[Improvement of the ear-surgery technique applying artificial temporal bones]. / Die vervollkommnung der operationstechnik des ohres mit der verwendung des künstlichen felsenbeines.

Study exercises on the temporal bones are a prerequisite for the knowledge of the anatomical features of the respective region and for the precise learning ot fundamentals of the tympanic cavity surgery. Natural human temporal bones however are not available in most countries. The search for artificial back-up temporal models are in a wide use therefore in last years. Basing upon the experiences of the handling and visualization of CT data for the three dimensional implant construction, a temporal bone model was developed in the Otorhinolaryngological Clinic of the University of Jena. The model has been distributed to surgeons for training. Classical procedures in mastoid surgery have been performed, exposing sigmoid sinus, facial nerve, labyrinth, dura mater, jugular bulb, and internal carotid artery. The Jena temporal-bone model is highly suitable for preparing exercises, particularly for beginners. The handling with drill and chisel can be learned easily. The calcium sulfate based temporal bone is an alternative training model for mastoid and middle-ear surgery, especially for beginners or in countries where human temporal bones are not available.

[Desmoid tumors of the head and neck]. / Desmoidtumoren im Kopf-Hals-Bereich.

Desmoid tumors of the head and neck, also known as aggressive fibromatoses, are rare. They are soft tissue neoplasms arising from musculoaponeurotic structures and characterized of locally aggressive infiltration and recurrences. Complete surgical excision of desmoid tumors is considered to be the only effective method of cure. It is likewise important to make a function-preserving surgery. In addition to the radicality this aspect should be a primary goal to minimize morbidity. MRI is the first choice in the preoperative evaluation of neck desmoids. We describe the successful treatment of desmoid tumors in two cases (M. sternocleidomastoideus, M. levator scapulae). Intraoperative neuromonitoring was very helpful for identification and protection of the motor nerves.

[Vestibular evoked muscle potentials dependency on neural origin and the location of an acoustic neuroma]. / Die vestibulär evozierten Muskelpotenziale in Abhängigkeit vom nervalen Ursprung und der Lage eines Akustikusneurinoms.

INTRODUCTION: Most acoustic neuromas (AN) originate from the inferior vestibular nerve (IVN). Vestibular evoked myogenic potentials (VEMP) are accepted as the only unilateral test for the function of the sacculus and the IVN. METHODS: The influence of the origin from the IVN and superior vestibular nerve (SVN), and the position of the AN in relation to the internal auditory canal on VEMPs was investigated. A total of 39 patients (aged: 30-67 years, mean: 53 years) were examined. The VEMPs were recorded on the activated sternocleidomastoid muscle and averaged over 200 stimuli. Tone bursts (95 dB nHL; 500 Hz; stimulation rate 5 Hz) were used to generate the VEMPs. RESULTS: The exact origin of the AN from the SVN or the IVN could be determined intraoperatively and correlated using VEMP in 28 patients. CONCLUSION: The origin of the AN has only a marginal influence on the results of VEMP measurements. The position of the AN in relation to the internal auditory canal seems to have more influence than the origin.

[Biallelic inactivation of the p16-Gen in a metachronous triple carcinoma in the oropharyngeal region]. / Biallelische Inaktivierung des p16-Gens bei metachronem Dreifachkarzinom im oropharyngealen Bereich.

AIMS: Recent studies have shown that most Dutch families with atypical multiple-mole melanoma (FAMMM) have a 19-bp deletion (p16-Leiden) in exon 2 of the p16 gene. Apart from reports on metachronous pancreatic tumors, other cancer types have never been described in such families. Due to heterozygous p16-Leiden constitution, our proband with multiple head and neck carcinomas was a suitable model for studying the type of p16 inactivation according to the Knudson-two-hit model. METHODS: p16 mutations in exons 1 and 2 were determined using PCR-SSCP-Sequencing analysis. p16 methylation was assessed by methylation-specific PCR. RESULTS: All three metachronous (larynx, pharynx, oral cavity) tumors had a methylated p16 promotor. The p16 protein loss detected by immunohistochemistry clearly confirmed a complete loss of p16 tumor suppressor function. Thus, all three tumors exhibited biallelic inactivation of p16, caused by aberrant methylation of the p16 promotor. CONCLUSIONS: This is the first report on p16-Leiden mutation in head and neck cancer. We provide evidence that the somatic methylation of p16 promotor is associated with the germline transmission of p16-Leiden mutation. This is an example for the rare event of in which aberrant methylation acting as the 'second hit' in a familial cancer syndrome. Our results show that this epigenetic event is equivalent to genetic alterations (mutation/LOH) confirming the Knudson's hypothesis for tumor suppressor gene inactivation.

[Postoperative haemorrhagia in a girl with congenital factor XI deficiency - successful treatment with desmopressin (DDAVP, Minirin(R))]. / Postoperative Blutung bei einem Mädchen mit angeborenem Faktor-XI-Mangel - erfolgreiche Therapie mit Desmopressin (DDAVP, Minirin(R)).

UNLABELLED: The rare factor XI deficiency is associated with different profuse bleeding without correlation to the severity of reduction of factor XI. Accordingly, traumata or surgical procedures may cause unexpected excessive bleeding in asymptomatic patients. After surgery of a nine-year-old girl with factor XI deficiency (8 per cent) profuse bleeding occurred which could only be stopped after infusion of desmopressin. After administration the factor XI activity was increased to 31 per cent, the factor VIII even to 290 per cent over the normal range. We suppose that the favorable clinical effectiveness is not only related to the increasing factor XI activity but also to the elevation of the factor VIII/von-Willebrand-complex. CONCLUSION: It is recommended to give desmopressin as firstline therapy of bleeding by factor XI deficiency since the only effective alternative such as substitution of factor XI by transfusion of fresh frozen plasma is associated with the risk of transmission of virus infections.

Visualization of inner ear dysplasias in patients with sensorineural hearing loss.

PURPOSE: We evaluated a data acquisition and post-processing protocol for inner ear (IE) assessment by MR imaging in patients, suffering from various labyrinth malformations. MATERIAL AND METHODS: MR IE studies of 158 consecutive patients (316 IEs) suffering from sensorineural hearing loss without evidence of an acoustic neurinoma were reviewed for pathologies of the IE and internal acoustic meatus. High-resolution MR data of all abnormal IE studies (n=45) were post-processed to previously standardized 3D volume rendered (VR) reconstructions. RESULTS: In 9 patients (5.7%) the following IE dysplasias were detected: malformation of the cochlea (6 IEs), vestibulum (4 IEs), semicircular canals (12 IEs) and vestibular aqueduct/endolymphatic sac (10 IEs). One patient showed evidence of an aplasia of the vestibulocochlear nerve. In 4 patients multiple IE dysplasias were encountered. Comprehensive 3D visualization of all labyrinthine dysplasias was achieved by the use of two VR reconstructions. The overall time for bilateral IE assessment amounted to 30-35 min. CONCLUSION: The imaging protocol allows for rapid and comprehensive visualization of various IE dysplasias, based on a limited number of VR reconstructions.

Virtual endoscopy of the tympanic cavity based on high-resolution multislice computed tomographic data.

OBJECTIVE: This study was designed to assess the value of high-resolution multislice computed tomography (MSCT) data of the petrous bone for the virtual endoscopic visualization of the tympanic cavity. BACKGROUND: The recently introduced MSCT technology has improved spatial resolution in the z axis as well as scan speed in computed tomography. Three-dimensional rendering of high-resolution MSCT data of the petrous bone may be expected to provide endoluminal views of superior image quality, thus competing with transtympanic endoscopy (otoendoscopy). SETTING: This study was conducted at a university teaching hospital. MATERIALS AND METHODS: Cadaveric phantom studies in a MSCT scanner were performed to define a data acquisition protocol, combining adequate detail resolution with low tube current. Subsequently, the cadaveric phantom underwent otoendoscopy. The postprocessing parameters of the three-dimensional rendering protocol were chosen to produce views closely resembling the corresponding otoendoscopic images. High-resolution data from 18 patients with pathologic conditions of the middle ear, as suggested by clinical findings and assessment of cross-sectional data, were postprocessed using the volume rendering technique to generate standardized virtual endoscopic views. A total of 36 virtual endoscopic scans of the tympanic cavity were generated. RESULTS: With regard to intermediate and high-density structures, virtual endoscopic images, based on MSCT data, yielded endoluminal views closely resembling corresponding otoendoscopic views. Virtual endoscopy seems useful for imaging ossicular pathologic conditions such as dysplasia and chain disruption as well as for assessing patient status before and after otosurgery. CONCLUSION: MSCT data sets allow for generating virtual endoscopic views closely resembling otoendoscopic images. The technique is especially useful when ossicular pathologic changes are present as well as for preoperative and postoperative imaging of otologic procedures.

[Virtual rendering techniques in otologic imaging]. / Virtuelle Darstellungstechniken in der otologischen Bildgebung1.

UNLABELLED: Virtual postprocessing techniques combine the advantages of condensing the large amounts of data provided by high-resolution (HR) cross-sectional imaging modalities with those of three-dimensional (3D) imaging. The techniques and indications for virtual representations in imaging of the middle ear (ME), internal ear (IE), and cerebellopontine angle (CPA) are presented together with practical examples. MATERIAL AND METHODS: HR data sets acquired by computed tomography (CT) and magnetic resonance imaging (MRI) in patients with ME, IE, and CPA pathologies were transferred to a workstation via an internal network to generate endo- or extraluminal 3D views by means of the volume rendering technique (VRT). The source data were acquired using scanners and imaging protocols with the highest resolution available at present: a multislice spiral CT (MSCT) with a slice thickness of 0.5 mm and a reconstruction increment of 0.2 mm and a 3D CISS sequence with a slice thickness of 0.5 mm for MRI. RESULTS: Virtual endoscopy was superior to cross-sectional images for assessing ME pathologies like dysplasia, postoperative changes, and destructive bone processes with extensive soft-tissue involvement; fibrous obliterations of the internal ear and labyrinthine dysplasia were depicted with a superior image quality on 3D renderings compared to conventional reconstruction techniques. Virtual endoscopy of the CPA and external acoustic meatus (EAM) was helpful in detecting and visualizing neurovascular conflicts and in assigning small intrameatal tumors to components of the acousticofacial bundle. A common feature of all applications was that the large numbers of source images could be reduced to a few 3D reconstructions for documentation and optimized communication of the findings between the radiologist and otologist. CONCLUSION: Virtual rendering makes an important contribution towards establishing, presenting, and documenting the findings when certain otologic pathologies have to be assessed. It can be used for routine imaging and allows for more efficient handling of the large amounts of imaging data generated by high-resolution cross-sectional imaging modalities.

Speech understanding with the CIS and the n-of-m strategy in the MED-EL COMBI 40+ system.

Speech tests have been performed on 6 subjects for comparing the standard 12-channel continuous interleaved sampling (CIS) strategy (CIS12), the 7-channel CIS strategy (CIS7) and the 7-of-12 strategy in the MED-EL COMBI 40+ system. An ABAB experimental design was used whereby each strategy was reversed and replicated. Speech tests were performed in quiet (vowels, consonants, monosyllables, sentences) and noise (sentences). Results showed that for vowels, CIS12 is significantly superior to CIS7, for consonants and sentences CIS12, CIS7 and 7-of-12 performed equally well, and that for monosyllables 7-of-12 is significantly superior to both CIS12 and CIS7. In addition, 7-of-12 is superior to CIS7 by almost the same amount as CIS12, but in this case the difference is not significant. Further, all strategies have been found to be equally robust in noise with respect to sentence understanding. The differences between CIS12 and 7-of-12 on the one hand and CIS7 on the other hand may be attributed to decreased spectral resolution of the latter. The fact that - in contrast to what has been reported for the SPEAK strategy - 7-of-12 is equally robust in noise as the CIS strategies is explained by the use of higher stimulation rates, wider frequency bands and a higher percentage of channels stimulated in each cycle.

[Second carcinomas in cancers of the mouth cavity, pharynx and larynx. Clinical, histopathologic and cell kinetic findings]. / Zweitkarzinome bei Karzinomen der Mundhöhle, des Pharynx und des Larynx. Klinische, histopathologische und zellkinetische Befunde.

BACKGROUND: The mortality because of malicious tumours of the oral cavity, of the pharynx and of the larynx has considerably increased in the Federal Republic of Germany during the last two decades. The prognosis not only shows a high ratio of recidivations and formation of metastases, but also a typical field canceration. There is a high rate of incidence of multiple primary, resp. secondary tumours that may occur synchronously or metachronously. Up to now we have learnt only little about the mechanisms concerning the development of tumours and their spread on the cellular molecular level. METHOD: The subject of the present retrospective study was the analysis of patients suffering from an epithelioma of the oral cavity, the pharynx and the larynx and the following secondary carcinomatas. The control group were patients suffering from primary tumours of the same location and subsequent recidivation or metachronous metastasis. By determination of the degree of malignity and keratinisation of the DNA ploidy, of the immunohistological expression of p53 and the immunohistological proliferation marker MIB1 conclusions had to be drawn with regard to the biological behaviour of tumours. RESULTS: The evaluation of the clinical parameters of the 122 patients on the whole revealed a clear increase of new disease as well as a shifting to the younger age within the last five years. Surprisingly, most common were secondary tumours in case of larynx carcinomatas. Patients suffering from secondary tumours show a bad prognosis, however, their maximum survival does not differ considerably from that of patients suffering from recidivations, resp. metastases. Because of the early diagnosis of the secondary carcinomatas the prognosis will depend on the primary carcinoma in most of the cases. The prognosis data indicate that independently from the fact whether there is a primary or a subsequent tumour, the therapy to be applied can only be a combined therapy consisting of operation and ray-therapy. The present examinations confirm reports with regard to disturbance of the p53 regulation that also play an important role in case of the head-neck area. The parallel analysis of MIB1 as a proliferation marker showed in case of the primary carcinomatas a correlation of positive p53-immunocolour with moderate and strong proliferation. Tumours in the hypopharynx and larynx showed a significantly smaller proliferation than that of the oral cavity and oropharynx. In case of the primary and secondary tumours the proliferation is more common in case of the G3-tumours. CONCLUSIONS: The in total modest differences between the primary and secondary carcinoma with regard to the DNA-ploidy; the proliferation and the p53-expression presumably originate in their formation within the scope of a so-called field canceration. Because of the field canceration supposed for the mucous membranes of the upper aerodigestive tract, the check-ups of these patients performed in regular intervals must not be limited in any case to the area of the primary tumour. They will have to consider the entire visible area of the upper respiratory and esophageal tract.

[Lipoma-like liposarcoma of the tongue]. / Lipomähnliches Liposarkom der Zunge.

BACKGROUND: Liposarcomas of the tongue are rare. To date only 7 cases have been reported. PATIENT: We report a new case of a well differentiated lipoma-like liposarcoma of the body of the tongue in a 71-year old woman. Complete uneventful resection was performed. Histologically the tumor consisted of fat cells with occasional lipoblasts and spindle cell areas. There is no sign of recurrence of the tumor 2 years later. RESULTS AND CONCLUSIONS: Liposarcomas of the tongue are rare neoplasms. All cases reported were histologically well differentiated. Liposarcomas have to be considered in the differential diagnosis of solid masses of the tongue.

[Anterior hyperostosis of the cervical spine: diagnostic studies and surgical therapy]. / Diagnostik und operative Therapie der hyperostotischen Spondylose der Halswirbelsäule.

BACKGROUND: Dysphagia due external compression by anterior hyperostosis of the cervical spine is rare. The diagnosis may be established by conventional X-ray of the spine, esophagogram, and CT. PATIENTS: We operated on three patients with large anterior osteophytes from C3 to C7. In two cases morphologic changes of the cervical spine were the main cause of dysphagia. One patient with progressive hypopharynx cancer had hyperostosis of cervical spine as secondary findings. RESULTS: The patients were asymptomatic, post-operatively. CONCLUSIONS: Cervical osteophytes can be detected in 20-30% of the population in asymptomatic patients. The therapeutic approach depends on the extent of dysphagic complaints. Painful dysphagia is a indication for surgery. The anterolateral extrapharyngeal approach is commonly preferred with anterior hyperostosis between C4 and C7. The transoral intrapharyngeal approach has been used in patients with hyperostosis of cervical vertebra C2/C3. Interdisciplinary orthopedic and ENT surgical treatment is without complications and yields good functional results.

Absence of an association between IgE-mediated atopic responsiveness and ADHD symptomatology.

There are mixed research results in the literature regarding a possible association between Attention Deficit Hyperactivity Disorder (ADHD) and atopic disorders. If such an association were supported, the implications for underlying pathophysiology would be significant. We evaluated level of atopic responsiveness (based on IgE-mediated response to skin prick tests) in 312 referrals to a pediatric allergist. Based on the atopy code, children were categorized as non-atopic, or moderately or severely atopic. Parents completed the Child Behavior Checklist (CBCL). Univariate analyses on the eight CBCL subscales revealed no differences between the atopic groups. Our results do not support an association between IgE-Mediated atopic responsiveness and ADHD, but they do not rule out an association between allergic symptoms and ADHD based on some other mechanism.

[Surgical therapy of oropharyngeal carcinoma within the scope of interdisciplinary tumor surgery]. / Die operative Therapie des Oropharynxkarzinoms im Rahmen der interdisziplinären Tumorchirurgie.

BACKGROUND: The results of the surgical therapy of patients with extensive squamous cell carcinoma of the oropharynx and their lymph node metastasis, postoperative follow-up, and rehabilitation depend on the collaboration between ear, nose, and throat surgeons and maxillofacial surgeons. Modern reconstruction techniques permit single-session surgical treatment with a reduced period of hospitalization. The most important therapeutic aim is to improve patient well-being. PATIENTS: Over a period of two years, 45 patients underwent the same surgical procedure. These patients were all in stages III or IV of their disease. RESULTS: This procedure consists of a temporary lateral osteotomy of the mandible with or without resection of the mandible, to provide broad exposure of the tumor of the oropharynx. Apart from the broad exposure, the advantage of this surgical method lies in simple reconstruction using a pedicled and microanastomosed flap. Internal fixation with six and four-hole titanium plates is a reliable method for restoring the stability of the mandible. In six patients, complications occurred in connection with the plate fixation. In four patients, we observed osteomyelitis of the fragment fissure or sequestration. Plate fracture and pseudarthrosis occurred in one patient each. CONCLUSIONS: In our clinical experience, the microvascular anastomosed radial flap proved to be the ideal free soft tissue transplant. One of its primary advantages is its plasticity and hence good post-therapeutic functional results. The jejunal flap has become less significant them the radial flap; the advantage of mucous membrane transfer is lost after radiation and a corresponding postoperative interval. In patients with large, voluminous defects, the use of myocutaneous flaps, especially from the latissimus dorsi, is still justified.

Extra-abdominal aggressive fibromatosis after treatment of a Morbus Hodgkin. A case report.

The proliferations of the connective tissue, which are summed up under the term fibromatoses, assume a special position with regard to their biologic behavior. While superficial fibromatoses are more likely to grow slowly, musculoaponeurotic fibromatoses (Desmoid tumor)--like malignant tumors--show a locally bound aggressive, rapid growth behavior. A metastazation, however, can never be proved. The research on etiology of fibromatoses has yielded only little knowledge. Besides genetic and hormonal factors, physical reasons are under discussion, too. The present study aims to add another description of a case report to the small number of case presentations on radiotherapy-associated fibromatosis. In 1977, a now 67-old-patient was irradiated because of a Morbus Hodgkin lymphoma in the left side of his neck. Prior to the radiation, the patient had undergone several surgeries. 17 years after the radiation, the patient developed an aggressive fibromatosis (prelaryngeal, right) in the formerly irradiated region. As the localization of the fibromatosis does not allow us to establish a connection to his numerous operations preceding the fibromatosis, a radiation-associated Desmoid tumor must be assumed. Immunohistologically, no estrogen receptors were detectable.

Lack of adverse reactions to measles, mumps, and rubella vaccine in egg-allergic children.

BACKGROUND: Controversy exists regarding allergic reactions to measles, mumps, and rubella (MMR) vaccine in egg-allergic patients. To date there have been only isolated reports describing egg-allergic patients with anaphylaxis to MMR vaccine. OBJECTIVE: Our study was designed to monitor possible adverse reactions in egg-allergic children receiving MMR vaccine. METHODS: Initially prick and intradermal testing with MMR vaccine was carried out. The first 120 children were screened in this fashion, with frequent irritant reactions occurring with skin testing but no subsequent reactions when full-strength vaccine was administered. The subsequent 380 children received the undiluted MMR vaccine without prior skin testing. DESIGN: Over an 8-year period, MMR vaccine was given to 500 egg-allergic children in outpatient setting. RESULTS: No anaphylactic reactions were observed in any of the 500 children immunized with MMR vaccine. Five children showed minor rashes within two hours of administration of the MMR vaccine. CONCLUSIONS: It is therefore felt the previously reported adverse reactions to MMR vaccine represent reactions to other vaccine components rather than the suspected egg antigens. The presence of egg allergy should therefore not be considered a contraindication to MMR immunization.