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https://www.readbyqxmd.com/read/29588140/cochlear-implant-function-in-a-patient-with-jervell-and-lange-nielsen-syndrome-after-defibrillation-by-countershock
#1
Shinsuke Kaneshiro, Harukazu Hiraumi, Kirito Shimamoto, Kaori Sasamori, Yumiko Kobayashi, Hiroaki Sato
Jervell and Lange-Nielsen syndrome (JLNS), a rare autosomal recessive congenital QT prolongation syndrome, is characterized by cardiac arrhythmias, syncopal episodes, and profound deafness. A cochlear implant (CI) for patients with JLNS is expected to result in hearing improvement. Sometimes, defibrillation is required if a patient experiences lethal arrhythmia. In this paper, we report a pediatric patient with JLNS who received defibrillation after CI surgery in his right ear at the age of 2 years. With intensive care, the post-operative course was uneventful, and the patient acquired satisfactory speech and hearing abilities...
March 24, 2018: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/29484971/music-training-can-improve-music-and-speech-perception-in-pediatric-mandarin-speaking-cochlear-implant-users
#2
Xiaoting Cheng, Yangwenyi Liu, Yilai Shu, Duo-Duo Tao, Bing Wang, Yasheng Yuan, John J Galvin, Qian-Jie Fu, Bing Chen
Due to limited spectral resolution, cochlear implants (CIs) do not convey pitch information very well. Pitch cues are important for perception of music and tonal language; it is possible that music training may improve performance in both listening tasks. In this study, we investigated music training outcomes in terms of perception of music, lexical tones, and sentences in 22 young (4.8 to 9.3 years old), prelingually deaf Mandarin-speaking CI users. Music perception was measured using a melodic contour identification (MCI) task...
January 2018: Trends in Hearing
https://www.readbyqxmd.com/read/29447813/long-term-outcomes-of-universiti-kebangsaan-malaysia-cochlear-implant-program-among-pediatric-implantees
#3
Bee-See Goh, Noraziana Fadzilah, Asma Abdullah, Basyariatul Fathi Othman, Cila Umat
OBJECTIVES: Cochlear implant (CI) greatly enhances auditory performance as compared to hearing aids and has dramatically affected the educational and communication outcomes for profoundly deaf children. Universiti Kebangsaan Malaysia (UKM) pioneered CI program in 1995 in the South East Asia. We would like to report the long-term outcomes of UKM paediatric cochlear implantation in terms of: the proportion of children who were implanted and still using the device, the children's modes of communication, their educational placements, and their functional auditory/oral performance...
February 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29382382/resolution-of-unilateral-sensorineural-hearing-loss-in-a-pediatric-patient-with-a-severe-phenotype-of-muckle-wells-syndrome-treated-with-anakinra-a-case-report-and-review-of-the-literature
#4
Cinzia Marchica, Faisal Zawawi, Dania Basodan, Rosie Scuccimarri, Sam J Daniel
BACKGROUND: Muckle-Wells syndrome (MWS) is a rare auto-inflammatory disease characterized by the presence of recurrent urticaria, deafness and amyloidosis. Progressive sensorineural hearing loss (SNHL) is reported to occur in up to 85% of patients occurring in the second and third decades and as early as the first decade in patients with a more severe phenotype, thus potentially having a significant impact on a child's development. IL-1 inhibitors, such as Anakinra, have been described to improve systemic inflammation, and stabilize or improve hearing status as well...
January 30, 2018: Journal of Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/29335387/clear-cell-carcinoma-of-the-abdominal-wall
#5
Ewa Kostrzeba, M Barczyk, M Wichtowski, R Garstecki, D Murawa
BACKGROUND: Clear cell carcinoma in scars after cesarean section is extremely rare, with only 22 cases reported in the literature. Management of this condition needs to be further explored. Here, we report of a patient with clear cell carcinoma of the abdominal wall that developed 35 years after cesarean section. CASE REPORT: The material of the study was a group of 61 patients divided into two groups. Group I - 35 deaf or with profound sensorineural hearing loss children (the pupils of the deaf and hard of hearing school), aged 5-17 years (average 9,2 years), 14 males, 21 females, II - control group comprised 26 normal hearing patients, aged 5-16 years (average 10,4 years), 14 males, 12 females (patients of Department of Pediatric Otolaryngology, Audiology and Phoniatrics, Medical University of Lodz)...
December 30, 2017: Polski Przeglad Chirurgiczny
https://www.readbyqxmd.com/read/29324475/bilateral-cochlear-implants-maximizing-expected-outcomes
#6
Kate E Wallis, Nathan J Blum, Stephanie A Waryasz, Marilyn Augustyn
Sonia is a 4 years 1 month-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 years 7 months years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate...
February 2018: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/29186598/favorable-long-term-functional-outcomes-and-safety-of-auditory-brainstem-implants-in-nontumor-patients
#7
Na Young Jung, Minsoo Kim, Won Seok Chang, Hyun Ho Jung, Jae Young Choi, Jin Woo Chang
BACKGROUND: Auditory brainstem implants (ABIs) were designed to restore hearing in deaf patients with auditory tumors or those unable to receive cochlear implants. Although ABIs may help some patients, their long-term outcomes have been rarely studied. OBJECTIVE: To assess the long-term benefits and safety of ABIs in nontumor patients with sensorineural hearing loss (SNHL). METHODS: We retrospectively reviewed the histories of 15 deaf patients (8 females, 7 males; mean age, 15...
December 1, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29141319/-homozygous-ectonucleotide-pyrophosphatase-phosphodiesterase-1-variants-in-a-girl-with-hypophosphatemic-rickets-and-literature-review
#8
Z Q Liu, X B Chen, F Y Song, K Gao, M F Qiu, Y Qian, M Du
Objective: To investigate the clinical features and genetic characteristics of patients with ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) gene variants. Method: The clinical data of a patient with ENPP1 homozygous variants from Capital Institute of Pediatrics was collected, the related literature was searched from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, National Center from Biotechnology Information and PubMed by using search term "ENPP1" , "hypophosphatemic rickets" ...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29130438/segmental-and-suprasegmental-perception-in-children-using-hearing-aids
#9
Kaitlyn A Wenrich, Lisa S Davidson, Rosalie M Uchanski
BACKGROUND: Suprasegmental perception (perception of stress, intonation, "how something is said" and "who says it") and segmental speech perception (perception of individual phonemes or perception of "what is said") are perceptual abilities that provide the foundation for the development of spoken language and effective communication. While there are numerous studies examining segmental perception in children with hearing aids (HAs), there are far fewer studies examining suprasegmental perception, especially for children with greater degrees of residual hearing...
November 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/29106853/sequential-pediatric-bilateral-cochlear-implantation-the-effect-of-time-interval-between-implants
#10
Giovanni Bianchin, Lorenzo Tribi, Patrizia Formigoni, Carmela Russo, Valeria Polizzi
OBJECTIVE: To examine speech intelligibility in children subjected to sequential bilateral cochlear implants (CI) surgery and to assess the influence of the inter-stage interval duration. INTRODUCTION: Binaural hearing recovery can have additional benefits, especially in speech and language development in patients with congenital profound sensorineural hearing loss; so recently there has been an increase in the number of children receiving bilateral CI. METHODS: Twenty-seven children who underwent sequential bilateral cochlear implant (SBCI) with a short (1-3 yrs), medium (4-6 yrs) and long (7-12 yrs) range interval between both implantations, respectively, were evaluated...
November 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29019629/health-disparities-and-child-development-after-prematurity
#11
Frances A Carter, Michael E Msall
There is increased recognition that preterm neonates require sequential surveillance to capture the spectrum of coordination, communication, learning, and behavior regulation disorders that may occur in the first 5 years of life and beyond. In particular, the framework of follow-up needs to go beyond the detection of cerebral palsy, blindness, and deafness in the first 2 years of life for only those at highest preterm risk (ie, <28 weeks gestation, with combinations of severe cranial sonographic abnormalities, bronchopulmonary dysplasia, and retinopathy of prematurity)...
October 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28946862/long-term-outcome-in-survivors-of-neonatal-tetanus-following-specialist-intensive-care-in-vietnam
#12
Huynh T Trieu, Nguyen Thi Kim Anh, Huynh Ngoc Thien Vuong, T T M Dao, Nguyen Thi Xuan Hoa, Vo Ngoc Cat Tuong, Pham Tam Dinh, Bridget Wills, Phan Tu Qui, Le Van Tan, Lam Minh Yen, Saraswathy Sabanathan, Catherine Louise Thwaites
BACKGROUND: Neonatal tetanus continues to occur in many resource-limited settings but there are few data regarding long-term neurological outcome from the disease, especially in settings with critical care facilities. METHODS: We assessed long-term outcome following neonatal tetanus in infants treated in a pediatric intensive care unit in southern Vietnam. Neurological and neurodevelopmental testing was performed in 17 survivors of neonatal tetanus and 18 control children from the same communities using tools previously validated in Vietnamese children...
September 25, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28895463/impact-of-primary-language-and-insurance-on-pediatric-hearing-health-care-in-a-multidisciplinary-clinic
#13
Brooke M Su, Jason S Park, Dylan K Chan
Objective This study aims to describe the effects of primary language and insurance status on care utilization among deaf or hard-of-hearing children under active otolaryngologic and audiologic care. Study Design Cross-sectional analysis. Setting Multidisciplinary hearing loss clinic at a tertiary center. Subjects and Methods Demographics, hearing loss data, and validated survey responses were collected from 206 patients aged 0 to 19 years. Two-sided t tests and χ(2) tests were used to obtain descriptive statistics and hypothesis testing...
October 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28841141/outcome-of-cochlear-implantation-in-prelingually-deafened-children-according-to-molecular-genetic-etiology
#14
Joo Hyun Park, Ah Reum Kim, Jin Hee Han, Seong Dong Kim, Shin Hye Kim, Ja-Won Koo, Seung Ha Oh, Byung Yoon Choi
OBJECTIVES: About 60% of Korean pediatric cochlear implantees could be genetically diagnosed (GD) and we previously reported that a substantial portion of undiagnosed cases by deafness gene panel sequencing were predicted to have a nongenetic or complex etiology. We aimed to compare the outcomes of cochlear implantation (CI) in GD and genetically undiagnosed (GUD) patients and attempted to determine CI outcomes according to etiology. DESIGN: Ninety-three pediatric cochlear implantees underwent molecular genetic testing...
September 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28801085/measuring-in-vivo-cerebral-maturation-using-age-related-t-2-relaxation-times-at-3t
#15
Eva Bültmann, Loukia M Spineli, Hans Hartmann, Heinrich Lanfermann
OBJECTIVE: To examine age-related changes in T2 relaxation times during infancy and childhood in order to assess T2 values obtained from routine MRI as a biomarker. METHODS: From our pool of clinical pediatric MRI examinations at 3T all patients with normal conventional MRI scans were retrospectively selected. Depending on their clinical findings the identified 99 patients (0-199months) were divided into 43 healthy controls and 56 diseased children with various clinical abnormalities (developmental delay, epilepsy, prematurity, and deafness)...
February 2018: Brain & Development
https://www.readbyqxmd.com/read/28776715/cost-effectiveness-of-pediatric-bilateral-cochlear-implantation-in-spain
#16
Jorge Pérez-Martín, Miguel A Artaso, Francisco J Díez
OBJECTIVES/HYPOTHESIS: To determine the incremental cost-effectiveness of bilateral versus unilateral cochlear implantation for 1-year-old children suffering from bilateral sensorineural severe to profound hearing loss from the perspective of the Spanish public health system. STUDY DESIGN: Cost-utility analysis. METHODS: We conducted a general-population survey to estimate the quality-of-life increase contributed by the second implant. We built a Markov influence diagram and evaluated it for a life-long time horizon with a 3% discount rate in the base case...
December 2017: Laryngoscope
https://www.readbyqxmd.com/read/28753163/cortical-processing-of-vocal-and-nonvocal-sounds-in-cochlear-implanted-children-an-electrophysiological-study
#17
David Bakhos, John Galvin, Sylvie Roux, Emmanuel Lescanne, Nicole Bruneau
OBJECTIVES: For prelingually deaf children, cochlear implants (CIs) can restore auditory input to the auditory cortex and the ability to acquire spoken language. Language development is strongly intertwined with voice perception. The aim of this electrophysiological study was to investigate human voice processing using measures of cortical auditory evoked potentials (AEPs) in pediatric CI users. DESIGN: Cortical AEPs were measured in 8 CI children (4 to 12 years old) with good auditory and language performance and 8 normal-hearing (NH) age-matched controls...
January 2018: Ear and Hearing
https://www.readbyqxmd.com/read/28724130/early-postimplant-speech-perception-and-language-skills-predict-long-term-language-and-neurocognitive-outcomes-following-pediatric-cochlear-implantation
#18
Cynthia R Hunter, William G Kronenberger, Irina Castellanos, David B Pisoni
Purpose: We sought to determine whether speech perception and language skills measured early after cochlear implantation in children who are deaf, and early postimplant growth in speech perception and language skills, predict long-term speech perception, language, and neurocognitive outcomes. Method: Thirty-six long-term users of cochlear implants, implanted at an average age of 3.4 years, completed measures of speech perception, language, and executive functioning an average of 14...
August 16, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28704896/long-term-speech-perception-outcomes-of-cochlear-implantation-in-gap-junction-protein-beta-2-related-hearing-loss
#19
Sung Hee Kim, Rajendra Nepali, Myung Hoon Yoo, Kwang-Sun Lee, Jong Woo Chung
BACKGROUND AND OBJECTIVES: The mutation of the gap junction protein beta 2 (GJB2) gene is the predominant cause of autosomal recessive non-syndromic hearing loss. The purpose of this study was to evaluate the speech perception outcome after cochlear implantation according to the presence of a GJB2 mutation. SUBJECTS AND METHODS: During the period from March 2004 to February 2005, 38 patients underwent cochlear implantation at Asan Medical Center. Genetic factors and speech perception were evaluated in all subjects, and the patients were grouped according to the presence of a GJB2 mutation...
July 2017: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/28688548/eeg-activity-as-an-objective-measure-of-cognitive-load-during-effortful-listening-a-study-on-pediatric-subjects-with-bilateral-asymmetric-sensorineural-hearing-loss
#20
Pasquale Marsella, Alessandro Scorpecci, Giulia Cartocci, Sara Giannantonio, Anton Giulio Maglione, Isotta Venuti, Ambra Brizi, Fabio Babiloni
OBJECTIVES: Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks...
August 2017: International Journal of Pediatric Otorhinolaryngology
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