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.

Listening to speech-in-noise with hearing aids: Do the self-reported outcomes reflect the behavioral speech perception task performance?

BACKGROUND: The aim of this study is to examine the relationship between behavioral speech-in-noise listening tasks and self-reported speech-in-noise outcomes of hearing aid user adult listeners. METHOD: To measure the self-reported outcomes of hearing, the Hearing Handicap Inventory for Adults [HHI-A], Speech, Spatial and Qualities of Hearing Scale (SSQ) and Amsterdam Inventory for Auditory Disability and Handicap were employed. To screen the cognitive abilities, Montreal Cognitive Assessment (MoCA) tool was used. Turkish matrix sentence test (TMST) was used for speech-in-noise test. Eighteen adult hearing aid users (mean age of 36.6 years) were participated. CONCLUSIONS: Results showed that some self-reported listening-in-noise outcomes are correlated with lab-based measurements of speech-in-noise test scores but not with the aided speech intelligibility thresholds. Given the present limitations of relying solely on self-report measures, it is important to complement them with objective measures to ensure a comprehensive evaluation.

Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis.

INTRODUCTION: Distal renal tubular acidosis (dRTA) is a disease that may develop either primarily or secondarily, resulting from urinary acidification defects in distal tubules. Hearing loss may accompany primary forms of dRTA. This study aims to determine the characteristics of hearing loss due to different gene mutations in patients with dRTA. METHODS: Behavioral and electrophysiological audiological evaluations were performed after otolaryngology examination in 21 patients with clinically diagnosed dRTA. Radiological imaging of the inner ear (n = 9) was conducted and results of genetic analyses using next-generation sequencing method (n = 16) were included. RESULTS: Twenty-one patients with dRTA from 20 unrelated families, aged between 8 months and 33 years (median = 12, interquartile range = 20), participated. All patients with ATP6V1B1 mutations (n = 9) had different degrees of hearing loss. There was one patient with hearing loss in patients with ATP6V0A4 mutations (n = 6). One patient with the WDR72 mutation had normal hearing. Large vestibular aqueduct syndrome (LVAS) was detected in 6 (67%) of 9 patients whose radiological evaluation results were available. CONCLUSIONS: LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.

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.

Speech in Noise: Implications of Age, Hearing Loss, and Cognition.

INTRODUCTION: Individuals with hearing loss have reduced hearing sensitivity and may not adequately process the temporal cues in acoustic signals. Cognitive skills that decrease with aging and hearing loss contribute negatively on the ability to understand speech. Hence, they may experience communication problems in noisy environments. The aim of the study was to investigate the effect of sloping high-frequency hearing loss on speech perception in noise and to examine the impact of temporal and cognitive processing in young and middle-age adults. METHODS: Speech in noise (SIN), temporal processing, and cognitive tests were conducted to hearing loss and normal hearing individuals aged 18-59 years. The measurements included the matrix sentence test, binaural temporal fine structure sensitivity (TFS) test, Visual Aural Digit Span (VADS), and Auditory Verbal Learning Test (AVLT). Twenty participants with normal hearing were recruited in the control group, whereas 20 participants with hearing loss at high frequencies were composed of the study group. RESULTS: Hierarchical regression analysis for SIN was performed by entering 3 separate blocks of independent variables. We entered age and hearing loss into the first block, which explained a significant amount of variability in SIN (R2 = 0.72, p < 0.001). Block 2 was comprised of scores from TFS sensitivity test, and this independent variable characterized temporal processing (R2 change = 0.002, p < 0.001). Block 3 was consisted of scores from VADS test and AVLT; these variables characterized cognitive processing and accounted for a good portion of SIN variance (R2 change = 0.04, p < 0.001). The age, hearing loss, and VADS contributed independently in the presence of all independent variables. CONCLUSION: The final model accounted for 76.2% of the variance in SIN. The results suggested that sloping hearing loss, aging, and cognitive decline affected auditory performance, and the poor performance starts from an early age. Additionally, the findings indicated that a more comprehensive approach might be recommended to evaluate the listening skills and identify communication problems.

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.

Assessment of temporal processing functions in early period cochlear implantation.

PURPOSE: The purpose of this study is to compare the temporal processing performance of children with cochlear implant (CI) according to the age of implantation and to determine their relation with auditory perception scores. METHODS: In this study, 30 cochlear implant users and ten normal hearing children at 9 and 10 years were included. Children with cochlear implants are divided into two groups according to the age of implantation: group I includes participants whose implantation age is between 13 and 35 months (20 children), group II includes participants whose implantation age is between 36 and 45 months (10 children). Individuals were evaluated with random gap detection test (RGDT), duration pattern test (DPT), frequency pattern test (FPT), the Mr. Potato Head task, word recognition, and sentence recognition test. RESULTS: A significant difference was found between the control and CI groups in temporal processing performance. The temporal processing ability of CI groups was significantly worse than those of normal hearing. Although there was no significant difference among the groups with cochlear implant in terms of temporal processing performance, children who started to use CI at an earlier age showed a tendency of better performance on temporal processing tasks. There was a significant relationship between Daily Sentence Test and FPT, and the Mr. Potato Head task and FPT rev (the score calculated by accepting the reverse patterns correctly). There was a significant relationship between duration of implant use and temporal ordering performance CONCLUSION: In this study, children with CI cannot perform as well as normal-hearing peers on temporal processing tasks, even if they had started to use their CIs at an early age. It is important to evaluate temporal processing in implanted individuals and to guide auditory training considering the evaluation results.

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.

Investigating Sensitivity to Auditory Cognition in Listening Effort Assessments: A Simultaneous EEG and Pupillometry Study.

BACKGROUND: It is still not fully explained what kind of cognitive sources the methods used in the assessment of listening effort are more sensitive to and how these measurement results are related to each other. The aim of the study is to ascertain which neural resources crucial for listening effort are most sensitive to objective measurement methods using differently degraded speech stimuli. METHODS: A total of 49 individuals between the ages of 19 and 34 with normal hearing participated in the study. In the first stage, simultaneous pupillometry, electroencephalogram (EEG), and single-task paradigm reaction time (RT) measurements were made during the challenging listening and repetition task with noise-vocoded speech. Two speech reception thresholds (SRT) (50% and 80%) for two vocoding conditions (16 and 6 channels) were collected, resulting in 4 conditions. In the second stage, the Rey Auditory Verbal Learning Test (RAVLT) and the test of attention in listening (TAIL) were applied. Stepwise linear regression analyses were conducted to examine the predictors of listening effort measurements. RESULTS: A significant difference was found between 6 and 16 channel stimuli in both pupil dilation change and EEG alpha band power change. In the hardest listening condition, whereas RAVLT scores are significant predictors of pupil dilation change, TAIL scores are significant predictors of EEG alpha power. As the stimulus difficulty decreased, the factors that predicted both EEG and pupillometry results decreased. In the single-task paradigm, a significant regression model could not be obtained at all four difficulty levels. CONCLUSION: As a result of the study, it was found that the pupil dilation change was more sensitive to auditory memory skills and the EEG alpha power change was more sensitive to auditory attention skills. To our knowledge, this study is the first to investigate the sensitivity of different listening effort measurement methods to auditory cognitive skills.

Unimodal versus bimodal auditory stimulation in inner ear malformations: Cognitive, language, and motor performance.

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.

Effects of Unilateral Audio-Vestibular Insufficiency on Spatial Hearing.

This study aimed to compare spatial hearing performance between adult individuals with the unilateral sensorineural hearing loss and unilateral loss of horizontal semicircular canal function (termed canal paresis/weakness) in the same ear and adults with normal hearing thresholds and normal vestibular function and to examine associated factors (duration of hearing loss and rate of canal paresis).The study participants consisted of 20 adults (aged 48±11 years) with unilateral sensorineural hearing loss and unilateral canal paresis (unilateral weakness≥25%) in the same ear. The control group comprised 25 adults (aged 45±13 years) with normal hearing and a unilateral weakness rate below 25%. Pure tone audiometry, bithermal binaural air caloric test, Turkish Spatial Hearing Questionnaire (T-SHQ), and Standardized Mini-Mental State Exam were applied to all the individuals. When the performance of the participants in T-SHQ was examined both in terms of the subscales and the total scale, there was a statistically significant difference between the two groups in relation to the scores. A statistically significant, high, negative correlation was detected between the duration of hearing loss, the rate of canal paresis and all the subscale scores and total score of T-SHQ. According to these results, as the duration of hearing loss increased, the scores obtained from the questionnaire decreased. As the rate of canal paresis increased, vestibular involvement increased, and the T-SHQ score decreased. This study showed that adults with unilateral hearing loss and unilateral canal paresis in the same ear had lower spatial hearing performance than those with normal hearing and balance. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03442-1.

The role of suprathreshold auditory processing abilities in children with specific learning disorder.

OBJECTIVE: To assess the suprathreshold auditory processing and speech recognition abilities in noise in children with specific learning disorder (SLD). METHODS: A group of twenty-five children diagnosed with SLD and a control group of twenty-five neuro-typical children were included in the study. All the participants were between 6-11 years old. To evaluate suprathreshold auditory processing abilities, the participants were given the Temporal Fine Structure (TFS) Sensitivity Test and the Temporal Envelope (TE) Sensitivity Test, as well as the Consonant Identification Test, was administered to evaluate speech recognition ability in noise. In addition, the Wechsler Intelligence Scale for Children - Fourth Edition (WISC-IV) intelligence test was applied to children with SLD, and the relationship between WISC-IV intelligence test scores in different skills and suprathreshold auditory processing and speech recognition abilities in noise was investigated. RESULTS: Significant differences were found between children diagnosed with SLD and neuro-typical children in terms of suprathreshold auditory processing tasks and speech recognition in noise. Additionally, no correlation was found between suprathreshold auditory processing tasks, speech recognition in noise, and intelligence tests. CONCLUSION: Suprathreshold auditory processing and speech recognition abilities in noise were found to be affected in children with SLD. A holistic evaluation including a multidisciplinary approach that includes suprathreshold auditory processing abilities is required for children diagnosed with SLD.

TEMPORARY REMOVAL: How The Child with Hearing Loss and Their Parents Affected During the Covid-19 Pandemic?

The publisher announces that this article has been temporarily removed. An edited version will be published under the same DOI as soon as possible. We thank you for your understanding. If you have any questions, please contact am-query@thieme.com.

Revision Surgery for Pediatric Nontumor Auditory Brainstem Implant Users: Audiological, Rehabilitative, and Surgical Outcomes.

OBJECTIVE: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. PATIENTS: Five pediatric ABI users who had revision surgery for device malfunctions. INTERVENTIONS: Revision surgery for ABI malfunctions. MAIN OUTCOME MEASURES: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared. RESULTS: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed. CONCLUSION: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development.

State-Trait Anxiety Inventory (STAI) assessment of mothers with language delayed children.

OBJECTIVES: In this study, we evaluated the continuous and state anxiety levels of mothers with children with language delay. METHODS: The study group consisted of the mothers of 18 children with language delay. The control group consisted of the mothers of 29 healthy children without language delay. To gain data about mothers, a personal information form and Spielberger's State-Trait Anxiety Inventory (STAI) form were applied to determine continuous and state-trait anxiety levels. RESULTS: State anxiety levels in the study group were significantly higher (by Student t-test) than that of the control group. For continuous anxiety level, no statistically significant difference was determined between two groups. In the study group, higher education levels of mothers and their husbands were associated with lower levels of both continuous and state anxiety. CONCLUSION: In the majority of the group of mothers with language delayed children and even mothers of children with normal language development, there were high levels concern. Mothers' concerns and anxiety levels may decrease with increasing levels of their education levels. We recommend providing detailed information regarding language development to the families at all stages of the childs' training programme.

Effects of Age at Auditory Brainstem Implantation: Impact on Auditory Perception, Language Development, Speech Intelligibility.

OBJECTIVE: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility. STUDY DESIGN: Retrospective single cohort design. SETTING: Tertiary referral center. PATIENTS: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ±â€Š6.5) months in the early group, 45.53 (ranged ±â€Š7.9) months in the late group. INTERVENTION(S): Retrosigmoid craniotomy and ABI placement. MAIN OUTCOME MEASURE(S): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room. RESULTS: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps. CONCLUSIONS: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

Auditory Reasoning Skills of Cochlear Implant Users.

OBJECTIVES: This study compared the auditory reasoning skills of school-going children with early and late cochlear implantation and assessed the relationship between auditory reasoning skills, language development, vocabulary knowledge, and communication skills. MATERIALS AND METHODS: In this case series study, 90 pre-lingually deaf children aged 7-10 years were assessed. Children were divided into two groups: early-implanted group with children who received cochlear implants before 3 years of age (mean, 23.45; 12-35 months) and late-implanted group with children implanted after 3 years of age (mean, 50.54; 36-84 months). Tests were performed in the auditory-visual condition. Correlational analyses were used to assess the relationships between daily communication skills, language development performances, vocabulary knowledge, and auditory reasoning skills of both the groups. RESULTS: Auditory reasoning skills were better in the early-implanted group than in the late-implanted group (Mann-Whitney U test=518, p<0.05). Language performances of the early-implanted group were significantly better than those of the late-implanted group (receptive language performances: Mann-Whitney U=522, p<0.05; expressive language performances: Mann-Whitney U=552, p<0.05). Stepwise regression analysis showed that expressive language performances, vocabulary knowledge, and chronological age could predict 82% of the variance. CONCLUSION: Reasoning skills of children with cochlear implants should be supported during the language-learning process.

Engineering a novel, stable dimeric streptavidin with lower isoelectric point.

We have engineered a soluble, stable two-chain dimeric streptavidin (TCD) in Escherchia coli. Examination of the three-dimensional structure of streptavidin aided by empirical binding free-energy calculations helped us to select mutations at subunit interfaces that dissociate the native tetramer and stabilize the desired dimer. We chose positions W120, L124, V125 and H127 and mutated them to 120D/124D/125D/127D (TCD-1); 120D/124N/125S/127D (TCD-2); and 120D/124D/125S/127D (TCD-3). The H127D mutation creates electrostatic repulsion that disrupts the dimer-dimer interface, but leaves it very hydrophobic. Therefore, W120, L124 and V125 were mutated to hydrophilic residues to increase dimer solubility. Among the three candidates, TCD-2 gave the best result: a stable, active dimer with K(d) for biotin of approximately 1x10(-7)M after purification by gel-filtration chromatography. The experimental results confirm the possibility of rational engineering of low-pI dimeric streptavidins. Reduced-size streptavidin mutants with a net negative charge may be more suitable than antibodies or wild-type streptavidin for the targeting step in radioimmunotherapy because they should clear faster from the bloodstream and the kidney.

Cochlear Implantation in Neurobrucellosis.

BACKGROUND: Neurobrucellosis is a disease consisting of a wide spectrum of complications such as peripheral neuropathy, cranial nerve involvement, ataxia, meningeal irritation, paraplegia, seizures, coma, and even death. The vestibulocochlear nerve seems to be the most commonly affected cranial nerve (10%). We present a patient with neurobrucellosis whose auditory perception and speech intelligibility skill performances improved after cochlear implantation. CASE REPORT: A 35 year-old woman was admitted to another hospital 2 years ago with the symptoms of headache, nausea, and altered consciousness, who was finally diagnosed with neurobrucellosis. She developed bilateral profound sensorineural hearing loss during the following 6 months. There was no benefit of using hearing aids. After successful treatment of her illness, she was found to be suitable for cochlear implantation. After the operation, her auditory perception skills improved significantly with a Categories of Auditory Performance (CAP) score of 5. According to clinical observations and her family members' statements, her Speech Intelligibility Rating (SIR) score was 3. Her speech intelligibility skills are still improving. CONCLUSION: Our case report represents the second case of hearing rehabilitation with cochlear implantation after neurobrucellosis. Cochlear implantation is a cost-effective and time-proven successful intervention in post-lingual adult patients with sensorineural hearing loss. Early timing of the surgery after appropriate treatment of meningitis helps the patient to achieve better postoperative results.

Long-term Results of ABI in Children With Severe Inner Ear Malformations.

OBJECTIVE: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral otolaryngology clinic. SUBJECTS AND METHODS: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined. RESULTS: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI. CONCLUSION: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.