Arbaclofen Given a Second Chance by the Simons Foundation

 Light at the end of the tunnel, for some

I did recently write about autism drugs that target the GABA_B receptor.

Western doctors have Baclofen and a few did have experimental use of the more potent version called Arbaclofen, or R-Baclofen.  We saw that Russian doctors have a wider choice.

The rights to use Arbaclofen have been acquired by the Simons Foundation, and they intend to restart autism trials in humans.

Mouse studies support second chance for fragile X drug

  

Arbaclofen was found to be effective in some people with Fragile-X and autism, but it failed its clinical trial and the developer, Seaside Therapeutics, went out of business.

The Simons Foundation, for those who do not know, is probably the best thing to ever happen to people with autism.  The founder of the foundation is an American multi-billionaire, former fund manager and mathematician.  He has a daughter with autism and decided to do something about it.

Having already funded a great deal of research, including by some of the scientists on my Dean’s List, it looks like he is going one step further and taking ownership over the trial drugs themselves.  Being a mathematician he is not averse to funding the most complex areas of research which include genetics and ion channels.  Being a fund manager he understands risk.  Being rich also helps, but you also need to be philanthropic.

Given the poor performance to date of developing practical therapies from the vast wealth of existing autism research, this is a very encouraging development.

There is now a large industry being made out of autism research, but the only coordinated part of it seems to be the Simons Foundation.  Interestingly the Simons Foundation focuses its effort on the very best scientists and not the existing autism researchers.  Apparently they want Nobel Laureates and future Nobel Laureates.  That sounds good to me.

Some people are concerned that by focusing on specific areas like genetics, the Simons Foundation may miss other possibly fruitful avenues.  But it is usually the case that an intelligent person's well thought out strategy is better than no strategy, and, at the end of the day, Simons’ billions are his to spend as he pleases.

Hopefully Simons will do for autism, what Bill and Melinda Gates are doing for polio and malaria.

Minimizing Summertime Autism Flare-ups in 2015

When I first connected histamine to autism, I did not realize that this might be a common problem.  The most frequently viewed post on this blog is one on histamine and autism; so at least 10,000 people out there have googled “autism and histamine”.

Two years later, the therapy is still evolving and it should be said that, what works best for one person may not help in another person.  The main point is that in some people with autism, they face a summertime regression due to the effect of allergy.  So bad behaviours and aggression increase and good behaviours and indeed cognitive function decrease.  This appears to be the result of histamine and a pro-inflammatory cytokine called IL-6.

For the 2015 pollen season, which started early where we live, this is what we are using:-

Azelastine nasal spray, this is an H1 antihistamine that is also inhibits mast cells from “degranulating” and emptying their load of pro-inflammatory substances.  Once a day.

Quercetin is a cheap flavonoid that has numerous actions including on histamine H1 receptors, mast cells, and inflammation. 125mg two or three times a day.

Verapamil is an L-type calcium channel blocker and also a mast cell stabilizer. 40mg three times a day

Fluticasone propionate 50 µg (micrograms) – see below.  It is a steroid that has recently been shown to have some unexpected effects on mast cells.  

I have found that oral antihistamines were effective for only a couple of hours, but their effect varies widely from person to person.

In theory, Rupatadine should be the most effective anti-histamine, since it is also a potent mast cell stabilizer.  The old first generation antihistamines (that make you drowsy) could in theory be better than the new ones like Claritin, Zyrtec, since they can also cross the blood brain barrier (BBB).

Ketotifen and cromolyn sodium should also be useful, but if the allergy is pollen related, you really need the nasal spray (nasalcrom etc) to get the most effect.  In some countries they sell eye drops and not the nasal spray.  Usually the eye drops are more diluted than the nasal spray.  For example, the Azelastine eye drops contain 50% less Azelastine than the nasal spray, but are otherwise the same.  Where we live they have run out of the nasal spray but not the eye drops, so you could refill the spray with eye drops and double the number of sprays to get the same dose.

Drugs like Claritin and Zyrtec are H1 antihistamines and also partial mast cell stabilizers; they have a positive behavioral effect in some people with ASD, who are apparently allergy free.

New for 2015

I expect that two recent anti-inflammatory therapies, the Tangeretin flavonoid and the Miyairi 588 bacteria/probiotic may have a beneficial, indirect, effect on our usual summertime regression.

A more convention approach is to add fluticasone propionate to reduce the inflammation caused by allergy.  This drug is a steroid and widely used either as an inhaler to control asthma and COPD, or as a nasal spray to treat allergies.

As Flixotide inhaler, Monty, aged 11 with ASD and asthma, has already been taking fluticasone propionate for a few years.  We now use a tiny dose (50 µg), since his autism therapies have greatly reduced any asthma tendencies.

Fluticasone propionate nasal spray (Flixonase, Flonase etc) is widely sold as a treatment for hay fever and rhinitis and was recently combined with Azelastine (see above) as a treatment for moderate to severe allergies in a product call Dymista.

The combination of H1 antihistamine, mast cell stabilizer and anti-inflammatory all in one spray does seem a good idea.  The steroid dose using Dymista is actually lower than the usual dose of steroid when using Fluticasone propionate nasal spray alone.  You want to minimize the amount of steroid absorbed in the blood. When used as a spray/inhaler the amount is tiny, but still should be considered.

Dymista (Azelastine + Fluticasone propionate) does indeed work better than Azelastine alone.  There is no sign of allergy at all (no red eyes, sneezing, itchy nose), with Azelastine you still have an itchy nose.

In our case, the allergy symptoms, even minors ones, do correlate with the change in behaviour and cognitive function; so the target is no allergy symptoms at all.

If anyone has other therapies for summertime flare ups, feel free to share them.

Tangeretin vs Ibuprofen, as PPARγ agonists for Autism. What about PPARγ for Epilepsy?

Summary of the therapeutic actions of PPARγ in diabetic nephropathy

From:  Peroxisome proliferator-activated receptors and renal diseases

I did write an earlier post about NSAIDs (Nonsteroidal anti-inflammatory drugs) like Ibuprofen, which I expected to have no effect on autism.

  

Cytokines from the Eruption of Permanent Teeth causing Flare-ups in Autism

However, to my surprise, I found that certain types of autism “flare-up” do respond very well to Ibuprofen.  Based on the comments I received, it seems that many other people have the same experience.

NSAIDs work by inhibiting something called COX-2, but they also inhibit COX-1.  The side effects of NSAIDs come from their unwanted effect on COX-1.

NSAIDs are both pain relievers and, in high doses, anti-inflammatory.  Long term use of NSAIDs is not recommended, due to their (COX-1 related) side effects.

Observational Study

All I can say is that in Monty, aged 11 with ASD, and with his last four milk teeth wobbly but refusing to come out, the increase in the cytokine IL-6 that the body uses to signal the roots of the milk teeth to dissolve seems to account for some of his flare-ups.  I do not think it is anything to do with pain.

This is fully treatable with occasional use of Ibuprofen and then “extreme behaviours” are entirely avoided.

Sytrinol (Tangeretin) vs Ibuprofen

Since Ibuprofen, when given long term, has known problems, I looked for something else.

On my list of things to investigate has been “selective PPAR gamma agonists”, which is quite a mouthful.  The full name is even longer.  The nuclear transcription factor peroxisome proliferator activated receptor gamma (PPARy) regulates genes in anti-inflammatory, anti-oxidant and mitochondrial pathways.  All three of these pathways are affected in autism.

We already know that non-selective PPARy agonists, like pioglitazone, developed to treat type 2 diabetes, can be used to treat autism.  The problem is that being “non-selective” they can have nasty side effects, leading to Pioglitazone being withdrawn in some markets.

  

Effect of pioglitazone treatment on behavioral symptoms in autistic children

  

While looking for a “better” PPARγ agonist, I came across the flavonoid Tangeretin, which is commercially available in a formulation called Sytrinol.

An effective PPARγ agonist would have many measurable effects.  The literature is full of natural substances that may, to some degree, be PPARγ agonists, but you might have to consume them by the bucket load to have any effect.

The attraction of Sytrinol is that it does have a measurable effect in realistic doses.  Sytrinol is sold as a product to lower cholesterol.  Tangeretin is a PPARγ agonist and you would expect a PPARγ agonist to improve insulin sensitivity and also reduce cholesterol. There are clinical trials showing this effect of Sytrinol.

Sytrinol (Tangeretin) Experiment

The most measurable effect of using Sytrinol for six weeks is that we no longer need any Ibuprofen.  It is measurable, since I am no longer needing to buy Ibuprofen any more.

About three days a week Monty’s assistant would need to give him Ibuprofen at school.  This all stopped, even though occasional complaints about wobbly teeth continue.

Nobody markets  Sytrinol (Tangeretin) as a painkiller.

Note:- Sytrinol capsules contain a blend of 270mg PMF (polymethoxylated flavones, consisting largely of tangeretin and nobiletin) + 30mg tocotrienols. Nobiletin is closely related to tangeretin, while tocotrienols are members of the vitamin E family.  All three should be good for you.

Tangeretin and Ibuprofen are both PPARγ agonists

The explanation for all this may indeed be that Tangeretin and Ibuprofen are both PPARγ agonists.  Inhibiting COX-2 may have been irrelevant.

Peroxisome Proliferator-activated Receptors α and γ Are Activated by Indomethacin andOther Non-steroidal Anti-inflammatory Drugs

  

It may be that by regulating the anti-inflammatory genes, via  PPARγ, the Sytrinol has countered the “flare-up” caused by the spike in IL-6.

Anyway, in the earlier post we did see that research shows that dissolving milk teeth is signalled via increased IL-6 and we do know that increased IL-6, caused by allergies, can trigger worsening autism. 

So it does make sense, at least to me.

Regular uses of Sytrinol/Tangeretin looks a much safer bet than any NSAID.

If anyone tries it, particularly those who regularly use NSAIDs, let us all know.

PPARγ and Epilepsy

If you Google PPARγ and autism you will soon end up back at this blog.

For any sceptics, better to Google PPARγ and Epilepsy.  Epilepsy looks to be the natural progression of un-treated classic autism.  If this progression can be prevented, that should be big news.

Prevention is always better than a cure.  All kinds of conditions appear to be preventable, or at least you can minimize their incidence.  

Here are just the ones I have stumbled upon while researching autism:- Asthma  (Ketotifen), type 2 diabetes (Verapamil), prostate cancer (Lycopene) and many types of cancer (Sulforaphane).

There are of course types of epilepsy unconnected to autism, but epilepsy, seizures and electrical activity are highly comorbid with classic autism

PPARgamma, Epilepsy and Therapeutics

Abstract

Approximately 30% of people with epilepsy do not achieve adequate seizure control with current anti-seizure drugs (ASDs). This medically refractory population has severe seizure phenotypes and is at greatest risk of sudden unexpected death in epilepsy (SUDEP). Therefore, there is an urgent need for detailed studies identifying new therapeutic targets with potential disease-modifying outcomes. Studies indicate that the refractory epileptic brain is chronically inflamed with persistent mitochondrial dysfunction. Recent evidence supports the hypothesis that both factors can increase the excitability of epileptic networks and exacerbate seizure frequency and severity in a pathological cycle. Thus, effective disease-modifying interventions will most likely interrupt this loop. The nuclear transcription factor peroxisome proliferator activated receptor gamma (PPARy) regulates genes in anti-inflammatory, anti-oxidant and mitochondrial pathways. Preliminary experiments in chronically epileptic mice indicate impressive anti-seizure efficacy. We hypothesize that (i) activation of brain PPARy in epileptic animals will have disease modifying effects that provide long-term benefits, and (ii) determining PPARy mechanisms will reveal additional therapeutic targets. Using a mouse model of developmental epilepsy, we propose to (1) elucidate the cellular, synaptic and network mechanisms by which PPARy activation restores normal excitability;(2) demonstrate the significant contribution of mitochondrial health in pathologic synaptic activity in epileptic brain;(3) demonstrate inflammatory regulation of PPARy in epileptic brain;and (4) determine whether PPARy activation extends the lifespan of severely epileptic animals. The proposed studies, spanning in vivo and in vitro systems using a combination of techniques in molecular biology, electrophysiology, microscopy, bioenergetics and pharmacology, will provide insight into the interplay of seizures, mitochondria, inflammation and homeostatic mechanisms. The results will have tremendous, immediate translational potential because PPARy agonists are currently used for clinical treatment of Type II Diabetes. PPARy is under investigation as treatment for a wide variety of other neurological diseases with cell death and inflammation as common denominators;therefore, the results of this proposal will have a broad impact.

Public Health Relevance

Approximately 30% of people with epilepsy do not achieve adequate seizure control with current anti-seizure drugs (ASDs). This medically refractory population has severe seizure phenotypes and is at greatest risk of sudden unexpected death in epilepsy (SUDEP). Therefore, there is an urgent need for detailed studies identifying new therapeutic targets with potential disease- modifying outcomes.

Anticonvulsant potential of the peroxisome proliferator-activated receptor γ agonist pioglitazone in pentylenetetrazole-induced acute seizures and kindling in mice.

Activation of cerebral peroxisome proliferator-activated receptors gamma exerts neuroprotection by inhibiting oxidative stress following pilocarpine-induced status epilepticus.

Abstract

Status epilepticus (SE) can cause severe neuronal loss and oxidative damage. As peroxisome proliferator-activated receptor gamma (PPARgamma) agonists possess antioxidative activity, we hypothesize that rosiglitazone, a PPARgamma agonist, might protect the central nervous system (CNS) from oxidative damage in epileptic rats. Using a lithium-pilocarpine-induced SE model, we found that rosiglitazone significantly reduced hippocampal neuronal loss 1 week after SE, potently suppressed the production of reactive oxygen species (ROS) and lipid peroxidation. We also found that treatment with rosiglitazone enhanced antioxidative activity of superoxide dismutase (SOD) and glutathione hormone (GSH), together with decreased expression of heme oxygenase-1 (HO-1) in the hippocampus. The above effects of rosiglitazone can be blocked by co-treatment with PPARgamma antagonist T0070907. The current data suggest that rosiglitazone exerts a neuroprotective effect on oxidative stress-mediated neuronal damage followed by SE. Our data also support the idea that PPARgamma agonist might be a potential neuroprotective agent for epilepsy.

Possible involvement of PPAR-gamma receptor and nitric oxide pathway in theanticonvulsant effect of acute pioglitazone on pentylenetetrazole-induced seizuresin mice

CONCLUSION:

The present study demonstrates the anticonvulsant effect of acute pioglitazone on PTZ-induced seizures in mice. This effect was reversed by PPAR-γ antagonist, and both a specific- and a non-specific nitric oxide synthase inhibitors, and augmented by nitric oxide precursor, L-arginine. These results support that the anticonvulsant effect of pioglitazone is mediated through PPAR-γ receptor-mediated pathway and also, at least partly, through the nitric oxide pathway.

Note that elsewhere in this blog I have already highlighted that PPAR alpha agonists also seem to have an effect against epilepsy.  For example in this research:-

PPAR-alpha agonists as novel antiepileptic drugs: preclinical findings.

          

I was originally interested in PPAR-alpha, because of its role in regulating mast cells.  It seems that PPARγ also affects mast cells.

Activation of PPARγ  restores mast cell numbers and reactivity in all oxan-diabetic rats by reducing the systemic glucocorticoid levels

  

PPARγ modulators – drugs vs neutraceuticals vs functional food

It does seem that many people with inflammatory diseases, epilepsy, autism and even people who are obese, might greatly benefit from selective PPARγ agonists.

The choice would be between drugs, “nutraceuticals” and functional (good) food.

The drugs have not yet arrived that are safe and selective.  The current Thiazolidinedione (TZD) class of drugs TZDs tend to increase fat mass as well as improving insulin sensitivity and glucose tolerance in both lab animals and humans.

PPARγ as a metabolic regulator: insights from genomics and Pharmacology

Since its identification in the early 1990s, peroxisome-proliferator-activated receptor γ (PPARγ), a nuclear hormone receptor, has attracted tremendous scientific and clinical interest. The role of PPARγ in macronutrient metabolism has received particular attention, for three main reasons: first, it is the target of the thiazolidinediones (TZDs), a novel class of insulin sensitisers widely used to treat type 2 diabetes; second, it plays a central role in adipogenesis; and third, it appears to be primarily involved in regulating lipid metabolism with predominantly secondary effects on carbohydrate metabolism, a notion in keeping with the currently in vogue ‘lipocentric’ view of diabetes. This review summarises in vitro studies suggesting that PPARγ is a master regulator of adipogenesis, and then considers in vivo findings from use of PPARγ agonists, knockout studies in mice and analysis of human PPARγ mutations/polymorphisms.

As usual there are numerous “natural substances” that may also modulate PPAR-γ

Modulation of PPAR-γ by Nutraceutics as Complementary Treatment for Obesity-Related Disorders and Inflammatory Diseases

A direct correlation between adequate nutrition and health is a universally accepted truth. The Western lifestyle, with a high intake of simple sugars, saturated fat, and physical inactivity, promotes pathologic conditions. The main adverse consequences range from cardiovascular disease, type 2 diabetes, and metabolic syndrome to several cancers. Dietary components influence tissue homeostasis in multiple ways and many different functional foods have been associated with various health benefits when consumed. Natural products are an important and promising source for drug discovery. Many anti-inflammatory natural products activate peroxisome proliferator-activated receptors (PPAR); therefore, compounds that activate or modulate PPAR-gamma (PPAR-γ) may help to fight all of these pathological conditions. Consequently, the discovery and optimization of novel PPAR-γ agonists and modulators that would display reduced side effects is of great interest. In this paper, we present some of the main naturally derived products studied that exert an influence on metabolism through the activation or modulation of PPAR-γ, and we also present PPAR-γ-related diseases that can be complementarily treated with nutraceutics from functional foods.

Conclusion

If you are one of those people successfully using NSAIDs, like Ibuprofen, to reduce autistic behaviors, you might well be in the group that would benefit from Sytrinol/Tangeretin.

If NSAIDs never help resolve your autism flare-ups, Sytrinol/Tangeretin may not help either.

Tangeretin does appear to have other effects, beyond not needing to use Ibuprofen.  It was found to be a potent antagonist at P2Y2 receptors.

Suramin is another potent P2Y2 antagonist and Suramin is showing a lot of promise in Robert Naviaux’s autism studies at the University of California at San Diego.  Suramin is not viewed as safe for regular use in humans.

Sustained Release NAC for Autism and Schizophrenia

“Pharmacokinetics” of a typical drug

Today’s post is about what should be the optimal anti-oxidant therapy for autism, schizophrenia, COPD and any other disease in which oxidative stress is present.  You will have to be able to swallow pills, to fully benefit.

NAC seems to be the most potent, safe, anti-oxidant, the only drawbacks are:-

·        Short half-life

·        Can taste/smell bad

In autism, NAC is normally given three times a day, but often it is not practical to give a drug at precise intervals throughout the day.

This is a common problem with many drugs and has been solved long ago – with the sustained release pill.

If you find that four hours after giving NAC there is an increase in irritability, anxiety or stimming, it may be that oxidative stress has already returned.  It may be that other factors have triggered a higher load of oxidative stress.  The way to be sure is just to give a small extra dose of NAC and wait 15 minutes.  If everything returns to normal, you found the problem.

Since you cannot always be present with an extra half dose of NAC, the answer is the sustained release form of NAC.

Since we have seen that oxidative stress triggers all kinds of secondary dysfunctions, the sustained release form of NAC might also help minimize them, since you could have 24 hour protection.  Oxidative stress does not go away while you sleep.

For example, I recall the Polish researcher at Harvard who suggested that oxidative stress might cause central hypothyroidism in autism (low levels of T3 in the brain).

Your body produces the pro-hormone T4 in the thyroid which then circulates throughout the body.  Special enzymes, produced locally, then convert the T4 into the active hormone called T3.  The researcher found that in the autistic brain this enzyme was reduced by oxidative stress.

Many “alternative” doctors, mainly in the US, do prescribe extra T3 hormone to people with autism and indeed other conditions.  Some older ladies across the world are buying T3 hormone, online from Mexico, since their doctor will not prescribe it.  They say it makes them feel better.

As your endocrinologist will tell you, hormones are controlled by so-called feedback loops.  So if you start adding extra T3 hormone, your thyroid will start producing less T4.  Then you need even more supplemental T3.

I did do a little experiment with a small dose of T3, to see if a short term increase in T3 affects “my” kind of autism.  It most definitely does; as does a short term spike in potassium levels.  These are useful diagnostic tests, rather than therapies.

This would suggest that minimizing oxidative stress 24 hours a day, may not just be possible, but also highly beneficial.

 

OTC Sustained Release NAC  (NAC SR)

There actually is an inexpensive Sustained Release NAC , available OTC (without prescription).

  

  

A directly-compressed, controlled release tablet contains N-acetyl cysteine as the active ingredient -   PATENT

The problem with currently-available granulated and effervescent tablet compositions is that they release N-acetyl cysteine very rapidly. Thus, the effervescent compositions as well as the granulate compositions currently available on the market achieve a maximum blood plasma level within 1 hr from administration. One matrix tablet formulation does show a maximum blood plasma level at 2-2.5 hrs after administration, although its recipe indicates that granulation was required. The problem with granulation of acetyl cysteine is that if any dissolves, the dissolved material starts to decompose into impurities.

In accordance with the present invention, this problem of overly-rapid release is obviated by providing the N-acetyl cysteine in the form of a tablet or other article made with the rheology modifying acrylic or methacrylic acid-based polymers, or analogues, described in commonly-assigned application Ser. No. 09/559,687, filed Apr. 27, 2000. Tablets made in this manner exhibit controlled release characteristics, thereby allowing the N-acetyl cysteine active ingredient to be released over a longer period of time.

The rheology modifying polymers used in the present invention provide controlled release of the N-acetyl cysteine and other biologically active compounds contained in the inventive tablet, if any, so that when placed in water or body fluid, the polymer swells to form a viscous gel which retards diffusion of the active material.

The advanced bilayer Sustain™ tablets combine 1/3 Quick Release and 2/3 Sustained Release formats to both immediately raise and to maintain blood levels over a longer period of time.* NAC Sustain^®  releases in the small intestine over a 8 hour period, compared to the 1.5 hour biological half-life of NAC in the bloodstream.*

NAC in published research

Much currently available data is from very early studies on NAC that indicated that the half-life was about 5 hours, but subsequent studies suggested it is very much shorter, perhaps just 90 minutes.

The following study is quite old, but compares the behaviour of different NAC formulations in 10 volunteers.

Pharmacokinetics of N-Acetylcysteine in Man 

Some definitions:-

Half Life

A biological half-life or elimination half-life is the time it takes for a substance (drug, radioactive nuclide, or other) to lose one-half of its pharmacologic, physiologic, or radiological activity. In a medical context, the half-life may also describe the time that it takes for the concentration in blood plasma of a substance to reach one-half of its steady-state value (the "plasma half-life").

The relationship between the biological and plasma half-lives of a substance can be complex, due to factors including accumulation in tissues, active metabolites, and receptor interactions

Mean Residence Time

For the medical field, residence time often refers to the amount of time that a drug spends in the body. This is dependent on an individual’s body size, the rate at which the Drug will move through and react within the person’s body, and the amount of the Drug administered. The Mean Residence Time (MRT) in Drug deviates from the previous equations as it is based on a statistical derivation. This still runs off a steady-state volume assumption but then uses the area under a distribution curve to find the average drug dose clearance time. The distribution is determined by numerical data derived from either urinary or plasma data collected. Each drug will have a different residence time based on its chemical composition and technique of administration. Some of these drug molecules will remain in the system for a very short time while others may remain for a lifetime. Since individual molecules are hard to trace, groups of molecules are tracked and the distribution of these is plotted to find a mean residence time.

Conclusion

This post may have been more useful for adult readers, with Asperger’s, who are self-treating.  Many people with Schizophrenia also self-treat with NAC, but they probably do not read autism blogs.

For those unable (yet) to swallow, pills you can have the option of breaking the effervescent tablets in half (or even quarters) to try and maintain a more stable level of NAC.  We sometimes do this, half a 600 mg tablet at school at 11 am,  when needed.  It only seems to be really needed in the pollen allergy season, which seems to trigger more oxidative stress as well as histamine and IL-6.  It works.

One reader of this blog is doing something similar with Bumetanide, he/she is giving it in three daily doses.  Bumetanide also has a short half-life, as does Verapamil.  There is no sustained release form of Bumetanide, but there is for Verapamil.

A final point raised is whether the benefit from NAC comes from it being a precursor to Glutathione (GSH), the body' own master antioxidant, or whether it is actually NAC's own free radical scavenging properties that really matter. It would appear to be the latter, based on the short half life of NAC and the short term beneficial effect.  This would imply that just normalizing GSH is not enough. Studies have shown that normalizing the reduced levels of GSH levels found in autism is readily achievable.

Autism and Deafness? Then no Cochlear Implant for You

Monty, now aged 11 with ASD, has an assistant who comes in the afternoon to run our ABA-inspired home program, let’s call her Stella.

Stella is a student training to be a teacher for deaf people, so her knowledge of ABA comes from her time with Monty.  Nobody here uses ABA to teach the deaf.

Her latest task was to try and teach 3 eight year old children to count to ten.  The problem being that two of the children are deaf and can only say “yes” and “no” and the third child is deaf, autistic and non-verbal.

Using ABA she managed to teach the two deaf children to count out loud to ten and to match objects marked with each number.  Very good and the regular teachers were very impressed.

My comment was that it was a pity nobody taught them to say something more useful.  How about “hello”, “my name is Tom” etc.

What about the deaf boy with autism?  It turns out, where we live, if you are deaf and have other “complex needs” you do not get a cochlear implant.

Cochlear implants, when implanted while the deaf person is very young, can be hugely successful.  About 400,000 people worldwide have received them.  You end up with a different kind of sound than that experienced by non-deaf people, but it gives the brain inputs which allow it to identify and process speech and other sounds.

Cochlear Implants

  

A cochlear implant (CI) is a surgically implanted electronic device that provides a sense of sound to a person who is profoundly deaf or severely hard of hearing.

Cochlear implants may help provide hearing in patients who are deaf because of damage to sensory hair cells in their cochleas. In those patients, the implants often can enable sufficient hearing for better understanding of speech. The quality of sound is different from natural hearing, with less sound information being received and processed by the brain. However, many patients are able to hear and understand speech and environmental sounds. Newer devices and processing-strategies allow recipients to hear better in noise, enjoy music, and even use their implant processors while swimming.

Cochlear implants for congenitally deaf children are considered to be most effective when implanted at a young age, during the critical period in which the brain is still learning to interpret sound. Hence they are implanted before the recipients can decide for themselves, on the assumption that deafness is a disability.

Children with cochlear implants are more likely to be educated orally, in the standard fashion, and without access to sign language (Spencer et al. 2003). They are often isolated from other deaf children and from sign language (Spencer 2003). Children do not always receive support in the educational system to fulfill their needs as they may require special education environments and Educational Assistants. According to Johnston (2004), cochlear implants have been one of the technological and social factors implicated in the decline of sign languages in the developed world.

Cochlear Implants and Autism

Since people with autism have various sensory issues, they are not considered suitable candidates for the only therapy that could give them the ability to hear.  At least that is the case in many countries.

The word is that in the first year it is hard to adjust to a Cochlear Implant and this is one reason why they have to be implanted while the person is very young.  It takes the brain a while to adjust, and the more plastic it is, the better it can adjust.

Since people with autism are not exactly flexible at the best of times and they can be prone to tantrums and violence, they are seen as a challenge too far.

This seems rather cruel to me and very short sighted.

As Stella said:- “for that first year, who is going to look after them?”

My response would be “who is going to look after them for the next 60+?”

After all, would you rather care for a sometimes violent toddler for 12 months during his adaptation to hearing, or a sometimes violent, deaf autistic adult for life?  I suppose they just drug the adults.

How common is deafness with autism?

According to some research, about 5% of people with deafness have autism and about 3.5% of people with autism have deafness.

I think when they say “autism” they mean serious autism, not the modern, all-inclusive, DSM 5 autism-lite.

What does the Research tell us?

As always, there is data on just about everything and this includes autism with deafness and the efficacy of Cochlear Implants.

Rather as I expected, it is not true that giving hearing to deaf people with autism is a bad idea.  The research actually shows the opposite.

Just as teaching deaf people to count aloud is possible, when you apply simple behavioral techniques, so is giving hearing to deaf people with autism.

Cochlear Implantation in Children With Autism Spectrum Disorder.

Results: Fifteen patients with history of ASD and cochlear implantation were analyzed and compared with 15 patients who received cochlear implant and have no other disability. Postoperatively, more than 67% of children with ASD significantly improved their speech perception skills, and 60% significantly improved their speech expression skills, whereas all patients in the control group showed significant improvement in both aspects. The top 3 reported improvements after cochlear implantation were name recognition, response to verbal requests, and enjoyment of music. Of all behavioral aspects, the use of eye contact was the least improved. Survey results in regard to improvements in patient interaction were more subtle when compared with those related to sound and speech perception. The most improved aspects in the ASD patients' lives after cochlear implantation seemed to be attending to other people's requests and conforming to family routines. Of note, awareness of the child's environment is the most highly ranked improvement attributed to the cochlear implant.

Conclusion: Cochlear implants are effective and beneficial for hearing impaired members of the ASD population, although development of language may lag behind that of implanted children with no additional disabilities. Significant speech perception and overall behavior improvement are noted.

Children with Cochlear Implants:  Complex Needs - Complex Outcomes

"Although the group of deaf children with complex needs is overall a heterogeneous one, there are subgroups that would benefit from further and detailed investigation in thinking about cochlear implantation, for instance deaf children with Down’s syndrome, Children with Autistic Spectrum Disorder, cerebral palsy."

  

Children With Cochlear Implants and Complex Needs: A Review of Outcome Research and Psychological Practice