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Pediatric hearing loss

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https://www.readbyqxmd.com/read/29675082/susac-s-syndrome-retinocochleocerebral-vasculopathy-follow-up-of-a-pediatric-patient
#1
Zeynep Selen Karalok, Birce Dilge Taskin, Alev Guven, Cemile Atilgan Ucgul, Omer Faruk Aydin
Susac's syndrome (SS) is a triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss as a result of microvascular occlusions of the brain, retina, and inner ear. It is also a disorder of autoimmune endotheliopathy. SS usually affects young women between the age of 20 and 40 years. SS can be misdiagnosed as multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM) because of similar findings. A 15-year-old girl presented in June 2015 with vomiting and severe headache...
October 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29664867/short-and-long-term-outcomes-of-titanium-clip-ossiculoplasty
#2
Charissa N Kahue, Brendan P O'Connnell, Matthew M Dedmon, David S Haynes, Alejandro Rivas
OBJECTIVE: To report short (∼4 mo) and long-term (>12 mo) audiometric outcomes following ossiculoplasty using a titanium clip partial ossicular reconstruction prosthesis. METHODS: Case series at a single tertiary referral center reviewing 130 pediatric and adult patients with conductive hearing loss (CHL) secondary to chronic otitis media (n = 121, 93%) or traumatic ossicular disruption (n = 9, 7%) who underwent partial ossiculoplasty from January 2005 to December 2015 with the CliP prosthesis...
April 16, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29664721/effect-of-adaptive-compression-and-fast-acting-wdrc-strategies-on-sentence-recognition-in-noise-in-mandarin-speaking-pediatric-hearing-aid-users
#3
Haihong Liu, Yuanhu Liu, Ying Li, Xin Jin, Jing Li, Yi Zhou, Wentong Ge, Xin Ni
BACKGROUND: Wide dynamic range compression (WDRC) has been widely used in hearing aid technology. However, several reports indicate that WDRC may improve audibility at the expense of speech intelligibility. As such, a modified amplification compression scheme, named adaptive compression, was developed. However, the effect of compression strategies on speech perception in pediatric hearing aid users has not been clearly reported. PURPOSE: The purpose of the present study was to investigate the effect of adaptive compression and fast-acting WDRC processing strategies on sentence recognition in noise with Mandarin, pediatric hearing aid users...
April 2018: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/29627839/genotype-phenotype-analysis-in-pediatric-patients-with-distal-renal-tubular-acidosis
#4
Eujin Park, Myung Hyun Cho, Hye Sun Hyun, Jae Il Shin, Joo Hoon Lee, Young Seo Park, Hyun Jin Choi, Hee Gyung Kang, Hae Il Cheong
BACKGROUND/AIMS: Primary distal renal tubular acidosis (dRTA) in children is a rare genetic disorder, and three causative mutated genes have been identified: SLC4A1, ATP6V1B1, and ATP6V0A4. We analyzed the prevalence and phenotypic differences of genetic mutations in children with dRTA. METHODS: A total of 17 children with dRTA were enrolled in the study. All patients underwent genetic testing for all three candidate genes. RESULTS: Pathogenic mutations, including six novel mutations, were detected in 15 (88...
March 29, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29605369/frequency-specific-auditory-brainstem-response-testing-with-age-appropriate-sedation
#5
Yael Levit, Dror Mandel, Idit Matot
OBJECTIVE: Auditory brainstem response (ABR) testing is the gold-standard procedure for hearing evaluation in pediatric patients who cannot complete a behavioral hearing test. The amount of audiological information obtained depends on the quality of the patient's sleep during the test. In this retrospective database review, we aimed to assess the amount and the characteristics of the audiological information obtained in ABR testing in pediatric patients with age-appropriate sedation. METHODS: A retrospective chart review was conducted on 501 consecutive ABR sedation sessions performed between January 2014 and June 2016 at the Tel Aviv Medical Center...
May 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29605351/evaluation-of-the-outcome-of-ct-and-mr-imaging-in-pediatric-patients-with-bilateral-sensorineural-hearing-loss
#6
E A van Beeck Calkoen, P Merkus, S T Goverts, J M van de Kamp, M F Mulder, E Sanchez Aliaga, E F Hensen
OBJECTIVE: To evaluate the clinically relevant abnormalities as visualized on CT and MR imaging in children with symmetric and asymmetric bilateral sensorineural hearing loss (SNHL), in relation to age and the severity of hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral otology and audiology center. PATIENTS AND DIAGNOSTIC INTERVENTIONS: From January 2006 until January 2016, a total of 207 children diagnosed with symmetric and asymmetric bilateral SNHL were included...
May 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29573766/-quality-of-life-of-children-with-otitis-media-and-impact-of-insertion-of-transtympanic-ventilation-tubes-in-a-portuguese-population
#7
Ana Rita Lameiras, Deodato Silva, Assunção O Neill, Pedro Escada
INTRODUCTION: Quality of life is an important measure for health-outcome evaluation. Although otitis media is one of the most common childhood diseases, its impact on Portuguese children's quality of life is unknown. The aim of this study is to determine the quality of life of Portuguese children with chronic otitis media with effusion and/or recurrent acute otitis media and the short-term impact of transtympanic ventilation tubes, using the Portuguese version of the OM-6 questionnaire, a valid, reliable and sensitive instrument to evaluate the health-related quality of life in children with otitis media...
January 31, 2018: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/29571077/neonates-with-congenital-cytomegalovirus-and-hearing-loss-identified-via-the-universal-newborn-hearing-screening-program
#8
William D Rawlinson, Pamela Palasanthiran, Beverly Hall, Laila Al Yazidi, Michael J Cannon, Carolyn Cottier, Wendy J van Zuylen, Monica Wilkinson
BACKGROUND: Congenital cytomegalovirus (CMV) is the most common non-genetic cause of sensorineural hearing loss. Currently, there are no universal CMV screening programs for newborns or routine CMV testing of neonates with hearing loss in Australia, or elsewhere. OBJECTIVES: This study was undertaken to determine the prevalence of congenital CMV infection in infants with hearing loss identified using routine resources via the Australian universal neonatal hearing screening (UNHS) program...
May 2018: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/29525391/an-evidence-based-practical-approach-to-pediatric-otolaryngology-in-the-developing-world
#9
REVIEW
Ryan H Belcher, David W Molter, Steven L Goudy
Despite humanitarian otolaryngology groups traveling in record numbers to resource-limited areas treating pediatric otolaryngology disease processes and training local providers, there remains a large burden of unmet needs. There is a meager amount of published information that comes from the developing world from an otolaryngology standpoint. As would be expected, the little information that does comes involves some of the most common pediatric otolaryngology diseases and surgical burdens including childhood hearing loss, otitis media, adenotonsillectomies, airway obstructions requiring tracheostomies, foreign body aspirations, and craniomaxillofacial surgeries, including cleft lip and palate...
March 7, 2018: Otolaryngologic Clinics of North America
https://www.readbyqxmd.com/read/29516058/use-of-porcine-small-intestinal-submucosa-for-pediatric-endoscopic-tympanic-membrane-repair
#10
REVIEW
Razan A Basonbul, Michael S Cohen
Tympanic membrane perforationsoccur frequently in children, and can result in hearing loss, otorrhea, pain, and cholesteatoma. Due to the narrower ear canal in children, a postauricular incision is often needed to access the tympanic membrane for surgical repair. Endoscopic approaches are increasingly being used for tympanic membrane repair, reducing the need for postauricular incisions. As the need for a postauricular incision decreases, the demand for non-autologous grafting material has increased. Acellular porcine small intestinal submucosa (SIS) has been described in the literature as an alternative to commonly used autologous grafts, and is well suited for use with transcanal endoscopic ear surgery as a minimally invasive approach...
September 2017: World journal of otorhinolaryngology—head and neck surgery
https://www.readbyqxmd.com/read/29501316/long-term-platinum-induced-ototoxicity-in-pediatric-patients
#11
Sofia Waissbluth, Angela Chuang, Álvaro Del Valle, Marcela Cordova
OBJECTIVES: Platinum-based chemotherapy treatments are effective against a variety of pediatric malignancies. However, its use can lead to permanent hearing loss. The aim of this study was to evaluate the long-term effect of platinum chemotherapy on hearing and evaluate its progression. METHODS: Prospective cohort study. All records of pediatric patients receiving platinum-based chemotherapy between 2001 and 2006 were reviewed. Demographics and audiograms performed before, during, and following chemotherapy were analyzed...
April 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29472286/a-novel-de-novo-capn5-mutation-in-a-patient-with-inflammatory-vitreoretinopathy-hearing-loss-and-developmental-delay
#12
Gabriel Velez, Alexander G Bassuk, Kellie A Schaefer, Brian Brooks, Lokesh Gakhar, MaryAnn Mahajan, Philip Kahn, Stephen H Tsang, Polly J Ferguson, Vinit B Mahajan
Mutations that activate the protease calpain-5 (CAPN5) cause a non-syndromic adult-onset autoinflammatory eye disease characterized by uveitis, altered synaptic signaling, retinal degeneration, neovascularization, and intraocular fibrosis. We describe a pediatric patient with severe inflammatory vitreoretinopathy accompanied by hearing loss and developmental delay associated with a novel, de novo CAPN5 missense mutation (c.865C>T, p.Arg289Trp) that shows greater hyperactivation of the calpain protease, indicating a genotype-phenotype correlation that links mutation severity to proteolytic activity and the possibility of earlier onset syndromic disease with auditory and neurological abnormalities...
February 22, 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29447896/bony-cochlear-nerve-canal-stenosis-in-pediatric-unilateral-sensorineural-hearing-loss
#13
Chae-Hyun Lim, Ji Hyung Lim, Doyoun Kim, Ho Sung Choi, Dong-Hee Lee, Dong-Kee Kim
OBJECTIVES: This study was performed to evaluate the frequency of bony cochlear nerve canal (BCNC) stenosis and its clinical significance in pediatric patients with unilateral sensorineural hearing loss (SNHL) of unknown etiology. MATERIALS AND METHODS: We analyzed the medical records and temporal bone computed tomography (CT) results of patients less than 13 years of age with a diagnosis of unilateral SNHL of unknown etiology between July 2007 and July 2017. We compared the BCNC diameter between both sides and analyzed the age at diagnosis, degree of hearing loss, and accompanying inner ear anomalies...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29447894/cochlear-implantation-in-pediatric-patients-with-cockayne-syndrome
#14
Renae D Van Wyhe, Claudia V Emery, Robert A Williamson
Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0-12% previously)...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29447794/plasticity-after-pediatric-cochlear-implantation-implication-from-changes-in-peripheral-plasma-level-of-bdnf-and-auditory-nerve-responses
#15
Razieh Alemi, Masoud Motassadi Zarandy, Mohammad Taghi Joghataei, Ali Eftekharian, Mohammad Reza Zarrindast, Nasim Vousooghi
INTRODUCTION: Sensory neural hearing loss could lead to some structural and physiological changes in the auditory pathways, such as alteration in the expression of neurotrophins. These factors, especially Brain-Derived Neurotrophic Factor (BDNF), play an important role in synaptic functions and experience-related plasticity. Restoring cochlear function after hearing loss is possible through cochlear implantation (CI). Evaluation of the blood concentration changes of neurotrophins as prerequisites of plasticity could help scientists to determine the prognosis of CI as in the candidacy procedure or enhancing prosthesis function by adding the exact needed amount of BDNF to the electrode array...
February 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29392049/sensorineural-hearing-loss-in-selective-immunglobulin-a-deficiency
#16
Erkan Eşki, Belgin Emine Usta, Suna Asilsoy, İsmail Yılmaz
Objective: To assess hearing functions in pediatric patients with selective immunoglobulin A (IgA) deficiency (SIGAD). Methods: Pure-tone audiometry, acoustic impedance, otoacoustic emission, and brainstem audiometric measurements were taken during a non-infectious period in 28 patients with SIGAD and 28 healthy children with normal otoscopic examination. The results of the hearing tests were compared between the two groups. Results: Two male patients and one female patient in the SIGAD group were found to have sensorineural hearing loss (SNHL)...
March 2017: Turkish archives of 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
#17
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/29380538/determining-the-prevalence-of-vestibular-screening-failures-in-pediatric-cancer-patients-whose-therapies-include-radiation-to-the-head-neck-and-platin-based-therapies-a-pilot-study
#18
Miranda L Camet, Susan S Hayashi, Belinda C Sinks, Jennifer Henry, Katie Gettinger, Ashley Hite, Juliann Kiefer, Caroline Mohrmann, Taryn Sandheinrich, Feng Gao, Robert J Hayashi
BACKGROUND: Sensorineural hearing loss due to ototoxic cancer therapy is well established; effects on the vestibular system are unknown. We examined the feasibility of implementing vestibular screens for pediatric cancer survivors exposed to ototoxic agents. The prevalence of screening failures is reported. METHODS: Cancer survivors who were 6-17 years, at least 1-month posttreatment, and received ototoxic therapy (radiation to the head/neck, cisplatin, carboplatin) were eligible...
January 30, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29366866/international-consensus-icon-on-audiological-assessment-of-hearing-loss-in-children
#19
A Farinetti, A Raji, H Wu, B Wanna, C Vincent
The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Early diagnosis and rehabilitation of congenital hearing loss are mandatory in order to achieve a satisfactory linguistic and cognitive development. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development. After promising results, neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out...
February 2018: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/29335387/clear-cell-carcinoma-of-the-abdominal-wall
#20
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
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