Ramsay Hunt syndrome and Hearing Loss

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.”

Phonak releases new Virto Paradise hearing aids

Phonak’s latest product, the Virto Paradise, is a custom-made hearing device which resembles a modern earbud. This revolutionary in-the-ear aid gives you all the benefits of the Paradise line, with customizable features and hands-free communication.

Phonak Virto Paradise

The Phonak Virto Paradise is the most recent hearing aid to join the Paradise platform. In August 2020, Paradise (P) was released for receiver in canal (RIC) wearers with the Audéo hearing aid. A year later, people with single sided deafness were able to get CROS P. Now there’s the Virto P.

The Virto P product line includes:

  • Virto P-312 (including Virto P Black)
  • Virto P-10 NWO – in the ear and invisible

Read more: Phonak announces Audeo Life waterproof hearing aids

Made for All Connectivity

The biggest difference with the Virto P 312 from other in-the-ear hearing aids on the market is that it has made for all (MFA) connectivity with two active pairings. It is actually the only Made For All [MFA] hearing aid currently available on the market. This means it offers hands-free communication.

“It is actually the only ‘Made for All’ hearing aid currently available on the market.”

“If your personal phone is an Android and your work device is iOS, you’re covered,” said Whitney Spagnola, Phonak U.S. Marketing Manager. “If you have a smart TV, Bluetooth compatible computer, an older flip phone, etc., any of these Bluetooth capable electronics can pair to your hearing aids. It may sound simple. But for hearing aid manufacturers, it’s not. The real estate of your ear is really small. As a result, it takes quite a bit of development time and forethought to provide MFA in hearing aids.”

Now with the Virto P, you can put your phone down on your kitchen island while cooking dinner and go about your various tasks all while still on the phone call. And it will be clear on both ends.

Customization

Audiologists use special software from Phonak to program hearing aids. Hearing aids are programmed by audiologists to fit the hearing loss of each individual, as everyone’s hearing is unique. Virto P brings additional customization at your fingertips via the myPhonak app,  which allows you to make your own adjustments based on the fitting provided by your audiologist. But until now, every Phonak Paradise hearing aid was built the same way and looks the same. Virto is different.

Virto P hearing aids are custom-built using special software that takes into account the patient’s individual ear anatomy. It extracts over 1,600 unique data points from the ear.

“We use this to refine the way the directional algorithms in the hearing aid help you hear,” Spagnola explained. “Each ear is unique. Look at your Grandpa’s, your nephew’s, your neighbor’s. They’re all different. The inside and outside are all there to help you hear better. And with the ear impression information, our Rapid Shell Modeling Software can actually extract your anatomical structure to change the way the device performs.”

Virto P-312

This is a fully-connected in-the-ear (ITE) hearing device that resembles a modern earbud. It can distinguish between streamed speech and music. The Virto M Black was first unveiled at CES 2020 (Consumer Electronics Show), where it received multiple awards and accolades for its “stigma-busting design that blurred the lines between a hearing aid and a hearable,” according to the Phonak press release. This is the second generation version. It comes in several colors, including the highly anticipated color of black.

Read more: Is an in-the-ear hearing aid right for me?

Virto Paradise hearing aids come in a variety of colors, accessories, and user options. They are now available in the U.S. Select markets worldwide will have them in the coming months.

Watch how a Virto P hearing aid is made (Note: this is meant for audiologists, so don’t fear the jargon!)

Early Intervention: Why parents need to follow up

Early Intervention programs for deaf and hard of hearing children are typically offered for ages 0-3 in the U.S. Over the last two years, during the height of the COVID-19 pandemic, early Intervention caseloads have been decreasing. This prompts researchers to ask the question: Are parents of deaf children not following up with early intervention hearing care? Why is Early Intervention so important?

Hearing Loss Diagnosis

A diagnosis of hearing loss for a child can be overwhelming to a new parent. In the last two years amidst a pandemic, it’s been more overwhelming. On top of the diagnosis, parents have to take many things into consideration, including:

  • COVID safety
  • Sick family members
  • Work
  • Uncertainty about whether child needs services

These decisions are not always easy. It can make it easy to overlook Early Intervention services provided by your state. The offerings and providers vary by state and not all programs are created equal.

Early Intervention Teams’ Caseloads Down

I spoke to five Teachers of the Deaf and Hard of Hearing (TODHH) in different states recently who confirmed that their caseload for children ages 0-3 is down. One TODHH noted that their caseload is down 65 percent. I wondered what was happening.

The audiologists I spoke to saw no difference in the number of referrals following hospital screening. Families are following up. Most families are following up on their Early Intervention intake.

The gap appears to be after the intake. Possible reasons include:

  • Deferring services
  • COVID shut downs
  • Fear of going to a classroom
  • Virtual environments not for everyone
  • Thinking services aren’t needed
  • Not enough time in a day for extra appointments

Early Intervention does require work and time on both the parent and the provider’s side. It means extra appointments and conversations, which can seem daunting. But if Early Intervention is offered by your state, you should be taking advantage of it.

“If Early Intervention is offered by your state, you should be taking advantage of it.”

The good news is that DHH programs are starting to pick back up. Of the five TODHHs I spoke with, four reported an increase in case numbers this year and getting back to normal.

Read more: How to advocate for your child with hearing loss

Benefits to Early Intervention

If you are feeling uncertain about Early Intervention services, here are some benefits:

1. Parent Confidence

  • EI services can help you understand what your child may be processing.
  • Learn how to be an advocate, which brings a confidence out that children get to witness. When children see their parents advocacy, they can learn to follow that lead.

2. Guidance

  • There is a lot to learn about options and communication. A TODHH from your Early Intervention team can help put things into perspective and set goals and timelines. This helps to relieve the initial overwhelm.
  • You will learn about your child’s hearing loss, their devices, and what to look out for that might be considered behavior (ie, not listening) when it might be listening fatigue.

3. Language

  • Does your child have access to language?
  • Are they communicating at an age appropriate level?
  • Your TODHH and Speech Language Pathologist (SLP) can help guide you in these areas. Note: the developmental language window is narrow, all the more reason to act quickly.

4. Community

  • You will hear about and may have the opportunity to meet other families. It helps to talk to other parents, as well potentially have your kids meet other DHH kids.
  • You can confide in your service providers what is on your mind. They are not your therapists, but they understand the process of what you may be going through and can give you specific next steps or ideas.

5. Goal-setting

  • Develop routines for your child’s learning and how to implement them into everyday life and activities.
  • You early intervention specialists can help you prepare with the right questions to ask whether you are visiting the audiologist or a new school.

6. Advocacy

  • If you need an advocate for your child’s classroom, oftentimes your TODHH can help. You can work together with the family and school to discuss the right accommodations.
  • A TODHH focuses on the whole child and also communicates with the audiologist, SLP and physical therapist or other service needed.

Early Intervention Works

On a personal note, as someone who is deaf/hard of hearing, I originally fell into the category of believing I did not need Early Intervention services. While I do have a lifetime of experience being deaf/hard of hearing, I had no experience raising deaf/hard of hearing children. It challenged my perception of language, behavior, and learning. We’re all better off for it. I feel so incredibly lucky for the team my girls had between the ages 0-3.

Read more: What to do early on for your deaf baby

Safe listening: Protect your ears

Hearing is one of the most magical and complicated senses humans have. Our ears are beautiful tiny pieces of engineering converting differences in air pressure into perceptions of sound. Yet, we as a society do not have a basic understanding of how these sound pressures can damage our hearing. Safe listening is important; protect your ears.

Hearing Care Education

Using hearing protection is key to safe listening. According to the World Health Organization, one in four people is projected to have hearing problems by 2050. That’s 25 percent of the population. One of the major factors that affect hearing health is lack of proper hearing care education. Why don’t we stare at the sun or why don’t we go to the beach without wearing sunscreen? If we follow the same principle, why don’t we wear hearing protection when we are going to a show knowing is going to be loud? Or why do we have our headphones embedded in our ears for a good part of the day?

Read more: Hearing Protection

Hearing Damage Can Happen Quickly

Hearing damage can happen in seconds depending on the intensity, proximity, or even frequency of the sound. Without getting too technical, a rock concert can get up to 115 db. It is known that safety exposure to that level of sound is only one minute. After that, there is a chance you are doing some permanent damage as hearing doesn’t regenerate.

I get it. Live music is great! It releases endorphins and make us feel alive. But why play this game that could have devastating consequences like hearing loss or tinnitus?

Read more: The Do’s and Don’t’s of Going to a Concert with Hearing Loss

Safe Listening

Have you ever come back from a night out with some ringing in your ears? Even if you’re lucky enough for it to go away after minutes, hours, or a few days? This is normally the first sign you are doing some damage to your hearing. If this happens frequently, there is a chance you will have permanently damaged your hearing and developed what is called “Tinnitus.”. Listen to the signs and take action!

If you like going out to noisy places, there are few things you can do. One is to maintain a distance from the speakers or to take regular breaks. If you are not sure about risky sound levels, learn about sound levels and download a decibel meter to your phone. Take it easy the next days after you have been out to give your ears some time to recover.

60/60 Rule

If you are a headphone user, apply the 60/60 rule: No more than 60 percent of the volume for no more than 60 minutes at a time. Invest in good quality headphones, preferably with active noise cancellation.

Read more: Who is at risk from noise-induced hearing loss?

Hearing Protection

Part of the issue with hearing health education is the stigma surrounding hearing protection, which is seen as uncool or somehow diminishing the audio quality. As a DJ, I used to think that louder was always better. In reality, it is not. It’s all about adjusting your brain. In sound production, there is a technique that requires you to listen and mix at the softest possible level. In this instance, the brain becomes super sensitive to sound. The differences in frequencies and volume become obvious so you can have a better mix in music. This shows that it is possible to change our collective idea as a society about sound levels and turn the volume down a notch.

Read more: My experience coping with tinnitus

Don’t Learn the Hard Way

Unfortunately, for most people with hearing damage, we learned the hard way. We often don’t realize the damage we are doing until it’s done. Nevertheless, it is never too late to start protecting your hearing and find good hearing protection. If you are concerned with the quality of sound as I am, there are good earplugs that reduce the volume overall without making the music sound muffled. In fact, the sound is better with them, especially at venues without a good quality sound system.

Read more: Hearing protection

Prevent Hearing Damage

The reality is that we live in an industrialized society, therefore we are constantly exposed to sound from various sources. It is inevitable. So, the best way to prevent hearing damage is to be diligent about it. If you think something is too loud turn it down, wear hearing protection, or move away. Use intuition and always remember, if you want to hear for life, listen with care!

Why Egypt’s newborn hearing screening initiative is important for the rest of the world

Egypt’s Newborn Hearing Screening Initiative

In September 2019, Egyptian President Al-Sisi and his Minister of Health and Population, Hala Zayed, launched an initiative to get otoacoustic hearing screening to newborns across Egypt. Over one million children have been tested within the first year, according to EgyptToday.com. With the 100 Million Scheme being rolled out across Africa, many more will follow. The hearing screening can’t come quickly enough, as an average of 130,000 Egyptian newborns have hearing loss.

The 100 Million Scheme covers all kinds of health screenings, from newborn hearing tests to breast cancer. With massive plans to improve Africa’s status as a continent, the 100 Million Scheme is just one part of a colossal effort to make Africa a world superpower by 2063. The move is known as the African Union Agenda 2063. The World Health Organization is backing the health initiative.

Hearing Screening for Babies

The earlier you catch hearing loss or deafness, the less severe the impact on the child’s development. Babies rely heavily on their hearing to learn speech and language. Understanding how words and letters sound can be crucial in learning to read and write. There’s also a developmental window when it comes to learning how to talk.

According to a study published in the Journal of Deaf Studies and Deaf Education, Deaf children can have a much harder time with literacy, due to not having phonetics to learn from. As a result, their general education can lag. People who acquire deafness later in life have already obtained a voice imprint, or the memory of what words sound like, according to the National Institute of Health. This makes it easier for them to learn to read.

In the U.S., hearing screening guidelines state that babies should have hearing tests before one month old. Those that fail need a full hearing test before three months of age. Children with hearing loss or deafness do much better if they’re supported early on so that they can obtain essential literacy and communication skills. The more awareness you have of your child’s condition, the better you’re able to support them.

Read more: Our journey after a failed newborn hearing screening 

Deafness and Hearing Loss in Egypt

With five percent of Egypt’s population having hearing loss, deafness and hearing loss are just as much of an issue in Egypt as they are in the U.S. and the rest of the world.

Being born with a hearing loss in developing countries means having a distinct disadvantage, especially if there isn’t adequate literacy for education. However, there is support available. Egypt is rapidly becoming a supportive country to live in for the deaf community. One example is the Egypt School for the Deaf offering classes in Egyptian Sign Language to parents of deaf children.

Egypt also has its own sign language known as Egyptian Sign Language or ESL. There isn’t much documentation about its history or usage. It is one of several Arabic sign languages. There is no united Arabic sign language. All attempts to implement such a language failed, as it means incorporating an entirely new sign language among users of existing ones. If you fancy giving ESL a go, check out SignPuddle, a dictionary of ESL signs.

Read more: Sign languages around the world