Application of Multiplanar Volume Reconstruction Technique for the Assessment of Electrode Location and Analysis of the Correlation to Cochlear Programming and Performance in Common Cavity Deformity

Purpose: Owing to the characteristic anatomy, cochlear implantation (CI) for common cavity deformity (CCD) has resulted in varied outcomes and frequent facial and vestibular nerve stimulation. The current study analyzed the correlation among the distance between each electrode and cavity wall (abbreviation, D), programming parameters, and performances outcomes.Materials and Methods: The current, retrospective study included 25 patients (27 ears) with CCD underwent CI. The multiplanar volume reconstruction (MPVR) techniques were employed to reconstruct and evaluate the postoperative temporal bone CT. The D and maximum comfortable level (MCL) 6 months after CI, facial and vestibular nerve stimulation, and outcomes 1, 2, and 3 years after CI pertaining to the questionnaires were documented and analyzed.Results: The patients were divided into symptomatic (10, 37%) and asymptomatic (17, 63%) groups according to with or without facial and vestibular nerve stimulation. The MCL pertaining to the symptomatic group was significantly lower than asymptomatic group, but Categories of Auditory Performance (CAP) scores 1 year after surgery was better (p < 0.05). The subjects were divided into flat (12, 44.4%) and curved (15, 55.6%) groups based on the contour of MCL map. The MCL and D were lower and shorter in the curved group than the flat group, and CAP score 1 year after surgery and Speech Intelligibility Rating (SIR) 3 years after surgery were better (p < 0.05).Conclusion: Although abnormal reactions such as facial and vestibular nerve stimulation were observed to be more frequent, lower MCL and better outcomes were observed in relation to the shorter D.

Auditory performance and speech intelligibility among children with cochlear implant under a state-sponsored insurance scheme: a cross-sectional study

Background: Cochlear implant is the intervention for improving the auditory performance and speech ability for children between 6-12 years with congenital deafness under the Chief Minister’s Comprehensive Health Insurance Scheme, a state-sponsored insurance scheme in Tamil Nadu. Until 2020, 4107 children had been provided with a cochlear implant under the scheme. However, the outcome of this intervention was not studied so far. Hence, as a program evaluation auditory performance and speech intelligibility among patients who received cochlear implant under the scheme is studied.Methods: A cross-sectional study was conducted among 203 patients who had received a cochlear implant at least 1 year ago. Children were evaluated for auditory performance and speech intelligibility using revised Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores by trained speech therapists in Government Medical College hospitals. The proportion of good scorers in CAP (level ≥7) and SIR (category ≥ 3) was computed. The difference in proportion between different demographics was tested using the Chi-square test and Fischer exact test.Results: The median CAP and SIR scores of the children were 8 (Interquartile Range – 4) and 3 (IQR – 2) respectively. Almost 2/3rd of the children had good auditory performance and speech intelligibility. Factors that were associated with good auditory performance and speech intelligibility were being a girl child, getting the implant before 3 years of age, and getting it in a private institution.Conclusions: Gender and age at implant influences auditory performance and speech intelligibility after cochlear implant. Hence, early screening for deafness should be made a routine to enable early detection and management, thereby preventing permanent disability.

Functional Outcomes of Cochlear Implantation in Children with Bilateral Cochlear Nerve Aplasia

Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. Currently, evidence on cochlear implantation (CI) outcomes in patients with CN aplasia is limited. We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, the majority of recipients with a 1-year postoperative CAP score ≤1 remained steady or achieved awareness of environmental sounds, while recipients with early stage hearing benefit had markedly improved auditory performance and could possibly discriminate some speech without lipreading. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. The dynamic range between T and C levels remained unchanged. Our results further refine those of previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

Predicting Quality of Life and Behavior and Emotion from Functional Auditory and Pragmatic Language Abilities in 9-Year-Old Deaf and Hard-of-Hearing Children

Children who are deaf or hard of hearing (DHH) are likely to exhibit difficulties in development of psychosocial skills, pragmatic language skills, and use of hearing for social communication in real-world environments. Some evidence suggests that pragmatic language use affects peer-relationships and school engagement in these children. However, no studies have investigated the influence of functional auditory performance and use of language and speech in real-world environments on children’s behavior and emotion, and on their health-related quality of life. This study explored the relationship in DHH children at 9 years of age. Data from 144 participants of the Longitudinal Outcomes of Children with Hearing Impairment study were analyzed. Parent reports were obtained on quality of life, behavior and emotion, pragmatic language skills, and auditory functional performance of children in real life. Children’s spoken language abilities and speech intelligibility were assessed by research speech pathologists. On average, performance of children in all domains was within the range of typically developing peers. There were significant associations among functional auditory performance, use of speech and language skills, psychosocial skills, and quality of life. Multiple linear regression analyses revealed that better auditory functional performance and pragmatic language skills, rather than structural language abilities, were associated with better psychosocial abilities and quality of life. The novel findings highlight the importance of targeted intervention for improving functional hearing skills and social communication abilities in DHH children, and emphasize the importance of collaborative approaches among medical, audiology, allied health, and educational professionals to identify those at risk so that timely referral and intervention can be implemented for improving psychosocial health and well-being in DHH children.

Spatial Hearing and Functional Auditory Skills in Children With Unilateral Hearing Loss

Purpose The purpose of this study was to characterize spatial hearing abilities of children with longstanding unilateral hearing loss (UHL). UHL was expected to negatively impact children's sound source localization and masked speech recognition, particularly when the target and masker were separated in space. Spatial release from masking (SRM) in the presence of a two-talker speech masker was expected to predict functional auditory performance as assessed by parent report. Method Participants were 5- to 14-year-olds with sensorineural or mixed UHL, age-matched children with normal hearing (NH), and adults with NH. Sound source localization was assessed on the horizontal plane (−90° to 90°), with noise that was either all-pass, low-pass, high-pass, or an unpredictable mixture. Speech recognition thresholds were measured in the sound field for sentences presented in two-talker speech or speech-shaped noise. Target speech was always presented from 0°; the masker was either colocated with the target or spatially separated at ±90°. Parents of children with UHL rated their children's functional auditory performance in everyday environments via questionnaire. Results Sound source localization was poorer for children with UHL than those with NH. Children with UHL also derived less SRM than those with NH, with increased masking for some conditions. Effects of UHL were larger in the two-talker than the noise masker, and SRM in two-talker speech increased with age for both groups of children. Children with UHL whose parents reported greater functional difficulties achieved less SRM when either masker was on the side of the better-hearing ear. Conclusions Children with UHL are clearly at a disadvantage compared with children with NH for both sound source localization and masked speech recognition with spatial separation. Parents' report of their children's real-world communication abilities suggests that spatial hearing plays an important role in outcomes for children with UHL.

Detailed clinical features and genotype–phenotype correlation in an OTOF-related hearing loss cohort in Japan

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype–phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype–phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a “typical” phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85–90% of the patients showed a hearing level of 20–39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed “true” auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype–phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype–phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

On the Effect of Bimodal Rehabilitation in Asymmetric Hearing Loss

Background: Bone conductive implants (BCI) have been reported to provide greater beneficial effects for the auditory and perceptual functions of the contralateral ear in patients presenting with asymmetric hearing loss (AHL) compared to those with single-sided deafness (SSD). The aim of the study was to assess the effects of wearing a conventional hearing aid in the contralateral ear on BCI in terms of an improved overall auditory performance. Methods: eleven AHL subjects wearing a BCI in their worse hearing ear underwent an auditory evaluation by pure tone and speech audiometry in free field. This study group was obtained by adding to the AHL patients those SSD subjects that, during the follow-up, showed deterioration of the hearing threshold of the contralateral ear, thus presenting with the features of AHL. Four different conditions were tested and compared: unaided, with BCI only, with contralateral hearing aid (CHA) only and with BCI combined with CHA. Results: all of the prosthetic conditions caused a significant improvement with respect to the unaided condition. When a CHA was adopted, its combination with the BCI showed significantly better auditory performances than those achieved with the BCI only. Conclusions: the present study suggests the beneficial role of a CHA in BCI-implanted AHL subjects in terms of overall auditory performance.

Round window versus conventional bony cochleostomy technique in pediatric cochlear implantation; A Randomized controlled double blinded trial.

<p><strong>Background:</strong> Objective of the study was to investigate the outcomes of cochlear implantation when done via two different techniques namely, the round window or the bony cochleostomy.</p><p><strong>Methods:</strong> A single-center, double-blinded randomized controlled trial including forty prelingual, bilateral severe to profoundly deaf children less than six years from the year 2014 to 2016 in a tertiary referral center in India were randomly allocated to round window and bony cochleostomy group. Our primary outcome measures were intraoperative neural response telemetry levels, behavioral threshold (t) and comfortable (c) loudness levels. The secondary subjective outcomes were measured via the category of auditory performance (CAP) and the meaningful auditory integration scale (MAIS) score. The cases were followed up for 9 months.</p><p><strong>Results:</strong> Intra-operative, electrically evoked compound action potentials (ECAP) showed comparable mean thresholds for both the techniques except intermediate electrodes (p~0.04) showed lower values for the round window. Similarly, a lower threshold (p~0.03) and comfortable mean current levels (p~0.03) were noticed for the round window group at 6 months post-implantation. Secondary speech perception outcome scores measured via category of auditory performance (CAP) and MAIS score were comparable. </p><p><strong>Conclusions:</strong> The round window insertion technique has physiological benefit as compared to the bony cochleostomy as evidenced by better stimulation levels in the intermediate electrodes and lower mean threshold and comfortable levels in the round window with more beneficial peri-modiolar position of electrode arrays. However, comparable speech perception outcomes revealed no clinical benefit in the cochlear implant performance depending on the technique of electrode insertion. </p>

Robot-Assisted Electrode Array Insertion Becomes Available in Pediatric Cochlear Implant Recipients: First Report and an Intra-Individual Study

Background: As an advanced surgical technique to reduce trauma to the inner ear, robot-assisted electrode array (EA) insertion has been applied in adult cochlear implantation (CI) and was approved as a safe surgical procedure that could result in better outcomes. As the mastoid and temporal bones are generally smaller in children, which would increase the difficulty for robot-assisted manipulation, the clinical application of these systems for CI in children has not been reported. Given that the pediatric candidate is the main population, we aim to investigate the safety and reliability of robot-assisted techniques in pediatric cochlear implantation.Methods: Retrospective cohort study at a referral center in Shanghai including all patients of simultaneous bilateral CI with robotic assistance on one side (RobOtol® system, Collin ORL, Bagneux, France), and manual insertion on the other (same brand of EA and CI in both side), from December 2019 to June 2020. The surgical outcomes, radiological measurements (EA positioning, EA insertion depth, mastoidectomy size), and audiological outcomes (Behavior pure-tone audiometry) were evaluated.Results: Five infants (17.8 ± 13.5 months, ranging from 10 to 42 months) and an adult (39 years old) were enrolled in this study. Both perimodiolar and lateral wall EAs were included. The robot-assisted EA insertion was successfully performed in all cases, although the surgical zone in infants was about half the size in adults, and no difference was observed in mastoidectomy size between robot-assisted and manual insertion sides (p = 0.219). The insertion depths of EA with two techniques were similar (P = 0.583). The robot-assisted technique showed no scalar deviation, but scalar deviation occurred for one manually inserted pre-curved EA (16%). Early auditory performance was similar to both techniques.Conclusion: Robot-assisted technique for EA insertion is approved to be used safely and reliably in children, which is possible and potential for better scalar positioning and might improve long-term auditory outcome. Standard mastoidectomy size was enough for robot-assisted technique. This first study marks the arrival of the era of robotic CI for all ages.