The increasing application of sophisticated electrophysiological, radiological and surgical methods to the diagnosis and treatment of laryngeal disorders requires a profound knowledge of the size and proportions of the human larynx and it's cartilaginous components. Only inadequate data regarding this subject have so far been accessible. The larynges of 53 patients (28 male and 25 female, age 25-88 years, in the means 59 years) were removed during routine autopsy 12-48 h post mortem and immediately submitted to morphometric investigation. None of the patients had histories or visible signs of laryngeal disease. Anatomical preparations were performed with customary surgical tools and morphometric measurements then carried out with a pair of compasses and a calliper rule. A total of 95 measurements were performed on each larynx. These included, aside from evaluation of the whole organ, identification of the internal and external diameters of the cricoid cartilage, height and length of the thyroid alae in different planes, angle of thyroid laminae, height of arytenoid cartilage, width and length of epiglottic cartilage, and internal and external diameter of first tracheal ring. The results obtained provide a full scale of data determining the size and extent not only of it's cartilaginous components, but of the laryngeal framework as a whole. The knowledge of these data may contribute to a precise positioning of electrodes in laryngeal electromyography, to the planning of laryngeal framework surgery, and to the analysis of CT- and MRI-scans of the larynx.
The present multicentric clinical study involves 19 centres, 16 of them in German-speaking countries, 1 British, 1 Polish and 1 Hungarian. 60 postlingually deafened adults with a mean age of 47.5 years (20–70) and a mean duration of deafness of 5.3 years (0.5–20) have been evaluated with the MED-EL COMBI 40 cochlear implant which implements a high-rate continuous-interleaved-sampling strategy with 8 channels. Safety and effectiveness data have been collected. Speech perception tests include a 16-consonant, an 8-vowel, a sentence and a monosyllabic-word test in all languages and a 2-digit figure test in all languages but English. Test intervals are 1,3,6 months and 1 year after first fitting. 41 of the 60 postlingually deafened adult study patients have completed their 6-month evaluation. While their pre-operative monosyllabic-word score was 0%, their mean monosyllabic-word score 6 months after first fitting was 48% (8–90) with a median of 50%. The mean sentence understanding was 84% (24–100) with a median of 90%. The respective values for the 1-year evaluations with 25 patients are a mean of 50% (5–85), with a median of 60%, for the monosyllables and a mean of 89% (30–100), with a median of 97%, for the sentences.
Background: Malfunction of the SLC26A4 protein leads to prelingual deafness often associated with mild thyroid dysfunction and goiter. It is assumed that SLC26A4 acts as a chloride/anion exchanger responsible for the iodide organification in the thyroid gland, and conditioning of the endolymphatic fluid in the inner ear. Methods: Chloride uptake studies were made using HEK293-Phoenix cells expressing human wild type SLC26A4 (pendrin) and a mutant (SLC26A4S28R) we recently described in a patient with hypothyroidism, goiter and sensorineural hearing loss. Results: Experiments are summarized showing the functional characterization of wild type SLC26A4 and a mutant (S28R), which we described recently. This mutant protein is transposed towards the cell membrane, however, its transport capability is markedly reduced if compared to wild-type SLC26A4. Furthermore, we show that the SLC26A4 induced chloride uptake in HEK293-Phoenix cells competes with iodide, and, in addition, that the chloride uptake can be blocked by NPPB and niflumic acid, whereas DIDS is ineffective. Conclusions: The functional characteristics of SLC26A4S28R we describe here, are consistent with the clinical phenotype observed in the patient from which the mutant was derived.
Safety and efficacy of the new bone conduction implant was demonstrated in children followed up to 3 months postoperatively.
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