cochlear implantation in a child with auditory neuropathy spectrum disorder

Objective To report outcomes of cochlear implantation (CI) in a child with auditory neuropathy spectrum disorder (ANSD) and to provide preliminary clinical evidence of the efficacy of CI in ANSD patien...     

Vestibular function in children with auditory neuropathy spectrum disorder

Objective

Children with auditory neuropathy spectrum disorder (ANSD) account for about 10% of paediatric patients referred for cochlear implantation. Vestibulopathy may be associated with ANSD, and may have implications when formulating management plans in this patient group. We wanted to determine the incidence and predictive factors for vestibulopathy in this patient group to guide vestibular testing in this patient population, and give insight to the aetiology of ANSD.

Methods

We reviewed the outcomes of vestibular function testing in a cohort of paediatric patients with ANSD.

Results

Probable or definite vestibulopathy was seen in 42% of patients who were tested. Vestibulopathy was associated with medical co-morbidities, but was not associated with imaging findings.

Conclusions

Vestibulopathy is relatively prevalent in this patient group, and should be considered when planning the investigation and management of children with ANSD.     

Identification of novel OTOF compound heterozygous mutations by targeted next-generation sequencing in a Chinese patient with auditory neuropathy spectrum disorder

Objectives

The molecular causes of auditory neuropathy spectrum disorder (ANSD) are not well known. Identification of the pathogenic mutations underlying nonsyndromic ANSD is difficult because of its extremely heterogeneous trait. The aim of the present study was to identify the genetic etiology of a single Chinese patient diagnosed with congenital ANSD by targeted next-generation sequencing.

Methods

Targeted next-generation sequencing of 79 known deafness genes was performed in a child that was clinically diagnosed with ANSD and received cochlear implantation. Candidate pathogenic variants were confirmed by Sanger sequencing. Post-implantation outcome were evaluated in a 40 months span.

Results

Novel compound heterozygous mutations p.R1583H/p.Q1883X in OTOF were identified as the pathogenic cause of the patient, correlated with a good post-implantation outcome in terms of sound detection and communication skills.

Conclusion

Targeted next-generation sequencing is effective for molecular diagnosis of ANSD and may provide important information for clinical management of this disease.     

The effect of age at cochlear implantation outcomes in Saudi children

Objectives

To determine for the effect of age (late versus early age) on the cochlear implant outcomes; in terms of language development, auditory skills, speech perception, and production outcomes).

Methods

67 children were included in the study out of 93 implanted cases in the study period. Children were classified into 2 groups according to age at time of implantation. Group 1 contained 43 children who were implanted before the age of 5 years. Group 2 contained 24 children who were implanted after the age of 5 years. All children were evaluated pre-operatively and at 3, 6, 12, 24 months device experience using the language screening test, Standardized Arabic Language test, Listening Progress Profile (LiP Test), the Monosyllabic-Trochee-polysyllabic Test (MTP), and the meaningful Auditory Integration Scale (MAIS) Test. Charts with incomplete data were excluded.

Results

Only 67 children had complete data out of 93 patients. The mean age (in months) for Group 1 was (43.37 ± 8.63) and for Group 2 was (70.38 ± 9.97) at time of implantation. Significantly higher mean values were detected for Group 2 in comparison to Group 1 in the pre-operative period. No significant difference was detected after 2 years evaluation using the test battery for language development and auditory skills.

Conclusions

Children who were implanted under the 5 years of age had a better outcome in the form of better auditory skills, speech perception, and language production. Limited resources and the absence of a national hearing screening program in Saudi Arabia result in the late presentation of children for evaluation and intervention of hearing problem; this late intervention reduces the benefits the late – implanted children derive from cochlear implantation.     

Auditory and speech performance in cochlear implanted ANSD children

Background: Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. Cochlear implantation was initially not recommended for ANSD children, later studies showed variable outcomes among those subjects.

Objective: To assess the auditory and speech performance of cochlear implanted children with auditory neuropathy spectrum disorder (ANSD) and to compare these results to those obtained from cochlear implanted children with sensorineural hearing loss (SNHL)

Material and methods: 18 cochlear implanted children with ANSD and 40 cochlear implanted children with SNHL were included in this study. Auditory and speech performance results were compared across both subject groups using the Category of Auditory Performance (CAP) and Speech Intelligibility Rate (SIR) tests, with measurements recorded one year post implantation.

Results: Cochlear implanted children with ANSD showed clinically significant improvements that were comparable to those observed from cochlear implanted subjects without ANSD.

Conclusions: Children with ANSD benefit from early cochlear implantation and can reach similar auditory and speech performance results as that achieved by children without ANSD.     

Auditory development after placement of bone-anchored hearing aids Softband among Chinese Mandarin-speaking children with bilateral aural atresia

Objective

To evaluate auditory developments of Chinese Mandarin-speaking children with congenital bilateral aural atresia after using Bone-anchored hearing aids (Baha) Softband and to compare them with matched peers with normal hearing.

Method

Sixteen patients (age ranging from 3 months to 6 years) with bilateral aural atresia and 29 children with normal hearing (age ranging from 8 months to 6 years) were studied. Auditory development was assessed at three time intervals: baseline, 6 months and 12 months. Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was conducted for children under 4 years old; Meaningful Auditory Integration Scale (MAIS), Chinese Mandarin lexical neighborhood test (MLNT) and sound field pure tone audiometry (PTA) were used for children of 4–6 years old.

Results

Mean IT-MAIS scores were 41 ± 24%, 60 ± 22% and 73 ± 7%, respectively at three time intervals. Mean MAIS scores were 66 ± 7%, 90 ± 5%, and 99 ± 2%. Mean speech discrimination scores at the three time intervals were 74 ± 19%, 86 ± 16%, and 95 ± 4% with the easy disyllabic (D-E) list; 48 ± 18%, 73 ± 15%, and 81 ± 7% with the hard disyllabic (D-H) list; 55 ± 17%, 74 ± 22%, and 83 ± 14% with the easy monosyllabic(M-E) list; and 31 ± 14%, 61 ± 15%, and 71 ± 13% with the hard monosyllabic (M-H) list.

Conclusions

Baha Softband is suitable for infants and young children with bilateral atresia. Results from these auditory development testing are encouraging. Baha Softband should be used as a bridge for surgical implantations when the temporal bone is thick enough.     

Evaluation of auditory development in infants and toddlers who received cochlear implants under the age of 24 months with the LittlEARS Auditory Questionnaire

Background and Aims

Newborn hearing screening and early intervention for congenital hearing loss have created a need for tools assessing the hearing development of very young children. A multidisciplinary evaluation of children's development is now becoming standard in clinical practice, though not many reliable diagnostic instruments exist. For this reason, the LittlEARS^® Auditory Questionnaire (LEAQ) was created to assess the auditory skills of a growing population of infants and toddlers who receive hearing instruments. The LEAQ relies on parent report, which has been shown to be a reliable way of assessing child development. Results with this tool in a group of children who received very early cochlear implantation are presented.

Methods

The LEAQ is the first module of the LittlEARS^® comprehensive test battery for children under the age of two who have normal hearing (NH), cochlear implants (CIs) or hearing aids (HAs). The LEAQ is a parent questionnaire comprised of 35 “yes/no” questions which can be completed by parents in less than 10 min. Sixty-three children who received unilateral CIs at a young age were assessed longitudinally and their performance was compared to that of a NH group.

Results

All CI children reached the maximum possible score on the LEAQ on average by 22 months of hearing age, i.e. 38 months of chronological age. In comparison, the NH group reached the maximum score by 24 months of age demonstrating that auditory skills of CI children often develop quicker than those of NH children. In the two comparison groups of children aged (a) younger and older than 12 months, and (b) between 6-9 and 21-24 months at first fitting, the early implanted children reached the highest scores faster than the later implanted children. Furthermore, three children with additional needs were tested. They showed slower growth over time but also received benefits from early implantation.

Conclusions

The LEAQ is a quick and effective tool for assessing auditory skills of very young children with or without hearing loss. In our study, the auditory skills of children with CI progressed very quickly after implantation and were comparable with those of NH peers.     

Auditory brainstem implant outcomes and MAP parameters: report of experiences in adults and children

The auditory brainstem implant (ABI) was first developed to help neurofibromatosis type 2 patients. Recently, its use has been recently extended to adults with non-tumor etiologies and children with profound hearing loss who were not candidates for a cochlear implant (CI). Although the results has been extensively reported, the stimulation parameters involved behind the outcomes have received less attention.

Objective

The aim of this study is to describe the audiologic outcomes and the MAP parameters in ABI adults and children at our center.

Methods

Retrospective chart review. Five adults and four children were implanted with the ABI24M from September 2005 to June 2009. In the adult patients, four had Neurofibromatosis type 2, and one had postmeningitic deafness with complete ossification of both cochleae. Three of the children had cochlear malformation or dysplasia, and one had complete ossified cochlea due to meningitis. Map parameters as well as the intraoperative electrical auditory brainstem responses were collected. Evaluation was performed with at least six months of device use and included free-field hearing thresholds, speech perception tests in the adult patients and for the children, the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and (ESP) were used to evaluate the development of auditory skills, besides the MUSS to evaluate.

Results

The number of active electrodes that did not cause any non-auditory sensation varied from three to nineteen. All of them were programmed with SPEAK strategy, and the pulse widths varied from 100 to 300 μs. Free-field thresholds with warble tones varied from very soft auditory sensation of 70 dBHL at 250 Hz to a pure tone average of 45 dBHL. Speech perception varied from none to 60% open-set recognition of sentences in silence in the adult population and from no auditory sensation at all to a slight improvement in the IT-MAIS/MAIS scores.

Conclusion

We observed that ABI may be a good option for offering some hearing attention to both adults and children. In children, the results might not be enough to ensure oral language development. Programming the speech processor in children demands higher care to the audiologist.     

Beneficial auditory and cognitive effects of auditory brainstem implantation in children

Conclusion: This preliminary study demonstrates the development of hearing ability and shows that there is a significant improvement in some cognitive parameters related to selective visual/spatial attention and to fluid or multisensory reasoning, in children fitted with auditory brainstem implantation (ABI). The improvement in cognitive paramenters is due to several factors, among which there is certainly, as demonstrated in the literature on a cochlear implants (CIs), the activation of the auditory sensory canal, which was previously absent. The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities with associated cognitive deficits should not be excluded from ABI implantation. Objectives: The indications for ABI have been extended over the last 10 years to adults with non-tumoral (NT) cochlear or cochlear nerve abnormalities that cannot benefit from CI. We demonstrated that the ABI with surface electrodes may provide sufficient stimulation of the central auditory system in adults for open set speech recognition. These favourable results motivated us to extend ABI indications to children with profound hearing loss who were not candidates for a CI. This study investigated the performances of young deaf children undergoing ABI, in terms of their auditory perceptual development and their non-verbal cognitive abilities. Patients and methods: In our department from 2000 to 2006, 24 children aged 14 months to 16 years received an ABI for different tumour and non-tumour diseases. Two children had NF2 tumours. Eighteen children had bilateral cochlear nerve aplasia. In this group, nine children had associated cochlear malformations, two had unilateral facial nerve agenesia and two had combined microtia, aural atresia and middle ear malformations. Four of these children had previously been fitted elsewhere with a CI with no auditory results. One child had bilateral incomplete cochlear partition (type II); one child, who had previously been fitted unsuccessfully elsewhere with a CI, had auditory neuropathy; one child showed total cochlear ossification bilaterally due to meningitis; and one child had profound hearing loss with cochlear fractures after a head injury. Twelve of these children had multiple associated psychomotor handicaps. The retrosigmoid approach was used in all children. Intraoperative electrical auditory brainstem responses (EABRs) and postoperative EABRs and electrical middle latency responses (EMLRs) were performed. Perceptual auditory abilities were evaluated with the Evaluation of Auditory Responses to Speech (EARS) battery – the Listening Progress Profile (LIP), the Meaningful Auditory Integration Scale (MAIS), the Meaningful Use of Speech Scale (MUSS) – and the Category of Auditory Performance (CAP). Cognitive evaluation was performed on seven children using the Leiter International Performance Scale – Revised (LIPS-R) test with the following subtests: Figure ground, Form completion, Sequential order and Repeated pattern. Results: No postoperative complications were observed. All children consistently used their devices for >75% of waking hours and had environmental sound awareness and utterance of words and simple sentences. Their CAP scores ranged from 1 to 7 (average =4); with MAIS they scored 2–97.5% (average =38%); MUSS scores ranged from 5 to 100% (average =49%) and LIP scores from 5 to 100% (average =45%). Owing to associated disabilities, 12 children were given other therapies (e.g. physical therapy and counselling) in addition to speech and aural rehabilitation therapy. Scores for two of the four subtests of LIPS-R in this study increased significantly during the first year of auditory brainstem implant use in all seven children selected for cognitive evaluation.     

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.     

听神经病谱系障碍患者人工耳蜗术后长期听力康复效果分析

目的 探讨听神经病谱系障碍患者人工耳蜗植入术后长期听力康复效果.方法 回顾性分析46例经我院诊断为听神经病谱系障碍的患者并行人工耳蜗植入(Cochlear Implantation,CI)患者的临床资料、听力及颞骨影像检查结果.对其中12例患儿通过听觉行为分级标准量表(CAP)、言语可懂度分级标准量表(SIR)两种问卷...     

Acquisition of early auditory milestones with a cochlear implant

Objective

Speech acquisition after cochlear implant is a long process. Various studies have followed the auditory milestones in the early period after implantation. The aim of the present study was to track the development of hearing skills in the early period after cochlear implantation and evaluate which factors influence the process.

Methods

195 records of children implanted in the Hadassah Medical Center were examined retrospectively. Data on etiology, age at implantation and type of implant were collected. In addition, information on the rate of progress was measured: the first time that there was detection and identification of Ling sounds, the first time it was possible to obtain SDT (speech detection threshold), SRT (speech reception threshold) and an audiogram, and the first accurate repetition of VCV (vowel consonant vowel) sounds.

Results

Results show a consistent pattern of auditory milestone acquisition similar to that of normal development, from milestones that do not require decoding beginning with SDT, detection of Ling sounds followed by an audiogram which requires cooperation, to tasks that involve decoding starting with SRT and repetition of Ling sounds and finally VCV repetition.The children implanted before 24 months of age achieved the auditory milestones later than children implanted between 2 and 6 years, apparently since these tasks involve cognitive abilities which are not yet developed in the youngest children.Previous hearing experience improved the rate of acquisition of the auditory milestones and progress was faster in the second implanted ear compared to the first implanted ear.

Conclusion

More research is needed to address the relationship between acquisition of early auditory milestones and performance with the cochlear implant later on in life.     

The role of the efferent auditory system in developmental dyslexia

Objective

To assess the role of the efferent auditory system by inhibition of contralateral otoacoustic emission in dyslexic children with auditory processing disorders.

Methods

The study sample was 34 children: 17 with dyslexia and 17 age-matched controls. Sensitive speech tests (low-pass filtered, time-compressed, distorted and dichotic) were performed to assess coexisting auditory processing disorder. Distortion-product otoacoustic emission (DPOAE) values were measured in basal condition and with contralateral broadband noise signal delivered via an earphone transducer at 60 dB SPL.

Results

The lower scores at sensitive speech testing confirmed the association of an auditory processing disorder in the dyslexic children. DPOAE values were significantly attenuated by contralateral inhibition only in the control group (p = 0.001; dyslexics, p = 0.19); attenuation was not significant at any frequency in the dyslexic group.

Conclusions

The differences in DPOAE attenuation between the groups, although not statistically significant, suggest alterations in the auditory efferent system in the dyslexic population. These alterations may affect language perception. If confirmed in further studies with larger samples, these results could provide insight into a possible pathophysiological background of dyslexia.     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

The Hebrew and the Arabic version of the LittlEARS® auditory questionnaire for the assessment of auditory development: results in normal hearing children and children with cochlear implants

Objective

With more children receiving cochlear implants at an early age, there is a need for evaluation and assessment of early auditory behavior. We present the translation of the LittlEARS^® Auditory Questionnaire into Hebrew and into Arabic. First the validation of the LittlEARS^® Auditory Questionnaire in normal hearing children was evaluated. Second, the auditory behavior and the progress in hearing skills of a group of children with cochlear implants were assessed.

Methods

A “back-translation” method was used to translate and adapt the LittlEARS^® Auditory Questionnaire into Hebrew and into Arabic. Normal hearing participants included 70 Hebrew speaking and 97 Arabic speaking parents of children from 1 to 24 months of age with normal hearing. An additional group of 42 parents of children with cochlear implants with a hearing age of up to 24 months completed the LittlEARS^® Auditory Questionnaire. 27 of them completed the questionnaire more than once at intervals, so that change and development could be recorded. Scores on the LittlEARS^® Auditory Questionnaire were compared to results of SIR and CAP scales and other available auditory data.

Results

The results of the first study show that the curves found for the Hebrew and the Arabic translations of the LittlEARS^® Auditory Questionnaire are essentially similar to those previously found for other languages. These curves reflect the age dependency of auditory skills. Furthermore, in the group with cochlear implants the developmental pattern was different than that of the normal hearing group, with an initial steep increase and a later slower improvement. This trend appeared both in curves of groups and in curves of individuals (individuals whose parents completed the questionnaire at several points in time). There was a high correlation between scores on the LittlEARS^® Auditory Questionnaire and results of other audiologic tests, showing validity of results with the LittlEARS^® Auditory Questionnaire.

Conclusion

Both the Hebrew and Arabic versions of the LittlEARS^® Auditory Questionnaire were found to be reliable and valid tools for assessment of the development of auditory behavior in children up to the age of 24 months. Furthermore, the LittlEARS^® Auditory Questionnaire in both languages is useful in monitoring the progress of children with cochlear implant.     

Auditory performance and speech intelligibility of Mandarin-speaking children implanted before age 5

Objectives

(1) To report the auditory performance and speech intelligibility of 84 Mandarin-speaking prelingually deaf children after using cochlear implants (CIs) for one, two, three, four, and five years to understand how many years of implant use were needed for them to reach a plateau-level performance; (2) to investigate the relation between subjective rating scales and objective measurements (i.e., speech perception tests); (3) to understand the effect of age at implantation on auditory and speech development.

Methods

Eighty-four children with CIs participated in this study. Their auditory performance and speech intelligibility were rated using the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales, respectively. The evaluations were made before implantation and six months, one, two, three, four, and five years after implantation. At the fifth year after implantation, monosyllabic-word, easy-sentence, and difficult-sentence perception tests were administered.

Results

The median CAP score reached a plateau at category 6 after three years of implant use. The median SIR arrived at the highest level after five years of use. With five years of CI experiences, 86% of the subjects understood conversation without lip-reading, and 58% were fully intelligible to all listeners. The three speech perception tests had a moderate-to-strong correlation with the CAP and SIR scores. The children implanted before the age of three years had significantly better CAP and monosyllabic word perception test scores.

Conclusions

Five years of follow-up are needed for assessing the post-implantation development of communication ability of prelingually deafened children. It is recommended that hearing-impaired children receive cochlear implantation at a younger age to acquire better auditory ability for developing language skills. Constant postoperative aural–verbal rehabilitation and speech and language therapy are most likely required for the patients to reach the highest level on the CAP and SIR scales.     

Cochlear implantation in children with auditory neuropathy spectrum disorders

Abstract

Aim

To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD).

Materials and methods

The pre- and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS).

Results

Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19–68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0–5) and 2.5 (range 0–6), while the post-CI scores 5.8 (range 2–9) and 7.7 (range 3–10), respectively.

Conclusions

Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids.     

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

Abstract

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.     

Infants versus older children fitted with cochlear implants: performance over 10 years

Objectives

To investigate the efficacy of cochlear implants (CIs) in infants versus children operated at later age in term of spoken language skills and cognitive performances.

Method

The present prospective cohort study focuses on 19 children fitted with CIs between 2 and 11 months (X = 6.4 months; SD = 2.8 months). The results were compared with two groups of children implanted at 12-23 and 24-35 months. Auditory abilities were evaluated up to 10 years of CI use with: Category of Auditory Performance (CAP); Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS); Peabody Picture Vocabulary Test (PPVT-R); Test of Reception of Grammar (TROG) and Speech Intelligibility Rating (SIR). Cognitive evaluation was performed using selected subclasses from the Griffiths Mental Development Scale (GMDS, 0-8 years of age) and Leiter International Performance Scale-Revised (LIPS-R, 8-13 years of age).

Results

The infant group showed significantly better results at the CAP than the older children from 12 months to 36 months after surgery (p < .05). Infants PPVT-R outcomes did not differ significantly from normal hearing children, whereas the older age groups never reached the values of normal hearing peers even after 10 years of CI use. TROG outcomes showed that infants developed significantly better grammar skills at 5 and 10 years of follow up (p < .001). Scores for the more complex subtests of the GMDS and LIPS-R were significantly higher in youngest age group (p < .05).

Conclusion

This study demonstrates improved auditory, speech language and cognitive performances in children implanted below 12 months of age compared to children implanted later.     

Validation of the LittlEARS Auditory Questionnaire in children with normal hearing

Objectives

With more children receiving cochlear implants during infancy, there is a need for validated assessments of pre-verbal and early verbal auditory skills. The LittlEARS^® Auditory Questionnaire is presented here as the first module of the LittlEARS^® test battery. The LittlEARS^® Auditory Questionnaire was developed and piloted to assess the auditory behaviour of normal hearing children and hearing impaired children who receive a cochlear implant or hearing aid prior to 24 months of age. This paper presents results from two studies: one validating the LittlEARS^® Auditory Questionnaire on children with normal hearing who are German speaking and a second validating the norm curves found after adaptation and administration of the questionnaire to children with normal hearing in 15 different languages.

Methods

Scores from a group of 218 German and Austrian children with normal hearing between 5 days and 24 months of age were used to create a norm curve. The questionnaire was adapted from the German original into English and then 15 other languages to date. Regression curves were found based on parental responses from 3309 normal hearing infants and toddlers. Curves for each language were compared to the original German validation curve.

Results

The results of the first study were a norm curve which reflects the age-dependence of auditory behaviour, reliability and homogeneity as a measure of auditory behaviour, and calculations of expected and critical values as a function of age. Results of the second study show that the regression curves found for all the adapted languages are essentially equal to the German norm curve, as no statistically significant differences were found.

Conclusions

The LittlEARS^® Auditory Questionnaire is a valid, language-independent tool for assessing the early auditory behaviour of infants and toddlers with normal hearing. The results of this study suggest that the LittlEARS^® Auditory Questionnaire could also be very useful for documenting children's progress with their current amplification, providing evidence of the need for implantation, or highlighting the need for follow-up in other developmental areas.