Toddler Meaningful Auditory Integration Scale Research Articles

Comprehensive Prediction Model, Including Genetic Testing, for the Outcomes of Cochlear Implantation.

Despite growing interest in the genetic contribution to cochlear implant (CI) outcomes, only a few studies with limited samples have examined the association of CI outcomes with genetic etiologies. We analyzed CI outcomes using known predictors and genetic testing results to obtain a comprehensive understanding of the impact of genetic etiologies. We retrospectively reviewed the medical records and images of patients who underwent cochlear implantation and genetic testing at a single tertiary medical institution, between May 2008 and December 2020. After excluding those whose speech test results were unavailable, and those in whom the implant was removed due to complications, such as infection or device failure, 203 patients were included in this study. The participants were categorized into adult (≥19 years), child (2-18 years), and infant (<24 months) groups. Outcomes were measured based on categories of auditory perception, monosyllable, disyllable, and sentence scores. For the infant group, the Infant-Toddler Meaningful Auditory Integration Scale score was used. Among the 203 participants, a causative genetic variant was identified in 117 (57.6%) individuals. The presence of a causative variant was significantly associated with better CI outcomes in the infant group (β = 0.23; 95% confidence interval, 0.01 to 0.47; p = 0.044), but not in the child and adult groups. In the genetically confirmed patients without cochlear malformation, genetic variants involving the spiral ganglion was a poor prognostic factor in the child group (β = -57.24; 95% confidence interval, -90.63 to -23.75; p = 0.004). The presence of known genetic etiology of hearing loss was associated with better CI outcomes in the infant group, but not in the child and adult groups. A neural-type genetic variant was a poor prognostic factor in the genetically diagnosed child subgroup without cochlear malformation. Careful genetic counseling should be performed before cochlear implantation.

Evaluation of the language development of children with cochlear implant users living in a multilingual environment

ObjectiveTo evaluate the effect of second language exposure on Turkish speaking skills in cochlear implant (CI) users. MethodsChildren living in mono and bilingual families, who underwent unilateral cochlear implant due to congenital severe to profound, or profound hearing loss, were examined. The fifty six children with a chronological age younger than 8 and a language age of 2–6 years were included the study. The Denver II Developmental Screening Test was used for identifying of pre-implant speech and development. The duration of implant use was also documented. Languages spoken at the patients' homes were divided into 4 categories: T/K: Predominantly Turkish/Kurdish, K/T: Predominantly Kurdish/Turkish, T/T: Just Turkish, A/T: Predominantly Arabic/Turkish. The CAP (Categories of Auditory Performance), SIR (Speech Intelligibility Rating), The Turkish Version Test of The Early Language Development (TELD-3:T),. The IT-MAIS (Infant Toddler Meaningful Auditory Integration Scale), MUSS (Meaningful Use of Speech Scale) were used to evaluate the language development of the patients. Changes in IT-MAIS and MUSS scores in the last two years were also evaluated. ResultsThe all children received their implants before age of 6 years. The average duration of cochlear implant use of children was 38,43 ± 11,64 months. The mean pre-implant speech age was 6,8 ± 2,13 months. There was no significant difference between the groups in terms of the CAP and SIR scores. In TELD-3:T scores, there was no significant difference between the groups except for the receptive raw scores. The Arabic/Turkish group had significantly lower receptive raw scores. This group also had significantly lower mother educational level.In IT-MAIS and MUSS scores, no significant difference was found between the groups. ConclusionsOur study supports the opinion that exposure to a second language at home does not affect the acquisition of the language of education in children using CI.

Outcomes of Acoustic and Linguistic Performances Following Cochlear Implantation in Large Vestibular Aqueduct Syndrome (LVAS).

This study aims to collate the outcomes in acoustic and linguistic performances after cochlear implantation in LVAS. In a hospital-based prospective interventional study, seven prelingual children with bilateral profound sensorineural hearing loss(SNHL) with LVAS were recruited. They underwent unilateral cochlear implantation between December 2013 and December 2015 in the Department of Otorhinolaryngology and Head Neck Surgery at a tertiary care center. Outcomes of auditory and speech performances were assessed in the form of revised categories of auditory perception (CAP), infant toddler meaningful auditory integration scale (IT-MAIS) and speech intelligibility rating (SIR) scores preoperatively, at one and two years follow up. The mean age of implantation was 4years. The median revised CAP, IT-MAIS, and SIR scores after one and two years of follow-up were 6, 27, 3, and 11, 36, 5 respectively. The mean follow-up duration was 21.3months. Pre op median revised CAP, IT-MAIS, and SIR scores of 0, 1, 1 showed improvement to 11, 36, 5 at two years follow up which was statistically significant. Hence, we conclude that good functional outcomes post-operatively advocate the significance of cochlear implantation in LVAS.

Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder

ObjectiveThe objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6). Study designCase series. SettingSingle tertiary care academic medical center.Subjects and Methods.All pediatric patients with a confirmed diagnosis of ANSD on Auditory Brainstem Response (ABR) testing who presented to a single tertiary medical center between September 2008 and September 2018 were included. Only patients that underwent Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS) and/or Ling 6 Sound Test (Ling 6) were included in the study. Audiologic testing performed after cochlear implantation was excluded. Results60 pediatric patients with ANSD were analyzed. There were 10 patients included in the study with documented hearing aid use who underwent IT-MAIS and/or Ling 6 testing. Average IT-MAIS score improved by 20.4% after initial or extended trial of amplification. Similarly, average Ling 6 score improved from 3.6 to 4.8 after initial or extended trial of amplification. The four patients who did not receive amplification had higher average IT-MAIS and Ling 6 scores. ConclusionIn most children with ANSD, IT-MAIS and Ling 6 Sound Test scores improved with initial hearing aid use and over time with extended hearing aid use. Long-term prospective, multi-institutional studies are needed to determine the impact of the natural history of ANSD, comorbidities, and socioeconomic variables on auditory function testing results in children with ANSD using hearing aids.

Auditory and language skills in children with auditory brainstem implants

ObjectiveThe aim of this study is to characterize the development of auditory and language skills in children during the first 3 years of auditory brainstem implant (ABI) use. MethodIt is a retrospective longitudinal analysis of auditory and language skills in 12 children and pre-adolescents with pre-lingual deafness following ABI surgery (mean age at surgery: 4 years; age range: 2–11 years). Responses were analyzed aboutInfant Toddler Meaningful Auditory Integration Scale (IT-MAIS), MAIS, and Meaningful Use of Speech Scale (MUSS) at 1, 3, 6, 12, 18, 24, and 36 months after ABI activation. ResultsMaximum IT-MAIS/MAIS and MUSS scores after 3 years of ABI use were 45.35% and 35.28%, respectively. ConclusionPediatric patients exhibit slow progressive development of auditory and language skills following ABI activation.

Pediatric Auditory Brainstem Implant Users Compared With Cochlear Implant Users With Additional Disabilities.

To evaluate long-term language development in children with prelingual deafness who received auditory brainstem implants (ABIs) compared with children who received cochlear implants (CIs) at the same hospital. Additional non-auditory disabilities were taken into account. Retrospective cohort study. Tertiary referral center. Ten children with bilateral malformations of the cochlea and/or cochlear nerve who received ABIs, including seven with additional disabilities, and 147 children with CIs as a reference group, including 22 children with additional disabilities. ABIs were implanted at 1.3 to 6.2 years of age. Follow-up ranged from 1.1 to 7.7 years. Receptive and expressive language abilities were assessed using the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Categories of Auditory Performance (CAP), the Meaningful Use of Speech Scale (MUSS), and the Speech Intelligibility Rate (SIR). Of the 10 children with ABIs, seven had long-term follow-up data. Within 1 year, six of the seven children with ABIs could identify sounds, respond to speech, and use their voice to attract attention. Language skills developed at a slower rate than in children with CIs and reached the same competence level when additional disabilities were absent. These language skills matched, on average, those of children with CIs with additional disabilities. For deaf children with bilateral inner ear malformations, ABIs provide satisfactory auditory input. Children with ABIs are able to develop receptive and expressive language skills comparable to those of children with CIs with additional disabilities. Using this knowledge, preoperative parent counselling can be refined.

Effects of bimodal intervention on the development of auditory and speech ability in infants with unilateral cochlear implantation

Objective: To explore the effects of bimodal intervention on the development of auditory and speech ability in the infants with unilateral cochlear implantation(CI). Methods: Total 35 bilateral profound sensorineural hearing loss infants with unilateral CI, aged 0.7 to 2.8 years old, were selected. The subjects were divided into two groups: the group with unilateral CI(cochlear implant alone, n= 15), and the bimodal group with CI and contralateral fitting hearing aid(n= 20). Their auditory and speech abilities were estimated at the different time points after switch-on(the 0th, 0.5th, 1st, 3rd, 6th, 12th, 18th, and 24th month, respectively) using Infant Toddler-Meaningful Auditory Integration Scale(IT-MAIS), Meaningful Use of Speech Scale(MUSS), Categories of Auditory Performance(CAP), and Speech Intelligibility Rating(SIR) scores. Results: The IT-MAIS scores of bimodal group after switch-on were higher than unilateral CI group(the 0.5th, 1st, 3rd, 6th, 12th, and 18th month), the statistical significances were identified at the 0.5th, 1st, 3rd, 6th, and 12th month, respectively(P<0.05). The CAP scores of bimodal group before CI operation and after switch-on(the 0.5th, 1st, 3rd, 6th, 12th, 18th and 24th month)were higher than unilateral CI group, the statistical significances were seen at the 3rd, 6th, 12th, 18th and 24th month after switch-on(P<0.05). The MUSS scores of bimodal group after switch-on were higher than unilateral CI group(the 1st, 3rd, 6th, 12th, 18th and 24th month), the statistical significances were found at the 12th, 18th and 24th month, respectively(P<0.05). The SIR scores of bimodal group after switch-on were higher than unilateral CI group(the 3rd, 6th, 12th, 18th and 24th month), and significant differences appeared at the 12th, 18th and 24th month after switch-on(P<0.05). Conclusion: Bimodal intervention could be helpful to the development of auditory and speech ability of infants.

The effects of stimulus rate on ABR morphology and its relationship to P1 CAEP responses and auditory speech perception outcomes in children with auditory neuropathy spectrum disorder: evidence from case reports

Objective: Auditory neuropathy spectrum disorder (ANSD) affects approximately 5–15% of children with sensorineural hearing loss (SNHL). ANSD is characterized by the presence of otoacoustic emissions (OAE) and an absent or abnormal auditory brainstem response (ABR). The purpose of this study was to investigate the prognostic value of slow-rate ABR in predicting the auditory cortical development and auditory speech perception outcomes in case studies of children with ANSD.Design: ABR waveform characteristics were collected at slow stimulation rates (5.1 clicks/second) and a fast stimulation rates (>11–31.1 clicks/second, rates typically used in a clinical setting) in three case reports of children with ANSD. P1 CAEP responses and measures of auditory speech perception using the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were also collected in these children. Retrospective analysis was performed to evaluate the prognostic value of slow- versus fast-rate ABR in predicting P1 CAEP responses and auditory speech perception outcomes in these children.Study sample: Participants included case reports of three paediatric participants with a clinical diagnosis of ANSD.Results: Slow-rate ABR did not elicit significant improvements in waveform morphology compared to fast-rate ABR. P1 CAEP results were present in 2 out of 3 cases and were consistent with auditory speech perception outcomes.Conclusions: Even when ABR stimulation rates were slowed, ABR responses in these children with ANSD did not display any characteristic or replicable pattern, and ABR responses were not predictive of cortical auditory maturation or behavioural performance. In contrast, P1 CAEP responses provided valuable information regarding the maturational status of the auditory cortex and P1 CAEP responses were consistent with behavioural measures of auditory speech perception. Overall, results highlight the high prognostic value of P1 CAEP testing when used in conjunction with behavioural measures of auditory speech perception in children with ANSD.

Benefits of computerized auditory training software for Kannada speaking children with hearing impairment – parent’s perspective

Purpose: The present study attempted to develop computer based auditory training software focusing on Kannada speaking children with hearing impairment (HI). In addition, it was also attempted to determine if there are any improvements in listening with and without the developed software.Materials and Methods: This was determined based on pre and post therapy changes in the parent’s perspective, and also on questionnaires. The software consisted of five modules namely, auditory awareness, auditory identification, auditory discrimination, auditory memory and sequencing and auditory comprehension. It was administered on ten individuals with HI who also received listening training, while he control group received only listening training.Results: The results of the study showed that there was an improvement in listening skills based on infant toddler-meaningful auditory integration scale (IT-MAIS) and parents’ evaluation of aural/oral performance of children (PEACH) questionnaires administered on parents of children with HI. A detailed interview of parents also suggested that there was an enhanced auditory attention and improvement in overall listening skills.Conclusions: Thus, the present software can act as a supplement to the usual listening training in Kannada speaking children with HI.

Auditory neuropathy spectrum disorder: a new approach to hearing aid fitting

ObjectiveThe aim of this study was to explore the outcome of hearing aid amplification in children with auditory neuropathy spectrum disorder (ANSD) using speech P1 cortical auditory evoked potential (P1-CAEP).Study designForty-five children were divided into three groups: the ANSD group (n=15), the sensorineural hearing loss (SNHL) group (n=15), and the normal hearing group (n=15). The ANSD group had a mean age of 48.2 (±29.4) months and included children with moderate-to-severe hearing loss, a history of bilateral hearing aid use for at least 6 months, and absence of comorbid disorders. The SNHL group was closely matched to the ANSD group. Verification of hearing aids was carried out twice with 6 months of interval and included evaluation of aided sound field and P1-CAEP, and evaluation using the Arabic version of the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS). The P1-CAEPs were elicited using the temporally modified synthetic ‘ba’ and ‘da’ syllables.ResultsIn the initial evaluation, 80% of ANSD children showed P1 response to the ‘ba’ stimulus and 87% of children to the ‘da’ stimulus. Only one child from the SNHL group did not show P1-CAEP responses to the ‘da’ stimulus. The latency of P1 was prolonged in both groups compared with the normal hearing loss group. At 6-month evaluation, the P1-CAEP latencies improved equally in the ANSD and SNHL groups. Children with absent responses persisted to have absent responses. The mean IT-MAIS scores was initially 45.5 (±20) in the ANSD group and 79 (±9) in the SNHL group and increased after 6 months in both groups. The IT-MAIS scores negatively correlated with the P1-CAEP latency in the two evaluation sessions and positively correlated with the age of hearing aid fitting.ConclusionAround 50% of ANSD children demonstrated benefit from amplification. They showed evidence of normal central auditory maturation and progress in auditory skill development. Longitudinal P1 recording is recommended in the comprehensive audiological test battery in ANSD population using temporally modified speech stimuli.

Percepção de fala em deficientes auditivos pré-linguais com desordem do espectro da neuropatia auditiva usuários de aparelho auditivo de amplificação sonora

To analyze speech perception in children with pre-lingual hearing loss with auditory neuropathy spectrum disorder users of bilateral hearing aid. This is a descriptive and exploratory study carried out at the Research Center Audiological (HRAC/USP). The study included four children aged between 8 years and 3 months and 12 years and 2 months. Lists of monosyllabic words, two syllables, nonsense words and sentences, the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS) and the Meaningful Use of Speech Scale (MUSS), hearing, and language categories were used. All lists were applied in acoustic booth, with speakers, in free field, in silence. The results showed an average 69.5% for the list of monosyllabic words, 87.75% for the list of two-syllable words, 89.92% for the list of nonsense syllables, and 92.5% for the list of sentences. The therapeutic process that includes the use of bilateral hearing aid was extremely satisfactory, since it allowed the maximum development of auditory skills.

Deeper insertion of electrode array result in better rehabilitation outcomes – Do we have evidence?

ObjectiveTo study the outcome analysis in cochlear implantees in relation to depth of insertion. Methods30 patients of non-syndromic congenital profound hearing loss in the age range of 2–12 years received cochlear implantation by a posterior tympanotomy round window approach. Depth of insertion was calculated using post-operative X-rays (modified Stenver's view) and categorized into four groups, viz. fair insertion (Group A <180°), good insertion (Group B 180–<270°), very good insertion(Group C 270–360°), excellent insertion (Group D >360°). The outcome analysis of each implantee was carried out in a follow up interval of every 3 months using Meaningful Auditory Integration Scale (MAIS), Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), Category of Auditory Performance (CAP), and Speech Intelligibility Rating (SIR). ResultsOverall 30, 29, 25, and 22 patients have completed 3, 6, 9, and 12 months follow up respectively. The MAIS scores in Group C were significantly better than Group B at 6, 9, and 12 months (P<0.05). The mean CAP score of Group C was more than rest of the groups with significant difference between Group C and Group D at 12 months (P<0.05). The mean SIR scores were maximum in Group C with significant difference between Group C and Group B at 9 and 12 months (P<0.05). ConclusionThe study demonstrates that insertion from 270° to 360° gives optimum hearing outcomes as compared to deeper insertion, although larger sample and long term follow-up is warranted for definite conclusions.

Auditory Cortical Maturation in a Child with Cochlear Implant: Analysis of Electrophysiological and Behavioral Measures.

The purpose of this study was to longitudinally assess the behavioral and electrophysiological hearing changes of a girl inserted in a CI program, who had bilateral profound sensorineural hearing loss and underwent surgery of cochlear implantation with electrode activation at 21 months of age. She was evaluated using the P1 component of Long Latency Auditory Evoked Potential (LLAEP); speech perception tests of the Glendonald Auditory Screening Procedure (GASP); Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS); and Meaningful Use of Speech Scales (MUSS). The study was conducted prior to activation and after three, nine, and 18 months of cochlear implant activation. The results of the LLAEP were compared with data from a hearing child matched by gender and chronological age. The results of the LLAEP of the child with cochlear implant showed gradual decrease in latency of the P1 component after auditory stimulation (172 ms–134 ms). In the GASP, IT-MAIS, and MUSS, gradual development of listening skills and oral language was observed. The values of the LLAEP of the hearing child were expected for chronological age (132 ms–128 ms). The use of different clinical instruments allow a better understanding of the auditory habilitation and rehabilitation process via CI.

Current trends in pediatric cochlear implant candidate selection and postoperative follow-up.

In this article, the authors describe current pediatric cochlear implant (CI) assessment and postoperative scheduling protocols in the United States. A survey was conducted in 2 phases. Response rates were similar between Phase I (10%) and Phase II (13%). Across phases, nearly all respondents reported assessing speech perception both preoperatively and postoperatively. The most frequently used parent questionnaires were the Infant-Toddler Meaningful Auditory Integration Scale (Robbins, Renshaw, & Berry, 1991), the Meaningful Auditory Integration Scale (Robbins et al., 1991), and LittlEARS (Kuehn-Inacken, Weichboldt, Tsiakpini, Coninx, & D'Haese, 2003). The most commonly used speech perception measure for children <23 months of age was the Early Speech Perception Test-Low Verbal (ESP-LV; Moog & Geers, 1990). The most commonly used measures for children 24-35 months of age were the ESP-LV, the Northwestern University of Children's Perception of Speech (Elliot & Katz, 1980), and the Mr. Potato Head task (Robbins, 1993). For children >36 months of age, there was a wide variety of speech perception tests utilized. Patient follow-up visits were weekly or biweekly immediately following CI activation, then every 3 months for the remainder of the 1st year. After the 1st year, most children were seen semiannually. Although trends emerged, there is a lack of consistency in the selection of speech perception measures utilized across centers for children <36 months of age. The development of a working group to establish a standard minimum pediatric test battery (similar to the adult Minimum Speech Test Battery) would promote uniformity in clinical protocols used to assess children who receive CIs.

Evaluation of the benefit with the use of the sound amplification inchildren and teenagers

Objective: Evaluate the proportionate benefit by the sound amplification in children and teenagers attended by the program of hearing health attention by the Federal University of Santa Maria (UFSM). Method: Participated from these study 63 individuals with age between 3 and 14 years and their family. For the evaluate the benefit proportionate by the sound amplification was applied the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), for children with less than 4 years old; the Meaningful Auditory Integration Scale (MAIS), for infants from 4 to 7 years and the questionnaire of evaluation from the benefit in infants and young, for the individuals from 8 to 14 years. Results: Only one individual could be evaluated through the MAIS scale, and the average punctuation found in the evaluation of the hearing behaviors was of 19,9. Were evaluated 52 individuals through the questionnaire of evaluation of benefit in infants and teenagers, and the positive answers related to the benefit proportionate by the prosthesis in different situations were the most found in all items. Conclusions: Was verified the benefit proportionate by the sound amplification, in different situations of daily life, such as from the patient's perspective as from his family. In some cases, where were found little or none benefit, there were influence of others clinical alterations, besides the hearing loss, influencing the effective use of the hearing aids.

Production of Infant Scale Evaluation (PRISE) in Italian normal hearing children: A validation study

ObjectivesParental questionnaires are important tools in the evaluation of auditory and language skills of very young children affected by sensorineural hearing loss. One of these instruments is the Production on Infant Scale Evaluation (PRISE). The purposes of this study were to adapt and validate the PRISE on Italian children with normal hearing; and to obtain normative data. MethodsA back translation technique was used to adapt the Italian version of PRISE. The PRISE was submitted to parents of 234 normal children aged between 3 and 18 months of life. All of them passed local universal newborn hearing screenings and they presented no audiological risk factors. ResultsThe PRISE internal reliability (Cronbach's alpha) was 0.87. Split-half reliability indexes were λ4=0.89 and λ6=0.89. Corrected item-total correlation coefficients were significant for all items. The correlation of PRISE with a modified Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), collected for convergent validity measurement purposes, was good (r=0.743). A positive correlation of PRISE scores with age was found, reflecting on the age-dependence of pre-verbal skills. ConclusionThese findings demonstrate high reliability and convergent validity of the Italian PRISE version. This questionnaire constitutes a robust tool for assessing early language development in infants and toddlers with normal hearing. It seems particularly sensitive to the normal language development in the first years of life, which can be very useful for early rehabilitation of hearing loss.

Central auditory maturation and behavioral outcome in children with auditory neuropathy spectrum disorder who use cochlear implants

Objective: We examined cortical auditory development and behavioral outcomes in children with ANSD fitted with cochlear implants (CI). Design: Cortical maturation, measured by P1 cortical auditory evoked potential (CAEP) latency, was regressed against scores on the infant toddler meaningful auditory integration scale (IT-MAIS). Implantation age was also considered in relation to CAEP findings. Study sample: Cross-sectional and longitudinal samples of 24 and 11 children, respectively, with ANSD fitted with CIs. Results: P1 CAEP responses were present in all children after implantation, though previous findings suggest that only 50–75% of ANSD children with hearing aids show CAEP responses. P1 CAEP latency was significantly correlated with participants’ IT-MAIS scores. Furthermore, more children implanted before age two years showed normal P1 latencies, while those implanted later mainly showed delayed latencies. Longitudinal analysis revealed that most children showed normal or improved cortical maturation after implantation. Conclusion: Cochlear implantation resulted in measureable cortical auditory development for all children with ANSD. Children fitted with CIs under age two years were more likely to show age-appropriate CAEP responses within six months after implantation, suggesting a possible sensitive period for cortical auditory development in ANSD. That CAEP responses were correlated with behavioral outcome highlights their clinical decision-making utility.

Cochlear implantation in children with "CHARGE syndrome": surgical options and outcomes

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with "CHARGE syndrome" submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy-posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid's audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.

The development of auditory skills in infants with isolated Large Vestibular Aqueduct Syndrome after cochlear implantation

Objective The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development. Method 435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant–Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills. Results The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B. Conclusion Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS.

C124 Environmental sounds identification in children with cochlear implant

Parental questionnaires are important tools in the evaluation of auditory and language skills of very young children affected by sensorineural hearing loss. One of these instruments is the Production on Infant Scale Evaluation (PRISE). The purposes of this study were to adapt and validate the PRISE on Italian children with normal hearing; and to obtain normative data.A back translation technique was used to adapt the Italian version of PRISE. The PRISE was submitted to parents of 234 normal children aged between 3 and 18 months of life. All of them passed local universal newborn hearing screenings and they presented no audiological risk factors.The PRISE internal reliability (Cronbach's alpha) was 0.87. Split-half reliability indexes were λ4 = 0.89 and λ6 = 0.89. Corrected item-total correlation coefficients were significant for all items. The correlation of PRISE with a modified Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), collected for convergent validity measurement purposes, was good (r = 0.743). A positive correlation of PRISE scores with age was found, reflecting on the age-dependence of pre-verbal skills.These findings demonstrate high reliability and convergent validity of the Italian PRISE version. This questionnaire constitutes a robust tool for assessing early language development in infants and toddlers with normal hearing. It seems particularly sensitive to the normal language development in the first years of life, which can be very useful for early rehabilitation of hearing loss.