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The author presents a characteristic of a fluctuating perception hearing disorder of unknown aetiology. The disease is considered a special nosological unit. Two examples are given. In the discussion the author analyzes the literature pertaining to the aetiology of the disease.
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A patient with multiple sclerosis (MS) in whom a lesion developed in the pons between successive nuclear magnetic resonance image scans (MRI) is described. The patient developed intolerance of loud sounds, with distorted perception of speech and music, and abnormalities of stapedius reflex threshold, masked speech audiometry, and masking level difference assessment in the presence of an unchanged pure tone threshold. These abnormalities partially resolved over a number of months. It is postulated that the efferent olivo-cochlear bundle was involved on one side of the pons and the abnormalities are interpreted in terms of bilateral removal of inhibition from the hair cells of the cochlea.
We administered a battery of both behavioral and electrophysiologic measures to a pair of fraternal twin girls, one of whom exhibited symptoms consistent with an auditory processing disorder. Both twins were within normal limits on standardized tests of cognitive and language skills. Basic audiometric measures, as well as behavioral tests of simultaneous masking, backward masking, gap detection, and frequency-sweep discrimination, showed little difference between the twins. Significant differences, however, were evident on event-related potentials (ERPs) in response to both within-channel and across-channel gap detection tasks. Substantial differences were also noted for ERPs to both linguistic and nonlinguistic targets in dichotic listening paradigms. The pattern of electrophysiologic results was consistent with a deficit in the efficiency of interhemispheric transfer of auditory information. A possible reason for the greater effectiveness of electrophysiologic over behavioral measures is discussed.
Generally, cortical deafness is not complicated by anarthria and cortical anarthria does not affect auditory perception. We report a case of simultaneous progressive cortical deafness and anarthria. At the age of 70 years, the patient, a woman, noticed hearing problems when using the telephone, which worsened rapidly over the next 2 years. She was then referred to our hospital for further examinations of her hearing problems. Auditory tests revealed threshold elevation in the low and middle frequencies on pure-tone audiometry, a maximum speech discrimination of 25% and normal otoacoustic emissions and auditory brainstem, middle- and long-latency responses. An articulation test revealed abnormal pronunciation. Because of these problems only written and not verbal communication was possible; her ability to read and write was unimpaired. She showed no other neurological problems. Brain MRI demonstrated atrophic changes of the auditory cortex and Wernicke's language center and PET suggested low uptake of (18F) 2-fluoro-2-deoxy-d-glucose around the Sylvian fissures in both hemispheres. Neurologically, the patient was suspected of having progressive aphasia or frontotemporal dementia. Her cortical deafness and anarthria are believed to be early signs of this entity.
We carried out extensive audiologic, electrophysiologic, and neuropsychologic testing on a young woman who complained that she had difficulty hearing in her educational environment. Conventional audiometric results, including pure-tone, speech, and immittance audiometry, were all within normal limits. The subject performed normally on tests involving the processing of rapidly changing temporal information, interaural time and intensity difference detection, and both absolute and relative sound localization. Early, middle, late and task-related auditory evoked potentials were essentially normal, although some asymmetry was observed in the middle latency (MLR) and late (LVR) responses. There was, however, a consistent left-ear deficit on dichotic sentence identification, on threshold and suprathreshold speech measures in the left sound field when various types of competition were delivered in the right sound field, and on cued-target identification in the left sound field in the presence of multitalker babble. Results suggest a central auditory processing disorder characterized by an asymmetric problem in the processing of binaural, noncoherent signals in auditory space. When auditory space was structured such that the target was directed to the left ear, and the competition to the right ear, unwanted background was less successfully suppressed than when the physical arrangement was reversed.
OBJECTIVE: To compare the auditory function of normal-hearing children attending mainstream schools who were referred for an auditory evaluation because of listening/hearing problems (suspected auditory processing disorders [susAPD]) with that of normal-hearing control children. DESIGN: Sixty-five children with a normal standard audiometric evaluation, ages 6-14 yr (32 of whom were referred for susAPD, with the rest age-matched control children), completed a battery of four auditory tests: a dichotic test of competing sentences; a simple discrimination of short tone pairs differing in fundamental frequency at varying interstimulus intervals (TDT); a discrimination task using consonant cluster minimal pairs of real words (CCMP), and an adaptive threshold task for detecting a brief tone presented either simultaneously with a masker (simultaneous masking) or immediately preceding it (backward masking). Regression analyses, including age as a covariate, were performed to determine the extent to which the performance of the two groups differed on each task. Age-corrected z-scores were calculated to evaluate the effectiveness of the complete battery in discriminating the groups. RESULTS: The performance of the susAPD group was significantly poorer than the control group on all but the masking tasks, which failed to differentiate the two groups. The CCMP discriminated the groups most effectively, as it yielded the lowest number of control children with abnormal scores, and performance in both groups was independent of age. By contrast, the proportion of control children who performed poorly on the competing sentences test was unacceptably high. Together, the CCMP (verbal) and TDT (nonverbal) tasks detected impaired listening skills in 56% of the children who were referred to the clinic, compared with 6% of the control children. Performance on the two tasks was not correlated. CONCLUSIONS: Two of the four tests evaluated, the CCMP and TDT, proved effective in differentiating the two groups of children of this study. The application of both tests increased the proportion of susAPD children who performed poorly compared with the application of each test alone, while reducing the proportion of control subjects who performed poorly. The findings highlight the importance of carrying out a complete auditory evaluation in children referred for medical attention, even if their standard audiometric evaluation is unremarkable.
It is commonly observed that 'tone deaf' individuals are unable to hear the beat of a tune, yet deficits on simple timing tests have not been found. In this study, we investigated rhythm processing in nine individuals with congenital amusia ('tone deafness') and nine controls. Participants were presented with pairs of 5-note sequences, and were required to detect the presence of a lengthened interval. In different conditions the sound sequences were presented isochronously or in an integer-ratio rhythm, and these were either monotonic or varied randomly in pitch. It was found that the 'tone deaf' participants exhibited inferior rhythm analysis for the sequences that varied in pitch compared to those that did not, whereas the controls obtained equivalent thresholds for these two conditions. These results suggest that the rhythm deficits in congenital amusia result from the pitch-variations in music.
Children have higher auditory backward masking (BM) thresholds than adults. One explanation for this is poor temporal resolution, resulting in difficulty separating brief or rapidly presented sounds. This implies that the auditory temporal window is broader in children than in adults. Alternatively, elevated BM thresholds in children may indicate poor processing efficiency. In this case, children would need a higher signal-to-masker ratio than adults to detect the presence of a signal. This would result in poor performance on a number of psychoacoustic tasks but would be particularly marked in BM due to the compressive nonlinearity of the basilar membrane. The objective of the present study was to examine the competing hypotheses of "temporal resolution" and "efficiency" by measuring BM as a function of signal-to-masker interval in children and adults. The children had significantly higher thresholds than the adults at each of the intervals. Subsequent modeling and analyses showed that the data for both children and adults were best fitted using the same, fixed temporal window. Therefore, the differences in BM threshold between adults and children were not due to differences in temporal resolution but to reduced detection efficiency in the children.
It has been proposed that specific language impairment (SLI) is caused by an impairment of auditory processing, but it is unclear whether this problem affects temporal processing, frequency discrimination (FD), or both. Furthermore, there are few longitudinal studies in this area, making it hard to establish whether any deficit represents a developmental lag or a more permanent deficit. To address these issues, the authors retested a group of 10 children with SLI and 12 control children first tested 42 months previously. At Time 1, the children with SLI (between 9 and 12 years of age) had significantly elevated FD thresholds compared to the matched controls. At Time 2, the thresholds of both groups had improved, but the children with SLI still had poorer FD thresholds than those of the controls. To assess temporal resolution, auditory backward masking was measured and it was found that most of the children with SLI performed as well as the controls, but 2 children had exceptionally high thresholds. There was also greater variability among the children with SLI compared to that measured among the controls on the FD task. These studies indicate considerable heterogeneity in auditory function among children with SLI and suggest that, as with auditory temporal deficits, difficulties in FD discrimination are important in this population.
The application of auditory processing tests to patients with sensorineural hearing loss is controversial. Several studies have shown that it is difficult to separate peripheral from central hearing processes. In the present study, a Dutch auditory processing test battery was administered to 24 subjects with mild, relatively flat, symmetrical sensorineural hearing loss. Tests were administered twice; the second time, the presentation level in four out of the six tests was adjusted according to the speech reception threshold. The scores of the hearing-impaired subjects were significantly poorer than those of the subjects with normal hearing on five out of the six tests, even with the adjusted presentation level. Significant correlations were found between test scores and PTA (pure-tone average); scores on words-in-noise, filtered-speech and binaural-fusion tests were additionally corrected according to PTA. In contrast to previous studies in the literature, the present dichotic-digit and pattern-recognition tests were greatly influenced by mild hearing loss. Therefore, this auditory processing test battery cannot readily be used to diagnose central auditory processing disorders in patients with flat sensorineural hearing loss. At least, both adjustment of presentation level and additional correction are needed.
A core difficulty in developmental dyslexia is the accurate specification and neural representation of speech. We argue that a likely perceptual cause of this difficulty is a deficit in the perceptual experience of rhythmic timing. Speech rhythm is one of the earliest cues used by infants to discriminate syllables and is determined principally by the acoustic structure of amplitude modulation at relatively low rates in the signal. We show significant differences between dyslexic and normally reading children, and between young early readers and normal developers, in amplitude envelope onset detection. We further show that individual differences in sensitivity to the shape of amplitude modulation account for 25% of the variance in reading and spelling acquisition even after controlling for individual differences in age, nonverbal IQ, and vocabulary. A possible causal explanation dependent on perceptual-center detection and the onset-rime representation of syllables is discussed.
We examined to what extent patients with fibromyalgia differ from painfree control subjects in the perception and processing not only of somatosensory but also of external stimuli. For this purpose the acoustic perception of 30 patients with fibromyalgia was compared with that of 36 generally pain-free age and gender matched subjects. The groups were also controlled for organic disease of pathological dysfunction of the ear and auditory nerves. Thresholds of unpleasantness and hearing thresholds were determined autiometrically for various frequencies. In addition the participants rated their experience of daily noise, vulnerability to acoustic stress, and functional and affective complaints associated with fibromyalgia. As expected the results show reduced unpleasantness thresholds for all frequencies and a nonsymptomatic hearing loss for higher frequencies. The elevated hearing threshold correlated significantly with experience of noise at the place of work, which was also elevated in the fibromyalgia group. Generalized pain had a high impact on the interaction between threshold of unpleasantness and daily noise experience. We interpret the differences in thresholds of hearing and of unpleasantness in patients with fibromyalgia as a form of either preconscious or conscious acts to protect against disturbing stimulation. Our results support the notion of a generalized disturbancy of perceptual thresholds in patients with fibromyalgia not restricted to the perception of pain.
BACKGROUND: The binaural masking level difference (BMLD) is defined as the contrast between homophasic and antiphasic signal conditions, and can be regarded as a measure for noise suppression by the auditory system. METHODS: An adaptive two interval forced choice (IFC) measurement method was designed to assess BMLDs in children. The target tone was a 500 Hz sine pip which was masked with narrow band noise. The presentation of homo- and antiphasic signals was staggered in order to take into account artefacts--such as changes in vigilance--in a similar manner for both conditions. RESULTS: Measurement was feasible in 57 out of 85 children (6-14 y.) with suspected auditory processing disorders. Mean BMLD was 7.4(+/-4.5 SD) dB. Five children revealed a BMLD close to 0 dB suggesting that they had no advantage in signal detection for the antiphasic condition. Eleven children were not able to cope with the task of measurement. In another 17 children, the measurements did not converge to a stable threshold. DISCUSSION: The proposed measurement method was feasible with most of the children. With slight modifications of the threshold convergence, this proportion can be further increased. Whether or not the large inter-individual scatter of the results is an expression of the heterogeneous clinical clientele can only be determined when normative data are available.
Evidence that deficits in early auditory processing occur in schizophrenia was first provided by an ERP study demonstrating that mismatch negativity (MMN) to duration increments is reduced in medicated patients. Our subsequent research, which is reviewed in this paper, demonstrates that duration MMN reduction cannot be attributed to neuroleptic medication, and is specific to schizophrenia. It is not dependent upon the nature of the task used to distract attention away from the auditory modality. Most importantly, a reduced duration MMN in schizophrenia is a replicable result, having been observed in multiple independently-selected groups of patients from two separate laboratories. It also occurs in unaffected first-degree relatives of patients, suggesting that it may be a vulnerability marker of the disorder. The most intriguing questions however, relate to what underpins the reduced MMN to duration increments in schizophrenia and therefore, what it reveals about the nature of the auditory system deficit in this disorder. Three hypotheses are considered here: a pervasive problem in auditory sensory memory; a specific impairment in duration processing; or an abnormality within the window of temporal integration, coincident with the early phase of auditory sensory memory. Our data so far offer preliminary support for the third hypothesis only, although the possibility of a more broadly defined deficit in temporal processing restricted to brief or rapidly-presented auditory stimuli is canvassed.
The Listening in Spatialized Noise test (LISN) produces a virtual three-dimensional auditory environment under headphones. Various measures assess the extent to which either spatial, vocal, or spatial and vocal cues combined increase a listener's ability to comprehend a target story in the presence of distracter sentences, without being affected by differences between participants in variables such as linguistic skills. Ten children at risk for auditory processing disorder (APD group) were assessed on the LISN, as well as a traditional APD test battery. The APD group performed significantly more poorly on all LISN measures than 48 age-matched controls. On the spatial advantage measure, the APD group achieved a mean advantage of only 3.7 dB when the distracters were spatially separated from the target by +/-90 degrees, compared to 10.0 dB for the controls-the 6.3 dB difference significant at p < 0.000001, with nine children scoring outside the normal range. The LISN was considered a promising addition to an APD test battery.
Uttenweiler's dichotic discrimination test is a valuable contribution to the diagnosis of auditory perception disorders. With this test it is possible to establish whether there is a deficit in a patient's ability to discriminate between differing spoken signals to each ear, as shown in a study contrasting elementary school age children with normal hearing with children having auditory perception disorders. Until now only a limited comparison of test results has been possible, as previous test modes have required an increase in volume when words could not be completely distinguished. A further problem is that very different hearing abilities may give rise to the same test configurations. This study presents an alternative test method that allows a direct individual comparison of results. The volume of the words spoken is no longer increased when words are not adequately understood. In addition, a point system is introduced that enables a quantitative analysis of the same test performances. This new evaluation scale is explained on the basis of actual examples.
The principal symptom of subjects suffering from King-Kopetzky syndrome is a perceived difficulty in recognizing and understanding speech in noisy backgrounds. For some patients, minor disturbances in auditory function, e.g. a deteriorated signal-to-noise ratio for speech, can be demonstrated; for others, all measurements of hearing are normal. A conceptual framework developed for the analysis of communication disorders is applied to King-Kopetzky syndrome in the present article. The usefulness of the framework is evaluated, and an extended system for the analysis of King-Kopetzky syndrome patients is suggested. It is emphasized that changes in demands resulting from external conditions and altered perception and evaluation of the conditions may generate the symptoms. A change in the subjects' desired self-image (preferendum) and a change in social role, new, unfamiliar vocabulary, as well as minor dysfunctions in peripheral and central auditory processing, might generate the characteristic features of the syndrome. The framework is related to the current literature, and arguments for the usefulness of the framework in the planning of support and rehabilitation are presented. Several new questions are generated for further analysis of King-Kopetzky syndrome.