Children with Auditory Brainstem Implants: Language Proficiency and Reading Comprehension Process.

INTRODUCTION: Auditory performance and language proficiency in young children who utilize auditory brainstem implants (ABI) throughout the first three years of life is difficult to predict. ABI users have challenges as a result of delays in language proficiency and the acquisition of reading comprehension, even if ABI technology offers auditory experiences that enhance spoken language development. The aim of this study was to evaluate about the impact of language proficiency on reading comprehension skills in children with ABI. METHOD: In this study, 20 children with ABI were evaluated for their reading comprehension abilities and language proficiency using an Informal Reading Inventory, Test of Early Language Development (TELD-3), Categories of Auditory Performance-II (CAP-II), and Speech Intelligibility Rating (SIR). Three distinct aspects of reading comprehension were assessed and analyzed to provide a composite score for reading comprehension abilities. TELD-3, which measures receptive and expressive language proficiency, was presented through spoken language. RESULTS: Studies have shown that there was a relationship between language proficiency and reading comprehension in children with ABI. In the present study, it was determined that the total scores of reading comprehension skills of the children who had poor language proficiency and enrolled in the school for the deaf were also low. The children use short, basic sentences, often repeat words and phrases, and have a restricted vocabulary. In addition, the children had difficulty reading characters and detailed paragraphs and could not remember events in a logical order. CONCLUSION: Children with ABI may potentially have complicated reading comprehension abilities due to lack of access to all the speech formants needed to develop spoken language. In addition, variables affecting the reading levels of children with ABI include factors such as age at implantation, duration of implant use, presence of additional disability, communication model and access to auditory rehabilitation. The reading comprehension skills of ABI users were evaluated in this study for the first time in the literature and may constitute a starting point for the examination of variables affecting reading comprehension in this area.

Does Mild and Moderate Hearing Loss Affect Verbal Working Memory and Language Skills in Children?

INTRODUCTION: For a comprehensive approach in children with hearing loss (HL), some cognitive and language skills should also be considered, along with auditory skills. The main aim of this study was to evaluate the working memory and language skills in children with mild to moderate HL. METHODS: Forty children with mild to moderate HL between the ages of 4 and 9 years were included in this study. The children with mild and moderate HL were evaluated in two groups. The Meaningless Word Repetition test, Test of Language Development-Primary test, and Meaningful Auditory Integration Scale were administered to assess working memory, language skills, and auditory perception, respectively. Also, the relationship between language and memory skills was evaluated. RESULTS: The study found statistically significant correlations between language skills and working memory test scores. Additionally, statistically significant differences were found between children with mild and moderate HL in terms of language and memory skills (p < 0.001). CONCLUSION: The children with moderate HL demonstrated poorer performance compared to those with mild HL. Even if it is mild, the degree of HL affects children's language and memory skills. It is emphasized that the relationship between language and working memory should be taken into consideration in auditory rehabilitation programs for these children.

Auditory performance and language skills in children with auditory brainstem implants and cochlear implants.

PURPOSE: This study aims to evaluate school-age language skills and auditory performance in different listening situations in children with cochlear implants and auditory brainstem implants. METHOD: The study included 60 children between the ages of 5 and 9 years with cochlear implants (CI) and auditory brainstem implants (ABI). The volunteer children were divided into two groups: bimodal CI-ABI and bilateral CI users. Test of Language Development: Primary (TOLD-P:4), which assesses components of language such as phonology, morphology, syntax and semantics, was used to evaluate school-age language skills. Children's Auditory Performance Scale (CHAPS) was used to measure their listening performance in quiet, noisy, multi-stimulus environments and their auditory attention and memory skills in daily life. The correlations between language and auditory performance were analyzed and compared between the two groups. RESULTS: Children with ABI showed poorer performance in school-age language skills and auditory performance in different listening environments (p < 0.05). Significant correlations were between school-age language skills and auditory performance (p < 0.05). CONCLUSION: Improved auditory performance is crucial for the development of school-age language skills. To improve auditory performance in children with ABI in different listening environments, assistive listening devices, acoustic environmental arrangements, informative activities, etc., should be used.

Perception of voice cues in school-age children with hearing aids.

The just-noticeable differences (JNDs) of the voice cues of voice pitch (F0) and vocal-tract length (VTL) were measured in school-aged children with bilateral hearing aids and children and adults with normal hearing. The JNDs were larger for hearing-aided than normal-hearing children up to the age of 12 for F0 and into adulthood for all ages for VTL. Age was a significant factor for both groups for F0 JNDs, but only for the hearing-aided group for VTL JNDs. Age of maturation was later for F0 than VTL. Individual JNDs of the two groups largely overlapped for F0, but little for VTL. Hearing thresholds (unaided or aided, 500-400 Hz, overlapping with mid-range speech frequencies) did not correlate with the JNDs. However, extended low-frequency hearing thresholds (unaided, 125-250 Hz, overlapping with voice F0 ranges) correlated with the F0 JNDs. Hence, age and hearing status differentially interact with F0 and VTL perception, and VTL perception seems challenging for hearing-aided children. On the other hand, even children with profound hearing loss could do the task, indicating a hearing aid benefit for voice perception. Given the significant age effect and that for F0 the hearing-aided children seem to be catching up with age-typical development, voice cue perception may continue developing in hearing-aided children.

Evaluation of the non-auditory responses in individuals with auditory brainstem implant.

OBJECTIVES: After auditory brainstem implant (ABI) surgery, stimulation of certain cranial nerves may result in a non-auditory response, and the electrodes that stimulate these nerves may be deactivated. The goals of this study are to compare the number of active electrodes in the initial activation and the last fitting, to investigate non-auditory response types and their frequency as a result of non-auditory stimulation, to compare the placements of deactivated electrodes as a result of non-auditory stimulation in the initial activation and the last fitting. METHODS: The computer software system was used to perform a retrospective analysis of the fitting data of 69 ABI users who underwent auditory brainstem implant surgery between January 1997 and January 2019. The non-auditory response types, deactive electrodes, and the positioning of the deactive electrodes horizontally and vertically were recorded in these users during the initial activation and the last fitting. RESULTS: There was no statistically significant difference between the number of active electrodes in the initial activation and the last fitting. The proportion of the users with deactive electrodes in the initial activation and the last fitting was not statistically significant different. In the horizontal and vertical placement classification, the placement of the deactive electrodes was not statistically different between initial activation and last fitting. The most common type of non-auditory response was facial nerve stimulation at the initial activation and no auditory perception at the last fitting. According to the difference between the number of active and deactive electrodes in the initial activation and the last fitting, as well as the auditory and non-auditory responses, it was found that the ABI users were statistically different between the initial activation and the last fitting. CONCLUSION: The results of this study show that not only auditory but also non-auditory responses occur in most ABI users. In addition, to the best of our knowledge, this study is the first to examine the frequencies of non-auditory response types, and the placement of the electrodes that cause these responses according to horizontal and vertical classifications.

Speech recognition and quality of life outcomes of adults with cochlear implants following a quarter-century of deafness: what should be the maximum duration?

PURPOSE: This study was aimed at examining the pre- and post-cochlear implant (CI) speech recognition and quality of life results of postlingually deaf adult CI users with a duration of deafness (DoD) > 25 years and determining the maximum DoD limit. METHODS: We enrolled 54 postlingually deaf CI users and divided them into ages ≤ 60 and > 60 years and DoDs ≤ 25 and > 25 years. All participants were evaluated using multisensory measures (auditory and auditory + visual) and open-set Speech Recognition Test (SRT) before CI and 3 years postoperatively. They were administered with The Hearing Handicap Inventory for the Elderly (HHIE) to determine the effects of hearing impairment on daily life. RESULTS: DoD and open-set SRT for auditory and auditory + visual stimuli showed a strong negative linear relationship (r = - 0.506, p < 0.01). Open-set SRT scores of patients with DoD aged ≤ 25 and > 25 years (p < 0.01) differed significantly. The chronological age and HHIE scores in social and emotional subfactors showed a strong negative linear relationship (r = - 0.519, p < 0.01). CONCLUSIONS: The present study showed that the number of years was a major factor determining that postlingual adults with profound hearing loss had hearing loss. The results support CI use as soon as possible in adults to prevent degeneration of the auditory pathways and possible central remodeling. However, auditory rehabilitation outcomes in adults using CI vary widely. Investigating the causes of this variability contributes to audiology.

Does glutaric aciduria type 1 affect hearing function?

This study aimed to evaluate audiological findings among patients with glutaric aciduria type 1 (GA-1). We used a large test battery for the audiological evaluation of 17 individuals with GA-1 (the study group) and 20 healthy individuals (the control group). Conventional audiometry (0.125-8 kHz), distortion product otoacoustic emissions (DPOAEs) (1, 1.5, 2, 3, 4, 6, and 8 kHz), contralateral suppression of otoacoustic emissions, and auditory brainstem response (ABR) ( 30, 50, 70 and 90 dB nHL) were measured for all participants (n = 37). Mild sensorineural hearing loss was found in 77.47% (n = 13) of the patients with GA-1, and normal hearing thresholds were seen in 23.53% (n = 4). There were three asymptomatic patients at the time of diagnosis [two developed mild mental motor retardation (MMR) and one developed severe MMR during the follow-up], one with a normal hearing threshold and two with mild hearing loss), and 14 symptomatic patients (three with normal hearing thresholds and 11 with mild hearing loss). Seven of the symptomatic patients diagnosed following an encephalopathic crisis required intensive care and showed significantly worse hearing thresholds than those without symptoms [20.86 ± 4.47 vs. 15.44 ± 3.96 decibel hearing level (dB HL), p = 0.039*], while five had mild-to-moderate hearing loss. Acute encephalopathic crisis had a negative effect on hearing function in the symptomatic patients. The emission and contralateral suppression amplitude values of the study group were significantly lower compared to the control group (p < 0.05). The I-V interpeak latency and absolute latencies of ABR waves I, III, and V of the study group were observed to be significantly prolonged and morphologically distorted compared to those of the control group (p < 0.05). Five patients had MMR, and three had moderate MMR; all eight had mild-to-moderate hearing loss. In addition, of the eight patients with mild MMR, four had mild hearing loss. In particular, the morphological findings of ABR waves were significantly worse in the patients with severe and moderate MMR (p < 0.05). There was a significant correlation between a macrocephaly history (12 patients) and hearing loss (p = 0.041*). Magnetic resonance imaging findings were evaluated in all the 17 patients with GA-1, and typical fronto-temporal atrophy and sylvian fissure enlargement were observed. Our findings support that GA-1 is associated with auditory impairment, primarily in symptomatic patients. Adequate audiological test battery evaluation is essential in this context, particularly for symptomatic patients with a history of encephalopathic crises.

Views of Syrian Mothers in Ankara on Infant Hearing Loss: Cross-sectional Survey.

BACKGROUND: Mothers' awareness of hearing loss, its risk factors, and available detection and intervention choices have been well known to play an essential role in the early detection and intervention of hearing loss. OBJECTIVE: To investigate the knowledge and attitude of Syrian mothers toward infant hearing loss, early identification, and intervention. METHODS: The "Maternal Views on Infant Hearing Loss" questionnaire was adapted and translated into Arabic and then administered to 100 Syrian mothers living in different neighborhoods in Ankara within the age range of 18-68 years old. The internal consistency for the main domains of the questionnaire was tested by Cronbach's alpha coefficient. Descriptive statistics and Spearman's rank correlation coefficient were used to evaluate the responses. RESULTS: The highest level of knowledge about the risk factors was about noise exposure (76%), while the lowest level of knowledge was about jaundice (25%). While 98% of the mothers had a positive attitude toward early detection, 97% of them did not mind the early intervention. CONCLUSIONS: The present findings showed the need to improve mothers' awareness about infant hearing loss risk factors, available detection, and intervention. Such results may help in performing programs that aim to increase awareness about hearing loss.

Written language skills in children with auditory brainstem implants.

PURPOSE: This study aimed to assess the written language skills of children with auditory brainstem implants (ABI). METHODS: In this study, 15 children (from second to eighth grades) with ABI were evaluated for their written language abilities using a written expression skill assessment form. Five different features of written expression points were scored and analyzed, yielding a composite score for written expression skills. RESULTS: This study showed that all children with ABI needed more verbal cues than spontaneously written samples. Moreover, these children used short and simple sentences with limited vocabulary and repeated words and sentences. Furthermore, these children were deficient in writing an introduction, the body, and the conclusion paragraphs and could not write events in a logical sequence. CONCLUSIONS: The written language skills of children with ABI depend on age at implantation, duration of implant use, and additional handicaps. Written expression skills in children with ABI are highly complex skills. The findings highlight the importance of ABI during the critical language development period and the enhancement of training programs for written language skills in children who underwent ABI.

Factors affecting phoneme discrimination in children with sequential bilateral cochlear implants.

OBJECTIVES: To investigate the effects of a number of variables on phoneme discrimination (PD) performance in children with sequential bilateral cochlear implants (SeqBiCIs) and compare PD performance between the 2 implantation sides and between children with bilateral cochlear implants (BiCIs) and their age-matched peers with normal hearing (NH). DESIGN: All participants completed the Auditory Speech Sound Evaluation Phoneme Discrimination Test. STUDY SAMPLE: The sample included 23 children with SeqBiCIs as the study group and 23 with NH as the control group. RESULTS: A significant difference was found between the scores of the two groups under the CI1 and CI2 conditions (p = 0.001), CI1 and BiCI conditions (p = 0.002), and CI2 and BiCI conditions (p = 0.001). PD scores with CI1 significantly depend on age at CI1 and duration of bilateral use. PD scores with CI1 were significant predictors of PD performance with CI2. Duration of BiCI use was a significant predictor of PD scores with BiCI. CONCLUSIONS: The age at CI1 and the duration of bilateral cochlear implant use were found to improve phoneme discrimination performance in children with a sequential bilateral cochlear implant. According to the success of the CI1, it is possible to predict the success of CI2 use.

Postural Control in Subjects with Incomplete Partition Inner Ear Malformations: A Comparison of Incomplete Partition Types.

INTRODUCTION: Children with inner ear malformation (IEM) are at risk of vestibular loss as well as hearing loss. Incomplete partition (IP) anomalies constitute about 41% of all IEMs. This study aimed to investigate the postural control in subjects with the same type of IP on both sides and to compare their results with cochlear implant (CI) users without IEM and healthy peers. METHODS: The study group consists of 17 subjects with the same IP types on both sides and using auditory implants on at least one side, with the following 3 groups: 6 IP-I subjects (mean age 12.28 ± 6.25), 6 IP-II subjects (mean age 12.90 ± 3.23), and 5 IP-III subjects (mean age 6.98 ± 3.10). Six unilateral CI users (mean age 11.38 ± 3.57) with normal inner ear structures were included in the CI control group, and 6 healthy peers (10.20 ± 4.79) were included in the healthy control group. The postural control was measured using the Bruininks-Oseretsky Test of Motor Proficiency Second Edition (BOT-2) balance subtest. All devices were turned off during the balance test. RESULTS: The BOT-2 balance scale scores were observed to be significantly different between the IP-I and healthy control group (medians of balance scores being 3.00 and 16.00, respectively, p < 0.001) and the IP-III and healthy control group (medians of balance scores being 6.60 and 16.00, respectively, p = 0.04). The IP-II group had better balance scores (median = 8.00) than those of the other IP groups, although there were no significant differences between the IP-II and other groups (p > 0.05). CONCLUSION: This study demonstrated that subjects with the same IP type on both sides and with early implantation may differ in terms of their postural control abilities depending on their IP type. Subjects with IP should be regularly followed up by the vestibular assessment and supported by their postural control ability by vestibular rehabilitation.

Children with Auditory Brainstem Implant: How Do They Perform in Motor and Language Skills?

BACKGROUND: Young children are able to explore new objects and practice language through the acquisition of motor skills that lead to their overall development. Congenital hearing loss and total vestibular loss may contribute to the delay in speech and motor skill development. OBJECTIVES: To investigate the relationship between motor development performance, speech perception, and language performance in children with auditory brainstem implant (ABI). METHOD: Ten children, aged 4-17 years (mean age 9.76 ± 4.03), fitted with unilateral ABI for at least 2 years due to the presence of labyrinthine aplasia and rudimentary otocyst at least 1 side were included in the study. Several standardized tests, such as Bruininks-Oseretsky Motor Proficiency Test-2 (BOT-2), Children's Auditory Perception Test Battery, Meaningful Auditory Integration Scale (MAIS), and Test of Early Language Development-3, were performed to evaluate their skills of fine motor control, balance, manual dexterity, language, and auditory perception. RESULTS: A significant correlation was established between the BOT-2 manual dexterity and MAIS scores (r = 0.827, p < 0.05) and between the manual dexterity and language skills (for expressive language, r = 0.762, p < 0.05; for receptive language, r = 0.650, p < 0.05). Some of the BOT-2 balance tasks, such as standing on 1 leg on a line with eyes closed, standing on 1 leg on a balance beam with eyes open, standing heel-to-toe on a balance beam, and walking forward heel-to-toe on a line, showed a strong correlation with their receptive and expressive language performance (p < 0.05). CONCLUSION: The current study has indicated that significantly poor manual and balance performances are associated with poor speech perception and language skills in children with ABI. The authors recommend performing a vestibular assessment before and after ABI surgery and the use of a holistic rehabilitation approach, including auditory and vestibular rehabilitation, to support development of the children with ABI.

Auditory perception skills in children receiving simultaneous bilateral cochlear implants: early speech-discrimination results.

PURPOSE: The objective of the present study is to evaluate early speech-perception abilities using VRISD in toddlers who received simultaneous bilateral CIs between 12 and 36 months of age and to compare them with the findings in NH infants and to monitor the development of speech-perception skills within 6 months after cochlear implantation. METHODS: The VRISD test was performed using video visual reinforcement to assess speech-discrimination ability in the CI and NH groups. Four stimuli were used for testing in the present study: /a/, /i/, /ba/, and /da/. The two contrasts used for the research were /a-i/ and /ba-da/. Auditory and listening skills in the CI group were assessed using the IT-MAIS. RESULTS: The responses to the /a-i/ and /ba-da/ phoneme contrast were found to be similar in the NH and CI groups. No statistically significant difference was found between the groups (p > 0.05). VRISD test result and the IT-MAIS score were highly correlated in CI group (p = 0.001, r = 0.822). CONCLUSION: The VRISD test can be effectively used to evaluate the development of speech-discrimination skills in hearing-impaired babies before and after CI. This research suggests that the development of speech-perception ability with CI is seriously influenced by environmental exposure and sound access. To the best of our knowledge, this is the first study to evaluate the speech-perception skills in toddlers with simultaneous bilateral CI.

Social competence in children with cochlear implants: is it possible to catch up with their peers?

PURPOSE: The objective of this study is to compare social competence skills in children with CI and their normal hearing peers. METHODS: Forty-six children with normal hearing and 46 children with CI between the ages of 42 and 72 months were included in the control group and study group, respectively. Preschool teachers rated children's social competence in the classroom using the Social Competence and Behavior Evaluation-Preschool Edition. Three subscales constitute the structure of the SCBE-30 scale: anger-aggression, social competence, and anxiety-withdrawal. RESULTS: The analyses showed that there were statistically significant differences between social competence scores of the study group and the control group. However, there was no statistically significant difference between anger-aggression scores and anxiety-withdrawal scores of the study group and the control group. There was a significant correlation found between anger-aggression score and the age of starting rehabilitation. CONCLUSION: Anger-aggression scores and anxiety-withdrawal scores were similar between children using cochlear implant and normal hearing peers, whereas children with CI show lower social competence abilities than normal hearing peers. Earlier beginning to the rehabilitation programs coincide with lower anger-aggression scores. To the best of our knowledge, this is the first study to reflect these findings more objectively, from the view of teachers.

Postural instability in children with severe inner ear malformations: Characteristics of vestibular and balance function.

OBJECTIVE: To investigate the postural instability and vestibular functions in children with severe inner ear malformations (IEMs). DESIGN: A prospective case-control study. STUDY SAMPLE: The study group consisted of 10 children using unilateral auditory brainstem implant (ABI) with labyrinthine aplasia or rudimentary otocyst. The age-matched control groups consisted of 10 unilateral cochlear implant (CI) users with normal inner ear structures and 10 healthy peers. All tests were performed to implant users when the implants were off. RESULTS: All median VOR gains in the ABI group (median anterior, lateral, and posterior canal 0.15, 0.05, and 0.05, respectively, for the non-implanted sides) were significantly lower than those of the control groups (median VOR gains ≥ 0.90 in both control groups). There were no oVEMP and cVEMP responses in the study group. The mean BOT-2 balance scores of the ABI (3.70 ± 1.34) group was dramatically lower than those of the CI (9.40 ± 2.88) and healthy control (16.20 ± 4.16, p < 0.001). CONCLUSIONS: The postural instability in children with severe IEMs was higher than those in CI users with normal inner ear structures and healthy peers. The level of deficiency in the labyrinthine was more important for postural stability in children.

Audiological Performance in Children with Inner Ear Malformations Before and After Cochlear Implantation: A Cohort Study of 274 Patients.

BACKGROUND AND OBJECTIVE: Inner ear malformations (IEMs) are common in children with hearing loss. The different types of IEMs form a unique subgroup of cochlear implant (CI) candidates. We aimed to evaluate the auditory perception outcomes of CI in children with different types of IEMs and compare them with CI users without IEMs. METHODS: The study included 274 CI users with and without IEMs as two groups (n = 137, each). Both groups' chronological age at implantation and duration of CI usage was matched (± 8 months). All subjects were evaluated pre-operatively and post-operatively by the Ling's sound test and the auditory perception test battery, which includes the Meaningful Auditory Integration Scale (MAIS), closed-set Pattern Perception Test (PPT) and open-set Sentence Recognition Test (SRT). Besides, children with IEMs were assessed for language development. RESULTS: Progress in the IEMs' group differed according to the type of ear anomaly. CI users with enlarged vestibular aqueduct had the highest scores, while users with common cavity had the lowest. Children with IEMs performed well on the closed-set test while having difficulty with the open-set test. CONCLUSION: Cochlear implantation outcomes are favourable in IEMs' patients with a cochlear nerve visible on magnetic resonance imaging. Our results indicate that it is critical to take the anatomical differences into account during follow-up and rehabilitation programmes. Each CI user should be evaluated according to his or her individual needs.

Evaluation of auditory temporal processing in patients fitted with bone-anchored hearing aids.

PURPOSE: The present study aimed to identify temporal processing abilities in users of bone-anchored hearing aid (BAHA). METHODS: Seventeen subjects with BAHA (SWB) users and 29 subjects with normal-hearing were compared using the frequency pattern test, duration pattern test, and speech-in-noise test. Besides behavioral temporal processing tests, a multi-feature mismatch negativity (MMN) test was performed in BAHA users to assess the accuracy of objective auditory discrimination at the cortical level, irrespective of the subjects' attention or behavioral task. RESULTS: The results of the multi-feature MMN test showed that BAHA users could perceive differences in frequency, duration, intensity. However, no response was elicited regarding localization of stimulus and gaps within tones. Word recognition performance in noise was significantly poorer compared with normal hearing groups. All age groups showed no differences in terms of temporal processing abilities except 30-39 years duration pattern ability weaker in SWB group. CONCLUSION: The present study was the first to indicate that the use of unilateral BAHA has no effect on the localization of auditory stimuli at the cortical level according to electrophysiological test. Although Baha users have near-normal levels of hearing thresholds and temporal ordering abilities with their devices, since they benefit from the unilateral device, they still have some difficulties in resolution, recognizing and distinguishing the spatial aspects of speech, especially in multiple and noisy listening environments.

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.

OBJECTIVE: To determine audiological outcomes of children who use a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the contralateral ear. DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PARTICIPANTS: Twelve children followed with CI and contralateral auditory brainstem implant (ABI) by Hacettepe University Department of Otorhinolaryngology and Audiology in Turkey. All children were diagnosed with different inner ear malformations with cochlear nerve aplasia/hypoplasia. CI was planned in the ear with better sound detection during behavioural testing with inserted ear phones and with better CN as seen on MRI. Due to the limited auditory and speech progress with the cochlear implant, ABI was performed on the contralateral ear in all subjects. MAIN OUTCOME MEASURES: Audiological performance and auditory perception skills of children with cochlear nerve deficiency (CND) who use bimodal electrical stimulation with CI and contralateral ABI. RESULTS: Mean age of the subjects was 84.00 ± 33.94 months. Age at CI surgery and ABI surgery was 25.00 ± 10.98 months and 41.50 ± 16.14 months, respectively. However, hearing thresholds only with CI and only with ABI did not reveal significant difference, and auditory perception scores improved with bimodal stimulation. The MAIS scores were significantly improved from unilateral CI to bimodal stimulation (P = .002). Pattern perception and word recognition scores were significantly higher with the bimodal condition when compared to CI only and ABI only conditions. CONCLUSION: Children with CND showed better performance with CI and contralateral ABI combined. Depending on the audiological and radiological results, bimodal stimulation should be advised for children with CND.

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults.

BACKGROUND: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. OBJECTIVES: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. METHODS: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants' audiological test results according to the radiological findings. RESULTS: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. CONCLUSIONS: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.

Is Early Cochlear Implant Device Activation Safe for All on-the-Ear and off-the-Ear Sound Processors?

BACKGROUND: Cochlear implantation (CI) is an effective treatment option for patients with severe-to-profound hearing loss. When CI first started, it was recommended to wait until at least 4 weeks after the CI surgery for the initial activation because of possible complications. Advances in the surgical techniques and experiences in fitting have made initial activation possible within 24 h. OBJECTIVES: To compare the complaints and complications after early activation between behind-the-ear (BTE) and off-the-ear (OTE) sound processors and to show the impact of early activation on the electrode impedance values. METHOD: CI surgeries performed between March 2013 and July 2018 were retrospectively analyzed from the database. In total, 294 CI users were included in the present study. The impedance measurements were analyzed postoperatively at the initial activation prior to the stimulation, and 4 weeks after the initial activation in the first-month follow-up visit. A customized questionnaire was administered in the first-month follow-up fitting session to caregivers and/or patients who were using CI at least for 6 months. Medical records were also reviewed to identify any postoperative complications. RESULTS: In the early activation group, impedance values were significantly lower than in the control group (p < 0.05) at first fitting. At the first-month follow-up, no significant difference was found between the groups (p > 0.05). The most common side effects were reported to be edema (6.1%) and pain (5.7%) in the early activation group. In patients with OTE sound processors, the rate of side effects such as skin infection, wound swelling, skin hyperemia, and pain was higher than in patients with BTE sound processors; however, a statistical significance was only observed in wound swelling (p = 0.005). Selecting the appropriate magnet was defined as a problem for the OTE sound processors during the initial activation. CONCLUSION: This study revealed that early activation of CI was clinically safe and feasible in patients with BTE sound processors. When using OTE sound processors, the audiologists should be careful during the activation period and inform patients of possible side effects. The first fitting should be delayed for 4 weeks after CI for OTE sound processors. This current study is the first to report this finding with 5 years of experience in a large cohort.