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PubMed1988

Evolution of recruitment at different frequencies during the development of endolymphatic hydrops in the guinea pig.

The study of human temporal bones has identified endolymphatic hydrops as a common feature of several diseases. In particular it is systematically found in those bones removed from patients with premortem Menière's disease. Menière's disease is known to induce sensorineural pathology with recruitment, which changes with the evolution of the hearing loss, and is suspected to induce a cochlear conductive loss by a possible increase in static pressure of endolymph. Amplitude/intensity functions of sensorineural responses can reflect recruitment and/or conduction loss. Experimentally induced hydrops in animals provokes cochlear physiological alterations, some of which closely resemble certain features of Menière's disease. In the present study using a guinea-pig animal model, we have examined amplitude/intensity functions at the round window for cochlear microphonics (RWCM), summating potentials (RWSP) and action potentials (CAP) at different stages of hearing loss in experimentally induced hydrops. During the period of fluctuating thresholds there was reduction of maximal RWCM amplitude, no change in RWSP and recruitment on the CAP. At a later stage when the audiogram was flat and fluctuations were no longer seen, RWCM remained unchanged. At this time RWSP could show recruitment while CAP amplitudes at all intensities were reduced, indicating either a cochlear conductive loss and/or a general depression of neural activity.

Animals↗
PubMed1982

Functional and morphological findings of endolymphatic sac.

After obliteration of endolymphatic sac and duct, no significant alteration in d.c. potential, K+ activity or protein content during the development of endolymphatic hydrops could be observed. The K+ activity of endolymphatic sac was only one-ninth of that in the cochlear part of the endolymph. After injection of Thorotrast into the endolymphatic sac, aggregated particles were found in the cochlear endolymph 2 days later. Ethacrynic acid (60 mg/kg) caused a decrease in K+ activity in the cochlear endolymph, but an increase in the endolymphatic sac. Intercellular edema was observed both in stria vascularis and in endolymphatic sac by light and electronmicroscopy after ethacrynic acid administration. These results suggest the existence of an active transport mechanism in the endolymphatic sac epithelium.

Animals↗
PubMed2000

FGF/FGFR-2(IIIb) signaling is essential for inner ear morphogenesis.

Interactions between FGF10 and the IIIb isoform of FGFR-2 appear to be crucial for the induction and growth of several organs, particularly those that involve budding morphogenesis. We determined their expression patterns in the inner ear and analyzed the inner ear phenotype of mice specifically deleted for the IIIb isoform of FGFR-2. FGF10 and FGFR-2(IIIb) mRNAs showed distinct, largely nonoverlapping expression patterns in the undifferentiated otic epithelium. Subsequently, FGF10 mRNA became confined to the presumptive cochlear and vestibular sensory epithelia and to the neuronal precursors and neurons. FGFR-2(IIIb) mRNA was expressed in the nonsensory epithelium of the otocyst that gives rise to structures such as the endolymphatic and semicircular ducts. These data suggest that in contrast to mesenchymal-epithelial-based FGF10 signaling demonstrated for other organs, the inner ear seems to depend on paracrine signals that operate within the epithelium. Expression of FGF10 mRNA partly overlapped with FGF3 mRNA in the sensory regions, suggesting that they may form parallel signaling pathways within the otic epithelium. In addition, hindbrain-derived FGF3 might regulate otocyst morphogenesis through FGFR-2(IIIb). Targeted deletion of FGFR-2(IIIb) resulted in severe dysgenesis of the cochleovestibular membraneous labyrinth, caused by a failure in morphogenesis at the otocyst stage. In addition to the nonsensory epithelium, sensory patches and the cochleovestibular ganglion remained at a rudimentary stage. Our findings provide genetic evidence that signaling by FGFR-2(IIIb) is critical for the morphological development of the inner ear.

Animals↗
PubMed1994

Activities of carbonic anhydrase in the cochleae of guinea pigs with early experimental endolymphatic hydrops.

Normal auditory function depends on maintenance of the unique ion composition in the endolymph. Carbonic anhydrase in the inner ear has been suggested to play an important role in maintaining the ion concentration and regulating fluids of the inner ear. Cochlear dysfunction may indicate changes in the biochemical components and osmotic pressure of the inner ear fluids as well as inadequate generation of intracellular metabolic energy. Dysfunction of the inner ear was investigated in the early stages of endolymphatic hydrops. Normal adult albino guinea pigs were operated on to obliterate the endolymphatic ducts and sacs of the right ears to induce endolymphatic hydrops. The auditory function of experimental guinea pigs was assessed according to the difference between the preoperative and postoperative hearing thresholds of the auditory brainstem response (ABR). Vibratome sections of the hydropic cochlea (right) and control cochlea (left) were stained histochemically for the activities of carbonic anhydrase in this study. Decreased activity of this enzyme was not shown consistently in the stria vascularis and organ of Corti of the hydropic cochlea in the early stage of endolymphatic hydrops whereas auditory dysfunction in the hydropic ear was noted from the ABR threshold for the experimental animals with 2 months survival. The results of the present study suggest that further investigation concerning the role of carbonic anhydrase in the cochlear auditory function is necessary.

Animals↗
PubMed1991

[Experimental model of endolymphatic hydrops].

We have investigated by electrophysiology, morphology and pharmacology, the consequences of the surgical blocking of the endolymphatic duct in the guinea pig. We have demonstrated an immediate fluctuant CAP sensitivity loss on the low frequencies. Some weeks later a very high frequency loss can also be detected and finally after several months the mid frequencies are also affected and audiogram becomes relatively flat. This type of evolution of sensitivity loss corresponds remarkably well to the type of evolution of hearing loss observed in Ménière's patients. We have demonstrated that the early low frequency fluctuant losses are almost certainly a result of the selective atrophy of the short and middle stereocilia on the outer hair cells in the upper three cochlear turns, corresponding to a new hait cell pathology. Since ion channels are likely to be localised close to inter-stereocilia, row tip links this type of stereocilia atrophy could account for the low frequency fluctuant CAP sensitivity losses in hydropic cochleas. In addition this type of atrophy recalls a retrograde step in the ontogenesis of hair cells. Application of hydrostatic pressure directly to the endolymph via the endolymphatic duct provoked a high frequency sensitivity loss suggesting that endolymphatic pressure might be implicated in the late phase in the evolution of the hearing loss. Long-terme treatment by the diuretic chlorthalidone appeared to slow down the evolution of early low frequency CAP sensitivity loss and could reduce the volume of the hydrops. However in the long-term the CAP sensitivity loss was not arrested.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1976

Familial branchio-oto-renal dysplasia: a new addition to the branchial arch syndromes.

The present report concerns a two-generation family of nine individuals in which the father and three of the six living children all had: (1) a mixed hearing loss with a Mondini type cochlear malformation and stapes fixation; (2) cup-shaped, anteverted pinnae with bilateral prehelical pits: (3) bilateral branchial cleft fistulas; and (4) bilateral renal dysplasia and anomalies of the collecting system. The father and one affected son also had aplasia of the lacrimal ducts. A fourth child who died at 5 months of age was reported to have branchial cleft fistulas and bilateral polycystic kidneys at autopsy. In addition, the concept of noso-embryologic communities is presented. Such groups are composed of syndromes whose total phenotypic spectra not only overlap but also share common elements in embryogenesis. This concept is illustrated with a group of branchial arch syndromes that are related in this way.

Abnormalities, Multiple↗
PubMedDate not supplied

Distribution of beta-tubulin in guinea pig inner ear.

Our previous research had suggested that beta-tubulin might be an autoantigen for autoimmune inner ear disease. In this study, the expression of beta-tubulin in inner ears of normal and tubulin-immunized guinea pigs was examined by immunohistochemical staining. Strong immunoreactivity to beta-tubulin monoclonal antibody was found in stria vascularis, neurons of the spiral ganglion, cochlear nerve fibers and spiral ligament. Diffuse staining was found in the stria vascularis and the neurons of the spiral ganglion, while dense network staining was found in the spiral ligament, the nerve fibers and the vestibular end organs. The semicircular canals, endolymphatic duct and sac were also positively stained. In inner ears of guinea pigs challenged with beta-tubulin, staining intensity was diminished in the stria vascularis, the spiral ligament, and the neurons of the spiral ganglion. The results suggest that beta-tubulin is distributed to most structures of guinea pig inner ear. A challenge to the inner ear by tubulin could change the beta-tubulin distribution and cause degeneration in the spiral ganglion. The results support the hypothesis that beta-tubulin might be an autoantigen for autoimmune inner ear disease.

Animals↗
PubMed1990

The effects of pressure on cochlear microphonics in experimentally induced hydropic ears in the guinea pig.

The effect of perilymphatic pressure on the cochlear microphonics (CM) threshold was examined in guinea pigs with experimentally induced hydrops. When a pressure of 300 mm H2O was applied to the scala tympani of the basal turn, the 500 Hz CM threshold shifts in ears during the 1st week following obliteration of the endolymphatic sac and duct were significantly smaller when compared with shifts in normal ears. Larger threshold shifts were then regained at 4-12 weeks after surgery. When acetazolamide was administered to hydropic animals, the effect of the increase of perilymphatic pressure on the CM threshold was also investigated. During the 1st postoperative week (4-7 days after surgery), CM threshold shifts were larger when compared to changes in hydropic animals without medication. The CM depression as the result of pressure is probably due to a mechanical effect on the intracochlear partition.

Acetazolamide↗
PubMed2005

Effects of lithium on endolymph homeostasis and experimentally induced endolymphatic hydrops.

There is evidence to suggest that water homeostasis in the inner ear is regulated via the vasopressin (VP)-aquaporin 2 (AQP2) system in the same fashion as in the kidney. The VP-AQP2 system in the kidney is well known to be inhibited by lithium, resulting in polyuria due to a decrease in reabsorption of water in the collecting duct of the kidney. Therefore, lithium is also likely to inhibit the VP-AQP2 system in the inner ear, and consequently exert some influence on inner ear fluid homeostasis. In this study, we investigated the effects of lithium on AQP2 expression in the rat inner ear, and on the cochlear fluid volume in hydropic ears of guinea pigs. A quantitative PCR study revealed that lithium reduced AQP2 mRNA expression in the cochlea and endolymphatic sac. Lithium application also decreased the immunoreactivity of AQP2 in the cochlea and endolymphatic sac. In a morphological study, lithium intake significantly reduced endolymphatic hydrops dose-dependently. These results indicate that lithium acts on the VP-AQP2 system in the inner ear, consequently producing a dehydratic effect on the endolymphatic compartment.

Animals↗
PubMed1982

Summating potential in Meniere's disease.

This study assesses several electrocochleographic (ECoG) duration and amplitude measures in a clinically defined Meniere's group and compares the results with those from a normal hearing control group and a hearing loss group (cochlear). The summating potential (SP)/action potential (AP) amplitude ratio was the most efficient diagnostic measure, with 62% of the Meniere's group demonstrating abnormal ratios compared to 4% of the normal control group and 17% of the cochlear group. The SP changes were then studied from three angles: 1. Comparison of SP changes with glycerol test results; 2. Comparison of ECoG results before and after shunt surgery; and 3. Recording of SP's in guinea pig ears in which hydrops had been created by obliterating the endolymphatic duct.

Action Potentials↗
PubMed2004

Enlarged endolymphatic duct and sac syndrome: relationship between MR findings and genotype of mutation in Pendred syndrome gene.

Pendred syndrome (PDS) is characterized by profound deafness in childhood, positive perchlorate challenge, and goiter. PDS is often associated with enlarged endolymphatic duct and sac (EEDS), and recently, PDS gene mutations have been reported even in those patients with EEDS without classic Pendred syndrome. In a previous report, the number of mutant alleles was correlated with the degree of subclinical thyroid abnormality, but not with hearing loss, in patients with missense mutation H723R. It also has been reported that the hearing loss in EEDS was not correlated with the EEDS volume, cochlear modiolar area, or signal intensity of the endolymphatic sac. We evaluated the correlations between the number of mutant alleles and these parameters in patients with EEDS to investigate the mechanisms underlying this condition. The study group was comprised of 16 Japanese patients with EEDS diagnosed by MR imaging. The H723R mutation was homozygous in six patients and heterozygous in six patients, with no mutation found in four patients. The modiolar area, EEDS volume, and signal intensity ratio (sac signal/cerebrospinal fluid signal) were not significantly correlated with the number of mutant alleles. PDS gene mutations may not be the only cause of EEDS, and the mechanisms underlying EEDS remain unclear.

Adolescent↗
PubMedDate not supplied

Inner ear anomalies in two cases of trisomy 18.

Two cases of trisomy 18 in which temporal bone defects were limited to the inner ears are described. Several abnormalities were present that have not been described previously in this syndrome. The first case involved a 1-month-old female infant who died of congenital heart defects. Cochlear nerve fibers were absent on the left side, with near-normal innervation on the right. Although the organ of Corti was present bilaterally, some of the outer hair cells were deformed, having small rounded cell bodies unsupported by Deiters' cells. On the right, similar abnormal cells were found in the tunnel of Corti. Vestibular defects in this case included reduced nerve supply of the left saccular macula, cysts in the superior and posterior cristae, and absence of the utriculoendolymphatic valve. The second case involved a newborn male infant with multiple congenital anomalies. The major cochlear defect was a deformity of the stria vascularis. In the lower apical turn, the stria was adherent to Reissner's membrane and extended beneath it into scala media. Large capillaries, which ran freely suspended in scala vestibuli, entered the upper portion of the stria. Severe atresia of the lateral and posterior semicircular ducts was found in the vestibular apparatus.

Chromosomes, Human, 16-18↗
PubMed1983

Meniere's syndrome and otitis media.

We here present a clinical study of 37 patients with Meniere's syndrome. Meniere's syndrome can occur subsequent to and in some cases simultaneously with chronic otitis media. When otitis media which has occurred many years earlier in childhood becomes inactive, leading to sequelae of Meniere's later in life, full-blown Meniere's symptom-complex with vertigo tends to occur; whereas when active chronic otitis media accompanies Meniere's, cochlear Meniere's syndrome tends to predominate. Endolymphatic hydrops is described in pathological cases of labyrinthitis and in 11 human temporal bone cases where there is evidence of chronic otitis media in the absence of visible labyrinthitis. A discussion of pathogenic factors includes considerations of quantity of endolymph due to hypodevelopment of the endolymphatic duct and sac related to mastoid hypocellularity and otomastoiditis in childhood and to other endolymphatic malabsorptions and also considerations of endolymph quality which can influence endolymph production as well as absorption.

Adolescent↗
PubMed1999

MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac.

BACKGROUND AND PURPOSE: Large endolymphatic duct and sac (LEDS) is one of the most common anomalies seen in patients with congenital sensorineural hearing loss (SNHL), and is known to occur with other inner ear findings. Our purpose was to use high-resolution T2-weighted fast spin-echo (FSE) MR imaging to describe the features and prevalence of specific anomalies that occur in association with LEDS. METHODS: We retrospectively reviewed MR images of the inner ear obtained in 63 patients with LEDS and in 60 control subjects. We evaluated each image for features of cochlear and vestibular dysplasia, including deficiency of the cochlear modiolus, gross cochlear dysmorphism, asymmetry of the cochlear scalar chambers, enlargement of the membranous vestibule, gross vestibular dysmorphism, and abnormality of the semicircular canals (SCC). RESULTS: Cochlear anomalies were present in 76% of ears with LEDS. Modiolar deficiency, gross dysmorphism, and scalar asymmetry were seen in 94%, 71%, and 65% of abnormal cochleas, respectively. Vestibular abnormalities were present in 40% of ears with LEDS. Simple enlargement, gross dysmorphism, and distortion of the lateral SCC were seen in 84%, 16%, and 32% of abnormal vestibules, respectively. CONCLUSION: Coexistent cochlear anomalies, vestibular anomalies, or both are present in most ears with LEDS, and appear as a spectrum of lesions, ranging from subtle dymorphism to overt dysplasia. The presence of coexistent anomalies in LEDS affects treatment decisions and prognosis. Newer techniques of high-resolution FSE MR imaging provide a means of exquisite characterization of LEDS, as well as more sensitive detection of associated vestibulocochlear anomalies.

Cochlea↗
PubMedDate not supplied

Prenatal low-dose gamma irradiation of the inner ear induces changes in the expression of intermediate filaments.

The expression of intermediate filaments (1F) was analysed in the inner ear in normally developed adult CBA/CBA mice and in mice of the same age which had been gamma irradiated in utero with a low dose 1-2 Gy single exposure. Well characterized monoclonal antibodies (mAbs) against all classes of intermediate filament proteins (cytokeratins-Cks, vimentin, neurofilaments, desmin and glial fibrillar acidic protein) were used. With the exception of neurofilament proteins, the expression of intermediate filament proteins was the same in adult normal and irradiated inner ears, irrespective of gestational age at exposure. A complex Ck pattern occurred in the various cell types comprising the membranous labyrinth. In spite of the differences in cell shape and internal organization of organelles, epithelia actively involved in inner ear fluid homeostasis (stria vascularis, dark cell epithelium, endolymphatic duct and sac) revealed, according to our mAbs, the same expression of Cks, except for the mouse counterpart of human Ck 7, which was found exclusively in the stria vascularis and the endolymphatic duct and sac. The pattern of intermediate filament composition in the labyrinth was the same in the mouse as in man. Irradiation on gestational days 12 or 13 (the otocyst stage)--but not at more advanced embryonic age--induced immunoreactivity for neurofilament proteins in vestibular hair cells (HC) and to a minor extent also in cochlear HC. No such positivity was found in the control material.

Abnormalities, Radiation-Induced↗
PubMed2005

Imaging and clinical findings in large endolymphatic duct and sac syndrome.

OBJECTIVE: Large endolymphatic duct and sac syndrome (LEDS) is known as the most common kind of inner ear malformations, which is radiologically detectable. Nevertheless, nowadays many questions are not fully cleared and LEDS is relatively unknown among general radiologists. The aim of this study was to evaluate the incidence of LEDS in the own patient population and to present our experiences regarding imaging findings, clinical presentation and follow up. MATERIALS AND METHODS: Based on a complete recording of all patients, sent from ENT department to radiology, we identified all radiological diagnosed cases of inner ear malformations including LEDS and all patients in whom an inner ear malformation was clinically suspected. The retrospective study included clinical records, HR-CT and MRI performed between 1994 and 2002. RESULTS: Among 169 patients (338 ear), 17 of patients (median age: 12 years) and 28 ears, respectively, had enlarged endolymphatic structures. In 10 patients - 6% - (15 ears), no other abnormalities were detected, called isolated LEDS, seven patients showed additional inner ear abnormalities. One patient showed a labyrinthine hemorrhage after sudden hearing loss. Audiometric data revealed sensorineural hearing loss in 22 ears, deafness in 5 ears and normal hearing in 1 case of 28 ears. In 10 (67%) of 15 ears with isolated LEDS, the hearing loss was downward-fluctuating progressive. Twelve patients (eight with isolated LEDS) had partly repeated sudden hearing losses. A trigger for worsening of hearing was found in five patients. A correlation between the severity of morphological changes on imaging and the degree of hearing disturbances could not be detected. Only four young patients underwent a radiological examination within the first or second year after onset of hearing loss. Three patients received a cochlear implant. CONCLUSIONS: LEDS might be the cause of progressive hearing loss and repeated acute hearing losses in children and young adults. Imaging plays an important role in making the diagnosis.

Adolescent↗
PubMed1992

Branchio-oto-renal syndrome: further delineation of an underdiagnosed syndrome.

We report on a woman who was diagnosed with branchio-oto-renal (BOR) syndrome after 2 pregnancies complicated by oligohydramnios due to renal hypoplasia and agenesis. Both babies died neonatally of pulmonary hypoplasia. Histopathology of the temporal bones of the second child showed marked immaturity of the middle ear cleft, ossicles, facial nerve and canal, and cochlear nerve. Maternal renal ultrasound study was normal although intravenous pyelography indicated renal hypoplasia. The frequency of BOR syndrome among cases of recurrent fetal renal hypoplasia/dysplasia or agenesis is unknown, and parental renal ultrasonography may not identify a heritable renal defect. Investigations should include a family history, and examination of relatives to look for preauricular pits, lacrimal duct stenosis, and branchial fistulae and/or cysts. Hearing studies and IVP may be indicated.

Abnormalities, Multiple↗
PubMedDate not supplied

Cytokeratin diversity in epithelia of the human inner ear.

The expression of cytokeratins (Cks) nos. 5, 7, the combination 5 and 8, 8, 10, 17, 18 and 19 was analysed in the inner ear of 14-24-week-old human fetuses, using 10 different well characterized monoclonal antibodies (mAbs). A complex pattern of Ck immunoreactivity was found. Cks 8 and 18 were identified in both cochlear and vestibular hair cells as well as in all other epithelia. Epithelia involved in fluid regulation (stria vascularis, Reissner's membrane and dark cell epithelium) revealed the same pattern of Ck immunoreactivity in spite of morphological differences in cell configuration. A specific Ck, no. 10 as detected with the mAbs RKSE 60, was found in the endolymphatic duct and sac. The expression of Cks in the human inner ear is in principle similar to that found in the labyrinth of other animal species, for instance in the mouse. However, the human inner ear had a more complex pattern than any inner ear so far known in animal species.

Antibodies, Monoclonal↗