Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

[Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation. German version]. / Management transmodiolärer und transmakulärer Vestibularis- und Cochlearisschwannome mit und ohne Cochleaimplantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

Implantation of a new active bone conduction hearing device with optimized geometry.

Here, we describe the surgical technique for implanting a new, active, transcutaneous bone conduction hearing aid. The implant technology is based on a system that has been in use reliably since 2012. The geometry of the new implant has been adapted based on experience with previously introduced implants. The surgery was feasible, standardized, and safe. Due to the optimized geometric design that improved the bone fit, it is not necessary to use specialized, detailed preoperative planning, except in challenging anatomical conditions; e.g., in young children, malformations, poor pneumatization, or after a canal wall down mastoidectomy.

[Implantation of a new active bone conduction hearing device with optimized geometry. German version]. / Implantation eines neuen, aktiven, knochenverankerten elektronischen Hörimplantats mit verkleinerter Geometrie.

Here, we describe the surgical technique for implanting a new, active, transcutaneous bone conduction hearing aid. The implant technology is based on a system that has been in use reliably since 2012. The geometry of the new implant has been adapted based on experience with previously introduced implants. The surgery was feasible, standardized, and safe. Due to the optimized geometric design that improved the bone fit, it is not necessary to use specialized, detailed preoperative planning, except in challenging anatomical conditions; e.g., in young children, malformations, poor pneumatization, or after a canal wall down mastoidectomy.

Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.

[Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants. German version]. / Intralabyrinthäre Schwannome : Chirurgisches Management und Hörrehabilitation mit Cochleaimplantaten.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebello-pontine angle (CPA), 1 a transotic including the CPA and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients opted for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single stage procedure; 1 patient received extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI following repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontile angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Provided performed early enough, cochlear implantation after surgical removal of ILS is an option for auditory rehabilitation, thus representing-in contrast to the "wait-and-test-and-scan" strategy-a therapeutic approach.

Intracochlear drug delivery in combination with cochlear implants : Current aspects.

Local drug application to the inner ear offers a number of advantages over systemic delivery. Local drug therapy currently encompasses extracochlear administration (i. e., through intratympanic injection), intracochlear administration (particularly for gene and stem cell therapy), as well as various combinations with auditory neurosensory prostheses, either evaluated in preclinical or clinical studies, or off-label. To improve rehabilitation with cochlear implants (CI), one focus is the development of drug-releasing electrode carriers, e. g., for delivery of glucocorticosteroids, antiapoptotic substances, or neurotrophins to the inner ear. The performance of cochlear implants may thus be improved by protecting neuronal structures from insertion trauma, reducing fibrosis in the inner ear, and by stimulating growth of neuronal structures in the direction of the electrodes. Controlled drug release after extracochlear or intracochlear application in conjunction with a CI can also be achieved by use of a biocompatible, resorbable controlled-release drug-delivery system. Two case reports for intracochlear controlled release drug delivery in combination with cochlear implants are presented. In order to treat progressive reduction in speech discrimination and increased impedance, two cochlear implant patients successfully underwent intracochlear placement of a biocompatible, resorbable drug-delivery system for controlled release of dexamethasone. The drug levels reached in inner ear fluids after different types of local drug application strategies can be calculated using a computer model. The intracochlear drug concentrations calculated in this way were compared for different dexamethasone application strategies.

[Intracochlear drug delivery in combination with cochlear implants : Current aspects]. / Intracochleäre Medikamentenapplikation in Verbindung mit Cochleaimplantaten : Aktuelle Aspekte.

Local drug application to the inner ear offers a number of advantages over systemic delivery. Local drug therapy currently encompasses extracochlear administration (intratympanic injection); intracochlear administration, particularly for gene and stem cell therapy; as well as various combinations with auditory neurosensory prostheses, either evaluated in preclinical or clinical studies, or off-label. To improve rehabilitation with cochlear implants (CI), one focus is the development of drug-releasing electrode carriers, e. g., to deliver glucocorticosteroids, antiapoptotic substances, or neurotrophines to the inner ear. By protecting neuronal structures from insertion trauma, reducing fibrosis in the inner ear, and by stimulating growth of neuronal structures in the direction of the electrodes, the performance of CIs should be improved. Controlled drug release after extracochlear or intracochlear application in conjunction with a CI can be achieved by, e.g., use of a biocompatible, resorbable controlled-release drug delivery systems. Two case reports are presented. In order to treat worsened speech discrimination and increased impedance, these CI-wearing patients successfully underwent intracochlear placement of a biocompatible, resorbable drug delivery system for controlled release of dexamethasone. The drug levels reached in inner ear fluids after different types of local drug application strategies can be calculated using computer models. The intracochlear drug concentrations calculated in this way were compared for different dexamethasone application strategies.

[Invasive aspergillosis of the skull base with orbit infiltration]. / Invasive Aspergillose der Schädelbasis mit Orbitaspitzensyndrom.

BACKGROUND: [corrected] Aspergillosis of the paranasal sinuses is subdivided into noninvasive and invasive types, depending on invasion of the tissue. The invasive form often occurs in immunodeficient patients and can be divided further into granulomatous, chronic invasive, and acute fulminating forms. CASE REPORT: We report the clinical course of an immunosuppressed 64-year-old male with invasive aspergillosis originating from the sphenoid sinus with infiltration of the orbit and intracranial extension into the cavernous sinus. The patient was referred to our hospital with loss of vision, ptosis, and ophthalmoplegia of 3-month duration. Additionally he suffered from diabetes mellitus II and kidney failure after kidney transplantation. After CT scanning, endonasal sinus debridement and decompression of the orbit were carried out immediately. Histology revealed invasive aspergillosis. Postoperatively, both systemic and local antimycotic therapy and antibiotic treatment were performed. According to recommendations of the Undersea and Hyperbaric Medicine Society, cerebral abscess is a certain indication of hyperbaric oxygenation. We decided to attempt therapy for that as well. The patient died 3 weeks after surgical intervention due to carotid dissection. CONCLUSION: Invasive aspergillosis of the paranasal sinuses and the skull base of immunsuppressed patients is usually lethal because of intracranial complications. Therefore, fast diagnosis using CT and MRI and surgical and antimycotic therapy are necessary.

[Sclerotherapy with OK-432 for cystic tumors in the neck region]. / Sklerotherapie zystischer Raumforderungen im Halsbereich mit OK-432.

BACKGROUND: The intralesional injection of OK-432 has been proposed as an effective treatment of lymphangiomas and ranulas. The aim of this study was to review our experience with sclerotherapy of benign cervical cysts as an alternative method to surgical extirpation. PATIENTS AND METHODS: We performed a case note review of six patients who received OK-432 injections in a concentration of 0.01 mg/ml between March 2002 and December 2003. RESULTS: Disappearance of the lesions was achieved after one to three injections in all patients. We found a complete remission in two of six patients and a marked reduction in four of six patients. There were no significant complications observed. CONCLUSION: OK-432 is an effective alternative in the treatment of benign cervical cysts, but it is very important to exclude malignancy-associated diseases before instillation of OK-432. Long-term results will be awaited.

[Fibrous dysplasia of the petrous bone]. / Fibröse Dysplasie des Felsenbeins.

Fibrous dysplasia named after Jaffè and Lichtenstein is a benign, self-limiting disorder of the bone structure. The normal bone is replaced by cellular fibrous connective tissue. Fibrous dysplasia can be differentiated into monostotic and polyostotic forms and the McCune-Albright syndrome (combination of polyostotic areas in the bone with hyperpigmentation of the skin and endocrinological disturbances). The rare disease is predominantly found in the 1st to 3rd decade of life in the femur or humerus. In about 15% of all cases it affects the facial bones, and one-third of these is located in the jaw bone. Extremely rare is the invasion of the petrous bone. A 45-year-old female patient was examined because of tinnitus persisting for 1 year. In addition, she was suffering from increased hearing reduction and recurrent vertigo. The CT and MRT showed a milky glass-like tumor, which was limited by an osteolytic process situated in the left petrous bone. The process was removed via the mastoid. Histological examination revealed fibrous dysplasia. A highly differentiated osteosarcoma and an ossifying fibroma as important differential diagnoses have to be excluded because of the different therapeutic consequences.

[Effect of Cyclosporin-A on gingiva]. / Auswirkungen von Cyclosporin-A auf die Gingiva.

Cyclosporin-A is a new immunosuppressive agent which has been used successfully to prevent organ rejection. Gingival overgrowth is one side-effect of the drug which is of interest for the dentist. The pathogenesis of the gingival alteration is unknown, but the dental plaque is discussed as a co-destructive factor.