The objective of this study was to present 5 years of surgical experience, and the extended results of hearing preservation (based on 3-year follow-up), with the Med-El Vibrant Soundbridge (VSB) in which the floating mass transducer (FMT) is placed directly against the round window membrane, and the fascia is used only as covering tissue to keep it in position. A retrospective survey of surgical and audiological data was conducted to evaluate the performance and stability of patient hearing, with audiometric measurements performed over fixed time intervals up to 36 months. 21 patients, aged 19–62 years (mean 48.4), with mixed or conductive, bilateral or unilateral hearing loss were included in this study. Surgical intervention involved monaural implantation of the Med-El VSB between 2006 and 2009. The results were assessed using pure tone audiometry. In 5 years of experience with the technique, no significant complications or device extrusion were observed except for two revision surgeries requiring FMT repositioning. In the 3-year follow-up, we observed stable hearing in the implanted ear. It is concluded that direct round window stimulation without interposed fascia is an alternative for patients with hearing impairment caused by chronic otitis media and/or lack of ossicles, especially after modified radical mastoidectomy. It allows good results in a selected group of patients, although further observation on a larger population is needed to confirm long-term validity and effectiveness.

After stapedotomy, with the use of a Teflon piston prosthesis, stimulation of inner ear structures diminishes, especially in higher frequencies.

Piston-stapedotomy is the most common method for hearing restoration in patients with otosclerosis. In this study, we have experimentally examined a prototype of a new chamber stapes prosthesis. The prototype was implanted in a human cadaver temporal bone. The round window vibrations before and after implantation were measured for the acoustic signal (90 dB SPL, 0.8–8 kHz) in the external auditory canal. In comparison with a 0.4-mm piston prosthesis, the chamber prosthesis induced significantly higher vibration of the round window, especially for frequencies above 1.5 kHz. Based on the results, it can be surmised that stapedotomy with a chamber stapes prosthesis could provide better hearing results in comparison with the piston-stapedotomy.

POU3F4 mutations (DFNX2) are the most prevalent among non-syndromic X-linked hearing loss (HL) identified to date. Clinical manifestations of DFNX2 usually comprise congenital HL either sensorineural or mixed, a tendency towards perilymphatic gusher during otologic surgery and temporal bone malformations. The aim of the present study was to screen for POU3F4 mutations in a group of 30 subjects with a suggestive clinical phenotype as well as a group (N = 1671–2018) of unselected hearing loss patients. We also planned to analyze audiological and radiological features in patients with HL caused by POU3F4 defects. The molecular techniques used to detect POU3F4 mutations included whole exome sequencing (WES), Sanger sequencing and real-time polymerase chain reaction. Hearing status was assessed with pure-tone audiometry and auditory brainstem response. Computer tomography scans were evaluated to define the pattern of structural changes in the temporal bones. Six novel (p.Gln27*, p.Glu187*, p.Leu217*, p.Gln275*, p.Gln306*, p.Val324Asp) and two known (p.Ala116fs141*, p.Leu208*) POU3F4 mutations were detected in the studied cohort. All probands with POU3F4 defects suffered from bilateral, prelingual, severe to profound HL. Morphological changes of the temporal bone in these patients presented a similar pattern, including malformations of the internal auditory canal, vestibular aqueduct, modiolus and vestibule. Despite different localization in the POU3F4 gene all mutations severely impair the protein structure affecting at least one functional POU3F4 domain, and results in similar and severe clinical manifestations. Sequencing of the entire POU3F4 gene is recommended in patients with characteristic temporal bone malformations. Results of POU3F4 mutation testing are important not only for a proper genetic counseling, but also for adequate preparation and conduction of a surgical procedure.

Piston stapes prostheses are implanted in patients with refractory conductive or mixed hearing loss due to stapes otosclerosis to stimulate the perilymph with varying degrees of success. The overclosure effect described by the majority of researchers affects mainly low and medium frequencies, and a large number of patients report a lack of satisfactory results for frequencies above 2 kHz. The mechanics of perilymph stimulation with the piston have not been studied in a systematic manner. The objective of this study was to assess the influence of stapedotomy surgery on round window membrane vibration and to estimate the postoperative outcomes using the finite element (FE) method. The study hypothesis is that the three-dimensional FE model developed of the human inner ear, which simulates the round window (RW) membrane vibration, can be used to assess the influence of stapedotomy on auditory outcomes achieved after the surgical procedure. An additional objective of the study was to enable the simulation of RW membrane vibration after stapedotomy using a new type of stapes prosthesis currently under investigation at Warsaw University of Technology. A three-dimensional finite element (FE) model of the human inner ear was developed and validated using experimental data. The model was then used to simulate the round window membrane vibration before and after stapedotomy surgery. Functional alterations of the RW membrane vibration were derived from the model and compared with the results of experimental measurements from temporal bones of a human cadaver. Piston stapes prosthesis implantation causes an approximately fivefold (14 dB) lower amplitude of the RW membrane vibrations compared with normal anatomical conditions. A satisfactory agreement between the FE model and the experimental data was found. The new prosthesis caused an increase of 20–30 dB in the RW displacement amplitude compared with the 0.4-mm piston prosthesis. In all frequencies, the FE model predicted a RW displacement curve that was above the experimental curves for the normal ear. The stapedotomy can be well simulated by the FE model to predict the auditory outcomes achieved following this otosurgery procedure. The 3D FE model developed in this study may be used to optimize the geometry of a new type of stapes prosthesis in order to achieve a similar sound transmission through the inner ear as for a normal middle ear. This should provide better auditory outcomes for patients with stapedial otosclerosis.

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Lesen Sie diesen Fall sorgfältig und beantworten Sie die Fragen auf S. 364 in diesem Heft. Wenn Sie am CME-Verfahren der DGN teilnehmen und mit dem Studium dieser Arbeit einen CME-Punkt erwerben wollen, faxen Sie bitte die Seite mit Ihren Antworten und Ihrem Arztstempel an das CME-Sekretariat der DGN c/o AKM, Weil am Rhein. Sie können das Blatt auch fotokopieren und mit der Post einsenden.Teil I Teil I. 1 Die klinische Situation Ein 68-jähriger Patient wurde wegen einer über ca. zehn Wochen progredienten Schwäche der Muskulatur, die in den letzten drei Tagen zur Gehunfähigkeit geführt hatte, aufgenommen. Er klagte weiterhin über Appetitlosigkeit, Übelkeit und über seit sechs Wochen bestehende Durchfälle.An Vorerkrankungen bestanden ein langjähriger Diabetes mellitus und eine chronische Herzinsuffizienz ischämischer Genese. Es bestand bei Aufnahme folgende Polypharmakotherapie: Insulin, Metoprolol, Lisinopril/Hydrochlorothiazid, Spironolak-ton50/Furosemid20, Torasemid, ASS, Simvastatin.