Neuronal Development of Hearing and Language: Cochlear Implants and Critical Periods.

The modern cochlear implant (CI) is the most successful neural prosthesis developed to date. CIs provide hearing to the profoundly hearing impaired and allow the acquisition of spoken language in children born deaf. Results from studies enabled by the CI have provided new insights into (a) minimal representations at the periphery for speech reception, (b) brain mechanisms for decoding speech presented in quiet and in acoustically adverse conditions, (c) the developmental neuroscience of language and hearing, and (d) the mechanisms and time courses of intramodal and cross-modal plasticity. Additionally, the results have underscored the interconnectedness of brain functions and the importance of top-down processes in perception and learning. The findings are described in this review with emphasis on the developing brain and the acquisition of hearing and spoken language.

Focal Brain Lesions Causing Acquired Amusia Map to a Common Brain Network.

Music is a universal human attribute. The study of amusia, a neurologic music processing deficit, has increasingly elaborated our view on the neural organization of the musical brain. However, lesions causing amusia occur in multiple brain locations and often also cause aphasia, leaving the distinct neural networks for amusia unclear. Here, we utilized lesion network mapping to identify these networks. A systematic literature search was carried out to identify all published case reports of lesion-induced amusia. The reproducibility and specificity of the identified amusia network were then tested in an independent prospective cohort of 97 stroke patients (46 female and 51 male) with repeated structural brain imaging, specifically assessed for both music perception and language abilities. Lesion locations in the case reports were heterogeneous but connected to common brain regions, including bilateral temporoparietal and insular cortices, precentral gyrus, and cingulum. In the prospective cohort, lesions causing amusia mapped to a common brain network, centering on the right superior temporal cortex and clearly distinct from the network causally associated with aphasia. Lesion-induced longitudinal structural effects in the amusia circuit were confirmed as reduction of both gray and white matter volume, which correlated with the severity of amusia. We demonstrate that despite the heterogeneity of lesion locations disrupting music processing, there is a common brain network that is distinct from the language network. These results provide evidence for the distinct neural substrate of music processing, differentiating music-related functions from language, providing a testable target for noninvasive brain stimulation to treat amusia.

Acquired amusia after a right middle cerebral artery infarction - a case study.

A 62-year-old musician-MM-developed amusia after a right middle-cerebral-artery infarction. Initially, MM showed melodic deficits while discriminating pitch-related differences in melodies, musical memory problems, and impaired sensitivity to tonal structures, but normal pitch discrimination and spectral resolution thresholds, and normal cognitive and language abilities. His rhythmic processing was intact when pitch variations were removed. After 3 months, MM showed a large improvement in his sensitivity to tonality, but persistent melodic deficits and a decline in perceiving the metric structure of rhythmic sequences. We also found visual cues aided melodic processing, which is novel and beneficial for future rehabilitation practice.

Right ventral stream damage underlies both poststroke aprosodia and amusia.

BACKGROUND AND PURPOSE: This study was undertaken to determine and compare lesion patterns and structural dysconnectivity underlying poststroke aprosodia and amusia, using a data-driven multimodal neuroimaging approach. METHODS: Thirty-nine patients with right or left hemisphere stroke were enrolled in a cohort study and tested for linguistic and affective prosody perception and musical pitch and rhythm perception at subacute and 3-month poststroke stages. Participants listened to words spoken with different prosodic stress that changed their meaning, and to words spoken with six different emotions, and chose which meaning or emotion was expressed. In the music tasks, participants judged pairs of short melodies as the same or different in terms of pitch or rhythm. Structural magnetic resonance imaging data were acquired at both stages, and machine learning-based lesion-symptom mapping and deterministic tractography were used to identify lesion patterns and damaged white matter pathways giving rise to aprosodia and amusia. RESULTS: Both aprosodia and amusia were behaviorally strongly correlated and associated with similar lesion patterns in right frontoinsular and striatal areas. In multiple regression models, reduced fractional anisotropy and lower tract volume of the right inferior fronto-occipital fasciculus were the strongest predictors for both disorders, over time. CONCLUSIONS: These results highlight a common origin of aprosodia and amusia, both arising from damage and disconnection of the right ventral auditory stream integrating rhythmic-melodic acoustic information in prosody and music. Comorbidity of these disabilities may worsen the prognosis and affect rehabilitation success.

Abnormal development of auditory responses in the inferior colliculus of a mouse model of Fragile X Syndrome.

Sensory processing abnormalities are frequently associated with autism spectrum disorders, but the underlying mechanisms are unclear. Here we studied auditory processing in a mouse model of Fragile X Syndrome (FXS), a leading known genetic cause of autism and intellectual disability. Both humans with FXS and the Fragile X mental retardation gene (Fmr1) knockout (KO) mouse model show auditory hypersensitivity, with the latter showing a strong propensity for audiogenic seizures (AGS) early in development. Because midbrain abnormalities cause AGS, we investigated whether the inferior colliculus (IC) of the Fmr1 KO mice shows abnormal auditory processing compared with wild-type (WT) controls at specific developmental time points. Using antibodies against neural activity marker c-Fos, we found increased density of c-Fos+ neurons in the IC, but not auditory cortex, of Fmr1 KO mice at P21 and P34 following sound presentation. In vivo single-unit recordings showed that IC neurons of Fmr1 KO mice are hyperresponsive to tone bursts and amplitude-modulated tones during development and show broader frequency tuning curves. There were no differences in rate-level responses or phase locking to amplitude-modulated tones in IC neurons between genotypes. Taken together, these data provide evidence for the development of auditory hyperresponsiveness in the IC of Fmr1 KO mice. Although most human and mouse work in autism and sensory processing has centered on the forebrain, our new findings, along with recent work on the lower brainstem, suggest that abnormal subcortical responses may underlie auditory hypersensitivity in autism spectrum disorders.NEW & NOTEWORTHY Autism spectrum disorders (ASD) are commonly associated with sensory sensitivity issues, but the underlying mechanisms are unclear. This study presents novel evidence for neural correlates of auditory hypersensitivity in the developing inferior colliculus (IC) in Fmr1 knockout (KO) mouse, a mouse model of Fragile X Syndrome (FXS), a leading genetic cause of ASD. Responses begin to show genotype differences between postnatal days 14 and 21, suggesting an early developmental treatment window.

Deficits in auditory sensory discrimination among children with attention-deficit/hyperactivity disorder.

Research into children with attention-deficit/hyperactivity disorder (ADHD) has focused on complex cognitive dysfunction, but less attention has been paid to sensory perception processes underlying the symptoms of ADHD. Based on signal detection theory, the present study compared the sensory discrimination ability and decision bias of children with and without ADHD. It also investigated the differences between ADHD with predominantly inattentive (ADHDi) and combined presentations (ADHDc). The sample of 75 children and adolescents with ADHD (24 ADHDi, 51 ADHDc) (16 females and 59 males) and 22 typical developing controls (TD) (8 females and 14 males) completed an auditory signal detection task. Participants were asked to detect signals against levels of transient background noise (35, 45, 55, and 65 dB). The results showed that with the increase of noise levels, both the ADHD and TD groups demonstrated decreased sensory discrimination. Although both groups successfully detected signal against noise levels from 35 to 55 dB, the ADHD group showed lower discrimination ability than that of the TD group. For decision bias, no group difference was found. Further comparisons regarding the predominant symptom presentation of ADHD sub-groups showed no differences. Current research has suggested that the deficit in ADHD people's signal detection performance can be attributed to sensory discrimination rather than decision bias. We suggest that background noise should be taken into account when using auditory stimuli to investigate cognitive functions in people with ADHD.

Behavioral assessment of auditory processing deficits in schizophrenia: Literature review and suggestions for future research.

During the last decades, a considerable number of studies about auditory processing deficits in schizophrenia have been published, some of them using the term of Auditory Processing Disorder (APD). Due to heterogeneous methodology and inconsistent results concrete conclusions may not be straightforward. We focused on studies that used at least one behavioral test for the assessment of auditory processing in schizophrenia, in order to identify new evidence on auditory processing deficits in schizophrenia, and to consider fields for future research. 28 studies met inclusion criteria and are presented in this review. The articles were divided into three groups, those that implemented a test battery approach, those that used only Dichotic Listening (DL), those that used DL along with imaging or electrophysiology techniques. Most of the studies that implemented a test battery showed significant performance differences between patients and controls. This indicates APD presence. Due to the limited number of studies implementing a test battery, this is not conclusive. The majority of all studies that used a DL task showed that patients' performance or laterality was significantly lower than that of controls. Age, duration of illness, total and specific positive symptoms seem to affect significantly DL performance and auditory laterality. The results support the existence of various schizophrenia subgroups that differ in their auditory processing performance, and also have structural and functional specificities. Further research in the field is needed, especially studies that implement a test battery, and studies that examine possible correlations between clinical variables and auditory processing deficits.

Cortical Representation of Interaural Time Difference Is Impaired by Deafness in Development: Evidence from Children with Early Long-term Access to Sound through Bilateral Cochlear Implants Provided Simultaneously.

Accurate use of interaural time differences (ITDs) for spatial hearing may require access to bilateral auditory input during sensitive periods in human development. Providing bilateral cochlear implants (CIs) simultaneously promotes symmetrical development of bilateral auditory pathways but does not support normal ITD sensitivity. Thus, although binaural interactions are established by bilateral CIs in the auditory brainstem, potential deficits in cortical processing of ITDs remain. Cortical ITD processing in children with simultaneous bilateral CIs and normal hearing with similar time-in-sound was explored in the present study. Cortical activity evoked by bilateral stimuli with varying ITDs (0, ±0.4, ±1 ms) was recorded using multichannel electroencephalography. Source analyses indicated dominant activity in the right auditory cortex in both groups but limited ITD processing in children with bilateral CIs. In normal-hearing children, adult-like processing patterns were found underlying the immature P1 (∼100 ms) response peak with reduced activity in the auditory cortex ipsilateral to the leading ITD. Further, the left cortex showed a stronger preference than the right cortex for stimuli leading from the contralateral hemifield. By contrast, children with CIs demonstrated reduced ITD-related changes in both auditory cortices. Decreased parieto-occipital activity, possibly involved in spatial processing, was also revealed in children with CIs. Thus, simultaneous bilateral implantation in young children maintains right cortical dominance during binaural processing but does not fully overcome effects of deafness using present CI devices. Protection of bilateral pathways through simultaneous implantation might be capitalized for ITD processing with signal processing advances, which more consistently represent binaural timing cues.SIGNIFICANCE STATEMENT Multichannel electroencephalography demonstrated impairment of binaural processing in children who are deaf despite early access to bilateral auditory input by first finding that foundations for binaural hearing are normally established during early stages of cortical development. Although 4- to 7-year-old children with normal hearing had immature cortical responses, adult patterns in cortical coding of binaural timing cues were measured. Second, children receiving two cochlear implants in the same surgery maintained normal-like input from both ears, but this did not support significant effects of binaural timing cues in either auditory cortex. Deficits in parieto-occiptal areas further suggested impairment in spatial processing. Results indicate that cochlear implants working independently in each ear do not fully overcome deafness-related binaural processing deficits, even after long-term experience.

[Impaired recognition of environmental sounds in patients with dementia : I can hear but I do not understand]. / Störung des Erkennens von Umweltgeräuschen bei Demenz : Ich höre aber ich verstehe nicht.

BACKGROUND: Impairment of central auditory processing is a well-known symptom of neurodegenerative dementia; however, whilst numerous studies have examined verbal processing impairment, to date few have attempted to describe impairments of non-verbal, environmental sound recognition in patients with dementia. As these impairments may have direct implications on patient support and care, such studies are urgently necessary. AIM OF THE STUDY: The aim of the study was to determine whether the recognition of meaningful environmental sounds is impaired in patients with mild or early stage neurodegenerative dementia. PATIENTS AND METHODS: We developed a test of non-verbal sound recognition consisting of 16 sound sequences from the familiar and unfamiliar environments. We included 18 patients with mild cognitive impairment and mild dementia caused by Alzheimer's disease and frontotemporal dementia, as well as 20 cognitively healthy controls. RESULTS: Patients and controls were given the test of recognizing 16 meaningful sounds from the familiar and unfamiliar environments. Patients with dementia performed significantly worse in comparison to cognitively healthy controls. Whilst healthy controls correctly recognized on average 12.1 ± 2.2 out of 16 sounds, cognitively impaired patients recognized 9.2 ± 2.5. Correlation analysis showed that the mini mental state examination (MMSE) scores were positively correlated with the number of correctly recognized sounds (MMSE: r = 0.556, p = 0.017). DISCUSSION: The fact that even in mild stages of Alzheimer's disease or frontotemporal dementia patients either do not recognize or misinterpret environmental sounds must be taken into consideration not only in everyday life but in particular when patients need to leave their familiar living environment, whether temporarily (e. g. hospitalization) or permanently (e. g. nursing home admission).

Neural Basis of Acquired Amusia and Its Recovery after Stroke.

UNLABELLED: Although acquired amusia is a relatively common disorder after stroke, its precise neuroanatomical basis is still unknown. To evaluate which brain regions form the neural substrate for acquired amusia and its recovery, we performed a voxel-based lesion-symptom mapping (VLSM) and morphometry (VBM) study with 77 human stroke subjects. Structural MRIs were acquired at acute and 6 month poststroke stages. Amusia and aphasia were behaviorally assessed at acute and 3 month poststroke stages using the Scale and Rhythm subtests of the Montreal Battery of Evaluation of Amusia (MBEA) and language tests. VLSM analyses indicated that amusia was associated with a lesion area comprising the superior temporal gyrus, Heschl's gyrus, insula, and striatum in the right hemisphere, clearly different from the lesion pattern associated with aphasia. Parametric analyses of MBEA Pitch and Rhythm scores showed extensive lesion overlap in the right striatum, as well as in the right Heschl's gyrus and superior temporal gyrus. Lesions associated with Rhythm scores extended more superiorly and posterolaterally. VBM analysis of volume changes from the acute to the 6 month stage showed a clear decrease in gray matter volume in the right superior and middle temporal gyri in nonrecovered amusic patients compared with nonamusic patients. This increased atrophy was more evident in anterior temporal areas in rhythm amusia and in posterior temporal and temporoparietal areas in pitch amusia. Overall, the results implicate right temporal and subcortical regions as the crucial neural substrate for acquired amusia and highlight the importance of different temporal lobe regions for the recovery of amusia after stroke. SIGNIFICANCE STATEMENT: Lesion studies are essential in uncovering the brain regions causally linked to a given behavior or skill. For music perception ability, previous lesion studies of amusia have been methodologically limited in both spatial accuracy and time domain as well as by small sample sizes, providing coarse and equivocal information about which brain areas underlie amusia. By using longitudinal MRI and behavioral data from a large sample of stroke patients coupled with modern voxel-based analyses methods, we were able provide the first systematic evidence for the causal role of right temporal and striatal areas in music perception. Clinically, these results have important implications for the diagnosis and prognosis of amusia after stroke and for rehabilitation planning.

Immuno-modulator inter-alpha inhibitor proteins ameliorate complex auditory processing deficits in rats with neonatal hypoxic-ischemic brain injury.

Hypoxic-ischemic (HI) brain injury is recognized as a significant problem in the perinatal period, contributing to life-long language-learning and other cognitive impairments. Central auditory processing deficits are common in infants with hypoxic-ischemic encephalopathy and have been shown to predict language learning deficits in other at risk infant populations. Inter-alpha inhibitor proteins (IAIPs) are a family of structurally related plasma proteins that modulate the systemic inflammatory response to infection and have been shown to attenuate cell death and improve learning outcomes after neonatal brain injury in rats. Here, we show that systemic administration of IAIPs during the early HI injury cascade ameliorates complex auditory discrimination deficits as compared to untreated HI injured subjects, despite reductions in brain weight. These findings have significant clinical implications for improving central auditory processing deficits linked to language learning in neonates with HI related brain injury.

Psychoacoustics and Neurophysiological Alterations after 30-36 Hours of Sleep Deprivation.

BACKGROUND AND AIM: Sleep deprivation (SD) may result in perceptual and cognitive alterations in healthy subjects. Our objective was to compare whether psychoacoustics and neurophysiological variables in healthy subjects were altered after SD of 30-36 h. METHOD: We examined 22 subjects by means of several psychoacoustics tests, P300 and mismatch negativity (MMN) recordings, and brainstem auditory evoked potentials (BAEP) before and after 30-36 h of SD. RESULTS: In the psychoacoustics tests, we found that after SD, difficulties were experienced by the left ear in the discrimination of words in noise and by the right ear in music discrimination. In the neurophysiological tests, we found delayed latencies of P300 and MMN wave; there was a delay of wave I in both ears, and wave V in the right ear in BAEP. We found significant correlations with positive direction between P300 latency and words in noise and music discrimination in the right ear. CONCLUSIONS: SD results in alterations of central auditory processing perception and delays of brain neurophysiological responses, with some correlations between the psychoacoustics and neurophysiological tests. These alterations may relate to other cognitive alterations that deserve more research in future studies.

Attention and pain: are auditory distractors special?

It is well established that manipulations of attention and emotional state can modulate pain. Some researchers have used olfactory or visual distractors to manipulate these factors in combination, and have found that attention and emotion have different effects on pain intensity and unpleasantness. Specifically, distraction from pain was found to markedly reduce its intensity while having little effect on its unpleasantness. Other evidence indicates, however, that the strength of intermodal attentional shifts depends on the specific modalities involved, with auditory-somesthetic shifts being relatively weak. The present study was, therefore, undertaken to determine how pain intensity and unpleasantness are affected when auditory, rather than olfactory or visual, distractors are used. Attention was directed either to the pain from noxious thermal stimuli, or to simultaneously presented environmental sounds that had either positive (e.g., bird chirping) or negative (e.g., alarm clock) associations. To manipulate attention, subjects were instructed to make two-alternative forced-choice discrimination judgments concerning the temperature of the thermal stimuli (in heat blocks) or the loudness of the sound clips (in sound blocks). Unpleasant sound clips were used during half of the heat blocks and half of the sound blocks, with pleasant sounds in the other half. Participants rated two components of pain: intensity and unpleasantness, after each block of trials. Although pain unpleasantness was influenced both by attentional direction and by the valence of the sound clips, pain intensity was not affected by either of these experimental manipulations. The failure of auditory distractors to modulate pain intensity differs from the previously documented ability of olfactory distractors to do so. Our findings are, however, consistent with evidence that one can attend simultaneously to auditory and cutaneous stimuli. Thus, environmental sounds are not effective at reducing pain intensity, but are capable of modulating pain unpleasantness, perhaps because it is constructed at a later stage.

Racial-Ethnic Differences in Word Fluency and Auditory Comprehension Among Persons With Poststroke Aphasia.

OBJECTIVES: To examine aphasia outcomes and to determine whether the observed language profiles vary by race-ethnicity. DESIGN: Retrospective cross-sectional study using a convenience sample of persons of with aphasia (PWA) obtained from AphasiaBank, a database designed for the study of aphasia outcomes. SETTING: Aphasia research laboratories. PARTICIPANTS: PWA (N=381; 339 white and 42 black individuals). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Western Aphasia Battery-Revised (WAB-R) total scale score (Aphasia Quotient) and subtest scores were analyzed for racial-ethnic differences. The WAB-R is a comprehensive assessment of communication function designed to evaluate PWA in the areas of spontaneous speech, auditory comprehension, repetition, and naming in addition to reading, writing, apraxia, and constructional, visuospatial, and calculation skills. RESULTS: In univariate comparisons, black PWA exhibited lower word fluency (5.7 vs 7.6; P=.004), auditory word comprehension (49.0 vs 53.0; P=.021), and comprehension of sequential commands (44.2 vs 52.2; P=.012) when compared with white PWA. In multivariate comparisons, adjusted for age and years of education, black PWA exhibited lower word fluency (5.5 vs 7.6; P=.015), auditory word recognition (49.3 vs 53.3; P=.02), and comprehension of sequential commands (43.7 vs 53.2; P=.017) when compared with white PWA. CONCLUSIONS: This study identified racial-ethnic differences in word fluency and auditory comprehension ability among PWA. Both skills are critical to effective communication, and racial-ethnic differences in outcomes must be considered in treatment approaches designed to improve overall communication ability.

Perspectives of Dutch health professionals regarding auditory processing disorders; a focus group study.

OBJECTIVE: This study investigated the perspectives of professionals from the Dutch audiological centres on the definition and care pathways of children with suspected auditory processing disorders (susAPD). DESIGN: focus group interviews. STUDY SAMPLE: In total, 45 professionals from 6 disciplines, representing 22 different audiological centres and one ambulatory service, participated in five parallel focus group interviews. Participants had a variety of experience in diagnosing and advising children with suspected APD. RESULTS: Qualitative analysis (open and thematic) identified four themes ("Definition", "Causes", "Diagnostic Procedures" and "Clinical Reasoning") expressing a variety of perspectives. Differences in perspectives were mainly affected by two debates: (1) whether or not APD exists as a pure (auditory) disorder and (2) whether or not current AP-tests are suitable in diagnosing children with listening difficulties. They also expressed a need for more guidance from the literature in their clinical decision making process. CONCLUSIONS: Professionals from the Dutch audiological centres share a broad perspective on children with APD. The ICF framework supports this perspective, thereby diminishing the need for a clear definition. The use of AP-tests should be limited to children where broader developmental disorders are first ruled out; a possible "pure" APD could then be diagnosed in a limited number of children.

Influence of Tinnitus on Auditory Spectral and Temporal Resolution and Speech Perception in Tinnitus Patients.

Although cochlear damage is believed to trigger the perception of tinnitus in the central auditory system, its pathophysiological mechanism is still unclear. We aimed to investigate the pathophysiology of tinnitus using psychoacoustic assessments of auditory spectral and temporal resolution and speech perception in noise. Human subjects with tinnitus and symmetric hearing thresholds were divided into three groups: nine unilateral tinnitus subjects with normal hearing thresholds (Group 1), 12 unilateral tinnitus subjects with hearing loss (HL; Group 2), and nine bilateral tinnitus subjects with HL. Fifteen normal-hearing subjects without tinnitus were also tested as a control group. Four different tests were administered: (1) the spectral-ripple discrimination (SRD) test, (2) the temporal modulation detection (TMD) test, (3) the Schroeder-phase discrimination (SPD) test, and (4) the speech recognition threshold (SRT) in noise test. There were no significant differences in SRD, TMD, and SPD between the tinnitus-affected ears (TEs) and non-tinnitus ears (NTEs) in Groups 1 and 2 (p > 0.05). In contrast, the TEs showed poorer SRTs than the NTEs in these two subject groups (p = 0.022 and 0.049). No inferiority of spectral/temporal resolution in TEs compared with NTEs suggests that there may be no more outer hair cell (OHC) damage on the tinnitus side given that damaged OHCs are associated with broadening the auditory filters. The decoupling of the SRT results from the spectral/temporal resolution data could imply that the occurrence of tinnitus does not depend upon the degree of damage to the OHCs, but rather upon different plastic changes in the central auditory system after cochlear damage. SIGNIFICANCE STATEMENT: We can easily find unilateral tinnitus patients who have symmetric hearing thresholds. Our research question was what kind of difference would be responsible for the tinnitus in the tinnitus-affected ears but not in the non-tinnitus ears of subjects with symmetric hearing thresholds. The answer to this fundamental question could help us to understand the pathophysiology of tinnitus. We evaluated the potential influence of tinnitus upon the subjects' auditory spectral and temporal resolution and speech perception in noise by comparing these psychoacoustic performances between tinnitus-affected ears and non-tinnitus ears in the same subjects.

A Computational Model of Implicit Memory Captures Dyslexics' Perceptual Deficits.

Dyslexics are diagnosed for their poor reading skills, yet they characteristically also suffer from poor verbal memory and often from poor auditory skills. To date, this combined profile has been accounted for in broad cognitive terms. Here we hypothesize that the perceptual deficits associated with dyslexia can be understood computationally as a deficit in integrating prior information with noisy observations. To test this hypothesis we analyzed the performance of human participants in an auditory discrimination task using a two-parameter computational model. One parameter captures the internal noise in representing the current event, and the other captures the impact of recently acquired prior information. Our findings show that dyslexics' perceptual deficit can be accounted for by inadequate adjustment of these components; namely, low weighting of their implicit memory of past trials relative to their internal noise. Underweighting the stimulus statistics decreased dyslexics' ability to compensate for noisy observations. ERP measurements (P2 component) while participants watched a silent movie indicated that dyslexics' perceptual deficiency may stem from poor automatic integration of stimulus statistics. This study provides the first description of a specific computational deficit associated with dyslexia. SIGNIFICANCE STATEMENT: This study presents the first attempt to specify the mechanisms underlying dyslexics' perceptual difficulties computationally by applying a specific model, inspired by the Bayesian framework. This model dissociates between the contribution of sensory noise and that of the prior statistics in an auditory perceptual decision task. We show that dyslexics cannot compensate for their perceptual noise by incorporating prior information. By contrast, adequately reading controls' usage of previous information is often close to optimal. We used ERP measurements to assess the neuronal stage of this deficit. We found that unlike their peers, dyslexics' ERP responses are not sensitive to the relations between the current observation and the prior observation, indicating that they cannot establish a reliable prior.

Auditory spatial processing in Alzheimer's disease.

The location and motion of sounds in space are important cues for encoding the auditory world. Spatial processing is a core component of auditory scene analysis, a cognitively demanding function that is vulnerable in Alzheimer's disease. Here we designed a novel neuropsychological battery based on a virtual space paradigm to assess auditory spatial processing in patient cohorts with clinically typical Alzheimer's disease (n = 20) and its major variant syndrome, posterior cortical atrophy (n = 12) in relation to healthy older controls (n = 26). We assessed three dimensions of auditory spatial function: externalized versus non-externalized sound discrimination, moving versus stationary sound discrimination and stationary auditory spatial position discrimination, together with non-spatial auditory and visual spatial control tasks. Neuroanatomical correlates of auditory spatial processing were assessed using voxel-based morphometry. Relative to healthy older controls, both patient groups exhibited impairments in detection of auditory motion, and stationary sound position discrimination. The posterior cortical atrophy group showed greater impairment for auditory motion processing and the processing of a non-spatial control complex auditory property (timbre) than the typical Alzheimer's disease group. Voxel-based morphometry in the patient cohort revealed grey matter correlates of auditory motion detection and spatial position discrimination in right inferior parietal cortex and precuneus, respectively. These findings delineate auditory spatial processing deficits in typical and posterior Alzheimer's disease phenotypes that are related to posterior cortical regions involved in both syndromic variants and modulated by the syndromic profile of brain degeneration. Auditory spatial deficits contribute to impaired spatial awareness in Alzheimer's disease and may constitute a novel perceptual model for probing brain network disintegration across the Alzheimer's disease syndromic spectrum.

'Noises in the head': a prospective study to characterize intracranial sounds after cranial surgery.

BACKGROUND: Patients often report sounds in the head after craniotomy. We aim to characterize the prevalence and nature of these sounds, and identify any patient, pathology, or technical factors related to them. These data may be used to inform patients of this sometimes unpleasant, but harmless effect of cranial surgery. METHODS: Prospective observational study of patients undergoing cranial surgery with dural opening. Eligible patients completed a questionnaire preoperatively and daily after surgery until discharge. Subjects were followed up at 14 days with a telephone consultation. RESULTS: One hundred fifty-one patients with various pathologies were included. Of these, 47 (31 %) reported hearing sounds in their head, lasting an average 4-6 days (median, 4 days, mean, 6 days, range, 1-14 days). The peak onset was the first postoperative day and the most commonly used descriptors were 'clicking' [20/47 (43 %)] and 'fluid moving' in the head [9/47 (19 %)]. A significant proportion (42 %, 32/77) without a wound drain experienced intracranial sounds compared to those with a drain (20 %, 15/74, p < 0.01); there was no difference between suction and gravity drains. Approximately a third of the patients in both groups (post-craniotomy sounds group: 36 %, 17/47; group not reporting sounds: 31 %, 32/104), had postoperative CT scans for unrelated reasons: 73 % (8/11) of those with pneumocephalus experienced intracranial sounds, compared to 24 % (9/38) of those without pneumocephalus (p < 0.01). There was no significant association with craniotomy site or size, temporal bone drilling, bone flap replacement, or filling of the surgical cavity with fluid. CONCLUSIONS: Sounds in the head after cranial surgery are common, affecting 31 % of patients. This is the first study into this subject, and provides valuable information useful for consenting patients. The data suggest pneumocephalus as a plausible explanation with which to reassure patients, rather than relying on anecdotal evidence, as has been the case to date.