Stem Cell Treatment Hearing Loss

Stem Cell Treatment Hearing Loss at SIRM

Two Types:

1. Conductive hearing loss

A conductive hearing impairment is present when the sound is not reaching the inner ear, the cochlea. This can be due to external ear canal malformation, dysfunction of the eardrum or malfunction of the bones of the middle ear. The ear drum may show defects from small to total resulting in hearing loss of different degree. Scar tissue after ear infections may also make the ear drum dysfunction as well as when it is retracted and adherent to the medial part of the middle ear.

Dysfunction of the three small bones of the middle ear; hammer, anvil and stapes may result in conductive hearing loss. The mobility of the ossicles may be impaired of different reasons and disruption of the ossicular chain due to trauma, infection or anchylosis may also result in hearing loss.

2. Sensorineural hearing loss

A sensorineural hearing loss is one resulting from dysfunction of the inner ear, the cochlea, the nerve that transmits the impulses from the cochlea to the hearing centre in the brain or damage in the brain. The most common reason for sensorineural hearing impairment is damage to the hair cells in the cochlea. As we grow older the hair cells degenerate and lose their function, and our hearing deteriorates. Depending on the definition it could be estimated that more than 50% of the population over the age of 70 has an impaired hearing. Impaired hearing is the most common physical handicap in the industrialized world.

Another common reason for hearing loss due to hair cell damage is noise-induced hearing loss. These types of hearing loss are often most pronounced in the high frequency range. This will often interfere with speech understanding, as it is in the high frequency range that we find the consonant sounds that are most important especially in noisy surroundings. Head trauma, ear infections, tumours and ototoxic drugs such as gentamyacin are other reasons for sensorineural hearing loss.

Mixed hearing loss

Mixed hearing loss is a combination of the two types discussed above. Chronic ear infection that is a fairly common diagnosis could result in a defect ear drum and/or middle ear ossicle damages.

Stem Cell Treatment for Hearing Loss

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Related Articles [Pathophysiology of hearing loss : Classification and treatment options]. HNO. 2017 Apr;65(4):290-297 Authors: Kral A Abstract From the therapeutic perspective, the etiology and pathophysiology of hearing loss can be classified based on the extent of the primary cause. Hearing loss can have very different consequences for cell preservation in the organ of Corti and the spiral ganglion. These not only have implications for prosthetic therapy outcome, but may also influence the potential for future causal molecular therapies. Etiologies leading to deficits that are limited to one or a few molecules without having an effect on cell survival have the greatest potential for future causal therapy using molecular and cellular approaches. Preliminary success for molecular therapy was recently reported in animal experiments. Unfortunately, the incidence of these types of hearing loss is very low and in the future the therapy of hearing loss will therefore also require several different approaches. In addition to peripheral pathophysiology, hearing loss has consequences on the functioning of the brain, which can vary greatly due to individual adaptation to the situation without hearing. The authors therefore argue for individualization of the diagnostics and therapy that focus not only the symptom of hearing loss, but also the individual pathophysiology and consequences. Only with individualized therapy can the success of treating hearing disorders be significantly improved. PMID: 27299892 [PubMed - indexed for MEDLINE]
Related Articles Structural changes in the inner ear over time studied in the experimentally deafened guinea pig. J Neurosci Res. 2017 Mar;95(3):869-875 Authors: Fransson A, Ulfendahl M Abstract Today a cochlear implant (CI) may significantly restore auditory function, even for people with a profound hearing loss. Because the efficacy of a CI is believed to depend mainly on the remaining population of spiral ganglion neurons (SGNs), it is important to understand the timeline of the degenerative process of the auditory neurons following deafness. Guinea pigs were transtympanically deafened with neomycin, verified by recording auditory brainstem responses (ABRs), and then sacrificed at different time points. Loss of SGNs as well as changes in cell body and nuclear volume were estimated. To study the effect of delayed treatment, a group of animals that had been deaf for 12 weeks was implanted with a stimulus electrode mimicking a CI, after which they received a 4-week treatment with glial cell-derived neurotrophic factor (GDNF). The electrical responsiveness of the SGNs was measured by recording electrically evoked ABRs. There was a rapid degeneration during the first 7 weeks, shown as a significant reduction of the SGN population. The degenerative process then slowed, and there was no difference in the amount of remaining neurons between weeks 7 and 18. © 2016 The Authors Journal of Neuroscience Research Published by Wiley Periodicals, Inc. PMID: 27400677 [PubMed - indexed for MEDLINE]
Related Articles Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe's disease). J Neurosci Res. 2016 Nov;94(11):1007-17 Authors: Bradbury AM, Bagel JH, Jiang X, Swain GP, Prociuk ML, Fitzgerald CA, O'Donnell PA, Braund KG, Ory DS, Vite CH Abstract Globoid cell leukodystrophy (GLD), or Krabbe's disease, is a debilitating and always fatal pediatric neurodegenerative disease caused by a mutation in the gene encoding the hydrolytic enzyme galactosylceramidase (GALC). In the absence of GALC, progressive loss of myelin and accumulation of a neurotoxic substrate lead to incapacitating loss of motor and cognitive function and death, typically by 2 years of age. Currently, there is no cure. Recent convincing evidence of the therapeutic potential of combining gene and cell therapies in the murine model of GLD has accelerated the requirement for validated markers of disease to evaluate therapeutic efficacy. Here we demonstrate clinically relevant and quantifiable measures of central (CNS) and peripheral (PNS) nervous system disease progression in the naturally occurring canine model of GLD. As measured by brainstem auditory-evoked response testing, GLD dogs demonstrated a significant increase in I-V interpeak latency and hearing threshold at all time points. Motor nerve conduction velocities (NCVs) in GLD dogs were significantly lower than normal by 12-16 weeks of age, and sensory NCV was significantly lower than normal by 8-12 weeks of age, serving as a sensitive indicator of peripheral nerve dysfunction. Post-mortem histological evaluations confirmed neuroimaging and electrodiagnostic assessments and detailed loss of myelin and accumulation of storage product in the CNS and the PNS. Additionally, cerebrospinal fluid psychosine concentrations were significantly elevated in GLD dogs, demonstrating potential as a biochemical marker of disease. These data demonstrate that CNS and PNS disease progression can be quantified over time in the canine model of GLD with tools identical to those used to assess human patients. © 2016 Wiley Periodicals, Inc. PMID: 27638585 [PubMed - indexed for MEDLINE]
Related Articles Biohybrid cochlear implants in human neurosensory restoration. Stem Cell Res Ther. 2016 Oct 07;7(1):148 Authors: Roemer A, Köhl U, Majdani O, Klöß S, Falk C, Haumann S, Lenarz T, Kral A, Warnecke A Abstract BACKGROUND: The success of cochlear implantation may be further improved by minimizing implantation trauma. The physical trauma of implantation and subsequent immunological sequelae can affect residual hearing and the viability of the spiral ganglion. An ideal electrode should therefore decrease post-implantation trauma and provide support to the residual spiral ganglion population. Combining a flexible electrode with cells producing and releasing protective factors could present a potential means to achieve this. Mononuclear cells obtained from bone marrow (BM-MNC) consist of mesenchymal and hematopoietic progenitor cells. They possess the innate capacity to induce repair of traumatized tissue and to modulate immunological reactions. METHODS: Human bone marrow was obtained from the patients that received treatment with biohybrid electrodes. Autologous mononuclear cells were isolated from bone marrow (BM-MNC) by centrifugation using the Regenlab™ THT-centrifugation tubes. Isolated BM-MNC were characterised using flow cytometry. In addition, the release of cytokines was analysed and their biological effect tested on spiral ganglion neurons isolated from neonatal rats. Fibrin adhesive (Tisseal™) was used for the coating of silicone-based cochlear implant electrode arrays for human use in order to generate biohybrid electrodes. Toxicity of the fibrin adhesive and influence on insertion, as well on the cell coating, was investigated. Furthermore, biohybrid electrodes were implanted in three patients. RESULTS: Human BM-MNC release cytokines, chemokines, and growth factors that exert anti-inflammatory and neuroprotective effects. Using fibrin adhesive as a carrier for BM-MNC, a simple and effective cell coating procedure for cochlear implant electrodes was developed that can be utilised on-site in the operating room for the generation of biohybrid electrodes for intracochlear cell-based drug delivery. A safety study demonstrated the feasibility of autologous progenitor cell transplantation in humans as an adjuvant to cochlear implantation for neurosensory restoration. CONCLUSION: This is the first report of the use of autologous cell transplantation to the human inner ear. Due to the simplicity of this procedure, we hope to initiate its widespread utilization in various fields. PMID: 27717379 [PubMed - indexed for MEDLINE]

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