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Objective: To report device failures, audiological signs and other reasons for revision cochlear implant surgery, and discuss indications for revision surgery. Methods: Revision procedures between November 1997 and August 2017 were retrospectively analysed. Over 20 years, 2181 cochlear implant operations were performed, and 114 patients underwent 127 revision operations. Results: The revision rate was 4.67 per cent. The full insertion rate for revision cochlear implant surgery was 88.2 per cent. The most frequent reasons for revision surgery were: device failure (59 per cent), wound breakdown (9.4 per cent) and electrode malposition (8.7 per cent). The device failure rate was: 2.78 per cent for Advanced Bionics, 1.82 per cent for Cochlear and 5.25 per cent for Med-El systems. The number of active electrodes was significantly increased only for Med-El devices after revision surgery. The most common complaints among 61 patients were: gradually decreased auditory performance, sudden internal device shutdown and headaches. Conclusion: The most common reason for revision surgery was device failure. Patients should be evaluated for device failure in cases of: no hearing despite appropriate follow up, side effects such as facial nerve stimulation, and rejection of speech processor use in paediatrics. After revision surgery, most patients have successful outcomes. [ABSTRACT FROM AUTHOR]

Objectives: To report unusual contralateral side effects in the case with auditory brainstem implant (ABI). Material and Methods: A 2-years-old girl was diagnosed with bilateral cochlear nerve aplasia and auditory brainstem hypoplasia. The family was reported left facial paralysis at the age of 5 months. She was implanted with auditory brainstem implant on the left side on 5th December 2017. The initial stimulation was done 1 months after the surgery under monitoring. After initial activation of ABI follow-up visits was scheduled as 1st month, 2nd month and 3rd month. Auditory perception abilities was evaluated with Ling sounds and MAIS scores. Results and Conclusions: During the initial stimulation facial nerve stimulation was observed on the contralateral side on 9 electrodes. Only 3 electrodes could be activated. In first month follow up visit, all electrodes checked for side effects. None of the deactivated electrodes could be activated. In case of 3 activated electrodes, she wanted to use sound processor daily, can recognize the environmental sound. MAIS scores improved 3 months after ABI. Contralateral side effects can be seen after ABI in cases with brainstem hypoplasia. During the ABI programming, two experienced pediatric audiologist should observe the patient. [ABSTRACT FROM AUTHOR]

Objective: The Cochlear Wireless Mini Microphone is a miniature, wireless remote microphone that transmits the audio signal captured at the microphone directly to the recipient's Nucleus 6 and Kanso Sound Processor via the proprietary GN Resound digital radio frequency transmission protocol on the 2.4 GHz band. It enhances speech recognition, sound quality and overall listening experience in situations that are known to provide the most difficulty for cochlear implant recipients. In the literature it was showed that the Mini Microphone resulted in significantly better speech recognition when compared to performance with the Nucleus 6 or Kanso alone. Aim: To assess the speech recognition ability in experienced cochlear implant recipients with and without the Mini Microphone. Material and Methods: 15 unilateral cochlear implant recipients participate in this study all who are fitted with Nucleus 6 or Kanso sound processors. Duration of cochlear implant use is at least 3 years. The recipients use the Mini Speech recognition ability in quiet and in noise were then assessed with and without the use of Mini Microphone. Recipients are seated 1 m from loud speakers which are located at +/-45 degree azimuths in a S-45N45 configuration. The Mini Microphone is positioned 20 cm directly below the loudspeaker that is used to present the target signal. Turkish Matrix Test was done for testing speech recognition ability. It was conducted via ten well-known Turkish names, numbers, adjectives, objects, verbs, from which syntactically fixed sentences were randomly composed. Results and Conclusion: Using the Cochlear wireless mini microphone improves speech recognition ability in cochlear implant recipients. When the SNR is increased in Turkish Matrix sentence test, speech recognition can show better scores. [ABSTRACT FROM AUTHOR]

Objectives: To point out the radiological and audiological characteristics of a recently defined cochlear hypoplasia type: cochlear hypoplasia type 4. To describe this malformation in relation to adjacent anatomial structures within temporal bone. Material and Methods: This study was a reprospective chart review of the cochlear hypoplasia type 4 (CH-IV). Cases were selected from our departments' database and their CT and MRI images together with audiological evaluation data were revised.Inclusion criteria was having CH-IV diagnosis in at least one ear and having both CT and MRI carried out in hospital. Axial and coronal CT of the temporal bone were evaluated to delineate the features of the malformation appropriately. MRI images were reviewed to evaluate the statos of the cochlear nerve (CN). CN was evaluated on axial and especially on sagittal-oblique T2 weighted images and classified as aplastic, hypoplastic, or normal. Results: There were 26 ears with CH-IV in our database. In CH-IV; the cochlea has a normal basal turn, but middle and apical turns are severely hypoplastic and located anterior and medially rather than in their normal central position. The labyrinthine segment of the facial nerve is usually located antero-superior to the cochlea rather than in its normal location. Six (27.3%) out of 26 with CH-IV had cochlear nerve hypoplasia. Four ears had naroow IAC. On axial CT scan; facial recess was evaluated also. An imaginary line crosses from the mastoid segment of the facial nerve to the basal turn and round window was created. In CH-IV cases; mastoid segment was anterior to this line. In transmastoid approach; exposure of the round window might be difficult because of this rotational malformation. 22 ears (84.6%) anomalous route of the labyrinthine segment of the facial route. In those cases labyrinthine segment was anterosuperoriorly displaced. 7.7% of the cases had slight to mild hearing loss(HL), 46.2% had moderate to moderately severe HL and 46.2% had severe to profound HL. Some selected cases did not require hearing aids and cochlear implantation. As the number of turns are smaller with narrower scalae, it is strongly advisable to use thin and shorter electrodes. Thick and long electrodes may not be inserted fully into the cochlea. It is important to diagnose CH-IV since the visualization of round window may be challenging and this anomaly has diverse audiological features which directly affect the treatment plan. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of the study was to assess the written language skills of children with auditory brainstem implants. Material and Method(s) when relevant: In this study 12 children with auditory brainstem implants were evaluated in terms of their written language abilities. The children were between 2nd and 8th grades and A Written Expression Skills Assessment Form was used. Five different features of written expression points were scored and analysed, yielding composite score for written expression skills. Result(s): The results of this study showed that all of children with ABI needed more verbal cues than spontaneously written samples. This study show that children with auditory brainstem implant use simple and short sentences with limited vocabulary and repetition of a word and a sentence. Children with ABI are deficient in writing an introduction, body and conclusion paragraphs or in logical sequences of events. Conclusion(s): Variables, which explain written language skills of the children who use ABI, depend on age at implantation, duration of implant use and additional handicaps. The findings of this study highlight the importance of auditory brainstem implantation during the critical period of language development and also enhancing training programmes for written language skills for the children who underwent ABI. Also, written expression skills in children with ABI is a highly complex skill. [ABSTRACT FROM AUTHOR]

Objective: To evaluate vestibular evoked myogenic potentials (VEMP) results of unilateral cochlear implant users with inner ear malformations. Method: VEMP is a short latency myogenic response recorded with surface electrodes placed over muscles. VEMP response could be recorded even in subjects with severe to profound hearing loss and this reveals intact vestibular organs. However, it is complicated that what happens in case of inner ear malformations. In the current study, 10 unilateral cochlear implant users with inner ear malformations were included. These subjects were selected randomly from institutional ear malformations database. Both cervical VEMP (cVEMP) and ocular VEMP (oVEMP) was applied for all subjects. Results: Age range was in between 7 years and 6 months and 40 years with 20,7 mean. All subjects had different cochleaovestibular malformations and in same subject both ear also had different malformations. 9 subjects had right CI and 1 subject left CI. All subjects had bilateral normal middle ear function. They were tested with cVEMP and oVEMP in a random order. In implanted ear, cVEMP response got in 4 subject, but oVEMP response got in 2 subjects. However in non-implanted ear, cVEMP and oVEMP responses got in 5 subjects. VEMP wave forms revealed some difference in both latency and amplitude form CI user with normal inner ear structure and normal subject's reports from literature. Conclusion: Cochlear implant could change vestibular functions and this could be evaluated with VEMP especially to see saccul and utricul functions. It was known that dizziness and balance problems commonly accompany with inner ear malformations also after CI their complaint may increase. Different cochleovestibular malformations result in different VEMP recordings. But it is not clear that absence of VEMP result from cochleaovestibular malformation or from CI. Therefore, further studies need. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate otorhinolaryngologic findings and the relationship between aminoglycoside (AG) exposure and hearing loss in paediatric patients with cystic fibrosis (cwCF). We also aimed to investigate the genetic predisposition to AG ototoxicity by screening for m.1555A>G mutations., Methods: CwCF who underwent otorhinolaryngologic and audiologic examinations were retrospectively included. Clinical characteristics, ear-nose-throat related symptoms, and a history of ototoxic drug exposure were recorded. m.1555A>G mutations were retrospectively screened among patients with audiologic evaluations., Results: Two hundred thirty-four cwCF were included in this study with a median age of 10.7 (range, 6.8-14.2) years. Nasal obstruction (14.1%) was the most common symptom. Fifty-two (22.2%) patients had chronic rhinosinusitis (CRS) with nasal polyps (CRSwNP). There was a positive correlation between CRSwNP and the symptom of nasal obstruction (r:.234, p < .001), snoring (r:.179, p = .006), and sleeping with mouth open (r:.138, p = .034). One hundred forty-nine (63.6%) patients had audiologic evaluations; 14 (9.4%) had hearing impairment. No statistical significance existed between ototoxicity and IV AG exposure (p = .90). Six (42.8%) of 14 patients did not receive ototoxic drugs. One hundred nineteen (50.8%) patients were screened for m.1555A>G mutations, and none were detected., Conclusions: Almost a quarter of the study population had CRSwNP. Neither the relationship between AGs exposure and hearing loss nor the genetic predisposition to AG ototoxicity could be shown in cwCF., (© 2024 Wiley Periodicals LLC.)

Introduction: Auditory performance and language proficiency in young children who utilize auditory brainstem implants (ABIs) throughout the first 3 years of life are 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-Third Edition (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., (© 2024 S. Karger AG, Basel.)

Objectives: This study aims to synthesize current knowledge and outcomes related to pediatric auditory brainstem implantation (ABI) in children with severe inner ear malformations (IEMs). It highlights the clinical management practices, challenges, and potential future directions for consensus development in this field., Methods: A systematic review of findings presented at the Third International Pediatric ABI Symposium organized by the Hacettepe Cochlear Implant team between 3 and 5 September 2020 was conducted, incorporating data from 41 departments across 19 countries. Relevant clinical outcomes, imaging techniques, surgical approaches, and rehabilitation strategies were analyzed to identify key trends and variability in practices., Results: The review indicates that children receiving ABIs exhibit diverse auditory outcomes influenced by individual anatomical variations and developmental factors. Early implantation, particularly before the age of three, positively correlates with better auditory and language development. Multicenter experiences underscore the necessity of tailored decision-making, which considers both surgical candidacy and comprehensive rehabilitation resources., Discussion:: The variability in outcomes emphasizes the need for improved consensus and guidelines regarding eligibility, surgical techniques, and multidisciplinary rehabilitation approaches. Notable complications and the necessity for thorough imaging assessments were also identified as critical components affecting clinical decisions., Conclusion: A formal consensus statement is warranted to standardize best practices in ABI management. This will not only enhance patient outcomes but also guide future research efforts to address the remaining challenges in the treatment of children with severe IEMs. Enhanced collaboration among team members will be pivotal in achieving these objectives.

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., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: New perspectives on rehabilitation options for inner ear malformations have still been studied in the literature. This study investigated the cognitive, language, and motor skills of auditory brainstem implant (ABI) users in unimodal and bimodal groups., Methods: The motor competency of the participants was assessed with Bruininks-Oseretsky Motor Proficiency Test-2 Short Form (BOT2 SF). Language performance was evaluated by the test of Early Language Development-3 and Speech Intelligibility Rating. Word identification, sentence recognition tests, and Categories of Auditory Performance were used to assess auditory perception skills. To examine the cognitive performance, Cancellation Test and Gesell Copy Form were administered. All the tests were conducted in a quiet environment without any distractions., Results: The participants were divided into two groups: (1) 17 children in the unimodal group and (2) 11 children in the bimodal (who used a cochlear implant on one side and ABI on the other side) group. There were significant correlations between the chronological age of participants and BOT2 SF total score, cancellation tests, auditory perception tests, and language performance. Similarly, there were significant correlations between the duration of ABI use and auditory perception tests, language performance, cancellation test, and some BOT2 SF subtests ( r  = -0.47 to -0.60, p  < .001). There was no significant difference between the unimodal and bimodal groups in any task ( p  > .05). However, there were moderate-to-strong correlations among the auditory perception tests, cancellation test, language test, and BOT2 SF total score and subtests ( r  = 0.40 to 0.55, p  < .05)., Conclusion: Although there were no significant differences between bimodal and unimodal groups, a holistic approach, which indicates that hearing and balance issues can have broader impacts on a person's physical, emotional, social, and psychological aspects, should be used in the assessment process., Level of Evidence: Level 4., Competing Interests: The authors declare no conflicts of interest., (© 2023 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals LLC on behalf of The Triological Society.)

Objectives: The aim of this study is to develop the Turkish version of hearing in noise test for children (HINT-C) by providing norms and correction factors for the children in different age groups. Methods: A total of 77 individuals with normal hearing - 62 children (6-12 years old) and 15 adults (18-30 years old) - were included. Twelve phonemically balanced 10-sentence lists were created from the adult version of the Turkish HINT (Study 1). Age-specific norms, correction factors and maturation effects were examined using the Turkish HINT-C (Study 2). Results: Mean performances under different listening conditions and Spatial Release from Masking (SRM) advantage values were obtained for the 6-, 8-, 10-, and 12-year-old and estimated for the 7-, 9-, and 11-year-old age groups, and correction factors were calculated for all children age groups. Turkish-speaking children did not achieve adult-like hearing in noise performance, until they were 12 years old. Conclusions: Twelve phonemically balanced 10-sentence lists of Turkish HINT-C were created, and the mean performances of children in different age groups were measured. In addition to the age-specific HINT-C norms and correction factors for the 6-, 8-, 10-, and 12-year-old age groups, the maturation effects were determined. Highlights The assessment of speech-in-noise perception is highly critical for children.To evaluate the speech-in-noise perception ability, 12 phonemically balanced 10-sentence lists of Turkish HINT-C were created.Speech-in-noise perception ability improves with age.Turkish-speaking children do not achieve adult-like hearing in noise performance, until they were 12 years old.

Objective: The present study aimed to evaluate binaural auditory skills in bimodal and bilateral pediatric cochlear implant (CI) users with incomplete partition type-II (IP-II) and to reveal the effect of IP-II on performance by comparing the results to pediatric CI users with normal cochlear morphology., Study Design: Cross-sectional study., Setting: Tertiary referral center., Methods: Forty-one CI users (mean age 8.8 ± 1.9) were grouped as bimodal (BIM-IP) and bilateral (BIL-IP) users with IP-II; bimodal (BIM-N) and bilateral (BIL-N) users with normal cochlear anatomy. Speech perception in noise and sound localization skills were compared under 2 conditions; binaural (bilateral or bimodal) and monaural (first CI alone)., Results: BIM-IP and BIL-IP showed no performance difference in binaural tasks. The BIM-N group showed remarkably poor performance in comparison to the groups of BIL-IP (p = .007), BIM-IP (p < .001), and BIL-N (p = .004) in terms of speech-in-noise skills. In sound localization abilities, similar significant differences were found between the group of BIM-N and the groups of BIL-IP (p = .001), BIM-IP (p < .001), and BIL-N (p = .004). All groups showed statistically significant improvements in binaural condition on both tasks (p < .05)., Conclusion: We revealed that bilateral and bimodal pediatric CI users with IP-II benefitted from implantation as much as bilateral users with normal anatomy. Differences in residual hearing between groups may explain the poor performance of bimodal users with normal cochlear morphology. To the best of our knowledge, it is the first study to unveil binaural performance characteristics in children diagnosed with a specific inner ear malformation subgroup., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objectives: To assess the quality of life (QoL) in child and adolescent cochlear implant users with inner ear malformations (IEM) and to compare their outcomes to their cochlear implant using peers with normal inner ear structures., Methods: The present sample consisted of 100 children (45 with IEM, 55 without IEM) and 100 adolescents (46 with IEM, 54 without IEM). The following QoL questionnaires were used to assess the hearing-related QoL: The Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26 for children between 7 and 12 years of age) and HEAR-QL-28 (for adolescents between 13 and 18 years of age). Both questionnaires were based on a 5-points Likert scale from 0 to 4, with higher scores indicating a better perception of QoL. The scores were converted to percentage values (never = 100, almost never = 75, sometimes = 50, often = 25, almost always = 0)., Results: For the patients with IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 50.4 (SD = 18.9) and 54.5 (SD = 19.6), respectively. For the patients without IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 72.7 (SD = 18.0) and 65.0 (SD = 19.1), respectively. For both child and adolescent subgroups, statistically significant differences were observed between QoL scores from patients with and without IEM (p < 0.001). There were no statistically significant effects of the malformation type on the QoL findings (p ≥ 0.05)., Conclusion: Child and adolescent cochlear implant users with IEM had significantly lower scores on validated HEAR-QL versions in comparison to their implanted peers without IEM., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objectives: The objectives of this study were to assess auditory perception and speech intelligibility outcomes in children with cochlear nerve (CN) hypoplasia who received cochlear implants (CIs) using Categories of Auditory Performance II (CAP II) and Speech Intelligibility Rating (SIR) scales., Methods: In total, 40 children who received CI and who were aged between 3 and 18 years were included in this study. The study group included 20 children with CN hypoplasia at least one ear, while the control group included 20 children with normal cochleas and cochlear nerve structures. All children in the study and control groups who participated were evaluated using the CAP II and the SIR scale. Demographic data were collected., Results: Significant differences were found between the study and control groups' CAP II and SIR scores (p < 0.001). It was found that CAP II scores were positively correlated with SIR scores in the study (r = 0.743, p < 0.001) and control (r = 0.601, p < 0.001) groups. In the study group, significant negative correlations were found between SIR scores and age at implantation (r = -0.674, p = 0.004) and between CAP II scores and age at implantation (r = 0.751, p = 0.003). In the control group, a significant negative correlation was found between age at implantation and CAP II scores (r = -0.805, p = 0.001). Similarly, a significant negative correlation was found between age at implantation and SIR scores (r = -0.702, p = 0.007)., Conclusion: Even for children with severe inner ear malformation and CN hypoplasia, CI is an effective treatment modality for auditory perception and speech production. However, it should be noted that CN hypoplasia affects auditory performance negatively in children with CI., (Copyright © 2022 Elsevier B.V. All rights reserved.)

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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 CI 1 and CI 2 conditions ( p  = 0.001), CI 1 and BiCI conditions ( p  = 0.002), and CI 2 and BiCI conditions ( p  = 0.001). PD scores with CI 1 significantly depend on age at CI 1 and duration of bilateral use. PD scores with CI 1 were significant predictors of PD performance with CI 2 . Duration of BiCI use was a significant predictor of PD scores with BiCI., Conclusions: The age at CI 1 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 CI 1 , it is possible to predict the success of CI 2 use.

Objectives: The aim was to evaluate the cochlear implant (CI) mapping parameters of CI users with inner ear malformation (IEM) and to reveal the changes in parameters over time., Methods: In total, 118 CI users were included with 127 ears (68-IEM; 59-normal cochlear anatomy) in present retrospective study. The impedance measurements, thresholds levels-THR, most comfortable levels- MCL, pulse width-PW and rate values were analyzed in both IEM and control group at the initial activation, 6th,12th and 24th months postoperatively., Results: There were statistically significant differences in impedance measurements in several time points. And also, there was a remarkable difference in THR & MCL and PW values between IEM and control groups in all time points (p < 0.05). THR & MCL levels and PW values increased significantly between all time periods in both groups (p < 0,008) and values of parameters in IEM-group were higher than those of control group. When comparing rates, statistically significant difference was observed only at the initial activation in both within (p < 0.001) and between groups (p = 0.03)., Conclusion: Pediatric CI users with IEM need individual changes in fitting parameters. More frequent map sessions should be planned as they require more PW, THR and MCL increase over time. The increase rate differs between IEM subgroups depending on the deviation of malformation from the normal cochlear anatomy. This study is the first to in its attempt to reveal the mapping characteristics and long-term changes in pediatric CI users with different IEM subgroups., (Copyright © 2022 Elsevier B.V. All rights reserved.)

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., (© 2021 S. Karger AG, Basel.)

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., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

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.

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.

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., (© 2020 John Wiley & Sons Ltd.)

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., (© 2021 S. Karger AG, Basel.)

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., (© 2019 John Wiley & Sons Ltd.)

Purpose: The aim of this study was to compare the quality of life (QoL) of adult CI users with the QoL of adults in the healthy and normal-hearing population., Materials and Methods: 31 patients with CIs were included in the CI group, and 31 normal-hearing subjects were included in the control group. The QoL was evaluated using the World Health Organization Quality of Life-BREF (WHOQOL-BREF) for all subjects., Results: A comparison of the QoL of the CI group to that of the control group found that the QoL of healthy adults was better than that of the CI users. The results obtained for the subdomains of physical health, psychological health, and social relations showed statistically significant differences between the two groups (p < 0.05). There were no statistically significant differences between the groups in the subdomains of environment and general health (p > 0.05)., Conclusions: The effect of a hearing disability on daily life continues after the CI. As expected, adults with CIs still face challenges in their daily lives due to the hearing impairment., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (© 2019 S. Karger AG, Basel.)

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., (© 2019 S. Karger AG, Basel.)

Objective: The aim of the current study is to evaluate audiologic and radiologic findings of cochlear hypoplasia which is a subgroup of inner ear malformations., Methods: This study was a prospective clinical study and based on voluntary participation from cases with cochlear hypoplasia diagnosis. The study was conducted at Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from an inner ear malformations database. Inclusion criteria were having cochlear hypoplasia for at least one ear. There were 66 subjects with an age range of 12 months and 60 years 5 months. For each subject, pure tone audiometry and tympanometry were applied according to chronological and cognitive age. And also, auditory brainstem response test was applied to when it is need. Subjects' radiologic results were reevaluated to confirm cochlear hypoplasia, cochlear nerve and cochlear aperture., Results: Cochlear hypoplasia types were statistically significantly different in terms of HL degree. This difference was caused by cochlear hypoplasia type IV group being was statistically different from the other three groups. Like with degree of HL, cochlear hypoplasia groups were statistically different from other three groups in terms of type of hearing loss. Cochlear aperture and cochlear nerve status showed variation according to cochlear hypoplasia type but these differences were not statistically approved., Conclusions: In the current study, incidence of cochlear hypoplasia was 23.5% in all inner ear malformation. With this study, it was seen that subtypes of cochlear hypoplasia showed variability in terms of degree and type of hearing loss and also cochlear aperture and cochlear nerve status. Especially cochlear hypoplasia type IV differs from other three cochlear hypoplasia types., (Copyright © 2016 Elsevier B.V. All rights reserved.)

The objective of the study was to discuss the findings of intraoperative electrically evoked auditory brainstem response (eABR) test results with a recently designed intracochlear test electrode (ITE) in terms of their relation to decisions of cochlear or auditory brainstem implantation. This clinical study was conducted in Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from inner ear malformation (IEM) database. Eleven subjects with profound sensorineural hearing loss were included in the current study with age range from 1 year 3 months to 4 years 3 months for children with prelingual hearing loss. There was only one 42-year-old post-lingual subject. eABR was recorded with an ITE and intraoperatively with an original cochlear implant (CI) electrode in 11 cases with different IEMs. Findings of eABR with ITE and their relation to the decision for CI or auditory brainstem implant (ABI) are discussed. Positive eABR test results were found to be dependent on close to normal cochlear structures and auditory nerve. The probability of positive result decreases with increasing degree of malformation severity. The prediction value of eABR via ITE on decision for hearing restoration was found to be questionable in this study. The results of eABR with ITE have predictive value on what we will get with the actual CI electrode. ITE appears to stimulate the cochlea like an actual CI. If the eABR is positive, the results are reliable. However, if eABR is negative, the results should be evaluated with preoperative audiological testing and MRI findings.

Purpose: Autosomal recessive nonsyndromic deafness (ARNSD) is characterized by a high degree of genetic heterogeneity, with reported mutations in 58 different genes. This study was designed to detect deafness-causing variants in a multiethnic cohort with ARNSD by using whole-exome sequencing (WES)., Methods: After excluding mutations in the most common gene, GJB2, we performed WES in 160 multiplex families with ARNSD from Turkey, Iran, Mexico, Ecuador, and Puerto Rico to screen for mutations in all known ARNSD genes., Results: We detected ARNSD-causing variants in 90 (56%) families, 54% of which had not been previously reported. Identified mutations were located in 31 known ARNSD genes. The most common genes with mutations were MYO15A (13%), MYO7A (11%), SLC26A4 (10%), TMPRSS3 (9%), TMC1 (8%), ILDR1 (6%), and CDH23 (4%). Nine mutations were detected in multiple families with shared haplotypes, suggesting founder effects., Conclusion: We report on a large multiethnic cohort with ARNSD in which comprehensive analysis of all known ARNSD genes identifies causative DNA variants in 56% of the families. In the remaining families, WES allows us to search for causative variants in novel genes, thus improving our ability to explain the underlying etiology in more families.Genet Med 18 4, 364-371.

Objectives: The aims of this study were to collect data on electrically evoked compound action potential (eCAP) and electrically evoked stapedius reflex thresholds (eSRT) in HiResolution(TM) cochlear implant (CI) users, and to explore the relationships between these objective measures and behavioural measures of comfort levels (M-levels)., Methods: A prospective study on newly implanted subjects was designed. The eCAP was measured intra-operatively and at first fitting through neural response imaging (NRI), using the SoundWave(TM) fitting software. The eSRT was measured intra-operatively by visual monitoring of the stapes, using both single-electrode stimulation and speech bursts (four electrodes stimulated at the same time). Measures of M-levels were performed according to standard clinical practice and collected at first fitting, 3 and 6 months of CI use., Results: One hundred seventeen subjects from 14 centres, all implanted unilaterally with a HiResolution CII Bionic Ear(®) or HiRes 90K(®), were included in the study. Speech burst stimulation elicited a significantly higher eSRT success rate than single-electrode stimulation, 84 vs. 64% respectively. The NRI success rate was 81% intra-operatively, significantly increasing to 96% after 6 months. Fitting guidelines were defined on the basis of a single NRI measurement. Correlations, analysis of variance, and multiple regression analysis were applied to generate a predictive model for the M-levels., Discussion: Useful insights were produced into the behaviour of objective measures according to time, electrode location, and fitting parameters. They may usefully assist in programming the CI when no reliable feedback is obtained through standard behavioural procedures.