Researchers from the Boston Children’s Hospital and Harvard Medical School used an innovative gene-editing system in order to recover the hearing abilities in mice that had genetic hearing-loss. The project was successful without harmful or unwanted side-effects.
The latest approach involves a more enhanced and precise version of the CRISPR-Cas9 gene-editing system. This system is better able to recognize a mutation which results in hearing loss that is progressive in an animal model called the "Beethoven mice." The tool provided researchers with a way to disable Tmc1 which is a hearing gene known as the defective-copy without affecting or disturbing the copy that is healthy.
David Corey, Ph.D., a co-senior investigator of the study stated that the aspect that was the most surprising was how well it worked, providing just about undetectable cutting when it came to the inappropriate genes. He went onto say that this significantly reduces the risks in clinical settings, and also means that the CRISPR approaches might be ready for use for the purpose of gene therapy a lot sooner than expected.
The Beethoven mice in this study underwent treatment for the very same genetic-mutation which causes hearing loss that is progressive in humans. Without any therapy, these mice go deaf completely after they have reached 6 months of age. With therapy, which was described recently in Nature Medicine, the mice are now able to detect sounds at around 45 dB, which can be compared to a standard conversation level. In the mice, this mutation is associated with 1 incorrect letter, which is an A instead of a T, in a DNA sequence of the Tmc1 gene. It is this 1 incorrect letter which marks the differences between deafness and normal hearing.
The system was able to recognize the 1 incorrect DNA letter from a defective-copy across 3 billion letters in the genome of a mouse. Regardless of this success, there is still a lot of work required before this therapy of gene-editing will be appropriate for human use, stated the authors of this study. However, this latest therapy has become necessary to improve the effectiveness and safety associated with the standard gene-editing technique.
Corey told the Hearing Journal that this specific mutation that they were studying in mice happens to be somewhat rare when it comes to humans, which somewhat limits the overall impact of this research. Corey does go onto say that when they analyzed the DNA sequences for the disease-causing mutations that are known in various other genes, they were surprised to discover that many of these, close to a quarter, could also be targeted with the modified SaCas9-KKH.
With the original CRISPR-Cas9 gene-editing tools, they worked in the way of using a "guiding molecule" known as gRNA, in order to identify a DNA sequence that is mutant. When the mutant DNA has been detected, the Cas9 enzyme cuts it off. Up until now, gene-editors have not been very accurate. In order to overcome this challenge, researchers have adapted the tool which was developed originally by Keith Joung, MD, Ph.D., and Ben Kleinstiver, Ph.D., in the way of using a modified Cas9 enzyme derived from Staphylococcus aureus rather than the bacterium Streptococcus pyogenes, that the original Cas9 was derived from. In order to improve the accuracy of detection, the latest system utilizes the gRNA to detect mutant genes along with the optimized Cas9 in order to find the specific DNA mutation.
The idea that is critical to this study has to do with how it is used, along with the guide RNA, of the “PAM site”, in a modified SaCas9-KKH enzyme which mediates a secondary-recognition of a mutant gene, states Corey. He goes onto say that they were hoping this approach would lower off-target effects of the Cas9, which involved either inappropriately cutting of a normal copy of the TMC1 gene or inappropriately cutting off any other genes that are unrelated.
Corey along with his team injected this treatment inside the inner ears of the mice. On further investigation, they discovered that this tool worked as the editing occurred only inside the inner ears of these mice. In order to study this evidence, they also stimulated hair cells of these mice which did not carry these mutant cells. The study also involved measuring auditory-brainstem responses in order to decide whether this therapy worked.
Due to the ability of the tool to target the single-point gene mutations, this system is able to mediate as much as 15 other types of deafness that are inherited which are also caused from a single-letter mutation in the DNA sequences of the other types of hearing genes.
Corey says that they will continue to work alongside other colleagues within the field linked to deafness and hearing in order to apply the method to other types of deafness that are hereditary. They are hoping that others that work in the community of gene therapy will take on these approaches to treat other disorders.
Clearly these are some promising studies being done in the world of research related to degenerative hearing loss. Keep us at Tinnitus & Hearing Center in mind for all of your questions related to hearing loss and your options to improve your hearing. Give us a call at (480) 831-6159 to schedule an appointment or with any questions.
At least 1 in every 10 people in the US suffers from a type of hearing loss. At least half of these people have never had their hearing checked. Keep in mind that hearing problems are likely going to get worse if ignored so you need to get your hearing tested, early and often. In some cases, the damage might be permanent.
Inside your ear, there are tiny hairs that send sound waves to your brain. If there is any damage to these hairs, they will never grow back. You might be doing something to your ear that damages it so you need to find out immediately. Did you know that normal drugs such as antibiotics or aspirin can actually cause hearing problems?
It's important to have your ears checked to take care of any hearing problems immediately. Nerve damage is one of the most common hearing problems. We're an audiologist in Phoenix, and we've seen plenty of patients that come in early enough that they can avoid hearing aids or surgery. But most people wait until their hearing damage is past the point of no return, and need hearing aids to keep their lifestyle intact.
Hearing Aids And Their Benefits
There are so many misconceptions about hearing aids that keep a lot of people from getting the help they need. In the last few years, these devices have actually come a long way. If you have mild hearing problems, you can definitely benefit from wearing them. Of course, you need to get a hearing test to determine the extent of your hearing loss and whether or not you need hearing aids.
You might assume that there is nothing you can do to cure your hearing loss. Previously, doctors were limited when it came to helping people who had hearing troubles in one ear, experienced nerve damage, or who couldn’t hear high-pitched sounds. However, technology has changed a lot of that. All these individuals can make a lot of strides with hearing aids.
"I Don't Want to Look Old" And Other Myths
As much as hearing aids might make you "look old," if you can’t hear, that's actually more of an "old person" stereotype. You need to remain sharp and hearing aids will help you get there. Note that with diminished hearing, you could develop a host of other issues such as dementia. Yes, hearing aids might be slightly noticeable to someone, but so what? Most patients we know would rather hear sounds than worry what people think about some tiny device on their ear. Hearing aids are so small these days that they're barely noticeable at all.
Note that the age of hearing aids making everything sound too loud is in the past. Now, you can enjoy a sane amount of volume to whatever you are listening to, with split-second compression that mutes loud sounds.
Keep in mind that having hearing loss can change the quality of life. Most people eventually develop anger, stress, loneliness, depression, and memory loss, and even dementia. It could hurt your chances of being promoted at work, and prevent you from hearing words from your loved ones, friends, and family. By using hearing aids, you can improve your self-esteem, physical health and much more.
For children, hearing loss needs to be treated very early. Note that if they can’t hear, it can seriously impact their development if they can’t talk, make friends, communicate or develop normally. When babies are born, their hearing should be checked in the hospital or at least when they are one month old. If there are any issues, they should receive professional help immediately. Check on your toddler who has frequent ear infections. Note that the hearing loss caused by ear infections may be temporary but it might lead to bigger problems in the future if not checked early enough.
At Tinnitus and Hearing Center of Arizona, we're a Phoenix Audiologist clinic that consults with patients about hearing loss and hearing aids, conduct hearing testing in Phoenix and the entire Phoenix Valley, including Scottsdale, Chandler, Gilbert, Sun Lakes, Fountain Hills, and more. We also work with tinnitus treatment, misophonia, and hyperacusis. For more information or to schedule an appointment contact us at (480) 831-6159 or visit our website at https://www.tinnitusaz.com/contact.
Know What Hearing Tests Are Before You Take Them
If you live in the metro area of Phoenix, hearing testing might come up when speaking to your doctor, and he/she may ask you to get your ears tested. This does not in any way mean that something is wrong. It is just that the doctor needs you to have a hearing test to make sure that your ears are working as they should.
Hearing loss becomes a possibility as you grow older. Between the ages of 45 and 64, 14% of people have some loss in hearing, and this percentage rises to 30% among people who are 65 years old or older. That is the reason doctors want to have your hearing tested every few years, instead of just once after you have become an adult.
It is recommended by experts in the audiology field to get your hearing tested once every ten years till you cross the age of 50, and once every three years after you have crossed this age. Of course, if you have any concerns about your hearing, you should get a hearing test immediately.
Thankfully, hearing tests are painless, quick, and inexpensive.
The Need for a Hearing Test
At times you may suspect that you have a hearing loss. You may have trouble hearing what is being said to you when you are in a crowded room or find that you have to turn on the volume high on your TV.
Not everybody realizes that they have a hearing problem. Hearing loss is a gradual process, and this prevents it from being realized. So, even if you think you might be fine, and the doctor suggests a hearing test, just do what they say!
Hearing loss in adults can be due to several reasons:
Older people who have hearing loss, and do not do anything about it, will feel they are being left out of social events which they have always enjoyed, just because they are unable to hear what is happening. This inability to hear well can be embarrassing and can lead to their avoiding both friends and family. This isolation can lead to depression unless they get themselves some help with their hearing.
What Can You Expect During Testing
The entire process of testing can take about half an hour, and there is no pain. Adults who undergo these tests will be asked to wear headphones while they listen to tones played at different pitches and volumes in both the ears, but one at a time. Your ability to listen to each short burst of sound will show whether you can hear low or high pitched sounds, loud or quiet sounds, and if any of the ears has hearing loss.
Some of the hearing tests involve listening to speeches at varying volumes and will require to hear them one ear at a time. The voices will come to you quietly in the earphones, and you are required to repeat the words that you have heard. This test can be carried out in rooms that are quiet or noisy, as your hearing of voices can be troubled when there is any background noise.
The Results and What They Mean
A test for hearing does not mean that you have passed or failed. The results will indicate your loss of hearing in each ear and the degree to which it is affected.
Sound intensities are measured in decibels. A whisper is 30 decibels. Normal speech measures 60 decibels, while anyone shouting in your ear will do so at 80 decibels or higher.
Adults who have normal hearing will have a loss of 25 decibels. The degrees of hearing loss are:
At times hearing test results can surprise you when they say you have mild, moderate or severe hearing loss, and this is because the loss of hearing is gradual.
Hearing loss can rarely be restored, but you can make up the loss and also protect whatever hearing you have left.
If you need to wear hearing aids than you can choose aids that come in many styles. They are nowadays much smaller than what the elders in your generation used to wear. Models can go into the ear or sit behind it. Some may be completely hidden in the ear canal.
Some hearing aids are devices that amplify the sound you want to hear. They will be able to make phone calls louder. Others can help you hear better when you are in a theatre or a place of worship. Some specialize in loud, crowded environments like restaurants, and others combine hearing amplification with tinnitus treatment.
Lip reading can help you understand what people are saying. You can learn how to do this with a little training.
Your audiologist can suggest the wearing of earplugs when you go to places where there is too much noise, concerts, restaurants, or even while using loud yard equipment. This can help to prevent any increase in hearing loss.
Sometimes those who live or work with someone that's "hard of hearing" find it difficult to figure out the best way to communicate with someone who suffers from hearing loss.
Certainly hearing aids are the easiest remedy for those who suffer from loss of hearing, but sometimes cost can prohibit the purchase. Lipreading is an important skill for many people who have hearing loss. They are able to recognize the lip shapes, gestures and facial movements of someone when they speak. This helps them to communicate well. Next time you speak with someone with the loss of hearing, assist them to lipread the speaker in a variety of ways.
Here are some simple tips to help you to effectively communicate with them.
Speak slowly, clearly and loudly
It is important that the person understands the conversation. Speak slowly, naturally, and loudly - but NOT shouting or exaggerating mouth movements. Use simple words and short sentences. Repeat yourself if necessary, or rephrase it. Make sure that one person talks at a time. Shouting distorts the sound of speech and the person will find it difficult to understand. Pause to check if the person has understood what you said before you continue the conversation.
Remember that the person with the hearing loss is certainly aware of their condition, but can be tremendously frustrated about their situation. Speak to them in a mature way - after all, it's only their hearing that's affected, not their mental capacity.
Include them in a group and address them by their name
It is important that the person understands the conversation. Speak slowly, naturally, without shouting or exaggerating mouth movements. Use simple words and short sentences. Repeat yourself if necessary, or rephrase it. Make sure that one person talks at a time. Shouting distorts the sound of speech and the person will find it difficult to understand. Pause to check if the person has understood what you said before you continue the conversation.
Reduce background noise
In a social gathering, find a quiet place to talk to help reduce background noise. It will be useful if you switch the TV or the music off when they come to visit. Some people with hearing loss may be very sensitive to loud sounds. You should be aware of this reduced tolerance and consequently avoid loud sounds.
Let the person see your expression
Make sure you are in a well-lighted area where the person can see your facial expression and gestures. This helps in better lipreading and understanding. Also, face the person and maintain eye contact. Remember, not to hide your mouth, eat, or chew gum while speaking. Beards and mustaches may act as barriers as well. Additionally, avoid talking from another room for the same reasons.
Be patient, stay positive and relaxed
If you are talking to someone who has loss of hearing, it is vital to make an effort by being patient and tolerant. If you relax and make the person feel relaxed, then it would help the person communicate better.
Familiarize the listener with the general topic of the conversation
If you change a topic without warning, it might confuse the person with hearing difficulty. Avoid sudden changes of topic and warn about the change. When you are in a group, it may help if you repeat the important points and information before you continue with the discussion.
Pay attention to the listener
Expression speaks a thousand words. If the person has a puzzled look, it may indicate misunderstanding. Be tactful and make sure the person has understood you, You may ask leading questions so you know your message got across.
Help them readjust their hearing
After a performance or a conference, allow the hearing-impaired person a few moments after the event is over to readjust their hearing. By either mentally or manually (changing the program on a hearing aid, for instance). They likely cannot hear you if you whisper to them in the middle of a performance as well.
Provide information in writing
Consider providing specific information like directions, phone number, work assignments in writing. You could talk them through the details and then provide the same thing in writing or drawings. That way, you would be sure that the person has the information. Alternatively, you could ask them to repeat the specifics back to you.
If you suffer from hearing loss, we would like to hear from you. Do you have any tips that would help us communicate better with you?
At Tinnitus & Hearing Center of Arizona, we know how challenging it can be for those with hearing loss, and their friends and family. The good news is that there are options to improve your hearing condition and your lifestyle. Give us a call to schedule a consultation and free screening, and we can discuss hearing testing as well as options to make life easier for you and those around you. We conduct the type of hearing tests Scottsdale and Phoenix valley residents appreciate for our kind and genuine approach. Call us at (480) 831-6159 or use our Contact page to schedule an online appointment or email us a message.
Throughout life, music plays an important role in individuals’ everyday experiences.
Presbycusis or age-related hearing loss is common in elderly people that occur because of natural neurodegenerative processes. It affects the cochlear receptor cells and brain circuits that are involved in auditory perception. Clinically, older people may have high-frequency hearing loss on both sides and reduced ability to understand speech. Using a hearing aid is the best way to deal with hearing loss at old age. However, you may wonder, if the ability to differentiate different notation of music changes too.
Can you enjoy music at old age?
Even though aging is associated with a decline in both cognitive and auditory abilities, whether aging damages music perception remains controversial. The question is, do you enjoy the harmony of different musical notes?
Harmony is the process when two or more notes are played at the same time. It is composed of simultaneously occurring frequencies, pitches (tones, notes), or chords and this is analyzed by hearing. When two musical notes are compatible, the musical chord is pleasing and evokes a sense of resolution or “consonance." A mix of different frequencies, on the other hand, might evoke a feeling of tension or “dissonance”. Harmony is a combination of this consonance and dissonance.
Even though anecdotal evidence suggests that harmony is enjoyed by the older population, interestingly, a study revealed that older listeners found consonant chords less pleasant compared to younger listeners and had less distinct neural representations of consonant and dissonant chords. Which means that there may be age-related differences in perception which is likely to be related to differences in neural temporal coding.
Despite aging, music perception is relatively spared. How does that happen?
Other studies, however, strongly suggest that the decline in music perception is relatively spared. Despite relying on auditory and cognitive abilities that tend to decline with age, it is likely that older adults engage in some form of a compensatory mechanism in the way the neurons in the brain processes music. Scientists have shown that the underlying mechanism to process music is very different between young and old but the ability to perceive it is similar.
Both could equally detect inconsistencies in music. Compared to the younger, the older person had reduced perception of amplitude but increased perception to detect out-of-key notes. This means that the perception of the tonal structure is preserved in older adults, despite aging.
Music training and education are important factors
Evidence shows that if older individuals were musically trained, their perception of music was better than the untrained older individuals. Also, the level of education seemed to make a difference. Performance of temporal tasks, which includes enjoying musical notes, was better in highly educated older individuals.
This means that music training and education are important factors as they can slow the deterioration of music perception produced by age-related hearing loss.
Aging does not alter specificity to music
Further evidence on the age differences in music perception reveals that even though the young and old have differences in the perception of valence and arousal in music, there is no difference in perceiving musical notes. Valence means whether the music has a positive or negative effect, and arousal measures how calming or exciting the music is.
The study also found that there was no difference in perception of the quality of the music. This reiterates that with aging, music perception does not change.
If you are above 65, you may need a hearing aid due to aging, but you will still distinctly recognize and enjoy all the musical notes.
Call us at (480) 831-6159 to schedule a consultation with Sun Lakes audiologist Tinnitus & Hearing Center of Arizona. We're a friendly and knowledgeable Audiologist clinic near Sun Lakes AZ that knows musicians' needs so much that we're known as "The Musician's Clinic!" Even if you simply love to listen to music and have concerns about your hearing, we'd love to show you some great options to improve your everyday life.
Dr. Rohe is a nationally-recognized audiologist specializing in Tinnitus Therapy and other hearing conditions.