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https://www.readbyqxmd.com/read/29788902/dystonia-deafness-syndrome-caused-by-actb-p-arg183trp-heterozygosity-shows-striatal-dopaminergic-dysfunction-and-response-to-pallidal-stimulation
#1
Inger Marie Skogseid, Oddveig Røsby, Ane Konglund, James P Connelly, Bård Nedregaard, Greg Eigner Jablonski, Nadja Kvernmo, Asbjørg Stray-Pedersen, Joel C Glover
BACKGROUND: Dystonia-deafness syndrome is a well-known clinical entity, with sensorineural deafness typically manifesting earlier than dystonia. ACTB p.Arg183Trp heterozygosity has been reported in six patients to cause combined infant-onset deafness and dystonia manifesting in adolescence or young adulthood. Three of these have received beneficial pallidal stimulation. Brain imaging to assess striatal function has not been reported previously, however. Nor has a comprehensive hypothesis been presented for how the pleiotropic manifestations of this specific beta-actin gene mutation originate developmentally...
May 22, 2018: Journal of Neurodevelopmental Disorders
https://www.readbyqxmd.com/read/29788544/cochlear-implantation-using-a-custom-guide-catheter-in-fourteen-patients-with-incomplete-partition-type-iii
#2
Hao Tian, Line Wang, Fenqi Gao, Wenqi Liang, Kevin A Peng
Incomplete partition type III (IP-III), also termed X-linked deafness with stapes gusher, is a heterogeneous condition that predominantly affects males; however, females demonstrating the phenotype also exist. The absence of a bony partition between the fundus of the internal auditory canal (IAC) and cochlea predisposes these patients to cerebrospinal fluid leak or electrode passage into the IAC when performing cochlear implantation. We describe a surgeon-fabricated guide catheter, made intraoperatively from a 16-gauge intravenous catheter, that helps avoid electrode passage into the IAC during CI for patients with IP-III...
May 22, 2018: Clinical Otolaryngology
https://www.readbyqxmd.com/read/29785464/contribution-of-cochlear-compression-to-discrimination-of-rippled-spectra-in-on-and-low-frequency-noise
#3
Olga N Milekhina, Dmitry I Nechaev, Alexander Ya Supin
The goal of the study was to assess cochlear compression when rippled-spectrum signals are perceived in noise assuming that the noise might produce both masking and confounding effects. In normal listeners, discrimination between rippled signals with and without ripple phase reversals was assessed in background noise. The signals were band-limited (0.5 oct at a - 6-dB level) rippled noise centered at 2 kHz, with a ripple density of 3.5 oct-1 . The noise (masker) was band-limited nonrippled noise centered at either 2 kHz (on-frequency masker) or 1 kHz (low-frequency masker)...
May 21, 2018: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/29784671/a-non-autonomous-function-of-the-core-pcp-protein-vangl2-directs-peripheral-axon-turning-in-the-developing-cochlea
#4
Satish R Ghimire, Evan M Ratzan, Michael R Deans
The cochlea is innervated by neurons that relay sound information from hair cells to central auditory targets. A subset of these are the TypeΙΙ spiral ganglion neurons which have nociceptive features and contribute to feedback circuits providing neuroprotection in extreme noise. TypeΙΙ neurons make a distinctive 90 degree turn towards the cochlear base to synapse with 10-15 outer hair cells. We demonstrate that this axon turning event requires Planar Cell Polarity (PCP) signaling, is disrupted in Vangl2 and Celsr1 knockout mice, and that VANGL2 acts non-autonomously from the cochlea to direct turning...
May 21, 2018: Development
https://www.readbyqxmd.com/read/29784430/microvascular-decompression-for-hemifacial-spasm-surgical-techniques-and-intraoperative-monitoring
#5
M Sindou, P Mercier
Primary hemifacial spasm with few exceptions is due to the vascular compression of the facial nerve that can be evidenced with high resolution MRI. Microvascular decompression is the only curative treatment for this pathology. According to literature review detailed in chapter "conflicting vessels", the compression is located at the facial Root Exit Zone (REZ) in 95% of the cases, and in 5% distally at the cisternal or the intrameatal portion of the root as the sole conflict or in addition to one at brainstem/REZ...
May 18, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29782445/comparing-two-hearing-aid-fitting-algorithms-for-bimodal-cochlear-implant-users
#6
Jantien L Vroegop, Nienke C Homans, Marc P van der Schroeff, André Goedegebure
OBJECTIVES: To investigate the possible advantage of the use of a dedicated bimodal hearing aid fitting formula, the Adaptive Phonak Digital Bimodal (APDB), compared with a frequently used standard hearing aid fitting formula, the NAL-NL2. We evaluated the effects of bimodal hearing aid fitting on provided hearing aid gain and on bimodal auditory functioning in a group of experienced bimodal cochlear implant (CI) users. A second aim of our study was to determine the effect of broadband loudness balancing on the prescribed gain of those two fitting formulas...
May 16, 2018: Ear and Hearing
https://www.readbyqxmd.com/read/29781875/feasibility-of-microrna-profiling-in-human-inner-ear-perilymph
#7
Matthew Shew, Athanasia Warnecke, Thomas Lenarz, Heike Schmitt, Sumedha Gunewardena, Hinrich Staecker
Hearing loss is common and caused by a wide range of molecular and cellular pathologies. Current diagnosis of hearing loss depends on a combination of physiologic testing, patient history, and in some cases genetic testing. Currently, no biopsy or equivalent procedure exists to diagnose inner ear disorders. MicroRNAs (miRNA) are short ribonucleic acids that regulate a variety of cellular processes. They have been found to be reliable markers for a variety of disease processes. In particular, a variety of miRNAs that are markers for neurodegenerative disease have been identified in cerebrospinal fluid...
May 17, 2018: Neuroreport
https://www.readbyqxmd.com/read/29780970/three-challenges-for-future-research-on-cochlear-implants
#8
REVIEW
David B Pisoni, William G Kronenberger, Michael S Harris, Aaron C Moberly
Cochlear implants (CIs) often work very well for many children and adults with profound sensorineural (SNHL) hearing loss. Unfortunately, while many CI patients display substantial benefits in recognizing speech and understanding spoken language following cochlear implantation, a large number of patients achieve poor outcomes. Understanding and explaining the reasons for poor outcomes following implantation is a very challenging research problem that has received little attention despite the pressing clinical significance...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780969/12-guiding-premises-of-pediatric-cochlear-implant-habilitation
#9
REVIEW
Amy McConkey Robbins
No abstract text is available yet for this article.
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780968/the-influence-of-obesity-on-operating-room-time-and-perioperative-complications-in-cochlear-implantation
#10
Jonathan L Hatch, Isabel M Boersma, Forest W Weir, Michael J Bauschard, Meredith A Holcomb, Paul R Lambert, Ted A Meyer
Objective: The rising incidence of obesity in the United States is associated with increased healthcare expenditures and resource allocation. Obesity has been associated with prolonged operating times during surgical procedures. The primary objective of this study is to compare body mass index (BMI) to length of surgery during cochlear implantation. Methods: A retrospective case control study from a tertiary academic referral center was performed. Patients included were adults who underwent primary, single-sided cochlear implantation with documented BMI and operating room (OR) times from January 2009 to July 2015...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780967/visual-working-memory-span-in-adults-with-cochlear-implants-some-preliminary-findings
#11
Aaron C Moberly, David B Pisoni, Michael S Harris
Objective: Neurocognitive functions, specifically verbal working memory (WM), contribute to speech recognition in postlingual adults with cochlear implants (CIs) and normal-hearing (NH) listener shearing degraded speech. Three hypotheses were tested: (1) WM accuracy as assessed using three visual span measures - digits, objects, and symbols - would correlate with recognition scores for spectrally degraded speech (through a CI or when noise-vocoded); (2) WM accuracy would be best for digit span, intermediate for object span, and lowest for symbol span, due to the increasing cognitive demands across these tasks...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780966/electro-tactile-stimulation-ets-enhances-cochlear-implant-mandarin-tone-recognition
#12
Juan Huang, Janice Chang, Fan-Gang Zeng
Objective: Electro-acoustic stimulation (EAS) is an effective method to enhance cochlear-implant performance in individuals who have residual low-frequency acoustic hearing. To help the majority of cochlear implant users who do not have any functional residual acoustic hearing, electro-tactile stimulation (ETS) may be used because tactile sensation has a frequency range and perceptual capabilities similar to that produced by acoustic stimulation in the EAS users. Methods: Following up the first ETS study showing enhanced English sentence recognition in noise,1 the present study evaluated the effect of ETS on Mandarin tone recognition in noise in two groups of adult Mandarin-speaking individuals...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780965/cochlear-implantation-in-infants-below-12-months-of-age
#13
Richard T Miyamoto, Bethany Colson, Shirley Henning, David Pisoni
Objectives: To provide safety and efficacy data on infants implanted below 12 months of age. Methods: With the wide application of newborn hearing screening programs, infants with deafness are being identified at birth. When a hearing aid trial fails, cochlear implantation is the only option to restore hearing. Mounting evidence suggests that age at implantation is a strong predictor of language outcomes. Using the minimally invasive surgical technique we have employed for nearly two decades, a limited clinical trial was initiated in the year 2000 because this age limitation fell outside of FDA guidelines...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780964/cochlear-implant-histopathology
#14
Fred H Linthicum, Joni K Doherty, Ivan A Lopez, Akira Ishiyama
The microscopic examination of fifty-five serially sectioned implanted temporal bones has provided insight into what is being stimulated; and the changes that are the result of the insertion and presence of the implant. The ganglion cell bodies (neurons) are structures being stimulated (two laboratories have reported an inverse relationship of the number of neurons and performance). Insertion through the round window, verses a cochleostomy, produces the least fibrosis and new bone. Fibrosis and new bone do not affect the implant function unless they form in the scala vestibuli in the region of the ductus reuniens, and, block it; and produce cochlear hydrops resulting in a delayed low tone loss of hearing in hybrid implants...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780963/the-cochlear-implant-and-possibilities-for-narrowing-the-remaining-gaps-between-prosthetic-and-normal-hearing
#15
Blake S Wilson
Background: The cochlear implant has become the standard of care for severe or worse losses in hearing and indeed has produced the first substantial restoration of a lost or absent human sense using a medical intervention. However, the devices are not perfect and many efforts to narrow the remaining gaps between prosthetic and normal hearing are underway. Objective: To assess the present status of cochlear implants and to describe possibilities for improving them...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780962/challenging-aspects-of-contemporary-cochlear-implant-electrode-array-design
#16
REVIEW
Pavel Mistrík, Claude Jolly, Daniel Sieber, Ingeborg Hochmair
Objective: A design comparison of current perimodiolar and lateral wall electrode arrays of the cochlear implant (CI) is provided. The focus is on functional features such as acoustic frequency coverage and tonotopic mapping, battery consumption and dynamic range. A traumacity of their insertion is also evaluated. Methods: Review of up-to-date literature. Results: Perimodiolar electrode arrays are positioned in the basal turn of the cochlea near the modiolus...
December 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29780422/cochlear-implantation-in-charcot-marie-tooth-disease-case-report-and-review-of-the-literature
#17
C Lane Anzalone, Sarah Nuhanovic, Amy P Olund, Matthew L Carlson
Introduction: Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. Methods: Case report and review of the English literature. Results: A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29780306/spontaneous-hair-cell-regeneration-is-prevented-by-increased-notch-signaling-in-supporting-cells
#18
Melissa M McGovern, Luyi Zhou, Michelle R Randle, Brandon C Cox
During embryonic development, differentiation of cochlear progenitor cells into hair cells (HCs) or supporting cells (SCs) is partially controlled through Notch signaling. Many studies have shown that inhibition of Notch signaling allows SCs to convert into HCs in both normal and drug damaged neonatal mouse cochleae. This mechanism is also implicated during HC regeneration in non-mammalian vertebrates; however, the mechanism of spontaneous HC regeneration in the neonatal mouse cochlea is less understood. While inhibition of Notch signaling can force SCs to convert into HCs and increase the number of regenerated HCs, it is currently unknown whether this pathway is involved in spontaneous HC regeneration observed in vivo ...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29779610/surgical-anatomy-for-hemifacial-spasm
#19
P Mercier, F Bernard
Classically in the cerebello-pontine angle the facial (CN VII) and vestibular-cochlear (CN VIII) nerves should run parallel with the anterior inferior cerebellar artery, whereas the lower nerves (CN IX-XI) continue with the posterior-inferior-cerebellar artery (PICA). In fact, this is not always true, particularly when dealing with hemispasm surgery where the relationships between CN VII, CN VIII and PICA are often different and closer. Knowledge of anatomical bases in surgical situation will help neurosurgeons to appreciate anatomical nuances, that are important to increase effectiveness and safety of hemifacial spasm surgery...
May 17, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29777327/intensity-discrimination-and-speech-recognition-of-cochlear-implant-users
#20
Colette M McKay, Natalie Rickard, Katherine Henshall
The relation between speech recognition and within-channel or across-channel (i.e., spectral tilt) intensity discrimination was measured in nine CI users (11 ears). Within-channel intensity difference limens (IDLs) were measured at four electrode locations across the electrode array. Spectral tilt difference limens were measured with (XIDL-J) and without (XIDL) level jitter. Only three subjects could perform the XIDL-J task with the amount of jitter required to limit use of within-channel cues. XIDLs (normalized to %DR) were correlated with speech recognition (r = 0...
May 17, 2018: Journal of the Association for Research in Otolaryngology: JARO
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