Our ears, the organs responsible for hearing, are part of an intricate system that allows humans to experience the audible world around them. Our ears allow us to communicate with our loved ones, stay aware of our surroundings, and connect with our environment. Additionally, the ears play a role in maintaining balance and equilibrium in the body.
As an integral part of the auditory system, issues with the ears can cause a noticeable change in the way one views and interacts with the world. Just imagine a future where hearing loss could be reversed with cutting-edge cell therapy.
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In this article, we’ll take a closer look at one important part of the ear that can lead to hearing problems and even tinnitus: the cochlea. We’ll also explore a groundbreaking new surgery that has the potential to reverse cochlear damage, offering hope to millions of individuals suffering from hearing loss due to inner ear damage.
Cochlear Damage Explained
The ear is composed of the outer ear, middle ear, and inner ear. The outer ear is the part we can see and touch. The ear canal contains cerumen, or ear wax, to keep it lubricated, protect the middle and inner ear from infection, and remove dead skin.
The middle ear is located beyond the ear canal, and includes the eardrum, and the three smallest bones in the human body, known as the ossicles. These bones amplify the sound from the outer ear into the inner ear.
Beyond the middle ear is the inner ear. The inner ear houses the organs responsible for balance, like the semicircular canals, and the organ responsible for hearing, called the cochlea.
Function In The Auditory System
Inside the cochlea, there are specific cells that connect to the auditory nerve called stereocilia, inner hair cells, and outer hair cells. These structures convert the vibration of sound waves into electrical signals that travel along the auditory nerve.
Under normal conditions, the microscopic outer hair cells are arranged in three rows and the inner hair cells in a single row. These hair cells allow us to hear clearly, but can be easily damaged, leading to hearing loss and or tinnitus.
Hearing loss can first be identified through a test that assesses outer hair cell function. This test called otoacoustic emissions (OAE) testing is commonly performed in an audiology clinic. The missing information from the damaged outer cells gets sent to the auditory nerve and up to the auditory brain. Tinnitus—ringing in the ears—often occurs as a result of this type of hearing loss.
Abnormal neural activity in the auditory system occurs when the brain creates phantom sounds associated with the frequencies it can no longer hear well. This phantom sound is described as tinnitus and can sound like ringing, hissing, buzzing, or a host of other sounds.
Popular Causes And Effects
Cochlear damage is also referred to as sensorineural hearing loss, where there’s damage to the sensory cells within the iinner ear. This is a permanent form of hearing loss, and the severity depends on the extent of the damage to the outer and inner ear cells.
Cochlear damage can occur due to:
- Noise exposure– This is one of the most common causes of hearing loss. The delicate hairs in our inner ear cannot tolerate sounds over 85 decibels for over eight hours and are unable to regenerate themselves once damaged. Examples of this include attending a loud concert, being around gunshots or fireworks, or listening to music too loudly through headphones.
- Head and neck trauma– These injuries send pressure waves to the cells in the inner ear which can cause them to become permanently damaged and lead to hearing loss and/or tinnitus even after receiving treatments for the physical injury.
- Ototoxic medication– Some medications have been known to affect the inner ear cells and their functioning temporarily. Examples include antimalarial drugs, certain antibiotics, loop diuretics, and cancer medication.
- Autoimmune disorders– Though rare, autoimmune conditions like Autoimmune inner ear disease (AIED), lupus, rheumatoid arthritis, and Sjögren’s syndrome can affect the way the cochlea functions and result in hearing and balance issues.
- Viral infections– When infections such as rubella, HSV, mumps, or measles affect the cochlea, the inner ear cells can get attacked and prompt an autoimmune response. This can lead to temporary or permanent damage, which can result in hearing loss, tinnitus, and/or dizziness.
- Aging– As we age, our hearing naturally declines, so as with other organs, the cochlea is also subject to the effects of aging. This is known as presbycusis or age-related hearing loss.
Hearing loss is the most obvious effect of cochlear damage. The extent of this damage determines the degree of the hearing loss. Hearing loss affects one’s perception and ability to listen and follow along in conversations with background noise, as well as in quiet. It can also result in tinnitus, causing a ringing or buzzing sound in one or both ears.
Cochlear damage can also cause strong psychological effects, like feelings of depression and anxiety. This may lead to social isolation, and negative feelings surrounding hearing loss, and the life changes that come with it.
Current Treatments For Cochlear Damage
Traditional treatments for cochlear damage include hearing aids and cochlear implants. Modern hearing aids are programmable, offer various settings for different listening environments, and can be customized to meet the wearer’s needs. For tinnitus, hearing aids can help reduce the perception of tinnitus, reducing its symptoms.
Cochlear implants are needed for more profound cases of sensorineural hearing loss. Once implanted, hearing and tinnitus can improve, however, this requires surgery under general anesthesia and comes with its own set of risks.
Traditional treatments like hearing aids or cochlear implants don’t regenerate lost cells or nerve fibers. They improve hearing only while the devices are in use. People with inner ear damage generally benefit from a combination of these devices, along with complementary therapies such as Auditory Training and Rehabilitation and Cognitive Behavioral Therapy (CBT).
Experimental therapies like gene therapy and pharmacological interventions show some promise, but they are still not safe or effective enough to be offered as treatments to patients with cochlear damage.
The Rinari Therapeutics Approach
Researchers at Rinari Therapeutics take a new approach to addressing cochlear damage. This new surgical method to improve the auditory nerve could help people who have difficulty hearing conversations in noisy environments where traditional hearing aids fall short. Hearing aids are useful when the cochlear damage is mild. The amplification provided is, however, not always enough to compensate for the auditory system’s damage. Sound distortion is a common complaint of those with cochlear damage, even when sounds are loud enough to be heard.
Using their OSPRAY platform, the experts at Rinari Therapeutics offer a minimally invasive approach to deliver regenerative cell therapy directly to the auditory nerve. The Rinari Therapeutics platform ensures the transplanted cells can survive, integrate, and form functional synapses with existing neural structures. This novel pathway accesses the cochlear nerve through the round window, a partition between the middle and inner ear, eliminating the need for invasive skull-based surgeries. The stem cell therapy easily reaches the auditory nerve through a membrane in the cochlea.
Rincell-1, their lead product, is the launchpad for human trials. The goal is to restore the neural connections between the inner ear and the brain by regenerating spiral ganglion neurons. Here’s how it works:
The procedure uses regenerative cells engineered to restore nerve connections between the inner ear and the brain. These cells are surgically delivered to the specific region of the cochlea through the round window using advanced, minimally invasive surgical tools. This ensures precise cell placement with minimal risk to surrounding structures. This process is considered groundbreaking as it can potentially restore communication between the cochlea and the brain, something not yet possible through current therapies.
Clinical trials for this procedure are expected to begin in 2025. Still, the preclinical studies have shown promising results with improved hearing levels of approximately 40% and reversals in hearing loss. Early research has shown that this procedure is an advancement in the delivery of cell therapies for hearing restoration. This includes a first-in-human clinical trial demonstrating its safety and feasibility without significant complications.
Is This A New Surgery For Tinnitus?
Restoring auditory nerve cells could reduce the abnormal neural activity linked to tinnitus. As we mentioned earlier, tinnitus is perceived in the auditory brain, which is connected to the ear. The ear sends signals through the auditory nerve to the brain, so even slight changes in the nerve and brain can impact the perception of tinnitus.
While this approach is promising, it is still in its early stages. More trials are needed to confirm its efficacy for tinnitus and hearing loss. Furthermore, not every tinnitus patient would benefit, as the root causes of tinnitus vary. For those whose tinnitus isn’t caused by hearing loss, this method might not be effective. Luckily, there are evidence-based treatments available for different kinds of tinnitus.
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