Improved directional hearing of children with congenital unilateral conductive hearing loss implanted with an active bone-conduction implant or an active middle ear implant.

Different amplification options are available for listeners with congenital unilateral conductive hearing loss (UCHL). For example, bone-conduction devices (BCDs) and middle ear implants. The present study investigated whether intervention with an active BCD, the Bonebridge, or a middle ear implant, the Vibrant Soundbridge (VSB), affected sound-localization performance of listeners with congenital UCHL. Listening with a Bonebridge or VSB might provide access to binaural cues. However, when fitted with the Bonebridge, but not with a VSB, binaural processing might be affected through cross stimulation of the contralateral normal hearing ear, and could interfere with processing of binaural cues. In the present study twenty-three listeners with congenital UCHL were included. To assess processing of binaural cues, we investigated localization abilities of broadband (BB, 0.5-20 kHz) filtered noise presented at varying sound levels. Sound localization abilities were analyzed separately for stimuli presented at the side of the normal-hearing ear, and for stimuli presented at the side of the hearing-impaired ear. Twenty-six normal hearing children and young adults were tested as control listeners. Sound localization abilities were measured under open-loop conditions by recording head-movement responses. We demonstrate improved sound localization abilities of children with congenital UCHL, when listening with a Bonebridge or VSB, predominantly for stimuli presented at the impaired (aided) side. Our results suggest that the improvement is not related to accurate processing of binaural cues. When listening with the Bonebridge, despite cross stimulation of the contralateral cochlea, localization performance was not deteriorated compared to listening with a VSB.

[Development of a computed tomography data-based Vibrant Bonebridge viewer]. / Entwicklung eines CT-Daten-basierten Vibrant-Bonebridge-Viewers.

BACKGROUND: Because of the anatomy of the mastoid and the size of the actuator, positioning of the Vibrant Bonebridge B-FMT can be difficult without prior evaluation of the individual computed tomography (CT) scan of the temporal bone. Development of a user-friendly CT data viewer to enable positioning of the B-FMT in the temporal bone model, whilst identifying individual, potential anatomic conflicts and offering possible solutions could provide a useful tool for preoperative positioning. OBJECTIVES: Aim of the study was to define the requirements of a Vibrant Bonebridge viewer and construct a prototype. MATERIALS AND METHODS: Based on a ZIBAmira software version and inclusion of a B-FMT model upon creation of a model of the temporal bone-which allows the intuitive estimation of individual, anatomic conflicts-a Vibrant Bonebridge viewer was constructed. RESULTS: The segmentation time of the individual digital imaging and communications in medicine (DICOM) data set is about 5 min. Positioning within the individual three-dimensional temporal bone model allows quantitative and qualitative estimation of conflicts (sigmoid sinus, middle cranial fossa) and determination of a preferred position for the B-FMT. Lifting of the B-FMT can be simulated with the help of a virtual washer. CONCLUSION: The Vibrant Bonebridge viewer reliably allows simulation of B-FMT positioning. The clinical value of the viewer still has to be evaluated.

Assessment of skills using a virtual reality temporal bone surgery simulator.

Surgery on the temporal bone is technically challenging due to its complex anatomy. Precise anatomical dissection of the human temporal bone is essential and is fundamental for middle ear surgery. We assessed the possible application of a virtual reality temporal bone surgery simulator to the education of ear surgeons. Seventeen ENT physicians with different levels of surgical training and 20 medical students performed an antrotomy with a computer-based virtual temporal bone surgery simulator. The ease, accuracy and timing of the simulated temporal bone surgery were assessed using the automatic assessment software provided by the simulator device and additionally with a modified Final Product Analysis Scale. Trained ENT surgeons, physicians without temporal bone surgical training and medical students were all able to perform the antrotomy. However, the highly trained ENT surgeons were able to complete the surgery in approximately half the time, with better handling and accuracy as assessed by the significant reduction in injury to important middle ear structures. Trained ENT surgeons achieved significantly higher scores using both dissection analysis methods. Surprisingly, there were no significant differences in the results between medical students and physicians without experience in ear surgery. The virtual temporal bone training system can stratify users of known levels of experience. This system can be used not only to improve the surgical skills of trained ENT surgeons for more successful and injury-free surgeries, but also to train inexperienced physicians/medical students in developing their surgical skills for the ear.

[Analysis of psychosocial changes following ear reconstruction with rib cartilage - development of a short test]. / Analyse psychosozialer Parameter nach Ohrrekonstruktionen mit Rippenknorpel - Entwicklung eines Kurztests.

BACKGROUND: The improvement of psychosocial well being in patients with microtia after ear reconstruction with rib cartilage is mainly assessed clinically. There are first prospective studies with established psychological questionnaires showing promising results but these tests are too extensive for everyday-use in clinical routine. Therefore, we examined a self-designed short version for the specific use in the head-neck-region. METHODS AND PATIENTS: The clinically established psychological questionnaire "Frankfurter Selbstkonzeptskalen/FSKN" consists of 78 items for self-assessment of performance abilities, self-esteem, and psychosocial attitude. We downsized the FSKN to 13 items that might be relevant for plastic reconstructive surgery in the head-neck-region. The pre- and postoperative development after ear reconstruction with rib cartilage was analyzed retrospectively in 68 patients and prospectively in 21 patients. The preoperative data of the prospective study group were compared with the results of 23 patients with microtia who declined any type of reconstruction after consultation. RESULTS: Especially the results of the psychosocial competence improved after ear reconstruction as well in the retrospective (median values 138,5 to points; p<0,01) as well as in the prospective study group (median values 126 to 141 points; p=0,01). We were able to demonstrate changes in the short version of the FSKN (median values 51,5 to 58,5 points; p<0,01 respectively, 50 to 56 points; p=0,02). Patients who declined ear surgery showed higher values in psychosocial competence (median values 126 to 154 points; p<0,01). Again, the short form displayed this difference clearly (median values 50 to 65 points; p<0,01). CONCLUSIONS: The short version of the FSKN has promising potential for the preoperative assessment and the documentation of psychological changes following reconstructive surgery. Further studies are necessary to validate the new instrument to obtain a valuable test for use in clinical routine in plastic surgery in the head-neck-region.

In vivo perfusion analysis of normal and dysplastic ears and its implication on total auricular reconstruction.

INTRODUCTION: During the first stage of total auricular reconstruction with autologous rib cartilage, according to Nagata, the cartilage framework is placed in a subcutaneous pocket. Its posterior skin flap is relatively large and can be prepared with or without a subcutaneous pedicle. This represents the crucial part of the procedure, as impaired healing and infection can occur due to low perfusion. Nothing was known about the blood supply of ear remnants or flap perfusion during reconstruction. It was not clear whether the preservation of the additional subcutaneous pedicle secures high blood supply. METHODS: We used laser fluorescence angiography with indocyanine green dye for anatomical and functional perfusion studies in eight normal ears. Subsequently the anatomical and functional vessel architecture of 18 dysplastic ears was investigated. Finally, five patients each were operated on with or without subcutaneous pedicle during auricular reconstruction and intraoperatively monitored with laser fluorescence angiography. RESULTS: We showed that the vessel structure of normal ears detected by fluorescence angiography is equivalent to anatomical preparations. The surrounding skin in high grade microtia remnants is biphasically perfused by deep perforators and by the cutaneous vessel network. The preservation of the subcutaneous pedicle during auricular reconstruction leads to significantly better perfusion of the posterior skin flap. No signs of critical perfusion or complications were observed in these patients. DISCUSSION: We present the feasibility of laser fluorescence angiography to simultaneously gain anatomical and functional data about skin blood supply. The first anatomical and functional description of blood supply of ear remnants in third grade microtia is given. Functional data of skin flap perfusion during and after complete ear reconstruction were evaluated. The present study shows that the subcutaneous pedicle of Nagata's procedure is of great importance for success of the first stage operation as it prevents impaired wound healing. In contrast, patients without a subcutaneous pedicle had a broad spectrum of reduced perfusion and therefore some had complications.

[Epidemiology of auricular trauma]. / Zur Epidemiologie von Ohrmuschelverletzungen.

BACKGROUND: Auricular trauma is rare but can seriously reduce self-acceptance and function. Epidemiologic data on the causes of trauma causes and patient characteristics are limited. PATIENTS AND METHODS: The case notes of 141 from 197 patients with ear injuries have been reviewed retrospectively according to trauma cause and to the distribution of age and gender. RESULTS: Two thirds of the documented cases occurred between the age of 11 and 40 years. Men outnumbered women at a ratio of 2 to 1. The most frequent causes for ear trauma were traffic accidents (43 %), accidents at home (33 %), and fights (14 %). CONCLUSIONS: Mainly younger and active groups of the population are affected by ear trauma. Therefore, it is of particular concern in the acute setting to select those therapeutic options that have turned out good aesthetic and functional results in the long-term perspective.

T-level downstaging and complete pathologic response after preoperative long-term radiochemotherapy for locally advanced rectal cancer.

Advantages of neoadjuvant chemoradiotherapy for locally advanced carcinoma of the middle and the lower third of the rectum are downstaging and downsizing of the tumor. Results of pathologic results are affected by post-treatment tissue changes and may influence the choice of surgical procedure. Forty-three consecutive patients (27 male, 16 female; mean age 64 years) were operated after receiving a long-term chemoradiotherapy during a period of 16 months. The data of initial staging procedure (high resolution magnetic resonance imaging) and results of pathological examination of the surgical specimens were analyzed. Regression of tumor was assessed by the absence of vital tumor cells and the post-treatment fibrotic tissue alterations. Regression of tumor size was seen in 42/43 patients leading to an improved T-stage in 27 patients. R0-resection was possible in all cases, although there was a perirectal tumor infiltration to less than 2 mm to circumference of the surgical specimen in 2 cases and unexpected small liver metastasis in 5 cases. Complete remission rate was 23.3% (10 cases). Detecting small amounts of vital tumor cells in altered tissue after chemoradiotherapy is a major problem of pathological examination procedure and should be taken into consideration by the surgeons. The choice of operation (resection vs. abdominoperineal extirpation vs. local excision) should be committed to the initial imaging procedure and not to any restaging procedure after neoadjuvant chemoradiotherapy.

[Integration of the active middle ear implant Vibrant Soundbridge in total auricular reconstruction]. / Integration des aktiven Mittelohrimplantates in die plastische Ohrmuschelrekonstruktion.

BACKGROUND: Patients with high-grade microtia and atresia require a sophisticated and specific treatment. Apart from the plastic reconstruction of the auricle, in some cases hearing rehabilitation is medically indicated or is desired by the patients. The long-term results of simultaneous middle ear reconstruction with tympanoplasty are often inadequate owing to a persisting air-bone gap, and new techniques in hearing rehabilitation are needed for these patients. METHODS: We present three cases of unilateral atresia to illustrate a combined approach integrating hearing rehabilitation using the active middle ear implant Vibrant Soundbridge (VSB) into plastic auricular reconstruction. The VSB was attached to the stapes suprastructure via the titanium clip in two of these cases and in the third case a subfacial approach was used to attach it directly to the membrane of the round window. RESULTS: The air-bone gap was reduced to 17 dB, 14 dB and 0.25 dB HL. In free-field speech recognition tests at 65 dB SPL the patients achieved 100%, 90% and 100% recognition with the activated implant. No postoperative complications such as facial nerve paresis, vertigo or inner ear damage were found. CONCLUSIONS: The integration of active middle ear implants in auricular reconstruction opens up a new approach in complete hearing rehabilitation. The additional implantation of the VSB does not have any negative effect on the healing process or the cosmetic outcome of the auricular reconstruction.

[Reconstruction of the auditory canal wall with bone chips from the temporal squama--preliminary results]. / Rekonstruktion der Gehörgangswand mit Knochenchips aus der Temporalschuppe--Erste Ergebnisse.

BACKGROUND: The canal wall down-technique in cholesteatoma surgery with the creation of an open mastoid cavity offers many disadvantages as prolonged healing time, necessity for periodic cleaning and subsequent infections. Therefore reconstruction of the posterosuperior canal wall is the more and more preferred technique to restore the physiologic anatomy of the external auditory canal without lacking a good surgical exposure to the tympanal and retrotympanal spaces. Cartilage is the mainly used material for this technique, as cartilage is easy to prepare and to fit into the defect with good viability. However, cartilage resorption and retraction is observed increasing the risk for recurrent cholesteatoma. In contrast, by the use of bone chips from the temporal squama a stable and close restoration of the auditory canal wall can be achieved. PATIENTS AND METHODS: In 23 patients (17 attic and 6 sinus cholesteatomas including two recurrences in each group) we have performed an anterior atticoantrostomy with retrograde cholesteatoma removal and reconstructed the posterosuperior wall of the auditory canal with bone chips harvested from the temporal squama by chisels. The bone chips and remaining gaps in the reconstructed wall were covered by bone paté and temporal fascia. In 4 cases the ossicular chain could be conserved (type-1 tympanoplasty [TP]), type-3 TP with PORP was performed in 13 and TORP-TP in 6 cases. The mean follow-up was 12.1 months (range 4 - 34 months). 8 patients had undergone a second-look procedure. RESULTS: On follow-up all patients showed a good healing of the external ear canal with no graft insufficiency. In one case we observed a slight ear canal narrowing due to bone excess, three patients developed retraction pockets of the attic. Recurrent cholesteatoma was not seen yet. Second-look tympanotomy revealed complete ingrowth of the transplanted bone chips into the surrounding bony structures in all cases. One residual cholesteatoma had to be removed and the canal wall had to be reconstructed again with bone chips. The average postoperative air-bone gap was 7.0 +/- 8.2 dB HL for type-1 TP, 8.6 +/- 3.0 dB HL for PORP-TP (83 % [100 %] of the patients < or = 10 dB HL [< or = 20 dB HL]) and 19.3 +/- 9.2 dB HL for TORP-TP (66 % of the patients < or = 20 dB). CONCLUSIONS: Osteoplastic atticoantrostomy allows adequate anatomic and physiologic restoration of the auditory canal even after extensive cholesteatoma removal. Bone stability may reduce recurrent cholesteatoma. However, due to the underlying impaired tubal function long-time results must be further evaluated.

[Auricular reconstruction for severe microtia. Schedule of treatment, operative strategy, and modifications]. / Ohrmuschelrekonstruktion bei hochgradiger Mikrotie. Behandlungsplan, plastische Operation und deren Sonderformen.

The incidence of microtia in Germany is 100-150 per year. These cases require a specific and challenging therapy. All patients need audiologic consultation. If desired plastic reconstruction is performed, which is aiming at achieving a lifelike as possible appearance corresponding to the shape of the opposite ear including an excellent skin color. The present paper describes background information, the interdisciplinary schedule of treatment, and the results of our operative strategy in two to three steps using autologous rib cartilage. Furthermore we expand on anomalous cases of microtia which require a modified procedure. In dystopic microtia, repositioning of the rudiment is necessary before reconstruction. In cases of excessive scar tissue due to injuries or previous operations, a one-step reconstruction using an axial fascia flap can be useful.

[Transmastoidal resection of an endaural encephalocele--a case report]. / Transmastoidale Resektion einer Gehörgangsenzephalozele--ein Fallbeispiel.

BACKGROUND: There are transmastoid, transtemporal and combined approaches for surgical management of endaural encephaloceles. The advantages of a transmastoid approach are the exploration and option of surgical treatment of the middle ear cavity without craniotomy. Nevertheless there are recent studies preferring the transtemporal or combined approach because of the better survey. PATIENT: We present a 27-year old male patient, who developed hearing loss and otorrhea of the right side. He underwent middle ear surgery associated with cholesteatoma three times before. On examination there was a smooth pulsatile mass taking origin in the roof and almost filling the external auditory canal. High-resolution CT and MRI scans of the petrous bone demonstrated a spherical soft tissue mass communicating with the middle cranial fossa. The encephalocele was exposed and resected through a transmastiodal approach. Behind the cele a relapsing cholesteatoma was found and resected in the same session. The laterobasilar defect was sealed with ear cartilage and a perichondrium flap. CONCLUSION: There are three different surgical approaches to manage laterobasilar encephaloceles. Due to the history of a previous cholesteatoma we performed a transmastoidal approach. A transtemporal approach alone would have failed to notice the relapsing cholesteatoma and would have led to a greater trauma.

Haplotype analysis of the CD11 gene cluster in patients with chronic Helicobacter pylori infection and gastric ulcer disease.

Helicobacter pylori infection leads to a broad spectrum of disease manifestations such as gastritis, ulcer disease, and even gastric carcinoma. The genetically determined immune response and subsequent inflammation influence the degree of mucosal damage. Adhesion molecules of the CD11 cluster play an important role in adherence of neutrophils to endothelial cells in inflammation. We conducted a haplotype-based analysis of the CD11 cluster in a sample of 315 patients with H. pylori infection and investigated associations with gastric erosions and ulcer disease. Twelve single nucleotide polymorphisms (SNPs) covering the genes CD11a, CD11b, and CD11c were genotyped by Taqman technology. Linkage disequilibrium (LD) was assessed within the CD11 cluster and haplotype case-control analysis was conducted. Sliding window haplotype analysis identified a haplotype consisting of the markers CD11c exon 15 and intron 31 associated with gastric ulcer disease. Patients carrying the haplotype GA bear a 2.4-fold increased risk. No significant associations of single markers with disease outcome were found. High-density LD mapping and mutation detection of CD11c in larger samples will be necessary to confirm our findings and identify the causative variant. Thus, we conclude that genetic variants in the CD11 cluster may play a role in the development of gastric ulcer in chronic H. pylori infection presumably by influencing leukocyte adhesion. The biological effect of genetic variants of CD11c in gastric inflammation needs further clarification.

[Gastrointestinal autonomic nerve tumor]. / Der gastrointestinale autonome Nerventumor.

Gastrointestinal autonomic nerve tumors (GAN-tumor) are rare malignant neurogenic stromal tumors of the intestinal tract. The origin is suspected in the autonomic nerve plexus Meissner or Auerbach with the interstitial cells of Cajal as precursors. We report on a 53-year-old patient with a clinical apparent and radiological 5 cm measuring tumor of the jejunum, which was resected and immunohistochemically verified as GAN-tumor. Within the follow-up of 29 months metastases appeared within the omentum majus with a diffuse peritoneal spreading. Several trials of adjuvant chemotherapy (adriamycine/ifosamide, taxotere, gemcitabine/xyloda) were ineffective. 15 months after the second operation the patient died. Since the first description of the GAN-tumor in 1984 87 patients were reported in the literature. No recurrences or metastasis were seen in tumors with a seize less than 5 cm. A tumor seize of more than 10 cm is associated with recurrences in 64% of the cases within 2 years. Since there is no option for medical treatment, surgical resection is the treatment of choice and has to be considered also in the case of recurrence.

[A rare cause of malignant ascites--peritoneal mesothelioma]. / Eine seltene Ursache für malignen Aszites--das Peritonealmesotheliom.

HISTORY AND CLINICAL FINDINGS: A 56-year-old patient (case 1) with recurrent haemorrhagic ascites for one year was admitted to our hospital for further investigation. Besides massive ascites he did not show abnormal physical signs. In addition, two 45-year-old patients were admitted (case 2 and 3) with clinical signs of acute abdomen--one having muscular guarding in the epigastric angle, the other in the right lower quadrant. All 3 patients did not have serious illnesses in the past; the first 2 patients had occupational asbestos exposure. INVESTIGATIONS: In patient 1 the ultrasound did not reveal abnormal findings besides ascites. Patients 2 and 3 underwent explorative laparotomy. DIAGNOSIS, TREATMENT AND COURSE: In the first case a diagnostic laparoscopy revealed diffuse tumor proliferations with nodular formations over the entire peritoneum--histologically a malignant peritoneal mesothelioma of the epithelial subtype. Patient 2 showed intraoperatively metastatic spread of tumour formations with infiltration of the peritoneum and transverse mesocolon. The histologic finding was similar to that in the first case. Patient 3 had a perforated sigma diverticulitis which was treated by resection of the sigmoid. Incidentally a well differentiated papillary peritoneal mesothelioma was found in the resected specimen. The first two patients were treated with alpha-interferon subcutaneously resulting in a decrease of ascites production. Because patient 3 showed neither ascites nor evidence for malignancy no interferon was administered. CONCLUSION: In case of haemorrhagic ascites of unknown cause a histological clarification by either laparoscopy or laparotomy is mandatory. Immunomodulation with interferon may be a promising approach.

Association between insertion mutation in NOD2 gene and Crohn's disease in German and British populations.

Background Genetic predisposition to inflammatory bowel disease (IBD) has been shown by epidemiological and linkage studies. Genetic linkage of IBD to chromosome 16 has been previously observed and replicated in independent populations. The recently identified NOD2 gene is a good positional and functional candidate gene since it is located in the region of linkage on chromosome 16q12, and activates nuclear factor (NF) kappaB in response to bacterial lipopolysaccharides. Methods We sequenced the coding region of the NOD2 gene and genotyped an insertion polymorphism affecting the leucine-rich region of the protein product in 512 individuals with IBD from 309 German or British families, 369 German trios (ie, German patients with sporadic IBD and their unaffected parents), and 272 normal controls. We then tested for association with Crohn's disease and ulcerative colitis. Findings Family-based association analyses were consistently positive in 95 British and 99 German affected sibling pairs with Crohn's disease (combined p<0.0001); the association was confirmed in the 304 German trios with Crohn's disease. No association was seen in the 115 sibling pairs and 65 trios with ulcerative colitis. The genotype-specific disease risks conferred by heterozygous and homozygous mutant genotypes were 2.6 (95% CI 1.5-4.5) and 42.1 (4.3-infinity), respectively. Interpretation The insertion mutation in the NOD2 gene confers a substantially increased susceptibility to Crohn's disease but not to ulcerative colitis.

[Lipomatous hypertrophy of the interatrial septum]. / Lipomatöse Hypertrophie des interatrialen Septums.

The echocardiographic examination of a female, overweight patient, 71 years of age, with left-sided breast cancer, performed in another hospital, revealed a tumor of the interatrial septum of 3.2 x 4.0 cm. The patient was transferred to our clinic and the finding was identified as a lipomatous hypertrophy of the interatrial septum by echocardiography, computer tomography and nuclear magnetic resonance imaging. It was confirmed by biopsy and histological examination. The lipomatous hypertrophy of the interatrial septum is a rare, benign disease, which usually does not cause a hemodynamic significant obstruction of the blood flow. An increased incidence of atrial arrhythmias is known to occur in this condition and seems to be the only functional restriction.