Australian families of deaf and hard of hearing children: Are they using sign?

BACKGROUND: Deaf and hard of hearing (DHH) children may experience communication delays, irrespective of early intervention and technology. Australian Sign Language (Auslan) is one approach in early intervention to address language delays. Current prevalence of Auslan use among Australian families with DHH children is unknown. AIMS: The first aim was to determine the proportion of families enrolled in an Australian statewide hearing loss databank who use Auslan with their DHH child. The second aim was to explore the relationships between indicators of child hearing loss (bilateral or unilateral hearing loss, degree of hearing loss, and device use: hearing aids and cochlear implants), family factors (maternal education, attendance at early intervention, family history of deafness, and socio-economic disadvantage) and the family's reported use of Auslan. METHODS: We analysed the enrolment data from 997 families who participated in an Australian statewide hearing loss databank between 2012 and 2021. We described the proportion of families who used Auslan with their DHH child at home. The association between indicators of child hearing loss and family factors, and the parental reports of communication approach were examined using correlation analyses. RESULTS: Eighty-seven of 997 parents (8.7%) reported using Auslan with their DHH child. Of these, 26 (2.6%) used Auslan as their primary language. The use of Auslan at home was associated with the following indicators of child hearing loss: bilateral hearing loss, profound compared to mild hearing loss, and cochlear implant and hearing aid use compared to no device use. The family factors associated with the use of Auslan were: referral or attendance at early intervention compared to those who did not attend, and a family history of deafness compared to those with none. No association was found between maternal education and socio-economic disadvantage and the use of Auslan. CONCLUSION: This Australian study found a low proportion (8.7%) of families with a DHH child who reported using Auslan. Seven child hearing loss and family factors were considered, and five were significantly associated with using Auslan at home. Children with a greater degree of hearing loss, attendance at early intervention and family history of deafness tended to use Auslan.

Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

Long-Term Cochlear Implant Sound Processor Usage in Children with Single-Sided Deafness.

OBJECTIVE: To assess cochlear implant (CI) sound processor usage over time in children with single-sided deafness (SSD) and identify factors influencing device use. STUDY DESIGN: Retrospective, chart review study. SETTING: Pediatric tertiary referral center. PATIENTS: Children with SSD who received CI between 2014 and 2020. OUTCOME MEASURE: Primary outcome was average daily CI sound processor usage over follow-up. RESULTS: Fifteen children with SSD who underwent CI surgery were categorized based on age of diagnosis and surgery timing. Over an average of 4.3-year follow-up, patients averaged 4.6 hours/day of CI usage. Declining usage trends were noted over time, with the first 2 years postactivation showing higher rates. No significant usage differences emerged based on age, surgery timing, or hearing loss etiology. CONCLUSIONS: Long-term usage decline necessitates further research into barriers and enablers for continued CI use in pediatric SSD cases.

Comparing pronunciation challenges in South Korean preschoolers with unilateral single-sided deafness due to cochlear nerve deficiency to a norm-referenced standard.

This study aimed to compare the development of pronunciation in South Korean preschoolers with unilateral cochlear nerve deficiency (CND) to that of age-matched preschoolers with normal hearing, a topic that has not been explored previously. In a retrospective analysis, 25 preschoolers with unilateral CND who had undergone a speech evaluation battery, including a pronunciation and vocabulary test, were enrolled. Utilizing the Urimal Test of Articulation and Phonation and customized language ability tests, pronunciation and vocabulary were assessed. The subjects' speech evaluation scores were converted into age-adjusted z-scores using normal controls' data. While vocabulary performance was within normal limits, their average pronunciation z-score was -2.90, significantly lower than both the zero reference point and their vocabulary z-scores. None of the subjects scored above average in pronunciation. Thirteen patients were recommended for articulation therapy, seven were considered as potential candidates for this therapy, and the remaining five were within normal limits. There was no observed correlation between the development of pronunciation and vocabulary. Notably, some subjects' pronunciation scores did not improve, even after serial follow-up during their preschool years. Despite typical vocabulary development, preschoolers with unilateral CND exhibit significant delays in pronunciation. These findings emphasize the necessity for vigilant monitoring of their language development.

Can Mandarin-speaking prelingual deaf adults benefit from cochlear implant?

BACKGROUND: With advances in cochlear implant (CI) technology, prelingual deaf adults may experience improved speech perception and quality of life (QoL). It is still a challenge for Mandarin-speaking CI user with tone recognition due to CI technology focused on intonation language. OBJECTIVES: To evaluate the long-term post-CI auditory performance and social-emotional benefits in prelingual deaf Mandarin-speaking adults and the difference between them and post-lingual deaf adults. MATERIAL AND METHODS: Fifty-five adult implanted ears were included (forty-six postlingual deaf group; nine prelingual deaf group). Post-CI long-term outcomes were using vowels, consonants, disyllabic words, Mandarin monosyllable words, categories of audiology performance, speech intelligibility rating, subjective social-emotional questionnaires. RESULTS: Post-CI auditory performance and speech intelligibility of prelingual deafness adults was significantly inferior to that of those with postlingual deafness. However, both groups presented improved social-emotional benefits, with no significant difference between both groups. CONCLUSIONS: Adult CI recipients who deaf before the age of 4 can experience benefits in social-emotional life functioning, regardless of their limited auditory performance and speech intelligibility. Therefore, prelingual Mandarin-speaking deaf adults, especially those using oral communication, can be considered as relative indications for cochlear implantation. SIGNIFICANCE: To clarify and validate the benefits among Mandarin-speaking prelingual deaf adult recipients.

Knowledge, attitude, and practice toward cochlear implants among deaf patients who received cochlear implants.

Despite the growing use of cochlear implants in deaf patients, there is a lack of data on their knowledge, attitude, and practice (KAP) toward cochlear implants. This study aimed to investigate the KAP toward cochlear implants among deaf patients who received cochlear implants. A web-based cross-sectional study was conducted between August 2022 and December 2022 among deaf patients who had received cochlear implants. A self-administered questionnaire was used to collect demographic characteristics and KAP scores. A total of 526 participants were enrolled; 54.18% were female, 65.40% were above 60 years old, and 61.03% were surveyed at less than 3 years after implantation. The mean knowledge, attitude, and practice scores were 8.15 ± 2.18 (possible range: 0-10), 43.63 ± 6.98 (possible range: 12-60), and 41.11 ± 7.42 (possible range: 11-55), respectively, indicating good knowledge, moderate attitude and practice. Multivariable logistic regression analysis showed that attitude [odd ratio (OR) = 1.24, 95% confidence interval (CI) 1.18-1.29, P < 0.001] and unemployment (OR = 0.33, 95% CI 0.17-0.63, P = 0.001) were independently associated with practice. Path analysis showed that knowledge directly influenced attitude (ß = 0.93, 95% CI 0.61-1.19, P < 0.001), attitude directly influenced practice (ß = 0.53, 95% CI 0.46-0.61, P < 0.001), and knowledge directly (ß = 0.77, 95% CI 0.53-1.01, P < 0.001) and indirectly (ß = 0.50, 95% CI 0.34-0.66, P < 0.001) influenced practice. Deaf patients who received cochlear implants showed good knowledge, moderate attitude and practice toward cochlear implants. Knowledge should be strengthened to improve attitude and practice toward cochlear implants, which could translate into realistic expectations toward cochlear implants devices and proper care and maintenance.

Processing of Plural Marking in Nouns by German-Speaking Children With Normal Hearing and Children With Cochlear Implants: An Eye-Tracking Study.

PURPOSE: Our goal is to understand how the different types of plural marking are understood and processed by children with cochlear implants (CIs): (a) how does salience affect the processing of plural marking, (b) how is this processing affected by the incomplete signal provided by the CIs, and (c) is it linked to individual factors such as chronological age, vocabulary development, and phonological working memory? METHOD: Sixteen children with CIs and 30 age-matched children with normal hearing (NH) participated in an eye-tracking study. Their task was to choose the corresponding picture to an auditorily presented singular or plural noun. Accuracy, reaction time, and gaze fixation were measured and analyzed with mixed-effect models. RESULTS: Group differences were found in accuracy but not in reaction time or gaze fixation. Plural processing is qualitatively similar in children with CIs and children with NH, with more difficulties in processing plurals involving stem-vowel changes and less with those involving suffixes. Age effects indicate that processing abilities still evolve between 5 and 11 years, and processing is further linked to lexical development. CONCLUSIONS: Our results indicate that early implantation seems to be beneficial for the acquisition of plural as indicated by very small between-group differences in processing and comprehension. Processing is furthermore affected by the type of material (i.e., phonetic, phonological, or morphological) used to mark plural and less so by their segmental salience. Our study emphasizes the need to take into account the form of the linguistic material in future investigations at higher levels of processing.

The challenge of a mature final diagnosis of hearing loss severity and early cochlear implantation.

PURPOSE: Despite the recent trend of cochlear implantation (CI) at the age of six or even four months is prevalent in many centers around the world, clinicians should be cautious because perinatal risk factors of auditory neuropathy and/or delayed maturation carry the possibility of reversible hearing loss, yielding better auditory performance at the age of one year. The purpose of this study is to raise awareness that early CI may not be universal for all patients. In addition, we specify the factors to be considered in the pre-operative evaluation of CI in infants younger than one year. METHODS AND RESULTS: This study describes four cases provisionally diagnosed with severe to profound sensorineural hearing loss that were presented to the CI clinic to determine candidacy for implantation. Two cases had histories of prematurity, one had Down syndrome, and one had a family history of hearing loss. None of the study cases were candidates for CI, as they had varying degrees of hearing improvement. CONCLUSION: Although early CI may yield better auditory performance, the final diagnosis should be made only after repeated subjective and objective measurements as well as family feedback on the child's auditory performance, especially in preterm children. Early auditory brainstem response (ABR) prior to the age of one year in children with cognitive, neurologic, or developmental comorbidities should be interpreted with caution, as ABR "alone" could not accurately represent the child's true hearing ability in this patient population.

Features of the speech processing network in post- and prelingually deaf cochlear implant users.

The onset of hearing loss can lead to altered brain structure and functions. However, hearing restoration may also result in distinct cortical reorganization. A differential pattern of functional remodeling was observed between post- and prelingual cochlear implant users, but it remains unclear how these speech processing networks are reorganized after cochlear implantation. To explore the impact of language acquisition and hearing restoration on speech perception in cochlear implant users, we conducted assessments of brain activation, functional connectivity, and graph theory-based analysis using functional near-infrared spectroscopy. We examined the effects of speech-in-noise stimuli on three groups: postlingual cochlear implant users (n = 12), prelingual cochlear implant users (n = 10), and age-matched individuals with hearing controls (HC) (n = 22). The activation of auditory-related areas in cochlear implant users showed a lower response compared with the HC group. Wernicke's area and Broca's area demonstrated differences network attributes in speech processing networks in post- and prelingual cochlear implant users. In addition, cochlear implant users maintain a high efficiency of the speech processing network to process speech information. Taken together, our results characterize the speech processing networks, in varying noise environments, in post- and prelingual cochlear implant users and provide new insights for theories of how implantation modes impact remodeling of the speech processing functional networks.

Pediatric cochlear implantation: Early surgical intervention and parental quality of life.

OBJECTIVES: Pediatric cochlear implantation (CI) provides sound perception to children with significant sensorineural hearing loss and, despite its challenging process, early implantation can enhance children's speech/language outcomes and potentially improve parental quality of life (PQoL). This study aims to examine parental perspectives on quality of life and parenting children with CI. METHODS: This study combined retrospective chart review and parent reported outcomes. Data were abstracted from medical charts of 85 children who underwent CI between 2016 and 2022 at a tertiary pediatric hospital. Parents were administered the Acceptance and Action Questionnaire (AAQ-MCHL), an 8-item self-report assessment of quality of life for parents of children with CI. Multivariate linear regression analyses examined clinical factors associated with PQoL scores. RESULTS: Parents whose children were implanted at less than two years of age reported significantly higher PQoL, indicated by lower AAQ scores, with a mean AAQ-MCHL of 7.6 + 5.7. In contrast, implantation at age >2 years yielded a mean AAQ-MCHL of 16.2 + 9.6. Parents interviewed within one year post-surgery reported lower PQoL, with a mean AAQ-MCHL of 12.3 + 8.8 compared to those interviewed after one year, with 20.5 + 10.4. CONCLUSION: Early identification of profound hearing loss in children, coupled with early surgical CI, may be associated with higher parental quality of life. The beneficial outcomes appear to be potentiated over time. Further research is essential to fully comprehend the impact of CI on the quality of life of children and their parents.

Variability in Receptive Language Development Following Bilateral Cochlear Implantation.

OBJECTIVES: The primary aim was to investigate the variability in language development in children aged 5-7.5 years after bilateral cochlear implantation (CI) up to the age of 2 years, and any impact of the age at implantation and additional noncognitive or anatomical disorders at implantation. DESIGN: Data of 84 congenitally deaf children that had received simultaneous bilateral CI at the age of ≤ 24 months were included in this retrospective study. The results of language comprehension acquisition were evaluated using a standardized German language acquisition test for normal hearing preschoolers and first graders. Data on speech perception of monosyllables and sentences in quiet and noise were added. RESULTS: In a monosyllabic test, the children achieved a median performance of 75.0 ± 12.88%. In the sentence test in quiet, the median performance was 89 ± 12.69%, but dropped to 54 ± 18.92% in noise. A simple analysis showed a significant main effect of age at implantation on monosyllabic word comprehension (p < .001), but no significant effect of comorbidities that lacked cognitive effects (p = .24). Language acquisition values correspond to the normal range of children with normal hearing. Approximately 25% of the variability in the language acquisition tests is due to the outcome of the monosyllabic speech perception test. CONCLUSIONS: Congenitally deaf children who were fitted bilaterally in the 1st year of life can develop age-appropriate language skills by the time they start school. The high variability in the data is partly due to the age of implantation, but additional factors such as cognitive factors (e.g., working memory) are likely to influence the variability.

Emotional abilities in preadolescents and adolescents with long-term cochlear implant use.

OBJECTIVES: Emotional abilities (EAs) are particularly important during preadolescence/adolescence, two challenging periods characterized by significant biological, physical, and cognitive changes. The present study attempted to a holistic EA approach in preadolescents/adolescents with cochlear implants (CI) and typical hearing (TH), considering various aspects such as perception, cognitive facilitation, comprehension, and management of emotions. A secondary aim was to identify significant demographic and audiological factors of EA development. METHODS: CI/TH groups were matched for chronological age, nonverbal IQ, gender, economic income, and maternal level of education. Each group consisted of 43 participants (age range 10-18 years). EAs were evaluated by using the multi-trait/method IE-ACCME test. Auditory-linguistic assessments included participants' lexical skills and Matrix performance as well. RESULTS: EA performance for perception and cognitive facilitation did not show any statistically significant CI/TH group differences (p > 0.05). Significant CI/TH differences emerged for emotion comprehension and management: CI group performed significantly worse in understanding emotional blends (t = 2.56, p = 0.014) but better in personal emotion management (t = -2.01, p = 0.048). For the CI group, gender showed statistically significant effects on cognitive facilitation in sensations, with males performing better than females (U = 129, p = 0.018). TH preadolescents showed significantly lower scores in understanding emotional changes in comparison to TH adolescents (U = 125.5, p = 0.01). Emotional blends understanding showed a weak negative correlation with Matrix performance (r = - 0.38, p = 0.013) and a moderate positive correlation with lexical skills (r = 0.40, p = 0.008). Relationships management showed various significant correlations: weak negative correlations with age at CI (r = - 0.38, p = 0.011) and Matrix performance (r = - 0.36, p = 0.016) as well as weak positive correlations with nonverbal-IQ (r = 0.38, p = 0.013) and positive moderate correlations with lexical skills (r = 0.49, p < 0.001). CONCLUSION: Cochlear implantation seems to show significant positive effects on emotional development in children, allowing them to achieve age appropriate EAs as they grow up and become preadolescents/adolescents. EA assessment in CI users may not only support monitorization of EA trajectory, but also early identification of any EA disorders, so that subjects with low EA profiles could be timely and properly intervened.

Early cochlear implantation for children with single sided deafness.

OBJECTIVES: Single-sided deafness (SSD) can have consequences for a child's language, educational, and social development. A cochlear implant (CI) is the only device which can restore true binaural hearing, yet they are only approved for children with (SSD) over the age of five in the United States. Reports on speech perception outcomes for children implanted at a younger age are limited. The present study aims to examine the effects of age at implantation, duration of deafness, hearing loss etiology, and presence of additional disabilities on device usage and speech perception outcomes. METHODS: A retrospective chart review was used to examine demographics and speech perception outcomes for 18 children implanted at age five or younger. RESULTS: Speech perception results were highly variable, with some children deriving significant benefit and others demonstrating no sound awareness through the implant alone. Age at implantation and duration of deafness did not have a clear impact on outcomes. Device usage was low in many children, often those with anatomical abnormalities such as a hypoplastic cochlear nerve. There are challenges to assessing speech perception in young children with SSD, leading to a lack of standardized outcome measures. CONCLUSIONS: Early CI for children with SSD may improve speech perception, but benefit is not guaranteed. Candidacy evaluation should consider both medical and audiological factors, in addition to the degree of family support and realistic expectations. Caution is especially warranted in children with significant anatomical anomalies.

Improving quality of life in the elderly: hearing loss treatment with cochlear implants.

BACKGROUND: Hearing loss impacts health-related quality of life and general well-being and was identified in a Lancet report as one of the largest potentially modifiable factors for the prevention of age-related dementia. There is a lack of robust data on how cochlear implant treatment in the elderly impacts quality of life. The primary objective was to measure the change in health utility following cochlear implantation in individuals aged ≥ 60 years. METHODS: This study uniquely prospectively recruited a large multinational sample of 100 older adults (mean age 71.7 (SD7.6) range 60-91 years) with severe to profound hearing loss. In a repeated-measures design, pre and post implant outcome measures were analysed using mixed-effect models. Health utility was assessed with the Health Utilities Index Mark III (HUI3). Subjects were divided into groups of 60-64, 65-74 and 75 + years. RESULTS: At 18 months post implant, the mean HUI3 score improved by 0.13 (95%CI: 0.07-0.18 p < 0.001). There was no statistically significant difference in the HUI3 between age groups (F[2,9228] = 0.53, p = 0.59). The De Jong Loneliness scale reduced by an average of 0.61 (95%CI: 0.25-0.97 p < 0.014) and the Lawton Instrumental Activities of Daily Living Scale improved on average (1.25, 95%CI: 0.85-1.65 p < 0.001). Hearing Handicap Inventory for the Elderly Screening reduced by an average of 8.7 (95%CI: 6.7-10.8, p < 0.001) from a significant to mild-moderate hearing handicap. Age was not a statistically significant factor for any of the other measures (p > 0.20). At baseline 90% of participants had no or mild depression and there was no change in mean depression scores after implant. Categories of Auditory perception scale showed that all subjects achieved a level of speech sound discrimination without lip reading post implantation (level 4) and at least 50% could use the telephone with a known speaker. CONCLUSIONS: Better hearing improved individuals' quality of life, ability to communicate verbally and their ability to function independently. They felt less lonely and less handicapped by their hearing loss. Benefits were independent of age group. Cochlear implants should be considered as a routine treatment option for those over 60 years with bilateral severe to profound hearing loss. TRIAL REGISTRATION: ClinicalTrials.gov ( http://www. CLINICALTRIALS: gov/ ), 7 March 2017, NCT03072862.

The impact of cochlear implantation on quality of life and psychological status in single-sided deafness or asymmetric hearing loss with tinnitus and influencing factors of implantation intention: a preliminary study.

PURPOSE: The current study aims to explore the therapeutic effect of cochlear implants (CIs) on tinnitus in patients with single-sided deafness or asymmetric hearing loss (SSD/AHL) as well as the improvement of tinnitus-related quality of life and psychological status. In addition, we also explored whether the levels of quality of life and psychological status was related to the patient's implantation intention. METHODS: Seven patients decided to receive cochlear implantation. Before and after implantation, they completed the Visual Analogue Scale (VAS) and the Tinnitus Questionnaire (TQ) to assess tinnitus severity, the Speech, Spatial and Qualities of Hearing Scale (SSQ), and the Medical Outcomes Study Short Form 36 Health Survey Questionnaire (SF-36) to assess the quality of life, the Simplified Coping Style Questionnaire (SCSQ) to assess psychological status. The other 8 SSD patients refused cochlear implantation. Their scores of the above questionnaires were compared with those of patients received implantation. RESULTS: Six months after cochlear implantations, the tinnitus perception, loudness, and annoyance significantly decreased compared to that before implantation. In terms of quality of life and physiological status, no statistically significant changes were detected in SSQ, SF-36, and SCSQ measurements. The score of annoyance subcategory of VAS and all subcategories of SSQ of patients refused implantation were better than those of implanted patients before implantation. CONCLUSIONS: These results suggest that CIs can significantly reduce tinnitus severity. Patients refused implantation had better status in the annoyance of VAS and all subcategories of SSQ scores than those received implantation.

Long-Term Outcome of Cochlear Implantation in Children With Congenital, Perilingual, and Postlingual Single-Sided Deafness.

OBJECTIVES: We investigated the long-term outcomes of children with single-sided deafness (SSD) after cochlear implant (CI) surgery, during and after rehabilitation, and compared the results of children with congenital, perilingual, and postlingual SSD. We evaluated the impact of SSD at age at onset and duration of deafness on their performance. DESIGN: Thirty-six children with SSD treated with CI participated in the study: 20 had congenital, seven perilingual (defined: >0 to 4 years), and nine had postlingual deafness (defined as >4 years of age). Their outcome with CI were measured on both subjective and objective scales: duration of device use, speech intelligibility in noise and in quiet, bilateral hearing and localization ability, quality of life and hearing, presence and loudness of tinnitus, and hearing ability of the better hearing ear. RESULTS: After a mean follow-up time of 4.75 years, 32 of the 36 children used their CI on a regular basis. The remaining four children were nonusers. These children had congenital SSD and were older than three years at the time of CI surgery. Overall, for congenital/perilingual and postlingual SSD, speech intelligibility in noise and the Speech, Spatial and Qualities of Hearing Scale (SSQ) speech subscore were significantly improved, as were their subjective and objective localization ability and hearing-related quality of life. Children with postlingual SSD benefited from the CI with regard to speech intelligibility, SSQ speech/spatial/total score, and localization error, and children with congenital SSD showed better results with a short duration of deafness of less than 3 years compared with those with a longer deafness period. CONCLUSIONS: Cochlear implantation is a successful treatment for children with congenital/perilingual or postlingual SSD. Results largely differed with respect to the onset and duration of deafness, and better outcomes were achieved by children with postlingual SSD and with a short duration of deafness. Our data also confirmed that children with congenital SSD should be implanted with a CI within three years of age.

Cochlear Implantation in Children With a Long Average Duration of Single Sided Deafness.

OBJECTIVES: Previous research has shown the positive effects of cochlear implantation in children with a short duration of single sided deafness (SSD). This case series assessed the impact of cochlear implantation in a cohort of children with a longer average duration of SSD. METHODS: A retrospective chart review of 6 children who received a cochlear implant for SSD. The mean age at time of cochlear implantation was 14.7 years old (median = 15.5, interquartile range (IQR) = 2.5) and mean duration of hearing loss before cochlear implantation was 10.8 years (median = 11.5, IQR = 5.3). Pre- and post-operative audiometric data for aided speech perception testing, sentence recognition in quiet, sentence recognition in noise, and word recognition scores were analyzed. RESULTS: When compared to preoperative hearing aid scores a 24% significant increase in median word score and a 64% significant increase in median sentence recognition score in quiet was observed at 12 months post-op. CONCLUSION: Cochlear implantation in children with a longer duration of SSD can provide benefit to speech recognition. LAY SUMMARY: This retrospective case series of children with prolonged single sided deafness demonstrated an improvement in word and sentence recognition within 12 months of receiving a cochlear implant.

Motor Processing in Children With Cochlear Implants as Assessed by Functional Near-Infrared Spectroscopy.

Auditory-motor and visual-motor networks are often coupled in daily activities, such as when listening to music and dancing; but these networks are known to be highly malleable as a function of sensory input. Thus, congenital deafness may modify neural activities within the connections between the motor, auditory, and visual cortices. Here, we investigated whether the cortical responses of children with cochlear implants (CI) to a simple and repetitive motor task would differ from that of children with typical hearing (TH) and we sought to understand whether this response related to their language development. Participants were 75 school-aged children, including 50 with CI (with varying language abilities) and 25 controls with TH. We used functional near-infrared spectroscopy (fNIRS) to record cortical responses over the whole brain, as children squeezed the back triggers of a joystick that vibrated or not with the squeeze. Motor cortex activity was reflected by an increase in oxygenated hemoglobin concentration (HbO) and a decrease in deoxygenated hemoglobin concentration (HbR) in all children, irrespective of their hearing status. Unexpectedly, the visual cortex (supposedly an irrelevant region) was deactivated in this task, particularly for children with CI who had good language skills when compared to those with CI who had language delays. Presence or absence of vibrotactile feedback made no difference in cortical activation. These findings support the potential of fNIRS to examine cognitive functions related to language in children with CI.

Long-Term Educational and Occupational Status of Prelingually Deaf Children Who Have Received a Cochlear Implant.

OBJECTIVE: To investigate the long-term educational and occupational status of prelingually bilateral deaf children who received a cochlear implant (CI) before the age of 7, and to identify factors that influence these outcomes. STUDY DESIGN: Retrospective chart review. SETTING: Single tertiary care center. METHODS: Seventy-one children who underwent CI surgery from 2000 to 2007 were included. The latest education and occupation status and word recognition score (WRS) were analyzed. RESULTS: The mean age at the time of surgery and the current age was 3.9 and 22.4 years. The age at CI showed a negative correlation with WRS. All subjects had graduated from high school or obtained an equivalent educational qualification. General high school graduates showed a higher WRS than those who attended a special education high school. The college entrance rate of CI patients (74.6% %) was comparable to that of the general population (72.5%). Subjects who went to college had a significantly better WRS than those who did not (51.4% vs 19.3%). Excluding 30 subjects currently enrolled in college, 26 (62%) of the remaining 41 were currently employed and engaged in various vocational activities, of which most (21 out of 26, 81%) were employed through vocational training institutes, or via special recruitment policy for the disabled. CONCLUSION: The long-term use of CI in prelingually deaf children enables not only speech perception but also produces comparable levels of education and employment to those of the general population. A good WRS and supportive policy were related to these successful outcomes.

The relation between cochlear implant programming levels and speech perception performance in post-lingually deafened adults: a data-driven approach.

PURPOSE: Programming a cochlear implant (fitting) is an essential part of a user's post-implantation journey, defining how sound will be translated into electrical stimulation and aiming to provide optimal speech perception outcomes. Currently, there are no established, evidence-based guidelines for fitting cochlear implant users, leading to a high degree of variability in fitting practices, users' parameters, and probably outcomes. In this study a data-driven approach is used to retrospectively investigate the relation between cochlear implant fitting parameters and speech perception outcomes in post-lingually deafened adults. METHODS: 298 data points corresponding to fitting parameters and speech audiometry test results for the same number of adult, post-lingually deafened, experienced CI users were analyzed. Correlation analysis was performed, after which parameters from the top-scoring and bottom-scoring tertiles were compared via the Mann-Whitney-Wilcoxon u test. RESULTS: Weak correlations between dynamic range and speech audiometry outcomes were identified, having p values lower than (albeit close to) 0.05. A significant (p < 0.05) difference in electrical dynamic range (the difference between the minimum and maximum amount of current which may be delivered by each electrode) was found, with top-scoring subjects having on average a wider dynamic range. CONCLUSION: The association between dynamic range and speech perception outcomes shown in this retrospective study highlights the need for deeper investigation into evidence-driven fitting. It might be a first step in the direction of evidence-based fitting, minimizing variability in outcomes for cochlear implant users and helping mitigate the issue of unexplained low performance.