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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
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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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