As parents, we want to do everything possible to protect our child’s health and well being. But what if there was something hiding in your child’s ear that could cause hearing loss?

Here is what you need to know about cholesteatomas and hearing loss.

What is a Cholesteatoma?

In simple terms, on Healthline, Elaine K. Luo, M.D., describes a cholesteatoma as “a noncancerous skin growth in the middle section of your ear, behind the eardrum. It can be a birth defect or result from repeated middle ear infections.  It can affect your hearing, balance, and facial muscle function.” She goes on to say: “a cholesteatoma often develops as a cyst, or sac, that sheds layers of old skin. As these dead skin cells accumulate, the growth can increase in size and destroy the delicate bones of the middle ear.”

In addition to recurring infections, a cholesteatoma can be the result of a malfunctioning eustachian tube. This tube connects from the back of the nose to the middle ear and allows air to flow through while equalizing pressure. The eustachian tube can become impaired due to various factors such as chronic ear infections, sinus infections, colds, or allergies.

Cholesteatomas are relatively rare but not uncommon. In the United States, one study from the Brazilian Journal of Otorhinolaryngology reported there were just six cholesteatomas per 100,000 people. Despite their relatively low occurrences, cholesteatomas make up a serious condition that requires medical attention and treatment.

Cholesteatomas and Hearing Loss

To better understand how cholesteatomas interfere with hearing, it is important to first identify how sound is processed within the ear. John Hopkins Medicine explains the process in the following way:

• The outer ear collects sound waves and directs them into the ear canal, where they cause the eardrum to vibrate.
• The vibrations from the eardrum are transmitted to the bones of the middle ear (the malleus, incus, and stapes) through the ossicular chain. The three bones amplify the vibration, pushing sound toward the inner ear, while the eustachian tube equalizes the middle ear pressure with the air from outside of the ear.
• In the inner ear, the cochlea receives the amplified vibration from the ossicular chain and converts it into electrical signals through the movement of hair cells.
• The electrical signals are then sent to the brain through the auditory nerve to be processed and interpreted as sound.

Read more: A study debunks how hearing works

Depending on the severity and progression of the cholesteatoma, hearing loss may be temporary or permanent. Below are three examples of how cholesteatomas interfere with hearing:

“Depending on the severity and progression of the cholesteatoma, hearing loss may be temporary or permanent.”

Destruction of the Middle Ear Structures

When cholesteatomas are present in the middle ear, they can disrupt the pathway that sound waves pass through. As the cholesteatomas increase in size, the delicate bones within the inner ear (the malleus, incus, and stapes), as well as the eardrum, are susceptible to damage. This can lead to conductive hearing loss. This is a type of hearing loss that occurs when sound waves are unable to reach the inner ear.

Eustachian Tube Dysfunction

Cholesteatomas may also cause a blockage of the eustachian tube, which connects the middle ear to the back of the throat. This can lead to the build up of pressure in the middle ear and limit hearing capabilities.

A peer-review study explaining eustachian tube dysfunction stated that “children are more vulnerable to pathologies of the middle ear, primarily due to the immature development of their eustachian tubes.”

Infections

Children who swim frequently may be more susceptible to ear infections as water can enter the middle ear and lead to infections. Over time, repeated middle ear infections can result in cholesteatomas.

According to the Brazilian Journal of Otorhinolaryngology, the disease’s seriousness is a result of two main factors: infection and compression. Overtime provided the pouch remains dry; the development of cholesteatomas can accumulate gradually without causing problems. That all changes when an infection is present. If cholesteatomas grow aggressively, they can cause destruction within the ear. This damage leads to hearing loss or deafness for most individuals with this condition.

Overall, when cholesteatomas are present, they can create a significant impact on the ability to hear.

It is important to identify hearing loss in children as soon as possible, so they can receive appropriate treatment and support. Schedule regular check-ups with an ear, nose, and throat (ENT) doctor for ongoing evaluations and proper diagnosis. Early intervention can help prevent or minimize the impact of hearing loss on a child’s development and future success.

One of the best predictors of who gets a hearing aid is how bothersome the hearing loss is.  Typically, this is assessed by the degree of hearing loss as measured with an audiogram.

But is this the right measure?  What’s fascinating is that often, two people are not bothered by the same audiometric loss to the same extent.

For example, we know that people vary naturally in how easily they get bored. So, if you’re the kind of person that doesn’t get bored very easily, maybe a certain level of hearing loss wouldn’t bother you as much in terms of your hearing loss extent. But if you’re a person who gets bored more easily and then suddenly, your sensory input is limited by hearing loss, then you’re going to be bothered by that hearing loss to a greater extent than someone who does not get bored more easily.

What made that question unique for us as researchers was that no one has ever explored this concept of boredom proneness in audiology. My understanding of prior research is that while there has been previous research looking at cognition and fatigue in relation to hearing loss, the idea of boredom has not yet been investigated.

Study results: Correlation between boredom and hearing loss

Dr. Mark Fenske and I decided to explore this relationship. Dr. Fenske, a cognitive neuroscientist, is an expert in cognition and motivation. Also involved in this work was a stellar team consisting of Carolyn Crawford, a graduate student who was project lead and who supervised a group of undergraduate students (Kalisha Ramlackhan, Hannah Brock, Ariella Golden, Sibley Hutchinson, and Brooke Party).

Our study involved close to 2000 participants. The participants, all first-time visitors of audiology clinics aged 50 years or older, had a mild case of hearing loss. The study examined the level of their hearing as well as their tendency to experience boredom.

What we learned was that not all people with a mild loss of hearing are the same.

Some people with mild hearing loss are not really bothered by it, while other people with a similar amount of hearing loss tend to be bothered to a greater extent. What this work uncovered is that this difference in the subjective impact of audiometric loss for a person is predicted by how easily someone gets bored.

Boredom is also highly tied to attention because we all want to be engaged in some sort of satisfying activity. So, when we talk about being engaged with anything — that concerns attention.

Clinical significance of this study

This study shows us that an individual’s boredom levels and difficulties in maintaining attention can be considered as potential personal factors which can be helpful to determine the extent to which hearing loss becomes bothersome for an individual. Such personal factors related to cognition could potentially prompt the likelihood of the person’s decision of whether treatment regarding their hearing loss may prove to be beneficial.

Often referred to as a snail shaped structure, the bony cochlea safely encloses our most precious hearing capabilities. Tucked inside are thousands of hair cells that transmit electrical wavelengths to the brain. Once they reach the brain, they are comprehended as the sounds one enjoys. A new study is debunking previously believed notions about accessibility to this part of the ear.

Discovering a Safe Passageway Into the Inner Ear

Though intensely studied, according to Radifah Kabir in an ABP Live article, the cochlea seemed to be inaccessible in years past. However, researchers from across the world came together to disprove this. The team consisted of Guy’s and St. Thomas’ NHS Foundation Trust in London, Uppsala University in Sweden, University of Sheffield in UK, University of Nottingham in UK, and Western University in Canada. Using advanced imaging technology, including temporal bone models, the researchers had exciting results.

Their published paper can be found in the Scientific Reports Journal, titled, “Unlocking the human inner ear for therapeutic intervention.” The sponsor of this study is Rinri Therapeutics, a group whose mission is discovering regenerative cell therapy to restore hearing. They found that the pathway into the cochlea is known as the Rosenthal’s canal. Rosenthal’s canal has a diameter of .1 to .05 mm. Inside this canal are auditory neurons. As one can imagine, this is an extremely tiny passageway. Although small in size, the team discovered that this canal may hold the secret to realistic access to the inner ear. With this newfound, safe access, researchers throughout the globe are encouraged to continue looking into gene therapies and regeneration possibilities.

With this newfound, safe access, researchers throughout the globe are encouraged to continue looking into gene therapies and regeneration possibilities.

Read more: How the Cochlea Contributes to Hearing

Methods

How did the researchers analyze such a narrow opening? How was safety considered? Researchers started by using specialized imaging technology called synchrotron radiation phase-contrast imaging (SR-PCl). This SR-PCl allows for the high-definition images to distinguish the edges of the soft tissue more accurately from the bone.

To assess safety, computer vision technology enabled the researchers to visualize the blood vessels and nerve pathways. This specialized technology enabled the team to gauge the risk of potential harm. These imaging studies allowed them to create an anatomically correct human inner ear. The fine-tuned accuracy of these imaging tools has enabled researchers and physicians to better understand the structure of the inner ear. Research and clinical practices continue to advance.

Read more: Unlocking the human inner ear for therapeutic intervention

Implications of This Research

Sensorineural hearing loss is steadily on the rise. According to the World Health Organization, it is estimated that about 700 million people will have hearing loss by 2050. Additionally, hearing loss can be caused by a variety factors including genetics, congenital conditions, infections, listening to loud sounds, etc. Due to the high prediction of increased hearing damage, there are goals to better both hearing technology and cell regeneration therapies.

This latest research is valuable as it shows an applicable access point of the cochlea. In a News Medical Life Sciences article, Professor Marcelo Rivolta explains why this is such a big deal: “Until now this region of the inner ear has been inaccessible in humans. This means that the pioneering advanced therapies to repair the auditory nerve, which have already proved successful in animal models, have been hampered by limited anatomical knowledge and the lack of a safe access to Rosenthal’s canal – the compartment that houses the auditory neurons within the central core of the cochlea.”

Scientists in the U.S. have discovered a “master gene” that can regrow lost ear hair cells. It is known as TBX2.

Ear Hair Cells

TBX2 functions by directing ear cells to produce inner or outer hair cells, according to the findings, which were published in the journal Nature. For human hearing to work, we need both types of ear hair cells to work together. Inner hair cells transmit to the brain. Outer hair cells, which amplify sound, are frequently damaged by factors like age, medications, or even substance abuse. If the outer hair cells are missing or damaged, signals don’t get to the inner hair cells. As a result, they aren’t sent to the brain, and we don’t hear the sound.

Ear hair cell loss is a significant cause of hearing loss, resulting in sensorineural hearing loss, or SNHL – the type of hearing loss responsible for around 90 percent of hearing loss. The other 10 percent is usually attributed to conductive hearing loss or some form of blockage in the ear. Hair cells are sensory receptors within the ear that are essential towards processing sound. Lost or damaged ear hair cells reduce hearing capacity and contribute heavily towards hearing loss.

What Does TBX2 Do?

Scientists now believe they can reproduce these essential ear hair cells to replace those lost to age or damage. Recreating these cells is a significant step forward. This could be the start of a new form of gene therapy for hearing loss, according to researchers.

“This could be the start of a new form of gene therapy for hearing loss.”

The discovery builds on existing science whereby researchers can create artificial hair cells. However, these cells cannot develop into inner or outer ear hair cells, which is where TBX2 fits in. Researchers at Northwestern University investigated ear hair cells in mice and revealed that TBX2 is a regulator of inner ear hair cell versus outer ear hair cell production. Scientists analyzed hair cell creation concerning inner and outer ear hair cells. When a hair cell was created, blocking the TBX2 gene ensured that the cell was an outer hair cell.

Read more: Researchers find a drug combination that could regenerate ear hair cells

What Happens Next?

While the discovery is a major step forward in neuroscience, it’s uncertain what happens next. Scientists hope to figure out how to use this new knowledge to create a cell development process that may be usable in treating hearing loss. Currently, it’s possible to create artificial hair cells. This method can’t differentiate between inner and outer hair cells, so some work still needs to be done.

In theory, if scientists can regrow ear hair cells, then they are one step further towards repairing or restoring lost hearing. However, there is still a long way to go. There’s no guarantee that this discovery will in any way lead to a “cure” for hearing loss. It does offer some hope, however, that ear hair cells might be replaceable one day.

Justin Bieber, the Canadian-born singer/songwriter and global pop sensation, announced Friday that he has been diagnosed with Ramsay Hunt Syndrome, a rare neurological disorder characterized by paralysis of the facial nerve, a rash affecting the ear or mouth, ringing in the ears (tinnitus) and hearing loss.

In a video posted on his Instagram account, the 28-year-old singer displayed signs of facial paralysis and expressed to his fans and followers that it was difficult to eat. He also showed how he was unable to smile on the right side of his face, blink his right eye and move his right nostril.

Ramsay Hunt syndrome affects about 5 of every 100,000 people each year in the United States, according to the National Organization for Rare Disorders (NORD). It is caused by the varicella zoster virus (VZV), which also causes chickenpox in children and shingles in adults. The disorder is also sometimes known as “herpes zoster oticus” because of the characteristic ear rash. However, physicians often use “herpes zostic oticus” only for the ear rash and “Ramsay Hunt syndrome” when the ear rash is accompanied by facial paralysis, according to NORD.

Ramsay Hunt Syndrome Prognosis

There is no way to prevent Ramsay Hunt syndrome, according to the Icahn School of Medicine at Mount Sinai. Recovery can occur within a few weeks to several months, however chances of recovery are better if treatment begins within 3 days after the symptoms begin, according to Mount Sinai.

Treatment includes anti-inflammatory drugs, antiviral medicines and pain killers.

When left untreated, Ramsay Hunt Syndrome can result in permanent weakness of the facial muscles or hearing loss, according to Healthline.com.

Ramsay Hunt Syndrome and Hearing Loss

Most cases of Ramsay Hunt syndrome result in symptoms of facial paralysis, and a rash that affects the outer (pinna) and external ear canal, as well as the mouth and throat.

Tinnitus, or the sensation of ringing in the ear, is also a common symptom, according to the National Organization for Rare Disorders. Some individuals can also develop sensorineural hearing loss, as a result of the affected auditory nerve not being able to transmit vibrations to the brain.

Read more: What is sensorineural hearing loss?

There is no clear correlation between the severity of facial weakness among patients with Ramsay Hunt syndrome and hearing loss, however, one study published in the Journal of Neurology, Neurosurgery and Psychiatry showed about 19% of patients had an abnormal audiogram.

According to a study in the journal “Medicine,” hearing loss was more severe in the high frequency range than in the low frequency range among patients with the Herpes zoster oticus virus. Hearing impairment was more severe in patients with vertigo than in those without vertigo in both the high and low frequency ranges. The degree of hearing impairment was not significantly different between patients with and without facial palsy (Ramsay Hunt Syndrome).

In another study by the Department of Otolaryngology, Ehime University School of Medicine in, Japan, complete recovery occurred in 85/173 (49%) adults and 33/42 (78%) patients younger than 16. Audiograms showed complete recovery in 66% of children with audiometry documented hearing loss compared with 37.7% of adults.

Read more: Why musicians should be more aware of hearing loss

Prevention and Recovery 

Ramsay Hunt syndrome is not contagious, according to the MayoClinic. While it can potentially still develop in anyone who has had the chickenpox, for people who haven’t had the chicken pox, a vaccine for the chickenpox virus and shingles is recommended.

Justin Bieber in recovery for Ramsay Hunt Syndrome

While recovering from Ramsay Hunt Syndrome, Justin Bieber has cancelled his upcoming tour dates and is practicing facial exercises as part of his healing, according to his Instagram post.

“I’m gonna get better,” he says in the video. “I’m doing all these facial exercises to get my face back to normal and it will go back to normal, it’s just time. We don’t know how much time it’s gonna be, but it’s gonna be okay.”