I am a 21 year multichannel cochlear implant user that is profoundly deaf bilaterally. I was severely hearing impaired at age three from a double case of mumps, and as a result of physicians using aminoglycoside antibiotics repeatedly for my recurring rhuematic fever I lost all of our hearing--every bit of it over time and balance in my vestibular organ. I was somewhat of a child prodigy, studied hard, and utilized speechreading (trained by the United Way Speechreading Program in Denver, Colorado in the early 1960s), all of which enabled me to attend hearing schools and to remain on the Dean's Honor Roll throughout school, and later college. Because of the cochlear implant, I was able to regain some footing after my speech severely degraded in my lecturing and went back to school to earn four more advanced degrees. I credit this technology as saving both my peronal and professional life, without which I would just have been another deaf man highlly and utterly dependent on society, instead of building enterprises that cumulatively employed thousands and brought many innovations and knowledge to the healthcare field, and also to the field of music, where I still perform and work to inspire kids everywhere to use music to overcome learning disabilities. Graham Clark's and  others' innovation of the cochlear implant has made possible nearly all that has transpired good in my life these past more than two decades. I will be forever grateful for this technology coming out at a time of my life when the lights were about to go out, and my life. personally and professionally, would have been severely limited.     

Dear Max,

I´m not able to access cochlea implant techniques, but I´m deeply impressed by your description of your personal fate after a mumps dilemma in your childhood. So reading about your disease I´m convinced, that cochlea implants have very important aspects.

But I´m not sure that not cured deafness really causes IQ deficits.

Dear Max Stanley Chartrand - thanks for your enlightening reply.

-Sundar

Dear Wolfgang, I'm a teacher who knows how much learning can take place through the human ears especially for Auditory Learners who just have to focus at lectures and remember stuff for a long time.  So I'm inclined to see the Pros.  And we have 2 ears to hear, and 1 mouth to speak, so let me talk less.  Let me place a link here, thanks.

http://www.theguardian.com/society/2014/aug/12/europe-leads-on-cochlear-implants

The IQ deficits goes back to every study, including John Myklebust of the 1980s, which shows the cognitive performance of deaf children as a group came out lower than normal hearing children, age and otherwise matched. I know it is a bitter pill to swallow, just as the finding that about 90% of the autistic spectrum are boys, and the these geneder proportions carry into the penal system, lifespan, special education in general, and just about every other demographic that describes men and women. To insist a child remain deaf and a burden to society is to consign them to a life of dependency and underachievement. True, a few savants and/or exceptions will break the stereotype, but they are rare and not in the norm by any means. To be deaf by choice is never a good thing despite the self-interests that may say otherwise. 

Max - your personal story makes for compelling reason to do cochlea implants.  I am not hearing impaired, so I do not feel adequately equipped to answer such question.  Having said that, I also wonder why the students at Gallaudet are opposed to such.  So much so, that when there was a nomination of the President of the university, there was opposition due to the cochlea implants she had received - http://en.wikipedia.org/wiki/Gallaudet_University.

Dear @Max, thanks for sharing this question with me. I am delighted by your 21 years of experience regarding cochlear implant! @Wolfgang, I have a mother in low who was not in situation to get the implant but hearing aids! I am very familiar with her problems.

In Serbia, the situation about Cochlear implant is different.10 years since the 1st Cochlear implantation in Serbia was celebrated on May, 17th!!!!!

http://www.serbia.embassy.gov.au/files/bgde/CochlearAUDIO%20BM%20conference%2017%20May%202013.pdf

Dear Max,

When I consider your accomplishments-- for instance, your publication of "How to Overcome ADHA and Other Learning Disabilities without Harmful Drugs" (DigiCare 2010)--, I can only rejoice that you had your cochlear implants, that you avoided IQ impairments, and that you are performing services for the world community of which most of us can never dream.  If anyone is reading this chain, I would urge him or her to go to Max Stanley Chartrand´s RG profile page and study the maximum  (and optimum) that the recipient of cochlear implants can do with his life. This would seem to obviate all objections against the procedure. 

Cochlear implants do the work of damaged parts of the inner ear (cochlea) to provide sound signals to the brain.

I agree upon using of cochlear implants simply because any "hearing" is better than "no hearing at all". As chemistry bothers a lot about safety, deaf persons will face real trouble if they are allowed to work in laboratories or industrial chemical plants.

Professors Muss &  Chartrand ought to be thanked for this important question. I really hope that there will be a motivation for some RG scholars to carry out "applied research" which aims at improvement of cochlear implants, reducing their costs, and increasing their efficiency so as to be suitable for all ages.

@Wolfgang and @Max,  I was not sufficiently informed on the cochlear implant. So I went to read up on the subject and I carefully read all the speeches in this forum. Aside from the pro's abundantly celebrated, it does not seem to have found some important con's  if we exclude some cases of infectious origin of the defect (meningitis). In view of the expertise and personal experience of our friend Max, I should just like to know if I understood what I red.

A disadvantage of cochlear implants was it that

patients (e.g. MS, stroke) could not be scanned with

magnet resonance. Here is an article about

an MRI-compatible implant (Wolf-Dieter Baumgartner,

AKH Wien).

http://derstandard.at/2000004365364/MR-kompatibles-Cochlea-Implantat-erstmals-in-Oesterreich-eingesetzt

I suppose it is this one:

http://www.presseportal.de/pm/62623/2764452/in-sync-with-natural-hearing-med-el-sets-next-milestone-with-a-new-cochlear-implant-system-foto

Regards,

Joachim

Dear @Wolfgang, thank you for the question; despite that I am not familiar with the issue of  Cochlea Implants' advantages and disadvantages but the answers of dear @Max are very informative that explain the issue clearly.

Uptil now CI with respect of its indication criteria is the best solution of bilateral profound SNHL. Also, biological hearing restoration by intracochlear stem cell implantation is a new promising advance but unfortunately it is still an experimental trials.

Dear all,

unfortunately I am not able to answer to all the posters meanwhile at once. So I shall do it step by step... I am overwhlemed!

Thank you Joachim for the really interesting web-URL of the most recent News on CI-Research and technology by MED-EL (Austria). The first URL links to a news-article in an Austrian Newspaper (dated 14th of August, 2014) which - in the Posting-section (see below the article, in German) initiated a "heavy" fray/skirmish (in part translated here) about origin,  (invention, development, transplant), stating also that the photo of the CI is not the cited one but an older CI from the "rival firm" ( of the  >>>Australian(SIC!)  firm:" Cochlear", shaming the poor recherche of the "STANDARD" news paper editorial office).

The new device, said to be developed by Prof. Hochmair  (http://de.wikipedia.org/wiki/Erwin_Hochmair) at the TU (Technical University) in Vienna (he moved later then to Innsbruck, Austria), was brought to series production readiness and market launch in Innsbruck at MED-EL (started with 4-10 employees in the 1990ies, now 1400 employees ). It may be / is the model "Synchrony" from MED-EL, and is said to be a miniaturized version of the ), but was transplanted some weeks ago (by Wolf-Dieter Baumgartner from Univ.Clinic. of Oto-Rhino-Laryngology, Medical Univ. Vienna, http://www.hear-ring.com/hearring/?q=en/expert/12; e-mail: [email protected]) to a 42 years old patient in Vienna, Capital of Austria.

Also it has been mentioned in the German postings that CI's including the magnet hitherto were MRI-able up to 1.5 Tesla. Additionally, one poster says "not mentioned were the contributions of Frank Rattay in the field of simulation and stimulation of neurons (approx. 50 „dissertations“ = publications I guess, Prof. Dr. scient.med., Frank Rattay,TU Vienna) as well as the work of Prof. Kurt Burian (1924-1996), who implanted on 16th Dec. 1977 the first multichannel microelectronic CI in Vienna.

http://www.medel.com/at/history/   (only in German), http://www.medel.com/history/ in English) or generally: http://www.medel.com/int/ (International/English)

The second URL links to a MED-EL press release, dated 18th of June 2014, so this is perhaps really the first source: ).

Hope this is of value to any reader, very best regards and thank you to ALL previous posters.

Regards, Wolfgang

All: The MRI compatibility issue and the very miniscule number of Meningitis cases with one company of which I am aware, are tiny objections to the implementation of the technology for the deaf. Mine is not MRI compatible, but has never posed a problem and I travel all over the world 100,000-150,000 miles per year for many years now without difficulty. The early single channel devices have nearly all had to be explanted to my knowledge (I used to work for Cochlear Americas, incidentally, and cannot speak to all the brands out there--but some have had problems)--I helped with about 100 of these cases about 20 years ago. The mutlichannel devices have been phenomenal, each generation of technology and reliability improving vastly over the past generations. I consider it a boon to the deaf who will avail themselves to taking advantage of it, although I recognize some might not be amenable to it. Those who lost their hearing in adulthood do much better with theirs than I do with mine, but I still count it a lifesaver and blessing to me in empowering me to stay connected to the hearing world. Thank you all for your insightful comments.

Thank you max for sharing your story with us.  I fully support cochlear implants as it enables people to be independent of others and participate actively in the community

I also support all previous comments.  Thanks for the story. 

Max and others:

There have already been many valuable contributions and comments - several quite inspiring - above on this critical topic introduced by my colleague Max Chartrand whose own comments and postings here represent exceptional intelligence and insight on this issue, so I will restrict my attention to a targeted focus and try to bring my expertise in evidence-based systematic review and meta-analysis to provide a robust evidentiary foundation to the questions of the efficacy and safety of cochlear implants, based on a critical review I recently conducted and completed. I provide below a brief plain-English summary of what the best critically appraised and systematically reviewed data has determined on these important issues.

The Issue of Safety: although no surgical intervention is free of complications, nonetheless hundreds of studies as well as systematic reviews and meta-analyses have decisively confirmed that cochlear implantation is a safe surgical hearing rehabilitation technique associated with a low complication rate: the global complication rate of major complications hovers at no more than 5% or less1,2 , making it one of the safest FDA approved medical devices currently marketed.

The Issue of Quality-of-Life (QoL): In addition, a recent comprehensive Systematic Review and Meta-analysis from the Coverage and Analysis Group at the Centers for Medicare & Medicaid Services of 42 studies was the first - and to date only - evaluation of the quality-of-life (QOL) benefits of cochlear implants, in addition to their efficacy3. The CAG-CMMS concluded that unilateral cochlear implantation was an effective method for improving speech perception and health-related QoL in adults with severe to profound sensorineural hearing loss and that compared with unilateral implantation, bilateral cochlear implantation provided further improvements in speech perception. These findings are consistent with and cross-confirmed by the results of the UK NICE (NIH Clinical Excellence) authoritative guidelines4, in particular the conclusion that speech perception and QoL improve with unilateral and bilateral implantation, especially in noisy conditions and that they improve academic performance and may increase the likelihood of children remaining in mainstream education, and facilitate social interactions, social dynamics, and social communication in both adults and children.

The issue of MRI Compatibility: This issue, although real for some - but by no means all - older cochlear implants, is now, given advances in cochlear implant technologies and in the selectivity of different MRI systems (under "Tesla" categories of MRI technology), as well as ingenuous new deployment of local protective bandaging for MRI patients with cochlear implants, is decidedly less critical than widely believed formerly. First, all cochlear implants made from nonmagnetic and nonconducting materials (hundreds) are rated MRI Safe. Second, middle ear ossicular prostheses type cochlear implants are rated MR Conditional for 1.5-Tesla MRI (except for the 1987 McGee prosthesis), with many also approved for 3-Tesla MRI systems. Third, all cochlear implants with removable magnets are approved for patients undergoing MRI at 1.5 Tesla after magnet removal. Fourth, the MED-EL PULSAR, SONATA, CONCERT, and CONCERT PIN cochlear implants are also approved for patients undergoing MRI at 1.5 Tesla by the application of a protective bandage, and the MED-EL COMBI 40+ can be used in 0.2-Tesla MR systems. Thus, there are hundreds more cochlear implants with acceptable MRI safety ratings5.

Further Reading and Researching: The indisputable definitive compilation, used by NIH as well as dozens of international regulatory authorities, is known as the MRISafety List6, an invaluable resource for patients and radiologists, maintained regularly by the esteemed Frank Shellock, radiologist and physiologist and USC, also Director of MRI Studies at the Biomimetic Microelectronic Systems of the National Science Foundation (NSF), and the Founder of the distinguished Institute for Magnetic Resonance Safety, Education, and Research (IMSER)7.

SUMMARY

In sum, from a perspective of robust efficacy, plus an uncommon level of safety and freedom from major complications, and a high level of personal and societal benefits to QoL, academic performance and global learning effects, and enhanced and productive social exchange and interaction, cochlear implants represent an inestimable technology contribution to human advancement, for which with complete justice, the prestigious Lasker–DeBakey Clinical Medical Research Award8 granted September 2013 recognized the contributions of three pioneers of cochlear implantation, Graeme Clark, Ingeborg Hochmair, and Blake Wilson, and concluded that "their collective efforts have transformed the lives of hundreds of thousands of people".

Methodology for this Review

A search of the PUBMED, Cochrane Library / Cochrane Register of Controlled Trials, MEDLINE, EMBASE, AMED (Allied and Complimentary Medicine Database), CINAHL (Cumulative Index to Nursing and Allied Health Literature), PsycINFO, ISI Web of Science (WoS), BIOSIS, LILACS (Latin American and Caribbean Health Sciences Literature), ASSIA (Applied Social Sciences Index and Abstracts), and SCEH (NHS Evidence Specialist Collection for Ethnicity and Health) was conducted without language or date restrictions, and updated again current as of date of publication, with systematic reviews and meta-analyses extracted separately. Search was expanded in parallel to include just-in-time (JIT) medical feed sources as returned from Terkko (provided by the National Library of Health Sciences - Terkko at the University of Helsinki). Unpublished studies were located via contextual search, and relevant dissertations were located via NTLTD (Networked Digital Library of Theses and Dissertations) and OpenThesis. Sources in languages foreign to this reviewer were translated by language translation software.

References

Constantine, once more I cannot express enough my gratitude for your thorough, insightful, factual, and timely analysis. This is publishable quality. Do we have your permisson to reference and to quote this along with appropriate credits intact?  

Dear Constantine, dear Max, dear all,

I am grateful too for all the important facts and the thorough, insightful, factual, and timely analysis you have provided to us/me.

I would have not been able to get all these data out of the web sources and research protocols whether they have (had) been published in journals or available as pages in the  www.

This will help enormously to advise or at least recommend or help someone affected or in need for thorough information along to get to the right conclusion.

Respectfully yours, Wolfgang

Sorry, it is totally out of my knowledge field!

(But I can 'hear' what the specialists will write here...)

Great and useful discussion. Thanks for the question

Said that great deaf & blind lady, Helen Keller: “The problems of deafness are deeper and more complex, if not more important, than those of blindness. Deafness is a much worse misfortune. For it means the loss of the most vital stimulus--the sound of the voice that brings language, sets thoughts astir and keeps us in the intellectual company of man.”

What Helen knew, and what popular thought overlooks, is that the human sense of hearing represents the primary cognitive window into life itself. “Blindness cuts us off from things; deafness cuts us off from people…to be cut off from hearing [people] is to be isolated indeed.”

In her later years Helen Keller confided in a letter to a friend that “after a lifetime in silence and darkness, to be deaf is a greater affliction than to be blind...Hearing is the soul of knowledge and information of a high order. To be cut off from hearing is to be isolated indeed.”

I am not an expert. It is really heartening to read Max story. A few publications of such applications:

Dear Max,

I agree 100%....and in the school for ("nearly" and totally ) deaf  (impaired) children my son in the 1980ies attended I have learned this too (fortunately NOT with my son, but with the 5 other children being until they left grammar school (around 9 or ten years of age). My son (now 36 of age) speaks just like a person (if people don't know about his hearing loss), naturally is able to "read" from the lips 99%, as well as is capable of using the gestures of deaf/ handicapped people and he also is able (carrier of only hearing aids bilaterally) to make phone calls, especially when he has learned or knows the voice melody or voice character of any person he spoke to present physically (naturally those he is familiar with) using also the phone magnetic adaptor device. But this age-group will do their communication faster by rather than by speech..(;-))

Apologize If I am using terms like "deaf", impaired or handicapped people...I am not really informed about how these terms nowadays should be replaced by "conform" terms.

Best regards, Wolfgang

This is a very good discussion, I learnt a lot!

Max:

Thanks for the appreciation.

And yes, the permission you request is hereby granted. I should note that there is a fuller version of the paper available both on my Academia.edu profile (https://independent.academia.edu/ConstantineKaniklidis) and my LinkedIn profile (https://www.linkedin.com/in/constantinekaniklidis), and also attach for convenience to this posting.  Should you need a version which is not accessed restricted, let me know and I can forward it directly to you.

Constantine

Wolfgang:

Your mode of expression is just fine, and your intent and insights, both personal and professional, comes across clearly and powefully. Thanks for your contribution!

Kind regards

Constantine

To be honest:

Dear all, and especially Constantine Kaniklidis and Jérémie Guignard, thank you so much for all the information given so far. This is really of great help.

@Constantine: thank you for granting your [using your papers for referencing or eventually handing over  /  forwarding  to others)  hopefully also to me and for your kind personal words in your recent post. 

I am also convinced now that cochlear implant technology has its

All good points, Jeremie, I worked with a couple of your colleagues in the early program at Mass Eye and Ear back in early, mid-90s establishing the early referral networks from regional hearing related practices. Your group did outstanding work in establishing habilitative programs in children's cochlear implantation. Your group was most instrumental in establishing concepts relative to neural plasticity in children and their brains' ability to adapt to the inputs of CI processors. Post-surgical hab/rehab are critical factors where the neural learning curve in children could go the full span of childhood development and adult rehabilitative span averaged about 2 years or more. The fact that early implant recipients like myself (implanted in 1993) are still going strong and the technology is still viable in terms of backwards/forward compatibility and support is one of the more stunning successes in free enterprise oto-prosthetics. The cost/benefit in terms of quality of life and lifetime earnings emanating from the procedure, technology, and rehabilitative supports (three totally separate entities, incidentally) are well established. Even with a handful hiccups alone the way--as you mentioned surgeon skill is paramount--the industry's sensitivity to these incidents is quite impressive. In many industries, when liabilities increase and bumps are run into, the tendency is to just write it off as risk and ignore the other guy's problems. But in this field my experience has been that competitors always assumed risks of the other companies and felt a community obligation to do what they could to rectify and unanswered failures when needed. Thank you for your most cogent assessment of the multifaceted CI field.

Thanks to Prof. Wolfgang H. Mussfor it’s really an interesting question. I am strongly agreed with the answers of Prof. Constantine Kaniklidis, Prof.Max Stanley Chartrand  & Prof. Joachim Pimiskern .

Dear Gopinath R., you're welcome.

Thank you for commenting this thread.

Just in case one of my colleagues or perhaps my "boss" will be reading this thread and the postings:

Since I am   i have clearly to state that I am no "Professor"...  did not reach that goal...   but I am happy to be addressed with my first name in this RG-platform...  since I am an informal person (as most RG-members are...) (:-))

best wishes and regards, Wolfgang

I once worked with a child who was deaf and her mom was reticent to go through the with the implant.  She finally did and I will tell you the world exploded for this little girl who spent 10 or so years while mom studied the development of the implants.   This little girl blossomed into a socialite when before she was a wall flower. I am sure this mom would do it all over again if she had to...

Fantastic, Carla. It's all about human development and potential. The cochlear implant is without a doubt one of the most beneficial of innovations, not to mention the habilitative/rehabilitative support behind them. Thank you for the remarkable case history.

I enjoyed reading this thread. It came to life personally through Max's description and technically through Constantine's review analysis summary and diagrams.

I believe this is the same Constantine Kaniklides who was so helpful to me in graduate school in introducing me to the writings of Gustav Herdan in the uses of quantitative methods in linguistics, and sharing interesting conversations about it. I appreciate his enthusiasm and public interest.

Herb

Herb, thank you for your thoughts. Yes, your description of Constantine Kanaclidis sounds like the one I know well from his work and at a distance. There's a wealth of information at his website www.evidencewatch.com, which I recommend often to colleagues and students alike. 

One of our readers of this Question wrote me that that CIs are "100%" effective and a miracle"---I would agree that close to 100% of those implanted receive measurable benefits, the outcomes of which may vary from as little as 20% hearing only speech discrim to as high as possibly 90% hearing only speech discrim--in quiet. In noise, both of these extremes of outcomes degrade terribly because in real life only the human brain can decide what is noise and what is not--technology has no clue as to which is which and can only assume that speech babble, for instance, is "noise", but to remove the babble is to remove components needed to have speech understanding...hence, the need for auditory rehabilitation when one is implanted or they might not enjoy the benefits they would otherwise enjoy. Attend and squelch, an acquired ability starting at about age five for normal human beings until they learn how to attend to that which they wish to hear and to squelch that which they wish to exclude, poses an extra challenge for the deaf who have either lost or never acquired it. We call it "habilitation" in cases like mine where hearing loss occurred before attend/squelch could be learned, and "rehabilitation" where it is relearned in cases of adulthood occurring deafness. Both are a challenge, but the former probably poses the biggest challenge. Even to this day I am still learning how to attend and squelch, some 21 years post-implantation.

Dear @Wolfgang, @Max and friends, I have just seen the following publication at RG that is just announced! It may be useful for some followers!

Article Cochlear implantation in children with bacterial meningitic ...

Dear Ljubomir, thanks for the hint.

Dear Max, Ljubomir,

thank you for the appreciated comment(s) on and (I fully agree! Something similar applies to hearing aids too)  and the aviso for the article  on bacterial infections and consequences for cochlear implant (the full text of which I have requested some minute ago).

and all other followers of this thread,

thank you  for your contributions,

have a beautiful and regenerating weekend,

Wolfgang

Yes, hearing aids share the same attributes in terms of overcoming atrophy of the neurological systems. If we don't use (the functional aspects) we definitely lose them--so the road back is a long one for many. The worse the loss and the greater time lapse before correction the longer the road back.

Who can know that to be deaf is more handicapped than to be blind? If the blind is asked about his first dream in life, he will say to be able to see. Also, if the deaf is asked about his first dream in life, he will say to be able to hear. Success in life  of a healthy or partially healthy person depends on the well of each person & a healthy environment because a healthy intelligent brain is not alone enough to succeed. Even healthy senses including vision & hearing are not enough to succeed. Our life includes many examples of handicapped succeeded persons. Also, many completely healthy persons with presence of all causes of success are non succeeded person. 

Handicaps are to be overcome--we all have them in some fashion; some are more visible than others, but we all have something to overcome even if its pride, arrogance, or learned helplessness (and that's before we get into the obvious ones like blindness, deafness, immobility, etc.). To overcome them is what life is all about, to be the best we can be and do the best we can do. 

Then, a handicapped is the one who can not overcome the cause of his inability. Completely healthy person is also a handicapped if he does not seek to success despite presence of all causes of success (health, intelligence & tolerant healthy environment) except the well. Sometimes a healthy person is resisted or prevented to succeed or to be more succeeded in spite of his well, ambition & durable trials to succeed. Injustice & absence of goodness are handicaps of his environment. So, a nonhandicapped is the person who continues to overcome his inability. 

Hazim makes some good points--I would say the handicapped person is one who will not strive to overcome their limitation, whatever it may be. A non-handicapped person, in my view, is one who is striving to achieve what they truly want to achieve despite the weaknesses or inabilities that otherwise might hamper them. Who would deny that Steve Hawking in not a great writer and thinker in his own right despite the lack of use of his hands; Charles Krauthammer (trained as a physician) is not a good driver though being quadrapalegic; Beethoven, a great composer and conductor, though not being able to hear the orchestra or piano? We have great painters that were visuallly impaired; musicians that were deaf; great physicians that never went to medical school; great orators, playwrights, religious leaders, architects, engineers, inventors, scholars, scientists, musicians, mechanics, business leaders who were too poor for a formal education in their day. I could add being a great mother, father, brother, sister, friend, etc.---The list of greatness is long, though often against the Zeigesit of their times, they achieved only to be recognized in later generations. The search for excellence knows no bounds, no handicap, no obstacle, no roadblock to success as long as the mind can visualize it and one is willing to work and sacrifice for it.    

Thanks Max, you also added a valuable comment. 

I will suggest that perhaps more productive is to speak less of handicaps and more of constraints or limitations on our intrinsic biofunctionality. Here's why:

The Biofunctionality Continuum and Enabling Technologies

Humans exhibit different degrees of biofunctionality, that is, biofunctional ability and expression, along a biofunctionality continuum, from biofunctional constraints as basic as plantar fasciitis or astigmatism, or more complicated impaired articulation disorders, on and through to hearing loss, visual impairment, gait disturbance, as well as the wide-spectrum complex limitations of ASD (autistic spectrum disorder), neurocognitive impairments like cancer-related cognitive impairment (widely known as "chemobrain" but more likely secondary to the malignancy, not to its treatment), neurocognitive disorders like MCI (mild cognitive impairment) and dementive disorders, among others, all representing different degrees and classes of functionally limited physical, sensory, cognitive, intellectual, psychological, and developmental constraints including secondary to various types of function-limiting chronic disease (asthma, diabetes, etc.).

With all of these human biofunctional constraints, there are associated diverse types of proactive therapeutic interventions which further enhance the manipulability and functionality and expressive ability of the human organism. Some of these are essentially corrective technologies, restoring lost or compromised functions back up to societally recognized norms, but others can raise ethical questions as they may be able to at some point in the future go beyond corrective intervention to (supra-)enhancive mode, where the functionality provided may be in excess of the typical borders and range of human functionality and common attainment (blurring the boundary between biology and technology), but all are a form of human functionality-restorative or functionality-enhancive enabling technologies: enabling capabilities/functions and expression that have been compromised and are perceived to be limiting to the human organism, or indeed to the human spirit (depressive disorders, schizophrenias, etc.).

Notice also I speak of enabling technologies, not just assistive technologies: such restorative or enhancive interventions (like cochlear implants), enabling technologies seek to benefit and improve the quality, richness and depth of the human experience (including expression, learning, and social dynamics, both interactions and communication), not merely to compensate for some perceived defect or limitation ("handicap").

As such, they help to expand and enrich our "humanness" in society.

Herb:

Greetings Herb, I do remember you well!

I am impressed with your reach of memory, back through the hazy mists of time to my young (mathematical) linguistics and philosophy of sciience days (although I was undertaking some early medical research even then, only furtively).

Sounds like you are engaged in some intriguing research with quaternions, and first chance I get I will turn to some of the resources you provided me and perhaps talk again. And here on ResearchGate we have some impressive talent in bridging the humantiies, the social, medical and physical sciences, with mathematics and philosphy of science, and I would strongly commend to your attention the contributions of Joachim Pimiskern, an exceptionally fertile and insightful integrative thinker.

Glad to be in touch again.

Constantine

Jérémie:

Thanks for your fine contributions, especially  on the theme of candidacy and selection, where my focus was more narrowly safety and efficay. And your suggestion of the Graeme Clark textbook, from one of the three "Fathers" of cochlear implant technoloogy is absolutely excellent. I would also add the Susan Waltzman & Thomas Roland (editors) text, whose third edition ("Cochlear Implants", Thieme, 2014) is highly valuable with an exceptionally strong and balanced discussion of "Cochlear implantation and deaf education : conflict or collaboration?" delving into the sometimes contentious politics of cochlear implantation especially from the Gallaudet University camp, and also with uncommonly update to date  coverage of emerging and frontier edge developments.

http://www.barnesandnoble.com/w/cochlear-implants-susan-b-waltzman/1119704241?ean=9781604069037

" I´m not sure that not cured deafness really causes IQ deficits."

"The IQ deficits goes back to every study, including John Myklebust of the 1980s, which shows the cognitive performance of deaf children as a group came out lower than normal hearing children, age and otherwise matched."

It is very important to distinguish between non-verbal, visuospatial or performance IQ, which is not impaired in the congenitally deaf, nor is there any reason to suppose general brain function or efficiency is affected.  Verbal IQ, and consequently reading ability and school attainment is another matter.  The brain areas for speech, language and verbal thought depend crucially on patterned auditory input in infancy.  However, I can see no evidence that Max has a low verbal IQ, but am not sure if I would have predicted this given that he had 3 years of (normal?) auditory input before he got mumps.

Dear Anthony, thanks for your hints. I agree with you explanation, the time for falling in deafness will play a major part for developping verbal IQ.

Yes, the growth of the Corpus Collosum, Frontal Lobe, etc. can further developed by exposure to the arts, music, literature, dance, sports. All of these, in our findings, contribute not only to improved verbal and cognitive function in the deaf (and normal hearing children, for that matter) but also in terms of actual physical changes to the brain and body. Hence, I attribute my early and ongoing cognitive development (and many of my impaired students over the years) to these very activities. I was fortunate to have a mother who was superior in those areas because of her own upbringing and were likewise instilled in me from the earliest age. The elephant in the boardroom of US Education today is the near total absense of these in the core curriculum, particularly in terms of music and fine arts education--nations that provide these at early ages are perrenially at the top of the world math and science surveys. Countries like the US, which ignore this paradigm are perennially at the bottom and will stay there until they go back to what we were doing during the 1950s and 1960s in that regard. The same affects the deaf, as well. My early musical training, despite deafness, made all the difference between me and the other deaf kids I knew. I did not realize why that diffference until I was in my fourth year of college and began to study brain development and the relationship of tasks that develop the mind and body. From there it has been a lifelong quest to bring these concepts to the fore and inspire others to do likewise. I still say the deaf and non-deaf alike need these in their training to ever rise out of mediocrity and subpar cognitive performance. I am attaching one of my lecture slide sets below (this one for parents and educators) that illustrates this a bit more.

Dear All: I recall that Anthony Gordon is an expert in the field of cognitive development and very much value his thoughts on these topics. Empowerment of the deaf and hearing impaired is one of my most ardent areas of work and his work contributes mightily to sorting out the constructs and theories involved. 

" Beethoven, a great composer and conductor, though not being able to hear the orchestra or piano"

Beethoven, and many other of his contempory composers, very likely had otosyphilis which was actually an advantage to them as it irritates the ear and produces musical hallucinations, which served as a basis for their compositions.  I don't think it is necessary for an adult composer to be able to hear their works.  As I recall, Beethoven was not a good conductor, which surely does depend on auditory feedback.

Beethoven showed first symptoms for deafness with 28 years. At this time he was already a piano virtuoso, so I think he already had developed an imagination of his music. He "heard" all he wrote.

"Beethoven showed first symptoms for deafness with 28 years"

As I recall, his first symptom was audiosensitivity, which was why he gave up piano playing as the auditory feedback was too loud and/or distorted.

Yes, Anthony is correct. Beethoven, from what we know today, had primarily a conductive loss called otosclerosis and likely some cochlear overlay. For by placing a wooden peg from the floor to his mastoid (behind the ear) when he played the piano, he could hear the sounds he played. But probably as troubling, if not more so, was reports of diplacusis--hearing two different sounds from cochlea to cochlea, creating a neural distortion that was most disconcerting to a man who otherwise had perfect (memory of) pitch.   

I think there are pros and cons to implantation. Parents of small children need to be invested and realize the implant will not "cure" deafness and that they will have to work with their child to develop their listening and speaking skills.

Speaking as a music researcher and a music therapist, who works with children with hearing loss and speech-language delays, even children with complex developmental issues can be implanted, provided they are appropriate for surgery. 

Overall, I believe the cons to be those associated with surgery and the healing process and the ability to accurately perceive music (a big area of research currently). For children who are prelingually deaf, however, the music they hear is "music" to them and they do not any differently.

The pros: ability to learn to hear, to speak, and participate in the hearing world are great. I do not discount those who choose to let their children become part of the Deaf community. It is a rich culture.

Personally, I would implant my children. 

Dear Virginia, thank you for your comment I appreciate really.

As father of a now 35 years of age "smart boy"  with hitherto unknown cause of being nearly deaf at the age of 1.5-2 years (now - with his wife also severely impaired with hearing loss - he is father of two healthy "hearing" sons!) I second 100% your second sentence:

Thanks for your joining in here! I would love to read - if you have time - at least as a short statement - how you work with the children (and of which ages they are) and what - in your opinion - the outcome in "hearing music" for these children will be. Needless to say that I know about the rich culture of the despite they often are told to be "out" of the  /hearing community though. Best regards, Wolfgang

Wolfgang, 

We provide music therapy in a group setting for children 18 mo to 6 years with hearing loss and speech language delays. It is provided in a collaborative setting with Speech-Language Pathologists and Audiologists. We also target the same goal areas, but my team uses music and music therapy techniques to address their delays in reception and production of speech and sounds. If you are interested in more, my supervisor and I published a clinical paper for music therapists regarding our work and ways in which we can incorporate our practices.  Feel free to contact me for more information.

Best regards,

Virginia

Article Music Therapy for Preschool Cochlear Implant Recipients

I really appreciate the kind you interchange your ideas in this thread.

Dear Virginia,

for my interest in gaining more knowledge about "modern" approaches (as compared to 1979/1981 in Salzburg Austria) I honestly requested the full text of your offered paper which I shall gladly incorporate in my e-library, if you allow...Since I am going to prepare a lecture/presentation in my field (diagn. EM) to be held next week in London I shall come back later on to you regarding "contact for more information". My e-mail-address at work is: w.muss(at)salk.at. Thank you so much! Wolfgang

Dear Wolfgang,

I have sent you the article via email. If you have issues accessing please let me know. For anyone else interested in the publication, you can find the full text at the attached link for free. 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3726054/

Dear Virginia,

thank you so much. I received all (the .pdf) safely and properly. First glance: really exciting and interesting, will go further tonight with it, regards, Wolfgang

"Anthony is correct. Beethoven, from what we know today, had primarily a conductive loss called otosclerosis and likely some cochlear overlay"

I am not convinced that he had any conductive component.   All his symptoms are consistent with endolymphatic hydrops secondary to syphilis (audiosensitivity, tinnitus, auditory distortion, fluctuating hearing, vertigo).   I don't think Rokitansky would have missed stapedial fixation at autopsy.

" For by placing a wooden peg from the floor to his mastoid (behind the ear) when he played the piano, he could hear the sounds he played."

It is often said that he used a stick resting on the piano, but I have had trouble authenticating this.  Does anyone have a primary source for his use of such a stick (or by anyone else for that matter)?

I believe Beethoven did both, Anthony. My first bachelors degree was in Music Composition, and my first doctorate is in Communicative Disorders, where I have published a number of textbooks and hundreds of articles. Our music history textbooks during the 1960s referenced both the peg to floor and peg to piano compensating mechanisms used by Beethoven (in one account, he cut off the legs of his piano and played the keyboard a few inches above the floor so the soundboard of the piano would resonate through the wooden floor better). In either case, that is conclusive evidence that his was not a sensorineural hearing loss, but primarily conductive--matched with history's recording of chronic ear infections during adulthood--of the otosclerotic type. Fixation or destruction of the ossicles (both types are part of the otosclerotic diagnosis)would be very difficult to determine at autopsy in that day and age. Besides,  sensorineural loss cannot be ameliorated by a mere peg to anything--that only occurs in conductive impairment. There would have to be substantial cochlear sensitivity for anything to be heard with a peg to the mastoid or temporal bone. 

Virginia raises the arguments we often hear surrounding the cochlear implant issue. I thank her for bringing these into the discussion. It is true that I am still profoundly and utterly deaf even though I wear my implant. The distinction would not really matter, however, if there were not billions of dollars of aid at stake in supporting the deaf population. And it is fine, in my book, if a child thinks they are more normal with the implant--their contribution to society is manifold greater than if they intentionally stayed a burden to society. Without my implant I cannot speak or see patients or lecture the world over or be able to employ hundreds of others in the enterprises I've developed. I perform virtuoso concerts on the clarinet and bass clarinet and the saxophones and lead some of the first choirs as an inspiration to others who wish to break out of the contraints of their developmental and learning disorders. None of this would happen without this technology. It would be a serious mistake for me to have succumbed to the many normal hearing deaf educators and interpreters who tried to dissuade me from having the cohclear implant. The number of those in the US dependent on government programs went from 885,000 deaf individuals to 325,000 by 1995 because of promotion and development of hearing aid and multichannel cochlear implant technologies. A common misconception is that the average deaf person is a cochlear implant candidate. In truth, only about 10% of the deaf meet the strictest criteria. The other 90% are better off with hearing aids, assistive devices, and coping strategies. When we tested an entire deaf community in North Texas in the 1980s we were surprised to find that the average PTA was 75dB in that population, and that my own thresholds were worse than 99% of the others. To purposely limit the deaf by making them feel guilty for wanting to join the hearing world is one of the worse things that can be done, It perpetuates the myth that the deaf have to depend on government services cradle to grave and that they rarely can be net contributors to society.      

Agreed, Max. And with the changing criteria (for high frequency hearing loss and low-frequency residual hearing), many more people who would not receive and implant now can. Our clinic is one where the "Hybrid" devices were pioneered, but that is another discussion for another thread, perhaps.

Yes, the criteria has changed dramatically from the days I received mine. I find sometimes that there has been a rush to the implant (in margina cases) before truly utilizing the hearing aid/assistive device/oral-aural route in the young patient. I have been called into assisting with a case of two young sisters that have not been able to utilize their cochlear implants because the residual hearing of the implanted ears gave them both a electrophonic effect, as Graham Clark referred to it. In both cases they utlize up to date power BTEs and other devices and are able to speak fairly well under this configuration. The electophonic phenomena, not well understood by many clinicias who are tasked with mapping these cases, can present both an acoustic artifact and the electrostimulus simultanously, creating a most disturbing sensation. Both of these sisters has hearing thresholds at about 75dB PTA in a fairly flat configuration to 6KHz. Caution needs to be exercised in rushing to implantation of such cases. Perhaps later in life they would become more appropriate candidates. But their experience in a deaf day school, where everyone is required to turn off implant, take off hearing aids, etc at the door of the school introduces a very troubling paradigm for this population. The antipathy toward becoming hearing is a scary and most egregious problem today. I would expect that from th dark ages; not from an enlightened people who want the best for the rising generation.  

Virginia, just reviewed some of your work--excellent and so needed. As a deaf musician who still performs and encourages young people to overcome their learning disabilities by development of musical skills, I can say that your work is exactly what we need today.

up to 6KHz? Wow! That is astounding! I would hope their electrodes at 6KHz and below have been deactivated to allow for an electro-acoustic stimulation rather than an electrophonic experience. 

We have a few children in a study with a hybrid device, but their residual hearing is monitored and the implant mapped appropriately to avoid significant overlap. There are several concerns regarding the two you mention: the stimulation and residual hearing and requirement to turn off the implant while in school both send up "red flags" to me. 

Yes, we have noted a number of schools that think they are doing the kids a favor by making them go "deaf" when they enter the school as a way to force proficiency in signing. This has caused a number of youth recipients of CIs to feel as if they've betrayed the Deaf (cultural) by trying to function in the larger hearing world. Looking forward to the day vested interests and the Tall Poppy Syndrome no longer dominate the question as to whether to hear or not to hear (to paraphrase the Bard)---but instead, the quest is for every child to reach for their highest attainment and potential utilizing the near miracles modern technology and rehabilitation have to offer. 

Max, your experience with cochlea implant is absolutely fantastic! As a sufferer of tinnitus in the last 20 years or so, I have noticed how much less I have been enjoying music since then. Your enthusiasm and optimistic view on life despite your impairment is of great help. Thanks!

The importance of these valuable technologies lies in the differences they make towards enhancing an individuals quality of life and enabling them to lead more satisfying and meaningful existence. I have worked with people with a wide range of disabilities and I have seen the differences technology such as Cochlea implants have made. For those who are unable to benefit this technology I have seen lives enhanced through communication devices, communication boards, and other mechanical aids. The importance of communicating in some way with the world around us cannot be over-emphasized.

I think this passage sums up nicely your point Max. Once you appreciate one of your blessings, one of your senses, your sense of hearing, then you begin to respect the sense of seeing and touching and tasting, you learn to respect all the senses. (Maya Angelou - African-American author, poet, dancer, actress and singer).

Truly, the cochlear implant is a blessing to those of us who need them, along with the ADA and a plethora of technoogies like TDD Relay, Closed Captioning, Alerting Devices, rehabiliative methodologies, etc. that supplement cochlear implant technology. The commitment that the companies who make these possible is far beyond the normal commitment of other consumer goods, for they are committing to support the hardware, software, and research necessary to follow recipients for decades down the road. And also the public carries some weight in the accolades, as well, for they finance the TDD Relay systems, closed captioning, and a plethora of services we would not have otherwise. I am ever grateful to anyone and everyone that makes it possible for us to enjoy these empowerments for the hearing impaired.    

The importance of these valuable technologies lies in the differences they make towards enhancing an individuals quality of life and enabling them to lead more satisfying and meaningful existence. I have worked with people with a wide range of disabilities and I have seen the differences technology such as Cochlea implants have made. For those who are unable to benefit this technology I have seen lives enhanced through communication devices, communication boards, and other mechanical aids. The importance of communicating in some way with the world around us cannot be over-emphasized.

I think this passage sums up nicely your point Max. Once you appreciate one of your blessings, one of your senses, your sense of hearing, then you begin to respect the sense of seeing and touching and tasting, you learn to respect all the senses. (Maya Angelou - African-American author, poet, dancer, actress and singer).

"As a sufferer of tinnitus in the last 20 years or so, I have noticed how much less I have been enjoying music since then."

I am a bit puzzled by this comment, as music would tend to mask tinnitus.  I think it is more likely due to cochlear distortion, as is common with endolymphatic hydrops or other Menieriform symptoms.  See, eg, Charles Darwin.

Yes, Anthony, tinnitus can be most disturbing. Usually tied to degenerating hair cells of the cochlea (particularly those atuned to the human heart sinus node tone @4KHz or multiples of it in the early stages of high frequency loss).  Consequently, the worse the loss of hearing thresholds the worse the tinnitus. For those of us with profound deafness we generally have the loudest of all, mine loud enough to actually hurt my ears when I have the implant off--with it on the tinnitus perception drops by a factor of about three. Hence, correction of  a hearing loss is usually the first major step in ameliorating the phantom-limb efffects of tinnitus, the rest variously involving abstaning from the offending medications (hypertension meds are strongly implicated in tinnitus) and resolving other issues that contribute mightily to the milieu. I am attaching one of my recent Seminar Handouts on the topic in case it is helpful to you or another reader here.

Anthony is also correct on the masking effect of presented by music over tinnitus--that is usually the case, but in many cases of severe tinnitus (what I and many cochlear implant users experience) there is the addition of cochlear distortion (diplacusis, recruitment, pitch distortions, etc.), which presents both the abnormal growth o f loudness perception and recruiting of neighboring hair cells which causes problems of comfort and pitch differentiation. As a lifelong musician I can attest that the destruction of hair cells and ganglion cells we experience from repeated episodes of Meniere's presents music in its most degraded electroacoustic form. We know (intuitively) that the dissonance of added 11ths, 13ths, and even 15ths are not really in the score, but that is what we hear and often about a quarter or a third lower in pitch than reality. I believe that is what  the commenter above is referring to about not enjoying music because of his tinnitus--rather the same mechanisms that bring his tinnitus also bring this disturbing perception of music. These are valuable and enlightening comments. Thank you. 

" I believe that is what the commenter above is referring to about not enjoying music because of his tinnitus--rather the same mechanisms that bring his tinnitus also bring this disturbing perception of music"

Thanks for clarifying the point I was trying to make as well (but lacking your detailed technical background!).

Patients often say they cannot hear or make out speech because of their tinnitus, when it is the underlying cochlear damage or dysfunction that is really responsible.

"hypertension meds are strongly implicated in tinnitus"

I don't recall seeing this observation in the tinnitus literature.  However, when reviewing the literature on musical hallucinations, I noticed that anti-hypertensive drugs were strongly implicated.   I think there is a simple explanation for this -- undershoot of the blood pressure, leading to hypotensive epidodes, perilymphatic hypotension and hence endolymphatic hydrops and inner ear hypersensitivity, with symptoms as described in Max's last posting.

Yes, Anthony, the harmful undershoot of blood pressure, because some of these meds are more prone to affect only the macrovascular component of hypertension, leaving the collapsed or blocked (due to artherosclerosis, inflammation, or both) microvascular component--which is more involved with auditory function and tinnitus, ie., stria vascularis--unremedied. We see this is in studies on dementia in older adults, also, so the effect of (over)medication to achieve a desired BP target can render unintended consequences that are quite pervasive. Now, before our other participants begin to think this is all off topic, we need to point out that these are among the factors considered in  determining causal factors of acquired deafness, whose population constitute the larger portion of adult cochlear implant recipients. In other words, the causal factors of adult hearing loss can be from a number of egregious causes, including toxicities to medication (aminogylcosides, anti-inflammatories, opioids, diuretics, etc.), certain acquired diseases during childhood and adulthood, heavy metal accumulations and exposure, and acoustic assaults, to name but a few. Thanks for adding richness to our discussion here, Anthony.     

Tinnitus and Antihypertensive Agents

There has always been some considerable confusion here, in part stemming from two competitive narratives re tinnitus pathology and etiology:

On the one hand, many practitioners appear to believe that diuretics may be of benefit in many vestibular disorders (and in widespread clinical use in the treatment of Meniere's) given that inner ear fluid accumulation in the form of secondary endolymphatic hydrops (SEH) within the cochlear or vestibular labyrinth has been confirmed to have a significant incidence of occurrence in patients with subjective idiopathic tinnitus (SIT) [1], with fluid dynamics and homeostasis playing a particularly large role in - but not restricted to - cochleovestibular-type tinnitus. However, these claims lack any robust supporting evidence and numerous reviews of RCTs, including a comprehensive Cochrane Review [2], have failed to uncover any evidentiary basis of clinical significant benefit in either Meniere's or tinnitus.

On the other hand, antihypertensives agents are demonstrably ototoxic and implicated in tinnitus reactive to drug-induced ototoxicity [1;4-11], aka 'drug-induced tinnitus [9], evidenced and further confirmed in the recent ERMPS (European Review for Medical and Pharmacological Sciences) comprehensive review [11], where tinnitus is induced in part secondary to Na-K-2Cl cotransporter (NKCC1) molecular pathway encoded by Slc12a2 in the inner ear [8], with agents like the loop diuretic furosemide (Lasix) being notorious in the induction of subjective and high-frequency tinnitus [3,12] (despite some low Methodological Quality Score (MQS) studies claiming benefit). Indeed furosemide ototoxicity presenting as tinnitus, vertigo, or hearing loss, although frequently reversible as is oft noted, may in fact result in permanent hearing loss in some non-trivial number of cases [12], and with now dozens and dozens of cases accumulated in FDA MedWatch, and with the University of Bologna prospective study finding tinnitus in 17.6% of patients receiving antihypertensive therapy, with use of diuretics and with low SBP (systolic blood pressure (

Thank you, Constantine, for a comprehensive and high quality answer to the topics under discussion. It has long been my opinion that we have many more examples of ill effects of medication on exacerbating or even causing tinnitus than examples of long term benefit. My own tinnitus has been diagnosed as resulting from aminoglycoside antibiotics (particularly streptomycin) in the early 1960s used to control acute bouts of rheumatic fever--causing notable threshold shifts in hearing acuity and simultaneously increasing levels of tinnitus some 6-12 months following administration of the antibiotics. My case was not a rare one, as we later found in the 1970s in a number of studies that identified this a more common (though delayed) side effect of aminoglycosides than previously realized. We hear a lot of talk about aspirin and NSAIs induced tinnitus, but these cases are almost always transient or temporary. Early on in my clinical work in tinnitus finding a solution for tinnitus (mostly the kind related to sensorineural hearing impairment) we emphasized the need for a comprehensive approach that involved addressing underlying causes of (chronic) disease--hydration, nutrition deficiencies, toxicities, stress, sedentary lifestyle, etc. coupled with wide band hearing correction was the most effective treatment for tinnitus. By so doing, the patient became healthy, eliminating the need for medications, their auditory deficit was addressed, and mental scores were improved commisurate with their degree of loss and lapse of time involved.

Any of the therapies you mentioned (TRT, etc.) we found to be short-lived if they ignored the more basic approach mentioned above.

Thank you again for your excellent information.  

"Thanks for adding richness to our discussion here, Anthony."    

Thanks for your kind words, which make me feel less guilty about my tendency to ramble off the point on RG questions.  In general, people should stick closely to the question, which makes it easier for people looking for information by searching the RG questions.  However, my justification for digression is that RG comments are picked up by Google, irrespective of where about on RG they occur.  Hence anyone wanting info on Tinnitus and Antihypertensive drugs is surely going to be rapidly directed by Google to Constantine's mini-review.  

These are all related to the Cochlear Implant experience, actually. Most older adult cochlear implant recipients are ototoxicity cases from disease and medication. In fact, few of them do not fall into this category. Of the younger recipients (and long standing ones like me), we just suffered the diseases and ototoxicity earlier than the older ones. A small handful of the younger ones are actual congenital cases. Meniere's, tinnitus, vertigo, auditory deprivation (atrophy), cochlear distortions, acoustic trauma, cochlear stroke--these are just landmarks on the pathway to deafness.  WIthout these assaults on hearing health, there would be little need for cochlear implantation. The general public and health policty makers and the medical community at large have wallowed in ignorance over these increasingly common constructs long enough. 

Anthony:

If your consistently insightful even penetrating observations - challenging us all to think both a lot more deeply, and a lot more critically to avoid facile and lazy assumptions about the themes of this topic - constitute ramblings, then we all need far more ramblings!

I am still just going back and finding and re-reading and re-reviewing your many rich contributions (only just re-read your right-on-target call to distinguish non-verbal, visuospatial or performance IQ and how brain areas for speech, language and verbal thought are crucially dependent on patterned auditory input in infancy, just two sentences in one of your postings that brought some of the discussion back closer to reality).

And the Beethoven observations are inestimable - and I might add, having been stimulated to read Hui and Wong's "Medical History of Beethoven" (HKMJ 2000;6:433-8; at: http://www.hkmj.org/article_pdfs/hkm0012p433.pdf), your prespectives uncannily on target, which in turn caused me to then discover your brilliant gem of a paper/letter on audiosensitivity in composers in the Journal of Neuropsychiatry and Clinical Neurosciences (2000;12:117-a-120., at: http://neuro.psychiatryonline.org/article.aspx?articleid=100701), must reading that I am still digesting.

So Anthony, you just, please, keep on "rambling" and we'll all just keep on benefiting (and be a lot less lazy in our thinking).

Kind regards

Constantine

"Most older adult cochlear implant recipients are ototoxicity cases from disease and medication."

" policty makers and the medical community at large have wallowed in ignorance over these increasingly common constructs long enough"

I think in the past I was one of these wallowers, but now I think the influence of drugs and toxins has been missed.  There is an interesting analogy with migraine, which is highly comorbid with Meniere's disease.  Patients for a long time have consistently blamed foods or drugs for triggering their headaches, but this has been dismissed because of negative provocation tests.  However, this has overlooked the point that a hypersensitive cochlea is a prerequisite for the drug to have its ototoxic effect, whether deafness, tinnitus, musical hallucinations or the vestibular components of a migraine.

"At this juncture we are left with the disconcerting scenario that a vast amount of agents (NSAIDs, antibiotics, salicylates, platinum-based chemotherapy, radiotherapy, antihypertensives, etc.) are tinnitus-inductive"

All the more reason for considering a common, general, non-specific underlying mechanism, vascular hypotension leading to alteration in cochlear pressure for example.   There can hardly be any pharmacological mechanism common to so many varying drugs.

"So Anthony, you just, please, keep on "rambling" and we'll all just keep on benefiting (and be a lot less lazy in our thinking)."

Thank you for your comments.  It is a pleasure to be on a troll-free academic blogging site like RG.   I have just been reported to the moderator on a more popular site for pointing out that controversial books need to be read before being criticised!

Anthony, while I worked for Cochlear Americas during the 1990s and conducted and participated in a good deal of their research during that time, I recall that a good 80% of the older CI recipients were acquired cases of deafness as a result of medication ototoxicity, which we dubbed "burned out Meniere's" cases. We also learned that otoscolerosis takes place in the cochlea and its incidence was quite high in older adults with severe and profound losses. So many previous perceptions on how people lose their hearing and the case history mileposts along the way, have been smashed because of what we learned from early recipients of CIs.

Admittedly, though, some of the data may have changed since the criteria for candidacy has changed so much, bringing many less profound and severe losses into the CI population. Perhaps someone closer to the more recent data can enlighten us on this. But I suspect the newer data has only confirmed what we were learning in the early days, and that the need for avoidance of toxicity and other other assaults on the hearing mechanisms has only increased as more and more people need the cochlear implant.  

The Global Problem of Ototoxicity

Although ototoxicity can result from occupational and/or environmental exposure to ototoxins, by far and away the majority of cases (63%+) result from drug therapy1. Here I use the term ototoxic to mean any agent or agency with the potential to cause toxic reactions to structures of the inner ear, including the cochlea, vestibule, semicircular canals, and otoliths, while drug-induced damage affecting specifically to the auditory systems constitutes cochleotoxicity, and to the vestibular systems constitutes vestibulotoxicity.

Many factors and trends are causing increased levels of induced ototoxicity. For one, we have noise potentiation of cochleotoxicity: noise-induced hearing loss is estimated to affect approximately 27 million (12.8%) of adults aged 20–69 years2 (with statistics showing that about 13-14% of the total population of the USA - some 39-42 million people as of 2005 - either currently experience noise-induced hearing loss or are at a high risk of developing it (whether occupational or not)15, and synergism can also occur between ototoxic agents, such as salicylates, aminoglycosides, and platinums, and noise which increases free radical formation and damage3-5.

In addition, use of analgesic is know to have climbed, and continues climbing rapidly, especially long-term use (2+ years) occasioning more ototoxicity; in the seminal Bangladesh study6, under moderate consumption (at least 3-4 times per week), severe damaged was observed in 52.77%, moderate damage in 30.55% cases, and mild damage in 16.66% cases.

At the same time, near epidemic use of acetaminophen/paracetamol has been observed - in the US, ~6% of adults are annually prescribed acetaminophen doses of more than 4 g/day, with 30,000 hospitalized for acetaminophen toxicity, and with liver injury in 17% of adults7 - with ototoxicity secondary to the depletion of glutathione8, which is known to be otoprotective of the cochlea from noise-induced damage9,10. Data from the large (n=62,261) Nurses' Health Study II11 and from the large (n=51,529) Health Professionals Follow-up Study12 further confirms the high ototoxicity of acetaminophen.

And higher consumption of alcohol has been confirmed prospectively in the Health Professionals Follow-up Study to be associated with an increased risk of hearing loss among those with lower intake of vitamin B12 (below the median (9.0 mcg/d)13.

The Sorry State of Prevention

At the same time as these alarming trends, insufficient resources have been conducted on the promising class of antioxidant otoprotective agents14 capable of preventing ototoxicity by neutralizing ROS generated especially by noise exposures and certain ototoxic agents, such as N-acetyl-L-cystine (L-NAC), acetyl-L-carnitine (ALCAR), magnesium, the glutathione peroxidase mimic Ebselen, and the pre-noise-exposure otoprotectives Auraquell and D-methionine (D-met), and melatonin or rosmarinic acid (the later two especially for platinum-induced ototoxicity), among several others in clinical trial.

Going Forward

As in oncology, we need to switch the paradigm of research from treatment interventions stages after hearing damage, to otoprotective preventive modalities to avoid the hearing damage in the first place, and currrently there is far too much therapeutic research and far too little preventive investigations, and we cannot morally wait for new vast generations of hearing-impaired populations before we more optimally realign our resources.

References

Outstanding analysis, Constantine. This has been on of my most ardent projects for many years--thank you for bringing us to date and opening the discussion on ototoxicity, which is as noted above is the number one reason for the need of cochlear implantation in older adults. The medical field's neglect of this is much like an overflowing bathtub--dipping out the water with a small ladle while both faucets are left full-on. 

" I recall that a good 80% of the older CI recipients were acquired cases of deafness as a result of medication ototoxicity, which we dubbed "burned out Meniere's" cases. We also learned that otoscolerosis takes place in the cochlea" 

Here's an idea for you to chew on, Max, concerning the frequent comorbidity of Meniere's disease and otosclerosis.  Otosclerosis often starts out with audiosensitivity and other inner ear symptoms (as noted by Itard).  Stapedial reflexes show an initial reversal (decreased compliance). I could never quite convince myself that this was sufficient to explain the increase in loudness, so now I think that this was a mechanical effect of mild endolymphatic hydrops, which in other conditions is due to decreased perilymph pressure.  This means that less perilymph seeps out of the cochlea into the middle ear, leading to gumming up of these routes, especially the fissula ante fenestram, triggering otosclerosis. 

"Many factors and trends are causing increased levels of induced ototoxicity. For one, we have noise potentiation of cochleotoxicity"

Thanks for this review.  I had not previously realised the magnitude of this problem.  When reviewing the literature on drug-induced musical hallucinations, I found that a hypersensitive inner ear was a necessary and probably sufficient cause, due to endolymphatic hydrops.  Loud noise does not often cause Meniere's disease, but there is consistent evidence that it does cause hydrops, probably due to increased perilymph leakage into the middle ear.  Likewise, head injuries, hence the explanation for intolerance to alcohol after mild head injuries insufficient to harm the brain.

Anthony, you've sparked all kinds of thoughts on this. Damage on cervical 1-2, which control so much of the stria vascularis can create the hydrops effect (vascularization, over production of endolymph and lack of same), and more and more we are seeing the dastardly unreported effects from the new neuroleptic and nerve deadening drugs that are invading the pain management field in the US having unexpected effects on the vestibular (and cochlear) system. It's as if medicine has gone off its rocker, treating symptoms and damn the consequences of ignoring underlying factors. Sudden deafness, Meniere's and Meniere's-like, tinnitus, and vestibular issues abound in the aftermath of the latest trends in overprescribing. Add extraordinarily high caffeine intake on top of the milieu and we are seeing more avoidable cases of cochleovestibular issues in our population. I don't know if you are seeing that in England, but we see the problem growing over here in the US.