Hearing disorders in brainstem lesions.

Auditory processing can be disrupted by brainstem lesions. It is estimated that approximately 57% of brainstem lesions are associated with auditory disorders. However diseases of the brainstem usually involve many structures, producing a plethora of other neurologic deficits, often relegating "auditory symptoms in the background." Lesions below or within the cochlear nuclei result in ipsilateral auditory-processing abnormalities detected in routine testing; disorders rostral to the cochlear nuclei may result in bilateral abnormalities or may be silent. Lesions in the superior olivary complex and trapezoid body show a mixture of ipsilateral, contralateral, and bilateral abnormalities, whereas lesions of the lateral lemniscus, inferior colliculus, and medial geniculate body do not affect peripheral auditory processing and result in predominantly subtle contralateral abnormalities that may be missed by routine auditory testing. In these cases psychophysical methods developed for the evaluation of central auditory function should be employed (e.g., dichotic listening, interaural time perception, sound localization). The extensive connections of the auditory brainstem nuclei not only are responsible for binaural interaction but also assure redundancy in the system. This redundancy may explain why small brainstem lesions are sometimes clinically silent. Any disorder of the brainstem (e.g., neoplasms, vascular disorders, infections, trauma, demyelinating disorders, neurodegenerative diseases, malformations) that involves the auditory pathways and/or centers may produce hearing abnormalities.

Future advances.

Future advances in the auditory systems are difficult to predict, and only educated guesses are possible. It is expected that innovative technologies in the field of neuroscience will be applied to the auditory system. Optogenetics, Brainbow, and CLARITY will improve our knowledge of the working of neural auditory networks and the relationship between sound and language, providing a dynamic picture of the brain in action. CLARITY makes brain tissue transparent and offers a three-dimensional view of neural networks, which, combined with genetically labeling neurons with multiple, distinct colors (Optogenetics), will provide detailed information of the complex brain system. Molecular functional magnetic resonance imaging (MRI) will allow the study of neurotransmitters detectable by MRI and their function in the auditory pathways. The Human Connectome project will study the patterns of distributed brain activity that underlie virtually all aspects of cognition and behavior and determine if abnormalities in the distributed patterns of activity may result in hearing and behavior disorders. Similarly, the programs of Big Brain and ENIGMA will improve our understanding of auditory disorders. New stem-cell therapy and gene therapies therapy may bring about a partial restoration of hearing for impaired patients by inducing regeneration of cochlear hair cells.

Conscious awareness in patients in vegetative states: myth or reality?

Do vegetative state (VS) and minimally conscious state (MCS) patients experience emotions and have conscious awareness of themselves and their surroundings? Can neuroimaging clarify these questions? Neuroimaging responses to stimuli are classified into four levels: level 0 indicates no response; level 1 indicates responses limited to the primary sensory cortices; level 2 indicates activation of primary sensory cortices and higher-order associative areas; level 3 indicates activation of cortical regions to either mental imagery tasks or high-level language stimuli requiring distinction of ambiguous from unambiguous words. Level 0 or level 1 was noted in 125 of 193 VS patients (65 %) and 46 of 121 MCS patients (38 %), suggesting no evidence of conscious awareness. Level 2 or level 3 was observed in 68 of 193 VS patients (35 %) and 75 of 121 MCS patients (62 %), indicating some cognitive processing. These data may denote the presence of conscious awareness or may simply identify neuronal processing without phenomenological awareness. The pro and cons of these conflicting interpretations are discussed.

Alcmaeon of Croton's observations on health, brain, mind, and soul.

Alcmaeon of Croton (sixth-fifth century BC), a pre-Socratic physician-philosopher, introduced the concept that mind and soul are located in the brain. Alcmaeon made observations about seeing, hearing, tasting, and smelling and distinguished perception from understanding. Alcmaeon contributed two major ideas to natural sciences: (1) the brain is the seat of human intelligence, and (2) physicians should draw conclusions from empirical observations, an idea that implicitly rejects the alternative notion that science should depend on "divine revelation." Two thousand and five-hundred years later, these two insights remain true and guarantee Alcmaeon a place in the history of neuroscience.

Unresponsive wakefulness syndrome: a new name for the vegetative state or apallic syndrome.

BACKGROUND: Some patients awaken from coma (that is, open the eyes) but remain unresponsive (that is, only showing reflex movements without response to command). This syndrome has been coined vegetative state. We here present a new name for this challenging neurological condition: unresponsive wakefulness syndrome (abbreviated UWS). DISCUSSION: Many clinicians feel uncomfortable when referring to patients as vegetative. Indeed, to most of the lay public and media vegetative state has a pejorative connotation and seems inappropriately to refer to these patients as being vegetable-like. Some political and religious groups have hence felt the need to emphasize these vulnerable patients' rights as human beings. Moreover, since its first description over 35 years ago, an increasing number of functional neuroimaging and cognitive evoked potential studies have shown that physicians should be cautious to make strong claims about awareness in some patients without behavioral responses to command. Given these concerns regarding the negative associations intrinsic to the term vegetative state as well as the diagnostic errors and their potential effect on the treatment and care for these patients (who sometimes never recover behavioral signs of consciousness but often recover to what was recently coined a minimally conscious state) we here propose to replace the name. CONCLUSION: Since after 35 years the medical community has been unsuccessful in changing the pejorative image associated with the words vegetative state, we think it would be better to change the term itself. We here offer physicians the possibility to refer to this condition as unresponsive wakefulness syndrome or UWS. As this neutral descriptive term indicates, it refers to patients showing a number of clinical signs (hence syndrome) of unresponsiveness (that is, without response to commands) in the presence of wakefulness (that is, eye opening).

International Federation of Clinical Neurophysiology: recommendations for visual system testing.

This document presents recommendations and guidelines for the use of visual electrophysiological testing in the clinical assessment of visual pathway function.

Studies of human visual pathophysiology with visual evoked potentials.

Visual evoked potentials (VEPs) offer reproducible and quantitative data on the function of the visual pathways and the visual cortex. Pattern reversal VEPs to full-field stimulation are best suited to evaluate anterior visual pathways while hemi-field stimulation is most effective in the assessment of post-chiasmal function. However, visual information is processed simultaneously via multiple parallel channels and each channel constitutes a set of sequential processes. We outline the major parallel pathways of the visual system from the retina to the primary visual cortex and higher visual areas via lateral geniculate nucleus that receive visual input. There is no best method of stimulus selection, rather visual stimuli and VEPs' recording should be tailored to answer specific clinical and/or research questions. Newly developed techniques that can assess the functions of extrastriate as well as striate cortices are discussed. Finally, an algorithm of sequential steps to evaluate the various levels of visual processing is proposed and its clinical use revisited.

Visual plasticity and its clinical applications.

Normal visual development requires: 1) environmental factors (i.e. sensory experience) and 2) molecular programs that are genetically determined. Experience determines the development and preservation of visual cortical circuitry in accordance with Hebb's principle. The molecular and genetic mechanisms that regulate visual plasticity are less known. Visual experience induces postnatal neural activity that triggers a cascade of molecular processes including release of neurotrophic factors from target neurons and genetic expression of protein synthesis, transcription factors and neurotransmitters. The continuous sensory experience induces activity-dependent tuning of synaptic connections. The present knowledge permits some manipulation of plasticity and the induction of functional changes beneficial for vision. Three areas of intervention will be discussed: 1) enhancement of visual experience for children with ocular disorders, 2) re-organization of visual cortical maps, 3) retinal and cortical implants (prostheses) and transplants.