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Laryngopedia

To educate about voice, swallowing, airway, coughing, and other head and neck disorders

Laryngopedia By Bastian Medical Media

A Journal of Observational Laryngology

Clinical observations, anecdotes, case series, and conceptual frameworks for further exploration


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Babies Who Cannot Burp, and Still Cannot as Adults.

 

The big picture

Babies who cannot burp are in terrible misery. So are their parents. While the child struggles with daily colic, spitting up, sometimes projectile vomiting, and gassiness, the parents must deal with hours of crying each evening and much medical attention may have provided no answers.

The explanation for why some babies can’t burp may be found in the story of adults who had this kind of trouble in their infancy – trouble that has continued into adulthood.

Adults with a lifelong inability to burp were recently diagnosed with retrograde cricopharyngeus dysfunction, or R- CPD. This condition was successfully treated for the first time on record in November of 2016. The first publication of both the comprehensive syndromic criteria for diagnosis and the treatment with Botox was by Bastian and Smithson in March of 2019, in a review of their first 51 cases of a much larger subsequent caseload.

Bastian and colleagues have also published additional articles about R-CPD, on: the use of cricopharyngeus myotomy for recurrent cases; an alternate EMG-guided technique for injection; and longer term results.

As background, here are the primary symptoms in adults, some of whom had trouble as infants

Summarized from a caseload of nearly 600 patients treated, R-CPD’s debilitating adulthood syndrome typically consists of all–or at least some–of the following:

  1. Inability to burp. This could be recognized in childhood, teenage years, etc. Victims routinely say they do not remember being able to burp, except for the rare occasion.
  2. Easily audible and therefore socially awkward gurgling noises like croaking frogs, creaking floorboards, etc. made worse after eating or drinking.
  3. Abdominal bloating (and often chest and even low-neck pressure).
  4. Flatulence that can be excessive or even extreme after meals, later in the day and into the night.

Other common but less universal symptoms

Can include painful hiccups, nausea after eating, a feeling of being so full it is almost hard to breathe when bloating is bad, and hypersalivation.

What is the linkage between this large adult caseload and babies and children who cannot burp?

A percentage of these adult patients know by family lore that they were unable to burp as infants. Parents who accompany them to their initial visit to BVI tell me that they (the parents) couldn’t burp them as infants. They also recount that this was in turn associated with extraordinary stress on the part of both baby and parents.

One mother said to me, “I couldn’t get him to burp conventionally, and he screamed in pain. Someone even told me to roll him on the floor, but he still wouldn’t burp.”

Another said, “He burped for the first two weeks of life, and then stop burping. At that point, he became incredibly gassy.”

Several parents have used words such as: “If she/he had been our first child, he/she would have been our only child.”

Others recount numerous trips to pediatricians searching in vain for a solution to the colic, projectile vomiting, ceaseless crying, and so forth.

One of the most extreme stories came from a father accompanying his teenage daughter. He told me that he and his wife had become concerned when their daughter was a baby because 20 to 30 minutes after every feeding, she would throw up what looked like everything they had just fed her. Fearing she would starve, they began measuring what they fed her and then standing by with a bowl to try to catch what she threw up to measure and compare.

Some describe a kind of failure to thrive and poor weight gain. One set of parents mentioned that even in toddlerhood, they had to chase the little one around to get him to the table to eat. And once there, he often refused the food they offered, due to the GI distress that inevitably followed.

What causes the disorder in adults and possibly in children too?

R-CPD is caused by a dysfunction of the upper esophageal sphincter, found at the level of mid-neck. The sphincter is comprised primarily of the cricopharyngeus muscle. It acts like a circular “gate” or “muscular purse-string” between the lower throat and upper esophagus. (There are 4 other sphincters in the GI tract, ending with the anus.)

Simplistically, think of the upper esophageal sphincter – cricopharyngeus muscle – being contracted/closed almost 24/7 and even during sleep. It relaxes very briefly, perhaps for 1 second, each time we swallow saliva, liquid, or solid food, clamping down on the “tail” of each bolus that passes through it and remaining contracted until the next swallow.

This same sphincter must also relax briefly to belch or to vomit. In persons with R-CPD, swallowing function (requiring antegrade sphincter relaxation) is normal. But opening for burping and often vomiting (requiring retrograde relaxation) is impossible or extremely difficult.

The result is severe daily misery caused by the combination of symptoms described above in bold.

For adults, injection of Botox into the upper esophageal sphincter can magically resolve this misery. And in about 80% of adults, a single injection of Botox into the sphincter can resolve or dramatically diminish symptoms permanently. Those who recur after Botox injection may achieve permanent relief after a second injection, and a small fraction who recur a second or third time after good relief may go on to cricopharyngeus myotomy.

Did all adults with R-CPD have difficulty burping as babies and children?

No. Only a subset seems to have had such trouble that early in life. In my most recent 77 patients (the latest additions to a Bastian Voice Institute caseload nearing 600), here’s the breakdown:

  • 18 were told they could not burp as infants (24%) and that it was a problem at some level
  • 24 definitely could burp as infants (32%)
  • 12 could burp as infants but had a history of colic, spitting up, vomiting, etc. (16%)
  • 22 had no information (28%)

Of course, it is possible that some of these adults who could burp as babies, but had GI distress, were burping incompletely. And some who could not burp as babies may not have had the dramatic GI distress described in some of my anecdotes above. In other words, they may have been on the mild end of the spectrum. And it is possible that some babies who couldn’t burp grew up to become adults who could…

Still, it appears that there are a small number of babies in the world even today who, together with their parents, suffer profoundly in infancy and beyond, and so far, have been unable to find an explanation or relief.
What might be done for this group of babies/children with no other explanation for their misery (after profuse medical evaluations) than potential R-CPD? Could a subset of babies who can’t burp—the ones with extreme manifestations, failure to thrive, and whose parents who are “on the ledge”—be helped by a Botox injection too?

The very thought is obviously off-putting. But hold this thought alongside the absolute misery and anguish in the child, and of the sleep deprived parents due to their crying baby.

Consider also, that some of these babies continue to suffer well into adulthood. In fact, given a 7-point scale to indicate their view of the overall severity of their symptoms, the most common response in our large adult population by far is “6” and many choose “7.” Hence, treatment of the baby could possibly serve as “training wheels” for burping and avoid all those early years of misery and even misery extending decades into adulthood…

What is the solution?

I believe that somewhere, a courageous physician will partner with courageous (and desperate) parents to offer this treatment to a toddler or pre-schooler on the extremely severe end of the R-CPD continuum. It will require input from one or more pediatricians who have exhausted other ideas and diagnoses. They will need to see both the diagnostic and potential therapeutic potential of Botox administration. And there will need to be a precise plan about how to follow and monitor afterwards.

And eventually, after gaining confidence from having helped this early group of young children, it is possible that this treatment will—on a highly selective basis—be offered to a small number of babies in extraordinary distress from R-CPD that presents in infancy.



Can’t Burp? Here Are the Symptoms of R-CPD

Robert W. Bastian M.D.

There is a group of people whose inability to burp causes severe daily distress. They are left without a solution (or even explanation) in spite of many doctor visits. Recently a major cause of inability to burp, retrograde cricopharyngeus dysfunction (R-CPD) has been codified for diagnosis and treatment.*

*A constellation of key symptoms powerfully “makes” the initial diagnosis. Patients can often “make” the tentative diagnosis by matching themselves to this clear syndrome of R-CPD.

THE BIG FOUR SYMPTOMS OF R-CPD THAT PROVIDE VIRTUALLY 100% ACCURACY IN DIAGNOSIS

  1. Inability to burp. This is almost always, but not exclusively “lifelong,” though persons may not recognize this as a “problem” or “difference from others” until early childhood or teenage years.
  2. Socially awkward gurgling noises. These noises can be mostly quiet and “internal,” but more often are loud enough to be embarrassing.  Mouth opening makes them louder. Almost everyone says they are easily heard several feet away; not infrequently “all the way to the door.” They engender social anxiety in most persons with R-CPD, causing some to avoid eating or drinking for hours before social occasions and even during them. Carbonation makes them much worse and is to be avoided at all costs. Some more colorful patient descriptions: Symphony of gurgles; Croaking frogs; Creaking floorboards; Dinosaur sounds; Strangled whale
  3. Bloating/pressure. Most common location is high central abdomen. Distention is common, especially later in the day. Using pregnancy as an analogy even in men, the usual degree of distention is described as “3 or 4 months.” “Six months” is not rare, and one slender young man was “full term.” Almost as often as abdominal distress, patients describe chest pressure, and for some that is the worst symptom. Some have pressure in the low neck. While “pressure” is the frequent descriptor, some experience occasional sharp pain in abdomen, back, or between shoulder blades. Some have to lie down after eating to find some relief.
  4. Flatulence. Routinely, this is described as “major,” or even “ridiculous.” Flatulence increases as the day progresses, and many experience it into the night. When around others, some scan their surroundings at all times for a place they can go briefly to pass gas. Understandably, the social ramifications of this problem can also be major.

COMMON BUT LESS UNIVERSAL R-CPD SYMPTOMS OR EFFECTS

  1. Nausea, especially after eating larger than normal amounts or drinking carbonated beverages.
  2. Painful hiccups, again more commonly after eating.
  3. Shortness of breath. A person can be so full of air that athletics, or even ability to climb stairs, etc. are impaired.
  4. Hypersalivation when symptoms of bloating are major.
  5. Inability to vomit. A few simply cannot vomit; more often it is possible but only after strenuous retching. Vomiting (spontaneous or self-induced) always begins with a very loud noise and major release of air in a phenomenon we call “air vomiting.”  Emetophobia can be major.
  6. Anxiety and social inhibition. This can be MAJOR due to gurgling, flatulence, and discomfort.
  7. (Still under evaluation): The question is whether the descending colon dilates over time if flatulence cannot be responded to, so that muscular effectiveness is diminished.

Check out our list of resources containing peer-reviewed articles, patient stories and more!

 

Abdominal Distention of R-CPD: Series of 3 photos

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Gastric Air Bubble (1 of 3)

This abdominal xray of an individual with R-CPD shows a remarkably large gastric air bubble (dotted line), and also excessive air in transverse (T) and descending (D) colon. All of this extra air can cause abdominal distention that increases as the day progresses.

Bloated Abdomen (2 of 3)

Flatulence in the evening and even into the night returns the abdomen to normal, but the cycle repeats the next day. To ask patients their degree of abdominal distention, we use pregnancy as an analogy in both men and women. Not everyone describes this problem. Most, however, say that late in the day they appear to be “at least 3 months pregnant.” Some say “6 months” or even “full term.” In a different patient with untreated R-CPD, here is what her abdomen looked like late in every day. Her abdomen bulges due to all of the air in her GI tract, just as shown in Photo 1.

Non-bloated Abdomen (3 of 3)

The same patient, a few weeks after Botox injection. She is now able to burp. Bloating and flatulence are remarkably diminished, and her abdomen no longer balloons towards the end of every day.

Can’t Burp: Progression of Bloating and Abdominal Distention – a Daily Cycle for Many with R-CPD

This young woman has classic R-CPD symptoms—the can’t burp syndrome. Early in the day, her symptoms are least, and abdomen at “baseline” because she has “deflated” via flatulence through the night.  In this series you see the difference in her abdominal distention between early and late in the day.  The xray images show the remarkable amount of air retained that explains her bloating and distention.  Her progression is quite typical; some with R-CPD distend even more than shown here especially after eating a large meal or consuming anything carbonated.

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Side view of a bloated abdomen (1 of 6)

Early in the day, side view of the abdomen shows mild distention. The patient’s discomfort is minimal at this time of day as compared with later.

Mild distension (2 of 6)

Also early in the day, a front view, showing again mild distention.

Front view (3 of 6)

Late in the same day, another side view to compare with photo 1. Accumulation of air in stomach and intestines is distending the abdominal wall.

Another view (4 of 6)

Also late in the day, the front view to compare with photo 2, showing considerably more distention. The patient is quite uncomfortable, bloated, and feels ready to “pop.” Flatulence becomes more intense this time of day, and will continue through the night.

X-ray of trapped air (5 of 6)

Antero-posterior xray of the chest shows a very large stomach air bubble (at *) and the descending colon is filled with air (arrow).

Side view (6 of 6)

A lateral view chest xray shows again the large amount of excess air in the stomach and intestines that the patient must rid herself of via flatulence, typically including through the night, in order to begin the cycle again the next day.

Shortness of Breath Caused by No-Burp (R-CPD)

Persons who can’t burp and have the full-blown R-CPD syndrome often say that when the bloating and distention are particularly bad—and especially when they have a sense of chest pressure, they also have a feeling of shortness of breath. They’ll say, for example, “I’m a [singer, or runner, or cyclist or _____], but my ability is so diminished by R-CPD.  If I’m competing or performing I can’t eat or drink for 6 hours beforehand.”  Some even say that they can’t complete a yawn when symptoms are particularly bad.  The xrays below explain how inability to burp can cause shortness of breath. 

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X-ray of trapped air (1 of 2)

In this antero-posterior xray, one can see that there is so much air in the abdomen, that the diaphragm especially on the left (right of xray) is lifted up, effectively diminishing the volume of the chest cavity and with it, the size of a breath a person can take.

Side view (2 of 2)

The lateral view again shows the line of the thin diaphragmatic muscle above the enormous amount of air in the stomach. The diaphragm inserts on itself so that when it contracts it flattens. That action sucks air into the lungs and simultaneously pushes abdominal contents downward. But how can the diaphragm press down all the extra air? It can’t fully, and the inspiratory volume is thereby diminished. The person says “I can’t get a deep breath.”

A Rare “abdominal crisis” Due to R-CPD (inability to burp)

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X-Ray of Abdominal Bloating (1 of 2)

This young man had an abdominal crisis related to R-CPD. He has had lifelong symptoms of classic R-CPD: inability to burp, gurgling, bloating, and flatulence. During a time of particular discomfort, he unfortunately took a “remedy” that was carbonated. Here you see a massive stomach air bubble. A lot of his intestines are air-filled and pressed up and to his right (left of photo, at arrow). The internal pressure within his abdomen also shut off his ability to pass gas. Note arrow pointing to lack of gas in the descending colon/rectum. NG decompression of his stomach allowed him to resume passing gas, returning him to his baseline “daily misery” of R-CPD.

X-Ray of Abdominal Bloating (2 of 2)

X-Ray without markings

More Interesting Esophageal Findings of R-CPD (Inability to Burp)

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Stretched Esophagus (1 of 4)

Using a 3.7mm ENT scope with no insufflated air, note the marked dilation of the esophagus by swallowed air the patient is unable to belch. T = trachea; A = aortic shelf; S = spine

Tracheal Wall (2 of 4)

The posterior wall of the trachea (T) is better seen here from a little higher in the esophagus. A = aorta

Over-dilation (3 of 4)

The photo is rotated clockwise at a moment when air from below is pushed upward so as to transiently over-dilate the esophagus. Note that the esophagus is almost stretching around the left side of the trachea in the direction of the arrow.

Bronchus (4 of 4)

Now deeper in the esophagus (with it inflated throughout the entire examination by the patient’s own air), it even appears that the left mainstem bronchus (B) is made visible by esophageal dilation stretching around it.

Dramatic Lateral Dilation of the Upper Esophagus: Series of 3 photos

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(1 of 3)

This photo is at the level of (estimated) C6 of the spine (at S). This person has known cervical arthritis, accounting for the prominence. Opposite the spine is the trachea (T). Note the remarkable lateral dilation (arrows) in this picture obtained with with no insufflated air using a 3.6mm ENF-VQ scope. It is the patient’s own air keeping the esophagus open for viewing.

(2 of 3)

At a moment when air from below further dilates the upper esophagus, the tracheal outline is particularly well-seen (T) opposite the spine (S). The “width” of the trachea indicated further emphasizes the degree of lateral dilation, which is necessary because spine and trachea resist anteroposterior dilation.

(3 of 3)

Just for interest, at mid-esophagus, the familiar aortic “shelf” is seen. Again, this esophagus is being viewed with a 3.6 mm scope with only the patient own (un-burped) air allowing this view.

What the Esophagus Can Look Like “Below A Burp”: Series of 3 photos

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Baseline (1 of 3)

Mid-esophagus of a person with R-CPD who is now burping well after Botox injection into the cricopharyngeus muscle many months earlier. The esophagus remains somewhat open likely due to esophageal stretching from the years of being unable to burp and also a “coming burp.”

Pre-burp (2 of 3)

A split-second before a successful burp the esophagus dilates abruptly from baseline (photo 1) as the excess air briefly enlarges the esophagus. An audible burp occurs at this point.

Post-burp (3 of 3)

The burp having just happened, the esophagus collapses to partially closed as the air that was “inflating it” has been released.

 

Where have patients traveled from?

 

R-CPD patients BVI treated across the USA

World Map of RCPD Patients



Still Coughing After COVID-19?

Covid-19 has intruded into our lives in innumerable ways. Worst of all for some, is that they contracted the virus.

 

Among those stricken by the virus are surely a number of persons who are some weeks or even months past the acute infection and who feel generally well. But, they are experiencing a lingering cough. Especially early on, this cough may be continuing to clear secretions that accompany resolving lung inflammation. In that case, the lingering cough has a purpose—it is accomplishing something.

For others, the cough is no longer solving a problem, but it IS the problem. That is because for them, the infection has “damaged,” or “sensitized” sensory nerve endings in the trachea, larynx, or throat. These “wild” nerve endings go “ZING!!” and demand that you cough.

Such persons may have developed a condition called sensory neuropathic cough (SNC).

Key features of SNC are:

  1. Each episode of coughing (whether a single cough, a few seconds of coughing, or a violent and protracted 30-second or longer attack) is initiated by a stereotyped sensation:  a “tickle,” “dry patch,” “itch,” “pinprick,” “dripping sensation,” etc. Commonest location is at the sternal notch—the lowest part of the neck before you reach the sternum.
  2. The cough is out of proportion to anything produced by that cough. The cough is dry, or it produces mucus only after hard coughing. The person says, “Who coughs this much to produce that much mucus?” The person may even cough hundreds of times a day, mostly briefly, but also have agonizing protracted and exhausting episodes a few times a day … to the point of embarrassment or even humiliation when they occur in public.
  3. Trigger phenomena. While coughing spells can occur spontaneously, certain actions make it more likely that you will cough. Things like talking, a loud laugh, breathing in air either colder or warmer than ambient (going outside, etc.), a strong odor, swallowing, change of position (such as when getting in bed or getting up in the night). Or touching a certain spot on the neck, and most commonly again at the sternal notch.
  4. Seeming futility of usual treatments. Taking an expression from the classic film, Casablanca, “rounding up the usual suspects” of allergy, acid reflux, and asthma is pointless.  Steroids may help but only while on them. Narcotic cough suppressants may provide some relief, but are purely a symptomatic treatment.
  5. The correct approach is to target the irritable nerve endings. Things like amitriptyline (or desipramine), gabapentin, citalopram, mirtazepine, venlafaxine, capsaicin spray, etc. Usually one of the first two is effective. Persons with resistant cases may need to work their way down a longer list of medication trials.  Occasionally, injection of a long-acting local anesthetic (mepivacaine, bupivacaine, or similar) combined with a locally-acting steroid (triamcinolone or similar) into a sensitized area can help.

What are the practicalities of getting help for a “Covid-19 Cough?”  Some ideas:

  1. If really early after the infection and the cough is congested and productive, stay the course of conventional Covid-19 treatments, possibly including steroids and standard cough suppressants.
  2. If the infection was weeks ago; you are feeling reasonably well; and the main impediment to recovery and return of quality of life is your cough, educate yourself more fully about sensory neuropathic cough. Laryngopedia.com has many articles, videos, patient interviews, etc. that you can find by typing “cough” into the search window. Or read this article on the subject: https://peerj.com/articles/816/ It is a peer-reviewed article found in an open-source medial journal (that is, not behind a paywall).
  3. Raise the question with your personal physician. Consider providing the above article for his or her perusal, since your doctor may not have encountered this diagnosis before. The treatment protocol is clearly outlined.
  4. Call nearby ENT or pulmonary doctor groups in particular and ask to speak to the triage nurse.Ask him or her “Do any of your doctors treat sensory neuropathic cough?” If “crickets,” call the next group with the same question.
  5. If you exhaust all local sources of help, consider a teleconversation with Laryngopedia.com, or with a Bastian Voice Institute physician. Doctors there would also be glad to help you in person, if convenient for you.

Here’s wishing you speedy resolution of your cough, maybe as a brief aftermath of Covid-19.  This information is offered just in case you struggle with coughing that won’t go away after many weeks, and despite many tests and treatments. If, as it appears, SNC has for all of human history been “caused” in many cases by endemic viruses, why not by a novel one like Covid-19?



The Gasping Syndrome

By Dr. Bastian M.D.

A few times a year, distressed patients present from their internists or pulmonologists to see if I can explain their shortness of breath. The patient has already undergone numerous tests: e.g. chest x-ray, pulmonary function tests, EKG, stress test, echocardiogram, CT, blood tests, and even bronchoscopy. Everything has come back normal, or anything even slightly abnormal (the reader can fill in the blank) has been treated maximally without any reduction of the patient’s symptoms.

In short, the patient has complied with every test and treatment recommendation; yet there is still no apparent explanation for this difficulty breathing, and no relief provided by numerous treatment trials.

The patient seems weary of the problem. My “antennae” do not pick up any signals of la belle indifference (a peculiar lack of concern about symptoms that when present suggests that the problem may be non-organic). She tells me that her breathing is never noisy, and that her shortness of breath may or may not be associated with exercise. I apply my subjective but extremely useful “flow-volume loop” by asking her to exhale to “empty” and then rapidly breathe in until completely full, and there is no unusual noise, and no prolongation of the time required to fill.

My initial thought in this scenario? The gasping syndromeWhat is that? A recognizable but to my knowledge previously undescribed disorderHere is my composite description, meaning that individual patients may not fit every element of what follows:

  1. Either with exertion or at rest, the patient has an abrupt* sense of “smothering,” or air hunger. It happens almost from one second to the next. Like the abrupt shooting pain of neuralgia, the abrupt tickle of sensory neuropathic cough / throat clearing, or the abrupt laryngeal closure of laryngospasm. The patient says she can be engrossed in a movie or a book strolling with the dog when suddenly her consciousness is invaded by a need to “get more air.”
  2. She responds by taking a deep breath, but there is no relief and the sensation remains. So, she takes a series of deep breaths, all of them to no avail. The feeling is oppressive, anxiety-provoking, gaspingThis sensation may last for a few seconds, to several hours.* Unfortunately, it can happen again some time later, or maybe just a time or two per day or even per week.

Having seen many people who fit the gasping syndrome across the decades, though not usually more than a handful per year, I have speculated that this is a sensory disturbance. After all, I ponder, if it represents more than a primary neuropathic sensation, why is oxygen saturation measured by oximetry, including during exercise, always normal? Why are all the tests of heart and lung function normal? Why no mitral valve prolapse, or something to explain this? Why nothing on imaging? Why no noisy breathing?

My mind goes to patients who describe a similar sensation at the moment IV contrast is injected for a CT scan. In addition to “warmth,” “a sensation of needing to urinate,” etc., they can have a sensation of abrupt, quite compelling, and thankfully transient smothering.  Or to someone I know who in the middle of an IV infusion of a biologic modifier for cancer, (with prior warning) experienced profound air hunger that started abruptly and was completely gone 20 seconds later. In each of these cases, it seems like a primary sensory phenomenon more than an alerting and protective sensation in response to low oxygen or high carbon dioxide levels.

How would one conceptualize a mechanism? Here is one thought experiment: consider that we are all supplied with pulmonary stretch receptors. They send messages “in the background” several times across the day and night to the respiratory center: Excuse me… Take a deep breath; expand those alveoli; your surfactant is giving you some atelectasis. And so, without being aware of it, we sigh now and then. We roll over in our sleep, mutter, grind our teeth, and take a deep breath. While reading a book we subconsciously shift in our seat and inspire deeply as we turn a page. Again, we are unaware of this, but an observer watching us read or, equipped with night vision goggles and watching us sleep, will see it.

What if the pulmonary stretch receptors send a signal Hey! Time to take a deep breath! and despite our taking a deeper breath outside of conscious awareness, the respiratory center does not receive the return communication, Action completed. Even with normal O2 and CO2 levels, if the brain thinks that deep breath did not happen, would it not intrude on conscious thought to re-command more urgently: Hey! Deep breath please!

Where does one go to help the patient with this thought experiment diagnosis? Just give her this explanation and leave it there? Or, maybe punt back to the primary care or pulmonary or cardiology physician…? But what if the person has already seen 3 of each specialty—one who is local, another at a nearby university center, and a third at a national referral center? What if “every conceivable test” has already been done 3 times?

A follow-on thought experiment: to the patient, and via a summary letter to her doctors, explain the concept of dysesthesia or sensory disturbance. Give her analogies: at the dentist, after anesthesia is in place to allow that root canal or filling, your tongue/lip can feel swollen, but a look in the mirror shows them to be normal. When a leg goes to sleep, it can feel fatter and heavier than the other one, but it is not. When you suffer nerve damage from diabetes, it can feel like bees are stinging your feet, but none are present in the room.

And then explain scenarios where persons have to accommodate to or neglect the feeling of contact lenses. The sound of new tinnitus. The tickling of an indwelling tracheotomy tube. The dramatic sensation a sword swallower must ignore during the show. Or the sensation of air hunger the pearl diver must overcome to stay submerged for 2 minutes while swimming vigorously.

Perhaps point out that it appears there is no danger from this ominous-feeling sensation.  Remind her of the multiplicity of prior “normal” tests. Maybe suggest that she experiment with assuming “control” of her response to the sensation by saying to herself: It is just a feeling! Or, Stupid pulmonary stretch receptors! Maybe gently alter behavior to see if it has any impact. Introduce inspiratory resistance via “straw-breathing.” Or a gentle Valsalva maneuver. Or, exhale slowly through pursed lips whose opening is the size of a coffee-stirrer.

And if nothing else works, suggest trying to mentally shrug and “throw the sensation over the shoulder.” And then keep going. The very worried individual can purchase an oximeter. Or even a stethoscope as a “crutch” to allay anxiety.

All of this can be offered with a physician’s apologies that he or she has nothing better to offer. And also with encouragement that these ideas have “liberated” other patients struggling with the same problem.

A final thought: just as neuromodulators can help persons suffering from neuralgia or sensory neuropathic cough/throat clearing, and laryngospasm, consider a “sensory neuropathic cough” strategy of working one-by-one, from medication to medication such as amitriptyline, gabapentin, etc., hoping to find one that helps.

*Some describe a more continuous, lower-grade background sensation of difficulty breathing with abrupt “peaks”

For more learning materials, check out our Multimedia Encyclopedia.

For more information on Dr. Bastian, go to our website at bastianvoice.com



Cricopharyngeus Spasm and What to Do About It

By Robert W. Bastian, MD

 

The information found below is a Q&A supplement to a teaching video on cricopharyngeus spasm:

Tell me more about the cricopharyngeus muscle. Where is it, and what is it for?

It is a sphincter, which is a circular muscle that makes a ring around the uppermost part of the esophagus to serve as a “valve” or “gateway” between the throat and esophagus. In the gastrointestinal tract, there is a series of additional sphincters: between lower esophagus and stomach; at the outlet of stomach into small intestine, and so forth down to the anus. Each of these sphincter muscles “at rest” is in a contracted state. When “in use,” each sphincter is momentarily relaxed.

When we swallow, the cricopharyngeus muscle sphincter must relax for a brief moment to let food or liquid out of the throat, through the sphincter, and into the upper esophagus. (It also relaxes momentarily, by the way, to release air from below upwards when we belch.) Normally we are unaware of this sphincter muscle because it functions outside of our consciousness.

You’ve used the word “spasm” as the cause of the sensation, but can you clarify what you mean?

By use of the word spasm, we don’t mean a cramp or a charley horse. It is instead sustained hypertonicity of the upper esophageal sphincter, or cricopharyngeus muscle. It is over-contracted. That’s why we become aware of it when this overcontraction gives rise to a feeling of pressure, choking, etc. defined as cricopharyngeus spasm.

By analogy, we are normally unaware of neck muscles that we use to hold up our heads. Yet, we have all had the experience of tight posterior neck muscles that we find to be bothersome and to intrude sufficiently into quality of life that we might ask a friend “Can you rub my neck?” In similar fashion, cricopharyngeus spasm often intrudes into a person’s mental space, and becomes oppressive, but there seems to be no way to relieve the sensation. Not only that, but the sensation causes us to worry, partly because many people with this problem search in vain to find someone to explain this to them, and they are therefore unable to put aside their worry about the sensation.

What is the list of features or symptoms that constitute the syndrome of cricopharyngeus spasm?

  1. Location of the sensation is at the level of the cricoid cartilage in the mid-to low neck.
  2. The nature of sensation is often described with words like choking, pressure, constriction, swelling…. Or, “It feels like a wad of phlegm.” One other particularly common description is “It feels like there is something stuck in my throat that I can’t swallow and can’t spit out.” Many say that this sensation is oppressive and preoccupying.
  3. The magnitude of the sensation tends to vary. Most often, it tends to become worse as the day progresses. A person might also notice that symptoms increase when they are stressed or fatigued, whatever the time of day.
  4. Active eating provides transient relief of the sensation. While the discomfort seems to originate in the swallowing passage, there is no interference with actual travel of food or drink.That is, the person’s ability to swallow food and liquids is fine. And in fact, during active eating,such as at lunch or dinner, the sensation subsides or even goes away completely, only to begin to return soon after finishing that meal.

Why does eating reduce the symptoms?

A person can’t rub the tight muscle directly, but each time a swallow happens, the sphincter muscle must let go momentarily, as already mentioned. This is then a kind of therapy to the over-contracted, hypertonic muscle. Swallowing saliva doesn’t help, because the volume we swallow is small, and the swallows are widely spaced in time. Active eating of a sandwich or plate of food during a mealtime, by contrast, relaxes the sphincter fully every 10 to 20 seconds across a span of 15 minutes or more. To repeat, this serves as a kind of unplanned “therapy” of the muscle and the symptoms subside for a time.

What can be done?

There is an old saying by Alexander Pope: “To know ourselves diseased is half our cure.” This seems to apply here as many patients say that simply coming to understand the disorder, as described above, is a major step towards relief. One woman said: “When I understood what was wrong, and that I didn’t have to worry about cancer, the problem started to gradually go away across the next several weeks.

For this to happen, however, the patient must accept and really believe in their diagnosis. Doctors can help them feel secure by making the diagnosis partially through what we call a “mind reading” exercise. It works like this: Right after the person says “Doctor, there is a lump…..”, we gently stop him or her, and ask if we can try to “read their mind.” The idea is that at the end of the “mind reading”, the patient is invited to say, “That’s it exactly!” Or, “That is mostly it but you said _________ and I would say __________.” Or, the patient might say “That isn’t at all what I am experiencing” and in this third case, attention is turned to a different diagnosis. If the physician does successfully “read the patient’s mind,” describing patient symptoms exactly, the patient finds it easier to accept and believe in the diagnosis because of the “mind-reading” involved.

Keep in mind that there really isn’t an examination finding, blood test, or X-ray to make the diagnosis for cricopharyngeus spasm. It is based in the precise 4-part syndrome described above.

Many such patients report that they can now “throw the sensation over their shoulders.” In other words, if particularly symptomatic, they can more easily say to themselves: “That crazy muscle….” It is a little like ignoring the sensation of new glasses, or contact lenses. And as mentioned, many say that knowing and trusting the diagnosis all by itself is the beginning of slow, steady, reduction and eventual disappearance of the symptoms across a few weeks’ time.

What if I want to do more than just “know myself diseased”? Isn’t there anything medical the doctor can do?

The occasional patient asks this and our list of further options includes:

  1. Work with a physical therapist or speech pathologist. The focus of that work is anterior neck relaxation, possibly by use of surface biofeedback electrodes, yawning maneuvers or very gentlestrap muscle massage (carefully avoiding going anywhere near the vascular compartments of theneck/ carotid arteries!!)
  2. A confirmatory “diagnostic” test in the form of a muscle relaxant, such as valium. That medication, called a benzodiazepine, has a tranquilizer effect, but we actually wish we could take it out of the medication. For our purposes, it is only the muscle relaxant property that we want. But they of course come together…. So here is how to do the diagnostic trial:
    1. We provide a prescription for four tablets of 5 mg each. These four tablets each represent a “diagnostic test.”
    2. Get into your pajamas about 2 hours before your normal bedtime on an evening whenyou are not planning to go out, and when you are also more than averagely symptomatic. Now take a moment to “commune” with your CPS sensation. “Measure” it on a 10-pointscale, for example. (Suppose you choose “6.”) Notify family that you are about to do your first diagnostic test of CPS. You can also set a loud timer for 90 minutes.
    3. Now, take Valium tablet #1.
    4. 90 minutes later, commune with/measure for a second time the level of the sensation. It is usually not gone altogether, but noticeably diminished. 25% to 75% reduction wouldbe what people commonly report. So on this particular evening, you might notice that“6” has become “2” or “3.” Again, this is the result of the direct muscle relaxant properties of Valium.
    5. If you fall asleep on the couch and waken in the middle of the night, you have three more opportunities to do the diagnostic test in coming days or weeks.
  3.  Rarely, a person can have the muscle injected with botulinum toxin. This is in the extreme casewhere the person isn’t worried about the diagnosis, but is simply plagued by the sensation itself. Of hundreds of persons we have diagnosed with this disorder, we have done this in a singlepatient, who achieved great benefit for the duration of botulinum toxin effect, approximately 4 months.

In summary, cricopharyngeus spasm occurs when hypertonicity in a circular, “valve” muscle mid- to low neck causes the sensation of a lump or choking, etc. It is a benign condition but especially while it remains undiagnosed, it can cause great distress and worry.



Swelling Checks to Detect Vibratory (Overuse) Injury to the Surface Tissue (Mucosa) of the Vocal Cords

 

 

Definition: Vocal tasks (swelling checks) that detect acute or chronic vocal fold mucosal injury reliably; Secondarily, they can also detect gaps between otherwise normal folds.
Purpose /rationale: To provide persons with a way to detect mucosal trouble for themselves. We are in effect “taking all of the clothes off the mucosa.”
Who they are for: Anyone who uses the voice extensively or vigorously—particularly vocal overdoers.*
What they are not for: Voice training or performance.
When they should be done: When first learning the tasks, they should be done often until the concept of one’s mucosal ceiling is understood (see below). Once both proficiency and ceiling are established, the tests require 20 seconds or less both morning and evening.

TEST I: “HAPPY BIRTHDAY”

  1. In your upper voice range, sing the first phrase of “Happy Birthday” as softly as you can, using a “boy soprano pianissimo.” Resist the temptation to “make it work” by getting louder!
  2. Repeat the phrase at progressively higher pitches.
  3. Verify carefully the pitch at which you falter (onset delays or air escape) or can’t go higher without getting louder. THIS IS YOUR MUCOSAL CEILING PITCH, FOR THIS TASK.
  4. If your mucosa is normal, the “soft voice” and “loud voice” ceilings should be about the same.
  5. If your mucosa is abnormal, the “loud” ceiling should be higher than the “soft.”

TEST II: STACCATO

  1. Sing again “boy soprano pianissimo” using the descending staccato figure so so so so so fa mi re do
    (5-5-5-5-5-4-3-2-1; e.g. G-G-G-G-G-F-E-D-C) Attack each note precisely in the middle of the continuum
    between an aspirated ho and a coupe de glotte. In other words, lightly, precisely, and with a little bounce.
  2. As for “Happy Birthday,” repeat at progressively higher pitches.
  3. Again carefully verify the pitch at which you experience onset delays or air escape or can’t go higher without getting louder. THIS IS YOUR MUCOSAL CEILING PITCH, FOR THIS TASK.

COMMON QUESTIONS

My mucosal ceiling is higher when I do the staccato exercise than it is when I do “Happy Birthday.” What does that mean?

Though needing verification via careful laryngeal examination, this phenomenon suggests that a small gap between the folds, rather than swelling, is the problem.

My mucosal ceiling is higher when I do the “Happy Birthday” exercise than it is when I do staccato. What does that mean?

Again needing verification, this phenomenon suggests a mucosal disturbance rather than a gap as the explanation.

I can figure out my mucosal ceiling easily enough, but how do I know if it is normal?

This can be answered best at the outset by individuals who can compare your performance with that of hundreds of others to whom they have applied these tests (e.g. laryngologist, speech pathologist, voice teacher). It is also helpful at the beginning to correlate your mucosal ceiling with high quality visualization of the vocal folds.

What if my ceiling isn’t normal as compared to others?

The swelling tests are nevertheless just as valuable! Here’s how: Suppose an individual’s initial mucosal ceiling is abnormal because of small vocal nodules, but the person is happy with the voice’s capabilities. Here, the swelling tests can be monitored to help the individual prevent additional mucosal injury, by not allowing the ceiling to descend any further. A different person whose initial ceiling is abnormal might be unhappy with
perceived limitations due to mucosal injury. Now, ongoing use of the swelling tests can confirm the benefits of medical, behavioral (voice therapy) or, eventually, surgical treatments, because the ceiling will rise with successful treatment. Furthermore, these tests can help to avoid recurrent injury.

What if I notice that my ceiling is abnormal (lower) as compared to my usual?

First, consider recent voice use for the possibility that it was “too much.” If so, and/or if the ceiling
remains lowered on subsequent trials of the tests later in the day, “back off” by reducing voice use
until the ceiling returns to your usual pitch, whether “normal” as compared to other persons or not.
Women: Some may find that the ceiling lowers routinely during pre-menstrual days, but returns to normal in a few days.

Do I need to cancel everything until the ceiling recovers?

This depends on the severity of the lowering of the ceiling. Generally, however, careful strategy concerning amount and manner of voice use during this time will allow the mucosa to recover while you continue to work or perform.

Are there common pitfalls in use of the swelling tests?

First and foremost, is the tendency to adjust how the voice is produced when the voice begins to falter. A singer will, for example, unconsciously get a bit louder or use a slight glottal attack to “make it work,” thereby reducing the sensitivity of the tasks. Another might be to perform them without a pitch reference at hand, so that the value of comparing with one’s known “ceiling” pitch is lost. A third might be to become a bit too obsessive and easily “spooked” with any ceiling change. And finally, comes the tendency to “lose the habit!”

* Vocal overdoer: Defined as an individual with both of the following:
  1. A high propensity to use the voice. Generally, “sixes and sevens” on a 7-point (maximum) intrinsic talkativeness scale.
  2. A high extrinsic opportunity or invitation to use voice, based on family, social, vocational, and avocational considerations.


How Do You Choke? Let Me Count the Ways…

By Robert W. Bastian, MD

Four examples of how the term “choking” can mean different things

When asked the reason he was calling for an appointment, a middle-aged man offered that he was “choking.” Staff not unreasonably marked on his chart that swallowing was his primary issue. In the first minutes of our time together, he focused my attention on “choking caused by swallowing.” But it shortly became clear that his choking was not “something going down the wrong pipe.” In fact, he had undergone prior xray evaluation of his swallowing function, and passed with flying colors. Rather, as he explained to me, a routine swallow would occasionally trigger a tickling sensation that set off a paroxysm of violent coughing. Bottom line? He was experiencing sensory neuropathic cough but with swallowing, especially of acidic or “scratchy” foods as one of his most prominent triggers.. He had used the word “choking” because the coughing was so intense and prolonged that he could only think of that word to describe the episode. Resolution: He was given the explanation he needed about sensory neuropathic cough and we began our search for best medication with low-dose amitriptyline…

Another “choking” patient was also initially designated as having a swallowing problem, but in his view, he ate and drank normally. During a bad cold 2 months earlier, he had initially “coughed his head off.” Although the cough had mostly subsided, he had started to awaken at night “choking,” and unable to breathe. He had endured 4 such frightening episodes across the past two months or so, thankfully with none in the past 3 weeks. Despite the recent quiescence, he was terrified it would happen again.

If a drip of saliva, or an upsurge of stomach acid had been the entire explanation, he would be expected to have simply awakened coughing. But his episodes had been much more than that — his laryngeal closure reflex was triggered, and the result was much more dramatic than coughing. He made loud inspiratory breathing noise for what seemed like an eternity but was actually about a minute. In fact, his events have a name: laryngospasm. Resolution: He was given a clear explanation of laryngospasm, an introduction to straw breathing, a suggestion that he take an acid-reducing medication for a month, and departed much relieved.

A third patient, an elderly person, gave “choking” as the reason for the visit, and the chart was so labeled. In this case it was swallowing at issue. This person had been taking longer to eat and was having to chew extra carefully. He had abandoned eating meat, gummy bread, and even mashed potatoes. His diet had become mostly soft foods, puree consistency, and liquids, because solid foods and some larger pills were getting stuck low in his throat regularly. The sensation was very uncomfortable and made him fear his airway would be blocked. A few times, he had tried to sip liquids to clear the sense of blockage, but the liquid wouldn’t pass and instead came out his nose. A videofluoroscopic swallow study confirmed that his trouble really was a disorder of the upper esophageal sphincter called cricopharyngeus dysfunction. Resolution: Initial swallowing therapy to work on ways to reduce the problem behaviorally through food choices and extra care in chewing, and also the offer of cricopharyngeus myotomy.

Yet a fourth patient, also elderly, described “choking” as the primary issue. Swallowing was also truly the problem here, too, except that it was happening mostly with liquids. Soft solids, and puree consistency worked quite well. A videofluoroscopic swallow study here showed good opening of the upper esophageal sphincter, but a pronounced tendency to pool some swallowed material in the throat after swallowing. When liquids were consumed, some tended to go into the airway—“down the wrong tube”—to cause coughing. Resolution: Swallowing strategies, thickening liquids to slow them down, and taking liquids as sips were recommended.

Patients and their doctors can work efficiently to an understanding of “choking” when the four common uses of the word are kept in mind, as described above, and summarized below.

1. I am choking. By that I mean that swallowing, (or a loud laugh, or breathing in cold air, or….) causes an abrupt tickling sensation which in turn initiates a violent attack of coughing. (Sensory neuropathic cough)

2. I am choking. By that I mean that suddenly, my throat seems to close; I can’t breathe, and I begin making loud noises when I try to breathe in. It is terrifying but seems to be over within a minute or two. (Laryngospasm)

3. I am choking. By that I mean that when I swallow, solid food and pills tend to get stuck in my throat <point to where> and sometimes I gag and try to spit to get it up. (Cricopharyngeus dysfunction with or without Zenker’s Diverticulum)

4. I am choking. By that I mean that when I swallow liquids in particular, it wants to go down the wrong tube and it makes me cough. (Aspiration)



When Mucus Seems to Be Causing Your Cough or Throat Clearing – But Isn’t

By Robert W. Bastian, MD

We all have experience trying to manage mucus that is causing us a problem. Sometimes, such as during an upper respiratory infection, our bodies over-produce mucus, in which case we cough it up, blow it out, or “dry it up” with decongestants. Other times, mucus may become thick and crusted, in which case we thin it out by drinking more fluids, using a humidifier and Neti pot, or taking over-the-counter mucolytics. And, if mucus becomes purulent, we treat it with antibiotics.

When Apparent Mucus Is Not the Problem
When it comes to coughing and throat-clearing, however, there are two kinds of circumstances in which mucus might seem to be the cause, but actually is not:

1. When a sensation of mucus is felt, but, while hard to believe, there is not any actual mucus present
2. When mucus is indeed present, but it is the result of coughing rather than its cause

At Bastian Voice Institute we have seen hundreds of cough and throat-clearing patients who at first cannot identify with either circumstance #1 or circumstance #2, but for whom one of the two above circumstances in fact applies. These patients were all diagnosed not with a “mucus” problem, but instead with sensory neuropathic cough or throat clearing. 1 2 For patients like these, it is crucial to recognize when the cause of their cough or throat clearing seems to be, but is not mucus; rather, the cause is a sensory neuropathic disorder. If a patient does not recognize this, he or she can get sidetracked, continuing to focus on and manage the mucus without ever improving the cough or throat clearing.

Circumstance #1 In More Detail: When Mucus Is Clearly “Felt” but the Mucus Is Not Actually There
This patient may interpret a completely convincing sensation of mucus (dripping or suddenly filling the throat) as being actual mucus, when in fact it isn’t. Consider, for example, the following composite anecdote.

A middle-aged woman had been coughing for more than 10 years. There were so many episodes of coughing each day, and some so violent and protracted, that it was seriously diminishing her quality of life. Co-workers were annoyed; family members were distressed. The several particularly bad episodes of coughing each day were brutal: she would vomit or nearly vomit due to the violence of her attacks. Sometimes she also experienced leaking of urine.

During her visit with me, she explained that the typical initiator of the coughing attacks was a sensation of mucus dripping down the back of her throat. Every conceivable treatment for this “dripping mucus,” applied across the years, had made no difference. Later, during that same initial evaluation, when a videoendoscope had been inserted and was recording a view inside her throat and voicebox, she suddenly began to exclaim, “There! It’s dripping! It’s dripping! It is going to make me cough!”

I asked her where it was dripping. Struggling not to cough, she told me urgently and animatedly that it was in her throat, and pointed to the upper-middle neck. All this time, the videoendoscope was still in position, recording video. She continued to insist that there was mucus actively “dripping” there, and after 20 or 30 seconds of valiantly struggling to resist, she began a severe episode of coughing. At no time was there any mucus visible. There was none within the throat or voicebox or upper windpipe, and none in the back of the nose.

I then reviewed the video-recorded examination with her, second by second, leading up to and including the initial 30 seconds of her attack, to show that
in fact there was no mucus, but instead only the sensation of mucus. I went on to explain the cause for this sensation: a neuropathy. A neuropathy (that is, damaged nerves) usually causes pain, but for individuals with sensory neuropathic cough or throat clearing, the neuropathy instead causes an abrupt false sensation of varying types – the sensation of a “feather,” “sandpaper,” “pinprick,” a “burning sensation,” a “bubble,” or, as with this patient, “dripping mucus.”

For most of the sensations described above, it is easy to grasp that there is not actually a feather, sandpaper, a pin, or a fire inside the person’s throat. However, since mucus is a normal inhabitant of the throat and chest, it escapes most people – doctors as well as patients – to realize that there may be a subset of people with SNC who experience “mucus” without there being any actual, or any sufficiently explanatory, mucus. Thus, the patient gets treated as if he or she did have “problem” mucus, of course with no benefit.

Circumstance #2: When Mucus Is Only the Result of Coughing
The second coughing scenario is one in which mucus is indeed produced and can even be spit out as proof (“foamy white” doesn’t count – that is
saliva), yet this mucus is not produced within the first seconds of coughing, but only near the end of a severe episode of coughing. In such cases, the coughing often ends soon after the mucus is produced. Below is an example of this scenario.

“Doctor,” says the patient, “can you give me something to get the mucus up faster, because once that comes up, my coughing stops.” This patient has already been tried on a long list of “mucus” medications, with no benefit.

“Does the mucus ever come up immediately at the beginning of a coughing attack?” I ask.

“No,” the patient says, “it is after I have been coughing for 20 seconds or a minute.”

“And do you turn red?” I ask. “And do your eyes tear, and does your nose run during this time?”

“Yes,” the patient says. “And I wet myself, and retch, and nearly throw up.”

“Well,” I suggest, “might it be that if your eyes are running and your nose is running, your lungs are ‘running’, too?”

For this group of sensory neuropathic cough patients, mucus production is the result rather than the cause of the coughing. Incidentally, I once sat and made myself cough extremely hard for 30 seconds. I didn’t need to cough, but just forced myself. I produced a surprising amount of phlegm.

Don’t Get Sidetracked
If your diagnosis is sensory neuropathic cough or throat clearing, and if your own experience or other doctors have strongly suggested that mucus is the
culprit behind your coughing or throat clearing, and if numerous “mucus” treatments have failed to provide any benefit, you may be ready to try out the
idea above: that although your sensation of mucus is real (circumstance #1) or the mucus produced by your coughing is real (circumstance #2), it can be the case that in neither case is mucus the actual cause of your coughing. The cause is instead a neuropathic disorder, and it is that neuropathic disorder – not mucus – that we will target with the treatment we prescribe.

References
  1. Bastian RW, Vaidya AM, Delsupehe KG. Sensory neuropathic cough: a common and treatable cause of chronic cough. Otolaryngol Head and Neck Surg. 2006; 135(1): 17-21.[]
  2. Bastian ZJ, Bastian RW. The use of neuralgia medications to treat sensory neuropathic cough: our experience in a retrospective cohort of thirty-two patients. PeerJ. 2015; 3:e816.[]


Vocal Underdoer Syndrome: When Voice Rest or Restraint Only Hurts

By Robert W. Bastian, MD

For a voice clinician, it is helpful not only to place each medical disorder in its own unique diagnostic “basket,” but also to search for organizing concepts that might show what certain disorders have in common with each other.

For example, vocal nodules, vocal polyps, and vocal fold hemorrhage are three distinct diagnoses that could be found in three different people. Yet these “different” diagnoses can be understood and addressed better when placed together under a common banner: the vocal overdoer syndrome. 1 That is, since each of these kinds of vocal fold injuries (along with other injuries like capillary ectasia and epidermoid cyst) arises from excessive voice use 2 , it is most helpful, when dealing with such an injury, to view the vocal overdoer syndrome as (almost always, but not exclusively) the primary diagnosis and the resultant injury as the secondary diagnosis.

Although it is now widely understood that many types of vocal fold injuries are caused by excessive voice use, it remains less clear to many voice clinicians that disorders can also occur because of vocal underuse. A person might underuse one’s voice for a variety of reasons: a personality that limits voice use; a false conclusion one has jumped to about his or her voice, triggering inappropriate, self-imposed voice rest; well-intentioned but misguided medical advice to conserve the voice; or a combination of these. All of these scenarios, when they lead to a voice problem, fall under the banner of what we call the vocal underdoer syndrome.

Background: vocal underdoers versus vocal overdoers
If we use a 7-point scale to rate a person’s talkativeness, vocal underdoers are usually 1’s and 2’s, whereas overdoers are 6’s and 7’s. If we use another 7-point scale to rate a person’s loudness, underdoers again tend to land lower on that scale (that is, they are more soft-spoken) as compared with overdoers. Typically, overdoers rate themselves accurately on the talkativeness and loudness scales, but underdoers are sometimes less self-aware, describing themselves as more talkative or louder than what their family or friends report.

In addition, even though overdoers are using the vocal fold muscles more vigorously than underdoers, it is paradoxically the vocal underdoers who much more often complain of general voice fatigue. To be sure, overdoers are the ones who may develop mucosal swelling and thus will report more mucosal “fatigue” symptoms (see swelling checks)—for example, a slight huskiness in the voice that leads them to say, “My voice is tired.” But they don’t typically describe the muscle-based fatigue symptoms that bring underdoers in for consultation: a choking sensation, anterior neck awareness or heaviness, a sense of effort, and so forth.

One reason that vocal underdoers experience more fatigue of the larynx and neck muscles may be that their muscles, being under-conditioned, are more easily fatigued. It is as though the professional ballet dancer whose back gets a vigorous daily workout rarely feels back strain, whereas the sedentary attorney who sits at a desk all day needs the chiropractor and massage. In addition, for some vocal underdoers, another cause of fatigue may be their tendency to produce voice with simultaneous over-contraction of antagonistic muscles—driving with the brakes on, as it were.

Because the problem of vocal underuse is not as well recognized by some voice clinicians as compared with the problem of vocal overuse, vocal underdoers can suffer through their voice problems much longer without getting a proper diagnosis and treatment. Below are the stories of four different vocal underdoers, each with his or her own reasons for underusing the voice. Each person came to harm while he or she went undiagnosed.

Vocal underdoer #1: Underuse by nature, abetted by medical advice
Near the beginning of my career, I saw an older, recently retired man who had lived for many years with what he experienced as a weak and inadequate voice. He explained that, early in his career, he had taught chemistry at the college level. He had begun to notice that his voice mechanism and throat in general seemed tired and almost achy at the end of the four to six single-hour lectures he gave each week. He said he seemed to become hoarse and run out of voice (with rapid recovery after brief voice rest) and that he eventually saw an ENT doctor about this.

Since this man’s problem was clearly experienced in the context of sustained projected voice use, and since recovery was associated with rest, he was advised to conserve voice whenever possible, in order to be ready for the next lecture. But the problem only became worse. He saw a second ENT doctor and received similar advice, so he redoubled his efforts to “protect” his voice. The problem continued, and so a third doctor told him it was clear that since the main problem occurred during lectures, he needed to give up teaching. He did this—regretfully, he told me—and spent his remaining working life as a research chemist.

His wife explained that this voice problem had interfered with much more than his work. “For the past 30 years, I don’t think we’ve ever made it through more than 20 minutes of a loud social event,” she said. “For example, after that span of time at a party, he will come over to me with a look of panic and say that his voice just can’t handle the situation, and we end up leaving early. Another example is that we can never stay and visit in the narthex after church; he has to flee situations like that.”

When I asked him to rate his innate degree of talkativeness/sociability on a 7-point scale, he and his wife together chose “2,” denoting a person who is—by nature—quiet and introverted. Still, by my assessment, his speaking voice sounded normal, and his range capability was as expected for his age. During “swelling checks” elicitation, there was no suggestion of mucosal abnormality.

When I modeled a loud voice—“Hey!”—in order to elicit the same production from this man, he initially demurred. “My voice won’t do that,” he said. I reassured, coaxed, and finally insisted that he try. He appeared anxious and even glanced towards his wife for help. Finally, he made what registered to me as guarded and half-hearted attempts. It was as if he could—or would—only turn up the volume to 2x, in response to my demonstration of 5x. Laryngeal examination was normal, other than mild bowing.

It appeared to me that there was nothing fundamentally wrong with the voice, and that this patient suffered from an exaggerated sense of vocal “vincibility” or vulnerability—almost like a mild phobia. It seemed that, based upon his own vocal discomforts, and abetted by the well-intentioned but misguided suggestions of his doctors, this man had come to believe deeply that his voice was weak and fragile and that he had to treat it with extraordinary gentleness. But all along, this man’s real problem (as proven below) was his wrong view of his voice and consequent minimal voice use.

This man was prescribed an initial “marathon session” of therapy with a voice-savvy speech pathologist. I suggested as much as two hours for the first session, during which he would be asked to project his voice nearly continuously, with appropriate coaching and modeling. He would be taught during this time to produce loud voice correctly, too. I advised him that I thought only a few additional sessions would complete the project.

This idea visibly frightened him. He wanted to know if I would be nearby during this therapy session, in case there was a problem. I assured him that, if necessary, I’d be glad to examine his larynx every 20 minutes with video equipment that would allow him to see for himself. (It never came to that.)

Within a few sessions, this man was almost miraculously let out of his 30-year vocal self-confinement. His wife told me that he had experienced an actual personality change and had become talkative even with strangers!

Vocal underdoer #2: Jumping to a wrong conclusion
I saw a young baritone who was pursuing a voice performance major at a nearby university. Early in the consultation, he told me with an undercurrent of desperation that his voice had been ruined a few months earlier, when he had undergone a tonsillectomy with endotracheal intubation. He said that the surgeon, knowing the patient was a serious singer, suggested a month should elapse before resuming singing, and he, the patient, had decided that if a month was good, two months might be better yet for his instrument, and that he would avoid not only singing, but also talking.

The problem that he explained in the consultation was that, in his words, “I have a different voice than I used to have.” He further stipulated that his new voice had no strength or depth or “body.” He didn’t remember any unusual early postoperative hoarseness, partly because during that time he had written messages instead of speaking. He had used his voice minimally and always gently during the remainder of the two months, and when he resumed using his voice two months after the procedure, he immediately came to believe that the breathing tube must have damaged his vocal folds.

At the time I saw him, he was continuing to rest his voice, but checking it briefly every day for recovery. Now, as he was about to begin the final month of the fall semester and without any apparent progress, he was in panic mode.

On the 7-point scale for innate talkativeness, this man chose “5.” Just as with the man in case 1, I was interested that this young singer’s speaking voice sounded normal—average man-on-the street for his age. He was only slightly tentative when asked to project voice. I did not notice any hypernasality, nor was there any leakage of air when he pretended to blow up a stiff balloon. (Rarely, tonsil surgery can unmask or create a palate deficiency such that it doesn’t fully seal off the nasopharynx during speech. 3 The result is a hypernasal resonance quality.) Swelling checks for mucosal injury were normal up into a fairly clear and high falsetto, and laryngeal examination, including stroboscopy, was also normal.

My diagnosis was that this voice was actually normal: there had been no injury to his vocal folds nor to his resonators at the time of the tonsillectomy. My alternate diagnosis was that this young man had developed a firm belief in injury, when in fact the problem was vocal deconditioning below the threshold needed for good singing. He was initially dubious. Therefore, with an eye to his skepticism, I asked him to adopt a regimen, with a timer as the arbiter.

He was to begin having voice practice sessions twice a day, on day one for five minutes per session, and then each day adding another five minutes to each practice session, so that by the end of a week he would be singing 30 minutes twice a day. And so forth. Within a few weeks, the problem vanished.

Vocal underdoer #3: Panic and extended voice rest from red herring findings
I saw a late-career, classically trained soprano who was quietly distraught. She announced that she had nodules and that her career was on hold waiting for them to resolve. She mentioned that singing was her livelihood, her sole means of support; she was now in financial distress because of the problem.

It all began three months earlier, when she had seen an otolaryngologist due to some allergy symptoms of sneezing, runny nose, and itchy eyes. In the course of a complete ear, nose, and throat examination, he had told her that he saw vocal nodules. She had panicked, gone into strict voice rest, and begun canceling performances around the country so that she could resolve her nodules. She was seeing me because, three months later, her voice was not improving at all, and in fact seemed to have gotten worse as a result of her voice rest.

I asked her to place herself on the 7-point innate talkativeness scale. Without a second’s thought and with a smile and a shrug, she chose “7.” (This is often the talkativeness profile of successful performers.) I then asked if the weeks or months leading up to the allergy symptom consultation had been particularly demanding vocally. She said no. I asked her if the reason for the visit three months earlier included any worries about her voice. She said that her voice had seemed fine—it was primarily her nose and eye symptoms that had caused her to make that appointment.

In the course of my evaluation of her voice, I noted that her swelling checks were very impaired, confirming mucosal injury. She began to struggle with onset delays as low as A4, a full octave below her necessary public singing range. Laryngeal examination confirmed small, pointed spicule-like vocal nodules.

Even though the soprano’s voice had been working at its normal baseline when her vocal nodules were first noticed, she had become convinced that she needed to rest her voice until those nodules went away. I was convinced that this well-intentioned voice rest had caused her voice to deteriorate. I believed that she had been singing with nodules at a professional level for years, and that rest had actually made her less able to coexist with or conceal these “baseline” nodules.

After all, I reasoned, she had been a “7” all her life. More importantly, there had been no sense of vocal indisposition or increased voice use prior to the time of her nodule diagnosis. I explained that she was like a premier ice skater with a few ounces of lead in her skates. She had indeed been laboring with an impediment, but quite successfully, and she could likely return to doing the same going forward, as these were probably stubborn, chronic nodules that might not resolve without the help of surgery. I predicted that, as long as she stayed in very good shape by vocalizing regularly, she could likely again “skate circles around others.” If I was wrong, or if she became unwilling to adapt to the “lead in the skates,” I explained, there would be an excellent surgical option to restore her voice.

As with case 2, I advised her to gradually build up her voice. She departed mostly unconvinced, but promised to “give it a try.” After the first week, she reported by telephone, “Well, it is definitely better, but I can’t return to performing with this voice.” We established together that “50%” described her voice at our first consultation, and now, she felt she had progressed to 75% of her baseline voice. After another week, however, it was up to 85%, and within three or four weeks, she stopped canceling performances and returned to her work.

Her nodule problem remained, causing detectable impairment of the swelling checks for the several additional years that I followed her, but her excellent technique and careful choice of repertoire allowed her to continue performing successfully for many more years.

Vocal underdoer #4: Inaccurate self-perception and loudness set-point
A thirty-something woman visited me because of irritated comments she was getting from co-workers and others. They were complaining, she said, that they could never hear her. The patient seemed perplexed and troubled by their frustration. During the entire encounter, she spoke like this, or less. Halfway through our lengthy conversation I remember feeling a little irritated at the effort needed to understand her, just as one might feel if forced to read a book chapter in extra-fine print. To the present, this person remains the most soft-spoken person I have ever met whose voice mechanism was normal.

During my evaluation, I did not notice any roughness or hoarseness. There was no air-wasting quality. When I elicited (by first demonstrating myself) loud voice, she repeated her “baseline” soft-spoken voice. That is, she responded still with this voice. Or perhaps at most this voice. I continued to request, and coax, and finally to insist that she produce this voice. But without continual cues and encouragement from me to keep this up, she reverted to this voice. And when I asked her what she thought of the normal-loudness voice she had produced briefly at my insistence, she said in all sincerity, “I could never talk like that—that’s yelling.”

This quiet woman manifested an inappropriate set-point for vocal loudness. Although others would be incredulous to hear her say it, she truly believed that her vocal loudness was normal, rather than recognizing, as everyone else did, that she was remarkably soft-spoken.

Here, I pointed out the concept of a “loudness set-point” to explain why people speak at differing loudness levels, each of which seems normal to them. I explained that just as someone else might go through life “yelling” as perceived by ears of everyone but themselves, she was going through life practically whispering in everyone’s view but her own.

We established that she would explain to family and friends the 7-point loudness scale and that she would give them permission to do “sound checks” with her. That is, they were permitted to hold up one finger, or three, or whatever the case might be, in order to indicate how loud her voice seemed to them in that moment, in comparison with the rest of the human race. In addition, I suggested that she occasionally solicit a “sound check.” For example, she might decide in advance to adopt a “5” loudness level at a dinner out with friends, and then mid-meal, request a “sound check” to see if her perception matched theirs.

Using these means, her assignment was to figuratively graft other people’s auditory perceptions into her own, in order to first understand and accurately perceive—and then possibly to realign—her own innate “set-point.” She would come to understand and measure for herself when she was talking like this, and when like this. She would no longer feel perplexed at how others experienced her voice.

Understanding vocal underdoers
What is the common thread in all these stories? In each case, the person’s voice problem did not arise primarily from a disorder or injury of the voice-making apparatus (weak, deconditioned muscles and nodules notwithstanding), but instead from a personality or a conviction, supported in some cases by misguided external advice, which caused that person to inappropriately rest or restrain the voice.

It is important to recognize that, like the soprano in case 3, an individual can have a lifelong classic vocal overdoer profile, and yet abruptly, due to some new life circumstance or belief, become instead a vocal underdoer. Also, as illustrated by her story, one can have an actual vocal disorder (nodules, in her case) that is a red herring alongside the main issue: quailing from voice use and disbelieving in the larynx’s capabilities. Others are innately vocal underdoers, like the man in case 1 and the young woman in case 4.

These vocal underdoer cases illustrate that voice rest is not always the answer, that rest can actually become a hindrance to having or re-gaining a normal voice. Just as vocal overdoers need to learn the skills of voice care and conservation in order to avoid injury to mucosa, and also to perform “swelling checks” regularly, vocal underdoers often need to get in touch with the “big voice” that they might never use across an entire lifetime due to personality, and to understand that the voice, like any other body part, can benefit from and “enjoy” a degree of vigorous use. Vocal overdoers need to learn vocal underdoer skills, and vocal underdoers need to learn vocal overdoer skills.

References
  1. Bastian RW. The vocal overdoer syndrome: a useful concept from the voice clinic. Journal of Singing. 2002; 58(5): 411-13.[]
  2. Johns MM. Update on the etiology, diagnosis, and treatment of vocal fold nodules, polyps, and cysts. Curr Opin Otolaryngol Head Neck Surg. 2003; 11(6):456-61.[]
  3. Haapanen ML, Ignatius J, Rihkanen H, Ertama L. Velopharyngeal insufficiency following palatine tonsillectomy. Eur Arch Otorhinolaryngol. 1994; 251(3):186-9.[]


Medical Jadedness and Treatment of Sensory Neuropathic Cough

By Robert W. Bastian, MD

People with sensory neuropathic cough often spend years searching in vain for a correct diagnosis and effective treatment. In a group of 110 consecutive patients diagnosed with sensory neuropathic cough by the author in 2005, the median duration of cough prior to diagnosis was 10 years, ranging from a few weeks to 62 years.

Such individuals will first visit their own primary care physician, and then an allergist, pulmonologist, gastroenterologist, and otolaryngologist. In each place, he or she may submit to a long list of tests, such as chest x-ray, various scans of the chest or sinuses, pulmonary function tests, allergy tests, bronchoscopy, esophagoscopy, 24-hour pH probe, and so forth. These tests are done to evaluate some “usual suspects,” for chronic cough—in particular, allergy, acid reflux, and cough-variant asthma.

Add to all of this that an individual with sensory neuropathic cough may have tried several to dozens of medications, including antihistamines, asthma inhalers, acid suppressors, antibiotics, cough suppressants, expectorants, and steroids—even when testing for the conditions for which those medications are used was negative. This leaves medicine chests cluttered with bottles of drugs that did not work.

The record for most grueling diagnosis search in my experience was an out-of-state patient who had been coughing for 16 years—she had seen more than 20 different physicians, undergone countless tests, and had tried 40 different medications. Her cough was the same or worse than at the beginning. After all of this time, effort, and money had been expended, with zero benefit, such a patient would understandably and reasonably be skeptical and even medically jaded, perhaps to the point of not actually complying (either overtly or covertly) with a new suggested treatment regimen.

On the other hand, the physician has not participated in the patient’s prior travails and instead may be focused on his or her previous treatment success with neuralgia medications. If the physician does not grasp the depth of the patient’s prior disappointments, he or she may not recognize when the patient is by appearances cordial and respectful, but is actually in great doubt about this “new” diagnosis.

Patient 1: Medically jaded but cordial
Several years ago, a middle-aged woman many years into an unsuccessful search for a correct diagnosis for her chronic cough came for a consultation, having heard of our work. Her history was classic for the syndrome of sensory neuropathic cough. 1 I educated her extensively and also had her watch a comprehensive teaching video we use to reinforce the nature of the diagnosis and explain the strategy for testing one or more neuralgia medications. She left the office with a prescription for amitriptyline and what we call our “roadmap,” detailing in a third format the strategy, voicemail instructions, and the six things we want to know when patients phone the office to give a status report and request further instructions.

Several weeks went by, and we didn’t hear from her. I believe we tried to contact her several months later, but our voicemail did not elicit a return call. Two years later, she reappeared on my schedule. After exchanging initial greetings, I said, “I notice that we have no information about your response to the first medication you tried, amitriptyline. Did your cough go away?”

No,” she said, “The amitriptyline did nothing, and I stopped it. The cough has been as bad as ever these past two years, and I’m ready to try your second medicine.”

“Why,” I asked, “didn’t you try it two years ago?”

She said, “Well doctor, I had already seen so many doctors, and tried so many things that when your first medicine didn’t work… I just blew you off.” She still seemed to me war-weary and uninspired about my diagnosis. Happily, the second medication, gabapentin, worked extremely well for her. I was struck to think that she had coughed aggressively, almost to the point of debilitation, needlessly, for two years.

Patient 2: Hostile and even contemptuous
Out of the flow of hundreds of patients across many years, one encounter stands out vividly. I entered the examination room to meet a new patient, a nurse in her 40s. The instant I entered the room, I sensed remarkably tangible hostility. I remember thinking for a moment that I must be two hours late, though I knew I was actually running nearly on time. Through the frost, I reviewed the story of her classic but as-yet undiagnosed sensory neuropathic cough. I spent more time than usual with her, trying to find common ground and thaw the ice. I spent more time that was necessary for my benefit on the details of prior evaluations, tests, and unsuccessful treatments.

Well into the consultation and after examination, I introduced her to the diagnosis. Sensing her skepticism, I explained with analogy after analogy the concept of sensory neuropathy causing not pain, but instead, the tickling sensation that initiated each of her coughing attacks. I suggested beginning with amitriptyline. She accepted the prescription and departed. I felt drained, thinking the entire encounter was a failure and that it would likely be my last with her.

The encounter had been so unpleasant that I remembered the name when it appeared on my schedule two weeks later. I strapped on a figurative suit of armor and consciously decided that I would be cordial but not work as hard this time around. I entered the room with my guard up. To my surprise, the atmosphere in the room was dramatically different. It was as though the sun was shining; the ice was melted. The patient’s face was strikingly transformed.

Even so, I sat down tentatively. “You were just here two weeks ago,” I said. “Is there something else we need to discuss?”

“Dr. Bastian,” she said, smiling, “I am not here for any medical reason. I made this appointment purely to apologize to you.” She went on, “You have to understand that when I saw you, I had been everywhere and done everything. I had gone to major national centers. Then I came here and you gave me this new diagnosis and I said to myself, ‘Right, another bright idea that will go nowhere.’”

She went on to say that her response to amitriptyline hadn’t yet abolished her cough, but it had been reduced to an amazing degree. She was simply thrilled.

The physician must bear the brunt
It is completely understandable for patients with sensory neuropathic cough to become cynical and feel that the medical profession has failed them. We can know that collectively the many prior physicians have worked very hard within existing paradigms, and we can also explain to ourselves and to the patient that physicians cannot diagnose what they do not know about. Nevertheless, it is up to us to compensate for the patients’ medical jadedness.

Here are a few ways that we as physicians can help the patient:

  1. Acknowledge the patient’s prior experience. I can imagine that you are exhausted, and skeptical by now of each new idea. But I want you to bear with me while I explain something you may not have heard of before.
  2. Stress that you want the patient to join you in a process that may take some weeks or months to complete. I really want to start you on a path that I believe can bring you some considerable relief. You might see some benefit from the first medicine, and within the first few days. But many who eventually get wonderful benefit don’t see that benefit until they try medication 2 or 3. Trying two or three different medications in sequence can take some weeks, and a lot of patience. Please do not give up. We sometimes say around here that you can’t give up unless I give up (and I never give up).
  3. Show good faith by your generosity of effort. It can take some back and forth communication between you and me to get to the best medication for you. We have prepared a sheet to help you do your part. We have listed voicemail instructions and also the six things we want to know from you, every time you call the office. We do it this way to spare you trips back and forth to the office.
  4. Set up a careful protocol with office staff to get messages back and forth, and prescriptions filled and refilled expeditiously. This conveys to patients the importance with which you take their dreadful problem.

The rewards are great
Patients with sensory neuropathic cough do not die. But, especially if the disorder is severe, their lives can be practically ruined. Until recently sensory neuropathic cough has been an orphan disorder, and patients with it, orphans in the medical community. Although it can be labor-intensive to work through the process of finding best treatment, going through numerous calls back and forth between patient and physician, with office staff as the hard-working go-between—working with this group of patients is extraordinarily gratifying.

References:

Bastian ZJ, Bastian RW. (2015) The use of neuralgia medications to treat sensory neuropathic cough: our experience in a retrospective cohort of thirty-two patients. PeerJ 3:e816 https://dx.doi.org/10.7717/peerj.816

 

References
  1. Bastian RW, Vaidya AM, Delsupehe KG. Sensory neuropathic cough: a common and treatable cause of chronic cough. Otolaryngol Head and Neck Surg. 2006; 135(1): 17-21.[]