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PubMed1997

Trapping genes expressed in the developing mouse inner ear.

Identification of the genes involved in the development of the mouse inner ear and developmental studies of mice that bear mutations in these genes is an important approach to understanding genetically determined human auditory dysfunction. Towards this end, we initiated a gene trap screen designed to simultaneously mark and mutate genes in mouse embryonic stem cells by the insertion of a lacZ reporter gene. Expression of beta-galactosidase in gene trap cell lines was monitored both before and after the addition of factors that are known to affect inner ear development. Gene trap cell lines that expressed beta-galactosidase under one or more culture conditions were used to create chimeric mouse embryos for studies of reporter gene expression in vivo. A high proportion of these gene trap insertions were expressed in the developing inner ear, suggesting that this strategy provides an effective means of identifying genes that may be involved in inner ear development or function.

Animals↗
PubMed2004

Characterization of a new mouse mutant, flouncer, with a balance defect and inner ear malformation.

HYPOTHESIS: Balance anomalies are often associated with abnormalities of the vestibular part of the inner ear. We studied a newly generated mouse mutant with balance defects and asked whether its behavioral anomalies were associated with inner ear defects. Furthermore, we asked whether the mutation responsible for the defects was located in the same region of mouse chromosome 4 as several other mouse mutations that we have previously described. BACKGROUND: Phenotypic and genotypic analysis of mouse mutants with hearing or balance problems has helped greatly with the identification of the genes involved in deafness and has contributed to the understanding of mechanisms of normal hearing and balance. This article describes a new mouse mutant, flouncer, that shows a balance defect. The flouncer mutation shows semidominant inheritance, and was generated by mutagenesis using N- ethyl-N- nitrosourea. METHODS: Hearing was assessed by the Preyer reflex (ear-flick) test. Behavioral tests including open field and swimming tests were performed. The morphology of the middle and inner ears was investigated by microdissection, clearing using glycerol, paint-filling of the labyrinth, and scanning electron microscopy. RESULTS: Flouncer mutants showed vestibular dysfunction but do respond to sounds. Phenotypically, mutants had various degrees of truncation of the lateral semicircular canals, small or obliterated round window of the cochlea, and mild morphologic anomalies of the stapes. Flouncer mutants showed circling behavior and hyperactivity. Linkage mapping using a backcross has indicated that the mutation lies in proximal chromosome 4 proximal to D4Mit171. CONCLUSION: The lateral semicircular canal has been described to be the most commonly affected part of the inner ear in humans, and flouncer provides a mouse model for genetic and developmental analysis of such defects.

Animals↗
PubMed2004

Hypergravity decreases carbonic anhydrase-reactivity in inner ear maculae of fish.

Previous investigations revealed that fish inner ear otolith growth depends on the amplitude and the direction of gravity. Both otolith total size, otolith bilateral size-asymmetry and the total and bilateral calcium-incorporation are also affected by gravity. Hypergravity, e.g., slows down otolith growth and diminishes bilateral otolith asymmetry as compared to 1 g control specimens raised in parallel. Since the enzyme carbonic anhydrase (CA) plays a prominent role in otolithic calcification, the reactivity of inner ear CA during otolith growth under hypergravity was investigated. CA-reactivity was demonstrated histochemically and densitometrically on sections of inner ear maculae of larval cichlid fish (Oreochromis mossambicus), that were kept for 6 hrs in a 3 g hypergravity centrifuge. The total unilateral macular CA-reactivity and the bilateral difference in CA between the left and the right maculae were significantly lower in 3 g animals than in 1g controls. The result is in complete agreement with previous studies indicating that a regulatory mechanism, which adjusts otolith size and asymmetry towards the gravity vector, acts via activation/deactivation of macular CA.

Acoustic Maculae↗
PubMedDate not supplied

Role of adhesion molecules for the immunological defense of the inner ear.

Previous experiments demonstrated an organ-specific migration of sensitized lymphocytes to the inner ear and enabled us to establish an animal model for an autoimmunological labyrinthitis in the guinea pig. For a nonrandom emigration from capillaries into tissues of their immunological destination, memory lymphocytes need homing receptors on their cell surface to interact with specialized postcapillary venules, the so-called high endothelial venules. These molecules were already demonstrated in organs of the mucosa-associated lymphatic system (MALT). This study was performed to demonstrate these adhesion molecules on endothelial cells and lymphocytes of the inner ear by using two monoclonal antibodies: MECA-325 and MEL-14. Special interest was directed to the posterior spiral modiolar vein as a known port of entry for lymphocytes during an immune response. Emphasis was placed on the development of new or additional binding sites for lymphocytes on the above-mentioned structures using immunohistochemistry during an immune response of the inner ear. The results suggest a constant recirculation of memory lymphocytes through the endolymphatic sac as known for other organs of the MALT. The acquisition of high endothelial venules in the cochlea during an immune response could serve for the cellular requirements of a sympathetic cochleolabyrinthitis with the spiral modiolar vein as port of entry for lymphocytes. Furthermore, this study supports the hypothesis of the endolymphatic sac as central immunological control organ of the inner ear and as the primary place for antigen processing.

Animals↗
PubMed1992

Inner ear damage and passage through the round window membrane of Pseudomonas aeruginosa exotoxin A in a chinchilla model.

By the use of computer-assisted morphometric analysis of the organ of Corti and/or measurements of action potential threshold changes, inner ear changes in chinchillas were evaluated 4 weeks after application on the round window membrane of a Pseudomonas aeruginosa exotoxin A solution. Severe inner ear damage was detected after application of 50 ng (5 microL at a concentration of 10 micrograms/mL) exotoxin A, whereas application of 5 ng exotoxin did not cause measurable inner ear damage. Perilymph concentrations of exotoxin A were measured with an enzyme-linked immunosorbent assay 1.5 to 19 hours after 50 ng, 0.5 micrograms, or 5 micrograms of exotoxin A was applied on the round window membrane. Only the highest concentration produced measurable levels of exotoxin in the inner ear fluids. It is concluded that exotoxin A present on the round window membrane of the chinchilla has the ability to penetrate into the inner ear and cause irreversible inner ear changes.

ADP Ribose Transferases↗
PubMed1997

Jean Marquet Award. Regeneration of the neurosensory structures in the mammalian inner ear.

Regeneration of the neurosensory structures in the mammalian inner ear. Motivated by the absence of treatment of neurosensory deafness apt to restore auditory function or alter the course of progressive hearing loss, we developed two different experimental strategies to approach these diseases: one is otoprotection, designed to prevent further degradation of auditory function; the other is regeneration which is defined as the replacement of hair cells in the deaf ear and their reconnection to the central nervous system through primary auditory neurons. In this paper, we summarize our data on the regeneration of auditory neurons and hair cells. Neuronal maintenance and regeneration was studied through an initial investigation of growth factors during inner development. Once the effect of various neurotrophic molecules was determined, the factors were tested on mature auditory neurons in vitro and in vivo. The hair cell regeneration was investigated on the basis of a concept derived from comparative physiology and from the study of the development of the inner ear. We showed that in young rats it is possible to induce the regeneration/repair of hair cells in the organ of Corti in cultures using retinoïc acid and transforming growth factor alpha. The clinical prospects of these findings of inner ear regeneration are discussed.

Animals↗
PubMed1996

[Scanning electron microscopic study of inner ear barotrauma: in the guinea pig under hypobaric pressure].

In order to investigate the mechanism of inner ear barotrauma, guinea pigs, with bilateral eustachian tube occlusion, were subjected to decompression and compression between 760 and 460 mmHg in a hypobaric pressure chamber. We divided the guinea pigs into two groups, A and B. Group A showed normal eustachian tubes, and group B showed bilaterally occluded eustachian tubes. Group B animals were divided into three types according to the rates of compression and decompression. After pressure loading, morphological changes in the hair cells of the organ of Corti were studied by means of scanning electron microscopy. There was no damage to hair cells in the setting of normal eustachian tube function, as in group A. On the other hand, mild to severe hair cell damage was observed with rapid decompression in group B. This observation suggests that relative positive pressure in the middle ear cavity is an important factor in inner ear barotrauma. The mechanism of hair cell damage due to inner ear barotrauma is presumed to be distortion of the organ of Corti caused by a difference in pressure between perilymph and endolymph resulting in injury to the stereocilica.

Animals↗
PubMed1990

[Improved inner ear diagnosis by controlled use of computerized tomography].

Problems in the examination of the inner ear using conventional CT are described. Intraoperative findings often differ from CT findings. A new development HR-CT is introduced. Findings of middle and inner ear malformations and otosclerosis are demonstrated. It is shown that HR-CT imaging using a new 1024 matrix, enables better spatial resolution. This new CT technique improves inner ear examination, especially in the preparation for cochlear implantation.

Child, Preschool↗
PubMed2005

Molecular mechanisms underlying inner ear patterning defects in kreisler mutants.

Prior studies have shown that kreisler mutants display early inner ear defects that are related to abnormal hindbrain development and signaling. These defects in kreisler mice have been linked to mutation of the kr/mafB gene. To investigate potential relevance of kr/mafB and abnormal hindbrain development in inner ear patterning, we analyzed the ear morphogenesis in kreisler mice using a paint-fill technique. We also examined the expression patterns of a battery of genes important for normal inner ear patterning and development. Our results indicate that the loss of dorsal otic structures such as the endolymphatic duct and sac is attributable to the downregulation of Gbx2, Dlx5 and Wnt2b in the dorsal region of the otocyst. In contrast, the expanded expression domain of Otx2 in the ventral otic region likely contributes to the cochlear phenotype seen in kreisler mutants. Sensory organ development is also markedly disrupted in kreisler mutants. This pattern of defects and gene expression changes is remarkably similar to that observed in Gbx2 mutants. Taken together, the data show an important role for hindbrain cues, and indirectly, kr/mafB, in guiding inner ear morphogenesis. The data also identify Gbx2, Dlx5, Wnt2b and Otx2 as key otic genes ultimately affected by perturbation of the kr/mafB-hindbrain pathway.

Animals↗
PubMed1983

Hereditary inner-ear abnormalities in animals. Relationships with human abnormalities.

A classification scheme for inner-ear abnormalities is defined, based on animals with hereditary inner-ear defects. Three major groups are (1) morphogenetic abnormalities, involving gross structural deformities of the labyrinth; (2) neuroepithelial abnormalities, in which there appears to be a primary organ of Corti defect and Reissner's membrane remains in its normal position; and (3) cochleosaccular abnormalities, showing collapse of Reissner's membranes and restriction of vestibular abnormalities to the saccule. Recent experimental findings in hearing-impaired animal mutants are taken into account. The relevance of the classification of scheme to conditions in humans is discussed. It is suggested that the Mondini and Michel deformities in humans are best considered together in the morphogenetic group. Some cases of inner-ear abnormality in humans might be considered as belonging to the neuroepithelial group.

Animals↗
PubMed2006

Gfi1 and Gfi1b act equivalently in haematopoiesis, but have distinct, non-overlapping functions in inner ear development.

Gfi1 is a transcriptional repressor essential for haematopoiesis and inner ear development. It shares with its paralogue Gfi1b an amino-terminal SNAG repressor domain and six carboxy-terminal zinc-finger motifs, but differs from Gfi1b in sequences separating these domains. Here, we describe two knock-in mouse models, in which the N-terminal SNAG repressor domain was mutated or in which the Gfi1 coding region was replaced by Gfi1b. Mouse mutants without an intact SNAG domain show the full phenotype of Gfi1 null mice. However, Gfi1:Gfi1b knock-in mice show almost normal pre-T-cell and neutrophil development, but lack properly formed inner ear hair cells. Hence, our findings show that an intact SNAG domain is essential for all functions of Gfi1 and that Gfi1b can replace Gfi1 functionally in haematopoiesis but, surprisingly, not in inner ear hair cell development, demonstrating that Gfi1 and Gfi1b have equivalent and domain-dependent, cell type-specific functions.

Animals↗
PubMed2002

Understanding inner ear development with gene expression profiling.

Understanding the development of the inner ear requires knowing the spatial and temporal pattern of gene expression, and the functions of those gene products. In the last decade, hearing research has benefited tremendously from the progress of the human and mouse genome projects, as amply illustrated by the identification of many deafness genes in both human and mouse. However, the sheer amount of information generated from the genome project has far outpaced the rate at which it is utilized. Microarray technology offers a means to quantify the expression level of transcripts at a whole-genome scale. Cross-tissue comparisons will identify genes unique to the inner ear, which will expedite the identification of new deafness genes. Microdissection and subtraction after ablation of cell types can reveal genes expressed in certain cells, such as hair cells. Expression profiling of both inner ear and other tissues, under a variety of conditions (such as during development, with drug treatment or in knock-out animals), can be used for cluster analysis to group genes of similar expression. Coexpression can suggest functional pathways and interactions between known genes, and can identify new genes in a structure or pathway. In this review we give examples for both transcription factors and cochlear structures.

Animals↗
PubMed1992

The effect of anaphylatoxin component on inner ear damage.

It has already been reported that anaphylatoxin (AT) is closely related to inner ear lesions. In this study the pathology of inner ear damage caused by AT was investigated in Hartley strain and C4 deficient guinea pigs (C4D-GP). Although the biological activity of C3a and C5a is strong, it is of short duration, and the resulting ear damage is considered to be reversible. Because of the cellular degeneration caused by this slight damage, the damage owing to continuously activated C4a is thought to be irreversible, that is, atrophy in the stria vascularis, degeneration and sloughing of the cochlear neurons, and stretching of Reissner's membrane, and C4a is therefore considered to be requisite for the manifestation of inner ear damage.

Anaphylatoxins↗
PubMedDate not supplied

Clinical study of the role of melanocytes in the inner ear of patients with Harada's disease.

Harada's disease is an autoimmune disorder involving melanocytes. Twenty patients with Harada's disease underwent otoneurological examination to investigate the inner ear involvement in Harada's disease and the function of the melanocytes in the inner ear. All 20 exhibited clinical and/or laboratory abnormal inner ear findings. The incidence of inner ear involvement in Harada's disease is very high. The melanocytes in the inner ear play important roles in both auditory and equilibrium function.

Adult↗
PubMed2006

Gene expression analysis of distinct populations of cells isolated from mouse and human inner ear FFPE tissue using laser capture microdissection--a technical report based on preliminary findings.

Laser Capture Microdissection (LCM) allows microscopic procurement of specific cell types from tissue sections that can then be used for gene expression analysis. We first tested this method with sections of adult mouse inner ears and subsequently applied it to human inner ear sections. The morphology of the various cell types within the inner ear is well preserved in formalin fixed paraffin embedded (FFPE) sections, making it easier to identify cell types and their boundaries. Recovery of good quality RNA from FFPE sections can be challenging, however, recent studies in cancer research demonstrated that it is possible to carry out gene expression analysis of FFPE material. Thus, a method developed using mouse FFPE tissue can be applied to human archival temporal bones. This is important because the majority of human temporal bone banks have specimens preserved in formalin and a technique for retrospective analysis of human archival ear tissue is needed. We used mouse FFPE inner ear sections to procure distinct populations of cells from the various functional domains (organ of Corti, spiral ganglion, etc.) by LCM. RNA was extracted from captured cells, amplified, and assessed for quality. Expression of selected genes was tested by RT-PCR. In addition to housekeeping genes, we were able to detect cell type specific markers, such as Myosin 7a, p27(kip1) and neurofilament gene transcripts that confirmed the likely composition of cells in the sample. We also tested the method described above on FFPE sections from human crista ampullaris. These sections were approximately a year old. Populations of cells from the epithelium and stroma were collected and analyzed independently for gene expression. The method described here has potential use in many areas of hearing research. For example, following exposure to noise, ototoxic drugs or age, it would be highly desirable to analyze gene expression profiles of selected populations of cells within the organ of Corti or spiral ganglion cells rather than a mixed population of cells from whole inner ear tissue. Also, this method can be applied for analysis of human archival ear tissue.

Animals↗
PubMed1990

Frequency selectivity and temporal resolution in patients with various inner ear disorders.

Both frequency selectivity and temporal resolution were examined in patients with various inner ear disorders. These included noise-induced hearing loss (n = 24), Menière's disease (n = 16), sudden deafness (n = 25), toxic inner ear damage (n = 14), presbyacusis (n = 38) and degenerative progressive inner ear hearing loss (n = 8). To facilitate quantitative comparison, various factors were introduced, namely, frequency resolution factor (FRF), temporal resolution factor (TRF) and a combined resolution factor (FTRF). The FRF of normal-hearing subjects in background noise conditions was found to be approximately 20% less than in comparative test conditions without noise, whereas the TRF of normal-hearing persons tested under background noise conditions showed a remarkable increase (factor 3). The frequency resolution performance and/or the temporal resolution performance were found to be impaired in all patient groups with inner ear hearing loss. This is particularly noticeable for temporal resolution in test conditions involving the addition of background noise. It can be concluded that in such cases, speech discrimination can be seriously jeopardized.

Hearing↗
PubMed2002

Inner ear pressure changes following square wave intracranial or ear canal pressure manipulation in the same guinea pig.

Inner ear pressure was measured in scala tympani with a micropipette during square wave pressure manipulation of the intracranial compartment and, subsequently, of the external ear canal (EEC) in the same guinea pig. As expected, the combination of the cochlear aqueduct and the inner ear behaves as a low-pass filtering system for intracranial pressure manipulation and as a complementary high-pass system for ear canal pressure manipulation. Time constants for pressure equalization were in the order of seconds and depended on the direction of flow through the cochlear aqueduct. Pressure equalization curves could not be fitted to a single exponential function; more complicated functions were needed for good fits, showing that the pressure equalization process is nonlinear. This means that the flow resistance of the cochlear aqueduct and/or the compliance of the cochlear windows is not constant, which is in accordance with a flow-direction dependent resistance of the cochlear aqueduct. An explanation for this can be found in the special structure of the periotic duct inside the aqueduct.

Animals↗
PubMed1977

[Do neomycin ointment dressings damage the inner-ear when used in the external auditory canal or the radical mastoidectomy cavity? (author's transl)].

When in low concentration (0.38%) in an ointment base neomycin within the middle ear does not damage the inner ear. Animal experiments show that doses 10 times greater do not damage the hair cells, but when in aqueous solution all hair cells are destroyed. Many years experience has confirmed that neomycin ointment packing for postoperative ears and infected radical mastoid cavities does not produce inner ear damage. These patients have been carefully monitored by audiometry.

Animals↗