67 results about "HL - Hearing loss" patented technology

The invention defines interdependent biofactors and biomolecules, and clinically useful formulations that are comprised of them. The active agents are demonstrated to be complementary in their physiologic functions especially as these relate to the quenching of free radicals and to the support of endothelial physiology, the reduction of hyperinsulinemia and improvements in vascular health. The active components of the invention are selected for inclusion in precise combinations specifically because they improve these various conditions and physiological functions, and by so doing reduce a variety of risks associated with hearing loss and tinnitus. The resulting enhancement of general systemic vascular health, improvement in local VIIIth nerve vascular health, modulation of conditions surrounding blood fluid dynamics, the consequences of hyperinsulinemia, and improvements in free radical defenses, all reduce the potential for cochlear hair cell death and VIIIth nerve atrophy, and the hearing loss and possible deafness that accompany them.

Compounds, compositions and methods for the treatment of retinal degenerative diseases, such as retinitis pigmentosa, Leber's congenital Amaurosis, Syndromic retinal degenerations, age-related macular degeneration and Usher Syndrome, and hearing loss associated with Usher Syndrome are described herein.

The invention relates to BCAT inhibitors and the use thereof for treating or preventing neuronal loss associated with stroke, ischemia, CNS trauma, hypoglycemia and surgery, as well as treating neurodegenerative diseases including Alzheimer's disease, amyotrophic lateral sclerosis, Huntington's disease and Down's syndrome, treating or preventing the adverse consequences of the overstimulation of the excitatory amino acids, treating anxiety, psychosis, convulsions, aminoglycoside antibiotics-induced hearing loss, migraine headache, chronic pain, neuropathic pain, Parkinson's disease, diabetic retinopathy, glaucoma, CMV retinitis, urinary incontinence, opioid tolerance or withdrawal, and inducing anesthesia, as well as for enhancing cognition.

The invention relates to BCAT inhibitors and the use thereof for treating or preventing neuronal loss associated with stroke, ischemia, CNS trauma, hypoglycemia and surgery, as well as treating neurodegenerative diseases including Alzheimer's disease, amyotrophic lateral sclerosis, Huntington's disease and Down's syndrome, treating or preventing the adverse consequences of the overstimulation of the excitatory amino acids, treating anxiety, psychosis, convulsions, aminoglycoside antibiotics-induced hearing loss, migraine headache, chronic pain, neuropathic pain, Parkinson's disease, diabetic retinopathy, glaucoma, CMV retinitis, urinary incontinence, opioid tolerance or withdrawal, and inducing anesthesia, as well as for enhancing cognition.

The invention provides a method of preventing or treating hearing loss in a mammal in need thereof. The method involves administering to the mammal an agent that increases intracellular NAD+ in the mammal. The invention also provides a method of determining if a compound acts as a neuroprotective agent.

The invention is directed, in part, to compounds of structure (I)to treat or prevent hearing loss. Compounds of the present invention also promote sensory hair cell regeneration. Particular compositions comprise compounds of structure (I), and optionally one or more small molecules that increase the proliferation of supporting cells.

A composition and method of treatment of neuromuscular, neuromuscular degenerative, neurodegenerative, autoimmune, developmental, traumatic, hearing loss related, and / or metabolic diseases, including spinal muscular atrophy (SMA) syndrome (SMA1, SMA2, SMA3, and SMA4, also called Type I, II, III and IV), traumatic brain injury (TBI), concussion, keratoconjunctivitis sicca (Dry Eye Disease), glaucoma, Sjogren's syndrome, rheumatoid arthritis, post-LASIK surgery, anti-depressants use, Wolfram Syndrome, and Wolcott-Rallison syndrome. The composition is selected from the group consisting of 2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP, bipartite 2,3-dinitrophenol, 2,4-dinitrophenol, 2,5-dinitrophenol, 2,6-dinitrophenol, 3,4-dinitrophenol, or 3,5-dinitrophenol (2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP) prodrugs; Gemini prodrugs, bioprecursor molecules, and combinations thereof. A dose of the composition for treatment of neurodegenerative diseases may be from about 0.01 mg / kg of body weight to about 50 mg / kg of body weight of the patient in need of treatment. A dose of the composition for treatment of metabolic diseases may be from about 1 mg / 70 kg of body weight to about 100 mg / 70 kg of body weight of the patient in need of treatment, and a maximum dose per day is about 200 mg / 70 kg of body weight of the patient in need of treatment.

Provided herein are compositions that include at least two different nucleic acid vectors, where each of the at least two different vectors includes a coding sequence that encodes a different portionof an otoferlin protein, and the use of these compositions to treat hearing loss in a subject.

The invention relates to BCAT inhibitor compounds of formula (I) and use thereof for treating or preventing neuronal loss associated with stroke, ischemia, CNS trauma, hypoglycemia and surgery, as well as treating neurodegenerative diseases including Alzheimer's disease, amyotrophic lateral sclerosis, Huntington's disease and Down's syndrome, treating or preventing the adverse consequences of the overstimulation of the excitatory amino acids, treating anxiety, psychosis, convulsions, aminoglycoside antibiotics-induced hearing loss, migraine headache, chronic pain, neuropathic pain, Parkinson's disease, diabetic retinopathy, glaucoma, CMV retinitis, urinary incontinence, opioid tolerance or withdrawal, and inducing anesthesia, as well as for enhancing cognition.

Described herein are optimally-modified GJB2 cDNA and associated genetic elements for use in recombinant adeno-associated virus (rAAV)-based gene therapy for the treatment of genetic hearing loss.

A system and a method for a healthcare provider to easily, accurately and efficiently provide a diagnostic hearing assessment, for their patients, in a way that accurately detects hearing loss and provides recommendations for seeking further assistance from remotely-located ear specialists is provided.

The disclosure provides polynucleotides containing regions of the Myosin 15 (Myo15) promoter, as well as vectors containing the same, that can be used to promote expression of a transgene specifically in hair cells. The polynucleotides described herein may be operably linked to a transgene, such as a transgene encoding a therapeutic protein, so as to promote hair cell-specific expression of the transgene. The polynucleotides described herein may be operably linked to a therapeutic transgene and used for the treatment of subjects having or at risk of developing hearing loss or vestibular dysfunction.

The present invention relates to the prophylaxis of acute noise-induced hearing loss by administering a compound of formula (I): wherein: W is group (Wa), group (Wb) or group (Wc):

The invention provides sgRNA of a targeted Tmc1 mutant (c.1235T > A; p.M412K), an expression vector, a CRISPRs-CasRx system, a kit and application of the sgRNA, and belongs to the technical field of genetic engineering. The sequence of sgRNA of the targeted Tmc1 mutant is shown as SEQIDNO.3; and CasRx containing the sgRNA can enable a Tmc1Bth transcript to be reduced by 82% under the condition that wild Tmc1 + is not interfered, CasRx based on an AAV-PHP.eB carrier is injected into inner ears of newborn Beethoven mice, it is found that Tmc1Bth is reduced by 70% within two weeks, and miss-target is not detected in a whole transcriptome. The sgRNA targeted Tmc1 mutant provided by the invention is high in specificity, and can improve the survival rate of Tmc1Bth mutant mouse hair cells, so that the form of cilia bundles is recovered, the mechanical transduction current is reduced, and progressive hearing loss is remarkably improved.

Polyamidoamine, its partially degraded products or its complexes-Math1 gene nanoparticles, method for preparing the same and use thereof, the gene nanoparticles can be produced through complex coacervating of polyamidoamine, or polyamidoamine complexes and a Math1 gene-containing plasmid. The gene nanoparticles are controllable in particle size, uniform in size, favorable for surface modification, can enhance the ability of expression and delivery of the Math1 gene, and is useful in a sensorineural hearing loss caused by hair cells loss due to noise, drug toxicity etc.

Methods and compositions using an inhibitor of EGFR signaling for prevention or an inhibitor of EGFR signaling and a nucleic acid molecule encoding an atonal-associated factor for treatment of hearingloss are described.

A composition and method of treatment of neuromuscular, neuromuscular degenerative, neurodegenerative, autoimmune, developmental, traumatic, hearing loss related, and / or metabolic diseases, including spinal muscular atrophy (SMA) syndrome (SMA1, SMA2, SMA3, and SMA4, also called Type I, II, III and IV), traumatic brain injury (TBI), concussion, keratoconjunctivitis sicca (Dry Eye Disease), glaucoma, Sjogren's syndrome, rheumatoid arthritis, post-LASIK surgery, anti-depressants use, Wolfram Syndrome, and Wolcott-Rallison syndrome. The composition is selected from the group consisting of 2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP, bipartite 2,3-dinitrophenol, 2,4-dinitrophenol, 2,5-dinitrophenol, 2,6-dinitrophenol, 3,4-dinitrophenol, or 3,5-dinitrophenol (2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP) prodrugs; Gemini prodrugs, bioprecursor molecules, and combinations thereof. A dose of the composition for treatment of neurodegenerative diseases may be from about 0.01 mg / kg of body weight to about 50 mg / kg of body weight of the patient in need of treatment. A dose of the composition for treatment of metabolic diseases may be from about 1 mg / 70 kg of body weight to about 100 mg / 70 kg of body weight of the patient in need of treatment, and a maximum dose per day is about 200 mg / 70 kg of body weight of the patient in need of treatment.

A system and a method for a healthcare provider to easily, accurately and efficiently provide a hearing test and a pressure test, i.e., a hearing assessment, for their patients, in a way that accurately detects hearing loss and provides recommendations for seeking further assistance from qualified hearing health specialists is provided.

The invention provides a non-syndromic hearing loss related gene mutant and application thereof, and particularly provides an MYO15A gene mutant and application thereof. Compared with a wild type MYO15A gene, the gene mutant has the c.10419-10423 del CAGCT mutation and / or the c.8791 del T mutation. The gene mutant is detectable, and by detecting whether the gene mutant is present in a biological sample or not, whether a biological sample suffers from non-syndromic hearing loss or not can be effectively detected. Detection and research of the hereditary hearing loss disease are expanded and perfected through discovery of the gene mutant, and a new detection site and a new detection method and way are provided for diagnosis or treatment of the disease.

Methods of treating Alport syndrome and other diseases associated with a type IV collagen deficiency, and preventing hearing loss associated the same, are provided, comprising administering a compound having structure (I),or a pharmaceutically acceptable salt thereof, or administering a pharmaceutical composition comprising the compound of structure (I) or pharmaceutically acceptable salt thereof.

The present invention relates to the prophylaxis of acute noise-induced hearing loss by administering a compound of formula (I): wherein: W is group (Wa), group (Wb) or group (Wc):

A system and a method for a healthcare provider to easily, accurately and efficiently provide a diagnostic hearing assessment, for their patients, in a way that accurately detects hearing loss and provides recommendations for seeking further assistance from remotely-located ear specialists is provided.

In certain embodiments the present invention provides a method of treating hearing loss comprising: (a) administering a gene suppression agent that suppresses both copies of an endogenous gene causing the hearing loss; and (b) administering an exogenous wild-type allele engineered to resist suppression by the gene suppression agent. The present invention provides in certain embodiments a method of treating a genetic hearing loss (GHL) in a patient in need thereof comprising: (a) identifying a mutation in a GHL-causing gene, wherein the mutation causes GHL in the patient; and (b) administering to the patient a pharmaceutical composition comprising a therapeutic miRNA and a pharmaceutically acceptable carrier, wherein the GHL therapeutic miRNA is of 18 to 25 nucleotides in length and knocks-down the GHL-causing gene function at a higher level than it knocks-down gene function in a corresponding wild-type gene.

The invention provides an SLC26A4 gene mutant and application thereof. The invention provides a gene mutation. Compared with a wild type SLC26A4 gene, the gene mutation has c.128delG mutation and / or (formula sees the description) mutation. The gene mutation is detectable, and whether a biological sample suffers from non-syndrome deafness can be effectively detected by detecting whether the gene mutation exists in the biological sample. By detecting the gene mutation, detection and research of hereditary hearing loss diseases are expanded and perfected, and a new detection site and a new detection method and way are provided for diagnosis or treatment of the diseases.

Methods and compositions for treating and preventing toxic side effects of platinum-based chemotherapy agents are disclosed, in which transplatin is administered to a subject. Transplatin is shown to have protective effects against cisplatin-induced ototoxicity, nephotoxicity and neurotoxicity. Anti-inflammatory activity of transplatin is demonstrated and methods and compositions for treating and preventing inflammatory pain are described.

The present invention concerns peptides and nucleic acids encoding the peptides, and their use for modulating large conductance Ca2+ activated K+ (BK) channel activity in cells; for treating conditions such as presbycusis (age-related hearing loss), audiogenic seizures, alcohol addiction, cancer, and neurodegenerative disease; and for delivering a cargo moiety to the brain of a subject through the blood-brain barrier.

Aspects of the disclosure relate to compositions and methods useful for treating hereditary hearing loss, for example, Usher syndrome type 3A or nonsyndromic hearing loss and deafness (DFNB1). In some embodiments, the disclosure provides isolated nucleic acids, vectors, and rAAV.9.PHP.B comprising a transgene encoding a Clarin-1 or a GJB2, and methods of treating hearing loss using the same.

The present invention relates to a composition for preventing or treating hearing loss, containing an HMG-CoA reductase inhibitor and, more specifically, to a composition for preventing or treating diabetes-mediated hearing loss, containing, as an active ingredient(s), one or more types selected from the group consisting of atorvastatin, fluvastatin, lovastatin, pitavastatin, pravastatin, rosuvastatin and simvastatin. A pharmaceutical composition containing an HMG-CoA reductase inhibitor, according to the present invention, inhibits auditory cell death caused by diabetes, and thus is very useful as an agent for preventing or treating diabetes-mediated sensorineural hearing loss.

A composition and method of treatment of neuromuscular, neuromuscular degenerative, neurodegenerative, autoimmune, developmental, traumatic, hearing loss related, and / or metabolic diseases, including spinal muscular atrophy (SMA) syndrome (SMA1, SMA2, SMA3, and SMA4, also called Type I, II, III and IV), traumatic brain injury (TBI), concussion, keratoconjunctivitis sicca (Dry Eye Disease), glaucoma, Sjogren's syndrome, rheumatoid arthritis, post-LASIK surgery, anti-depressants use, Wolfram Syndrome, and Wolcott-Rallison syndrome. The composition is selected from the group consisting of 2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP, bipartite 2,3-dinitrophenol, 2,4-dinitrophenol, 2,5-dinitrophenol, 2,6-dinitrophenol, 3,4-dinitrophenol, or 3,5-dinitrophenol (2,3-DNP, 2,4-DNP, 2,5-DNP, 2,6-DNP, 3,4-DNP, or 3,5-DNP) prodrugs; Gemini prodrugs, bioprecursor molecules, and combinations thereof. A dose of the composition for treatment of neurodegenerative diseases may be from about 0.01 mg / kg of body weight to about 50 mg / kg of body weight of the patient in need of treatment. A dose of the composition for treatment of metabolic diseases may be from about 1 mg / 70 kg of body weight to about 100 mg / 70 kg of body weight of the patient in need of treatment, and a maximum dose per day is about 200 mg / 70 kg of body weight of the patient in need of treatment.

The disclosure provides polynucleotides containing outer hair cell-specific promoters, as well as vectors containing the same, that can be used to promote expression of a transgene specifically in outer hair cells. The polynucleotides described herein may be operably linked to a transgene, such as a transgene encoding a therapeutic protein, so as to promote outer hair cell-specific expression of the transgene. The polynucleotides described herein may be operably linked to a therapeutic transgene and used for the treatment of subjects having or at risk of developing hearing loss.