[Functional assessment of cochlear nerve and hearing]. / Exploration fonctionnelle du nerf cochléaire et de l'audition.

BACKGROUND/PURPOSE: Since otoneurosurgical pathologies are being diagnosed increasingly early, the functional prognosis of surgery is gaining in importance. Hearing and cochlear nerve exploration can be carried out using three principal methods: pure-tone and speech audiometry, evoked otoacoustic emissions (EOAEs), and brainstem auditory evoked potentials (BAEPs). The aim of this paper is to present the current knowledge on auditory functional assessment. MATERIALS AND METHODS: The medical and scientific literature from the Pubmed-Medline database was reviewed. Recently published related books were also included. RESULTS/DATA SYNTHESIS: Preoperative audiological evaluation makes it possible to assess pathology consequences on hearing. Depending on auditory status, conservative surgery, including intraoperative auditory monitoring, can be planned. From this perspective, prognostic factors for hearing preservation - such as the speech intelligibility score, the presence of transient EOAEs, and the presence of the BAEPs wave III - were identified. However, several surgical teams argue for a systematic conservative procedure, particularly when EOAEs are present, whatever the patient's preoperative hearing status. Postoperative audiological evaluation provides the opportunity for monitoring: in the short term, to detect complications early, through BAEPs recorded in the nonoperated ear; in the long term, to detect recurrence of the pathology. Moreover, the consequences of surgery on hearing can be assessed and long-term follow-up of auditory function can be carried out. Finally, the comparison of pre- and postoperative audiological status provides inductive feedback on surgery and electrophysiological monitoring, giving the otoneurosurgeon and neurophysiologist the opportunity to improve the entire procedure. CONCLUSION: Auditory functional assessment is essential for optimal care of tumor or functional pathologies involving the cochlear nerve and/or the cerebellopontine angle.

[Functional anatomy of the cochlear nerve and the central auditory system]. / Anatomie fonctionnelle du nerf cochléaire et du système auditif central.

The auditory pathways are a system of afferent fibers (through the cochlear nerve) and efferent fibers (through the vestibular nerve), which are not limited to a simple information transmitting system but create a veritable integration of the sound stimulus at the different levels, by analyzing its three fundamental elements: frequency (pitch), intensity, and spatial localization of the sound source. From the cochlea to the primary auditory cortex, the auditory fibers are organized anatomically in relation to the characteristic frequency of the sound signal that they transmit (tonotopy). Coding the intensity of the sound signal is based on temporal recruitment (the number of action potentials) and spatial recruitment (the number of inner hair cells recruited near the cell of the frequency that is characteristic of the stimulus). Because of binaural hearing, commissural pathways at each level of the auditory system and integration of the phase shift and the difference in intensity between signals coming from both ears, spatial localization of the sound source is possible. Finally, through the efferent fibers in the vestibular nerve, higher centers exercise control over the activity of the cochlea and adjust the peripheral hearing organ to external sound conditions, thus protecting the auditory system or increasing sensitivity by the attention given to the signal.

Lateralization of the effects of the benzodiazepine drug oxazepam on medial olivocochlear system activity in humans.

Benzodiazepines (Bzd) are known to interact with GABAergic inhibitory neurotransmission. Previous research on their effect on human auditory efferent pathways--through evoked otoacoustic emissions suppression by contralateral acoustic stimulation (CAS)--indicated a decrease in medial olivocochlear (MOC) efferent system inhibitory activity, after oral intake of oxazepam--representative of the Bzd drug class. To date, this pharmacological effect was only assessed in the right ear. Since a leftward asymmetry of Bzd receptors localization in human auditory cortex has been described recently, we explored in this study the hypothesis of an asymmetrical action of Bzd on MOC efferent functioning. The results revealed a significant difference of Bzd effect probing the right ear versus the left ear, with CAS-induced suppression being less effective in the right than left ear after oxazepam intake. This finding raises the question of possible neurochemical left-right asymmetry in the descending auditory pathways. The potential localization of this asymmetry is discussed.

Relationship between auditory intensity discrimination in noise and olivocochlear efferent system activity in humans.

Recent physiological data in animals suggest a role of the medial olivocochlear system (MOCS) in auditory intensity discrimination in noise. In this study, the existence of statistical relationships between intensity difference limens (IDLs) and MOCS functioning was tested in humans. IDLs were measured in conditions of quiet and of ipsilateral, contralateral, and dichotic noise. MOCS functioning was assessed through the contralateral evoked otoacoustic emission (EOAE) amplitude attenuation effect. A first finding was that IDLs measured in the presence of ipsilateral noise were reduced when contralateral noise was added. Furthermore, the observed shift in IDL appeared to be significantly correlated to the contralateral EOAE amplitude attenuation effect. These results support the hypothesis that MOCS functioning plays a role in intensity discrimination in dichotic noise in humans.

Difference in cochlear efferent activity between musicians and non-musicians.

The present study aimed to confirm and extend the finding, suggested by the results of a previous study, of different auditory neural efferent functioning in musicians compared with non-musicians. The activity of the medial olivocochlear bundle (MOCB), an auditory efferent subsystem, was measured through the contralaterally induced attenuation of the amplitude of evoked otoacoustic emissions (EOAEs) in two groups, one of musicians and one of non-musicians, paired for age and sex. The results indicated a statistically significant difference between the two groups, with the musicians showing greater amplitude reduction upon contralateral noise stimulation than non-musicians (U-test, U = 204, p < 0.025, n = 32). These results indicate greater MOCB activity in musicians than in non-musicians. The possible origins and implications of this finding are discussed.

Stronger bilateral efferent influences on cochlear biomechanical activity in musicians than in non-musicians.

The auditory sensory end organ is under the control of the central nervous system via efferent projections. Contralateral suppression of otoacoustic emissions (acoustic signatures of the cochlear biomechanical activity) provides a non-invasive approach to assess olivocochlear efferent activity in humans. Using this approach, the present study compared professional musicians with musically-inexperienced subjects. The results revealed stronger bilateral cochlear suppression, suggesting larger efferent influences in both ears, in musicians. Furthermore, in indicating no difference in left/right asymmetry of efferent-mediated suppression between the two groups, the present findings suggest that the observed differences in olivocochlear activity reflect bilaterally-enhanced activity of the cortical auditory structures in musicians rather than differences in cerebral hemispheric asymmetry between the two groups.

[Vertebro-basilar ischemic strokes and aseptic meningitis, late complications of Fabry's disease]. / Accidents ischémiques vertébrobasilaires et méningite aseptique compliquant une maladie de Fabry.

Fabry's disease, also called angiokeratoma corporis diffusum universal, is a rare cause of stroke in the young. We report the case of a 39-year-old man who presented with relapsing vertebro-basilar ischemic strokes. Biological tests showed the presence of an aseptic meningitis and a biological inflammatory syndrome, suggesting a particularly progressive illness. The possible mechanisms involved in this exceptional association are discussed.

[Hashimoto's encephalopathy: an anatomicoclinical observation]. / Encéphalopathie d'Hashimoto: une observation anatomo-clinique.

Hashimoto's encephalopathy (HE) is a rare neurological complication of chronic lymphocytic thyroiditis. As its clinical presentation is aspecific, other etiologies of acute encephalopathy have to be ruled out. We report the case of a 29-year old woman with neuropsychiatric signs preceding coma, myoclonus and epileptic seizures. Clinical and electroencephalographic features were consistent with the diagnosis of new variant of Creutzfeldt-Jakob disease. However, high titres of antithyroid antibodies in serum directed towards the diagnosis of HE. Despite oral steroids, the patient died five months later. Neuropathological findings ruled out spongiform encephalopathy and disclosed aspecific activated microglia. Our observation suggests that this process could be involved in the pathogenesis of HE. Even in the absence of clinical dysthyroidism, HE diagnosis has to be suspected in the settings of acute encephalopathy associated with seric antithyroid antibodies.

[Practical recommendations for management of the biopsy in an expansive intra-cerebral process]. / Recommandations pratiques pour la prise en charge de la biopsie d'un processus expansif intra-cérébral.

Diagnosis of an intracerebral mass requires a good collaboration between clinicians and pathologists. Different techniques may be necessary. The interest of cytological preparations and immunohistochemistry is reported. The main characteristic features of cerebral tumors are described and the differential diagnosis with reactive and inflammatory masses simulating neoplasia is discussed.

[Peripheral auditory lateralization]. / Existence d'une latéralisation de l'appareil auditif périphérique.

Neuropsychological studies have demonstrated that language perception is lateralized at the hemispheric level. There is also much evidence for a peripheral lateralization of the auditory system in humans. Spontaneous otoacoustic emissions are more frequent in the right than in the left ear. The medial olivo-cochlear system is also more functional in the right than in the left ear in normal subjects. The study of specific samples shows that this asymmetry is found in professional musicians which have more functional efferent system in both ears. A link is hypothesized between peripheral and central laterality because of a dysfunctional peripheral asymmetry in pathological cases which show hemispheric lateralization disorders. Consequences of those pathological data will be discussed.

[Related noise exposure and auditory consequence during transcranial magnetic stimulation: new insights and review of the literature]. / Exposition sonore et répercussions auditives au cours de la stimulation magnétique transcrânienne répétitive: données récentes et revue de la littérature.

Repetitive transcranial magnetic stimulation (rTMS) is a non-invasive neurostimulation tool with increasing therapeutic applications in neurology, psychiatry and in the treatment of chronic tinnitus, and with a growing interest in cognitive neuroscience. One of its side effects is the loud click sound generated simultaneously to the magnetic pulse, which depends both on the equipment and rTMS intensity. This impulse sound could transiently modify peripheral hearing mechanisms, and hence hearing thresholds, both in patients and in rTMS practitioners. Furthermore, if no precautions are taken, especially in subjects with several risks factors for hearing loss, it is possible that the repetition of exposure could lead to more definitive changes in hearing thresholds. These issues are often neglected, although they could have specific relevance in rTMS treatment for tinnitus or in auditory cognitive neuroscience. This review specifically deals with noise exposure during rTMS and its potential consequences on the auditory system. It provides several practical solutions to help minimize exposure.

Myasthenia gravis exacerbation or unmasking associated with telithromycin treatment.

Telithromycin, a new ketolide antibiotic, has been implicated in the exacerbation or unmasking of myasthenia gravis. This retrospective study presents two clinical reports and summarizes eight other suspected cases notified to the French pharmacovigilance system, highlighting a potentially life-threatening risk of telithromycin treatment in myasthenic patients. An important common feature was that, in seven cases, symptomatology occurred within 2 hours of first telithromycin intake--notably in cases of severe exacerbation.