Autosomal dominant compelling helio ophthalmic outburst syndrome

Synonyms

4

Overview

It is a genetic autosomal dominant trait that causes variably difficult to control sneezing in response to numerous stimuli, such as looking at bright lights or periocular (surrounding the eyeball) injection. The condition affects 18-35% of the population, but its exact mechanism of action is not well understood.

Symptoms

The photic sneeze reflex manifests itself in the form of uncontrollable sneezing in response to a stimulus which would not produce a sneeze in people without the trait. The sneezes generally occur in bursts of 1 to 10 sneezes, followed by a refractory period that can be as long as 24 hours.

Photic sneezing:
A photic sneeze is the most common manifestation of the photic sneeze reflex. A photic sneeze is a sneeze that results from exposure to a bright light. The photic sneeze reflex seems to be caused by a change in light intensity rather than due to a specific wavelength of light. Photic sneezes are harmless annoyances for the most part, but can pose physical risk to people who are operating vehicles or machinery that involve precise movement and reactions.

A study conducted by the School of Optometry at the University of Alabama at Birmingham, found that females represent 67% of photic sneezers, and Caucasians represent 94%. The study also found statistically significant correlations between photic sneezing and the presence of a deviated nasal septum. The study also showed that photic sneezing is more likely to be acquired than inherited.

Response to periocular injection:
During surgeries in and around the eye, such as corneal transplant surgery, the patient often requires injection of a local anesthetic into the eye. In patients who show the photic sneeze reflex, an injection into the eye, such as that undergone in a retrobulbar or peribulbar block, can often elicit a sneeze from the patient. During these procedures, the patient may be sedated prior to the periocular injection. The patient begins to sneeze just as the needle is inserted into the eye, often resulting in the anesthesiologist having to remove the needle before injecting the local anesthetic in order to avoid damaging the patient's eye.

Sneezing after eating:
A condition called gustatory rhinitis can cause some individuals to sneeze after eating, particularly after the consumption of spicy foods. Stomach fullness is another example of a stimulus that can cause uncontrollable sneezing. Those who exhibit this symptom or disorder, called snatiation, undergo uncontrollable fits of 3–15 sneezes immediately after eating large meals that completely fill the stomach, regardless of the type of food eaten. Snatiation is not believed to be an allergic reaction of any kind. Even less well-understood than photic sneezing and sneezing in response to periocular injection, the trait appears to be inherited in an autosomal dominant fashion.

Treatment

While this phenomenon is poorly understood, recent research has shown that antihistamines being used to treat rhinitis due to seasonal allergies may also reduce the occurrence of photic sneezes in people affected by both conditions. Those affected by ACHOO may find relief by shielding their eyes and/or faces with hats, scarves, and sunglasses. There are many remedial fixes for sneezing, such as placing a finger horizontally below the nose or holding the nose closed when the beginnings of a sneeze are felt.

Another remedy is to deliberately cause the onset of sneezing in a safe environment before moving into an environment where the condition could be a danger. The person will then be protected as long as the refractory period lasts.

The most helpful way to avoid the risks stated above is to be aware of any inclination to sneeze in response to strange stimuli. If a pilot knows he or she is at risk for experiencing a photic sneeze during a flight, he or she can wear polarized goggles to block out the sun, or at the very least be prepared for a sneeze and have measures planned to minimize the risk from such a sneeze. Any patient with a history of uncontrollable sneezing who requires periocular surgery should tell the doctor or anesthesiologist, so that they can take appropriate measures to minimize the risk of injury in case of a sneeze during the surgical procedure. People who know they have a tendency to experience sneezing fits after consuming a large meal can make an effort to reduce the size of their meals, since snatiation seems to occur only as a result of an extremely full stomach.

Resources

• NIH