Active middle ear implant coupled bilaterally to the round window despite bilateral implanted stapes prostheses.

After stapes surgery, patients with mixed or moderate hearing loss have limited possibilities for hearing improvement. We are reporting on a patient who underwent stapedotomy bilaterally 20 years ago and had sensorineural and mixed hearing loss. Recurrent otitis externa prevented the use of hearing aids. This patient was treated bilaterally with the Vibrant Soundbridge (Med-El, Innsbruck, Austria) successively. The Schuknecht piston stapes prostheses remained in situ. The Floating Mass Transducer (FMT; Med-El) was coupled to the round window (RW) and provided good acoustic reinforcement bilaterally. In conclusion, for patients with otosclerosis and stapes surgery, the FMT-RW coupling (Bess AG, Berlin, DE) is a safe procedure with good acoustic amplification. Laryngoscope, 2016 127:500-503, 2017.

Gender-specific risk factors in post-tonsillectomy hemorrhage.

There are gender-specific differences in the frequency and course of different diseases. Specifically, some studies have shown an increased risk of post-tonsillectomy hemorrhage (PTH). The aim of the study was to investigate gender-specific risk factors for hemorrhage after tonsillectomy (TE)/abscess-TE. We anonymously reviewed and recorded the relevant data of all patients (≥14 years) who underwent a TE/abscess-TE between 2011 and 2013 in the ENT Clinic of the Charité Universitätsmedizin Berlin, Campus Benjamin Franklin. A patient survey was used to complete missing data. We analyzed gender-specific risk factors for PTH. During the study period, 460 operations were performed and the data of 250 patients were analyzed (213 TE and 37 abscess-TE). The median patient age was 27 years (ranging from 14 to 83 years). The rate of primary PTH (<24 h after TE/abscess-TE) was 3 %, and the rate of secondary PTH (>24 h) was 23 %. A significantly higher PTH rate was associated with males (p = 0.037), which was still apparent in ages 21-30 after sub-classification. Multivariate analysis calculated diagnosis, regular alcohol consumption and administration of glucocorticoids to be independent risk factors associated with gender. In conclusion, the PTH rate is gender-specific, and male patients are at higher risk, especially in young adulthood. Therefore, doctors should advise male patients of the increased risk of bleeding and stress the importance of compliance. Also, close postoperative follow-up is desirable.

Meta-analysis of survival in patients with HNSCC discriminates risk depending on combined HPV and p16 status.

Data indicate a better prognosis for human papillomavirus (HPV)-associated head and neck squamous cell carcinoma (HNSCC). HPV and p16 detection are established markers for HPV-related HNSCC. Both are accepted as survival-independent predictors. Previous studies investigating the survival in HNSCC patients depending on HPV(+/-) and p16(+/-) status consistently found discordant results with p16(-)/HPV(+) and p16(+)/HPV(-). However, no meta-analysis regarding the survival according to combined HPV/p16 status has been performed yet. The objective of this study was to discriminate the impact of combined HPV(+/-) and p16(+/-) status on survival. Data sources were identification and review of publications assessing survival of the distinct subgroups with both p16 and HPV investigated in HNSCC until February, 2015. A meta-analysis was performed to classify survival and clinical outcomes. 18 out of 397 articles (4424 patients) were eligible for the meta-analysis. The percent proportion of the subgroups was 25 % for HPV(+)/p16(+), 61.2 % for HPV(-)/p16(-), 7.1 % for HPV(-)/p16(+) and 6.8 % for HPV(+)/P16(-). The meta-analysis showed a significantly improved 5-year overall survival (OS), 5-year disease-free survival and their corresponding hazard ratio for HPV(+)/p16(+) HNSCC in comparison to HPV(-)/p16(-), HPV(+)/p16(-) and HPV(-)/p16(+). The 5-year OS of the HPV(-)/p16(+) subgroup was intermediate while HPV(+)/p16(-) and HPV(-)/p16(-) HNSCC had the shortest survival. With current therapeutic strategies, survival of patients with HNSCC is better if associated with HPV(+)/p16(+) or HPV(-)/p16(+). Clinical trials are needed to confirm the distinct survival pattern and to investigate possible differences in survival for HPV(+)/p16(-) and HPV(-)/p16(+) HNSCC. To further differentiate p16(+) HNSCC, HPV testing may be advisable.

Incidence and long-term survival of patients with de novo head and neck carcinoma after liver transplantation.

BACKGROUND: Liver transplant recipients have an increased risk of developing de novo malignancies. METHODS: We conducted a prospective evaluation of clinicopathological data and predictors for overall survival (OS) in patients with head and neck squamous cell carcinoma (HNSCC) after liver transplantation (1988 to 2010). RESULTS: Thirty-three of 2040 patients who underwent liver transplantation (1.6%) developed de novo HNSCC. The incidence of HNSCC in liver transplant recipients with end-stage alcoholic liver disease (26) was 5%. After a median follow-up of 9 years, 1-year, 3-year, and 5-year OS rates were 74%, 47%, and 34%, respectively. Tumor size, cervical lymph node metastases, tumor site, and therapy (surgery only vs surgery and adjuvant radiotherapy [RT]/chemoradiotherapy [CRT] vs RT/CRT only; p < .0001) were significantly associated with OS in univariate analysis. However, surgery only predicted OS independently in multivariate analysis. CONCLUSION: Early diagnosis and surgical treatment of de novo HNSCC are crucial to the outcome. HNSCC risk should be taken into close consideration during posttransplantation follow-up examinations, especially among patients with a positive history of smoking and alcohol consumption.

Biology and immunology of cancer stem(-like) cells in head and neck cancer.

Immunological approaches against tumors including head and neck squamous cell carcinoma (HNSCC) have been investigated for about 50 years. Such immunotherapeutic treatments are still not sufficiently effective for therapy of HNSCC. Despite the existence of immunosurveillance tumor cells may escape from the host immune system by a variety of mechanisms. Recent findings have indicated that cancer stem(-like) cells (CSCs) in HNSCC have the ability to reconstitute the heterogeneity of the bulk tumor and contribute to immunosuppression and resistance to current therapies. With regard to the CSC model, future immunotherapy possibly in combination with other modes of treatment should target this subpopulation specifically to reduce local recurrence and metastasis. In this review, we will summarize recent research findings on immunological features of CSCs and the potential of immune targeting of CSCs.

Inhibition of pan-class I phosphatidyl-inositol-3-kinase by NVP-BKM120 effectively blocks proliferation and induces cell death in diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma (DLBCL) is the most frequent aggressive lymphoma, with a great demand for novel treatments for relapsing and refractory disease. Constitutive activation of the phosphatidyl-inositol-3-kinase (PI3K)/Akt/mammalian target of rapamycin (mTOR) signaling pathway is often detected in this lymphoma. Inhibition of this signaling cascade with the pan-class I PI3K inhibitor NVP-BKM120 decreased cell proliferation and increased apoptotic cell death. DLBCL proliferation was further decreased if NVP-BKM120-induced autophagy was blocked. Treatment with NVP-BKM120 was associated with an increase of the pro-apoptotic BH3-only proteins Puma and Bim and down-regulation of the anti-apoptotic Bcl-xL and Mcl-1. Translation of Bcl-xL and Mcl-1 is facilitated by cap-dependent mRNA translation, a process that was partially inhibited by NVP-BKM120. Overall, we demonstrated here the potential of NVP-BKM120 for the treatment of DLBCL.

RORγt⁺ innate lymphoid cells acquire a proinflammatory program upon engagement of the activating receptor NKp44.

RORγt⁺ innate lymphoid cells (ILCs) are crucial players of innate immune responses and represent a major source of interleukin-22 (IL-22), which has an important role in mucosal homeostasis. The signals required by RORγt⁺ ILCs to express IL-22 and other cytokines have been elucidated only partially. Here we showed that RORγt⁺ ILCs can directly sense the environment by the engagement of the activating receptor NKp44. NKp44 triggering in RORγt⁺ ILCs selectively activated a coordinated proinflammatory program, including tumor necrosis factor (TNF), whereas cytokine stimulation preferentially induced IL-22 expression. However, combined engagement of NKp44 and cytokine receptors resulted in a strong synergistic effect. These data support the concept that NKp44⁺ RORγt⁺ ILCs can be activated without cytokines and are able to switch between IL-22 or TNF production, depending on the triggering stimulus.

Long-term performance of cochlear implants in postlingually deafened adults.

OBJECTIVE: To evaluate the stability of long-term hearing performance after cochlear implantation (CI) in postlingually deafened adults and to explore the boundaries and limitations of the present test batteries for adult CI patients. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A cohort of 1005 postlingually deafened adult cochlear implantees, who received their implants after age 18 years, was unilaterally implanted and had no inner ear malformations or cochlear ossification. Hearing performance with cochlear implant was evaluated with the help of 5 standard German speech tests. RESULTS: The average performance improved significantly during the first 6 months in all tests (learning phase) and afterward entered a plateau phase in which no statistically significant improvements or deteriorations were observed for more than 20 years of follow-up. For each test, the average performance of the cohort, the ceiling effect, and the average results for high and low performers are presented. CONCLUSIONS: In this study, postlingually deafened adults required about 6 months to learn how to process the artificial signals delivered by the cochlear implant. After this learning phase, the hearing performance entered a stable plateau phase for more than 20 years. This stability reveals the long-term reliability of the technology and the biological stability of the electrode-nerve interface over years. In this study, the authors also evaluated the "ceiling effect" with 5 standard German speech tests, used for evaluation of postlingually deafened adult CI patients.

Preoperative prediction of vestibular schwannoma's nerve of origin with posturography and electronystagmography.

CONCLUSION: Chances of hearing preservation are better in patients with vestibular schwannoma (VS) from the superior vestibular nerve (SVN) than from the inferior vestibular nerve (IVN). Pathologic posturography and electronystagmography (ENG) findings are more common in schwannomas from the SVN compared with tumors from the IVN. In this way, computerized dynamic platform posturography (CDPP) and ENG contribute to predict the nerve of origin of VS and can be used indirectly as a prognostic factor for hearing preservation. OBJECTIVES: To test whether preoperative CDPP or ENG results contribute to predict the nerve of origin in VS and also to evaluate the influence of the nerve of origin on hearing preservation. METHODS: Eighty-nine patients with VS originating from the IVN and 22 patients with VS from the SVN were included. Hearing loss due to surgery was calculated on postoperative and preoperative audiograms. Caloric response was measured by ENG and condition 5 and 6 score were recorded by CDPP before surgery. RESULTS: Hearing loss due to surgery was significantly lower in patients with tumors from the SVN than from the IVN (p = 0.011). Pathologic results in preoperative ENG (p < 0.0001) and CDPP (p = 0.025) were significantly more frequent in subjects with SVN than with IVN schwannomas.

Artifacts caused by cochlear implants with non-removable magnets in 3T MRI: phantom and cadaveric studies.

The aim of this study was to evaluate artifacts produced by cochlear implants (CI) during 3.0 Tesla (T) magnetic resonance imaging of the brain using different sequences on phantom and cadaveric specimens. A phantom and three cadaveric specimens with CIs were imaged using a 3.0 T clinical scanner. Artifacts were analyzed quantitatively and according to the sequence used. Different brain regions were evaluated for image distortion and limitation of diagnostic significance. In cadaver studies, all sequences generated signal-void areas around the implant. In T2-weighted sequences, additional periodic shadowing was discovered. Anatomical structures of the brain on the contralateral side of the CI were for the most part undistorted. At 3T, artifacts around CIs with non-removable magnets compromise image quality of the nearby brain regions and diagnosis of brain lesions is limited. In the contralateral hemisphere, diagnostic accuracy is only marginally limited.

Demagnetization of cochlear implants and temperature changes in 3.0T MRI environment.

OBJECTIVE: To investigate the level of demagnetization of the magnets and temperature changes in cochlear implants (Cis) in a 3.0 tesla (3.0T) MRI. STUDY DESIGN: Experimental. SUBJECTS AND METHODS: Demagnetization and remagnetization measurements were done on magnets for different types of CIs. Temperature of different body and electrode sides was measured in the MRI environment. RESULTS: Demagnetization of the magnets of the CI is dependent on the angle between the magnetic field of the CI magnet and the MRI. When this angle was greater than 80 degrees, relevant demagnetization occurred and sufficient remagnetization was not possible with the 3.0T MRI magnet. Maximum temperature rise was 0.5 degrees C. CONCLUSIONS: Patients carrying CIs with non-removable magnets should not enter a 3.0T MRI device in a routine clinical setup. Under special conditions (angle between the two magnets less than 80 degrees) imaging in a 3.0T MRI may be possible without harming the patient or the implant.

Intratympanic membrane cholesteatoma after tympanoplasty with the underlay technique.

OBJECTIVE: To evaluate the incidence of intratympanic membrane cholesteatoma (ITMC) in patients after tympanoplasty with the underlay technique. DESIGN: Retrospective study. SETTING: Ear, nose, and throat department, Tabriz University of Medical Sciences, Tabriz, Iran. PATIENTS: A total of 1121 patients with central tympanic membrane perforation were evaluated after tympanoplasty. INTERVENTIONS: Tympanoplasty was performed with the underlay technique using temporal facial graft. MAIN OUTCOME MEASURE: The patients were followed up to assess the postoperative incidence of ITMC. RESULTS: During the follow-up period of 5 years, ITMC was observed in 9 patients (0.8%). Of these 9 patients, 8 were asymptomatic and had intact tympanic membranes. The asymptomatic cases were detected 1 to 2 years after surgery during routine follow-up examinations. Only 1 of 9 patients had otorrhea, which was due to a posterior perforation away from the location of the ITMC. The most common site of the ITMC was near the umbo. CONCLUSIONS: Even after tympanoplasty with underlay technique, ITMC may develop between the layers of the tympanic membrane. The most common location of these cholesteatomas is near the umbo, which may be the result of insufficient removal of the residual squamous epithelium from the handle of the malleus. The cholesteatomas are usually asymptomatic and can be detected during routine follow-up examinations 1 to 2 years after surgery. Although ITMCs are usually noninvasive in nature, a review of the literature revealed that in rare cases they can also show a rapid and invasive growth pattern. Early detection and removal of these asymptomatic cholesteatomas during routine postoperative follow-up examinations can prevent their progression as well as consequent residual problems and complications.

A comparative study of the audiological outcomes with Retro-X (semi-implantable hearing aid system) and conventional open fitting hearing aids.

The objective of this study was to compare the results of hearing augmentation with the Retro-X semi-implantable hearing aid to a conventional non-implantable open canal hearing aid using the same software technology (Titan-X, auric Hearing Systems, HiKaNo.:13.20.03.0047). Nineteen subjects (20 ears) with mild to moderate high-frequency sensory-neural hearing loss were included in this clinical study. All the subjects were first fitted with a conventional open canal hearing aid (Titan-X). After a period of 4-6 weeks audiological evaluations were performed using standardized speech tests in quiet and noise. Subjective evaluation was performed with the help of standardized questionnaires. After this phase the patients received the semi-implantable Retro-X device under local anaesthesia and 4 weeks later were fitted with a hearing module with the same software technology as the conventional Titan-X hearing aid. Four weeks following the first fitting the same audiological evaluations were performed under similar conditions and the patients were evaluated again using the same questionnaires in order to compare the results of the Retro-X semi-implantable system with the conventional open canal hearing aid. Audiological evaluations revealed better results with the semi-implantable Retro-X in the adaptive speech in noise test; however, the results of the monosyllabic word test in quiet were similar for the Retro-X and conventional open canal hearing aid. The patients subjectively preferred the improved wearing comfort of the semi-implantable device. The Retro-X semi-implantable hearing aid provides better speech perception in noise and higher wearing comfort in comparison to the conventional open canal hearing aids. Considering the simple implantation procedure under local anaesthesia with low complication rate, Retro-X is an alternative to the conventional open canal hearing aids in patients with mild to moderate high frequency sensory-neural hearing loss.

A true minimally invasive approach for cochlear implantation: high accuracy in cranial base navigation through flat-panel-based volume computed tomography.

OBJECTIVE: High-precision intraoperative navigation using high-resolution flat-panel volume computed tomography makes feasible the possibility of minimally invasive cochlear implant surgery, including cochleostomy. Conventional cochlear implant surgery is typically performed via mastoidectomy with facial recess to identify and avoid damage to vital anatomic landmarks. To accomplish this procedure via a minimally invasive approach--without performing mastoidectomy--in a precise fashion, image-guided technology is necessary. With such an approach, surgical time and expertise may be reduced, and hearing preservation may be improved. INTERVENTIONS: Flat-panel volume computed tomography was used to scan 4 human temporal bones. A drilling channel was planned preoperatively from the mastoid surface to the round window niche, providing a margin of safety to all functional important structures (e.g., facial nerve, chorda tympani, incus). MAIN OUTCOME MEASURES: Postoperatively, computed tomographic imaging and conventional surgical exploration of the drilled route to the cochlea were performed. RESULTS: All 4 specimens showed a cochleostomy located at the scala tympani anterior inferior to the round window. The chorda tympani was damaged in 1 specimen--this was preoperatively planned as a narrow facial recess was encountered. CONCLUSION: Using flat-panel volume computed tomography for image-guided surgical navigation, we were able to perform minimally invasive cochlear implant surgery defined as a narrow, single-channel mastoidotomy with cochleostomy. Although this finding is preliminary, it is technologically achievable.

Extra- and intracranial dumbbell-shaped hemangiopericytoma.

Hemangiopericytomas are malignant tumors arising from pericytic cells and account for less than 1% of all vascular neoplasms. We report a rare case of an extra- and intracranial dumbbell-shaped hemangiopericytoma originating from the soft tissue of the neck and penetrating the skull base with invasion into the posterior cranial fossa. The 59-year-old female patient presented with a large pulsating neck mass and reported weakness, abnormal fatigue and headache. MRI revealed an inhomogeneously enhancing tumor and cerebral angiography showed intensive vascularization. Preoperative embolization was performed in order to decrease the operative blood loss. The tumor was operated via a far lateral approach through an osteoclastic suboccipital craniotomy. Total resection of both the intra- and extracranial part of the neoplasm (grade I by Simpson) could be achieved. The histopathological analysis revealed a mesenchymal, hypervascular tumor with the classic staghorn vascular pattern. In this article, we discuss the clinical presentation and multidisciplinary management of hemangiopericytoma and describe the radiological and pathological features of this tumor entity.

Electrical stimulation of the midbrain for hearing restoration: insight into the functional organization of the human central auditory system.

The cochlear implant can restore speech perception in patients with sensorineural hearing loss. However, it is ineffective for those without an implantable cochlea or a functional auditory nerve. These patients can be implanted with the auditory brainstem implant (ABI), which stimulates the surface of the cochlear nucleus. Unfortunately, the ABI has achieved limited success in its main patient group [i.e., those with neurofibromatosis type 2 (NF2)] and requires a difficult surgical procedure. These limitations have motivated us to develop a new hearing prosthesis that stimulates the midbrain with a penetrating electrode array. We recently implanted three patients with the auditory midbrain implant (AMI), and it has proven to be safe with minimal movement over time. The AMI provides loudness, pitch, temporal, and directional cues, features that have shown to be important for speech perception and more complex sound processing. Thus far, all three patients obtain enhancements in lip reading capabilities and environmental awareness and some improvements in speech perception comparable with that of NF2 ABI patients. Considering that our midbrain target is more surgically exposable than the cochlear nucleus, this argues for the use of the AMI as an alternative to the ABI. Fortunately, we were able to stimulate different midbrain regions in our patients and investigate the functional organization of the human central auditory system. These findings provide some insight into how we may need to stimulate the midbrain to improve hearing performance with the AMI.

A case of multiple primary tumors of the anterior skull base.

We report a case of synchronous olfactory bulb meningioma and undifferentiated carcinoma of the nose and paranasal sinuses that involved and destroyed the anterior skull base and mimicked intracranial invasion by a carcinoma. The heterogeneity of tissue types in the skull base gives rise to a diverse variety of benign and malignant neoplasms which have totally different prognoses. Synchronous development of benign and malignant primary tumors both originating from and involving the skull base at the same location is very rare and may cause confusion for both the skull base surgeon and neuroradiologist.

Cavernous hemangioma of the internal auditory canal.

Hemangiomas rarely occur in the internal auditory canal. These tumors originate from the capillary bed of the epineurium surrounding the nerve and can either compress or infiltrate the nerve. Depending on location and the nerve of origin, these lesions can cause severe and progressive sensorineural hearing loss, tinnitus, facial nerve palsy, or vertigo even when they are relatively small. The presence of a small contrast-enhancing tumor in the internal auditory canal accompanied by severe sensorineural hearing loss and facial nerve palsy, should raise the suspicion of a hemangioma. Early recognition and surgical intervention in these benign tumors may improve the chance of preserving the functional integrity of the facial nerve and provides better results after nerve reconstruction. Due to their relative small size, the temporal bone CT-scan may show no evidence of pathological widening of the internal auditory canal or the typical intralesional calcifications at the time of presentation. MRI with Gadolinium is the imaging method of choice and a high index of clinical suspicion is necessary for the diagnosis of these tumors. In this paper we report about a 51-year-old male presented with right-sided sensory-neural deafness and facial nerve palsy, accompanied by severe tinnitus and ipsilateral loss of vestibular function due to a cavernous hemangioma in the internal auditory canal.

Auditory midbrain implant: a combined approach for vestibular schwannoma surgery and device implantation.

HYPOTHESIS: The lateral suboccipital approach is a well-established route for safe removal of vestibular schwannomas in neurofibromatosis Type 2 (NF2) patients. The goal of this study was to assess if this approach can be extended to a lateral supracerebellar infratentorial approach to enable insertion of an auditory midbrain implant (AMI) penetrating array along the tonotopic gradient of the inferior colliculus central nucleus (ICC). BACKGROUND: The AMI is a new auditory prosthesis designed for penetrating stimulation of the ICC in patients with neural deafness. The initial candidates are NF2 patients who, because of the growth and/or surgical removal of bilateral acoustic neuromas, develop neural deafness and are unable to benefit from cochlear implants. The ideal surgical approach in NF2 patients must first enable safe removal of vestibular schwannomas and then provide sufficient exposure of the midbrain for AMI implantation. METHODS: This study was performed on formalin-fixed and fresh cadaver specimens. Computed tomography scan and magnetic resonance imaging were used to study the heads of the specimens and for surgical navigation. RESULTS: The lateral suboccipital craniotomy enabled sufficient exposure of the cerebellopontine angle and internal auditory canal for tumor removal. It could then be extended to a lateral supracerebellar infratentorial approach that provided good exposure of the dorsolateral aspect of the tentorial hiatus and mesencephalon for implantation of the AMI along the tonotopic gradient of the ICC. This approach did not endanger the trochlear nerve or any major midline venous structures in the quadrigeminal cistern. CONCLUSION: This modified lateral suboccipital approach ensures safe removal of large vestibular schwannomas and provides sufficient exposure of the inferior colliculus for ideal AMI implantation.

Primary central nervous system lymphoma presenting as bilateral tumors of the internal auditory canal.

The increasing incidence of central nervous system (CNS) lymphoma in the general population, especially in young adults with AIDS, should alert the otolaryngologist that in future there will be an even greater incidence of this rare disease. It may be responsible for hearing loss accompanied by other neurological deficits. CNS lymphoma often has a rapidly progressive course. Early diagnosis and rapid therapy are crucial for a better prognosis. We report a case of primary CNS lymphoma involving both internal auditory canals that presented with sudden deafness and disequilibrium accompanied by facial and abducens nerve palsy.