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Biomedical subjects

Frank E Musiek

Publications and source records attributed to Frank E Musiek.

10 recordsLinked to original sources
PubMed2004

Deficient auditory interhemispheric transfer in patients with PAX6 mutations.

PAX6 mutations are associated with absence/hypoplasia of the anterior commissure and reduction in the callosal area in humans. Both of these structures contain auditory interhemispheric fibers. The aim of this study was to characterize central auditory function in patients with a PAX6 mutation. We conducted central auditory tests (dichotic speech, pattern, and gaps in noise tests) on eight subjects with a PAX6 mutation and eight age- and sex-matched controls. Brain magnetic resonance imaging showed absent/hypoplastic anterior commissure in six and a hypoplastic corpus callosum in three PAX6 subjects. The control group gave normal central auditory tests results. All the PAX6 subjects gave abnormal results in at least two tests that require interhemispheric transfer, and all but one gave normal results in a test not requiring interhemispheric transfer. The left ear scores in the dichotic speech tests was significantly lower in the PAX6 than in the control group. These results are consistent with deficient auditory interhemispheric transfer in patients with a PAX6 mutation, which may be attributable to structural and/or functional abnormalities of the anterior commisure and corpus callosum, although the exact contribution of these two formations to our findings remains unclear. Our unique findings broaden the possible functions of PAX6 to include neurodevelopmental roles in higher order auditory processing.

Adult↗
PubMed2004

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.

Audiometry, Pure-Tone↗
PubMed2004

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.

Adult↗
PubMed2004

Central deafness associated with a midbrain lesion.

Central deafness has been linked historically to bihemispheric involvement of the temporal lobe, with more recent findings suggesting that compromise of other cortical and subcortical structures can also result in this disorder. The present investigation extends our understanding of the potential anatomical correlates to central deafness by demonstrating that bilateral involvement of an auditory structure within the midbrain can additionally result in this condition. Our subject was a 21-year-old male with a subarachnoid bleed affecting both inferior colliculi. Significant auditory deficits were noted for the middle and late auditory evoked potentials, while electrophysiologic measures of the periphery indicated normal function. The patient was enrolled in a rehabilitation program for approximately 14 weeks. Although initially unresponsive to sounds, the patient regained most of his auditory abilities during the 10 months he was followed. This case documents the range of auditory deficits that may be associated with damage to the inferior colliculi, and it profiles a hierarchical recovery of auditory function consistent with test findings.

Adult↗
PubMed2004

An unusual case of X-linked adrenoleukodystrophy with auditory processing difficulties as the first and sole clinical manifestation.

X-linked adrenoleukodystrophy (X-ALD) is characterized by demyelination that is associated with a deficient beta-oxidation of very long chain fatty acids. We report the unusual case of a male adult with X-ALD who was diagnosed at the age of 26 by a brain MRI performed because his brother had been diagnosed with a rapidly deteriorating form of X-ALD. His sole symptom was hearing difficulties in the presence of a normal audiogram since childhood. He has remained stable for seven years. Central auditory testing in our patient revealed severe deficits in several auditory processes. These findings correlated with involvement of the auditory pathway at the level of the trapezoid body, and posterior corpus callosum in particular, on his brain MRI. This case highlights not only the need for thorough audiological investigation of the patient who complains of hearing difficulties in the presence of a normal audiogram, but also that audiological investigations could be of value in the phenotypic evaluation of cases with adrenoleukodystrophy.

Acoustic Impedance Tests↗
PubMed2004

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.

Adult↗
PubMed2003

The insula (Island of Reil) and its role in auditory processing. Literature review.

The insular cortex is a complex structure which contains areas that subserve visceral sensory, motor, vestibular, and somatosensory functions. The role of the insular cortex in auditory processing was poorly understood until recently. However, recent case studies indicate that bilateral damage to the insulae may result in total auditory agnosia. Functional imaging studies demonstrate that the insulae participate in several key auditory processes, such as allocating auditory attention and tuning in to novel auditory stimuli, temporal processing, phonological processing and visual-auditory integration. These studies do not clarify the issue of further specialisation within the insular cortex, e.g. whether the posterior insulae are primarily sensory areas, while the anterior insulae serve mainly as integration/association auditory areas, two hypotheses that would be compatible with the cytoarchitectonic structure and connectivity of the insulae. The functional characterisation of the insulae remains incomplete, underlining the need for further studies.

Animals↗
PubMed2002

Interaural asymmetries revealed by dichotic listening tests in normal and dyslexic children.

Normal and dyslexic right-handed children were assessed with three dichotic listening tests, the Dichotic Digits test, the Competing Words subtest of the SCAN, and the Dichotic Consonant-Vowel test. Performance was measured as both number and percentage of correct responses in the right and left ears. Laterality was defined as a simple difference in percentage between the two ears. Differences across the tests were revealed for all children, with the greatest differences occurring for left-ear responses. Only one dichotic listening test, Competing Words from the SCAN, produced a consistent right-ear advantage across all of the children tested. Between groups of children, differences in performance and in laterality were demonstrated. Using a criterion of poorer than 76 percent correct for the left ear, the Competing Words subtest of the SCAN identified 7 of the 10 dyslexic children as abnormal, with no false alarms in the control group.

Auditory Perceptual Disorders↗