal function patients, p = 0.042) and respiratory toxicity (40% of renal dysfunction patients vs. 14% of normal renal function patients, p = 0.0037) were observed. The majority of heart failure, myocardial infarction, and atrial fibrillation cases occurred in the renal dysfunction group. The odds of developing grade ≥3 cardiotoxicity did not differ significantly between patients with and without baseline cardiac comorbidities (OR 1.43, p = 0.43). Conclusions This study noted a higher incidence of grade ≥3 cardiac and respiratory toxicities in decitabine-treated acute myeloid leukemia patients with renal dysfunction compared to normal renal function. This may prompt closer monitoring, regardless of baseline cardiac comorbidities. Further evaluation of decitabine in patients with renal dysfunction is needed.

  18. Relation between acute and late irradiation impairment of four basic tastes and irradiated tongue volume in patients with head-and-neck cancer

    SciTech Connect

    Yamashita, Hideomi . E-mail: yamachan07291973@yahoo.co.jp; Nakagawa, Keiichi; Nakamura, Naoki; Abe, Keiko; Asakage, Takahiro; Ohmoto, Makoto; Okada, Shinji; Matsumoto, Ichiro; Hosoi, Yoshio; Sasano, Nakashi; Yamakawa, Sen; Ohtomo, Kuni

    2006-12-01

    Purpose: Taste loss is a major cause of morbidity in patients undergoing head-and-neck irradiation. The relationship between the time course and the degree of taste disorder was studied in both acute and late phases. Methods and Materials: Taste ability was measured by the taste threshold for the four basic tastes using a filter paper disc method in patients before, during, and after radiotherapy. The subjects were divided into two groups. In Group A, Radiation fields included most of the tongue (n = 100), and in Group B Radiation fields did not include the tip of the tongue (n = 18). Results: In Group A, there was a significant impairment of the threshold of all four basic tastes at 3 weeks after starting radiotherapy (RT), and this impairment remained at 8 weeks (p < 0.05). This was not seen in Group B. In Group A, there was no significant difference in the patterns of taste sensitivity change between the high-dose (>20 Gy) and low-dose ({<=}20 Gy) groups. In the late phase, recovery of taste loss was seen in both groups since 4 months after completing RT. Conclusions: Unless the anterior part of the tongue was irradiated, taste loss was not observed during RT. When the anterior part of the tongue was irradiated, a difference by radiation dose was not observed in the taste loss pattern. Additionally, radiation-induced taste dysfunction appears to be a temporal effect.

  19. Reconciling conceptualisations of the body and person-centred care of the older person with cognitive impairment in the acute care setting.

    PubMed

    Rushton, Carole; Edvardsson, David

    2016-11-23

    In this article, we sought reconciliation between the "body-as-representation" and the "body-as-experience," that is, how the body is represented in discourse and how the body of older people with cognitive impairment is experienced. We identified four contemporary "technologies" and gave examples of these to show how they influence how older people with cognitive impairment are often represented in acute care settings. We argued that these technologies may be mediated further by discourses of ageism and ableism which can potentiate either the repressive or productive tendencies of these technologies resulting in either positive or negative care experiences for the older person and/or their carer, including nurses. We then provided examples from research of embodied experiences of older people with dementia and of how nurses and other professionals utilized their inter-bodily experiences to inform acts of caring. The specificity and individuality of these experiences were more conducive to positive care experiences. We conclude the article by proposing that the act of caring is one way nurses seek to reconcile the "body-as-representation" with the "body-as-experience" to mitigate the repressive effects of negative ageism and ableism. The act of caring, we argue, is the essence of caring enacted through the provision of person-centred care which evokes nurses to respond appropriately to the older person's "otherness," their "variation of being" while enabling them to enact a continuation of themselves and their own version of normality.

  20. Low and moderate doses of acute ethanol do not impair spatial cognition but facilitate accelerating rotarod performance in adolescent and adult rats.

    PubMed

    Novier, Adelle; Van Skike, Candice E; Chin, Vivien S; Diaz-Granados, Jaime L; Matthews, Douglas B

    2012-03-14

    Adolescents and adult rodents have differing sensitivities to the acute effects of ethanol on a variety of behavioral and electrophysiological measures. Often, these differences are revealed using high ethanol doses and consequently little is known about these age-related effects using lower ethanol doses. We sought to determine if low-dose ethanol produces differential effects on cognition and motor behavior in adolescent and adult rats. Adolescent (postnatal day PD 30-32) and adult (PD 70-72) male Sprague Dawley rats were trained on the standard version of the Morris Water Maze (MWM) for 5 days or received 5 training trials on an accelerating rotarod (ARR). Adolescents learned the location of the submerged platform in the MWM significantly slower than adults during training and, acute ethanol administration (0.5 g/kg, 0.75 g/kg, or 1.0 g/kg) 30 min before testing did not impair spatial memory in either age group. On the ARR test, adolescent rats spent significantly more time on the rotarod compared to adults and, alcohol exposure (1.0 g/kg) significantly increased ARR performance 30 min following administration. Our findings address the utility of investigating low and moderate doses of ethanol during different developmental stages in rats.

  1. Near Infrared Spectroscopy (NIRS) as a New Non-Invasive Tool to Detect Oxidative Skeletal Muscle Impairment in Children Survived to Acute Lymphoblastic Leukaemia

    PubMed Central

    Lanfranconi, Francesca; Pollastri, Luca; Ferri, Alessandra; Fraschini, Donatella; Masera, Giuseppe; Miserocchi, Giuseppe

    2014-01-01

    Background Separating out the effects of cancer and treatment between central and peripheral components of the O2 delivery chain should be of interest to clinicians for longitudinal evaluation of potential functional impairment in order to set appropriate individually tailored training/rehabilitation programmes. We propose a non-invasive method (NIRS, near infrared spectroscopy) to be used in routine clinical practice to evaluate a potential impairment of skeletal muscle oxidative capacity during exercise in children previously diagnosed with acute lymphoblastic leukaemia (ALL). The purpose of this study was to evaluate the capacity of skeletal muscle to extract O2 in 10 children diagnosed with ALL, 1 year after the end of malignancy treatment, compared to a control group matched for gender and age (mean±SD = 7.8±1.5 and 7.3±1.4 years, respectively). Methods and Findings Participants underwent an incremental exercise test on a treadmill until exhaustion. Oxygen uptake (), heart rate (HR), and tissue oxygenation status (Δ[HHb]) of the vastus lateralis muscle evaluated by NIRS, were measured. The results showed that, in children with ALL, a significant linear regression was found by plotting vs Δ[HHb] both measured at peak of exercise. In children with ALL, the slope of the HR vs linear response (during sub-maximal and peak work rates) was negatively correlated with the peak value of Δ[HHb]. Conclusions The present study proves that the NIRS technique allows us to identify large inter-individual differences in levels of impairment in muscle O2 extraction in children with ALL. The outcome of these findings is variable and may reflect either muscle atrophy due to lack of use or, in the most severe cases, an undiagnosed myopathy. PMID:24956391

  2. Resolution of sudden sensorineural hearing loss following a roller coaster ride.

    PubMed

    Kumar, Aman; Sinha, Amrita; Al-Waa, Ahmad M

    2011-07-01

    We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal prior to the episode). Following resolution of the patient's symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient's symptoms was attributed to a patent cochlear aqueduct.

  3. Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia

    PubMed Central

    Poburski, Dörte; Boerner, Josefine Barbara; Koenig, Michel; Ristow, Michael

    2016-01-01

    ABSTRACT Friedreich ataxia is a neurodegenerative disease caused by a GAA triplet repeat expansion in the first intron of the frataxin gene, which results in reduced expression levels of the corresponding protein. Despite numerous animal and cellular models, therapeutic options that mechanistically address impaired frataxin expression are lacking. Here, we have developed a new mammalian cell model employing the Cre/loxP recombination system to induce a homozygous or heterozygous frataxin knockout in mouse embryonic fibroblasts. Induction of Cre-mediated disruption by tamoxifen was successfully tested on RNA and protein levels. After loss of frataxin protein, cell division, aconitase activity and oxygen consumption rates were found to be decreased, while ROS production was increased in the homozygous state. By contrast, in the heterozygous state no such changes were observed. A time-resolved analysis revealed the loss of aconitase activity as an initial event after induction of complete frataxin deficiency, followed by secondarily elevated ROS production and a late increase in iron content. Initial impairments of oxygen consumption and ATP production were found to be compensated in the late state and seemed to play a minor role in Friedreich ataxia pathophysiology. In conclusion and as predicted from its proposed role in iron sulfur cluster (ISC) biosynthesis, disruption of frataxin primarily causes impaired function of ISC-containing enzymes, whereas other consequences, including elevated ROS production and iron accumulation, appear secondary. These parameters and the robustness of the newly established system may additionally be used for a time-resolved study of pharmacological candidates in a HTS manner. PMID:27106929

  4. Estimation of the contribution of norketamine to ketamine-induced acute pain relief and neurocognitive impairment in healthy volunteers

    PubMed Central

    Olofsen, Erik; Noppers, Ingeborg; Niesters, Marieke; Kharasch, Evan; Aarts, Leon; Sarton, Elise; Dahan, Albert

    2012-01-01

    Background The N-methyl-D-receptor antagonist ketamine is metabolized in the liver into its active metabolite norketamine. No human data are available on the relative contribution of norketamine to ketamine-induced analgesia and side effects. One approach to assess the ketamine and norketamine contributions is by measuring ketamine-effect at varying ketamine and norketamine plasma concentrations using the CYP450 inducer rifampicin. Methods In 12 healthy male volunteers the effect of rifampicin versus placebo pretreatment on S-ketamine (a 2-h infusion of 20 mg/h)-induced analgesia and cognition was quantified. The relative ketamine and norketamine contribution to effect was estimated using a linear additive population pharmacokinetic-pharmacodynamic model. Results S-ketamine produced significant analgesia, psychotropic effects (drug high), and cognitive impairment (including memory impairment, reduced psychomotor speed, reduced reaction time, reduced cognitive flexibility). Modeling revealed a negative contribution of S-norketamine to S-ketamine-induced analgesia and absence of contribution to cognitive impairment. At ketamine and norketamine effect concentrations of 100 ng/ml and 50 ng/ml, respectievly, the ketamine contribution to analgesia is −3.8 cm (visual analogue pain score) versus a contribution of norketamine of +1.5 cm, causing an overall effect −2.3 cm. The blood-effect-site equilibration half-life ranged from 0 (cognitive flexibility) to 11.8 (pain intensity) min, and averaged across all end-points was 6.1 min. Conclusions This first observation that norketamine produces effects in the opposite direction of ketamine requires further proof. It can explain the observation of ketamine-related excitatory phenomena (such as hyperalgesia and allodynia) upon the termination of ketamine infusions. PMID:22692377

  5. Impaired acquired resistance of mice to Klebsiella pneumoniae infection induced by acute NO/sub 2/ exposure

    SciTech Connect

    Bouley, G.; Azoulay-Dupuis, E.; Gaudebout, C.

    1985-12-01

    The natural resistance of nonimmunized C57B1/6 mice to an intraperitoneal Klebsiella pneumoniae challenge was not significantly affected by prior continuous exposure to 20 ppm NO/sub 2/ for 4 days. In contrast, the acquired resistance of mice immunized just before and infected just after NO/sub 2/ exposure was seriously impaired. This could not be explained by the loss of appetite (about 30%) observed in NO/sub 2/ treated mice, for neither the natural nor acquired resistance of control air exposure mice given approximately 70% ad libitum food and water were significantly modified.

  6. Cochlear implantation for severe sensorineural hearing loss caused by lightning.

    PubMed

    Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun

    2012-01-01

    Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation.

  7. Sudden sensorineural hearing loss after non-otologic surgery.

    PubMed

    Page, Joshua Cody; Peters, Bob

    2015-01-01

    Sudden sensorineural hearing loss following non-otologic surgery is a rare event described in the medical literature. Cardiopulmonary bypass surgery is most commonly associated with this type of hearing loss. Our case report and review of the literature describe two cases with postoperative hearing loss - neither of which are cardiac surgeries - making them exceedingly rare in the medical literature. Regardless of the rarity of this unfortunate event, the possibility for permanent hearing loss is a potentially devastating unanticipated complication and one that all surgeons should be aware.

  8. Implications of Sensorineural Hearing Loss With Hydrocodone/Acetaminophen Abuse

    PubMed Central

    Novac, Andrei; Iosif, Anamaria M.; Groysman, Regina; Bota, Robert G.

    2015-01-01

    Sensorineural hearing loss is an infrequently recognized side effect of pain medication abuse. Chronic pain patients treated with opiates develop different degrees of tolerance to pain medications. In many cases, the tolerance becomes the gateway to a variety of cycles of overuse and unmasking of significant psychiatric morbidity and mortality. An individualized approach utilizing combined treatment modalities (including nonopiate pharmaceuticals) is expected to become the norm. Patients can now be provided with multidisciplinary care that addresses an individual’s psychiatric, social, and medical needs, which requires close cooperation between physicians of varying specialties. This report describes a patient who experienced hearing loss from hydrocodone/acetaminophen abuse. PMID:26835162

  9. Renal diagnosis without renal biopsy. Nephritis and sensorineural deafness.

    PubMed

    Richardson, D; Shires, M; Davison, A M

    2001-06-01

    Two examples of hereditary nephropathy within the context of clinical syndromes are described. Emphasis is put on the ability to make a renal diagnosis without renal biopsy and the benefits of screening relatives once a diagnosis is achieved. A variant of Alport's syndrome with associated macrothrombocytic thrombocytopenia, known as Epstein's syndrome, is reported. In addition siblings with Alström's syndrome characterized by pigmentary retinal degeneration (causing blindness in early childhood), progressive sensorineural hearing loss, and progressive renal failure are reported. Both cases had previously presented for non-renal pathology in advance of the onset of symptomatic renal failure and may have benefited from appropriate screening.

  10. C-Terminus of Heat Shock Cognate 70 Interacting Protein Increases Following Stroke and Impairs Survival Against Acute Oxidative Stress

    PubMed Central

    Stankowski, Jeannette N.; Zeiger, Stephanie L.H.; Cohen, Evan L.; DeFranco, Donald B.; Cai, Jiyang

    2011-01-01

    Abstract The decision to remove or refold oxidized, denatured, or misfolded proteins by heat shock protein 70 and its binding partners is critical to determine cell fate under pathophysiological conditions. Overexpression of the ubiquitin ligase C-terminus of HSC70 interacting protein (CHIP) can compensate for failure of other ubiquitin ligases and enhance protein turnover and survival under chronic neurological stress. The ability of CHIP to alter cell fate after acute neurological injury has not been assessed. Using postmortem human tissue samples, we provide the first evidence that cortical CHIP expression is increased after ischemic stroke. Oxygen glucose deprivation in vitro led to rapid protein oxidation, antioxidant depletion, proteasome dysfunction, and a significant increase in CHIP expression. To determine if CHIP upregulation enhances neural survival, we overexpressed CHIP in vitro and evaluated cell fate 24 h after acute oxidative stress. Surprisingly, CHIP overexpressing cells fared worse against oxidative injury, accumulated more ubiquitinated and oxidized proteins, and experienced decreased proteasome activity. Conversely, using small interfering RNA to decrease CHIP expression in primary neuronal cultures improved survival after oxidative stress, suggesting that increases in CHIP observed after stroke like injuries are likely correlated with diminished survival and may negatively impact the neuroprotective potential of heat shock protein 70. Antioxid. Redox Signal. 14, 1787–1801. PMID:20677910

  11. Role of Cystathionine Gamma-Lyase in Immediate Renal Impairment and Inflammatory Response in Acute Ischemic Kidney Injury

    PubMed Central

    Markó, Lajos; Szijártó, István A.; Filipovic, Milos R.; Kaßmann, Mario; Balogh, András; Park, Joon-Keun; Przybyl, Lukasz; N’diaye, Gabriele; Krämer, Stephanie; Anders, Juliane; Ishii, Isao; Müller, Dominik N.; Gollasch, Maik

    2016-01-01

    Hydrogen sulfide (H2S) is known to act protectively during renal ischemia/reperfusion injury (IRI). However, the role of the endogenous H2S in acute kidney injury (AKI) is largely unclear. Here, we analyzed the role of cystathionine gamma-lyase (CTH) in acute renal IRI using CTH-deficient (Cth−/−) mice whose renal H2S levels were approximately 50% of control (wild-type) mice. Although levels of serum creatinine and renal expression of AKI marker proteins were equivalent between Cth−/− and control mice, histological analysis revealed that IRI caused less renal tubular damage in Cth−/− mice. Flow cytometric analysis revealed that renal population of infiltrated granulocytes/macrophages was equivalent in these mice. However, renal expression levels of certain inflammatory cytokines/adhesion molecules believed to play a role in IRI were found to be lower after IRI only in Cth−/− mice. Our results indicate that the systemic CTH loss does not deteriorate but rather ameliorates the immediate AKI outcome probably due to reduced inflammatory responses in the kidney. The renal expression of CTH and other H2S-producing enzymes was markedly suppressed after IRI, which could be an integrated adaptive response for renal cell protection. PMID:27273292

  12. Identification of a novel mutation in the SLC26A4 gene in an Italian with fluctuating sensorineural hearing loss.

    PubMed

    Cama, Elona; Alemanno, Maria Stella; Bellacchio, Emanuele; Santarelli, Rosamaria; Carella, Massimo; Zelante, Leopoldo; Palladino, Teresa; Inches, Ingrid; di Paola, Francesco; Arslan, Edoardo; Melchionda, Salvatore

    2009-10-01

    Pendred syndrome is an autosomal recessive disorder characterized by congenital sensorineural deafness, goitre and defective iodide organification. Congenital and profound hearing loss is the hallmark of the syndrome, while goitre and thyroid dysfunction are highly variable even within the same family. Clinical features are due to altered formation of pendrin, a chloride/iodide transporter protein expressed in the inner ear, thyroid gland and kidney. A novel substitution was found in exon 7 of the pendrin encoding gene (SLC26A4) that leads to a stop codon, S314X. The new variation was found in compound heterozygosity with L445W mutation in a hearing impaired patient with bilateral Mondini's dysplasia and goitre.

  13. Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike.

    PubMed

    Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

    2015-01-01

    In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature.

  14. Sudden onset unilateral sensorineural hearing loss after rabies vaccination.

    PubMed

    Okhovat, Saleh; Fox, Richard; Magill, Jennifer; Narula, Antony

    2015-12-15

    A 33-year-old man developed profound sudden onset right-sided hearing loss with tinnitus and vertigo, within 24 h of pretravel rabies vaccination. There was no history of upper respiratory tract infection, systemic illness, ototoxic medication or trauma, and normal otoscopic examination. Pure tone audiograms (PTA) demonstrated right-sided sensorineural hearing loss (thresholds 90-100 dB) and normal left-sided hearing. MRI internal acoustic meatus, viral serology (hepatitis B, C, HIV and cytomegalovirus) and syphilis screen were normal. Positive Epstein-Barr virus IgG, viral capsid IgG and anticochlear antibodies (anti-HSP-70) were noted. Initial treatment involved a course of high-dose oral prednisolone and acyclovir. Repeat PTAs after 12 days of treatment showed a small improvement in hearing thresholds. Salvage intratympanic steroid injections were attempted but failed to improve hearing further. Sudden onset sensorineural hearing loss (SSNHL) is an uncommon but frightening experience for patients. This is the first report of SSNHL following rabies immunisation in an adult.

  15. Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

    PubMed Central

    Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

    2015-01-01

    In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature. PMID:26161278

  16. Endothelial Dysfunction in Idiopathic Sudden Sensorineural Hearing Loss: A Review

    PubMed Central

    Quaranta, Nicola; De Ceglie, Vincenzo; D’Elia, Alessandra

    2016-01-01

    An endothelial dysfunction has been described in idiopathic sudden sensorineural hearing loss (ISSHL) patients. The purpose of our review was to: i) identify, evaluate and review recent research about cardiovascular risk factors involvement and signs of endothelial dysfunction in ISSHL; ii) implication of these discovering in clinical practice and future research. A Medline literature search was conducted to identify any study on the involvement of endothelial dysfunction in ISSHL, published in the English language in the last decade. The following MEDLINE search terms were used: sudden sensorineural hearing loss (SSHL) and endothelial dysfunction (text words). Additional studies were identified by hand searching the references of original articles and review articles. Studies were not excluded on the basis of the qualitative or quantitative definitions of SSHL, treatment regimens, or outcome measures. Data were extracted from included papers by a reviewer. Information on the patients, investigations, methods, interventions, and outcomes were systematically analyzed. Characteristics and results of all included studies were reviewed systematically. High levels of adhesion molecules, hyperhomocysteinemia and lower folate levels, unbalanced oxidative status, a lower value of flow-mediated dilatation of brachial artery and a reduced percentage of circulating endothelial progenitor cells in patients affected by ISSHL support the hypothesis that this syndrome should be considered as a microcirculation disorder based on endothelial dysfunction and drive clinicians to implement all the traditional strategies used for preventing cardiovascular events, to also reduce the likelihood of ISSHL occurrence. PMID:27588164

  17. [Aggravation after Diagnosis of Sudden Sensorineural Hearing Loss].

    PubMed

    Fukumoto, Ichiro; Nemoto, Toshimitsu; Tsukuda, Tomoko; Koshizuka, Keiichi

    2015-03-01

    Among 95 patients with sudden sensorineural hearing loss who received inpatient treatment at our hospital within the 27-month period between October 2009 and December 2011, those in whom hearing loss was aggravated after diagnosis were compared with a control group. Hearing loss aggravation was defined as a decrease by 10 dB or more in the mean hearing threshold at 5 frequencies from 250 to 4,000 Hz or decrease of 15 dB or more in the hearing threshold at 2 consecutive frequencies. Hearing loss was aggravated after diagnosis in 22 (23.2%) of the 95 patients, showing a similar tendency to that previously reported. Although the grades of hearing loss in these patients were higher than those in 73 control group patients, according to the sudden hearing loss severity classification, their outcomes were favorable. The hearing loss aggravation group consisted of those with steroid-dependent hearing loss (6) and those who had undergone perilymphatic fistula repair (4), in addition to a large number of patients with idiopathic hearing loss, including suspicious perilymphatic fistula (10). When hearing loss becomes aggravated after the diagnosis of sudden sensorineural hearing loss, it may be important to determine the most appropriate approach in each case, such as a careful and gradual decrease in the adenocortical steroid dose and the consideration of perilymphatic fistula repair.

  18. Acute leukemia after radiotherapy in a patient with Turcot's syndrome. Impaired colony formation in skin fibroblast cultures after irradiation

    SciTech Connect

    Li, F.P.; Little, J.B.; Bech-Hansen, N.T.; Paterson, M.C.; Arlett, C.; Garnick, M.B.; Mayer, R.J.

    1983-02-01

    Colonic polyposis and carcinoma developed in a woman with Turcot's syndrome at the age of 31 years; astrocytoma developed when she was 37. Her brother and sister had died of astrocytoma at the ages of 18 and 33 years, respectively. Progressive neutropenia developed in the patient three months after radiotherapy for her brain tumor and acute myelomonocytic leukemia 19 months after treatment. Three laboratories independently evaluated cultures of her skin fibroblasts for in vitro sensitivity to cell killing (loss of colony-forming ability) by x-rays. Survival assays consistently revealed slight but significant radiosensitivity in an early-passage (six to 10 doublings) fibroblast subculture. A later subculture (21 to 29 doublings) showed no abnormality, a possible effect of selective in vitro loss of radiosensitive cells.

  19. Exaggerated Acute Lung Injury and Impaired Antibacterial Defenses During Staphylococcus aureus Infection in Rats with the Metabolic Syndrome

    PubMed Central

    Feng, Xiaomei; Maze, Mervyn; Koch, Lauren G.; Britton, Steven L.; Hellman, Judith

    2015-01-01

    Rats with Metabolic Syndrome (MetaS) have a dysregulated immune response to the aseptic trauma of surgery. We hypothesized that rats with MetaS would have dysregulated inflammation, increased lung injury, and less effective antibacterial defenses during Staphylococcus (S.) aureus sepsis as compared to rats without MetaS. Low capacity runner (LCR; a model of MetaS) and high capacity runner (HCR) rats were challenged intravenously with S. aureus bacteria. After 48 h, inflammatory mediators and bacteria were quantified in the blood, bronchoalveolar lavage fluid (BALF), and lung homogenates. Lungs were analyzed histologically. BALF protein and lung wet-dry ratios were quantified to assess for vascular leak. Endpoints were compared in infected LCR vs HCR rats. LCR rats had higher blood and lung S. aureus counts, as well as higher levels of IL-6 in plasma, lungs and BALF, MIP-2 in plasma and lung, and IL-17A in lungs. Conversely, LCR rats had lower levels of IL-10 in plasma and lungs. Although lactate levels, and liver and renal function tests were similar between groups, LCR rats had higher BALF protein and lung wet-dry ratios, and more pronounced acute lung injury histologically. During S. aureus bacteremia, as compared with HCR rats, LCR (MetaS) rats have heightened pro-inflammatory responses, accompanied by increased acute lung injury and vascular leak. Notably, despite an augmented pro-inflammatory phenotype, LCR rats have higher bacterial levels in their blood and lungs. The MetaS state may exacerbate lung injury and vascular leak by attenuating the inflammation-resolving response, and by weakening antimicrobial defenses. PMID:25978669

  20. Impaired Circulating CD4+LAP+ Regulatory T Cells in Patients with Acute Coronary Syndrome and Its Mechanistic Study

    PubMed Central

    Mao, Xiao-Bo; Yu, Kun-Wu; Zhang, Wei; Zhu, Peng-Fei; Ren, Ze-Peng; Wu, Bang-Wei; Ji, Qin-Wei; Wang, Xiang; Zeng, Qiu-Tang

    2014-01-01

    Objective CD4+ latency-associated peptide (LAP)+ regulatory T cells (Tregs) are a newly discovered T cell subset in humans and the role of these cells in patients with acute coronary syndrome (ACS) has not been explored. We designed to investigate whether circulating frequency and function of CD4+LAP+ Tregs are defective in ACS. Methods One hundred eleven ACS patients (acute myocardial infarction and unstable angina) and 117 control patients were enrolled in the study. The control patients consisted of chronic stable angina (CSA) and chest pain syndrome (CPS). The frequencies of circulating CD4+LAP+ Tregs and the expression of the transmembrane protein glycoprotein-A repetitions predominant (GARP) on CD4+ T cells were determined by flow cytometry. The function of CD4+LAP+ Tregs was detected using thymidine uptake. Serum interleukin-10 (IL-10) and transforming growth factor-β protein (TGF-β) levels were detected using ELISA and expression of GARP mRNA in peripheral blood mononuclear cells (PBMCs) was measured by real time-polymerase chain reaction. Results We found ACS patients had a significantly lower frequency of circulating CD4+LAP+ Tregs, and the function of these cells was reduced compared to controls. The expression of GARP in CD4+ T cells and the serum levels of TGF-β in ACS patients were lower than those of control patients. The serum levels of IL-10 were similar between the two cohorts. Conclusions A novel regulatory T cell subset, defined as CD4+LAP+ T cells is defective in ACS patients. PMID:24558424

  1. Acute Heat-Evoked Temperature Sensation Is Impaired but Not Abolished in Mice Lacking TRPV1 and TRPV3 Channels

    PubMed Central

    Reynders, Ana; Gaillard, Stéphane; Moqrich, Aziz

    2014-01-01

    The discovery of heat-sensitive Transient Receptor Potential Vanilloid ion channels (ThermoTRPVs) greatly advanced our molecular understanding of acute and injury-evoked heat temperature sensation. ThermoTRPV channels are activated by partially overlapping temperatures ranging from warm to supra-threshold noxious heat. TRPV1 is activated by noxious heat temperature whereas TRPV3 can be activated by warm as well as noxious heat temperatures. Loss-of-function studies in single TRPV1 and TRPV3 knock-out mice have shown that heat temperature sensation is not completely abolished suggesting functional redundancies among these two channels and highlighting the need of a detailed analysis of TRPV1::TRPV3 double knock-out mice (V1V3dKO) which is hampered by the close proximity of the loci expressing the two channels. Here we describe the generation of a novel mouse model in which trpv1 and trpv3 genes have been inactivated using bacterial artificial chromosome (BAC)-based homologous recombination in embryonic stem cells. In these mice, using classical thermosensory tests such hot plate, tail flick and the thermotaxis gradient paradigms, we confirm that TRPV1 is the master channel for sensing noxious heat temperatures and identify a cooperative role of TRPV1 and TRPV3 for sensing a well-defined window of acute moderate heat temperature. Using the dynamic hot plate assay, we unravel an intriguing and unexpected pronounced escape behavior in TRPV1 knock-out mice that was attenuated in the V1V3dKO. Together, and in agreement with the temperature activation overlap between TRPV1 and TRPV3 channels, our data provide in vivo evidence of a cooperative role between skin-derived TRPV3 and primary sensory neurons-enriched TRPV1 in modulation of moderate and noxious heat temperature sensation and suggest that other mechanisms are required for heat temperature sensation. PMID:24925072

  2. Acute heat-evoked temperature sensation is impaired but not abolished in mice lacking TRPV1 and TRPV3 channels.

    PubMed

    Marics, Irène; Malapert, Pascale; Reynders, Ana; Gaillard, Stéphane; Moqrich, Aziz

    2014-01-01

    The discovery of heat-sensitive Transient Receptor Potential Vanilloid ion channels (ThermoTRPVs) greatly advanced our molecular understanding of acute and injury-evoked heat temperature sensation. ThermoTRPV channels are activated by partially overlapping temperatures ranging from warm to supra-threshold noxious heat. TRPV1 is activated by noxious heat temperature whereas TRPV3 can be activated by warm as well as noxious heat temperatures. Loss-of-function studies in single TRPV1 and TRPV3 knock-out mice have shown that heat temperature sensation is not completely abolished suggesting functional redundancies among these two channels and highlighting the need of a detailed analysis of TRPV1::TRPV3 double knock-out mice (V1V3dKO) which is hampered by the close proximity of the loci expressing the two channels. Here we describe the generation of a novel mouse model in which trpv1 and trpv3 genes have been inactivated using bacterial artificial chromosome (BAC)-based homologous recombination in embryonic stem cells. In these mice, using classical thermosensory tests such hot plate, tail flick and the thermotaxis gradient paradigms, we confirm that TRPV1 is the master channel for sensing noxious heat temperatures and identify a cooperative role of TRPV1 and TRPV3 for sensing a well-defined window of acute moderate heat temperature. Using the dynamic hot plate assay, we unravel an intriguing and unexpected pronounced escape behavior in TRPV1 knock-out mice that was attenuated in the V1V3dKO. Together, and in agreement with the temperature activation overlap between TRPV1 and TRPV3 channels, our data provide in vivo evidence of a cooperative role between skin-derived TRPV3 and primary sensory neurons-enriched TRPV1 in modulation of moderate and noxious heat temperature sensation and suggest that other mechanisms are required for heat temperature sensation.

  3. Exaggerated Acute Lung Injury and Impaired Antibacterial Defenses During Staphylococcus aureus Infection in Rats with the Metabolic Syndrome.

    PubMed

    Feng, Xiaomei; Maze, Mervyn; Koch, Lauren G; Britton, Steven L; Hellman, Judith

    2015-01-01

    Rats with Metabolic Syndrome (MetaS) have a dysregulated immune response to the aseptic trauma of surgery. We hypothesized that rats with MetaS would have dysregulated inflammation, increased lung injury, and less effective antibacterial defenses during Staphylococcus (S.) aureus sepsis as compared to rats without MetaS. Low capacity runner (LCR; a model of MetaS) and high capacity runner (HCR) rats were challenged intravenously with S. aureus bacteria. After 48 h, inflammatory mediators and bacteria were quantified in the blood, bronchoalveolar lavage fluid (BALF), and lung homogenates. Lungs were analyzed histologically. BALF protein and lung wet-dry ratios were quantified to assess for vascular leak. Endpoints were compared in infected LCR vs HCR rats. LCR rats had higher blood and lung S. aureus counts, as well as higher levels of IL-6 in plasma, lungs and BALF, MIP-2 in plasma and lung, and IL-17A in lungs. Conversely, LCR rats had lower levels of IL-10 in plasma and lungs. Although lactate levels, and liver and renal function tests were similar between groups, LCR rats had higher BALF protein and lung wet-dry ratios, and more pronounced acute lung injury histologically. During S. aureus bacteremia, as compared with HCR rats, LCR (MetaS) rats have heightened pro-inflammatory responses, accompanied by increased acute lung injury and vascular leak. Notably, despite an augmented pro-inflammatory phenotype, LCR rats have higher bacterial levels in their blood and lungs. The MetaS state may exacerbate lung injury and vascular leak by attenuating the inflammation-resolving response, and by weakening antimicrobial defenses.

  4. Perception of dissonance by people with normal hearing and sensorineural hearing loss.

    PubMed

    Tufts, Jennifer B; Molis, Michelle R; Leek, Marjorie R

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  5. Perception of dissonance by people with normal hearing and sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Tufts, Jennifer B.; Molis, Michelle R.; Leek, Marjorie R.

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  6. The clinical characteristics and treatment for sudden sensorineural hearing loss with vestibular schwannoma.

    PubMed

    Lin, Chang; Gong, Qilin; Zuo, Wenjing; Zhang, Rong; Zhou, Aidong

    2015-04-01

    The aim of this study is to analyze the clinical characteristics and treatment of sudden sensorineural hearing loss (SSNHL) patients with vestibular schwannoma (VS). The clinical features of the VS patients were explored by retrospectively analyzing the clinical data from 542 cases of SSNHL patients between January 2008 and March 2013. There were 10 cases (10 ears) diagnosed with VS in 542 cases of SSNHL patients (10 ears, 1.85 %), 3 males, 7 females, with a range of 28-57 years. Among all the cases, eight patients with abnormal ABR, ten with ear ipsilateral stapedius reflexes which were completely not elicited and seven patients with healthy ear contralateral stapedius reflexes which were completely not elicited. Neuromas were classified by Koos grades according to size (8 of grade I, 1 of grade II, 1 of grade IV). Eight small VS  patients were taken waiting and MRI therapy strategies. Meanwhile, we used glucocorticoid treatment and timely and short-term medication to improve the microcirculation of the inner ear for these patients. And four cases' hearing was improved. Some vestibular schwannomas have SSNHL as initial symptoms, especially the small ones in internal auditory canal. To prevent misdiagnosis or leak-diagnosis, MRI should be performed as a routine test for SSNHL, and ABR is sometimes necessary for SSNHL patients. It is also necessary to give appropriate treatment to protect hearing of the small vestibular schwannoma patients whose first symptoms are diagnosed as SSNHL in acute phase.

  7. Repeated Acute Oral Exposure to Cannabis sativa Impaired Neurocognitive Behaviours and Cortico-hippocampal Architectonics in Wistar Rats.

    PubMed

    Imam, A; Ajao, M S; Akinola, O B; Ajibola, M I; Ibrahim, A; Amin, A; Abdulmajeed, W I; Lawal, Z A; Ali-Oluwafuyi, A

    2017-03-06

    The most abused illicit drug in both the developing and the developed world is Cannabis disposing users to varying forms of personality disorders. However, the effects of cannabis on cortico-hippocampal architecture and cognitive behaviours still remain elusive.  The present study investigated the neuro-cognitive implications of oral cannabis use in rats. Eighteen adult Wistar rats were randomly grouped to three. Saline was administered to the control rats, cannabis (20 mg/kg) to the experimental group I, while Scopolamine (1 mg/kg. ip) was administered to the last group as a standard measure for the cannabis induced cognitive impairment. All treatments lasted for seven consecutive days. Open Field Test (OFT) was used to assess locomotor activities, Elevated Plus Maze (EPM) for anxiety-like behaviour, and Y maze paradigm for spatial memory and data subjected to ANOVA and T test respectively. Thereafter, rats were sacrificed and brains removed for histopathological studies. Cannabis significantly reduced rearing frequencies in the OFT and EPM, and increased freezing period in the OFT. It also reduced percentage alternation similar to scopolamine in the Y maze, and these effects were coupled with alterations in the cortico-hippocampal neuronal architectures. These results point to the detrimental impacts of cannabis on cortico-hippocampal neuronal architecture and morphology, and consequently cognitive deficits.

  8. Selective TNF-α targeting with infliximab attenuates impaired oxygen metabolism and contractile function induced by an acute exposure to air particulate matter.

    PubMed

    Marchini, Timoteo; D'Annunzio, Verónica; Paz, Mariela L; Cáceres, Lourdes; Garcés, Mariana; Perez, Virginia; Tasat, Deborah; Vanasco, Virginia; Magnani, Natalia; Gonzalez Maglio, Daniel; Gelpi, Ricardo J; Alvarez, Silvia; Evelson, Pablo

    2015-11-15

    Inflammation plays a central role in the onset and progression of cardiovascular diseases associated with the exposure to air pollution particulate matter (PM). The aim of this work was to analyze the cardioprotective effect of selective TNF-α targeting with a blocking anti-TNF-α antibody (infliximab) in an in vivo mice model of acute exposure to residual oil fly ash (ROFA). Female Swiss mice received an intraperitoneal injection of infliximab (10 mg/kg body wt) or saline solution, and were intranasally instilled with a ROFA suspension (1 mg/kg body wt). Control animals were instilled with saline solution and handled in parallel. After 3 h, heart O2 consumption was assessed by high-resolution respirometry in left ventricle tissue cubes and isolated mitochondria, and ventricular contractile reserve and lusitropic reserve were evaluated according to the Langendorff technique. ROFA instillation induced a significant decrease in tissue O2 consumption and active mitochondrial respiration by 32 and 31%, respectively, compared with the control group. While ventricular contractile state and isovolumic relaxation were not altered in ROFA-exposed mice, impaired contractile reserve and lusitropic reserve were observed in this group. Infliximab pretreatment significantly attenuated the decrease in heart O2 consumption and prevented the decrease in ventricular contractile and lusitropic reserve in ROFA-exposed mice. Moreover, infliximab-pretreated ROFA-exposed mice showed conserved left ventricular developed pressure and cardiac O2 consumption in response to a β-adrenergic stimulus with isoproterenol. These results provides direct evidence linking systemic inflammation and altered cardiac function following an acute exposure to PM and contribute to the understanding of PM-associated cardiovascular morbidity and mortality.

  9. Low MMP-8/TIMP-1 reflects left ventricle impairment in takotsubo cardiomyopathy and high TIMP-1 may help to differentiate it from acute coronary syndrome

    PubMed Central

    Parkkonen, Olavi; Nieminen, Mikko T.; Vesterinen, Paula; Tervahartiala, Taina; Perola, Markus; Salomaa, Veikko; Jousilahti, Pekka; Sorsa, Timo; Pussinen, Pirkko J.; Sinisalo, Juha

    2017-01-01

    Background Matrix metalloproteinase 8 (MMP-8) is the most potent type-I collagen protease. Such collagen mainly constitutes the transient fibrosis in takotsubo cardiomyopathy (TTC) endomyocardial biopsies. High MMP-8 and tissue-inhibitor of matrix metalloproteinase-1 (TIMP-1) levels are implicated in acute coronary syndrome (ACS). We compared MMP-8 and TIMP-1 levels in consecutive TTC and ACS patients, and their association to TTC severity. Methods and results In 45 acute serum samples of TTC, 2072 ACS and 1000 controls, TIMP-1 differed between ACS 146.7ng/mL (115.0–186.3) (median (interquartile range)), TTC 115.7 (94.3–137.7) and controls 80.9 (73.2–90.4), (p<0.0001). MMP-8 levels were similar between ACS and TTC. In receiver-operating characteristics analysis, TIMP-1 differentiated TTC from ACS with an area under the curve (AUC) of 0.679 (p<0.0001) surpassing troponin T (TnT) at 0.522 (p = 0.66). Compared to other differing factors (age, sex, smoking), TIMP-1 improved diagnostic specificity and sensitivity from AUC of 0.821 to 0.844 (p = 0.007). The MMP8/TIMP-1 molar ratio differentiated normal ejection fraction (EF) at 0.27 (0.13–0.51) from decreased EF<50% at 0.08 (0.05–0.20), (p = 0.04) in TTC, but not in ACS. Conclusions Even with other differing factors considered, TIMP-1 differentiated TTC from ACS better than TnT. In TTC, the low MMP-8/TIMP-1 molar ratio may reflect decreased proteolysis and increased transient fibrosis, perhaps in part explaining the left-ventricle impairment. PMID:28278213

  10. Histamine deficiency exacerbates myocardial injury in acute myocardial infarction through impaired macrophage infiltration and increased cardiomyocyte apoptosis.

    PubMed

    Deng, Long; Hong, Tao; Lin, Jinyi; Ding, Suling; Huang, Zheyong; Chen, Jinmiao; Jia, Jianguo; Zou, Yunzeng; Wang, Timothy C; Yang, Xiangdong; Ge, Junbo

    2015-08-17

    Histamine is a biogenic amine that is widely distributed and has multiple functions, but the role it plays in acute myocardial infarction (AMI) remains unclear. In this study, we investigated the origin and contribution of endogenous histamine to AMI. Histidine decarboxylase (HDC) is the unique enzyme responsible for histamine generation. Using HDC-EGFP bacterial artificial chromosome (BAC) transgenic mice in which EGFP expression is controlled by the HDC promoter, we identified HDC expression primarily in CD11b(+)Gr-1(+) immature myeloid cells (IMCs) that markedly increase in the early stages of AMI. Deficiency of histamine in HDC knockout mice (HDC(-/-)) reduced cardiac function and exacerbated the injury of infarcted heart. Furthermore, administering either an H1 receptor antagonist (pyrilamine) or an H2 receptor antagonist (cimetidine) demonstrated a protective effect of histamine against myocardial injury. The results of in vivo and in vitro assays showed that histamine deficiency promotes the apoptosis of cardiomyocytes and inhibits macrophage infiltration. In conclusion, CD11b(+)Gr-1(+) IMCs are the predominant HDC-expressing sites in AMI, and histamine plays a protective role in the process of AMI through inhibition of cardiomyocyte apoptosis and facilitation of macrophage infiltration.

  11. von Frey anesthesiometry to assess sensory impairment after acute spinal cord injury caused by thoracolumbar intervertebral disc extrusion in dogs.

    PubMed

    Song, R B; Basso, D M; da Costa, R C; Fisher, L C; Mo, X; Moore, S A

    2016-03-01

    Sensory threshold (ST) was measured using an electric von Frey anesthesiometer (VFA) in all limbs of 20 normal dogs and 29 dogs with acute thoracolumbar spinal cord injury (SCI) caused by spontaneous intervertebral disc extrusion. ST values were measured at three separate time points in normal dogs and on days 3, 10 and 30 following decompressive surgery in dogs with SCI. ST values were compared between groups and correlated with locomotor recovery in SCI-affected dogs. ST values were significantly higher (consistent with hypoalgesia) in the pelvic limbs of SCI-affected dogs at day 3, day 10 and day 30 when compared to normal dogs (P <0.05), while no significant difference in thoracic limb ST values was observed between groups. A progressive decrease in pelvic limb ST values occurred in SCI-affected dogs over time, consistent with improvement toward normal sensation or development of allodynia. This finding correlated inversely with locomotor score at 3 and 10 days after surgery. A significant decline in ST values across testing sessions was observed for all limbs of normal and SCI-affected dogs and may be related to patient acclimation, operator training effect, or effect of analgesic medications. This study supports the feasibility of VFA to assess differences in ST between normal and SCI-affected dogs. However, future studies must focus on techniques to minimize or compensate for clinical, environmental and behavioral factors which may impact ST values in the clinical setting.

  12. Does Herpes Virus Reactivation Affect Prognosis in Idiopathic Sudden Sensorineural Hearing Loss?

    PubMed Central

    Park, Sang Man; Han, Chun; Lee, Jae Woo; Kong, Tae Hoon; Seo, Young Joon

    2017-01-01

    Objectives Several etiologies have been proposed to underlie idiopathic sudden sensorineural hearing loss (ISSNHL), including viral infection, vascular disturbance, and immune-mediated mechanisms. However, none of these mechanisms are conclusive. Should ISSNHL be caused by reactivation of a viral infection, antiviral treatment would be an important option. Thus, in this study, we reported the prognosis according to serologic test results of herpes viruses in patients with ISSNHL. We also evaluated treatment response with acyclovir and corticosteroids versus corticosteroids alone in herpes simplex virus (HSV) seropositive ISSNHL patients. Methods We compared hematologic examinations and the results of audiometry testing in 232 patients with ISSNHL. Statistical analyses for initial hearing impairment, progression of hearing impairment, recovery of hearing loss, and laboratory results were performed in all patients. All statistical analyses were performed using SPSS software. Results The 232 ISSNHL patients were divided into two subgroups according to HSV immunoglobulin M (IgM) serologic results (seropositive or seronegative). When the seropositive group was compared to the seronegative group, age, gender, body mass index (BMI), white blood cell, absolute neutrophil count, absolute monocyte count, and platelet count were not significantly different. Initial hearing level, final recovery hearing level, and recovery time were also not significantly different between the two groups (P>0.05). Though there were no significant differences in age, gender, BMI, or viral HSV IgM titer, the two groups had similar initial hearing level, final recovery hearing level, and recovery time. The difference in hearing threshold before and after treatment was larger (18.7±37.1) in the group receiving antiviral medication than in the corticosteroids group (11.0±44.5), but this difference was not significant (P=0.619). Conclusion There was no significant difference of prognosis between

  13. An Across-Frequency Processing Deficit in Listeners with Hearing Impairment Is Supported by Acoustic Correlation

    ERIC Educational Resources Information Center

    Healy, Eric W.; Kannabiran, Anand; Bacon, Sid P.

    2005-01-01

    It has been recently suggested that listeners having a sensorineural hearing impairment (HI) may possess a deficit in their ability to integrate speech information across different frequencies. When presented with a task that required across-frequency integration of speech patterns, listeners with HI performed more poorly than their normal-hearing…

  14. Acute insulin resistance in ST-segment elevation myocardial infarction in non-diabetic patients is associated with incomplete myocardial reperfusion and impaired coronary microcirculatory function

    PubMed Central

    2014-01-01

    acute phase of the first anterior STEMI in patients without diabetes treated by pPCI is independently associated with poorer myocardial reperfusion, impaired coronary microcirculatory function and potentially with larger final infarct size. PMID:24708817

  15. Characteristics and Spontaneous Recovery of Tinnitus Related to Idiopathic Sudden Sensorineural Hearing Loss

    PubMed Central

    Mühlmeier, Guido; Baguley, David; Cox, Tony; Suckfüll, Markus; Meyer, Thomas

    2016-01-01

    Objective: To evaluate the characteristics and spontaneous recovery of tinnitus related to idiopathic sudden sensorineural hearing loss (ISSNHL). Study Design: Retrospective analysis from two randomized placebo-controlled clinical trials for treatment of ISSNHL within 48 hours from onset (Study A), or of tinnitus related to ISSNHL within 3 months from onset (Study B). Setting: Forty-eight European sites (academic tertiary referral centers, private ENT practices). Patients: One hundred thirteen adult patients of which 65 with hearing loss ≥30 dB (Study A) and 48 with persistent acute tinnitus (Study B) at baseline. Interventions: Intratympanic (i.t.) injection of placebo gel in single dose or in triple dose during 3 consecutive days. Main Outcome Measures: Frequency of tinnitus, subjective tinnitus loudness, rates of complete tinnitus remission, and complete hearing recovery during 3 months follow-up. Results: In acute ISSNHL, tinnitus loudness decreased rapidly in cases of mild-moderate hearing loss, and tinnitus had completely resolved in two-thirds of patients after 3 months. Hearing recovery preceded tinnitus resolution. When associated with severe-profound hearing loss, tinnitus improved significantly less. Complete hearing recovery and full tinnitus remission were both about three times more frequent in mild-moderate hearing loss patients than in severe-profound cases. Improvement in tinnitus loudness over time can be approximated by a negative exponential function. Conclusions: Prognosis for ISSNHL-related tinnitus is relatively poor in case of severe-profound hearing loss and the longer it has persisted. Alleviation or management of tinnitus should be a key therapeutic objective especially in pronounced ISSNHL cases. PMID:27228021

  16. Real-time point-of-care measurement of impaired renal function in a rat acute injury model employing exogenous fluorescent tracer agents

    NASA Astrophysics Data System (ADS)

    Dorshow, Richard B.; Fitch, Richard M.; Galen, Karen P.; Wojdyla, Jolette K.; Poreddy, Amruta R.; Freskos, John N.; Rajagopalan, Raghavan; Shieh, Jeng-Jong; Demirjian, Sevag G.

    2013-02-01

    Renal function assessment is needed for the detection of acute kidney injury and chronic kidney disease. Glomerular filtration rate (GFR) is now widely accepted as the best indicator of renal function, and current clinical guidelines advocate its use in the staging of kidney disease. The optimum measure of GFR is by the use of exogenous tracer agents. However current clinically employed agents lack sensitivity or are cumbersome to use. An exogenous GFR fluorescent tracer agent, whose elimination rate could be monitored noninvasively through skin would provide a substantial improvement over currently available methods. We developed a series of novel aminopyrazine analogs for use as exogenous fluorescent GFR tracer agents that emit light in the visible region for monitoring GFR noninvasively over skin. In rats, these compounds are eliminated by the kidney with urine recovery greater than 90% of injected dose, are not broken down or metabolized in vivo, are not secreted by the renal tubules, and have clearance values similar to a GFR reference compound, iothalamate. In addition, biological half-life of these compounds measured in rats by noninvasive optical methods correlated with plasma derived methods. In this study, we show that this noninvasive methodology with our novel fluorescent tracer agents can detect impaired renal function. A 5/6th nephrectomy rat model is employed.

  17. Sensorineural hearing loss associated with a factitious disorder.

    PubMed

    Maruyama, Ayako; Noguchi, Yoshihiro; Ito, Taku; Narushima, Kenji; Kitamura, Ken

    2015-12-01

    Factitious disorders are characterized by intentionally abnormal physical and/or psychological behavior, and affected patients often make up their symptoms and clinical histories. The most serious and chronic type of factitious disorder is Munchausen syndrome. We report the case of a 24-year-old woman with a 2-year history of sensorineural hearing loss (SNHL) who later confessed to feigning her hearing loss. She was eventually diagnosed with a factitious disorder. During those 2 years, she was able to induce her SNHL by exposing herself to excessive noise or high doses of aspirin. To the best of our knowledge, this is the first report describing an association between a factitious disorder and SNHL.

  18. Dichotic listening in adults with sensorineural hearing loss.

    PubMed

    Roeser, R J; Johns, D F; Price, L L

    1976-01-01

    Digits and consonant-vowel (CV) nonsense syllables were presented dichotically to 36 normal-hearing subjects and 36 subjects with bilaterally symmetrical sensorineural hearing loss. The normal-hearing subjects performed significantly better in the recall of both digits and CV nonsense syllables, and recall decreased significantly as the degree of hearing loss increased. The expected right ear advantage was observed for the normal-hearing subjects on both tests. As a group, the hearing-loss subjects showed no significant ear preference, and ear preference did not vary significantly with the degree of hearing loss. However, individual hearing-loss subjects showed marked ear asymmetry for the dichotic digits. For both dichotic tests, individual ear preference increased as the degree of hearing loss increased. Significant but low correlations were observed between better ear speech discrimination scores for the hearing-loss subjects and the preferred ear for dichotic CV nonsense syllables.

  19. An Acute-phase Protein as a Regulator of Sperm Survival in the Bovine Oviduct: Alpha 1-acid-glycoprotein Impairs Neutrophil Phagocytosis of Sperm In Vitro

    PubMed Central

    LIU, Jinghui; MAREY, Mohamed A.; KOWSAR, Rasoul; HAMBRUCH, Nina; SHIMIZU, Takashi; HANEDA, Shingo; MATSUI, Motozumi; SASAKI, Motoki; HAYAKAWA, Hiroyuki; PFARRER, Christiane; MIYAMOTO, Akio

    2014-01-01

    We have previously shown that polymorphonuclear neutrophils (PMNs) are present in bovine oviduct fluid under physiological conditions, and that the oviduct provides a microenvironment that protects sperm from phagocytosis by PMNs. Alpha 1-acid glycoprotein (AGP) is a major acute-phase protein produced mainly in the liver that has immunomodulatory functions. AGP mRNA is expressed in extrahepatic organs, such as the lung, kidney, spleen, lymph node, uterus, and ovary. Therefore, in this study, we investigated, 1) the local production of AGP in the bovine oviduct, 2) the effect of AGP on the phagocytic activity of PMNs for sperm and superoxide production and 3) the impact of AGP desialylation on the PMN phagocytosis of sperm. The AGP gene was expressed in cultured bovine oviduct epithelial cells (BOECs) and AGP protein was detected in oviduct fluid. Preexposure of PMNs to AGP at physiological levels impaired PMN phagocytosis for sperm and superoxide generation. The desialylation of AGP eliminated these suppressive effects of AGP on PMN. Scanning electron microscopy revealed that AGP drastically reduced the formation of DNA-based neutrophil extracellular traps (NETs) for sperm entanglement. Additionally, AGP dose-dependently stimulated BOECs to produce prostaglandin E2 (PGE2) which has been shown to partially contribute to the regulation of sperm phagocytosis in the bovine oviduct. AGP and PGE2 at concentrations detected in the oviducts additively suppressed sperm phagocytosis by PMNs. These results provide evidence that locally produced AGP may be involved in protecting sperm from phagocytosis by PMNs in the bovine oviduct. PMID:24931131

  20. A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: Case Evidence Showcasing Treatment Efficacy.

    PubMed

    Formby, Craig; Sherlock, LaGuinn P; Hawley, Monica L; Gold, Susan L

    2017-02-01

    Case evidence is presented that highlights the clinical relevance and significance of a novel sound therapy-based treatment. This intervention has been shown to be efficacious in a randomized controlled trial for promoting expansion of the dynamic range for loudness and increased sound tolerance among persons with sensorineural hearing losses. Prior to treatment, these individuals were unable to use aided sound effectively because of their limited dynamic ranges. These promising treatment effects are shown in this article to be functionally significant, giving rise to improved speech understanding and enhanced hearing aid benefit and satisfaction, and, in turn, to enhanced quality of life posttreatment. These posttreatment sound therapy effects also are shown to be sustained, in whole or part, with aided environmental sound and to be dependent on specialized counseling to maximize treatment benefit. Importantly, the treatment appears to be efficacious for hearing-impaired persons with primary hyperacusis (i.e., abnormally reduced loudness discomfort levels [LDLs]) and for persons with loudness recruitment (i.e., LDLs within the typical range), which suggests the intervention should generalize across most individuals with reduced dynamic ranges owing to sensorineural hearing loss. An exception presented in this article is for a person describing the perceptual experience of pronounced loudness adaptation, which apparently rendered the sound therapy inaudible and ineffectual for this individual. Ultimately, these case examples showcase the enormous potential of a surprisingly simple sound therapy intervention, which has utility for virtually all audiologists to master and empower the adaptive plasticity of the auditory system to achieve remarkable treatment benefits for large numbers of individuals with sensorineural hearing losses.

  1. Association of TMTC2 With Human Nonsyndromic Sensorineural Hearing Loss

    PubMed Central

    Runge, Christina L.; Indap, Amit; Zhou, Yifan; Kent, Jack W.; King, Ericka; Erbe, Christy B.; Cole, Regina; Littrell, Jack; Merath, Kate; James, Roland; Rüschendorf, Franz; Kerschner, Joseph E.; Marth, Gabor; Hübner, Norbert; Göring, Harald H. H.; Friedland, David R.; Kwok, Wai-Meng; Olivier, Michael

    2016-01-01

    IMPORTANCE Sensorineural hearing loss (SNHL) is commonly caused by conditions that affect cochlear structures or the auditory nerve, and the genes identified as causing SNHL to date only explain a fraction of the overall genetic risk for this debilitating disorder. It is likely that other genes and mutations also cause SNHL. OBJECTIVE To identify a candidate gene that causes bilateral, symmetric, progressive SNHL in a large multigeneration family of Northern European descent. DESIGN, SETTING, AND PARTICIPANTS In this prospective genotype and phenotype study performed from January 1, 2006, through April 1, 2016, a 6-generation family of Northern European descent with 19 individuals having reported early-onset hearing loss suggestive of an autosomal dominant inheritance were studied at a tertiary academic medical center. In addition, 179 unrelated adult individuals with SNHL and 186 adult individuals reporting nondeafness were examined. MAIN OUTCOMES AND MEASURES Sensorineural hearing loss. RESULTS Nine family members (5 women [55.6%]) provided clinical audiometric and medical records that documented hearing loss. The hearing loss is characterized as bilateral, symmetric, progressive SNHL that reached severe to profound loss in childhood. Audiometric configurations demonstrated a characteristic dip at 1000 to 2000 Hz. All affected family members wear hearing aids or have undergone cochlear implantation. Exome sequencing and linkage and association analyses identified a fully penetrant sequence variant (rs35725509) on chromosome 12q21 (logarithm of odds, 3.3) in the TMTC2 gene region that segregates with SNHL in this family. This gene explains the SNHL occurrence in this family. The variant is also associated with SNHL in a cohort of 363 unrelated individuals (179 patients with confirmed SNHL and 184 controls, P = 7 × 10−4). CONCLUSIONS AND RELEVANCE A previously uncharacterized gene, TMTC2, has been identified as a candidate for causing progressive SNHL in humans

  2. Changes in the default mode networks of individuals with long-term unilateral sensorineural hearing loss.

    PubMed

    Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Chen, H; Zhang, P-P; Li, J; Chen, J-Y; Liu, L-J; Wang, J; Teng, G-J

    2015-01-29

    Hearing impairment contributes to cognitive dysfunction. Previous studies have found changes of functional connectivity in the default mode network (DMN) associated with cognitive processing in individuals with sensorineural hearing loss (SNHL). Whereas the changes in the DMN in patients with long-term unilateral SNHL (USNHL) is still not entirely clear. In this work, we analyzed resting-state functional magnetic resonance imaging (fMRI) data and neuropsychological test scores from normal hearing subjects (n = 11) and patients (n = 21) with long-term USNHL. Functional connectivity and nodal topological properties were computed for every brain region in the DMN. Analysis of covariance (ANCOVA) and post hoc analyses were conducted to identify differences between normal controls and patients for each measure. Results indicated that the left USNHL presented enhanced connectivity (p < 0.05, false discovery rate (FDR) corrected), and significant changes (p < 0.05, Bonferroni corrected) of the nodal topological properties in the DMN compared with the control. More changes in the DMN have been found in the left than right long-term USNHL (RUSNHL). However, the neuropsychological tests did not show significant differences between the USNHL and the control. These findings suggest that long-term USNHL contributes to changes in the DMN, and these changes might affect cognitive abilities in patients with long-term USNHL. Left hearing loss affects the DMN more than the right hearing loss does. The fMRI measures might be more sensitive for observing cognitive changes in patients with hearing loss than clinical neuropsychological tests. This study provides some insights into the mechanisms of the association between hearing loss and cognitive function.

  3. The significance of routine laboratory parameters in patients with sudden sensorineural hearing loss.

    PubMed

    Yasan, Hasan; Tüz, Mustafa; Yariktaş, Murat; Aynali, Giray; Tomruk, Onder; Akkuş, Omer

    2013-12-01

    There are several factors (viral infections, metabolic and ototoxic disorders etc.) accused for the development of sudden sensorineural hearing loss. Some prognostic factors (late onset of treatment etc.) had been evaluated in the literature. There is no sufficient data on the effect of routine laboratory parameters on the development and/or prognosis of sudden sensorineural hearing loss. The aim of this study is to investigate the effects of routine blood chemistry and hematological parameters on the development and prognosis of disease in patients with idiopathic sudden sensorineural hearing loss. One hundred and forty-seven patients with the diagnosis of idiopathic sudden sensorineural hearing loss followed up during the periods of 2000-2010 years were included in this study. One hundred and three septoplasty patients with no otologic complaints were enrolled as control group. Following the clinical and demographic evaluations, patients with idiopathic sudden sensorineural hearing loss and control groups, and patients treated successfully and patients with poor outcome were compared with each other. Data were analyzed by T test. All hematological and biochemical parameters were compared. Hemoglobin, hematocrit, white blood cell count, total and direct bilirubin, fasting blood glucose level and aspartate aminotransferase were significantly different between idiopathic sudden sensorineural hearing loss and control groups. There was no significantly different parameter between patients treated successfully and patients with poor outcome. Hemoglobin, hematocrit, white blood cell count, total and direct bilirubin, fasting blood glucose level and AST all can be risk factors for SHL, or they can be the result of undetermined pathology, because these parameters have no effect on the prognosis. Other routine parameters seem to have no effect on the development and/or prognosis of idiopathic sudden sensorineural hearing loss.

  4. Idiopathic sensorineural hearing loss in the only hearing ear.

    PubMed

    Berrettini, S; De Vito, A; Bruschini, L; Fortunato, S; Forli, F

    2016-04-01

    A retrospective chart review was used for 31 patients with sudden, progressive or fluctuating sensorineural hearing loss (SHL) in the only hearing ear who had been consecutively evaluated at the ENT, Audiology and Phoniatrics Unit of the University of Pisa. The group of patients was evaluated with a complete history review, clinical evaluation, imaging exam (MRI, CT), audiologic tests (tone and speech audiometry, tympanometry, study of stapedial reflexes, ABR and otoacoustic emission) evaluation. In order to exclude genetic causes, patients were screened for CX 26 and CX30 mutations and for mitochondrial DNA mutation A1555G. Patients with sudden or rapidly progressive SHL in the only hearing ear were treated with osmotic diuretics and corticosteroids. In patients who did not respond to intravenous therapy we performed intratympanic injections of corticosteroid. Hearing aids were fitted when indicated and patients who developed severe to profound SHL were scheduled for cochlear implant surgery. The aim of this study is to report and discuss the epidemiology, aetiopathogenesis, therapy and clinical characteristic of patients affected by SHL in the only hearing hear and to discuss the issues related to the cochlear implant procedure in some of these patients, with regard to indications, choice of the ear to implant and results.

  5. Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation

    PubMed Central

    Che, Jing; Li, Qiang; Chai, Chao; Zheng, Meizhu; Shen, Wen

    2017-01-01

    Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL) and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF) and regional homogeneity (ReHo) of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45) was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age. PMID:28255465

  6. The novel c.247_249delTTC (p.F83del) GJB2 mutation in a family with prelingual sensorineural deafness.

    PubMed

    Petersen, Michael B; Grigoriadou, Maria; Koutroumpe, Maria; Kokotas, Haris

    2012-07-01

    Non-syndromic hearing loss is one of the most common hereditary determined diseases in human, and the disease is a genetically heterogeneous disorder. Mutations in the GJB2 gene, encoding connexin 26 (Cx26), are a major cause of non-syndromic recessive hearing impairment in many countries and are largely dependent on ethnic groups. Due to the high frequency of the c.35delG GJB2 mutation in the Greek population, we have previously suggested that Greek patients with sensorineural, non-syndromic deafness should be tested for the c.35delG mutation and the coding region of the GJB2 gene should be sequenced in c.35delG heterozygotes. Here we present on the clinical and molecular genetic evaluation of a family suffering from prelingual, sensorineural, non-syndromic deafness. A novel c.247_249delTTC (p.F83del) GJB2 mutation was detected in compound heterozygosity with the c.35delG GJB2 mutation in the proband and was later confirmed in the father, while the mother was homozygous for the c.35delG GJB2 mutation. We conclude that compound heterozygosity of the novel c.247_249delTTC (p.F83del) and the c.35delG mutations in the GJB2 gene was the cause of deafness in the proband and his father.

  7. Effects of pulmonary static inflation with 50% xenon on oxygen impairment during cardiopulmonary bypass for stanford type A acute aortic dissection

    PubMed Central

    Jin, Mu; Yang, Yanwei; Pan, Xudong; Lu, Jiakai; Zhang, Zhiquan; Cheng, Weiping

    2017-01-01

    Abstract Background: The goal of this study was to investigate the effects of pulmonary static inflation with 50% xenon on postoperative oxygen impairment during cardiopulmonary bypass (CPB) for Stanford type A acute aortic dissection (AAD). Methods: This prospective single-center nonrandomized controlled clinical trial included 100 adult patients undergoing surgery for Stanford type A AAD at an academic hospital in China. Fifty subjects underwent pulmonary static inflation with 50% oxygen from January 2013 to January 2014, and 50 underwent inflation with 50% xenon from January 2014 to December 2014. During CPB, the lungs were inflated with either 50% xenon (xenon group) or 50% oxygen (control group) to maintain an airway pressure of 5 cm H2O. The primary outcome was oxygenation index (OI) value after intubation, and 10 minutes and 6 hours after the operation. The second outcome was cytokine and reactive oxygen species levels after intubation and 10 minutes, 6 hours, and 24 hours after the operation. Results: Patients treated with xenon had lower OI levels compared to the control group before surgery (P = 0.002); however, there was no difference in postoperative values between the 2 groups. Following surgery, mean maximal OI values decreased by 18.8% and 33.8%, respectively, in the xenon and control groups. After surgery, the levels of interleukin-6 (IL-6), tumor necrosis factor alpha, and thromboxane B2 decreased by 23.5%, 9.1%, and 30.2%, respectively, in the xenon group, but increased by 10.8%, 26.2%, and 26.4%, respectively, in the control group. Moreover, IL-10 levels increased by 28% in the xenon group and decreased by 7.5% in the control group. There were significant time and treatment-time interaction effects on methane dicarboxylic aldehyde (P = 0.000 and P = 0.050, respectively) and myeloperoxidase (P = 0.000 and P = 0.001 in xenon and control groups, respectively). There was no difference in hospital mortality and 1-year

  8. Visual Impairment

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Visual Impairment KidsHealth > For Teens > Visual Impairment Print A ... with the brain, making vision impossible. What Is Visual Impairment? Many people have some type of visual ...

  9. [The very severe sensorineural deafness patients caused by rubella virus infection: two cases report].

    PubMed

    Ma, Jing; Wan, Lang; Xu, Fen

    2015-09-01

    To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.

  10. Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss

    PubMed Central

    Li, Feng-Jiao; Wang, Da-Yong; Wang, Hong-Yang; Wang, Li; Yang, Feng-Bo; Lan, Lan; Guan, Jing; Yin, Zi-Fang; Rosenhall, Ulf; Yu, Lan; Hellstrom, Sten; Xue, Xi-Jun; Duan, Mao-Li; Wang, Qiu-Ju

    2016-01-01

    Background: The prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL. Methods: One hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors. Results: Among the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies. Conclusions: CSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators

  11. Phosphodiesterase 4D gene polymorphisms in sudden sensorineural hearing loss.

    PubMed

    Chien, Chen-Yu; Tai, Shu-Yu; Wang, Ling-Feng; Hsi, Edward; Chang, Ning-Chia; Wang, Hsun-Mo; Wu, Ming-Tsang; Ho, Kuen-Yao

    2016-09-01

    The phosphodiesterase 4D (PDE4D) gene has been reported as a risk gene for ischemic stroke. The vascular factors are between the hypothesized etiologies of sudden sensorineural hearing loss (SSNHL), and this genetic effect might be attributed for its role in SSNHL. We hypothesized that genetic variants of the PDE4D gene are associated with susceptibility to SSNHL. We conducted a case-control study with 362 SSNHL cases and 209 controls. Three single nucleotide polymorphisms (SNPs) were selected. The genotypes were determined using TaqMan technology. Hardy-Weinberg equilibrium (HWE) was tested for each SNP, and genetic effects were evaluated according to three inheritance modes. We carried out sex-specific analysis to analyze the overall data. All three SNPs were in HWE. When subjects were stratified by sex, the genetic effect was only evident in females but not in males. The TT genotype of rs702553 exhibited an adjusted odds ratio (OR) of 3.83 (95 % confidence interval = 1.46-11.18) (p = 0.006) in female SSNHL. The TT genotype of SNP rs702553 was associated with female SSNHL under the recessive model (p = 0.004, OR 3.70). In multivariate logistic regression analysis, TT genotype of rs702553 was significantly associated with female SSNHL (p = 0.0043, OR 3.70). These results suggest that PDE4D gene polymorphisms influence the susceptibility for the development of SSNHL in the southern Taiwanese female population.

  12. Risk of sudden sensorineural hearing loss in stroke patients

    PubMed Central

    Kuo, Chin-Lung; Shiao, An-Suey; Wang, Shuu-Jiun; Chang, Wei-Pin; Lin, Yung-Yang

    2016-01-01

    Abstract Poststroke sudden sensorineural hearing loss (SSNHL) can hinder communication between patients and healthcare professionals, thereby restricting participation in rehabilitation programs and limiting improvements in physical performance. However, the relationship between stroke and SSNHL remains unclear. This study employed a nationwide population-based dataset to investigate the relationship between stroke and SSNHL. The Taiwan Longitudinal Health Insurance Database was used to compile data from 11,115 stroke patients and a comparison cohort of 33,345 matched nonstroke enrollees. Each patient was followed for 5 years to identify new-onset SSNHL. Stratified Cox proportional-hazard regression analysis was used to examine the association of stroke with subsequent SSNHL. Among the 44,460 patients, 66 patients (55,378 person-years) from the stroke cohort and 105 patients (166,586 person-years) from the comparison cohort were diagnosed with SSNHL. The incidence of SSNHL was approximately twice as high among stroke patients than among nonstroke patients (1.19 and 0.63/1000 person-years, respectively). Stroke patients had a 71% increased risk of SSNHL, compared with nonstroke patients (adjusted hazard ratio [HR] 1.71, 95% confidence interval [CI] 1.24–2.36). We also observed a remarkable increase in risk of SSNHL in stroke patients within 1-year of follow-up (adjusted HR 5.65, 95% CI 3.07–10.41) or under steroid therapy during hospitalization (adjusted HR 5.14, 95% CI 2.08–12.75). Patients with stroke had a higher risk of subsequent SSNHL compared with patients without stroke. In particular, stroke patients within 1-year follow-up and those undergoing steroid therapy during hospitalization should be treated with the utmost caution, considering that the risk of SSNHL increases by more than 5-fold. PMID:27603402

  13. Novel association of achalasia with hereditary sensory and motor neuropathy with sensorineural deafness.

    PubMed

    Asthana, A K; Lubel, J S; Kohn, G P

    2016-08-01

    Achalasia is a primary esophageal motility disorder. Unlike diffuse esophageal spasm, it has not previously been described in association with hereditary sensory and motor neuropathy (HSMN). An 18-year-old-male with HSMN with sensorineural deafness presented with a 2-day history of dysphagia to solids and liquids. Achalasia was diagnosed after extensive investigations, and his symptoms resolved with endoscopic and definitive surgical management. His monozygotic twin brother had also been diagnosed with HSMN and suffered from chronic dysphagia, which was also subsequently diagnosed with achalasia. This is the first case to illustrate an association between HSMN with sensorineural deafness and achalasia.

  14. Sudden Sensorineural Hearing Loss after Orthopedic Surgery under Combined Spinal and Epidural Anesthesia

    PubMed Central

    Vilhena, Ditza; Pereira, Luís; Duarte, Delfim; Oliveira, Nuno

    2016-01-01

    Postoperative hearing loss following nonotologic surgery is rare. For patients undergoing subarachnoid anesthesia, the loss of cerebral spinal fluid and hence the drop in intracranial pressure can result in hearing loss and cranial nerve palsy. We report a case in which a patient sustained orthopedic surgery under combined spinal and epidural anesthesia complicated by severe and persistent sensorineural hearing loss. This report is a reminder that postoperative sudden sensorineural hearing loss is a poorly understood complication. A high index of suspicion is required for early diagnosis of this complication, although prompt treatment does not guarantee a good outcome. PMID:26904339

  15. The effect of sensorineural hearing loss and tinnitus on speech recognition over air and bone conduction military communications headsets.

    PubMed

    Manning, Candice; Mermagen, Timothy; Scharine, Angelique

    2016-10-27

    Military personnel are at risk for hearing loss due to noise exposure during deployment (USACHPPM, 2008). Despite mandated use of hearing protection, hearing loss and tinnitus are prevalent due to reluctance to use hearing protection. Bone conduction headsets can offer good speech intelligibility for normal hearing (NH) listeners while allowing the ears to remain open in quiet environments and the use of hearing protection when needed. Those who suffer from tinnitus, the experience of perceiving a sound not produced by an external source, often show degraded speech recognition; however, it is unclear whether this is a result of decreased hearing sensitivity or increased distractibility (Moon et al., 2015). It has been suggested that the vibratory stimulation of a bone conduction headset might ameliorate the effects of tinnitus on speech perception; however, there is currently no research to support or refute this claim (Hoare et al., 2014). Speech recognition of words presented over air conduction and bone conduction headsets was measured for three groups of listeners: NH, sensorineural hearing impaired, and/or tinnitus sufferers. Three levels of speech-to-noise (SNR = 0, -6, -12 dB) were created by embedding speech items in pink noise. Better speech recognition performance was observed with the bone conduction headset regardless of hearing profile, and speech intelligibility was a function of SNR. Discussion will include study limitations and the implications of these findings for those serving in the military.

  16. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome).

    PubMed

    Desir, Julie; Abramowicz, Marc

    2008-10-15

    Harboyan syndrome is a degenerative corneal disorder defined as congenital hereditary endothelial dystrophy (CHED) accompanied by progressive, postlingual sensorineural hearing loss. To date, 24 cases from 11 families of various origin (Asian Indian, South American Indian, Sephardi Jewish, Brazilian Portuguese, Dutch, Gypsy, Moroccan, Dominican) have been reported. More than 50% of the reported cases have been associated with parental consanguinity. The ocular manifestations in Harboyan syndrome include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. They are apparent at birth or within the neonatal period and are indistinguishable from those characteristic of the autosomal recessive CHED (CHED2). Hearing deficit in Harboyan is slowly progressive and typically found in patients 10-25 years old. There are no reported cases with prelinglual deafness, however, a significant hearing loss in children as young as 4 years old has been detected by audiometry, suggesting that hearing may be affected earlier, even at birth. Harboyan syndrome is caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13-p12, indicating that CHED2 and Harboyan syndrome are allelic disorders. A total of 62 different SLC4A11 mutations have been reported in 98 families (92 CHED2 and 6 Harboyan). All reported cases have been consistent with autosomal recessive transmission. Diagnosis is based on clinical criteria, detailed ophthalmological assessment and audiometry. A molecular confirmation of the clinical diagnosis is feasible. A variety of genetic, metabolic, developmental and acquired diseases presenting with clouding of the cornea should be considered in the differential diagnosis (Peters anomaly, sclerocornea, limbal dermoids, congenital glaucoma). Audiometry must be performed to differentiate Harboyan syndrome from CHED2. Autosomal recessive types of CHED (CHED2 and Harboyan syndrome) should

  17. Profound Bilateral Sensorineural Hearing Loss in Nigerian Children: Any Shift in Etiology?

    ERIC Educational Resources Information Center

    Dunmade, A. D.; Segun-Busari, S.; Olajide, T. G.; Ologe, F. E.

    2007-01-01

    Deafness, profound hearing loss, is a global problem. However, the causes of, attitudes toward, and management options for deafness differ considerably from region to region. This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence…

  18. Prognostic effect of hyperbaric oxygen therapy starting time for sudden sensorineural hearing loss.

    PubMed

    Yıldırım, Erol; Murat Özcan, K; Palalı, Mehmet; Cetin, Mehmet Ali; Ensari, Serdar; Dere, Hüseyin

    2015-01-01

    Sudden sensorineural hearing loss is one of the otological emergencies whose pathogenesis is uncertain and associated with total or partial loss of hearing function. The aim of this study was to investigate whether the hyperbaric oxygen therapy starting time affects the management of sudden sensorineural hearing loss. Fifty-nine patients with sudden sensorineural hearing loss admitted to our clinic between 2008 and 2012 were retrospectively included in this study. All patients received hyperbaric oxygen therapy. In addition, each patient received intravenous piracetam and 37 patients received steroid therapy. Hyperbaric oxygen therapy was initiated between 1 and 7 days with 20 patients determined as Group A, between 8 and 14 days with 25 patients determined as Group B and between 15 and 28 days with 14 patients determined as Group C. Hearing gains of these three groups were statistically evaluated. Each of them showed statistically significant improvement. Lowest hearing gain was observed in Group C and the gain of this group was statistically less than the other two groups. There was no significant difference between the hearing gains of the Group A and Group B. Starting hyperbaric oxygen therapy in patients with sudden sensorineural hearing loss within the first 14 days has positive effect on the prognosis of the disease.

  19. Can Mild Bilateral Sensorineural Hearing Loss Affect Developmental Abilities in Younger School-Age Children?

    ERIC Educational Resources Information Center

    Ðokovic, Sanja; Gligorovic, Milica; Ostojic, Sanja; Dimic, Nadežda; Radic-Šestic, Marina; Slavnic, Svetlana

    2014-01-01

    The research study was conducted for the purpose of examining the influence of mild bilateral sensorineural hearing loss (MBSNHL) on developmental abilities of younger school-age children. The sample encompassed 144 children with MBSNHL, aged 7.5-11 (M = 8.85). MBSNHL (20-40 dB HL) was identified by pure tone audiometry. The control group…

  20. Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

    PubMed Central

    Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2013-01-01

    Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing. PMID:25992037

  1. Neurodevelopmental Disorders in Children with Severe to Profound Sensorineural Hearing Loss: A Clinical Study

    ERIC Educational Resources Information Center

    Chilosi, Anna M.; Comparini, Alessandro; Scusa, Maria F.; Berrettini, Stefano; Forli, Francesca; Battini, Roberta; Cipriani, Paola; Cioni, Giovanni

    2010-01-01

    Aim: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation…

  2. Eye color as a risk factor for acquired sensorineural hearing loss: a review.

    PubMed

    Mujica-Mota, Mario A; Schermbrucker, Jonah; Daniel, Sam J

    2015-02-01

    Eye color may be an indicator of inner ear melanin content and has been associated with hearing loss. There is controversy as to whether eye color has an effect on acquired causes of sensorineural hearing loss. This review was conducted to analyze the literature evaluating the relationship between eye color and causes of sensorineural hearing loss. Six databases were searched to identify eligible studies. Included articles were independently assessed for quality by two authors. Eighteen articles were eligible for review. Eye color was not found to have an effect in the non-exposed population or in presbycusis. In noise-induced sensorineural hearing loss, light-eyed patients had more significant loss following noise exposure, although the variability reported due to eye color was modest (r(2) = 0.01-0.14). Two out of three studies reported that dark eye color is associated with cisplatin ototoxicity. In one study, green-eyed patients were found to be at higher risk of radiation-induced hearing loss. Eye color does not appear to play a role in hearing loss in non-exposed individuals or presbycusis. It is possible that dark-eyed individuals, with greater inner ear melanin content, are better protected against noise-induced hearing loss. Evidence suggests that melanin can be protective against radiation-induced sensorineural hearing loss, but may predispose individuals to cisplatin ototoxicity. Future studies are required to support these conclusions.

  3. Some Non-Occupational Aspects of Sensorineural Loss of Hearing Acuity - A Proposed Design for Study.

    DTIC Science & Technology

    1985-06-01

    last twelve months only one person was retired from the U.S. Air Force solely due to hearing loss ( Caraway , Per- sonal interview, 1985). Sensorineural...Loss in Teenage Boys, Scandinavian Audiological Journal 1981; 10 (2): 91-6. Caraway , Rebecca, Captain, USAF, Personal Interview, Division of Medical

  4. Inflectional morphology in German hearing-impaired children.

    PubMed

    Penke, Martina; Wimmer, Eva; Hennies, Johannes; Hess, Markus; Rothweiler, Monika

    2016-01-01

    Despite modern hearing aids, children with hearing impairment often have only restricted access to spoken language input during the 'critical' years for language acquisition. Specifically, a sensorineural hearing impairment affects the perception of voiceless coronal consonants which realize verbal affixes in German. The aim of this study is to explore if German hearing-impaired children have problems in producing and/or acquiring inflectional suffixes expressed by such phonemes. The findings of two experiments (an elicitation task and a picture-naming task) conducted with a group of hearing-impaired monolingual German children (age 3-4 years) demonstrate that difficulties in perceiving specific phonemes relate to the avoidance of these same sounds in speech production independent of the grammatical function these phonemes have.

  5. [The active middle ear implant for the rehabilitation of sensorineural, mixed and conductive hearing losses].

    PubMed

    Sprinzl, G M; Wolf-Magele, A; Schnabl, J; Koci, V

    2011-09-01

    Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

  6. Transient impairment of hippocampus-dependent learning and memory in relatively low-dose of acute radiation syndrome is associated with inhibition of hippocampal neurogenesis.

    PubMed

    Kim, Joong-Sun; Lee, Hae-June; Kim, Jong Choon; Kang, Seong Soo; Bae, Chun-Sik; Shin, Taekyun; Jin, Jae-Kwang; Kim, Sung Ho; Wang, Hongbing; Moon, Changjong

    2008-09-01

    Neurogenesis in the adult hippocampus, which occurs constitutively, is vulnerable to ionizing radiation. In the relatively low-dose exposure of acute radiation syndrome (ARS), the change in the adult hippocampal function is poorly understood. This study analyzed the changes in apoptotic cell death and neurogenesis in the DGs of hippocampi from adult ICR mice with single whole-body gamma-irradiation using the TUNEL method and immunohistochemical markers of neurogenesis, Ki-67 and doublecortin (DCX). In addition, the hippocampus-dependent learning and memory tasks after single whole-body gamma-irradiation were examined in order to evaluate the hippocampus-related behavioral dysfunction in the relatively low-dose exposure of ARS. The number of TUNEL-positive apoptotic nuclei in the dentate gyrus (DG) was increased 6-12 h after acute gamma-irradiation (a single dose of 0.5 to 4 Gy). In contrast, the number of Ki-67- and DCX-positive cells began to decrease significantly 6 h postirradiation, reaching its lowest level 24 h after irradiation. The level of Ki-67 and DCX immunoreactivity decreased in a dose-dependent manner within the range of irradiation applied (0-4 Gy). In passive avoidance and object recognition memory test, the mice trained 1 day after acute irradiation (2 Gy) showed significant memory deficits, compared with the sham controls. In conclusion, the pattern of the hippocampus-dependent memory dysfunction is consistent with the change in neurogenesis after acute irradiation. It is suggested that a relatively low dose of ARS in adult ICR mice is sufficiently detrimental to interrupt the functioning of the hippocampus, including learning and memory, possibly through the inhibition of neurogenesis.

  7. Chronic ethanol ingestion impairs alveolar type II cell glutathione homeostasis and function and predisposes to endotoxin-mediated acute edematous lung injury in rats.

    PubMed Central

    Holguin, F; Moss, I; Brown, L A; Guidot, D M

    1998-01-01

    Chronic alcohol abuse increases the incidence and mortality of the acute respiratory distress syndrome (ARDS) in septic patients. To examine a potential mechanism, we hypothesized that ethanol ingestion predisposes to sepsis-mediated acute lung injury by decreasing alveolar type II cell glutathione homeostasis and function. Lungs isolated from rats fed ethanol (20% in water for >/= 3 wk), compared with lungs from control-fed rats, had greater (P < 0. 05) edematous injury (reflected by nonhydrostatic weight gain) after endotoxin (2 mg/kg intraperitoneally) and subsequent perfusion ex vivo with n-formylmethionylleucylphenylalanine (fMLP, 10(-7) M). Ethanol ingestion decreased (P < 0.05) glutathione levels in the plasma, lung tissue, and lung lavage fluid, and increased (P < 0.05) oxidized glutathione levels in the lung lavage fluid. Furthermore, ethanol ingestion decreased type II cell glutathione content by 95% (P < 0.05), decreased (P < 0.05) type II cell surfactant synthesis and secretion, and decreased (P < 0.05) type II cell viability, in vitro. Finally, treatment with the glutathione precursors S-adenosyl-L-methionine and N-acetylcysteine in the final week of ethanol ingestion significantly reduced lung edema during perfusion ex vivo. We conclude that ethanol ingestion in rats alters alveolar type II cell glutathione levels and function, thereby predisposing the lung to acute edematous injury after endotoxemia. We speculate that chronic alcohol abuse in humans predisposes to ARDS through similar mechanisms. PMID:9466970

  8. Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis.

    PubMed

    Kuhn, Maggie; Heman-Ackah, Selena E; Shaikh, Jamil A; Roehm, Pamela C

    2011-09-01

    Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram.

  9. Muckle-Wells syndrome: a treatable cause of congenital sensorineural hearing loss.

    PubMed

    Stew, B T; Fishpool, S J C; Owens, D; Quine, S

    2013-01-01

    Muckle-Wells syndrome (MWS) is a rare autosomal dominant condition with variable expression. It is a subset of auto-inflammatory diseases characterised by recurrent inflammatory crises and is associated with chronic recurrent urticaria, sensorineural deafness, periodic arthritis and secondary amyloidosis. The diagnosis of MWS is a clinical one with sufferers classically presenting in childhood with a moderate fever and non-pruiginous urticaria. We describe a case of a six-year-old girl who was successfully diagnosed and treated with Anakinra. Muckle and Wells originally described this syndrome in 1962; however, only recently was it discovered to be genetically linked to chromosome 1q44 and subsequently to missense mutations in the CIAS1/NALP3/PYPAF1 gene. Since then, treatment has evolved and it remains one of few treatable causes of congenital profound sensorineural hearing loss.

  10. Congenital sensorineural deafness in dogs: a molecular genetic approach toward unravelling the responsible genes.

    PubMed

    Rak, Simone G; Distl, Ottmar

    2005-03-01

    Deafness is often diagnosed in different dog breeds and has been identified as a significant problem for breeders, owners and clinicians. The aetiology can be inherited or acquired, and a distinction must be made between sensorineural and conductive forms of deafness. This paper provides a brief overview of the varieties of findings in different dog breeds and in one breed in particular including prevalence, phenotypic and gender associations, histology, modes of inheritance and the number of contributing genes in congenital sensorineural deafness. We have also described molecular genetic approaches to canine hearing loss and discuss how comparative genomics could help reduce the prevalence of deafness in affected breeds leading to new insights into the molecular mechanisms of auditory function in both dogs and humans.

  11. Effects of stimulus material on the dichotic listening performance of patients with sensorineural hearing loss.

    PubMed

    Speaks, C; Niccum, N; Van Tasell, D

    1985-03-01

    Dichotic listening scores were obtained from 27 patients with sensorineural hearing loss in response to four dichotic speech tests: digits, vowel words, consonant words, and CV nonsense syllables. Monotic performance-intensity functions for each ear were defined with the CV syllables, and the four dichotic tests were administered at an intensity that produced asymptotic monotic performance for both ears with the CV syllables. Four blocks of 30 pairs of items per block were presented for each dichotic test, which produced 240 responses per test for each patient. Reliable differences among left-ear scores, right-ear scores, performance level, and the ear advantage were observed among the four tests. The digit test appeared to be most promising for assessing central auditory function when the patient had a sensorineural hearing loss because performance for the digits was only slightly affected by the peripheral loss.

  12. Inhibition of endoplasmic reticulum-resident glucosidases impairs severe acute respiratory syndrome coronavirus and human coronavirus NL63 spike protein-mediated entry by altering the glycan processing of angiotensin I-converting enzyme 2.

    PubMed

    Zhao, Xuesen; Guo, Fang; Comunale, Mary Ann; Mehta, Anand; Sehgal, Mohit; Jain, Pooja; Cuconati, Andrea; Lin, Hanxin; Block, Timothy M; Chang, Jinhong; Guo, Ju-Tao

    2015-01-01

    Endoplasmic reticulum (ER)-resident glucosidases I and II sequentially trim the three terminal glucose moieties on the N-linked glycans attached to nascent glycoproteins. These reactions are the first steps of N-linked glycan processing and are essential for proper folding and function of many glycoproteins. Because most of the viral envelope glycoproteins contain N-linked glycans, inhibition of ER glucosidases with derivatives of 1-deoxynojirimycin, i.e., iminosugars, efficiently disrupts the morphogenesis of a broad spectrum of enveloped viruses. However, like viral envelope proteins, the cellular receptors of many viruses are also glycoproteins. It is therefore possible that inhibition of ER glucosidases not only compromises virion production but also disrupts expression and function of viral receptors and thus inhibits virus entry into host cells. Indeed, we demonstrate here that iminosugar treatment altered the N-linked glycan structure of angiotensin I-converting enzyme 2 (ACE2), which did not affect its expression on the cell surface or its binding of the severe acute respiratory syndrome coronavirus (SARS-CoV) spike glycoprotein. However, alteration of N-linked glycans of ACE2 impaired its ability to support the transduction of SARS-CoV and human coronavirus NL63 (HCoV-NL63) spike glycoprotein-pseudotyped lentiviral particles by disruption of the viral envelope protein-triggered membrane fusion. Hence, in addition to reducing the production of infectious virions, inhibition of ER glucosidases also impairs the entry of selected viruses via a post-receptor-binding mechanism.

  13. Rosuvastatin versus placebo for delirium in intensive care and subsequent cognitive impairment in patients with sepsis-associated acute respiratory distress syndrome: an ancillary study to a randomised controlled trial

    PubMed Central

    Needham, Dale M; Colantuoni, Elizabeth; Dinglas, Victor D; Hough, Catherine L; Wozniak, Amy W; Jackson, James C; Morris, Peter E; Mendez-Tellez, Pedro A; Ely, E Wesley; Hopkins, Ramona O

    2016-01-01

    Summary Background Delirium is common in mechanically ventilated patients and is associated with cognitive impairment lasting at least 1 year after hospital discharge. Preclinical and observational studies suggest that the use of statins might reduce delirium in intensive care. We assessed whether the pleiotropic effects of statins can reduce delirium in intensive care and decrease subsequent cognitive impairment in a randomised controlled trial. Methods We did this ancillary study within the SAILS trial, a randomised controlled trial assessing mortality and ventilator-free days for rosuvastatin versus placebo for patients with sepsis-associated acute respiratory distress syndrome. This study was done at 35 hospitals in the USA. Patients were randomly assigned in permuted blocks of eight and stratified by hospital to receive either rosuvastatin (40 mg loading dose and then 20 mg daily until the earliest of 3 days after discharge from intensive care, study day 28, or death) or placebo. Patients and investigators were masked to treatment assignment. Delirium was assessed with the validated Confusion Assessment Method for intensive care. Cognitive function was assessed with tests for executive function, language, verbal reasoning and concept formation, and working, immediate, and delayed memory. We defined cognitive impairment as having one of these domains at least two SDs below population norms or at least two domains at least 1·5 SDs below norms. The primary endpoint was daily delirium status in intensive care up to 28 days in the intention-to-treat population and secondary endpoints were cognitive function at 6 months and 12 months. This trial is registered with ClinicalTrials.gov (NCT00979121 and NCT00719446). Findings 272 patients were assessed for delirium daily in intensive care. The mean proportion of days with delirium was 34% (SD 30%) in the rosuvastatin group versus 31% (29%) in the placebo group; hazard ratio 1·14, 95% CI 0·92–1·41, p=0·22. At 6

  14. Allan-Herndon-Dudley syndrome with unusual profound sensorineural hearing loss.

    PubMed

    Gagliardi, Lucia; Nataren, Nathalie; Feng, Jinghua; Schreiber, Andreas W; Hahn, Christopher N; Conwell, Louise S; Coman, David; Scott, Hamish S

    2015-08-01

    The Allan-Herndon-Dudley syndrome is caused by mutations in the thyroid hormone transporter, Monocarboxylate transporter 8 (MCT8). It is characterized by profound intellectual disability and abnormal thyroid function. We report on a patient with Allan-Herndon-Dudley syndrome (AHDS) with profound sensorineural hearing loss which is not usually a feature of AHDS and which may have been due to a coexisting nonsense mutation in Microphthalmia-associated transcription factor (MITF).

  15. Intratympanic steroid therapy for treatment of idiopathic sudden sensorineural hearing loss.

    PubMed

    Bear, Zachary W; Mikulec, Anthony A

    2014-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is the sudden loss of unilateral hearing of unknown etiology. The standard treatment consist of a high dose oral steroid taper. This article serves to review the current literature on intratympanic steroid injections for ISSHL. Current literature suggested intratympanic steroids are equivalent to oral steroid therapy, primary combined therapy is superior to either alone and intratympanic steroids should be offered for salvage therapy in ISSHL.

  16. Sudden Sensorineural Hearing Loss in the Only Hearing Ear: Large Vestibular Aqueduct Syndrome

    PubMed Central

    Bal, Kemal Koray; Bucioglu, Helen; Vayısoğlu, Yusuf; Gorur, Kemal

    2016-01-01

    Sudden hearing loss in the only hearing ear cases are rarely published in the English literature; most of the cases are idiopathic. It is an otologic emergency needing urgent treatment. Delayed diagnosis can interfere with patient's social life with interrupting the verbal communication. In this case report we presented a 33-year-old female patient having sudden sensorineural hearing loss in the only hearing ear diagnosed as bilateral large vestibular aqueduct syndrome. PMID:28018692

  17. Alteration of frequency range for binaural beats in acute low-tone hearing loss.

    PubMed

    Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

    2005-01-01

    The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

  18. Gain of cellular adaptation due to prolonged p53 impairment leads to functional switchover from p53 to p73 during DNA damage in acute myeloid leukemia cells.

    PubMed

    Chakraborty, Juni; Banerjee, Shuvomoy; Ray, Pallab; Hossain, Dewan Md Sakib; Bhattacharyya, Sankar; Adhikary, Arghya; Chattopadhyay, Sreya; Das, Tanya; Sa, Gaurisankar

    2010-10-22

    Tumor suppressor p53 plays the central role in regulating apoptosis in response to genotoxic stress. From an evolutionary perspective, the activity of p53 has to be backed up by other protein(s) in case of any functional impairment of this protein, to trigger DNA damage-induced apoptosis in cancer cells. We adopted multiple experimental approaches to demonstrate that in p53-impaired cancer cells, DNA damage caused accumulation of p53 paralogue p73 via Chk-1 that strongly impacted Bax expression and p53-independent apoptosis. On the contrary, when p53 function was restored by ectopic expression, Chk-2 induced p53 accumulation that in turn overshadowed p73 activity, suggesting an antagonistic interaction between p53 family members. To understand such interaction better, p53-expressing cells were impaired differentially for p53 activity. In wild-type p53-expressing cancer cells that were silenced for p53 for several generations, p73 was activated, whereas no such trend was observed when p53 was transiently silenced. Prolonged p53 interference, even in functional p53 settings, therefore, leads to the "gain of cellular adaptation" in a way that alters the cellular microenvironment in favor of p73 activation by altering p73-regulatory proteins, e.g. Chk1 activation and dominant negative p73 down-regulation. These findings not only unveil a hitherto unexplained mechanism underlying the functional switchover from p53 to p73, but also validate p73 as a promising and potential target for cancer therapy in the absence of functional p53.

  19. Intratympanic injection of dexamethasone for treatment of tinnitus in patients with sudden sensorineural hearing loss.

    PubMed

    Yoshida, Tadao; Teranishi, Masaaki; Iwata, Tomoyuki; Otake, Hironao; Nakashima, Tsutomu

    2012-01-09

    The purpose of this study is to test the effectiveness of intratympanic dexamethasone injections as a treatment for severe tinnitus in idiopathic sudden sensorineural hearing loss (SNHL). We studied 37 patients who received intratympanic dexamethasone injections and 14 control patients who did not receive it, with severe tinnitus after onset of unilateral sudden SNHL. Hearing level did not change during this study in any patient. The relationship between the duration of tinnitus and effectiveness of treatment was investigated in sudden SNHL. We used a visual analogue scale to evaluate 51 patients with severe tinnitus at the stage of stable hearing level after idiopathic sudden sensorineural hearing loss. Forty-one per cent of patients showed significant improvement after treatment. The average period between onset of sudden sensorineural hearing loss and initiation of intratympanic dexamethasone injection was significantly shorter (207 days) in the improved group than in the unchanged group (482 days) (P<0.001). In control group, one of 14 patients presented significant improvement spontaneously. Intratympanic dexamethasone treatment may be effective in treatment of severe tinnitus after sudden SNHL at the stage of stable hearing level, and the shorter the period from onset of sudden deafness to the start of intratympanic dexamethasone treatment, the greater the improvement in tinnitus that can be expected.

  20. Impaired expression of DICER and some microRNAs in HBZ expressing cells from acute adult T-cell leukemia patients

    PubMed Central

    Gazon, Hélène; Belrose, Gildas; Terol, Marie; Meniane, Jean-Come; Mesnard, Jean-Michel; Césaire, Raymond; Peloponese, Jean-Marie

    2016-01-01

    Global dysregulation of microRNAs (miRNAs), a class of non-coding RNAs that regulate genes expression, is a common feature of human tumors. Profiling of cellular miRNAs on Adult T cell Leukemia (ATL) cells by Yamagishi et al. showed a strong decrease in expression for 96.7% of cellular miRNAs in ATL cells. However, the mechanisms that regulate the expression of miRNAs in ATL cells are still largely unknown. In this study, we compared the expression of 12 miRs previously described for being overexpress by Tax and the expression of several key components of the miRNAs biogenesis pathways in different HBZ expressing cell lines as well as in primary CD4 (+) cells from acute ATL patients. We showed that the expression of miRNAs and Dicer1 were downregulated in cells lines expressing HBZ as well as in fresh CD4 (+) cells from acute ATL patients. Using qRT-PCR, western blotting analysis and Chromatin Immunoprecipitation, we showed that dicer transcription was regulated by c-Jun and JunD, two AP-1 transcription factors. We also demonstrated that HBZ affects the expression of Dicer by removing JunD from the proximal promoter. Furthermore, we showed that at therapeutic concentration of 1mM, Valproate (VPA) an HDAC inhibitors often used in cancer treatment, rescue Dicer expression and miRNAs maturation. These results might offer a rationale for clinical studies of new combined therapy in an effort to improve the outcome of patients with acute ATL. PMID:26849145

  1. Acute and impaired wound healing: pathophysiology and current methods for drug delivery, part 1: normal and chronic wounds: biology, causes, and approaches to care.

    PubMed

    Demidova-Rice, Tatiana N; Hamblin, Michael R; Herman, Ira M

    2012-07-01

    This is the first installment of 2 articles that discuss the biology and pathophysiology of wound healing, review the role that growth factors play in this process, and describe current ways of growth factor delivery into the wound bed. Part 1 discusses the latest advances in clinicians' understanding of the control points that regulate wound healing. Importantly, biological similarities and differences between acute and chronic wounds are considered, including the signaling pathways that initiate cellular and tissue responses after injury, which may be impeded during chronic wound healing.

  2. PP2A impaired activity is a common event in acute myeloid leukemia and its activation by forskolin has a potent anti-leukemic effect.

    PubMed

    Cristóbal, I; Garcia-Orti, L; Cirauqui, C; Alonso, M M; Calasanz, M J; Odero, M D

    2011-04-01

    Protein phosphatase 2A (PP2A) is a human tumor suppressor that inhibits cellular transformation by regulating the activity of several signaling proteins critical for malignant cell behavior. PP2A has been described as a potential therapeutic target in chronic myeloid leukemia, Philadelphia chromosome-positive acute lymphoblastic leukemia and B-cell chronic lymphocytic leukemia. Here, we show that PP2A inactivation is a recurrent event in acute myeloid leukemia (AML), and that restoration of PP2A phosphatase activity by treatment with forskolin in AML cells blocks proliferation, induces caspase-dependent apoptosis and affects AKT and ERK1/2 activity. Moreover, treatment with forskolin had an additive effect with Idarubicin and Ara-c, drugs used in standard induction therapy in AML patients. Analysis at protein level of the PP2A activation status in a series of patients with AML at diagnosis showed PP2A hyperphosphorylation in 78% of cases (29/37). In addition, we found that either deregulated expression of the endogenous PP2A inhibitors SET or CIP2A, overexpression of SETBP1, or downregulation of some PP2A subunits, might be contributing to PP2A inhibition in AML. In conclusion, our results show that PP2A inhibition is a common event in AML cells and that PP2A activators, such as forskolin or FTY720, could represent potential novel therapeutic targets in AML.

  3. Traumatic Labyrinthine Concussion in a Patient with Sensorineural Hearing Loss

    PubMed Central

    Chiaramonte, R.; Bonfiglio, M.; D'Amore, A.; Viglianesi, A.; Cavallaro, T.; Chiaramonte, I.

    2013-01-01

    Blunt head trauma without any temporal bone fracture or longitudinal temporal bone fracture, with an associated fracture of the labyrinth may cause labyrinthine injury with sensor neural hearing loss and vertigo because of a concussive injury to the membranous labyrinth. Sudden sensory neural hearing loss is relatively frequent. In most cases, the etiology is not discovered. One of the possible causes for sudden deafness is inner labyrinth bleeding or concussion, which were difficult to diagnose before the advent of magnetic resonance imaging. Vertigo without a demonstrable fracture may also be the result of labyrinthine concussion, cupololithiasis and perilymphatic fistula. We describe the clinical case of a patient with acute traumatic hearing loss and vertigo, without skull base fracture detected on computed tomography. Magnetic resonance study was also performed. We have integrated the discussion with features that allow the differential diagnosis from other similar conditions. PMID:23859168

  4. Corticosteroid treatment of idiopathic sudden sensorineural hearing loss: analysis of an RCT and material drawn from the Swedish national database.

    PubMed

    Hultcrantz, Elisabeth; Nosrati-Zarenoe, Ramesh

    2015-11-01

    A randomized placebo-controlled study has demonstrated no effect of prednisolone in customary dosage on idiopathic sudden sensorineural hearing loss (ISSNHL). The aim of the present paper is to analyse a larger patient group by meta-analysis of data from the RCT together with a corresponding material drawn from the Swedish national database for ISSNHL. Data from 192 patients, 18-80 years with ISSNHL, were available. All had an acute hearing loss of at least 30 dB measured as PTA in the three most affected contiguous frequencies. All patients had been enrolled within one week after onset and evaluated by audiograms after 3 months. 45/99 (RCT) and 54/99 (the database) had been treated with prednisolone in tapering doses from 60 mg daily and 42/93 with placebo (RCT) or 51/93 with no treatment (the database). Primary outcome was the mean hearing improvement on day 90 for the different groups. A mean difference of >10 dB improvement was required to demonstrate a treatment effect for prednisolone compared to placebo/no treatment. No significant difference was seen between the prednisolone group and placebo/no treatment (p = 0.06). Total recovery was 38% in prednisolone group, 40% in the placebo and 14% in the no treatment group. Vertigo at the onset of hearing loss and age at onset had an equal negative prognostic value in all groups and signs of inflammation had a positive effect. Prednisolone in customary dosage does not influence recovery after ISSNHL.

  5. Two large French pedigrees with non syndromic sensorineural deafness and the mitochondrial DNA T7511C mutation: evidence for a modulatory factor.

    PubMed

    Chapiro, Elise; Feldmann, Delphine; Denoyelle, Françoise; Sternberg, Damien; Jardel, Claude; Eliot, Marie-Madeleine; Bouccara, Didier; Weil, Dominique; Garabédian, Eréa-Noel; Couderc, Rémy; Petit, Christine; Marlin, Sandrine

    2002-12-01

    Hearing impairment is the most frequent sensory defect in children, with a genetic basis in about 50% of cases. Several point mutations and deletions in mitochondrial DNA (mtDNA) have been identified in non-syndromic sensorineural hearing loss (NSSNHL). Beside the frequent A1555G mutation, a number of mutations in tRNAs have been reported recently, but their incidence remains unknown. We identified the T7511C mutation in the tRNASer(UCN) gene in two French families with isolated deafness. Maternal transmission was obvious in both. The 15 patients with hearing impairment exhibited a variable disease phenotype in terms of onset, severity, and progression. T7511C was present in all the patients screened. Homoplasmic and heteroplasmic levels were observed and did not correlate with the severity of the disease. T7511C was also present in 12 hearing offspring of the oldest deaf mothers, confirming the existence of modulatory factors. Our data suggest that this mtDNA mutation should be screened for in all cases of familial NSSNHL compatible with maternal transmission.

  6. [The problems of hearing impairment in the flying staff of commercial aviation in Russia].

    PubMed

    Pankova, V B; Bushmanov, A Y U

    2014-01-01

    The authors discuss the problems pertaining to the growing incidence of hearing impairment in the members of the flying staff employed in commercial aviation of Russia and the main criteria used to elucidate the causes behind occupational diseases of the organs of hearing. Special attention is given to the principal normative documents regulating the methodological basis on which the acoustic factor in the aircraft cockpit is evaluated, peculiarities of occupational sensorineural hearing impairment and the methods for its detection. The main errors in the determination of the relationship between the working conditions and the diseases of the organs of hearing are discussed.

  7. Hearing impairment in Stickler syndrome: a systematic review

    PubMed Central

    2012-01-01

    Background Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. Methods English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. Results 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). Conclusions Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired. PMID:23110709

  8. [Non-syndromic hereditary hearing impairment].

    PubMed

    Birkenhäger, R; Aschendorff, A; Schipper, J; Laszig, R

    2007-04-01

    Hearing impairment is the most common sensorineural disorder in humans. Approximately one of thousand new-borns is affected by severe to profound deafness at birth or during early childhood. Genetic causes account for around half of these cases of prelingual hearing impairment and the remainder are attributed to environmental factors. Genetic causes of hearing impairment in combination with a syndrome as Usher, Pendred are distinguished from non-syndromic hearing impairment. In the last years a tremendous growth in the localisation and identification of genes for non-syndromic hereditary hearing impairment has evolved. It has become clear that these conditions are genetically extremely heterogeneous. Approximately 120 different gene loci associated with non syndromic hearing impairment have been identified. Presently 54 gene loci associated with autosomal dominant mode of inheritance and 67 gene loci with autosomal recessive mode of inheritance have been identified; 7 are X-chromosome linked and 4 mitochondrial. Of these, 19 genes have been characterised for autosomal dominant (DFNA), 20 for autosomal recessive (DFNB), and 2 for X-linked (DFN) disorders. These genes encode proteins of diverse functions, including transcription factors, cytoskeletal and extracellular matrix components, and ion channels. Despite this heterogeneity, up to 50 % of prelingual recessive non-syndromic deafness can be attributed to mutations in the GJB2 gene (Connexin-26, gap-junction protein). However, the diversity of genes and genetic loci implicated in hearing loss illustrates the complexity of the genetic basis of hearing. Knowing the gene and the function of its products helps understanding the mechanisms of hearing.

  9. Impaired Driving

    MedlinePlus

    Impaired driving is dangerous. It's the cause of more than half of all car crashes. It means operating a ... texting Having a medical condition which affects your driving For your safety and the safety of others, ...

  10. Taste - impaired

    MedlinePlus

    ... longer. Causes of impaired taste include: Bell's palsy Common cold Flu and other viral infections Nasal infection, nasal ... your diet. For taste problems due to the common cold or flu, normal taste should return when the ...

  11. Enzymatic evidence of impaired reperfusion in diabetic patients after thrombolytic therapy for acute myocardial infarction: a role for plasminogen activator inhibitor?

    PubMed Central

    Gray, R P; Yudkin, J S; Patterson, D L

    1993-01-01

    OBJECTIVE--To compare the activity of plasminogen activator inhibitor (PAI-1) in diabetic and non-diabetic patients admitted with acute myocardial infarction and to determine whether PAI-1 activity influences reperfusion after thrombolytic therapy. DESIGN--Prospective study of patients admitted with acute myocardial infarction. SETTING--District general hospital. MAIN OUTCOME MEASURES--Reperfusion assessed by time to peak release of creatine kinase-MB isoenzyme. RESULTS--Baseline PAI-1 activity and antigen concentrations were significantly higher in diabetic patients (n = 45) than in non-diabetic patients (n = 110) (24.6 (6.9) v 18.6 (7.9) AU/ml (AU = arbitrary units) (p = 0.0001) and 58.8 (13.1-328.8) v 41.0 (10.9-125.4) ng/ml (p = 0.004). Time to peak release of creatine kinase-MB was calculated in 123 (80%) patients. In 98 who received thrombolytic therapy the median time to peak enzyme release was 15.5 h (7.5-24 h) in diabetic patients (n = 26) and 12 h (5-26 h) in non-diabetic patients (n = 72) (p = 0.005). In those with a time to peak release of < or = 12 h, indicating likely successful reperfusion, PAI-1 activity was 17.5 (7.8) AU/ml compared with 22.8 (7.7) AU/ml in those with a time to peak release of > 12 h (p = 0.001). In multiple regression analysis both diabetes (p = 0.0001) and PAI-1 activity at admission (p = 0.029) were independently related to successful reperfusion. In 13 patients with evidence of reinfarction in hospital PAI-1 activity on day 3 was 26.7 (6.4) AU/ml compared with 21.7 (6.3) AU/ml in those without evidence of reinfarction (p = 0.032). CONCLUSION--Both raised PAI-1 activity on admission and diabetes were associated with a reduced likelihood of enzymatic evidence of reperfusion after thrombolytic therapy. Increased PAI-1 activity on day 3 was associated with an increased risk of reinfarction. Diabetic patients had higher PAI-1 activity on admission. This may partly explain their reduced likelihood of reperfusion. PMID:8280517

  12. How porphyrinogenic drugs modeling acute porphyria impair the hormonal status that regulates glucose metabolism. Their relevance in the onset of this disease.

    PubMed

    Matkovic, Laura B; D'Andrea, Florencia; Fornes, Daiana; San Martín de Viale, Leonor C; Mazzetti, Marta B

    2011-11-28

    This work deals with the study of how porphyrinogenic drugs modeling acute porphyrias interfere with the status of carbohydrate-regulating hormones in relation to key glucose enzymes and to porphyria, considering that glucose modulates the development of the disease. Female Wistar rats were treated with 2-allyl-2-isopropylacetamide (AIA) and 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) using different doses of AIA (100, 250 and 500mg/kg body weight) and a single dose of DDC (50mg DDC/kg body weight). Rats were sacrificed 16h after AIA/DDC administration. In the group treated with the highest dose of AIA (group H), hepatic 5-aminolevulinic acid synthase (ALA-S) increased more than 300%, phosphoenolpyruvate carboxykinase (PEPCK) and glycogen phosphorylase (GP) activities were 43% and 46% lower than the controls, respectively, plasmatic insulin levels exceeded normal values by 617%, and plasmatic glucocorticoids (GC) decreased 20%. GC results are related to a decrease in corticosterone (CORT) adrenal production (33%) and a significant reduction in its metabolization by UDP-glucuronosyltransferase (UGT) (62%). Adrenocorticotropic hormone (ACTH) stimulated adrenal production 3-fold and drugs did not alter this process. Thus, porphyria-inducing drugs AIA and DDC dramatically altered the status of hormones that regulate carbohydrate metabolism increasing insulin levels and reducing GC production, metabolization and plasmatic levels. In this acute porphyria model, gluconeogenic and glycogenolytic blockages caused by PEPCK and GP depressed activities, respectively, would be mainly a consequence of the negative regulatory action of insulin on these enzymes. GC could also contribute to PEPCK blockage both because they were depressed by the treatment and because they are positive effectors on PEPCK. These disturbances in carbohydrates and their regulation, through ALA-S de-repression, would enhance the porphyria state promoted by the drugs on heme synthesis and destruction

  13. Timbre discrimination in normal-hearing and hearing-impaired listeners under different noise conditions.

    PubMed

    Emiroglu, Suzan; Kollmeier, Birger

    2008-07-18

    In an attempt to quantify differences in object separation and timbre discrimination between normal-hearing and hearing-impaired listeners with a moderate sensorineural hearing loss of two different configurations, psychoacoustic measurements were performed with a total of 50 listeners. The experiments determined just noticeable differences (JND) of timbre in normal-hearing and hearing-impaired subjects along continua of "morphed" musical instruments and investigated the variance of JND in silence and different background noise conditions and on different sound levels. The results show that timbre JNDs of subjects with a steep hearing loss are significantly higher than of normal-hearing subjects, both in silence and noise, whereas timbre JNDs of flat/diagonal hearing-impaired subjects are similar to JNDs of normal-hearing subjects for signal levels above 55 dB (plus appropriate amplification for hearing-impaired). In noise (SNR=+10 dB) timbre JNDs of all subject groups are significantly higher than in silence. In the condition testing, transferability from silence to noise (i.e., the ability to imagine how the stimulus heard in silence would sound in noise), no significant JND differences across listener groups were found. The results can be explained by primary factors involved in sensorineural hearing loss and contradict the hypothesis that hearing-impaired people generally have more problems in object discrimination than normal-hearing people.

  14. Hearing Instruments for Unilateral Severe-to-Profound Sensorineural Hearing Loss in Adults: A Systematic Review and Meta-Analysis

    PubMed Central

    Smith, Sandra Nelson; Lucas, Laura

    2016-01-01

    Objectives: A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe to profound sensorineural hearing loss. Design: The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL, and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until February 11, 2015. Studies were included that (a) assessed the impact of any form of hearing instrument, including devices that reroute signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe to profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device with placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively defined criteria were subjected to random effects meta-analyses. Results: Twenty-seven studies reported in 30 articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically significant benefits to speech perception in noise for devices that rerouted the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit, 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit, 3.1 dB) when noise was rerouted to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localization also precluded the synthesis of evidence across studies. Evidence for

  15. Detrimental ELAVL-1/HuR-dependent GSK3β mRNA stabilization impairs resolution in acute respiratory distress syndrome

    PubMed Central

    Hoffman, Olivia; Burns, Nana; Vadász, István; Eltzschig, Holger K.; Edwards, Michael G.

    2017-01-01

    A hallmark of acute respiratory distress syndrome (ARDS) is accumulation of protein-rich edema in the distal airspaces and its removal is critical for patient survival. Previous studies have shown a detrimental role of Glycogen Synthase Kinase (GSK) 3β during ARDS via inhibition of alveolar epithelial protein transport. We hypothesized that post-transcriptional regulation of GSK3β could play a functional role in ARDS resolution. To address this hypothesis, we performed an in silico analysis to identify regulatory genes whose expression correlation to GSK3β messenger RNA utilizing two lung cancer cell line array datasets. Among potential regulatory partners of GSK3β, these studies identified the RNA-binding protein ELAVL-1/HuR (Embryonic Lethal, Abnormal Vision, Drosophila-Like) as a central component in a likely GSK3β signaling network. ELAVL-1/HuR is a RNA-binding protein that selectively binds to AU-rich elements of mRNA and enhances its stability thereby increasing target gene expression. Subsequent studies with siRNA suppression of ELAVL-1/HuR demonstrated deceased GSK3β mRNA and protein expression and improved clearance of FITC-albumin in A549 cells. Conversely, stabilization of ELAVL-1/HuR with the proteasome inhibitor MG-132 resulted in induction of GSK3β at mRNA and protein level and attenuated FITC-albumin clearance. Utilizing ventilator-induced lung injury or intra-tracheal installation of hydrochloric acid to induce ARDS in mice, we observed increased mRNA and protein expression of ELAVL-1/HuR and GSK3β. Together, our findings indicate a previously unknown interaction between GSK3β and ELAV-1 during ARDS, and suggest the inhibition of the ELAV-1- GSK3β pathways as a novel ARDS treatment approach. PMID:28196122

  16. Detrimental ELAVL-1/HuR-dependent GSK3β mRNA stabilization impairs resolution in acute respiratory distress syndrome.

    PubMed

    Hoffman, Olivia; Burns, Nana; Vadász, István; Eltzschig, Holger K; Edwards, Michael G; Vohwinkel, Christine U

    2017-01-01

    A hallmark of acute respiratory distress syndrome (ARDS) is accumulation of protein-rich edema in the distal airspaces and its removal is critical for patient survival. Previous studies have shown a detrimental role of Glycogen Synthase Kinase (GSK) 3β during ARDS via inhibition of alveolar epithelial protein transport. We hypothesized that post-transcriptional regulation of GSK3β could play a functional role in ARDS resolution. To address this hypothesis, we performed an in silico analysis to identify regulatory genes whose expression correlation to GSK3β messenger RNA utilizing two lung cancer cell line array datasets. Among potential regulatory partners of GSK3β, these studies identified the RNA-binding protein ELAVL-1/HuR (Embryonic Lethal, Abnormal Vision, Drosophila-Like) as a central component in a likely GSK3β signaling network. ELAVL-1/HuR is a RNA-binding protein that selectively binds to AU-rich elements of mRNA and enhances its stability thereby increasing target gene expression. Subsequent studies with siRNA suppression of ELAVL-1/HuR demonstrated deceased GSK3β mRNA and protein expression and improved clearance of FITC-albumin in A549 cells. Conversely, stabilization of ELAVL-1/HuR with the proteasome inhibitor MG-132 resulted in induction of GSK3β at mRNA and protein level and attenuated FITC-albumin clearance. Utilizing ventilator-induced lung injury or intra-tracheal installation of hydrochloric acid to induce ARDS in mice, we observed increased mRNA and protein expression of ELAVL-1/HuR and GSK3β. Together, our findings indicate a previously unknown interaction between GSK3β and ELAV-1 during ARDS, and suggest the inhibition of the ELAV-1- GSK3β pathways as a novel ARDS treatment approach.

  17. Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit

    PubMed Central

    Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

    2016-01-01

    We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas. PMID:27975074

  18. Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

    PubMed

    Grahn, Anna; Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

    2016-10-01

    We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

  19. [The revised version of the german guidelines "sudden idiopathic sensorineural hearing loss"].

    PubMed

    Michel, O

    2011-05-01

    The new revised version (expires 2012) for sudden, unilateral, sensorineural hearing loss stresses the urgence but not the emergency of diagnostics and therapy in this inner ear disfunction with still increasing incidence. Minimum diagnostics should comprise ENT examination with earmicroscopy, pure tone and tuning fork hearing tests, tympano- and vestibulometry. Classification by frequency loss seems of interest in regard to different underlying pathology, which is still obscure. Therefore from experience glucocorticoids and rheological therapy are recommended in the light of some favoring recent studies. The intratympanic appliCation of glucokorticoids is considered as spare therapy.

  20. [Barotherapy and interval hypoxic training in the treatment of sensorineural deafness].

    PubMed

    Troll', V G; Vishniakov, V V

    2009-01-01

    Barotherapy or normoxic therapeutic compression is a novel technique based on the exposure of the patients to an air and oxygen mixture in combination with antioxidants under slightly elevated pressure in the hyperbaric chamber. The efficiency of this method was compared with that of different therapeutic modalities in a group of 105 patients suffering sensorineural deafness. Barotherapy was given to 30 patients, 28 were offered interval hypoxic training, and 47 comprised the control group. Results of the study were used to develop criteria for the selection of patients to be treated with barotherapy and interval hypoxic training.

  1. A case of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

    PubMed

    Hong, Young-Ho; Mun, Seog-Kyun

    2011-10-01

    The reasons behind sudden sensorineural hearing loss are mostly unknown, but viral infections, blood disorders, ototoxicity, noise trauma, autoimmune disease, acoustic tumor, and even mental stress may be related to the disease. In cases of hearing loss as a result of psychogenic factors, early diagnosis and adequate treatment under collaboration with the psychiatric department are crucial, since failure to take appropriate measures may result in permanent sequela. We report a case, with a review of the literature, of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

  2. Niikawa-Kuroki (Kabuki) syndrome with congenital sensorineural deafness: evidence for a wide spectrum of inner ear abnormalities.

    PubMed

    Tekin, Mustafa; Fitoz, Suat; Arici, Serap; Cetinkaya, Ergun; Incesulu, Armagan

    2006-05-01

    Hearing loss, mainly due to recurrent otitis media, has been reported in approximately 40% of individuals with Niikawa-Kuroki (Kabuki) syndrome (NKS). Sensorineural hearing loss leading to congenital or prelingual deafness has been described rarely. We have identified two unrelated individuals with Niikawa-Kuroki syndrome among 535 probands who have severe to profound sensorineural deafness. Bilateral absence of the cochlea with dilated dysplastic vestibule and unilateral enlarged vestibule were demonstrated in these two individuals. In conclusion, Niikawa-Kuroki syndrome should be kept in mind when evaluating an individual with congenital deafness and a wide spectrum of inner ear abnormalities occurs in this syndrome.

  3. Hereditary gingival fibromatosis and sensorineural hearing loss in a 42-year-old man with Jones syndrome.

    PubMed

    Kasaboğlu, O; Tümer, C; Balci, S

    2004-01-01

    Hereditary gingival fibromatosis and sensorineural hearing loss in a 42-year-old man with Jones syndrome: Gingival fibromatosis is a rare disease, which can be seen as an isolated condition or associated with some uncommon syndromes. This case report describes the evaluation and treatment of a 42-year-old male patient with hereditary gingival fibromatosis, sensorineural hearing loss, undescended testis and maxillary odontogenic cyst (Jones Syndrome). Six years follow up of the index patient after the surgery revealed no recurrence of the gingival fibromatosis. This report also describes the anamnestic data of the patient's family that showed progressive deafness and gingival enlargement in three generations.

  4. Impairment of the cellular immune response in acute murine toxoplasmosis: regulation of interleukin 2 production and macrophage-mediated inhibitory effects.

    PubMed Central

    Haque, S; Khan, I; Haque, A; Kasper, L

    1994-01-01

    infected and normal splenocytes. These results indicate that during acute murine toxoplasmosis, there is a well-defined period (day 7) during which both the T-cell mitogen and parasite antigen-associated lymphoproliferative response are reduced. Further, there is a reduction in the production of IL-2 and an increase in IL-10, which appear to mediate, in part, the observed downregulation of immunity to T. gondii. PMID:8005679

  5. Physical Impairment

    NASA Astrophysics Data System (ADS)

    Trewin, Shari

    Many health conditions can lead to physical impairments that impact computer and Web access. Musculoskeletal conditions such as arthritis and cumulative trauma disorders can make movement stiff and painful. Movement disorders such as tremor, Parkinsonism and dystonia affect the ability to control movement, or to prevent unwanted movements. Often, the same underlying health condition also has sensory or cognitive effects. People with dexterity impairments may use a standard keyboard and mouse, or any of a wide range of alternative input mechanisms. Examples are given of the diverse ways that specific dexterity impairments and input mechanisms affect the fundamental actions of Web browsing. As the Web becomes increasingly sophisticated, and physically demanding, new access features at the Web browser and page level will be necessary.

  6. All Vision Impairment

    MedlinePlus

    ... Home > Statistics and Data > All Vision Impairment All Vision Impairment Vision Impairment Defined Vision impairment is defined as the ... Ethnicity 2010 U.S. Age-Specific Prevalence Rates for Vision Impairment by Age and Race/Ethnicity Table for ...

  7. Role of Platelet Parameters on Sudden Sensorineural Hearing Loss: A Case-Control Study in Iran

    PubMed Central

    2016-01-01

    Sudden sensorineural hearing loss (SSNHL) is a common otological disorder characterized by a hearing loss greater than 30 dB over three consecutive frequencies, in less than 72 hours. It has been established that platelet parameters, such as mean platelet volume, are associated with ischemic heart events, whose clinical manifestations are similar to those of SSNHL. Hence, we aimed to determine if the platelet count, mean platelet volume and platelet distribution width are related to the occurrence and severity of sudden sensorineural hearing loss. A case-control prospective study was conducted in a teaching hospital in Iran. One hundred-eight patients with SSNHL and an equal number of healthy, age- and sex-matched controls were enrolled in the study. Peripheral venous blood samples were collected from the subjects, and the platelet count, mean platelet volume and platelet distribution width were measured with an automated blood cell counter. Analysis of the audiometry and hematological test results using SPSS22 software showed no statistical correlation between the platelet parameters and the occurrence of SSNHL, but correlation coefficients showed a significant correlation between PDW and hearing loss severity in patients group. However, further investigation is required to unequivocally establish the absence of correlation between the platelet parameters and occurrence of SSNHL. PMID:26829393

  8. Homozygous mutation in PTRH2 gene causes progressive sensorineural deafness and peripheral neuropathy.

    PubMed

    Sharkia, Rajech; Shalev, Stavit A; Zalan, Abdelnaser; Marom-David, Milit; Watemberg, Nathan; Urquhart, Jill E; Daly, Sarah B; Bhaskar, Sanjeev S; Williams, Simon G; Newman, William G; Spiegel, Ronen; Azem, Abdussalam; Elpeleg, Orly; Mahajnah, Muhammad

    2017-04-01

    PTRH2 is an evolutionarily highly conserved mitochondrial protein that belongs to a family of peptidyl-tRNA hydrolases. Recently, patients from two consanguineous families with mutations in the PTRH2 gene were reported. Global developmental delay associated with microcephaly, growth retardation, progressive ataxia, distal muscle weakness with ankle contractures, demyelinating sensorimotor neuropathy, and sensorineural hearing loss were present in all patients, while facial dysmorphism with widely spaced eyes, exotropia, thin upper lip, proximally placed thumbs, and deformities of the fingers and toes were present in some individuals. Here, we report a new family with three siblings affected by sensorineural hearing loss and peripheral neuropathy. Autozygosity mapping followed by exome sequencing identified a previously reported homozygous missense mutation in PTRH2 (c.254A>C; p.(Gln85Pro)). Sanger sequencing confirmed that the variant segregated with the phenotype. In contrast to the previously reported patient, the affected siblings had normal intelligence, milder microcephaly, delayed puberty, myopia, and moderate insensitivity to pain. Our findings expand the clinical phenotype and further demonstrate the clinical heterogeneity related to PTRH2 variants.

  9. Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

    PubMed

    Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

    2015-01-01

    Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss.

  10. Modeling the Effects of Sensorineural Hearing Loss on Sound Localization in the Median Plane

    PubMed Central

    Majdak, Piotr; Laback, Bernhard

    2016-01-01

    Listeners use monaural spectral cues to localize sound sources in sagittal planes (along the up-down and front-back directions). How sensorineural hearing loss affects the salience of monaural spectral cues is unclear. To simulate the effects of outer-hair-cell (OHC) dysfunction and the contribution of different auditory-nerve fiber types on localization performance, we incorporated a nonlinear model of the auditory periphery into a model of sagittal-plane sound localization for normal-hearing listeners. The localization model was first evaluated in its ability to predict the effects of spectral cue modifications for normal-hearing listeners. Then, we used it to simulate various degrees of OHC dysfunction applied to different types of auditory-nerve fibers. Predicted localization performance was hardly affected by mild OHC dysfunction but was strongly degraded in conditions involving severe and complete OHC dysfunction. These predictions resemble the usually observed degradation in localization performance induced by sensorineural hearing loss. Predicted localization performance was best when preserving fibers with medium spontaneous rates, which is particularly important in view of noise-induced hearing loss associated with degeneration of this fiber type. On average across listeners, predicted localization performance was strongly related to level discrimination sensitivity of auditory-nerve fibers, indicating an essential role of this coding property for localization accuracy in sagittal planes. PMID:27659486

  11. Intratympanic Steroid Treatment for Idiopathic Sudden Sensorineural Hearing Loss after Failure of Intravenous Therapy

    PubMed Central

    Ferri, Emanuele; Frisina, Antonio; Fasson, Anna Chiara; Armato, Enrico; Spinato, Giacomo; Amadori, Maurizio

    2012-01-01

    Purpose. The aim of this study is the investigation of the effectiveness of intratympanic steroids therapy (IST) in patients with idiopathic sudden sensorineural hearing loss (ISSHL) who had not responded to intravenous treatment, evaluating the overall hearing recovery and comparing the results with different variables. Materials and Methods. Our study consisted of 55 patients with refractory ISSHL who, at the end of 10 days of therapy with intravenous steroids, had puretone 4-frequency average (PTA) of worse than 30 dB. The patients received 0.5 mL of methylprednisolone by direct intratympanic injection. The procedure was carried out up to 7 times within a 20-days period. Statistical analysis was carried out. Results. Overall 29 patients (52.7%) showed improvement in PTA, 24 (43.8%) had no change in hearing, and 2 (3.5%) worsened. There was a significant statistical correlation between hearing recovery and time to onset of symptoms, severity of hearing loss and frequency of hearing loss. Conclusions. IST is an effective and safe therapy in sudden sensorineural hearing loss cases that are refractory to standard treatment. The earlier IST, the hearing losses less than 90 dB and the involvement of the low frequencies seem to influence positively the hearing recovery. PMID:23724270

  12. Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

    PubMed

    Hendriks, Y M; Laan, L A; Vielvoye, G J; van Haeringen, A

    1999-09-10

    We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.

  13. Acute liver failure impairs function and expression of breast cancer-resistant protein (BCRP) at rat blood-brain barrier partly via ammonia-ROS-ERK1/2 activation.

    PubMed

    Li, Ying; Zhang, Ji; Xu, Ping; Sun, Binbin; Zhong, Zeyu; Liu, Can; Ling, Zhaoli; Chen, Yang; Shu, Nan; Zhao, Kaijing; Liu, Li; Liu, Xiaodong

    2016-07-01

    We once reported that P-glycoprotein (P-GP) and multidrug resistance-associated protein 2 (MRP2) were oppositely regulated at the blood-brain barrier (BBB) of thioacetamide-induced acute liver failure (ALF) rats. This study aimed to investigate whether ALF affected function and expression of breast cancer-resistant protein (BCRP) at the BBB of rats and the role of ammonia in the regulation. ALF rats were developed by intraperitoneal (i.p.) injection of thioacetamide (300 mg/kg) for 2 days. Hyperammonemic rats were developed by NH4 Ac (i.p. 4.5 mmol/kg). BCRP function and expression were measured by brain distribution of specific substrates (prazosin and methotrexate) and western blot, respectively. MDCK-BCRP cells and primarily cultured rat brain microvessel endothelial cells (rBMECs) were employed to investigate possible mechanisms through which ammonia regulated BCRP function and expression. The results showed that both ALF and hyperammonemia significantly weakened function and expression of BCRP in the brain of rats. The function and expression of BCRP in MDCK-BCRP cells and rBMECs were strikingly decreased after exposure to NH4 Cl and H2 O2 , accompanied by remarkable increases in the levels of phosphorylated ERK1/2 and reactive oxygen species (ROS). The altered BCRP expression and function by ammonia and H2 O2 were restored by ROS scavenger N-acetylcysteine and ERK1/2 inhibitor U0126. Markedly increased levels of ERK1/2 phosphorylation and ROS were found in the brains of ALF rats and hyperammonemic rats. All above results indicated ALF down-regulated expression and function of BCRP at BBB of rats partly via hyperammonemia. Activation of ROS-mediated ERK1/2 phosphorylation may be one of the reasons that ammonia impaired BCRP expression and function at the BBB. The present study showed that the expression and function of breast cancer resistant protein (BCRP) at blood-brain barrier (BBB) of thioacetamide-induced ALF rats were down-regulated which partly

  14. Bilateral sudden sensorineural deafness with vertigo as the sole presenting symptoms of diabetes mellitus - a case report.

    PubMed

    Misra, Vilas; Agarwal, C G; Bhatia, Naresh; Shukla, G K

    2010-06-01

    This Paper reports a late uncontrolled diabetic presenting to an otolaryngologist with sudden severe sensorineural hearing loss of immediate origin with vertigo and tinnitus as the symptoms. Appropriate investigative and treatment measure resulted in deterioration of hearing in the right ear and mild improvement of hearing in the left ear, with no recovery of imbalance.

  15. A Comparison of Phonological Processing Skills of Children with Mild to Moderate Sensorineural Hearing Loss and Children with Dyslexia

    ERIC Educational Resources Information Center

    Park, Jungjun; Lombardino, Linda J.

    2012-01-01

    Using the Comprehensive Test of Phonological Processes (Wagner, Torgesen, & Rashotte, 1999), the researchers compared strengths and weaknesses in phonological processing skills in three groups: 21 children with mild to moderate sensorineural hearing loss (MSNH group), 29 children with dyslexia, and 30 age-matched controls. The MSNH group showed…

  16. Systemic Polyarteritis Nodosa as the Cause of Sudden Onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Infection

    DTIC Science & Technology

    2016-07-05

    distribution is unlimited. UNCLASSIFIED Title: Systemic polyarteritis nodosa as the cause of sudden-onset bilateral sensorineural hearing loss...tissues from infected primates revealed pathology consistent with systemic polyarterisis nodosa (PAN), an autoimmune disorder associated with sudden...etiology for LASV-associated sudden-onset hearing loss. One Sentence Summary: Systemic polyarteritis nodosa is the proposed underlying pathology

  17. Sensorineural deafness, hypospadias, and synostosis of metacarpals and metatarsals 4 and 5: a previously apparently undescribed MCA/MR syndrome.

    PubMed

    Pfeiffer, R A; Kapferer, L

    1988-09-01

    We report on a young man with a syndrome of mental retardation, abnormalities of the brain, sensorineural deafness, hypospadias, bilateral synostosis of the 4th and 5th metacarpals and metatarsals, and abnormal dermatoglyphics. This appears to be a previously undescribed MCA/MR syndrome.

  18. [Cochlear implantation in a child with congenital sensorineural deafness due to 35 DELG mutation in GJB2 (connexin 26) gene].

    PubMed

    Teriutin, F M; Barashkov, N A; Dzhemileva, L U; Posukh, O L; Fedotova, E E; Gurinova, E E; Fedorova, S A; Tavartkiladze, G A; Khusnutdinova, E K

    2009-01-01

    This paper reports the first case of cochlear implantation performed in this country in a child with congenital non-syndromic sensorineural loss of hearing having hereditary etiology and attributable to autosomal-recessive 35 delG mutation in locus DFNB1 (13q.11-q12) of GJB2 (connexin 26) gene.

  19. Acute pancreatitis and acute renal failure complicating doxylamine succinate intoxication.

    PubMed

    Lee, Yang Deok; Lee, Soo Teik

    2002-06-01

    Doxylamine succinate is an antihistaminic drugwith additional hypnotic, anticholinergic and local anesthetic effects first described in 1948. In Korea and many other countries, it is a common-over-the counter medication frequently involved in overdoses. Clinical symtomatology of doxylamine succinate overdose includes somnolence, coma, seizures, mydriasis, tachycardia, psychosis, and rhabdomyolysis. A serious complication may be rhabdomyolysis with subsequent impairment of renal function and acute renal failure. We report a case of acute renal failure and acute pancreatitis complicating a doxylamine succinate intoxication.

  20. Impact of aerobic exercise training during chemotherapy on cancer related cognitive impairments in patients suffering from acute myeloid leukemia or myelodysplastic syndrome - Study protocol of a randomized placebo-controlled trial.

    PubMed

    Zimmer, P; Oberste, M; Bloch, W; Schenk, A; Joisten, N; Hartig, P; Wolf, F; Baumann, F T; Garthe, A; Hallek, M; Elter, T

    2016-07-01

    Cancer related cognitive impairments (CRCI) are frequently reported by patients prior to, during and after medical treatment. Although this cognitive decline severely affects patients' quality of life, little is known about effective treatments. Exercise programs represent a promising supportive strategy in this field. However, evidence is sparse and existing studies display methodological limitations. In the planned study, 83 men and women newly diagnosed with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS) will be randomized into one of three treatment groups. During 4weeks of induction chemotherapy with Anthracycline and Cytarabin patients allocated to exercise group will cycle 3×/week for 30min at moderate to vigorous intensity on an ergometer. Patients allocated to placebo group will receive a supervised myofascial release training (3×/week, approx. 30min) and patients at control group will get usual care. As primary endpoints a cognitive test battery will be conducted measuring performances depending on verbal/spatial memory and executive functioning. Secondary endpoints will be self-perceived cognitive functioning, as well as neurotrophic and inflammatory serum markers. All assessments will be conducted immediately after hospitalization and before chemotherapy is commenced, immediately before discharge of hospital after 4-5weeks as well as before continuing medical treatment 3-4weeks after discharge. This will be the first study investigating the impact of an aerobic exercise training on CRCI in AML/MDS patients. We hope that the study design and the state-of-the-art assessments will help to increase knowledge about CRCI in general and exercise as potential treatment option in this under investigated population.

  1. Sensorineural deafness

    MedlinePlus

    ... Meniere disease and acoustic neuromas ) Ringing or buzzing sound in the ears ( tinnitus ) Causes The inner part of the ear contains tiny hair cells (nerve endings), that change sounds into electric signals. The nerves then carry these ...

  2. Progressive sensorineural hearing loss, subjective tinnitus and vertigo caused by elevated blood lipids.

    PubMed

    Pulec, J L; Pulec, M B; Mendoza, I

    1997-10-01

    The otologist frequently sees patients with progressive sensorineural hearing loss, subjective aural tinnitus and vertigo with no apparent cause. Elevated blood lipids may be a cause of inner ear malfunction on a biochemical basis. To establish the true incidence of this condition, all new patients (4,251) seen during an eight-year period were evaluated; of these, 2,332 patients had complaints of inner ear disease. All had a complete neurotologic examination, appropriate audiometric and vestibular studies and imaging, and blood tests including lipid phenotype studies. Hyperlipoproteinemia was found in 120 patients (5.1%). Most patients were found to be overweight and had additional coexisting conditions such as diabetes mellitus. Treatment with vasodilators and a 500-calorie, high-protein, low-carbohydrate diet yielded improvement of symptoms in 83% of patients within five months of initiation of treatment.

  3. Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss.

    PubMed

    Lan, Ming-Ying; Shiao, Jiun-Yih; Ho, Ching-Yin; Hung, Hao-Chun

    2009-09-01

    The objective of this study is to use standardized measurements of the inner ear to see whether there are subtle bony malformations in children with congenital sensorineural hearing loss (SNHL) whose temporal bone computed tomography (CT) are grossly normal. The study includes 45 ears with congenital SNHL and grossly normal temporal bone CT scans and 45 ears with normal inner ear structures and normal hearing. Standardized measurements of the inner ear structures were made on axial temporal bone CT scans. Student's t test was performed to compare the measurements of the two groups. There were significant differences in the measurements of the bony island width of the superior semicircular canal, bony island width of the lateral semicircular canal and maximal height of cochlea between two groups (P < 0.05). In conclusion, standardized measurements of bony labyrinth of inner ear on temporal bone CT can identify subtle abnormalities of inner ear in patients with congenital SNHL having grossly normal radiological images.

  4. X-linked ocular albinism and sensorineural deafness: Linkage to Xp22. 3

    SciTech Connect

    Winship, I.M.; Babaya, M.; Ramesar, R.S. )

    1993-11-01

    X-linked ocular albinism with late-onset sensorineural deafness (OASD) is an autonomous disorder that poses significant clinical problems, causing affected individuals to be blind and deaf by early middle age. Classical X-linked ocular albinism (without deafness; OA1) has recently been linked to markers in the Xp22.2-Xp22.3 region of the human genome. In the present report, a large South African family with OASD was investigated at the molecular level and tight linkage was found to the DXS452 locus at Xp22.3 using 25 informative meioses, with a maximum lod score of 7.1 at a recombination fraction of 0.00. These findings suggest that OA1 and OASD are allelic variants or that they may be due to contiguous gene defects. 12 refs., 1 fig.

  5. Infantile variant of Bartter syndrome and sensorineural deafness: A new autosomal recessive disorder

    SciTech Connect

    Landau, D.; Shalev, H.; Carmi, Rivka; Ohaly, M.

    1995-12-04

    The infantile variant of Bartter syndrome (IBS) is usually associated with maternal polyhydramnios, premature birth, postnatal polyuria and hypokalemic hypochloremic metabolic alkalosis and a typical appearance. IBS is thought to be an autosomal recessive trait. Several congenital tubular defects are associated with sensorineural deafness (SND). However, an association between the IBS and SND has not been reported so far. Here we describe 5 children of an extended consanguineous Bedouin family with IBS and SND. In 3 of the cases, the typical electrolyte imbalance and facial appearance were detected neonatally. SND was detected as early as age 1 month, suggesting either coincidental homozygotization of 2 recessive genes or a pleiotropic effect of one autosomal recessive gene. This association suggests that evaluation of SND is warranted in every case of IBS. 35 refs., 2 figs., 2 tabs.

  6. Unilateral sensorineural hearing loss and facial nerve paralysis associated with low-voltage electrical shock.

    PubMed

    Ozkiris, Mahmut

    2014-02-01

    Electrical injuries can occur as a result of contact with low- or high-voltage electricity. Low-voltage injuries are more common, as they usually occur in the home, but reports in the literature are few. After exposure to electric current, almost every organ system in the body is affected. The severity of an injury depends on many factors, including the type of current, the duration of exposure, and the resistance of the tissue involved. Reported cases of hearing loss and facial nerve paralysis associated with low-voltage electrical shock are rare, and minimal information is available about this circumstance. In this article, the author describes a case of low-voltage electrical shock in a 20-year-old man. To the best of the author's knowledge, this is the first report in the literature of a resolution of unilateral sensorineural hearing loss and facial nerve paralysis caused by a low-voltage electrical shock.

  7. A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in children.

    PubMed

    Hudson, V E; Elniel, A; Ughratdar, I; Zebian, B; Selway, R; Lin, J P

    2017-01-01

    Cochlear implants for sensorineural deafness in children is one of the most successful neuromodulation techniques known to relieve early chronic neurodisability, improving activity and participation. In 2012 there were 324,000 recipients of cochlear implants globally.

  8. [Lightning strike to a vehicle causing acute acoustic trauma].

    PubMed

    Angerer, F; Hoppe, U; Schick, B

    2009-10-01

    The cochlea and vestibular organ is often affected in lightning strikes. A lightning strike to a motor vehicle with cochlear injury has not been described hitherto in the literature. We report the case of a 44-year-old male patient with sensorineural hearing loss and tinnitus after his car was struck by lightning. While hearing loss recovered using intravenous therapy, tinnitus persisted 6 months after the lightning strike. Cochlear injuries as an acute acoustic trauma have to be considered in lightning strikes and can occur when a car is struck by lightning.

  9. Maternally inherited hearing impairment in a family with the mitochondrial DNA A7445G mutation.

    PubMed

    Hutchin, T P; Lench, N J; Arbuzova, S; Markham, A F; Mueller, R F

    2001-01-01

    Despite the increasing number of reports of families with hearing impairment and mitochondrial DNA (mtDNA) mutations, the frequency of these mutations as causes of non-syndromic sensorineural hearing impairment (NSSHI) remains unknown. Mutations such as A1555G, A7445G and 7472insC have been found in several unrelated families implying they are more frequent than initially thought. We describe a family with NSSHI due to the presence of the homoplasmic mtDNA A7445G mutation in the tRNASer(UCN) gene. This is the fourth such family described with this mutation, all of different genetic backgrounds. Our study also demonstrates the difficulties sometimes encountered in establishing mitochondrial inheritance of hearing impairment in some families.

  10. Acute necrosis after Gamma Knife surgery in vestibular schwannoma leading to multiple cranial nerve palsies.

    PubMed

    Kapitza, Sandra; Pangalu, Athina; Horstmann, Gerhard A; van Eck, Albert T; Regli, Luca; Tarnutzer, Alexander A

    2016-08-01

    We discuss a rare acute complication after Gamma Knife therapy (Elekta AB, Stockholm, Sweden) in a single patient. A 52-year-old woman presented with vertigo, facial weakness and hearing loss emerging 48hours following Gamma Knife radiosurgery for a right-sided vestibular schwannoma. Neurological examination 6days after symptom onset showed right-sided facial palsy, spontaneous left-beating nystagmus and pathologic head-impulse testing to the right. Pure-tone audiogram revealed right-sided sensorineural hearing loss. A diagnosis of acute vestibulocochlear and facial neuropathy was made. Brain MRI demonstrated focal contrast sparing within the schwannoma, likely related to acute radiation necrosis. Acute multiple cranial neuropathies of the cerebellopontine angle after Gamma Knife treatment should raise suspicion of acute tissue damage within the schwannoma and should result in urgent MRI. Treatment with steroids may be considered based on accompanying swelling and edema.

  11. Cortical Visual Impairment

    MedlinePlus

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Cortical Visual Impairment En Español Read in Chinese What is cortical visual impairment? Cortical visual impairment (CVI) is a decreased ...

  12. GJB2 Gene Mutations in Syndromic Skin Diseases with Sensorineural Hearing Loss.

    PubMed

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-11-01

    The GJB2 gene is located on chromosome 13q12 and it encodes the connexin 26, a transmembrane protein involved in cell-cell attachment of almost all tissues. GJB2 mutations cause autosomal recessive (DFNB1) and sometimes dominant (DFNA3) non-syndromic sensorineural hearing loss. Moreover, it has been demonstrated that connexins are involved in regulation of growth and differentiation of epidermis and, in fact, GJB2 mutations have also been identified in syndromic disorders with hearing loss associated with various skin disease phenotypes. GJB2 mutations associated with skin disease are, in general, transmitted with a dominant inheritance pattern. Nonsyndromic deafness is caused prevalently by a loss-of-function, while literature evidences suggest for syndromic deafness a mechanism based on gain-of-function. The spectrum of skin manifestations associated with some mutations seems to have a very high phenotypic variability. Why some mutations can lead to widely varying cutaneous manifestations is poorly understood and in particular, the reason why the skin disease-deafness phenotypes differ from each other thus remains unclear. This review provides an overview of recent findings concerning pathogenesis of syndromic deafness imputable to GJB2 mutations with an emphasis on relevant clinical genotype-phenotype correlations. After describing connexin 26 fundamental characteristics, the most relevant and recent information about its known mutations involved in the syndromic forms causing hearing loss and skin problems are summarized. The possible effects of the mutations on channel expression and function are discussed.

  13. Role of worry in patients with chronic tinnitus and sensorineural hearing loss: a preliminary study.

    PubMed

    Caldirola, Daniela; Teggi, Roberto; Daccò, Silvia; Sangiorgio, Erika; Bussi, Mario; Perna, Giampaolo

    2016-12-01

    Tinnitus-related distress appears to be more strongly associated with multiple psychological factors than with any perceptual properties of tinnitus. Prior studies have not investigated the role of worry in tinnitus sufferers. Worry is a dispositional cognitive trait that involves a pervasive, non-specific, future-oriented proneness to fretting, which can foster negative affective states and catastrophic thinking about a specific trouble when the trouble is actual and present. We examined the relationship between worry and self-perceived anxiety and depressive symptoms and handicap in 54 outpatients with chronic tinnitus and sensorineural hearing loss who had been previously recruited for a randomized double-blind study on the efficacy of transmeatal low-level laser therapy for tinnitus. We measured the current anxiety and depressive symptoms with the State-Trait Anxiety Inventory Form Y-1/Self-evaluation Depression Scale, the handicap with the Tinnitus Handicap Inventory, and the proneness to worry with the Penn State Worry Questionnaire. For the psychoacoustic tinnitus measures, we considered the loudness match and the minimum masking level. We found that tinnitus-related anxiety and depressive symptoms and handicap were significantly associated with proneness to worry (linear regression models, p < 0.01), whereas no associations were found with the psychoacoustic measures. This suggests the usefulness of worry assessment when managing chronic tinnitus in clinical practice. Early therapeutic interventions for reducing proneness to worry may facilitate better adaptation to tinnitus.

  14. Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction.

    PubMed

    Martines, Francesco; Dispenza, Francesco; Gagliardo, Cesare; Martines, Enrico; Bentivegna, Daniela

    2011-01-01

    Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic resonance imaging showed an ischemic infarct in the right AICA territory. This case represents the fifth in the literature to date but it confirms that AICA occlusion can cause sudden deafness even without brainstem or cerebellar signs. Therefore, we recommend submitting the patient for neuroimaging, as an emergency, in order to exclude infarction of the AICA territory. By doing this, it may be possible to limit the extent of the lesion by commencing early therapy.

  15. Abnormal Magnetic Resonance Imaging Findings in Patients With Sudden Sensorineural Hearing Loss

    PubMed Central

    Jeong, Kyung-Hwa; Choi, Jin Woo; Shin, Jung Eun; Kim, Chang-Hee

    2016-01-01

    Abstract The etiology of sudden sensorineural hearing loss (SSNHL) remains unclear in most cases. This study aimed to assess abnormal magnetic resonance imaging (MRI) findings in patients with SSNHL and evaluate the value of MRI in identifying the cause of SSNHL. A retrospective analysis of the charts and MRI findings of 291 patients with SSNHL was performed. In 291 patients, MRI abnormality, which was considered a cause of SSNHL, was detected in 13 patients. Vestibular schwannoma involving the internal auditory canal (IAC) and/or cerebellopontine angle was observed in 9 patients. All 9 patients had intrameatal tumors, and 6 of the 9 patients displayed extrameatal extension of their tumors. The tumor was small (<1 cm) or medium-sized (1.1–2.9 cm) in these 6 patients. Intralabyrinthine schwannoma, labyrinthine hemorrhage, IAC metastasis, and a ruptured dermoid cyst were each observed in 1 patient. The most commonly observed MRI abnormality in patients with SSNHL was vestibular schwannoma, and all of the lesions were small or medium-sized tumors involving the IAC. PMID:27124066

  16. PSIP1/LEDGF: a new gene likely involved in sensorineural progressive hearing loss

    PubMed Central

    Girotto, Giorgia; Scheffer, Déborah I.; Morgan, Anna; Vozzi, Diego; Rubinato, Elisa; Di Stazio, Mariateresa; Muzzi, Enrico; Pensiero, Stefano; Giersch, Anne B.; Corey, David P.; Gasparini, Paolo

    2015-01-01

    Hereditary Hearing Loss (HHL) is an extremely heterogeneous disorder. Approximately 30 out of 80 known HHL genes are associated with autosomal dominant forms. Here, we identified PSIP1/LEDGF (isoform p75) as a novel strong candidate gene involved in dominant HHL. Using exome sequencing we found a frameshift deletion (c.1554_1555del leading to p.E518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a variable eye phenotype (i.e. uveitis, optic neuropathy). This deletion led to a premature stop codon (p.T519X) with truncation of the last 12 amino acids. PSIP1 was recently described as a transcriptional co-activator regulated by miR-135b in vestibular hair cells of the mouse inner ear as well as a possible protector against photoreceptor degeneration. Here, we demonstrate that it is ubiquitously expressed in the mouse inner ear. The PSIP1 mutation is associated with a peculiar audiometric slope toward the high frequencies. These findings indicate that PSIP1 likely plays an important role in HHL. PMID:26689366

  17. Idiopathic sudden sensorineural hearing loss: cardiovascular risk factors do not influence hearing threshold recovery.

    PubMed

    Ciorba, A; Hatzopoulos, S; Bianchini, C; Iannini, V; Rosignoli, M; Skarzynski, H; Aimoni, C

    2015-04-01

    Previous studies have suggested that risk factors for ischaemic vascular disease, such as cigarette smoking, hypertension and hyperlipidaemia, can also be considered risk factors for the development of idiopathic sudden sensorineural hearing loss (ISSNHL). In this study, we have evaluated the hypothesis that these factors can influence hearing threshold recovery in patients affected by ISSNHL. A total of 141 subjects who suffered an episode of ISSNHL were included. All subjects were assessed with tonal audiometry, auditory brainstem responses and MRI to exclude retrocochlear pathology. Hearing tests were conducted at ISSNHL onset (t = 0) and after 30 days. Patients were divided into three classes according to the presence/absence of one or more cardiovascular risk factors including: history of smoking, total serum cholesterol/triglycerides, history of hypertension and diabetes mellitus. Values of hearing threshold recovery were estimated and comparisons were conducted across the three risk factor classes. 75% of patients affected by ISSNHL showed a threshold recovery. However, the threshold recovery was found to be class-independent (average recovery value of 18 dB HL per classes) and also independent of age and gender. Even if cardiovascular risk factors have been found to be involved in the pathogenesis of ISSNHL, the present study suggests that these factors do not have any significant influence on the threshold recovery in ISSNHL.

  18. Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation.

    PubMed

    Young, Joseph Y; Ryan, Maura E; Young, Nancy M

    2014-01-01

    Cochlear implantation is the only U.S. Food and Drug Administration-approved treatment for children with marked bilateral sensorineural hearing loss. It provides auditory benefits that range from simple sound detection to substantial word understanding. Improved hearing through cochlear implantation has been demonstrated to enhance the rate of language acquisition, enable development of spoken language, and advance literacy in deaf children. Magnetic resonance imaging and computed tomography both have roles in the preoperative assessment of inner-ear abnormalities, cochlear nerve deficiency, and variant anatomy that may affect the decision to implant and the prognosis for auditory improvement and increase the risk for complications. Most cochlear abnormalities may be successfully treated with cochlear implantation, but the presence of a cochlear malformation may increase the risk for intraoperative cerebrospinal fluid leakage and postoperative bacterial meningitis. Eighth-nerve deficiency correlates with poor auditory outcomes and may affect eligibility for cochlear implantation. Another important consideration for implantation is the presence of labyrinthitis ossificans in some children with deafness resulting from bacterial meningitis, which may cause obstruction that limits electrode insertion. Anatomic variations of the facial nerve or middle-ear cavity, which are more common in syndromic patients, may also affect the surgical approach and make implantation difficult.

  19. Endocranial anatomy of lambeosaurine hadrosaurids (Dinosauria: Ornithischia): a sensorineural perspective on cranial crest function.

    PubMed

    Evans, David C; Ridgely, Ryan; Witmer, Lawrence M

    2009-09-01

    Brain and nasal cavity endocasts of four corythosaurian lambeosaurines (Dinosauria: Ornithischia) were investigated to test hypotheses of cranial crest function related to sensorineural systems. Endocasts were generated through computed tomography and three-dimensional rendering and visualization software. The sample comprises a range of ontogenetic stages from the taxa Lambeosaurus, Corythosaurus, and Hypacrosaurus. Results show that the morphology of brain endocasts differs little from that of hadrosaurines. The strikingly convoluted nasal vestibule of Hypacrosaurus altispinus, when interpreted in the context of lambeosaurine phylogeny, suggests selective pressure for nasal cavity function independent from changes in the external shape of the crest and associated visual display function. The plesiomorphically small olfactory bulbs and apparently small olfactory region of the nasal cavity argues against the hypothesis that increased olfactory acuity played a causal role in crest evolution. The elongate cochlea of the inner ear reveals that hearing in lambeosaurines emphasized low frequencies consistent with the hypothesized low-frequency calls made by the crests under the resonation model of crest function. The brain is relatively large in lambeosaurines compared with many other large dinosaurs, and the cerebrum is relatively larger than that of all non-hadrosaurian ornithischians and large theropods, but compares favorably with hadrosaurine hadrosaurids as well as some maniraptoran theropods. It is concluded that the large brains of lambeosaurines are consistent with the range of social behaviors inferred when the crest is interpreted as an intraspecific signaling structure.

  20. Role of p63 and the Notch pathway in cochlea development and sensorineural deafness.

    PubMed

    Terrinoni, Alessandro; Serra, Valeria; Bruno, Ernesto; Strasser, Andreas; Valente, Elizabeth; Flores, Elsa R; van Bokhoven, Hans; Lu, Xin; Knight, Richard A; Melino, Gerry

    2013-04-30

    The ectodermal dysplasias are a group of inherited autosomal dominant syndromes associated with heterozygous mutations in the Tumor Protein p63 (TRP63) gene. Here we show that, in addition to their epidermal pathology, a proportion of these patients have distinct levels of deafness. Accordingly, p63 null mouse embryos show marked cochlea abnormalities, and the transactivating isoform of p63 (TAp63) protein is normally found in the organ of Corti. TAp63 transactivates hairy and enhancer of split 5 (Hes5) and atonal homolog 1 (Atoh1), components of the Notch pathway, known to be involved in cochlear neuroepithelial development. Strikingly, p63 null mice show morphological defects of the organ of Corti, with supernumerary hair cells, as also reported for Hes5 null mice. This phenotype is related to loss of a differentiation property of TAp63 and not to loss of its proapoptotic function, because cochleas in mice lacking the critical Bcl-2 homology domain (BH-3) inducers of p53- and p63-mediated apoptosis--Puma, Noxa, or both--are normal. Collectively, these data demonstrate that TAp63, acting via the Notch pathway, is crucial for the development of the organ of Corti, providing a molecular explanation for the sensorineural deafness in ectodermal dysplasia patients with TRP63 mutations.

  1. Congenital sensorineural deafness in dalmatian dogs associated with quantitative trait loci.

    PubMed

    Kluth, Susanne; Distl, Ottmar

    2013-01-01

    A genome-wide association study (GWAS) was performed for 235 Dalmatian dogs using the canine Illumina high density bead chip to identify quantitative trait loci (QTL) associated with canine congenital sensorineural deafness (CCSD). Data analysis was performed for all Dalmatian dogs and in addition, separately for brown-eyed and blue-eyed dogs because of the significant influence of eye colour on CCSD in Dalmatian dogs. Mixed linear model analysis (MLM) revealed seven QTL with experiment-wide significant associations (-log10P>5.0) for CCSD in all Dalmatian dogs. Six QTL with experiment-wide significant associations for CCSD were found in brown-eyed Dalmatian dogs and in blue-eyed Dalmatian dogs, four experiment-wide significant QTL were detected. The experiment-wide CCSD-associated SNPs explained 82% of the phenotypic variance of CCSD. Five CCSD-loci on dog chromosomes (CFA) 6, 14, 27, 29 and 31 were in close vicinity of genes shown as causative for hearing loss in human and/or mouse.

  2. Prevalence, heritability and genetic correlations of congenital sensorineural deafness and pigmentation phenotypes in the Border Collie.

    PubMed

    De Risio, Luisa; Lewis, Tom; Freeman, Julia; de Stefani, Alberta; Matiasek, Lara; Blott, Sarah

    2011-06-01

    The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes.

  3. A regressive model analysis of congenital sensorineural deafness in German Dalmatian dogs.

    PubMed

    Juraschko, Kathrin; Meyer-Lindenberg, Andrea; Nolte, Ingo; Distl, Ottmar

    2003-08-01

    The objective of the present study was to analyze the mode of inheritance for congenital sensorineural deafness (CSD) in German Dalmatian dogs by consideration of association between phenotypic breed characteristics and CSD. Segregation analysis with regressive logistic models was employed to test for different mechanisms of genetic transmission. Data were obtained from all three Dalmatian kennel clubs associated with the German Association for Dog Breeding and Husbandry (VDH). CSD was tested by veterinary practitioners using standardized protocols for Brainstem Auditory-Evoked Response (BAER). The sample included 1899 Dalmatian dogs from 354 litters in 169 different kennels. BAER testing results were from the years 1986 to 1999. Pedigree information was available for up to seven generations. The segregation analysis showed that a mixed monogenic-polygenic model including eye color as covariate among all other tested models best explained the segregation of affected animals in the pedigrees. The recessive major gene segregated in dogs with blue and brown eye color as well as in dogs with and without pigmented coat patches. Models which took into account the occurrence of patches, percentage of puppies tested per litter, or inbreeding coefficient gave no better adjustment to the most general (saturated) model. A procedure for the simultaneous prediction of breeding values and the estimation of genotype probabilities for CSD is expected to improve breeding programs significantly.

  4. GJB2 Gene Mutations in Syndromic Skin Diseases with Sensorineural Hearing Loss.

    PubMed Central

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-01-01

    The GJB2 gene is located on chromosome 13q12 and it encodes the connexin 26, a transmembrane protein involved in cell-cell attachment of almost all tissues. GJB2 mutations cause autosomal recessive (DFNB1) and sometimes dominant (DFNA3) non-syndromic sensorineural hearing loss. Moreover, it has been demonstrated that connexins are involved in regulation of growth and differentiation of epidermis and, in fact, GJB2 mutations have also been identified in syndromic disorders with hearing loss associated with various skin disease phenotypes. GJB2 mutations associated with skin disease are, in general, transmitted with a dominant inheritance pattern. Nonsyndromic deafness is caused prevalently by a loss-of-function, while literature evidences suggest for syndromic deafness a mechanism based on gain-of-function. The spectrum of skin manifestations associated with some mutations seems to have a very high phenotypic variability. Why some mutations can lead to widely varying cutaneous manifestations is poorly understood and in particular, the reason why the skin disease-deafness phenotypes differ from each other thus remains unclear. This review provides an overview of recent findings concerning pathogenesis of syndromic deafness imputable to GJB2 mutations with an emphasis on relevant clinical genotype-phenotype correlations. After describing connexin 26 fundamental characteristics, the most relevant and recent information about its known mutations involved in the syndromic forms causing hearing loss and skin problems are summarized. The possible effects of the mutations on channel expression and function are discussed. PMID:22547955

  5. Comprehensive Genetic Analysis of Japanese Autosomal Dominant Sensorineural Hearing Loss Patients

    PubMed Central

    Iwasa, Yoh-ichiro; Nishio, Shin-ya; Usami, Shin-ichi

    2016-01-01

    Background In general, autosomal dominant inherited hearing loss does not have a founder mutation, with the causative mutation different in each family. For this reason, there has been a strong need for efficient diagnosis methods for autosomal dominant sensorineural hearing loss (ADSNHL) patients. This study sought to verify the effectiveness of our analysis algorithm for the screening of ADSNHL patients as well as the usefulness of the massively parallel DNA sequencing (MPS). Subjects and Methods Seventy-five Japanese ADSNHL patients from 53 ENT departments nationwide participated in this study. We conducted genetic analysis of 75 ADSNHL patients using the Invader assay, TaqMan genotyping assay and MPS-based genetic screening. Results A total of 46 (61.3%) ADSNHL patients were found to have at least one candidate gene variant. Conclusion We were able to achieve a high mutation detection rate through the combination of the Invader assay, TaqMan genotyping assay and MPS. MPS could be used to successfully identify mutations in rare deafness genes. PMID:27911912

  6. Value of genetic testing in the otological approach for sensorineural hearing loss.

    PubMed

    Matsunaga, Tatsuo

    2009-12-01

    Sensorineural hearing loss (SNHL) is one of the most common disabilities in human, and genetics is an important aspect for SNHL, especially in children. In recent 10 years, our knowledge in genetic causes of SNHL has made a significant advance, and now it is used for diagnosis and other clinical practices. Hereditary hearing loss can be classified into syndromic and nonsyndromic hearing loss. As the nonsyndromic deafness genes, more than 100 loci for deafness genes have been determined, and more than 40 genes were identified. Furthermore, more than 300 forms of syndromic hearing loss have been characterized, and each syndrome may have several causative genes. In childhood hearing loss, early educational intervention is required in addition to medical intervention for normal development of speech and language. In addition, even severe to profound hearing loss may be restored very effectively by hearing aids or cochlear implants. Because of these features of SNHL, genetic testing has exceptionally high value in the medical practice for hereditary hearing loss. Several strategies are used for genetic testing of SNHL for accurate and efficient identification of the genetic causes, and the results were used for explanation of the cause, prediction of auditory features, prevention of deafness, management of associated symptoms, determination of therapy, and genetic counseling. Identification of damaged cells in the inner ear and the underlying mechanism by genetic testing undoubtedly facilitates development and introduction of novel and specific therapies to distinct types of SNHL.

  7. Transplantation of human umbilical cord mesenchymal stem cells in cochlea to repair sensorineural hearing

    PubMed Central

    Ma, Yueying; Guo, Weiwei; Yi, Haijin; Ren, Lili; Zhao, Lidong; Zhang, Yue; Yuan, Shuolong; Liu, Riyuan; Xu, Liangwei; Cong, Tao; EK, Oghagbon; Zhai, Suoqiang; Yang, Shiming

    2016-01-01

    To examine if transplantation of human umbilical cord mesenchymal stem cells (UMSC) into cochlea can be used to repair sensorineural hearing. Here we transplanted the fifth and sixth generations of UMSCs through the subarachnoid cavity of congenital deaf albino pigs. Auditory brainstem responses (ABR) were measured before and after UMSC transplantation. Cochlear samples were collected at 2 hrs, 3 days, 1, 2, 3, 4 and 8 weeks after transplantation. Immunohistochemistry was used to detect the proliferated cell nuclear antigen (PCNA). The UMSCs were found in different regions of the cochlea, including the stria vascularis, the basal membrane and the spiral ganglions, 3 days to 4 weeks after the transplantation. UMSCs and their DNA were found also in the areas of the brain, the heart, the liver, the kidney and the lung. ABR tests displayed a new waveform in the congenital deaf albino pigs after the UMSCs transplantation. We conclude that human UMSCs injected into the subarachnoid space can migrate into the inner ear, the central nervous system and the periphery organs. The presence of UMSCs in the cochlea maybe associated with changes of ABR waveforms in the congenital deaf albino pigs. PMID:28077998

  8. Non-Mendelian mitochondrial inheritance as a cause of progressive genetic sensorineural hearing loss.

    PubMed

    Gold, M; Rapin, I

    1994-08-01

    Awareness of non-Mendelian mitochondrial inheritance and of its role as an agent of genetic sensorineural hearing loss (SNHL) is recent. Mitochondria are passed on exclusively from the ovum to all the offspring of both sexes, a novel pattern of inheritance. Owing to the critical role of mitochondria in cellular energy metabolism, deletions or point mutations of the mitochondrial DNA often cause progressive SNHL and a variety of disorders in other organ systems (mitochondrial cytopathies). The clinical expression of mitochondrial diseases varies and depends on the proportion of mutated mitochondria in various body tissues, as well as the nature of the mutation or deletion. In order to determine how often SNHL occurs in mitochondrial diseases and what is its presenting symptom, and also whether SNHL is a marker for particular phenotypes, we carried out a review of published case reports of patients with an established diagnosis of mitochondrial disease. The review indicates that SNHL occurs at all ages and in virtually all variants of mitochondrial diseases. It is not clear whether SNHL is a marker for a more severe and more rapid course of disease; the lower prevalence of SNHL in descriptions of live patients than of those who had died may be an artifact of case selection reported in the literature. Mitochondrial disease needs to be considered in progressive hearing loss and better longitudinal audiometric study of established cases will be required to answer these questions.

  9. Sensorineural hearing loss in patients with sickle cell anaemia in Kenya.

    PubMed

    Tsibulevskaya, G; Oburra, H; Aluoch, J R

    1996-07-01

    The auditory function of sixty two Kenyan sickle cell anaemia patients aged seven to thirty years was compared to age-matched fifty five healthy controls with haemoglobin AA. Sensorineural hearing loss of 30 db and above was demonstrated in 25 (40%) of sickle cell anaemia patients and in three (5.5%) control subjects. Both sexes were equally affected. Bilateral lesion registered in 16%. Hearing threshold level was normal in 97% of the sickle cell group. High frequencies were commonly affected with hearing loss of 30-40 db. Two cases with severe unilateral deafness at all frequencies had severe recruitment suggestive of cochlea lesion. There were no cases of acoustic reflex decay in all study patients. The hearing loss was of slow onset. The high risk of deafness in Kenyan sickle cell anaemia patients may be a reflection of the severe course of the disease due to specific Kenyan haematological profile (haplotype 20 with low Hb F level), the level of medical care available and the geographical distribution in the tropics together with other factors.

  10. Meteorological Conditions Related to the Onset of Idiopathic Sudden Sensorineural Hearing Loss

    PubMed Central

    Seo, Jae-Hyun; Park, Yong-Soo; Kim, JunHyun; Chang, Ki-Hong; Yeo, Sang-Won

    2014-01-01

    Purpose The objective of this study was to evaluate the effect of meteorological factors on the onset of idiopathic sudden sensorineural hearing loss (ISSHL). Materials and Methods Meteorological data from 2005 to 2011 were obtained from the web-based "Monthly Weather Reports of the Meteorological Administration" database. Patients with ISSHL who visited our hospital during this same period and presented the precise day on which hearing loss developed were included in this retrospective study. Twelve meteorological factors were analyzed between the days when ISSHL onset was observed as well as the days when ISSHL did not occur. The weather conditions occurring 1-7 days before ISSHL onset were also analyzed to assess any possible delayed effects of meteorological factors on the onset of ISSHL. Results During the study period, 607 patients were included for the study. Although mean and maximal wind velocities were higher for the days when ISSHL occurred than the days without ISSHL onset, after adjusting the value for multiple comparisons, we cannot find any significant relationship between any of meteorological factors and the onset of ISSHL. However, in analysis of time lag effect of the weather conditions, we found that there was still a significant difference in maximum wind speed on 5 days before ISSHL onset even after applying Bonferroni correction. Conclusion The result of this study suggests that stronger wind speed may be related to the occurrence of ISSHL. PMID:25323908

  11. Cystitis - acute

    MedlinePlus

    Uncomplicated urinary tract infection; UTI - acute cystitis; Acute bladder infection; Acute bacterial cystitis ... cause. Menopause also increases the risk for a urinary tract infection. The following also increase your chances of having ...

  12. Recognition Memory Is Impaired in Children after Prolonged Febrile Seizures

    ERIC Educational Resources Information Center

    Martinos, Marina M.; Yoong, Michael; Patil, Shekhar; Chin, Richard F. M.; Neville, Brian G.; Scott, Rod C.; de Haan, Michelle

    2012-01-01

    Children with a history of a prolonged febrile seizure show signs of acute hippocampal injury on magnetic resonance imaging. In addition, animal studies have shown that adult rats who suffered febrile seizures during development reveal memory impairments. Together, these lines of evidence suggest that memory impairments related to hippocampal…

  13. [Acute respiratory distress syndrome].

    PubMed

    Hecker, M; Weigand, M A; Mayer, K

    2012-05-01

    Acute respiratory distress syndrome (ARDS) is the clinical manifestation of an acute lung injury caused by a variety of direct and indirect injuries to the lung. The cardinal clinical feature of ARDS, refractory arterial hypoxemia, is the result of protein-rich alveolar edema with impaired surfactant function, due to vascular leakage and dysfunction with consequently impaired matching of ventilation to perfusion. Better understanding of the pathophysiology of ARDS has led to the development of novel therapies, pharmacological strategies, and advances in mechanical ventilation. However, protective ventilation is the only confirmed option in ARDS management improving survival, and few other therapies have translated into improved oxygenation or reduced ventilation time. The development of innovative therapy options, such as extracorporeal membrane oxygenation, have the potential to further improve survival of this devastating disease.

  14. [Analysis of the relevant factors for recurrent sudden sensorineural hearing loss].

    PubMed

    Liang, H; Zhong, S X

    2016-09-07

    Objective: To investigate the possible factors related to recurrence and prognosis of sudden sensorineural hearing loss(SSNHL). Methods: Four hundred and ninety-five patients with unilateral sudden sensorineural hearing loss between January 2013 to April 2014 were analyzed retrospectively(34 patients lost to follow-up with a dropout rate of 6.87%). Twenty of the 495 patients were diagnosed as recurrent SSNHL and treated again in the same hospital. The data of the patients were summarized to analyze the related factors which might influence the recurrence and prognosis of SSNHL. Results: In the 20 patients with recurrent SSNHL, 19 had the second attack in same ear as the first attack, and the other one had in both ears. There were seven male patients, and thirteen female patients. Patients ranged in age from 24 to 77years, with a median age of 39.5 years. Types of hearing loss: low frequency in eight patients, high frequency in two patients, flat frequency in eight patients, total deafness in two patients, the types of the second attack in 17 patients were same as the first attack, only one patient was changed from total deafness to flat frequency, one case was changed from flat frequency to high frequency, one case changed from flat frequency to total deafness. The intervals between of the first attack time and the second attack time were 1-36 months with the median time of 3.5 months. After systemic oral and (or) transtympanic steroid treatment, recovered in three cases, effective in three cases and 14 cases invalid, the cure rate was 15%, and the total effective rate was 30%. There were statistically significant differences in the recovery rate(χ(2)=8.640, P<0.05) and the overall response rate(χ(2)=12.379, P<0.01)between the first and the second treatments. For the patients with vertigo and/or dizziness, with a history more than seven days, with hypertension or diabetes mellitus, and with a type of hearing loss except low frequency type, the treatment effect

  15. Fully implantable Otologics MET Carina™ device for the treatment of sensorineural hearing loss. Preliminary surgical and clinical results

    PubMed Central

    Bruschini, L; Forli, F; Santoro, A; Bruschini, P; Berrettini, S

    2009-01-01

    Summary Middle ear implants overcome some of the common problems of conventional hearing aid technology, such as feedback, signal distortion, ear canal occlusion and associated issues. The Otologics MET Carina™, Boulder, CO, USA, is a fully implantable hearing prosthesis designed to address the amplification needs of adults (> 18 years of age), with moderate to severe sensorineural hearing loss and normal middle ears, providing a mechanical direct stimulation of middle ear ossicles. Recently, it has been successfully used also in patients with conductive hearing loss. In the present report, personal surgical and clinical experience with the fully implantable Carina™ is described in 5 adults with moderate to severe sensorineural hearing loss, operated upon between November 2007 and May 2008 in the ENT Unit, University of Pisa. Mean follow-up was 10.2 months of device use (range 7-13). Surgery was performed under general anaesthesia, in ~3 hours, with no surgical complications in any of the patients. In these 5 cases, no significant post-operative variation was observed in hearing thresholds, either for air or bone conduction, indicating absence of surgical damage to the cochlea. All patients showed improvements in hearing thresholds, in free field and in speech perception abilities, with the device functioning, moreover, they reported subjective benefits. With regard to post-operative adverse effects, no cases of extrusion of the device, device failure, loss of external communication or increased charging times were observed. Problems of feedback noise occurred, which were resolved with minor fitting adjustments in 4 cases, while a second operation was required to change the microphone position in the other patient. The present results, in agreement with those reported in the literature, confirm that the Otologics MET Carina™ is viable treatment for moderate to severe sensorineural hearing loss and, in selected cases, may represent an alternative to

  16. Is Intratympanic Dexamathasone Injection Effective for the Treatment of Idiopathic Sudden Sensorineural Hearing Loss?

    PubMed Central

    Lee, Kyu Ho; Ryu, Sung Hoon; Lee, Hyung Min; Park, Su Kyoung; Kim, Hyung-Jong

    2015-01-01

    Background and Objectives The purpose of this study is to identify the effectiveness of intratympanic (IT) steroids when used as an initial therapy in sudden sensorineural hearing loss (SSNHL), and when it is used as a salvage therapy in a refractory SSNHL. Subjects and Methods This retrospective study included 122 patients with SSNHL. First, patients were divided into three groups according to the initial treatment; IT steroid, systemic steroid and combined (systemic and IT steroid) groups. Second, patients were divided into two groups according to the salvage treatment in a refractory SSNHL; IT steroid and the control (non IT group). Fifty eight patients who did not respond to initial therapy were included in the second analysis. Hearing was assessed immediately before the treatment and 2 weeks, 4 weeks and 3 months after the treatments. Hearing recovery was defined as an improvement of >15 dB and the final hearing of 25-45 dB in the audiogram. Results When we analyzed the hearing recovery in initial treatment, the comparison of audiogram among three groups did not result in significantly different outcomes. There were no differences in the recovery rate in 2 and 4 weeks throughout all the frequencies. Also, the analysis of the salvage treatment demonstrated that neither IT steroid group nor control group was significantly effective in treating the refractory SSNHL. Conclusions The results suggest that initial treatment of SSNHL with IT steroid alone is as effective as systemic steroid alone or a combination therapy. Also, salvage IT steroids for refractory SSNHL did not have any additional beneficial effects. PMID:26771014

  17. Intratympanic dexamethasone injection vs methylprednisolone for the treatment of refractory sudden sensorineural hearing loss

    PubMed Central

    Berjis, Nezamoddin; Soheilipour, Saeed; Musavi, Alireza; Hashemi, Seyed Mostafa

    2016-01-01

    Background: During the past years various drugs have been used for sudden sensorineural hearing loss (SSNHL) treatment including steroids that are shown to be beneficial. Directed delivery of high doses of steroids into the inner ear is suggested for its potential and known as intratympanic steroids therapy (IST). Despite the use of dexamethasone and methylprednisolone as the traditional treatments, there are still debates about the optimal dosage, preferred drug, and the route of administration. Materials and Methods: We performed a randomized clinical trial study in which 50 patients suffering from SSNHL and resistant to standard therapy were employed. Each patient took 0.5 ml methylprednisolone (40 mg/mg) along with bicarbonate or dexamethasone (4 mg/mL) through direct intratympanic injection. This method was performed and scheduled once every 2 days for three times only for the dexamethasone receiving group. Hearing test was carried out and the results were analyzed according to a four-frequency (0.5, 1.0, 2.0, 3.0 kHz) pure tone average (PTA) and Siegel's criteria. Results: According to Siegel's criteria, three out of 25 (12%) dexamethasone receiving patients were healed in 1 and 4 (16%), 9 (32%) were respectively recovered in Siegel's criteria 2, 3, and 9 (32%) showed no recovery. In the group receiving methylprednisolone, recovery was found in 6 (24%), 8 (32%), 7 (28%) patients in the Siegel's criteria 1, 2, 3, respectively, and in 4 (16%) patients no recovery was recorded. In methylprednisolone group, hearing was significantly improved compared to the dexamethasone group (P < 0.05). The general hearing improvement rate was 84% in methylprednisolone receiving patients showing a significantly higher improvement than 64% in the dexamethasone group. Conclusions: Topical intratympanic treatment with methylprednisolone is safe and an effective treatment approach for those SSNHL cases that are refractory to the common therapies by Dexamethasone. PMID:27403406

  18. Prognostic factors for profound sudden idiopathic sensorineural hearing loss: a multicenter retrospective study.

    PubMed

    Lee, Ho Yun; Kim, Dong-Kee; Park, Yong-Ho; Cha, Wang Woon; Kim, Geun Jeon; Lee, Seung Hun

    2017-01-01

    The aim of this study was to assess the outcomes of various treatment modalities for profound idiopathic sudden sensorineural hearing loss (ISSNHL) and confirm the prognostic factors. In total, 191 patients were enrolled after a thorough medical chart review of patients diagnosed with unilateral, profound ISSNHL (≥90 dB). Epidemiological profiles, therapeutic regimens, and the results of pure tone audiometry tests were recorded for all patients. Final recovery was assessed according to Siegel's criteria and by comparing the final hearing level of the affected ear with that of the unaffected ear. The mean follow-up duration and the final hearing level were 75 ± 54 days and 77 ± 24 dB, respectively. None of the evaluated prognostic factors were significantly associated with complete recovery (<25 dB). However, improved hearing in both ears, the absence of dizziness, the use of lipo-prostaglandin E1 (lipo-PGE1), and the use of plasma volume expanders were independently associated with a final hearing level of up to 45 dB (p < 0.05). Steroid dose reduction, worse initial hearing, and non-use of lipo-PGE1 increased the possibility of no recovery. Although the efficacy of oral steroid treatment for profound ISSNHL has been questioned, steroid dose reduction was significantly associated with no recovery. Therefore, adequate oral corticosteroid doses should be considered in the absence of contraindications. In addition, the use of lipo-PGE1 and/or a plasma volume expander seems preferable for better recovery, and their use for the management of profound ISSNHL should be considered.

  19. Association between sudden sensorineural hearing loss and anxiety disorder: a population-based study.

    PubMed

    Chung, Shiu-Dong; Hung, Shih-Han; Lin, Herng-Ching; Sheu, Jau-Jiuan

    2015-10-01

    Anxiety disorder (AD) is commonly associated with a number of physical illnesses. No previous study has investigated the association between AD and sudden sensorineural hearing loss (SSNHL). In this study, we investigated the association between prior AD and SSNHL using a population-based dataset in Taiwan. Sampled subjects of this case-control study were retrieved from the Taiwan "Longitudinal Health Insurance Database". We identified 3,522 patients who had a diagnosis of SSNHL as cases and 10,566 age- and gender-matched subjects without SSNHL as controls. A conditional logistic regression was used to calculate the odds ratio (OR) for having previously been diagnosed with AD between cases and controls. We found that of 14,088 patients, 13.4% had a prior AD diagnosis, 17.8 and 11.9% for the SSNHL group and controls, respectively. After adjusting for patient socioeconomic characteristics and comorbid medical disorders, SSNHL patients were more likely to have prior AD than the controls (OR 1.49, 95% confidence interval (CI) 1.34-1.66, p < 0.001). Furthermore, we found that the significant relationship between SSNHL and prior AD decreased with age. The relationship was the most pronounced among those aged ≤44 years, with an adjusted OR of 1.86 (95% CI 1.48-2.33, p < 0.001) for cases compared to controls. We concluded that patients with SSNHL had a higher proportion of prior AD than non-SSNHL-diagnosed controls. Further study is needed to confirm our findings and explore the underlying pathomechanisms.

  20. Sensorineural Hearing Loss After Treatment of Nasopharyngeal Carcinoma: A Longitudinal Analysis

    SciTech Connect

    Chan, S.H. Ng, W.T.; Kam, K.L.; Lee, Michael C.H.; Choi, C.W.; Yau, T.K.; Lee, Anne W.M.; Chow, S.K.

    2009-04-01

    Purpose: To analyze the effects of radiotherapy (RT) and chemotherapy in relation to sensorineural hearing loss (SNHL) after contemporary treatment of nasopharyngeal carcinoma. Methods and Materials: A total of 87 nasopharyngeal carcinoma patients were treated with RT or chemoradiotherapy using either three-dimensional conformal RT or intensity-modulated RT between 2004 and 2005. Tympanometry and pure-tone audiogram assessments were performed before treatment and then serially at 6-month intervals. The dose-volume data of the cochlea were analyzed. The effects of cisplatin administered in concurrent and nonconcurrent phases was explored. Results: Of the 170 eligible ears, RT (n = 30) and chemoradiotherapy (n = 140) resulted in 40% (n = 12) and 56.4% (n = 79) persistent SNHL ({>=}15 dB loss), respectively, after a median follow-up of 2 years. SNHL at a high frequency was more frequent statistically in the chemoradiotherapy group than in the RT-alone group (55% vs. 33.3%, p < 0.01), but not at a low frequency (7.9% vs. 16.7%, p = 0.14). Within the chemoradiotherapy group, the mean cochlea dose and concurrent cisplatin dose were important determinants of high-frequency SNHL, with an odds ratio of 1.07/Gy increase (p = 0.01) and an odds ratio of 1.008/mg/m{sup 2} increase (p < 0.01), respectively. Age, gender, and nonconcurrent cisplatin dose were not statistically significant factors. A mean radiation dose to the cochlea of <47 Gy would result in <15% of patients developing severe ({>=}30 dB) high-frequency SNHL. Conclusion: The results of our study have shown that high-frequency SNHL is significantly related to the mean cochlea dose and the concurrent cisplatin dose. A mean dose constraint of 47 Gy to the cochlea is recommended to minimize SNHL after chemoradiotherapy.

  1. Hyperbaric oxygen therapy as salvage treatment for sudden sensorineural hearing loss: a prospective controlled study.

    PubMed

    Pezzoli, M; Magnano, M; Maffi, L; Pezzoli, L; Marcato, P; Orione, M; Cupi, D; Bongioannini, G

    2015-07-01

    The most commonly used treatment for sensorineural sudden hearing loss (SSHL) in clinical practice is the administration of steroids; however, a favorable result is not always obtained. We studied 58 patients who failed to recover after primary treatment with IV steroids, 44 of these met our inclusion criteria (mean age 50.7, 27 males, range 30-74). We treated 23 patients (mean age 47.3, 16 males, age range 22-74) with hyperbaric oxygen therapy (HBO) (2.5 ATA for 60 min for 15 treatments), while 21 (mean age 54.5, 11 males, age range 22-71) patients refused to be treated and served as a non-randomized control group. Patients treated with HBO had a mean improvement of 15.6 dB (SD ± 15.3), with 1 of them completely healed, 5 with a good recovery, 10 with a fair recovery and 7 unchanged. Patients who were not treated had a spontaneous mean improvement of 5.0 dB (SD ± 11.4) with 3 patients with a good recovery, 1 patient with a fair recovery and 17 patients unchanged. Mean improvement was significantly better in patients treated with HBO compared to controls (p = 0.0133). Patients with worst hearing had the greater degree of improvement whether or not they were treated in the first 10 days after the onset of the hearing loss or between 11 and 30 days. In conclusion, hyperbaric oxygen therapy can lead to significant improvement of pure tone hearing thresholds in patients with SSHL who failed primary corticosteroid treatment and are within 4 weeks of the onset of deafness.

  2. Prediction model for hearing outcome in patients with idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Mori, Takanori; Hashida, Koichi; Shibata, Minori; Nguyen, Khac-Hung; Wakasugi, Tetsuro; Hohchi, Nobusuke

    2011-04-01

    The aim of this study is to develop a regression model for predicting hearing outcome in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A total of 174 consecutive patients with ISSNHL (average of the hearing levels at 250, 500, 1,000, 2,000, and 4,000 Hz was ≥40 dB; time from onset to treatment was ≤30 days) were retrospectively analyzed. They received steroid administration (400 mg/day of hydrocortisone sodium succinate followed by tapered doses) in combination with hyperbaric oxygen therapy. The hearing improvement rate compared to the unaffected contralateral ear was calculated. Correlations between the hearing improvement rate and four prognostic factors (patient's age, days from onset to treatment, initial hearing level, and the presence of vertigo) were examined by simple and multiple regression analyses. In the simple regression analysis, significant correlations were observed between the hearing improvement rate and all four prognostic factors. In the multiple regression analysis, the correlation was significant for patient's age, days from onset to treatment, and the presence of vertigo with partial correlation coefficients of -0.221, -0.324, and -0.329, respectively, but was not significant for the initial hearing level. We subsequently formulated a multiple regression equation for predicting the hearing improvement rate. The multiple correlation coefficient was 0.495 with a p value of 1.42 × 10(-9). Using this regression model, the hearing improvement rate is still difficult to predict with 95% probability, but is predictable with 70% probability.

  3. Childhood sensorineural hearing loss: effects of combined exposure with aging or noise exposure later in life.

    PubMed

    Aarhus, Lisa; Tambs, Kristian; Nafstad, Per; Bjørgan, Eskil; Engdahl, Bo

    2016-05-01

    The aim of the study was to examine childhood high-frequency sensorineural hearing loss (HF-SNHL) and the effects of combined exposure with aging or noise exposure on HF hearing thresholds in adulthood. Population-based cohort study of 30,003 adults (mean age 40 years) underwent an audiometry and completed a hearing questionnaire. At age 7-13 years, the same people had participated in a longitudinal school hearing investigation, in which 283 participants were diagnosed with HF-SNHL [PTA 3-8 kHz ≥ 25 dB HL (mean 45 dB HL), worse hearing ear], and 29,720 participants had normal hearing thresholds. The effect of childhood HF-SNHL on adult hearing threshold was significantly moderated by age. Age stratified analyses showed that the difference in HF hearing thresholds between adults with and without childhood HF-SNHL was 33 dB (95 % CI 31-34) in young adults (n = 173, aged 20-39 years) and 37 dB (95 % CI 34-39) in middle-aged adults (n = 110, aged 40-56 years). The combined exposure of childhood HF-SNHL and noise exposure showed a simple additive effect. It appears to be a super-additive effect of childhood-onset HF-SNHL and aging on adult hearing thresholds. An explanation might be that already damaged hair cells are more susceptible to age-related degeneration. To exclude possible birth cohort effects, the finding should be confirmed by a study with several audiometries in adulthood.

  4. Discovery of CDH23 as a Significant Contributor to Progressive Postlingual Sensorineural Hearing Loss in Koreans

    PubMed Central

    Lee, Chung; Kim, So Young; Kim, Nayoung K. D.; Chang, Mun Young; Rhee, Jihye; Park, Mi-Hyun; Koo, Soo Kyung; Kim, Min Young; Han, Jin Hee; Oh, Seung-ha; Park, Woong-Yang; Choi, Byung Yoon

    2016-01-01

    CDH23 mutations have mostly been associated with prelingual severe-to-profound sensorineural hearing loss (SNHL) in either syndromic or nonsyndromic SNHL (DFNB12). Herein, we demonstrate the contribution of CDH23 mutations to postlingual nonsyndromic SNHL (NS-SNHL). We screened 32 Korean adult probands with postlingual NS-SNHL sporadically or in autosomal recessive fashion using targeted panel or whole exome sequencing. We identified four (12.5%, 4/32) potential postlingual DFNB12 families that segregated the recessive CDH23 variants, qualifying for our criteria along with rapidly progressive SNHL. Three of the four families carried one definite pathogenic CDH23 variant previously known as the prelingual DFNB12 variant in a trans configuration with rare CDH23 variants. To determine the contribution of rare CDH23 variants to the postlingual NS-SNHL, we checked the minor allele frequency (MAF) of CDH23 variants detected from our postlingual NS-SNHL cohort and prelingual NS-SNHL cohort, among the 2040 normal control chromosomes. The allele frequency of these CDH23 variants in our postlingual cohort was 12.5%, which was significantly higher than that of the 2040 control chromosomes (5.53%), confirming the contribution of these rare CDH23 variants to postlingual NS-SNHL. Furthermore, MAF of rare CDH23 variants from the postlingual NS-SNHL group was significantly higher than that from the prelingual NS-SNHL group. This study demonstrates an important contribution of CDH23 mutations to poslingual NS-SNHL and shows that the phenotypic spectrum of DFNB12 can be broadened even into the presbycusis, depending on the pathogenic potential of variants. We also propose that pathogenic potential of CDH23 variants and the clinical fate of DFNB12 may be predicted by MAF. PMID:27792758

  5. Analysis of systematic effects on congenital sensorineural deafness in German Dalmatian dogs.

    PubMed

    Juraschko, K; Meyer-Lindenberg, A; Nolte, I; Distl, O

    2003-09-01

    We have analysed the systematic influences, phenotypic colour markers and the additive genetic variation for congenital sensorineural deafness (CSD) in German Dalmatian dogs in order to help elucidate the importance of phenotypic breed characteristics for genetic differences of CSD. Linear animal models using restricted maximum likelihood methods were employed to estimate variance components. Data were obtained from all three German Dalmatian kennel clubs associated with the German Association for Dog Breeding and Husbandry (VDH). CSD was recorded by standardized protocols for brainstem auditory-evoked response (BAER). The material included 1899 German Dalmatian dogs from 354 litters in 169 different kennels. BAER testing results were from the years 1986 to 1999. Pedigree information was available for up to seven generations. The animal model regarded the fixed effects of sex, coat colour, eye colour, presence of patches, litter size, percentage of examined puppies per litter, kennel club, and inbreeding coefficient. The common environment of the litter and kennel as well as the additive genetic effect of the animal were taken into account as randomly distributed effects. The fixed effects of eye colour, percentage of puppies examined per litter and kennel club were significant in the mixed model analysis. A significant proportion of additive genetic variation could be shown despite corrections for phenotypic colour variants. The heritability estimate for CSD in German Dalmatian dogs was h(2)=0.27+/-0.07. The additive genetic correlation of CSD with presence of blue eyes was r(g)=0.53+/-0.41 and with presence of patches r(g)=-0.36+/-0.24. We concluded that additional genes other than those associated with phenotypic colour markers in German Dalmatian dogs significantly contribute to the occurrence of CSD.

  6. [Phenotypic trends and breeding values for canine congenital sensorineural deafness in Dalmatian dogs].

    PubMed

    Blum, Meike; Distl, Ottmar

    2014-01-01

    In the present study, breeding values for canine congenital sensorineural deafness, the presence of blue eyes and patches have been predicted using multivariate animal models to test the reliability of the breeding values for planned matings. The dataset consisted of 6669 German Dalmatian dogs born between 1988 and 2009. Data were provided by the Dalmatian kennel clubs which are members of the German Association for Dog Breeding and Husbandry (VDH). The hearing status for all dogs was evaluated using brainstem auditory evoked potentials. The reliability using the prediction error variance of breeding values and the realized reliability of the prediction of the phenotype of future progeny born in each one year between 2006 and 2009 were used as parameters to evaluate the goodness of prediction through breeding values. All animals from the previous birth years were used for prediction of the breeding values of the progeny in each of the up-coming birth years. The breeding values based on pedigree records achieved an average reliability of 0.19 for the future 1951 progeny. The predictive accuracy (R2) for the hearing status of single future progeny was at 1.3%. Combining breeding values for littermates increased the predictive accuracy to 3.5%. Corresponding values for maternal and paternal half-sib groups were at 3.2 and 7.3%. The use of breeding values for planned matings increases the phenotypic selection response over mass selection. The breeding values of sires may be used for planned matings because reliabilities and predictive accuracies for future paternal progeny groups were highest.

  7. Sensorineural hearing loss amplifies neural coding of envelope information in the central auditory system of chinchillas.

    PubMed

    Zhong, Ziwei; Henry, Kenneth S; Heinz, Michael G

    2014-03-01

    People with sensorineural hearing loss often have substantial difficulty understanding speech under challenging listening conditions. Behavioral studies suggest that reduced sensitivity to the temporal structure of sound may be responsible, but underlying neurophysiological pathologies are incompletely understood. Here, we investigate the effects of noise-induced hearing loss on coding of envelope (ENV) structure in the central auditory system of anesthetized chinchillas. ENV coding was evaluated noninvasively using auditory evoked potentials recorded from the scalp surface in response to sinusoidally amplitude modulated tones with carrier frequencies of 1, 2, 4, and 8 kHz and a modulation frequency of 140 Hz. Stimuli were presented in quiet and in three levels of white background noise. The latency of scalp-recorded ENV responses was consistent with generation in the auditory midbrain. Hearing loss amplified neural coding of ENV at carrier frequencies of 2 kHz and above. This result may reflect enhanced ENV coding from the periphery and/or an increase in the gain of central auditory neurons. In contrast to expectations, hearing loss was not associated with a stronger adverse effect of increasing masker intensity on ENV coding. The exaggerated neural representation of ENV information shown here at the level of the auditory midbrain helps to explain previous findings of enhanced sensitivity to amplitude modulation in people with hearing loss under some conditions. Furthermore, amplified ENV coding may potentially contribute to speech perception problems in people with cochlear hearing loss by acting as a distraction from more salient acoustic cues, particularly in fluctuating backgrounds.

  8. Sensorineural hearing loss and status epilepticus associated with ulcerative colitis: Is there enough evidence to support immune-related mechanisms?

    PubMed Central

    Yazici, Sinem; Benbir, Gulcin; Ince, Birsen

    2015-01-01

    Ulcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms. PMID:25552855

  9. Bilateral sensorineural deafness and hydrocephalus due to foramen of Monro obstruction in sibs: A newly described autosomal recessive disorder

    SciTech Connect

    Chudley, A.E.; McCullough, C.; McCullough, D.W.

    1997-01-31

    We identified a Canadian-Mennonite family in which a brother and sister have hydrocephalus due to obstruction at the foramen of Monro and profound bilateral sensorineural deafness. This appears to be a unique combination of anomalies and, to our knowledge, has not been reported previously. Both parents and a brother are phenotypically normal. The parents are second cousins. Thus, on the basis of consanguinity, affected sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. 37 refs., 5 figs.

  10. A role of the microphthalmia-associated transcription factor in congenital sensorineural deafness and eye pigmentation in Dalmatian dogs.

    PubMed

    Stritzel, S; Wöhlke, A; Distl, O

    2009-02-01

    Microphthalmia-associated transcription factor (MITF) is involved in white spotting and deafness associated with lack of pigmentation in human and mice. In the present study, we employed MITF-associated markers to evaluate MITF as a candidate for canine congenital sensorineural deafness (CCSD) in Dalmatian dogs. We performed an association study using MITF flanking and intragenic markers for 88 Dalmatian dogs of different hearing and eye pigmentation status. A significant association was identified for MITF-related markers with CCSD and blue iris colour. We conclude that MITF might play a role in CCSD and blue eye colour in Dalmatian dogs.

  11. Acute pancreatitis, acute hepatitis and acute renal failure favourably resolved in two renal transplant recipients.

    PubMed

    Voiculescu, Mihai; Ionescu, Camelia; Ismail, Gener; Mandache, Eugen; Hortopan, Monica; Constantinescu, Ileana; Iliescu, Olguta

    2003-03-01

    Renal transplantation is often associated with severe complications. Except for acute rejection, infections and toxicity of immunosuppressive treatment are the most frequent problems observed after transplantation. Infections with hepatic viruses (HBV, HDV, HCV, HGV) and cytomegalic virus (CMV) are the main infectious complications after renal transplantation. Cyclosporine toxicity is not unusual for a patient with renal transplantation and is even more frequent for patients with hepatic impairment due to viral infections. The subjects of this report are two renal transplant recipients with acute pancreatitis, severe hepatitis and acute renal failure on graft, receiving immunosuppressive therapy for maintaining renal graft function

  12. Impaired hepatitis C virus (HCV)-specific effector CD8+ T cells undergo massive apoptosis in the peripheral blood during acute HCV infection and in the liver during the chronic phase of infection.

    PubMed

    Radziewicz, Henry; Ibegbu, Chris C; Hon, Huiming; Osborn, Melissa K; Obideen, Kamil; Wehbi, Mohammad; Freeman, Gordon J; Lennox, Jeffrey L; Workowski, Kimberly A; Hanson, Holly L; Grakoui, Arash

    2008-10-01

    A majority of patients infected with hepatitis C virus (HCV) do not sustain an effective T-cell response, and viremia persists. The mechanism leading to failure of the HCV-specific CD8(+) T-cell response in patients developing chronic infection is unclear. We investigated apoptosis susceptibility of HCV-specific CD8(+) T cells during the acute and chronic stages of infection. Although HCV-specific CD8(+) T cells in the blood during the acute phase of infection and in the liver during the chronic phase were highly activated and expressed an effector phenotype, the majority was undergoing apoptosis. In contrast, peripheral blood HCV-specific CD8(+) T cells during the chronic phase expressed a resting memory phenotype. Apoptosis susceptibility of HCV-specific CD8(+) T cells was associated with very high levels of programmed death-1 (PD-1) and low CD127 expression and with significant functional T-cell deficits. Further evaluation of the "death phase" of HCV-specific CD8(+) T cells during acute HCV infection showed that the majority of cells were dying by a process of cytokine withdrawal, mediated by activated caspase 9. Contraction during the acute phase occurred rapidly via this process despite the persistence of the virus. Remarkably, in the chronic phase of HCV infection, at the site of infection in the liver, a substantial frequency of caspase 9-mediated T-cell death was also present. This study highlights the importance of cytokine deprivation-mediated apoptosis with consequent down-modulation of the immune response to HCV during acute and chronic infections.

  13. Contiguous deletion of SLC6A8 and BAP31 in a patient with severe dystonia and sensorineural deafness.

    PubMed

    Osaka, Hitoshi; Takagi, Atsushi; Tsuyusaki, Yu; Wada, Takahito; Iai, Mizue; Yamashita, Sumimasa; Shimbo, Hiroko; Saitsu, Hirotomo; Salomons, Gajja S; Jakobs, Cornelis; Aida, Noriko; Toshihiro, Shinka; Kuhara, Tomiko; Matsumoto, Naomichi

    2012-05-01

    We report here a 6-year-old boy exhibiting severe dystonia, profound intellectual and developmental disability with liver disease, and sensorineural deafness. A deficient creatine peak in brain (1)H-MR spectroscopy and high ratio of creatine/creatinine concentration in his urine lead us to suspect a creatine transporter (solute carrier family 6, member 8; SLC6A8) deficiency, which was confirmed by the inability to take up creatine into fibroblasts. We found a large ~19 kb deletion encompassing exons 5-13 of SLC6A8 and exons 5-8 of the B-cell receptor-associated protein (BAP31) gene. This case is the first report in which the SLC6A8 and BAP31 genes are both deleted. The phenotype of BAP31 mutations has been reported only as a part of Xq28 deletion syndrome or contiguous ATP-binding cassette, sub-family D, member 1 (ABCD1)/DXS1375E (BAP31) deletion syndrome [MIM ID #300475], where liver dysfunction and sensorineural deafness have been suggested to be attributed to the loss of function of BAP31. Our case supports the idea that the loss of BAP31 is related to liver dysfunction and hearing loss.

  14. Binaural speech discrimination under noise in hearing-impaired listeners

    NASA Technical Reports Server (NTRS)

    Kumar, K. V.; Rao, A. B.

    1988-01-01

    This paper presents the results of an assessment of speech discrimination by hearing-impaired listeners (sensori-neural, conductive, and mixed groups) under binaural free-field listening in the presence of background noise. Subjects with pure-tone thresholds greater than 20 dB in 0.5, 1.0 and 2.0 kHz were presented with a version of the W-22 list of phonetically balanced words under three conditions: (1) 'quiet', with the chamber noise below 28 dB and speech at 60 dB; (2) at a constant S/N ratio of +10 dB, and with a background white noise at 70 dB; and (3) same as condition (2), but with the background noise at 80 dB. The mean speech discrimination scores decreased significantly with noise in all groups. However, the decrease in binaural speech discrimination scores with an increase in hearing impairment was less for material presented under the noise conditions than for the material presented in quiet.

  15. Impaired Waters and TMDLs

    EPA Pesticide Factsheets

    The 303(d) program provides guidance and technical resources to assist States in submitting lists of impaired waterbodies and the development of Total Maximum Daily Loads of the pollutant causing the impairment.

  16. Comparison of two different steroid treatments with hyperbaric oxygen for idiopathic sudden sensorineural hearing loss.

    PubMed

    Sevil, Ergun; Bercin, Sami; Muderris, Togay; Gul, Fatih; Kiris, Muzaffer

    2016-09-01

    The purpose of the study was to assess the efficacy of the association of intratympanic (IT) steroid and hyperbaric oxygen (HBO) therapy in patients presenting with idiopathic sudden sensorineural hearing loss (ISSNHL), and to compare this protocol with another consisting of intravenous (IV) steroid administration and HBO therapy. A total of 80 patients diagnosed with ISSNHL were included in this prospective trial. Patients were divided into three categories: a mild-to-moderate ISSNHL group with a pure-tone average (PTA) ≤60 decibels (dB), a severe ISSNHL group with a PTA of 60-80 dB, and a profound ISSNHL group with a PTA ≥81 dB. The first protocol consisted of 20 sessions of HBO therapy together with IV methylprednisolone 1 mg/kg body weight and a 10 mg taper every 3 days for 10 days. The second protocol consisted of HBO therapy for 20 sessions, together with an IT injection of dexamethasone at a dose of 4 mg/mL, 0.5-0.7 mL once a day for 7 consecutive days, performed 3 h before the HBO therapy. In the mild-to-moderate ISSNHL patients, the mean hearing gain and successful treatment rate was 19 (0-27) dB and 78.9 %, respectively in the IT + HBO treatment group, and 18 (3-44) dB and 70.5 % in the IV + HBO therapy group. In the severe ISSNHL patients, the mean hearing gain and successful treatment rate was 33 (1-54) dB and 81.8 %, respectively in the IT + HBO treatment group and 33.5 (7-57) dB and 58.2 % in the IV + HBO group. In the profound ISSNHL patients, the mean hearing gain and successful treatment rate was 36 (4-69) dB and 40 %, respectively in the IT + HBO therapy group, and 39.5 (0-92) dB and 72.7 % in the IV + HBO treatment group. The results demonstrated that patients with severe hearing loss success rate was superior in the group submitted to IT + HBO treatment, conversely IV + HBO therapy may be benefit for patients with profound hearing loss. Nevertheless, these clinical results were not statistically significant.

  17. [Effect of cochlear implantation on sound localization for patients with unilateral sensorineural hearing loss].

    PubMed

    Liu, J F; Dai, J S; Wang, N Y

    2016-08-07

    The aim of this review was to examine the current literature regarding application of cochlear implantation on patients with unilateral sensorineural hearing loss (USNHL) for improvement on sound localization. The literature were searched in the PubMed database with 'cochlear implantation AND single-sided deafness' or 'cochlear implantation AND unilateral deafness' as keywords. The publication date of the articles was up to 2015-2-12. A total of 12 articles were included. The results show that the ability of sound localization for most of the USNHL subjects (90%) with cochlear implantation was significantly improved than that without CI, which suggests that CI is a superior auditory rehabilitation treatment than BAHA and CROS hearing aids for patients with USNHL, because of the re-establishment of the benefits of binaural hearing. In addition, the benefit of CI for USNHL requires a period of auditory experience or training. About 30% subjects showed significantly improvement on sound localization ability after CI worked for three months. For most of the patients (90%), the sound localization ability improved after CI worked for six months. When CI worked for nine months, all the subjects would show improvement on sound localization ability. Sound localization of the USNHL subjects with a CI is based primarily on interaural level differences (ILD) while interaural time differences (ITD) provide little advantage or probably not perceptible at all. The younger subject suffers from USNHL, the stronger the plasticity of the auditory center shows, which results in more obvious degeneration of the affected side and adaptive enhancement of the contralateral side of the auditory pathway. Similarly, the longer duration of USNHL lead to more obvious degeneration of the affected side and adaptive enhancement of the contralateral side. An adaptive enhancement of auditory pathway corresponding to the healthy ear will rely more on the monaural spatial cues that available to the

  18. Vitamins A, C, and E and selenium in the treatment of idiopathic sudden sensorineural hearing loss.

    PubMed

    Kaya, Hakan; Koç, Arzu Karaman; Sayın, İbrahim; Güneş, Selçuk; Altıntaş, Ahmet; Yeğin, Yakup; Kayhan, Fatma Tülin

    2015-05-01

    This study evaluated the effectiveness of vitamins A, C, and E, with selenium, in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). This was a prospective, controlled study performed at a tertiary teaching and research hospital. Over a 32-month period, patients were treated with either our standard ISSNHL treatment regimen plus vitamins A, C, and E and selenium (ACE+ group) or with only our standard ISSNHL treatment regimen (ACE- group). The demographics, additional symptoms, mean initial and final hearing levels, mean hearing gain, and recovery data were compared between the two groups. The ACE+ group, consisting of 70 (55.5 %) patients, received vitamin A (natural beta-carotene, 26,000 IU), vitamin C (ascorbic acid, 200 mg), vitamin E (d-alpha-tocopherol, 200 IU), and selenium (50 μg) twice daily for 30 days in addition to our ISSNHL treatment regimen: methylprednisolone at an initial dose of 1 mg/kg body weight per day, tapered over 14 days; Rheomacrodex(®) [(10 g of dextran and 0.9 g of NaCl)/100 ml] 500 ml daily for 5 days; Vastarel(®) 20-mg tablet (20 mg of trimetazidine dihydrochloride) three times daily for 30 days; and ten 60-min hyperbaric oxygen (HBO) sessions (2.5 absolute atmospheres of 100 % O2), once daily, starting the day of hospitalization. The ACE- group comprised 56 (44.4 %) patients, who received only our ISSNHL treatment regimen. The mean hearing gains were 36.2 ± 20.3 dB in the ACE+ group and 27.1 ± 20.6 dB in the ACE- group. The mean hearing gain rates were significantly higher in the ACE+ group than in the ACE- group (p = 0.014). Treatment with vitamins A, C, and E and selenium was effective in ISSNHL patients undergoing treatment with methylprednisolone, dextran, trimetazidine dihydrochloride, and HBO, and might be more effective when the initial hearing level is below 46 dB.

  19. Acute Bronchitis

    MedlinePlus

    ... can also cause acute bronchitis. To diagnose acute bronchitis, your health care provider will ask about your symptoms and listen to your breathing. You may also have other tests. Treatments include rest, fluids, and aspirin (for adults) or ...

  20. Adapting for Impaired Patrons.

    ERIC Educational Resources Information Center

    Schuyler, Michael

    1999-01-01

    Describes how a library, with an MCI Corporation grant, approached the process of setting up computers for the visually impaired. Discusses preparations, which included hiring a visually-impaired user as a consultant and contacting the VIP (Visually Impaired Persons) group; equipment; problems with the graphical user interface; and training.…

  1. Epiphyseal dysplasia of the femoral head, mild vertebral abnormality, myopia, and sensorineural deafness: report of a pedigree with autosomal dominant inheritance.

    PubMed Central

    MacDermot, K D; Roth, S C; Hall, C; Winter, R M

    1987-01-01

    A family is presented with short stature, femoral epiphyseal dysplasia, mild vertebral changes, and sensorineural deafness inherited as an autosomal dominant trait. Myopia and retinal detachment presenting in adult life were also present in some affected members. We suggest that this disorder may be a distinct entity within the spondyloepiphyseal dysplasia group of disorders. Images PMID:3681905

  2. Phonology Matters: A Comprehensive Investigation of Reading and Spelling Skills of School-Age Children with Mild to Moderate Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Park, Jungjun; Lombardino, Linda J.; Ritter, Michaela

    2013-01-01

    The investigators measured 7 literacy skills in a group of 21 school-age children with mild to moderate sensorineural hearing loss (MSNH group), and compared the scores to those of 2 age-matched groups: children with dyslexia (DYS group) and, as a control, typically developing hearing children (CA group). The MSNH group performed consistently…

  3. Effect of Early Intervention on Language Development in Hearing-Impaired Children

    PubMed Central

    Shojaei, Elahe; Jafari, Zahra; Gholami, Maryam

    2016-01-01

    Introduction: Hearing loss from birth up to the age of 3 years has a negative effect on speech/language development and results in sensory, cognitive, emotional, and academic defects in adulthood by causing delayed development of communicative-linguistic abilities. The present study was performed in order to assess the effect of early intervention on language development in Persian children aged 6-7 years with severe sensorineural hearing loss. Materials and Methods: Thirty boys and girls aged 6-7 years participated in this study, all of them had severe congenital sensorineural hearing loss in both ears. All children were using bilateral behind-the-ear hearing aid, and had similar economic/socio-cultural backgrounds. Subjects were categorized into two groups based on the age of identification/intervention of hearing loss (3-6 and 12-15 months of age). The Persian TOLD-P3 test was used to evaluate language development in all subjects. Data collection was accomplished by observation, completion of questionnaires, and speech recording. Results: There was a significant difference in language development in 11 sub-tests and five lingual gains on the Persian TOLD-P3 test between early (3-6 months of age) and late identified/intervened (12-15 months of age) hearing-impaired children (P<0.05). Early identified/intervened hearing-impaired children had a notable preference in all assessed sub-tests and lingual gains. Conclusion: Early identification/intervention of hearing loss before the age of 6 months has a significant positive effect on a child’s language development in terms of picture/relational/oral vocabulary, grammatical comprehension, sentence combining, grammatical completion, phonologic analysis, word differentiation, word production, semantics, and syntax. Moreover, early identification/ intervention of hearing loss develops the hearing-impaired child’s lingual gains in visual vocabulary, grammatical completion, word differentiation, phonologic analysis, and

  4. Acute disseminated encephalomyelitis associated with acute Toxoplasma gondii Infection.

    PubMed

    Aksoy, Ayse; Tanir, Gonul; Ozkan, Mehpare; Oguz, Melek; Yıldız, Yasemin Tasci

    2013-03-01

    Acute disseminated encephalomyelitis is an acute demyelinating disorder of the central nervous system, which principally affects the brain and spinal cord. It usually follows a benign infection or vaccination in children. Although a number of infectious agents have been implicated in acute disseminated encephalomyelitis, Toxoplasma gondii infection has not been described previously in children. Acquired T. gondii infection presents with lymphadenopathy and fever and usually spontaneously resolves in immunocompetent patients. We describe a previously healthy 10-year-old boy with acute disseminated encephalomyelitis associated with acute acquired Toxoplasma gondii infection, the symptoms of which initially began with nuchal stiffness, difficulty in walking, and urinary and stool incontinence; he later had development of motor and sensory impairment in both lower extremities and classical magnetic resonance imaging lesions suggestive of the disease. The patient recovered completely after the specific therapy for acquired T. gondii infection and pulse prednisolone. Although acute acquired Toxoplasma gondii infection has not been reported previously in association with acute disseminated encephalomyelitis, clinicians should keep in mind this uncommon cause of a common disease when evaluating a patient with acute disseminated encephalomyelitis.

  5. A novel missense mutation in ACTG1 causes dominant deafness in a Norwegian DFNA20/26 family, but ACTG1 mutations are not frequent among families with hereditary hearing impairment.

    PubMed

    Rendtorff, Nanna D; Zhu, Mei; Fagerheim, Toril; Antal, Torben L; Jones, MaryPat; Teslovich, Tanya M; Gillanders, Elizabeth M; Barmada, Michael; Teig, Erik; Trent, Jeffrey M; Friderici, Karen H; Stephan, Dietrich A; Tranebjaerg, Lisbeth

    2006-10-01

    The gamma-actin gene (ACTG1) encodes a major cytoskeletal protein of the sensory hair cells of the cochlea. Recently, mutations in ACTG1 were found to cause autosomal dominant, progressive, sensorineural hearing impairment linked to the DFNA20/26 locus on chromosome 17q25.3 in four American families and in one Dutch family. We report here the linkage of autosomal dominant, progressive, sensorineural hearing impairment in a large Norwegian family to the DFNA20/26 locus. Sequencing of ACTG1 identified a novel missense mutation (c.1109T>C; p.V370A) segregating with the hearing loss. Functional analysis in yeast showed that the p.V370A mutation restricts cell growth at elevated temperature or under hyperosmolar stress. Molecular modelling suggested that the p.V370A mutation modestly alters a site for protein-protein interaction in gamma-actin and thereby modestly alters gamma-actin-based cytoskeletal structures. Nineteen Norwegian and Danish families with autosomal, dominant hearing impairment were analyzed for mutations in ACTG1 by sequencing, but no disease-associated mutations were identified. Finally, a long-term follow-up of the hearing loss progression associated with the p.V370A mutation in ACTG1 is provided. The present study expands our understanding of the genotype-phenotype relationship of this deafness gene and provides a sensitive and simple functional assay for missense mutations in this gene, which may assist future molecular diagnosis of autosomal-dominant hearing impairment. Finally, the present results do not indicate that mutations in ACTG1 are a frequent cause of autosomal-dominant postlingual sensorineural hearing impairment in Norway nor Denmark.

  6. Memory Impairment in Children with Language Impairment

    ERIC Educational Resources Information Center

    Baird, Gillian; Dworzynski, Katharina; Slonims, Vicky; Simonoff, Emily

    2010-01-01

    Aim: The aim of this study was to assess whether any memory impairment co-occurring with language impairment is global, affecting both verbal and visual domains, or domain specific. Method: Visual and verbal memory, learning, and processing speed were assessed in children aged 6 years to 16 years 11 months (mean 9y 9m, SD 2y 6mo) with current,…

  7. Visual impairment in the hearing impaired students

    PubMed Central

    Gogate, Parikshit; Rishikeshi, Nikhil; Mehata, Reshma; Ranade, Satish; Kharat, Jitesh; Deshpande, Madan

    2009-01-01

    Background: Ocular problems are more common in children with hearing problems than in normal children. Neglected visual impairment could aggravate educational and social disability. Aim: To detect and treat visual impairment, if any, in hearing-impaired children. Setting and Design: Observational, clinical case series of hearing-impaired children in schools providing special education. Materials and Methods: Hearing-impaired children in selected schools underwent detailed visual acuity testing, refraction, external ocular examination and fundoscopy. Ocular motility testing was also performed. Teachers were sensitized and trained to help in the assessment of visual acuity using Snellen's E charts. Refractive errors and squint were treated as per standard practice. Statistical Analysis: Excel software was used for data entry and SSPS for analysis. Results: The study involved 901 hearing-impaired students between four and 21 years of age, from 14 special education schools. A quarter of them (216/901, 24%) had ocular problems. Refractive errors were the most common morbidity 167(18.5%), but only 10 children were using appropriate spectacle correction at presentation. Fifty children had visual acuity less than 20/80 at presentation; after providing refractive correction, this number reduced to three children, all of whom were provided low-vision aids. Other common conditions included strabismus in 12 (1.3%) children, and retinal pigmentary dystrophy in five (0.6%) children. Conclusion: Ocular problems are common in hearing-impaired children. Screening for ocular problems should be made mandatory in hearing-impaired children, as they use their visual sense to compensate for the poor auditory sense. PMID:19861747

  8. Frequency Discrimination and Literacy Skills in Children with Mild to Moderate Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Halliday, L. F.; Bishop, D. V. M.

    2005-01-01

    It has been suggested that specific reading disability (SRD) may be attributable to an impaired ability to perceive spectral differences between sounds that leads to a deficit in frequency discrimination and subsequent problems with language and literacy. The objective of the present study was threefold. We aimed to (a) determine whether children…

  9. Predicted effects of sensorineural hearing loss on across-fiber envelope coding in the auditory nervea

    PubMed Central

    Swaminathan, Jayaganesh; Heinz, Michael G.

    2011-01-01

    Cross-channel envelope correlations are hypothesized to influence speech intelligibility, particularly in adverse conditions. Acoustic analyses suggest speech envelope correlations differ for syllabic and phonemic ranges of modulation frequency. The influence of cochlear filtering was examined here by predicting cross-channel envelope correlations in different speech modulation ranges for normal and impaired auditory-nerve (AN) responses. Neural cross-correlation coefficients quantified across-fiber envelope coding in syllabic (0–5 Hz), phonemic (5–64 Hz), and periodicity (64–300 Hz) modulation ranges. Spike trains were generated from a physiologically based AN model. Correlations were also computed using the model with selective hair-cell damage. Neural predictions revealed that envelope cross-correlation decreased with increased characteristic-frequency separation for all modulation ranges (with greater syllabic-envelope correlation than phonemic or periodicity). Syllabic envelope was highly correlated across many spectral channels, whereas phonemic and periodicity envelopes were correlated mainly between adjacent channels. Outer-hair-cell impairment increased the degree of cross-channel correlation for phonemic and periodicity ranges for speech in quiet and in noise, thereby reducing the number of independent neural information channels for envelope coding. In contrast, outer-hair-cell impairment was predicted to decrease cross-channel correlation for syllabic envelopes in noise, which may partially account for the reduced ability of hearing-impaired listeners to segregate speech in complex backgrounds. PMID:21682421

  10. [Efficacy of intratympanic steroid treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous steroid treatment].

    PubMed

    Kawano, Toshiro; Matsuura, Masaki; Ishitoya, Junichi; Oridate, Nobuhiko

    2014-06-01

    This study investigated the efficacy of intratympanic steroid (ITS) therapy as a salvage treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous steroid (IVS) therapy. Systemic steroid therapy is the only standard drug therapy. However, ethically, we could not simply compare ITS with IVS. Conventionally, we have treated idiopahic sudden sensorineural hearing loss patients after failure of systemic steroid therapy with the double combined therapy IVS and hyperbaric oxygen (HBO), as the salvage modality. We examined the effect of ITS by adding it to the double combined therapy with IVS and HBO. Retrospectively, we clinically examined the effect of double combined therapy with IVS and HBO (A group) for 31 patients (12 men and 19 women) (median age: 54 years) with sudden hearing loss after failure of systemic steroid therapy between June, 2003 and July, 2010. Prospectively, we also examined clinically the effect of triple combined therapy with IVS and HBO, ITS (B group) for 29 patients (17 men and 12 women) (median age: 51 years) with sudden hearing loss after failure of systemic steroid therapy between August, 2010 and April, 2012. In the examination of patients treated within 30 days from the onset, one patient (3.2%) demonstrated remarkable recovery, 6 patients (19.4%) demonstrated mild recovery, while no change was noted in 24 patients (77.4%) in the A group. In the B group, 5 patients (17.2%) demonstrated complete recovery, 3 patients (10.3%) demonstrated remarkable recovery, mild recovery was seen in 14 patients (48.3%), and the remaining 7 patients (24.1%) showed no change. There was a significant difference (p < 0.05) between the A group and the B group. Furthermore, the hearing improvement in group B in five pure tone average was significantly better than in the group A (p < 0.05). We concluded that the B group demonstrated better hearing improvement than the A group. Therefore, ITS could be effective for idiopathic sudden

  11. [Acute muscle weakness: differential diagnoses].

    PubMed

    Antoniuk, Sérgio A

    2013-09-06

    Acute muscle weakness, a common disorder in pediatrics, can occur from impairment of any part of the motor unit, including the upper motor neuron, lower motor neuron, peripheral nerve, neuromuscular junction or muscle. It usually manifests itself as an acute or hyperacute motor disorder of progressive or rapidly progressive course. Acute muscle weakness is a neuromuscular emergency, especially if it affects the respiratory or oropharyngeal musculature. The location of the motor weakness and associated neurological signs and symptoms usually indicate the location of the lesion. The onset, speed and clinical evolution, as well as other data from the patient's history, suggest the pathophysiological differential diagnosis. Successful treatment depends on the immediate and correct differential diagnosis. This paper presents the main differential diagnosis of main neuromuscular diseases that cause acute muscle weakness in children.

  12. Associations between hand-wrist musculoskeletal and sensorineural complaints and biomechanical and vibration work constraints.

    PubMed

    Malchaire, J; Piette, A; Cock, N

    2001-08-01

    A 3-year prospective epidemiological study was conducted to investigate the relationship between musculoskeletal complaints (MS) and sensorineural complaints (SN) of the workers in the hand-wrist region. A group of 69 workers (G1) using vibrating tools in eight different working situations was compared to a group of 62 workers (G2) performing heavy work without vibration and 46 workers (G3) performing light work without vibration. Biomechanical constraints (force, postures, repetitiveness and movement velocities) were analysed for each working situation and the vibration exposure at the eight workplaces with the 69 workers. MS and SN data were collected using the nordic questionnaire, modified to collect information about the frequency intensity and duration of complaints. The prevalence of complaints at the start of the study was significantly greater for G1 (72.5%) than for G2 (56.5%), itself greater than for G3 (30.4%). The prevalence of SN was about 40% for G1 and 2.5 times smaller in the two other groups. During the two years follow-up, new cases of 'serious' MS and SN developed. The annual incidence was respectively 8.3 and 5.4% on average. The incidence of MS was slightly but not statistically significantly greater for G1, while the incidence of SN was statistically higher (P<0.01) for G1 (10.9%) than for the two other groups (4.1 and 2.1%). Forces and angular repetitiveness were the only biomechanical factors significantly greater for G1. The vibration exposure duration of the G1 workers varied, in average, from 10 to 70% of the work time and the weighted personal exposure amplitude (A(EPw)) varied from 0.5 to 25.4 ms(-2). The probabilities of complaints at the beginning of the study (cross-sectional study) were estimated using multiple logistic regression models. The prevalence odds ratio (POR) for MS was equal to 4 for G1 compared to G2 and equal to 9 compared to G3. Force and vibration exposure were the main constraint parameters associated with this

  13. A novel biallelic splice site mutation of TECTA causes moderate to severe hearing impairment in an Algerian family.

    PubMed

    Behlouli, Asma; Bonnet, Crystel; Abdi, Samia; Hasbellaoui, Mokhtar; Boudjenah, Farid; Hardelin, Jean-Pierre; Louha, Malek; Makrelouf, Mohamed; Ammar-Khodja, Fatima; Zenati, Akila; Petit, Christine

    2016-08-01

    Congenital deafness is certainly one of the most common monogenic diseases in humans, but it is also one of the most genetically heterogeneous, which makes molecular diagnosis challenging in most cases. Whole-exome sequencing in two out of three Algerian siblings affected by recessively-inherited, moderate to severe sensorineural deafness allowed us to identify a novel splice donor site mutation (c.5272+1G > A) in the gene encoding α-tectorin, a major component of the cochlear tectorial membrane. The mutation was present at the homozygous state in the three affected siblings, and at the heterozygous state in their unaffected, consanguineous parents. To our knowledge, this is the first reported TECTA mutation leading to the DFNB21 form of hearing impairment among Maghrebian individuals suffering from congenital hearing impairment, which further illustrates the diversity of the genes involved in congenital deafness in the Maghreb.

  14. A Novel De Novo Dominant Mutation in GJB2 Gene Associated with a Sporadic Case of Nonsyndromic Sensorineural Hearing Loss.

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-12-01

    Mutations in the GJB2 gene are the most common known cause of hereditary congenital hearing loss. Rapid genomic DNA extraction (RGDE) method was used for genomic DNA extraction. After amplification of coding region of CX26 gene with specific primers, expected PCR products with 724bp length were subjected to direct sequencing in both directions. We describe here a novel heterozygous -T to -C transition at codon 202 (TGC→CGC) of the GJB2 gene in a patient, 40-year-old Iranian woman, which replaces a cysteine with an arginine residue (C202R). The dominant mutation C202R associated with non-syndromic sensorineural hearing loss. This mutation has not previously been described in affected or control samples from other populations investigated for GJB2 mutations, indicating that it is a rare substitution. This dominant mutation was recorded in NCBI GenBank with accession number KF 638275.

  15. Complete recovery following hyperbaric oxygen therapy in idiopathic sudden sensorineural hearing loss--a report of two cases.

    PubMed

    Agrawal, Swati; Sharma, Nishi

    2016-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL), a common otologic emergency, presents mostly as an abrupt onset unilateral hearing loss, aural fullness, often with vertigo and tinnitus, usually upon awakening in the morning. Its etiopathogenesis is multifactorial, so a number of different therapeutic regimens are in practice, hyperbaric oxygen (HBO2) therapy being an effective yet underutilized one. Not all cases recover completely even after treatment. Here we describe two cases of ISSHL, diagnosed on the basis of clinical examination and pure-tone audiometry, who had a complete recovery following administration of HBO2 therapy in addition to medical treatment. These cases are reported to highlight the effectiveness of this modality in a physician's armamentarium.

  16. Sensorineural hearing loss in insulin-like growth factor I-null mice: a new model of human deafness.

    PubMed

    Cediel, R; Riquelme, R; Contreras, J; Díaz, A; Varela-Nieto, I

    2006-01-01

    It has been reported that mutations in the gene encoding human insulin-like growth factor-I (IGF-I) cause syndromic hearing loss. To study the precise role of IGF-I in auditory function and to hypothesize the possible morphological and electrophysiological changes that may occur in the human inner ear, we have analysed the auditory brainstem response in a mouse model of IGF-I deficiency. We show here that homozygous Igf-1(-/-) mice present an all-frequency involved bilateral sensorineural hearing loss. Igf-1(-/-) mice also present a delayed response to acoustic stimuli; this increases along the auditory pathway, indicating a contribution of the central nervous system to the hearing loss in Igf-1(-/-) mice. These results support the use of the Igf-1(-/-) mouse as a new model for the study of human syndromic deafness.

  17. Prevalence of GBJ2 mutations in patients with severe to profound congenital nonsyndromic sensorineural hearing loss in Bulgarian population.

    PubMed

    Popova, Diana P; Kaneva, Radka; Varbanova, Sonya; Popov, Todor M

    2012-06-01

    Objective of the study is to assess the prevalence of Connexin 26 (GJB2) mutation in patients with congenital nonsyndromic sensorineural hearing loss in Bulgarian population. Study design is done prospectively. Patient inclusion criteria for this study were diagnosis of congenital nonsyndromic hearing loss, and absence of potential sibling relationships between patients included in the study (anamnestic pedigree for at least three generations). Patients were excluded from the study group if one of the following conditions were present: secondary hearing loss (cytomegalovirus, rubella, meningo-encephalitis, mastoiditis, other infections, posterior fossa tumors, etc.), exposure to drugs or other prenatal or perinatal etiology of deafness, and congenital syndromic hearing loss. Genomic DNA samples from whole blood were tested with sequence analysis for mutations in the coding region of the GJB2. Results state that 51 patients were analyzed for GJB2 mutations. Twenty of the patients (39%) with mutant alleles were homozygous for the c.35delG mutation (c.35delG/c.35delG) and four patients (8%) presented as heterozygotes (c.35delG/WT). In one patient, who carried a heterozygous mutation c.35delG, a second mutation was found-312del114. Additionally, in two other patients were discovered the mutations Trp24X (W24X) and, respectively, Arg127His(R127H), both in heterozygous states. From the whole study group there was only one patient with compound heterozygous genotype-p.Leu90Pro(L90P)/p.Ile121Asn. The latter one has never been reported in the literature so far. In conclusion, this study determines the importance of connexin 26 mutations in Bulgarian children with severe to profound congenital nonsyndromic sensorineural hearing loss, the prevalence of the different mutation variants and their relationship with the ethnical background of the patients. In addition, we report for the first time a novel mutation in the GJB2 gene.

  18. Acute liver failure due to acute fatty liver of pregnancy.

    PubMed

    Wand, S; Waeschle, R M; Von Ahsen, N; Hawighorst, T; Bräuer, A; Quintel, M

    2012-04-01

    Acute fatty liver of pregnancy (AFLP) is a rare but serious liver disease and typically occurs during the third trimester. It carries the risk for significant perinatal and maternal mortality. Therefore an early diagnosis and delivery, followed by close monitoring and optimized management of the impaired liver function with all associated problems are necessary to prevent maternal and foetal death. This case report focuses on the management of acute liver failure due to AFLP in a 31 year old women treated in our intensive care unit (ICU) after an emergency C-section.

  19. The effect of symmetrical and asymmetrical hearing impairment on music quality perception.

    PubMed

    Cai, Yuexin; Zhao, Fei; Chen, Yuebo; Liang, Maojin; Chen, Ling; Yang, Haidi; Xiong, Hao; Zhang, Xueyuan; Zheng, Yiqing

    2016-09-01

    The purpose of this study was to investigate the effect of symmetrical, asymmetrical and unilateral hearing impairment on music quality perception. Six validated music pieces in the categories of classical music, folk music and pop music were used to assess music quality in terms of its 'pleasantness', 'naturalness', 'fullness', 'roughness' and 'sharpness'. 58 participants with sensorineural hearing loss [20 with unilateral hearing loss (UHL), 20 with bilateral symmetrical hearing loss (BSHL) and 18 with bilateral asymmetrical hearing loss (BAHL)] and 29 normal hearing (NH) subjects participated in the present study. Hearing impaired (HI) participants had greater difficulty in overall music quality perception than NH participants. Participants with BSHL rated music pleasantness and naturalness to be higher than participants with BAHL. Moreover, the hearing thresholds of the better ears from BSHL and BAHL participants as well as the hearing thresholds of the worse ears from BSHL participants were negatively correlated to the pleasantness and naturalness perception. HI participants rated the familiar music pieces higher than unfamiliar music pieces in the three music categories. Music quality perception in participants with hearing impairment appeared to be affected by symmetry of hearing loss, degree of hearing loss and music familiarity when they were assessed using the music quality rating test (MQRT). This indicates that binaural symmetrical hearing is important to achieve a high level of music quality perception in HI listeners. This emphasizes the importance of provision of bilateral hearing assistive devices for people with asymmetrical hearing impairment.

  20. Telephone speech enhancement for hearing-impaired listeners using multi-channel compression

    NASA Astrophysics Data System (ADS)

    Natarajan, Harikrishna P.; Krishnamurthy, Ashok K.; Feth, Lawrence L.

    2002-05-01

    Elderly listeners with sensorineural hearing impairment usually have difficulty with telephone communication if they do not use a hearing aid or telephone amplifier. In many cases, these devices may not be readily available or maybe uncomfortable to use. We describe here an alternative strategy to enhance the speech by pre-processing the signal before sending it over the telephone line. The pre-processing is based on a multi-channel compression algorithm that modifies the critical band spectrum of the speech signal to bring it within the dynamic hearing range of the listener, while also compensating for the effects of the telephone line. We describe the algorithm and the results of a simulation study in which the Articulation Index (AI) is used to measure the intelligibility enhancement, and the Glasberg, Moore and Stone (1999) model is used to simulate the effects of the hearing loss.

  1. A Physiological Signal Transmission Model to be Used for Specific Diagnosis of Cochlear Impairments

    NASA Astrophysics Data System (ADS)

    Saremi, Amin; Stenfelt, Stefan

    2011-11-01

    Many of the sophisticated characteristics of human auditory system are attributed to cochlea. Also, most of patients with a hearing loss suffer from impairments that originate from cochlea (sensorineural). Despite this, today's clinical diagnosis methods do not probe the specific origins of such cochlear lesions. The aim of this research is to introduce a physiological signal transmission model to be clinically used as a tool for diagnosis of cochlear losses. This model enables simulation of different bio-mechano-electrical processes which occur in the auditory organ of Corti inside the cochlea. What makes this model different from many available computational models is its loyalty to physiology since the ultimate goal is to model each single physiological phenomenon. This includes passive BM vibration, outer hair cells' performances such as nonlinear mechanoelectrical transduction (MET), active amplifications by somatic motor, as well as vibration to neural conversion at the inner hair cells.

  2. Acoustic Schwannoma Presenting as Acute Posterior Fossa Hematoma

    PubMed Central

    Ghobashy, Ashraf; Loveren, Harry van

    1993-01-01

    Acoustic schwannomas usually present with gradually progressive unilateral sensorineural hearing loss. As the tumor enlarges, symptoms and signs develop when the adjacent cranial nerves, cerebelhim, and/or brainstem become compressed. Rarely, acoustic tumors present with acute subarachnoid or intratumoral hemorrhage. Of the 12 cases of acoustic schwannoma with tumoral hemorrhage presented in the literature of which we are aware, this is the third such case of a patient presenting with spontaneous pure intratumoral hemorrhage and the first such case presenting with sudden multiple cranial nerve palsies, The purpose of this report is to increase the awareness of this rare form of presentation of acoustic schwannoma in the hope of achieving better preservation of cranial nerves. ImagesFigure 1Figure 2Figure 3 PMID:17170903

  3. Cognitive impairment and stroke in elderly patients

    PubMed Central

    Lo Coco, Daniele; Lopez, Gianluca; Corrao, Salvatore

    2016-01-01

    We reviewed current knowledge about the interaction between stroke and vascular risk factors and the development of cognitive impairment and dementia. Stroke is increasingly recognized as an important cause of cognitive problems and has been implicated in the development of both Alzheimer’s disease and vascular dementia. The prevalence of cognitive impairment after stroke is high, and their combined effects significantly increase the cost of care and health resource utilization, with reflections on hospital readmissions and increased mortality rates. There is also substantial evidence that vascular risk factors (such as hypertension, diabetes, obesity, dyslipidemia, and tobacco smoking) are independently associated with an increased risk of cognitive decline and dementia. Thus, a successful management of these factors, as well as optimal acute stroke management, might have a great impact on the development of cognitive impairment. Notwithstanding, the pathological link between cognitive impairment, stroke, and vascular risk factors is complex and still partially unclear so that further studies are needed to better elucidate the boundaries of this relationship. Many specific pharmacological treatments, including anticholinergic drugs and antihypertensive medications, and nonpharmacological approaches, such as diet, cognitive rehabilitation, and physical activity, have been studied for patients with vascular cognitive impairment, but the optimal care is still far away. Meanwhile, according to the most recent knowledge, optimal stroke care should also include cognitive assessment in the short and long term, and great efforts should be oriented toward a multidisciplinary approach, including quality-of-life assessment and support of caregivers. PMID:27069366

  4. Education for the Hearing Impaired (Auditorily Impaired).

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Education for the hearing impaired is discussed in nine conference papers. J. N. Howarth describes "The Education of Deaf Children in Schools for Hearing Pupils in the United Kingdom" and A.I.Dyachkov of the U.S.S.R. outlines Didactical Principles of Educating the Deaf in the Light of their Rehabilitation Goal." Seven papers from…

  5. Development or Impairment?

    ERIC Educational Resources Information Center

    Hakansson, Gisela

    2010-01-01

    Joanne Paradis' Keynote Article on bilingualism and specific language impairment (SLI) is an impressive overview of research in language acquisition and language impairment. Studying different populations is crucial both for theorizing about language acquisition mechanisms, and for practical purposes of diagnosing and supporting children with…

  6. Hereditary non-syndromic sensorineural hearing loss: transforming silence to sound.

    PubMed

    Schrijver, Iris

    2004-11-01

    Tremendous progress has been made in our understanding of the molecular basis of hearing and hearing loss. Through recent advances, we have begun to understand the fascinating biology of the auditory system and unveiled new molecular mechanisms of hearing impairment. Changes in the diagnostic impact of genetic testing have occurred, as well as exciting developments in therapeutic options. Molecular diagnosis, which is already a reality for several hearing-associated genes, will doubtlessly continue to increase in the near future, both in terms of the number of mutations tested and the spectrum of genes. Genetic analysis for hearing loss is mostly used for diagnosis and treatment, and relatively rarely for reproductive decisions, in contrast to other inherited disorders. Inherited hearing loss, however, is characterized by impressive genetic heterogeneity. An abundance of genes carry a large number of mutations, but specific mutations in a single gene may lead to syndromic or non-syndromic hearing loss. Some mutations predominate in individual ethnic groups. For clinical and laboratory diagnosticians, it is challenging to keep abreast of the unfolding discoveries. This review aims to provide the framework pertinent to diagnosticians and a practical approach to mutation analysis in the hearing impaired.

  7. Dorsal hippocampal NMDA receptor blockade impairs extinction of naloxone-precipitated conditioned place aversion in acute morphine-treated rats by suppressing ERK and CREB phosphorylation in the basolateral amygdala

    PubMed Central

    Wang, Wei-Sheng; Chen, Zhong-Guo; Liu, Wen-Tao; Chi, Zhi-Qiang; He, Ling; Liu, Jing-Gen

    2015-01-01

    BACKGROUND AND PURPOSE Substantial evidence shows that negative reinforcement resulting from the aversive affective consequences of opiate withdrawal may play a crucial role in drug relapse. Understanding the mechanisms underlying the loss (extinction) of conditioned aversion of drug withdrawal could facilitate the treatment of drug addiction. EXPERIMENTAL APPROACH Naloxone-induced conditioned place aversion (CPA) of Sprague-Dawley rats was used to measure conditioned aversion. An NMDA receptor antagonist and MAPK kinase inhibitor were applied through intracranial injections. The phosphorylation of ERK and cAMP response element-binding protein (CREB) was detected using Western blot. KEY RESULTS The extinction of CPA behaviour increased the phosphorylation of ERK and CREB in the dorsal hippocampus (DH) and basolateral amygdala (BLA), but not in the central amygdala (CeA). Intra-DH injection of AP5 or intra-BLA injection of AP-5 or U0126 before extinction training significantly attenuated ERK and CREB phosphorylation in the BLA and impaired the extinction of CPA behaviour. Although intra-DH injections of AP-5 attenuated extinction training-induced activation of the ERK-CREB pathway in the BLA, intra-BLA injection of AP5 had no effect on extinction training-induced activation of the ERK-CREB pathway in the DH. CONCLUSIONS AND IMPLICATIONS These results suggest that activation of ERK and CREB in the BLA and DH is involved in the extinction of CPA behaviour and that the DH, via a direct or indirect pathway, modulates the activity of ERK and CREB in the BLA through activation of NMDA receptors after extinction training. Understanding the mechanisms underlying the extinction of conditioned aversion could facilitate the treatment of drug addiction. LINKED ARTICLES This article is part of a themed section on Opioids: New Pathways to Functional Selectivity. To view the other articles in this section visit http://dx.doi.org/10.1111/bph.2015.172.issue-2 PMID:24597568

  8. Vascular Cognitive Impairment.

    PubMed

    Dichgans, Martin; Leys, Didier

    2017-02-03

    Cerebrovascular disease typically manifests with stroke, cognitive impairment, or both. Vascular cognitive impairment refers to all forms of cognitive disorder associated with cerebrovascular disease, regardless of the specific mechanisms involved. It encompasses the full range of cognitive deficits from mild cognitive impairment to dementia. In principle, any of the multiple causes of clinical stroke can cause vascular cognitive impairment. Recent work further highlights a role of microinfarcts, microhemorrhages, strategic white matter tracts, loss of microstructural tissue integrity, and secondary neurodegeneration. Vascular brain injury results in loss of structural and functional connectivity and, hence, compromise of functional networks within the brain. Vascular cognitive impairment is common both after stroke and in stroke-free individuals presenting to dementia clinics, and vascular pathology frequently coexists with neurodegenerative pathology, resulting in mixed forms of mild cognitive impairment or dementia. Vascular dementia is now recognized as the second most common form of dementia after Alzheimer's disease, and there is increasing awareness that targeting vascular risk may help to prevent dementia, even of the Alzheimer type. Recent advances in neuroimaging, neuropathology, epidemiology, and genetics have led to a deeper understanding of how vascular disease affects cognition. These new findings provide an opportunity for the present reappraisal of vascular cognitive impairment. We further briefly address current therapeutic concepts.

  9. Acute Pancreatitis and Pregnancy

    MedlinePlus

    ... Pancreatitis Acute Pancreatitis and Pregnancy Acute Pancreatitis and Pregnancy Timothy Gardner, MD Acute pancreatitis is defined as ... pancreatitis in pregnancy. Reasons for Acute Pancreatitis and Pregnancy While acute pancreatitis is responsible for almost 1 ...

  10. Acute intracerebral treatment with amyloid-beta (1–42) alters the profile of neuronal oscillations that accompany LTP induction and results in impaired LTP in freely behaving rats

    PubMed Central

    Kalweit, Alexander Nikolai; Yang, Honghong; Colitti-Klausnitzer, Jens; Fülöp, Livia; Bozsó, Zsolt; Penke, Botond; Manahan-Vaughan, Denise

    2015-01-01

    Accumulation of amyloid plaques comprises one of the major hallmarks of Alzheimer’s disease (AD). In rodents, acute treatment with amyloid-beta (Aβ; 1–42) elicits immediate debilitating effects on hippocampal long-term potentiation (LTP). Whereas LTP contributes to synaptic information storage, information is transferred across neurons by means of neuronal oscillations. Furthermore, changes in theta-gamma oscillations, that appear during high-frequency stimulation (HFS) to induce LTP, predict whether successful LTP will occur. Here, we explored if intra-cerebral treatment with Aβ(1–42), that prevents LTP, also results in alterations of hippocampal oscillations that occur during HFS of the perforant path-dentate gyrus synapse in 6-month-old behaving rats. HFS resulted in LTP that lasted for over 24 h. In Aβ-treated animals, LTP was significantly prevented. During HFS, spectral power for oscillations below 100 Hz (δ, θ, α, β and γ) was significantly higher in Aβ-treated animals compared to controls. In addition, the trough-to-peak amplitudes of theta and gamma cycles were higher during HFS in Aβ-treated animals. We also observed a lower amount of envelope-to-signal correlations during HFS in Aβ-treated animals. Overall, the characteristic profile of theta-gamma oscillations that accompany successful LTP induction was disrupted. These data indicate that alterations in network oscillations accompany Aβ-effects on hippocampal LTP. This may comprise an underlying mechanism through which disturbances in synaptic information storage and hippocampus-dependent memory occurs in AD. PMID:25999827

  11. Impaired preprodynorphin, but not preproenkephalin, mRNA induction in the striatum of mGluR1 mutant mice in response to acute administration of the full dopamine D(1) agonist SKF-82958.

    PubMed

    Mao, Limin; Conquet, François; Wang, John Q

    2002-05-01

    Metabotropic glutamate receptor 1 (mGluR1) is highly expressed in striatonigral projection neurons of rat striatum. To define the role of mGluR1 in the regulation of striatal gene expression, the responsiveness of the three neuropeptide gene expression to a single injection of the dopamine D(1) agonist SKF-82958 was compared between mGluR1 mutant and wild-type control mice. We found that acute injection of SKF-82958 increased preprodynorphin (PPD), substance P (SP), and preproenkephalin (PPE) mRNAs in the dorsal and ventral striatum of mutant and wild-type mice in a dose-dependent manner (0.125, 0.5, and 2 mg/kg, i.p.) as revealed by quantitative in situ hybridization. However, the induction of PPD mRNA in both the dorsal and ventral striatum of mGluR1 minus sign/minus sign mice was significantly less than that of wild-type +/+ mice in response to the two higher doses of SKF-82958. In contrast to PPD, SP and PPE in the dorsal and ventral striatum of mGluR1 mutant mice were elevated to a similar level as that of wild-type mice. There were no differences in basal levels and distribution patterns of all three mRNAs between the two genotypes of mice treated with saline. These results indicate that mGluR1 selectively participates in striatonigral PPD induction in response to D(1) receptor stimulation.

  12. Impairment in Non-Word Repetition: A Marker for Language Impairment or Reading Impairment?

    ERIC Educational Resources Information Center

    Baird, Gillian; Slonims, Vicky; Simonoff, Emily; Dworzynski, Katharina

    2011-01-01

    Aim: A deficit in non-word repetition (NWR), a measure of short-term phonological memory proposed as a marker for language impairment, is found not only in language impairment but also in reading impairment. We evaluated the strength of association between language impairment and reading impairment in children with current, past, and no language…

  13. Hearing or speech impairment - resources

    MedlinePlus

    Resources - hearing or speech impairment ... The following organizations are good resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell. ...

  14. Speech impairment (adult)

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/003204.htm Speech impairment (adult) To use the sharing features on ... 2017, A.D.A.M., Inc. Duplication for commercial use must be authorized in writing by ADAM ...

  15. Kids' Quest: Vision Impairment

    MedlinePlus

    ... most important job. Return to Steps World-Wide Web Search Kids Health: What is Vision Impairment What ... for the Blind (AFB) created the Braille Bug web site to teach sighted children about braille, and ...

  16. Evaluation of hearing impairment.

    PubMed

    Zadeh, M H; Selesnick, S H

    2001-01-01

    Hearing impairment is among the most common medical condition presenting to health care professionals. Ear anatomy, physiology, and pathology resulting in hearing loss are discussed. A systematic approach to evaluation, diagnosis, and treatment is presented.

  17. [Acute pancreatitis].

    PubMed

    Hecker, M; Mayer, K; Askevold, I; Collet, P; Weigand, M A; Krombach, G A; Padberg, W; Hecker, A

    2014-03-01

    Acute pancreatitis is a potentially fatal disease with individually differing expression of systemic involvement. For this reason early diagnosis with subsequent risk stratification is essential in the clinical management of this frequent gastroenterological disorder. Severe forms of acute pancreatitis occur in approximately 20 % of cases often requiring intensive care monitoring and interdisciplinary therapeutic approaches. In the acute phase adequate fluid replacement and sufficient analgesic therapy is of major therapeutic importance. Concerning the administration of antibiotics and the nutritional support of patients with acute pancreatitis a change in paradigms could be observed in recent years. Furthermore, endoscopic, radiological or surgical interventions can be necessary depending on the severity of the disease and potential complications.

  18. Bronchitis - acute

    MedlinePlus

    ... to breathe. Other symptoms of bronchitis are a cough and coughing up mucus. Acute means the symptoms ... diagnosed with chronic bronchitis, you must have a cough with mucus on most days for at least ...

  19. Acute Bronchitis

    MedlinePlus

    ... bronchitis? Acute bronchitis is inflammation of your bronchial tree. The bronchial tree consists of tubes that carry air into your ... weeks or months. This happens because the bronchial tree takes a while to heal. A lasting cough ...

  20. Advanced IT Education for the Vision Impaired via e-Learning

    ERIC Educational Resources Information Center

    Armstrong, Helen L.

    2009-01-01

    Lack of accessibility in the design of e-learning courses continues to hinder students with vision impairment. E-learning materials are predominantly vision-centric, incorporating images, animation, and interactive media, and as a result students with acute vision impairment do not have equal opportunity to gain tertiary qualifications or skills…

  1. [Inner Ear Hearing Loss Part II: Sudden Sensorineural Hearing Loss, Therapeutic Options].

    PubMed

    Hesse, Gerhard

    2016-07-01

    The great majority of hearing disorders generates from pathologies in the inner ear, mainly the outer hair cells, as mentioned in the first part of this review. Very often, however, hearing loss appears suddenly and even without external causes like noise exposure. This sudden hearing loss is mostly unilateral, recovers very often spontaneously and should be treated, if persisting. Only in this acute stage there are therapeutic options available. If the inner ear hearing loss is chronic there is no curative therapy, an effective management of the hearing disorder is only possible through rehabilitation. This is due to the fact, that hair cells of all mammals, incl. humans, have no regenerative capacity and neither pharmaceutic agents nor other means can induce regeneration and recovery of hair cells. Even a gen-therapy is not available yet. In the second part of this review the main focus lies in sudden hearing loss and general therapeutic options for inner ear hearing loss.

  2. Treatment of Post-Traumatic Cognitive Impairments

    PubMed Central

    Wortzel, Hal S.; Arciniegas, David B.

    2012-01-01

    Opinion statement Cognitive impairment is a common consequence of traumatic brain injury (TBI) and a substantial source of disability. Across all levels of TBI severity, attention, processing speed, episodic memory, and executive function are most commonly affected.The differential diagnosis for posttraumatic cognitive impairments is broad, and includes emotional, behavioral, and physical problems as well as substance use disorders, medical conditions, prescribed and self-administered medications, and symptom elaboration. Thorough neuropsychiatric assessment for such problems is a pre-requisite to treatments specifically targeting cognitive impairments.First-line treatments for posttraumatic cognitive impairments are non-pharmacologic, including education, realistic expectation setting, environmental and lifestyle modifications, and cognitive rehabilitation.Pharmacotherapies for posttraumatic cognitive impairments include uncompetitive N-methyl-D-aspartate receptor (NMDA) antagonists, medications that directly or indirectly augment cerebral catecholaminergic or acetylcholinergic function, or agents with combinations of these properties.In the immediate post-injury period, treatment with uncompetitive NMDA receptor antagonists reduces duration of unconsciousness. The mechanism for this effect may involve attenuation of neurotrauma-induced glutamate-mediated excitotoxicity and/or stabilization of glutamate signaling in the injured brain.During the sub-acute or late post-injury periods, medications that augment cerebral acetylcholinergic function may improve declarative memory. Among responders to this treatment, secondary benefits on attention, processing speed, and executive function impairments as well as neuropsychiatric disturbances may be observed. During these post-injury periods, medications that augment cerebral catecholaminergic function may improve hypoarousal, processing speed, attention, and/or executive function as well as comorbid depression or apathy

  3. Discrimination and identification of vowels by young, hearing-impaired adults

    NASA Astrophysics Data System (ADS)

    Richie, Carolyn; Kewley-Port, Diane; Coughlin, Maureen

    2003-11-01

    This study examined the effects of mild-to-moderate sensorineural hearing loss on vowel perception abilities of young, hearing-impaired (YHI) adults. Stimuli were presented at a low conversational level with a flat frequency response (approximately 60 dB SPL), and in two gain conditions: (a) high level gain with a flat frequency response (95 dB SPL), and (b) frequency-specific gain shaped according to each listener's hearing loss (designed to simulate the frequency response provided by a linear hearing aid to an input signal of 60 dB SPL). Listeners discriminated changes in the vowels /smcapi ee eh invv æ/ when F1 or F2 varied, and later categorized the vowels. YHI listeners performed better in the two gain conditions than in the conversational level condition. Performances in the two gain conditions were similar, suggesting that upward spread of masking was not seen at these signal levels for these tasks. Results were compared with those from a group of elderly, hearing-impaired (EHI) listeners, reported in Coughlin, Kewley-Port, and Humes [J. Acoust. Soc. Am. 104, 3597-3607 (1998)]. Comparisons revealed no significant differences between the EHI and YHI groups, suggesting that hearing impairment, not age, is the primary contributor to decreased vowel perception in these listeners.

  4. First audiometric results with the Vibrant soundbridge, a semi-implantable hearing device for sensorineural hearing loss.

    PubMed

    Snik, A F; Cremers, C W

    1999-01-01

    The Vibrant soundbridge is a semi-implantable hearing device. The implanted electromagnetic transducer is attached to the incus and it is linked by telemetry to the externally worn audio processor. In Nijmegen, this device has been applied to seven patients with moderate or severe sensorineural hearing loss (PTA between 43 and 71 dB HL) who could not tolerate ear moulds. As the amplification of the device depends on the input level (amplifier with wide dynamic range compression), loudness scaling measurements were performed. The gain as a function of input level was determined from aided and unaided loudness growth curves. The mean gain was 21 dB at an input level of 40 dB SPL. The mean gain decreased to 5 dB at an input level of 90 dB SPL. Measured gain values were lower than target values prescribed by the FIG6 method, mainly however for the low-frequency range and for low-level sounds. It was concluded that this device is very promising for patients who cannot tolerate an ear mould.

  5. High Total Cholesterol in Peripheral Blood Correlates with Poorer Hearing Recovery in Idiopathic Sudden Sensorineural Hearing Loss.

    PubMed

    Quaranta, Nicola; Squeo, Valentina; Sangineto, Moris; Graziano, Giusi; Sabbà, Carlo

    2015-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is a common otologic emergency whose cause is still unclear. The importance of blood lipids in the pathogenesis of ISSHL is widely reported in literature. In fact elevated levels of low density lipoprotein cholesterol (LDL), total cholesterol (TC) and apolipoprotein B (Apo-B) have been proposed as risk factors for this pathology. No correlation has been described between serum lipid parameters and the prognosis of ISSHL. Aim of the present study was to identify prognostic factors associated with hearing recovery in a group of patients affected by ISSHL. Ninety-four patients with the diagnosis of ISSHL hospitalized between March 2013 and October 2014 were included in this study. Patients' blood sampling and hearing assessments were carried out. Patients were divided into two groups as "recovered" and "unrecovered", according to their response to the treatment. We found a statistically significant higher level of total cholesterol in the unrecovered group compared to the recovered one (p = 0.03). None of the other routine laboratory parameters have shown a statistically significant difference between the patients successfully treated and patients with poor outcomes. Total cholesterol concentrations may be a prognostic factor for recovery in ISSHL and should be assessed together with routine tests in patients with this condition. The other routine laboratory parameters seem to have no effect on the development and prognosis of this pathology.

  6. High Total Cholesterol in Peripheral Blood Correlates with Poorer Hearing Recovery in Idiopathic Sudden Sensorineural Hearing Loss

    PubMed Central

    Quaranta, Nicola; Squeo, Valentina; Sangineto, Moris; Graziano, Giusi; Sabbà, Carlo

    2015-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is a common otologic emergency whose cause is still unclear. The importance of blood lipids in the pathogenesis of ISSHL is widely reported in literature. In fact elevated levels of low density lipoprotein cholesterol (LDL), total cholesterol (TC) and apolipoprotein B (Apo-B) have been proposed as risk factors for this pathology. No correlation has been described between serum lipid parameters and the prognosis of ISSHL. Aim of the present study was to identify prognostic factors associated with hearing recovery in a group of patients affected by ISSHL. Ninety-four patients with the diagnosis of ISSHL hospitalized between March 2013 and October 2014 were included in this study. Patients’ blood sampling and hearing assessments were carried out. Patients were divided into two groups as “recovered” and “unrecovered”, according to their response to the treatment. We found a statistically significant higher level of total cholesterol in the unrecovered group compared to the recovered one (p = 0.03). None of the other routine laboratory parameters have shown a statistically significant difference between the patients successfully treated and patients with poor outcomes. Total cholesterol concentrations may be a prognostic factor for recovery in ISSHL and should be assessed together with routine tests in patients with this condition. The other routine laboratory parameters seem to have no effect on the development and prognosis of this pathology. PMID:26208311

  7. Combined intratympanic and systemic use of steroids for idiopathic sudden sensorineural hearing loss: a meta-analysis.

    PubMed

    Gao, Yang; Liu, Dong

    2016-11-01

    The main objective of the meta-analysis was to investigate whether intratympanic steroid injections in combination with systemic steroids would provide an additional advantage over systemic steroid therapy (SST) alone in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The results will provide a meaningful suggestion in clinical therapy of ISSNHL. The electronic database search was based on the database in OVID Medline, Embase and PubMed up to December 15, 2015 with the goal of identifying all available observational studies examining the effects of combination therapy and SST in ISSNHL patients. Observational studies that compared the pure tone average (PTA) improvement and recovery rate between combination therapy and SST group in ISSNHL patients were selected. Finally we have identified eight eligible studies that focused on comparing the combination therapy and SST in ISSNHL from designated researches. In the PTA improvement group, seven studies have been analyzed to compare the pooled mean differences between two therapy modalities and subgroups based on initial hearing loss and treatment delay. In the recovery rate group, six studies were calculated for pooled risk ratios and subgroup analysis was also conducted. Through our meta-analysis, we have reached the conclusion that combination therapy exhibited better outcomes in PTA improvement than SST alone, especially in severe-profound initial hearing loss cases. Combination therapy also showed advantages in recovery rate. Whether time of treatment delay would influence the PTA improvement and recovery rate requires further researches.

  8. Hearing outcome does not depend on the interval of intratympanic steroid administration in idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Koizumi, Hiroki; Ohkubo, Jun-Ichi; Hohchi, Nobusuke; Ikezaki, Shoji; Kitamura, Takuro

    2016-10-01

    We studied the effect of intratympanic steroid administration with different intervals on hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The subjects were 197 consecutive patients (197 ears) with ISSNHL (hearing level ≥40 dB, interval between onset and treatment ≤30 days). They received systemic administration of prednisolone (100 mg followed by tapered doses) combined with intratympanic injection of dexamethasone (4 mg/ml). Intratympanic injection was performed once a week for 4 weeks in 105 patients (long-interval group), or 4 times in 1 week in 92 patients (short-interval group). The hearing outcomes were evaluated at two points of time: 1 week from the start of treatment, and 1-2 months after the completion of treatment when the hearing level reached a plateau. There was no significant difference in the cure rate, marked-recovery rate, recovery rate, hearing gain, hearing level, or percent hearing improvement between the long- and short-interval groups at either point of time. Multiple regression analysis also showed that the final hearing level did not depend on the interval of intratympanic steroid injection. These results indicate that the hearing outcome of ISSNHL does not improve even if the interval of intratympanic injection is shortened. This implies that a lower total number of intratympanic steroid injections may be as effective as the present protocol.

  9. Synaptopathy in the noise-exposed and aging cochlea: Primary neural degeneration in acquired sensorineural hearing loss.

    PubMed

    Kujawa, Sharon G; Liberman, M Charles

    2015-12-01

    The classic view of sensorineural hearing loss (SNHL) is that the "primary" targets are hair cells, and that cochlear-nerve loss is "secondary" to hair cell degeneration. Our recent work in mouse and guinea pig has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of cochlear-nerve/hair-cell synapses. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained hidden for three reasons: 1) the spiral ganglion cells, the cochlear neural elements commonly assessed in studies of SNHL, survive for years despite loss of synaptic connection with hair cells, 2) the synaptic terminals of cochlear nerve fibers are unmyelinated and difficult to see in the light microscope, and 3) the degeneration is selective for cochlear-nerve fibers with high thresholds. Although not required for threshold detection in quiet (e.g. threshold audiometry or auditory brainstem response threshold), these high-threshold fibers are critical for hearing in noisy environments. Our research suggests that 1) primary neural degeneration is an important contributor to the perceptual handicap in SNHL, and 2) in cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from spiral ganglion neurons and re-establishment of their peripheral synapses. This article is part of a Special Issue entitled .

  10. CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss.

    PubMed

    Joshi, Varsha M; Navlekar, Shantanu K; Kishore, G Ravi; Reddy, K Jitender; Kumar, E C Vinay

    2012-01-01

    Imaging plays an important role in the evaluation of congenital sensorineural hearing loss. In children who are candidates for cochlear implantation surgery, it provides vital preoperative information about the inner ear, the vestibulocochlear nerve, and the brain. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging provide excellent delineation of the intricate anatomy of the inner ear: CT depicts the minute details of osseous structures, and MR imaging allows visualization of the fluid-filled spaces and the vestibulocochlear nerve. Together, these complementary modalities can aid decision making about the best management strategy by facilitating the identification and characterization of inner ear malformations and any associated neurologic abnormalities. It is important that the radiologist be familiar with the key imaging features when interpreting CT and MR images obtained in this patient group. A broad spectrum of inner ear malformations have been described and linked to developmental insults at different stages of embryogenesis, and various systems have been proposed for classifying them. In this article, these malformations are described by using classification systems used by otolaryngologists for ease of interpretation. The relevant normal anatomy and development of the inner ear are briefly surveyed, standard imaging protocols for studying the inner ear are reviewed, and the imaging appearances of frequently observed inner ear malformations are described and illustrated. The impact of the identification of these malformations and commonly associated brain abnormalities on clinical management and prognosis also is discussed.

  11. NLRP3 Is Expressed in the Spiral Ganglion Neurons and Associated with Both Syndromic and Nonsyndromic Sensorineural Deafness

    PubMed Central

    Chen, Penghui; He, Longxia; Pang, Xiuhong; Wang, Xiaowen

    2016-01-01

    Nonsyndromic deafness is genetically heterogeneous but phenotypically similar among many cases. Though a variety of targeted next-generation sequencing (NGS) panels has been recently developed to facilitate genetic screening of nonsyndromic deafness, some syndromic deafness genes outside the panels may lead to clinical phenotypes similar to nonsyndromic deafness. In this study, we performed comprehensive genetic screening in a dominant family in which the proband was initially diagnosed with nonsyndromic deafness. No pathogenic mutation was identified by targeted NGS in 72 nonsyndromic and another 72 syndromic deafness genes. Whole exome sequencing, however, identified a p.E313K mutation in NLRP3, a gene reported to cause syndromic deafness Muckle-Wells Syndrome (MWS) but not included in any targeted NGS panels for deafness in previous reports. Follow-up clinical evaluation revealed only minor inflammatory symptoms in addition to deafness in six of the nine affected members, while the rest, three affected members, including the proband had no obvious MWS-related inflammatory symptoms. Immunostaining of the mouse cochlea showed a strong expression of NLRP3 in the spiral ganglion neurons. Our results suggested that NLRP3 may have specific function in the spiral ganglion neurons and can be associated with both syndromic and nonsyndromic sensorineural deafness. PMID:27965898

  12. Treatment of central and sensorineural tinnitus with orally administered Melatonin and Sulodexide: personal experience from a randomized controlled study.

    PubMed

    Neri, G; De Stefano, A; Baffa, C; Kulamarva, G; Di Giovanni, P; Petrucci, G; Poliandri, A; Dispenza, F; Citraro, L; Croce, A

    2009-04-01

    Since very little is understood about the exact aetiology of tinnitus, this has made treatment of the condition difficult. Even though approximately 10-15% of the general population suffer from tinnitus, only 2% consider it serious enough to warrant any treatment. The main problem arising from tinnitus is the disturbance it causes not only in day to day life but also in sleep, leading to fatigue and general discomfort. The present study focused on the effect of Melatonin in conjunction with Sulodexide as a treatment method for tinnitus. Overall, 102 patients suffering from tinnitus were evaluated in a prospective randomised controlled study conducted in a tertiary care ENT department. After randomisation, 34 patients were treated with Melatonin and Sulodexide, another 34 were treated with Melatonin alone, while the remaining 34 (control group) were managed without treatment in order to evaluate spontaneous variations in the quality of tinnitus. Patients were assessed prospectively with the Tinnitus Handicap Inventory and Acufenometry, both pre- and post-treatment. Among the patients studied, better results with both Tinnitus Handicap Inventory and Acufenometry were found in the group who received Melatonin and Sulodexide compared to those receiving Melatonin alone. No improvement was observed in the control group. In conclusion, Melatonin in combination with Sulodexide is, in our opinion, a viable treatment option for patients suffering from central or sensorineural tinnitus.

  13. A Retrospective Study of the Clinical Characteristics and Post-treatment Hearing Outcome in Idiopathic Sudden Sensorineural Hearing Loss

    PubMed Central

    Kothandaraman, Purushothaman Pavanjur; Swapna, Simham; Manchaiah, Vinaya

    2017-01-01

    The purpose of this retrospective study was to analyze the clinical characteristics and document hearing recovery in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). 122 patients diagnosed with unilateral ISSNHL, from March 2009 to December 2014, were treated with oral steroids and pentoxifylline. Hearing change was evaluated by comparing pre-treatment and post-treatment pure-tone average (PTA) (500, 1K, and 2K Hz), and categorized into complete, partial, and no recovery of hearing. T-test, Wilcoxon Signed Rank test and Regression analysis were employed to analyze the statistical significance. Of the 122 patients, seventy-one (58%) had complete recovery and 34 (28%) had partial recovery. The average pre-treatment PTA was 78.3±16.9 dB whereas post-treatment average was 47.0±20.8 dB, showing statistically significant improvement (t=24.89, P≤0.001). The factors such as presence of tinnitus (P=0.005) and initial milder hearing loss (P=0.005) were found to be significant predictors for hearing recovery. Conventional steroid regimes produced a recovery rate in ISSNHL, which exceeds the spontaneous recovery rate. The current study results highlight the importance of medical treatment in the management of ISSNHL. PMID:28286637

  14. Spatial release from masking in normally hearing and hearing-impaired listeners as a function of the temporal overlap of competing talkersa

    PubMed Central

    Best, Virginia; Mason, Christine R.; Kidd, Gerald

    2011-01-01

    Listeners with sensorineural hearing loss are poorer than listeners with normal hearing at understanding one talker in the presence of another. This deficit is more pronounced when competing talkers are spatially separated, implying a reduced “spatial benefit” in hearing-impaired listeners. This study tested the hypothesis that this deficit is due to increased masking specifically during the simultaneous portions of competing speech signals. Monosyllabic words were compressed to a uniform duration and concatenated to create target and masker sentences with three levels of temporal overlap: 0% (non-overlapping in time), 50% (partially overlapping), or 100% (completely overlapping). Listeners with hearing loss performed particularly poorly in the 100% overlap condition, consistent with the idea that simultaneous speech sounds are most problematic for these listeners. However, spatial release from masking was reduced in all overlap conditions, suggesting that increased masking during periods of temporal overlap is only one factor limiting spatial unmasking in hearing-impaired listeners. PMID:21428524

  15. Trainable Mentally Impaired/Severely Multiply Impaired/Autistic Impaired/Severely Mentally Impaired. Product Evaluation Report 1989-1990.

    ERIC Educational Resources Information Center

    Claus, Richard N.; And Others

    The evaluation report describes special education services provided to trainable mentally impaired (TMI), autistic impaired (AI), severely multiply impaired (SXI), and severely mentally impaired (SMI) students at and through the Melvin G. Millet Learning Center (Bridgeport, Michigan). The eight program components are described individually and…

  16. Hearing impairment in patients with rheumatoid arthritis: association with anti-citrullinated protein antibodies.

    PubMed

    Lobo, Fabrício Silva; Dossi, Mario Orlando; Batista, Lígia; Shinzato, Márcia Midori

    2016-09-01

    It has been suggested that hearing impairment (HI) is one of the extra-articular features of rheumatoid arthritis (RA). Nevertheless, the prevalence and nature of HI in RA is still uncertain. The objectives were to study hearing function in patients with RA using audiometric tests and to examine whether HI correlates with autoantibodies. Hearing functions were investigated in 43 consecutive RA patients and 23 control subjects (less than 60 years old). Their sera were evaluated for the presence of rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), and anti-mutated citrullinated vimentin (anti-MCV) antibodies. HI was observed in 46.5 % of RA patients and in 30.4 % of control subjects, p = 0.32. HI was characterized as sensorineural in 80 and 85.7 % of RA patients and control subjects with HI, respectively, p = 1.00. RA patients had a worse hearing threshold for air conduction at 6 kHz in the right ear (p = 0.019) and had a decreased amplitude of otoacoustic emissions (OAEs) at 2 kHz bilaterally (p = 0.04) compared with control subjects. In the RA group, patients with and without HI were 80 and 34.78 % anti-CCP positive, respectively, p = 0.008. RA patients with and without HI were 85 and 43.48 % anti-MCV positive, respectively, p = 0.013. HI in RA patients was mainly sensorineural and was associated with anti-CCP and anti-MCV antibodies.

  17. Social communication impairments: pragmatics.

    PubMed

    Russell, Robert L

    2007-06-01

    Social communication or pragmatic impairments are characterized and illustrated as involving inappropriate or ineffective use of language and gesture in social contexts. Three clinical vignettes illustrate different pragmatic impairments and the wealth of diagnostic information that can be garnered from observation of a child's social communication behavior. Definitions of, and developmental milestones in, domains of pragmatic competence are provided. Several screening instruments are suggested for use in assessing pragmatic competence within the time-frame of a pediatric examination. Frequent comorbid psychiatric conditions are described and a sample of current neurobiologic research is briefly summarized.

  18. Association between idiopathic hearing loss and mitochondrial DNA mutations: A study on 169 hearing-impaired subjects

    PubMed Central

    GUARAN, VALERIA; ASTOLFI, LAURA; CASTIGLIONE, ALESSANDRO; SIMONI, EDI; OLIVETTO, ELENA; GALASSO, MARCO; TREVISI, PATRIZIA; BUSI, MICOL; VOLINIA, STEFANO; MARTINI, ALESSANDRO

    2013-01-01

    Mutations in mitochondrial DNA (mtDNA) have been shown to be an important cause of sensorineural hearing loss (SNHL). In this study, we performed a clinical and genetic analysis of 169 hearing-impaired patients and some of their relatives suffering from idiopathic SNHL, both familial and sporadic. The analysis of four fragments of their mtDNA identified several polymorphisms, the well known pathogenic mutation, A1555G, and some novel mutations in different genes, implying changes in the aminoacidic sequence. A novel sporadic mutation in 12S rRNA (MT-RNR1), not previously reported in the literature, was found in a case of possible aminoglycoside-induced progressive deafness. PMID:23969527

  19. Acute Pancreatitis

    PubMed Central

    Geokas, Michael C.

    1972-01-01

    For many decades two types of acute pancreatitis have been recognized: the edematous or interstitial and the hemorrhagic or necrotic. In most cases acute pancreatitis is associated with alcoholism or biliary tract disease. Elevated serum or urinary α-amylase is the most important finding in diagnosis. The presence of methemalbumin in serum and in peritoneal or pleural fluid supports the diagnosis of the hemorrhagic form of the disease in patients with a history and enzyme studies suggestive of pancreatitis. There is no characteristic clinical picture in acute pancreatitis, and its complications are legion. Pancreatic pseudocyst is probably the most common and pancreatic abscess is the most serious complication. The pathogenetic principle is autodigestion, but the precise sequence of biochemical events is unclear, especially the mode of trypsinogen activation and the role of lysosomal hydrolases. A host of metabolic derangements have been identified in acute pancreatitis, involving lipid, glucose, calcium and magnesium metabolism and changes of the blood clotting mechanism, to name but a few. Medical treatment includes intestinal decompression, analgesics, correction of hypovolemia and other supportive and protective measures. Surgical exploration is advisable in selected cases, when the diagnosis is in doubt, and is considered imperative in the presence of certain complications, especially pancreatic abscess. PMID:4559467

  20. Emotionally Impaired Elementary Curriculum.

    ERIC Educational Resources Information Center

    Taulbee, Dianne R.; And Others

    A curriculum is presented for teaching emotionally impaired elementary students. The curriculum document describes program management techniques, strategies for developing and maintaining teacher-student relationships, and therapy/change systems. It outlines referral and eligibility procedures and exit criteria. It contains job descriptions for…

  1. Emotionally Impaired Elementary Curriculum.

    ERIC Educational Resources Information Center

    Taulbee, Dianne R.; And Others

    The Jackson County (Michigan) Intermediate School District curriculum for teaching emotionally impaired elementary students is presented. The curriculum document describes program management techniques, strategies for developing and maintaining teacher-student relationships, and therapy/change systems. It outlines referral and eligibility…

  2. Anarthria impairs subvocal counting.

    PubMed

    Cubelli, R; Nichelli, P; Pentore, R

    1993-12-01

    We studied subvocal counting in two pure anarthric patients. Analysis showed that they performed definitively worse than normal subjects free to articulate subvocally and their scores were in the lower bounds of the performances of subjects suppressing articulation. These results suggest that subvocal counting is impaired after anarthria.

  3. Specific Language Impairment

    MedlinePlus

    ... children with true language impairments. After studying a large group of Hispanic children who speak English as a ... trying out an intervention program with a small group of bilingual first graders with SLI to find techniques and strategies to help them succeed academically. Diagnostic ...

  4. Efficacy comparison of oral steroid, intratympanic steroid, hyperbaric oxygen and oral steroid + hyperbaric oxygen treatments in idiopathic sudden sensorineural hearing loss cases.

    PubMed

    Alimoglu, Yalcin; Inci, Ender; Edizer, Deniz Tuna; Ozdilek, Alper; Aslan, Mehmet

    2011-12-01

    Idiopathic sudden sensorineural hearing loss is a rare disorder of unknown pathogenesis in which hearing is lost partially or totally. About 60 treatment modalities have been described. We aimed to compare the efficacy of hyperbaric oxygen, oral steroid, intratympanic steroid therapy and their combinations in idiopathic sudden sensorineural hearing loss patients. Files of patients who were followed up between 2004 and 2010 in our clinic were examined retrospectively. Patients were divided into four groups according to the therapy received: Oral steroid, oral steroid + hyperbaric oxygen, intratympanic steroid and hyperbaric oxygen. Treatment success was assessed by Siegel criteria and mean gains using pre-treatment and post-treatment audiograms. 217 patients and 219 ears were examined. The proportion of patients responding to therapy was the highest in the oral steroid + hyperbaric oxygen group with 86.88% (53/61) followed by the oral steroid group with 63.79% (37/58), the intratympanic steroid group with 46,51% (20/43) and the hyperbaric oxygen group with 43.85% (25/57). The proportion of patients who had complete recovery was the highest in the oral steroid + hyperbaric oxygen group with 42.6% (26/61) followed by the oral steroid group with 19.0% (11/58), the hyperbaric oxygen group with 17.5% (10/57) and the intratympanic steroid group with 11.6% (5/43). The oral steroid + hyperbaric oxygen group has the highest mean hearing gain among all groups (p < 0.05). Idiopathic sudden sensorineural hearing loss patients receiving oral steroid + hyperbaric oxygen combination therapy have a higher likelihood of recovery than patients receiving oral steroids, hyperbaric oxygen or intratympanic steroids alone.

  5. Development of the stria vascularis and potassium regulation in the human fetal cochlea: Insights into hereditary sensorineural hearing loss

    PubMed Central

    de Groot, John C.M.J.; van Iperen, Liesbeth; Huisman, Margriet A.; Frijns, Johan H.M.

    2015-01-01

    ABSTRACT Sensorineural hearing loss (SNHL) is one of the most common congenital disorders in humans, afflicting one in every thousand newborns. The majority is of heritable origin and can be divided in syndromic and nonsyndromic forms. Knowledge of the expression profile of affected genes in the human fetal cochlea is limited, and as many of the gene mutations causing SNHL likely affect the stria vascularis or cochlear potassium homeostasis (both essential to hearing), a better insight into the embryological development of this organ is needed to understand SNHL etiologies. We present an investigation on the development of the stria vascularis in the human fetal cochlea between 9 and 18 weeks of gestation (W9–W18) and show the cochlear expression dynamics of key potassium‐regulating proteins. At W12, MITF+/SOX10+/KIT+ neural‐crest‐derived melanocytes migrated into the cochlea and penetrated the basement membrane of the lateral wall epithelium, developing into the intermediate cells of the stria vascularis. These melanocytes tightly integrated with Na+/K+‐ATPase‐positive marginal cells, which started to express KCNQ1 in their apical membrane at W16. At W18, KCNJ10 and gap junction proteins GJB2/CX26 and GJB6/CX30 were expressed in the cells in the outer sulcus, but not in the spiral ligament. Finally, we investigated GJA1/CX43 and GJE1/CX23 expression, and suggest that GJE1 presents a potential new SNHL associated locus. Our study helps to better understand human cochlear development, provides more insight into multiple forms of hereditary SNHL, and suggests that human hearing does not commence before the third trimester of pregnancy. © 2015 Wiley Periodicals, Inc. Develop Neurobiol 75: 1219–1240, 2015 PMID:25663387

  6. Changes of the directional brain networks related with brain plasticity in patients with long-term unilateral sensorineural hearing loss.

    PubMed

    Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Li, J; Chen, J-Y; Chen, H; Zhang, P-P; Liu, L-J; Wang, J; Teng, G-J

    2016-01-28

    Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain.

  7. Smartphone-based hearing test as an aid in the initial evaluation of unilateral sudden sensorineural hearing loss.

    PubMed

    Handzel, Ophir; Ben-Ari, Oded; Damian, Doris; Priel, Maayan M; Cohen, Jacob; Himmelfarb, Mordechai

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) can cause significant morbidity. Treatment with steroids can improve outcome. Delay in initiation of treatment reduces the chance to regain hearing. For this reason SSNHL is considered an emergency. Diagnosis is based on history, physical examination and a standard audiogram, the latter requiring specialized equipment and personnel. Standard audiogram may not be available at the time and place of patient presentation. A smartphone or tablet computer-based hearing test may aid in the decision to prescribe steroids in this setting. In this study the uHear™ hearing test application was utilized. The output of this ear-level air conduction hearing test is reported in hearing grades for 6 frequencies ranging from 250 to 6000 Hz. A total of 32 patients with unilateral SSNHL proven by a standard audiogram were tested. The results of standard and iPod hearing tests were compared. Based on the accepted criterion of SSNHL (at least 30 dB loss - or 2 hearing grades - in 3 consecutive frequencies) the test had a sensitivity of 0.76 and specificity of 0.91. Using a less stringent criterion of a loss of 2 hearing grades over at least 2 frequencies the sensitivity was 0.96 and specificity 0.86. The correlation coefficient for the comparison of the average hearing grade across the 6 measured frequencies of the study and standard audiogram was 0.83. uHear more accurately reflected hearing thresholds at mid and high tones. Similarly to previously published data, low frequency thresholds could be artificially elevated. In conclusion, uHear can be useful in the initial evaluation of patients with single-sided SSNHL by providing important information guiding the decision to initiate treatment before a standard audiogram is available.

  8. Ischemia as a potential etiologic factor in idiopathic unilateral sudden sensorineural hearing loss: Analysis of posterior circulation arteries.

    PubMed

    Kim, Chulho; Sohn, Jong-Hee; Jang, Min Uk; Hong, Sung-Kwang; Lee, Joong-Seob; Kim, Hyung-Jong; Choi, Hui-Chul; Lee, Jun Ho

    2016-01-01

    The association between idiopathic sudden sensorineural hearing loss (ISSNHL) and the radiologic characteristics of the vertebrobasilar artery is unclear. We hypothesized that the degree and direction of vertebrobasilar artery curvature in the posterior circulation contribute to the occurrence of ISSNHL. We consecutively enrolled patients diagnosed with unilateral ISSNHL in two tertiary hospitals. Magnetic resonance images were performed in all patients to exclude specific causes of ISSNHL, such as vestibular schwannoma, chronic mastoiditis, and anterior inferior cerebellar artery infarct. We measured the following parameters of posterior circulation: vertebral and basilar artery diameter, the degree of basilar artery curvature (modified smoker criteria), and vertebral artery dominance. Pure tone audiometries were performed at admission and again 1 week and 3 months later. A total of 121 ISSNHL patients (mean age, 46.0 ± 17.3 years; 48.8% male) were included in these analyses. The proportion of patients with the left side hearing loss was larger than the proportion with the right side hearing loss (left, 57.9%; right, 42.1%). The majority of patients were characterized by a left dominant vertebral artery and right-sided basilar artery curvature. The direction of the basilar artery curvature was significantly associated with hearing loss lateralization (p = 0.036). Age and sex matched multivariable analyses revealed the absence of diabetes and right-sided basilar artery curvature as significant predictors for left sided hearing loss. There was no statistical difference between atherosclerotic cardiovascular risk score (high versus low) and hearing outcomes at 3 months. In ISSNHL, the laterality of hearing loss was inversely associated with the direction of basilar artery curvature. Our results, therefore, indicate the importance of vascular assessment when evaluating ISSNHL.

  9. A common variant in CLDN14 causes precipitous, prelingual sensorineural hearing loss in multiple families due to founder effect.

    PubMed

    Pater, Justin A; Benteau, Tammy; Griffin, Anne; Penney, Cindy; Stanton, Susan G; Predham, Sarah; Kielley, Bernadine; Squires, Jessica; Zhou, Jiayi; Li, Quan; Abdelfatah, Nelly; O'Rielly, Darren D; Young, Terry-Lynn

    2017-01-01

    Genetic isolates provide unprecedented opportunities to identify pathogenic mutations and explore the full natural history of clinically heterogeneous phenotypes such as hearing loss. We noticed a unique audioprofile, characterized by prelingual and rapid deterioration of hearing thresholds at frequencies >0.5 kHz in several adults from unrelated families from the island population of Newfoundland. Targeted serial Sanger sequencing of probands for deafness alleles (n = 23) that we previously identified in this founder population was negative. Whole exome sequencing in four members of the largest family (R2010) identified a CLDN14 (DFNB29) variant [c.488C>T; p. (Ala163Val)], likely pathogenic, sensorineural hearing loss, autosomal recessive. Although not associated with deafness or disease, CLDN14 p.(Ala163Val) has been previously reported as a variant of uncertain significance (VUS). Targeted sequencing of 169 deafness probands identified one homozygote and one heterozygous carrier. Genealogical studies, cascade sequencing and haplotype analysis across four unrelated families showed all subjects with the unique audioprofile (n = 12) were also homozygous for p.(Ala163Val) and shared a 1.4 Mb DFNB29-associated haplotype on chromosome 21. Most significantly, sequencing 175 population controls revealed 1% of the population are heterozygous for CLDN14 p.(Ala163Val), consistent with a major founder effect in Newfoundland. The youngest CLDN14 [c.488C>T; p.(Ala163Val)] homozygote passed newborn screening and had normal hearing thresholds up to 3 years of age, which then deteriorated to a precipitous loss >1 kHz during the first decade. Our study suggests that genetic testing may be necessary to identify at-risk children in time to prevent speech, language and developmental delay.

  10. Consequences of a screening programme on the prevalence of congenital hereditary sensorineural deafness in the Australian Cattle Dog.

    PubMed

    Sommerlad, S F; Morton, J M; Johnstone, I; O'Leary, C A; Seddon, J M

    2014-12-01

    Genetic disease testing programmes are used in domestic animal breeds to guide selective breeding with the aim of reducing disease prevalence. We assessed the change in the prevalence of canine congenital hereditary sensorineural deafness (CHSD) in litters of Australian Cattle Dogs following the introduction of a brainstem auditory evoked response (BAER) testing programme. We studied 608 pups from 122 litters from 10 breeding kennels. Despite 10 years of testing (1998-2008), no substantial reduction in prevalence of CHSD was evident in these 10 breeding kennels. Even for the subset of litters in which both parents were BAER tested as normal hearing (305 pups from 58 litters), there was no evidence of substantial reduction in prevalence. Odds ratios for CHSD in pups for each extra year since testing in the kennel commenced were 1.01 (95% CI, 0.88-1.17) and 1.03 (95% CI, 0.82-1.30) respectively for these populations. Amongst 284 dogs from 54 litters with extended pedigrees and both parents BAER-tested normal hearing, observed prevalences of CHSD were highest in pups with no BAER-tested normal grandparents (17% or 5/29) and lowest in pups with all four grandparents tested normal (0% or 0/9). In pups for which one, two and three grandparents tested negative, prevalences of CHSD were 12% (9/74), 9% (9/101) and 8% (6/71) respectively. Hence, testing programmes based on phenotypic screening may not lead to a substantial reduction in recessive genetic disease prevalence over the medium term, even when only tested normal parents are used. Exclusive breeding of litters in which both parents and all four grandparents are BAER-tested normal is expected to reduce CHSD prevalence in pups to the greatest extent over the long term.

  11. Association between the methylenetetrahydrofolate reductase gene C677T polymorphism and sudden sensorineural hearing loss: a meta-analysis.

    PubMed

    Shu, Jingcheng; Yin, Shihua; Tan, An-Zhou; He, Meirong

    2015-09-01

    A variety of epidemiological studies have evaluated the association between methylenetetrahydrofolate reductase (MTHFR) gene C677T polymorphism and sudden sensorineural hearing loss (SSNHL), but the results were inconsistent. The aim of this meta-analysis was to clarify more accurately the association of this polymorphism with SSNHL. A systematic literature search of the associated studies up to May 1, 2014, was conducted using the following electronic databases: PubMed, Embase, Medline, and the China National Knowledge Infrastructure. Statistical analyses were performed by STATA12.0 software, with odds ratios (ORs) and their 95 % confidence intervals (CIs). Six eligible studies including covering 1,271 objects were identified. A pooled analysis of these studies showed no significant association between C677T polymorphism and risk of SSNHL: T vs. C (OR = 1.334, POR = 0.105); TT vs. CC (OR = 1.580, POR = 0.231); CT vs. CC (OR = 1.500, POR = 0.123); TT vs. CC + CT (OR = 1.326, POR = 0.293); and TT + CT vs. CC (OR = 1.540, POR = 0.102). But in subgroup analysis, a significant association was found in European populations (T vs. C, OR = 1.542, 95 % CI 1.008-2.359, P = 0.046; TT vs. CT + CC, OR = 1.856, 95 % CI 1.245-2.767, P = 0.002). There was no significant association in any model in the Asian populations. The present meta-analysis suggests that MTHFR gene C677T polymorphism is significantly associated with increased risk of SSNHL disease in European populations, but no statistically significant association was found between the MTHFR C677T gene mutation and SSNHL in Asian. Further large and well-designed studies are needed to confirm this association.

  12. Impact of methionine synthase gene and methylenetetrahydrofolate reductase gene polymorphisms on the risk of sudden sensorineural hearing loss.

    PubMed

    Gross, Menachem; Friedman, Gideon; Eliashar, Ron; Koren-Morag, Nira; Goldschmidt, Neta; Atta, Iman Abou; Ben-Yehuda, Arie

    2006-01-01

    Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological disease of unknown etiology. In recent years, several inherited risk factors have been found in the pathogenesis of vascular diseases. In the present study, we determined whether specific polymorphism or the combination of polymorphisms in folate-dependent homocysteine metabolism genes can act as predisposing inherited vascular risk factors in the development of SSNHL. We conducted a prospective case-control study using DNA samples extracted from 81 patients diagnosed as suffering from SSNHL and 264 healthy control subjects. Three functional polymorphisms were analyzed by polymerase chain reaction amplification, restriction enzyme digestion, and DNA fragment separation by electrophoresis: methylenetetrahydrofolate reductase (MTHFR) C677T, MTHFR A1298C, and methionine synthase (MTR) A2756G polymorphisms. The prevalence of the homozygous genotype of MTR 2756GG in the SSNHL patients (9%) was significantly higher than in the control group (4%) (p = 0.011). The allelic frequency of the G allele of the MTR A2756G polymorphism among SSNHL patients (12.5%) was also significantly higher than in the control group (5%) (p = 0.033). The prevalence of patients possessing two polymorphisms (31%) and three polymorphisms (17%) in the SSNHL group was significantly higher than in the control group (23 and 9%, respectively; p = 0.019). The frequency of patients with a very high rank risk (double homozygous) was significantly higher in the SSNHL group, MTHFR 677TT/MTR 2675GG--7%, than the frequency of patients in the control group, MTHFR 677TT/MTR 2675GG--3% (p = 0.030). Certain polymorphisms in genes encoding enzymes in the folate-dependent homocysteine metabolism are associated with SSNHL. In our case-control study, a significant association between MTR 2756GG genotype and SSNHL was found which may represent an inherited vascular risk factor in the pathogenesis of SSNHL.

  13. Dose-Response Analysis of Developmental Iodide Deficiency: Reductions in Thyroid Hormones and Impaired Hippocampal Synaptic Transmission

    EPA Science Inventory

    Iodide is an essential nutrient for thyroid hormone synthesis and severe iodide deficiency (ID) during early development is associated with neurological impairments. Several environmental contaminants can perturb the thyroid axis and this perturbation may be more acute under cond...

  14. Acute scurvy during treatment with interleukin-2.

    PubMed

    Alexandrescu, D T; Dasanu, C A; Kauffman, C L

    2009-10-01

    The association of vitamin C deficiency with nutritional factors is commonly recognized. However, an acute form of scurvy can occur in patients with an acute systemic inflammatory response, which is produced by sepsis, medications, cancer or acute inflammation. The frequency of acute hypovitaminosis C in hospitalized patients is higher than previously recognized. We report the occurrence of acute signs and symptoms of scurvy (perifollicular petechiae, erythema, gingivitis and bleeding) in a patient hospitalized for treatment of metastatic renal-cell carcinoma with high-dose interleukin-2. Concomitantly, serum vitamin C levels decreased to below normal. Better diets and longer lifespan may result a lower frequency of acute scurvy and a higher frequency of scurvy associated with systemic inflammatory responses. Therefore, increased awareness of this condition can lead to early recognition of the cutaneous signs of acute scurvy in hospitalized patients with acute illnesses or in receipt of biological agents, and prevent subsequent morbidity such as bleeding, anaemia, impaired immune defences, oedema or neurological symptoms.

  15. Grammatical Impairments in PPA

    PubMed Central

    Thompson, Cynthia K.; Mack, Jennifer E.

    2015-01-01

    Background Grammatical impairments are commonly observed in the agrammatic subtype of primary progressive aphasia (PPA-G), whereas grammatical processing is relatively preserved in logopenic (PPA-L) and semantic (PPA-S) subtypes. Aims We review research on grammatical deficits in PPA and associated neural mechanisms, with discussion focused on production and comprehension of four aspects of morphosyntactic structure: grammatical morphology, functional categories, verbs and verb argument structure, and complex syntactic structures. We also address assessment of grammatical deficits in PPA, with emphasis on behavioral tests of grammatical processing. Finally, we address research examining the effects of treatment for progressive grammatical impairments. Main Contribution PPA-G is associated with grammatical deficits that are evident across linguistic domains in both production and comprehension. PPA-G is associated with damage to regions including the left inferior frontal gyrus (IFG) and dorsal white matter tracts, which have been linked to impaired comprehension and production of complex sentences. Detailing grammatical deficits in PPA is important for estimating the trajectory of language decline and associated neuropathology. We, therefore, highlight several new assessment tools for examining different aspects of morphosyntactic processing in PPA. Conclusions Individuals with PPA-G present with agrammatic deficit patterns distinct from those associated with PPA-L and PPA-S, but similar to those seen in agrammatism resulting from stroke, and patterns of cortical atrophy and white matter changes associated with PPA-G have been identified. Methods for clinical evaluation of agrammatism, focusing on comprehension and production of grammatical morphology, functional categories, verbs and verb argument structure, and complex syntactic structures are recommended and tools for this are emerging in the literature. Further research is needed to investigate the real

  16. Loss-of-Function Mutations of ILDR1 Cause Autosomal-Recessive Hearing Impairment DFNB42

    PubMed Central

    Borck, Guntram; Rehman, Atteeq Ur; Lee, Kwanghyuk; Pogoda, Hans-Martin; Kakar, Naseebullah; von Ameln, Simon; Grillet, Nicolas; Hildebrand, Michael S.; Ahmed, Zubair M.; Nürnberg, Gudrun; Ansar, Muhammad; Basit, Sulman; Javed, Qamar; Morell, Robert J.; Nasreen, Nabilah; Shearer, A. Eliot; Ahmad, Adeel; Kahrizi, Kimia; Shaikh, Rehan S.; Ali, Rana A.; Khan, Shaheen N.; Goebel, Ingrid; Meyer, Nicole C.; Kimberling, William J.; Webster, Jennifer A.; Stephan, Dietrich A.; Schiller, Martin R.; Bahlo, Melanie; Najmabadi, Hossein; Gillespie, Peter G.; Nürnberg, Peter; Wollnik, Bernd; Riazuddin, Saima; Smith, Richard J.H.; Ahmad, Wasim; Müller, Ulrich; Hammerschmidt, Matthias; Friedman, Thomas B.; Riazuddin, Sheikh; Leal, Suzanne M.; Ahmad, Jamil; Kubisch, Christian

    2011-01-01

    By using homozygosity mapping in a consanguineous Pakistani family, we detected linkage of nonsyndromic hearing loss to a 7.6 Mb region on chromosome 3q13.31-q21.1 within the previously reported DFNB42 locus. Subsequent candidate gene sequencing identified a homozygous nonsense mutation (c.1135G>T [p.Glu379X]) in ILDR1 as the cause of hearing impairment. By analyzing additional consanguineous families with homozygosity at this locus, we detected ILDR1 mutations in the affected individuals of 10 more families from Pakistan and Iran. The identified ILDR1 variants include missense, nonsense, frameshift, and splice-site mutations as well as a start codon mutation in the family that originally defined the DFNB42 locus. ILDR1 encodes the evolutionarily conserved immunoglobulin-like domain containing receptor 1, a putative transmembrane receptor of unknown function. In situ hybridization detected expression of Ildr1, the murine ortholog, early in development in the vestibule and in hair cells and supporting cells of the cochlea. Expression in hair cell- and supporting cell-containing neurosensory organs is conserved in the zebrafish, in which the ildr1 ortholog is prominently expressed in the developing ear and neuromasts of the lateral line. These data identify loss-of-function mutations of ILDR1, a gene with a conserved expression pattern pointing to a conserved function in hearing in vertebrates, as underlying nonsyndromic prelingual sensorineural hearing impairment. PMID:21255762

  17. Acute Vestibulopathy

    PubMed Central

    Cha, Yoon-Hee

    2011-01-01

    The presentation of acute vertigo may represent both a common benign disorder or a life threatening but rare one. Familiarity with the common peripheral vestibular disorders will allow the clinician to rapidly “rule-in” a benign disorder and recognize when further testing is required. Key features of vertigo required to make an accurate diagnosis are duration, chronicity, associated symptoms, and triggers. Bedside tests that are critical to the diagnosis of acute vertigo include the Dix-Hallpike maneuver and canalith repositioning manuever, occlusive ophthalmoscopy, and the head impulse test. The goal of this review is to provide the clinician with the clinical and pathophysiologic background of the most common disorders that present with vertigo to develop a logical differential diagnosis and management plan. PMID:23983835

  18. Acute Blindness.

    PubMed

    Meekins, Jessica M

    2015-09-01

    Sudden loss of vision is an ophthalmic emergency with numerous possible causes. Abnormalities may occur at any point within the complex vision pathway, from retina to optic nerve to the visual center in the occipital lobe. This article reviews specific prechiasm (retina and optic nerve) and cerebral cortical diseases that lead to acute blindness. Information regarding specific etiologies, pathophysiology, diagnosis, treatment, and prognosis for vision is discussed.

  19. [Diabetes mellitus in acute pancreatitis].

    PubMed

    Díaz-Rubio, José Luis; Torre-Delgadillo, Aldo; Robles-Díaz, Guillermo

    2002-01-01

    Exocrine and endocrine components of pancreas are interrelated anatomically and functionally. Exocrine pancreatic dysfunction often accompanies endocrine pancreatic impairment and vice versa. Diabetes mellitus resulting from alterations of exocrine pancreas, such as acute or chronic pancreatitis, is known as pancreatic diabetes. Hyperglycemia during acute pancreatitis (AP) can be due to abnormalities in insulin secretion, increase in counterregulatory hormones release, or decrease in glucose utilization by peripheral tissues. Causal association is suggested between diabetic ketoacidosis and AP and is attributed to alternation in metabolism of triglycerides. High blood glucose levels are associated with severe AP and constitute factor of worst prognosis. Some patients are discharged with diabetes after AP episode, while others develop diabetes during first year of follow-up. Origin and frequency of glycemic abnormalities associated with AP have not been settled yet accurately. Also, predictive factors for diabetes development and persistence after AP have not been recognized to date.

  20. Frailty and cognitive impairment: Unique challenges in the older emergency surgical patient

    PubMed Central

    Moug, SJ; Stechman, M; McCarthy, K; Pearce, L; Myint, PK; Hewitt, J

    2016-01-01

    Introduction Older patients (>65 years of age) admitted as general surgical emergencies increasingly require improved recognition of their specific needs relative to younger patients. Two such needs are frailty and cognitive impairment. These are evolving research areas that the emergency surgeon increasingly requires knowledge of to improve short- and long-term patient outcomes. Methods This paper reviews the evidence for frailty and cognitive impairment in the acute surgical setting by defining frailty and cognitive impairment, introducing methods of diagnosis, discussing the influence on prognosis and proposing strategies to improve older patient outcomes. Results Frailty is present in 25% of the older surgical population. Using frailty-scoring tools, frailty was associated with a significantly longer hospital stay and higher mortality at 30 and 90 days after admission to an acute surgical unit. Cognitive impairment is present in a high number of older acute surgical patients (approximately 70%), whilst acute onset cognitive impairment, termed delirium, is documented in 18%. However, patients with delirium had significantly longer hospital stays and higher in-hospital mortality than those with cognitive impairment. Conclusions Improved knowledge of frailty and delirium by the emergency surgeon allows the specialised needs of older surgical patients to be taken into account. Early recognition, and consideration of minimally invasive surgery or radiological intervention alongside potentially transferable successful elective interventions such as comprehensive geriatric assessment, may help to improve short- and long-term patient outcomes in this vulnerable population. PMID:26890834

  1. Butalbital and driving impairment.

    PubMed

    Yeakel, Jillian K; Logan, Barry K

    2013-07-01

    Butalbital (Fiorinal(®)), used in the treatment of migraines and muscle pain, is the most commonly encountered barbiturate in impaired driving cases. It has central nervous system (CNS) depressant properties, including sedation, drowsiness, and feelings of intoxication, which can contribute to driving impairment. Twenty-six driving under the influence cases are reviewed including results from field sobriety tests and toxicology testing. Blood samples were screened using enzyme multiplied immunoassay technique immunoassay, and the presence of butalbital was confirmed and quantified using gas chromatography/mass spectrometry, gas chromatography with flame ionization detection, or gas chromatography nitrogen/phosphorus detection. Butalbital concentrations ranged from 1.0 to 30.2 mg/L, with a mean and median of 16.0 mg/L. General impairment indicators in these cases included horizontal and vertical nystagmus, lack of convergence, poor motor coordination, and balance and speech problems, which are common to CNS depressant intoxication, similar to that associated with alcohol. These findings indicate the importance of toxicological testing for butalbital in cases where CNS depressants are indicated.

  2. Neurological Impairment: Nomenclature and Consequences.

    ERIC Educational Resources Information Center

    Spears, Catherine E.; Weber, Robert E.

    Neurological impairment as discussed includes a range of disabilities referred to as neurological impairment: minimal brain dysfunction/damage, developmental disability, perceptual handicap, learning disability, hyperkinetic behavioral syndrome, and others. Defined are causes of neurological impairment and methods of diagnosis. Symptoms…

  3. Assessment of Hearing