[Impact of CI use and CI fitting on speech production in very early cochlear-implanted infants]. / Bedeutung von CI-Nutzungsverhalten und CI-Anpassung für sprachproduktive Leistungen sehr früh cochleaimplantierter Kinder.

BACKGROUND: Children's age at implantation is an important factor for their outcome in auditory and language skills with a cochlear implant (CI). CI use and frequency of CI fitting may also influence speech performance. Purpose of this study was to evaluate CI use and CI fitting of very early implanted infants and its potential influence on age-related speech production performance. METHODS: Data of 34 bilaterally cochlea-implanted infants (age at CI in months: M = 8,8; SD = 1,7) were included. During the third year of life speech production performance was evaluated and related to datalogging-based CI use and number of CI fitting sessions. RESULTS: About half of the cohort achieved speech production level within the normal range of hearing peers. Daily time of CI use was approximately 8 h. Analysis of listening environment showed that infants were exposed most of the time to quiet environment and least amount of time to speech in noise. Daily time of CI use seems to be a significant predictor of speech production, speech-exposition particularly predicts word production. Number of daily disconnection between CI-processor and implant as well as the monthly number of CI fitting sessions were not correlated with speech production. CONCLUSION: Very early cochlear implanted infants may achieve age-appropriate speech production performance in the third year of life. Time of daily CI use in the study cohort is comparable to results of other studies. Time of daily CI use and exposure to speech seem to be important factors for early speech production. These findings should be integrated in pre- and postoperative parent counselling.

Speech recognition with hearing aids for 10 standard audiograms : English version.

BACKGROUND: Improvement of speech perception in quiet is an important goal of hearing aid provision. In practice, results are highly variable. The aim of this study was to investigate the relationship between type and extent of hearing loss (audiogram type), maximum word recognition score, and aided speech perception. MATERIALS AND METHODS: Pure tone and speech audiometric data of 740 ears in 370 patients were reviewed. All subjects visited our hearing center for hearing aid evaluation between 2012 and 2017. The maximum word recognition score (WRSmax) and the monosyllabic speech recognition score with hearing aids, WRS65(HA) were analyzed for 10 different standard audiogram types. RESULTS: The WRS65(HA) with hearing aids for different degrees of hearing loss is, within error boundaries, comparable to previous investigations and shows a difference of 10-20 percentage points to the WRSmax. This difference tends to be larger for flat and moderately sloping audiograms compared to steep-sloping audiograms. The ratio WRS65(HA)/WRSmax can be interpreted as an efficiency factor for hearing aid provision, since it relates speech recognition with hearing aids to the maximally achievable information carrying capacity of the hearing impaired. CONCLUSION: The expectation regarding hearing aid provision has to be adjusted according to maximum word recognition score, the derived quality measures, degree of hearing loss, and audiogram type.

[Speech recognition with hearing aids for 10 standard audiograms]. / Sprachverstehen mit Hörgeräten für 10 Standardaudiogramme.

BACKGROUND: Improvement of speech perception in quiet is an important goal of hearing aid provision. In practice, results are highly variable. The aim of this study was to investigate the relationship between type and extent of hearing loss (audiogram type), maximum word recognition score, and aided speech perception. MATERIALS AND METHODS: Pure tone and speech audiometric data of 740 ears in 370 patients were reviewed. All subjects visited our hearing center for hearing aid evaluation between 2012 and 2017. The maximum word recognition score (WRSmax) and the monosyllabic speech recognition score with hearing aids, WRS65(HA) were analyzed for 10 different standard audiogram types. RESULTS: The WRS65(HA) with hearing aids for different degrees of hearing loss is, within error boundaries, comparable to previous investigations and shows a difference of 10-20 percentage points to the WRSmax. This difference tends to be larger for flat and moderately sloping audiograms compared to steep-sloping audiograms. The ratio WRS65(HA)/WRSmax can be interpreted as an efficiency factor for hearing aid provision, since it relates speech recognition with hearing aids to the maximally achievable information carrying capacity of the hearing impaired. CONCLUSION: The expectation regarding hearing aid provision has to be adjusted according to maximum word recognition score, the derived quality measures, degree of hearing loss, and audiogram type.

[Auditory group therapy in adult cochlear implant rehabilitation]. / Hörtherapeutische Gruppenkonzepte in der Cochleaimplantat-Rehabilitation bei Erwachsenen.

BACKGROUND: Auditory training is an established intervention in adult cochlear implant (CI) aural rehabilitation. In most cases, training is implemented in an individual therapy setting. Increasing patient numbers and the associated time-economic and cost-related demands as well as psychosocial and communicative aspects support the use of aural group interventions. OBJECTIVES: This study aimed 1) to describe concepts and contents of group interventions for adult CI users and 2) to present results of a questionnaire-based evaluation. METHODS: Group interventions have been offered at the CI Centre Erlangen CICERO for several years. In Auditory Training Groups, exercises have priority, while Thematic Group Workshops focus on psychosocial aspects and provide information for the participants. The Auditory Training Groups were evaluated based on a patient questionnaire. Additionally, the reliability of the questionnaire was analyzed. RESULTS: The median overall satisfaction of CI users participating in Auditory Training Groups was rated as good. Training of speech perception in noise as well as communicative exchange are of great importance for CI users. They rated the therapeutic design as very good to good. Reliability analysis showed significant positive intercorrelations of the questionnaire items. CONCLUSION: Group interventions are well accepted by CI users and represent a useful complement to individual therapy during the rehabilitation process. As group interventions place special demands on patients and therapists, structured and well-proven concepts should be used in practice.

[Maximum monosyllabic score as a predictor for cochlear implant outcome]. / Das maximale Einsilberverstehen als Prädiktor für das Sprachverstehen mit Cochleaimplantat.

OBJECTIVE: This study investigated the speech perception of cochlear implant (CI) recipients with measurable preoperative ipsilateral speech perception. These data should support improved individual counselling of CI candidates. MATERIALS AND METHODS: Pre- and postoperative speech audiometric parameters were analyzed, including maximum score for phonemically balanced words (PBmax) and monosyllabic score at a normal conversational level of 65 dBSPL, with hearing aids one hand and CI on the other. Data of 284 experienced adult CI wearers were grouped and evaluated in terms of preoperative PBmax. RESULTS: The preoperative PBmax was exceeded by the postoperative monosyllabic score in 96% of cases. The overall median postoperative score was 72.5%. The groups with preoperative PBmax > 0% showed significantly better speech perception scores with CI than the group with PBmax = 0 %. Median improvement compared to the preoperative monosyllabic score with hearing aids was 65 percentage points, independent of preoperative PBmax. CONCLUSION: The preoperatively measured PBmax may be used as a predictor for the minimum speech perception obtained with CI. This is of high clinical relevance for CI candidates with a PBmax above zero.

Control of Fundamental Frequency in Dysphonic Patients During Phonation and Speech.

PURPOSE: The pitch-shift reflex (PSR) is the adaptation of the fundamental frequency during phonation and speech and describes the auditory feedback control. Speakers without voice and speech disorders mostly show a compensation of the pitch change in the auditory feedback and adapt their fundamental frequency to the opposite direction. Dysphonic patients often display problems with the auditory perception and control of their voice during therapy. Our study focuses on the auditory and kinesthetic control mechanisms of patients with muscle tension dysphonia (MTD) and speakers without voice and speech problems. Main purpose of the study is the analysis of the functionality of the control mechanisms within phonation and speech between patients with MTD and normal speakers. METHOD: Sixty-one healthy subjects (17 male, 44 female) and 22 patients with MTD (7 male, 15 female) participated following two paradigms including a sustained phonation (vowel /a/) and speech (['mama]). Within both paradigms the fundamental frequency of the auditory feedback was increased synthetically. For the analysis of the PSR the electroencephalogram, electroglottography, the voice signal, and the high-speed endoscopy data were recorded simultaneously. The PSR in the electroencephalogram was detected via the N100 and the mismatch negativity. Statistical tests were applied for the detection of the PSR in the physiological response within the electroglottography, voice, and high-speed endoscopy signals. The results were compared between both groups. RESULTS: No differences were found between the controls and patients with MTD regarding latency and magnitude of the perception of the pitch shift in both paradigms, but for the magnitude of the behavioral response. Differences also could be found for both groups between the "no pitch" and "pitch" condition of the two paradigms regarding vocal fold dynamics and voice quality. Patients with MTD showed more vibrational irregularities during the PSR than the controls, especially regarding the symmetry of vocal fold dynamics. CONCLUSION: Patients with MTD seem to have a disturbed interaction between the auditory and kinesthetic feedback inducing the execution of an overriding behavioral response.

[Postoperative legal disputes involving patients with cochlear implants]. / Postoperative Rechtsstreitigkeiten bei Trägern von Cochleaimplantaten.

BACKGROUND: After the implantation of one or two cochlear implants additional problems often arise concerning the question which further costs have to be covered by statutory insurances or other insurance providers, e.g. within the framework of integration aid. OBJECTIVE: This article provides an overview of judgments rendered by the German social courts. It was investigated whether and in which cases it is advisable for a patient to go to court, and how long the proceedings may take. MATERIAL AND METHODS: A search was made for judgments in the two biggest commercial legal databases and in the database of the German social courts, using combinations of the search parameters "Cochlear", "Cochlea", "Implant", and "Implantat". The reviewed judgments were issued between 2002 and 2017. RESULTS: A total of 13 judgments were found. The results varied according to the specifics of the individual case and 54% of the patients won their cases. The court procedures took between 2 months (starting from the filing of a complaint, date of application in this case unknown), and 6 years and 11 months. CONCLUSION: This study showed that it is uncertain whether costs that are not caused directly by the surgery or the postoperative treatment will be remunerated. As each case is in principle unique, the results vary. Nevertheless, taking legal action is recommended after thorough consideration in cases where statutory insurances or other providers refuse to meet expenses incurred.

[Subjective improvement of hearing through cochlear implantation]. / Subjektive Hörverbesserung durch Cochleaimplantatversorgung.

BACKGROUND: During the past few decades various questionnaires have been developed to evaluate quality of life using audiological measures. The Hearing Implant Sound Quality Index (HISQUI) was developed especially for patients with a cochlear implant (CI). The aim of this study was to evaluate subjective hearing improvement after CI surgery and to correlate the results with objective measuring methods such as pure-tone and speech audiometry. MATERIALS AND METHODS: Pre- and postoperative data of 57 patients who received a cochlear implant (CIs) were retrospectively analyzed. For evaluation of subjective hearing ability the HISQUI questionnaire consisting of 19 questions was used as reference. Pure-tone audiometry and Freiburger monosyllable word tests were used as objective measurements for hearing. RESULTS: The mean HISQUI score increased on average from 53.0 prior to surgery to 69.0 after surgery. In addition, there were significant improvements in all seven subgroups of the questionnaire divisions. A correlation of rSp = 0.53 before surgery and rSp = 0.36 after surgery between the HISQUI scores and Freiburg monosyllable word test was recorded. In addition the 4FPTA (four frequency pure tone average) value correlated negatively with rSp = -0.48 with the HISQUI total score before surgery. CONCLUSION: The moderate correlation of both subjective and objective measurement methods underlines the importance of enquiring the patients' subjective perception and satisfaction. Through the combination of subjective statements and measurements of speech comprehension it is possible to obtain a comprehensive picture of the hearing performance of CI recipients.

[Cochlear implants in the social courts]. / Das Cochleaimplantat in der sozialgerichtlichen Rechtsprechung.

BACKGROUND: Since the indication for receiving a cochlear implant (CI) has widened (single-sided deafness [SSD], electric acoustic stimulation [EAS], bilateral CI, CI for long-term deafness), more and more patients come into consideration for such a treatment. Hence, disputes increasingly arise between patients and their insurance companies concerning the question of whether surgery and follow-up treatment have to be paid for by statutory health insurance. OBJECTIVE: This work provides an overview of judgments rendered by the German social courts. We investigated whether and in which cases it is advisable for a patient to go to court, and how long the proceedings may take. MATERIALS AND METHODS: We looked for judgments in the two biggest commercial legal databases and in the database of the German social courts, using combinations of the search parameters "Cochlear," "Cochlea," "Implant," and "Implantat." Three verdicts were attained by directly contacting the court; another one was mentioned in an article. The reviewed judgements were issued between 2003 and 2017. RESULTS: A total of 12 judgments were found. The patients won in all but one of the main proceedings. The case that was lost concerned exceptional circumstances. One patient didn't get the desired interim measure, but won in the main proceedings. The proceedings took between 1 year and 8 months, and 9 years and 5 months. CONCLUSION: Despite the amount of time the patient has to invest, taking legal action is worthwhile. The proceedings at the social courts are generally exempt from charges. In most cases, the statutory health insurance is ordered to pay for a CI.

[Hearing restoration with cochlear implants after translabyrinthine vestibular schwannoma resection]. / Hörrehabilitation mit Cochleaimplantaten nach translabyrinthärer Vestibularisschwannomresektion.

BACKGROUND: Hearing restoration after translabyrinthine vestibular schwannoma resection is a challenge. Because the cochlea can begin to ossify a few months after cochlear or labyrinthine injury, the time interval for cochlear implant surgery is limited. To avoid complete ossification and to prolong the time interval until cochlear implantation, it is possible to insert a placeholder (depth gauge) into the cochlea and perform the cochlear implant surgery at a later time point (two-stage approach). OBJECTIVE: The aim of this retrospective case series was to present the outcomes after restoration of hearing with cochlea implants in six patients and to evaluate the use of the depth gauge in practice. METHODS: The hearing outcome of all patients with (n = 3) and without (n = 3) insertion of a depth gauge was measured with the Freiburg monosyllabic test without background noise at 65 dB. The first measurement was performed prior to the translabyrinthine vestibular schwannoma resection, the last measurement was performed up to 48 months after cochlear implantation. RESULTS: All 6 patients reached 22.5 ± 36.57% prior to vestibular schwannoma resection and 41.3 ± 26% 12 months after cochlear implantation. The understanding values of the patients with a depth gauge were 25.8 ± 16% after 12 months which is below the values of the other patients with 56.6 ± 25.0%. No severe intraoperative or postoperative complications occurred in any patient. CONCLUSION: The two-stage approach for cochlear implantation with depth gauge insertion following labrynthine incision and intact nerve appears to represent a very promising and safe variation for hearing restoration. Intensified research on this approach seems to be justified and necessary.

Long-QT syndrome-associated caveolin-3 mutations differentially regulate the hyperpolarization-activated cyclic nucleotide gated channel 4.

Background Caveolin-3 (cav-3) mutations are linked to the long-QT syndrome (LQTS) causing distinct clinical symptoms. Hyperpolarization-activated cyclic nucleotide channel 4 (HCN4) underlies the pacemaker current If. It associates with cav-3 and both form a macromolecular complex. Methods To examine the effects of human LQTS-associated cav-3 mutations on HCN4-channel function, HEK293-cells were cotransfected with HCN4 and wild-type (WT) cav-3 or a LQTS-associated cav-3 mutant (T78M, A85T, S141R, or F97C). HCN4 currents were recorded using the whole-cell patch-clamp technique. Results WT cav-3 significantly decreased HCN4 current density and shifted midpoint of activation into negative direction. HCN4 current properties were differentially modulated by LQTS-associated cav-3 mutations. When compared with WT cav-3, A85T, F97C, and T78M did not alter the specific effect of cav-3, but S141R significantly increased HCN4 current density. Compared with WT cav-3, no significant modifications of voltage dependence of steady-state activation curves were observed. However, while WT cav-3 alone had no significant effect on HCN4 current activation, all LQTS-associated cav-3 mutations significantly accelerated HCN4 activation kinetics. Conclusions Our results indicate that HCN4 channel function is modulated by cav-3. LQTS-associated mutations of cav-3 differentially influence pacemaker current properties indicating a pathophysiological role in clinical manifestations.

Prenatal and adult androgen activities in alcohol dependence.

OBJECTIVE: Alcohol dependence is more prevalent in men than in women. The evidence for how prenatal and adult androgens influence alcohol dependence is limited. We investigated the effects of prenatal and adult androgen activity on alcohol dependence. Moreover, we studied how the behaviours of pregnant women affect their children's prenatal androgen load. METHOD: We quantified prenatal androgen markers (e.g., second-to-fourth finger length ratio [2D : 4D]) and blood androgens in 200 early-abstinent alcohol-dependent in-patients and 240 controls (2013-2015, including a 12-month follow-up). We also surveyed 134 women during pregnancy (2005-2007) and measured the 2D : 4D of their children (2013-2016). RESULTS: The prenatal androgen loads were higher in the male alcohol-dependent patients compared to the controls (lower 2D : 4D, P = 0.004) and correlated positively with the patients' liver transaminase activities (P < 0.001) and alcohol withdrawal severity (P = 0.019). Higher prenatal androgen loads and increasing androgen levels during withdrawal predicted earlier and more frequent 12-month hospital readmission in alcohol-dependent patients (P < 0.005). Moreover, stress levels (P = 0.002), alcohol (P = 0.010) and tobacco consumption (P = 0.017), and lifetime stressors (P = 0.019) of women during pregnancy related positively to their children's prenatal androgen loads (lower 2D : 4D). CONCLUSION: Androgen activities in alcohol-dependent patients and behaviours of pregnant women represent novel preventive and therapeutic targets of alcohol dependence.

[Speech audiometry for indication of conventional and implantable hearing aids]. / Sprachaudiometrie bei der Indikation von Hörhilfen und Hörimplantaten.

The social function of the human hearing apparatus is comprehension of speech. Auditory rehabilitation aims to enhance speech perception in everyday life. Consequently, audiological evaluation contains speech perception measurement. Many speech audiometric methods have been developed in German-speaking countries, which are suitable for quantification of speech perception abilities in quiet and in noise to address specific diagnostic questions. For establishment of the indication for technical hearing systems such as hearing aids and cochlear implants, the Freiburg monosyllabic test has been employed successfully for many years. Particularly sentence tests have been suggested as a complementary measure for hearing aid indication and assessment. This paper describes the characteristics and range of application of various speech audiometric methods, and highlights the methodological limitations of the individual tests.

[Cochlear implant fitting strategies]. / Anpassung von Cochleaimplantatsystemen.

Cochlear implant (CI) fitting is the technical adjustment of a CI processor to the individual needs of a subject. Fitting comprises the choice of stimulation strategy and determination of the lower and upper stimulation levels for the individual. This electrical dynamic range defines the stimulation range for the real-time functioning of the CI system. All of the CI manufacturers provide a large set of parameters which have to be optimized for proper hearing and speech comprehension. As a consequence of the widening of indication criteria for CIs and lowering the age of implantation for children, the fitting process has changed dramatically over the years. This includes replacement of behavioral responses by other data from objective electrophysiologic measures and from expert knowledge. Recent developments aim to structure and automatize the CI fitting process. This reduces the time requirement for CI fitting and leads to fast settings which can be tested in the real-time situation. This review provides an overview of state-of-the-art fitting procedures and recent developments for fitting strategies. These will be discussed with respect to practicability and quality assurance.

[Improvement in Phoneme Discrimination in Noise in Normal Hearing Adults]. / Trainierbarkeit der Diskrimination von Phonemen im Störgeräusch bei normalhörenden Erwachsenen.

Objective: The study's aim was to examine the possibility to train phoneme-discrimination in noise with normal hearing adults, and its effectivity on speech recognition in noise. A specific computerised training program was used, consisting of special nonsense-syllables with background noise, to train participants' discrimination ability. Material and Methods: 46 normal hearing subjects took part in this study, 28 as training group participants, 18 as control group participants. Only the training group subjects were asked to train over a period of 3 weeks, twice a week for an hour with a computer-based training program. Speech recognition in noise were measured pre- to posttraining for the training group subjects with the Freiburger Einsilber Test. The control group subjects obtained test and restest measures within a 2-3 week break. For the training group follow-up speech recognition was measured 2-3 months after the end of the training. Results: The majority of training group subjects improved their phoneme discrimination significantly. Besides, their speech recognition in noise improved significantly during the training compared to the control group, and remained stable for a period of time. Conclusions: Phonem-Discrimination in noise can be trained by normal hearing adults. The improvements have got a positiv effect on speech recognition in noise, also for a longer period of time.

[Phoneme discrimination training with experienced cochlear implant listeners]. / Training der Phonemdiskrimination mit erfahrenen Cochleaimplantatträgern.

BACKGROUND: Computer-based auditory training programmes seem to be a useful tool in the process of auditory rehabilitation after cochlear implantation (CI). Currently, little is known about the learning mechanism and efficiency of such programs. The aim of the study was to evaluate a specific auditory training programme for phoneme discrimination in experienced CI listeners. MATERIALS AND METHODS: A total of 15 CI adult listeners with more than 2 years' CI experience participated in the auditory training. Over a period of 3 weeks they were instructed to train their phoneme discrimination via computer twice a week. Training material consisted of special syllables for consonants (vCv) and vowels (cVc) discrimination. RESULTS: The discrimination abilities for consonants and vowels improved significantly over the training period for training group participants, whereas the changes for the consonants were higher. In addition, the improvement for voiced and unvoiced consonants was significant. CONCLUSION: Computerised auditory training with phonemes improves CI listeners' discrimination abilities for consonants and vowels.