Stem Cell Research Might Lead to Hereditary Hearing Loss Treatment
What is Hereditary Hearing Loss?
Hearing loss is a common medical condition in the United States, affecting 48 million Americans. The two most common forms of hearing loss are age-related (presbycusis) and noise-induced (due to exposure to loud noise, over a long period of time or in a one-time traumatic event).
Hereditary hearing loss is unlike these two forms, as it is not an acquired form of hearing loss. Hereditary hearing loss – like other forms of hearing loss – may be conductive, sensorineural, or a combination of both. It is also categorized as syndromic (due to malformations of the outer ear or organ systems) or non-syndromic (with no visible abnormalities of the outer ear or other medical problems). Hereditary hearing loss may be pre-lingual (before language develops) or post-lingual (after language develops).
Like other forms of hearing loss, hereditary hearing loss is identified through a series of otologic and physical examinations, which will simultaneously help to identify the type of hearing loss experienced as well as the appropriate treatments, namely hearing aids.
Prevalence of Hereditary Hearing Loss
According to a 2009 study, “Hearing loss is the most common birth defect and the most prevalent sensorineural condition in developed countries.” (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2847850/)
Researchers have identified “more than 400 genetic syndromes that include hearing loss.” The most common type of syndromic hearing loss is Waardenburg syndrome, which causes both sensorineural hearing loss as well as abnormalities with skin and hair (white forelock). Another syndrome, Branciootorenal, has been known to cause conductive, sensorineural, or mixed hearing loss, and has been associated with malformation of the outer ear.
Current Solutions to Hereditary Hearing Loss
Currently, hereditary hearing loss is treated with the use of hearing aids. Children older than age 12, with severe to profound hearing loss, may receive treatment in the form of cochlear implants. Some forms of hereditary hearing loss may be treated with surgery.
As with all forms of hearing loss, identifying and treating the hearing loss is crucial to one’s overall well-being. Generally speaking, amplification (with the use of hearing aids or cochlear implants) is “essential for optimal cognitive development in children,” according to researchers at the University of Iowa Department of Otolaryngology and University of Antwerp.
Research on Stem Cells and Hereditary Hearing Loss
In March 2016, results were released from a study conducted between Wenzhou Medical University in China, along with Cincinnati Children’s Hospital Medical Center and Emory University in the US. This team of researchers had begun to develop new research toward a therapy that would effectively treat hereditary sensorineural hearing loss. Using a framework in which stem cells treat disease-specific genes, lead researcher Jin-Fu Wang, PhD and his team decided to target sensorineural hearing loss.
Researchers theorized that “a protein coding gene called MYO7A plays an important role in the assembly of stereocilia into bundles. (Stereocilia are the part of the hair cells that respond to fluid motion. Their bending is how hair cells sense sounds.) If they could use stem cells to repair the MYO7A, that might result in proper growth of the stereocilia and correct the hearing loss.”
According to another researcher, Dr. Min-Xin Guan, “Current findings confirmed our hypothesis and might prove further insight into what is behind sensorineural hearing loss.”
While studies are still in their nascent stages, researchers of this joint study are optimistic that further trials will produce an efficient treatment and development of stem cells to counteract hereditary hearing loss.
You don’t have to live with untreated hearing loss. For a consultation, contact us at Greentree Hearing & Audiology today!