Research Areas
Brainstem and Inner Ear Interactions
The auditory nerve carries sound information from
the inner ear to centers in the brainstem,
which interact with the hair cells by
sending information back to them. For instance,
sound
played into one ear triggers a mechanism
which suppresses the oto-acoustic emission of the
other ear. In some unusual pathological
situations,
these emissions are not suppressed and
the person is unable to hear or understand sounds
when there is a noisy background. Kresge
Lab's
Charles
I. Berlin, PhD, and Linda
J. Hood, PhD were among the first to quantify
this
suppression and its characteristics of
time, frequency and intensity. Using a powerful
computer program [note: there appears
to be
a problem in running this program on
Gateway computers---to download click here: (em40.exe)]
developed at Kresge by Engineer Han Wen,
Berlin and Hood have been studying the
phenomenon of suppression and persons in who it
does
not occur. While this investigation is
still in its early stages, it has aroused world-wide
interest. It promises to help us understand
some feedback mechanisms of the nervous
system
auditory system in general and of the
auditory system in particular.
Deafness Genes
Remarkable progress has been made over the past
ten years in the identification of genes for hearing
loss, in large part because of new technologies
and resources that have become available as a result
of the human genome project. The complete nucleotide
sequences of the human genome, as well as model
organisms, are now in public databases. The mouse,
in particular, is an excellent model organism for
genetic hearing loss because many deaf inbred strains
are available for research. We now know that humans
have about 35,000 genes encoding the proteins that
are needed for the growth, development and functioning
of the organs and tissues of our bodies. Abnormalities
(mutations) in more than 100 genes are now known
to be associated with hearing loss.
Bronya Keats,
PhD, together with other Kresge scientists,
is working on the genetic causes of both syndromic
and non-syndromic forms of hearing loss. She
is particularly interested in the gene that causes
Usher syndrome type I in the Acadian population
of south Louisiana. One component of her work
is
creating a mouse that contains the human mutation
in this gene, in order to facilitate the development
of an effective therapy for this devastating
disorder. Another of her projects is focused on
identifying
the genetic causes of deafness in schools
for the Deaf.
Hair Cells
The process that turns sound waves into nerve impulses
occurs in the hair cells of the inner
ear, about which so much has been learned in recent
years.
There are new developments almost daily from the
Molecular Hearing Division. They are isolating individual,
living hair cells from the rest of the ear and
recording their responses to various stimuli. In
these studies, they are asking three questions
about hair cells:
What are the molecular mechanisms
that allow them to change sound into nerve
impulses?
How do drugs affect their functioning?
Do they play
a role in tinnitus - the peculiar type of
hearing loss in which one hears "ringing" or
other noises in the ear? The outer hair cells normally produce faint sounds,
called oto-acoustic emissions, which have been
the subject of much interest.
|