Eleven years ago, a distraught father drove his 16-year-old daughter down from Canada to my tinnitus and hyperacusis clinic in Oregon for help. The girl complained of an extreme reaction to everyday sounds, such as the eating or breathing noises of other people; she had dropped out of high school and refused to sit at the family dining table. She had suffered the sudden onset of these symptoms at age 13, and the number of "triggers" was increasing and creating a seriously limited lifestyle. Her reactions to annoying sounds were immediate and severe: emotional outbursts, rage, a sense of a strong need to flee the scene, and uncontrollable anger. This was upsetting to a previously happy family life--thus the drive down to Oregon.

All physical evaluations had revealed perfect health, including perfect hearing. There was no history of psychological or emotional pathology and, according to her home-schooling tutor, the girl was making straight As. She did not have any reduction in her loudness discomfort levels (LDLs) in the booth; as a matter of fact, the auditory system was working perfectly, as far as I could tell. There was no tinnitus or hyperacusis.

This was the first case of selective sound sensitivity syndrome (4S) that I had encountered. Sinc  e that time, 11 long years ago, thousands of similar cases have presented to my clinic with relatively identical case histories and etiologies. In the vast majority of cases, the children or youth are often referred by their primary care physicians to an ear specialist and an audiologist for evaluation. Audiologists do need to be aware of the syndrome and its symptoms, which are characteristic and demonstrate a similar profile in most cases.

4S appears most of the time in children between the ages of 8 and 13. It generally has a sudden onset point and the subjects are able to describe the event with great detail and precision. Most often the first negative encounter with these softer sounds is with a primary family member--usually mom or dad, but sometimes it is a sibling that presents the problem first. These "trigger" people are important in the syndrome, as the child rapidly forms strong negative associations with the activities and events that create the potential for the "trigger" to emerge, e.g., preparing for dinner at home. Over time, these triggers tend to expand and include other people or other situations.

There appears to be a genetic influence in 4S due to the high number of subjects who report a relative with similar issues related to sound and negative associations. A typical reaction of the family to the subject is to insist that the reaction stop because "no one else is bothered by dad's chewing sounds, so just knock it off." Over time, the child realizes that this is a unique situation and that others do not or cannot share these reactions; a sense of shame develops about the problem. This constricts the flow of open conversation in most cases, and the child begins to adopt avoidance behaviors that circumvent exposure to the noxious sounds, e.g., not hungry at dinnertime, but later wants to eat.

Figure 1 shows the results of a recent survey conducted by my clinic with 100 self-selected participants from an Internet support group. To my knowledge, this survey provides the first data collection of any type for 4S.

To date, the vast majority of the subjects have been prescribed mood- or brain-chemistry-altering drugs. Out of 100 subjects in the survey, 99 had tried one or more medications for their 4S. It appears that these medications have little or no effect on auditory system problems with soft everyday sounds. Similar results have been found in the dozens of patients who have directly consulted my clinic for help over the past decade.

The 16-year-old patient from Canada provided a clue for a possible therapy, as she remarked the one place she enjoyed at school was the large noisy cafeteria. As a matter of fact, that was the only place in the school that was comfortable for her. Since "cafeteria chatter" is a standard background noise stimulus used by audiologists, I considered how that sound is actually a blend of noise, tones and pitches that creates a band of steady sound. It was a small leap to see that tinnitus retraining therapy (TRT) devices, with their broad band stimuli output, might be of use.

Using Jastreboff's neurophysiological model of tinnitus, I applied the use of bilateral sound generators to assist in significantly increasing the ambient sound levels of patients.^{1, 2} Using an open-ear model, the broad band signal generators stimulate much of the cochlea and appear to improve tolerance in a large number of subjects. The units engage the auditory system and lessen the amount of auditory contrast between a quiet background noise floor and the offending trigger sounds.

 Children have been able to wear their nearly invisible devices into classrooms and at home and resume many of their former activities. While the use of these sound devices does not appear to result in long term recovery or diminishment of the symptoms of 4S, it eases the severity of the reactions and allows the wearer to enjoy more of daily life. Providing a trial period makes sense, as it allows for a test-run time to assess the real value of the units.

The formal identification and definition of 4S has yet to begin. An online support group currently has about 800 members who have formed several networks of international information and education sites and have issued with appeals to the National Institutes of Health (NIH) for help in studying the syndrome. 4S problems do not appear to dissipate with adulthood; rather, people with 4S tend to structure their entire lives around accommodating their noise sensitivity issues in work and social settings.

In the tinnitus and hyperacusis clinic, of course, the need for adjuvant therapies is very important, and a strong network of like-minded medical providers has slowly coalesced to include neuro-psychologists, neuro-psychiatrists, neurologists, and otologists. It is important to work within a framework of specialists in these cases to be sure that every angle is considered and recognized.

Recently I was giving a talk about tinnitus at a seminar for audiologists; there were a few moments for a free topic and I introduced the subject of 4S to the group. I had hardly spent 5 minutes describing the information I have gathered when a young audiologist to my right side raised her hand. "I have that!" she said. And, like the vast majority of the subjects I encounter every day, she was pleased and relieved to discover she has thousands of "cousins" with the same problems and concerns and that her problem even has a name!

References

1. Jastreboff, PJ, Hazell, JWP. (1993). A neurophysiological approach to tinnitus: clinical implications. British Journal of Audiology, 27:7-17.
2. Jastreboff, PJ, Gray, WC, Gold, SL. (1996). Neurophysiological approach to tinnitus patients. American Journal of Otology, 17:236-240.

Marsha Johnson, AuD, opened the Oregon Tinnitus & Hyperacusis Treatment Clinic in the summer of 1997 to devote herself full-time to the evaluation, diagnosis and treatment of these auditory disorders. For more information: www.hyperacusis.org, www.tinnitus-audiology.com, Oregon7@aol.com.