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Auditory neuropathy spectrum disorder

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https://www.readbyqxmd.com/read/28357181/prevalence-of-vestibular-symptoms-in-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#1
Prashanth Prabhu, Pratyasha Jamuar
The objective of the study was to retrospectively determine the prevalence of vestibular symptoms in individuals with auditory neuropathy spectrum disorder (ANSD). It was also attempted to determine the prevalence of vestibular symptoms and factors (gender and age of reported hearing loss) that could affect the prevalence in individuals with ANSD. The vestibular symptoms reported in the case history were analyzed in individuals diagnosed with ANSD. The symptoms reported by a total of 316 individuals (185 females and 131 males) with ANSD were analyzed...
February 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28335750/novel-compound-heterozygous-mutations-in-the-otof-gene-identified-by-whole-exome-sequencing-in-auditory-neuropathy-spectrum-disorder
#2
Fengzhu Tang, Dengke Ma, Yulan Wang, Yuecai Qiu, Fei Liu, Qingqing Wang, Qiutian Lu, Min Shi, Liang Xu, Min Liu, Jianping Liang
BACKGROUND: Many hearing-loss diseases are demonstrated to have Mendelian inheritance caused by mutations in single gene. However, many deaf individuals have diseases that remain genetically unexplained. Auditory neuropathy is a sensorineural deafness in which sounds are able to be transferred into the inner ear normally but the transmission of the signals from inner ear to auditory nerve and brain is injured, also known as auditory neuropathy spectrum disorder (ANSD). The pathogenic mutations of the genes responsible for the Chinese ANSD population remain poorly understood...
March 23, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28209736/pejvakin-a-candidate-stereociliary-rootlet-protein-regulates-hair-cell-function-in-a-cell-autonomous-manner
#3
Marcin Kazmierczak, Piotr Kazmierczak, Anthony W Peng, Suzan L Harris, Prahar Shah, Jean-Luc Puel, Marc Lenoir, Santos J Franco, Martin Schwander
Mutations in the Pejvakin (PJVK) gene are thought to cause auditory neuropathy and hearing loss of cochlear origin by affecting noise-induced peroxisome proliferation in auditory hair cells and neurons. Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing loss and outer hair cell degeneration in mice. Pejvakin binds to and colocalizes with the rootlet component TRIOBP at the base of stereocilia in injectoporated hair cells, a pattern that is disrupted by deafness-associated PJVK mutations...
March 29, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28089576/conditional-deletion-of-pejvakin-in-adult-outer-hair-cells-causes-progressive-hearing-loss-in-mice
#4
Suzan L Harris, Marcin Kazmierczak, Tina Pangršič, Prahar Shah, Nadiya Chuchvara, Alonso Barrantes-Freer, Tobias Moser, Martin Schwander
Mutations in the Pejvakin (Pjvk) gene cause autosomal recessive hearing loss DFNB59 with audiological features of auditory neuropathy spectrum disorder (ANSD) or cochlear dysfunction. The precise mechanisms underlying the variable clinical phenotypes of DFNB59 remain unclear. Here, we demonstrate that mice with conditional ablation of the Pjvk gene in all sensory hair cells or only in outer hair cells (OHCs) show similar auditory phenotypes with early-onset profound hearing loss. By contrast, loss of Pjvk in adult OHCs causes a slowly progressive hearing loss associated with OHC degeneration and delayed loss of inner hair cells (IHCs), indicating a primary role for pejvakin in regulating OHC function and survival...
March 6, 2017: Neuroscience
https://www.readbyqxmd.com/read/27942602/audiological-profile-of-adult-persons-with-auditory-neuropathy-spectrum-disorders
#5
Pradeep Yuvaraj, Mannarukrishnaiah Jayaram
BACKGROUND AND OBJECTIVES: The auditory profile of a large number of persons with late onset auditory neuropathy spectrum disorder (ANSD) is recently described in the Indian context. The purpose of study was 1) to profile data on routine audiological parameters, cortical evoked potentials, and temporal processing, 2) to analyze the benefit from hearing aids for persons with ANSD, and 3) to understand the association between benefit from hearing aids and auditory profile. SUBJECTS AND METHODS: Thirty-eight adults with late onset ANSD and a matched group of 40 normally hearing adults participated in the study...
December 2016: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/27885979/gender-differences-in-audiological-findings-and-hearing-aid-benefit-in-255-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#6
Vijaya Kumar Narne, Prashanth Prabhu, Hunsur S Chandan, Mahadeva Deepthi
BACKGROUND: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. PURPOSE: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27843504/relationship-between-patients-with-clinical-auditory-neuropathy-spectrum-disorder-and-mutations-in-gjb2-gene
#7
Guilherme M de Carvalho, Priscila Z Ramos, Arthur M Castilho, Alexandre C Guimarães, Edi L Sartorato
The auditory neuropathy is a condition which there is a dyssynchrony in the nerve conduction of the auditory nerve fibers. There is no evidence about the relationship between patients with clinical auditory neuropathy spectrum disorder and mutations in GJB2 gene. There are only two studies about this topic in the medical literature. Connexin 26 (GJB2 gene) mutations are common causes of genetic deafness in many populations and we also being reported in subjects with auditory neuropathy. OBJECTIVE: To analyze the pattern of clinical relationship between patients with clinical diagnosis with auditory neuropathy spectrum disorder and GJB2 gene...
2016: Open Neurology Journal
https://www.readbyqxmd.com/read/27755139/performance-intensity-function-and-aided-improvement-in-individuals-with-late-onset-auditory-neuropathy-spectrum-disorder
#8
Jijo Pottackal Mathai, Asha Yathiraj
OBJECTIVES: The purpose of the study was to explore the effect of intensity on speech perception in individuals with late-onset auditory neuropathy spectrum disorder (ANSD) by obtaining their performance-intensity (PI) function. Additionally, the study investigated the effect of PI function on aided speech perception. It was hypothesized that speech perception abilities of individuals with ANSD vary with intensity and may provide information regarding their aided performance. DESIGN: A factorial research design was used to obtain the PI function and aided performance...
October 13, 2016: Ear and Hearing
https://www.readbyqxmd.com/read/27688594/the-p1-biomarker-for-assessing-cortical-maturation-in-pediatric-hearing-loss-a-review
#9
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
https://www.readbyqxmd.com/read/27587922/detection-rates-of-cortical-auditory-evoked-potentials-at-different-sensation-levels-in-infants-with-sensory-neural-hearing-loss-and-auditory-neuropathy-spectrum-disorder
#10
Kirsty Gardner-Berry, Hsiuwen Chang, Teresa Y C Ching, Sanna Hou
With the introduction of newborn hearing screening, infants are being diagnosed with hearing loss during the first few months of life. For infants with a sensory/neural hearing loss (SNHL), the audiogram can be estimated objectively using auditory brainstem response (ABR) testing and hearing aids prescribed accordingly. However, for infants with auditory neuropathy spectrum disorder (ANSD) due to the abnormal/absent ABR waveforms, alternative measures of auditory function are needed to assess the need for amplification and evaluate whether aided benefit has been achieved...
February 2016: Seminars in Hearing
https://www.readbyqxmd.com/read/27579218/evaluation-of-depression-anxiety-and-stress-in-adolescents-and-young-adults-with-auditory-neuropathy-spectrum-disorder
#11
Prashanth Prabhu
The aim of the present study was to determine the severity of stress, anxiety, and depression using Depression Anxiety Stress Scales (DASS) in adolescents and young adults with auditory neuropathy spectrum disorder (ANSD). DASS was administered to 20 individuals with auditory neuropathy spectrum disorder. The effect of gender on severity of anxiety, stress, and depression on DASS scores was determined. It was attempted to determine the correlation of severity of anxiety, stress, and depression with the reported onset of the problem, degree of hearing loss, and speech identification scores...
2016: Scientifica
https://www.readbyqxmd.com/read/27340976/performance-of-cochlear-implants-in-pediatric-patients-with-auditory-neuropathy-spectrum-disorder
#12
REVIEW
Guilherme Machado de Carvalho, Priscila Ramos, Castilho Arthur, Alexandre Guimarães, Edi Sartorato
OBJECTIVE: To describe the performance and results of CIs (cochlear implant) in patients with AN (auditory neuropathy) and to present a medical literature review. MATERIALS AND METHODS: Retrospective chart review of patients with AN who were treated with CI. The mesh terms used for the review in the Pubmed and Scopus databases were as follows: "hearing loss, cochlear implants, rehabilitation of persons with hearing impairment, auditory neuropathy". STATISTICAL ANALYSES: The Mann-Whitney test was performed...
April 2016: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/27340636/prevalence-and-audiological-characteristics-of-auditory-neuropathy-spectrum-disorder-in-pediatric-population-a-retrospective-study
#13
S S Vignesh, V Jaya, A Muraleedharan
Auditory neuropathy spectrum disorder (ANSD) is a type of hearing disorder which is challenging for assessment and rehabilitation. This disorder has been studied over a decade and prevalence of the disorder is variable. The study aimed at estimating the prevalence and audiological characteristics of ANSD in children. A retrospective study was conducted from the medical records of pediatric patients evaluated at Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai to estimate the prevalence of ANSD...
June 2016: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/27266370/contemporary-surgical-issues-in-paediatric-cochlear-implantation
#14
Joseph D Wasson, Robert J S Briggs
OBJECTIVE: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. DESIGN: Narrative literature review. RESULTS: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure...
2016: International Journal of Audiology
https://www.readbyqxmd.com/read/27260582/diverse-etiologies-manifesting-auditory-neuropathy-characteristics-from-infants-with-profound-hearing-loss-and-clinical-implications
#15
Shin Hye Kim, Hyun Seok Choi, Young Eun Han, Byung Yoon Choi
OBJECTIVE: Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder with impaired signal transmission from the inner ear to the brain. The electrophysiological characteristics of auditory neuropathy (AN characteristics) are marked with presence of otoacoustic emissions (OAE) or cochlear microphonics (CM) in the absence of auditory brainstem response (ABR). This study aimed to review etiologies related to AN characteristics from infants with profound hearing loss (HL), either unilaterally or bilaterally...
July 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27235210/bilirubin-induced-audiologic-injury-in-preterm-infants
#16
REVIEW
Cristen Olds, John S Oghalai
Although hyperbilirubinemia is extremely common among neonates and is usually mild and transient, it sometimes leads to bilirubin-induced neurologic damage (BIND). The auditory pathway is highly sensitive to the effects of elevated total serum/plasma bilirubin (TB) levels, with damage manifesting clinically as auditory neuropathy spectrum disorder. Compared to full-term neonates, preterm neonates are more susceptible to BIND and suffer adverse effects at lower TB levels with worse long-term outcomes. Furthermore, although standardized guidelines for management of hyperbilirubinemia exist for term and late preterm neonates, similar guidelines for neonates less than 35 weeks gestational age are limited...
June 2016: Clinics in Perinatology
https://www.readbyqxmd.com/read/27220871/auditory-neuropathy-spectrum-disorder-ansd-in-referrals-from-neonatal-hearing-screening-at-a-well-baby-clinic
#17
A Boudewyns, Frank Declau, Jenneke van den Ende, Anouk Hofkens, Sara Dirckx, Paul Van de Heyning
UNLABELLED: Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing...
July 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27101692/-the-review-of-newborn-hearing-screening-program-in-neonatal-intensive-care-unit
#18
REVIEW
Beier Qi, Hui En, Lihui Huang
The incidence of hearing impairment in neonatal intensive care unit (NICU) was much higher than that of well-baby nursery. The incidence of the former was 2%-4%, whereas that of the latter was 0.1%-0.3%. Furthermore, the incidence of auditory neuropathy spectrum disorder, progressive and delayed hearing loss was also higher than those of other infants. Therefore, the newborn hearing screening program in NICU has become an important part of pediatric audiology. In this paper, we reviewed the previous studies and suggested the special procedure of hearing screening and following-up which based on the physiological and pathological characteristics of NICU in order to detect hearing impaired as early as possible...
December 2015: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/27099109/international-survey-of-cochlear-implant-candidacy
#19
D Vickers, L De Raeve, J Graham
BACKGROUND: The goal of this work was to determine international differences in candidacy based on audiometric and speech perception measures, and to evaluate the information in light of the funding structure and access to implants within different countries. METHOD: An online questionnaire was circulated to professionals in 25 countries. There were 28 respondents, representing the candidacy practice in 17 countries. RESULTS: Results showed differences in the funding model between countries...
April 2016: Cochlear Implants International
https://www.readbyqxmd.com/read/27097229/feasibility-of-a-recasting-and-auditory-bombardment-treatment-with-young-cochlear-implant-users
#20
Danielle Encinas, Elena Plante
PURPOSE: There is little to guide clinicians in terms of evidence-based interventions for children with cochlear implants who demonstrate morpheme errors. This feasibility study tested the utility of a treatment targeting grammatical morpheme errors. METHOD: Three children (ages 4-5 years) received Enhanced Conversational Recast treatment, a version of conversational recast treatment that focuses on a single morpheme error at a time, emphasizes attention to clinician input, and uses high linguistic variability with clinician input...
April 1, 2016: Language, Speech, and Hearing Services in Schools
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