Minimal Reporting Standards for Active Middle Ear Hearing Implants.

There is currently no standardized method for reporting audiological, surgical and subjective outcome measures in clinical trials with active middle ear implants (AMEIs). It is often difficult to compare studies due to data incompatibility and to perform meta-analyses across different centres is almost impossible. A committee of ENT and audiological experts from Germany, Austria and Switzerland decided to address this issue by developing new minimal standards for reporting the outcomes of AMEI clinical trials. The consensus presented here aims to provide a recommendation to enable better inter-study comparability.

Management of Life-Threatening Tracheal Stenosis and Tracheomalacia in Patients with Mucopolysaccharidoses.

Several different lysosomal storage diseases, mainly mucopolysaccharidosis (MPS) type I, II, and VI, are complicated by severe obstruction of the upper airways, tracheobronchial malacia, and/or stenosis of the lower airways. Although enzyme replacement therapies (ERTs) are available, the impact of these on tracheobronchial alterations has not been reported. By extending the life expectancy of MPS patients with ERTs, airway problems may become more prevalent at advanced ages. These airway abnormalities can result in severe, potentially fatal, difficulties during anesthetic procedures. Usually, upper airway obstruction is treated by tracheostomy. However, with lower airway malacia and/or stenosis, there are no procedures available to date to address these difficulties. We report the first cases using a new technique of tracheal stenting in patients with MPS type VI (Maroteaux-Lamy syndrome) and type II (Hunter syndrome) who had almost complete tracheal occlusion and total airway collapse. An updated literature review is also reported.

Head and neck paragangliomas: Report of 175 patients (1989-2010).

BACKGROUND: Attention of the otorhinolaryngologist needs to be drawn to the versatile aspects of head and neck paragangliomas (PGLs). METHODS: This study is a retrospective, nonrandomized clinical study of all 175 individuals with PGLs treated in our department between 1989 and 2010. A genetic analysis was performed on 86 patients. RESULTS: The 175 patients presented 224 head and neck PGLs as well as 2 thyroid papillary carcinomas. Genetic analysis resulted in 1 patient positive for a von Hippel-Lindau (VHL) gene mutation and 34 for succinate dehydrogenase (SDH) gene mutations (22 SDHD, 7 SDHC, and 5 SDHB), 12 of the latter carrying a novel mutation. Thirty-three patients (18.9%) had multiple PGLs and 11 patients (6.3%) had a malignant paraganglioma. SDH-mutation carriers had multiple tumors in 64.7% and malignant paragangliomas in 20.6%. CONCLUSIONS: Multifocal occurrence, potential malignancy, genetic aspects, possible coincidence of thyroid carcinoma, and hormone production have to be considered in patients with head and neck PGLs.

Intratympanic dexamethasone/hyaluronic acid mix as an adjunct to intravenous steroid and vasoactive treatment in patients with severe idiopathic sudden sensorineural hearing loss.

OBJECTIVE: To evaluate differences in effectiveness (hearing recovery rates) between idiopathic sudden sensorineural hearing loss (ISSNHL) patients treated with intravenous therapy alone and patients treated with a combination of intravenous and intratympanic therapy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital center. PATIENTS AND INTERVENTIONS: Ninety-four patients with moderate ISSNHL treated with an intravenous steroid and vasoactive regimen (duration of therapy, 9 ± 2.76 d) and 76 patients with severe ISSNHL treated with a combination regimen of intravenous and intratympanic therapy (duration of therapy, 10 ± 2.71 d) were reviewed. In the latter patients' group, a series of 3 intratympanic injections of a dexamethasone/hyaluronic acid mix solution were applied every 2 days. MAIN OUTCOME MEASURE: Pure-tone audiometric thresholds at 0.5, 1, 2, 4, and 8 kHz were compared between groups using the Wilcoxon test. RESULTS: Combination therapy in severe ISSNHL did not show any statistically significant difference in effectiveness to intravenous therapy in moderate ISSNHL (p > 0.05). CONCLUSION: In patients with severe ISSNHL, starting intratympanic steroid therapy as an adjunct early in the course of intravenous steroid and vasoactive therapy improves hearing to a level which is obtained in patients with less severe (moderate) ISSNHL treated with intravenous therapy alone.

Hearing results after hypotympanotomy for glomus tympanicum tumors.

OBJECTIVE: We postulate, that glomus tympanicum tumors (GTTs) may be safely removed without interference with the ossicular chain via a hypotympanotomy approach. STUDY DESIGN: Prospective, nonrandomized anatomic and clinical study. SETTING: Tertiary referral center. PATIENTS: All 17 patients between 1989 and 2009 with GTTs without involvement of the lumen of the jugular bulb. INTERVENTIONS: We used a modified hypotympanotomy approach. Our technique is a modification of the one first published by Shambaugh (1955). Pure-tone audiograms were performed in all patients. Preoperative and postoperative audiograms were modeled in a linear mixed model evaluating hearing threshold for air and bone conduction and air-bone gap at 500, 1,000, 2,000, and 3,000 Hz. In an effort to preserve the normal sound conducting apparatus and hearing, we used a retroauricular approach, exposing widely the jugular bulb, the carotid artery, the protympanum, and even the bony part of the Eustachian tube via a hypotympanotomy. Three formalin-fixed and one macerated temporal bones were dissected step by step under the operating microscope to demonstrate the approach in cadaver dissections. MAIN OUTCOME MEASURE: To evaluate if GTTs can be completely resected without interference with the ossicular chain to improve conductive hearing loss. RESULTS: We found a substantial improvement of hearing threshold after surgery at all frequencies in air conduction. For bone conduction, there was only a slight gain within random variation. The air-bone gap decreased significantly after surgery. CONCLUSION: Our approach demonstrated a safe avenue for complete tumor removal without interference with the continuity of the ossicular chain.

Polysomnography and ApneaGraph in patients with sleep-related breathing disorders.

PURPOSE: To evaluate whether ApneaGraph (AG) and polysomnography (PSG) deliver comparable results in patients with sleep-related breathing disorders. PROCEDURES: A prospective study was performed, which included 14 patients with obstructive sleep apnea syndrome. Apnea-hypopnea index (AHI), hypopnea index (HI), apnea index (AI), obstructive, central and mixed apnea, oxygen saturation (SaO2), pulse and body position were simultaneously assessed by PSG and AG in each individual. RESULTS: There was a good correlation between measurements of AG and PSG for AHI, pulse, SaO2, body position and central apnea. However, our study showed differences between PSG and AG for AI (p = 0.002), HI (p = 0.013), mixed apnea (p = 0.003) and obstructive apnea (p = 0.013). AG indicated that 2/14 patients had a pure upper airway obstruction, 6/14 patients had a predominance of lower obstruction and 6/14 patients had a predominance of upper obstruction. CONCLUSION: AG provides comparable results for AHI, pulse, SaO2, body position and central apnea when compared to PSG, but not for the rest of the measurements. Using AG, the distribution of sites of obstructive events could be identified in this study in all of the patients.

Steroid and vasoactive treatment for acute deafness after attempted hearing preservation acoustic neuroma surgery.

OBJECTIVE: To investigate whether intravenous steroid and vasoactive therapy in the acute postoperative period improves hearing outcome in patients who develop acute deafness after attempted hearing preservation surgery for acoustic neuroma (AN) through a retrosigmoid or a middle cranial fossa approach. STUDY DESIGN AND SETTING: Retrospective controlled study in a tertiary care center. Thirty-six patients who had developed acute deafness after hearing preservation surgery for treatment of an AN were reviewed. Preoperative AAOHNS hearing class was A in 2, B in 2 and D in 32 patients. Twenty-seven patients were treated with prednisolone, hydroxyethyl starch 3% and pentoxifylline intravenously for a period of at least 5 days. Nine patients (controls) did not receive any specific steroid or vasoactive therapy. RESULTS: All patients in both groups remained deaf. CONCLUSIONS: Intravenous therapy with prednisolone, hydroxyethyl starch 3% and pentoxifylline in the acute postoperative period does not improve hearing in patients who develop acute deafness after attempted hearing preservation surgery for AN.

Myeloperoxidase in nasal secretion as a cell-activation marker in acute sinusitis.

BACKGROUND: Acute bacterial infections of the upper respiratory tract, including the nasal sinuses, lead to an activation of neutrophil granulocytes. The protein myeloperoxidase (MPO) is released by this cell type only and therefore can act as a marker for activity of inflammation. METHODS: In a prospective multicenter study, 45 patients with acute bacterial sinusitis (diagnosed by clinical examination, ultrasonography, and X ray or computed tomography scan) were treated with two different antibiotics (Cefditoren or Cefuroxim twice a day for 10 days). Nasal secretions were collected with absorbing foam-rubber samplers and a concentration of MPO was evaluated before the therapy and after 7 +/- 1 days and 14 +/- 2 days of antibiotic treatment. The concentrations of MPO were measured by a radioimmunoassay method and the dilution factors were determined by the lithium method. RESULTS: Significant differences in the MPO concentration in nasal secretions between the first and third visit could be found in both groups (p < 0.01 each). No significant difference was seen within the two different antibiotics used. CONCLUSION: Measurement of MPO concentration in nasal secretions showed a significant change after antibiotic treatment of acute bacterial rhinosinusitis. The reduction of MPO levels correlated with the observed clinical and radiological improvement of the disease. Additional investigations are needed to achieve more knowledge about basal concentrations of MPO in healthy persons and different activation patterns before it can be determined whether the MPO measurement could be a suitable method for monitoring the success of an antibiotic treatment in acute bacterial sinusitis.

Long-term follow-up of patients with microscopic endonasal dacryocystorhinostomy.

BACKGROUND: Stenosis of the nasolacrimal drainage system is a relatively frequent complication of acute or chronic inflammation, trauma, tumor, or congenital malformation. Symptoms include epiphora, intermittent purulent secretion, and swelling of the nasolacrimal sac. The principle of dacryocystorhinostomy (DCR) is to create an artificial opening of the lacrimal sac into the nasal cavity. This can be done via an external as well as an endonasal route. METHODS: In this retrospective study, we examined 74 microscopic endonasal DCRs. which were performed on 70 patients between 1990 and 2000. All of the 74 DCR operations had no major complications intraoperatively. RESULTS: Sixty DCR procedures (81.1%) were successful after a mean follow-up time of 3.18 years. This success rate is lower than those rates reported in the literature for the external approach, which range between 85 and 99%, although follow-up times were shorter in most of these studies. CONCLUSIONS: Advantages of the endonasal approach such as saving the medial palpebral ligament, the lacrimal pump mechanism, and the horizontal apparatus are obvious. If necessary, additional management of sinus, septal, and conchal disease can be performed simultaneously. Our results reveal that the microscopic endonasal approach is a safe and reliable procedure in the management of postsaccular or saccular nasolacrimal duct obstruction and is an alternative to the traditional external route.

Diffusion-weighted MRI of cholesteatomas of the petrous bone.

PURPOSE: To investigate if primary cholesteatomas of the petrous bone show high signal in diffusion-weighted imaging (DWI). MATERIALS AND METHODS: In this blinded study, we compared 15 patients with clinically certain cases and later surgically proven cholesteatomas vs. 12 patients with clinically acute otitis of the middle ear and 20 volunteers without petrous bone disease. Two blinded readers without knowledge of the clinical data decided in consensus agreement whether there was a pathologic signal increase in the petrous bone in an anisotropic single-shot echo-planar imaging (EPI) DWI sequence, an artifact, or no signal increase. RESULTS: Thirteen of 15 patients with cholesteatomas showed bright signal in EPI DWI, whereas 10 of 12 patients with acute otitis media and all volunteers presented the usual low signal of petrous bone. CONCLUSION: EPI DWI is a fast diagnostic method that may be an additional valuable tool in the workup of suspected cholesteatomas. The ability of this technique to differentiate between cholesteatomas and granulomas or chronic otitis is not yet available.

Nasal allergen provocation with Dermatophagoides pteronyssinus in patients with chronic rhinitis referred to a rhinologic surgical center.

BACKGROUND: Skin tests and specific immunoglobulin (Ig)E determinations identify the presence of some immune response to allergens, but frequently do not correlate to allergic disease in vivo. OBJECTIVE: To compare clinical history, skin tests and specific IgE determinations with the results of nasal provocation tests (NPT) to confirm clinically relevant allergy to Dermatophagoides pteronyssinus (DP) in patients with chronic rhinitis referred to a rhinologic surgical center. METHODS: Clinical data of 2,194 patients with chronic nasal disorders treated at two rhinologic referral centers between 1992 and 1998 were evaluated. Clinical allergy work-up was routinely performed corresponding to German and European guidelines providing consistent data in both centers. RESULTS: Of chronic rhinitis patients, 752 of 2,194 (34%) had at least one positive skin reaction to a perennial allergen common in Central Europe. Three hundred seventy-three patients had a positive skin test to DP and were paradigmatically evaluated in detail. In 235 patients, a complete dataset including structured allergic history, skin test, specific IgE determinations, and NPT was available. Quality and severity of symptoms, clinical history, skin tests, and allergen-specific IgE-determinations were unreliable predictors of the outcome of NPT with DP. CONCLUSION: In patients referred for surgical treatment of chronic rhinitis, diagnosis of perennial allergy is unreliable unless confirmed with NPT.