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[Exploratory analysis of risk factors of acute inner ear dysfunctions].

Aside from the clinical picture, the frequent occurrence of vascular risk factors favors a vascular pathogenesis of acute sensorineural hearing loss. However, the data on the association of vascular risk factors with sensorineural hearing loss are controversial. Achieving a sufficient number of patients and establishing a suitable control group is difficult. We analyzed the data of 393 patients suffering from acute sensorineural hearing loss. Risk factors, general, audiological, and laboratory parameters were investigated. Mean hearing loss and remission during 10 days of hospital treatment were calculated. The patient group was divided into patients with a certain risk factor and those without this risk factor, and mean remission and hearing loss were compared. Patients suffering from hyperlipidemia or hypotension had substantial and significantly higher mean hearing losses than patients not suffering from these diseases. Thrombosis, embolism, and hypertension were associated with a significantly worse remission of hearing loss. Repeated episodes of sensorineural hearing loss showed a significant worse remission, but less mean hearing loss before therapy. Smoking, diabetes mellitus, infections of the upper airways, and allergies had no influence on remission and mean hearing loss.

Adolescent↗

[Petrous bone tumors: primary non-Hodgkin lymphoma of the inner ear canal and cerebellopontile angle].

BACKGROUND: We report about a primary Non-Hodgkin Lymphoma (NHL) of the internal auditory canal. The only previously known manifestations of a NHL in the temporal bone have been infiltrations or hemorrhagic complications due to a late manifestation or advanced systemic disease. Involvement of both temporal bones is typical. CLINICAL CASE: The 60-year-old female patient complained of an acute one-sided deafness, accompanied by a high-pitched tinnitus, rotating vertigo, and paralysis of the left half of the face. RESULTS: We found a deafness in the left ear, spontaneous nystaxis, which was interpreted as a deficiency in excitement of the vestibular organ, and a complete peripheral facial paralysis. Diagnostic imaging studies revealed a large, intrameatal solid mass in the temporal bone, measuring 1.2 x 0.8 cm. Histologic examination after translabyrinthine tumor removal demonstrated a centroblastic Non-Hodgkin Lymphoma. The following extensive interdisciplinary staging examination showed no other tumor manifestations; the CSF analysis was negative. CONCLUSIONS: The uniqueness of this case lies in the detection of a primary nongeneralized centroblastic lymphoma of the internal auditory canal. In contrast to infiltrations of systemic NHL in the same location, in which the advanced disease is responsible for the bad prognosis, this isolated lymphoma of the internal auditory canal seems analogous to extranodal MALT Lymphomas with a better prognosis. The primary extranodal NHL of the temporal bone, not reported in previous studies, is discussed with regard to clinical symptoms, differential diagnoses, and therapeutic strategies.

Combined Modality Therapy↗

[Hormone receptors in the inner ear].

Vasopressin binding sites could be clearly demonstrated in the cochlea. Membrane staining was mainly limited to the apical and ciliar membranes of the cochlear and vestibular hair cells, and hence to membranes in which adenylate cyclase activity could not be demonstrated. In addition to V2-vasopressin receptors that mediate hormonal signals by adenylate cyclase activation and cAMP release, in V1-vasopressin-receptors extracellular vasopressin signal is mediated by the breakdown of inositol phosphates and the release of inositol-triphosphates and diacylglycerol. Inositol triphosphates were found to be responsible for the intracellular mobilization of calcium. The localization of vasopressin binding sites at the hair cell membranes, therefore, suggests that vasopressin contributes to the breakdown and release of phospholipid messenger molecules and is thus probably involved in cochlear and vestibular signal transduction.

Animals↗

Gap junctional hemichannel-mediated ATP release and hearing controls in the inner ear.

Connexin gap junctions play an important role in hearing function, but the mechanism by which this contribution occurs is unknown. Connexins in the cochlea are expressed only in supporting cells; no connexin expression occurs in auditory sensory hair cells. A gap junctional channel is formed by two hemichannels. Here, we show that connexin hemichannels in the cochlea can release ATP at levels that account for the submicromolar concentrations measured in the cochlear fluids in vivo. The release could be increased 3- to 5-fold by a reduction of extracellular Ca2+ or an increase in membrane stress, and blocked by gap junctional blockers. We also demonstrated that extracellular ATP at submicromolar levels apparently affected outer hair cell (OHC) electromotility, which is an active cochlear amplifier determining cochlear sensitivity to sound stimulation in mammals. ATP reduced OHC electromotility and the slope factor of the voltage dependence and shifted the operating point to reduce the active amplifier gain. ATP also reduced the generation of distortion products. Immunofluorescent staining showed that purinergic receptors P2x2 and P2x7 were distributed on the OHC surface. Blockage of P2 receptors eliminated the effect of ATP on the OHC electromotility. The data revealed that there is a hemichannel-mediated, purinergic intercellular signaling pathway between supporting cells and hair cells in the cochlea to control hearing sensitivity. The data also demonstrated a potential source of ATP in the cochlea.

Adenosine Triphosphate↗

Notch signaling in the development of the inner ear: lessons from Drosophila.

The sensory patches in the ear of a vertebrate can be compared with the mechanosensory bristles of a fly. This comparison has led to the discovery that lateral inhibition mediated by the Notch cell-cell signaling pathway, first characterized in Drosophila and crucial for bristle development, also has a key role in controlling the pattern of sensory hair cells and supporting cells in the ear. We review the arguments for considering the sensory patches of the vertebrate ear and bristles of the insect to be homologous structures, evolved from a common ancestral mechanosensory organ, and we examine more closely the role of Notch signaling in each system. Using viral vectors to misexpress components of the Notch pathway in the chick ear, we show that a simple lateral-inhibition model based on feedback regulation of the Notch ligand Delta is inadequate for the ear just as it is for the fly bristle. The Notch ligand Serrate1, expressed in supporting cells in the ear, is regulated by lateral induction, not lateral inhibition; commitment to become a hair cell is not simply controlled by levels of expression of the Notch ligands Delta1, Serrate1, and Serrate2 in the neighbors of the nascent hair cell; and at least one factor, Numb, capable of blocking reception of lateral inhibition is concentrated in hair cells. These findings reinforce the parallels between the vertebrate ear and the fly bristle and show how study of the insect system can help us understand the vertebrate.

Animals↗

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

Mutations in the potassium channel gene KCNQ4 underlie DFNA2, an autosomal dominant form of progressive hearing loss in humans. In the mouse cochlea, the transcript has been found exclusively in the outer hair cells. By using specific antibodies, we now show that KCNQ4 is situated at the basal membrane of these sensory cells. In the vestibular organs, KCNQ4 is restricted to the type I hair cells and the afferent calyx-like nerve endings ensheathing these sensory cells. Several lines of evidence suggest that KCNQ4 underlies the I(K,n) and g(K,L) currents that have been described in the outer and type I hair cells, respectively, and that are already open at resting potentials. KCNQ4 is also expressed in neurons of many, but not all, nuclei of the central auditory pathway, and is absent from most other brain regions. It is present, e.g., in the cochlear nuclei, the nuclei of the lateral lemniscus, and the inferior colliculus. This is the first ion channel shown to be specifically expressed in a sensory pathway. Moreover, the expression pattern of KCNQ4 in the mouse auditory system raises the possibility of a central component in the DFNA2 hearing loss.

Amino Acid Sequence↗

Buffered papaverine facilitates passage of intratympanic dexamethasone to the inner ear.

CONCLUSION: We conclude that when papaverine is intratympanically administered together with dexamethasone after buffering, passage of dexamethasone to the inner ear is increased. OBJECTIVE: To determine the influences of papaverine and buffered papaverine on passage of dexamethasone administered intratympanically to the inner ear. MATERIALS AND METHODS: Twenty-seven Vienna white rabbits were divided into five groups: eight received intratympanic dexamethasone (4 mg/ml) (group 1), seven received intratympanic papaverine (10 mg/ml) + dexamethasone (2 mg/ml) (group 2), six received buffered (with sodium bicarbonate 8.4%) intratympanic papaverine (7.5 mg/ml) + dexamethasone (1.5 mg/ml) (group 3), three had basal cortisol levels in venous blood and perilymph measured (group 4), and three received intravenous papaverine + dexamethasone (group 5). At 1 h after the administration of the drugs, dexamethasone levels in ipsilateral and contralateral perilymph and venous blood were measured by radioimmunoassay. RESULTS: Animals in group 3 demonstrated the highest levels of ipsilateral perilymph dexamethasone. Ipsilateral perilymph levels were significantly higher in groups 1 and 3 than they were in the other groups (p<0.05). Although the perilymph levels observed in animals in group 2 were slightly higher than those in group 4, no significant difference existed (p = 0.160).

Animals↗

THE ULTRASTRUCTURE OF THE KINOCILIUM OF THE SENSORY CELLS IN THE INNER EAR AND LATERAL LINE ORGANS.

The bundle of sensory hairs protruding from the top of each receptor cell in the vestibular and lateral line organs in the teleost fish (burbot) Lota vulgaris is composed of a number of stereocilia and one kinocilium located in the periphery of the bundle. The ultrastructure of the kinocilium and its basal body is described. It is found that the kinocilium is morphologically polarized by the asymmetric arrangement of its component fibers and of the basal body by the presence of a basal foot. Peripheral fibers 5 and 6 of the kinocilium and the basal foot of the basal body are oriented away from the stereocilia; that is, in a direction coinciding with the direction of excitatory stimulation. The findings are discussed in terms of directional sensitivity.

Animals↗