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auditory neuropathy

Zixin Yong, Po-Jang Hsieh, Dan Milea
Acquired auditory-visual synesthesia (AVS) is a rare neurological sign, in which specific auditory stimulation triggers visual experience. In this study, we used event-related fMRI to explore the brain regions correlated with acquired monocular sound-induced phosphenes, which occurred 2 months after unilateral visual loss due to an ischemic optic neuropathy. During the fMRI session, 1-s pure tones at various pitches were presented to the patient, who was asked to report occurrence of sound-induced phosphenes by pressing one of the two buttons (yes/no)...
October 19, 2016: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
Ahmad Alodaib, Nara Sobreira, Wendy A Gold, Lisa G Riley, Nicole J Van Bergen, Meredith J Wilson, Bruce Bennetts, David R Thorburn, Corinne Boehm, John Christodoulou
Recent advances in next-generation sequencing strategies have led to the discovery of many novel disease genes. We describe here a non-consanguineous family with two affected boys presenting with early onset of severe axonal neuropathy, optic atrophy, intellectual disability, auditory neuropathy and chronic respiratory and gut disturbances. Whole-exome sequencing (WES) was performed on all family members and we identified compound heterozygous variants (c.[760C>A];[1528G>C];p.[(Gln254Lys);(Ala510Pro)] in the polyribonucleotide nucleotidyltransferase 1 (PNPT1) gene in both affected individuals...
October 19, 2016: European Journal of Human Genetics: EJHG
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
Sanjiv B Amin, Satish Saluja, Arvind Saili, Nirupama Laroia, Mark Orlando, Hongyue Wang, Asha Agarwal
AIM: Jaundice may cause auditory toxicity (auditory neuropathy and hearing loss). However, total serum bilirubin (TSB) does not discriminate neonates at risk for auditory toxicity. We compared TSB, bilirubin:albumin molar ratio (BAMR), and unbound bilirubin for their association with auditory toxicity in neonates with severe jaundice (TSB ≥342μmol/L, or that met exchange transfusion). METHOD: Neonates greater or equal to 34 weeks gestational age with severe jaundice during the first 2 postnatal weeks were eligible for prospective cohort study, unless they had craniofacial malformations, chromosomal disorders, toxoplasmosis, other infections, rubella, cytomegalovirus, herpes simplex infections, surgery, or family history of congenital deafness...
October 8, 2016: Developmental Medicine and Child Neurology
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
Min Young Lee, Seung-Hyun Bae, So-Young Chang, Jae-Hun Lee, Se-Hyung Kim, Jin-Chul Ahn, Phil-Sang Chung, Wesley Moy, Jae Yun Jung
Auditory neuropathy is a hearing disorder caused by impaired auditory nerve function. The lack of information about the pathophysiology of this disease limits early diagnosis and further treatment. Laser therapy is a novel approach to enhance nerve growth or induce axonal regeneration. We induced auditory neural degeneration sparing the sensory epithelium with local ouabain application in an animal model and observed the rescue effect of photobiomodulation (PBM), showing recovered auditory function and favorable histologic outcome...
October 28, 2016: Neuroscience Letters
Ana Sánchez-Martínez, José I Benito-Orejas, Juan J Tellería-Orriols, María J Alonso-Ramos
No abstract text is available yet for this article.
September 19, 2016: Acta Otorrinolaringológica Española
Nadia M Al-Wardy, Mohammed N Al-Kindi, Mazin J Al-Khabouri, Yahya Tamimi, Guy Van Camp
OBJECTIVES: To identify genetic defects in an Omani family diagnosed with deafness.  METHODS: A cross-sectional association study was conducted at the Department of Biochemistry, College of Medicine and Health Sciences, Sultan Qaboos University, Al-Khoud, Oman and the Centre of Medical Genetics, University of Antwerp, Antwerp, Belgium between August 2010 and September 2014. Microsatellites markers for nine non-syndromic genes were used to genotype the defective locus using the extracted DNA from family members...
October 2016: Saudi Medical Journal
E Villalón, M R Jones, C Sibigtroth, S J Zino, J M Dale, D S Landayan, H Shen, D D W Cornelison, M L Garcia
Charcot-Marie-Tooth (CMT) is the most common inherited peripheral neuropathy, affecting approximately 2.8 million people. The CMT leads to distal neuropathy that is characterized by reduced motor nerve conduction velocity, ataxia, muscle atrophy and sensory loss. We generated a mouse model of CMT type 2E (CMT2E) expressing human neurofilament light E396K (hNF-L(E396K) ), which develops decreased motor nerve conduction velocity, ataxia and muscle atrophy by 4 months of age. Symptomatic hNF-L(E396K) mice developed phenotypes that were consistent with proprioceptive sensory defects as well as reduced sensitivity to mechanical stimulation, while thermal sensitivity and auditory brainstem responses were unaltered...
September 19, 2016: Genes, Brain, and Behavior
Anna R Chambers, Juan J Salazar, Daniel B Polley
Neurons at higher stages of sensory processing can partially compensate for a sudden drop in peripheral input through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where >95% of afferent synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, auditory cortex (ACtx) processing and perception recover despite the absence of an auditory brainstem response (ABR) or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC), an auditory midbrain nucleus...
2016: Frontiers in Neural Circuits
Anastasia M Fedick, Chaim Jalas, Ananya Swaroop, Eric E Smouha, Bryn D Webb
Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population...
2016: Application of Clinical Genetics
Ronald van Toorn, Philip Brink, Johan Smith, Christelle Ackermann, Regan Solomons
The clinical expression of bilirubin-induced neurological dysfunction varies according to severity and location of the disease. Definitions have been proposed to describe different bilirubin-induced neurological dysfunction subtypes. Our objective was to describe the severity and clinico-radiological-neurophysiological correlation in 30 consecutive children with bilirubin-induced neurological dysfunction seen over a period of 5 years. Thirty children exposed to acute neonatal bilirubin encephalopathy were included in the study...
September 2, 2016: Journal of Child Neurology
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
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
Donald M Caspary, Daniel A Llano
Tinnitus is defined as a phantom sound (ringing in the ears), and can significantly reduce the quality of life for those who suffer its effects. Ten to fifteen percent of the general adult population report symptoms of tinnitus with 1-2% reporting that tinnitus negatively impacts their quality of life. Noise exposure is the most common cause of tinnitus and the military environment presents many challenging high-noise situations. Military noise levels can be so intense that standard hearing protection is not adequate...
August 20, 2016: Hearing Research
Katsuki Niwa, Kunio Mizutari, Toshiyasu Matsui, Takaomi Kurioka, Takeshi Matsunobu, Satoko Kawauchi, Yasushi Satoh, Shunichi Sato, Akihiro Shiotani, Yasushi Kobayashi
The ear is the organ that is most sensitive to blast overpressure, and ear damage is most frequently seen after blast exposure. Blast overpressure to the ear results in sensorineural hearing loss, which is untreatable and is often associated with a decline in the quality of life. In this study, we used a rat model to demonstrate the pathophysiological and structural changes in the inner ear that replicate pure sensorineural hearing loss associated with blast injury using laser-induced shock wave (LISW) without any conductive hearing loss...
2016: Scientific Reports
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
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
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
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
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