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Spontaneous cerebrospinal fluid otorrhoea via oval window: an obscure cause of recurrent meningitis.

Spontaneous cerebrospinal fluid (CSF) leak via the oval window is uncommon and can result in recurrent bacterial meningitis. Current understanding of spontaneous CSF otorrhoea is reviewed and a diagnostic algorithm is presented. A seven-year-old boy presented with bilateral congenital deafness and recurrent meningitis. High-resolution computed tomography (HRCT) of the temporal bone showed a labyrinthine deformity and communication between the internal auditory canal (IAC) and the cochlea. Subtotal petrosectomy with closure of the external acoustic meatus and eustachian tube was performed. Post-operatively, the child had no further episodes of meningitis. This rare and obscure cause of recurrent childhood meningitis requires a high index of suspicion and the use of diagnostic tools, especially HRCT.

Cerebrospinal Fluid Otorrhea↗

Labyrinthine otosclerosis and sensorineural deafness. Pathologic findings of the spiral ligament.

In severe labyrinthine otosclerosis, spiral ligament changes occur predominantly adjacent to the stria vascularis, and consist of a decrease or loss of the capillary-perilymph system. It is suggested that this results in increased concentrations of metabolic products in the cochlear fluids that can no longer be absorbed effectively. If vascular shunts form they might represent collateral channels, with the flow of blood from the cochlea into the otosclerotic focus, bypassing the deficient capillary network in the spiral ligament. The zone of dense collagenization is described as a widening of the soft tissues endosteum of the cochlear capsule due to erosion of the bone, with the bone surface now set further back. This may result in greater mobility or loosening of the suspension mechanism of the basilar membrane.

Aged↗

Computed tomography and magnetic resonance imaging of the inner ear.

The majority of temporal bone radiographic studies are obtained either for middle ear and mastoid disease or in the evaluation of retrocochlear pathology. With recent technologic advances, diagnostic imaging of the inner ear has developed an increasing role in the evaluation and management of diseases that affect the cochlea, semicircular canals, and the vestibular and cochlear aqueducts. High-resolution computed tomography (CT) provides excellent detail of the osseous labyrinth, whereas magnetic resonance imaging (MRI) generates images derived from the membranous labyrinth and its associated neural elements. Optimal techniques for obtaining high quality CT and MRI images of the normal and diseased inner ear are presented. CT has proved useful in the evaluation of inner ear malformations, cochlear otosclerosis, labyrinthine fistulization from cholesteatoma, translabyrinthine fractures, otic capsule osteodystrophies, in the assessment of cochlear patency before cochlear implantation, and in the localization of prosthetic devices such as stapes wires and cochlear implants. While MRI produces discernible images of the soft tissue and fluid components of the inner ear, it has yet to demonstrate any unique advantages in the evaluation of inner ear disease. However, MRI produces excellent and highly useful images of the audiovestibular and facial nerves, cerebellopontine angle, and brain.

Cholesteatoma↗

Magnetic resonance imaging of the intratemporal facial nerve in idiopathic facial paralysis in the dog.

The most common cause of peripheral facial nerve paralysis in dogs, in the absence of otitis media, is thought to be idiopathic. Gadolinium-enhanced (Gd) magnetic resonance (MR) imaging has been used to study peripheral facial weakness in humans with a wide variety of disorders, including Bell's palsy, the clinical equivalent of idiopathic facial nerve paralysis in dogs. Gd-MR imaging may be useful to demonstrate abnormal enhancement of the intratemporal facial nerve. The aim of this study was to define the role of the Gd-MR imaging in dogs with idiopathic facial nerve paralysis, with regard to pattern of enhancement, and to search for prognostic information. Six dogs with peripheral facial nerve paralysis, followed between 2003 and 2005, were studied. Physical and neurologic examinations, as well as clinical tests, were performed, including routine hematology, serum biochemistry, thyroid screening, cerebrospinal fluid analysis, and MR imaging. The time interval between the onset of the clinical signs, the progress of the disease, and the final recovery was noted in each dog. The following four intratemporal segments of the facial nerve were analyzed: internal acoustic meatus, labyrinthine segment/geniculate ganglion, tympanic segment, and mastoid segment. Along its length, contrast enhancement was found in four dogs. In this group, contrast enhancement of the facial nerve was found in all segments of two dogs, in three segments of one dog, and in one segment of the other dog. In the four dogs with enhancement, one recovered completely in 8 weeks and three have not recovered completely. The two dogs without evidence of enhancement recovered completely in an average time of 4 weeks.

Animals↗

Mechanical indentation of the vestibular labyrinth and its relationship to head rotation in the toadfish, Opsanus tau.

1. In the present study we examine the response of the semicircular canal of the toadfish (Opsanus tau) to head rotation and to mechanical indentation of the membranous labyrinth. The relationship between the two stimuli is described by a new elastohydrodynamic model that delineates the three-dimensional (3-D) spatiotemporal distribution of endolymph pressure and flow. In vivo electrophysiological recordings of primary afferents supplying the horizontal canal (HC) were employed to validate the model predictions. Data were collected from 213 afferents in 18 fish during independent head rotation. HC indentation, utricle (U) indentation, and paired stimuli. To quantify the afferent response and the relationship between the applied sinusoidal stimuli, the magnitude (gain) and temporal relationship (phase) of the first harmonic of modulation were calculated and compared with theoretical predictions. 2. A mathematical based extensively on the 3-D morphology of a toadfish labyrinth and the physical properties of endolymph is presented to describe the relationship between head rotation and mechanical indentation. All model parameters specifying labyrinthine morphology and physical properties of endolymph are known; the model contains no free parameters. New results are independent of the structural properties of the cupula. The analysis employs an asymptotic solution of the Navier-Stokes equations in the three toroidal ducts that includes the 3-D fluid-structure interaction taking place within the enlarged ampulla. The solution addresses the differential pressure (delta P) acting across the cupula and the dilatational pressure acting on both sides of the cupula. The analysis quantifies the hydrodynamics of the HC for mechanical indentations of the long and slender portion of the canal duct (HC indentation) and the U (U indentation). Results specifically relate the indentation stimuli to head rotation. Linear commutations of HC indentation, U indentation, and rotation stimuli are analyzed by matching delta P acting across the cupula for the three stimulus modalities. 3. HC afferents show a linear correspondence between HC indentation, U indentation, and rotation stimuli. Specific experimental results for sinusoidal stimuli at frequencies < 2 Hz show 1) +/- 1 micron-HC indentation commutates with +/- 4 degrees/s rotation, 2) + 1-micron HC indentation commutates with -/+ 15-microns U indentation, and 3) -/+ 15-microns U indentation commutates with +/- 4 degrees/s rotation. These results were obtained by adjusting the relative amplitude and phase of two stimuli presented simultaneously to achieve destructive interaction that minimizes the afferent modulation (balanced). Equivalent results were obtained using afferent responses to the stimuli applied independently.(ABSTRACT TRUNCATED AT 400 WORDS)

Afferent Pathways↗

Petrous apex cholesteatoma: diagnostic and treatment dilemmas.

The diagnosis and treatment of petrous apex cholesteatoma is a difficult surgical challenge. This study is a review of 14 cases of cholesteatoma involving the petrous apex. These cholesteatomas originated as a congenital primary lesion or secondary to an acquired lesion. The cases were evaluated according to the clinical features, the intraoperative findings, the radiological findings, and the surgical approaches. In this series, 83% of the patients presented with hearing loss and 50% presented with facial nerve weakness or paralysis (House grade II to VI). Intraoperative and radiological features revealed frequent direct labyrinthine and supralabyrinthine cell spread. The transpetrous surgical approach was used in all cases. The main factors affecting the surgical approach to be adopted are the inaccessible nature of the petrous apex, the extent of disease, the degree of facial nerve function, and the need for the prevention of cerebrospinal fluid leaks and the recurrence of the lesion.

Adult↗

CT and MR imaging characteristics of intravestibular lipoma.

BACKGROUND AND PURPOSE: Intracranial lipomas are uncommon congenital malformations that most often are asymptomatic. A rare subset of intracranial lipomas arises within the vestibule of the inner ear, which can cause sensorineural hearing loss. We present the CT and MR imaging characteristics of lipomas within the vestibule of the inner ear and propose a pathogenetic mechanism for this malformation. METHODS: We retrospectively reviewed five cases of vestibular lipoma. T1-weighted axial conventional spin-echo and T2-weighted axial fast spin-echo sequences were performed in all five cases. Four patients underwent T1-weighted fat-saturated imaging, and one underwent CT scanning with Hounsfield units measured. RESULTS: Four female patients ranging in age from 10 to 40 years and one 26-year-old male patient had sensorineural hearing loss. Two patients had progressive high-frequency loss; one, sudden-onset high-frequency loss; and two, congenital high-frequency loss. All had lesions within the vestibule that displayed hyperintensity on T1-weighted images, isointensity to slight hypointensity on T2-weighted fast spin-echo images, hypointensity with fat saturation, and fat attenuation on CT scans. Three of the cases were associated with lipoma of the cerebellopontine angle. In none of these cases were the cerebellopontine angle and labyrinthine lipomas contiguous. CONCLUSION: Intravestibular lipoma is considered when a focal hyperintense lesion is identified within the vestibule of the inner ear on T1-weighted MR images. Lesion hypointensity on fat-saturated T1-weighted MR images verifies its lipomatous nature and separates it from intralabyrinthine hemorrhage or highly proteinaceous fluid.

Adolescent↗

Spontaneous CSF otorrhea caused by abnormal development of the facial nerve canal.

In two patients with surgically proved CSF fistula through the facial nerve canal, MR and CT examinations showed smooth enlargement of the geniculate fossa with CSF signal. In the clinical setting of CSF otorrhea or rhinorrhea, the presence of an enlarged labyrinthine facial nerve canal and enlarged geniculate fossa on CT scans and CSF intensity on MR images strongly suggests a CSF fistula through the facial nerve canal.

Adult↗

Hyperactivation of mammalian sperm.

Mammalian sperm commonly show hyperactivated motility just before fertilization. The movement of hyperactivated sperm appears different in fluids of different viscosity and elasticity and in different species, but basically it involves an increase in flagellar bend amplitude and, usually, beat asymmetry. Hyperactivation may be critical to the success of fertilization, because it enhances the ability of sperm to detach from the wall of the oviduct, to move around in the labyrinthine lumen of the oviduct, to penetrate mucous substances and, finally, to penetrate the zona pellucida of the oocyte. Presumably, a signal or signals exist in the oviduct to initiate hyperactivation at the appropriate time; however, none have yet been identified with certainty. While the signal transduction cascade regulating hyperactivation remains to be completely described, it is clear that calcium ions interact with the axoneme of the flagellum to switch on hyperactivation. Although hyperactivation often occurs during the process of capacitation, divergent pathways regulate the two events.

Animals↗

The developmental pathology of maternally derived Thp fetuses.

The Thair pin (Thp) mutation is a deletion of 5 centimorgans of chromosome 17 in the mouse. When the mutant chromosome is passed to the fetus through the female, the heterozygous fetuses (Thp/+) die in utero. If the chromosome is passed through the male, the heterozygotes are viable and display a short-tailed phenotype. These maternally derived mutant embryos provide an excellent model system to study the effects of an incomplete female genome on development. The results reported here describe the findings of a pathological study of the affected fetuses from day 14 of development to birth. These observations indicate that the maternally derived Thp fetuses die in utero of congestive heart failure. The mutant fetuses displayed an enlarged heart, primarily the right side, and other cardiovascular abnormalities including ventricular septal defects, aortic stenosis, pulmonary artery dilation, and dilation of the venous circulatory system. The fetuses also displayed abnormal accumulation of extrafetal fluid in the visceral yolk sac and amion, as well as massive subcutaneous edema and ascites. The Thp fetuses were often pale and anemic, and they showed a decreased number of red blood cells per unit volume of blood and an increase in circulating nucleated red blood cells. Defects in the development of the labyrinthine and spongiotrophoblast regions of the placenta were also observed. The pathogenesis of the defects is discussed.

Animals↗

Scanning electron microscopy of the juxtaglomerular apparatus in the freeze-fractured rat kidney.

The morphology of the juxtaglomerular apparatus of the rat kidney was investigated by scanning electron microscopy of the freeze-fractured tissue. The macula densa cells have a dome-shaped apical surface abundantly provided with short microvilli. Their basal and lateral aspects may show labyrinthine spaces which are consistently more developed than in the epithelium across the tubular lumen. The Goormaghtigh cells display the well known complex pattern of cytoplasmic extensions forming the lacis. Fractured juxtaglomerular cells show distinct nuclei and well defined secretory granules. The well developed labyrinth of the macula densa suggest a considerable trans-epithelial fluid transfer at this level.

Animals↗

Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment.

We report the use of MRI in the diagnosis, follow-up and therapeutic management of three cases of intralabyrinthine Schwannoma. The diagnosis was based on the history and initial and follow-up MRI findings. The main feature suggesting the diagnosis was a nodular intralabyrinthine mass of low signal intensity on T2-weighted images, and high or isointense signal on T1-weighted images (relative to cerebrospinal fluid), which showed contrast enhancement. Follow-up imaging showed growth of the tumour in one patient. One patient underwent surgery for severe tinnitus. To detect these lesions, MRI should be focussed on the inner ear, using thin-section T2-weighted and T1-weighted images before and after contrast medium. MRI allowed informed surgical planning.

Adult↗

[The subfornical organ today: morphological aspects and functional role].

The recognition of the role played by the subfornical organ (SFO) in the central regulation of body water balance has recently aroused new interest in this anatomical formation which remained ignored for a long time. The SFO is included in the group of the circumventricular organs. In higher vertebrates it is adherent to the ventral surface of the fornix and protrudes into the third ventricle at the level of the interventricular foramina, partially covered by the choroid plexus. The SFO appears as a small nodule, rounded or ovoidal in shape, consisting of highly vascularized nervous tissue and lined by ependyma at the ventricular surface. Its structural organization is fundamentally constant and presents only minor differences in the various species. The SFO neuronal perikarya show different aspects which have been classified in four types. However, it is not yet clearly defined if such aspects refer to distinct cell types or to different transitional features. Nerve and glial cell processes form a dense plexus through the SFO and the subependymal area, as well as in the connective tissue perivascular spaces. These may be narrow or wide and surround fenestrated and non-fenestrated capillaries, assuming sometimes a labyrinthine aspect. The ependymal lining of the SFO ventricular surface shows large variations and regional differences concerning the cell height, the number and development of microvilli, the cilia distribution. The structural properties of SFO, which is characterized by a rich and highly permeable capillary bed, by a wide surface area of contact and exchange with the cerebrospinal fluid, by direct and indirect neural connections with a number of regulatory structures, have been considered as the basis for the role of neurohumoral integration that SFO plays in regulating physiological and behavioral responses to water-mineral changes. Much experimental evidence substantiates this function. However, the studies on SFO are increasingly enriching the literature with new experimental, especially physiological and cytochemical, data which may suggest for this organ connections even more extensive and functions even more complex than those until now ascertained.

Animals↗

Spatial and molecular organization of lymph node T cell cortex: a labyrinthine cavity bounded by an epithelium-like monolayer of fibroblastic reticular cells anchored to basement membrane-like extracellular matrix.

Naive T cells encounter antigen-presenting cells within the cortex of lymph nodes to initiate primary immune responses. Within this T cell cortex is the reticular network (RN)--a system of collagen fibers and extracellular matrix (ECM) wrapped by fibroblastic reticular cells (FRC). We have investigated the distribution of various molecules, including ECM proteins and proteoglycans, in the T cell cortex of both human and rodent lymph node. We confirm and extend reports of matrix elements in the RN. In addition, we find that staining for the laminin-alpha3 chain and for tenascin reveals a 'hollow' reticular pattern, consistent with localization to the basement membrane-like covering of reticular fibers. In contrast, keratan sulfate is observed in a fine linear pattern within the RN, suggesting it is localized to the core of the fibers. Staining with the marker ER-TR7 indicates that FRC cover all identifiable ECM surfaces of the T cell cortex. Based on these findings and previous reports, we conclude that cortical lymphocytes migrate within a 'labyrinthine cavity' free of fibrillar ECM, distinguishing the T cell cortex from other loose connective tissues, and that the FRC lining of the cavity constitutes an epithelium-like boundary. We propose that this spatial organization facilitates ameboid leukocyte crawling along preformed paths of least resistance and that the basement membrane-like ECM of the FRC may facilitate fluid transport within the RN by limiting leakage from the fiber.

Animals↗

Anterior clinoidectomy and opening of the internal auditory canal using an ultrasonic bone curette.

OBJECTIVE: During cranial base surgery, use of a high-speed drill for osteotomy has become common. We performed anterior clinoidectomy and opening of the internal auditory canal using an ultrasonic bone curette, and we report the advantages and clinical applications of this method. DESCRIPTION OF INSTRUMENTATION: The ultrasonic surgical equipment comprises a power supply unit, footswitch, and handpiece (weight, 110 g; diameter, 20 mm; length, 140 mm from tip to angled section). The handpiece tip is 2 mm wide, and the amplitude of longitudinal vibration can be varied from 120 to 365 microm at an ultrasonic frequency of 25 kHz. Cool-controlled irrigation fluid emerges near the tip, through the sheath. EXPERIENCE AND RESULTS: We performed anterior clinoidectomy in eight cases of paraclinoid aneurysm and opening of the internal auditory canal in six cases of acoustic neuroma without damage to the dura mater or nearby structures such as brain tissue, blood vessels, and cranial nerves. In addition, no damage to the facial nerve or labyrinthine organ resulted from heat or vibration caused by the ultrasonic bone curette. CONCLUSION: Ultrasonic bone curettage represents safe instrumentation for performance of anterior clinoidectomy and opening of the internal auditory canal without damage to surrounding structures. This technique allows surgeons to perform procedures on deep areas without incurring psychomotor stress.

Craniotomy↗

Time course of hearing loss in an animal model of pneumococcal meningitis.

A leading cause of morbidity from bacterial meningitis is an irreversible, usually profound sensorineural hearing loss, with an incidence as high as 30% in some studies. Bacterial meningitis remains the most common cause of acquired postnatal sensorineural deafness. Although several clinical studies have examined the long-term outcome of hearing in meningitis, few studies have examined the time course of hearing loss during the acute course of the disease. We have developed an animal model of meningogenic hearing loss in the rat and have plotted the time course of that hearing loss. Serial auditory brain stem responses (ABRs) were measured in rats inoculated in the cisterna magna (subarachnoid space) with Streptococcus pneumoniae (10(5) to 10(7) colony-forming units). All rats injected developed meningitis as evidenced by increased cerebrospinal fluid (CSF) white cell counts and positive CSF cultures. Serial ABR measurements taken 6, 12, 15, 18, 21, and 24 hours after inoculation demonstrated significant threshold shifts and eventual loss of the ABR waveform as compared with measurements in control rats injected with sterile culture medium. Hearing loss began approximately 12 to 15 hours after inoculation and progressed to complete loss by 24 hours (17 of 18 animals). No correlation was found between the magnitude of hearing loss and CSF white cell count or bacterial titer. Temporal bone histology of rats with meningitis shows a dense inflammatory cell infiltrate throughout the subarachnoid space. Labyrinthine inflammatory cells were confined to the scala tympani. The cochlear aqueduct is the proposed route of infection from the meninges to the labyrinth (scala tympani). Endolymphatic hydrops was also noted throughout the cochlea. These experiments both establish a reproducible animal model of meningogenic hearing loss and support the hypothesis that this hearing loss is progressive rather than abrupt in onset and is related to the duration of untreated infection. CSF inflammatory cells appear to enter the cochlea through the cochlear aqueduct. This reliable animal model will enable future studies directed toward further understanding the pathogenesis and pathophysiology of this hearing loss.

Acute Disease↗

A post-processing protocol for three-dimensional visualization of the inner ear using the volume-rendering technique based on a standard magnetic resonance imaging protocol.

A superior diagnostic quality compared to other post-processing (PP) techniques for three-dimensional (3D) inner ear imaging has been attributed to volume rendering (VR). We defined and assessed a VR protocol for 3D visualization of the inner ear in a routine imaging setting. Following definition of a VR protocol by using lower threshold values, surface shading, perspective views and related parameters, standardized 3D views of the inner ear were generated and evaluated in 32 patients suffering from sensorineural or combined hearing loss. Comprehensive inner ear visualization was achieved in 28 patients by means of two 3D shaded-surface views. Incomplete data acquisition (1/32), motion and pulsation artefacts (2/32) and interposed fluid-retaining mastoid cells (1/32) were responsible for non-diagnostic image quality in 4/32 patients. In 5/32 patients modifications of the PP protocol involving the threshold value and depth-cueing parameters helped to establish diagnostic image quality. Mean post-processing time amounted to 5.8 min per site. 3D imaging with the VR technique is suitable for routine inner ear assessment if direct VR, predefined PP protocols and standardized labyrinthine views are used.

Artifacts↗

Exposure of the lateral extremity of the internal auditory canal through the retrosigmoid approach: a radioanatomic study.

The recent trend toward earlier diagnosis of acoustic neuroma has substantially increased the number of candidates suitable for surgery with an attempt at hearing preservation. Although the retrosigmoid approach affords the possibility of saving hearing in selected cases, it is associated with a somewhat greater morbidity that other approaches, in terms of persistent headache, cerebrospinal fluid leakage, and cerebellar dysfunction. For this reason, it is best used selectively, when the probability of success in hearing conservation is high. Only a portion of the internal auditory canal can be exposed through the retrosigmoid approach without violating the inner ear, a maneuver that greatly reduces the chance of preserving residual hearing. Substantial variability exists between individuals as to just how far laterally the internal auditory canal may be opened without compromising labyrinthine integrity. To assess the magnitude of this variability, measurements were obtained from 60 high-resolution temporal bone computed tomography scans with a schema intended to model the surgical angle of view used during the retrosigmoid procedure. Intraoperative measurements in a series of cases established that the actual surgical point of view is situated along a line that passes approximately 1.5 cm behind the sigmoid sinus. In this typical surgical position, these data predict that an average of 3.0 mm (32% of the internal auditory canal length) must be left unexposed to avoid labyrinthine injury, with a range between 1.1 mm and 5.3 mm (9% to 58% of the internal auditory canal). Each additional 1-cm retraction on the cerebellum beyond that customarily used affords approximately 1 mm (10% of the internal auditory canal) further exposure of the canal. When considering the retrosigmoid approach to an acoustic neuroma, the clinician is urged to evaluate each patient individually to estimate the amount of internal auditory canal accessible without the removal of a portion of the inner ear. This can be ascertained from an axially oriented, gadolinium-enhanced magnetic resonance imaging scan in the internal auditory canal plane by drawing a line that originates 1.5 cm behind the posterior margin of the sigmoid sinus and passes tangential to the most medial extent of the labyrinth. If this line intersects the posterior margin of the internal auditory canal at least 2 mm lateral to the deepest point of tumor penetration, then adequate exposure with preservation of the labyrinth is likely an achievable goal.

Craniotomy↗