keyword
https://read.qxmd.com/read/38539359/unilateral-sensorineural-hearing-loss-in-children-etiology-audiological-characteristics-and-treatment
#1
JOURNAL ARTICLE
Mirko Aldè, Diego Zanetti, Umberto Ambrosetti, Eleonora Monaco, Anna Maria Gasbarre, Lorenzo Pignataro, Giovanna Cantarella, Stefania Barozzi
The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45...
March 9, 2024: Children
https://read.qxmd.com/read/38529662/early-results-with-the-new-active-bone-conduction-hearing-implant-a-systematic-review-and-meta-analysis
#2
REVIEW
Alexander Lein, Wolf-Dieter Baumgartner, Dominik Riss, Wolfgang Gstöttner, Lukas D Landegger, David T Liu, Thomas Thurner, Erich Vyskocil, Faris F Brkic
OBJECTIVE: The bone conduction implant (BCI) 602 is a new transcutaneous BCI with smaller dimensions. However, limited patient numbers restrict the statistical power and generalizability of the current studies. The present systematic review and meta-analysis summarize early audiological and medical outcomes of adult and pediatric patients implanted with the BCI 602 due to mixed or conductive hearing loss. DATA SOURCE: Following the Preferred Reporting items for Systematic Reviews and Meta-analyses guidelines, 108 studies were reviewed, and 6 (5...
March 26, 2024: Otolaryngology—Head and Neck Surgery
https://read.qxmd.com/read/38525688/large-vestibular-aqueduct-associated-symptoms-endolymphatic-duct-blockage-as-a-surgical-treatment
#3
JOURNAL ARTICLE
Issam Saliba, Sarah Alshehri, Isabelle Fournier, Nasser Altamami
OBJECTIVE: This study aimed to evaluate the effectiveness of endolymphatic duct blockage (EDB) on dizziness control in patients with a large vestibular aqueduct (LVA) and to evaluate its effect on hearing. STUDY DESIGN: This is a prospective nonrandomized study. SETTING: Five adults and one child with dizziness and five children with progressive hearing loss were referred to our tertiary centers. METHODS: The dizziness handicap inventory (DHI) and DHI-PC (dizziness handicap inventory-patient caregiver) questionnaires were used before and after surgery...
March 18, 2024: Audiology Research
https://read.qxmd.com/read/38512716/more-than-40-years-of-cochlear-implant-research-a-bibliometric-analysis
#4
JOURNAL ARTICLE
Jaouad Abari, Ahmet M Tekin, Ilhan Bahşi, Vedat Topsakal
OBJECTIVES: Cochlear implantation is the most effective treatment for patients with severe-to-profound sensorineural hearing loss. Much scientific work has been published since their inception. There is a need for a critical reflection on how and what we publish on cochlear implantation. METHODS: All Science Citation Index Expanded featured articles between 1980 and 2022 with the word 'cochlear implants' or 'cochlear implantation' were collected from the Web of Science database...
March 21, 2024: Cochlear Implants International
https://read.qxmd.com/read/38510079/speech-language-functional-communication-psychosocial-outcomes-and-qol-in-school-age-children-with-congenital-unilateral-hearing-loss
#5
JOURNAL ARTICLE
Linda Cupples, Teresa Y C Ching, Sanna Hou
INTRODUCTION: Children with early-identified unilateral hearing loss (UHL) might be at risk for delays in early speech and language, functional communication, psychosocial skills, and quality of life (QOL). However, a paucity of relevant research prohibits strong conclusions. This study aimed to provide new evidence relevant to this issue. METHODS: Participants were 34 children, ages 9;0 to 12;7 (years;months), who were identified with UHL via newborn hearing screening...
2024: Frontiers in Pediatrics
https://read.qxmd.com/read/38509666/gaucher-disease-coexisting-with-cytomegalovirus-infection-a-rare-presentation-in-an-infant
#6
JOURNAL ARTICLE
Zhaoxia Zhang, Dong Liu, Zhangbin Yu, Zhihui Xiao, Keying Zhou, Bo Li
BACKGROUND Gaucher disease is a rare autosomal recessive disorder characterized by mutations in the glucocerebrosidase gene, resulting in deficient enzyme activity and accumulation of glucocerebroside in macrophages, which leads to pathological changes in affected organs. The atypical clinical manifestations of Gaucher disease often contribute to delays in diagnosis and treatment. CASE REPORT We present the case of a 4-month-old female infant admitted to the Department of Pediatrics with progressive hepatosplenomegaly since birth...
March 21, 2024: American Journal of Case Reports
https://read.qxmd.com/read/38498908/-adaptation-and-validation-for-european-portuguese-of-the-auditory-performance-categories-ii-and-infant-toddler-meaningful-auditory-integration-scale-for-children-with-cochlear-implant
#7
JOURNAL ARTICLE
Luí S Roque Reis, Kaamil Gani, André, Carlota Peres, Gonçalo Nunes, Ricardo Santos, Assunção O'Neill, Pedro Escada
INTRODUCTION: The Categories of Auditory Performance II (CAP-II) scale and the Infant-Toddler Meaningful Audit Integration Scale (IT-MAIS) are simple and quick questionnaires that allow assessment of the auditory performance of children with cochlear implant (CI). The aim of this study was to translate, adapt and validate the European Portuguese version of the CAP-II and IT-MAIS scales. METHODS: A total of 85 participants completed the European Portuguese version of the CAP-II and IT-MAIS questionnaires, of which 45 were parents of children with pediatric cochlear implants (9...
March 18, 2024: Acta Médica Portuguesa
https://read.qxmd.com/read/38487823/predictors-of-the-outcome-at-2-years-in-neonates-with-congenital-cytomegalovirus-infection
#8
JOURNAL ARTICLE
Jacques Fourgeaud, Jean-François Magny, Sophie Couderc, Patricia Garcia, Anne-Marie Maillotte, Melinda Benard, Didier Pinquier, Philippe Minodier, Dominique Astruc, Hugues Patural, Sophie Parat, Bernard Guillois, Armelle Garenne, Tiffany Guilleminot, Marine Parodi, Laurence Bussières, Idir Ghout, Yves Ville, Marianne Leruez-Ville
BACKGROUND: Approximately 20% of neonates with congenital cytomegalovirus (cCMV) develop long-term sequelae. The ability to accurately predict long-term outcomes as early as the neonatal period would help to provide for appropriate parental counseling and treatment indications. With this study, we aimed to identify neonatal predictive markers of cCMV long-term outcomes. METHODS: As this study's subjects, we chose neonates diagnosed with cCMV in 13 hospitals throughout France recruited from 2013 to 2017 and evaluated for at least 2 years with thorough clinical, audiology, and imaging evaluations and psychomotor development tests...
March 15, 2024: Pediatrics
https://read.qxmd.com/read/38478407/long-term-cochlear-implant-sound-processor-usage-in-children-with-single-sided-deafness
#9
JOURNAL ARTICLE
Raimundo J Garcia-Matte, Luke M O'Neil, Carl Chase, Pia Leeming, Rachel Clack, Steve Rodrigues, Jafri Kuthubutheen
OBJECTIVE: To assess cochlear implant (CI) sound processor usage over time in children with single-sided deafness (SSD) and identify factors influencing device use. STUDY DESIGN: Retrospective, chart review study. SETTING: Pediatric tertiary referral center. PATIENTS: Children with SSD who received CI between 2014 and 2020. OUTCOME MEASURE: Primary outcome was average daily CI sound processor usage over follow-up...
April 1, 2024: Otology & Neurotology
https://read.qxmd.com/read/38466937/pediatric-hearing-aid-daily-wear-time-is-significantly-impacted-by-clinician-family-language-discordance
#10
JOURNAL ARTICLE
Nicole Brigham, Emily C Thompson, Erin M Picou, Hilary Davis, Anne Marie Tharpe
PURPOSE: The purpose of this study was to evaluate the potential contribution of limited English proficiency on daily hearing aid wear time for children with hearing loss. METHOD: A retrospective chart review was completed to evaluate hearing aid wear time based on data logging information available at the time of a follow-up visit following an initial hearing aid fitting. Children were included in the study if they had permanent bilateral hearing loss and were less than 60 months of age at the time of their first follow-up visit...
March 11, 2024: American Journal of Audiology
https://read.qxmd.com/read/38461681/world-of-sounds-seslerin-d%C3%A3-nyas%C3%A4-a-mobile-auditory-training-game-for-children-with-cochlear-implants
#11
JOURNAL ARTICLE
Merve Meral Çetinkaya, Özlem Konukseven, Ali Efe İralı
OBJECTIVES: The aim of this study is to develop a mobile auditory training application based on gaming for children aged 3-5 years using cochlear implants and to evaluate its usability. METHODS: 4 games were developed in the scope of the application World of Sounds; the crucible sound for auditory awareness, mole hunting for auditory discrimination, find the sound for auditory recognition, and choo-choo for auditory comprehension. The prototype was applied to 20 children with normal hearing and 20 children with cochlear implants, all of whom were aged 3-5...
March 6, 2024: International Journal of Pediatric Otorhinolaryngology
https://read.qxmd.com/read/38456989/risk-factors-for-infant-hearing-loss-a-meta-analysis
#12
JOURNAL ARTICLE
Yiwei Han, Shangbin Li, Yankun Song, Jingfei Sun, Weichen Yan, Jie Wang, Xiong Gao, Xueying Li, Changjun Ren, Qian Zhao
Hearing loss is a common disability in infants that significantly impacts their cognitive, language, and literacy development. This study aimed to systematically assess the risk factors for the early identification and intervention in infant hearing loss. Databases were searched for meta-analyses of observational studies until November 2023. The quality assessment was performed using the Cochrane risk of bias tool, and the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach was used to assess the certainty of the evidence...
March 8, 2024: European Journal of Pediatrics
https://read.qxmd.com/read/38451298/an-unusual-finding-of-an-anaplastic-meningioma-in-nf2-related-schwannomatosis
#13
JOURNAL ARTICLE
Siegfried J Adelhoefer, James Feghali, Sharika Rajan, Charles G Eberhart, Verena Staedtke, Alan R Cohen
NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids...
March 7, 2024: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://read.qxmd.com/read/38450295/bioinformatics-characterization-of-variants-of-uncertain-significance-in-pediatric-sensorineural-hearing-loss
#14
JOURNAL ARTICLE
Sloane Clay, Adele Evans, Regina Zambrano, David Otohinoyi, Chindo Hicks, Fern Tsien
INTRODUCTION: Rapid advancements in Next Generation Sequencing (NGS) and bioinformatics tools have allowed physicians to obtain genetic testing results in a more rapid, cost-effective, and comprehensive manner than ever before. Around 50% of pediatric sensorineural hearing loss (SNHL) cases are due to a genetic etiology, thus physicians regularly utilize targeted sequencing panels that identify variants in genes related to SNHL. These panels allow for early detection of pathogenic variants which allows physicians to provide anticipatory guidance to families...
2024: Frontiers in Pediatrics
https://read.qxmd.com/read/38448330/costs-analysis-of-cochlear-implantation-in-children
#15
JOURNAL ARTICLE
M Benifla, N Martelli, E Brenet, C Compagnon, X Dubernard, M Labrousse
OBJECTIVES: The study assessed the direct medical costs of the cochlear implantation pathway from the healthcare payer's perspective, in children with bilateral severe to profound hearing loss, from diagnosis to 3 years' follow-up after first implantation. We also compared costs between two populations: congenital and progressive deafness. MATERIAL AND METHODS: A retrospective costs analysis was performed for 56 children who received a cochlear implant in one French pediatric ENT center...
March 5, 2024: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://read.qxmd.com/read/38448301/long-term-efficacy-of-intrathecal-cyclodextrin-in-patients-with-niemann-pick-disease-type-c
#16
JOURNAL ARTICLE
Muneaki Matsuo, Takafumi Sakakibara, Yoshio Sakiyama, Tetsumin So, Motomichi Kosuga, Toshihiko Kakiuchi, Fumio Ichinose, Takuji Nakamura, Yoichi Ishitsuka, Tetsumi Irie
BACKGROUND AND OBJECTIVES: Niemann-Pick type C (NPC) is a rare lysosomal storage disease characterized by hepatosplenomegaly and progressive neurological deterioration due to abnormal intracellular cholesterol transport. Cyclic oligosaccharide 2-hydroxypropyl-β-cyclodextrin (HPBCD) is an effective treatment for NPC; however, few reports have shown its long-term efficacy and safety. To demonstrate long-term efficacy and safety of intrathecal HPBCD (IT-HPBCD) treatment for NPC, we herein reports five patients with NPC treated using IT-HPBCD for 4-11 years...
March 5, 2024: Brain & Development
https://read.qxmd.com/read/38440641/audiological-and-speech-and-language-characteristics-of-a-case-with-hunter-s-syndrome
#17
JOURNAL ARTICLE
Krithi Rao, Prajith Carthik, Aleena Varghese, Divya Seth, Mayur Bhat
Hearing loss is a common manifestation of Hunter's syndrome, with reported rates ranging from 67.3 to 94%. The aim is to highlight the audiological profile and pathophysiology of mixed hearing loss in individuals with hunter's syndrome. A 7.6-year-old male child was brought to the department of audiology with a complaint of not responding to name call and regression in the speech and language skills. Detailed audiological showed severe to profound mixed hearing loss. REELS and 3DLAT results showed RLA to be 9 to 10 months and ELA to be 6 to 7 months...
February 2024: Indian Journal of Otolaryngology and Head and Neck Surgery
https://read.qxmd.com/read/38440496/cochlear-implantation-in-children-with-meningitis-a-multicenter-study-on-auditory-performance-and-speech-production-outcomes
#18
JOURNAL ARTICLE
Mohsen Rajati, Mohamad Reza Afzalzadeh, Ahmad Daneshi, Mohammad Ajalloueyan, Seyed Basir Hashemi, Navid Nourizadeh, Mohammad Mahdi Ghasemi, Ali Moradi, Mohammad Farhadi, Alimohamad Asghari, Saleh Mohebbi
BACKGROUND: This study aims to evaluate speech production outcomes and auditory performance in children with post-meningitis deafness who were treated with cochlear implants. Additionally, the study assesses the impact of electrode insertion depth on surgical outcomes.". METHODS: We conducted a study on 66 pediatric patients with bilateral postmeningitis hearing loss who were being prepared for cochlear implantation at four tertiary referral academic institutions...
February 2024: Indian Journal of Otolaryngology and Head and Neck Surgery
https://read.qxmd.com/read/38432637/a-neurodevelopmental-disorder-associated-with-a-loss-of-function-missense-mutation-in-rab35
#19
JOURNAL ARTICLE
Adriana Aguila, Somaya Salah, Gopinath Kulasekaran, Moatasem Shweiki, Nava Shaul-Lotan, Hagar Mor-Shaked, Muhannad Daana, Tamar Harel, Peter S McPherson
Rab35 is a small GTPase that regulates endosomal membrane trafficking and functions in cell polarity, cytokinesis and growth factor signaling. Altered Rab35 function contributes to progression of glioblastoma, defects in primary cilia formation and altered cytokinesis. Here we report a pediatric patient with global developmental delay, hydrocephalus, a Dandy-Walker malformation, axial hypotonia with peripheral hypertonia, visual problems, and conductive hearing impairment. Exome sequencing identified a homozygous missense variant in the GTPase fold of RAB35 (c...
March 1, 2024: Journal of Biological Chemistry
https://read.qxmd.com/read/38427998/auditory-brainstem-implantation-surgical-experience-and-audiometric-outcomes-in-the-pediatric-population
#20
JOURNAL ARTICLE
Yosef M Dastagirzada, Alexander Eremiev, Jeffrey H Wisoff, Emily Kay-Rivest, William H Shapiro, Ansley Unterberger, Susan B Waltzman, J Thomas Roland, John G Golfinos, David H Harter
OBJECTIVE: Pediatric data regarding treatment via an auditory brainstem implant (ABI) remains sparse. The authors aimed to describe their experience at their institution and to delineate associated demographic data, audiometric outcomes, and surgical parameters. METHODS: An IRB-approved, retrospective chart review was conducted among the authors' pediatric patients who had undergone auditory brainstem implantation between 2012 and 2021. Demographic information including sex, age, race, coexisting syndrome(s), history of cochlear implant placement, average duration of implant use, and follow-up outcomes were collected...
March 1, 2024: Journal of Neurosurgery. Pediatrics
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