Auditory agnosia as a clinical symptom of childhood adrenoleukodystrophy.

OBJECTIVE: To investigate detailed auditory features in patients with auditory impairment as the first clinical symptoms of childhood adrenoleukodystrophy (CSALD). SUBJECTS AND METHODS: Three patients who had hearing difficulty as the first clinical signs and/or symptoms of ALD. Precise examination of the clinical characteristics of hearing and auditory function was performed, including assessments of pure tone audiometry, verbal sound discrimination, otoacoustic emission (OAE), and auditory brainstem response (ABR), as well as an environmental sound discrimination test, a sound lateralization test, and a dichotic listening test (DLT). The auditory pathway was evaluated by MRI in each patient. RESULTS: Poor response to calling was detected in all patients. Two patients were not aware of their hearing difficulty, and had been diagnosed with normal hearing by otolaryngologists at first. Pure-tone audiometry disclosed normal hearing in all patients. All patients showed a normal wave V ABR threshold. Three patients showed obvious difficulty in discriminating verbal sounds, environmental sounds, and sound lateralization and strong left-ear suppression in a dichotic listening test. However, once they discriminated verbal sounds, they correctly understood the meaning. Two patients showed elongation of the I-V and III-V interwave intervals in ABR, but one showed no abnormality. MRIs of these three patients revealed signal changes in auditory radiation including in other subcortical areas. CONCLUSION: The hearing features of these subjects were diagnosed as auditory agnosia and not aphasia. It should be emphasized that when patients are suspected to have hearing impairment but have no abnormalities in pure tone audiometry and/or ABR, this should not be diagnosed immediately as psychogenic response or pathomimesis, but auditory agnosia must also be considered.

Cortical oscillatory power changes during auditory oddball task revealed by spatially filtered magnetoencephalography.

OBJECTIVE: To investigate the neural sources and associated changes in oscillatory activity involved in auditory attention and memory updating processing using spatially filtered magnetoencephalography. METHODS: We recorded magnetic responses during an auditory oddball task in 12 normal subjects. Synthetic aperture magnetometry (SAM)-permutation analysis was used to visualize the multiple brain regions associated with event-related magnetic fields (ERFs), and event-related oscillations during target detection processing. RESULTS: SAM-permutation results showed the topographical distribution of N1m over the bilateral primary auditory cortex. Post-stimulus delta (1.5-4 Hz) activity sources, likely related to the P300 slow-waveform, were distributed over the right frontocentral and parietal regions. Source locations of theta (4-8 Hz) and alpha (8-13 Hz) event-related synchronization (ERS) were identified over the dorsolateral and medial prefrontal cortex. We visualized bilateral central-Rolandic suppresions for mu (8-15 Hz), beta (15-30 Hz), and low-gamma (30-60 Hz) activities, more dominant in the hemisphere contralateral to the moving hand (button-pressing in response to target stimuli). CONCLUSIONS: Prefrontal theta and alpha ERS, and frontocentral-parietal delta ERS are functionally engaged in auditory attention and memory updating process. SIGNIFICANCE: Spatially filtered MEG is valuable for detection and source localization of task-related changes in the ongoing oscillatory activity during oddball tasks.

Time may be compressed in sound representation as replicated in sensory memory.

The flow of time in the real world may differ from the one in the neural representation of auditory scene stored in the sensory memory, because the encoded information is free from the rule of actual time. The sensory memory underlying the automatic discriminative system is reflected by the mismatch negativity (MMN). The time-wise image of preceding sounds is integrated into the sensory memory as a 160-170 ms epoch. We measured the mismatch field (MMNm) and the reaction time responding to the omitted segments incorporated into a complex sound. The main result was that the encoded period from early to late omitted segment was shorter than the actual one, suggesting that time may be compressed in the sound representation.

Magnetoencephalographic study of the cortical activity elicited by human voice.

In an attempt to identify voice-specific neural activities in auditory cortex in humans, we recorded cortical magnetic responses. Volunteers were instructed to listen to vocal and instrumental sounds matched in fundamental-frequency, duration, temporal envelope and average root mean square power. The stimuli were sounds produced by four singers and four musical instruments at each of two fundamental frequencies: 220 Hz (musical note A3) and 261.9 Hz (C3). Two components of the evoked responses were analyzed, one at approximately 100 ms (N1m) and the other 400 ms after the stimulus onset (sustained field, SF). The source locations of equivalent current dipoles for both components were estimated around the Heschl's gyrus in both hemispheres. Compared with the instrumental sound, the source strength of the SF component for the voice was significantly larger.

Cortical activities relating to modulation of sound frequency: how to vocalize?

This is the first report to clarify the underlying mechanisms of processing in the modulation of frequencies (tones) in humans using magnetoencephalography (MEG). Volunteers were instructed to vocalize a simple vowel sound (/u/) after receiving a cue (S2) for either one of three (low, middle, or high fundamental frequencies) (F0s). Three tasks, (1) the modulated vocalization task in which the subjects were asked to modulate vocalization tones according to S2, (2) the non-modulated vocalization task in which the subjects were asked to vocalize the same sound (/u/) with a fixed F0, and (3) the image task in which the subjects had to modulate according to S2 and imagine the vowel (/u/) sound, but not vocalize it. In all tasks, two clear components, 1M and 2M, were recorded at approximately 190 and 290 ms after the S2. Since both were identified even in the Image task, they appear to be specifically related to activity for modulation. The equivalent current dipoles of both 1M and 2M were estimated to lie mainly in the inferior frontal lobe or insula in both hemispheres. Therefore, the activity relating to modulation mainly took place in the inferior frontal lobe or insula in both hemispheres starting about 200 ms after the viewing of a cue.

Cortical evidence of the perceptual backward masking effect on /l/ and /r/ sounds from a following vowel in Japanese speakers.

We examined the influence of stimulus duration of foreign consonant vowel stimuli on the MMNm (magnetic counter part of mismatch negativity). In Experiment 1, /ra/ and /la/ stimuli were synthesized and subjects were native Japanese speakers who are known to have difficulty discriminating the stimuli. "Short" duration stimuli were terminated in the middle of the consonant-to-vowel transition (110 ms). They were nevertheless clearly identifiable by English speakers. A clear MMNm was observed only for short-duration stimuli but not for untruncated long-duration (150-ms) stimuli. We suggest that the diminished MMNm for longer duration stimuli result from more effective masking by the longer vowel part. In Experiment 2 we examined this hypothesis by presenting only the third formant (F3) component of the original stimuli, since the acoustic difference between /la/ and /ra/ is most evident in the third formant, whereas F1 and F2 play a major role in vowel perception. If the MMNm effect depends on the acoustic property of F3, a stimulus duration effect comparable to that found with the original /la/ and /ra/ stimuli might be expected. However, if the effect is attributable to the masking effect from the vowel, no influence of stimulus duration would be expected, since neither stimulus contains F1 and F2 components. In fact, the results showed that the "F3 only" stimuli did not show a duration effect; MMNm was always elicited independent of stimulus duration. The MMN stimulus duration effect is thus suggested to come from the backward masking of foreign consonants by subsequent vowels.