Tullio's Phenomenon

Timothy C. Hain, MD,  

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Content last updated: 4/2002

Types of Fistulae

 

Definition

The "Tullio phenomenon; consists of dizziness induced by sound. For example, use of one's own voice or a musical instrument. It occurs mainly in four ear conditions: perilymph fistula, Meniere's syndrome, post fenestration surgery, and vestibulofibrosis. Meniere's syndrome is the most common because of its relatively high prevalence (about 2/1000). Perilymph fistula, and especially the superior canal dehiscence syndrome, is probably the second most common cause. Vestibulofibrosis, or the attachment of a motion sensitive part of the ear to the stapes, is probably the third most common. There are only a very few people who have had fenestration surgery for otosclerosis. Accurate figures regarding the prevalence of fistula and vestibulofibrosis are presently not available. Tullio's is probably more common in persons who wear hearing aids, although this has never been studied.

Tullio's often is accompanied by hearing loss, tinnitus, and pressure sensitivity. The latter symptom is rare, but arises from similar mechanisms and disease processes as does sound sensitivity. Sensitivity to Valsalva is another type of pressure sensitivity.

History:

There are probably several mechnisms for Tullio's phenomenon as several types of disease processes have been associated with Tullios.

Tullio (1929) originally investigated sound-induced eye and head movement in pigeons. He made openings in the semicircular canals and demonstrated that sound waves spread primarily into the canals that had been opened. Huizinga (1935) proposed that the openings allow sound energy to move the ampulla of the semicircular canal, and furthermore, that the net effect is greater in the direction that excites the vestibular nerve. This is similar to Ewald's second law. Rottach and associates, implicated stimulation of the horizontal canal (1996).

Nadol suggested that the stapes foot plate may form fibrous adhesions to the utricle spontaneously or as a post infectious process, terming this process "vestibulofibrosis". Nadol also suggested that pathologic dilation of the otolithic sac, as in Ménière's disease, might cause it to become more sensitive to movement of the stapes. Finally, collapse of perilymphatic membranes, called , "vestibular atelectasis" or "floating labyrinth", might allow it to become a force conduit between the stapes and the utricle.

With respect to mechanisms involving abnormal stapes movement, Dieterich (1989) suggested that a hypermobile stapes with annular ligament damage or subluxation of the stapes may cause abnormal utricular contact. Combining both ideas, Kwee (1976) postulated that a congenital middle ear bone malformations may predispose the utricle to abnormal fluid movement generated by an irregularly shaped stapes.

Fenestration surgery, or in other words, the making of openings in canals, was used for Otosclerosis during the 1940's, before the invention of the stapes operation for otosclerosis. There are still many patients who have fenestrations. Nearly all of these patients have Tullio's.

Superior canal dehiscence, a thinning or absence of the roof over the superior semicircular canal has recently been described. Approximately 2% of the population has SCD on autopsy. SCD is associated with movements in the plane of the superior canal on stimulation. SCD is also associated with low-threshold sound evoked vestibulocollic reflexes (Brantberg et al, 1999; Watson et al, 2000).

Diagnosis

This is basically a symptom, not a disease, so you diagnose it by simply making the observation that loud noises make you dizzy. We don't mean that loud noses make you uncomfortable -- chalk screeching on the blackboard and all that, but literally dizzy as in spinning vertigo. It is particularly important to try to determine which ear is the problem. The next step should be to consult with a doctor who knows something about this. Generally this will be an otolaryngologist who specializes in ear problems (an otologist). This doctor will likely do a hearing test, take a careful history, and get a temporal bone CT scan to look for superior canal dehiscence and other ear problems.

Ben-David et al (1997) reported that MVP could be used to diagnose Tullio. This seems worth pursuing.

Treatment

Treatment is based on the cause. Generally it is not terribly effective. Fistulae can be patched, Meniere's can be treated medically and surgically. Fenestrations may be closed, although this is done very rarely. Vestibulofibrosis can be treated with approaches that destroy vestibular function, disconnecting the ear from the brain. More practically, you may find it useful to wear ear plugs, and avoid loud noises.

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