Otosclerosis is a phrase derived from two archaic words: “oto” (ear) and “sclerosis” (abnormal hardening of human tissue). Otosclerosis is a condition caused by aberrant bone remodeling in the middle ear. Bone remodeling is a continuous process that renews bone tissue by replacing old tissue with new tissue. It affects around 4 million people globally. Otosclerosis is thought to be hereditary in many cases. White women in their forties and fifties are the most vulnerable.
A cascade of actions takes place inside the ear to transform sound waves in the air into electrochemical signals.
Higher-pitched sounds, such as a cell phone ringing, are detected by hair cells towards the base of the cochlea. People in the middle detect lower-pitched sounds, such as a huge dog barking.
Otosclerosis normally arises when one of the middle ear bones, the stapes bone, becomes stuck in place. The pinna, temporal bone, and ear canal are all part of the outer ear. The eardrum, malleus, incus, and stapes are all located in the middle ear. The semicircular canals, Eustachian tube, cochlea, vestibular nerve, and auditory nerve are all found in the inner ear.
It is unclear why this occurs, but scientists believe it is linked to a past measles infection, stress fractures in the bone tissue around the inner ear, or immune system abnormalities. Otosclerosis also runs in families.
It could also be the result of an interaction between three different immune system cells known as cytokines. The appropriate balance of these three chemicals, according to researchers, is necessary for optimal bone remodeling. Researchers also suggest that an imbalance in these chemicals’ levels may promote the aberrant remodeling seen in otosclerosis.
Individuals with otosclerosis have increasing hearing loss that is particularly noticeable at low frequencies, making it harder for them to distinguish vowels or male voices. Also,
An otolaryngologist’s auditory examination, which includes liminal tonal audiometry, audiometry, vocal audiometry, and impedance testing with stapedial reflexes, is essential for diagnosing otosclerosis.
Complementary investigations, such as computed tomography (CT), will be required to determine the best course of treatment.
Physical examination is required to rule out various disorders of the external auditory canal or tympanic membrane that may cause conductive hearing loss. Otoscopy is routine in cases of otosclerosis.
Otosclerosis is diagnosed by doctors who specialize in hearing. An otolaryngologist (a doctor who specializes in diseases of the ear, nose, throat, and neck (ENT), an otologist (a doctor who specializes in diseases of the ear), or an audiologist are examples of these specialists (a health professional trained to identify, measure and treat hearing disorders). The first step in diagnosing otosclerosis is to rule out other diseases or health issues that may cause similar symptoms.
Hearing tests to determine hearing sensitivity (audiogram) and sound transmission in the middle ear are the next steps (tympanogram). Otosclerosis is sometimes diagnosed with diagnostic imaging, such as a computed tomography scan (CT scan).
There is hope that current research on bone remodeling may lead to the discovery of viable novel therapeutics. Moderate otosclerosis can be treated with a hearing aid that amplifies sound, but more severe cases usually necessitate surgery. The surgeon installs a prosthetic into the middle ear that passes around the aberrant bone and allows sound waves to go to the inner ear, restoring hearing, in an operation known as a stapedectomy.
It is critical to consult with an ear expert before undergoing any surgical operation to understand the potential dangers and limitations of the technique. Some hearing loss, for example, may persist after a stapedectomy, and in rare situations, surgery might exacerbate hearing loss.
The inner ear’s complicated anatomy makes it challenging for scientists to research this portion of the body. Researchers must evaluate ear bone samples from cadavers provided for research because they cannot extract and analyze samples from the inner ear of a person with otosclerosis (or other hearing problems).
It also pushes scientists to merge cutting-edge biology, informatics, and imaging technologies with data from patient medical histories and pathology reports. It expects to discover new hints and remedies for hearing impairments caused by bone anomalies in this way.
NIDCD also finances genetic research and bone remodeling research in order to better understand the causes of otosclerosis and to investigate potential novel treatments. Researchers supported by the NIDCD are currently studying the effectiveness of an implanted device in animals. To treat bone irregularities that underlie otosclerosis, this device could send a medication that suppresses bone formation straight into the inner ear. If the results are favorable, human research will be carried out in the future.
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