[Rare complication after Le Fort I osteotomy]. / Eine seltene Komplikation nach Le-Fort-I-Osteotomie.

Malfunctions of the eustachian tube after Le Fort I osteotomies are rare. A 22-year-old woman was treated by Le Fort I osteotomy for maxillary retrognathism. Postoperatively she developed recurrent tubal malfunction and middle ear effusions on the left side, with no improvement after adenotomy, tonsillectomy, and grommet insertion. In consecutive computed tomography and magnetic resonance imaging scans, a forward dislocation of the left pterygoid hamulus was demonstrated. In addition, damage to the tensor veli palatini muscle was evident. Both postoperative sequelae appear to be responsible for the unilateral tubal dysfunction.

Tandem mass spectrometry protein identification on a PC grid.

We present a method to grid-enable tandem mass spectrometry protein identification. The implemented parallelization strategy embeds the open-source x!tandem tool in a grid-enabled workflow. This allows rapid analysis of large-scale mass spectrometry experiments on existing heterogeneous hardware. We have explored different data-splitting schemes, considering both splitting spectra datasets and protein databases, and examine the impact of the different schemes on scoring and computation time. While resulting peptide e-values exhibit fluctuation, we show that these variations are small, caused by statistical rather than numerical instability, and are not specific to the grid environment. The correlation coefficient of results obtained on a standalone machine versus the grid environment is found to be better than 0.933 for spectra and 0.984 for protein identification, demonstrating the validity of our approach. Finally, we examine the effect of different splitting schemes of spectra and protein data on CPU time and overall wall clock time, revealing that judicious splitting of both data sets yields best overall performance.

Conservation of signaling pathways of xenobiotic-sensing orphan nuclear receptors, chicken xenobiotic receptor, constitutive androstane receptor, and pregnane X receptor, from birds to humans.

Chicken xenobiotic receptor, pregnane X receptor, and constitutive androstane receptor are orphan nuclear receptors that have recently been discovered to regulate drug- and steroid-mediated induction of hepatic cytochromes P450 (CYP). This induction is part of an adaptive response involving numerous genes to exposure to drugs and chemicals and has major clinical and toxicological implications. Here we report experiments in the chicken hepatoma cell line LMH that suggest evolutionary conservation of the signaling pathways triggered by pregnane X receptor, constitutive androstane receptor, and chicken xenobiotic receptor. Thus, the phenobarbital-inducible enhancer units of the mouse Cyp2b10, rat CYP2B2, and human CYP2B6 genes were activated in reporter gene assays by the same compounds that activate the chicken CYP2H1 phenobarbital-inducible enhancer units. Chicken xenobiotic receptor, pregnane X receptor, and constitutive androstane receptor all bound to the CYP2H1 phenobarbital-inducible enhancer units in gel-shift experiments. In CV-1 cell transactivation assays, mammalian pregnane X receptors activate the chicken phenobarbital-inducible enhancer units to the same extent as does chicken xenobiotic receptor, each receptor maintaining its species-specific ligand spectrum. To assess the reported role of protein phosphorylation in drug-mediated induction, we treated LMH cells with okadaic acid and observed increased mRNA of delta-aminolevulinate synthase and CYP2H1 whereas expression of CYP3A37 was decreased. The effects of okadaic acid and other modifiers of protein phosphorylation in LMH cells are comparable to those seen on CYP2Bs and CYP3As in mammalian primary hepatocyte cultures. These results indicate that closely related nuclear receptors, transcription factors, and signaling pathways are mediating the transcriptional activation of multiple genes by xenobiotics in chicken, rodents, and man.

Studies on the anatomy of the facial nerve.

The present study on the anatomy of the human facial nerve is based on the results of a series of gross dissections, and histological and electro-diagnostic examinations. From our findings it follows that there is a definite spatial orientation of the peripheral branches in the facial nerve trunk. The results of the morphologic studies are in agreement with those obtained by electric stimulation combined with electromyography.

A randomized, double-blind, placebo-controlled study of dextran/pentoxifylline medication in acute acoustic trauma and sudden hearing loss.

The effectiveness of any therapy in acute acoustic trauma or sudden hearing loss of unknown origin has not been demonstrated convincingly. The assessment is difficult because of a relatively high rate of spontaneous recovery. Nevertheless, many different forms of treatment are recommended. We tested one form, treatment with rheoactive substances, in a prospective, randomized, double-blind trial and compared treatment with (a) infusions of dextran-40 with pentoxifylline, (b) saline infusions with pentoxifylline, and (c) saline infusions with placebo medication. Pure-tone hearing thresholds served as control parameters and were taken before treatment and at 1 and 4 weeks after the onset of therapy. Three hundred eighty-two patients were included in the trial, 331 (87%) could be analyzed, 184 patients were treated because of sudden hearing loss, 147 because of acute acoustic trauma. The three treatment groups were comparable in their basic characteristics including the amount of initial hearing loss. In patients with sudden hearing loss, no significant differences of hearing recovery were detected between the three treatment groups. Hearing recovery was also similar in patients with acute acoustic trauma. A power analysis of the study revealed that possible true treatment differences of a hearing recovery of 10 dB would have lead to significance with a probability of over 90%. It is concluded that there were, in fact, no clinically relevant differences in hearing gains of sudden hearing loss or acute acoustic trauma between treatments with saline infusions together with placebo medication and treatment with dextran-40 and/or pentoxifylline.

[Highligts from the interdisciplinary consultation for diseases of the mouth in Basel]. / Erfahrungen aus der interdisziplinären Basler Mundhöhlensprechstunde.

A number of medical specialties traditionally face changes and lesions in the mouth, specific for diseases seen routinely in their everyday practice: gastroenterologists, paediatricians, orthodontists, to name just a few, will easily identify the typical aspect of the tongue, the Koplik spots or a dysgnathia respectively, and the treatment of the underlying diseases in question will resolve the local problem. However, more unusual lesions with chronic or recurrent course may cause a much longer patient career, which may last for months or even years and is interspersed with more or less ineffective local antiseptic or steroid treatment regimens before underlying diseases like pemphigus, lichen, diseases of haematopoetic organs, paraneoplastic lesions or even simple mechanical consequences of parafunctions like bruxismus, are identified as the underlying cause. The otorhinolaryngologist, the dermatologist and the dentist-oral-maxillofacial surgeon offer an interdisciplinary consultation at the Center for Dental Medicine at the University of Basel, in which the advantages of an advanced diagnostic armamentarium and a combined pool of experience proves to be a gain for both the patients and involved medical persons. The otolaryngologist offers diagnostic procedures of the entire upper airways, the dermatologist bridges the gap to allergology, immunology and systemic diseases well known to his specialty. Dental medicine offers diagnostic and therapeutic alternatives, of which the first two have but scarce knowledge. In this article, the different approaches to the most frequently seen diseases and associated problems are described by the dermatologist and dental specialist. The dermatologist, in a succinct state of the art review, describes the problem of pemphigus and pemphigoid, diseases which may cause diagnostic problems because of their tendency to stay limited to the oral mucosa for a longer time. Recurrent aphtosis is another problem encountered frequently, in which a vast research effort has not yet abutted into pertinent knowledge on ethiology and effective therapy for all cases. The dentist-oral-maxillofacial surgeon is especially interested in the specific problem in the diagnosis and treatment of lichen planus i.e. the differentiation of true idiopathic lichen and the lichenoid reaction due to foreign bodies in the mouth. He describes the modern diagnostic and treatment procedures. In spite of missing parts in the puzzle of all these diseases, special consideration in every individual case contributes to more effective management and offers a gain in experience and knowledge to all medical persons involved.

Hearing loss and fluctuating hearing levels in X-linked hypophosphataemic osteomalacia.

BACKGROUND AND OBJECTIVE: X-linked hypophosphataemic osteomalacia is the most common of the genetically determined forms of osteomalacia. The occurrence of hearing loss in X-linked hypophosphataemic osteomalacia has been known since 1984. However, observations on the progression of such hearing loss, and suggestions regarding possible therapy, have not previously been published. METHODS: Case report of a patient with X-linked hypophosphataemic osteomalacia and hearing loss, with three years' audiological follow up, description of empirical therapy and literature review. RESULTS: The patient presented with fluctuating hearing. An audiogram showed mild to severe sensorineural hearing loss mainly in the low and high frequencies. A temporary improvement of 20-40 dB after steroid therapy was observed. Four weeks later, hearing had deteriorated again, mainly in the low frequencies. After one year of fluctuating hearing levels, stabilisation occurred. CONCLUSIONS: In X-linked hypophosphataemic osteomalacia, hearing loss occurs predominantly in the low and high frequencies. The hearing loss type and progression pattern point to an endolymphatic hydrops as the pathogenetic mechanism. Steroid therapy may be of some benefit.

[Hearing loss after lumbar myelography]. / Hörverlust nach Myelographie.

Hearing loss is a possible sequel of myelography. We describe a case of bilateral hearing loss of -60 dB, which recovered completely after an epidural blood patch. In hearing loss or tinnitus in association with a possible cerebrospinal fluid loss after a diagnostic or therapeutic puncture of the lumbar dural sack, an epidural blood patch is a viable and recommended therapeutic option.

Facial nerve disorders: anatomical, histological and clinical aspects.

Three main problems of facial nerve pathology and surgery are considered in this paper. The intraneural anatomy of the facial nerve with its consequences for surgery, especially surgery for facial hyperkinesia, is studied and an original method of selective funicular neurolysis is proposed as a symptomatic operation. Quantitative and qualitative findings in the normal facial nerve are compared to findings in nerves after palsy. The results show that the introduction of neurometric methods for the quantitative assessment may yield further parameters in the judgement of past pathological processes within the nerve. Finally, prognosis of peripheral facial palsy, seen through electrophysiological testing methods, is discussed.

Electroneuronography in prognosis of intratemporal facial palsy, as compared to measurements of rheobase and chronaxy.

Electroneuronography is compared to indicrect chronaximetry in the prognosis of intratemporal facial palsies. In 86 patients, the two methods were found to be congruent and in a number of cases complementary in determining impending degeneration. Both techniques are described and their results evaluated statistically. Our observations indicate that electroneuronography allows to some extent a quantitative evaluation by means of numeric parameters whereas the results of chronaximetry only give a qualitative account of nerve degeneration depending largely on the examiner's experience. Both methods, however, have a certain margin of failure and for more accurate diagnosis and prognosis simultaneous use of both methods is advocated.

Histological and ultrastructural changes in idiopathic facial palsy.

Light- and electron-microscopic findings in the facial nerve are reported in a patient who died 8 months after the onset of acute idiopathic facial palsy. There were signs of regeneration after axonal destruction, and inflammatory infiltrates confined to the intracanalicular part of the nerve were found. These findings suggest a viral or immunological etiology, which may become pathogenetic due to the length and narrowness of the Fallopian canal.

[Fixation of the temporarily removed posterior wall of the auditory canal with plate osteosynthesis: a new technique]. / Fixation der temporär entfernten hinteren Gehörgangswand mit Plattenosteosynthese: Eine neue Technik.

BACKGROUND: Temporary removal of the posterior external ear canal wall allows excellent exposure of the middle ear and epitympanum without the negative sequelae of a cavity as can occur after canal-wall down procedures. Safe fixation of the bony canal wall, however, has not always been possible with a risk for prolonged healing and bone necrosis. MATERIAL AND METHODS: A new technique permits rigid internal fixation of the posterior canal wall. A titanium miniplate of 10 holes length commonly used for orbital rim surgery is adapted to the cortical surface of the mastoid just posterior to the external ear canal. Six holes for 1.3 mm screws are drilled into the cortical bone: two screws behind the ear canal, two screws on the temporal line, and two on the mastoid tip. All fixation material is then removed and a mastoidectomy is carried out with preservation of the cortical bone for the screws. The external ear canal skin, which has been incised previously near the fibrous annulus with lateral extensions, is mobilized laterally using a newly designed retroflected microraspatory. This creates a vital skin flap that might be essential to avoid bone necrosis. No other skin incisions are needed in the canal. The posterior bony canal wall is cut using an oscillating saw Osseoscalpel, secured by facial nerve monitoring. After middle ear surgery and tympanoplasty have been completed, the canal wall is repositioned and fastened precisely in its place with rigid internal fixation allowing a secure stabilization. RESULTS: This new technique has proven to be safe and reliable on the first five patients. There has been no bone necrosis within an observation period of 18 months. CONCLUSIONS: Rigid internal fixation is an alternative technique to safely readapt the posterior auditory canal wall.

CXR, a chicken xenobiotic-sensing orphan nuclear receptor, is related to both mammalian pregnane X receptor (PXR) and constitutive androstane receptor (CAR).

Nuclear receptors constitute a large family of ligand-modulated transcription factors that mediate cellular responses to small lipophilic molecules, including steroids, retinoids, fatty acids, and exogenous ligands. Orphan nuclear receptors with no known endogenous ligands have been discovered to regulate drug-mediated induction of cytochromes P450 (CYP), the major drug-metabolizing enzymes. Here, we report the cloning of an orphan nuclear receptor from chicken, termed chicken xenobiotic receptor (CXR), that is closely related to two mammalian xenobiotic-activated receptors, the pregnane X receptor (PXR) and the constitutive androstane receptor (CAR). Expression of CXR is restricted to tissues where drug induction of CYPs predominantly occurs, namely liver, kidney, small intestine, and colon. Furthermore, CXR binds to a previously identified phenobarbital-responsive enhancer unit (PBRU) in the 5'-flanking region of the chicken CYP2H1 gene. A variety of drugs, steroids, and chemicals activate CXR in CV-1 monkey cell transactivation assays. The same agents induce PBRU-dependent reporter gene expression and CYP2H1 transcription in a chicken hepatoma cell line. These results provide convincing evidence for a major role of CXR in the regulation of CYP2H1 and add a member to the family of xenobiotic-activated orphan nuclear receptors.

[Necrotizing sialometaplasia of the nasal cavity]. / Nekrotisierende Sialometaplasie in der Nasenhöhle.

In the present paper we report two cases of "necrotizing sialometaplasia" in uncommon locations. The relevant literature is reviewed. Necrotizing sialometaplasia is self-limiting and is characterized by necrosis and squamous pseudohypertrophy. Typically occurring in the minor salivary glands of the oral mucosa, our cases appear to be the sixth and seventh cases of involvement of the nasal cavity reported in the literature. Owing to the similarity of histological findings, the term "sialometaplasia" is also used for this disorder. This rare disease is a benign process but may be misdiagnosed as mucoepidermoid or squamous cell carcinoma. Therefore, the possibility of this disease in the nose should again be presented. Both of our cases show that with a histological examination considering defined morphological findings, the diagnosis of necrotizing sialometaplasia can be verified and a diagnosis of a carcinoma can be excluded. Especially with regard to radical and possibly unnecessary invasive therapeutic procedures we emphasize the importance of considering "necrotizing sialometaplasia" as part of the differential diagnosis of nasal lesions.

[Diagnostic importance of electrophysiologic tests in Bell's palsy: report on a longterm follow-up study in 100 cases (author's transl)]. / Ergebnisse elektrophysiologischer Verlaufskontrollen bei der idiopathischen Fazialisparese

The present study is based upon the results of repeated electrodiagnostic tests carried out in a series of 100 cases of Bell's palsy (Determination of Rheobase and Chronaxy). The first symptoms of denervation can be observed during the second week after the onset of the paralysis. This seems to be the critical period in which the ultimate consequences of the lesion are determined: partial or total recovery or complete irreversible denervation. In cases showing a rapid increase of chronaxy during the 2nd week, prognosis is rather poor because a difference of 240% between the affected and the normal side indicates a progressive denervation. Cases in which the difference of chronaxy values during the 2nd week is increased upto 240% (i.e. partial denervation) recover incompletely in 15% of the cases. Estimation of prognosis in Bell's palsy and timing of a facial nerve decompression must be based on reliable facts, such as the results of repeated electrodiagnostic tests (modified measurement of Rheobase and Chronaxy) carried out at least every secont but preferably every day during the first 3 weeks after the onset of the paresis.

[Bullous dermatoses in otorhinolaryngology (author's transl)]. / Manifestationen bullöser Dermatosen im ORL-Bereich.

The early clinical and histological diagnosis of acquired bullous dermatoses (pemphigus, bullous pemphigoid and the cicatricical pemphigoid of mucous membranes) may prove to be difficult when the disease is confined to the upper air and food passages. Histological differentiation between these diseases can also be difficult. In a number of cases it could be shown that the first signs of the disease were frequently mistaken for inflammatory conditions and that routine histological examination rarely confirmed the diagnosis. Only after immunohistological and immunoserological examination could the diagnosis be confirmed. In pemphigus, the treatment can be individually adapted to the titre of antibodies. The typical otorhinolaryngological findings in each of the three diseases are described.

Oral hairy leukoplakia in a HIV-negative renal transplant patient: a marker for immunosuppression?

We report the case of a 58-year-old renal transplant patient who developed oral hairy leukoplakia. Examination for HIV-1 and HIV-2 infection was negative. Biopsy of the lateral tongue showed ballooned prickle cells and electron microscopy revealed herpes-type viruses. In situ hybridization and examinations with the Southern blot technique yielded Epstein-Barr virus. Serology for Epstein-Barr virus was reactive. Immunological investigation of the patient showed a marked decrease of T-helper and T-suppressor cells as the result of immunosuppressive regimen. Oral hairy leukoplakia may be a marker for severe immunosuppression but is not necessarily associated with HIV infection.

[Results of a prospective open study of therapy of sudden deafness with flunarizine]. / Ergebnisse einer prospektiven offenen Studie zur Therapie des Hörsturzes mit Flunarizine.

The treatment effect of the lipophilic calcium antagonist flunarizine on the recovery of sudden hearing loss was evaluated in a prospective phase-II study. Thirty-seven patients presenting with recent and sudden onset of unilateral sensorineural hearing loss were treated with 50 mg flunarizine intravenously for the first two days followed by 10 mg flunarizine orally for four weeks. Pure-tone audiometry was done before the beginning of the treatment and after one and four weeks. The absolute and relative hearing gains after one and four weeks were compared to those of 67 similar patients previously treated with saline infusions and placebo tablets in the same institutions during a double blind study (Probst et al. Acta Otolaryngol. 112 (1992) 435-443). The mean absolute hearing gains of the flunarizine group was 17 dB and 21 dB, after one and four weeks respectively (placebo group: 16 dB and 24 dB). The corresponding relative hearing gains (defined as absolute gain divided by initial hearing loss) were 35% and 43% for the flunarizine group and 33% and 42% for the placebo group. We conclude that flunarizine treatment does not improve the recovery of sudden hearing loss.

[EBV infection as etiology of otomastoiditis with peripheral facial paralysis in the child]. / EBV-Infektion als Ursache einer Otomastoiditis mit peripherer Fazialisparese beim Kind.

Case report on a 15-month-old girl with acute bilateral otomastoiditis with facial nerve palsy in the course of an EBV infection. Although the initial mononucleosis blood test was negative the diagnosis was established by the presence of blastic transformed lymphocytes with immunoreaction for the latent membrane protein of EBV, and the presence of EBV-mRNA in the majority of lymphocytes in the biopsy specimen. The occurrence of facial nerve palsy in the course of an EBV infection is relatively rare but well known. The present case involved a viral otomastoiditis with a typical course, which was complicated by facial nerve palsy after a few days of illness. A case of this kind has only once been described in an adult patient and has never been reported in a child.