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Objectives: This is the first clinical trial to evaluate the suitability of a new titanium stapes prosthesis, which we developed jointly with the Kurz Company (Duslingen). Design: In a prospective clinical study, patients with otosclerosis underwent stapes surgery using our new titanium stapes prosthesis during a period of 14 months. One year after surgery pre- and postoperative audiograms were performed and all patients participated in a structured interview. Setting: Tertiary otorhinolaryngological university department Participants: Of 49 patients with otosclerosis, entering the study, two patients were excluded, because they were not located any more. Main outcome measures and results: All patients experienced a hearing improvement of 21 dB on average except one patient. Sixty percentage of patients achieved a reduction of the air–bone gap to less than 10 dB and 31% of patients having their air–bone gap closed to within 20 dB – averaged across 0.5, 1.0, 2.0 and 4 kHz. The hearing level improved significantly in the air and in the bone conduction thresholds. Conclusions: This paper presents our first ‘1 year postimplantation’ results of our titanium stapes prosthesis. We consider the development of this implant to be beneficial. The results confirm significant improvement in hearing status and tinnitus levels.

Background: Recently, in patients with normal tension glaucoma (NTG) elevated levels of antiphosphatidylserine antibodies (APSA), a subgroup of antiphospholipid antibodies (APLA) were found. Progressive sensorineural hearing loss (PSHL) is associated with autoimmune diseases and also the presence of APLA. Methods: To investigate a possible association between NTG and PSHL, 34 patients (age range 31–81 years) with NTG were evaluated for evidence of audiovestibular disorders. Besides ophthalmological standard examinations (slit lamp, IOP, funduscopy, perimetry) scanning laser tomography and polarimetry were performed. From all patients’ audiograms, stapedial thresholds and otoacoustic emissions were obtained. The serological testing of patients and controls (40 healthy blood donors older than 50 years) concerned IgG and IgM levels of antibodies against phosphatidylserine (APSA) and β2 glycoprotein. Results: 23 of 34 NTG patients had hearing loss (PSHL n = 11; presbyacusis n = 12). The NTG patients had significantly higher APSA levels than controls. Elevated APSA concentrations were significantly more frequent in patients with NTG and hearing loss compared with NTG patients with normacusis. Conclusions: These findings show that NTG and hearing loss have a high coincidence. The elevated APSA levels may indicate an association with similar systemic autoimmune processes.

Total reconstruction of the auricle requires a skilful surgical technique and an appropriate material for the shape-supporting frame. Up to now, there is no such material apart from autologous rib cartilage. The combination of chronic microtraumatisation of adjacent tissue caused by the mobility of an implant bed such as the auricle and the foreign-body reaction to currently available artificial polymers frequently results in extrusion. In our animal model (rats), polymers of different elasticity were implanted in a moving implant bed to analyse differences in foreign-body reaction related to implant elasticity. The results were significantly better for a rather stiff control material (porous polyethylene). A contributing factor may be better fixation of the implant material by tissue ingrowth into its micropores.

The use of tissue plasminogen activator (tPA) in community hospitals has been limited by a lack of neurologic support. The authors developed a telephone network to support community emergency departments (EDs) in the use of tPA. Their experience demonstrates equivalent results for patients treated by telephone and those treated in-house. Their number of tPA-treated patients increased 72%. Requirements included willingness of community EDs to use tPA and willingness of the stroke center to provide support.

Cancer surgery, malformation or trauma may cause broad facial defects that cannot be covered by patients because of their exposed site. Such defects lead to functional deficits and enormous psychological strain and require rehabilitation at all ages. Prosthetic supply has been developed into a functional and aesthetic alternative to plastic and reconstructive surgery.The condensed experience with prosthetic supply after persistent surgical defects of more than twenty patients during the recent years is described.Efficient prosthetic supply in the exposed facial area requires a well scheduled and close cooperation between the surgeon and anaplastologist. Preoperative diagnostic evaluation and operative filling of the magnetic fixation in cooperation with the anaplastologist provide a satisfying functional and cosmetic prosthetic outcome for the patient. Advantages of this procedure combine early cosmetic rehabilitation with the option to directly investigate the tumor site after resection. Novel magnet systems provide a high degree of flexibility combined with confident fixation.Prosthetic rehabilitation should be offered and discussed with patients ahead of cancer surgery in the head and neck area. This procedure should be considered in particular in patients that reject or are not feasible for multi-step reconstructive surgery.

Regarding the satisfaction of patients efficiency and outcome of surgery on the nasal septum and the turbinates regularly is the topic of controversial discussion and occasionally some health authorities doubt there is an indication for these procedures in general. Subjective and objective evaluation of surgical outcome do not necessarily match in all cases.A standardized questionnaire was sent to patients having undergone surgery on the nasal septum and the turbinates 33-45 months ago at a university hospital and a non-university hospital. Topics (10 all together) were e. g.: nasal aeration, sense of smell, rhinorrhea, snoring, general condition. Statistic significance of each parameter was verified.138 questionnaires were reviewed. 82% of the candidates reported better aeration of the nose after surgery. 71% stated to have benefited from the operation. A relevant positive effect on snoring, rhinorrhea, headache and sense of smell could not be proved. The results did no different whether surgery was performed at a university hospital or at a non-university-hospital. Older patients seem to profit more from the procedure than younger ones.The survey clearly shows, that surgery on the nasal septum with turbinoplasty has a positive effect on the nasal aeration and the general condition. Therefore its performance is well justified when after careful verification of the pre-conditions the indication for surgery is given.

Basosquamous carcinoma (BSC) is a rare malignancy with specific histopathological features of both basal cell (BCC) and squamous cell carcinoma (SCC). Therefore, the histological diagnosis is challenging. Due to its low incidence there is no consensus on the surgical management of BSC.We describe the (immunohistological) diagnostic and therapeutic aspects of nine cases (8 male symbol : 1 female symbol, on average 68.6 years of age (range: 47-81)) of BSC. Of these, seven were located on the pinna, one on the forehead and zygomatic region and one in the retroauricular region.Immunochemical staining for epithelial membrane antigen was negative (apart from the typical areas of epithelial pearl formation) and BerEP4 was positive in all cases. Therapy consisted of partial removal of the pinna in four, and total removal in three cases. One patient was treated by partial removal of the auricle with superficial parotidectomy and ipsilateral neck dissection. In the case of the carcinoma on the forehead, a local excision was performed. The median follow-up was 45 months. One patient had a local recurrence.The histological diagnosis of BSC is confirmed by the use of EMA and BerEP4 immunohistological staining. Clinically, BSC is a rare, aggressive skin tumor. Despite the histological similarity to basal cell carcinoma, BSC has an imminent risk of metastasizing. Hence, therapy should be similar to that for SCC, taking into consideration the age and general state of the usually elderly patient.

The excellent results yielded by hyperfractionated and accelerated radiotherapy associated with concurrent chemotherapy in locally advanced oropharyngeal and hypopharyngeal carcinomas led to investigation of this therapeutic regimen in nasopharyngeal carcinomas also.Thirty-five patients with stage III and IV nasopharyngeal carcinomas received accelerated hyperfractionated radiotherapy with concurrent chemotherapy (5-FU, mitomycin C + leucovorin). In the first 3 weeks of treatment five 2-Gy doses per week were delivered to the primary tumour and regional lymph nodes. The fractionation was then accelerated, with 1.4 Gy given twice daily until a total dose of 72 Gy had been administered.The overall objective response rate was 100%. The median follow-up period was 71 months. Salvage surgery of the lymph nodes was performed in 10 patients, revealing vital tumour tissue in 6 of these. The actuarial 5-year local control rate was 64% (95%CI: 47-81%), while overall actuarial survival at 5 years was 70% (95%CI: 53-86%).Hyperfractionated accelerated radiotherapy with concurrent chemotherapy is effective and feasible in locally advanced nasopharyngeal carcinoma.

Iatrogenic esophageal perforations are still a life-threatening clinical entity.We present the case reports of six patients to demonstrate different treatment options and we focus on new therapeutic strategies which have evolved in the interdisciplinary management of iatrogenic esophageal perforations. Two patients with perforations in the cervical esophagus were operated and in another patient the perforation was closed with fibrin glue. Three patients with perforations in the thoracic esophagus were treated with self-expandable plastic stents.The surgical therapy of esophageal perforations still is regarded to be the gold standard and nonadherence should only be considered based on interdisciplinary decisions in individual cases. However, positive results are increasingly being reported for conservative endoscopic treatment, particularly for thoracic perforations.

Smoking has not properly been investigated as a predisposing factor for the development of a peritonsillar abscess.In a retrospective study a group of 541 abscess tonsillectomies--performed in the department of Otorhinolaryngology (University of Duisburg/Essen) between 1994-2003--was retrospectively analysed for smoking habits of the patients. Further on we investigated the influence of smoking on the risk for a postoperative haemorrhage.Smoking habits of 519/541 were known, 296/519 (57%) were smokers, which is much more than expected with regard to the prevalence of smokers in the German population. Beneath the 2.2% of patients with a postoperative haemorrhage that had to be arrested under general anaesthesia no statistical relation between the occurrence of a postoperative haemorrhage and smoking was found.Smoking could be an important predisposing factor for the development of peritonsillar abscess e. g. due to alteration of the mucosa and microbiological changes. We could not identify a statistical relation between smoking and the risk of a postoperative haemorrhage after tonsillectomy à chaud.

The simulation of deafness rarely occurs in the course of medical expertise. However, it is a problem that needs to be recognized and demonstrated by proving methods of investigation. For this purpose, a number of subjective and objective hearing tests exist which can provide evidence for the simulation of uni- or bilateral hearing loss. These methods include the measurement of oto-acoustic emissions, brainstem-evoked response audiometry and the determination of the stapedius reflex threshold as well as subjective methods such as Stenger- and Lee-test. Among the expertise carried out in our department during the past few years, we found 5 cases of simulation of deafness. In these cases -- as opposed to simulated traffic accidents -- there was no criminal prosecution regarding insurance fraud.

According to the guidelines for cancer of the skin in the head and neck region of the German association of Otolaryngology, Head and Neck surgery squamous cell carcinoma (SCC) of the pinna are classified by using the current TNM system of skin cancer. As soon as cartilage is infiltrated, irrespective of other criteria like tumour size, SCC of the thin skin of the pinna are classified as T4 category. As therapy considerably depends on the TNM stage a review of the prognostic value of cartilage infiltration as well as other histological criteria seems to be justified.Medical records of all patients (n = 36) being operated for SCC of the pinna between August 1988 and January 2004 at our department were retrospectively analysed with regard to a statistical correlation of histological criteria, cervical lymph node metastases and prognosis.In 36 cases a histological re-evaluation could be performed on the original tumour samples (34 male symbol : 2 female symbol; average age was 76 years, with an age interval of 54 - 99 years). 26 SCC cases were smaller than 2 cm, 8 cases between 2 and 5 cm and 2 cases bigger than 5 cm of size. 36 % of SCC cases had infiltration of the auricular cartilage. Statistical analysis did not show a statistical correlation of either cartilage infiltration, tumour size bigger than 2 cm or 1 cm, tumour grading and tumour depth with regard to lymph node metastases.According to our results, cartilage infiltration as single criterion of inclusion into a T4 category should be analysed cautiously. The anatomical peculiarity of the pinna where cartilage lies directly beneath very thin skin should be taken into account. A survey of a bigger group of patients e. g. as a multicenter study would be desirable for such a rare malignancy.

Carbon-dioxide-laser-tonsillotomy is reconsidered as a procedure to relieve patients in early childhood from obstructive symptoms caused by tonsillar hyperplasia such as snoring or sleep apnea without influencing the immunological function.The protruding part of the tonsils was removed by a CO (2)-laser delivering 10 - 15 W. During 1993 to 2003, 83 children received laser-tonsillotomy, combined with adenoidectomy 51 children were available for follow-up with a standard questionnaire. The parents were surveyed in average 39 months postoperatively. 5 patients required a subsequent tonsillectomy due to a recurrence of tonsillar hyperplasia. Histological investigations were done. 15 children were reevaluated by clinical examination.Most of the patients were relieved from obstructive symptoms. There was no occurrence of postoperative hemorrhage or peritonsillar abscesses. The histological investigations on the specimens from later performed tonsillectomy (n = 5) showed no evidence of scarring opened crypts. The clinical examination did not reveal any signs of chronic infections.The tonsillotomy by carbon-dioxide-laser appears as a suitable and safe technique for the treatment of tonsillar hyperplasia without severe throat infection in early childhood.

Data for all patients with ear malignancies being operated in our department between August 1988 and March 2001 were retrospectively analyzed for tumor localisation, stage, biometric data, anesthesiological risk factors, therapy and recurrence of the disease.Thirty of 79 patients (29 male, one female; average age 77.2 years, range 54-99 years) with cutaneous malignancies of the external ear were diagnosed as SCC ( n=32 SCC). A total of 17 SCC were smaller than 2 cm, 12 were between 2-5 cm, and three were larger than 5 cm. Only two patients had regional nodal disease, none had distant metastases. The anesthesiological risk was estimated according to the recommendations of the American Society of Anesthesiologists (ASA); 16/30 patients were classified as group 3 or 4, having severe general disease with a decrease in vitality or even vital risks. A total of 24 SSC were primarily operated under local anesthesia. Depending on histology, localisation and size of the SCC local excision, partial or total removal of the auricle was performed. In the remaining 8/32 cases, the primary intervention was performed under general anesthesia, mostly in combination with an ipsilateral neck dissection and a superficial parotidectomy. In 8/32 cases, the SCC had to be re-operated after primary R1 resection. Altogether, eight patients received radiotherapy. In 5/32 cases there was a recurrence of the disease. The average follow-up period of the 13 patients who are still alive is 50 months (17-113). One patient died as a result of the metastasized SCC and 16 patients died due to other diseases.Considering the high age and the age-associated general diseases of the patients with SCC of the auricle, differentiation between a radical concept of therapy and its risks and possible therapy-related damage is important. Therefore, individual concepts such as partial removal of the pinna without neck dissection and parotidectomy for the N(0) stage are justified if relevant anesthesiological risk factors have to be taken into account.

Local recurrence of nasopharyngeal carcinomas can be treated in different ways. One option is re-irradiation e. g. as stereotactically guided convergent beam. An operative approach is possible in small recurrent tumours. Brachytherapy is a good alternative because of the steep dose gradient in the pre-irradiated area.First step is the creation of a wide approach to the nasopharynx. This leads to a tumour mass reduction and gives space for the applicator. The radioactive substance will be brought into contact with the tumour by an afterloading procedure. The advantage of this therapy is the possibility to protect the surrounding tissue whereas the tumour receives a relatively high dosage. Between 2/90 and 12/96 10 men and 3 women were treated according to this protocol.The median age was 56 years (37- 66 years), the average follow-up period was 49,7 months (8 -123 months). 2 non keratinising and 11 undifferentiated carcinomas were treated. 5 of 13 patients were still alive at the end of the follow-up period. The 5 year over all survival rate was 46 %. 3 patients are free of disease for 5 years or longer. The patients were treated 2 to 6 times with 7 Gy in 5 mm depth.The results show that good palliation can be achieved by the applied method but larger studies are required to give a definite statement.

Gap junction channels consist of different connexin proteins and play an important role in the physiology of hearing. Connexin26 and connexin30 have been demonstrated in the inner ear by immunohistochemistry and Northern Blot analysis. Mutations in the genes for connexin26 and connexin30 have been described to be responsible for non-syndromic hearing loss.We investigated the prevalence of connexin26 and connexin30 mutations in patients with profound hearing loss or deafness by SSCP-analysis and sequencing.30 connexin26 mutations (22 %) were detected among 134 patients with profound hearing loss or deafness. The most frequent connexin26 mutation 30delG was found in 25 patients. In 5 patients other connexin26 mutations were identified. No connexin30 mutation was found.Therefore connexin26 mutations also play an important role for non-syndromic hearing loss in Germany. We propose that every patient with suspected hereditary hearing loss should be screened for a connexin26 mutation.

Mobilising the stapes via the removal of the tympanosclerotic plaques from the oval window niche (effodation) and stapedectomy or malleovestibulopexy are the different procedures generally available for the surgical therapy of stapes fixation due to tympanosclerosis. These techniques bear a significant risk of sensory hearing loss. Here we analyse our results using the mobilisation technique together with locally applied antibiotics.Nineteen ears in seventeen patients with tympanosclerosis involving the stapes and its footplate which underwent stapes mobilisation between 1991 and 1999 have been investigated retrospectively. According to the literature this operation has a high risk of cochlear hearing loss. To reduce this risk, azlocillin was instilled locally during removal of tympanosclerotic plaques.Different operation techniques have been used: classic type III with placement of a cartilage disc on the head of the stapes (4), interposition of the incus (3), interposition of the head of malleus (1), interposition of a ceramic-PORP (6) and cartilage columella in cases of significant stapes footplate erosion (3). In two operations the chain was intact and no reconstruction was necessary. Pure-tone-audiometry showed no significant decrease of bone-conduction thresholds. Preoperatively 4 (21.1%) ears had an average air-bone-gapor = 30 dB, while postoperatively 15 (78.9%) ears had this level of hearing.Until the exact causes of the loss of hearing after mobilisation or stapedectomy in cases of tympanosclerosis are known, the local administration of antibiotics is certainly recommended, bearing in mind the initial hypothesis that infection may be jointly responsible for cochlear hearing loss on mobilisation or stapedectomy in cases of tympanosclerosis.

Tullio phenomenon is defined as noise induced vertigo. Other authors have attributed this symptom to either a perilymphatic fistula or postinflammatory adhesions between the stapes foot plate and the vestibular end organs.In this paper two cases are described in which acoustically induced vertigo was explained by abnormal mobility of the stapes.The stapes was stabilized by the placement of cartilage chips beside the crurae of the stapes. Both cases demonstrated long term success, i.e. 4 and 5 years postoperatively.

The epithelioid hemangioendothelioma is a soft tissue tumor of vascular origin. Typical localisations are subcutis, cutis, liver, lungs and bones. It has been described in 1982 by Weiss and Enzinger as a separate tumor entity. Due to the unpredictable biological behaviour of the tumor, a borderline malignancy is assumed.We report on the case of a 44-year old woman with a metastasising epithelioid hemangioendothelioma in the head and neck area. The primary tumor was located in the subcutis of the left forehead. Due to local recurrences surgical treatment was performed three times after the initial removal in 1993. At the time of the last local recurrence in 1996, a tumor in the left parotid gland was diagnosed and was the reason for a lateral parotidectomy. Pathohistologically, a metastasis of the epithelioid hemangioendothelioma was found. Postoperative radiotherapy was performed and no recurrence occurred until now (22 months follow-up).Metastatic epithelioid hemangioendotheliomas are rarely found in the head and neck area. Literature has not yet reported on metastasis formation in the parotid gland. The case illustrates the potentially malignant behaviour of epithelioid hemangioendotheliomas. Hence, therapy should consist of a combination of radical tumor removal and post-operative radiotherapy.

Forty-one patients with locally advanced hypopharyngeal carcinomas were followed for at least 3 years (median, 60 months) after simultaneous radiochemotherapy. Conventionally fractionated radiotherapy was administered as 5 x 2 Gy/week to a total dose of 30 Gy within 3 weeks. From the fourth week an accelerated hyperfractionated schedule was used as 2 x 1.4 Gy/day five days weekly given exclusively to the first order target volume of macroscopic tumor (adding up to a total dose of 72 Gy in six weeks). The second and third order target volumes received conventional fractionation only to 60 Gy and 50 Gy, respectively. The moderate acceleration of the concomitant boost scheme in the second half was counterbalanced during the first week by the introduction of a 5-fluorouracil bolus of 350 mg/M2 with 200 mg/M2 folinic acid and a subsequent continuous infusion using the same dose each 24 h for 5 days. Additionally, a Mitomycin-C bolus of 10 mg/M2 was infused at the fifth day and on the first day of the sixth week. Six weeks after treatment the patients were restaged. In cases with residual carcinoma salvage surgery was performed (11 patients). Late effects of therapy were analyzed according to the Lent-Soma index and life quality according to the European Organisation for Research and Treatment of Cancer-Module. Late effects of treatment were tolerable and were controlled locally. The 3-year-survival rate was 39%, with a local-regional recurrence-free control rate of 71%. Fifty-two percent of all cases of death were caused by distant metastases, secondary carcinomas or other diseases not related to tumor recurrence. The poor prognosis of hypopharyngeal carcinomas despite acceptable local tumor control may be due to specific biological factors present in affected patients.

The treatment of severe forms of bilateral Menière's disease remains an especially challenging task. Similar problems also occur in debilitating Menière's disease in the only hearing ear. The intramuscular titration therapy with streptomycin has been the means of choice since 1984 to minimize the risk of total hearing loss in cases of severe bilateral disease.Since 1989 we have treated six out of 21 cases of bilateral Menière's disease by intravenous application of 2 x 120 mg gentamicin in Ringer's solution for several days. Additionally we reported on two cases in 1988. Only minor amounts of gentamicin were applied to sedate the function of both vestibular organs while avoiding damage to the cochlea.In two cases hearing approved approximately about 10 dB, in two cases hearing remained stable, and in two cases hearing worsened about 10 dB. Five of six patients showed minor excitability in caloric tests on both sides, they did not complain of vertigo attacks one to five years after therapy.Given that only very small amounts of gentamicin are applied to sedate the function of the vestibular organ while causing almost no damage to the cochlea, this method seems to be an excellent means for treatment of bilateral Menière's disease. Patients do not experience severe problems with equilibrium afterwards, and the treatment can be repeated as often as necessary.

As causal therapy of Rendu-Osler-Weber syndrome is not yet possible, there are many different therapeutical approaches. It seemed expedient to investigate the efficacy of super-selective angiography in treating Rendu-Osler-Weber syndrome after this technique proved effective in other therapeutic settings. This method is known to be efficient in controlling tumor bleeding.Between March 1993 and September 1995, eight patients with an average age of 57 years were treated by super-selective intraarterial embolization.In all cases epistaxis occurred on both sides. Unilateral embolization was performed in ten cases and bilateral embolization in five cases. In two of three patients with nasal packing the embolization permitted removal of the packing. Nevertheless nosebleeds reoccurred on an average of 3.5 days after therapy. Two of eight patients showed long-term improvement after therapy. They suffered fewer nosebleeds than before. Complications included one patient who suffered from partial hemiparesis after embolization, which lasted 1.5 hours and was completely reversible. Another patient died because of pulmonic bleeding caused by manifestation of the disease in this organ. Often patients complained of facial pain or headache which occasionally lasted for a few weeks.Endovasal embolization obviously is an alternative to artery ligation in emergency cases whereas the long-term success is currently impaired by formation of new anastomoses. Further development of this technique is necessary.

The feasibility and effectiveness of a combined chemo-radiotherapy treatment modality for locally advanced head and neck cancer was tested in a phase II trial. Between 1990 and 1993, 74 patients (20 female/54 male) with head and neck cancer stage III (n = 12) and IV (n = 62) were treated with accelerated radiotherapy (72 Gy) and simultaneous chemotherapy (5-FU, folinic acid, mitomycin C). The median follow-up time was 43 months (1-72). Complete remission (CR) was absent in 76% (56/74) of patients and, after subsequent resection of residual lymph nodes, another 8 patients achieved CR. The cumulative local control rate was 72% and disease-specific survival rate was 59% at 4 years. Two patients died with treatment-related conditions (pancytopenia, larynx oedema). By multivariate analysis, only lymph node status was an independent parameter for local control (P = 0.04). This treatment was feasible and toxicity was not a treatment-limiting factor. As a consequence, a German multicentre phase III trial was initiated in 1995.

The interval between the onset of symptoms in patients with head and neck cancers and the introduction of treatment was evaluated. During a 1-year period, patients were interviewed to determine the duration of any delays from the onset of complaints until the first visit to a doctor. Also asked was how long it took to be referred to an ENT specialist and actual referral to our department. We then determined the interval from diagnosis to the introduction of therapy. It was found that the delay of patients who went directly to an otorhino-laryngologist was shorter (median, 8 weeks) than that of patients who first went to their family doctors (median, 13 weeks) (P0.02). The latter then took an additional 4 weeks before sending patients to an otorhinolaryngologist. Referral from the ENT specialist to our department took another 2 weeks, as well as our staging and pre-therapeutic management. Our findings show that the patient himself is the critical factor in delaying diagnosis and therapy. Tumor patients with greater professional qualification went to their doctors earlier (P0.0001) or more often went directly to an ENT specialist (P0.002). Consultations of physicians without experience in otorhinolaryngology caused yet further delays.

Abnormal eustachian tube function has been a major problem in improving hearing with middle ear surgery. In 13 patients, a newly developed gold tube conductor was placed in the eustachian tube via the middle ear cavity. Patients were examined 2-50 months postoperatively, at which time middle ear aeration was found to be significantly improved in 11 of the cases.

Amyloidosis is a term that describes a heterogeneous group of disorders in which various tissues contain the characteristic insoluble fibrillar protein known as amyloid. A number of different forms of presentation have been described and include senile, familial or hereditary, systemic, localized, secondary and primary forms. We now report our management of a 79-year old woman who suffered from primary A-lambda-light chain amyloidosis. Amyloid changes first presented in the tissues of the tongue and floor of mouth and were seen clinically as a macroglossia. Further investigation led to a primary diagnosis of multiple myeloma IIa, as categorized by Durie and Salmon. Several cases have been reported to date describing secondary development of macroglossia due to long-term known and treated multiple myeloma. However, there has been only one report of macroglossia as a primary finding leading to the diagnosis of multiple myeloma.

This case report describes a lethal complication of retrograde air insufflation through the Eustachian tube after myringotomy. A 35-year-old woman presented with otalgia and hearing loss in her left ear, which had occurred after an upper respiratory infection. Otoscopy showed a middle ear effusion. A myringotomy was performed under mask anesthesia in a private clinic. During subsequent retrograde Eustachian tube insufflation, the patient experienced sudden convulsions, followed by cardiac and respiratory arrest. The patient then died some hours later from generalized cardiac and circulation break-down in the intensive care unit of a nearby hospital. The cause of the cardiac and circulation collapse could not be defined completely, but it is believed than an anatomical variant of the tegmen tympani was responsible for the tragic outcome because of an air embolus.

Aseptic necrosis and cholesteatoma of the external auditory canal can be stages of the same pathological entity. The majority of these patients suffered from symptoms similar to those of external otitis. It is suggested that minor mechanical trauma, e.g. cotton lip swabs, plays a role in the pathogenesis of external ear canal cholesteatoma. During the last 10 years we have been surgically treating 22 patients with diagnosis of aseptic necrosis and cholesteatoma of the bony external auditory canal. All 22 patients registered significant postoperative improvement.

To evaluate the feasibility and effectiveness of a concurrent radio-chemotherapy regimen for locally advanced carcinomas of the pharynx and floor of the mouth.Since January 1990, 97 patients with locally advanced carcinomas of the naso-, oro-, hypopharynx and the oral cavity in UICC stages III and IV were treated according to an accelerated hyperfractionated radiotherapy schedule with concurrent chemotherapy. The primary tumor and positive lymph nodes received a total dose of 72 Gy in 6 weeks. In the first 3 weeks, large fields were irradiated 5 times per week with 2 Gy per fraction. Thereafter, treatment was accelerated, giving 2 daily fractions of 1.4 Gy with minimal intervals of 6 hours. Target volumes were reduced after 49.6 and 59.4 Gy, excluding clinically uninvolved lymph node regions of low and high risk. On day 1, 350 mg/m2 5-FU and 50 mg/m2 folinic acid were given as intravenous bolus followed by continuous infusion of 350 mg/m2 5-FU and 100 mg/m2 folinic acid, days 1 to 5. 10 mg/m2 mitomycin C was given on day 5 and 36 of the treatment. Salvage surgery was offered for residual disease 5 to 6 weeks after the end of radiotherapy.70 male, 27 female; age: 27 to 81 years; T2/T3/T4: 7/30/60; N0/N1/N2/N3: 20/18/53/6; nasopharynx/oropharynx/hypopharynx/oral cavity: 16/33/36/12. Median follow-up is 26 months.Overall survival and recurrence-free survival at 2 years wereOverall survival and recurrence-free survival at 2 years were 68 +/- 5% and 74 +/- 5%. Multivariate analysis revealed a significant influence of the geometric mean neck node diameter or the N-stage on loco-regional control and survival. T-stage, tumor volume, or tumor localisation did not become significant. Acute toxicity of this schedule was acceptable but required optimised supportive care. Treatment related grade 4+ late toxicity of mucosa, soft tissue and bone were observed with a cumulative frequency of 13% at 2 years. Two patients died during a phase of severe leuko- or thrombocytopenia.This phase I to II trial shows favourable results using an intensified treatment radio-chemotherapy protocol. A phase III study is now planned, comparing this regime with an accelerated hyperfractionated radio-therapy alone to an increased total dose of 77.6 Gy.

1% of thyroglossal duct cysts are supposed to develop carcinoma. In most cases papillary carcinoma is found deriving from ectopic thyroid tissue. As far as squamous cell carcinomas are concerned, worldwide only eleven cases have been reported so far. In this article, three cases of carcinoma in a thyroglossal duct cyst are presented (1 case of squamous cell carcinoma, 2 cases of papillary carcinoma). The diagnostic procedures as well as the therapeutic strategies are discussed. The diagnosis of a thyroglossal duct cyst should be confirmed by ultrasonography. This can provide information about the presence or absence of a normal thyroid gland and makes radioisotope scanning unnecessary.

In this study we examined the feasibility of rebuilding a stable stapes superstructure for a new articulated ossicular chain by using bioactive ceramics. Dense hydroxylapatite and ceravital were tested in guinea pigs. The ceramic prosthesis was placed on the remnants of the footplate to create a bony connection without touching the tympanic membrane, thereby protecting it from relative movements. The implants were explanted together with the stapes footplate at six weeks and three months, respectively. Histologically, bony connections were found between all ceramic implants and the remnants of the crura even at six weeks and became more pronounced after three months.

We examined 105 patients who underwent partial stapedectomies at the age of 65 years or older as treatment for combined hearing losses due to otosclerosis. Among other things investigated was the influence of the operation on the hearing of both the operated and unoperated ears, as well as on tinnitus existing preoperatively and the use of a hearing aid. Findings showed that tinnitus decreased or vanished in 75% of the cases. Sixty-one percent of the patients needed a hearing aid continuously preoperatively, with 48% not needing it post-operatively and 13% using it only occasionally. The degree of sensorineural hearing loss had by the other patients was so severe that the use of a hearing aid was also necessary post-operatively. However, in some of these cases, a successful use of a hearing aid became possible for the first time due to the surgical improvement in hearing.

The clinical occurrence of isolated sphenoid sinusitis is rare. Due to a long history and few initial symptoms, diagnosis of this potentially fatal infections can be difficult. Radiological aspects in 10 patients are presented (e.g. type and design of examination and whether inflammatory or destructive changes could be shown), as well as clinical aspects (cranial nerve palsy, concurrent inflammation, duration of disease and therapy). CT proved to be superior in diagnosing the extent of any bony changes, while MRI was more useful in detecting inflammation.

Tissue sections of 32 squamous cell carcinomas (SCCs) of the head and neck were investigated for the expression of the epithelium-specific cell adhesion molecule E-cadherin. We found that E-cadherin expression is inversely correlated both with the loss of differentiation of the tumor and with lymph node metastasis. The well-differentiated SCCs expressed E-cadherin, often as strongly as the normal stratified epithelium (12 cases were tested); the moderately differentiated SCCs expressed intermediate amounts of E-cadherin or were heterogeneous (15 cases were analyzed); whereas the poorly differentiated SCCs were all E-cadherin-negative (five cases were investigated). Furthermore, seven of eight infiltrated lymph nodes of SCCs were E-cadherin-negative. These data indicate that the loss of the cell adhesion molecule E-cadherin in fact plays an important role in the progression of human squamous cell carcinomas, i.e., that down-regulation of expression is associated with dedifferentiation and metastasis of the tumor cells in vivo.

About 30 cases of a persistent stapedial artery have been described in the literature so far. It is usually associated with other malformations, suggesting an inhibition of normal embryonic development. For the first time, it was possible to demonstrate this using high-resolution computed tomography.

Five years after cholesteatoma surgery (primary operation in 1981 and 1982) we examined 112 patients to determine the hearing and clinical results in follow-up. In 12 (10.7%) patients Cholesteatoma recurred. During the period of follow-up 28 (25%) ears underwent second surgery. Comparing the open (50.9%) with the closed (49.1%) technique a better air bone gap was gained for the closed technique procedures. This was confirmed when the different types of tympanoplasty were compared between the two groups. In 69.5% of the closed procedures the air bone gap was less than 20 dB, whereas the value for the open technique was 51.1%. In 30 patients a second-look operation was recommended, which was performed in 15 persons; 8 recurrent cholesteatoma were detected. Reducing the application of the closed procedures from a rate of 78.1% in the years 1971/1972 to 49.1% in 1981/1982, we saw a distinct reduction of cholesteatoma recurrence from 25% in 1971/1972 to 10.7% in the present study. The data show that the advice for a second-look operation should be seriously considered. Furthermore, the study indicates that due to modern tympanoplastic techniques also in longterm follow-up good hearing results can be obtained.

The following parameters have been determined or calculated in isolated adipocytes from subcutaneous human adipose tissue from normal weight and obese subjects, obtained during surgical interventions or from volunteers who received local anaesthesia. Mean cell diameter, mean cellular lipid content, mean fat cell volume, and volume of lipid-free space. In fat cell extracts the activities of the following enzymes were measured: Glucose-6-phosphate-dehydrogenase, Phosphoglucomutase, Fructose-6-phosphatekinase, Hexosephosphate-isomerase, Aldolase, Glycerolaldehyde-phosphate-dehydrogenase, Phosphoglycerate-kinase, Pyruvate-kinase, Lactate-dehydrogenase. — In subjects with more than 50% overweight, the mean fat cell diameter was significantly increased. Mean fat cell volume calculated from fat cell diameter increased from 376±115 mm3 × 10−6 in normal weight to 799±99.5 mm3 × 10−6 in obesity. — In obese subjects the mean lipid content of individual adipocytes was twice as high as in normal weight subjects. The lipidfree residual space was also significantly larger in obesity. Nevertheless, this lipid-free space occupied a significantly smaller percentage of a defined fat cell mass in obese than in normal weight subjects. — Related to triglyceride content, the activities of HIM, G6PDH, and Aldolase were significantly reduced in adipocytes of subjects with more than 50% overweight, in a second series this was true for LDH also. There were significant negative correlations of the logarithms of these enzymes to the logarithm of overweight. There was an increased mean absolute enzyme content in adipocytes of obese subjects compared with those of normal weight persons. However, this increase was significant for LDH and HIM only. It is not certain that the enzyme pattern in the fat cells of obese subjects is related to the increase in cell volume. When related to a defined fat cell mass after correction for extra-cellular space, diminished activities for Aldolase, G6PDH, HIM, and LDH were found.

A long-term review of 140 ears in 133 patients was carried out. All ears had first been operated on in 1971 and 1972 for cholesteatoma in the E.N.T. clinic. A closed technique was used in 110 ears and in the remaining 30 ears an open technique, depending on the pneumatisation of the mastoid, the extent of the cholesteatoma, tubal function and the age and social circumstances of the patient. At that time, second-look operations were carried out on relatively few patients after a closed operation. While the short-term results were favourable, recurrent cholesteatomas still developed 8 to 10 years later; 9 were discovered at this review alone. The long-term incidence of recurrence was 25%. Recurrent cholesteatomas were found in 57% of patients who were first operated on for a cholesteatoma before the age of 21, 24% of those between 21 and 40, and 11% of those between 41 and 60. These results reaffirm the necessity for a planned second-look operation in all young patients and also the importance of outpatient follow-up over many years.

Twentyfour chemodectomas (non-chromaffin paragangliomas) of the neck seen in 20 patients are presented. The investigation including angiography and the surgery in respect to risk factors and preoperative assessment is discussed. The long term results are also presented. Special note is made of the familial incidence since two twin brothers who had bilateral tumours are included.

The clinical data and follow-up of five patients with Lermoyez syndrome are reported. Three of them presented with marked lesions of the cervical spine. Serological antibodies against nuclei and sarcolemma as well as against heart muscle and gastric cells were found. Tests of the sera against guinea pig cochlear tissue showed antinuclear antibodies in one patient and against basilar membrane structures in another. Three sera were negative despite positive heterologous finding. Therapy included infusions active on the inner ear, decompression of the saccus endolymphaticus in two cases and physiotherapy for the cervical spine. These achieved marked improvement or long term relief. The available literature supports the conclusions based on pathophysiological considerations of our results. We cannot postulate an independent immunopathogenesis for Lermoyez Syndrome, but we think that the immunological findings conform with the clinical and experimental data found in the literature.

Radical or palliative resection of tumours in the region of the midface often require a combined approach by maxillary-facial surgeons, as well as by oto-rhino-laryngologists. This holds true not only for the excision of the tumour, but also for adequate reconstructive measures. These are necessary to alleviate as much disfiguration as possible, and to ensure correct physiological and mechanical function, i.e. nasal patency and chewing ability. We are aware of the increasingly important rôle of microsurgically transplanted myocutaneous and osteomyocutaneous flaps. In this paper we demonstrate our operative technique by means of several selected cases, primarily in the use of the following pegged-vascularized flaps: 1. free myocutaneous latissimus dorsi flaps, 2. iliac osteomyocutaneous flaps and 3. modified rectus abdominis flaps.

Because of the slow lingering course of this disease, the diagnosis of hypothyroidism is generally made by chance particularly because the euthyroidism can change unobserved into manifest hypothyroidism. In old age the course of hypothyroidism may be oligosymptomatic: only adynamia and abnormal susceptibility to cold may be diagnosed. Because of the augmented oxygen requirement of tissues during therapy with thyroid hormones the medical treatment of hypothyroidism has to start - especially in old age - with a fractional part of the final dose. In old age bland struma more and more changes to nodular struma, where small autonomic areas are often developed. Conservative therapy with thyroid hormones predominates in this case. Radioiodotherapy is indicated when medical treatment has been without success and operation is contraindicated because of high risk. Cancer of the thyroid is important - with respect to differential diagnosis - especially in older patients. Cancer of the thyroid is most common in patients aged between 50-70 years, and the anaplastic carcinoma is the most frequently observed malignant growth of old age. Concerning therapy, a radical operation comes into consideration; if radical operation is combined with too high risks, remaining tissue may be treated by radioiodotherapy without danger.