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Harvard studies protein on the hair cells in the cochlea

EERecordist

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Its failure can cause specific kinds of deafness.

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This can be very promising, also in context of another, rather rare phenomenon: There are "a handful" of people, who almost don't develop hearing loss with age, even despite noisy workplace and such. There must be some genetic/biochemical cause, but I haven't seen much research on this. From anamnesis (maybe 10 such patients in my working life) it seems to run in families, so probably genetically based. But no guarantees - my grandmother was such a case, she could hear very well aged 90, my mother had to get hearing aids before 80.

Maybe we can "undeaf the deaf" one day, who knows...
 
Or, if we're less lucky, we'll be back to the stone age (hopefully not).
 
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COMMENT:
Certain helices of the CIB2 structural complex which has a negatively charged electrostatic potential connects to a TMC1 pocket for normal hearing. Genetic mutation of the loop EF3 (represented above in orange) has a molecular residue with altered conformation. Thus shifting it in such a way that it has poor binding to calcium for signal activation and that plays out as reduced TMC1 binding so the ear hair cells can't perform properly.

See free full text of below review available on-line for more details.


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This can be very promising, also in context of another, rather rare phenomenon: There are "a handful" of people, who almost don't develop hearing loss with age, even despite noisy workplace and such. There must be some genetic/biochemical cause, but I haven't seen much research on this. From anamnesis (maybe 10 such patients in my working life) it seems to run in families, so probably genetically based. But no guarantees - my grandmother was such a case, she could hear very well aged 90, my mother had to get hearing aids before 80.

Maybe we can "undeaf the deaf" one day, who knows...
A friend has some kind of degenerative cochlear disease she knew about in college and prepared. She now has a cochlear implant. She benefitted from language development in the cortex from hearing when young and studied at a top university. In all my air travels, I have had two most moving conversations with a seat mate. One was with a mother bringing her second child back from a cochlear implant. On birth of the second, the first asked if the new second would be deaf. My university was in the same town as a major deaf school, so I follow the topic. Thanks for participating in ASR.
 
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A friend has some kind of degenerative cochlear disease she knew about in college and prepared. She now has a cochlear implant. She benefitted from language development in the cortex from hearing when young and studied at a top university. In all my air travels, I have had two most moving conversations with a seat mate. One was with a mother bringing her second child back from a cochlear implant. My university was in the same town as a major deaf school, so I follow the topic. Thanks for participating in ASR.
Remains an option for my mother, should she go "stone deaf". IDK however, if she will want surgery at this age. Luckily, so far the hearing aids are sufficient. Fun fact: She prefers watching TV with headphones (Beyerdynamic DT-990) over these hearing aids (Signia, with DSP).
This way she can listen less loud and understands dialogs better. No room influence.
Peculiar, but works for her.
 
This can be very promising, also in context of another, rather rare phenomenon: There are "a handful" of people, who almost don't develop hearing loss with age, even despite noisy workplace and such. There must be some genetic/biochemical cause, but I haven't seen much research on this. From anamnesis (maybe 10 such patients in my working life) it seems to run in families, so probably genetically based. But no guarantees - my grandmother was such a case, she could hear very well aged 90, my mother had to get hearing aids before 80.

Maybe we can "undeaf the deaf" one day, who knows...
That's highly interesting indeed. I imagine it must be very hard to research because while patients/subjects may be able to tell you what they hear and what they don't, it's difficult to test beyond basic response/ear pickup capability. And the biomechanical and chemical reasons are even harder to examine.

Great times for science anyway, examination and research technology has made great strides the last decades.
 
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