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pediatric deaf

Maja Svrakic, J Thomas Roland, Sean O McMenomey, Mario A Svirsky
OBJECTIVE: To describe our initial operative experience and hearing preservation results with the Advanced Bionics (AB) Mid Scala Electrode (MSE). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Sixty-three MSE implants in pediatric and adult patients were compared with age- and sex-matched 1j electrode implants from the same manufacturer. All patients were severe to profoundly deaf. INTERVENTION: Cochlear implantation with either the AB 1j electrode or the AB MSE...
October 12, 2016: Otology & Neurotology
Marc S Schwartz, Eric P Wilkinson
OBJECTIVE: Auditory brainstem implants (ABIs), which have previously been used to restore auditory perception to deaf patients with neurofibromatosis type 2 (NF2), are now being utilized in other situations, including treatment of congenitally deaf children with cochlear malformations or cochlear nerve deficiencies. Concurrent with this expansion of indications, the number of centers placing and expressing interest in placing ABIs has proliferated. Because ABI placement involves posterior fossa craniotomy in order to access the site of implantation on the cochlear nucleus complex of the brainstem and is not without significant risk, we aim to highlight issues important in developing and maintaining successful ABI programs that would be in the best interests of patients...
September 26, 2016: Laryngoscope
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
Kathryn Y Noonan, Jack Russo, Jun Shen, Heidi Rehm, Sara Halbach, Einar Hopp, Sarah Noon, Jacqueline Hoover, Clifford Eskey, James E Saunders
OBJECTIVE: To investigate the prevalence and relative risk of semicircular canal dehiscence (SCD) in pediatric patients with CDH23 pathogenic variants (Usher syndrome or non-syndromic deafness) compared with age-matched controls. STUDY DESIGN: Retrospective cohort study. SETTING: Multi-institutional study. PATIENTS: Pediatric patients (ages 0-5 years) were compared based on the presence of biallelic pathogenic variants in CDH23 with pediatric controls who underwent computed tomography (CT) temporal bone scan for alternative purposes...
September 14, 2016: Otology & Neurotology
Hannah Glick, Anu Sharma
This review explores cross-modal cortical plasticity as a result of auditory deprivation in populations with hearing loss across the age spectrum, from development to adulthood. Cross-modal plasticity refers to the phenomenon when deprivation in one sensory modality (e.g. the auditory modality as in deafness or hearing loss) results in the recruitment of cortical resources of the deprived modality by intact sensory modalities (e.g. visual or somatosensory systems). We discuss recruitment of auditory cortical resources for visual and somatosensory processing in deafness and in lesser degrees of hearing loss...
September 6, 2016: Hearing Research
Aaron Baker, David Fanelli, Sangam Kanekar, Huseyin Isildak
OBJECTIVE: Bone-anchored hearing aid has been shown to be effective in hearing rehabilitation for conductive loss or single-sided deafness. Current FDA guidelines allow implantation in patients over 5 years old. This guideline is at least partially due to concern for thickness of bone stock at the implant site. We aim to investigate whether temporal bone thickness should be a deterrent to implantation in those younger than five. STUDY DESIGN: A retrospective review of high-resolution temporal bone computed tomographies (CTs) comparing measurements between ears with chronic disease and controls...
October 2016: Otology & Neurotology
Ting Chen, Xiang-Hui Lu, Hui-Fang Wang, Rui Ban, Hua-Xu Liu, Qiang Shi, Qian Wang, Xi Yin, Chuan-Qiang Pu
BACKGROUND: Myopathies with rimmed vacuoles are a heterogeneous group of muscle disorders with progressive muscle weakness and varied clinical manifestations but similar features in muscle biopsies. Here, we describe a novel autosomal dominant myopathy with rimmed vacuoles in a large family with 11 patients of three generations affected. METHODS: A clinical study including family history, obstetric, pediatric, and development history was recorded. Clinical examinations including physical examination, electromyography (EMG), serum creatine kinase (CK), bone X-rays, and brain magnetic resonance imaging (MRI) were performed in this family...
August 5, 2016: Chinese Medical Journal
Irina Castellanos, David B Pisoni, William G Kronenberger, Jessica Beer
PURPOSE: The objective of the present article was to document the extent to which early expressive language skills (measured using the MacArthur-Bates Communicative Development Inventories [CDI; Fenson et al., 2006]) predict long-term neurocognitive outcomes in a sample of early-implanted prelingually deaf cochlear implant (CI) users. METHOD: The CDI was used to index the early expressive language skills of 32 pediatric CI users after an average of 1.03 years (SD = 0...
August 1, 2016: American Journal of Speech-language Pathology
Marzena Mielczarek, Anna Zakrzewska, Jurek Olszewski
INTRODUCTION: GJB2 mutations are the most frequent reason of genetic congenital hearing loss. The aim of the study was to assess the prevalence of GJB2 mutations in the deaf and profound hearing loss children. MATERIAL AND METHODS: 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...
June 30, 2016: Otolaryngologia Polska. the Polish Otolaryngology
Niu Li, Y U Ding, Tingting Yu, Juan Li, Yongnian Shen, Xiumin Wang, Qihua Fu, Yiping Shen, Xiaodong Huang, Jian Wang
Uniparental disomy (UPD), which is the abnormal situation in which both copies of a chromosomal pair have been inherited from one parent, may cause clinical abnormalities by affecting genomic imprinting or causing autosomal recessive variation. Whole Exome Sequencing (WES) and chromosomal microarray analysis (CMA) are powerful technologies used to search for underlying causal variants. In the present study, WES was used to screen for candidate causal variants in the genome of a Chinese pediatric patient, who had been shown by CMA to have maternal uniparental isodisomy of chromosome 10...
June 2016: Experimental and Therapeutic Medicine
S Burdo, A Giuliani, L Dalla Costa
OBJECTIVE: To identify the influence of the first implanted ear on the performance of the delayed sequentially implanted ear in pediatric patients. STUDY DESIGN: Retrospective case series review. SETTING: Outpatient Cochlear Implant (CI) center. PATIENTS: Congenitally deaf children who underwent unilateral implant at various ages followed by sequential implant of the contralateral ear with various delays were enrolled in the study...
June 2016: European Annals of Otorhinolaryngology, Head and Neck Diseases
Stephane Roman, Françoise Rochette, Jean-Michel Triglia, Daniele Schön, Emmanuel Bigand
UNLABELLED: While the positive benefits of pediatric cochlear implantation on language perception skills are now proven, the heterogeneity of outcomes remains high. The understanding of this heterogeneity and possible strategies to minimize it is of utmost importance. Our scope here is to test the effects of an auditory training strategy, "sound in Hands", using playful tasks grounded on the theoretical and empirical findings of cognitive sciences. Indeed, several basic auditory operations, such as auditory scene analysis (ASA) are not trained in the usual therapeutic interventions in deaf children...
July 2016: Hearing Research
Sidharth V Puram, Samuel R Barber, Elliott D Kozin, Parth Shah, Aaron Remenschneider, Barbara S Herrmann, Ann-Christine Duhaime, Fred G Barker, Daniel J Lee
There are no approved Food and Drug Administration indications for pediatric auditory brainstem implant (ABI) surgery in the United States. Our prospective case series aims to determine the safety and feasibility of ABI surgery in pediatric patients <5 years old with congenital deafness at a tertiary North American center. The inclusion criterion was pre- or postlinguistic deafness in children not eligible for cochlear implantation. Seventeen candidates were evaluated (mean ± SD: age, 2.52 ± 0.39 years)...
July 2016: Otolaryngology—Head and Neck Surgery
Sheila Moodie, Eileen Rall, Leisha Eiten, George Lindley, Dave Gordey, Lisa Davidson, Marlene Bagatto, Susan Scollie
BACKGROUND: There is broad consensus that screening and diagnosis of permanent hearing loss in children must be embedded within a comprehensive, evidence-based, family-centered intervention program. Clinical practice guidelines (CPGs) for pediatric hearing assessment and hearing aid verification aim to reduce variability in practice and increase the use of effective evidence-based diagnostic and treatment options so that optimal outcomes may be achieved. To be of value, guidelines must be translated and implemented into practice and ongoing monitoring of their use in practice should occur...
March 2016: Journal of the American Academy of Audiology
Parth V Shah, Elliott D Kozin, Alyson B Kaplan, Daniel J Lee
INTRODUCTION: The auditory brainstem implant (ABI) is a neuroprosthetic device that provides sound sensations to individuals with profound hearing loss who are not candidates for a cochlear implant (CI) because of anatomic constraints. Herein we describe the ABI for family physicians. METHODS: PubMed was searched to identify articles relevant to the ABI, as well as articles that contain outcomes data for pediatric patients (age <18 years) who have undergone ABI surgery...
March 2016: Journal of the American Board of Family Medicine: JABFM
Carmen Ares, Francesca Albertini, Martina Frei-Welte, Alessandra Bolsi, Michael A Grotzer, Gudrun Goitein, Damien C Weber
To assess the clinical outcome and late side effect profile of pencil beam scanning proton therapy (PT) delivered to children with intracranial ependymoma. Between July-2004 and March-2013, 50 patients with intracranial ependymoma (n = 46, grade 3) received involved-field PT at Paul Scherrer Institute (PSI). Median age at time of PT was 2.6 years (range 1.1-15.2). Thirty-six patients had infratentorial and 14 supratentorial ependymomas. Seventeen patients presented with macroscopic residual disease after subtotal resection before starting PT (8 with ≤1...
May 2016: Journal of Neuro-oncology
Cortlyn Brown, Megan Rowlands, Daniel Lee, Joel A Geffin, John Huang
We report the first case of Boston Keratoprosthesis (KPro) implantation in a 7-year-old girl with keratitis-ichthyosiform-deafness syndrome and persistent, highly vascular corneal surface disease. An adult aphakic KPro with 8.5 mm backplate was implanted successfully and without operative or postoperative complications following 3 failed penetrating keratoplasties. Visual acuity improved from hand motions to 20/70 in the left eye 22 months after KPro surgery and from hand motion to hand motion with direction in the right eye...
February 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
Anu Sharma, Hannah Glick, Julia Campbell, Jennifer Torres, Michael Dorman, Daniel M Zeitler
HYPOTHESIS: The purpose of this study was to examine changes in cortical development and neuroplasticity in a child with single-sided deafness (SSD) before and after cochlear implantation (CI). BACKGROUND: The extent to which sensory pathways reorganize in childhood SSD is not well understood and there is currently little evidence demonstrating the efficacy of CI in children with SSD. METHODS: High-density 128-channel electroencephalography (EEG) was used to collect cortical auditory evoked potentials (CAEP), cortical visual evoked potentials (CVEP), and cortical somatosensory evoked potentials (CSSEP) in a child with SSD, pre-CI and at subsequent sessions until approximately 3 years post-CI in her right ear which occurred at age 9...
February 2016: Otology & Neurotology
David R Friedmann, Omar H Ahmed, Sean O McMenomey, William H Shapiro, Susan B Waltzman, J Thomas Roland
OBJECTIVES: Although there are various available treatment options for unilateral severe-to-profound hearing loss, these options do not provide the benefits of binaural hearing since sound is directed from the poorer ear to the better ear. The purpose of this investigation was to review our center's experience with cochlear implantation in such patients in providing improved auditory benefits and useful binaural hearing. STUDY DESIGN: Retrospective chart review...
February 2016: Otology & Neurotology
Maiko Miyagawa, Shin-Ya Nishio, Shin-ichi Usami
OBJECTIVE: Cochlear implantation is the most important treatment currently available for profound sensorineural hearing loss. The aim of this study was to investigate the etiology of hearing loss in patients with cochlear implantation, and to compare outcomes. METHODS: Japanese hearing loss patients who received cochlear implants (CIs) or electric acoustic stimulation (EAS) in Shinshu University hospital (n = 173, prelingual onset: 92, postlingual onset: 81) participated in this study...
February 2016: Otology & Neurotology
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