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https://www.readbyqxmd.com/read/29451456/clival-meningocele-causing-bilateral-hearing-loss-in-a-child-due-to-superficial-siderosis-of-the-central-nervous-system-case-report
#1
Stephen J Johans, Kevin N Swong, Daniel J Burkett, Michael P Wemhoff, Sean M Lew, Chirag R Patel, Anand V Germanwala
Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated...
February 16, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29451107/bilaterally-combined-electric-and-acoustic-hearing-in-mandarin-speaking-listeners-the-population-with-poor-residual-hearing
#2
Duo-Duo Tao, Ji-Sheng Liu, Zhen-Dong Yang, Blake S Wilson, Ning Zhou
The hearing loss criterion for cochlear implant candidacy in mainland China is extremely stringent (bilateral severe to profound hearing loss), resulting in few patients with substantial residual hearing in the nonimplanted ear. The main objective of the current study was to examine the benefit of bimodal hearing in typical Mandarin-speaking implant users who have poorer residual hearing in the nonimplanted ear relative to those used in the English-speaking studies. Seventeen Mandarin-speaking bimodal users with pure-tone averages of ∼80 dB HL participated in the study...
January 2018: Trends in Hearing
https://www.readbyqxmd.com/read/29450971/evaluation-of-an-abutment-level-superpower-sound-processor-for-bone-anchored-hearing
#3
Arjan J Bosman, Ivo J Kruyt, Emmanuel Am Mylanus, Myrthe Ks Hol, Ad Fm Snik
OBJECTIVES: Performance of an abutment-level superpower sound processor for bone-anchored hearing, the Ponto 3 SuperPower from Oticon Medical (BCD2), was compared to an earlier model from Oticon Medical (BCD1). DESIGN: A comparative study in which each patient serves as its own control. SETTING: Tertiary clinic. PARTICIPANTS: Eighteen experienced BCD1 users with profound mixed hearing loss. MAIN OUTCOME MEASURES: speech reception thresholds in noise; APHAB and SSQ questionnaires...
February 16, 2018: Clinical Otolaryngology
https://www.readbyqxmd.com/read/29450493/the-impact-of-age-background-noise-semantic-ambiguity-and-hearing-loss-on-recognition-memory-for-spoken-sentences
#4
Margaret A Koeritzer, Chad S Rogers, Kristin J Van Engen, Jonathan E Peelle
Purpose: The goal of this study was to determine how background noise, linguistic properties of spoken sentences, and listener abilities (hearing sensitivity and verbal working memory) affect cognitive demand during auditory sentence comprehension. Method: We tested 30 young adults and 30 older adults. Participants heard lists of sentences in quiet and in 8-talker babble at signal-to-noise ratios of +15 dB and +5 dB, which increased acoustic challenge but left the speech largely intelligible...
February 15, 2018: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/29450484/neurological-manifestations-among-us-government-personnel-reporting-directional-audible-and-sensory-phenomena-in-havana-cuba
#5
Randel L Swanson, Stephen Hampton, Judith Green-McKenzie, Ramon Diaz-Arrastia, M Sean Grady, Ragini Verma, Rosette Biester, Diana Duda, Ronald L Wolf, Douglas H Smith
Importance: From late 2016 through August 2017, US government personnel serving on diplomatic assignment in Havana, Cuba, reported neurological symptoms associated with exposure to auditory and sensory phenomena. Objective: To describe the neurological manifestations that followed exposure to an unknown energy source associated with auditory and sensory phenomena. Design, Setting, and Participants: Preliminary results from a retrospective case series of US government personnel in Havana, Cuba...
February 15, 2018: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29449017/why-do-hearing-aids-fail-to-restore-normal-auditory-perception
#6
REVIEW
Nicholas A Lesica
Hearing loss is a widespread condition that is linked to declines in quality of life and mental health. Hearing aids remain the treatment of choice, but, unfortunately, even state-of-the-art devices provide only limited benefit for the perception of speech in noisy environments. While traditionally viewed primarily as a loss of sensitivity, hearing loss is also known to cause complex distortions of sound-evoked neural activity that cannot be corrected by amplification alone. This Opinion article describes the effects of hearing loss on neural activity to illustrate the reasons why current hearing aids are insufficient and to motivate the use of new technologies to explore directions for improving the next generation of devices...
February 12, 2018: Trends in Neurosciences
https://www.readbyqxmd.com/read/29447894/cochlear-implantation-in-pediatric-patients-with-cockayne-syndrome
#7
Renae D Van Wyhe, Claudia V Emery, Robert A Williamson
Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously)...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29447820/how-does-a-bilingual-environment-affect-the-results-in-children-with-cochlear-implants-compared-to-monolingual-matched-children-an-italian-follow-up-study
#8
Francesca Forli, Giada Giuntini, Annalisa Ciabotti, Luca Bruschini, Ulrika Löfkvist, Stefano Berrettini
OBJECTIVES: To compare the results after cochlear implantation achieved by monolingual and bilingual deaf children implanted at our Institution, with the aim of understanding if there are any differences between the two groups and if there is a correlation between the outcomes and some patients' variables. METHODS: The study group was composed by 14 bilingual deaf children and the control group by the same number of monolingual children implanted at our Institution...
February 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446191/speech-understanding-in-noise-in-elderly-adults-the-effect-of-inhibitory-control-and-syntactic-complexity
#9
Eline C van Knijff, Martine Coene, Paul J Govaerts
BACKGROUND: Previous research has suggested that speech perception in elderly adults is influenced not only by age-related hearing loss or presbycusis but also by declines in cognitive abilities, by background noise and by the syntactic complexity of the message. AIMS: To gain further insight into the influence of these cognitive as well as acoustic and linguistic factors on speech perception in elderly adults by investigating inhibitory control as a listener characteristic and background noise type and syntactic complexity as input characteristics...
February 15, 2018: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29441214/syndromic-hearing-loss-a-brief-review-of-common-presentations-and-genetics
#10
REVIEW
John D Gettelfinger, John P Dahl
Congenital hearing loss is one of the most common birth defects worldwide, with around 1 in 500 people experiencing some form of severe hearing loss. While over 400 different syndromes involving hearing loss have been described, it is important to be familiar with a wide range of syndromes involving hearing loss so an early diagnosis can be made and early intervention can be pursued to maximize functional hearing and speech-language development in the setting of verbal communication. This review aims to describe the presentation and genetics for some of the most frequently occurring syndromes involving hearing loss, including neurofibromatosis type 2, branchio-oto-renal syndrome, Treacher Collins syndrome, Stickler syndrome, Waardenburg syndrome, Pendred syndrome, Jervell and Lange-Nielsen syndrome, Usher syndromes, Refsum disease, Alport syndrome, MELAS, and MERRF...
March 2018: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/29439330/hypoxia-induced-signaling-activation-in-neurodegenerative-diseases-targets-for-new-therapeutic-strategies
#11
Niraj Kumar Jha, Saurabh Kumar Jha, Renu Sharma, Dhiraj Kumar, Rashmi K Ambasta, Pravir Kumar
For the maintenance of cellular homeostasis and energy metabolism, an uninterrupted supply of oxygen (O2) is routinely required in the brain. However, under the impaired level of O2 (hypoxia) or reduced blood flow (ischemia), the tissues are not sufficiently oxygenated, which triggers disruption of cellular homeostasis in the brain. Hypoxia is known to have a notable effect on controlling the expression of proteins involved in a broad range of biological processes varying from energy metabolism, erythropoiesis, angiogenesis, neurogenesis to mitochondrial trafficking and autophagy, thus facilitating neuronal cells to endure in deprived O2...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29437513/cleft-lip-and-palate-in-charge-syndrome-phenotypic-features-that-influence-management
#12
Kathryn V Isaac, Ingrid M Ganske, Stephen A Rottgers, So Young Lim, John B Mulliken
OBJECTIVE: Infants with syndromic cleft lip and/or cleft palate (CL/P) often require more complex care than their nonsyndromic counterparts. Our purpose was to (1) determine the prevalence of CL/P in patients with CHARGE syndrome and (2) highlight factors that affect management in this subset of children. DESIGN: This is a retrospective review from 1998 to 2016. PATIENTS: Patients with CHARGE syndrome were diagnosed clinically and genetically...
March 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29432110/the-cochlear-implant-eeg-artifact-recorded-from-an-artificial-brain-for-complex-acoustic-stimuli
#13
Luise Wagner, Natasha Maurits, Bert Maat, Deniz Baskent, Anita E Wagner
Electroencephalographic (EEG) recordings provide objective estimates of listeners' cortical processing of sounds and of the status of their speech perception system. For profoundly deaf listeners with cochlear implants (CIs), the applications of EEG are limited because the device adds electric artifacts to the recordings. This restricts the possibilities for the neural-based metrics of speech processing by CI users, for instance to gauge cortical reorganization due to individual's hearing loss history. This paper describes the characteristics of the CI artifact as recorded with an artificial head substitute, and reports how the artifact is affected by the properties of the acoustical input signal versus the settings of the device...
February 2018: IEEE Transactions on Neural Systems and Rehabilitation Engineering
https://www.readbyqxmd.com/read/29431654/enhanced-operant-extinction-and-prefrontal-excitability-in-a-mouse-model-of-angelman-syndrome
#14
Michael S Sidorov, Matthew C Judson, Hyojin Kim, Marie Rougie, Alejandra I Ferrer, Viktoriya D Nikolova, Natallia V Riddick, Sheryl S Moy, Benjamin D Philpot
Angelman syndrome (AS), a neurodevelopmental disorder associated with intellectual disability, is caused by loss of maternal allele expression of UBE3A in neurons. Mouse models of AS faithfully recapitulate disease phenotypes across multiple domains, including behavior. Yet in AS, there has been only limited study of behaviors encoded by the prefrontal cortex, a region broadly involved in executive function and cognition. Because cognitive impairment is a core feature of AS, it is critical to develop behavioral readouts of prefrontal circuit function in AS mouse models...
February 5, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29431348/new-technique-for-large-mandibular-and-surrounding-soft-tissue-reconstruction-that-uses-an-intraoral-three-dimensional-distractor-for-one-step-transport-disc-distraction-osteogenesis
#15
Antonio Cortese, Mario Caggiano, Giuseppe Pantaleo, Massimo Amato, Pier P Claudio
BACKGROUND: Mandibular reconstruction after ablative surgery is challenging not only for the amount of bone tissue needed following bone loss after benign or malignant tumor surgical removal, but mainly to reconstruct bone shape and structure necessary for subsequent implant insertion to preserve the function (speech, swallowing and chewing) and aesthetic of the lower face. Several different techniques for maxillary bone defects reconstruction have been described. The aim of our work is to describe a new technique for mandibular reconstruction that uses an intramural distractor, which is placed in one surgical step and without the need for a skin incision...
February 9, 2018: Minerva Stomatologica
https://www.readbyqxmd.com/read/29430091/endodontic-treatment-of-chronically-infected-primary-teeth-using-triple-antibiotic-paste-an-in-vivo-study
#16
G Anuradha Reddy, E Sridevi, A J Sai Sankar, K Pranitha, M J S Pratap Gowd, C Vinay
Aim: Maintenance of primary dentition is imperative for the development of arch form, esthetics, function, mastication, and normal eruption of permanent teeth. Premature loss can cause various problems such as ectopic eruption, space loss for the successor permanent teeth, alterations in speech, and impairment of function. Hence, decayed primary teeth should not be extracted and treated whenever possible. Pulpectomy is generally recommended as a treatment of choice in such cases. The purpose of this study was to evaluate the clinical and radiographic success of pulpectomized primary teeth with chronic infection using a mixture of metronidazole, ciprofloxacin, and minocycline (3MIX)-MP as an intracanal medicament before the obturation...
November 2017: Journal of Conservative Dentistry: JCD
https://www.readbyqxmd.com/read/29427102/spinocerebellum-ataxia-type-6-molecular-mechanisms-and-calcium-channel-genetics
#17
Xiaofei Du, Christopher Manuel Gomez
Spinocerebellar ataxia (SCA) type 6 is an autosomal dominant disease affecting cerebellar degeneration. Clinically, it is characterized by pure cerebellar dysfunction, slowly progressive unsteadiness of gait and stance, slurred speech, and abnormal eye movements with late onset. Pathological findings of SCA6 include a diffuse loss of Purkinje cells, predominantly in the cerebellar vermis. Genetically, SCA6 is caused by expansion of a trinucleotide CAG repeat in the last exon of longest isoform CACNA1A gene on chromosome 19p13...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29415585/semantic-context-improves-speech-intelligibility-and-reduces-listening-effort-for-listeners-with-hearing-impairment
#18
Emma Holmes, Paula Folkeard, Ingrid S Johnsrude, Susan Scollie
OBJECTIVE: We investigated whether speech intelligibility and listening effort for hearing-aid users is affected by semantic context and hearing-aid setting. DESIGN: Participants heard target sentences spoken in a reverberant background of cafeteria noise and competing speech. Participants reported each sentence verbally. Eight participants also rated listening effort after each sentence. Sentence topic was either the same as, or different from, the previous target sentence...
February 7, 2018: International Journal of Audiology
https://www.readbyqxmd.com/read/29410512/clinical-and-molecular-characteristics-of-newly-reported-mitochondrial-disease-entity-caused-by-biallelic-pars2-mutations
#19
Elżbieta Ciara, Dariusz Rokicki, Michal Lazniewski, Hanna Mierzewska, Elżbieta Jurkiewicz, Monika Bekiesińska-Figatowska, Dorota Piekutowska-Abramczuk, Katarzyna Iwanicka-Pronicka, Edyta Szymańska, Piotr Stawiński, Joanna Kosińska, Agnieszka Pollak, Maciej Pronicki, Dariusz Plewczyński, Rafał Płoski, Ewa Pronicka
Most of the 19 mitochondrial aminoacyl-tRNA synthetases (mt-aaRSs) involved in mitochondrial protein synthesis are already linked to specific entities, one of the exceptions being PARS2 mutations for which pathogenic significance is not finally validated. The aim of the study was to characterize the PARS2- related phenotype.Three siblings with biallelic PARS2 mutations presented from birth with infantile spasms, secondary microcephaly, and similar facial dysmorphy. Mental development was deeply impaired with speech absence and no eye contact...
February 6, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29405837/asymmetric-hearing-loss-prompting-mri-referral-in-a-military-population-redefining-audiometric-criteria
#20
Anthony M Tolisano, Ricardo M Burgos, Michael B Lustik, Lex A Mitchell, Philip D Littlefield
Objective To reevaluate asymmetric sensorineural hearing loss (ASNHL) criteria used to justify magnetic resonance imaging (MRI) in the evaluation of retrocochlear tumors in a military population. Study Design Retrospective case-control study. Setting Tertiary care military medical center. Subjects and Methods Patients with military service and a history of ASNHL prompting referral for MRI, with or without retrocochlear tumors, were compared between 2005 and 2016. Predictor variables included pure tone ASNHL, speech audiometry, and a history of noise exposure...
February 1, 2018: Otolaryngology—Head and Neck Surgery
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