International Consensus Statements on Intraoperative Testing for Cochlear Implantation Surgery.

OBJECTIVES: A wide variety of intraoperative tests are available in cochlear implantation. However, no consensus exists on which tests constitute the minimum necessary battery. We assembled an international panel of clinical experts to develop, refine, and vote upon a set of core consensus statements. DESIGN: A literature review was used to identify intraoperative tests currently used in the field and draft a set of provisional statements. For statement evaluation and refinement, we used a modified Delphi consensus panel structure. Multiple interactive rounds of voting, evaluation, and feedback were conducted to achieve convergence. RESULTS: Twenty-nine provisional statements were included in the original draft. In the first voting round, consensus was reached on 15 statements. Of the 14 statements that did not reach consensus, 12 were revised based on feedback provided by the expert practitioners, and 2 were eliminated. In the second voting round, 10 of the 12 revised statements reached a consensus. The two statements which did not achieve consensus were further revised and subjected to a third voting round. However, both statements failed to achieve consensus in the third round. In addition, during the final revision, one more statement was decided to be deleted due to overlap with another modified statement. CONCLUSIONS: A final core set of 24 consensus statements was generated, covering wide areas of intraoperative testing during CI surgery. These statements may provide utility as evidence-based guidelines to improve quality and achieve uniformity of surgical practice.

Clinically recorded cortical auditory evoked potentials from paediatric cochlear implant users fitted with electrically elicited stapedius reflex thresholds.

OBJECTIVES: This study aimed to objectively evaluate access to soft sounds (55 dB SPL) in paediatric CI users, all wearing MED-EL (Innsbruck, Austria) devices who were fitted with the objective electrically elicited stapedius reflex threshold (eSRT) fitting method, to track their cortical auditory evoked potential (CAEP) presence and latency, and to compare their CAEPs to those of normal-hearing peers. METHODS: Forty-five unilaterally implanted, pre-lingually deafened MED-EL CI users, aged 12-48 months, underwent CAEP testing in the clinic at regular monthly intervals post switch-on. CAEPs were recorded in response to short speech tokens /m/, /g/ and /t/ presented in the free field at 55 dB SPL. Twenty children with normal hearing (NH), similarly aged, underwent CAEP testing once. RESULTS: The proportion of present CAEPs increased and CAEP P1 latencies reduced significantly with post-implantation duration. CAEPs were scored based on their presence and age-appropriate P1 latency. These CAEP scores increased significantly with post-implantation duration. CAEP scores were significantly worse for the /m/ speech token compared to the other two tokens. Compared to the NH group, CAEP scores were significantly smaller for all post-implantation test intervals. CONCLUSIONS: This study provides clinicians with a first step towards typical ranges of CAEP presence, latency, and derived CAEP score over the first months of MED-EL CI use. CAEPs within these typical ranges could validate intervention whereas less than optimum CAEPs could prompt clinicians to seek solutions in a timely manner. CAEPs could clinically validate whether a CI provides adequate access to soft sounds. This approach could form an alternative to behavioural soft sound access verification.

Comparing eSRT and eCAP measurements in pediatric MED-EL cochlear implant users.

INTRODUCTION: Electrically evoked compound action potentials (eCAP) and electrically evoked stapedius reflexes are the most frequently used objective measurements for programming a cochlear implant (CI) audio processor. Objective methods are particularly beneficial for children and CI users that encounter difficulties in providing feedback. In this study, we compared the threshold and the slope of the eCAP amplitude growth function with the electrically evoked stapedius reflex threshold (eSRT) in pediatric CI users. Furthermore, the duration times required to perform eCAP and eSRT recordings were compared. METHODS: During a regular fitting session, 52 pediatric CI users with recordable eSRTs having MED-EL devices (MED-EL GmbH, Innsbruck, Austria) were programmed using the eSRT fitting method. The eCAP thresholds and the slopes of the amplitude growth function were measured across one apical, one medial, and one basal electrode contact. RESULTS: There was a weak to medium correlation between eCAP thresholds and eSRTs. The eCAP threshold profile did not correlate with the eSRT profile. Typically ECAP thresholds were at a lower stimulation charge than eSRTs with only 4/152 being higher. An eCAP threshold was found on 152/156 electrode contacts with eSRTs. On average, the eCAP measurements took 4.2 times longer to record per electrode than eSRT measurements (median durations 35 s vs. 120 s). CONCLUSION: eSRTs were significantly higher than eCAP thresholds and eSRT and eCAP profiles were generally different from each other reducing the clinical relevance of eCAP testing for setting MCLs across the array. Additionally, the eSRT measurements were faster to record than the eCAP threshold and slope determination measurements.

Assessment of early language development in Turkish children with a cochlear implant using the TEDIL test.

OBJECTIVES: To analyse language development of children with a cochlear implant (CI) in relation to length of CI use and age at implantation and to examine the suitability of the TEDIL as an assessment tool for measuring early language development in Turkish children. METHODS: A total of 119 children implanted with a CI before 5 years of age were assessed acutely on sound field thresholds, speech recognition thresholds, open-set and closed-set monosyllabic word tests, the TEDIL, categories of auditory performance (CAP), and speech intelligibility rating (SIR). The outcome scores were analysed in relation to length of CI use (3, 4, and 5 years) and age at implantation (<24 months vs. >24 months). The TEDIL scores were compared to all other outcome measures. RESULTS: Scores significantly increased with CI experience. CAP and SIR were significantly higher in the younger implanted group. No significant difference was observed between the younger and older implanted group on the closed-set and open-set monosyllabic tests and the TEDIL. The TEDIL scores significantly correlated with CAP, SIR, and the closed-set and open-set word scores. The mean TEDIL standard score was close to average. CONCLUSIONS: Performance of CI users improves with increased CI experience. CI users implanted <24 months tend to have better auditory skills and clearer speech than CI users implanted >24 months. CI users implanted between 24 months and 60 months tend to develop language similarly to CI users implanted <24 months. The TEDIL is a suitable tool for assessing early receptive and expressive language development in Turkish children.

Auditory brainstem implant in postlingual postmeningitic patients.

OBJECTIVES/HYPOTHESIS: The aim of this study was to evaluate outcomes of postlingual postmeningitic patients who received an auditory brainstem implant (ABI). STUDY DESIGN: Retrospective analysis was performed on postlingual postmeningitic patients with bilateral profound sensorineural hearing loss who underwent ABI between the years 2007 and 2014 METHODS: All patients were postlingually deaf due to cochlear ossification as a consequence of bacterial meningitis. The patients received a MED-EL or Neurelec ABI. All patients were operated on at different hospitals by the same primary surgeon. The patients were tested using Ling 5 sound detection, sound field implant thresholds between 250 Hz and 6 kHz, and 6 to 12 choice closed-set word and sentence tests. RESULTS: Nine patients with postmeningitic cochlear ossification received an ABI. Five of nine ABI users (55.5%) wear their audio processors (AP) most of the time. Four (44.5%) with no perceivable benefit have become nonusers. Three of the five consistent ABI users reported good benefit. The other two ABI users who do wear their APs do not respond to sound in daily living but reported benefits such as "feeling sound" in a good way. CONCLUSIONS: In this study, five of nine patients (55.5%) with bilateral ossified cochlea had some degree of benefit from their ABI. An ABI may be useful in hearing restoration in postlingual patients with bilateral ossified cochlea due to meningitis. However, poor results may be related to side effects, which may necessitate deactivation of electrodes, long duration of auditory deprivation, or impairments in the auditory neural structures as a result of meningitis. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1889-1892, 2016.

LittlEARS(®) early speech production questionnaire validation in hearing Turkish infants.

AIM: The LEESPQ validated on hearing, German infants is a standardized tool examining preverbal speech development. This study aims to validate the LEESPQ on hearing, Turkish infants. MATERIALS AND METHODS: This is a prospective, cross-sectional validation study using the LEESPQ in Turkish. The LEESPQ was filled in once for each hearing infant. Data for ≥10 infants was collected at 19 test intervals, ≥ 0-1, ≥ 1-2……, ≥18-19 months. Psychometric validation was performed through confirmatory factor analysis, item response analysis, item analysis, and analysis of reliability and validity. RESULTS: The LEESPQ was found to be gender independent, have high predictive accuracy and almost exclusively assess speech production ability. A very high correlation between total score and chronological age means score can be interpreted as child's speech production developmental age. Expected and minimum scores were defined for each monthly interval. CONCLUSION: The LEESPQ (Turkish) has clinical value to confirm typical speech production development and detect potential problems.

Cochlear implant programming: a global survey on the state of the art.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

Methods and preliminary outcomes of pediatric auditory brainstem implantation.

OBJECTIVE: The objective was to provide information about methods used and preliminary outcomes for pediatric ABI (auditory brainstem implant). STUDY DESIGN: An analysis of outcome was performed in children who received an ABI. METHODS: Twelve children received a MED-EL ABI system. Progress in audition and language was monitored through parental reports, questionnaires, profiles, and closed-set tests. RESULTS: The median number of active electrodes was 9 of 12. Seven of 12 users consistently respond to sound, and 5 of 12 do not. Highest performers can recognize words in small sets and have begun to use some words. CONCLUSION: Auditory brainstem implants appear to be beneficial for some pediatric patients who cannot benefit from traditional cochlear implant surgery. Benefits in the short-term can be recognition of environmental sounds, recognition of some words and very commonly used phrases, and the beginning use of words. Although some of our ABI users demonstrate no response to sound, they do want to wear their sound processors all waking hours. The cause of lack of response may be related to the second intervention, which might have led to displacement of the electrode array, or presence of additional handicaps or syndromes. However, the results are less than optimal. The relatively short postoperative follow-up duration is a considered weakness of this study.

An investigation of the first lexicon of Turkish hearing children and children with a cochlear implant.

INTRODUCTION: Research on early cochlear implantation and first language milestones is limited. To compare language performance in cochlear implant (CI) users and hearing children, the establishment of normative data for both groups would be of benefit. To aid the data collection for Turkish hearing children and children with a CI diaries can be used. AIMS: This study aimed to document the first 100-word lexicon acquired by Turkish hearing children and children with a CI during the first 2 years of hearing experience, to determine the distribution of the first 100-word lexicon per word category, the rate of acquisition of words and the effects of age at implantation on language performance. METHODS: First word data was collected from 63 Turkish hearing children and 71 CI users implanted under 36 months of chronological age using a diary. The mean number of words recorded at each time interval was calculated. The time taken to achieve the first 100-word lexicon and the categories of the first words were documented. Performance under 18 months and over 24 months of age at first fitting was compared. RESULTS: By 19-21 months of hearing age both hearing and CI user's vocabularies were of similar size. CI users developed a lexicon earlier than hearing children, but once hearing children started to acquire words their acquisition rate was faster. The distribution of words acquired per category were similar. 83% of first words were shared by both groups. No significant difference in performance was found between: hearing versus: implanted children; or earlier (<18 months) versus later (≥24 months) implanted children. CONCLUSIONS: The vocabulary of hearing children compared to CI users are similar in size and the category. Early access to auditory stimuli facilitates children with a hearing loss to develop vocabularies similar to hearing children in the short term.

Young cochlear implant users' auditory development as measured and monitored by the LittlEARS® Auditory Questionnaire: a Turkish experience.

OBJECTIVE: This study aims to assess the usefulness of the LittlEARS(®) Auditory Questionnaire (LEAQ) in determining the audiological development of Turkish children who have received a cochlear implant. METHODS: 20 children received a cochlear implant before their 3rd birthday. Each child's progress was evaluated with the LittlEARS(®) Auditory Questionnaire at first device fitting and then at 3-month intervals for 2 years. Scores were compared with the age-related norms established by hearing children. RESULTS: All children showed a significant increase in LittlEARS(®) Auditory Questionnaire scores over time. Nearly all children showed a growth in auditory skills similar to that of hearing children. Children without additional needs showed more development than did children with additional needs. CONCLUSIONS: The LittlEARS(®) Auditory Questionnaire is useful for monitoring the audiological development of young children with a cochlear implant. Confirmation that a cochlear implant user is achieving typical auditory milestones serves to boost parental morale during a child's pre-verbal stage when parents may be anxious about their child's ability to talk. The questionnaire could also be useful as an early warning system. Poor scores likely indicate that something is impeding the child's development. This should prompt professionals to try to identify the impediment, whether technical, medical, social or educational and, possibly, eliminate/mitigate its effects while the child is still in his/her critical development stages.

Developing a music programme for preschool children with cochlear implants.

UNLABELLED: Although music perception is especially challenging for cochlear implant (CI) users, young CI users' musical perception abilities are improved by participation in structured musical activities. OBJECTIVES: To design, implement, evaluate, and publish a music training programme with a monitoring tool for preschool CI users, for use in family-centred habilitation programmes. METHODS: We devised a programme of musical activities, Musical EARS®, and a curriculum-related hierarchical Evaluation Form to represent performance. The programme included sections on singing; recognizing songs, tunes, and timbre; and responding appropriately to music and rhythm. It was implemented over 18 months at Ilkses Rehabilitation Centre, with 25 paediatric MED-EL CI users split into three groups of varying age, duration of CI use, and ability. RESULTS: Mean total scores increased significantly for all groups. Scores increased unevenly across subscales. Participation in and enjoyment of musical activities increased for both children and parents. Significant correlations were found between scores and length of CI use. DISCUSSION: The training programme effectively enriches child CI users' musical experience. To varying degrees, children learned to perform the Musical EARS® activities. The study allowed us to validate the lesson content and the hierarchical nature of the Evaluation Form. We conclude that prelingually deafened CI users should be systematically involved in musical activities to help them acquire skills acquired more easily by hearing peers.

Comparing auditory perception and speech production outcomes: non-language specific assessment of auditory perception and speech production in children with cochlear implants.

Language-independent assessment tools evaluate the progress of children who receive a cochlear implant, allowing large pooling of data for better access to insurers and other health care professionals. One hundred and seventeen children from centres in the United Kingdom, Iran and Turkey were assessed on two measures over a five-year test interval. There is a significant improvement over time for the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) measures. There was a significant difference between scores for different language groups: accounted for by the differences in age at implantation. There was a significant effect of age at implantation up to three years of device use. There were high correlations between the CAP and SIR scores. A longer duration of deafness resulted in a higher score for both scales; however, there was no relationship when correlated for age. Finally, the CAP pre-operative score allows us to predict the post-operative SIR scores. The scales are validated; reliable measures which can be used across countries and languages. This allows greater ability to pool data allowing data to be generalised across population groups, providing more power to prove that cochlear implantation is a viable treatment for children with bilateral severe-to-profound hearing loss.