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372 Disorders of the Auditory System
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associated auditory processing difficulties
(see Chermak & Musiek, 2014, for an indepth review).
Case 7–11: CAPD Associated
with Learning Difficulties
History
This was a 13-year-old male who had a long
history of learning difficulties, especially
in the verbally related subjects in school.
He was having difficulty in school despite
investing many study hours a week. He
also struggled hearing in noise and following directions for many years (likely
for most of his school years) as noted by
teachers and his parents. After years of
considering a potential auditory processing deficit, he was referred for evaluation.
Audiology
This student demonstrated normal puretone thresholds and excellent speech recognition scores bilaterally. As can be seen
in Figure 7–11, he demonstrated a left ear
deficit on dichotic listening (digits, sentences, and staggered spondees) and a
mildly depressed score for the left ear on
filtered speech, while his performance on
the frequency patterns test (assessed in
the sound field) was normal.
Audiologic Management
Given that this was essentially a dichotic problem, the student was enrolled
in dichotic interaural intensity difference
(DIID) training (see Musiek, Weihing, &
Lau, 2008; Weihing & Musiek, 2007). This
auditory training involves dichotic listening with an intensity (or temporal) advantage provided to the poorer ear. After sev-
eral months of training, the left ear deficit
noted during his central auditory evaluation improved on each of the three dichotic speech tests that initially showed left
deficits (see Figure 7–11). Within the next
school year, both his parents and teachers
reported that his performance in school
had improved.
summaRy
This chapter highlighted selected disorders that can and do affect the CANS.
Mass lesions, vascular lesions, degenerative disorders, neurotoxic agents, head
trauma, temporal lobe epilepsy, and
learning difficulties are among the more
commonly encountered disorders that
can compromise the CANS. However,
the reader should be aware that many
other central nervous system disorders
not mentioned in this chapter can result
in central auditory deficits. Each of these
disorders could not be elaborated on here
due to the scope of this chapter. Among
the disorders not addressed, but deserving of comment, is central presbycusis.
Both the peripheral and central auditory
systems undergo normal aging processes.
In some people, there is more central
aging; in others, there is greater peripheral auditory system aging. Therefore,
whenever possible, it will be important
to assess both the peripheral and central
auditory systems when evaluating an
elderly patient. A common central deficit noted in many older individuals is a
left ear deficit on dichotic listening tests
that is secondary to compromise of the
corpus callosum due to normal aging
effects (Bellis & Wilber, 2001). Other central trends are not as obvious but should
be pursued clinically.

Figure 7–11. Pre- and
https://t.me/medicina_free
posttherapy (retest) central
behavioral test results
7–11).
obtained from a 13-year-old
male with a long history
of learning problems
(Case
373

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Hydrocephalus is another disorder
that should be mentioned. Third, fourth,
and lateral ventricle hydrocephalus can
easily compromise the CANS due the
close proximity of these ventricles to
central auditory nuclei and tracts (Musiek et al., 1994). Leukodystrophies are a
category of degenerative disorders often
inherited that can result in myelin damage. The auditory evoked potentials often
are abnormal in this category of disease
(De Meirleir, Taylor, & Logan, 1988). More
recently, Bamiou and colleagues (2007)
have shown auditory interhemispheric
processing difficulties in children with
congenital aniridia due to PAX6 genetic
mutations. Auditory deprivation from
long-standing peripheral hearing loss is
another condition that can result in central auditory deficits. Auditory hallucinations associated with schizophrenia
have garnered much interest in certain
audiology circles. This is likely happening because emerging data has shown
that some patients with schizophrenia
(i.e., those who experience auditory hallucinations) are demonstrating changes
in the auditory cortex (reduced volumes)
and decreased performance on central
auditory tests (see Musiek et al., 2007).
In the early 1980s, Morest (1983) profiled
research in animals with peripheral hearing deficits that demonstrated trans-synaptic degeneration of auditory neurons
and altered cortical organization due to
a lack of auditory stimulation in these
animals. This research has since been
well accepted and expanded upon and
has major implications across a variety
of disorders, consistent with the concept
of reduced neural connectivity that was
discussed earlier. The foregoing disorders
are some of the additional types of central nervous system compromise that can
affect the central auditory structures. As
we learn more about central mechanisms,
we undoubtedly will uncover other disorders that can compromise the CANS.
Acknowledgments. The authors grate-
fully acknowledge the contributions of
Erik Musiek, MD, PhD, Associate Professor, Department of Neurology, Washington University School of Medicine to this
chapter.
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