Idiopathic sudden sensorineural hearing loss (ISSNHL) is a heterogenic disease. Multiple factors influencing aetiology and prognosis are discussed. A retrospective clinical characterisation and analysis of family history of ISSNHL patients was performed to investigate influences on the disease. 186 inpatients diagnosed with ISSNHL were characterised by health records and a standardised questionnaire. Audiograms were observed. 75 controls that had never experienced an event of ISSNHL were questioned about family members being affected by ISSNHL. 63.4% of all patients could be assigned to at least one group with similar causes of ISSNHL (noise exposure, positive family history, infectious diseases, hypothyreoidism and fibromuscular dysplasia). A positive family history for ISSNHL has not been reported so far. Therefore, we accentuated the characterisation of patients with positive family history. 21.4% affirmed a positive family history. In ten families, at least two family members were reported as ISSNHL patients. In comparison with patients with negative family history, they tend to be younger, experience more events of ISSNHL and show less improvement of hearing abilities under therapeutic treatment (non-significant). Differences intensified between smokers with positive family history and non-smokers with negative family history. Differences concerning average age were statistically significant ( p = 0.001). Within 75 controls 11 families were reported with one member being affected by ISSNHL. In the control group we did not detect any family with more than one ISSNHL patient. The results indicated that patients with positive family history tend to have an aggravated course of ISSNHL. Further studies should help to confirm these results and to identify environmental or genetic factors leading to ISSNHL. This might support a better understanding of the aetiology of ISSNHL and offer new possibilities for prevention and therapy.

Recombinant human bone morphogenetic protein-2 (rhBMP-2) is a growth factor of the transforming growth factor-beta superfamily. Members of this protein family are involved in the development of various mammalian tissues, including the inner ear. As their notations indicate, they also have well-known effects on bone formation and regeneration. In this study, we examined the influence of rhBMP-2 on spiral ganglion (SG) neurite growth in vitro and showed the presence of its most preferred receptor BMPR-IB in spiral ganglion cells both in vitro and in vivo. SG explants of postnatal day 4 rats were analysed for neurite length and number after organotypical cell culture for 72 h, fixation and immunolabeling. Different concentrations of rhBMP-2 were used in a serum-free culture media. Neurite growth was compared with control groups that lacked stimulative effects; with neutrophin-3 (NT-3), which is a well-established positive stimulus on neurite length and number; and with combinations of these parameters. The results display that neurite number and total neurite length per explant in particular concentrations of rhBMP-2 increased by a maximum factor of two, while the mean neurite length was not affected. NT-3 demonstrated a much more potent effect, delivering a maximum increase of a factor of five. Furthermore, a combination of both growth factors shows a predominant effect on NT-3. Immunohistological detection of BMPR-IB was successful both in cell culture explants and in paraffin-embedded sections of animals of different ages. The results show that rhBMP-2 is, among other growth factors, a positive stimulus for SG neurite growth in vitro. Most growth factors are unstable and cannot be attached to surfaces without loss of their biological function. In contrast, rhBMP-2 can be attached to metal surfaces without loss of activity. Our findings suggest in vivo studies and a future clinical application of rhBMP-2 in cochlear implant technology to improve the tissue/electrode interface.

Mit der zunehmenden Verbreitung und Weiterentwicklung des Kochleaimplantats (CI) ist das Einführen von Fremdmaterialien in das Innenohr beim Menschen ein etabliertes Verfahren. Jedoch ist die Biokompatibilität des Elektrodenmaterials sowie die Wechselwirkung der Elektrode mit den neuronalen Strukturen noch nicht abschließend geklärt.

With the increasing use of cochlear implants (CIs), the insertion of alloplastic material into the inner ear is nowadays an established treatment for severe to profound hearing loss in children and adults. Beyond its widespread use, the biocompatibility of the CI electrode and its interaction with the neural structures of the cochlea is not yet established. To investigate the survival and growth behavior of spiral ganglion neurons on different CI materials, spiral ganglion explants from newborn rats were cultured on silicone and platinum, on a surface combination of silicone and platinum, and, finally, on a CI electrode. The results of this study indicate that the growth of spiral ganglion neurons in vitro is strongly influenced by the different materials and their arrangement, with platinum exhibiting the highest degree of biocompatibility with respect to neurite extension. Level differences in the surface structure between silicone and platinum lead to inhibition of neurite outgrowth. Furthermore, the culturing of spiral ganglion explants on a CI electrode leads to neurite sprouting toward the electrodes made of platinum. The biocompatibility of CI materials with spiral ganglion neurons was shown in this study, but it differs with different CI materials. Besides the material itself, the arrangement of the materials can affect the neurite extension.

Die wichtigsten Teilgebiete, in denen zukünftig we sentliche Neuerungen zu erwarten sind, umfassen: –  Onkologie von Kopf-Hals-Tumoren einschließlich Stammzelltherapie –  Gentherapie und „new targets“ –  Regeneration von Strukturen des Innenohrs –  gezielte medikamentöse Therapie bestimmter Organe („drug delivery“) –  Implantate neuen Designs und mit modifizierten, „biologisierten“ Oberflächen

BACKGROUND:This study was designed to assess the postoperative quality of life (QoL) of 30 patients successfully treated with a laryngectomy.METHODS:QoL was evaluated with two validated questionnaires (EORTC-QLQ-C30 and EORTC-QLQ-H&N35) during the regular outpatient follow-up. To analyse the long-term quality of life, we divided our collective into two groups of patients; one consisting of patients whose treatment had been completed less than five years ago and one whose therapy had been finished more than five years ago.RESULTS:The collective consisted of 24 men (80 %) and 6 (20 %) women with a mean age of 58.8 +/- 8.7 years. Mean follow-up was 53 months (range: 12 - 110). Most patients (n = 15, 50 %) had a tumor stadium IV after UICC. Voice rehabilitation was achieved in all patients with a Provoxreg voice prosthesis. The global QoL was rated as satisfying with a mean value of 62. The analysis of the specific questionnaire showed mostly a limitation of smell. Patients, whose treatment was longer than five years ago, showed a slight improvement in the global QoL. Overall we could not find a significant difference concerning the QoL between the two groups.CONCLUSIONS:In our study the QoL of laryngectomized patients was satisfying. The most common symptoms were problems with smell, cough and dyspnoea.

Background: Utilising the enormous proliferation and multi-lineage differentiation potentials of somatic stem cells represents a possible therapeutical strategy for diseases of non-regenerative tissues like the inner ear. In the current study, the possibility of murine neural stem cells to contribute to the developing inner ear following blastocyst injection was investigated.Methods: Fetal brain-derived neural stem cells from the embryonic day 14 cortex of male mice were isolated and expanded for four weeks in neurobasal media supplemented with bFGF and EGF. Neural stem cells of male animals were harvested, injected into blastocysts and the blastocysts were transferred into pseudo-pregnant foster animals. Each blastocyst was injected with 5-15 microspheres growing from single cell suspension from neurospheres dissociated the day before. The resulting mice were investigated six months postpartum for the presence of donor cells. Brainstem evoked response audiometry (BERA) was performed in six animals. To visualize donor cells Lac-Z staining was performed on sliced cochleas of two animals. In addition, the cochleas of four female animals were isolated and genomic DNA of the entire cochlea was analyzed for donor contribution by Y-chromosome-specific PCR.Results: All animals had normal thresholds in brainstem evoked response audiometry. The male-specific PCR product indicating the presence of male donor cells were detected in the cochleas of three of the four female animals investigated. In two animals, male donor cells were detected unilateral, in one animal bilateral.Conclusion: The results suggest that descendants of neural stem cells are detectable in the inner ear after injection into blastocysts and possess the ability to integrate into the developing inner ear without obvious loss in hearing function.

Background: The etiology and factors influencing the prognosis of idiopathic sensorineural hearing loss are still in focus. To determine pathogenetic relevant factors, a detailed clinical characterization of patients with idiopathic sudden sensorineural hearing loss remains to be performed.Methods: The history of 103 patients who presented with a sudden sensorineural hearing loss and received a standard treatment with prednisolone und pentoxyphylline (Stennert-Schema) has been investigated with a new questionnaire. In additon, audiological and serological investigations were analysed.Results: A partial or total recovery of hearing after therapy was reported by 57 % of patients, on average 15 dB. Half of the patients sustained at least one recurrent idiopathic sensorineural hearing loss. In single cases, fibromuscular dysplasia and hypothyroidism in autoimmunthyroidism were detected. Additionally, smoking was identified as a disease modulating factor. The age of admission to the hospital was significantly reduced in the group of smokers compared to non-smokers (p = 0.02). Non-smokers reported more episodes of sensorineural hearing loss compared to smokers (p=0.02) according to their higher age. One third of the patients reported a high level of noise exposure in private and business, which was related with the sudden sensorineural hearing loss.Conclusion: By collecting an exact medical history, in more than 50% of cases etiological and predisposing factors of sudden sensorineural hearing loss can be detected which lead to further diagnostic investigations and therapies.

Objective: This case is reported in order to demonstrate the importance of detailed clinical analysis, including evaluation of personal and family history, in the differential diagnosis of sudden sensorineural hearing loss.Case report: A 50-year-old woman presented with a sudden onset of sensorineural hearing loss in her right ear. She had experienced three previous episodes of sudden sensorineural hearing loss in her left ear, at the ages of 35, 48 and 50 years. She also reported suffering two strokes with left hemiparesis due to fibromuscular dysplasia of her right internal carotid artery. A positive family history of stroke among maternal relatives suggested autosomal dominant inheritance. The patient's personal and family history suggested a rare cause of sudden sensorineural hearing loss, for which alternative therapeutic modalities may be applicable in selected cases.Conclusions: Careful follow up of any patient with sudden sensorineural hearing loss and evaluation of their personal and family history is essential, in order to uncover evidence of rare underlying causes of sudden sensorineural hearing loss. For patients with such rare diagnoses, alternative therapy and surveillance modalities may be useful in disease management, depending on pre-existing pathology. Those patients should be managed via a multidisciplinary approach, including genetic counselling, in order to achieve the best possible outcome.

Objectives: To investigate posterior external ear canal wall reconstruction with a composite cartilage titanium mesh graft in canal wall down tympanoplasty and revision surgery for open mastoids.Study design: Retrospective case review.Setting: Tertiary referral centre.Methods: As a preliminary study, 15 selected patients underwent reconstruction of a posterior ear canal wall defect with titanium mesh. Large defects of the posterior external auditory canal wall, resulting from canal wall down tympanoplasty or present in revision surgery, were eliminated by reconstruction using a titanium mesh. The mesh was covered with conchal cartilage and attached to the cortical mastoid bone using 3-mm titanium screws.Results: All patients maintained a normal contour of the external ear canal, without depression, extrusion or infection. There were no failures, based on short-term post-operative controls. However, two procedures had to be revised due to incomplete coverage of the titanium mesh.Conclusions: This study shows that reconstruction of the posterior ear canal wall with a composite cartilage titanium mesh is a valuable method for preserving the morphology of the external auditory canal in selected cases. Problems occurring in canal wall down tympanomastoidectomy and radical cavities may therefore be avoided. However, long-term results have yet to be evaluated.

Background: Commonly used staging procedures often cannot predict the absence of lymphatic micro- metastases in squamous cell carcinoma (SCC) of the head and neck. Therefore in many cases an elective neck dissection (ND) is necessary. In the surgical therapy of melanoma or breast cancer the presence of metastases can be evaluated securely by identification and examination of the sentinel lymph node (SLN). The type of surgical procedure is usually chosen in regard to the histopathological result. The present study evaluates the applicability of this concept for SCC of the head and neck.Methods: Radioactive labeled microalbumine particles were injected preoperatively around the tumor in 38 patients without proven metastases. Following the excision of the primary tumor the sentinel lymph node/s were detected and dissected and ND was completed. Histological examination of the tissue was performed to evaluate whether the SLN reflected the lymphatic status.Results: In two cases (5,1%) no SLN were detected. ND was completed in 32 cases. In nine cases (28,1%) the SLN were infiltrated by the primary tumor. In 22 cases (68,8 %) SLN and ND revealed a No stage. In one case (3,1 %) we could not identify a metastasis because of the anatomical closeness of the SLN to the primary. The negative predictive value was 96%.Conclusion: Predictive value regarding metastases to the neck was higher with the detection of SLN than with conventional staging procedures. Whether the detection of a tumor-free SLN is an indication not to perform an elective neck dissection is a matter of discussion, especially under the aspect of the effective reduction of postoperative morbidity.

BACKGROUND:Commonly used staging procedures often cannot predict the absence of lymphatic micro- metastases in squamous cell carcinoma (SCC) of the head and neck. Therefore in many cases an elective neck dissection (ND) is necessary. In the surgical therapy of melanoma or breast cancer the presence of metastases can be evaluated securely by identification and examination of the sentinel lymph node (SLN). The type of surgical procedure is usually chosen in regard to the histopathological result. The present study evaluates the applicability of this concept for SCC of the head and neck.METHODS:Radioactive labeled micro-albumin particles were injected preoperatively around the tumor in 38 patients without proven metastases. Following the excision of the primary tumor the sentinel lymph node/s were detected and dissected and ND was completed. Histological examination of the tissue was performed to evaluate whether the SLN reflected the lymphatic status.RESULTS:In two cases (5.1 %) no SLN were detected. ND was completed in 32 cases. In nine cases (28.1 %) the SLN were infiltrated by the primary tumor. In 22 cases (68.8 %) SLN and ND revealed a N(0) stage. In one case (3.1 %) we could not identify a metastasis because of the anatomical closeness of the SLN to the primary. The negative predictive value was 96 %.CONCLUSION:Predictive value regarding metastases to the neck was higher with the detection of SLN than with conventional staging procedures. Whether the detection of a tumor-free SLN is an indication not to perform an elective neck dissection is a matter of discussion, especially under the aspect of the effective reduction of postoperative morbidity.

Introduction: The question of whether a decompression of the optic nerve should be performed after skull trauma is still discussed controversially. Different studies using corticosteroids to reduce an edema of the optic nerve within its bony canal show similar results in enhancing the vision as do studies that performed a surgical decompression of the nerve. The question of whether a decompression should be performed several days after the trauma is even more difficult.

At birth, one in 850 babies are profoundly deaf, and hearing loss affects more than 50% of all people over 60 years of age. While hearing loss caused by disease of the external and/or middle ear is treatable, hearing loss as a result of damage to and loss of hair cells and/or auditory neurons can only be alleviated using prosthetic devices such as hearings aids or cochlear implants.

Ephrine und Eph-Rezeptoren (Eph) sind bidirektionale Signalmoleküle, die eine wesentliche Rolle in der Entwicklung des Nervensystems spielen. Aktuellen immunhistochemischen Studien zufolge wird EphA4 in der Lamia ossis spiralis (OSL) der embryonalen und postnatalen Cochlea exprimiert. Dies kann funktionelle Bedeutung haben, da die OSL von Spiralganglienneuriten auf ihrem Weg in das Cortische Organ durchwachsen wird. Auch Liganden des EphA4, die Ephrine B2 und B3, werden von Spiralganglienzellen exprimiert. In vorangegangenen Studien konnten wir zeigen, dass EphA4 eine repulsive Wirkung auf Neuriten von Spiralganglienzellen hat.

Gehörgangsexostosen sind multiple, bilaterale, breitbasige Wucherungen tympanalen Knochens des medialen äußeren Gehörgangs. Oft werden die asymptomatischen Tumore als Zufallsbefund während einer otologischen Untersuchung entdeckt. In der Regel wird die Indikation zur chirurgischen Entfernung der Exostosen gestellt, wenn 80% oder mehr des Kanallumens verschlossen oder die Patienten symptomatisch sind.

Einleitung: P75NTR ist ein niederaffiner Rezeptor für Neutrophine. Studien bezüglich der Interaktion zwischen p75NTR und den hochaffinen Tyrosin Kinase Rezeptoren (Trk) in verschiedenen neuronalen Systemen unterstreichen die Bedeutung von p75NTR für die Modulation von Signalkaskaden der Neutrophine. In der vorliegenden Studie wurde der Einfluss von p75NTR auf die Entwicklung der Säugetiercochlea in vivo und in vitro untersucht.

Studies have demonstrated different pathogenetic key factors in gentamicin-induced hair cell death. The production of reactive oxygen species (ROS), as well as apoptosis-related genes, play a critical role. However, a coordinated large-scale investigation of gene expression in the organ of Corti (OC) exposed to gentamicin has not yet been conducted. Here we used DNA microarray technology to compare the expression profile of OC exposed to gentamicin to the expression profile of untreated OC. The OCs of Sprague Dawley rats were dissected and the basal turns were cultured. Two-thirds of the explants were then exposed to l00 microM gentamicin, for 4 and 8 h, while one-third of the explants remained in culture medium alone. Gene expression was analyzed using DNA microarray technology and the dChip software package. Based on the results, the 4-h time-point was chosen for further analysis. In these assays, out of 8800 genes, 12 genes were identified on the basis of differential expression in the OC exposed to gentamicin vs. control OC. The identity of these genes suggests that the response of the OC to the gentamicin challenge involves down-regulation of specific gene families in order to alleviate ROS and N-methyl-D-aspartate (NMDA) receptor-mediated cellular stress.

Rhinogene Hirnabszesse gehen meist von einer Stirnhöhleneiterung aus, selter vom Siebbeinsystem, der Keilbeinhöhle oder der Kieferhöhle. Dennoch ist ein Entzündungstransfer von einem isolierten Kieferhöhlenempyem zum Endokranium möglich.