Anemia, Pernicious & Sickle Cell

[waltky]

Uncle Ferd thinks dat's why Granny always sayin', "Huh, whad ya say?...

Some Hearing Loss May Be Related to Anemia, Study Finds
January 03, 2017 - Hearing impairment in some people may be related to anemia, a common condition in which there is not enough iron in the blood, researchers think.

In a study of more than 300,000 U.S. adults aged 21 to 90, researchers at Pennsylvania State University's College of Medicine found the prevalence of hearing loss among participants overall was 1.6 percent, compared with 3.4 percent among those with iron-deficiency anemia. The findings were published in the journal JAMA Otolaryngology-Head & Neck Surgery.

The most common forms of hearing loss associated with anemia in the study were sensorineural hearing loss, in which the nerve pathway to the brain is damaged, and conductive hearing loss. In that condition, there is a problem with external sounds being conducted to the middle ear and eardrum. Many patients in the study who had suffered hearing loss had both kinds. However, researchers say, it's possible that correcting anemia with supplemental iron could improve the conditions. That's a future area of study.

A research audiologist conducts a hearing test on a patient at the Phonak US Audiology Research Center in Warrenville, Ill.

Researchers say it makes sense that loss of hearing might be caused by anemia. The inside of the ear is very sensitive to oxygen, and it's possible that the nerves there are not getting enough in someone who is iron-deficient. Iron is an essential element for the production of hemoglobin, the red blood cell protein that carries and releases oxygen throughout the body.

Hearing loss is common as people age. When the loss is sudden, in particular, researchers say doctors ought to consider iron-deficiency anemia as a possible cause. According to the American Speech-Language-Hearing Association, approximately 15 percent of U.S. adults complained of hearing loss in 2014. To the extent that iron deficiency may play a role, experts suggest eating a well-balanced diet, containing an adequate amount of nutrients and iron for optimal health.

http://www.voanews.com/a/hearing-los...s/3661824.html

[waltky]

Pioneering treatment to change DNA reverses sickle-cell anemia...

Teenager's sickle cell reversed with world-first therapy
Thu, 02 Mar 2017 - World-first therapy has reversed one French boy's blood disease.

A French teenager's sickle cell disease has been reversed using a pioneering treatment to change his DNA. The world-first procedure at Necker Children's Hospital in Paris offers hope to millions of people with the blood disorder. Scientists altered the genetic instructions in his bone marrow so it made healthy red blood cells. So far, the therapy has worked for 15 months and the child is no longer on any medication.Sickle cell disease causes normally round red blood cells, which carry oxygen around the body, to become shaped like a sickle.

Healthy red blood cells are round, but the genetic defect makes them sickle shaped

These deformed cells can lock together to block the flow of blood around the body. This can cause intense pain, organ damage and can be fatal. The teenager who received the treatment had so much internal damage he needed to have his spleen removed and his hips replaced. Every month he had to go into hospital to have a blood transfusion to dilute his defective blood. But when he was 13, doctors at the Necker Children's Hospital in Paris did something unique.

'No sign of disease'

Doctors removed his bone marrow - the part of the body that makes blood. They then genetically altered it in a lab to compensate for the defect in his DNA that caused the disease. Sickle cell is caused by a typo in the instructions for making the protein haemoglobin, which is densely packed into red blood cells. A virus was used to infect the bone marrow with new, correct instructions. The corrected bone marrow was then put back into the patient.

The results in the New England Journal of Medicine showed the teenager has been making normal blood since the procedure 15 months ago. Philippe Leboulch, a professor of medicine at the University of Paris, told the BBC News website: "So far the patient has no sign of the disease, no pain, no hospitalisation. He no longer requires a transfusion so we are quite pleased with that. "But of course we need to perform the same therapy in many patients to feel confident that it is robust enough to propose it as a mainstream therapy."

'Given his life back'

Prof Leboulch is nervous about using the word "cure" as this is just the first patient to come through clinical trials. But the study does show the potential power of gene therapy to transform the lives of people with sickle cell. "I think it's very significant, essential they've given him his life back," said Dr Deborah Gill from the gene medicine research group at the University of Oxford.

A normal red blood cell next to a sickle cell

She told the BBC: "I've worked in gene therapy for a long time and we make small steps and know there's years more work. "But here you have someone who has received gene therapy and has complete clinical remission - that's a huge step forward." However, the expensive procedure can only be carried out in cutting-edge hospitals and laboratories, while most sickle cell patients are in Africa. The next big challenge will be to transform this pioneering science into something that really can help millions of people.

What is sickle cell disease?

[resister]

Uncle resister says, eat some really fine iron filings, it'll fix ya right up!

[waltky]

Restoring hearing to gerbils may lead to high-tech cochlear implants...


Pulses of light restored hearing in gerbils. Could that lead to higher-tech cochlear implants?
Jul 14, 2018 - Could light one day be used to restore hearing?

To try to answer that question, a team of German bioengineers surgically installed coiled strips of optical fibers in the ears of deaf gerbils. While they still had their hearing, the gerbils had learned to hurdle a small barrier upon hearing an alarm. Now researchers sent a pulse of blue laser light deep into the animals’ ears. They jumped. he experiment was part of a study published Wednesday seeking to improve upon cochlear implants — electronic devices that stimulate auditory neurons to partially restore hearing. Instead of using electrical currents, scientists are trying to determine whether optogenetics, a new field that uses light to control living cells, could one day help improve someone’s sense of hearing.


Although it could take decades to use optogenetics-based technologies in humans, researchers are beginning to demonstrate that light, not electricity, may be the best way to convey the rich information contained in sound. “Through my experience with patients, I’ve recognized the huge potential of cochlear implants,” said Tobias Moser, director of the Institute for Auditory Neuroscience at the University Medical Center Göttingen and lead author of the new study. “At the same time I’ve also witnessed the shortcomings.” Patients with cochlear implants often describe the sound quality through the devices as harsh and tinny. Listening to music or picking out one voice from several others is often impossible. The goal of the new research, said Moser, is to improve the technology and create “a more natural hearing so that patients can recognize the melody in music and speech.”


At the most basic level, the act of hearing is transforming sound into electrochemical signals, the language of neurons, that the brain can then interpret. Much of this process occurs in the cochlea, a snail-shaped organ within the inner ear lined with specialized sensory cells called hair cells. When hair cells detect vibration through thin protrusions on their surface, they generate electrical current in neighboring nerve cells that travel to the brain. In people who have dysfunctional or dead hair cells, cochlear implants work by electrically stimulating auditory nerve cells directly. According to Dan Polley, an associate professor at Harvard Medical School and director of the Lauer Tinnitus Research Center who was not involved in the study, the successes and limitations of the cochlear implant are determined by the anatomy of the inner ear.


Within the cochlea, hair cells rest on an organic platform known as the basilar membrane that is floppy and wide on one end and narrow and taut on the other. This biomechanical organization causes hair cells to wiggle in response to specific sound frequencies or pitches that are mapped out smoothly from low to high, like keys on a piano. When implanting cochlear implants, Polley explained, surgeons thread electrodes into specific locations along the basilar membrane to target nerves that are sensitive to particular frequencies. With cochlear implants, however, electrical current spreads itself over a large area and activates not only the targeted nerves but also neighboring cells as well. This impercision distorts and muffles sound.


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