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C C Wu, Y H Chen, T H Yang, K N Lin, S Y Lee, T C Liu, C J Hsu
Congenital ossicular chain anomalies are rare, but are clinically important because hearing loss in most cases is correctable by appropriate surgical interventions. The great diversity in anatomic variations makes congenital ossicular chain anomalies a surgical challenge for otologists. Theoretically, endoscopic surgery might be suitable for management of congenital ossicular chain anomalies by improving visualization of lesions and preserving uninvolved anatomic structures as compared to conventional microscopic surgery...
October 19, 2016: Clinical Otolaryngology
Bas P Hartel, Martijn J H Agterberg, Ad F Snik, Henricus P M Kunst, A John V Opstal, Arjan J Bosman, Ronald J E Pennings
OBJECTIVES: Usher syndrome is the leading cause of hereditary deaf-blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localization abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal processing on binaural hearing, including sound localization. DESIGN AND PARTICIPANTS: In this prospective study, patients were fitted with hearing aids with a nonlinear (compression) and linear amplification program...
October 19, 2016: Clinical Otolaryngology
Debra W Yen, Dennis C Nguyen, Gary B Skolnick, Sybill D Naidoo, Kamlesh B Patel, Lynn Marty Grames, Albert S Woo
BACKGROUND: Reconstruction of the levator musculature during cleft palate repair has been suggested to be important in long-term speech outcomes. In this study, we compare the need for postoperative speech therapy between 2 intravelar veloplasty techniques. METHODS: Chart review was performed for patients with nonsyndromic cleft palate who underwent either primary Kriens or overlapping intravelar veloplasty before 18 months of age. All subjects completed a follow-up visit at approximately 3 years of age...
October 17, 2016: Annals of Plastic Surgery
Kevin K Ohlemiller, Sherri M Jones, Kenneth R Johnson
Laboratory mice (Mus musculus) have become the major model species for inner ear research. The major uses of mice include gene discovery, characterization, and confirmation. Every application of mice is founded on assumptions about what mice represent and how the information gained may be generalized. A host of successes support the continued use of mice to understand hearing and balance. Depending on the research question, however, some mouse models and research designs will be more appropriate than others...
October 17, 2016: Journal of the Association for Research in Otolaryngology: JARO
Yuan Zhang, Yong-Hua Cui, Ying Hu, Zhong-Yao Mao, Qiu-Xia Wang, Chu Pan, Ai-Guo Liu
The purpose of the study was to observe changes in endolymphatic hydrops by using intratympanic injection of gadolinium and magnetic resonance imaging (MRI) before and after endolymphatic sac surgery in patients with unilateral Meniere's disease. Thirteen patients with unilateral Meniere's disease undergoing endolymphatic sac surgery were retrospectively and prospectively analyzed. Three-dimensional fluid-attenuated inversion recovery or three-dimensional real inversion recovery MRI was performed 24 h after an intratympanic injection of gadolinium to grade the presence of endolymphatic hydrops...
October 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
Christian Güldner, Isabell Diogo, Eva Bernd, Stephanie Dräger, Magis Mandapathil, Afshin Teymoortash, Hesham Negm, Thomas Wilhelm
Cone beam computed tomography (CBCT, syn. digital volume tomography = DVT) was introduced into ENT imaging more than 10 years ago. The main focus was on imaging of the paranasal sinuses and traumatology of the mid face. In recent years, it has also been used in imaging of chronic ear diseases (especially in visualizing middle and inner ear implants), but an exact description of the advantages and limitations of visualizing precise anatomy in a relevant number of patients is still missing. The data sets of CBCT imaging of the middle and inner ear of 204 patients were analyzed regarding the visualization of 18 different anatomic structures...
October 17, 2016: European Archives of Oto-rhino-laryngology
Jason Fleming, Dianna Quan
We present a patient with congenital spinal muscular atrophy associated with pain, subjective sensory loss, right talipes equinovarus, delayed walking, and progressive gait impairment. A sister and niece reportedly had Charcot-Marie-Tooth 1A, but the patient's electromyogram showed an axonal motor neuropathy or neuronopathy. We identified a c.806G>A TRPV4 gene mutation causing an Arg269His amino acid substitution. TRPV4 mutations cause variable phenotypes including axonal sensorimotor neuropathy and motor neuropathy or neuronopathy...
September 16, 2016: Neuromuscular Disorders: NMD
Hyun-Min Lee, Keun-Ik Yi, Jae-Hoon Jung, Il-Woo Lee
We report an extremely rare case of hearing aid silicone impression material as a foreign body in the middle ear. Symptoms of the patient were otorrhea and vertigo after taking of a mold impression on his only hearing ear, and the symptoms mimicked chronic otitis media. A temporal bone CT scan revealed foreign body material in the middle ear and Eustachian tube. An intact canal wall mastoidectomy with a facial recess approach and type IV tympanoplasty was performed to remove the silicone impression material...
September 28, 2016: American Journal of Otolaryngology
Michael C Topf, David W Hsu, Douglas R Adams, Tingting Zhan, Stanley Pelosi, Thomas O Willcox, Brian McGettigan, Kyle W Fisher
PURPOSE: To determine the rate of persistent tympanic membrane perforation after intratympanic steroid injection. To determine which comorbid conditions and risk factors are associated with prolonged time to perforation closure following intratympanic steroid injection. MATERIALS AND METHODS: Clinical data were gathered for patients who had undergone intratympanic steroid injection to treat sudden sensorineural hearing loss or Ménière's disease. Primary outcomes analysis included rate of persistent tympanic membrane perforation, defined as perforation at least 90days following last injection, and time to perforation healing...
September 28, 2016: American Journal of Otolaryngology
L Westover, G Faulkner, W Hodgetts, D Raboud
This study presents the Advanced System for Implant Stability Testing (ASIST) which provides a non-invasive, quantitative measure of the stability of percutaneous implants used for craniofacial rehabilitation such as bone anchored hearing aids or dental implants. The ASIST uses an impact technique coupled with an analytical model which allows the measure to be independent of the system components. This paper presents a laboratory evaluation of the ASIST for the Oticon Medical Ponto and the Cochlear Baha Connect bone anchored hearing aid (BAHA) systems...
October 6, 2016: Journal of Biomechanics
Abby R Rosenberg, Joanne Wolfe, Lori Wiener, Maureen Lyon, Chris Feudtner
Importance: For clinicians caring for adolescent patients living with progressive, life-threatening illness, discussions regarding prognosis, goals of care, and treatment options can be extremely challenging. While clinicians should respect and help to facilitate adolescents' emerging autonomy, they often must also work with parents' wishes to protect patients from the emotional distress of hearing bad news. Observations: We reviewed the ethical justifications for and against truth-telling, and we considered the published ethical and practice guidance, as well as the perspectives of patients, parents, and clinicians involved in these cases...
October 17, 2016: JAMA Pediatrics
Hilary C McCrary, Erynne A Faucett, Saranya Reghunathan, F Zahra Aly, Rihan Khan, Raymond F Carmody, Abraham Jacob
OBJECTIVE: Cutaneous neuroendocrine lesions of the external auditory canal (EAC) are exceptionally rare, with only five cases reported in the literature. In this case report, we present a patient with a recurrent carcinoid tumor in the EAC, which has yet to be described. PATIENTS: A 38-year-old woman presenting with otalgia, aural fullness, and decreased hearing was found to have a recurrent EAC carcinoid tumor, 8-years after initial resection at an outside facility...
September 30, 2016: Otology & Neurotology
Yuka Morita, Kuniyuki Takahashi, Shuji Izumi, Yamato Kubota, Shinsuke Ohshima, Arata Horii
OBJECTIVE: Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. STUDY DESIGN: Retrospective chart review...
September 30, 2016: Otology & Neurotology
Nikolaus E Wolter, Sharon L Cushing, Luis D Vilchez Madrigal, Adrian L James, Jennifer Campos, Blake C Papsin, Karen A Gordon
OBJECTIVE: To determine if children with unilateral sensorineural hearing loss (UHL) demonstrate impaired balance compared with their normal hearing (NH) peers. STUDY DESIGN: Prospective, case-control study. METHODS: Balance was assessed in14 UHL and 14 NH children using the Bruininks-Oseretsky Test-2 (BOT-2) and time to fall (TTF) in an immersive, virtual-reality laboratory. Postural control was quantified by center of pressure (COP) using force plates...
September 30, 2016: Otology & Neurotology
Thomas Zahnert, Marie-Luise Metasch, Hannes Seidler, Matthias Bornitz, Nicoloz Lasurashvili, Marcus Neudert
HYPOTHESIS: Electromagnetical excitation of ossicular vibration is suitable for middle ear transmission measurements in the experimental and clinical setting. Thereby, it can be used as a real-time monitoring system for quality control in ossiculoplasty. BACKGROUND: Positioning and coupling of middle ear prosthesis are a precondition for good postoperative hearing results, but at the same time completely dependent upon the surgeon's subjective judgment during surgery...
October 4, 2016: Otology & Neurotology
Thomas Sené, Olivier Lidove, Joel Sebbah, Jean-Marc Darondel, Hervé Picard, Laurent Aaron, Olivier Fain, Thierry Zenone, Dominique Joly, Philippe Charron, Jean-Marc Ziza
The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy...
October 2016: Medicine (Baltimore)
Koenraad S Rhebergen, Thijs H Maalderink, Wouter A Dreschler
OBJECTIVES: The effects of nonlinear signal processing on speech intelligibility in noise are difficult to evaluate. Often, the effects are examined by comparing speech intelligibility scores with and without processing measured at fixed signal to noise ratios (SNRs) or by comparing the adaptive measured speech reception thresholds corresponding to 50% intelligibility (SRT50) with and without processing. These outcome measures might not be optimal. Measuring at fixed SNRs can be affected by ceiling or floor effects, because the range of relevant SNRs is not know in advance...
October 4, 2016: Ear and Hearing
Angel M Dzhambov
There is compelling evidence from animal experiments that noise exposure suppresses testosterone in males by affecting the hypothalamic-pituitary-testicular axis. Virtually nothing is known about its effect in humans. Therefore, the aim of this study was to explore the association between occupational noise exposure and serum testosterone in a representative sample of the general population. The sample has been taken from the National Health and Nutrition Examination Survey (NHANES) data for the period between 1999 and 2004 and is limited to employed men aged 16-85+ years at the time...
September 1, 2016: Arhiv za Higijenu Rada i Toksikologiju
Hao Zhang, Hua Yue, Chun Wang, Weiwei Hu, Jiemei Gu, Jinwei He, Wenzhen Fu, Yunqiu Hu, Miao Li, Zhenlin Zhang
Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by brittle bone fractures. The aim of the present study was to investigate the pathogenic gene mutation spectrum and clinical manifestations of mutations in collagen type I, alpha 1 (COL1A1) and collagen type I, alpha 2 (COL1A2) genes in Chinese patients with OI. A total of 61 unrelated Chinese OI patients with COL1A1 and COL1A2 mutations were recruited. All the exons and the exon-intron boundaries of the COL1A1 and COL1A2 genes were amplified and directly sequenced and lumbar spine bone mineral density was measured by dual‑energy X‑ray absorptiometry...
October 12, 2016: Molecular Medicine Reports
Rebecca Carroll, Verena Uslar, Thomas Brand, Esther Ruigendijk
OBJECTIVE: The authors aimed to determine whether hearing impairment affects sentence comprehension beyond phoneme or word recognition (i.e., on the sentence level), and to distinguish grammatically induced processing difficulties in structurally complex sentences from perceptual difficulties associated with listening to degraded speech. Effects of hearing impairment or speech in noise were expected to reflect hearer-specific speech recognition difficulties. Any additional processing time caused by the sustained perceptual challenges across the sentence may either be independent of or interact with top-down processing mechanisms associated with grammatical sentence structure...
September 28, 2016: Ear and Hearing
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