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SSNHL and MD demonstrated the highest speech perception score at 3 months (93 and 95%), and "Other" demonstrated the lowest scores at 88%. All 3 groups demonstrated nadir in speech scores at 6 months before improving at 12 months, but the "Other" diagnoses maintained the lowest speech testing across all time points. All 3 groups reported improved quality of life on CCIQ. CONCLUSIONS Subjects with SSNHL and MD demonstrate excellent speech perception and quality of life outcomes after cochlear implantation for SSD. Subjects with "Other" diagnoses underlying their SSD demonstrated lower scores on speech testing but nonetheless reported improved quality of life.OBJECTIVE Cochlear implant (CI) users struggle with tasks of pitch-based prosody perception. Pitch pattern recognition is vital for both music comprehension and understanding the prosody of speech, which signals emotion and intent. Research in normal-hearing individuals shows that auditory-motor training, in which participants produce the auditory pattern they are learning, is more effective than passive auditory training. We investigated whether auditory-motor training of CI users improves complex sound perception, such as vocal emotion recognition and pitch pattern recognition, compared with purely auditory training. STUDY DESIGN Prospective cohort study. SETTING Tertiary academic center. PATIENTS Fifteen postlingually deafened adults with CIs. INTERVENTION(S) Participants were divided into 3 one-month training groups auditory-motor (intervention), auditory-only (active control), and no training (control). Auditory-motor training was conducted with the "Contours" software program and auditory-only training was completed with the "AngelSound" software program. MAIN OUTCOME MEASURE Pre and posttest examinations included tests of speech perception (consonant-nucleus-consonant, hearing-in-noise test sentence recognition), speech prosody perception, pitch discrimination, and melodic contour identification. RESULTS Participants in the auditory-motor training group performed better than those in the auditory-only and no-training (p  less then  0.05) for the melodic contour identification task. No significant training effect was noted on tasks of speech perception, speech prosody perception, or pitch discrimination. CONCLUSIONS These data suggest that short-term auditory-motor music training of CI users impacts pitch pattern recognition. This study offers approaches for enriching the world of complex sound in the CI user.OBJECTIVES The HiFocus Mid-Scala electrode array (HFms) is designed to sit within the scala tympani without touching either the lateral wall or the modiolus. The aim of this study was to compare the HFms to the Helix perimodiolar electrode array. METHOD Two groups of recipients with Helix (n = 22 ears) and HFms (n = 29 ears) electrode arrays were retrospectively identified and matched by age at implantation and duration of severe to profound deafness. Most comfortable listening levels (M), impedances, Freiburger Monosyllables in quiet, and Oldenburg sentences in adaptive noise were compared at 3, 6, and 12 months postimplant. RESULTS Median scores for monosyllables in quiet for the HFms group were significantly better than the Helix group at each test interval (p  less then  0.05). Speech perception in quiet also significantly improved from 3 to 12 months for both groups (p  less then  0.001). There was no significant difference between the groups for speech in noise. Impedances were significantly lower for the HFms group at 12 months (p  less then  0.05) except at the basal end and M levels were generally higher. CONCLUSIONS The HFms group had better median performance for monosyllables in quiet than the Helix group at each test interval, although performance in noise was similar. For speech in noise, the HFms group appear to reach optimum performance quicker than the Helix group. Impedances were lower in the HFms group across the array, other than at the most basal end, and support our hypothesis that the HFms assumes a more lateral position within the cochlea than the Helix electrode, although our article did not include imaging data.OBJECTIVE This study investigated the audiological and tinnitus outcomes of cochlear implantation (CI) in adults with single-sided deafness (SSD) and tinnitus. STUDY DESIGN Multicentered prospective, non-randomized intervention study. SETTING Six French CI centers. PATIENTS Twenty-six patients with SSD and incapacitating tinnitus (Tinnitus Handicap Inventory [THI] >58) underwent cochlear implantation. INTERVENTIONS First, CIs delivered only masking white noise stimulation for 1 month and then standard CI stimulation. MAIN OUTCOME MEASURES Before and after CI surgery, patients completed the THI, Tinnitus Reaction Questionnaire (TRQ), Subjective Tinnitus Severity Scale (STSS), and two visual analogue scales quantifying tinnitus loudness and annoyance. Speech perception in spatialized noise was tested at 13 months. RESULTS The first month of white noise stimulation triggered a significant improvement in THI scores (72 ± 9 to 55 ± 20, p  less then  0.05). No change was observed for the other measures. After 1 year of standard CI stimulation, 23 patients (92%) reported a significant improvement in tinnitus. This improvement started 1 to 2 months after CI and exceeded 40% improvement for 14 patients (54%). Average speech-in-noise perception after 1 year significantly improved for the 23 patients who completed these measures. CONCLUSIONS CI is efficacious to reduce the handicap of patient with SSD and incapacitating tinnitus, leading to a decrease in reported tinnitus and partial restoration of binaural hearing abilities.OBJECTIVE Cochlear implant (CI) technology and techniques have advanced over the years. There has not been the same degree of change in programming and there remains a lack of standardization techniques. The purpose of this study is to compare performance in cochlear implant subjects using experienced clinician (EC) standard programming methods versus an Artificial Intelligence, FOX based algorithm for programming. STUDY DESIGN Prospective, nonrandomized, multicenter study using within-subject experimental design SETTING Tertiary referral centers. PATIENTS Fifty-five adult patients with ≥ 3 months experience with a Nucleus 5, 6, Kanso, or 7 series sound processor. INTERVENTION Therapeutic Main Outcome Measures CNC words and AzBio sentences in noise (+10 dB SNR) tests were administered in a soundproof booth followed by a direct connect psychoacoustic battery using the EC program. Tests were repeated 1 month later using the optimized FOX program. Subjective measures of patient satisfaction were also measured. check details RESULTS Performance for the EC program was compared to the FOX program for both measures. Group mean results revealed equivalent performance (Kruskal-Wallis ANOVA p = 0.934) with both programming methods. While some patients had better performance with the FOX method and some performed more poorly, the majority had equivalent performance and preferred the FOX system. CONCLUSION The study demonstrated that on average, FOX outcomes are equivalent to those using traditional programming techniques. In addition, the FOX programming method can effect standardization across centers and increase access for many individuals who could benefit.OBJECTIVE To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients. DATA SOURCES A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay." REVIEW METHODS Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up. RESULTS Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%. CONCLUSION Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.OBJECTIVE The aim of the study is to examine trends in the age of patients receiving cochlear implants and to determine the effect of age on the rate of perioperative complications. STUDY DESIGN Retrospective analysis of deidentified administrative claims data from a US commercial insurance database (Optum). PATIENTS Individuals undergoing cochlear implantation between 2003 and 2016. SETTING US hospital and outpatient facilities serving commercially insured patients. INTERVENTION Cochlear implantation. MAIN OUTCOME MEASURES Age at implantation, incidence of perioperative complications within 30 days identified by ICD9/10 codes including device problems, myocardial infarction, stroke, venous thromboembolism, local infection, meningitis, stroke, cerebrospinal fluid leak, and facial weakness. RESULTS Between 2003 and 2016, 3420 patients underwent a total of 4154 cochlear implants. The number of implants per year increased annually from 171 in 2003 to 531 in 2016, with the greatest growth demonstrated in those aged 60 and older.The age of patients undergoing implantation increased annually from an average of 26.6-57.2 years (p  less then  0.001). The implantation rates from 2003 to 2016, per 100,000 enrollees, increased from 1.64 to 6.82 for patients 60-79 years of age, and 0 to 11.57 for patients greater than 80 years of age (p  less then  0.001). No significant differences in 30-day complication rates were found between patients when grouped by age in decades, except for device related problems, which was significantly higher in younger patients ( less then 18 years). CONCLUSION Over the past decade and a half, cochlear implantation is more frequently being performed, and in an increasingly aging population. This trend does not seem to alter the risk of perioperative complications.OBJECTIVE USH2A-related disorders are characterised by genetic and phenotypic heterogeneity, and are associated with a spectrum of sensory deficits, ranging from deaf blindness to blindness with normal hearing. It has been previously proposed that the presence of specific USH2A alleles can be predictive of unaffected hearing. This study reports the clinical and genetic findings in a group of patients with USH2A-related disease and evaluates the validity of the allelic hierarchy model. PATIENTS AND INTERVENTION USH2A variants from 27 adults with syndromic and nonsyndromic USH2A-related disease were analyzed according to a previously reported model of allelic hierarchy. The analysis was replicated on genotype-phenotype correlation information from 197 individuals previously reported in 2 external datasets. MAIN OUTCOME MEASURE Genotype-phenotype correlations in USH2A-related disease. RESULTS A valid allelic hierarchy model was observed in 93% of individuals with nonsyndromic USH2A-retinopathy (n = 14/15) and in 100% of patients with classic Usher syndrome type IIa (n = 8/8).

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