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D
Figure 6–6. continued
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ear response was abnormal with the absence of a wave I; however, waves II through V were present at normal laten­cies and a normal III–V interwave inter­val was noted. No significant abnormali­ties were observed for either ear at the high repetition rate of 77.7 clicks/sec).
Medical Examination
The patient presented with an essentially normal otolaryngologic examination. Imaging revealed a very prominent ante­rior inferior cerebellar artery on the right side (Figure 6–6D).
Impression
Right-sided vascular loop syndrome.
Audiologic Recommendations and Management
It was recommended that the patient undergo aural rehabilitation to include intense training exercises to build skills in binaural integration and the process­ing of degraded speech. This was particu­larly important for this patient as she is employed as an operating room nurse. In addition, a mild gain hearing aid was recommended in order to assist with the auditory processing deficit. Although the patient considered both recommenda­tions, she opted not to proceed with any intervention at the time of her evaluation; however, she continues to be monitored annually.
Medical Recommendations and Management
benefits of surgery were discussed with the patient. The patient continues to be seen for otologic evaluations annually as she elected to forego surgery.
summaRy
Acoustic neuromas (vestibular schwanno­mas), auditory neuropathy (ANSD), and vascular loops (compression) are all disor­ders that can compromise auditory nerve function. Acoustic neuromas require early detection so they can be managed optimally. Early detection is dependent on interaction between audiologists and otologists. Audiologists also can be involved in monitoring and managing these patients after surgery. The main treatments, however, are surgery, radia­tion, and monitored observation. ANSD remains, at least from some perspec­tives, somewhat controversial. It affects newborns as well some older children and adults. It is defined by behavioral, electroacoustic, and electrophysiologic audiologic test results. Cochlear implants have provided significant help to many of the patients with this disorder. Vascular loops or vascular compression syndrome results from pressure on the eighth and/ or adjacent cranial nerves resulting from a misplaced blood vessel in the CPA. Hearing loss, hemifacial spasm, tinnitus, vestibular problems, and even facial pain can be symptoms. Although commonly diagnosed and treated, the nature and characterization of this disorder remain controversial.
The option of microvascular decompres­sion of the vascular loop away from the facial nerve and the vestibulocochlear nerve complex along with the risks and
Acknowledgments. The authors grate-
fully acknowledge Eric Smouha, MD, Neu­rotologogist, ENT and Allergy Associates, LLP, for his contributions to this chapter.
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Disorders of the Central
Auditory Nervous System
intRoduction
When considering disorders of the central auditory nervous system (CANS), it must be understood that it is not necessarily the type of disorder but rather the loca­tion of the lesion and the specific effects of the disorder on the CANS (i.e., the disease mechanisms) that are the crucial issues. Anatomically, the CANS begins at the level of caudal pons, specifically at the cochlear nucleus. At a similar level, but located deep in the pons, is the next major group of nuclei: the superior oli­vary complex (SOC). The SOC projects fibers along the lateral lemniscus, a major brainstem pathway that also has a group of nuclei in the upper half of the pons. The next nucleus in the CANS is the inferior colliculus. This nucleus is located in the midbrain and receives input from practi­cally all of the more caudally located audi­tory nuclei and projects to the underside of the thalamus to the medial geniculate body (MGB). The MGB sends fibers to the auditory cortex, specifically Heschl’s
gyrus and secondary auditory areas such as the insula. The corpus callosum con­nects the right and left hemispheres of the brain and has a specific auditory region where impulses are exchanged between the two hemispheres.
Many disorders can result in audio­logic deficits if there is insult to the audi­tory neural substrate within the CANS. However, the effects of CANS damage often result in audiologic findings that are not unique to a particular disorder but rather to the site of the CANS lesion. Some of the more significant disorders that can result in central auditory deficits are discussed in this chapter. Many of these disorders are often overlooked by health­care professionals due to the fact that other comorbid conditions (e.g., paralysis, vertigo) often overshadow the auditory symptoms. In some cases, these disorders may not result in hearing deficits, but in others, they certainly can and do. When they do, it is important to determine the nature and degree of the deficit(s) so that optimal medical and/or audiologic treat­ment can be realized.
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Audiology
The audiology sections in this chapter are organized in a slightly different manner than in previous chapters. This was done for efficiency. We discuss the audiologic profiles associated with various types of CANS disorders as has been done in other chapters; however, due to the similarity of the audiologic findings in mass and vascular lesions, rather than dedicating a separate section to the audiologic find­ings for each of these lesion sites, the dis­cussion is combined. Audiologic findings are provided separately for the following lesion types: degenerative disorders, neu­rotoxicity, traumatic brain injury, tempo­ral lobe epilepsy, surgical compromise of the CANS, and learning difficulties.
Anatomic Factors
In the assessment of various disorders of the CANS, it is useful to understand that various tests have anatomic limita­tions. Some central tests are efficient for the assessment of brainstem involvement, whereas others may be better suited for use with cortical or interhemispheric com­promise. Also, some disorders may mani­fest their dysfunction primarily in one of these three anatomic regions, whereas others may affect multiple areas. This, of course, influences the types of tests selected for administration during evalu­ation of the patient. For example, a small tumor of the low brainstem would have a focal effect in the pons. On the other hand, heavy metal neurotoxicity could involve the entire auditory system. Therefore, the selection of the tests to be administered to a particular patient will depend on the patient’s case history and presenting
symptomatology, as well as the results of any tests that are initially adminis­tered during the patient’s evaluation, as these may indicate that additional testing is needed to explore different (or poten­tially additional) auditory processes and/ or sites of lesion.
Types of Tests
Two main categories of central tests are discussed in this chapter. These include psychophysical (i.e., behavioral) tests and electrophysiologic tests. The psychophys­ical test category includes dichotic listen­ing, temporal processing, low redundancy speech, and binaural interaction tests, whereas the electrophysiologic procedures to be discussed include the auditory brain­stem response (ABR), the middle latency response (MLR), the N1 and P2 late poten­tials, and the P300 (also referred to as the P3 potential). On occasion, a brief discus­sion of other auditory evoked potentials also is included. Finally, there are some electroacoustic tests (e.g., otoacoustic emissions) that are often used to help dif­ferentiate a central site of lesion from a peripheral one. Although the otoacoustic emissions test does not assess the integ­rity of the CANS, it can be used to rule out a significant peripheral hearing loss.
In cases of CANS involvement, the goal of testing generally is not to make the diagnosis as in many instances, the dis­order is already known and many medi­cal procedures are better at defining the lesion than are the central auditory tests. However, there are three major goals of audiologic testing when CANS involve­ment is either suspected or confirmed. The first is as a screener. The audiologist may be the first professional to see a patient
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with a CANS disorder and therefore has the responsibility to make the appropri­ate referral for medical and/or psycho­logical/psychiatric follow-up. This, of course, cannot be done unless central dysfunction is determined. Although not a common occurrence, this situation does happen and when it does, appropriate follow-up and management of the patient is essential. The second major goal in uti­lizing central tests in CANS disease is to determine if the central auditory system is involved. For example, a patient with a long-standing diagnosis of multiple scle­rosis might seek audiologic assessment because of a new symptom of hearing dif­ficulty. The key here is to determine if this new symptom is really auditory in nature or not, and if it is, whether it is due to peripheral or central system compromise. The third goal of central auditory assess­ment is to corroborate the medical and communicative symptoms and/or com­plaints of the patient with test measures and to determine the degree of the patient’s functional deficit(s). All three of these goals lead to another important aspect of audiol­ogy; that is, the appropriate management of the patient. Without the appropriate diagnostic information, proper manage­ment of the patient with confirmed or sus­pected CANS involvement is difficult.
Brain Plasticity
Plasticity of the CANS is a factor in all brain lesions. Natural compensation by the brain for central dysfunction often occurs over time. Therefore, some lesion effects may not be as severe if the patient is assessed some time after the initial occurrence of the CANS disorder or insult as they would have been had the patient
been assessed closer in time to the original disease process or CANS compromise. On the other hand, some CANS lesions may progress, creating greater problems over time, as potentially would be the case in progressive CANS disorders such as mul­tiple sclerosis. Given these considerations, central auditory testing can be used not only to initially document the auditory deficits associated with CANS involve­ment, but also to monitor subsequent changes in the patient’s audiologic pro­file that may result as a function of brain plasticity, audiologic intervention, and/or disease progression.
mass lesions
Introduction
Mass lesions are space occupying lesions located within the brain. Lesions that are located within or close to the audi­tory areas of the brain, of course, are at risk for influencing central auditory func­tion. Sometimes, vascular lesions such as aneurysms and hematomas could be viewed as mass lesions in that they can be space occupying as they often are quite large. However, these types of lesions are classified as vascular disorders. In the brainstem, mass lesions are divided into intra-axial and extra-axial categories depending on whether the tumor resides primarily within (intra-axial) or outside (extra-axial) the brain tissue. The first reports on central auditory disorders by Bocca, Calearo, and Cassinari (1954) were on patients with temporal lobe tumors. In many cases, these patients reported vary­ing auditory symptoms, depending on the characteristics of their tumors.