Auditory neuropathy

A hearing disorder in which sound enters the INNER EAR normally but the transmission of signals from the inner ear to the brain is impaired. Germline mutations in the OTOF gene have been identified for Auditory Neuropathy, Autosomal Recessive, 1 (OMIM: 601071)

Also Known As:

Networked: 265 relevant articles (8 outcomes, 40 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sensorineural Hearing Loss
2.	CAPOS syndrome
3.	Deafness (Deaf Mutism)
4.	Hearing Loss (Hearing Impairment)
5.	Jaundice (Icterus)

Experts

1.	Sharma, Anu: 5 articles (03/2021 - 05/2011)
2.	Buchman, Craig A: 5 articles (01/2017 - 06/2006)
3.	Kim, Lee-Suk: 5 articles (07/2013 - 09/2004)
4.	Rivas, Alejandro: 4 articles (04/2019 - 12/2012)
5.	Labadie, Robert F: 4 articles (10/2018 - 05/2002)
6.	Ji, Fei: 4 articles (01/2015 - 08/2008)
7.	Jeong, Sung-Wook: 4 articles (07/2013 - 10/2007)
8.	Cardon, Garrett: 3 articles (03/2021 - 05/2011)
9.	Choi, Byung Yoon: 3 articles (01/2021 - 11/2018)
10.	Qi, Yue: 3 articles (01/2021 - 04/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Auditory neuropathy:

1.	Ouabain (G Strophanthin)IBA 01/01/2016 - "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. " 01/01/2020 - "To study the effects of Lin28 on endogenous glial cells after loss of auditory neurons in vivo, we produced a model of auditory neuropathy by selectively damaging auditory neurons with ouabain. " 10/01/2019 - "The NSC cells were isolated from neonatal Balb/c mice and an auditory neuropathy gerbil model was established by microinjection of ouabain. " 02/01/2019 - "We used 8-week-old CBA/CaJ male mice and created ouabain-induced auditory neuropathy. " 11/01/2015 - "Estimates of f(Tr) before and after inducing auditory neuropathy-loss of auditory nerve responses with preserved hair cell responses from neurotoxic treatment with ouabain-showed that the neural excitation from low-frequency tones contributes to the magnitude of f(Tr) but not the sigmoidal, saturating, nonlinear morphology."
2.	trigonellineIBA 08/29/2008 - "This study examined the auditory neuropathy induced by diabetes and investigated the action of coffee, trigonelline, and caffeine to determine whether they improved diabetic auditory neuropathy in mice. " 04/01/2009 - "The present findings demonstrate that high-dose pyridoxine administration can be used to produce a new mouse model for AN, and coffee or trigonelline as a main active compound of coffee extract can potentially facilitate recovery from pyridoxine-induced auditory neuropathy." 01/01/2012 - "Trigonelline has hypoglycemic, hypolipidemic, neuroprotective, antimigraine, sedative, memory-improving, antibacterial, antiviral, and anti-tumor activities, and it has been shown to reduce diabetic auditory neuropathy and platelet aggregation. "
3.	CoffeeFDA Link 08/29/2008 - "These findings demonstrate that diabetes can produce a mouse model of auditory neuropathy and that coffee consumption potentially facilitates recovery from diabetes-induced auditory neuropathy. " 08/29/2008 - "Coffee improves auditory neuropathy in diabetic mice." 04/01/2009 - "High-dosage pyridoxine-induced auditory neuropathy and protection with coffee in mice." 08/29/2008 - "This study examined the auditory neuropathy induced by diabetes and investigated the action of coffee, trigonelline, and caffeine to determine whether they improved diabetic auditory neuropathy in mice. " 04/01/2009 - "The present findings demonstrate that high-dose pyridoxine administration can be used to produce a new mouse model for AN, and coffee or trigonelline as a main active compound of coffee extract can potentially facilitate recovery from pyridoxine-induced auditory neuropathy."
4.	Caffeine (No Doz)FDA LinkGeneric 10/01/2022 - "The effects of caffeine on the auditory and vestibular system have been investigated under normal and pathological conditions, such as acoustic trauma, ototoxicity, auditory neuropathy, and vestibular disorders, using various tests. " 08/29/2008 - "This study examined the auditory neuropathy induced by diabetes and investigated the action of coffee, trigonelline, and caffeine to determine whether they improved diabetic auditory neuropathy in mice. "
5.	Rutin (Rutoside)IBA 08/01/2021 - "In this study, we assessed the efficacy of rutin in treating diabetic auditory neuropathy in an experimental rat model. " 08/01/2021 - "Rutin shows promise as a treatment modality for diabetic auditory neuropathy, but more trials are warranted for its clinical application." 08/01/2021 - "Significant improvements in auditory neuropathy were observed in the rutin-treated groups in comparison with the diabetic group (P<0.05). " 08/01/2021 - "Effect of Rutin on Diabetic Auditory Neuropathy in an Experimental Rat Model."
6.	TaurineFDA Link 10/01/2019 - "Transplantation of cochlear NSCs combined with taurine significantly improved ouabain-induced auditory neuropathy in gerbils. "
7.	BilirubinIBA 08/01/2019 - "Therefore, auditory neuropathy is the most important or even the only clinical symptom of bilirubin neurological dysfunction. " 01/01/2016 - "The auditory pathway is highly sensitive to the effects of elevated total serum/plasma bilirubin (TB) levels, with damage manifesting clinically as auditory neuropathy spectrum disorder. " 02/01/2015 - "This paper explores the societal impact of bilirubin-induced auditory impairments, inclusive of hearing impairments and auditory neuropathy spectrum disorders, on these infants, their families, and on the community in resource-limited countries (per capita income of US$6,000 or less). " 11/01/2012 - "Bilirubin induces auditory neuropathy in neonatal guinea pigs via auditory nerve fiber damage." 10/01/2008 - "Mean total serum bilirubin, serum Tau, and S100B levels of infants who manifested auditory neuropathy, neurologic abnormalities, or electroencephalogram abnormalities were significantly higher than in infants without these abnormalities (P < 0.05). "
8.	Biomarkers (Surrogate Marker)IBA 12/13/2012 - "In individuals with auditory neuropathy due to both axonal and demyelinating mechanisms the assessment provides a measure of functional hearing ability, a biomarker capable of tracking the natural history of progressive disease and a potential means of evaluating the effectiveness of interventions." 10/01/2021 - "This study aimed to explore the central auditory system development in children with auditory neuropathy spectrum disorder (ANSD) using cochlear implants (CIs) and to find the correlation of CAEP biomarkers with speech perception. " 01/01/2021 - "The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). " 05/01/2011 - "Clinical Application of the P1 Cortical Auditory Evoked Potential Biomarker in Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder." 12/01/2015 - "Finally, we present clinical case studies in which the P1 CAEP biomarker proved useful in clinical decision-making regarding intervention in cases of single-sided deafness, auditory neuropathy spectrum disorder, mild hearing loss and hypoplastic auditory nerve."
9.	Proteins (Proteins, Gene)FDA Link 01/01/2023 - "Transgenic mutant mice exhibited late-onset auditory neuropathy, and their altered XKR8 protein localization in the inner ear confirmed the damaging effects of this variant. " 07/01/2006 - "Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy." 09/01/2013 - "DFNB59 gene encodes the protein pejvakin (PJVK) and its mutations cause autosomal recessive auditory neuropathy as well as other forms of sensorineural hearing loss. " 10/01/2007 - "The current case and a review of available reports indicate that auditory neuropathy or neural dys-synchrony may be another phenotype of the condition possibly related to abnormal expression of the protein beta-catenin mapped to 5p. " 12/03/2022 - "To determine the genetic etiology of deafness in the pedigree with a heterozygous missense variant in the gene TNN encoding tenascin-W associated with autosomal recessive nonsyndromic auditory neuropathy, the cochlear expression of tenascin-W was evaluated at mRNA and protein levels in mice, and Tnn knock out mice were generated and utilized to study the function of Tnn in the auditory system. "
10.	Pyridoxine (Pyridoxin)FDA LinkGeneric 06/01/2019 - "Therefore, the present study explored a direct mechanism underlying the effects of pyridoxine on auditory neuropathy in organ of Corti (OC) explants ex vivo and cochlear neuroblast cell line, VOT-33 in vitro. " 06/01/2019 - "Auditory neuropathy due to toxicity mechanism of pyridoxine has not yet been fully documented. " 06/01/2019 - "Pyridoxine Preferentially Induces Auditory Neuropathy Through Mitochondrial Dysfunction and Endoplasmic Reticulum Stress-Mediated Apoptosis." 04/01/2009 - "High-dosage pyridoxine-induced auditory neuropathy and protection with coffee in mice." 04/01/2009 - "The present findings demonstrate that high-dose pyridoxine administration can be used to produce a new mouse model for AN, and coffee or trigonelline as a main active compound of coffee extract can potentially facilitate recovery from pyridoxine-induced auditory neuropathy."

Therapies and Procedures

1.	Cochlear Implantation 06/01/2006 - "Prior investigations showed good results due to replacing a synchronous impulse by means of cochlear implantation in patients with auditory neuropathy." 01/01/2005 - "In many sensory-neural profound hearing losses as well as auditory neuropathy cases good results may be obtained by cochlear implantation. " 12/01/2019 - "The pediatric patients, who underwent cochlear implantation with the diagnosis of auditory neuropathy at İzmir Bozyaka Training and Research Hospital ENT Clinic between January 1997 and May 2017, were included to the study. " 12/01/2019 - "The aim of this study is to evaluate the quality of life in pediatric patients with auditory neuropathy according to the perspective of their parents after cochlear implantation. " 12/01/2018 - "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). "
2.	Cochlear Implants (Cochlear Implant) 02/01/2023 - "The primary aim of this study was to analyze the benefit of cochlear implants for patients with auditory neuropathy. " 07/01/2013 - "Auditory neuropathy spectrum disorder: predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification." 08/01/2011 - "The results of this study show that the temporal processing ability in children with auditory neuropathy (AN) can be restored to some degree by electrical stimulation through a cochlear implant. " 02/01/1999 - "In this study the results observed in a child with auditory neuropathy who received a cochlear implant are presented and compared with those of a matched group of children who were recipients of implants. " 10/04/2023 - "The objective of this overview of systematic reviews (SRs; umbrella review) was to systematically summarize and critically appraise current evidence of cochlear implant (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). "
3.	Hearing Aids (Hearing Aid) 09/01/2021 - "The objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6). " 10/01/2017 - "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). " 07/01/2017 - "Introduction  The studies on hearing aid benefit in individuals with auditory neuropathy spectrum disorder (ANSD) shows limited benefit. " 12/01/2015 - "The purpose of the present study was to investigate the relationship between cortical processing of speech and benefit from hearing aids in individuals with auditory dys-synchrony. " 09/01/2021 - "To investigate phrase perception and subjective quality preference of temporal enhancement-based speech processing strategies: Deep-band modulation and Stretching, and hearing aid processed speech in adolescents with late-onset Auditory Neuropathy. "
4.	Transplantation 02/01/2019 - "Transplantation of human limbus-derived mesenchymal stromal cells via occipital approach improves hearing in animal auditory neuropathy." 11/08/2012 - "In a major breakthrough, Chen and colleagues recently reported the restoration of hearing thresholds by up to 46% following the transplantation of human pluripotent stem cells in a rodent auditory neuropathy model. " 04/01/2011 - "Transplantation of neural differentiated human mesenchymal stem cells into the cochlea of an auditory-neuropathy guinea pig model." 06/01/2008 - "Transplantation of mouse embryonic stem cells into the cochlea of an auditory-neuropathy animal model: effects of timing after injury." 02/01/2019 - "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. "
5.	Therapeutics 01/17/2023 - "Current Advances in Gene Therapies of Genetic Auditory Neuropathy Spectrum Disorder." 10/01/2022 - "New therapeutic approaches, such as stem cell therapy and gene therapy are the future trends in the treatment of auditory neuropathy. " 01/01/2020 - "This suggests the potential for inner ear glia to be converted into neurons as a regeneration therapy for neural replacement in auditory neuropathy." 12/01/2019 - "Evaluation and therapy outcome in children with auditory neuropathy spectrum disorder (ANSD)." 04/01/2014 - "In light of our findings we support the combined medical therapy as an option for patients with auditory neuropathy spectrum disorder."