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1/26. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.

    A case of 'central deafness' is presented in a 3-year-old male Caucasian child with moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills. ( info)

2/26. Multidimensional approach to the differential diagnosis of central auditory processing disorders in children.

    Central auditory processing disorder (CAPD) may be viewed as a multidimensional entity with far-reaching communicative, educational, and psychosocial implications for which differential diagnosis not only is possible but also is essential to an understanding of its impact and to the development of efficacious, deficit-specific management plans. This paper begins with a description of some behavioral central auditory assessment tools in current clinical use. Four case studies illustrate the utility of these tools in clarifying the nature of auditory difficulties. Appropriate treatment options that flow logically from the diagnoses are given in each case. The heterogeneity of the population presenting with auditory processing problems, not unexpected based on this model, is made clear, as is the clinical utility of central auditory tests in the transdisciplinary assessment and management of children's language and learning difficulties. ( info)

3/26. A review of objective methods of evaluating auditory neural pathways.

    OBJECTIVES: review physiological methods of evaluating function of the auditory neural pathways in infants, children, and adults. Present two case studies to demonstrate the usefulness of physiological measures in assessing abnormalities of the auditory neural pathways. STUDY DESIGN: review of applications of physiological measures of auditory neural function. methods: review otoacoustic emissions, auditory evoked potentials, and efferent reflexes, and discuss their use in identifying disorders of the auditory neural pathways from the cochlea to the cortex. RESULTS: Auditory disorders occur from peripheral to central areas of the neural system. patients with disorders of the peripheral nerve and/or the input from the cochlear inner hair cells, such as the patients presently described as having auditory neuropathy, demonstrate abnormal efferent reflexes, including middle ear muscle reflexes and efferent suppression of otoacoustic emissions, as well as grossly abnormal brainstem evoked potentials. In contrast, patients with more central disorders can be differentiated by normal results on tests of the neural periphery and abnormal findings on cortical evoked potentials and other measures of central function. CONCLUSIONS: Physiological measures are sensitive, objective, and less variable in assessing neural disorders than traditional behavioral measures such as pure-tone or speech audiometry. ( info)

4/26. Electrical status epilepticus in slow wave sleep: prospective case study of a cortical hearing impairment.

    The development of a central hearing impairment is described in a young girl with risk factors for hearing impairment that included mosaic down syndrome, leukemia, and chemotherapy. This case is unusual in the prospective regularity with which hearing was assessed from birth. The diagnosis is electrical status epilepticus in slow wave sleep, a rare childhood disorder, which was associated with lack of responsiveness to auditory signals, regression of emerging speech and language and other cognitive skills, and abnormal electroencephalographic (EEG) activity in both hemispheres. Treatment of the disorder with anticonvulsant medications and steroids has ameliorated the condition by suppressing the abnormal EEG activity and allowing substantial improvements in cognitive and social skills, although communication skills are improving more slowly. ( info)

5/26. Cortical deafness to dissonance.

    Ordinary listeners, including infants, easily distinguish consonant from dissonant pitch combinations and consider the former more pleasant than the latter. The preference for consonance over dissonance was tested in a patient, I.R., who suffers from music perception and memory disorders as a result of bilateral lesions to the auditory cortex. In Experiment 1, I.R. was found to be unable to distinguish consonant from dissonant versions of musical excerpts taken from the classical repertoire by rating their pleasantness. I.R.'s indifference to dissonance was not due to a loss of all affective responses to music, however, since she rated the same excerpts as happy or sad, as normal controls do. In Experiment 2, I.R.'s lack of responsiveness to varying degrees of dissonance was replicated with chord sequences which had been used in a previous study using PET, in examining emotional responses to dissonance. A CT scan of I.R.'s brain was co-registered with the PET activation data from normal volunteers. Comparison of I.R.'s scan with the PET data revealed that the damaged areas overlapped with the regions identified to be involved in the perceptual analysis of the musical input, but not with the paralimbic regions involved in affective responses. Taken together, the findings suggest that dissonance may be computed bilaterally in the superior temporal gyri by specialized mechanisms prior to its emotional interpretation. ( info)

6/26. SPET monitoring of auditory cortex activation by electric stimulation in a patient with auditory brainstem implant.

    auditory cortex activation following multifrequency acoustic stimulation has been evaluated by means of single photon emission tomography (SPET) in one patient before and after an auditory brainstem implant (ABI). No activation could be observed after acoustic stimulation before ABI. After ABI stimulation in the coronal and axial slices, the activation within the temporal cortex contralateral to the stimulated ear was twice (43.76%) that of normal controls (23.94 /- 2.74%). This marked difference was not present in other selected cortical auditory areas (homolateral temporal, homolateral and contralateral parietal cortices). The temporal cortex was also examined with six consecutive sagittal slices from 18.75 mm up to 56.25 mm lateral to the midline. A very strong activation (51.20%) compared with that of normal controls (9.94 /- 7.45%) was detected in the 25.26-mm sagittal slice of the temporal cortex contralateral to the stimulated side. The remaining sagittal slices showed an almost normal post-stimulatory activation. As the 25.26-mm sagittal slice corresponds to the medial part of the auditory temporal cortex, its activation suggests that electrode stimulation is concentrated on the region of the cochlear nucleus in which the neurons that transduce high frequencies are located. SPET can be considered useful, in combination with electric auditory-evoked potentials, to obtain information on ABI placement and function, effectiveness of acoustic stimulation, degree of cortical stimulation and tonotopic spatial distribution of auditory cortex activation. ( info)

7/26. Central auditory deficits associated with compromise of the primary auditory cortex.

    The subject of this study was a 46-year-old female who had suffered a cerebrovascular accident (CVA). magnetic resonance imaging revealed damage in the area of the distribution of the middle cerebral artery involving most, if not all, of the primary auditory area of the left hemisphere. No auditory problems were noted prior to the CVA; however, following the CVA, the subject reported a number of auditory difficulties. Pure-tone thresholds were normal post-CVA, and performance on speech recognition testing was good in both ears if ample time was provided between a response and the presentation of the next test item. Duration pattern, intensity discrimination, and middle latency response test results were abnormal for both ears, and right ear deficits were evident on an auditory fusion test and two dichotic speech tests (digits and rhymes). This case is significant in that it demonstrates a good correlation between damage to known key auditory regions and central auditory test results. ( info)

8/26. Assessment and remediation of an auditory processing disorder associated with head trauma.

    This case study involves a 41-year-old female who had sustained a mild traumatic brain injury during a horseback riding accident. The patient was seen for medical and neuropsychological testing following this incident and was referred to a speech-language pathologist for rehabilitative services. At 13 months posttrauma, the patient, who was frustrated by a lack of significant progress, requested an audiologic work-up. Results of testing conducted at this time revealed normal peripheral hearing and significant central auditory deficits. Based on these findings, an auditory rehabilitation program was developed and implemented. The components of this patient's rehabilitation program are reviewed, and the posttherapy improvements noted in her auditory functions are detailed. The case is important in that it demonstrates (1) that auditory deficits can be a sequel to minor head injury, (2) that these deficits are often subtle and may not be detected unless central auditory testing is conducted, and (3) that these deficits may be amenable to remediation. ( info)

9/26. Audiological correlates to a rupture of a pontine arteriovenous malformation.

    This is a report of a female patient in her midthirties who sustained a hemorrhage secondary to an arteriovenous malformation in the region of the pons. The patient's initial symptoms included hearing loss and tinnitus, which were followed by the more characteristic symptoms of headache and loss of consciousness. Results of audiological testing at three months postaccident documented the presence of a hearing loss and a central auditory processing disorder, and the patient was provided an auditory rehabilitation program. Follow-up testing over the course of an additional year documented improvement in both pure-tone threshold and central test results; however, at 15 months postaccident, some auditory deficits remained, especially in the ear ipsilateral to the primary site of lesion. The anatomical correlates of these deficits are discussed, as are the potential contributions of both the auditory rehabilitation program and spontaneous recovery mechanisms to the documented improvements in auditory function. ( info)

10/26. Characteristics of electrically evoked potentials in patients with auditory neuropathy/auditory dys-synchrony.

    The primary goal of this study was to compare the characteristics of the post-implant evoked potentials with preimplant evoked potentials in patients with auditory neuropathy (AN) or dys-synchrony. AN is typically characterized by sensorineural hearing loss, reduced speech perception, abnormal temporal processing, and unusual patterns of results with various objective audiological tests. In some cases, these patients may be appropriate candidates for a cochlear implant. In this article, we highlight evoked potential findings in two children diagnosed with AN who were provided with multichannel cochlear implants. Preoperative, interoperative and postoperative evoked potential measures show that the restoration of neural synchrony may occur at multiple levels of the auditory pathways in patients with AN when appropriate diagnostic tests, cochlear implantation and rehabilitation are provided. ( info)
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