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PubMed2002

The molecular architecture of the inner ear.

The inner ear is structurally complex. A molecular description of its architecture is now emerging from the use of contemporary methods of cell and molecular biology, and from studies of ontogenetic development. With the application of clinical and molecular genetics, it has now become possible to identify genes associated with inherited, non-syndromic deafness and balance dysfunction in humans and in mice. This work is providing new insights into how the tissues of the inner ear are built to perform their tasks, and into the pathogenesis of a range of inner ear disorders.

Animals↗
PubMed1993

Descriptive and experimental analysis of the epithelial remodellings that control semicircular canal formation in the developing mouse inner ear.

The inner ear of the mouse develops from a roughly spherical epithelial vesicle, the otocyst, which undergoes a series of complex shape changes to produce the functionally important parts of an adult inner ear; in particular, a coiled cochlea--which houses the auditory apparatus, a saccule and utricle containing sensors of gravity and linear acceleration, and three precisely shaped and oriented semicircular canals, with which angular acceleration is detected. This paper follows the development of the shape of the mouse inner ear from simple otocyst until a stage when the vesicle has become a rather squat miniature model of its adult self. We have been able to visualize clearly these complex shape changes by injecting an opaque marker into the lumina of a series of fixed ears. We have further concentrated on the mechanism of formation of the semicircular canals using light-, electron-microscopic, and dye-marking techniques. Classic embryological texts describe the canals developing from outpocketings of the epithelial ear rudiment, whose opposite walls meet, fuse, and "disappear" in the central canal plate region to leave a tube of epithelium encircling their margin. To trace the fate of these "disappearing" epithelial cells we have dye-marked all of the otic epithelial cells at a stage prior to canal formation in mouse embryos which we then grow in roller culture until their canals have formed. From these marking experiments we show that the disappearing cells of the canal plate neither die nor become transformed into mesenchymal cells, but rather, most are retracted back into the canal tube epithelium at either side of the site of fusion. We speculate that this mechanism of epithelial resorption may be a common way of "losing" epithelial cells during embryonic morphogenetic remodellings.

Animals↗
PubMed2006

Fgf19 expression patterns in the developing chick inner ear.

The inner ear is a complex sensorial structure with hearing and balance functions. A key aim of developmental biology is to understand the molecular and cellular mechanisms involved in the induction, patterning and innervation of the vertebrate inner ear. These developmental events could be mediated by the expression of regulating genes, such as the members of the family of Fibroblast Growth Factors (Fgfs). This work reports the detailed spatial and temporal patterns of Fgf19 expression in the developing inner ear from otic cup (stage 14) to 8 embryonic days (stage 34). In the earliest stages, Fgf19 and Fgf8 expressions determine two subdomains within the Fgf10-positive proneural-sensory territory. We show that, from the earliest stages, the Fgf19 expression was detected in the acoustic-vestibular ganglion and the macula utriculi. The Fgf19 gene was also strongly, but transiently, expressed in the macula lagena, whereas the macula neglecta never expressed this gene in the period analysed. The Fgf19 expression was also clearly observed in some borders of various sensory elements. These results could be useful from further investigations into the role of FGF19 in otic patterning.

Animals↗
PubMed1998

The development of the vertebrate inner ear.

The inner ear is a complex sensory organ responsible for balance and sound detection in vertebrates. It originates from a transient embryonic structure, the otic vesicle, that contains all of the information to develop autonomously into the mature inner ear. We review here the development of the otic vesicle, bringing together classical embryological experiments and recent genetic and molecular data. The specification of the prospective ectoderm and its commitment to the otic fate are very early events and can be related to the expression of genes with restricted expression domains. A combinatorial gene expression model for placode specification and diversification, based on classical embryological evidence and gene expression patterns, is discussed. The formation of the otic vesicle is dependent on inducing signals from endoderm, mesoderm and neuroectoderm. Ear induction consists of a sequence of discrete instructions from those tissues that confer its final identity on the otic field, rather than a single all-or-none process. The important role of the neural tube in otic development is highlighted by the abnormalities observed in mouse mutants for the Hoxa1, kreisler and fgf3 genes and those reported in retinoic acid-deficient quails. Still, the nature of the relation between the neural tube and otic development remains unclear. Gene targeting experiments in the mouse have provided evidence for genes potentially involved in regional and cell-fate specification in the inner ear. The disruption of the mouse Brn3.1 gene identifies the first mutation affecting sensory hair-cell specification, and mutants for Pax2 and Nkx5.1 genes show their requirement for the development of specific regions of the otic vesicle. Several growth-factors contribute to the patterned cell proliferation of the otic vesicle. Among these, IGF-I and FGF-2 are expressed in the otic vesicle and may act in an autocrine manner. Finally, little is known about early mechanisms involved in guiding ear innervation. However, targeted disruption of genes coding for neurotrophins and Trk receptors have shown that once synaptic contacts are established, they depend on specific trophic interactions that involve these two gene families. The accessibility of new cellular and molecular approaches are opening new perspectives in vertebrate development and are also starting to be applied to ear development. This will allow this classical and attractive model system to see a rapid progress in the near future.

Animals↗
PubMed1988

Histopathology of chloroform-induced inner ear damage.

Inner ear function loss was caused in guinea pigs and rats by injecting chloroform into the middle ear. After symptoms for cochlear and vestibular deficit had been registered, the animals were permitted to survive for one day to five months. Ear histopathology was then studied in celloidin sections. In both species, hair cells and afferent nerve fibers were intact at all survival times. The acute stage of functional loss in guinea pigs was associated with inner ears of normal histological appearance. Within days after chloroform injection a severe otitis media developed which led to fibrous occlusion of the round window and eventually to new bone growth in the middle ear space around the otic capsule. A secondary labyrinthitis was also observed, resulting in endolymphatic hydrops at longer survival times. Different histopathological changes were seen in rats. The tectorial membrane appeared swollen in all cases, the swelling being more severe in more apical turns at longer survival times. It is concluded that only secondary sequela of the initial functional insult can be detected by standard light microscopic histopathology. Chloroform does not cause a chemical labyrinthectomy as previously assumed, although it is severely ototoxic.

Animals↗
PubMed1997

Extracellular matrix and cell adhesion molecules in the developing inner ear.

The inner ear is a complex sensory organ that forms from a simple epithelial placode. The expression patterns of cell adhesion molecules and extracellular matrix components that have been described in the developing inner ear to date are summarized. Whilst our knowledge of the distribution of some of the known elements involved in cell-cell and cell-matrix interactions is in some instances quite limited, these studies generally suggest many potential roles for cell-cell and cell-matrix interactions in various aspects of inner ear development. However, there is a serious need for experimental studies to assess these possibilities.

Journal Article↗
PubMed1998

Nkx5-1 controls semicircular canal formation in the mouse inner ear.

The inner ear develops from the otic vesicle, a one-cell-thick epithelium, which eventually transforms into highly complex structures including the sensory organs for balance (vestibulum) and hearing (cochlea). Several mouse inner ear mutations with hearing and balance defects have been described but for most the underlying genes have not been identified, for example, the genes controlling the development of the vestibular organs. Here, we report the inactivation of the homeobox gene, Nkx5-1, by homologous recombination in mice. This gene is expressed in vestibular structures throughout inner ear development. Mice carrying the Nkx5-1 null mutation exhibit behavioural abnormalities that resemble the typical hyperactivity and circling movements of the shaker/waltzer type mutants. The balance defect correlates with severe malformations of the vestibular organ in Nkx5-1(-/-) mutants, which fail to develop the semicircular canals. Nkx5-1 is the first ear-specific molecule identified to play a crucial role in the formation of the mammalian vestibular system.

Animals↗
PubMed2005

Expression and functional phenotype of mouse ERG K+ channels in the inner ear: potential role in K+ regulation in the inner ear.

An outcome of the intricate K+ regulation in the cochlear duct is the endocochlear potential (EP), approximately 80 mV, the "battery" that runs hair-cell transduction; however, the detailed molecular mechanisms for the generation of the EP remain unclear. We provide strong evidence indicating that the intermediate cells (ICs) of the stria vascularis (StV) express outward K+ current that rectifies inwardly at positive potentials. The channel belongs to the ether-a-go-go-related gene (erg) family of K+ channels. We cloned an ERG1a channel in the mouse inner ear (MERG1a). The cellular distribution of MERG1a in the cochlea displayed the highest levels of immunoreactivity in the ICs and modest reactivity in the marginal cells as well as in several extrastrial cells (e.g., hair cells). Functional expression of the StV-specific MERG1a channel reveals a current that activates at relatively negative potentials (approximately-50 mV) and shows rapid inactivation reflected as inward rectification at depolarized potentials. The current was sensitive to the methanesulfonanilide drug E-4031 (IC50, approximately 165 nM) and the recombinant peptide rBeKm-1 (IC50, approximately 16 nM), and the single-channel conductance in symmetrical K+ was approximately 14 pS. The site of expression of MERG1a and its functional phenotype (e.g., modulation of the current by external K+ make it one of the most likely candidates for establishing the high throughput of K+ ions across ICs to generate EP. In addition, the property of the channel that produces marked K+ extrusion in increased external K+ may be important in shaping the dynamics of K+ cycling in the inner ear.

Amino Acid Sequence↗
PubMed1994

A study of neurotransmitters in human inner ear. Preservation of human temporal bone and value of organ donation for inner ear research.

Twenty-nine human temporal bones (TBs) from 3 different groups of patients were used to investigate an effective preservation method of the inner ear and to study the suitability of TBs from organ donors for human inner ear research. Inner ears were fixed by perilymphatic perfusion and immersion fixation. Choline acetyltransferase (ChAT) and gamma-aminobutyric acid (GABA) activities were detected either by an indirect immunostaining method or by the peroxidase-anti-peroxidase (PAP) technique. The results show that the cytoarchitecture of the sensory epithelia is excellently preserved in specimens fixed within 2 h after death. ChAT- and GABA-immunoreactivities were revealed in the efferent nerve endings and fibers of the cochlea. Morphological preservation of the sensory epithelia was also good in specimens fixed within 5 h after death. However, inner ear sensory epithelia of organ donors that had died from head trauma and were in the definite brain death state for at least 7 1/2 h were severely damaged and showed cellular debris due to autolysis, although they were fixed within 2 1/2 h after death. The mechanisms underlying this damage of the sensory epithelia are discussed.

Adult↗
PubMed2001

Proteomics and the inner ear.

The inner ear, one of the most complex organs, contains within its bony shell three sensory systems, the evolutionary oldest gravity receptor system, the three semicircular canals for the detection of angular acceleration, and the auditory system--unrivaled in sensitivity and frequency discrimination. All three systems are susceptible to a host of afflictions affecting the quality of life for all of us. In the first part of this review we present an introduction to the milestones of inner ear research to pave the way for understanding the complexities of a proteomics approach to the ear. Minute sensory structures, surrounded by large fluid spaces and a hard bony shell, pose extreme challenges to the ear researcher. In spite of these obstacles, a powerful preparatory technique was developed, whereby precisely defined microscopic tissue elements can be isolated and analyzed, while maintaining the biochemical state representative of the in vivo conditions. The second part consists of a discussion of proteomics as a tool in the elucidation of basic and pathologic mechanisms, diagnosis of disease, as well as treatment. Examples are the organ of Corti proteins OCP1 and OCP2, oncomodulin, a highly specific calcium-binding protein, and several disease entities, Meniere's disease, benign paroxysmal positional vertigo, and perilymphatic fistula.

Animals↗
PubMed1997

[Active electronic hearing implants for middle and inner ear hearing loss--a new era in ear surgery. III: prospects for inner ear hearing loss].

The perspectives for active hearing implants lie in the treatment of patients with sensorineural hearing loss (SNHL). The majority of patients with SNHL suffer from a cochlea amplifier (CA) failure which is discernible by a positive recruitment and loss of otoacoustic emissions (OAE). Therefore, the electronic implant is expected to partially replace functions of the CA. Thus, the implant is thought to function as a CAI (cochlea amplifier implant). An approved implant for routine use is not yet available. Clinical studies have thus far only used the high energy consuming (HEC), narrow-band, electromagnetic floating-mass transducer, as well as the Maniglia-HEC implant. The high energie consuming, yet broadband Canadian Fredrickson implant is soon to be used in humans. Of the piezoelectrical implants, a German CAI (Tübingen implant) at present consisting of a piezoelectrical transducer and a microphone has thus far been acutely implanted in first patient. It is a low energy consuming (LEC), broad-band implantable system for patients with sensorineural hearing loss. Routine surgical treatment of patients with sensorineural hearing loss with a CAI will only be achieved if complete implants (with transducer, microphones, batteries, and control unit) are made available. They combine distinct acoustic superiority with invisibility (end of stigmatization), an open ear canal, and hopefully, the end of feedback whistling. Among the implants mentioned, the German CAI is the only LEC implant. Its energy requirements are so low that with today's technologie implantable batteries (e.g., in pacemakers), the additional implantation of an energy carrier seems feasible. Since the implantable microphone is already available in the German system, the only essential part missing for a totally implantable CAI is the implantable control unit.

Cochlear Implants↗
PubMed1988

Pressure transmission properties from the externa ear canal to the inner ear. An experimental study using guinea pigs.

The inner ear pressure (PIE) in response to pressure changes in the external ear canal was measured in guinea pigs while alternatively opening and closing the perforation of the otic bulla. When the bulla was opened, only a transient degree of applied pressure was transmitted to the inner ear and the amplitude of the PIE was smaller than that of the corresponding PIE when the bulla was closed. This was because the applied pressure was exclusively transmitted to the inner ear via the ossicular chain. When the otic bulla was closed, the pressure was transmitted not only via the ossicular chain but also via the round window (RW) through the middle ear cavity. When the bulla was closed, the amplitude of PIE was larger by a positive pressure load than by the corresponding negative one. The amplitude of PIE showed a linear relationship to ear canal pressure of at least within the +/- 200 mmH2O range, as long as pressure was slowly applied to the ear canal. When the loading pressure was abruptly changed, a bouncing response, possibly reflecting elasticity of the RW, was evoked, which diminished or disappeared when the round window was artificially ruptured.

Acoustic Impedance Tests↗
PubMed1985

Damage to the middle ear and the inner ear in underwater divers.

Postmortem human tissue from recently deceased divers was processed histologically to assess any inner and middle ear damage that could have resulted from the effects of pressure during diving. The following new findings are particularly noteworthy. In one diver, ascent while breath holding resulted in the rupture of the ear drum and blood in the middle ear, in addition to pulmonary barotrauma. In a second diver, following inner ear decompression sickness, new bone growth, similar to that described earlier in experimental studies with the squirrel monkey, was observed in the arms of one of the semicircular canals. These observations are further confirmation that otologic disorders can be a serious threat to divers.

Barotrauma↗
PubMed1998

Molecular cloning of a zinc finger gene eZNF from a human inner ear cDNA library, and in situ expression pattern of its mouse homologue in mouse inner ear.

We have isolated and characterized the cDNA for eZNF, a zinc finger gene expressed in human inner ear, from a kinetically enriched human inner ear cDNA library. The sequence of full length cDNA was determined and its expression pattern characterized. A high degree of homology is shared between eZNF and rat transcription factor Kid-1. It belongs to the C2H2 class of zinc finger genes, contains a Kruppel-associated box (KRAB) domain near the N-terminus, and has consensus sites for phosphorylation. The gene is expressed in kidney and inner ear structures of mouse and human as determined by Northern blot analysis. In situ hybridization was used to demonstrate specific expression of the mouse eZNF homologue in epithelial layers of the saccule, semicircular canals, and the cochlea of newborn mice. The genomic clone corresponding to the cDNA was isolated and used for fluorescence in situ hybridization to localize it to human chromosome 5qter. The identification of genes expressed in human inner ear by representational difference analysis, their chromosomal location, and expression pattern of their homologues in developing mouse inner ear comprise a strategy that can potentially identify genes important in hearing and deafness.

Amino Acid Sequence↗
PubMed1997

Induction of an inner-ear-specific autoreactive T-cell line for the diagnostic evaluation of an autoimmune disease of the inner ear.

Different patterns of sensorineural hearing loss with a potential improvement in auditory function following immunosuppressive therapy might be caused by an isolated autoimmune disease of the inner ear. Because of the lack of well-defined detection methods to identify autoimmune processes within the inner ear and the fact that the human inner ear is one of the few organs of the body not amenable to diagnostic biopsy, there has been great interest in developing animal models. Previous studies found evidence that this entity might be cellular mediated. By heterologeous immunization of inbred Lewis rats with inner-ear tissue, an autoreactive inner-ear-specific T-cell line was established. After passive transfer of these cells, a labyrinthitis was induced in recipient animals. The experimental design can serve as an animal model for a cellular-mediated autoimmune disease of the inner ear. Further studies have to split the cochlear proteins and to identify the protein with the strongest autoimmunological potency. After biotechnical production of this protein, a clinical test to diagnose an autoimmune disease of the inner ear in man should be possible.

Animals↗
PubMed1991

Inner ear fine structures of the hamster in frozen sections used for immunohistochemical assays for inner ear diseases.

Frozen sections of the inner ear of the hamster enable detailed investigations of the fine structures in immunofluorescence assays. At high magnification single mitochondria can be identified by their reactions with an antiserum containing antibodies against mitochondria. In the positive reaction with an antiserum against nuclei, the typical green fluorescence is restricted to the nuclei, which are mostly separated by the surrounding cytoplasm. The method of immunohistochemical assay using frozen sections from the non-decalcified inner ear is very time-consuming and cannot be recommended for the routine diagnosis of inner ear diseases, although it may be useful in research and for studying critical clinical cases.

Animals↗
PubMed1997

Expression of P-glycoprotein in inner ear capillary endothelial cells of the guinea pig with special reference to blood-inner ear barrier.

Expression of P-glycoprotein (P-gp) was detected immunohistochemically in the guinea pig inner ear using mouse anti-P-gp monoclonal antibody C219. P-gp was found only in the capillary endothelial cells of the cochlea and vestibule. The pattern of P-gp immunostaining in the inner ear was similar to that of the brain. The present investigation suggested that P-gp expression in inner ear capillary endothelial cells might play an important role in the blood-inner ear barrier by acting as an extrusion pump.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1998

A role for neural cell adhesion molecule in the formation of the avian inner ear.

The inner ear forms by a series of folds within an ectodermal placode. Previous work has shown that changes in surrounding tissues play a more prominent role in invagination than changes in the cytoskeleton of the primordium. Interference with the integrity of the extracellular matrix causes abnormalities in the folding process, primarily related to abnormalities in the paraxial mesoderm which lies ventral to the placode. In this study, the role of the neural cell adhesion molecule (N-CAM) was investigated, based on the expression of this component of the plasmalemma at the time the otic placode begins to fold. Microinjection of blocking antibodies to N-CAM into the paraxial mesoderm adjacent to the otic placode resulted in two major classes of defects, detachment of the primordium from the neural tube and interference with formation of the folds. Microinjection of saline, control immunoglobulin, or antibody against cytoplasmic domain had no effect. These defects correlate with the pattern of N-CAM expression at the time of injection, along the neural ectoderm and otic epithelium and the mesenchyme cells ventral to the primordium. It seems likely that N-CAM is playing a role in heterophilic associations rather than through the homophilic binding domain during formation of the otic vesicle.

Animals↗