Clinical High-Resolution Imaging of the Inner Ear by Using Magnetic Resonance Imaging (MRI) and Cone Beam Computed Tomography (CBCT).

PURPOSE: Imaging of the delicate inner ear morphology has become more and more precise owing to the rapid progress in magnetic resonance imaging (MRI). However, in clinical practice, the interpretation of imaging findings is hampered by a limited knowledge of anatomical details which are frequently obscured by artifacts. Corresponding review articles are as rare in journals as they are in reference books. This shortness prompted us to perform a direct comparison of imaging with anatomical whole-mount sections as a reference. It was the intention of this paper to compare the microscopic anatomy of a human inner ear as shown on anatomical whole-mount sections with high-resolution MRI and cone beam computed tomography (CBCT). Both are available in clinical routine and depict the structures with maximum spatial resolution. It was also a goal of this work to clarify if structures that were observed on MRI in a regular manner correlate with factual inner ear anatomy or correspond with artifacts typical for imaging. METHODS: A fresh human anatomical specimen was examined on a clinical 3-Tesla MRI scanner using a dedicated surface coil. The same specimen was then studied with CBCT. In each imaging modality, high-resolution 3D data sets which enabled multiplanar reformatting were created. In the second step, anatomical whole-mount sections of the specimen were cut and stained. This process enabled a direct comparison of imaging with anatomical conditions. RESULTS: Clinical MRI was able to depict the inner ear with remarkable anatomical precision. Strongly T2-weighted imaging protocols are exquisitely capable of showing the fluid-filled components of the inner ear. The macular organs, ampullar crests and cochlear aqueduct were clearly visible. Truncation artifacts are prone to be confused with the delicate membrane separating the endolymphatic from the perilymphatic compartment. However, it was not possible to directly depict this borderline. CONCLUSIONS: With the maximum resolution of magnetic resonance tomography, commonly used in everyday clinical practice, even the smallest details of the inner ear structures can be reliably displayed. However, it is important to distinguish between truncation artifacts and true anatomical structures. Therefore, this study can be useful as a reference for image analysis.

German guidelines on community-acquired acute bacterial meningitis in adults.

INTRODUCTION: The incidence of community-acquired acute bacterial meningitis has decreased during the last decades. However, outcome remains poor with a significant proportion of patients not surviving and up to 50% of survivors suffering from long-term sequelae. These guidelines were developed by the Deutsche Gesellschaft für Neurologie (DGN) under guidance of the Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF) to guide physicians through diagnostics and treatment of adult patients with acute bacterial meningitis. RECOMMENDATIONS: The most important recommendations are: (i) In patients with suspected acute bacterial meningitis, we recommend that lumbar cerebrospinal fluid (with simultaneous collection of serum to determine the cerebrospinal fluid-serum glucose index and blood cultures) is obtained immediately after the clinical examination (in the absence of severely impaired consciousness, focal neurological deficits, and/or new epileptic seizures). (ii) Next, we recommend application of dexamethasone and empiric antibiotics intravenously. (iii) The recommended initial empiric antibiotic regimen consists of ampicillin and a group 3a cephalosporin (e.g., ceftriaxone). (iv) In patients with severely impaired consciousness, new onset focal neurological deficits (e.g. hemiparesis) and/or patients with newly occurring epileptic seizures, we recommend that dexamethasone and antibiotics are started immediately after the collection of blood; we further recommend that -if the imaging findings do not indicate otherwise -a lumbar CSF sample is taken directly after imaging. (v) Due to the frequent occurrence of intracranial and systemic complications, we suggest that patients with acute bacterial meningitis are treated at an intensive care unit in the initial phase of the disease. In the case of impaired consciousness, we suggest that this is done at an intensive care unit with experience in the treatment of patients with severe CNS diseases. CONCLUSIONS: The German S2k-guidelines give up to date recommendations for workup, diagnostics and treatment in adult patients with acute bacterial meningitis.

Endovascular intervention in treatment of refractory epistaxis.

Objective: Angiographic embolization is an established method of treating intractable cases of posterior epistaxis. This study aimed to test the effectiveness and safety of this method in treating cases refractory to conservative methods. Methods: A descriptive retrospective analysis of consecutive cases referred to the department of interventional radiology with refractory epistaxis from January 2001 to December 2018 and received a selective angiographic embolization of the sphenopalatine artery was done. Only epistaxis of idiopathic origin was included in the study. Results: During this period, 98 embolizations were performed. The success rate reached 81.6%. Minor complications were registered in 5%, with no single major complication. The length of stay was 10.5 ± 5.6. Conclusion: Selective angiographic embolization is an effective, safe, and minimally invasive method in treating refractory epistaxis.

Auditory Brainstem Implants-Hearing Restoration in Congenitally Deaf Children.

BACKGROUND: Children who are born deaf can learn to hear and to speak with the aid of a cochlear implant (CI). If the implantation of a CI is not possible for anatomical reasons, an auditory brainstem implant (ABI) is the only surgical option for auditory rehabilitation. It is estimated that about 5 to 45 children could potentially benefit from this treatment in Germany each year. In this article, we present and discuss the current state of the scientific evidence. METHODS: The PubMed and Embase databases were searched for relevant publications from 2010 onward. 15 articles reporting at least 10 cases with at least one year of auditory follow-up were included in the analysis. The results, including CAP ("categories of auditory performance") scores on a scale of 0 to 7, are presented and compared with the authors' own findings in a series of 38 patients. RESULTS: All of the publications show that children who do not suffer from impairments of other kinds hear significantly better with an ABI than those with additional handicaps. Early implantation is advantageous, under the age of three years if possible. The results vary widely across publications and from patient to patient. The mean CAP score in all publications is 3.57 (standard deviation [SD], 1.04). 38.24% of the patients (SD 18.68) achieved the ability to understand spoken language (CAP ≥= 5), more specifically, the ability to communicate in everyday life without lip reading, in person and some even succeed in conversing over the telephone. CONCLUSION: ABI is a safe and effective treatment for sensorineural deafness in congenitally deaf children who cannot be treated with a cochlear implant. In particular, children without any other impairments have a good chance of developing the ability to understand spoken language, especially if the implantation is performed early.

Postoperative Cochlear Obliteration after Retrosigmoid Approach in Patients with Vestibular Schwannoma.

Objective Vestibular schwannomas (VSNs) account for the vast majority of lesions located in the cerebellopontine angle (CPA). The goals of VSN surgery are possible total tumor removal, intact facial nerve function, and preservation of cochlear nerve function. In cases of pre- or postoperative deafness, restoration of hearing with auditory brainstem implant (ABI) or cochlear implant (CI) is a promising treatment option with normally better results in CI than in ABI. The aim of this retrospective study is to evaluate cochlear ossification or obliteration secondary to a retrosigmoid approach, which is important for later CI, especially in single-sided deafness. Materials and Methods We retrospectively reviewed data from our suboccipital retrosigmoid operation database for the period from January 2008 to February 2015. A total of 65 patients with VSN could be analyzed retrospectively. The patient's data (age, gender, side of operation, tumor entities, and the duration of follow-up) were evaluated. The most recent T2-weigted MRI exams were taken into account for the evaluation of cochlea. To compare left and right sides, the coronal reformatted images were reconstructed in a symmetrical way. Results Twenty-two out of 65 (33%) patients had cochlear obliteration in our survey. The cochlear obliteration was more common in females (44 vs. 20% in males). The median follow-up was 28 months. The size and the intrameatal extension of tumors could not be considered as factors influencing the cochlear obliteration rate. According to our survey, 33% of suboccipital approaches manifested cochlear obliteration to some extent. It was more common in females. The intrameatal extension of tumors played statistically no role in the occurrence of postoperative cochlear obliteration. Whether the prohibition of cochlear ossification can be best achieved by retrosigmoid approaches, compared to other approaches to the CPA, or not could be a subject of future studies.

Reconstruction of the mastoid using a titanium cage.

OBJECTIVES: Evaluate reconstruction of the posterior canal wall, including the mastoid cavity after canal wall-down cholesteatoma removal. METHODS: A titanium mesh metal cage was used to rebuild the posterior auditory canal wall and mastoid cell system in 12 patients undergoing cholesteatoma removal. The cage was then covered with pieces of cartilage to reconstruct the soft tissue surface. RESULTS: All reconstructions epithelialized completely. There was no denudation of the titanium material or signs of extrusion. Recurrences of cholesteatoma occurred twice according to the condition of the underlying disease, not related to the material. CONCLUSION: The titanium cage showed good biocompatibility in the middle ear space and the potential for rebuilding the mastoid cell system, making it a versatile tool for primary cases with large defects and in revision cases where soft tissue with cartilage-only reconstructions failed. Reconstruction of the posterior canal wall and mastoid by means of a titanium cage can be used in situations where adhesive tensions need a more stable construction and support of the posterior canal wall.

Pulsatile tinnitus: imaging and differential diagnosis.

BACKGROUND: Pulsatile tinnitus, unlike idiopathic tinnitus, usually has a specific, identifiable cause. Nonetheless, uncertainty often arises in clinical practice about the findings to be sought and the strategy for work-up. METHODS: Selective literature review and evaluation of our own series of patients. RESULTS: Pulsatile tinnitus can have many causes. No prospective studies on this subject are available to date. Pulsatile tinnitus requires both a functional organ of hearing and a genuine, physical source of sound, which can, under certain conditions, even be objectified by an examiner. Pulsatile tinnitus can be classified by its site of generation as arterial, arteriovenous, or venous. Typical arterial causes are arteriosclerosis, dissection, and fibromuscular dysplasia. Common causes at the arteriovenous junction include arteriovenous fistulae and highly vascularized skull base tumors. Common venous causes are intracranial hypertension and, as predisposing factors, anomalies and normal variants of the basal veins and sinuses. In our own series of patients, pulsatile tinnitus was most often due to highly vascularized tumors of the temporal bone (16%), followed by venous normal variants and anomalies (14%) and vascular stenoses (9%). Dural arteriovenous fistulae, inflammatory hyperemia, and intracranial hypertension were tied for fourth place (8% each). CONCLUSION: The clinical findings and imaging studies must always be evaluated together. Thorough history-taking and clinical examination are the basis for the efficient use of imaging studies to reveal the cause of pulsatile tinnitus.

Imaging of cerebrospinal fluid leaks.

The present article provides an overview of epidemiology, clinical signs and symptoms, imaging techniques and specific imaging findings in cerebrospinal fluid leaks. Imaging protocols are supplemented with practical hints for invasive techniques. Emphasis is on the full scope of imaging findings in traumatic and nontraumatic cranial and spinal leaks.

Histologic and morphologic evaluation of explanted bone anchors from bone-anchored hearing aids.

Bone-anchored hearing aids are a standard option in rehabilitation of patients with conductive or mixed hearing loss, and also CROS fitting. However, the skin-penetrating bone anchor repeatedly gives reason for discussion about the risk of infection of surrounding tissues as a major cause of malfunction. In the present study, explanted bone anchors with surrounding bone and soft tissue were examined and compared with the morphology of lost implants. The anchors originated from five patients. Two needed explantation due to deafness with the need of cochlea implantation. A third patient underwent explantation due to meningeal irritation by the bone anchor. Another patient lost the implant due to mechanical stress shortly after implantation. The last implant was lost in a child without apparent reason. All implants were clinically free of infection and had been stable for a median implantation period of 12 months. During the explantation procedure, the fixtures were recovered together with the attached soft tissue and bone. The specimens were examined by light microscopy or scanning electron microscopy (SEM). Sectioning for light microscopy was performed with a diamond-coated saw microtome. Histopathologic examination of the surrounding skin and subcutaneous soft tissue showed slight inflammation in one case only. The bone was regularly vital, presenting no signs of inflammation. The threads of the fixtures were filled with bone, with particularly strong attachment to the flank of traction. The SEM investigation exposed the ultrastructural interaction of bone with the implant surface. Filiform- and podocyte-like processes of osteocytes attach to the implant; lost implants did not reflect these features. Implant integration involves both osseointegration as well as soft tissue integration. Titanium oxide as the active implant surface promotes this integration even in unstable implants. The morphologic analysis exposed structural areas of the implant with weak bone-to-metal contact. Optimized implant design with modified surface and threads may additionally improve osseointegration of hearing aid bone anchors.

Reconstruction of middle ear malformations.

Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients.

Secreted glycoprotein myocilin is a component of the myelin sheath in peripheral nerves.

The structure of the myelin sheath in peripheral nerves requires the expression of a specific set of proteins. In the present study, we report that myocilin, a member of the olfactomedin protein family, is a component of the myelin sheath in peripheral nerves. Myocilin is a secreted glycoprotein that forms multimers and contains a leucine zipper and an olfactomedin domain. Mutations in myocilin are responsible for some forms of glaucoma, a neurodegenerative disease that is characterized by a continuous loss of optic nerve axons. Myocilin mRNA was detected by Northern blotting in RNA from the rat sciatic and ophthalmic nerves. By one- and two-dimensional gel electrophoresis of proteins from the rat and human sciatic nerves, myocilin was found to migrate at an isoelectric point (pI) of 5.2-5.3 and a molecular weight of 55-57 kDa. Immunohistochemistry showed immunoreactivity for myocilin in paranodal terminal loops of the nodes of Ranvier and outer mesaxons and basal/abaxonal regions of the myelin sheath. Double-labeling experiments with antibodies against myelin basic protein showed no overlapping, while overlapping immunoreactivity was observed with antibodies against myelin-associated glycoprotein. The expression of myocilin in the sciatic nerve became detectable at postnatal day (P) 15 and reached adult levels at P20. No or minor expression of myocilin mRNA was found in brain, spinal cord, and optic nerve. mRNA of myocilin was detected in schwannoma cells in situ, but at considerably lower levels than in myelinated nerves. Myocilin might significantly contribute to the structure of the myelin sheath in peripheral nerves.

Interaction of spiral ganglion neuron processes with alloplastic materials in vitro(1).

The cochlear implant (CI) involves the introduction of alloplastic materials into the cochlea. While current implants interact with cochlear neurons at a distance, direct interactions between spiral ganglion (SG) neurites and implants could be fostered by appropriate treatment with neurotrophic factors. The interactions of fibroblasts and osteoblasts with alloplastic materials have been well studied in vitro and in vivo. However, interactions of inner ear neurons with such alloplastic materials have yet to be described. To investigate survival and growth behavior of SG neurons on different materials, SG explants from post-natal day 5 rat SG were cultured for 72 h in the presence of neurotrophin-3 (10 ng/ml) on titanium, gold, stainless steel, platinum, silicone and plastic surfaces that had been coated with laminin and poly-L-lysine. Neurite outgrowth was investigated after immunohistological staining for neurofilament, by image analysis to determine neurite extension and directional changes. Neurite morphology and adhesion to the alloplastic material were also evaluated by scanning electron microscopy (SEM). On titanium, SG neurites reached the highest extent of outgrowth, with an average length of 662 microm and a mean of 31 neurites per explant, compared to 568 microm and 21 neurites on gold, 574 microm and 24 neurites on stainless steel, 509 microm and 16 neurites on platinum, 281 microm and 12 neurites on silicone and 483 microm and 31 neurites on plastic. SEM revealed details of adhesion of neurites and interaction with non-neuronal cells. The results of this study indicate that the growth of SG neurons in vitro is strongly influenced by alloplastic materials, with titanium exhibiting the highest degree of biocompatibility with respect to neurite extension. The knowledge of neurite interaction with different alloplastic materials is of clinical interest, as development in CI technology leads to closer contact of implanted electrodes with surviving inner ear neurons.

Revision surgery after saccotomy for Meniere's disease: does it make sense?

Saccotomy is an established surgical treatment for Meniere's disease that involves exposing and opening the endolymphatic sac and draining the endolymph. However, in patients experiencing recurrent vertigo after saccotomy, it is questionable whether revision saccotomy should be recommended as opposed to more invasive procedures, such as neurectomy of the vestibular nerve. To determine the utility of revision saccotomy, we conducted a retrospective study of 29 patients undergoing this procedure for recurrent vertigo. Patient charts were reviewed for the re-occurrence of any vertiginous symptoms and outcomes with regard to hearing results and post-operative complications. An attempt was made to correlate vertigo status post-revision with surgical findings at the time of revision. After an average follow-up of 20 months, 18 patients (62%) were free of vertigo, and four patients (14%) had a significant decrease in the frequency and intensity of vertiginous attacks. Two patients (7%) relapsed after a vertigo-free period of 18 months, and five patients (17%) experienced no clinical improvement after revision surgery. In patients with a successful sac revision, a common finding at the time of revision was new bone formation in the endolymphatic sac area (n=11), which may have caused blocking of the endolymphatic drainage. Our results demonstrate that revision saccotomy should be considered for recurrent Meniere disease before other, more invasive, surgical options.