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auditory neuropathy

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https://www.readbyqxmd.com/read/28708635/the-management-of-pediatric-hearing-loss-caused-by-auditory-neuropathy-spectrum-disorder
#1
Nguyen S Pham
PURPOSE OF REVIEW: Auditory neuropathy spectrum disorder (ANSD) is a condition in which auditory testing reveals normal otoacoustic emissions, but auditory brainstem testing is abnormal or absent and speech discrimination is poor. This constellation of findings ostensibly suggests that the cochlea is healthy and an abnormality of conduction or processing of sound occurs along the nerve fibers. As more is learned about this condition, it is becoming clear that ANSD describes heterogeneous, distinct clinical entities that must be taken into account when devising treatment modalities...
July 13, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28704898/a-case-of-auditory-neuropathy-caused-by-pontine-hemorrhage-in-an-adult
#2
Seung-Hyun Chung, Sung Wook Jeong, Lee-Suk Kim
A pontine hemorrhage can evoke several neurological symptoms because the pons contains various nuclei and nerve fibers. Hearing loss can develop as a result of a pontine hemorrhage because there is an auditory conduction pathway in the cochlear nucleus of the pons. However, very few cases of hearing loss caused by pontine lesions have been reported, and there have been no reports of auditory neuropathy that developed following a pontine hemorrhage. Recently we had a patient who experienced a nontraumatic pontine hemorrhage who was diagnosed with auditory neuropathy...
July 2017: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/28688553/clinical-role-of-electrocochleography-in-children-with-auditory-neuropathy-spectrum-disorder
#3
Tatyana E Fontenot, Christopher K Giardina, Holly F Teagle, Lisa R Park, Oliver F Adunka, Craig A Buchman, Kevin D Brown, Douglas C Fitzpatrick
OBJECTIVES: To assess electrocochleography (ECochG) to tones as an instrument to account for CI speech perception outcomes in children with auditory neuropathy spectrum disorder (ANSD). MATERIALS & METHODS: Children (<18 years) receiving CIs for ANSD (n = 30) and non-ANSD (n = 74) etiologies of hearing loss were evaluated with ECochG using tone bursts (0.25-4 kHz). The total response (TR) is the sum of spectral peaks of responses across frequencies. The compound action potential (CAP) and the auditory nerve neurophonic (ANN) in ECochG waveforms were used to estimate nerve activity and calculate nerve score...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28687782/human-cochlear-histopathology-reflects-clinical-signatures-of-primary-neural-degeneration
#4
Jessica E Sagers, Lukas D Landegger, Steven Worthington, Joseph B Nadol, Konstantina M Stankovic
Auditory neuropathy is a significant and understudied cause of human hearing loss, diagnosed in patients who demonstrate abnormal function of the cochlear nerve despite typical function of sensory cells. Because the human inner ear cannot be visualized during life, histopathological analysis of autopsy specimens is critical to understanding the cellular mechanisms underlying this pathology. Here we present statistical models of severe primary neuronal degeneration and its relationship to pure tone audiometric thresholds and word recognition scores in comparison to age-matched control patients, spanning every decade of life...
July 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28680492/effectiveness-of-low-cut-modified-amplification-using-receiver-in-the-canal-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#5
Prashanth Prabhu, Animesh Barman
Introduction  The studies on hearing aid benefit in individuals with auditory neuropathy spectrum disorder (ANSD) shows limited benefit. Low cut modified amplification is found to be effective in few individuals with ANSD. With advancement in technology, receiver in the canal (RIC) hearing aids have proven to be more effective than traditional behind the ear (BTE) hearing aids. Objective  Thus, the present study attempts to determine the effectiveness of low cut modified amplification using RIC and BTE...
July 2017: International Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/28601886/auna2-a-novel-type-of-non-syndromic-slowly-progressive-auditory-synaptopathy-auditory-neuropathy-with-autosomal-dominant-inheritance
#6
Ruth Lang-Roth, Eva Fischer-Krall, Cornelia Kornblum, Gudrun Nürnberg, Dieter Meschede, Ingrid Goebel, Peter Nürnberg, Dirk Beutner, Christian Kubisch, Martin Walger, Alexander E Volk
BACKGROUND: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative...
June 10, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28555600/audiological-findings-in-charcot-marie-tooth-disease-type-4c
#7
Rafael Sivera, Laura Cavalle, Juan J Vílchez, Carmen Espinós, Herminio Pérez Garrigues, Teresa Sevilla
OBJECTIVE: Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary demyelinating early onset neuropathy with prominent unsteadiness and occasional cranial nerve involvement. Vestibulopathy caused by the dysfunction of cranial nerve VIII has been demonstrated in a high percentage of these patients, but the presence and degree of auditory neuropathy are unknown. The aim of the study was to characterize the hearing abnormalities of a series of patients with CMT4C and to determine the presence and severity of auditory neuropathy (AN) in these patients...
April 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28555599/speech-perception-in-quiet-and-in-noise-condition-in-individuals-with-auditory-neuropathy-spectrum-disorder
#8
Kumari Apeksha, Ajith U Kumar
OBJECTIVE: The study investigated the effect of noise on syllable perception in individuals with Auditory Neuropathy Spectrum Disorder (ANSD) and compared that with the normal hearing individuals. MATERIALS AND METHODS: A total of 54 participants were considered, out of which 26 individuals were diagnosed with ANSD and 28 with normal hearing sensitivity. Syllable identification and discrimination were assessed in both the groups in quiet as well as +10 dB SNR. RESULTS: All the individuals with ANSD performed poorer on syllable identification and syllable discrimination tasks compared to individuals with normal hearing...
April 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28503992/dose-dependent-effects-of-ouabain-on-spiral-ganglion-neurons-and-schwann-cells-in-mouse-cochlea
#9
Zhi-Jian Zhang, Hong-Xia Guan, Kun Yang, Bo-Kui Xiao, Hua Liao, Yang Jiang, Tao Zhou, Qing-Quan Hua
OBJECTIVE: This study aimed in fully investigating the toxicities of ouabain to mouse cochlea and the related cellular environment, and providing an optimal animal model system for cell transplantation in the treatment of auditory neuropathy (AN) and sensorineural hearing loss (SNHL). METHODS: Different dosages of ouabain were applied to mouse round window. The auditory brainstem responses and distortion product otoacoustic emissions were used to evaluate the cochlear function...
May 14, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28503341/peripheral-and-central-nervous-system-involvement-in-recently-diagnosed-cases-of-hypothyroidism-an-electrophysiological-study
#10
N Gupta, M Arora, R Sharma, K S Arora
BACKGROUND: Hypothyroidism, one of the most common endocrine disorders, may induce neurological abnormalities at an early stage of the disease. AIM: The study was designed to assess the electrophysiological alterations of some selected variables of nerve conduction, brainstem auditory evoked potentials (BAEPs), and visual evoked potentials (VEPs) in hypothyroid patients. SUBJECTS AND METHODS: Sixty patients of newly diagnosed hypothyroidism and an equal number of age-matched controls were selected for the study...
September 2016: Annals of Medical and Health Sciences Research
https://www.readbyqxmd.com/read/28502273/modiolar-ossification-in-paediatric-patients-with-auditory-neuropathy
#11
L Wang, Z Wang, F Gao, K A Peng
OBJECTIVE: To describe our finding of increased ossification of the modiolus in paediatric patients with auditory neuropathy who met criteria for cochlear implantation. METHODS: A retrospective case series with a comparison group at a tertiary referral centre is described. Seven paediatric patients with auditory neuropathy who met criteria for and underwent cochlear implantation were identified. Fifteen paediatric implantees with bilateral profound sensorineural hearing loss were included as the comparison group...
July 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28485679/effectiveness-of-low-cut-modified-amplification-strategy-and-channel-free-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#12
Prashanth Prabhu, Animesh Barman
OBJECTIVE: The present study attempted to compare the aided benefit using low-cut modified amplification and channel-free hearing aids in individuals with auditory neuropathy spectrum disorder (ANSD). It was also attempted to determine these effects in good and poor performers with ANSD. DESIGN: Cross-sectional within group pretest, post-test design. STUDY SAMPLE: Twenty-five individuals with acquired ANSD were selected for the study. The study sample included 11 males and 14 females between the age ranges of 17-40 years (mean age of 24...
May 9, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28484666/hearing-and-balance-disorders-in-the-state-of-hawai-i-demographics-and-demand-for-services
#13
Mohsin Ahmed M Shaikh, James W Hall, Cindy McManus, Henry L Lew
Hearing and balance disorders affect people of all ages. Among children, hearing loss affects speech and language development, academic performance, and psychosocial development. Hearing loss in adults negatively impacts work productivity, cognitive function, and psychosocial status. Prevalence of hearing loss in children in Hawai'i is higher than the national average. Research indicates that hearing loss is a prevalent condition among veterans and advanced age adults. This is of particular concern in Hawai'i as the state is home to many military training facilities and has a large elderly population...
May 2017: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/28483258/universal-newborn-hearing-screening-in-southwestern-iran
#14
Nader Saki, Arash Bayat, Reza Hoseinabadi, Soheila Nikakhlagh, Majid Karimi, Rezvan Dashti
OBJECTIVES: The implementation of Neonatal Hearing Screening (NHS) program is still at the preliminary stage particularly in developing countries despite the burden of permanent congenital and early-onset hearing impairment. The purpose of this study was to report results for universal newborn NHS in a cohort of children born in the southwestern region of Iran, as part of a national screening program set up by the Iranian National Health System. METHODS: During this cross-sectional study, which took place between March 2013 and April 2016, healthy newborns were screened using transient evoked otoacoustic emissions (TEOAEs) and automated auditory brainstem responses (AABRs) methods at several points in time as early as possible after birth...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28473856/recovery-of-abnormal-abr-in-neonates-and-infants-at-risk-of-hearing-loss
#15
Ioannis Psarommatis, Charalampos Voudouris, Ioannis Kapetanakis, Faselida Athanasiadi, Konstantinos Douros
The purpose of this retrospective study is to present the clinical experience of a single institution on the recovery of ABR thresholds in a large population of neonates and infants at risk of hearing loss. Potential prognostic factors associated with this phenomenon were also investigated. Out of 2248 high risk infants, 384 had abnormal ABR at initial hearing evaluation and 168 of them had absent ABR or a threshold ≥80 dBnHL. From this subgroup, a significant percentage showed complete or partial recovery on reexamination (32...
2017: International Journal of Otolaryngology
https://www.readbyqxmd.com/read/28357181/prevalence-of-vestibular-symptoms-in-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#16
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/28346294/diagnosis-and-treatment-of-vestibular-neuritis-neuronitis-or-peripheral-vestibulopathy-pvp-open-questions-and-possible-answers
#17
Stefan C A Hegemann, Angela Wenzel
: The acute vestibular syndrome is a clinically defined entity consisting of vertigo or dizziness that develops acutely over minutes to hours and is accompanied by nausea/vomiting, gait instability, head motion intolerance, and nystagmus, while persisting over a day or more. When it is caused by a peripheral vestibular lesion and is not associated with clinically manifest auditory deficits, it is mostly labeled vestibular neuritis/neuronitis/neuropathy or sometimes peripheral vestibulopathy. Here, we propose hypotheses and discuss current research advances on viral or vascular factors in the pathogenesis, the recurrence, the site of lesion, old and new treatment options, contraindicated measures, the differential diagnosis, and the prognosis of vestibular neuritis/neuronitis/neuropathy or vestibulopathy...
June 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28335750/novel-compound-heterozygous-mutations-in-the-otof-gene-identified-by-whole-exome-sequencing-in-auditory-neuropathy-spectrum-disorder
#18
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/28257146/randomized-controlled-trial-of-neurofeedback-on-chemotherapy-induced-peripheral-neuropathy-a-pilot-study
#19
Sarah Prinsloo, Diane Novy, Larry Driver, Randall Lyle, Lois Ramondetta, Cathy Eng, Jennifer McQuade, Gabriel Lopez, Lorenzo Cohen
BACKGROUND: Chemotherapy-induced peripheral neuropathy (CIPN) is a significant problem for cancer patients, and there are limited treatment options for this often debilitating condition. Neuromodulatory interventions could be a novel modality for patients trying to manage CIPN symptoms; however, they are not yet the standard of care. This study examined whether electroencephalogram (EEG) neurofeedback (NFB) could alleviate CIPN symptoms in survivors. METHODS: This was a randomized controlled trial with survivors assigned to an NFB group or a wait-list control (WLC) group...
June 1, 2017: Cancer
https://www.readbyqxmd.com/read/28225734/auditory-performance-and-electrical-stimulation-measures-in-cochlear-implant-recipients-with-auditory-neuropathy-compared-with-severe-to-profound-sensorineural-hearing-loss
#20
Joseph Attias, Tally Greenstein, Miriam Peled, David Ulanovski, Jay Wohlgelernter, Eyal Raveh
OBJECTIVES: The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). DESIGN: The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3...
March 2017: Ear and Hearing
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