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Auditory Neuropathy

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Lin28 reprograms inner ear glia to a neuronal fate.

Judith S Kempfle, Ngoc-Nhi C Luu, Marco Petrillo, Reef Al-Asad, Andrea Zhang, Albert S B Edge,

Sensorineural hearing loss is irreversible and can be caused by loss of auditory neurons. Regeneration of neural cells from endogenous cells may offer a future tool to restore the auditory circuit and to enhance the performance of implantable hearing devices. Neurons and glial cells in the peripheral nervous system are ... Read more >>

Stem Cells (Stem cells (Dayton, Ohio))
[2020, :]

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Facial myokymia in inherited peripheral nerve hyperexcitability syndrome.

Clara Gontijo Camelo, André Macedo Serafim Silva, Cristiane Araújo Martins Moreno, Ciro Matsui-Júnior, Carlos Otto Heise, José Luiz Pedroso, Edmar Zanoteli,

Peripheral nerve hyperexcitability syndrome comprises a heterogeneous group of diseases, clinically characterised by myokymia, fasciculation, muscle cramps and stiffness. The causes are either immune mediated or non-immune mediated. Non-immune-mediated forms are mostly genetic, relating to two main genes: KCNQ2 and KCNA1 Patients with KCNQ2 gene mutations typically present with epileptic ... Read more >>

Pract Neurol (Practical neurology)
[2020, :]

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A novel variant in diaphanous homolog 1 (DIAPH1) as the cause of auditory neuropathy in a Chinese family.

Kan Wu, Hongyang Wang, Jing Guan, Lan Lan, Cui Zhao, Mengqian Zhang, Dayong Wang, Qiuju Wang,

OBJECTIVES:To determine the genetic cause of non-syndromic autosomal dominant deafness segregating in a Chinese Auditory neuropathy (AN) family. INTRODUCTION:AN is a genetically related rare disease characterized by sensorineural hearing loss and retention of hair cell function. Diaphanous Homolog 1 (DIAPH1) is the causative gene of DFNA1. To date, no evidence ... Read more >>

Int. J. Pediatr. Otorhinolaryngol. (International journal of pediatric otorhinolaryngology)
[2020, 133:109947]

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Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD).

Désirée Ehrmann-Müller, Daniela Back, Heike Kühn, Rudolf Hagen, Wafaa Shehata-Dieler,

INTRODUCTION:The present article shows long-term results in the hearing and speech development of children with auditory neuropathy spectrum disorder (ANSD). Some children were followed up for nearly 20 years, monitoring their progress through childhood into adulthood. METHODS:This retrospective study examined data from 10 children who were diagnosed and treated at ... Read more >>

Int. J. Pediatr. Otorhinolaryngol. (International journal of pediatric otorhinolaryngology)
[2020, 132:109938]

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The characteristics of monosyllable recognition in Mandarin-speaking patients with auditory neuropathy.

Liangwei Xu, Wei Chen, Shuolong Yuan, Shiming Yang, Fei Ji,

Background: A set of Chinese Mandarin monosyllable test lists has been widely used in clinical diagnosis, while the performance of the Mandarin-speaking patients with auditory neuropathy (AN) in the monosyllable tests was still unknown.Objectives: To analyze the characteristics of monosyllable recognition in Mandarin-speaking patients with AN.Materials and methods: Nineteen Mandarin-speaking ... Read more >>

Acta Otolaryngol. (Acta oto-laryngologica)
[2020, :1-8]

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Postpartum psychosis in peripartum cardiomyopathy: a case report.

Odhiambo Henry Owuor, James Amisi Akiruga, Jeremiah Laktabai, Samuel Ateya, Salwa Mohamed Omar,

BACKGROUND:This case report highlights the rare occurrence of postpartum psychosis in the setting of peripartum cardiomyopathy, which can have rare presentations like arrhythmias and pulmonary edema; and the challenges one should anticipate while managing these conditions together. Caution is advised whenever antipsychotic drugs are to be administered to a patient ... Read more >>

BMC Psychiatry (BMC Psychiatry)
[2020, 20(1):114]

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AIF knockdown induce apoptosis and mitochondrial dysfunction in cochlear spiral ganglion neurons in vitro.

Liang Zong, Jiandong Zhao, Wenming Wu, Jialing Wang, Deliang Huang, Mingbo Liu,

The underlying mechanism involved in auditory neuropathy spectrum disorder (ANSD) remains largely unclear. It has been previously reported that mutations in the apoptosis-inducing factor (AIF) gene are associated with auditory neuropathy and delayed peripheral neuropathy, which can eventually cause ANSD. In the present study, the regulatory effects of AIF knockdown ... Read more >>

Mol Med Rep (Molecular Medicine Reports)
[2020, 21(4):1910-1920]

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Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base.

Ryan A Bartholomew, Amir A Zamani, Grace S Kim, Jennifer C Alyono, Haley Steinert, Vera Fridman, Reza Sadjadi, Robert K Jackler, C Eduardo Corrales,

Objective:To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications. Methods:We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy diagnoses who had undergone gadolinium-enhanced magnetic resonance imaging (MRI) of the brain or skull ... Read more >>

Laryngoscope Investig Otolaryngol (Laryngoscope investigative otolaryngology)
[2020, 5(1):11-18]

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Enolate-forming compounds provide protection from platinum neurotoxicity.

Brian C Geohagen, Daniel A Weiser, David M Loeb, Lars U Nordstroem, Richard M LoPachin,

Cisplatin (CisPt) and other platinum (Pt)-based antineoplastic drugs (e.g., carboplatin, oxaliplatin) are highly effective and widely used in the treatment of solid tumors in both pediatric and adult patients. Although considered to be life-saving as a cancer treatment, Pt-based drugs frequently result in dose-limiting toxicities such as chemotherapy-induced peripheral neuropathies ... Read more >>

Chem. Biol. Interact. (Chemico-biological interactions)
[2020, 317:108961]

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Music and psychoacoustic perception abilities in cochlear implant users with auditory neuropathy spectrum disorder.

Mustafa Yüksel, Ayça Çiprut,

OBJECTIVE:Auditory neuropathy spectrum disorder (ANSD) is a condition wherein the pre-neural or cochlear outer hair cell activity is intact, but the neural activity in the auditory nerve is disrupted. Cochlear implant (CI) can be beneficial for subjects with ANSD; however, little is known about the music perception and psychoacoustic abilities ... Read more >>

Int. J. Pediatr. Otorhinolaryngol. (International journal of pediatric otorhinolaryngology)
[2020, 131:109865]

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A novel frameshift mutation in SOX10 causes Waardenburg syndrome with peripheral demyelinating neuropathy, visual impairment and the absence of Hirschsprung disease.

Elizabeth A Burke, Kyle E Reichard, Lynne A Wolfe, Brian P Brooks, John J DiGiovanna, Donald W Hadley, Tanya J Lehky, Andrea L Gropman, Cynthia J Tifft, William A Gahl, Camilo Toro, David Adams,

Waardenburg syndrome (WS) is a group of genetic disorders associated with varying components of sensorineural hearing loss and abnormal pigmentation of the hair, skin, and eyes. There exist four different WS subtypes, each defined by the absence or presence of additional features. One of the genes associated with WS is ... Read more >>

Am. J. Med. Genet. A (American journal of medical genetics. Part A)
[2020, :]

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Vestibular dysfunction in patients with auditory neuropathy detected by vestibular evoked myogenic potentials.

Juan Hu, Zichen Chen, Yuzhong Zhang, Yong Xu, Weijun Ma, Yan Zhang, Junli Wang, Yanfei Chen, Min Xu, Hui Yang, Qing Zhang,

OBJECTIVES:This study aimed to determine vestibular involvement in patients with auditory neuropathy (AN) using ocular vestibular evoked myogenic potential (oVEMP), cervical vestibular evoked myogenic potential (cVEMP), caloric tests, video Head Impulse Tests (vHIT), and Suppression Head Impulse Paradigm (SHIMP) tests. METHODS:Twenty-two patients with AN (study group) and 50 age-and-gender-matched healthy ... Read more >>

Clin Neurophysiol (Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology)
[2020, :]

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Long-term Follow-up of a Patient With Auditory Neuropathy and Normal Hearing Thresholds.

Travis White-Schwoch, Samira Anderson, Nina Kraus,

JAMA Otolaryngol Head Neck Surg (JAMA otolaryngology-- head & neck surgery)
[2020, :]

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Hidden Hearing Loss: A Disorder with Multiple Etiologies and Mechanisms.

David C Kohrman, Guoqiang Wan, Luis Cassinotti, Gabriel Corfas,

Hidden hearing loss (HHL), a recently described auditory disorder, has been proposed to affect auditory neural processing and hearing acuity in subjects with normal audiometric thresholds, particularly in noisy environments. In contrast to central auditory processing disorders, HHL is caused by defects in the cochlea, the peripheral auditory organ. Noise ... Read more >>

Cold Spring Harb Perspect Med (Cold Spring Harbor perspectives in medicine)
[2020, 10(1):]

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Cryptococcal meningitis and immune reconstitution inflammatory syndrome in a pediatric patient with HIV after switching to second line antiretroviral therapy: a case report.

Ssegujja Boniface Joseph Otto, Paul E George, Rebecca Mercedes, Nicolette Nabukeera-Barungi,

BACKGROUND:Cryptococcal meningitis (CCM) is a common and deadly disease among HIV-infected patients. Notable about CCM is its association with the immune reconstitution inflammatory syndrome (IRIS). Though it has been posited a switch from first to second-line antiretroviral therapy (ART) can induce CCM IRIS, a case presentation of CCM IRIS has ... Read more >>

BMC Infect. Dis. (BMC infectious diseases)
[2020, 20(1):68]

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Imaging findings in Möbius-Poland syndrome.

Vincent G Parenti, Xiaozhou Liu, Akshita Mehta, Rithvik Malireddy, Lydia A Sutherlun, Cory M Pfeifer,

Poland syndrome refers to a chest wall disorder in which there is a deficiency of the pectoral musculature. Möbius syndrome is a rare disorder in which there is absence or hypoplasia of the facial or abducens nerve, either unilaterally or bilaterally. Described here is a case in a newborn male ... Read more >>

Radiol Case Rep (Radiology Case Reports)
[2020, 15(4):379-381]

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Functional MRI study in a case of Charles Bonnet syndrome related to LHON.

V Vacchiano, C Tonon, M Mitolo, S Evangelisti, M Carbonelli, R Liguori, R Lodi, V Carelli, C La Morgia,

INTRODUCTION:Charles Bonnet syndrome is characterized by simple or complex visual hallucinations (VH) due to damage along the visual pathways. We report a functional MRI study of brain correlates of VH in the context of a severe optic atrophy in a patient with Leber's Hereditary Optic Neuropathy (LHON). CASE REPORT:A 62-year-old ... Read more >>

BMC Neurol (BMC neurology)
[2019, 19(1):350]

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Escherichia coli: a rare cause of meningitis in immuno-competent adult.

Maryam Zafar, Abubakar Tauseef, Muhammad Sohaib Asghar, Narmin Khan, Nabeeha Farooqui, Mustafa Dawood, Tanvir Alam, Durre Naman,

A 35-year-old male of Asian ethnicity presented with complaints of high-grade fever, severe headache, vomiting, generalized seizures, deteriorated conscious level and neck stiffness. After all baseline workup Computed Tomography (CT) scan of the brain was done, which turned out to be unremarkable. CT scan was then followed by lumbar puncture ... Read more >>

J Community Hosp Intern Med Perspect (Journal of community hospital internal medicine perspectives)
[2020, 10(1):69-72]

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Safety of Levodopa-Carbidopa Intestinal Gel Treatment in Patients with Advanced Parkinson's Disease Receiving ≥2000 mg Daily Dose of Levodopa.

Cindy Zadikoff, Werner Poewe, James T Boyd, Lars Bergmann, Horia Ijacu, Pavnit Kukreja, Weining Z Robieson, Janet Benesh, Angelo Antonini,

Background:Levodopa-carbidopa intestinal gel (LCIG) provides continuous levodopa administration and clinical benefits to patients with advanced Parkinson's disease (PD). This report evaluates long-term safety and efficacy of high-dose LCIG in PD patients. Methods:Data were collected from several prospective, phase III clinical studies and an observational registry. The phase III program (N = 412) ... Read more >>

Parkinsons Dis (Parkinson's disease)
[2020, 2020:9716317]

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Isolated auditory neuropathy at birth in congenital cytomegalovirus infection.

Fabio Natale, Mario De Curtis, Bianca Bizzarri, Maria Patrizia Orlando, Massimo Ralli, Giuseppina Liuzzi, Barbara Caravale, Francesco Franco, Aurelia Gaeta, Antonella Giancotti, Francesca Yoshie Russo, Rosaria Turchetta,

BACKGROUND:Congenital cytomegalovirus (cCMV) infection is the most frequent non-genetic cause of sensorineural hearing-loss (SNHL) (i.e., hearing loss due to a cochlear and/or auditory nerve damage). It is widely accepted that SNHL at birth, when associated to cCMV symptomatic infection involving the central nervous system, benefits from antiviral therapy started in ... Read more >>

Ital J Pediatr (Italian journal of pediatrics)
[2020, 46(1):3]

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Postoperative Intracochlear Electrocochleography in Pediatric Cochlear Implant Recipients: Association to Audiometric Thresholds and Auditory Performance.

Joseph Attias, David Ulanovski, Ohad Hilly, Tally Greenstein, Merav Sokolov, Suhail HabibAllah, Hen Mormer, Eyal Raveh,

OBJECTIVES:The aim of this study was to compare intracochlear-recorded cochlear microphonics (CM) responses to behavioral audiometry thresholds in young children, with congenital hearing loss, 2 to 5 years after cochlear implantation early in life. In addition, differences in speech and auditory outcomes were assessed among children with and without residual ... Read more >>

Ear Hear (Ear and hearing)
[2020, :]

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Peroxisomal Dysfunction in Neurological Diseases and Brain Aging.

Ndidi-Ese Uzor, Louise D McCullough, Andrey S Tsvetkov,

Peroxisomes exist in most cells, where they participate in lipid metabolism, as well as scavenging the reactive oxygen species (ROS) that are produced as by-products of their metabolic functions. In certain tissues such as the liver and kidneys, peroxisomes have more specific roles, such as bile acid synthesis in the ... Read more >>

Front Cell Neurosci (Frontiers in Cellular Neuroscience)
[2020, 14:44]

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Clinical and economic burden of invasive meningococcal disease: Evidence from a large German claims database.

Liping Huang, Olivia Denise Heuer, Sabrina Janßen, Dennis Häckl, Niklas Schmedt,

BACKGROUND:Limited data is available to describe clinical characteristics, long-term outcomes, healthcare resource use and the attributable costs of invasive meningococcal disease (IMD) in Germany. We aimed to examine demographic and clinical characteristics as well as healthcare resource use and related costs. METHODS:We conducted a retrospective cohort study based on the ... Read more >>

PLoS ONE (PloS one)
[2020, 15(1):e0228020]

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Analysis of gene variants in the GASH/Sal model of epilepsy.

Elena Díaz-Casado, Ricardo Gómez-Nieto, José M de Pereda, Luis J Muñoz, María Jara-Acevedo, Dolores E López,

Epilepsy is a complex neurological disorder characterized by sudden and recurrent seizures, which are caused by various factors, including genetic abnormalities. Several animal models of epilepsy mimic the different symptoms of this disorder. In particular, the genetic audiogenic seizure hamster from Salamanca (GASH/Sal) animals exhibit sound-induced seizures similar to the ... Read more >>

PLoS ONE (PloS one)
[2020, 15(3):e0229953]

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Ocular Hypotonia and Transient Decrease of Vision as a Consequence of Exposure to a Common Toad Poison.

Renato Pejic, Marija Simic Prskalo, Josip Simic,

The common toad produces venom (bufotoxin) that is produced in the parotid gland of the toad as well as in the skin. This toxic compound is a potent inhibitor of Na+/K+-ATPase activity. Physiological effects of bufotoxin are similar to those of digitalis and cause increased heart rate and muscle contractions. ... Read more >>

Case Rep Ophthalmol Med (Case reports in ophthalmological medicine)
[2020, 2020:2983947]

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