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Progressive hearing loss

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https://www.readbyqxmd.com/read/28334328/vestibular-aqueduct-midpoint-width-and-hearing-loss-in-patients-with-an-enlarged-vestibular-aqueduct
#1
Mustafa S Ascha, Nauman Manzoor, Amit Gupta, Maroun Semaan, Cliff Megerian, Todd D Otteson
Importance: Elucidating the relationship between vestibular aqueduct size and hearing loss progression may inform the prognosis and counseling of patients who have an enlarged vestibular aqueduct (EVA). Objectives: To examine the association between vestibular aqueduct size and repeated measures of hearing loss. Design, Setting, and Participants: For this retrospective medical record review, 52 patients with a diagnosis of hearing loss and radiologic diagnosis of EVA according to the Valvassori criterion were included...
March 23, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28328494/hearing-aid-technology-for-the-21st-century-a-proposal-for-universal-wireless-connectivity-and-improved-sound-quality
#2
Richard Einhorn
Approximately 360 million people in the world live with a debilitating hearing loss. The most common conditions-age-related and noise-induced sensorineural hearing loss-are both progressive and, for the foreseeable future, neither curable nor reversible.
March 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28328138/homozygous-mutation-in-ptrh2-gene-causes-progressive-sensorineural-deafness-and-peripheral-neuropathy
#3
Rajech Sharkia, Stavit A Shalev, Abdelnaser Zalan, Milit Marom-David, Nathan Watemberg, Jill E Urquhart, Sarah B Daly, Sanjeev S Bhaskar, Simon G Williams, William G Newman, Ronen Spiegel, Abdussalam Azem, Orly Elpeleg, Muhammad Mahajnah
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...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28327385/acoustic-startle-modification-as-a-tool-for-evaluating-auditory-function-of-the-mouse-progress-pitfalls-and-potential
#4
REVIEW
Amanda M Lauer, Derik Behrens, Georg Klump
Acoustic startle response (ASR) modification procedures, especially prepulse inhibition (PPI), are increasingly used as behavioral measures of auditory processing and sensorimotor gating in rodents due to their perceived ease of implementation and short testing times. In practice, ASR and PPI procedures are extremely variable across animals, experimental setups, and studies, and the interpretation of results is subject to numerous caveats and confounding influences. We review considerations for modification of the ASR using acoustic stimuli, and we compare the sensitivity of PPI procedures to more traditional operant psychoacoustic techniques...
March 18, 2017: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/28322503/a-novel-aberrant-splice-site-mutation-in-col27a1-is-responsible-for-steel-syndrome-and-extension-of-the-phenotype-to-include-hearing-loss
#5
Nesrin Gariballa, Afif Ben-Mahmoud, Makanko Komara, Aisha M Al-Shamsi, Anne John, Bassam R Ali, Lihadh Al-Gazali
Steel syndrome is an autosomal recessive disease characterized by skeletal abnormalities and dysmorphic features. The first mutation associated with this syndrome was reported in Puerto Rican children. In this study, we identified a novel homozygous splice site variant in COL27A1 (c.3556-2A>G) in a consanguineous Emirati family with a child affected by Steel syndrome. In addition, the affected child had severe non-progressive sensorineural hearing loss not reported previously. The variant segregated in the family in an autosomal recessive manner and we show that the variant alters mRNA splicing...
March 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28318405/the-association-between-age-related-hearing-impairment-and-metabolic-syndrome-in-korean-women-5-year-follow-up-observational-study
#6
Tae Su Kim, Eun Hui Kim, Jong Woo Chung
BACKGROUND: Although several observational studies showed a relationship between various conditions of metabolic syndrome (MetS) and hearing threshold, there are no studies about longitudinal audiometric results related MetS. The aim of this study was to investigate the association between MetS and age-related hearing impairment (ARHI) through a large, average 5-year longitudinal follow-up, clinical comparative analysis. MATERIALS AND METHODS: We recruited 1381 women older than 50 years who were enrolled in 2007 and reevaluated in 2012...
March 20, 2017: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/28314816/characterization-of-a-human-point-mutation-of-vglut3-p-a211v-in-the-rodent-brain-suggests-a-non-uniform-distribution-of-the-transporter-in-synaptic-vesicles
#7
Lauriane Ramet, Johannes Zimmermann, Tiphaine Bersot, Odile Poirel, Stéphanie De Gois, Katlin Silm, Diana Yae Sakae, Nina Mansouri-Guilani, Marie-Josée Bourque, Louis-Eric Trudeau, Nicolas Pietrancosta, Stéphanie Daumas, Véronique Bernard, Christian Rosenmund, Salah El Mestikawy
The atypical vesicular glutamate transporter type 3 (VGLUT3) is expressed by sub-populations of neurons using acetylcholine, GABA or serotonin as neurotransmitters. In addition, VGLUT3 is expressed in the inner hair cells of the auditory system. A mutation (p.A211V) in the gene that encodes VGLUT3 is responsible for progressive deafness in two unrelated families. In this study, we investigated the consequences of the p.A211V mutation in cell cultures and in the central nervous system (CNS) of a mutant mouse...
March 17, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28296514/can-unilateral-progressive-or-sudden-hearing-loss-be-immune-mediated-in-origin
#8
Francesca Atturo, Roberta Colangeli, Giorgio Bandiera, Maurizio Barbara, Simonetta Monini
OBJECTIVE: The aim of the present study was to demonstrate that the positivity of nonspecific immunological tests could be found not only in bilateral hearing loss but also in unilateral cases, either sudden or progressive. METHOD: An observational case series study included subjects suffering from unilateral or bilateral, sudden or progressive, symmetric or asymmetric sensorineural hearing loss (SNHL). All the patients underwent pure tone audiometry and the following battery of blood exams: anti-nuclear antibody (ANA), extractable nuclear antigen (ENA) antibody screening, anti-thyroperoxidase (anti-TPO), anti-thyroglobulin and anti-smooth muscle antibody (ASMA)...
March 15, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28283078/distortion-product-otoacoustic-emissions-in-young-adult-and-geriatric-cats
#9
George M Strain, Kain A McGee
Recordings of distortion product otoacoustic emissions (DPOAEs) were taken from 15 geriatric cats (mean age ± standard deviation, SD, 13.6 ± 2.7 years; range 10.2-19.4 years) and 12 young adult control cats (mean ± SD 4.6 ± 0.5 years; range 3.4-5 years) to identify frequency-specific age-related changes in cochlear responses. Recordings were performed for primary frequencies from 2 to 12 kHz in 2 kHz increments. Cats were considered to be geriatric > 11.9 ± 1.9 years of age...
March 2017: Veterinary Journal
https://www.readbyqxmd.com/read/28281033/a-treatable-cause-of-myelopathy-and-vision-loss-mimicking-neuromyelitis-optica-spectrum-disorder-late-onset-biotinidase-deficiency
#10
Sanem Yilmaz, Mine Serin, Ebru Canda, Cenk Eraslan, Hande Tekin, Sema Kalkan Ucar, Sarenur Gokben, Hasan Tekgul, Gul Serdaroglu
Biotinidase deficiency is characterized by severe neurological manifestations as hypotonia, lethargy, ataxia, hearing loss, seizures and developmental retardation in its classical form. Late-onset biotinidase deficiency presents distinctly from the classical form such as limb weakness and vision problems. A 14-year-old boy presented with progressive vision loss and upper limb weakness. The patient was initiated steroid therapy with a preliminary diagnosis of neuromyelitis optica spectrum disorder due to the craniospinal imaging findings demonstrating optic nerve, brainstem and longitudinally extensive spinal cord involvement...
March 9, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28280462/the-role-of-glia-in-the-peripheral-and-central-auditory-system-following-noise-overexposure-contribution-of-tnf-%C3%AE-and-il-1%C3%AE-to-the-pathogenesis-of-hearing-loss
#11
Verónica Fuentes-Santamaría, Juan Carlos Alvarado, Pedro Melgar-Rojas, María C Gabaldón-Ull, Josef M Miller, José M Juiz
Repeated noise exposure induces inflammation and cellular adaptations in the peripheral and central auditory system resulting in pathophysiology of hearing loss. In this study, we analyzed the mechanisms by which noise-induced inflammatory-related events in the cochlea activate glial-mediated cellular responses in the cochlear nucleus (CN), the first relay station of the auditory pathway. The auditory function, glial activation, modifications in gene expression and protein levels of inflammatory mediators and ultrastructural changes in glial-neuronal interactions were assessed in rats exposed to broadband noise (0...
2017: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/28269468/a-home-automation-based-environmental-sound-alert-for-people-experiencing-hearing-loss
#12
Matthias Mielke, Rainer Bruck
Different assistive technologies are available for deaf people (i.e. deaf, deafened, and hard of hearing). Besides the well-known hearing aid, devices for detection of sound events that occur at home or at work (e.g. doorbell, telephone) are available. Despite the technological progress in the last years and resulting new possibilities, the basic functions and concepts of such devices have not changed. The user still needs special assistive technology that is bound to the home or work environment. In this contribution a new concept for awareness of events in buildings is presented...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28241924/primary-human-cytomegalovirus-hcmv-infection-in-pregnancy
#13
Horst Buxmann, Klaus Hamprecht, Matthias Meyer-Wittkopf, Klaus Friese
BACKGROUND: In 0.5-4% of pregnancies, the prospective mother sustains a primary infection with human cytomegalovirus (HCMV). An HCMV infection of the fetus in the first or second trimester can cause complex post-encephalitic impairment of the infant brain, leading to motor and mental retardation, cerebral palsy, epilepsy, retinal defects, and progressive hearing loss. METHODS: This review is based on pertinent publications from January 2000 to October 2016 that were retrieved by a selective search in PubMed employing the terms "cytomegalovirus and pregnancy" and "congenital cytomegalovirus...
January 27, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28241884/radiotherapy-of-nasopharyngeal-cancer-using-rapidarc-dosimetric-study-of-military-teaching-hospital-mohamed-v-morocco
#14
Issam Lalya, El Amin Marnouche, Maghous Abdelhak, Noha Zaghba, Khalid Andaloussi, Mohamed Elmarjany, Laila Baddouh, Keltoum Dahmani, Khalid Hadadi, Hassan Sifat, Hamid Mansouri
BACKGROUND: The aim of the present study is to assess efficacy and efficiency of Volumetric-modulated arc therapy (VMAT) technique in treatment of nasopharyngeal cancer in our institution and to report toxicity related to this technique. METHODS: Between June 2013 and January 2015, thirty-two patients with non metastatic nasopharyngeal cancer were curatively treated using VMAT Rapidarc. Dose prescription was performed using two different schedules, it consisted of either simultaneous integrated boost or simultaneous modulated accelerated radiation therapy delivering 70 Gy in 35 fractions and 69...
February 28, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28240983/validation-of-a-computer-administered-version-of-the-digits-in-noise-test-for-hearing-screening-in-the-united-states
#15
Robert L Folmer, Jay Vachhani, Garnett P McMillan, Charles Watson, Gary R Kidd, M Patrick Feeney
BACKGROUND: The sooner people receive treatment for hearing loss (HL), the quicker they are able to recognize speech and to master hearing aid technology. Unfortunately, a majority of people with HL wait until their impairments have progressed from moderate to severe levels before seeking auditory rehabilitation. To increase the number of individuals with HL who pursue and receive auditory rehabilitation, it is necessary to improve methods for identifying and informing these people via widely accessible hearing screening procedures...
February 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28227718/a-home-automation-based-environmental-sound-alert-for-people-experiencing-hearing-loss
#16
Matthias Mielke, Rainer Bruck, Matthias Mielke, Rainer Bruck, Rainer Bruck, Matthias Mielke
Different assistive technologies are available for deaf people (i.e. deaf, deafened, and hard of hearing). Besides the well-known hearing aid, devices for detection of sound events that occur at home or at work (e.g. doorbell, telephone) are available. Despite the technological progress in the last years and resulting new possibilities, the basic functions and concepts of such devices have not changed. The user still needs special assistive technology that is bound to the home or work environment. In this contribution a new concept for awareness of events in buildings is presented...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28226077/superficial-siderosis-of-the-central-nervous-system-is-a-rare-and-possibly-underdiagnosed-disorder
#17
Yara Dadalti Fragoso, Tarso Adoni, Joseph Bruno Bidin Brooks, Sidney Gomes, Marcus Vinicius Magno Goncalves, Cassio Lemos Jovem, Andre Palma da Cunha Matta, Joao Filipe Oliveira, Fabio Siquinelli, Carlos Bernardo Tauil, Guilherme Navarro Troiani, Paulo Roberto Wille
Methods: Series of cases collected from Brazilian centers. Results: We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion: SS-CNS is a rare disease that may remain undiagnosed for long periods...
February 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28225734/auditory-performance-and-electrical-stimulation-measures-in-cochlear-implant-recipients-with-auditory-neuropathy-compared-with-severe-to-profound-sensorineural-hearing-loss
#18
Joseph Attias, Tally Greenstein, Miriam Peled, David Ulanovski, Jay Wohlgelernter, Eyal Raveh
OBJECTIVES: The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). DESIGN: The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3...
March 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28224282/micrornas-effective-elements-in-ear-related-diseases-and-hearing-loss
#19
REVIEW
Mohammad-Reza Mahmoudian-Sani, Ameneh Mehri-Ghahfarrokhi, Fereshteh Ahmadinejad, Morteza Hashemzadeh-Chaleshtori, Massoud Saidijam, Mohammad-Saeid Jami
miRNAs are important factors for post-transcriptional process that controls gene expression at mRNA level. Various biological processes, including growth and differentiation, are regulated by miRNAs. miRNAs have been demonstrated to play an essential role in development and progression of hearing loss. Nowadays, miRNAs are known as critical factors involved in different physiological, biological, and pathological processes, such as gene expression, progressive sensorineural hearing loss, age-related hearing loss, noise-induced hearing loss, cholesteatoma, schwannomas, and inner ear inflammation...
February 21, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28223190/association-of-tagsnp-in-lncrna-hotair-with-susceptibility-to-noise-induced-hearing-loss-in-a-chinese-population
#20
Boshen Wang, Enmin Ding, Huanxi Shen, Jianfeng Wang, Kai Sun, Shi Chen, Lei Han, Hengdong Zhang, Baoli Zhu, Ming Xu
BACKGROUND: Noise-induced hearing loss (NIHL) is a multifactorial disease, and dysregulation of oxidative stress is universally acknowledged as one crucial pathogenic factor for this disease. Recently studies have found the LncRNA HOTAIR is involved in the alteration of oxidative stress level, cell proliferation, cell cycle progression, and apoptosis. Considering the effects of lncRNA HOTAIR in cellular oxidative stress, we sought to investigate the influence of lncRNA HOTAIR variants on the risk of NIHL...
February 18, 2017: Hearing Research
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