1/27. 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.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/27. Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions.Auditory neuropathy is defined as absent or severely distorted auditory brainstem responses with preserved otoacoustic emissions and cochlear microphonics. This entity can be found in various circumstances including pre-lingual children. An almost universal characteristic reported from adult patients is the ineffectiveness of traditional hearing aids. Adequate management of pre-lingual cases therefore remains an open problem. This paper describes two pre-lingual children whose follow-up data demonstrated a selective loss of the otoacoustic emissions, whereas the cochlear microphonics remained preserved. In one of the patients, hearing aid fitting as soon as she lost her otoacoustic emissions proved successful. These findings have important implications for the operational definition of the condition, since one must be prepared to encounter cases with absent otoacoustic emissions. The present data also demonstrate that conventional amplification can benefit pre-lingual auditory neuropathy cases, at least once they have lost their otoacoustic emissions.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
3/27. Auditory neuropathy and a mitochondrial disorder in a child: case study.A child was referred for an audiologic evaluation, to include auditory brainstem evoked response testing, due to inconsistent responses to sound and delayed speech and language development. Results were characteristic of auditory neuropathy. In view of subsequent decline in motor function, a genetics evaluation was conducted, revealing a mitochondrial disorder. A brief overview of mitochondrial genetics in association with hearing loss is presented. The patient's audiologic profile is described and the implications for management are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
4/27. language development in a child with left hemispherectomy.A longitudinal study of a left hemispherectomized boy (AB) was conducted to document linguistic evolution and maturation and determine the extent to which right hemisphere processes allow development of language. Resection of the left hemisphere occurred at age 5 years 6 months, following intractable epilepsy. Tests of language comprehension (pointing, understanding of prepositions, understanding of narratives) and production (naming, repetition, lexical diversity, grammatical production) were administered at ages 6:2, 6:4, 6:6, and 6 years 9 months. Observations showed little progress, if any, in most aspects of linguistic performance. In contrast to studies with left-hemispherectomized children, AB showed only a modest expansion of the semantic lexicon and the phonological repertoire more than a year after the surgical intervention. These observations indirectly suggest (1) poor functional involvement of the right hemisphere in the development of adequate linguistic abilities, (2) the necessary integrity of the LH for adequate development of language, or (3) that variations in individual brain maturation rates may account for AB's linguistic progress.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
5/27. Central auditory processing disorder as the presenting manifestation of subtle brain pathology.The objective of this case-report study was to assess the presence of central auditory impairment in a patient with a normal neurological examination. This subject was a 45-year-old female with gradually deteriorating hearing difficulties over a period of 5 years and a borderline normal audiogram. Behavioural central auditory tests were used, including Dichotic Sentence Identification Test, Competing Sentences Test, and auditory event-related potentials (mismatch negativity). Behavioural central auditory tests and mismatch negativity results were abnormal and indicated disordered central auditory processing. Subsequent magnetic resonance imaging of the brain identified subtle changes consistent with small-vessel ischaemic disease. adult patients who present with hearing difficulties that cannot be explained on the basis of their audiogram should undergo central auditory assessment, as the auditory symptoms may be the first and only manifestation of central nervous system pathology.- - - - - - - - - - ranking = 1.6666666666667keywords = brain (Clic here for more details about this article) |
6/27. Worster-Drought and congenital perisylvian syndromes-a continuum?A 5-year-old female was evaluated because of severe speech and expressive language delay. On examination, she could hardly speak and communicated through gestures. She manifested severe dysarthria and difficulty in protruding and moving her tongue laterally. She lacked coordination of the swallowing process, with drooling and an increased mental reflex. Her cognitive development was normal, and no associated neurologic dysfunction of the limbs was noted. On follow-up, the child experienced two episodes of seizures at 6 years of age. magnetic resonance imaging of the brain demonstrated perisylvian and frontal polymicrogyria. Clinical and radiologic findings demonstrated a similarity and continuum between congenital suprabulbar paresis (Worster-Drought syndrome) and perisylvian syndrome.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
7/27. Influence of otitis media on hearing and development.otitis media affects nearly every child at some time; many children have mild-to-moderate hearing losses for prolonged periods. The effects of these losses on language and educational development may be significant; possible mechanisms are discussed. Especially in suboptimal listening situations, speech perception may be impaired by even a mild hearing loss. Patterns of imperception are predicted by comparison of composite audiometric data from children with middle ear effusions with speech power data, and by analysis of sound pressure waveforms of speech filtered to simulate the typical hearing loss of these patients. A new method of analysis of brain stem evoked responses, yielding response components attributable to binaural interaction, is reported. This and other evoked response techniques may be able to identify objective changes in auditory nervous system function attributable to relative sensory deprivation during development. Finally, directions for further research in this area are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
8/27. association of Chiari I malformation, mental retardation, speech delay, and epilepsy: a specific disorder?OBJECTIVE: The Chiari I malformation is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Although Chiari I malformation is considered to derive from a mesodermal disorder resulting in underdevelopment of the posterior fossa relative to its content, evidence for a possible heterogeneous etiology also has been reported. The aim of the present study is to elucidate the relationship between Chiari I malformation and mental retardation, speech delay, and epilepsy to consider a possible specific pathogenetic background. methods: Thirty-five patients with Chiari I malformations were identified by use of magnetic resonance imaging during a period between 1993 and 1999. The study consisted of nine patients (four boys and five girls) who were affected by mental retardation, speech delay, and epilepsy. All patients underwent electroencephalography and brain and cervical spine magnetic resonance imaging. RESULTS: All patients were mentally retarded with a mean intelligence quotient of 50. Seven patients had a positive history for speech delay, and five were epileptic. Electroencephalograms demonstrated abnormalities in seven patients. The mean tonsillar displacement was 10.1 mm. A thin corpus callosum and a wide cavum septum pellucidum were present in three patients. Neither hydromyelia nor scoliosis was observed. No correlation between the degree of the ectopia and clinical manifestation was noted. CONCLUSION: The association of Chiari I malformation with epilepsy, speech delay, and mental retardation may not be a mere incidental finding but may be a marker for a different pathogenetic background.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
9/27. Language dysfunction in epileptic conditions.epilepsy may disrupt brain functions necessary for language development by its associated intellectual disabilities or directly as a consequence of the seizure disorder. Additionally, in recent years, there has been increasing recognition of the association of epileptiform electroencephalogram (EEG) abnormalities with language disorders and autism spectrum disorders. Any process that impairs language function has long-term consequences for academic, social, and occupational adjustments in children and adolescents with epilepsy. Furthermore, impairments in specific language abilities can impact memory and learning abilities. This article reviews interictal language function in children and adults with epilepsy; epilepsy surgery and language outcome; and language disorders associated with abnormal EEGs. The relationship between epilepsy and language function is complicated as the neuroanatomic circuits common to both overlap. We demonstrate how magnetoencephalography (MEG) offers the ability to analyze the relationship of language, EEG abnormalities, and epilepsy.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
10/27. A fluent language disorder following antepartum left-hemisphere brain injury.This article describes the development of language in a left-handed girl with a left middle cerebral artery infarction. Seven language samples of parent-child interaction, obtained when she was between 36 and 60 months of age, were transcribed and analyzed using the child language Data Exchange System. At 36 months of age, only 42 (20%) of the child's 214 utterances contained words; the other 80% were composed of jargon or interactional markers such as "uh-huh" and "uhn-uhn." Jargon incorporated familiar intonational contours and prosodic features to convey emotional states and communicative functions. Between 36 and 45 months of age, her jargon became differentiated into increasing approximations of English sentences. Simultaneously, her use of words and word combinations increased. By 54 months of age, no jargon was heard. The pattern of development observed in this child can be described as a transient jargon or fluent aphasia. It may have resulted from initial reliance on an uninjured right hemisphere. However, given the similarity between this pattern and the expressive or gestalt style of learning seen in some normal children, the pattern may also be related to other variables including characteristics of the parental input.- - - - - - - - - - ranking = 1.3333333333333keywords = brain (Clic here for more details about this article) |
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