Comparison of Clinician Versus Parental Perspectives of Outcomes in Cochlear Implantees: A South Indian Experience

Outcomes of cochlear implantation (CI) are generically assessed using standard validated measures like CAP, SIR, MAIS and MUSS scales. Although this reflects the improvement in auditory verbal skills among the implantees with habilitation over one year, the overall perception of their skill development may vary between the parents of these children and the clinicians who provide the habilitation. This study aimed to compare the CAP and SIR scores sequentially over habilitation and further analyzes the correlation between clinician assessment (with CAP/SIR scores) and parental perspective (with MAIS/MUSS scores), at the end of one year of habilitation. 388 children aged 1–6 years who underwent unilateral CI were included in the study. Their baseline CAP and SIR scores were recorded post implantation. All children received 1 year of intensive auditory verbal therapy and their 12 month CAP, SIR, MAIS and MUSS scores were then recorded. The baseline CAP/SIR scores were compared with 12 month CAP/SIR scores and then their 12 month CAP/SIR scores were correlated with 12 month MAIS/MUSS scores respectively. There was significant difference between baseline and the 12 month CAP/SIR scores (p < 0.001). There was strong positive correlation between CAP and SIR scores after 12 months of habilitation (r = 0.7), while there was moderate positive correlation between CAP and MAIS scores (r = 0.59) and between SIR and MUSS scores (r = 0.49) respectively. Though the parents note significant improvement in child’s communication abilities, the parental perspective of final outcomes does not always match with the clinician’s assessments at the end of habilitation, as highlighted by the moderate correlations. A more precise method of holistic assessment is lacking currently and stands warranted.     

A Retrospective Clinical Audit of Outcomes of Cochlear Implantation in Children with Multiple Disabilities in Comparison with Normal Implantees: A South Indian Experience

AimsCochlear implantation (CI) is established as a standard remedy for children with congenital bilateral profound hearing loss to attain hearing perception and thereby develop speech and language. A subgroup includes children with multiple disabilities in whom the implant helps to improve their quality of life and also of their families via enhanced communication skills. Cochlear implants today form an integral part of their multi-handicap rehabilitation process.Material and MethodsA retrospective cohort study was carried out on children with and without multiple handicaps who have received cochlear implantation at the cochlear implant clinic of MERF, Chennai, India over the past decade. Category of Auditory Performance (CAP) scores, Speech Intelligibility Rating (SIR) scores, and also Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) scores were compared at set time frequencies of 6 months and 12 months post-implantation between the two groups of implanted children.ResultsAll the four CAP, SIR, MAIS and MUSS scores showed improvement over time with auditory and speech therapy in both groups of children as reflected by the improvement in their quality of life. The normative group of implantees showed better improvement compared to the group of children with multiple disabilities.ConclusionIntensive habilitation is essential especially for children with multiple disabilities who have received cochlear implantation in which their special needs are addressed individually and optimised for the best outcome. The study shows that restoration of the special sense of hearing helps as a remedy to alleviate their other multi-handicaps to a notable extent.     

Speech Recognition Performance in Children with Cochlear Implants Using Bimodal Stimulation

Cochlear implantees have considerably good speech understanding abilities in quiet surroundings. But, ambient noise poses significant difficulties in understanding speech for these individuals. Bimodal stimulation is still not used by many Indian implantees in spite of reports that bimodal stimulation is beneficial for speech understanding in noise as compared to cochlear implant alone and also prevents auditory deprivation in the un-implanted ear. The aim of the study is to evaluate the benefits of bimodal stimulation in children in an Indian cochlear implant clinic. A group of 14 children who have been using cochlear implants served as subjects in this study. They were fitted with advanced digital hearing aids in their un-implanted ears to provide bimodal stimulation. Results revealed that bimodal stimulation did not bring greater change in speech scores in quiet surroundings but have shown a noticeable improvement in noisy ambience. Hence the present study suggests that bimodal stimulation would benefit children with cochlear implants especially in adverse listening conditions.     

Abschätzung der Hörschwelle mit stationären auditorischen evozierten Potentialen: Probleme der digitalen Signalverarbeitung

The evaluation of hearing thresholds in human subjects using steady-state auditory evoked potentials (AEP) requires an appropriate test procedure to detect the response at poor signal to noise ratios. Differences in signal processing between transient and steady-state AEP are discussed and optimal conditions for recording of steady-state AEP in the frequency domain are established. 40-Hz-Click-AEP (5 subjects) und 40 Hz-AMFR (Amplitude Modulation Following Response, 11 subjects) were recorded in normal hearing subjects at stimulus levels ranging from 10 to 80 dB (nHL). An estimate of residual background noise for a narrow frequency band (30 … 50 Hz) was plotted as a function of the number of signal periods analysed. Differences in response amplitudes and signal to noise ratios between 40-Hz-Click-AEP and 40 Hz-AMFR were discussed.     

Magnet migration in a cochlear implantee — a serendipitous diagnosis

The successful outcome of a cochlear implant habilitation program depends upon a multitude of vital factors, including the avid cooperation of the cochlear implantee, committed participation of the parents/family members and total dedication of the habilitation team of implant audiologists and auditory verbal therapists. In a rare situation, where the implantee’s performance is suboptimal or poor inspite of successful implantation, anxious moments engulf the parents and the implant team, with the morbid fear of a device failure lingering at the back of their minds. We report such an incident in a 13-year-old cochlear implantee, who was an excellent cochlear implant performer for 8 years, following which she had rapid deterioration of her auditory verbal skills within the next few weeks. The hidden etiology, was the inconspicuous migration of the internal magnet of the Receiver-Stimulator Coil placed in the mastoid temporal bone of her skull, due to unexplained reasons. We share our experience with the diagnosis and management of this condition and review the existing world literature on this rarely reported entity.     

Neurofibromatosis 2: Primary Modality of Hearing Rehabilitation with Cochlear Implant

Auditory Brainstem Implants have been recommended as the gold standard in hearing rehabilitation of Neurofibromatosis Type 2 patients who lose hearing completely in both the ears and the cochlear nerves are not stimulable. Some patients have undergone cochlear implantation, in whom the cochlear nerve was spared during surgery or have undergone stereotactic radiotherapy preserving the function of the cochlear nerve. Here we report a case in whom we chose cochlear implantation prior to any definitive treatment for the tumour itself during the ‘wait and watch’ period. The reasons in favour of this approach have been discussed in this article. Post switch-on the implant is benefitting the patient satisfactorily and she is on regular follow up for monitoring the bilateral tumours.     

New SMS Classification of Cochleovestibular Anomalies: Our Experience with 25 Cases of Type I Anomaly

PurposeTo study and analyse the radiological and surgical findings of 25 cochlear implantees with SMS type—I cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and post-operatively.MethodsRetrospective analysis of cochlear implanted candidates over a period of 8 year from 3 institutions was undertaken and 25 patients suffering from pre-lingual profound congenital sensori-neural hearing loss along with presence of SMS type I cochleovestibular malformation were studied. Pre-operative radiology, surgical difficulties and complication, and post-operative hearing and speech outcomes upto a period of 2 years, using IT-MAIS scores were noted. Statistical comparison pre- and post-implantation was done using Wilcoxon signed rank test and a p-value of < 0.05 was considered significant.ResultsIn 25 cases, we encountered 2 posterior SCC dysplasia, 15 enlarged vestibular aqueducts (LVA), 2 dilated vestibules, 4 superior SCC dysplasia, 2 cases with both posterior and superior SCC dysplasia and of those 1 had a dilated vestibule also. Intra-operatively, CSF leak was observed in 10 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS scores increased from a pre-operative mean of 4.32 to 34.56 two years post-operatively and this difference was found to be statistically significant.ConclusionOur experience with SMS Type I malformations show promising and motivating results with less chances of complications, similar to implant candidates with normal anatomy. The outcomes in this group of patients are definitely not affected by any malformations in semicircular canals, vestibule or vestibular aqueduct.     

Excitation characteristic of a bipolar stimulus for broadband stimulation in measurements of electrically evoked auditory potentials

Objective

The aim of this study was to determine the optimum electrical stimulation mode for the measurement of electrically evoked potentials. To quantify the broadband excitation electrically evoked compound action potentials were recorded in cochlear implant recipients. The full width half maximum was determined for spread of excitation along the electrode array.

Electrode Translocations in Perimodiolar Cochlear Implant Electrodes: Audiological and Electrophysiological Outcome

IntroductionDespite using the soft-surgery technique, cochlear implantation may increase the damage of the intracochlear structures due to a scalar translocation of the electrode. The aim of this work was to investigate the incidence as well as the audiological and electrophysiological outcome for electrode translocations and complete scala tympani insertions of perimodiolar electrodes within a large group of patients.Material and methodsThe investigations were performed retrospectively on 255 adult subjects with a Nucleus Contour Advance or Slim Modiolar electrode (Cochlear Ltd.). The scalar position was assessed by postoperative rotational tomography. Intraoperative and one year after CI activation measured ECAP thresholds were examined as well as the postoperative speech recognition in quiet using the Freiburg monosyllable word test.ResultsThe incidence of a translocation was significantly lower with the Slim Modiolar than with the Contour Advance electrode (5.1% versus 32.3%; p< 0.05). With a scala tympani placement the median speech recognition score was 75% (range: 20- 100%) with the Contour Advance and 72.5% (range: 27.5-95%) with the Slim Modiolar electrode. In cases with an electrode translocation, speech recognition scores show a median of 75% (range: 45-100%) and 73.8% (range: 40-80%), respectively. No significant differences in speech recognition were found between translocations and scala tympani insertions with both electrodes. Compared to scala tympani insertions, electrode translocations yielded higher ECAP thresholds at apical and medial electrode contacts (p< 0.05).ConclusionThe incidence of an electrode translocation is determined for both perimodiolar electrode types analyzed in this work. ECAP measurements provide additional information for detecting translocations compared to radiological imaging. However, the postoperative speech recognition in quiet was not affected by the scalar position in the electrodes examined here.     

Notes for developing a molecular test for the full characterization of circulating tumor cells

The proved association between the circulating tumor cell (CTC) levels and the patients’ survival parameters has been growing interest to investigate the molecular profile of these neoplastic cells among which hide out precursors capable of initiating a new distant metastatic lesion. The full characterization of the tumor cells in peripheral blood of cancer patients is expected to be of help for understanding and (prospectively) for counteracting the metastatic process. The major hitch that is hampering the successful gaining of this result is the lack of a consensus onto standard operating procedures (SOPs) for performing what we generally define as the “liquid biopsy”. Here we review the more recent acquisitions in the analysis of CTCs and tumor related nucleic acids, looking to the main open questions that are hampering their definitive employ in the routine clinical practice.     

Early Speech Perception Test Outcome in Children with Severe Sensorineural Hearing Loss with Unilateral Cochlear Implants Alone versus Bimodal Stimulation

Bilateral stimulation of the auditory system has clear advantages over unilateral hearing. Hearing-impaired children are, therefore, generally fitted with hearing aids in both ears so that they can have the benefits of binaural hearing. Children who use acochlear implant in one ear and no acoustic stimulation in the opposite ear are at a definite disadvantage. This study was undertaken to determine the advantages of bimodal stimulation in pediatric population especially in terms of speech recognition. This study comprised of 30 children between 3 and 6 years of age with profound bilateral sensorineural hearing loss with cochlear implant in one ear and fitted with digital hearing aid in non-implanted ear. Speech recognition performance was compared in unilateral cochlear implant only and with bimodal hearing stimulation in the same set of children. A statistically significant difference was found between speech reception scores in children with a unilateral cochlear implant only and those with a cochlear implant in one ear and a hearing aid in the non implanted ear in quiet surroundings. It is suggested that the use of bimodal fitting be considered as an effective management method to obtain the advantage of binaural hearing in children who undergo unilateral cochlear implantation.     

MiR-449a suppresses cell invasion by inhibiting MAP2K1 in non-small cell lung cancer

Increasing evidence reveals that deregulation of miRNAs contributes to carcinogenesis of the human non-small cell lung cancer (NSCLC). Our study discovered that the expression of miR-449a was markedly decreased in NSCLC cells with high metastatic capacity and tissues of positive lymph node metastasis. Moreover, our results showed that miR-449a could act as a tumor suppressor by inhibiting the invasion of NSCLC cells in vitro and in vivo. Mechanistically, miR-449a inhibited the expression of MAP2K1 by direct targeting its 3’UTR, and regulated the activity of MEK1/ERK1/2/c-Jun pathway through an auto-regulatory feedback loop. Furthermore, the histone methylation mediated the decreased expression of miR-449a through SUZ12. Taken together, the novel connection between miR-449a and MAP2K1 demonstrated here provided a new, potential therapeutic target for the treatment of non-small cell lung cancer.     

乳腺癌改良根治术中瑞芬太尼复合丙泊酚全凭静脉麻醉方法的探讨

目的：在听觉诱发电位指导下探讨瑞芬太尼复合丙泊酚全凭静脉麻醉在乳腺癌改良根治术麻醉维持与恢复质量。方法：选择ASAⅠ～Ⅱ级择期行乳腺癌改良根治术的患者60例,随机分为两组。试验组（A组）,通过监测听觉诱发电位指数（AAI）调节丙泊酚输注速率,维持AAI〈30;对照组（B组）,根据血压及心率调节丙泊酚输注速率（收缩压或心率上升及下降幅度超过基础值的20％时）,每5min增减丙泊酚0．01mg／（kg·min）。诱导时两组均以5ng／ml血浆靶浓度TCI瑞芬太尼开始,术中瑞芬太尼血药靶浓度均维持不变。记录基础值（T1）、插入喉罩时（1、2）、切皮（1、3）、分离深部组织（T4）、术毕（T5）、睁眼（T6）6个时间点的MAP、HR、AAI值及T2～T5时间点的丙泊酚给药速率和均值。记录患者睁眼的时间、定向力恢复时间、出手术室时间。结果：两组各时间点AAI差异有统计学意义（P〈0．05）,与B组相比,A组输注丙？白酚速率以及总用量减少（P〈0．05）。在睁眼和定向力恢复时间方面,B组明显高于A组（P〈0．05）。术后24h随访患者均无术中知晓。结论：AAI指导全凭静脉麻醉输注瑞芬太尼复合丙泊酚用于乳腺癌改良根治术患者,可较好的控制麻醉深度,指导合理用药,有助于患者较快地恢复,避免术中知晓。     

Correlation of PET standard uptake value and CT window-level thresholds for target delineation in CT-based radiation treatment planning

PURPOSE: To develop standardized correlates of [18F]fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) standard uptake value (SUV) to computed tomography (CT)-based window and levels. METHODS AND MATERIALS: Nineteen patients with non-small-cell lung cancer who underwent imaging with positron emission tomography (PET) and CT were selected. A method of standardizing SUV within CT planning software was developed. A scale factor, determined by a sensitivity calibration of the PET scanner, converts voxel counts to activity per gram in tissue, allowing SUVs to be correlated to CT window and levels. A method of limiting interobserver variations was devised to enhance "edges" of regions of interest based on SUV thresholds. The difference in gross tumor volumes (GTVs) based on CT, PET SUV >or= 2.5, and regions of 40% maximum SUV were analyzed. RESULTS: The mean SUV was 9.3. Mean GTV volumes were 253 cc for CT, 221 cc for SUV >or= 2.5, and 97 cc for SUV40%Max. Average volume difference was -259% between >or=2.5 SUV and CT and -162% between SUV40%Max and CT. Percent difference between GTV >or= 2.5 SUV and SUV40%Max remained constant beyond SUV > 7. For SUVs 4-6, best correlation among SUV thresholds occurred at volumes near 90 cc. Mean percent change from GTVs contoured according to CT (GTV CT) was -260% for GTV2.5 and -162% for GTV40%Max. Using the SUV40%Max threshold resulted in a significant alteration of volume in 98% of patients, while the SUV2.5 threshold resulted in an alteration of volume in 58% of patients. CONCLUSIONS: Our method of correlating SUV to W/L thresholds permits accurate displaying of SUV in coregistered PET/CT studies. The optimal SUV thresholds to contour GTV depend on maximum tumor SUV and volume. Best correlation occurs with SUVs >6 and small volumes <100 cc. At SUVs >7, differences between the SUV threshold filters remain constant. Because of variability in volumes obtained by using SUV40%Max, we recommend using SUV >or= 2.5 for radiotherapy planning in non-small-cell lung cancer.     

Measurement of implant stability and auditory pure-tone thresholds of Baha patients comparing osteosysthesis and osseointegrated systems

With bone-anchored hearing systems the implant-bone junction is critical for the transmission of mechanical vibrations to the skull. The implant stability might differ between available and widely applied implants and can be estimated by resonance frequency analysis.The implant stability and the audiological performance of ten adult long-time users where a bone-anchored hearing system was connected by an Baha osseointegrated implant (Cochlear Ltd, Mölnlycke, Sweden) were compared with the implant stability of fourteen adult patients provided with a Ti-epiplating osteosysthesis system (Medicon, Tuttlingen, Germany). The acute implant stability was compared between these two implants with one cadaveric skull.The results show higher resonance frequencies and thus higher implant stability for the osseointegrating system of both, measuring acute and after long-time use of the implants. The audiological outcomes show frequency dependent differences between both systems as determined by comparing pure-tone bone-conduction audiometry. However, measuring thresholds by a direct stimulation of the implant reveals better hearing with low frequencies for the osseointegrated system and for high frequencies with the osteosysthesis system. At all, the implant selection has an impact on the implant stability and on the pure-tone hearing.     

Oral high-dose medroxyprogesterone acetate (MAP) in treatment of advanced breast cancer

Summary Medroxyprogesterone acetate (MAP) in the treatment of advanced breast cancer has been regarded as a minor agent according to the previous reports when used at low doses (<500 mg/day). High doses of more than 500 mg which have come into use since 1973 give a response rate of over 40%, but sometimes cause gluteal abcess or induration because of daily intramuscular injections.In order to administer easily and to avoid the side effect, we have attempted to use oral administration in a daily dose of 1200mg (400 mg × 3). Of those 20 patients treated with oral high-dose MAP, 1 showed complete response, 6 showed partial response, 7 no change, and 6 progressive disease. As for site of lesion, 4 out of 6 (67%) in skin and 4 out of 16 (25%) in bone responded. Neither severe side effects nor abnormal laboratory data were seen.Then, we examined the blood levels of MAP in vivo by RIA in 9 patients. The blood level of MAP reached 39–250 ng/ml in 3 days and was maintained at least over 50 ng/ml for 1 or 2 months of continuous administration. Subsequently, we examined the effects of MAP on binding to ER in vitro. The inhibition of binding of estradiol-17 to ER was about 60% at 10^–5 M MAP. The blood level of 50 ng/ml in vivo corresponds to about 1.3 × 10^–5 M. Address for reprints: Dr. Masaru Izuo, Department of Surgery, Gunma University School of Medicine, Maebashi, Japan     

Psychometric Properties of the Thai Version of Supportive Care Needs Survey-Partners and Caregivers (T- SCNS-P&C) for Cholangiocarcinoma Caregivers

Background:Unmet needs of cancer patients and caregivers are also closely linked with patients and their family well-being. Identifying and focusing on caregivers’ unmet need have been recommended to reduce their burden and improve their quality of life (QOL). This study aimed to evaluate the psychometric properties of the Thai version of Supportive Care Needs Survey for Partners and Caregivers (T-SCNS-P&C) among Cholangiocarcinoma (CCA) caregivers. Methods:The T-SCNS-P&C was developed using standardized translation methodology. A total of 231 CCA caregivers completed the T- SCNS-P&C. The internal consistency of the scale was examined with Cronbach’s alpha. Construct validity was analyzed using Pearson correlations coefficient with the physical effects, stage, anxiety and depression, age, and education level. To assess the factorial validity of the T-SCNS-P&C, confirmatory factor analysis (CFA) was performed. Results:The T- SCNS-P&C indicated good readability and high content validity for use as an assessment tool among Thai CCA caregivers. All Cronbach’s α coefficients were above the minimum acceptable criterion of ≥0.70. For construct validity, higher physical effect scores and higher anxiety and depression scores, as well as poorer QOL scores and younger caregivers, were significantly positively associated with higher levels of unmet needs. CFA indicated that the four factor structure of the T-SCNS-P&C was a good fit to the data.Conclusion: The T-SCNS-P&C demonstrated acceptable reliability and validity for assessing unmet needs among CCA caregivers in Thailand. Using this simple assessment to target the individual needs of these caregivers can help healthcare professionals providing effective personalized care.Key Words: Caregivers, Thai, psychometric validation, supportive care, unmet needs     

A Case Series of Metastatic Malignant Gastrointestinal Neuroectodermal Tumors and Comprehensive Genomic Profiling Analysis of 20 Cases

Malignant gastrointestinal neuroectodermal tumor (GNET) is an ultra-rare soft tissue sarcoma, therefore often misdiagnosed and has no available standard treatment. Here, we report 3 cases of metastatic GNET with variable clinical courses. Our small case series as well as extensive literature review, further support that GNET is a spectrum of diseases with variable inherent biology and prognosis. Surgical management in the setting of recurrent/metastatic disease may be appropriate for GNET with indolent nature. Response to systemic treatments including chemotherapy and targeted treatments is variable, likely related to heterogenous biology as well. Furthermore, we retrospectively identified 20 additional GNET cases from Foundation Medicine’s genomic database and expanded on their clinicopathological and genomic features. Comprehensive genomic profiling (CGP) with DNA and RNA sequencing of this cohort, in the course of clinical care, demonstrated recurrent EWSR1 chromosomal rearrangements and a sparsity of additional recurrent or driver genomic alterations. All cases had low tumor mutational burden (TMB) and were microsatellite stable.