Speech Perception and Production in Cochlear Implant Recipients with Pendred Syndrome.

AIMS: Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients. METHODS: Ten patients with Pendred syndrome were analyzed for speech perception and production. The control group consisted of 41 non-syndromic implanted patients. All implantees were scored according to speech perception, speech production, and the sum of both. The data were statistically analyzed. RESULTS: No statistical difference was found in language acquisition and production in implantees with Pendred syndrome when compared to non-syndromic patients with cochlear implants. Nor there was any difference in speech production and acquisition between the 2 compared groups regarding surgical age, time elapsed after surgery, or age during the testing. CONCLUSION: In this study evaluating language and speech production and acquisition, patients with Pendred syndrome who underwent cochlear implants show comparable results to their implanted peers with deafness of a different etiology.

Fas-Fas Ligand Interplay in the Periphery of Salivary Gland Carcinomas as a New Checkpoint Predictor for Disease Severity and Immunotherapy Response.

Salivary gland carcinomas (SGCs) are extremely morphologically heterogeneous, and treatment options for this disease are limited. Immunotherapy with immune checkpoint inhibitors (ICIs) represents a revolutionary treatment approach. However, SGCs remain largely resistant to this therapy. An increasing body of evidence suggests that resistance to ICI therapy is modulated by the Fas (CD95)-Fas ligand (FasL, CD178) interplay between tumor cells and immune cells. In this study, we examined the Fas-FasL interplay between tumor cells and tumor-infiltrating immune cells (TIICs) in the center and periphery of SGCs from 62 patients. We found that the Fas-expressing tumor cells accumulated in the center of SGC tumors with increasing tumor stage. Furthermore, this accumulation occurred regardless of the presence of TIICs expressing high levels of FasL. On the contrary, a loss of Fas-expressing TIICs with increasing tumor stage was found in the tumor periphery, whereas FasL expression in tumor cells in the tumor periphery correlated with tumor stage. These data suggest that SGC cells are resistant to FasL-induced apoptosis by TIICs but could utilize FasL to eliminate these cells in high-stage tumors to provide resistance to immunotherapy.

The Periphery of Salivary Gland Carcinoma Tumors Reveals a PD-L1/PD-1 Biomarker Niche for the Evaluation of Disease Severity and Tumor-Immune System Interplay.

The treatment options for patients with advanced salivary gland cancers (SGCs) are limited. Immune checkpoint inhibitors (ICIs) have revolutionized cancer treatment. However, the response to ICI immunotherapy is largely driven by the immune cell signatures within the tumor tissue and the para-tumoral tissue compartments. To date, there are no data on the expression of programed cell death protein-1/programed cell death protein-ligand 1 (PD-1/PD-L1) in SGC, which may enable the implementation of ICI immunotherapy for this disease. Thus, we performed an immunohistochemical analysis of PD-1 and PD-L1 expression in tumor cells and tumor-infiltrating immune cells (TIICs) in the tumor center and periphery of 62 SGC patients. The tumor periphery showed significantly higher expression of PD-L1 in tumor cells than in TIICs. Moreover, peripheral TIICs had significantly higher PD-1 expression than peripheral tumor cells. PD-1-positive tumor cells were detected exclusively in the tumor center of high-grade tumors, and most importantly, the presence of lymph node (LN) metastases and primary tumor stage significantly correlated with the presence of PD-L1-positive tumor cells in the tumor periphery. The PD-1/PD-L1 molecular signatures in SGC are clustered predominantly in the tumor periphery, reflect disease severity, and may predict the response to ICI immunotherapy in SGC patients.

Thromboelastometry as an Alternative Method for Coagulation Assessment in Pediatric Patients Undergoing Invasive Procedures: A Pilot Study.

INTRODUCTION: Standard coagulation tests (activated partial thromboplastin time [aPTT] and prothrombin time [PT]) are used for the assessment of coagulation profile in critically ill pediatric patients undergoing invasive interventions such as insertion of central venous catheter, tonsillectomy, laparotomy, etc. However, these tests do not reflect the profile of whole blood coagulation. Rotational thromboelastometry (ROTEM) as a point of care (POC) viscoelastic test may serve as an alternative method. Due to its ability to assess coagulation profile of the whole blood, it might yield normal results despite prolonged aPTT/PT results. The aim of this study was to find out if there was any severe bleeding during or after invasive procedures if ROTEM test was normal despite prolonged values of aPTT/PT in pediatric patients. MATERIALS AND METHODS: We retrospectively analyzed data for the years 2015 to 2017 for pediatric patients with prolonged values of aPTT or PT and normal ROTEM tests-internal thromboelastometry (INTEM) (assessing internal pathway of coagulation) and external thromboelastometry (EXTEM) (assessing external pathway of coagulation)-and we looked for severe bleeding during or after invasive procedures. RESULTS: In 26 pediatric patients (children from 2 months to 17 years old), we found that INTEM and EXTEM tests showed normal coagulation despite prolonged values of aPTT ratio with a median of 1.47 (minimum 1.04 and maximum 2.05), international normalized ratio with a median of 1.4 (minimum 0.99 and maximum 2.10), and PT ratio with a median of 1.30 (minimum 0.89 and maximum 2.11). In these patients, no severe bleeding was observed during interventions or postoperatively. CONCLUSION: Our data support using thromboelastometry method as an alternative coagulation test for the assessment of coagulation profile in pediatric patients undergoing surgical or other invasive procedures, especially using it as a POC test. All invasive procedures in our study were performed without severe bleeding despite prolonged values of PT/aPTT with normal ROTEM results. It seems that ROTEM assessment of coagulation may lead to decreased administration of fresh frozen plasma and shorten time of patient preparation for intervention.

Cochlear Implantation.

Historically, cochlear implants are the first substitute for a sensory modality, hearing. In past decades, they have become a standard method of a compensation for a hearing loss, both for children and adults. Because of restored hearing by cochlear implants, people were able to keep their jobs, marriages and other social ties. Many children born deaf could attend standard schools from basic education to universities, together with their hearing peers, the fact which had influenced greatly their future lives. Unfortunately, not all suitable candidates for cochlear implants get them. Paradoxically, not due to insufficient financing as before or in undeveloped countries nowadays, but due to lack of a correct information and a lack of interest both in patients and specialists. In our contribution, a principle of the cochlear implant is explained, together with proper indications, modes of application and results of its usage. Because of a limited text span, a history of development of cochlear implants is skipped completely. Nevertheless, we believe that the information proper will reach all those, who could benefit from it.

Application of "banana cochleostomy" and looped electrode insertion for cochlear implantation in children with common cavity malformation and cystic forms of cochlear hypoplasia.

OBJECTIVES: Patients with profound hearing loss due to inner ear malformations may benefit from cochlear implantation; however, the surgery may present a substantial problem for the cochlear implant surgeon due to anatomical variations. The authors describe a new surgical and technical advancement for implantation in patients with small inner ear cavities that make the surgery easier and safer. On the basis of experience involving five consecutive surgeries performed in four patients with inner ear malformations, we present the advantages and application possibilities of the technique. METHODS: The technique does not change the surgical approach in general; however, modification of the cochleostomy shape and looping of the cochlear implant electrode enables safe advancement of the electrode with optimal positioning in the cavity. Additionally, these modifications protect against the insertion of the electrode into the internal auditory canal minimizing the risk of gushing and extracochlear stimulation. RESULTS: The present technique has been used in five cases of cystic implantable inner ear spaces in three independent institutions by different surgeons. It has proven to be a reliable, relatively easy and safe procedure performed with very good anatomic and initially functional effects (positive intraoperative neural response telemetry measurements). CONCLUSIONS: We hope that utilization of the "banana cochleostomy" and insertion of the looped cochlear implant electrode in the implantable cystic spaces of children with malformed inner ears will facilitate and simplify the surgical technique in this difficult procedure and additionally, in revision surgical cases. To our knowledge, the looped insertion and banana-shaped cochleostomy have not been reported previously.

[30 years of the cochlear implantations in the Czech Republic]. / 30 let kochleárních implantací v Ceské republice.

Cochlear implant is the unique sensory neuroprosthesis and still the only one used in clinical praxis. The function of the inner ear is replaced with direct electrical stimulation of the cochlear nerve. It is 30 years since the first cochlear implantation has been performed with the Czech single-channel cochlear neuroprosthesis. There are more than one thousand users of cochlear implants in the Czech Republic nowadays. Cochlear implants have become a standard of care of patients with severe hearing loss. It allows user inclusion to the society with only a minimum of obstacles.Key words: cochlear implant, history, neuroprosthesis, severe hearing loss.

Baha implant as a hearing solution for single-sided deafness after retrosigmoid approach for the vestibular schwannoma: surgical results.

Skull base tumors and, in particular, vestibular schwannoma (VS) are among the etiological reasons for single-sided deafness (SSD). Patients with SSD have problems in understanding speech in a noisy environment and cannot localize the direction of sounds. Baha is one of the most frequently used systems for SSD compensation. Out of 38 patients with SSD after retrosigmoid removal of VS who underwent testing with Baha softband, 16 were satisfied and were indicated for Baha implantation. Two surgical approaches have been used-the Nijmegen linear incision technique with subdermal thinning (Group I, implant BI300) and fast surgery technique without subdermal thinning (Group II, implant BIA400). The duration of the surgery, the implant stability measured by Ostell, and skin or soft tissue reactions in long range follow-up were evaluated and compared between Group I and II. There was a difference in duration of surgery, in Group II procedures averaged significantly faster (p > 0.001). In both groups, there was a similar trend of the gradual increase of implant stability. In the Group I and II, there was comparable rate of the skin or soft tissue reactions grade 0, I, II, or III. We have proved Baha to be a suitable possibility for SSD patients after the removal of VS, regardless of the approach. After the retrosigmoid approach to the VS, the key step of Baha implantation must be to reach intact healthy bone to avoid implantation into scar tissue.

Baha implant as a hearing solution for single-sided deafness after retrosigmoid approach for the vestibular schwannoma: audiological results.

Skull base tumors and, in particular, vestibular schwannoma (VS) are among the etiological reasons for single-sided deafness (SSD). Patients with SSD have problems in understanding speech in a noisy environment and cannot localize the direction of sounds. For the majority, this is the handicap for which they try to find a solution. Apart from CROS hearing aids, Baha is one of the most frequently used systems for SSD compensation. 38 patients with single-sided deafness after retrosigmoid removal of a vestibular schwannoma underwent testing with a Baha softband from September 2010 to August 2014. Sixteen patients (42 %) finally decided to accept Baha implantation. Subjective experience with the Baha softband was evaluated by patients using the BBSS questionnaire immediately after testing. Objective evaluation of the effect was performed as a measurement of the sentence discrimination score in noise and side horizontal discrimination without a Baha and 6 weeks and 12 months after a sound processor fitting. There was a significant improvement in sentence discrimination in the 6 week (64.0 %) and 1 year (74.6 %) interval of follow-up in comparison with understanding without Baha (24.0 %, p = 0.001) in situations when sentences are coming from the side of the non-hearing ear and noise contralaterally with SNR -5 dB. Baha can significantly improve sentence discrimination in complex-listening situation in patients with SSD after the VS surgery.

A case report of a cochlear implant infection - A reason to explant the device?

CLINICAL PRESENTATION: Case history of a paediatric patient with a cochlear implant and a surgical site infection that developed as a result of acute otitis media is presented. INTERVENTION: After conservative management including wound debridement it was decided to explant a functioning device. OBJECTIVE AND IMPORTANCE: In a number of cases, it is necessary to remove the infected albeit functioning device, especially in the event of formation of the biofilm has occurred. It is necessary to review and evaluate the methods with which these major complications are routinely managed with the aim to increase the survival ratio for the implanted device.

Does attempt at hearing preservation microsurgery of vestibular schwannoma affect postoperative tinnitus?

BACKGROUND: The aim of this study was to analyze the effect of vestibular schwannoma microsurgery via the retrosigmoid-transmeatal approach with special reference to the postoperative tinnitus outcome. MATERIAL AND METHODS: A prospective study was performed in 89 consecutive patients with unilateral vestibular schwannoma indicated for microsurgery. Patient and tumor related parameters, pre- and postoperative hearing level, intraoperative findings, and hearing and tinnitus handicap inventory scores were analyzed. RESULTS: Cochlear nerve integrity was achieved in 44% corresponding to preservation of preoperatively serviceable hearing in 47% and useful hearing in 21%. Main prognostic factors of hearing preservation were grade/size of tumor, preoperative hearing level, intraoperative neuromonitoring, tumor consistency, and adhesion to neurovascular structures. Microsurgery led to elimination of tinnitus in 66% but also new-onset of the symptom in 14% of cases. Preservation of useful hearing and neurectomy of the eighth cranial nerve were main prognostic factors of tinnitus elimination. Preservation of cochlear nerve but loss of preoperative hearing emerged as the main factor for tinnitus persistence and new onset tinnitus. Decrease of THI scores was observed postoperatively. CONCLUSIONS: Our results underscore the importance of proper pre- and intraoperative decision making about attempt at hearing preservation versus potential for tinnitus elimination/risk of new onset of tinnitus.

Complications of microsurgery of vestibular schwannoma.

BACKGROUND: The aim of this study was to analyze complications of vestibular schwannoma (VS) microsurgery. MATERIAL AND METHODS: A retrospective study was performed in 333 patients with unilateral vestibular schwannoma indicated for surgical treatment between January 1997 and December 2012. Postoperative complications were assessed immediately after VS surgery as well as during outpatient followup. RESULTS: In all 333 patients microsurgical vestibular schwannoma (Koos grade 1: 12, grade 2: 34, grade 3: 62, and grade 4: 225) removal was performed. The main neurological complication was facial nerve dysfunction. The intermediate and poor function (HB III-VI) was observed in 124 cases (45%) immediately after surgery and in 104 cases (33%) on the last followup. We encountered disordered vestibular compensation in 13%, permanent trigeminal nerve dysfunction in 1%, and transient lower cranial nerves (IX-XI) deficit in 6%. Nonneurological complications included CSF leakage in 63% (lateral/medial variant: 99/1%), headache in 9%, and intracerebral hemorrhage in 5%. We did not encounter any case of meningitis. CONCLUSIONS: Our study demonstrates that despite the benefits of advanced high-tech equipment, refined microsurgical instruments, and highly developed neuroimaging technologies, there are still various and significant complications associated with vestibular schwannomas microsurgery.

A new strategy for the surgical management of RLN infiltrated by well-differentiated thyroid carcinoma.

Well-differentiated thyroid carcinoma (WDTC) represents the most common endocrine malignancy. Despite excellent prognoses exceeding 90% in 10-year follow-up, there are clinically controversial issues. One of these is extrathyroidal tumour extension invading recurrent laryngeal nerve (RLN). The spread outside of the thyroid parenchyma and invasion to the surrounding structures, classified as always T4a, are the most important negative prognostic factor for the WDTC. Conversely, resection of the RLN leads to vocal cord paralysis with hoarseness, possible swallowing problems, and finally decreased quality of life. We propose a new algorithm for intraoperative management based on the MACIS classification, which would allow swift status evaluation pre/intraoperatively and consider a possibility to preserve the infiltrated RLN without compromising an oncological radicality. In the case of a preoperative vocal cord paralysis (VCP) and confirmation of the invasive carcinoma, a resection of the RLN and the nerve graft reconstruction are indicated. Preoperatively, unaffected vocal cord movement and intraoperatively detected RLN infiltration by the invasive WDTC require an individual assessment of the oncological risk by the proposed algorithm. Preservation of the infiltrated RLN is oncologically acceptable only in specific groups of patients of a younger age with a minor size of primary tumour.

Impact of video-endoscopy on the results of retrosigmoid-transmeatal microsurgery of vestibular schwannoma: prospective study.

Endoscopy-assisted microsurgery represents modern trend of treatment of the cerebellopontine angle (CPA) pathologies including vestibular schwannoma (VS). Endoscopes are used in adjunct to microscope to achieve better functional results with less morbidity. Angled optics, magnification and illumination enable superior view in the operative field. Consecutive 89 patients with untreated unilateral sporadic vestibular schwannoma undergoing tumor resection via a retrosigmoid approach during 2008-2010 were prospectively analysed. Endoscopy-assisted microsurgical (EA-MS) removal was performed in 39 cases (Grade 1: 2, Grade 2: 5, Grade 3: 9, Grade 4: 22, Grade 5: 1) and microsurgical (MS) removal was performed in 50 cases (Grade 1: 1, Grade 2: 3, Grade 3: 9, Grade 4: 34, Grade 5: 3). Minimally invasive approach with craniotomy ≤ 2.5 cm was employed for small tumors (Grade 1 and 2) in the EA-MS group. Endoscopic technique was used for monitoring of neuro-vascular anatomy in CPA, during dissection of the meatal portion of tumors, assessment of radicality and for identification of potential pathways for CSF leak formation. All cases in MS group were deemed as radically removed. In the EA-MS group, residual tumor tissue in the fundus of internal auditory canal not observable with microscope was identified with endoscope in four cases. Such cases were radicalized. Tumor recurrence was not observed during the follow-up in EA-MS group. There is a suspicious intrameatal tumor recurrence on the repeated MRI scan in one patient in the MS group. Neither mortality nor infection was observed. The most common complication was pseudomeningocele (EA-MS 20 cases; MS 23). It was managed with aspiration with or without tissue-gluing in all cases without the need for any surgical revision. Adjunctive use of endoscope in the EA-MS group identified potential pathways for CSF leak formation, which was not observable with the microscope in five patients. Improved cochlear nerve (EA-MS: 22, MS: 14; p = 0.012), brainstem auditory evoked potentials (EA-MS: 3 of 8, MS: 0 of 4) and hearing (EA-MS: 14 of 36, MS: 4 of 45; p = 0.001) preservation were observed in EA-MS group. Despite the trend for better useful hearing (Gardner-Robertson class 1 and 2) preservation (EA-MS: 8 of 26, MS: 1 of 16) there were no significant differences in the postoperative hearing handicap inventory in both groups. There were no differences in the postoperative tinnitus in both groups. Better facial nerve preservation (EA-MS: 39, MS: 44; p = 0.027) and excellent-very good (House-Brackmann 1 or 2) facial nerve function (EA-MS: 31, MS: 29; p = 0.035) were observed in EA-MS group. Postoperative compensation of vestibular lesion, symptoms typical for VS, patients assessed by dizziness handicap inventory, facial disability index were comparable in both studied groups. Adjunctive use of endoscope during the VS surgery due to its magnification and illumination enable superior view in the operative field. It is valuable for assessment of radicality of resection in the region of internal auditory meatus. Improved information about critical structures and tumor itself helps the surgeon to preserve facial nerve and in selected cases also hearing. These techniques can help to decrease incidence of postoperative complications.