Aversion, awareness, and attraction: investigating claims of hyperacusis in the Williams Syndrome phenotype

Dr. Levitin discuss some helpful observations on how people with Williams Syndrome hear.

The results of this study indicate that people with WS
present four unusual behaviors of auditory perception:
hyperacusis, odynacusis, auditory aversions,
and auditory fascinations. The hyperacusis might be
characterized as an early awareness of sounds that
are either too soft for others to hear or simply inconsequential
to others. Both odynacusis and auditory
allodynia create distress/discomfort in people with
WS, and together constitute aversion. Finally, we
documented that people with WS may show an intense
fascination for or attraction to certain classes of
sounds, often the same sounds of which they were
frightened at a younger age. Thus, the concepts of
aversion, awareness and attraction seem to characterize
the auditory anomalies observed in WS, and
may help to describe the WS phenotype, although we
note that reports of true hyperacusis were significantly
less commonthan of aversions and attractions.
The present study contributes significantly to a
clinical and conceptual understanding of auditory
abnormalities in WS. Because the genotypes in WS,
DNS and AUT are clearly defined, the study of these
distinct populations offers an unprecedented
opportunity to link genes, brain, and behavior. This
effort is greatly facilitated by the ability to document
such striking phenotypic differences as exist in
auditory function among the groups. Although the
precise mechanisms underlying the four auditory
anomalies in WS remain unknown, the present
paper documents the existence of abnormalities in
auditory perception which previous reports had
confounded. Ongoing work in our laboratories
focuses on the functional and structural neuroanatomical
components of these disorders, with the
goal of eventually understanding how genes, brain,
and cognition are linked. People with WS may provide
important clues to the neurogenetic basis of
cognition, perception, and complex behaviors.