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PubMed2003

Subcellular localisation, secretion, and post-translational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9.

Five missense mutations in the FCH/LCCL domain of the COCH gene, encoding the protein cochlin, are pathogenic for the autosomal dominant hearing loss and vestibular dysfunction disorder, DFNA9. To date, the function of cochlin and the mechanism of pathogenesis of the mutations are unknown. We have used the biological system of transient transfections of the entire protein coding region of COCH into several mammalian cell lines, to investigate various functional properties of cochlin. By western blot analysis of lysates prepared from transfected cells, we show that cochlin is a secreted protein. Immunocytochemistry shows concentrated localisation of cochlin in perinuclear structures consistent with the Golgi apparatus and endoplasmic reticulum, showing intracellular passage through these secretory compartments. We detected that cochlin is proteolytically cleaved between the FCH/LCCL domain and the downstream vWFA domains, resulting in a smaller cochlin isoform of approximately 50 kDa. Interestingly, this isoform lacks the entire mutation bearing FCH/LCCL domain. We have also shown that cochlin is N-glycosylated in its mature secreted form. Previous studies of the FCH/LCCL domain alone, expressed in bacteria, have demonstrated that three of four DFNA9 mutations cause misfolding of this domain. Characteristic eosinophilic deposits in DFNA9 affected inner ear structures could be the result of aberrant folding, secretion, or solubility of mutated cochlins, as in certain other pathological states in which misfolded proteins accumulate and aggregate causing toxicity. To examine the biological consequences of cochlin misfolding, we made separate constructs with three of the DFNA9 mutations and performed parallel studies of the mutated and wild type cochlins. We detected that mutated cochlins are not retained intracellularly, and are able to be secreted adequately by the cells, through the Golgi/ER secretory pathway, and also undergo proteolytic cleavage and glycosylation. These results suggest that DFNA9 mutations may manifest deleterious effects beyond the point of secretion, in the unique environment of the extracellular matrix of the inner ear by disrupting cochlin function or interfering with protein-protein interactions involving the FCH/LCCL domain. It is also possible that the mutations may result in aggregation of cochlin in vivo over a longer time course, as supported by the late onset and progressive nature of this disorder.

3T3 Cells↗

PubMed1997

[Computerized tomography in children and adolescents with suspected petrous bone abnormality].

PURPOSE: To demonstrate HRCT findings and their therapeutic relevance in suspected congenital hearing disorders. MATERIAL AND METHODS: It was checked in 96 young patients if HRCT findings of the temporal bone could explain functional findings. Furthermore, the therapeutic consequences were noted. RESULTS: Normal CT and normal functional findings were obtained in 49 temporal bones (TB). In conductive hearing loss (41 TB), dysplasias of the conducting apparatus (37 TB) and inflammatory changes (3 TB) were found. Combined hearing loss (18 TB) was clarified completely or partially in half the cases. There were 22 dysplasias of the inner ear, 3 dysplasias of the middle ear, 1 abandoned examination (2 TB), and 55 normal CT findings in senorineural hearing disorders (82 TB). 1 retardate had a malformation of the inner ear and, contralaterally, inflammatory middle ear. In cases of vestibular disorders (24 TB), 14 malformations of the inner ear were detected. An indication for an operation was given in 23 TB. In 22 TB, it was contraindicated. The CT was one preliminary examination to a cochlea implant in 19 patients. The therapy was carried on with hearing devices in the other patients. CONCLUSION: HRCT is an important method in diagnosis and therapeutic planning of suspected malformations of the temporal bone.

Adolescent↗

PubMed1988

Changing patterns of eye-head coordination during 6 h of optically reversed vision.

1) This study investigates the early development of adaptive changes in oculomotor function associated with coordinated eye-head tracking of the optically reversed image of an earth-fixed target seen through horizontally reversing dove prism goggles attached to the skull. 2) Two tasks comprised a) fixation of a single target during head rotation which causes the seen target's image to move in the direction of head motion by an amount exactly equal to the head movement itself (the 1-Target task), and b) change of gaze onto a displaced target with head free to move (2-Target task). 3) The 1-Target task requires the eyes to move in a direction opposite to that of the normal vestibulo-ocular reflex (VOR). The 2-Target task is identical, except that reorientation onto the new target calls for an initial saccadic eye movement in a direction opposite to that of the ensuing head movement, which is contrary to the normal pattern of eye-head coordination during gaze shifts. 4) Eye (EOG) and head (potentiometer) movements were continuously recorded (0-250 Hz) in an apparatus which permitted sudden, unexpected, electromagnetic braking of the head movement, either just before or during the intended manoeuvre. 5) Early adaptive strategies employed reduction of VOR gain, rearrangement of timing, amplitude and shape of "catch-up" saccades and the introduction of centrally programmed eye movements uncovered by the braking manoeuvres. 6) All of these phenomena were detectable in an initial series of 60 trials, in which the total exposure to visual-vestibular conflict was less than 30 s. They became more systematized and more marked after 6 h of active reversed vision experience. 7) Specifically, mean VOR gain, measured within the first 80 ms of head movement (deemed free of visuomotor influence), became markedly attenuated (25% in the first test series; 66% after 6 h of active vision-reversed exercise). In addition (not included in the above percentages) there were numerous occasions of complete absence of measurable VOR during head rotation, in both the first and final test series. 8) In the 1-Target task, the latency of the first "catch-up" saccade (re onset of head movement) tended to offset residual VOR by becoming shortened to the point of synchrony with head movement onset. This saccade (not present in control tests) continued to occur on those occasions when the head was unpredictably prevented from moving, and when head movements were made in the dark.(ABSTRACT TRUNCATED AT 400 WORDS)

Adaptation, Physiological↗

PubMed1976

Creeping attachment after free gingival grafts.

Free gingival grafts were placed in the lower anterior vestibular region of 30 patients each of whom had a gingival recession. According to the width of the recession and to the surgical procedure, the patients were divided into four groups. Group 1 showed a "narrow" (less than 3 mm) type of recession, while Group 2 had a "wide" type. In both of these groups the graft was placed over the denuded root. In Group 3 (narrow recession) and Group 4 (wide), a collar of vestibular gingiva was left in place on the lower margin of the denuded root at the moment of grafting. Measurements and photographs taken before and 1 month after surgery, 6 months and 2 years postoperatively, showed varying degrees of root covering by the phenomenon of creeping attachment. An optimal recovery of the gingival profile was obtained in the three patients of Group 1. In the other categories of patients the creeping of the attachment apparatus was relatively small (between 0 and 33%) within the 2 years of observation. The factors which seem to have a definite influence upon the phenomenon of creeping attachment are the following: width of the recession, position of the graft, bone resorption, position of the tooth and hygiene of the patient.

Adolescent↗

PubMedDate not supplied

[State of labyrinth tonic posture reflexes during administration to rabbits of microelements (copper, manganese and cobalt)].

The influence of varying doses of copper, manganese and cobalt chlorides on the labyrinthine tonic reflexes of rabbits was studied electromyographically. The presence and intensity of the reflexes were measured with respect to changes in the electric activity of muscles-extensors of forelimbs and occiput during the rotation of an animal along the bitemporal axis. Under the influence of cobalt (0.01 and 0.001 mg/kg), copper (0.1 mg/kg) and manganese (0.01 mg/kg) the threshold of adequate stimulation of the labyrinthine apparatus decreased and the time of the reflex response increased. This can be related to increased excitability of stem centers that are responsible for the realization of these reflexes. Larger doses of copper (1.0 mg/kg) and manganese (0.1 mg/kg) resulted in a significant and prolonged inhibition of tonic reflexes from the labyrinth to the limbs of rabbits. These data suggest an important role played by trace elements in the regulation and adjustment of the vestibular system.

Animals↗

PubMed2006

Differential expression of espin isoforms during epithelial morphogenesis, stereociliogenesis and postnatal maturation in the developing inner ear.

The espins are a family of multifunctional actin cytoskeletal proteins. They are present in hair cell stereocilia and are the target of mutations that cause deafness and vestibular dysfunction. Here, we demonstrate that the different espin isoforms are expressed in complex spatiotemporal patterns during inner ear development. Espin 3 isoforms were prevalent in the epithelium of the otic pit, otocyst and membranous labyrinth as they underwent morphogenesis. This espin was down-regulated ahead of hair cell differentiation and during neuroblast delamination. Espin also accumulated in the epithelium of branchial clefts and pharyngeal pouches and during branching morphogenesis in other embryonic epithelial tissues, suggesting general roles for espins in epithelial morphogenesis. Espin reappeared later in inner ear development in differentiating hair cells. Its levels and compartmentalization to stereocilia increased during the formation and maturation of stereociliary bundles. Late in embryonic development, espin was also present in a tail-like process that emanated from the hair cell base. Increases in the levels of espin 1 and espin 4 isoforms correlated with stereocilium elongation and maturation in the vestibular system and cochlea, respectively. Our results suggest that the different espin isoforms play specific roles in actin cytoskeletal regulation during epithelial morphogenesis and hair cell differentiation.

Animals↗

PubMed1975

The circular eye-tracking test. I. Simultaneous recording of the horizontal and vertical component of eye movement in the eye-tracking test.

Almost all of the conventional clinical reports on the eye-tracking test are made on the horizontal component of the smooth pursuit function of oculomotor system; few are made on the vertical component. One reason is that during the conventional vertical eye-tracking test the artifacts caused by movement of the upper eyelids often make it difficult to analyze the data. To overcome this problem the authors have developed a method to record simultaneously the horizontal and vertical components of eye movement in the eye-tracking test, and moreover, to reduce artifacts as much as possible. Our method, the circular eye-tracking test, is performed by means of an apparatus that provides a visual stimulus drawing a circle clockwise, at a rate of 120 degrees/sec, with a radius of 25.5 cm, and viewed at a distance of 70 cm.

Brain Diseases↗

PubMed1995

Head direction cell activity monitored in a novel environment and during a cue conflict situation.

1. Recent conceptualizations of the neural systems used during navigation have classified two types of sensory information used by animals: landmark cues and internally based (idiothetic; e.g., vestibular, kinesthetic) sensory cues. Previous studies have identified neurons in the postsubiculum and the anterior thalamic nuclei that discharge as a function of the animal's head direction in the horizontal plane. The present study was designed to determine how animals use head direction (HD) cells for spatial orientation and the types of sensory cues involved. 2. HD cell activity was monitored in the postsubiculum and anterior thalamic nucleus of rats in a dual-chamber apparatus in an experiment that consisted of two phases. In the first phase, HD cell activity was monitored as an animal moved from a familiar environment to a novel environment. It was hypothesized that if HD cells were capable of using idiothetic sensory information, then the direction of maximal discharge should remain relatively unchanged as the animal moved into an environment where it was unfamiliar with the landmark cues. In the second phase, HD cells were monitored under conditions in which a conflict situation was introduced between the established landmark cues and the animal's internally generated sensory cues. 3. HD cells were initially recorded in a cylinder containing a single orientation cue (familiar environment). A door was then opened, and the rat entered a U-shaped passageway leading to a rectangular chamber containing a different prominent cue (novel environment). For most HD cells, the preferred direction remained relatively constant between the cylinder and passageway/rectangle, although many cells showed a small (6-30 degrees) shift in their preferred direction in the novel environment. This directional shift was maintained across different episodes in the passageway/rectangle. 4. Before the next session, the orientation cue in the cylinder was rotated 90 degrees, and the animal returned to the cylinder. The cell's preferred direction usually shifted between 45 and 90 degrees in the same direction. 5. The rat was then permitted to walk back through the passageway into the now-familiar rectangle. Immediately upon entering the passageway, the preferred direction returned to its original (prerotation) orientation and remained at this value while the rat was in the rectangle. When the rat was allowed to walk back into the cylinder, one of three outcomes occurred: 1) the cell's preferred direction shifted, such that it remained linked to the cylinder's rotated cue card; 2) the cell's preferred direction remained unchanged from its orientation in the rectangle; or 3) the cell's preferred direction shifted to a new value that lay between the preferred directions for the rotated cylinder condition and rectangle. 6. There was little change in the HD cell's background firing rate, peak firing rate, or directional firing range for both the novel and cue-conflict situations. 7. Simultaneous recordings from multiple cells in different sessions showed that the preferred directions remained "in register" with one another. Thus, when one HD cell shifted its preferred direction a specific amount, the other HD cell also shifted its preferred direction the same amount. 8. Results across different series within the same animal showed that the amount the preferred direction shifted in the first Novel series was about the same amount as the shifts observed in subsequent Novel series. In contrast, as the animal experienced more Conflict series, HD cells tended to use the cylinder's cue card less as an orientation cue when the animal returned to the rotated cylinder condition from the rectangle. 9. These results suggest that HD cells in the postsubiculum and anterior thalamic nuclei receive information from both landmark and idiothetic sensory cues, and when both types of cues are available, HD cells preferentially use the landmark cues as long as they are perceived

Animals↗

PubMed1999

Mechano-electrical transduction in mice lacking the alpha-subunit of the epithelial sodium channel.

Sensory hair cells of the vertebrate inner ear use mechanically gated transducer channels (MET) to perceive mechanical stimuli. The molecular nature of the MET channel is not known but several findings suggested that the amiloride-sensitive epithelial Na+ channel, ENaC, might be a candidate gene for this function. In order to test this hypothesis, we examined knockout mice deficient in the alpha-subunit of ENaC, and therefore in ENaC function. First, neonatal alphaENaC(-/-) mice exhibited vestibular reflexes not different from wildtype littermates thus indicating normal vestibular function. We used organotypic cultures of cochlear outer hair cells from newborns to rescue the hair cells from the perinatal death of alphaENaC(-/-) mice. When hair bundles of cochlear outer hair cells of alphaENaC(-/-) mice were mechanically stimulated by a fluid jet in whole cell voltage clamp experiments, transducer currents were elicited that were not significantly different from those of alphaENaC(+/-) or (+/+) cochlear outer hair cells. These results suggest that the vertebrate mechano-electrical transducer apparatus does not include the alpha-subunit of the epithelial Na+ channel.

Animals↗

PubMed1984

[The ultrastructure of the paratympanic organ (Vitali) in Gallus domesticus: preliminary studies].

The sensory epithelium of the paratypanic organ (Vitali) was studied by means of the electron microscope. Two kinds of cells are present. One type extends from the basement membrane to the surface of the epithelium; their nuclei are arranged close to the connective tissue and are surrounded by a pale cytoplasm. The distal part of these cells, which are denser and richer in organelles, possess microvilli. The cells of the second type are located above the basement membrane and are found between the upper parts of the cells of the first type. Their cytoplasm is rich in small round vesicles, free ribosomes and cisternae of rough endoplasmic reticulum are present especially in the infranuclear zone. The apical part contains a Golgi apparatus lysosomes and multive sicular bodies. At the apex each cell possesses a cuticular plate numerous stereocilia and one kinocilium. The stereocilia become increasingly longer from one side of the cell surface to the other and the kinocilium is situated on the side where the stereocilia are longest. Nervous fibers are present in the epithelium and are in close contact with the cells of the second type. The cells we have described are remarkably similar to the supporting and hair cells of the vestibular sensory epithelium.

Animals↗

PubMed1982

Taurine in the mammalian cerebellum: demonstration by autoradiography with [3H]taurine and immunocytochemistry with antibodies against the taurine-synthesizing enzyme, cysteine-sulfinic acid decarboxylase.

Taurine neurons and their dendrites and axons were visualized in the mammalian cerebellum by autoradiography, after in vivo injections of [(3)H]taurine directly into the cerebellar cortex or deep cerebellar nuclei, and by immunocytochemistry at the light- and electron-microscope levels with antibodies against cysteine-sulfinic acid decarboxylase (CSADCase; L-cysteine-sulfinate carboxylyase, EC 4.1.1.29). Uptake and sequestration of [(3)H]taurine labeled numerous Purkinje cell somata, primary dendrites, and axons; many granule cell somata, dendrites, and parallel fibers; stellate, basket, and Golgi cells; the larger neurons in all deep cerebellar nuclei; the largest neurons in the lateral vestibular nucleus; and, more rarely, Purkinje cell axonal terminals in the neuropil. The label at all sites was diminished by preinjection into the cerebellum of hypotaurine, p-chloromercuriphenylsulfonic acid, or beta-alanine, and was virtually eliminated by strychnine. Immunocytochemical labeling with polyclonal antibodies directed against CSADCase, the enzyme responsible for the synthesis of hypotaurine from cysteine sulfinic acid and taurine from cysteic acid, had a similar distribution. In electron micrographs, immunoreactivity within Purkinje cell somata and dendrites was localized to the Golgi apparatus, the inner plasma membrane, and condensed nonmembranous foci (120 nm in diameter) marked by clumps of peroxidase reaction product. Large Nissl bodies were usually not CSADCase immunoreactive. Numerous immunoreactive granule cells, dendrites, and parallel fibers were recognized. Pretreatment of the animals with colchicine increased the intensity of CSADCase immunoreactivity but did not change the number or distribution of labeled cells. These experiments indicate that taurine is synthesized and involved in a specific uptake process by cerebellar neurons. Neuroglial cells do not synthesize taurine but some neuroglia take up [(3)H]taurine. These findings call for a reexamination of the physiological function of taurine in the cerebellum. A hypothesis is proposed that taurine may be involved in the regulation of calcium, in dendritic spike generation, and in the inhibition of impulse propagation in major Purkinje cell dendrites.

Animals↗

PubMed2001

[Pendred's syndrome. Current features].

Introduction Pendred's syndrome is a recessive autosomal disease, traditionally defined as the association of deaf-mutism, goiter and dysfunctional iodide organization revealed by the perchlorate discharge test. It represents 4 to 10% of the causes of congenital hypoacusis. Although described more than a 100 years ago, the association of thyroid and cochleo-vestibular damage remained unclear for many years. Genetic abnormalities Progress in molecular biology has revealed that the disease is related to alterations in the PDS gene situated on chromosome 7. The PDS gene is responsible for the production of pendrine, protein involved in anion (l-, Cl-) transportation, notably in the apical pole of the thyreocyte and the cochlear duct, where the endolympha is produced. Practical implications The truncation of pendrine related to the genetic alterations be responsible for the morpho-functional alterations in the cochlear apparatus and the thyroid. In this perspective, Pendred's syndrome would appear as a genetic disorder in anion transportation.

Carrier Proteins↗

PubMed2000

Postsynaptic actin filaments at the giant mossy fiber-unipolar brush cell synapse.

The unipolar brush cell (UBC), a small interneuron occurring at high density in the granular layer of the mammalian vestibulocerebellum, receives a giant glutamatergic synapse from a single mossy fiber (MF) rosette, usually on a brush of dendritic branchlets. MF stimulation produces a current in the UBC several orders of magnitude greater in duration than at other glutamatergic synapses. We assumed that the cytoskeleton would have a special role in plasticity of the MF-UBC synapse. Neurofilaments and microtubules are enriched in the UBC somatodendritic compartment but are conspicuously absent in close proximity to the giant synapse, where standard electron microscopy reveals a granulo-flocculent material. Because osmium tetroxide fixation during sample preparation for standard electron microscopy destabilizes actin filaments, we hypothesized that this subsynaptic granulo-flocculent material is actin-based. After actin stabilization, we observed prominent, but loosely organized, bundles of microfilaments at the subsynaptic region of the MF-UBC synapse that linked the postsynaptic density with the cytoskeletal core of the dendritic branchlets. Confocal fluorescence microscopy and pre- and postembedding immunogold labeling with phalloidin and actin antibodies showed that these microfilaments consist of f-actin and contain little beta-actin. This extraordinary postsynaptic actin apparatus is ideally situated to form a dynamic framework for glutamate receptors and other postsynaptic molecules, and to mediate activity-dependent plastic rearrangements of the giant synapse.

Actin Cytoskeleton↗

PubMed1990

[Experimental endolymphatic hydrops and its related morphological and functional changes in guinea pigs].

The endolymphatic hydrops was induced successfully in 13 guinea pigs by blocking the endolymphatic duct and sac, and the morphological and functional alterations (especially on semicircular canal function) after hydrops were studied by means of ENG, EcochG and serial section techniques. Hydrops in the cochlear duct was evident in the apical and/or near apical turns, especially in earlier stages. Dilation of the saccule was seen in all samples, but was barely noticeable in the utricle and semicircular canal's endolymphatic space. Degeneration in the corti's apparatus, stria vasculi and spiral ganglion occurred late. No evident changes of the sensory components in the saccule, utricle and semicircular canals were seen under the light microscope. The hearing loss in earlier stages of hydrops was evident at lower frequencies: later stages showed effects at all frequencies. The cochlear recruitment and fluctuation phenomena were observed in a few of the operated animals. The value of -SP/CAP in a few animals was significantly after operation. The functional changes of semicircular canals were demonstrated by the semicircular paralysis (CP), directional preponderance (DP), vestibular recruitment (VR) and elevated threshold of nystagmus to angular acceleration stimuli. There was no significant correlation between the degrees of hearing loss and impaired semicircular function. The criteria of ENG elicited by the quantitative angular acceleration stimuli for evaluating the semicircular canal function in guinea pigs is proposed for the first time in our experiment.

Animals↗

PubMed1997

The unipolar brush cells of the mammalian cerebellum and cochlear nucleus: cytology and microcircuitry.

The unipolar brush cell (UBC) is a novel type of small neuron that is characterized by sets of morphological and chemical phenotypes. UBCs occur in the granular layer of the mammalian cerebellar cortex, particularly in folia of the vestibulocerebellum, and in the granule cell domains of the dorsal cochlear nucleus. The UBC is characterized by a single dendrite that terminates with a brush-like tip of dendrioles. The soma, the dendritic stem, and especially the dendrioles emit short, non-synaptic appendages. The dendrioles represent the main synaptic apparatus of the UBC and articulate tightly with a single mossy fiber rosette forming a glomerular array characterized by an extraordinarily extensive synaptic contact. Electron microscopic and electrophysiological observations indicate that the unusual synaptic ultrastructure may produce entrapment of neurotransmitter in the synaptic cleft. While ionotropic glutamate receptors are enriched in correspondence of the postsynaptic density, metabotropic glutamate receptors are situated extrasynaptically and are particularly enriched at the appendages, which usually do not bear synaptic junctions. Some of the UBCs receive their input from choline acetyltransferase-positive mossy rosettes originating from the vestibular nuclei, suggesting that ACh and glutamate are co-released at these synapses. The UBC brush occupies a glomerulus where granule cell dendrites are intermixed with the UBC dendrioles, both of which receive synapses from the same mossy fiber rosette and portions of the Golgi axonal plexus. In addition, the dendrioles are presynaptic to granule cell dendrites, forming dendrodendritic contacts that display features of excitatory synapses. Branches of the UBC axon in the granular layer bear large endings resembling mossy fibers. The UBCs may represent an extraordinary device for feedforward, excitatory links along the mossy fiber pathways of cerebellum and dorsal cochlear nucleus.

Animals↗

PubMed2001

[Skeletal configurations of cervical column during rest to motion transition: generalization that underlie the motor repertoire in rodents].

During forward locomotion, guinea pigs adopted a posture which, although different, was as stereotyped as at rest. The whole extent of the vertebral column was extended, parallel to the earth-horizontal plane. This result supports the hypothesis that, in mammals, gaze and postural control by the central nervous system would be simplified by the adoption of a limited number of skeletal configurations. They would be optimized for energy saving, biomechanical efficacy and limitation of the number of degrees of freedom of the skeletal apparatus. Transition between rest and forward locomotion displayed two prominent characteristics: the head was stabilized versus space throughout the whole transition and the modifications of the skeletal geometry were confined to the median plane and to two major articular regions. Such an organization revealed a fourth characteristic of the guinea pig postural control: the skeletal configurations retained by evolution would ensure that smooth transitions take place between rest and the various activities of its motor repertoire. Finally, our purely descriptive data shed new light on the architecture of the neuronal network which underlies the vestibular control of gaze and posture.

Animals↗

PubMed1991

Eye movement induced by lateral tilt and its clinical significance.

We have studied ocular reflexes caused by tilting stimulations in test subjects placed in an upright sitting position on a chair apparatus which tilts continuously. In healthy persons, a weak nystagmus can be observed of minor amplitude and showing a small number of beats. The actual incidence of nystagmus in the younger group was minimal, but it tended to increase with age. This occurrence is considered to be due to age-related degeneration of the otolithic organ. Patients with vertigo and/or dizziness clearly develop nystagmus. These responses are classified as follows: type I is of fixed direction: type II is of changed direction; and type III is a combination of the first and second types. Among the peripheral vestibular disorders, particularly involving patients with benign paroxysmal positional vertigo, a nystagmus of changed direction was found. This type was rarely seen in patients with other vestibular disorders, but was a common occurrence in healthy individuals who readily experienced motion sickness. These observations have led us to conclude that patients with benign paroxysmal positional vertigo have definite reactions which are similar to those of individuals with motion sickness, and that this depends on the susceptibility of the otolithic organs.

Adult↗

PubMed2002

Cochleovestibular impairment in pediatric Cogan's syndrome.

Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the long-term, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits.

Adolescent↗