Genetic aspects of familial Ménière's disease.

Objective: Ménière's disease (MD) is a chronic illness characterized by sensorineural hearing loss, recurring vertigo attacks, and tinnitus. It is possibly of multifactorial origin, although several families with autosomal dominant inheritance and reduced penetrance have been described. To elucidate the genetic basis of MD, patients and their families were investigated, and linkage analysis was performed.

[How to create a self-cleaning open mastoid cavity].

Our experience from multiple open mastoid cavity revisions is that some of the well-proven techniques to create a self-cleaning small open cavity are not anymore practiced everywhere.The crucial steps are to considerably drill down the facial ridge, as well as the lateral bone (to the level of the sigmoid sinus) and - in cases of a well pneumatized mastoid - to ground down the mastoid tip. The bony rims should be smoothened with a diamond burr.

Hyperfractionated accelerated radiotherapy versus conventional fractionation both combined with chemotherapy in patients with locally advanced head and neck carcinomas: a retrospective analysis of a monoinstitutional series.

Objective: Hyperfractionated accelerated radiotherapy (HART) has been combined with chemotherapy (CC) for locally advanced head and neck cancer, but no data from randomized trials are available for a comparison with conventionally fractionated radiotherapy (CFRT) and CC.

Methods: This monoinstitutional retrospective study compares the results of both treatment schedules: 315 patients with locally advanced carcinoma (UICC stage III and IV) of the oral cavity and the orohypopharynx were treated from January 1990 to March 2006 with a radiochemotherapy combination based on mitomycin C and fluorouracil (HART-CC: 203 patients, CFRT-CC: 112 patients, total dose: 70-72 Gy) with curative intent.

Results: Two- and 4-year survival was 60 and 42 (HART-CC) and 59 and 42% (CFRT-CC; p = 0.

The GNAS1 T393C polymorphism predicts survival in patients with advanced squamous cell carcinoma of the larynx.

Objectives/hypothesis: In previous studies, we have demonstrated that the T-allele of a specific single nucleotide polymorphism (SNP) in the Galphas gene (T393C) correlates with increased Galphas expression and hence apoptosis. The T-allele was associated with a favorable outcome in a variety of human cancers, for example, carcinoma of the urinary bladder, kidney, colorectal, oro- and hypopharynx.

Study Design: The prognostic value of the T393C SNP was retrospectively evaluated in an unselected series of patients treated with curative intent for laryngeal squamous cell carcinomas including all tumor stages with different therapeutic regimens.

Association study of the G-protein beta3 subunit C825T polymorphism with disease progression an overall survival in patients with head and neck squamous cell carcinoma.

The T-allele of a common C825T single nucleotide polymorphism (SNP) in the gene GNB3, encoding the G3 subunit of heterotrimeric G-proteins, is associated with a truncated form of the G3 protein that imparts a greater signaling capacity than the alternative C-allele encoding a nontruncated protein. We analyzed the C825T-allele status with regard to disease progression in patients with head and neck squamous cell carcinoma (HNSCC). The prognostic value of the SNP was evaluated in an unselected series of 341 patients treated with curative intent for HNSCC including all tumor stages with different therapeutic regimens.

Overall and relapse-free survival in oropharyngeal and hypopharyngeal squamous cell carcinoma are associated with genotypes of T393C polymorphism of the GNAS1 gene.

Purpose: In previous studies, we have shown that the T allele of a specific single-nucleotide polymorphism (SNP) in the Galphas gene (T393C) correlates with increased Galphas expression and hence apoptosis. The T allele was associated with a favorable outcome in a variety of human cancers, e.g.

Immunohistochemical evidence of BMP-2, -4 and -7 activity in otospongiosis.

Conclusion: This study is the first to show that bone morphogenetic proteins (BMP)-2, -4 and -7 play a role in active phase otosclerotic bone remodelling (otospongiosis).

Objectives: The role of BMPs in various tissue growth and repair mechanisms is an ongoing topic in the literature. BMP-2, -4 and -7 are known to be of major importance in bone formation and repair.

Infections with human rhinovirus induce the formation of distinct functional membrane domains.

The plasma membrane contains distinct domains that are characterized by a high concentration of sphingolipids and cholesterol. These membrane microdomains also referred to as rafts, seem to be intimately involved in transmembranous signaling and often initiate interactions of pathogens and the host cell membranes. Here, we investigated the further reorganization of membrane rafts in cultured epithelial cells and ex vivo isolated nasal cells after infection with rhinoviruses.

Signaling by way of type IB and II bone morphogenetic protein receptors regulates bone formation in otospongiosis.

Hypothesis: The main goal of this study was to perform an immunohistologic analysis of bone morphogenetic protein receptors (BMPR) in otospongiosis.

Background: BMP-2, -4, and -7 play an essential role in bone formation and repair. They do so as well in otosclerosis.

Revision stapes surgery.

We present the results of our revision stapes operations from 1989 to 2004 (n = 217). Long-term follow-up was performed in the first 135 cases. Eighteen of these patients were revised because of inner ear symptoms, predominantly within the first year.

Prototype of a silicon nitride ceramic-based miniplate osteofixation system for the midface.

Objective: The favorable properties of silicon nitride (Si3N4) ceramics, such as high mean strength level and fracture toughness, suggest biomedical use as an implant material. Minor reservations about the biocompatibility of Si3N4 ceramics were cleared up by previous in vitro and in vivo investigations.

Study Design And Setting: A Si3N4 prototype minifixation system was manufactured and implanted for osteosynthesis of artificial frontal bone defects in 3 minipigs.

Unusual aggressive course of a giant cell tumor of soft tissue during immunosuppressive therapy.

Giant cell tumor of soft tissue with low malignant potential (GCT-ST) is a low-grade, primary soft tissue sarcoma with histological and clinical features similar to giant cell tumor of the bone. The main tumor localizations are the extremities, but it may also occur in the head and neck region. GCT-ST shows a recurrence rate of approximately 15%, but it very rarely metastasizes.

Laser-tonsillotomy for treatment of obstructive tonsillar hyperplasia in early childhood: a retrospective review.

Introduction: Tonsillar hyperplasia leading to dyspnea, dysphagia and other symptoms of obstruction represents a common problem especially in young children where tonsillectomy should be avoided in order to preserve the immunological function of the tonsils. Aim of the study was to assess carbon-dioxide-laser-tonsillotomy as a considered alternative procedure to reduce the tonsillar volume in these children.

Methods: Between 1993 and 2004, 109 children with tonsillar hyperplasia without former episodes of tonsillitis received laser-tonsillotomy mostly (n=98) combined with adenoidectomy.

Antiphospholipid antibodies in patients with sensorineural hearing loss.

Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Sixty patients (mean age 47 years, range 18-76 years) with sudden sensorineural hearing loss were studied with audiograms, stapedial thresholds, otoacoustic emissions, positional and caloric testing. The serologic testing included antibodies against phosphatidylserine and beta(2)-glycoprotein.

Hyperfractionated accelerated chemoradiation with concurrent fluorouracil-mitomycin is more effective than dose-escalated hyperfractionated accelerated radiation therapy alone in locally advanced head and neck cancer: final results of the radiotherapy cooperative clinical trials group of the German Cancer Society 95-06 Prospective Randomized Trial.

Purpose: To report the results and corresponding acute and late reactions of a prospective, randomized, clinical study in locally advanced head and neck cancer comparing concurrent fluorouracil (FU) and mitomycin (MMC) chemotherapy and hyperfractionated accelerated radiation therapy (C-HART; 70.6 Gy) to hyperfractionated accelerated radiation therapy alone (HART; 77.6 Gy).

Immunohistochemical investigations on external auditory canal cholesteatomas.

Objective: The aim of the study was to analyze the biological behavior of external auditory canal cholesteatoma (EACC). The expression and distribution of relevant markers such as transforming growth factor-alpha (TGF-alpha), epidermal growth factor-receptor (EGFR), and the proliferation marker MIB 1 were studied in comparison with normal auditory meatal skin.

Background: EACC are uncommon and knowledge concerning etiology and pathogenesis is limited.

Type III tympanoplasty applying the palisade cartilage technique: a study of 61 cases.

Objective: To determine the morphologic and hearing results of the combined application of the palisade cartilage technique and titanium ossicular replacement prostheses in Type III tympanoplasty.

Study Design: Retrospective review of 61 tympanoplasties.

Setting: Tertiary referral center.

Reconstruction of the stapes superstructure with a combined glass-ceramic (Bioverit) implant in guinea pigs.

Background: Reconstruction of the stapes superstructure continues to be a problem for otologists. Optimal fixation of implants on the stapes footplate has not yet been achieved.

Methods: We postulated that bony fixation, between implant and stapes, could be confined exclusively to the footplate region by modifying the bioactivity of the implant material.

Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential.

The gap junction protein connexin30 (Cx30) is expressed in a variety of tissues that include epithelial and mesenchymal structures of the inner ear. We generated Cx30 (Gjb6) deficient mice by deletion of the Cx30 coding region. Homozygous mutants (Cx30((-/-))) were born at the expected Mendelian frequency, developed normally and were fertile.