Meniere’s disease is a disorder of the inner ear that can lead to episodes of dizziness and hearing loss, and in most cases, the disease affects only one ear.
Meniere’s disease can occur at any age, but it usually begins between puberty and young adulthood. It is considered a chronic condition, but various treatments can help relieve symptoms and reduce the long-term impact on your life.
In fact, 10 percent of patients, Those with an inner ear disorder that causes vertigo and tinnitus They have one or more relatives with the disease. This disease also has a genetic component that has been demonstrated in family aggregation studies.
The study, led by Pablo Roman-Naranjo and José Antonio López-Escamez, researcher in charge of the Institute for Biohealth Research in Granada (ibs.GRANADA), and published in the journal Hearing Research, analyzed data on a total of 62 families with this disease, mostly from Spain.
The results of the study indicated a relationship between Meniere’s disease and various ear genes, and the main candidate for this disease is the MYO7A gene, which releases a protein called myosin VIIa, specific to inner ear hair cells.
It is worth noting that the function of the ear depends on thousands of genes, and when changed due to rare mutations, the ability to maintain balance and hearing can be impaired. In the case of Meniere’s disease, the researchers explained in this study that genetic or genetic inheritance, in which a mutation in two different genes is necessary to cause a specific disease or phenotype.
This is because the proteins encoded by these genes are functionally related. There are different types of possible interactions between genes that determine disease onset, severity or age.
In some cases, a major locus and another modifier may be considered, or both can be equally important for disease progression. Therefore, genetic inheritance requires the interaction of two different genes to express the phenotype.
In some families, the disease appears to have its origin in rare mutations in the MYO7A gene, with mutations in other genes, such as CDH23, PCDH15 or ADGRV1, with which MYO7A both interact. This hypothesis is based on the results obtained in the study, which confirmed that nine of the participating families had rare mutations in these genes.
The researchers also noted in the medical study, that these genes are represented in a specific region of the ear, which is the ciliary space of the sensory neurons in the inner ear. These cells are responsible for transmitting sound in the organ of Corti, as well as perceiving acceleration in the posterior labyrinth, and sending nerve impulses to the central nervous system. Grouped strocilia protrusions were found in each of these cells.
According to the researchers, mutations in the MYO7A gene and other genes may cause changes to appear in several proteins, which bind stereocilia together, eventually leading to hearing loss and dizziness.
They explained that “rare mutations in the MYO7A gene, alone or in combination with other mutations that interact with the MYO7A gene, may cause changes in the morphological structure of the vacuolar cilia, causing a loss of cohesion between them, with the abnormal opening of the mechanotransportation complex, which is found in the cilia. Finally there is the possibility of hearing loss or vertigo.
In addition, the MYO7A gene has been associated with sensorineural hearing loss or retinitis pigmentosa, which can also result in another rare disease that causes blindness.”
In conclusion, studying Meniere’s disease and identifying new genes that could explain the origin of this disorder can help in deciphering its mechanisms, facilitating early genetic diagnosis of it, as well as developing and applying new drugs to treat it.
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