Meniere's Disease - A New Theory And A Promising Vestibular Prosthesis

According to researchers at University of Colorado School of Medicine there is a strong association between Meniere's disease and conditions involving temporary low blood flow in the brain such as migraine headaches.

Foster explains that these attacks can be caused by a combination of two factors: 1) a malformation of the inner ear, endolymphatic hydrops (the inner ear dilated with fluid) and 2) risk factors for vascular disease in the brain, such as migraine, sleep apnea, smoking and atherosclerosis.

The researchers propose that a fluid buildup in part of the inner ear, which is strongly associated with Meniere's attacks, indicates the presence of a pressure-regulation problem that acts to cause mild, intermittent decreases of blood flow within the ear. When this is combined with vascular diseases that also lower blood flow to the brain and ear, sudden loss of blood flow similar to transient ischemic attacks (or mini strokes) in the brain can be generated in the inner ear sensory tissues. In young people who have hydrops without vascular disorders, no attacks occur because blood flow continues in spite of these fluctuations. However, in people with vascular diseases, these fluctuations are sufficient to rob the ear of blood flow and the nutrients the blood provides. When the tissues that sense hearing and motion are starved of blood, they stop sending signals to the brain, which sets off the vertigo, tinnitus and hearing loss in the disorder.

Restoration of blood flow does not resolve the problem. Scientists believe it triggers a damaging after-effect called the ischemia-reperfusion pathway in the excitable tissues of the ear that silences the ear for several hours, resulting in the prolonged severe vertigo and hearing loss that is characteristic of the disorder. Although most of the tissues recover, each spell results in small areas of damage that over time results in permanent loss of both hearing and balance function in the ear.

Since the first linkage of endolymphatic hydrops and Meniere's disease in 1938, a variety of mechanisms have been proposed to explain the attacks and the progressive deafness, but no answer has explained all aspects of the disorder, and no treatment based on these theories has proven capable of controlling the progression of the disease. This new theory, if proven, would provide many new avenues of treatment for this previously poorly-controlled disorder.

Many disorders of the inner hear which affect both hearing and balance can be hugely debilitating and are currently largely incurable. Cochlear implants have been used for many years to replace lost hearing resulting from inner ear damage. However, to date, there has not been an analogous treatment for balance disorders resulting from inner ear disease. One potential new treatment is an implantable vestibular prosthesis which would directly activate the vestibular nerve by electrical stimulation.

Phillips and his colleagues have developed a vestibular prosthesis which delivers electrical stimulation to the fluid inside the semi-circular canals of the ear. In effect, the stimulation of the fluid makes the brain believe that the body is moving or swaying in a certain direction. This then causes a compensatory postural reflex to stabilize the posture thereby helping to restore balance.

This prosthesis was inserted into the ears of four subjects all suffering from long-term Meniere's disease and differing degrees of hearing loss which was resistant to other management strategies. The researchers found that electrical stimulation of the fluid in the semicircular canals of the affected ear did result in a change in posture, the direction of which was dependent on which ear was stimulated. However, each subject had different sway responses to the stimulation given. The authors believe this could be caused by small differences in the location of the electrode between subjects. Thus fine tuning and individual calibration for each electrode implant would be required for it to be effective. Overall the results illustrate that this type of prosthesis may eventually be a possible treatment for balance issues caused by Meniere's disease.


References:

1. Carol Foster, MD and Robert Breeze, MD. The Meniere attack: An ischemia/reperfusion disorder of inner ear sensory tissues. Medical Hypotheses, December 2013
2. Phillips, C. et al. Postural responses to electrical stimulation of the vestibular end organs in human subjects. Experimental Brain Research, 2013 DOI:10.1007/s00221-013-3604-3

Role Of Multisensory Plasticity In Tinnitus

That area, called the dorsal cochlear nucleus, is the first station for signals arriving in the brain from the ear via the auditory nerve. But it's also a center where "multitasking" neurons integrate other sensory signals, such as touch, together with the hearing information.

In tinnitus, some of the input to the brain from the ear's cochlea is reduced, while signals from the somato-sensory nerves of the face and neck, related to touch, are excessively amplified.  

Susan Shore, Ph.D., the senior author of the paper, explains that her team has confirmed that a process called stimulus-timing dependent multisensory plasticity is altered in animals with tinnitus -- and that this plasticity is "exquisitely sensitive" to the timing of signals coming in to a key area of the brain.  It's as if the signals are compensating for the lost auditory input, but they overcompensate and end up making everything noisy," says Shore.

The new findings illuminate the relationship between tinnitus, hearing loss and sensory input and help explain why many tinnitus sufferers can change the volume and pitch of their tinnitus's sound by clenching their jaw, or moving their head and neck.  But it's not just the combination of loud noise and overactive somatosensory signals that are involved in tinnitus, its the precise timing of these signals in relation to one another that prompt the changes in the nervous system's plasticity mechanisms, which may lead to the symptoms known to tinnitus sufferers. 

In this study, only half of the animals receiving a noise-overexposure developed tinnitus. This is similarly the case with humans -- not everyone with hearing damage ends up with tinnitus. An important finding in the new paper is that animals that did not get tinnitus showed fewer changes in their multisensory plasticity than those with evidence of tinnitus. In other words, their neurons were not hyperactive.

Shore is now working with other students and postdoctoral fellows to develop a device that uses the new knowledge about the importance of signal timing to alleviate tinnitus. The device will combine sound and electrical stimulation of the face and neck in order to return to normal the neural activity in the auditory pathway.

"If we get the timing right, we believe we can decrease the firing rates of neurons at the tinnitus frequency, and target those with hyperactivity," says Shore. She and her colleagues are also working to develop pharmacological manipulations that could enhance stimulus timed plasticity by changing specific molecular targets.

Ref:

1. Seth D. Koehler And Susan E. Shore. Stimulus Timing-Dependent Plasticity in Dorsal Cochlear Nucleus Is Altered in Tinnitus. Journal of Neuroscience, December 2013