Hiccups and Laryngospasm

So one day I was having some bad hiccups... you know, the obnoxious kind complete with strangled noise and chest jerks.

However, this experience unlike the numerous prior episodes of hiccups triggered an intellectual brainstorm about breathing and stridor in general.

FACT:
Hiccups (aka singultus) are due to brief, intermittent, and involuntary diaphragm contractions against a closed glottis. For the layperson, this means during a hiccup:

• Your body takes a quick breath inwards (inhalation)
• Your body brings the vocal cords together
• Both above actions occur simultaneously, involuntarily, and briefly 

Which brought me to consider another disorder called laryngospasm which is the most severe form of vocal cord dysfunction. In this particular condition, the vocal cords come together involuntarily just as in hiccups... BUT, it may last for seconds to minutes while the breathing is still voluntary. At worst, it results in complete airway obstruction causing a loud high-pitched squeal called stridor (check out this movie below). Click here for more information about this condition.



FACT:
Laryngospasm is due to sustained and involuntary vocal cord closure WHILE the person voluntarily tries to breath. For the layperson, this means during a laryngospasm attack:

• Your body tries to breath voluntarily
• Your vocal cords involuntarily closes for a sustained period of time
• The above actions occur simultaneously

So... when you get down to it, the only difference between hiccups which is common and afflicts many people and laryngospasm which is rare is: 1) how long it lasts for and 2) whether the diaphragmatic movement is voluntary or involuntary.

Which got me thinking... is there actually a medical condition that is BOTH a hiccup and laryngospasm at the same time?

In fact, there is... though I must preface by saying there has been only one case report (that I'm aware of) of this super-rare condition called "Diaphragmatic Flutter with Stridor."

Reported back in 1995, the journal Chest reported a 13 years old girl who presented with rapid stridulous panting that occurred only during inspiration while awake (symptoms disappeared while asleep). Laryngoscopy showed an abnormal closed glottis. ECG noted noncardiac electrical activity due to involuntary diaphragmatic contractions occurring at a rate of 200/min. Fluoroscopy of her diaphragm revealed rapid myoclonic contractions of the left hemidiaphragm.

In essence, she was "hiccuping" 200 times per minute while having a sustained laryngospasm attack. The suffering this child and her family must have gone through must have been unbelievable and I'm sure tracheostomy must have been entertained at some point.

Treatment (cure) ultimately was achieved by crushing the patient's left phrenic nerve.

So let's take a look at all three conditions in table format:

	Diaphragm Contraction	Vocal Cord Closure	Timing
Hiccups	Involuntary	Involuntary	Brief
Laryngospasm	Voluntary	Involuntary	Sustained
Diaphragmatic Flutter	Both	Involuntary	Sustained

The common theme between all three disorders is the involuntary vocal cord closure resulting in airway obstruction. The variation in presentation is utterly dependent on whether the diaphragm contraction is involuntary or not and how long the "attack" lasts for.

References:
Diaphragmatic Flutter Presenting as Inspiratory Stridor. 10.1378/chest.107.3.872 CHEST March 1995 vol. 107 no. 3

Laryngospasm and Other Forms of Vocal Cord Dysfunction. Fauquier ENT.

Bursts of Fast Irregular Clicking Noise in the Ear

Every once in awhile, I see a patient who complains of an irregular clicking (or vibration) noise in the ear that occurs in bursts and may last anywhere from a few minutes every few days to as long as days or weeks at a time.

Click.... click... click, click, click... 

CLICK... click, click... 

click... Click Click... click... ... ... ... CLICK

These irregular clicking noises in the ear should NOT be confused with tinnitus. Rather, irregular clicking noises in the ear are almost always due to muscle spasms... just like "eye twitching" or "facial twitching". BUT, instead of being able to "see" the twitching as with eye twitching, one hears the twitching instead since the muscles are located around or in the ear.

1) Palatal Myoclonus
2) Tensor tympani muscle spasms
3) Stapedius muscle spasms

The first potential cause (palatal myoclonus) can be diagnosed by seeing "spasms" of the soft palate that occurs in time with the clicking. Spasms of the soft palate can be visualized by simply looking in the mouth or by nasal endoscopy. Click here to watch a video of these spasms.

The reason one is able to "hear" the twitching is because the muscles of the soft palate extend up into the ear via the eustachian tube.

Treatment of palatal myoclonus is by botox injections to the muscles that are twitching.

Just like botox treatment anywhere else in the body, the effects are only temporary and needs to be repeated every 3-6 months.

Regarding tensor tympani and stapedius muscle spasms... these muscle are not able to be visualized as they are located within the middle ear. The tensor tympani attaches to the malleus ossicle (the "hammer" of the 3 middle ear bones) and the stapedius muscle attaches to the stapes ossicle (the "stirrup").

Image taken from wikipedia.

Just like any muscle in the body, these muscles can also "twitch" rapidly causing the clicking noise in the ear.

Unfortunately, there is no "test" once can obtain to diagnose this problem with absolute certainty. It is based purely on history and excluding palatal myoclonus as a diagnosis (no soft palate twitching seen even though the patient is able to hear it).

Even more unfortunately, the only way this problem can be treated is surgical... the muscle gets cut. Botox can not be utilized as one needs to be able to "see" or "feel" the muscle in order to inject botox. This surgery is performed by a neuro-otologic surgeon.

See the full list of surgeons who can perform this surgery here.

Read more about this problem here.