747 results match your criteria Auditory Neuropathy


Taurine enhances mouse cochlear neural stem cell transplantation via the cochlear lateral wall for replacement of degenerated spiral ganglion neurons via sonic hedgehog signaling pathway.

Cell Tissue Res 2019 Apr 17. Epub 2019 Apr 17.

Department of Otolaryngology and Head & Neck surgery, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Changsha, 410011, China.

The aim of this paper is to investigate the potential beneficial effects of taurine in cochlear neural stem cell (NSC) transplantation and elucidate the underlying molecular mechanism. The NSC cells were isolated from neonatal Balb/c mice and an auditory neuropathy gerbil model was established by microinjection of ouabain. The spiral ganglion neurons (SGN) were characterized with immunofluorescence stained with Tuj1 antibody. Read More

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http://dx.doi.org/10.1007/s00441-019-03018-6DOI Listing

Auditory and speech performance in cochlear implanted ANSD children.

Acta Otolaryngol 2019 Apr 5:1-5. Epub 2019 Apr 5.

c ENT Surgery Unit, Department of Surgical Specialists , King Fahad Medical City , Riyadh , Saudi Arabia.

Background: Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. Cochlear implantation was initially not recommended for ANSD children, later studies showed variable outcomes among those subjects.

Objective: To assess the auditory and speech performance of cochlear implanted children with auditory neuropathy spectrum disorder (ANSD) and to compare these results to those obtained from cochlear implanted children with sensorineural hearing loss (SNHL) Material and methods: 18 cochlear implanted children with ANSD and 40 cochlear implanted children with SNHL were included in this study. Read More

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http://dx.doi.org/10.1080/00016489.2019.1571283DOI Listing
April 2019
1 Read

Effect of acoustic features on discrimination ability in individuals with auditory neuropathy spectrum disorder: an electrophysiological and behavioral study.

Eur Arch Otorhinolaryngol 2019 Apr 1. Epub 2019 Apr 1.

Department of Audiology, All India Institute of Speech and Hearing, Mysore, India.

Purpose: The present study aimed to investigate the discrimination ability for acoustic cues in individuals with auditory neuropathy spectrum disorder (ANSD) using both behavioral and neural measures and to compare the result with normal hearing individuals.

Methods: Four naturally produced syllables /ba/, /da/, /ma/ and /pa/ were used to study discrimination skills. They were combined in pairs such that the two syllables differ in acoustic features, that is place (/ba/-/da/), manner (/ba/-/ma/) and voicing (/ba/-/pa/) cues. Read More

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http://dx.doi.org/10.1007/s00405-019-05405-9DOI Listing

The use of animal models to study cell transplantation in neuropathic hearing loss.

Hear Res 2019 Mar 19;377:72-87. Epub 2019 Mar 19.

Centre for Stem Cell Biology and Department of Biomedical Sciences, University of Sheffield, Sheffield, S10 2TN, United Kingdom. Electronic address:

Auditory neuropathy (AN) is a form of sensorineural deafness specifically affecting the conduction of the nerve impulse from the cochlear hair cells to the auditory centres of the brain. As such, the condition is a potential clinical target for 'cell replacement therapy', in which a functioning auditory nerve is regenerated by transplanting an appropriated neural progenitor. In this review, we survey the current literature and examine possible experimental models for this condition, with particular reference to their compatibility as suitable hosts for transplantation. Read More

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http://dx.doi.org/10.1016/j.heares.2019.03.014DOI Listing

Protection of Spiral Ganglion Neurons and Prevention of Auditory Neuropathy.

Adv Exp Med Biol 2019 ;1130:93-107

Otolaryngology-Head and Neck Surgery, Shandong Provincial ENT Hospital, Shandong Provincial ENT Hospital Affiliated to Shandong University, Jinan, China.

In the auditory system, the primary sensory neurons, spiral ganglion neurons (SGNs), transmit complex acoustic information from hair cells to the second-order sensory neurons in the cochlear nucleus for sound processing, thus building the initial bridge between the physical world of sound and the perception of that sound. Cochlear SGN loss causes irreversible hearing impairment because this type of neural cell cannot regenerate. A better understanding of the molecular mechanisms of formation, structure, degeneration, and protection of SGNs will help to design potential therapeutic strategies for preservation and replacement of them in the cochlear implant recipient. Read More

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http://link.springer.com/10.1007/978-981-13-6123-4_6
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http://dx.doi.org/10.1007/978-981-13-6123-4_6DOI Listing
January 2019
2 Reads

The Prevalence and Causes of Auditory Neuropathy/Dys-synchrony (AN/AD) in Children with Hearing Impairment.

Indian J Otolaryngol Head Neck Surg 2019 Mar 17;71(1):71-75. Epub 2018 Sep 17.

1School of Rehabilitation, Hamadan University of Medical Sciences, Hamadan, Iran.

There are a wide variety of hearing impairments that part of it is auditory neuropathy/dys-synchrony (AN/AD). So, the object of this study was determination the prevalence and causes of AN/AD in children with hearing impairment. This study was a descriptive cross-sectional survey. Read More

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http://link.springer.com/10.1007/s12070-018-1494-1
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http://dx.doi.org/10.1007/s12070-018-1494-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6401023PMC
March 2019
6 Reads

[Cochlear implant in children].

Rev Prat 2018 Oct;68(8):870-873

Service ORL, CHU de Rennes, France.

Cochlear implant in children. In a newborn, sensitive auditory stimulations allow the progressive central auditory development. In a congenital severe or profound deaf child, a cochlear implantation can be considered if auditory thresholds are too low to provide an efficient auditory stimulation with hearing aids. Read More

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October 2018
4 Reads

Assessing and monitoring the impact of the national newborn hearing screening program in Israel.

Isr J Health Policy Res 2019 Mar 11;8(1):30. Epub 2019 Mar 11.

Department of Maternal and Child Health, Public Health Services, Ministry of Health, Jerusalem, Israel.

Background: The Israeli Newborn Hearing Screening Program (NHSP) began operating nationally in January 2010. The program includes the Otoacoustic Emissions (OAE) test for all newborns and Automated Auditory Brainstem Response (A-ABR) test for failed OAE and infants at risk for auditory neuropathy spectrum disorders. NHSP targets are diagnosis of hearing impairment by age three months and initiation of habilitation by six months. Read More

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https://ijhpr.biomedcentral.com/articles/10.1186/s13584-019-
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http://dx.doi.org/10.1186/s13584-019-0296-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410489PMC
March 2019
4 Reads

GJB2 c.235delC variant associated with autosomal recessive nonsyndromic hearing loss and auditory neuropathy spectrum disorder.

Genet Mol Biol 2019 Jan-Mar;42(1):48-51. Epub 2019 Feb 28.

Third Xiangya Hospital, Center for Experimental Medicine and Department of Neurology, Central South University, Changsha, Hunan, China.

Autosomal recessive nonsyndromic hearing loss (ARNSHL) is a genetically heterogeneous neurosensory disorder, usually characterized by congenital or prelingual hearing loss. We report a Han Chinese male, born to consanguineous parents, presenting with nonsyndromic sensorineural hearing loss, whose clinical phenotype was also consistent with auditory neuropathy spectrum disorder (ANSD). After exome sequencing, a gap junction protein beta 2 gene (GJB2) c. Read More

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http://dx.doi.org/10.1590/1678-4685-gmb-2017-0318DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428124PMC
February 2019
1 Read

[The molecular mechanisms underpinning auditory neuropathy].

Authors:
K Wang L Luo Z Z He

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2019 Feb;54(2):149-156

Department of Biomedical Sciences, Creighton University, Omaha, Nebraska 68178, USA.

Auditory neuropathy (AN) is a hearing disorder where cochlear inner hair cell and/or the auditory nerve function is disrupted while outer hair cell function is normal. It can affect people of all ages, from infancy to adulthood. People with auditory neuropathy may have normal hearing threshold, or hearing loss ranging from mild to severe; they always have poor speech-perception abilities. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1673-0860.2019.02.013DOI Listing
February 2019

Auditory Neuropathy: Bridging the Gap Between Hearing Aids and Cochlear Implants.

Otolaryngol Clin North Am 2019 Apr 12;52(2):349-355. Epub 2019 Feb 12.

The Otology Group of Vanderbilt, Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, 7209 Medical Center East, South Tower 1215 21st Avenue South, Nashville, TN 37232-8605, USA. Electronic address:

Auditory neuropathy spectrum disorder (ANSD) is a complex and heterogeneous disorder associated with altered neural synchrony with respect to auditory stimuli. Patients have characteristic auditory findings including normal otoacoustic emissions in the setting of abnormal auditory brainstem response. Patients with ANSD have a high incidence of comorbid developmental delay that may impact speech outcomes. Read More

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http://dx.doi.org/10.1016/j.otc.2018.11.016DOI Listing

Relationship Between Objective and Behavioral Audiology for Young Children Being Assessed for Cochlear Implantation: Implications for CI Candidacy Assessment.

Otol Neurotol 2019 Mar;40(3):e252-e259

Cochlear Implant Clinic, Royal Victorian Eye and Ear Hospital.

Objective: This study aimed to evaluate the feasibility of making cochlear implant recommendations based on diagnostic ABR and ASSR results. The goal was to challenge the need for behavioral audiometry as part of the standard cochlear implant assessment battery for infants with profound hearing loss and to reduce the age at which cochlear implant recommendation was made.

Study Design: A retrospective review of 123 patient files for children referred to the pediatric cochlear implant service before 3 years of age over a 3-year period was undertaken. Read More

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http://dx.doi.org/10.1097/MAO.0000000000002125DOI Listing
March 2019
1 Read

[The clinical application of gene diagnosis and genetic counseling on hereditary hearing loss].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2019 Jan;33(1):58-62

Department of Otorhinolaryngology Head and Neck Surgery, Peking University First Hospital, Beijing, 100034, China.

To summarize the clinic procedure and experience about gene diagnosis and genetic counseling on hereditary hearing loss, and explore the strategy and principle about gene diagnosis and genetic counseling on hereditary hearing loss.A retrospective analysis was used on the clinical data of 151 cases who aim at genetic counseling. The all cases were divided into 5 groups according to the purpose of genetic counseling, such as the occurrence risk of hearing loss, the etiological analysis, the choice of the intervention way, the examination guidance, the prevention of hearing loss and the usage requirement of Aminogly cosides drugs. Read More

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http://dx.doi.org/10.13201/j.issn.1001-1781.2019.01.012DOI Listing
January 2019

Phenotype prediction of Mohr-Tranebjaerg syndrome (MTS) by genetic analysis and initial auditory neuropathy.

BMC Med Genet 2019 Jan 11;20(1):11. Epub 2019 Jan 11.

Institute of Otolaryngology, Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing, 100853, China.

Background: Mohr-Tranebjaerg syndrome (MTS) is a rare X-linked recessive neurodegenerative disorder resulting in early-onset hearing impairment, gradual dystonia and optic atrophy. MTS is caused by variations in the nuclear TIMM8A gene, which is involved in mitochondrial transport of metabolites. This study aimed to identify the pathogenic gene variations in three Chinese families associated with predicted MTS with or without X-linked agammaglobulinaemia. Read More

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https://bmcmedgenet.biomedcentral.com/articles/10.1186/s1288
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http://dx.doi.org/10.1186/s12881-018-0741-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330410PMC
January 2019
19 Reads
2.083 Impact Factor

Transplantation of human limbus-derived mesenchymal stromal cells via occipital approach improves hearing in animal auditory neuropathy.

Int J Pediatr Otorhinolaryngol 2019 Feb 15;117:67-72. Epub 2018 Nov 15.

Department of Otolaryngology-Head and Neck Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, 114, Taiwan; Graduate Institute of Medical Sciences, National Defense Medical Center, Taipei, 114, Taiwan.

Objective: To develop a surgical approach for cell transplantation into mouse cochlear nerves via an intracranial route and investigate whether transplantation of human limbus-derived mesenchymal stromal cells (HL-MSCs) can improve hearing in this model of auditory neuropathy.

Methods: We used 8-week-old CBA/CaJ male mice and created ouabain-induced auditory neuropathy. The surgical approach passed through the cerebellum to reveal the superior semicircular canal and brainstem, allowing access to the auditory nerve. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01655876183058
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http://dx.doi.org/10.1016/j.ijporl.2018.11.018DOI Listing
February 2019
12 Reads

Language outcome in children with congenital hearing impairment: The influence of etiology.

Int J Pediatr Otorhinolaryngol 2019 Feb 3;117:37-44. Epub 2018 Nov 3.

Department of Otorhinolaryngology, Head and Neck Surgery, and Audiology, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, 2100, Copenhagen, Denmark.

Objectives: To investigate the possible association between the etiology of hearing impairment (HI) and language outcome in children with congenital HI after an early medical-technical intervention and three years of AVT.

Methods: A retrospective, two-center study was conducted of 53 patients who were divided in four categories of etiology (degeneratio labyrinthi acustici (DLA) congenita hereditaria, DLA congenita non specificata, DLA congenita postinfectiosa and auditory neuropathy). Language outcome was assessed by examining receptive vocabulary (Peabody Picture Vocabulary Test, PPVT-4), receptive language (Reynell test) and productive language (the Danish 'Viborgmaterialet'). Read More

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http://dx.doi.org/10.1016/j.ijporl.2018.11.002DOI Listing
February 2019
1 Read

Bilirubin-induced neurotoxic and ototoxic effects in rat cochlear and vestibular organotypic cultures.

Neurotoxicology 2019 Mar 19;71:75-86. Epub 2018 Dec 19.

Department of Otorhinolaryngology, Affiliated Sixth People's Hospital of Shanghai Jiaotong University, 600 Yishan Road, Shanghai 200233, PR China.

Exposure to high levels of bilirubin in hyperbilirubinemia patients and animal models can result in sensorineural deafness. However, the mechanisms underlying bilirubin-induced damage to the inner ear, including the cochlear and vestibular organs, remain unknown. The present analyses of cochlear and vestibular organotypic cultures obtained from postnatal day 3 rats exposed to bilirubin at varying concentrations (0, 10, 50, 100, or 250 μM) for 24 h revealed that auditory nerve fibers (ANFs) and vestibular nerve endings were destroyed even at low doses (10 and 50 μM). Read More

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http://dx.doi.org/10.1016/j.neuro.2018.12.004DOI Listing
March 2019
2 Reads
3.379 Impact Factor

Findings from aetiological investigation of Auditory Neuropathy Spectrum Disorder in children referred to cochlear implant programs.

Int J Pediatr Otorhinolaryngol 2019 Jan 11;116:79-83. Epub 2018 Oct 11.

Cochlear Implant Department, Great Ormond Street Hospital, London, United Kingdom. Electronic address:

Objectives: Auditory neuropathy spectrum disorder (ANSD) is an audiological diagnosis characterised by hearing dysfunction in the presence of intact outer hair cell function in the cochlea. ANSD is thought to account for 7-10% of all childhood permanent hearing impairment, and can result from a range of pathological processes. This paper describes the rationale, methods and findings from the aetiological investigation of ANSD. Read More

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http://dx.doi.org/10.1016/j.ijporl.2018.10.010DOI Listing
January 2019

[Research progress on auditory neuropathy spectrum disorder and cochlear implantation in infants and young children].

Authors:
J Y Chen Y X Li

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Jul;32(14):1120-1124

Auditory neuropathy is a disorder characterized by absent or severely abnormal auditory brainstem response (ABR) with intact outer hair cell function, as evidenced by the presence of evoked otoacoustic emissions and/or cochlear microphonics. Unlike patients with sensory hearing loss who show clinical evidence of impaired outer hair cell function. For ANSD patients, clinical rehabilitation is mainly limited to hearing aid wearing and cochlear implantation. Read More

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http://dx.doi.org/10.13201/j.issn.1001-1781.2018.14.020DOI Listing

Mutational and phenotypic spectrum of OTOF-related auditory neuropathy in Koreans: eliciting reciprocal interaction between bench and clinics.

J Transl Med 2018 11 27;16(1):330. Epub 2018 Nov 27.

Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 300 Gumi-dong, Bundang-gu, Seongnam, 13620, Republic of South Korea.

Background: While auditory neuropathy spectrum disorder (ANSD) is a heterogeneous disorder and its management quite varies depending upon the etiology, even including self-resolution, OTOF is an important molecular etiology of prelingual ANSD and has emerged as an attractive target for implementation of precision medicine in terms of timing and prognosis prediction of auditory rehabilitation. However, to date, the literature is lacking in the genotype-phenotype relationship of this gene as well as efficient molecular testing strategy in the clinic in many populations and to make things more complicated in Koreans, the most prevalent variant p.Arg1939Gln among Korean ANSD children frequently evaded detection by next generation sequencing (NGS), resulting in delayed genetic diagnosis and late cochlear implantation (CI). Read More

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http://dx.doi.org/10.1186/s12967-018-1708-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260760PMC
November 2018
16 Reads
3.930 Impact Factor

Elongated EABR wave latencies observed in patients with auditory neuropathy caused by mutation.

Laryngoscope Investig Otolaryngol 2018 Oct 24;3(5):388-393. Epub 2018 Sep 24.

National Institute of Sensory Organs Tokyo Japan.

Objectives: We sought to determine how the pathology altered electrically evoked auditory brainstem responses (EABRs) in patients with hearing loss by evaluating EABRs in auditory neuropathy patients with mutations comparing with various types of congenital deafness.

Methods: We included 15 patients with congenital hearing loss, grouped according to pathology: mutations (n = 4), mutations (n = 4), mutations (n = 4), or cytomegalovirus infections (n = 3). EABRs were recorded when patients underwent cochlear implantation surgery. Read More

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http://doi.wiley.com/10.1002/lio2.210
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http://dx.doi.org/10.1002/lio2.210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6209615PMC
October 2018
6 Reads

Measurement of Thresholds Using Auditory Steady-State Response and Cochlear Microphonics in Children with Auditory Neuropathy.

J Am Acad Audiol 2018 Nov 8. Epub 2018 Nov 8.

Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Fudan University, Shanghai, China.

Background: The detection of precise hearing thresholds in infants and children with auditory neuropathy (AN) is challenging with current objective methods, especially in those younger than six months of age.

Purpose: The aim of this study was to compare the thresholds using auditory steady-state response (ASSR) and cochlear microphonics (CM) in children with AN and children with normal hearing.

Research Design: The thresholds of CM, ASSR, and visual reinforcement audiometry (VRA) tests were recorded; the ASSR and VRA frequencies used were 250, 500, 1000, 2000, and 4000 Hz. Read More

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http://dx.doi.org/10.3766/jaaa.17013DOI Listing
November 2018
5 Reads

Hearing Loss and Failed Newborn Hearing Screen.

Clin Perinatol 2018 12 24;45(4):629-643. Epub 2018 Sep 24.

Department of Pediatric Otolaryngology, University of Utah, 100 North Mario Capercchi Drive, Salt Lake City, UT 84113, USA.

Hearing loss is the most common congenital defect. With early diagnosis and intervention, we are able to improve speech and language outcomes in this population. In this article, we discuss the implications of the newborn hearing screen, as well as diagnostic interventions, management, and intervention, and the increasing role of congenital cytomegalovirus screening. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00955108183139
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http://dx.doi.org/10.1016/j.clp.2018.07.004DOI Listing
December 2018
11 Reads

Targeted next generation sequencing reveals OTOF mutations in auditory neuropathy spectrum disorder.

Int J Pediatr Otorhinolaryngol 2018 Dec 14;115:19-23. Epub 2018 Sep 14.

Department of Otorhinolaryngology, Hainan General Hospital, Haikou, 570311, PR China. Electronic address:

Objective: To study the genetic etiology of auditory neuropathy spectrum disorder (ANSD) in a Chinese family and perform a literature review of OTOF mutations and cochlear implantation (CI).

Methods: Sequential targeted next generation sequencing (NGS) and CI was performed for the proband. Further, 50 DNA samples from unrelated families with nonsyndromic deafness were examined for frequency determination. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01655876183046
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http://dx.doi.org/10.1016/j.ijporl.2018.09.008DOI Listing
December 2018
12 Reads

Cochlear implantation in children with auditory neuropathy: Lessons from Brown-Vialetto-Van Laere syndrome.

Cochlear Implants Int 2019 Jan 18;20(1):31-38. Epub 2018 Oct 18.

b Paediatric ENT Department , Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK.

Objective: Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurodegenerative disorder associated with auditory neuropathy (AN). The decision process for CI in AN is evolving with increasing evidence of efficacy. We evaluated the benefit of CI in children with BVVL syndrome. Read More

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http://dx.doi.org/10.1080/14670100.2018.1534035DOI Listing
January 2019
2 Reads

Tone-in-noise detection deficits in elderly patients with clinically normal hearing.

Am J Otolaryngol 2019 Jan - Feb;40(1):1-9. Epub 2018 Sep 17.

Center for Hearing and Deafness, University at Buffalo, Buffalo, NY 14214, USA; Department of Audiology and Speech-Language Pathology, Asia University, Taichung, Taiwan, ROC. Electronic address:

Purpose: One of the most common complaints among the elderly is the inability to understand speech in noisy environments. In many cases, these deficits are due to age-related hearing loss; however, some of the elderly that have difficulty hearing in noise have clinically normal pure-tone thresholds. While speech in noise testing is informative, it fails to identify specific frequencies responsible for the speech processing deficit. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01960709183075
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http://dx.doi.org/10.1016/j.amjoto.2018.09.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6286197PMC
April 2019
8 Reads

Behavioural and Electrophysiological Correlates of Aided Performance in Individuals with Late Onset Auditory Neuropathy Spectrum Disorder: A Review.

J Audiol Otol 2018 Oct 20;22(4):171-177. Epub 2018 Sep 20.

JSS Institute of Speech and Hearing, Kelageri, Dharwad, India.

Hearing aids are one of the rehabilitative options for individuals with late onset auditory neuropathy spectrum disorder (ANSD). There is a growing body of evidence that supports the effectiveness of hearing aids among these individuals. The objective of the study was to review the behavioural and electrophysiological factors that determine the effectiveness of amplification in individuals with late onset ANSD. Read More

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http://dx.doi.org/10.7874/jao.2018.00031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6233941PMC
October 2018

Evaluation of saccular and inferior vestibular nerve function in children with auditory neuropathy spectrum disorder.

Eur Arch Otorhinolaryngol 2018 Dec 26;275(12):2925-2931. Epub 2018 Sep 26.

Audio-Vestibular Unit, Minia University, Minia, Egypt.

Purpose: The aim of the current work was to evaluate the function of the saccule and inferior vestibular nerve in children with auditory neuropathy spectrum disorder (ANSD) by recording the cervical-evoked myogenic potentials (C-VEMP) on those children and to compare C-VEMP results in ANSD children of pre-lingual onset to those in ANSD children of post-lingual onset.

Methods: The study included 38 ANSD children of pre-lingual onset, 16 ANSD children of post-lingual onset, and 20 control children. All participant children were subjected to C-VEMP testing using 500 Hz tone burst stimuli. Read More

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http://dx.doi.org/10.1007/s00405-018-5149-3DOI Listing
December 2018

Unilateral Versus Bilateral Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder (ANSD).

Otol Neurotol 2018 Oct;39(9):e810-e816

Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University, Nashville, Tennessee.

Objective: To evaluate audiologic outcomes following unilateral cochlear implantation with contralateral hearing aid (unilateral CI + HA) versus bilateral CI in children with auditory neuropathy spectrum disorder (ANSD).

Study Design: Retrospective case review.

Setting: Tertiary Care Otologic Practice. Read More

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http://dx.doi.org/10.1097/MAO.0000000000001971DOI Listing
October 2018
17 Reads

Synergistic Transcriptional Changes in AMPA and GABA Receptor Genes Support Compensatory Plasticity Following Unilateral Hearing Loss.

Neuroscience 2018 Sep 1. Epub 2018 Sep 1.

Eaton-Peabody Laboratories, Massachusetts Eye and Ear Infirmary, Boston MA 02114, USA; Dept. of Otolaryngology, Harvard Medical School, Boston MA 02114, USA. Electronic address:

Debilitating perceptual disorders including tinnitus, hyperacusis, phantom limb pain and visual release hallucinations may reflect aberrant patterns of neural activity in central sensory pathways following a loss of peripheral sensory input. Here, we explore short- and long-term changes in gene expression that may contribute to hyperexcitability following a sudden, profound loss of auditory input from one ear. We used fluorescence in situ hybridization to quantify mRNA levels for genes encoding AMPA and GABA receptor subunits (Gria2 and Gabra1, respectively) in single neurons from the inferior colliculus (IC) and auditory cortex (ACtx). Read More

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http://dx.doi.org/10.1016/j.neuroscience.2018.08.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395571PMC
September 2018
7 Reads

[Auditory evoked potentials in children with auditory neuropathy spectrum disorder].

Vestn Otorinolaringol 2018;83(4):15-20

Russian Research Centre for Audiology and Hearing Rehabilitation, Russian Medico-Biological Agency, 123, Moscow, Russia, 117513; Russian Medical Academy of Continuous Professional Education, Ministry of Health of the Russian Federation, Moscow, Russia, 123395.

The objective of the present study was to estimate peculiarities of the auditory brainstem evoked potentials (ABR), auditory steady-state responses (ASSR) and cortical auditory evoked potentials (CAEP) in the children presenting with bilateral auditory neuropathy spectrum disorder (ANSD). The study included 100 patients with bilateral ANSD diagnosed based on the positive response of otoacoustic emissions (OAEs) and/or cochlear microphonic (CM) detection, while no synchronous neural activity was detected in the ABR test. Cochlear microphonic was the main clue for the ANSD diagnosing, because OAE was absent in both ears of 49 children. Read More

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http://dx.doi.org/10.17116/otorino201883415DOI Listing
January 2019
2 Reads

Novel OTOF gene mutations identified using a massively parallel DNA sequencing technique in DFNB9 deafness.

Acta Otolaryngol 2018 Oct 3;138(10):865-870. Epub 2018 Aug 3.

b Medical Genetics Center, Southwest Hospital , Army Medical University , Chongqing , China.

Objectives: This study examined the causative genes in patients with early-onset hearing loss from two Chinese families.

Method: Massively parallel sequencing, designed to screen all reported genes associated with hearing loss, was performed in a large number of Chinese individuals with hearing loss. This study enrolled patients with the same OTOF mutation and analyzed their phenotype-genotype correlations. Read More

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http://dx.doi.org/10.1080/00016489.2018.1476777DOI Listing
October 2018
23 Reads
0.990 Impact Factor

An Frameshift Variant Associated with Auditory Neuropathy Spectrum Disorder.

Curr Genomics 2018 Aug;19(5):370-374

Center for Experimental Medicine and Department of Neurology, The Third Xiangya Hospital, Central South University, Changsha, China.

Background: Auditory Neuropathy Spectrum Disorder (ANSD) is manifested as impairment of auditory nerve activity but preservation of the outer hair cell function.

Objective: This study was to detect the disease-causing gene and variant(s) in a Chinese ANSD family.

Methods: A four-generation consanguineous Chinese ANSD family and 200 unrelated healthy controls were enrolled. Read More

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http://dx.doi.org/10.2174/1389202919666171113152951DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6030853PMC
August 2018
6 Reads

Outcomes of Cochlear Implantation in Auditory Neuropathy Spectrum Disorder and the Role of Cortical Auditory Evoked Potentials in Benefit Evaluation.

Turk Arch Otorhinolaryngol 2018 Mar 1;56(1):15-20. Epub 2018 Mar 1.

Department of Otorhinolaryngology, Madras ENT Research Foundation, Chennai, India.

Objective: To compare the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorder (ANSD) and age-matched controls with profound sensorineural hearing loss, using categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), and meaningful use of speech scale (MUSS), and to determine the role of Cortical Auditory Evoked Potentials (CAEP) in benefit evaluation after CI.

Methods: Ten patients (8 males and two females) with ANSD who underwent CI were included in the study. Auditory and speech scores were compared between baseline and after 12 months of habilitation in children with ANSD. Read More

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http://dx.doi.org/10.5152/tao.2017.2537DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6017206PMC

Dynamics of Speech Perception in the Auditory-Visual Mode: An Empirical Evidence for the Management of Auditory Neuropathy Spectrum Disorders.

J Audiol Otol 2018 Oct 4;22(4):197-203. Epub 2018 Jul 4.

Department of Audiology, All India Institute of Speech and Hearing, Mysuru, India.

Background And Objectives: The present study probed into the relative and combined contribution of auditory and visual modalities in the speech perception of individuals with auditory neuropathy spectrum disorders (ANSD). Specifically, the identification scores of consonantvowel (CV) syllables, visual enhancement (VE), and auditory enhancement in different signal to noise ratios (SNRs) were compared with that of the control group. Subjects and. Read More

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http://dx.doi.org/10.7874/jao.2018.00059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6233939PMC
October 2018
1 Read

The First Case of Riboflavin Transporter Deficiency in sub-Saharan Africa.

Semin Pediatr Neurol 2018 07 4;26:10-14. Epub 2017 Apr 4.

Division of Paediatric Neurology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.

This report describes the first case of a child with genetically confirmed Brown-Vialetto-van Laere syndrome in sub-Saharan Africa. This is an extremely rare clinical condition that presents with an auditory neuropathy, bulbar palsy, stridor, muscle weakness, and respiratory compromise that manifests with diaphragmatic and vocal cord paralysis. It is an autosomal recessive condition for which the genetic mutation has only recently been linked to a riboflavin transporter deficiency. Read More

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http://dx.doi.org/10.1016/j.spen.2017.03.002DOI Listing
July 2018
24 Reads

A study on vestibular-evoked myogenic potentials via galvanic vestibular stimulation in normal people.

J Otol 2018 Mar 6;13(1):16-19. Epub 2017 Oct 6.

National Institute of Sensory Organs, Tokyo Medical Center, Japan.

Objectives: The aim of our study is to examine vestibular-evoked myogenic potentials (VEMPs) elicited by the galvanic vestibular stimulation in the sternocleidomastoid muscle (SCM) in healthy subjects for clinical applications of auditory neuropathy or vestibular neuropathy in the future.

Methods: We enrolled sixteen healthy subjects to record the average responses of SCM to galvanic vestibular stimulation (GVS) [current 3 mA; duration 1 ms] by electromyography (EMG). SPSS18. Read More

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http://dx.doi.org/10.1016/j.joto.2017.09.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6002623PMC
March 2018
4 Reads

A case of auditory neuropathy revealed by OTOF gene mutation analysis in a junior high school girl.

J Otol 2017 Dec 15;12(4):202-206. Epub 2017 Jul 15.

National Institute of Sensory Organs, Tokyo Medical Center, Japan.

Objective: Congenital auditory neuropathy (AN) affects hearing and speech development. The degree of hearing difficulty in congenital AN varies as a function of pathology at the inner ear hair cell (IHC) synapses or the auditory nerve. We report a case of a Chinese girl with AN revealed by OTOF (otoferlin) gene mutation analysis who had only a mild hearing loss. Read More

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http://dx.doi.org/10.1016/j.joto.2017.07.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6002630PMC
December 2017
6 Reads

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder.

Audiol Neurootol 2018 22;23(1):39-47. Epub 2018 Jun 22.

Department of Otolaryngology-HNS, University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada.

Background: Cochlear nerve aplasia (CNA) may present with features of auditory neuropathy spectrum disorder (ANSD), having detectable otoacoustic emissions (OAE) but profound hearing loss. We propose that some children with CNA have a distinct form of afferent ANSD in which efferent cochlear nerve function can be detected using contralateral suppression of OAE.

Methods: Children were prospectively enrolled with MRI and auditory brainstem response evidence of unilateral CNA, a normal contralateral ear, and detectable OAE bilaterally. Read More

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https://www.karger.com/Article/FullText/487584
Publisher Site
http://dx.doi.org/10.1159/000487584DOI Listing
June 2018
6 Reads

French Society of ENT (SFORL) guidelines (short version): Audiometry in adults and children.

Eur Ann Otorhinolaryngol Head Neck Dis 2018 Oct 19;135(5):341-347. Epub 2018 Jun 19.

Inserm 1051, service otologie-otoneurologie, plateforme d'audiologie I-PaudioM, CHU de Montpellier, 34090 Montpellier, France.

Introduction: French Society of ENT (SFORL) good practice guidelines for audiometric examination in adults and children.

Methods: A multidisciplinary working group performed a review of the scientific literature. Guidelines were drawn up, reviewed by an independent reading group, and finalized in a consensus meeting. Read More

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http://dx.doi.org/10.1016/j.anorl.2018.05.009DOI Listing
October 2018
15 Reads

[International consensus (ICON) on audiological assessment of hearing loss in children].

Authors:
J Y Chen J Yang

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Jun;32(12):886-890

The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. Read More

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http://www.cnki.net/kcms/doi/10.13201/j.issn.1001-1781.2018.
Publisher Site
http://dx.doi.org/10.13201/j.issn.1001-1781.2018.12.002DOI Listing
June 2018
6 Reads

Reorganization of Destabilized Nodes of Ranvier in β Mutants Uncovers Critical Timelines for Nodal Restoration and Prevention of Motor Paresis.

J Neurosci 2018 Jul 15;38(28):6267-6282. Epub 2018 Jun 15.

Department of Cellular and Integrative Physiology, Center for Biomedical Neuroscience, Long School of Medicine, University of Texas Health Science Center, San Antonio, Texas 78229-3900 and

Disorganization of nodes of Ranvier is associated with motor and sensory dysfunctions. Mechanisms that allow nodal recovery during pathological processes remain poorly understood. A highly enriched nodal cytoskeletal protein βIV spectrin anchors and stabilizes the nodal complex to actin cytoskeleton. Read More

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http://dx.doi.org/10.1523/JNEUROSCI.0515-18.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6041796PMC
July 2018
2 Reads

Role of Targeted Next Generation Sequencing in the Etiological Work-Up of Congenitally Deaf Children.

Otol Neurotol 2018 Jul;39(6):732-738

Centre of Medical Genetics, University of Antwerp and Antwerp University Hospital, Edegem.

Objectives: The purpose of this study is to report the results of a comprehensive etiological work-up for congenitally deaf children including targeted next generation sequencing.

Study Design: Retrospective case review.

Setting: Tertiary referral center. Read More

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http://dx.doi.org/10.1097/MAO.0000000000001847DOI Listing
July 2018
18 Reads

βIV Spectrinopathies Cause Profound Intellectual Disability, Congenital Hypotonia, and Motor Axonal Neuropathy.

Am J Hum Genet 2018 06 31;102(6):1158-1168. Epub 2018 May 31.

Department of Neuroscience and Integrative Molecular and Biomedical Sciences Graduate Program, Baylor College of Medicine, Houston, TX 77030, USA. Electronic address:

βIV spectrin links ankyrinG (AnkG) and clustered ion channels at axon initial segments (AISs) and nodes of Ranvier to the axonal cytoskeleton. Here, we report bi-allelic pathogenic SPTBN4 variants (three homozygous and two compound heterozygous) that cause a severe neurological syndrome that includes congenital hypotonia, intellectual disability, and motor axonal and auditory neuropathy. We introduced these variants into βIV spectrin, expressed these in neurons, and found that 5/7 were loss-of-function variants disrupting AIS localization or abolishing phosphoinositide binding. Read More

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http://dx.doi.org/10.1016/j.ajhg.2018.04.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5992132PMC
June 2018
11 Reads
10.930 Impact Factor

Auditory function in Pelizaeus-Merzbacher disease.

J Neurol 2018 Jul 3;265(7):1580-1589. Epub 2018 May 3.

Nemours Biomedical Research, Center for Applied Clinical Genomics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Pelizaeus-Merzbacher disease (PMD; MIM 312080), an inherited defect of central nervous system myelin formation, affects individuals in many ways, including their hearing and language abilities. The aim of this study was to assess the auditory abilities in 18 patients with PMD by examining the functional processes along the central auditory pathways using auditory brainstem responses (ABR) and cortical auditory evoked potentials (CAEP) in response to speech sounds. The significant ABR anomalies confirm the existence of dyssynchrony previously described at the level of the brainstem in patients with PMD. Read More

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http://dx.doi.org/10.1007/s00415-018-8884-xDOI Listing
July 2018
4 Reads

Cochlear Microphonic and Summating Potential Responses from Click-Evoked Auditory Brain Stem Responses in High-Risk and Normal Infants.

J Am Acad Audiol 2018 May;29(5):427-442

Communication Sciences Research Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Background: Examination of cochlear and neural potentials is necessary to assess sensory and neural status in infants, especially those cared for in neonatal intensive care units (NICU) who have high rates of hyperbilirubinemia and thus are at risk for auditory neuropathy (AN).

Purpose: The purpose of this study was to determine whether recording parameters commonly used in click-evoked auditory brain stem response (ABR) are useful for recording cochlear microphonic (CM) and Wave I in infants at risk for AN. Specifically, we analyzed CM, summating potential (SP), and Waves I, III, and V. Read More

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http://dx.doi.org/10.3766/jaaa.17085DOI Listing
May 2018
5 Reads

Comparison of Cochlear Microphonics Magnitude with Broad and Narrow Band Stimuli in Healthy Adult Wistar Rats.

Iran J Child Neurol 2018 ;12(2):58-65

Department of Medical Physics and Biomedical Engineering, Schoolof Medicine, Tehran University of Medical Sciences, Tehran, Iran.

Objective: Cochlear microphonic (CM) is a cochlear AC electric field, recorded within, around, and remote from its sources. Nowadays it can contribute to the differential diagnosis of different auditory pathologies such as auditory neuropathy spectrum disorder (ANSD). This study compared CM waveforms (CMWs) and amplitudes with broad and narrow band stimuli in 25 healthy male young adults Wistar rats. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5904739PMC
January 2018
4 Reads

Deterioration in Distortion Product Otoacoustic Emissions in Auditory Neuropathy Patients With Distinct Clinical and Genetic Backgrounds.

Ear Hear 2019 Jan/Feb;40(1):184-191

Division of Hearing and Balance Research, National Institute for Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan.

Objectives: Auditory neuropathy (AN) is a clinical disorder characterized by the absence of auditory brainstem response and presence of otoacoustic emissions. A gradual loss of otoacoustic emissions has been reported for some cases of AN. Such cases could be diagnosed as cochlear hearing loss and lead to misunderstanding of the pathology when patients first visit clinics after the loss of otoacoustic emissions. Read More

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http://Insights.ovid.com/crossref?an=00003446-900000000-9894
Publisher Site
http://dx.doi.org/10.1097/AUD.0000000000000586DOI Listing
April 2019
6 Reads

TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad.

Mol Genet Genomic Med 2018 Apr 19. Epub 2018 Apr 19.

Department of Otolaryngology and Communication Sciences, Medical College of Wisconsin, Milwaukee, WI, USA.

Background: Sensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized.

Methods: A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD. Read More

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http://dx.doi.org/10.1002/mgg3.397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081214PMC
April 2018
7 Reads

Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model.

Biomed Res Int 2018 13;2018:6280969. Epub 2018 Feb 13.

Neurosensory Biophysics, INSERM UMR 1107, Clermont Auvergne University, Clermont-Ferrand, France.

Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or "hidden" synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18-30 stages when high-frequency auditory thresholds remained near-normal. Read More

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http://dx.doi.org/10.1155/2018/6280969DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832037PMC
September 2018
43 Reads