Tumoración en el colículo inferior como causa de acúfeno unilateral / Tumor at inferior colliculus causing unilateral tinnitus

Resumen La patología del sistema nervioso central, habitualmente, no provoca síntomas auditivos unilaterales, ya que la vía auditiva central está formada por una red de conexiones cruzadas entre los diferentes núcleos que la forman. Además, hay que considerar que una lesión pequeña puede extenderse a más de una estructura provocando varios déficits neurológicos debido a la proximidad de los tractos y núcleos nerviosos. Las lesiones unilaterales circunscritas en el colículo inferior son infrecuentes. No obstante, se han descrito casos en los que lesiones unilaterales de diversas etiologías en esta localización causaban síntomas auditivos. Ya que la vía auditiva central es cruzada, síntomas auditivos detectados con más frecuencia afectaban concretamente a la capacidad de localización del sonido o la comprensión verbal. Presentamos el caso de un hombre de 44 años con acúfeno unilateral derecho de larga evolución, sin otra clínica asociada quien fue diagnosticado de un tumor en el colículo inferior derecho mediante resonancia magnética cerebral. Se exponen los hallazgos clínicos y radiológicos del caso.

Abstract Central nervous system diseases usually do not cause auditory symptoms because the central auditory pathway consists on a network of crossed connections between the different nuclei that form it. In addition, we must consider that a small lesion might extend to more than one structure producing many neurologic symptoms due to the proximity of tracts and nuclei in the midbrain. Unilateral circumscribed lesions at inferior colliculus are rare. Nevertheless, lesions at this location causing auditory symptoms have been described. Because of the crossed central auditory pathway, the most commonly detected auditory symptoms specifically affected the ability to locate sound or verbal comprehension. We present the case of a 44-year-old man with a long-term monoaural right-sided tinnitus without other complaints who was diagnosed of a tumour at right inferior colliculus by neuroimaging. Clinical and radiological findings of this case are discussed.

Antioxidant Treatments Recover the Alteration of Auditory-Evoked Potentials and Reduce Morphological Damage in the Inferior Colliculus after Perinatal Asphyxia in Rat.

Maturation of the auditory pathway is dependent on the central nervous system myelination and it can be affected by pathologies such as neonatal hypoxic ischemic (HI) encephalopathy. Our aim was to evaluate the functional integrity of the auditory pathway and to visualize, by histological and cellular methods, the damage to the brainstem using a neonatal rat model of HI brain injury. To carry out this morphofunctional evaluation, we studied the effects of the administration of the antioxidants nicotine, melatonin, resveratrol and docosahexaenoic acid after hypoxia-ischemia on the inferior colliculus and the auditory pathway. We found that the integrity of the auditory pathway in the brainstem was altered as a consequence of the HI insult. Thus, the auditory brainstem response (ABR) showed increased I-V and III-V wave latencies. At a histological level, HI altered the morphology of the inferior colliculus neurons, astrocytes and oligodendricytes, and at a molecular level, the mitochondria membrane potential and integrity was altered during the first hours after the HI and reactive oxygen species (ROS) activity is increased 12 h after the injury in the brainstem. Following antioxidant treatment, ABR interpeak latency intervals were restored and the body and brain weight was recovered as well as the morphology of the inferior colliculus that was similar to the control group. Our results support the hypothesis that antioxidant treatments have a protective effect on the functional changes of the auditory pathway and on the morphological damage which occurs after HI insult.

Reversible pure word deafness due to inferior colliculi compression by a pineal germinoma in a young adult.

We report a clinical case of a 19-year-old male patient who developed pure word deafness due to the local compressive effect of a pineal germinoma on the inferior colliculi of the quadrigeminal plate. After percutaneous radiation therapy the size of the tumor decreased significantly, while audiometry demonstrated a complete regression of the auditory deficit. Since pure word deafness is commonly attributed to temporal lesions, the inferior colliculi represent an exceptional site for these symptoms. The pathophysiological background and the scarce literature on pure word deafness, especially the one related to neoplasms of the tectal region, are briefly discussed.

Cytoarchitectural and functional abnormalities of the inferior colliculus in sudden unexplained perinatal death.

The inferior colliculus is a mesencephalic structure endowed with serotonergic fibers that plays an important role in the processing of acoustic information. The implication of the neuromodulator serotonin also in the aetiology of sudden unexplained fetal and infant death syndromes and the demonstration in these pathologies of developmental alterations of the superior olivary complex (SOC), a group of pontine nuclei likewise involved in hearing, prompted us to investigate whether the inferior colliculus may somehow contribute to the pathogenetic mechanism of unexplained perinatal death. Therefore, we performed in a wide set of fetuses and infants, aged from 33 gestational weeks to 7 postnatal months and died of both known and unknown cause, an in-depth anatomopathological analysis of the brainstem, particularly of the midbrain. Peculiar neuroanatomical and functional abnormalities of the inferior colliculus, such as hypoplasia/structural disarrangement and immunonegativity or poor positivity of serotonin, were exclusively found in sudden death victims, and not in controls. In addition, these alterations were frequently related to dysgenesis of connected structures, precisely the raphé nuclei and the superior olivary complex, and to nicotine absorption in pregnancy. We propose, on the basis of these results, the involvement of the inferior colliculus in more important functions than those related to hearing, as breathing and, more extensively, all the vital activities, and then in pathological conditions underlying a sudden death in vulnerable periods of the autonomic nervous system development, particularly associated to harmful risk factors as cigarette smoking.

Effects of chronic administration of efavirenz on the chromatophilic substance of the intracranial auditory relay centres of adult Wistar rats / Efectos de la administración crónica de efavirenz sobre la sustancia cromatofílica de los centros de relevo auditivos intracraneales de ratas Wistar adultas

The effects of chronic administration of efavirenz commonly used as part of highly active antiretroviral therapy (HAART) for the treatment of Human Immunodeficiency Virus (HIV) type-1 therapy on the chromatophilic substance of the intracranial auditory relay centre namely the inferior colliculus and medial geniculate body of adult wistar rats were carefully studied. The rats of both sexes (n=20), with an average weight of 200g were randomly assigned into treatment (n=10) and control (n=10) groups. The rats in the treatment group received 600 mg/70kg body weight of efavirenz dissolved in distilled water daily for 30 days through the orogastric tube. The control group received equal volume of distilled water daily for 30 days through the same route. The rats were fed with grower's mash obtained from Edo Feeds and Flour Mill Limited, Ewu, Edo state, Nigeria and given water liberally. The rats were sacrificed by cervical dislocation method on the thirty-first day of the experiment. The inferior colliculus and medial geniculate body were carefully dissected out and quickly fixed in 10 percent formal saline for histological study. The histological findings indicated that the treated sections of the inferior colliculus and medial geniculate body showed that the chromatophilics substances were less intensely stained as compared to the control. The parenchyme was vacuolated and with evidence of hypertrophy and more spaces between the axonal mesh around the sparsely distributed neurons as compared to the control group. The treated section of the inferior colliculus showed neurons with faintly stained chromatophilics substances in large, medium and small sized neurons while that of the medial geniculate body showed less intense and enlarge chromatophilics substances with some vacuolations. Chronic administration of efavirenz may therefore have an adverse effect on the chromatophilics substances of the inferior colliculus and medial geniculate body of adult wistar rats...

Fueron estudiados los efectos de la administración crónica del efavirenz, comúnmente utilizado como parte del tratamiento antirretroviral de gran actividad para el VIH tipo 1, sobre la sustancia cromatofílica del centro de relevo auditivo intracraneal, el colículo inferior y cuerpo geniculado medial, en ratas Wistar adultas. Ratas de ambos sexos (n = 20), con un peso promedio de 200g fueron asignadas aleatoriamente a tratamiento (n = 10) y control (n = 10). Las ratas del grupo tratado recibieron 600mg/70kg peso corporal de efavirenz disuelto en agua destilada durante 30 días a través de sonda orogástrica. El grupo de control recibió un volumen igual de agua destilada durante 30 días por la misma vía. Las ratas fueron alimentadas con puré agricultor obtenido de Edo Feeds and Flour Mill Limited, Ewu, estado de Edo, Nigeria y agua ad-libitum. Las ratas se sacrificaron por dislocación cervical el día 31. El colículo inferior y el cuerpo geniculado medial fueron disecados cuidadosamente y se fijaron en solución de formalina salina al 10 por ciento. Los hallazgos histológicos indicaron que en las secciones tratadas del colículo inferior y el cuerpo geniculado medial la sustancia cromatofílica fue menos intensamente teñidas en comparación con el control. El parénquima se vacuoló, con evidencia de hipertrofia y más espacios entre la red axonal alrededor de neuronas escasamente distribuidas en comparación con el grupo control. La sección tratada del colículo inferior mostró neuronas con sustancia cromatofílica débilmente teñida en las neuronas de tamaño grande, mediano y pequeño, mientras que las del cuerpo geniculado medial mostraron sustancia cromatofílica menos intensa, con algunas vacuolaciones amplias. La administración crónica de efavirenz puede tener un efecto adverso sobre las sustancias cromatofílica del colículo inferior y del cuerpo geniculado medial de ratas Wistar adultas. Se recomienda realizar estudios adicionales...

The impact of hearing experience on signal integration in the auditory brainstem: a c-Fos study of the rat.

In this study we investigated the pattern of c-Fos expression in anteroventral (AVCN) and dorsal cochlear nucleus (DCN) and central inferior colliculus (CIC) following electrical intracochlear stimulation (EIS) of anesthetized adult rats that were neonatally deafened. The animals never experienced acoustic sensations as their hair cells were destroyed by daily kanamycin injections between postnatal days 10 to 20, resulting in a rise of hearing threshold by about 90 dB. Unilateral EIS was applied through a cochlear implant inserted into the medial turn of the left cochlea and lasted for 45 or 73 min, 2, 3:15, or 5h. Following EIS at 50Hz, a high number of c-Fos positive nuclei were observed showing only marginal tonotopic order in ipsilateral AVCN, in DCN bilaterally, and in contralateral CIC. Quantifying the number of c-Fos positive nuclei in ipsilateral AVCN, we found a steady increase with stimulation time. By contrast, the population of neurons expressing c-Fos in DCN and CIC revealed a transient maximum at 73 min. A direct comparison with our previous study (Rosskothen-Kuhl, N., Illing, R.-B., 2010. Nonlinear development of the populations of neurons expressing c-Fos under sustained electrical intracochlear stimulation in the rat auditory brainstem. Brain Res. 1347, 33-41) reveals that absence of hearing experience has far-reaching consequences for the interneuronal communication within networks of the auditory brainstem. When hearing fails, EIS entails expression of c-Fos in populations of neurons that are much larger than normally, essentially disregard tonotopic order, and lack much of spatio-temporal variations seen in hearing-experienced rats.

Calcifying pseudoneoplasm of the inferior colliculus: an unusual location for a rare tumor: case report.

OBJECTIVE: Quadrigeminal plate lesions are rare and usually present with a silent clinical course. Tumors, vascular lesions, inflammatory and infectious processes have been described in this region. Calcifying pseudoneoplasms, also reported as fibro-osseous lesions, cerebral calculi, and brain stones, are unusual lesions in the central nervous system. They can be revealed by cranial radiography, computed tomography, and magnetic resonance imaging as calcified masses and should be differentiated from neoplastic, inflammatory, and vascular lesions. To the best of our knowledge, the occurrence of a calcifying pseudoneoplasm located at the quadrigeminal plate has not yet been reported. CLINICAL PRESENTATION: A 67-year-old woman with a 6-month history of several daily attacks of dizziness presented to our service. Magnetic resonance imaging studies revealed a tumor in the right inferior colliculus. This lesion was isointense on T1-weighted imaging, hypointense on T2-weighted imaging, and homogeneously enhanced with contrast. INTERVENTION: The lesion at the quadrigeminal plate was completely removed, and the patient was successfully treated without any new neurological deficit. At the time of follow-up, all preoperative symptoms had resolved. CONCLUSION: We report the first case of a calcifying pseudoneoplasm of the inferior colliculus. Complete surgical removal of this type of tumor is feasible. We propose surgical treatment in this location when this tumor becomes symptomatic.

Bilateral cochlear ablation in postnatal rat disrupts development of banded pattern of projections from the dorsal nucleus of the lateral lemniscus to the inferior colliculus.

Axonal projections from the dorsal nucleus of the lateral lemniscus (DNLL) distribute contralaterally in a pattern of banded layers in the central nucleus of the inferior colliculus (IC). The banded pattern of DNLL projections is already in the IC by onset of hearing in postnatal rat pups. Previously, it was shown that unilateral cochlear ablation in neonatal rat pups disrupted the banded pattern in IC for the projections of the DNLL contralateral to the ablation but not those of the DNLL ipsilateral to the ablation. In the present study, bilateral cochlear ablation or sham surgery was performed at postnatal day 9 (P9) after which rat pups were killed at P12 and the brains removed to study axonal projections of the DNLL. A lipophilic carbocyanine dye, 1,1'-dioctadecyl-3,3,3',3'-tetramethylindocarbocyanine perchlorate (DiI), was placed in the dorsal tegmental commissure of Probst to label decussating DNLL axons that end in the central nucleus of the contralateral IC. The distribution of labeled fibers across the central nucleus of the IC was analyzed in digital images by comparing the pattern of labeling with a sine model of periodic distribution of banded layers. In the control group, labeled axons formed a regular pattern of dense banded layers in IC. In the bilateral cochlear ablation group, labeled axons in the IC were distributed diffusely and there was little or no regular pattern of dense bands of axonal labeling. The influence of the cochlea on developing auditory circuits possibly mediated by activity-dependent mechanisms is discussed.

Reduction of inhibition in the inferior colliculus after inner hair cell loss.

Effects of carboplatin-induced partial loss of inner hair cells on rate-level functions of neurons in the central nucleus of the inferior colliculus of the same chinchillas before and 1-3 months after carboplatin treatment were examined. Partial inner hair cell loss caused only a small elevation of response thresholds but induced a significant increase in the proportion of monotonic and a significant decrease in the proportion of non-monotonic rate-level functions at the characteristic frequency of 210 multiunits. This indicates that inhibition in the inferior colliculus is reduced after sensory deafferentation. We conclude that some of the functional changes reported in the auditory cortex after peripheral deafferentation are already realized at the level of the inferior colliculus.

Distribution of Fos immunoreactivity in the rat brain after freezing or escape elicited by inhibition of glutamic acid decarboxylase or antagonism of GABA-A receptors in the inferior colliculus.

It has been shown that electrical stimulation of the central nucleus of the inferior colliculus (IC) at freezing or escape thresholds activates different neural circuits in the brain. Since electrical stimulation activates cell bodies and fibers of passage it is necessary to use chemical stimulation that activates only post-synaptic receptors. To examine this issue in more detail, we took advantage of the fact that GABAergic neurons exert tonic control over the neural substrates of aversion in the IC. Reduction of GABA transmission in this structure was performed with the use of semicarbazide - an inhibitor of the GABA synthesizing enzyme glutamic acid decarboxylase (GAD) - and the GABA-A receptor antagonist bicuculline. Depending on the dose employed local infusions of semicarbazide (6.0 microg/0.2 microl) or bicuculline (40 ng/0.2 microl) into this region caused freezing and escape, respectively. The results obtained showed that freezing behavior induced by semicarbazide was associated with an increase in Fos expression in the dorsomedial column of the PAG (dmPAG) only, while bicuculline-induced escape was related to widespread increase in Fos labeling, notably in the periaqueductal gray, hypothalamus nuclei, amygdaloid nuclei, the laterodorsal nucleus of thalamus (LD), the cuneiform nucleus (CnF) and the locus coeruleus (LC). Thus, the present data support the notion that freezing and escape behaviors induced by GABA blockade in the IC are neurally segregated: acquisition of aversive information of acoustic nature from the IC probably uses the dmPAG column as a relay station to higher brain centers whereas bicuculline-induced escape activates structures involved in both sensory processing and motor output of defensive behavior. These results support the existence of distinct neural circuits mediating the sensory and motor responses of the defense reaction. The extent of the brain activation during freezing appears to be limited to the anatomical connections of the dmPAG, whereas an overall activation of the limbic system predominates during escape behavior induced by IC stimulation.

Auditory agnosia caused by a tectal germinoma.

The authors describe a patient with auditory agnosia caused by a tectal germinoma. Despite having normal audiometric tests, the patient failed to recognize words and musical characters. On head MRI, the inferior colliculi were infiltrated by tumor. Neuropsychological tests revealed severe impairment in recognition of environmental sounds and words, defective musical perception, and stop consonant-vowel discrimination. Inferior colliculus may play a role in the analysis of sound properties.

c-Fos immunohistochemical mapping of the audiogenic seizure network and tonotopic neuronal hyperexcitability in the inferior colliculus of the Frings mouse.

The Frings mouse is a model of audiogenic seizure (AGS) susceptibility. The genetic locus responsible for the AGS phenotype in the Frings mouse has been named monogenic audiogenic seizure-susceptible (MASS1). MASS1 is unique in that it is one of only two identified seizure loci that are not associated with an ion channel mutation. Furthermore, Frings mice display a robust AGS phenotype demonstrating very high and prolonged susceptibility to sound-induced tonic extension seizures. The purpose of this investigation was to use c-Fos immunohistochemistry to map the brain structures involved in the Frings AGS and to examine neuronal hyperexcitability in the inferior colliculus, the brain structure that is recognized as the site of AGS initiation. AGS mapping revealed that intense seizure-induced neuronal activation was mostly limited to structures involved in a brainstem seizure network, including the external and dorsal nuclei of the inferior colliculus, as observed in other AGS rodents. Acoustically induced c-Fos expression in the central nucleus of the inferior colliculus to sub-AGS threshold tone stimulations displayed a greater level of neuronal activation in AGS-susceptible Frings, DBA/2J and noise-primed C57BL/6J mice compared to AGS-resistant C57BL/6J and CF1 mice. The AGS-susceptible mice also displayed c-Fos immunoreactivity that was more focused within the tonotopic response domain of the inferior colliculus compared to AGS-resistant mice. Furthermore, Frings mice displayed significantly greater tonotopic hyper-responsiveness compared to other AGS-susceptible mice.

Central hearing loss with a bilateral inferior colliculus lesion.

A case is presented of a 43-year-old man with bilateral lesions of the inferior colliculus (IC) due to central nervous system lymphoma. Our patient was found to have relatively normal pure-tone averages with severely reduced bilateral word recognition scores. This case illustrates that the presentation of a partial bilateral IC lesion may be of severely reduced word recognition accompanied by nearly normal pure-tone hearing.

Dural arteriovenous fistula after craniotomy for pilocytic astrocytoma in a patient with protein S deficiency.

BACKGROUND: We report an unusual case, which may provide insight into the etiology and pathogenesis of dural arteriovenous malformation. CASE DESCRIPTION: A 24-year-old woman presented with hemorrhage into a pilocytic astrocytoma of the collicular plate. Angiography was normal and the tumor was surgically resected. She developed sigmoid sinus thrombosis and a transverse/sigmoid sinus dural arteriovenous fistula 11 months after this and was found to have protein S deficiency. The fistula was not treated. Angiography 4 years later was unchanged. CONCLUSION: This report illustrates an acquired etiology of a dural arteriovenous fistula. To our knowledge this is the first reported case of postoperative sigmoid sinus thrombosis along with arteriovenous fistula in a patient with previously undetected protein S deficiency.

Cross-modal neuroplasticity in neonatally enucleated hamsters: structure, electrophysiology and behaviour.

Potential auditory compensation in neonatally bilaterally enucleated Syrian hamsters was explored anatomically, electrophysiologically and behaviourally. Gross morphology of the visual cortex appeared normal and no obvious cytoarchitectural malformation was discerned. However, enucleation induced a significant increase in the spontaneous firing rate of visual cortex cells. Further, auditory stimuli elicited field potentials and single unit responses in the visual cortex of enucleated, but not normal, animals. About 63% of the cells isolated in the visual cortex of 16 enucleated hamsters responded to at least one type of auditory stimulus. Most of the responses were less vigorous and less time-locked than those of auditory cortex cells, and thresholds were typically higher. Projection tracing with WGA-HRP disclosed reciprocal connections between the visual cortex and the dorsal lateral geniculate nucleus in both intact and enucleated animals. However, in the enucleated animals retrogradely labelled cells were also found in the inferior colliculus, the major midbrain auditory nucleus. Behaviourally determined auditory sensitivity across the hearing range did not differ between enucleated and intact hamsters. Minimum audible angle, as determined by a conditioned suppression task, ranged from around 17 to 22 degrees, with no significant difference between normal and enucleated animals. The two groups also did not differ with regard to the direction of their unconditioned head orientating response to intermittent noise. However, the enucleated animals showed a more vigorous response and were slower to habituate to the noise. These results show that bilateral enucleation of newborn hamsters results in auditory activation of visual targets, in addition to the typical activation of the intact auditory pathway. Behaviourally it appears that enucleated hamsters, compared with their normal littermates, are slower to habituate in their response to an unexpected source of sound.

Activity-dependent plasticity in the adult auditory brainstem.

Over the past few years we have studied the plasticity of the adult auditory brainstem in the rat following unilateral changes to the pattern of sensory activation, either by intracochlear electrical stimulation or by deafening. We discovered that modifications to afferent activity induced changes in the molecular composition and cellular morphology throughout the auditory brainstem, including its major centers: the cochlear nucleus complex, the superior olivary complex, and the inferior colliculus. The time window studied ranged from 2 h to over 1 year following induction of changes to afferent activity. The molecular markers employed include the NMDA receptor subunit type 1, the cAMP response element binding protein (CREB), the immediate early gene products c-Fos, c-Jun and Egr-1, the growth and plasticity-associated protein GAP-43 and its mRNA, the calcium binding protein calbindin, the cell adhesion molecule integrin-alpha(1), the microtubule-associated protein MAP-1b, and the neurofilament light chain (NF-L). As a consequence of the specific electrical stimulation of the auditory afferents or the loss of hearing, a cascade of events is triggered that apparently modifies the integrative action and computational abilities of the central auditory system. An attempt is made to relate the diverse phenomena observed to a common molecular signaling network that is suspected to bridge sensory experience to changes in the structure and function of the brain. Eventually, a thorough understanding of these events will be essential for the specific diagnosis of patients, optimal timing for implantation, and suitable parameters for running of a cochlear implant or an auditory brainstem implant in humans. In this report an overview of the results obtained in the past years in our lab is presented, flanked by an introduction into the history of plasticity research and a model proposed for intracellular signal cascades related to activity-dependent plasticity.

Changes in cochlear electrical stimulation induced Fos expression in the rat inferior colliculus following deafness.

Fos immunoreactive (IR) staining was used to examine changes in excitatory neuronal activity in the rat inferior colliculus (IC) between normal hearing and 21 day deaf rats evoked by basal or apical monopolar cochlear electrical stimulation. The location of evoked Fos IR neurons was consistent with expected tonotopic areas. The number of Fos IR cells increased as stimulation intensity increased in both normal and 21 day deaf animals. Stimulation at 1. 5x threshold evoked fewer Fos IR cells in 21 day deafened animals compared to normal hearing animals. At 5x and above, however, significantly increased numbers of Fos IR neurons (in a larger grouping) were evoked in 21 day deafened animals compared to normal hearing animals. Another group of animals had 7 days of deafness followed by 14 days of chronic basal cochlear electrical stimulation. In this group basal monopolar stimulation at 5x evoked not only a greater number of Fos IR neurons, compared to normal hearing animals, but the location of their grouping was slightly shifted to a more dorso-lateral region in the contralateral IC, compared to the normal hearing and 21 day deaf groups. These observations indicate that both deafness and chronic electrical stimulation may alter central auditory processing.

Effects of intracochlear factors on spiral ganglion cells and auditory brain stem response after long-term electrical stimulation in deafened kittens.

Using an animal model, we have studied the response of the auditory brain stem to cochlear implantation and the effect of intracochlear factors on this response. Neonatally, pharmacologically deafened cats (100 to more than 180 days old) were implanted with a 4-electrode array in both cochleas. Then, the left cochlea of each cat was electrically stimulated for total periods of up to 1000 hours. After a terminal (14)C-2-deoxyglucose (2DG) experiment, the fraction of the right inferior colliculus with a significant accumulation of 2DG label was calculated. Using 3-dimensional computer-aided reconstruction, we examined the cochleas of these animals for spiral ganglion cell (SGC) survival and intracochlear factors such as electrode positions, degeneration of the organ of Corti, and the degree of fibrosis of the scala tympani. The distribution of each parameter was calculated along the organ of Corti from the basal end. There was a positive correlation between SGC survival and the level of fibrosis in the scala tympani, and a negative correlation between SGC survival and the degree of organ of Corti degeneration. Finally, there was a negative correlation between the 2DG-labeled inferior colliculus volume fraction and the degree of fibrosis, particularly in the 1-mm region nearest the pair of electrodes, and presumably in the basal turn.