[Exostosis of the internal auditory canal in a patient with myotonic dystrophy]. / Exostosenbildung des inneren Gehörganges bei einem Patienten mit myotoner Dystrophie Curschmann-Steinert.

A 53-year-old patient with myotonic dystrophy presented to our clinic with progressive bilateral hearing loss. The ENT status and particularly the otological examination were without pathological signs. Pure tone audiograms showed a bilateral moderate to severe sensorineural hearing loss. Routinely performed computed tomography of the temporal bones revealed the rare picture of exostosis of the internal auditory canals and the medial surface of the petrous bones. To our knowledge, this is the first report describing exostosis of the internal auditory canal in a patient with myotonic dystrophy, although at present it remains unclear in how far there is a causal connection between these two pathologies.

[Characterisation of the gene expression profiles in the inner ear and the colliculus inferior of normal and deafened rats by gene-array-technology]. / Charakterisierung der Genexpression im Innenohr und der Hörbahn hörender und ertaubter Ratten mittels Gene-Arrays.

BACKGROUND: The phenotype of deafness and its mechanisms are morphologically and electrophysiologically well characterised. However, the molecular mechanisms and the consequences of deafness are poorly understood. METHODS: In this study we investigated changes in gene expression profiles in subfractions of the cochlea and the colliculus inferior, a non-cochlear tissue, of normal and deafened (10 % Neomycin) rats using the gene-array-technology. RNA was prepared from modiolus (Mo) und sensorineural epithel/lateral wall (SnE/Lw) und Colliculus inferior (IC), reverse transcribed with gene specific primers, labeled with (32)P-dATP and hybridised with its complementary sequences of 1200 rat ESTs. RESULTS: Similar gene expression profiles were detected in Mo- and SnE/Lw in normal as well in deafened rats differing significantly from those found in IC. In deafened animals differences in mRNA levels were determined in IC for 8 genes, in Mo für 17 genes and in SnE/Lw for 25 genes in comparison to those of normal rats. By using gene-arrays many genes described in the literature previously could be detected. Otherwise most of the genes found in the cochlea are unknown. CONCLUSIONS: The gene-array-technology is a valuable tool in otological research for gene expression analysis and, therefore, for comprehensive understanding of molecular processes in the inner ear. Furthermore gene screening for candidate genes could be a big step ahead in developing therapies of diseases of the inner ear.

Technical report: modification of a cochlear implant electrode for drug delivery to the inner ear.

OBJECTIVE: To investigate the possibility of modifying a cochlear implant electrode for the purpose of drug delivery to the cochlea. BACKGROUND: Animal experiments suggest that local therapy of the inner ear could be a promising new approach to the interventional treatment of inner ear disease, and that pharmacologic intervention could possibly enhance cochlear implant performance. One of the key aspects is the deployment of a means of drug delivery to the human inner ear. METHODS: The tip of the Contour electrode array was cut to open the lumen of the array, and a connecting piece was developed to connect the electrode to a pump. The feasibility of using the array for drug delivery was tested using both an Alzet mini-osmotic pump and a mechanical pump. The connection was tested for its stability in terms of leakage and resistance to tractive forces. The system was also applied to temporal bones to evaluate its applicability to the human cochlea. RESULTS: The modified Contour electrode is easy to handle in temporal bones and can be used to simulate drug delivery to the inner ear. The connection to the pump was sealed for all tested pump rates and resisted tractive forces up to 50 N. CONCLUSIONS: The described modified electrode could provide a safe and easy-to-handle means of combining electrical stimulation with the beneficial effects of a local drug therapy applied to the inner ear.

Telomerase activity, telomere length, and apoptosis: a comparison between acquired cholesteatoma and squamous cell carcinoma.

BACKGROUND: Cholesteatoma disease is characterized by accumulation of keratinizing epithelium. Several molecular markers of tumor formation have been found in cholesteatoma (e.g. upregulation of matrix metalloproteinases, c and activation of angiogenesis). Other molecular findings clearly distinguish between cholesteatoma and malignant tumors (e.g., lack of chromosomal instability, intact checkpoint responses). To further distinguish the molecular mechanisms in cholesteatoma from malignant tumors, the authors determined telomerase activity and telomere length in both tissue types. METHODS: To evaluate the role of telomerase activation and telomere length in cholesteatoma, 29 cholesteatoma samples and 9 squamous cell carcinomas were analyzed for telomerase activity and telomere length. In addition, the rate of apoptosis was determined in both groups, using the TdT-mediated dUTP nick end labeling technique. RESULTS: As previously described, a high proportion of squamous cell carcinoma exhibited telomerase activity (6/9, 66%). By contrast, a significantly lower rate of telomerase activity was found in cholesteatoma samples (1/29, 3.4%, p = 0.0002). Despite the differences in telomerase activity, the telomere length was similar in cholesteatoma (mean length 7.43 kb) and in squamous cell carcinoma (mean length 7.99 kb; difference not significant, p = 0.1364). The low rate of telomerase activity in cholesteatoma was accompanied by significantly higher rates of apoptosis in cholesteatoma (mean 30%) compared with squamous cell carcinoma tissue (mean 3%, p = 0.0031). CONCLUSIONS: Taken together, these data show that telomerase activation is a rare event in cholesteatoma and that the absence of telomerase activity is accompanied by high rates of apoptosis in cholesteatoma. It is proposed that the absence of telomerase limits the proliferative capacity of cholesteatoma by induction of apoptosis, whereas the presence of telomerase allows immortal growth of squamous cell carcinoma.