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https://www.readbyqxmd.com/read/29776323/low-frequency-sensorineural-hearing-loss-in-familial-hemophagocytic-lymphohistiocytosis-type-5
#1
Aren Bezdjian, Hanneke Bruijnzeel, Julia Pagel, Sam J Daniel, Hans G X M Thomeer
INTRODUCTION: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. CASE PRESENTATION: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature...
June 2018: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29770223/profound-hearing-loss-following-surgery-in-pediatric-patients-with-posterior-fossa-low-grade-glioma
#2
Yahya Ghazwani, Ibrahim Qaddoumi, Johnnie K Bass, Shengjie Wu, Jason Chiang, Frederick Boop, Amar Gajjar, Zsila Sadighi
Background: Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods: We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology...
May 2018: Neuro-oncology Practice
https://www.readbyqxmd.com/read/29761140/hearing-impairment-among-children-referred-to-a-public-audiology-clinic-in-gaborone-botswana
#3
Francis M Banda, Kathleen M Powis, Agnes B Mokoka, Moalosi Mmapetla, Katherine D Westmoreland, Thuso David, Andrew P Steenhoff
Objective . To describe and quantify hearing impairment among children referred to the audiology clinic in Princess Marina Hospital, a public referral hospital in Botswana. Methods . In a retrospective case series, we reviewed medical records of children aged 10 years and younger whose hearing was assessed between January 2006 and December 2015 at the audiology clinic of Princess Marina Hospital in Gaborone, Botswana. Results . Of 622 children, 50% were male, and median age was 6.7 years (interquartile range = 5...
2018: Global Pediatric Health
https://www.readbyqxmd.com/read/29761033/diagnosis-and-treatment-of-congenital-sensorineural-hearing-loss
#4
Divya A Chari, Dylan K Chan
Purpose of Review: The aim of this report is to review current literature regarding the work-up and management of congenital sensorineural hearing loss. Recent Findings: Diagnostic evaluation of a newborn with sensorineural hearing loss begins with a complete audiologic evaluation and comprehensive history and physical exam. This review presents a diagnostic algorithm for the work-up of congenital hearing loss, focusing on the three following modalities: cytomegalovirus testing, genetic evaluation, and imaging...
December 2017: Current Otorhinolaryngology Reports
https://www.readbyqxmd.com/read/29753496/an-evidence-based-protocol-for-managing-neonatal-middle-ear-effusions-in-babies-who-fail-newborn-hearing-screening
#5
Brittany C Weber, Scott M Whitlock, Kaidi He, Blake S Kimbrell, Craig S Derkay
OBJECTIVES: To evaluate the prevalence of middle ear disease in infants referred for failed newborn hearing screening (NBHS) and to review patient outcomes after intervention in order to propose an evidence-based protocol for management of newborns with otitis media with effusion (OME) who fail NBHS. METHODS: 85 infants with suspected middle ear pathology were retrospectively reviewed after referral for failed NBHS. All subjects underwent a diagnostic microscopic exam with myringotomy with or without placement of a ventilation tube in the presence of a middle ear effusion and had intra-operative auditory brainstem response (ABR) testing or testing at a later date...
April 12, 2018: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/29748705/hearing-loss-in-phace-syndrome-clinical-and-radiologic-findings
#6
Mark D Mamlouk, Bree Zimmerman, Erin F Mathes, Kristina W Rosbe
PURPOSE: To characterize the types of hearing loss, auditory-related imaging findings, and hemangioma characteristics in patients with Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and abnormalities of the Eye (PHACE) syndrome. METHODS: Retrospective medical records, audiologic data, and imaging review of all patients presenting to a tertiary care children's hospital with a proven diagnosis of PHACE syndrome from 2005 to 2016. RESULTS: Twelve patients were identified with hearing and imaging data...
May 10, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29742543/psychometric-properties-and-factor-structure-of-a-new-scale-to-measure-hyperacusis-introducing-the-inventory-of-hyperacusis-symptoms
#7
Benjamin Greenberg, Megan Carlos
OBJECTIVES: Despite increasing interest in hyperacusis and other disorders of auditory sensitivity, there is still a lack of valid, standardized assessment tools to measure symptom severity, treatment outcomes, and diagnostic differentiation. Accordingly, this study sought to create a new scale that is reliable, valid, brief, and easy to score with the purpose of filling this gap. DESIGN: Original items were constructed through review of currently existing models of hyperacusis measurement, as well as qualitative data collected from professional audiologists and individuals reporting heightened audiological sensitivity with tinnitus...
May 8, 2018: Ear and Hearing
https://www.readbyqxmd.com/read/29741433/familial-nonsyndromic-hearing-loss-with-incomplete-partition-type-ii-caused-by-novel-dspp-gene-mutations
#8
Wan-Xin Li, Hong Peng, Le Yang, Qing-Qing Hao, Wei Sun, Fei Ji, Wei-Wei Guo, Shi-Ming Yang
BACKGROUND: Familial nonsyndromic hearing loss (NSHL) with incomplete partition type II (IP-II) is a very rare condition. AIMS/OBJECTIVES: To determine the audiological feature, inheritance patterns and genetic etiology of familial NSHL with IP-II in a Chinese family with eight family members. MATERIAL AND METHODS: Clinical data were collected from all eight family members, selected deafness genes were sequenced in proband and whole genome sequencing of seven family members was performed...
May 9, 2018: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/29739340/mutation-analysis-of-slc26a4-pendrin-gene-in-a-brazilian-sample-of-hearing-impaired-subjects
#9
Renata Watanabe Nonose, Karina Lezirovitz, Maria Teresa Balester de Mello Auricchio, Ana Carla Batissoco, Guilherme Lopes Yamamoto, Regina Célia Mingroni-Netto
BACKGROUND: Mutations in the SLC26A4 gene are associated with Pendred syndrome and autosomal recessive non-syndromic deafness (DFNB4). Both disorders have similar audiologic characteristics: bilateral hearing loss, often severe or profound, which may be associated with abnormalities of the inner ear, such as dilatation of the vestibular aqueduct or Mondini dysplasia. But, in Pendred syndrome (OMIM #274600), with autosomal recessive inheritance, besides congenital sensorineural deafness, goiter or thyroid dysfunctions are frequently present...
May 8, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29734694/hearing-loss-in-adult-survivors-of-childhood-cancer-treated-with-radiotherapy
#10
Amber Khan, Amy Budnick, Dana Barnea, Darren R Feldman, Kevin C Oeffinger, Emily S Tonorezos
The ototoxic effects of radiotherapy have been poorly characterized. We examined adult survivors of childhood cancer who were treated with radiotherapy, which included the head, before the age of 22 years and between 1952 and 2016. Those who received platinum chemotherapy were excluded. Demographic, diagnosis, and treatment outcomes were captured. Audiograms were graded using the Chang and International Society of Paediatric Oncology ototoxicity (SIOP) scales. Among 276 patients with a history of radiation to sites that included the brain, orbit, nasopharynx, and total body irradiation, the median age at treatment was 10...
May 4, 2018: Children
https://www.readbyqxmd.com/read/29734179/the-effectiveness-of-parental-questionnaires-in-the-assessment-of-speech-language-and-auditory-function-in-children
#11
Renee Fabus, Abbey L Berg, Yula C Serpanos, Mary Jo Cooley Hidecker
OBJECTIVE: This study investigates the use of a parental questionnaire to determine its effectiveness as a screening tool in identifying speech-language and/or auditory impairments in children. PATIENTS AND METHODS: Parents completed speech-language (n = 36) or audiology (n = 23) questionnaires prior to their child's speech-language and/or auditory evaluations. The speech-language and audiology evaluations were conducted by clinicians at three different universities not familiar with the administration and scoring of the parental questionnaires...
May 7, 2018: Folia Phoniatrica et Logopaedica
https://www.readbyqxmd.com/read/29729903/restoration-of-spatial-hearing-in-adult-cochlear-implant-users-with-single-sided-deafness
#12
Ruth Y Litovsky, Keng Moua, Shelly Godar, Alan Kan, Sara M Misurelli, Daniel J Lee
In recent years, cochlear implants (CIs) have been provided in growing numbers to people with not only bilateral deafness but also to people with unilateral hearing loss, at times in order to alleviate tinnitus. This study presents audiological data from 15 adult participants (ages 48 ± 12 years) with single sided deafness. Results are presented from 9/15 adults, who received a CI (SSD-CI) in the deaf ear and were tested in Acoustic or Acoustic + CI hearing modes, and 6/15 adults who are planning to receive a CI, and were tested in the unilateral condition only...
April 14, 2018: Hearing Research
https://www.readbyqxmd.com/read/29728184/an-audiological-evaluation-of-syndromic-and-non-syndromic-craniosynostosis-in-pre-school-going-children
#13
Liang Chye Goh, Ali Azman, Hufaidah Binti Konting Siti, Wee Vien Khoo, Premala A/P Muthukumarasamy, Meow Keong Thong, Zulkiflee Abu Bakar, Anura Michelle Manuel
OBJECTIVE: To study the audiological outcome and early screening of pre-school going children with craniosynostosis under follow-up at the University of Malaya Medical Center(UMMC), Kuala Lumpur, Malaysia over a 10 year period. METHODS: A retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination...
June 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29726623/contemporary-analysis-of-practicing-otolaryngologists
#14
Willard C Harrill, David E Melon, Merritt J Seshul, Marc S Katz, Adam M Zanation
OBJECTIVES/HYPOTHESIS: To investigate contemporary issues facing practicing otolaryngologists including workforce dynamics, ancillary service modeling, otolaryngic allergy integration, ambulatory surgery center utilization, and relevant certificate of need legislation. STUDY DESIGN: A cross-sectional survey analysis of academic and private practicing otolaryngologists in North and South Carolina in 2016. METHODS: A cross-sectional survey was e-mailed to 510 practicing otolaryngologists in North and South Carolina...
May 4, 2018: Laryngoscope
https://www.readbyqxmd.com/read/29708490/audiology-assistants-results-of-a-multicenter-survey
#15
Roanne Karzon, Lisa Hunter, Wendy Steuerwald
BACKGROUND: Although audiologists have been using support personnel for over 45 yr, controversy and variability continue with respect to the entry-level education, training methods, and scope of practice. PURPOSE: As part of a larger clinical practices survey, this report focuses on use of audiology assistants (AAs) for pediatric settings and "life-span" facilities that had a significant population of pediatric patients. RESEARCH DESIGN: A questionnaire was sent to 116 facilities in geographically diverse locations...
May 2018: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/29708487/referral-and-diagnosis-of-developmental-auditory-processing-disorder-in-a-large-united-states-hospital-based-audiology-service
#16
David R Moore, Stephanie L Sieswerda, Maureen M Grainger, Alexandra Bowling, Nicholette Smith, Audrey Perdew, Susan Eichert, Sandra Alston, Lisa W Hilbert, Lynn Summers, Li Lin, Lisa L Hunter
BACKGROUND: Children referred to audiology services with otherwise unexplained academic, listening, attention, language, or other difficulties are often found to be audiometrically normal. Some of these children receive further evaluation for auditory processing disorder (APD), a controversial construct that assumes neural processing problems within the central auditory nervous system. This study focuses on the evaluation of APD and how it relates to diagnosis in one large pediatric audiology facility...
May 2018: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/29708064/time-of-day-and-hearing-aid-adoption
#17
Gurjit Singh, Stefan Launer
To date, there is little understanding of how contextual factors may influence the decisions individuals make regarding the adoption of options for hearing rehabilitation. This explorative retrospective study investigated whether hearing aid adoption and return rates are associated with the time of the day at which an appointment takes place. The study sample consisted of 24,842 patients experiencing their first audiology appointment. It was observed that hearing aid adoption was significantly associated with appointment times whereby lower hearing aid adoption rates were observed at noon and 4 p...
January 2018: Trends in Hearing
https://www.readbyqxmd.com/read/29705727/does-screening-for-congenital-cytomegalovirus-at-birth-improve-longer-term-hearing-outcomes
#18
REVIEW
Cathie Hilditch, Bianca Liersch, Nicola Spurrier, Emily J Callander, Celia Cooper, Amy K Keir
Currently, the diagnosis of congenital cytomegalovirus (cCMV) infection in most highly resourced countries is based on clinical suspicion alone. This means only a small proportion of cCMV infections are diagnosed. Identification, through either universal or targeted screening of asymptomatic newborns with cCMV, who would previously have gone undiagnosed, would allow for potential early treatment with antiviral therapy, ongoing audiological surveillance and early intervention if sensorineural hearing loss (SNHL) is identified...
April 28, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29697646/-the-hearing-function-in-the-premature-children-following-their-treatment-with-the-use-of-ototoxic-antibiotics
#19
I N D'yakonova, I V Rakhmanova, Yu S Ishanova, D S Burmistrova
The objective of the present study was the evaluation of the state of the auditory function in the premature children during the first year of life who underwent the neonatal treatment with various ototoxic antibiotics. A total of 232 newborn infants were available for the examination by the methods designed for recording distortion product optoacoustic emission (DPOAE) and short-latency auditory evoked potentials (SAEPs). The 'Statgraphics Centurion XV' program was used for the statistical treatment of the data obtained in the study...
2018: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/29697645/-the-influence-of-central-auditory-processing-on-the-intelligibility-of-the-speech-in-the-patients-presenting-with-hearing-impairment
#20
M Yu Boboshko, I P Berdnikova, E S Garbaruk, M A Salakhbekov, N V Mal'tseva
The objective of the present study was to evaluate to what degree the function of the central auditory pathways influences the intelligibility of the speech in the patients suffering from the sensorineural loss of hearing (SNLH). The study included a total of 20 patients at the age varying from 31 to 80 years presenting with moderate to moderately severe symmetrical SNLN. All the patients were permanent hearing aid users. They underwent the standard audiological examination including pure-tone threshold and suprathreshold audimetries together with impedancemetry, the evaluation of the functional ability of the central auditory pathways with special reference to temporal resolution and differential frequency sensitivity, binaural integration, and the ability to distinguish a sound stimulus against the background noise...
2018: Vestnik Otorinolaringologii
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