CASE REPORT
D.L. is a 10-year-old left-handed male, born at 6 pounds, 3 ounces following a slightly premature (371/2 weeks) gestation delivered by caesarean section due to breech positioning. (Normal gestation is 38 to 40 weeks.) Mother experienced high blood pressure and edema during pregnancy. D.L. became ambulatory at 13 months; his speech commenced at age two, short sentences spoken at age three. Toilet training attained at three years, six months. Medical history is positive for febrile seizures, ear infections, and seasonal allergies. A maternal uncle and D.L’s father were also positive for febrile seizures, and the uncle’s visual-spatial style of learning was subsequently equated with D.L.’s style of learning. D.L’s early behavioral and developmental history seemed otherwise relatively normal to his parents. Academic issues began in kindergarten. He began experiencing difficulty with classroom activities that required language processing and verbal expression. Verbal directions often needed to be repeated when D.L. had difficulty with discrimination of letter sounds. At age 6 years, 1 month, a school psychologist administered a battery of intelligence tests, but these were inconclusive. Both his verbal (82) and performance (84) scores on the Wechsler Intelligence Scale for Children – Third Edition (WISC-III) were within the low normal range of cognitive functioning. Overall, he displayed average to low average performance in visual-spatial analysis, visual-motor integration, and visual processing speed. In fact, D.L.’s performance with tasks that required visual recall suggested no deficit and reflected no areas of relative delay. At that time there was no diagnosis, but D.L. was offered speech and language therapy, focusing on expressive language. At age 6 years, 9 months, D.L. was reassessed for purposes of evaluating progress. He was judged to be within normal limits (age appropriate) for skill areas such as oral peripheral (able to produce speech), articulation, phonological awareness, vocabulary development, expressive vocabulary, language development, and auditory processing. But he was well below normal in auditory word and number memory and interpretation of directions. Resourceful, D.L. often attempted to compensate by using meaning, structural, and visual cues to self-correct. At age 7 years 8 months, D.L. was given a central auditory processing evaluation. He was found to be easily distracted by background sounds, had difficulty recalling auditory information, and had difficulty learning through the auditory channel. He was beginning to have difficulty with reading comprehension and mathematics. In a filtered word subtest, he scored technically within the average range, but was on the verge of an auditory processing disorder. He would hear a simple word and it sounded slightly distorted or muffled. Problems on this test seemed to indicate an auditory processing disorder. A pitch pattern sequence test (PPST) proved more problematic. Instructed to listen to three tones that were in combinations of high and low pitches and then discriminate between high and low, he performed poorly, suggesting a compromise of the inter-hemispheric pathways or of the left hemisphere. Although D.L. possessed an excellent ability to understand speech in quiet listening conditions, in competing sound environments, he demonstrated a distinct right ear weakness. Specifically, he had difficulty selectively attending to speech in the presence of background noise and difficulty decoding auditory information coming from different locations. As a consequence of this evaluation, he was fitted with a “hearing aid,” more precisely, a FM Auditory Assistive Listening Device, to improve signal-to-noise ratio (teacher’s voice relative to the background noise) and improve D.L’s comprehension in the classroom. He was also enrolled in Earobics — an auditory enhancement program for slow listeners. It was strongly advised that a neuropsychological evaluation be performed to rule out a neurologically based language or learning disability. As a second grader, D.L. continued to have difficulty focusing and following oral directions, as well as difficulty with new concepts, in particular the language of mathematics. Despite his deficits, D.L. tested well within the normal range (42 percentile) on the Iowa Tests of Basic Skills as a second grader. At age eight years ten months, additional developmental tests revealed considerable strengths as a visual-spatial learner; for instance, he possessed very strong artistic abilities, especially in drawing and copying figures. He showed mixed dominance with left hand and right eye dominance. His gross motor function was above average, as shown by his ability to perform rapid alternating movements, hop in place, perform a sideways tandem gait, and catch a ball. As language tasks became more complex and abstract, however, D.L. had much more difficulty. Memory was another troublesome area. For instance, drawing from memory was difficult for D.L. and sequencing when drawing figures was erratic. While he was able to name the days of the week backwards, he proved unable to learn a series of seven words in four tries and could not learn a visual pattern in four tries. Reading comprehension was well below normal; he was observed to be a “very well-behaved boy who copes well with his substantial learning disabilities.” A physician’s comments in the evaluative summary finally observed that D.L. was afflicted with more than an auditory processing disorder. “D.L. has a much more complex processing problem, with overall processing issues of both visually and auditorially presented information.” This more ominous report became a prediction of diagnosis when D.L. underwent a more conclusive Brain MRI with contrast spectroscopy at age 10 years 2 months. What the magnetic resonance scan discovered was quite significant, but not entirely unexpected from a clinician’s perspective. Essentially, it revealed a large diffuse cortical malformation with associated heterotopic gray matter visible in the left hemisphere. A right subependymal nodular heterotopia and probable right frontal cortical malformation was also existent. He also was diagnosed with a variant circle of Willis vascular anatomy. The MRI detail confirmed a large area of heterotopic gray matter. In addition, focal nodular areas of similar intermediate signal intensity (positive for brain anomalies) were noted in the subependymal areas of the frontal horn of the right lateral ventricle, which was also diagnosed as likely gray matter heterotopias. The left caudate head and basal ganglia were malformed, appearing to be fused. An abnormal gyral pattern was observed in the right frontal lobe with a focal cortical malformation involving this lobe as well. The ventricles and CSF spaces were prominent, but remained within normal limits. What enabled D.L. to function at all was that his midline structures were otherwise normal with no evidence of mass or shift, and that his brainstem, cerebellum, and craniocervical junction were normal. Note was made of an azygos anterior cerebral artery arising from the right internal carotid artery, and that the left internal carotid artery terminated in the left middle cerebral artery as well. But no evidence of intraparenchymal hemorrhage or extra-axial fluid was identified. Finally, the MR spectroscopy demonstrated normal NAA, creatine, and choline ratios without evidence of abnormal metabolites.