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Progressive hearing loss

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https://www.readbyqxmd.com/read/28538470/progression-of-unilateral-hearing-loss-in-children-with-and-without-ipsilateral-cochlear-nerve-canal-stenosis-a-hazard-analysis
#1
Patricia L Purcell, Justin R Shinn, Scott S Coggeshall, Grace Phillips, Angelisa Paladin, Kathleen C Y Sie, David L Horn
OBJECTIVE: To investigate the risk of hearing loss progression in each ear among children with unilateral hearing loss associated with ipsilateral bony cochlear nerve canal (BCNC) stenosis. SETTING: Tertiary pediatric referral center. PATIENTS: Children diagnosed with unilateral hearing loss who had undergone temporal bone computed tomography imaging and had at least 6 months of follow-up audiometric testing were identified from a prospective audiological database...
May 22, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28527170/oculo-otological-manifestations-in-a-case-of-granulomatosis-with-polyangiitis
#2
Archana Sonawale, Anjali Rajadhyaksha, Shreepriya Mangalgi
A 44 year old lady presented with acute onset of loss of vision in the right eye and cough with mucopurulent expectoration for two months. Ophthalmic examination revealed central retinal artery occlusion (CRAO). Chest radiograph showed multiple cavitatory nodules with fluid levels. Sputum was negative for AFB and ANCA was strongly positive suggestive of a diagnosis of Granulomatosis with Polyangiitis (GPA). Within the next few weeks the patient had rapid detenoration due to left eye CRAO, progressive bilateral hearing loss, facial palsy and retro orbital mass...
April 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28527079/endolymphatic-hydrops-in-idiopathic-intracranial-hypertension-prevalence-and-clinical-outcome-after-lumbar-puncture-preliminary-data
#3
Angelo Ranieri, Michele Cavaliere, Stefania Sicignano, Pietro Falco, Federico Cautiero, Roberto De Simone
Idiopathic intracranial hypertension is characterized by raised intracranial pressure (ICP) without any underlying pathology, presenting with (IIH) or without papilledema (IIHWOP). Headache, often on daily basis, is the most frequent symptom. Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports. Endolymphatic hydrops (ELH) is the typical histopathologic feature of Ménière disease, a condition featured by episodes of vertigo, dizziness, fluctuating hearing loss, tinnitus, and aural fullness...
May 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28516959/adaptive-psychological-structure-in-childhood-hearing-impairment-audiological-correlations
#4
A Serra, G Spinato, S Cocuzza, L Licciardello, P Pavone, L Maiolino
The present research deals with the clinical and social problems present during linguistic and cognitive development of deaf children. Currently, the development of Theory of Mind represents an important research field in deafness studies. These international studies highlighted a significant alteration in the development of Theory of Mind in deaf children compared to normal hearing children, especially in cases of congenital or preverbal hearing loss. In particular, the research focuses on the skills of deaf children in recognising emotions and desires, through both perceptive and cognitive methods, by evaluation of psycho-cognitive skills of children with severe hearing loss using a set of questions to be administered to hearing loss patients...
June 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28509597/use-of-technological-aids-and-interpretation-services-among-children-and-adults-with-hearing-loss
#5
Jesper Dammeyer, Christine Lehane, Marc Marschark
OBJECTIVES: The technological development of communication aids for people with hearing loss has progressed rapidly over the last decades. Quality has improved and the number of different types of aids has increased. However, few studies have examined the prevalence of technology use and interpreting services use among people with hearing loss as they relate to demographic characteristics of this population. DESIGN: This study reports from national surveys of children and adults with hearing loss...
May 16, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28502323/a-case-report-on-the-exceptional-coincidence-of-two-inherited-renal-disorders-adpkd-and-alport-syndrome%C3%A2
#6
Kathrin Ebner, Nadine Reintjes, Markus Feldkötter, Friederike Körber, Mato Nagel, Jörg Dötsch, Bernd Hoppe, Lutz Thorsten Weber, Bodo B Beck, Max C Liebau
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of dialysis-requiring end-stage renal disease in adults and is characterized by the slowly progressing replacement of renal tissue by focal macrocysts. Alport syndrome (AS; hereditary nephritis) is a rare, inherited disorder of the basement membrane associated with hematuria, proteinuria, and loss of kidney function as well as sensorineural hearing loss and ocular abnormalities. Here, we report on a family in which both ADPKD and AS are present...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28498272/paget-s-disease-of-the-temporal-bone-a-single-institution-contemporary-review-of-27-patients
#7
Nicholas L Deep, Jake G Besch-Stokes, John I Lane, Colin L W Driscoll, Matthew L Carlson
OBJECTIVES: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). STUDY DESIGN: Retrospective chart review of patients evaluated from 1998 to 2016. SETTING: Quaternary referral center. PATIENTS: Patients with radiographically confirmed PDTB. MAIN OUTCOME MEASURES: Clinical, audiological, and radiological features and management strategies of PDTB...
May 11, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28498079/outcomes-of-cochlear-implantation-for-the-patients-with-specific-genetic-etiologies-a-systematic-literature-review
#8
Shin-Ya Nishio, Shin-Ichi Usami
CONCLUSION: Most of the cases with gene mutations of intra-cochlear etiology showed relatively good CI outcomes. To progress toward more solid evidence-based CI intervention, a greater number of reports including CI outcomes for specific gene mutations are desired. BACKGROUND: Cochlear implantation (CI) is the most important and effective treatment for patients with profound sensorineural hearing loss. However, the outcomes of CI vary among patients. One of the reasons of this heterogeneous outcome for cochlear implantation is thought to be the heterogeneous nature of hearing loss...
February 24, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28487795/sequence-and-timing-of-intracranial-changes-in-cytomegalovirus-in-pregnancy-a-case-report-and-literature-review
#9
Cynthia O'Sullivan, Shankari Arulkumaran, Lorin Lakasing, Eric Jauniaux, Karl Murphy
Cytomegalovirus (CMV) is the most common cause of intrauterine infection, occurring in up to 2% of all live births. Most women are asymptomatic or experience nonspecific symptoms, which can lead to long-term sequelae in newborns including neurological impairment, hearing loss, and mental retardation. A 41-year-old woman (G6 P2), with a medical history of epilepsy, presented for her routine anomaly scan at 20 + 4/40. A single finding of echogenic bowel was noted on ultrasound which prompted a full investigation...
2017: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28487633/cochlear-homocysteine-metabolism-at-the-crossroad-of-nutrition-and-sensorineural-hearing-loss
#10
REVIEW
Teresa Partearroyo, Néstor Vallecillo, María A Pajares, Gregorio Varela-Moreiras, Isabel Varela-Nieto
Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28483396/early-diagnosis-of-capos-syndrome-before-acute-onset-ataxia-review-of-the-literature-and-a-new-family
#11
Anna Duat Rodriguez, Michaela Prochazkova, Saturnino Santos Santos, Oscar Rubio Cabezas, Veronica Cantarin Extremera, Luis Gonzalez-Gutierrez-Solana
BACKGROUND: CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare disease that has been reported in 22 patients so far. In all cases, the mutation c.2452G>A (p.Glu818Lys) in the ATP1A3 gene was identified. Patients typically present at an early age with an acute-onset fever-induced episode of ataxia frequently associated with encephalopathy and weakness. They usually present one to three episodes. The acute symptoms improve within days, but most patients show slow progression afterward...
January 25, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28483240/management-of-non-organic-hearing-loss-in-children-a-case-study
#12
Piotr Henryk Skarzynski, Danuta Raj-Koziak, Joanna Jadwiga Rajchel, Henryk Skarzynski
A 10 year-old girl was admitted due to the claim of progressively developing hearing loss. The impedance audiometry showed no abnormalities but it was impossible to obtain reliable outcomes during pure tone audiometry assessment. The girl was additionally sent for speech audiometry, indicating a bilateral hearing loss and objective evaluations such as distortion product otoacoustic emissions and auditory brainstem responses, which results indicated a normal hearing. On the second day, repeated subjective audiometric tests showed also normal hearing, despite constantly reported hearing loss...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28472857/c-fos-and-arc-arg3-1-expression-in-auditory-and-visual-cortices-after-hearing-loss-evidence-of-sensory-crossmodal-reorganization-in-adult-rats
#13
M Pernia, S Estevez, C Poveda, I Plaza, J Carro, M Juiz, M A Merchan
Cross-modal reorganization in the auditory and visual cortices has been reported after hearing and visual deficits mostly during the developmental period, possibly underlying sensory compensation mechanisms. However, there are very few data on the existence or nature and timeline of such reorganization events during sensory deficits in adulthood. In this study, we assessed long-term changes in activity-dependent immediate early genes c-Fos and Arc/Arg3.1 in auditory and neighboring visual cortical areas after bilateral deafness in young adult rats...
May 4, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/28471784/hearing-threshold-shifts-among-11-to-35-year-olds-with-early-hearing-impairment
#14
Lyndal Carter, Deborah Black
OBJECTIVES: Data obtained from the clinical records of selected 11- to 35-year-olds with preadult onset hearing impairment were analyzed with 2 primary aims: (1) to determine the incidence of hearing threshold level (HTL) shift in this cohort and, (2) to examine the relationship between HTL shift, whole-of-life noise exposure, and other factors. DESIGN: Cross-sectional cohort study. Retrospective HTL + survey data for a sample of 237 young Australians receiving hearing (re)habilitation services were obtained...
May 2, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28458811/cochlear-impairment-and-autoimmune-ear-disorder-in-a-patient-with-breast-cancer
#15
Alessandra Fioretti, Vittoria Di Rubbo, Giorgia Peri, Elisa Vitti, Sara Cisternino, Theodoros Varakliotis, Alberto Eibenstein
The purpose of this study was to consider the possible role of autoimmune diseases and paraneoplastic syndrome in the genesis of tinnitus. The incidence of autoimmune inner ear disease (AIED) is rare, accounting for <1% of all cases of hearing impairment and dizziness. In presence of auditory and vestibular deficit in oncological patients, a paraneoplastic syndrome with cochleovestibulopathy should be considered. We described a 50-year-old Caucasian woman came to our attention with complaints of severe disabling bilateral tinnitus (Tinnitus Handicap Inventory, THI: 96), ear fullness and headache...
February 1, 2017: Audiology Research
https://www.readbyqxmd.com/read/28458810/autoimmune-ear-disease-clinical-and-diagnostic-relevance-in-cogan-s-sydrome
#16
Luigi Maiolino, Salvatore Cocuzza, Angelo Conti, Luisa Licciardello, Agostino Serra, Salvatore Gallina
The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan's syndrome. Cogan's syndrome is a chronic inflammatory disorder that most commonly affects young adults...
February 1, 2017: Audiology Research
https://www.readbyqxmd.com/read/28457383/kidney-transplantation-in-alstr%C3%A3-m-syndrome-case-report
#17
L Poli, G Arroyo, M Garofalo, E Choppin de Janvry, G Intini, A Saracino, R Pretagostini, F Della Pietra, P B Berloco
The Alström syndrome is a rare genetic disorder, inherited in an autosomal recessive manner. It has recently been classified as a ciliopathic disorder. Alström syndrome is a multiorgan pathology characterized by cone-rod dystrophy, hearing loss, childhood truncal obesity, insulin resistance and hyperinsulinemia, type 2 diabetes mellitus, dyslipidemia, short stature in adulthood, hypothyroidism, hypogonadism, dilated or restrictive cardiomyopathy, and progressive pulmonary, hepatic, and renal dysfunction. End-stage renal disease can occur as early as the late teens and is the leading cause of death...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28457225/otoacoustic-emissions-in-rural-nicaragua-cost-analysis-and-implications-for-newborn-hearing-screening
#18
Lye-Yeng Wong, Francisca Espinoza, Karen Mojica Alvarez, Dave Molter, James E Saunders
Objective (1) Determine the incidence and risk factors for congenital hearing loss. (2) Perform cost analysis of screening programs. Study Design Proportionally distributed cross-sectional survey. Setting Jinotega, Nicaragua. Subjects and Methods Otoacoustic emissions (OAEs) were used to screen 640 infants <6 months of age from neonatal intensive care unit, institutional, and home birth settings. Data on 15 risk factors were analyzed. Cost of 4 implementation strategies was studied: universal screening, screening at the regional health center (RHC), targeted screening, and screening at the RHC plus targeted screening...
May 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28437363/intratympanic-dexamethasone-in-the-treatment-of-m%C3%A3-ni%C3%A3-re-s-disease-a-comparison-of-two-techniques
#19
Jason A Beyea, Ryan S Instrum, Sumit K Agrawal, Lorne S Parnes
OBJECTIVE: To assess the efficacy and safety of two different intratympanic dexamethasone (IT Dex) injection protocols for intractable unilateral Ménière's disease. STUDY DESIGN: Prospective case series. SETTING: Tertiary neurotology clinic. PATIENTS: One hundred six consecutive adult patients with definite unilateral Ménière's disease who had failed medical management were studied for an average of 1,061 days. None had previous oral steroid, IT steroid, or ablative treatment...
April 21, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28430993/a-multi-systemic-mitochondrial-disorder-due-to-a-dominant-p-y955h-disease-variant-in-dna-polymerase-gamma
#20
Triinu Siibak, Paula Clemente, Ana Bratic, Helene Bruhn, Timo E S Kauppila, Bertil Macao, Florian A Schober, Nicole Lesko, Rolf Wibom, Karin Naess, Inger Nennesmo, Anna Wedell, Bradley Peter, Christoph Freyer, Maria Falkenberg, Anna Wredenberg
Mutations in the mitochondrial DNA polymerase, POLG, are associated with a variety of clinical presentations, ranging from early onset fatal brain disease in Alpers syndrome to chronic progressive external ophthalmoplegia. The majority of mutations are linked with disturbances of mitochondrial DNA (mtDNA) integrity and maintenance. On a molecular level, depending on their location within the enzyme, mutations either lead to mtDNA depletion or the accumulation of multiple mtDNA deletions, and in some cases these molecular changes can be correlated to the clinical presentation...
April 17, 2017: Human Molecular Genetics
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