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Normalization of poor auditory brainstem response in infants and children.

In these past 10 years, the usefulness of auditory brainstem response (ABR) has been demonstrated in the clinical evaluation of audiological or neurological disorders, and the absence of an ABR is commonly accepted as consistent with a peripheral hearing loss caused by middle or inner ear diseases. In this article, the authors report nine infants and children who had an absent or difficult to detect ABR at the first examination with subsequent normalization of ABR. Repeated otological examinations were always normal in these patients. The final diagnoses were delayed development, Waardenburg syndrome with congenital heart disease and epilepsy, healthy infant, infantile convulsion, mental retardation with history of low birth weight, in each case, and chromosomal aberration in four patients. The reason for normalization of ABR in our patients is not clear and is yet to be ascertained. However, the authors believe that neurological maturation rather than an improved primary peripheral disorder is responsible for the noted normalization of the ABR, because of the high incidence of neurological abnormalities in these patients.

Audiometry, Evoked Response↗

The neuropsychology of trichotillomania.

The pathophysiology of trichotillomania (TM) is not well understood. Overlap with obsessive compulsive disorder (OCD) has been proposed, although extant data are inconsistent in this regard. In the neuropsychological domain, some data have supported the proposed TM-OCD overlap. However, the available studies are limited in number, and they typically have sampled a restricted range of performance domains. To examine further neuropsychological functioning in TM, the present study compared performance of 21 patients with TM and 17 normal control (NC) participants on a broad battery of tests assessing intellectual functioning, auditory perception and language, visual perception, somatosensory function, motor ability, memory, concept formation, attention and information processing speed, impulsivity, and cerebral dominance. The TM group demonstrated poorer performance on all measures of divided, but not focused, attention. Correlational data suggested the potentially important role of negative affect in TM. Implications of the data for the conceptualization of TM are discussed.

Adolescent↗

Cholinergic modulation of experience-dependent plasticity in human auditory cortex.

The factors that influence experience-dependent plasticity in the human brain are unknown. We used event-related functional magnetic resonance imaging (fMRI) and a pharmacological manipulation to measure cholinergic modulation of experience-dependent plasticity in human auditory cortex. In a differential aversive conditioning paradigm, subjects were presented with high (1600 Hz) and low tones (400 Hz), one of which was conditioned by pairing with an electrical shock. Prior to presentation, subjects were given either a placebo or an anticholinergic drug (0.4 mg iv scopolamine). Experience-dependent plasticity, expressed as a conditioning-specific enhanced BOLD response, was evident in auditory cortex in the placebo group, but not with scopolamine. This study provides in vivo evidence that experience-dependent plasticity, evident in hemodynamic changes in human auditory cortex, is modulated by acetylcholine.

Acetylcholine↗

Altered performance of schizophrenia patients in an auditory detection and discrimination task: exploring the 'self-monitoring' model of hallucination.

It has been suggested that perceptual hallucination results from a bias in attributing a self-generated event to an external source. The 'self-monitoring' model proposes that the impairment arises from a defective corollary discharge in perceptual decisions. However, psychophysical studies in schizophrenia patients have not found consistent support for the model. We re-explored this issue by including subjects with mood disorders as patient controls and employed signals of variable intensities in an auditory detection and discrimination task. Using signal detection theory, we found decreased sensitivity in auditory detection and discrimination in schizophrenia patients, as compared to control subjects. Moreover, the psychometric functions of schizophrenics had a shallower slope than those of the controls. According to the uncertainty theory of signal detection, this suggested that schizophrenia patients monitored a smaller number of perceptual channels. On the other hand, hallucinating and non-hallucinating schizophrenia patients did not differ from each other, nor did they as a group differ from control subjects in terms of response bias. Overall, our results do not support the idea that hallucination results from an altered bias in the online processing of perceptual signals.

Adult↗

Brain responses to number sequences with and without active task requirement.

OBJECTIVE: To examine the effect of the active task involvement versus the passive knowledge of the structure of the stimulation on event-related potentials (ERPs) to violation in number sequences. This should contribute to future development of an ERP test for neurological patients. METHODS: ERPs were recorded in subjects who listened to 4-item sequences of numbers incrementing by one. Half of the sequences had an appropriate end item, whereas the other half ended inappropriately. The role of active versus passive condition was investigated in a between-subject design. RESULTS: Incorrectly ending sequences yielded a large P300 wave in all groups. Subjects who were explicitly informed about the nature of the stimulation but did not have a motor task displayed a slow negativity which followed P300 and slightly suppressed it. In addition, incorrect sequence endings elicited an early (70-200 ms) negativity at frontal sites. This effect was inverted on mastoids. CONCLUSIONS: Auditory perception of 3 consecutive numbers constituting a simple incrementing row leads to building a strong expectancy concerning the subsequent number, and unexpected events are detected already after the initial phoneme.

Acoustic Stimulation↗

Interhemispheric transfer in high-functioning children and adolescents with autism spectrum disorders: a controlled pilot study.

Autism is a neurodevelopmental disorder with strong genetic influences. Clinical experience and limited empirical evidence support the view that autism may be associated with aberrant interhemispheric information transfer. This empirical controlled study examined whether, at neuropsychological testing, children with autism showed problems with interhemispheric information transfer. The study included auditory, visual, and motor measures covering information transfer within, as well as across, modalities. Thirty children (24 males, 6 females; mean age 12 years 8 months, SD 2 years 8 months; range 9 years 5 months to 17 years 5 months) without learning disability but with autism spectrum disorders were compared with 30 children from a mainstream school matched for age, sex, and IQ>75. Children with autism spectrum disorder performed significantly worse than the comparison group on most of the tests (p=0.02 for auditory perception and attention, p=0.005 for visual perception, p=0.0001 for motor control, p=0.04 for tactile perception). Results support the notion that aberrant interhemispheric transfer may be involved in the pathogenesis or clinical course of autism. The findings were not accounted for by lower IQ in the group with autism.

Adolescent↗

Neurodevelopmental pattern of succinic semialdehyde dehydrogenase deficiency (gamma-hydroxybutyric aciduria).

Succinic semialdehyde dehydrogenase (SSADH deficiency) (MIM 271980) is a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB) and causing neurological and cognitive disorders of varying severity. The non-specific nature and the difficulties in detection of urinary GHB explain why this disorder is largely underdiagnosed. Of 350 patients identified worldwide, to date only six adults with SSADH deficiency have been reported in the literature. Here we describe two additional cases in brothers up to ages 26 and 28 years. This retrospective report sheds light on the clinical features of SSADH deficiency in relation to the physiopathological involvement of GHB, and tries to identify the specific neurodevelopmental pattern of this learning disability.* Features of this are: early impaired psychomotor development with hypotonia and disturbances in motor coordination; impaired development of language, mainly due to poor auditory perception; and seizures and psychotic features in late adolescence or adulthood. Moreover, narcolepsy-like symptoms could be a consistent feature of the disease.

Adolescent↗

Auditory brainstem evoked potentials in sudden deafness.

Auditory Brainstem Evoked Potentials (ABEP) were recorded from 53 adult subjects suffering from sudden deafness at the time of examination. The onset of sudden deafness was up to three years prior to the study. ABEP were recorded in response to 75 dBHL clicks presented at rates of 10/sec and 40/sec. ABEP peak latencies as well as interpeak latency differences, and also the effect of increasing stimulus rate, were determined for each patient and compared with the clinical symptoms. The psychoacoustic and ABEP detection thresholds were also compared. A highly significant correlation was observed between ABEP detection threshold and the psychoacoustically determined hearing threshold, and the differences between them were found to be insignificant. The results of this study indicate a central component in sudden deafness. This impairment manifests itself in abnormal effects of increasing stimulus rate on ABEP, and may be related to reduced central synaptic efficacy.

Adult↗

Can self assessment of communication predict hearing loss?

A total of 120 subjects with hearing loss (75 men, 45 women), within the age range 18-70 years (mean, 38 years), and 15 normal subjects were administered a modified Hindi adaptation of the 'self assessment of communication' hearing loss inventory. The study aimed to determine whether there was any correlation between subjects' average pure tone thresholds and their inventory scores. Data was analysed using the Pearson coefficient of correlation and regression analysis. A negative correlation was obtained stating that the greater the hearing loss, the lower the inventory score. An equation could also be derived for the bilateral symmetrical sensorineural hearing loss group and the bilateral symmetrical conductive hearing loss group to enable calculation of patients' average hearing loss from their inventory scores, in the absence of an audiogram. This could aid rehabilitation in cases with either type of hearing loss (in which no medical intervention was required) when pure tone audiometry is not possible.

Adolescent↗

Auditory signal detectability and facilitation of simple reaction time in psychiatric patients and non-patients.

Patients diagnosed on the basis of structured interviews as having affective psychoses were less sensitive in detecting the presence of a transient auditory signal than were schizophrenic patients or non-patients. Patients with affective psychoses also benefited more (their reaction time was more reduced) than the other two groups from the presence of a second auditory transient.

Acoustic Stimulation↗

Mapping the neural systems that mediate the Paced Auditory Serial Addition Task (PASAT).

The paced auditory serial addition task (PASAT), in which subjects hear a number-string and add the two most-recently heard numbers, is a neuropsychological test sensitive to cerebral dysfunction. We mapped the brain regions activated by the PASAT using positron emission tomography (PET) and 15O-water to measure cerebral blood flow. We parsed the PASAT by mapping sites activated by immediate repetition of numbers and by repetition of the prior number after the presentation of the next number in the series. The PASAT activated dispersed non-contiguous foci in the superior temporal gyri, bifrontal and biparietal sites, the anterior cingulate and bilateral cerebellar sites. These sites are consistent with the elements of the task that include auditory perception and processing, speech production, working memory, and attention. Sites mediating addition were not identified. The extent of the sites activated during the performance of the PASAT accounts for the sensitivity of this test and justifies its use in a variety of seemingly disparate conditions.

Acoustic Stimulation↗