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https://www.readbyqxmd.com/read/29331570/meningeal-marginal-zone-b-cell-lymphoma-the-meningioma-trap
#1
Alexandre Villeneuve, François Rubin, Pierre Bonfils
OBJECTIVE: To report a case of marginal zone MALT lymphoma of the temporal dura mater, initially mistaken for temporal meningioma. CASE REPORT: A 60-year-old immunocompetent woman, followed for more than 10 years for temporal meningioma causing vertigo and mixed hearing loss, presented with cervical lymphadenopathy, revealing marked progression of an intracranial lesion, leading to a diagnosis of marginal zone MALT lymphoma based on histological examination of a cervical lymph node...
January 10, 2018: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/29327735/acquired-sensorineural-hearing-loss-in-children-current-research-and-therapeutic-perspectives
#2
M Ralli, R Rolesi, R Anzivino, R Turchetta, A R Fetoni
The knowledge of mechanisms responsible for acquired sensorineural hearing loss in children, such as viral and bacterial infections, noise exposure, aminoglycoside and cisplatin ototoxicity, is increasing and progressively changing the clinical management of affected patients. Viral infections are by far the most relevant cause of acquired hearing loss, followed by aminoglycoside and platinum derivative ototoxicity; moreover, cochlear damage induced by noise overexposure, mainly in adolescents, is an emerging topic...
December 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/29327200/exocyst-complex-member-exoc5-is-required-for-survival-of-hair-cells-and-spiral-ganglion-neurons-and-maintenance-of-hearing
#3
Byeonghyeon Lee, Jeong-In Baek, Hyehyun Min, Seung-Hyun Bae, Kyeonghye Moon, Min-A Kim, Ye-Ri Kim, Ben Fogelgren, Joshua H Lipschutz, Kyu-Yup Lee, Jinwoong Bok, Un-Kyung Kim
The exocyst, an octameric protein complex consisting of Exoc1 through Exoc8, was first determined to regulate exocytosis by targeting vesicles to the plasma membrane in yeast to mice. In addition to this fundamental role, the exocyst complex has been implicated in other cellular processes. In this study, we investigated the role of the exocyst in cochlear development and hearing by targeting EXOC5, a central exocyst component. Deleting Exoc5 in the otic epithelium with widely used Cre lines resulted in early lethality...
January 11, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29326878/ten-year-long-enzyme-replacement-therapy-shows-a-poor-effect-in-alleviating-giant-leg-ulcers-in-a-male-with-fabry-disease
#4
Jun Okada, Mohammad Arif Hossain, Chen Wu, Takashi Miyajima, Hiroko Yanagisawa, Keiko Akiyama, Yoshikatsu Eto
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A (α-gal A), leading to the progressive accumulation of glycosphingolipids. Classical hemizygous males usually present symptoms, including pain and paresthesia in the extremities, angiokeratoma, hypo- or anhidrosis, abdominal pain, cornea verticillata, early stroke, tinnitus, and/or hearing loss, during early childhood or adolescence. Moreover, proteinuria, renal impairment, and cardiac hypertrophy can appear with age...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29324475/bilateral-cochlear-implants-maximizing-expected-outcomes
#5
Kate E Willis, Nathan J Blum, Stephanie A Waryasz, Marilyn Augustyn
Sonia is a 4 1/12-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 7/12 years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate...
January 10, 2018: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/29319131/temozolomide-therapy-for-aggressive-pituitary-crooke-s-cells-corticotropinoma-causing-cushing-s-disease-a-case-report-with-literature-review
#6
Aleksandra Gilis-Januszewska, Małgorzata Wilusz, Jacek Pantofliński, Renata Turek-Jabrocka, Grzegorz Sokołowski, Anna Sowa-Staszczak, Łukasz Kluczyński, Dorota Pach, Grzegorz Zieliński, Alicja Hubalewska-Dydejczyk
AbstractContext: Aggressive pituitary tumours causing Cushing's Disease are very rare, difficult to treat, and usually resistant to conventional therapy. There is growing evidence for the use of temozolomide (TZM), an alkylating chemotherapeutic agent, as first line chemotherapy in tumours resistant to repeated neurosurgery, radiotherapy and adrenalectomy. OBJECTIVE: To present the response to TMZ in a rare case of an aggressive pituitary tumour in the course of Cushing's Disease and to review the literature referring to similar cases...
January 10, 2018: Endokrynologia Polska
https://www.readbyqxmd.com/read/29318324/strategies-for-a%C3%A2-regenerative-therapy-of-hearing-loss
#7
REVIEW
M Diensthuber, T Stöver
Despite impressive technical progress in the field of conventional hearing aids and implantable hearing systems, the hopes for the treatment of inner ear diseases such as hearing loss and tinnitus have become increasingly directed toward regenerative therapeutic approaches. This review discusses the currently most promising strategies for hair cell regeneration in the inner ear to treat hearing loss, including stem cell-based, gene transfer-based, and pharmacological interventions. Furthermore, previous milestones and ground-breaking work in this scientific field are identified...
January 9, 2018: HNO
https://www.readbyqxmd.com/read/29317961/successful-treatment-of-rapidly-progressive-life-threatening-esophageal-submucosal-hematoma-in-a-patient-with-van-der-hoeve-syndrome
#8
Yasuhiro Watanabe, Naomi Shimizu, Masahiro Iwakawa, Takashi Yamaguchi, Noriko Ban, Hidetoshi Kawana, Atsuhito Saiki, Emiko Sakaida, Chiaki Nakaseko, Yasuhiro Matsuura, Nobuyuki Aotsuka, Hideaki Bujo, Ichiro Tatsuno
Osteogenesis imperfecta (OI) is a rare inherited disorder of the connective tissue with many reports on its association with bleeding diatheses. OI patients with blue sclera, hearing loss, and bone vulnerability are classified as having van der Hoeve syndrome. Here, we report the first case of rapidly progressing, massive esophageal submucosal hematoma in this syndrome. Bleeding in OI is reportedly due to defective capillary integrity and platelet dysfunction; however, our patient did not show such findings...
February 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29315190/audiological-outcomes-in-growing-vestibular-schwannomas-managed-either-conservatively-or-with-stereotactic-radiosurgery
#9
Thomas Daniel Milner, Richard R Locke, Georgios Kontorinis, John A Crowther
OBJECTIVE: Recent studies have suggested good hearing preservation following stereotactic radiosurgery (STRS) in patients with vestibular schwannomas (VS). This study aims to assess audiological outcomes in patients with growing VS treated either with STRS, or managed conservatively. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Out of 540 patients with VS, 69 patients with growing VS fulfilled the inclusion criteria; 24 treated conservatively and 45 with STRS...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29315188/relationship-between-the-extent-of-endolymphatic-hydrops-and-the-severity-and-fluctuation-of-audiovestibular-symptoms-in-patients-with-meni%C3%A3-re-s-disease-and-mri-evidence-of-hydrops
#10
Claudia Jerin, Sonja Floerke, Rebecca Maxwell, Robert Gürkov
OBJECTIVE: To characterize the short-, middle-, and long-term occurrence of vertigo attacks in a large population of Menière's disease (MD) and to investigate the relationship between the extent of endolymphatic hydrops (ELH) and the severity of audiovestibular symptoms. STUDY DESIGN: Prospective observational study. METHODS: One hundred ninety-two patients with clinically definite MD participated in this study. The degree of ELH was visualized by locally enhanced inner ear magnetic resonance imaging...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29315178/utilization-of-nerve-integrity-monitor-for-promontory-stimulation-testing-prior-to-cochlear-implant
#11
Elizabeth A Kelly, Samuel Levine, Kristin E Gravel, Dianna L Hart, Tina Huang
OBJECTIVE: To demonstrate the feasibility of a nerve integrity monitor as a tool for promontory stimulation testing in patients with profound sensorineural hearing loss considering cochlear implantation. PATIENTS: Adult patients considered for cochlear implantation with no auditory response on audiometric testing INTERVENTION:: Promontory stimulation testing using the nerve integrity monitor. MAIN OUTCOME MEASURE: By using a facial nerve stimulator and the nerve integrity monitor, transtympanic promontory stimulation testing was performed to assess auditory nerve function and determine candidacy for cochlear implantation...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29309637/congress-of-neurological-surgeons-systematic-review-and-evidence-based-guidelines-on-the-role-of-radiosurgery-and-radiation-therapy-in-the-management-of-patients-with-vestibular-schwannomas
#12
Isabelle M Germano, Jason Sheehan, Johnathan Parish, Tyler Atkins, Anthony Asher, Constantinos G Hadjipanayis, Stuart H Burri, Sheryl Green, Jeffrey J Olson
Question: What are the indications for stereotactic radiosurgery (SRS) treatment vs observation for patients with intracanalicular vestibular schwannomas without evidence of radiographic progression? Recommendation: Level 3: If tinnitus is not observed at presentation, it is recommended that intracanalicular vestibular schwannomas and small tumors (<2 cm) without tinnitus be observed as observation does not have a negative impact on tumor growth or hearing preservation compared to treatment...
December 20, 2017: Neurosurgery
https://www.readbyqxmd.com/read/29307789/inner-ear-involvement-in-fabry-disease-clinical-and-audiometric-evaluation-of-a-large-cohort-of-patients-followed-in-a-reference-centre
#13
Jorge Rodrigues, Olga Azevedo, Nuno Sousa, Damião Cunha, Alexandre Mexedo, Rui Fonseca
BACKGROUND: Fabry disease (FD) is a lysosomal storage disorder (LSD) that involves the cochleovestibular system. Tinnitus and progressive sensorineural hearing loss are frequent complains. A stabilization of hearing function has been reported with enzyme replacement therapy (ERT). This study aims to characterize the inner ear involvement, identify factors associated to hearing loss and evaluate the effect of ERT on the hearing function of FD patients. METHODS: We reviewed the clinical records of patients with confirmed diagnosis of FD followed in a Reference Centre on LSD in the North of Portugal...
January 4, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29302704/a-randomized-phase%C3%A2-iii-study-between-sequential-versus-simultaneous-integrated-boost-intensity-modulated-radiation-therapy-in-nasopharyngeal-carcinoma
#14
Chawalit Lertbutsayanukul, Anussara Prayongrat, Danita Kannarunimit, Chakkapong Chakkabat, Buntipa Netsawang, Sarin Kitpanit
PURPOSE: This study was performed to compare the acute and late toxicities between sequential (SEQ) and simultaneous integrated boost (SIB) intensity-modulated radiotherapy (IMRT) in nasopharyngeal carcinoma (NPC). MATERIALS AND METHODS: Stage I-IVB NPC patients were randomized to receive SEQ-IMRT or SIB-IMRT. SEQ-IMRT consisted of two plans: 2 Gy × 25 fractions to low-risk planning target volume (PTV) followed by a sequential boost (2 Gy × 10 fractions) to high-risk PTV, while SIB-IMRT treated low- and high-risk PTVs with doses of 56 and 70 Gy in 33 fractions...
January 4, 2018: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
https://www.readbyqxmd.com/read/29299506/longitudinal-up-regulation-of-endolymphatic-hydrops-in-patients-with-meniere-s-disease-during-medical-treatment
#15
Munehisa Fukushima, Tadashi Kitahara, Ryohei Oya, Shiro Akahani, Hidenori Inohara, Shinji Naganawa, Noriaki Takeda
Objective/Hypothesis: Meniere's disease (MD) is a common inner ear disease characterized by repeated episodic vertigo, fluctuating sensorineural hearing loss, and tinnitus. Its pathology is defined as endolymphatic hydrops (EH) in the inner ear and EH has been hypothesized to correlate with the clinical symptoms of MD. We presented the dynamics of in vivo EH in MD patients during medical treatments. Study Design: Prospective, single-arm repeated measures. Methods: Eleven MD patients were enrolled...
December 2017: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/29289454/audiometric-assessment-of-pediatric-patients-with-cystic-fibrosis
#16
Kathryn L Kreicher, Michael J Bauschard, Clarice S Clemmens, Concetta Maria Riva, Ted A Meyer
BACKGROUND: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). METHODS: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss...
December 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29287890/a-novel-pathogenic-variant-c-975g-a-p-trp325-in-the-pou3f4-gene-in-yakut-family-eastern-siberia-russia-with-the-x-linked-deafness-2-dfnx2
#17
Nikolay A Barashkov, Leonid A Klarov, Fedor M Teryutin, Aisen V Solovyev, Vera G Pshennikova, Edilia E Konnikova, Georgii P Romanov, Alexander V Tobokhov, Igor V Morozov, Alexander A Bondar, Olga L Posukh, Lilya U Dzhemileva, Mikhail I Tomsky, Elza K Khusnutdinova, Sardana A Fedorova
Here, we report a novel hemizygous transition c.975G>A (p.Trp325*) in POU3F4 gene (Xq21) found in two deaf half-brothers from one Yakut family (Eastern Siberia, Russia) with identical inner ear abnormalities ("corkscrew" cochlea with an absence of modiolus) specific to X-linked deafness-2 (DFNX2). Comprehensive clinical evaluation (CT and MR-imaging, audiological and stabilometric examinations) of available members of this family revealed both already known (mixed progressive hearing loss) and additional (enlargement of semicircular canals and postural disorders) clinical DFNX2 features in affected males with c...
January 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29287879/a-novel-splicing-mutation-in-smpx-is-linked-to-nonsyndromic-progressive-hearing-loss
#18
Zhijie Niu, Denise Yan, Sara Bressler, Lingyun Mei, Yong Feng, Xuezhong Liu
OBJECTIVE: X-linked nonsyndromic hearing impairment is the rarest form of genetic hearing loss and represents only a minor fraction of all cases. The aim of this study was to investigate the cause of X-linked nonsyndromic sensorineural hearing loss in a three-generation American family. METHODS: Whole-exome sequencing and co-segregation analysis were used to identify disease-causing genes. RESULTS: In this study, we described in detail the clinical characteristics of the family and identified a novel frameshift mutation creating a premature stop codon (c...
January 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29287867/slc52a2-mutations-cause-scabd2-phenotype-a-second-report
#19
Mojgan Babanejad, Omid Ali Adeli, Nooshin Nikzat, Maryam Beheshtian, Hakimeh Azarafra, Farnaz Sadeghnia, Marzieh Mohseni, Hossein Najmabadi, Kimia Kahrizi
INTRODUCTION: Autosomal recessive cerebellar ataxias (ARCAs) are a large group of neurodegenerative disorders that manifest mainly in children and young adults. Most ARCAs are heterogeneous with respect to age at onset, severity of disease progression, and frequency of extracerebellar and systemic signs. METHODS: The phenotype of a consanguineous Iranian family was characterized using clinical testing and pedigree analysis. Whole-exome sequencing was used to identify the disease-causing gene in this family...
January 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29287859/hearing-loss-in-children-with-primary-ciliary-dyskinesia
#20
Kathryn L Kreicher, Heather K Schopper, Akash N Naik, Jonathan L Hatch, Ted A Meyer
OBJECTIVES: To evaluate the type and severity of hearing impairment in pediatric patients with primary ciliary dyskinesia (PCD) and relate these measures to patient demographics, treatment options, and other otologic factors. METHODS: A retrospective analysis of children with a diagnosis of PCD, Kartagener's syndrome, or situs inversus in the AudGen Database was conducted. Audiograms were analyzed for type of hearing loss (HL), severity, laterality, and progression...
January 2018: International Journal of Pediatric Otorhinolaryngology
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