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https://www.readbyqxmd.com/read/28346653/how-successful-is-the-fitting-of-digital-hearing-aids-implications-for-the-allocation-of-resources-within-national-health-systems
#1
Petros V Vlastarakos, Alastair I Cameron, Thomas P Nikolopoulos
We conducted a retrospective study to determine the success rate of initial fittings in digital hearing aid (DHA) users. We also addressed the implications of national health systems' continuing to provide access to these devices. We identified 1,597 consecutively presenting adults who had undergone a first fitting or a new fitting (i.e., an upgrade from an analogue hearing aid in the first or second ear) of a behind-the-ear DHA during the previous year. We further sought to identify all nominal reprogram appointments that had taken place within 6 months after the first or new fitting; we found 460 such appointments (28...
March 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28346292/bilateral-labyrinthine-and-internal-auditory-canal-enhancement-in-an-infant-with-severe-labyrinthine-dysplasia-a-previously-unreported-phenomenon
#2
Charles L Anzalone, Mai Lan Ho, Neil S Patel, Lisa A Schimmenti, Melissa D DeJong, Matthew L Carlson
OBJECTIVE: To describe a novel case of congenital profound bilateral sensorineural hearing loss in a patient with bilateral nodular internal auditory canal and labyrinthine enhancement and temporal bone dysplasia. PATIENTS: A 76-day-old female was referred to the authors' center for evaluation of congenital deafness. Behavioral observations and objective audiometric evaluation demonstrated bilateral profound sensorineural hearing loss and a comprehensive multidisciplinary evaluation identified compound heterozygous pathogenic variants in MYO7A, a gene associated with Usher Syndrome Type 1B or DFNB2...
March 24, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28343629/biallelic-variants-in-otud6b-cause-an-intellectual-disability-syndrome-associated-with-seizures-and-dysmorphic-features
#3
Teresa Santiago-Sim, Lindsay C Burrage, Frédéric Ebstein, Mari J Tokita, Marcus Miller, Weimin Bi, Alicia A Braxton, Jill A Rosenfeld, Maher Shahrour, Andrea Lehmann, Benjamin Cogné, Sébastien Küry, Thomas Besnard, Bertrand Isidor, Stéphane Bézieau, Isabelle Hazart, Honey Nagakura, LaDonna L Immken, Rebecca O Littlejohn, Elizabeth Roeder, Bulent Kara, Katia Hardies, Sarah Weckhuysen, Patrick May, Johannes R Lemke, Orly Elpeleg, Bassam Abu-Libdeh, Kiely N James, Jennifer L Silhavy, Mahmoud Y Issa, Maha S Zaki, Joseph G Gleeson, John R Seavitt, Mary E Dickinson, M Cecilia Ljungberg, Sara Wells, Sara J Johnson, Lydia Teboul, Christine M Eng, Yaping Yang, Peter-Michael Kloetzel, Jason D Heaney, Magdalena A Walkiewicz
Ubiquitination is a posttranslational modification that regulates many cellular processes including protein degradation, intracellular trafficking, cell signaling, and protein-protein interactions. Deubiquitinating enzymes (DUBs), which reverse the process of ubiquitination, are important regulators of the ubiquitin system. OTUD6B encodes a member of the ovarian tumor domain (OTU)-containing subfamily of deubiquitinating enzymes. Herein, we report biallelic pathogenic variants in OTUD6B in 12 individuals from 6 independent families with an intellectual disability syndrome associated with seizures and dysmorphic features...
March 21, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28338496/infants-and-adults-use-of-temporal-cues-in-consonant-discrimination
#4
Laurianne Cabrera, Lynne Werner
OBJECTIVES: Adults can use slow temporal envelope cues, or amplitude modulation (AM), to identify speech sounds in quiet. Faster AM cues and the temporal fine structure, or frequency modulation (FM), play a more important role in noise. This study assessed whether fast and slow temporal modulation cues play a similar role in infants' speech perception by comparing the ability of normal-hearing 3-month-olds and adults to use slow temporal envelope cues in discriminating consonants contrasts...
March 23, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28338374/the-clinical-features-and-prognosis-of-mumps-associated-hearing-loss-a-retrospective-multi-institutional-investigation-in-japan
#5
Shinya Morita, Keishi Fujiwara, Atsushi Fukuda, Satoshi Fukuda, Shin-Ya Nishio, Ryosuke Kitoh, Naohito Hato, Tetsuo Ikezono, Kotaro Ishikawa, Kimitaka Kaga, Atsushi Matsubara, Tatsuo Matsunaga, Takaaki Murata, Yasushi Naito, Kazunori Nishizaki, Kaoru Ogawa, Hajime Sano, Hiroaki Sato, Michihiko Sone, Mikio Suzuki, Haruo Takahashi, Tetsuya Tono, Hiroshi Yamashita, Tatsuya Yamasoba, Shin-Ichi Usami
CONCLUSIONS: The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss. OBJECTIVE: The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes...
March 24, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28335700/cochlear-implants-in-the-management-of-hearing-loss-in-neurofibromatosis-type-2
#6
Frances Harris, James R Tysome, Neil Donnelly, Juliette Durie-Gair, Gemma Crundwell, Yu Chuen Tam, Richard D Knight, Zebunnisa H Vanat, Nicola Folland, Patrick Axon
OBJECTIVE: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime. METHOD: Retrospective review of 12 patients, managed over the period 2009-2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI)...
March 24, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/28334328/vestibular-aqueduct-midpoint-width-and-hearing-loss-in-patients-with-an-enlarged-vestibular-aqueduct
#7
Mustafa S Ascha, Nauman Manzoor, Amit Gupta, Maroun Semaan, Cliff Megerian, Todd D Otteson
Importance: Elucidating the relationship between vestibular aqueduct size and hearing loss progression may inform the prognosis and counseling of patients who have an enlarged vestibular aqueduct (EVA). Objectives: To examine the association between vestibular aqueduct size and repeated measures of hearing loss. Design, Setting, and Participants: For this retrospective medical record review, 52 patients with a diagnosis of hearing loss and radiologic diagnosis of EVA according to the Valvassori criterion were included...
March 23, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28330595/electrophysiological-exploration-of-hearing
#8
D Bakhos, M Marx, A Villeneuve, E Lescanne, S Kim, A Robier
Electrophysiologic hearing tests have been developed since the 1960s to determine hearing thresholds objectively. They are now implemented in newborn hearing screening. While they determine thresholds, interpretation requires subjective pure-tone and speech audiometry to determine the type of hearing loss. Each examination tests a different anatomic region, enabling the auditory system to be explored from the organ of Corti to the auditory cortex. Thus, the various objective audiometric examinations are complementary...
March 16, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28323747/supporting-a-youth-with-cerebellar-ataxia-into-adolescence
#9
Veronica Meneses, Zurisadai Gonzalez-Castillo, Veronica B Edgar, Marilyn Augustyn
Zoe, a 13-year-old white girl, presents as a new patient to your pediatric clinic with complaints of frequent emesis, anxiety, and learning problems, and previous diagnosis of cerebellar ataxia. Parents accompany Zoe and state, "it is really hard for her to go out, she gets sick and falls easily." She was born full term by vaginal delivery without complications. Given globally delayed milestones, she received early intervention services. Feeding problems began at infancy, including gastroesophageal reflux and aspiration pneumonia...
March 17, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28322114/predictive-value-of-gjb2-mutation-status-for-hearing-outcomes-of-pediatric-cochlear-implantation
#10
Yasin Abdurehim, Alexandre Lehmann, Anthony G Zeitouni
Objective To systematically review and quantify current evidence regarding the association of GJB2 mutation status with outcomes of pediatric cochlear implantation. Data Sources PubMed, Embase, and the Cochrane Library were searched for "GJB2,"pediatric hearing loss," and "cochlear implantation" and their synonyms, with no language restrictions, until December 2, 2015. Review Methods Studies were included that investigated the status of GJB2 mutation and its predictive value for outcomes of pediatric cochlear implantation...
March 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28319479/brainstem-evoked-potential-indices-of-subcortical-auditory-processing-after-mild-traumatic-brain-injury
#11
Kathy R Vander Werff, Brian Rieger
OBJECTIVES: The primary aim of this study was to assess subcortical auditory processing in individuals with chronic symptoms after mild traumatic brain injury (mTBI) by measuring auditory brainstem responses (ABRs) to standard click and complex speech stimuli. Consistent with reports in the literature of auditory problems after mTBI (despite normal-hearing thresholds), it was hypothesized that individuals with mTBI would have evidence of impaired neural encoding in the auditory brainstem compared to noninjured controls, as evidenced by delayed latencies and reduced amplitudes of ABR components...
March 17, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28303288/-technical-advancements-in-cochlear-implants-state-of-the-art
#12
A Büchner, L Gärtner
Twenty years ago, cochlear implants (CI) were indicated only in cases of profound hearing loss or complete deafness. While from today's perspective the technology was clumsy and provided patients with only limited speech comprehension in quiet scenarios, successive advances in CI technology and the consequent substantial hearing improvements over time have since then resulted in continuous relaxation of indication criteria toward residual hearing. While achievements in implant and processor electronics have been one key factor for the ever-improving hearing performance, development of electro-acoustic CI systems-together with atraumatic implantation concepts-has led to enormous improvements in patients with low-frequency residual hearing...
March 16, 2017: HNO
https://www.readbyqxmd.com/read/28301390/pre-and-postoperative-binaural-unmasking-for-bimodal-cochlear-implant-listeners
#13
Benjamin M Sheffield, Gerald Schuchman, Joshua G W Bernstein
OBJECTIVES: Cochlear implants (CIs) are increasingly recommended to individuals with residual bilateral acoustic hearing. Although new hearing-preserving electrode designs and surgical approaches show great promise, CI recipients are still at risk to lose acoustic hearing in the implanted ear, which could prevent the ability to take advantage of binaural unmasking to aid speech recognition in noise. This study examined the tradeoff between the benefits of a CI for speech understanding in noise and the potential loss of binaural unmasking for CI recipients with some bilateral preoperative acoustic hearing...
March 15, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28291986/considerations-for-pediatric-cochlear-implant-recipients-with-unilateral-or-asymmetric-hearing-loss-assessment-device-fitting-and-habilitation
#14
Laura Greaver, Hannah Eskridge, Holly F B Teagle
Purpose: The purpose of this clinical report is to present case studies of children who are nontraditional candidates for cochlear implantation because they have significant residual hearing in 1 ear and to describe outcomes and considerations for their audiological management and habilitation. Method: Case information is presented for 5 children with profound hearing loss in 1 ear and normal or mild-to-moderate hearing loss in the opposite ear and who have undergone unilateral cochlear implantation...
March 14, 2017: American Journal of Audiology
https://www.readbyqxmd.com/read/28289594/a-9-year-old-girl-with-phelan-mcdermid-syndrome-who-had-been-diagnosed-with-an-autism-spectrum-disorder
#15
I Görker, H Gürkan, S Demir Ulusal, E Atlı, E Ikbal Atlı
Phelan McDermid Syndrome (PHMDS) (OMIM #606232), is a contiguous gene disorder resulting from deletion of the distal long arm of chromosome 22. The 22q13.3 deletions and mutations that lead to a loss of a functional copy of SHANK3 (OMIM *606230) cause the syndrome, characterized by moderate to profound intellectual disability, severely delayed or absent speech, hypotonia, and autism spectrum disorder (ASD) or ASD traits. In this study, we present the case of a 9-year-old girl who had earlier been diagnosed with an ASD...
December 1, 2016: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28286368/a-sound-therapy-based-intervention-to-expand-the-auditory-dynamic-range-for-loudness-among-persons-with-sensorineural-hearing-losses-case-evidence-showcasing-treatment-efficacy
#16
REVIEW
Craig Formby, LaGuinn P Sherlock, Monica L Hawley, Susan L Gold
Case evidence is presented that highlights the clinical relevance and significance of a novel sound therapy-based treatment. This intervention has been shown to be efficacious in a randomized controlled trial for promoting expansion of the dynamic range for loudness and increased sound tolerance among persons with sensorineural hearing losses. Prior to treatment, these individuals were unable to use aided sound effectively because of their limited dynamic ranges. These promising treatment effects are shown in this article to be functionally significant, giving rise to improved speech understanding and enhanced hearing aid benefit and satisfaction, and, in turn, to enhanced quality of life posttreatment...
February 2017: Seminars in Hearing
https://www.readbyqxmd.com/read/28286367/structured-counseling-for-auditory-dynamic-range-expansion
#17
REVIEW
Susan L Gold, Craig Formby
A structured counseling protocol is described that, when combined with low-level broadband sound therapy from bilateral sound generators, offers audiologists a new tool for facilitating the expansion of the auditory dynamic range (DR) for loudness. The protocol and its content are specifically designed to address and treat problems that impact hearing-impaired persons who, due to their reduced DRs, may be limited in the use and benefit of amplified sound from hearing aids. The reduced DRs may result from elevated audiometric thresholds and/or reduced sound tolerance as documented by lower-than-normal loudness discomfort levels (LDLs)...
February 2017: Seminars in Hearing
https://www.readbyqxmd.com/read/28285827/our-auditory-results-using-the-vibrant-soundbridge-on-the-long-process-of-the-incus-20-years-of-data
#18
Anaïs Grégoire, Jean-Philippe Van Damme, Chantal Gilain, Benoit Bihin, Pierre Garin
OBJECTIVE: After 20 years of experience with different types of middle ear implants, we analyzed our database about the Vibrant Soundbridge (VSB) to know the rate of complications, the effect on the residual hearing and the audiometric gain in our center. METHOD: The study was retrospective and included all VSB implants bound to the long process of the incus in our tertiary medical center between january 1999 and february 2015. We observed the effect of surgery on residual hearing by comparing bone and air conduction thresholds before and after implantation...
March 9, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28283378/the-effects-of-synthetic-cannabinoid-ur-144-on-the-human-body-a-review-of-39-cases
#19
Piotr Adamowicz, Joanna Gieroń, Dominika Gil, Wojciech Lechowicz, Agnieszka Skulska, Bogdan Tokarczyk
UR-144 [(1-pentyl-1H-indol-3-yl)(2,2,3,3-tetramethylcyclopropyl)methanone] is a synthetic cannabinoid, which has been detected in many 'legal highs', seized from the global drug market since the beginning of 2012. It has gained popularity as a 'legal' alternative to classic cannabis in countries where it was not controlled. Despite the widespread distribution of this substance, the data on its effects on the human body are scarce. Therefore, this paper describes the results of analysis and observed effects in 39 cases in which UR-144 was determined in blood...
March 7, 2017: Forensic Science International
https://www.readbyqxmd.com/read/28277214/pediatric-minimum-speech-test-battery
#20
Kristin Uhler, Andrea Warner-Czyz, Rene Gifford, Pmstb Working Group
BACKGROUND: Assessment of patient outcomes and documentation of treatment efficacy serves as an essential component of (re)habilitative audiology; however, no standardized protocol exists for the assessment of speech perception abilities for children with hearing loss. This presents a significant challenge in tracking performance of children who utilize various hearing technologies for within-subjects assessment, between-subjects assessment, and even across different facilities. PURPOSE: The adoption and adherence to a standardized assessment protocol could help facilitate continuity of care, assist in clinical decision making, allow clinicians and researchers to define benchmarks for an aggregate clinical population, and in time, aid with patient counseling regarding expectations and predictions regarding longitudinal outcomes...
March 2017: Journal of the American Academy of Audiology
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