Reduced Channel Interaction Improves Timbre Recognition Under Vocoder Simulation of Cochlear Implant Processing.

OBJECTIVE: This study aimed to investigate the influence of the number of channels and channel interaction on timbre perception in cochlear implant (CI) processing. By utilizing vocoder simulations of CI processing, the effects of different numbers of channels and channel interaction were examined to assess their impact on timbre perception, an essential aspect of music and auditory performance. STUDY DESIGN, SETTING, AND PATIENTS: Fourteen CI recipients, with at least 1 year of CI device use, and two groups (N = 16 and N = 19) of normal hearing (NH) participants completed a timbre recognition (TR) task. NH participants were divided into two groups, with each group being tested on different aspects of the study. The first group underwent testing with varying numbers of channels (8, 12, 16, and 20) to determine an ideal number that closely reflected the TR performance of CI recipients. Subsequently, the second group of NH participants participated in the assessment of channel interaction, utilizing the identified ideal number of 20 channels, with three conditions: low interaction (54 dB/octave), medium interaction (24 dB/octave), and high interaction (12 dB/octave). Statistical analyses, including repeated-measures analysis of variance and pairwise comparisons, were conducted to examine the effects. RESULTS: The number of channels did not demonstrate a statistically significant effect on TR in NH participants ( p > 0.05). However, it was observed that the condition with 20 channels closely resembled the TR performance of CI recipients. In contrast, channel interaction exhibited a significant effect ( p < 0.001) on TR. Both the low interaction (54 dB/octave) and high interaction (12 dB/octave) conditions differed significantly from the actual CI recipients' performance. CONCLUSION: Timbre perception, a complex ability reliant on highly detailed spectral resolution, was not significantly influenced by the number of channels. However, channel interaction emerged as a significant factor affecting timbre perception. The differences observed under different channel interaction conditions suggest potential mechanisms, including reduced spectro-temporal resolution and degraded spectral cues. These findings highlight the importance of considering channel interaction and optimizing CI processing strategies to enhance music perception and overall auditory performance for CI recipients.

Intracochlear electrocochleography findings in cochlear implant recipients with auditory neuropathy spectrum disorder.

OBJECTIVES: This study aimed to compare intracochlear electrocochleography (ECochG) findings in a group of cochlear implant (CI) recipients with auditory neuropathy spectrum disorder (ANSD) with a group of CI recipients with sensorineural hearing loss (SNHL). Auditory outcome and spectral resolution findings were also compared among CI recipients with and without cochlear microphonic (CM) responses. METHODS: This single-center, prospective cohort study was undertaken at a tertiary referral center. CM responses by the intracochlear ECochG test were recorded in CI recipients at 0.25-2 kHz. Speech, spatial, and hearing quality (SSQ) outcomes and spectral resolution measured with the spectral-temporally modulated ripple test were obtained for each recipient. The study included 62 implanted ears in 46 recipients, of which 59% (n = 27) were male and 41% (n = 19) were female. Twenty-nine ears with ANSD and 33 ears with SNHL were included. The mean age of the participants was 11 years. The results compared the intracochlear ECochG findings of the ANSD group with those of the SNHL group. RESULTS: Participants were divided into two groups with and without obtainable CM responses. CM responses were obtained in 13 of 29 ears in the ANSD group and 14 of 33 ears in the SNHL group. CM thresholds obtained were better according to behavioral audiometric responses in some frequencies in the ANSD group. No significant difference was found in the auditory outcome and spectral resolution among CI recipients with and without CM responses. CONCLUSIONS: Intracochlear ECochG has a limited potential clinical value for monitoring ANSD. CM thresholds obtained using ECochG may not reflect behavioral hearing thresholds.

Emotions and Psychological Mechanisms of Listening to Music in Cochlear Implant Recipients.

OBJECTIVES: Music is a multidimensional phenomenon and is classified by its arousal properties, emotional quality, and structural characteristics. Although structural features of music (i.e., pitch, timbre, and tempo) and music emotion recognition in cochlear implant (CI) recipients are popular research topics, music-evoked emotions, and related psychological mechanisms that reflect both the individual and social context of music are largely ignored. Understanding the music-evoked emotions (the "what") and related mechanisms (the "why") can help professionals and CI recipients better comprehend the impact of music on CI recipients' daily lives. Therefore, the purpose of this study is to evaluate these aspects in CI recipients and compare their findings to those of normal hearing (NH) controls. DESIGN: This study included 50 CI recipients with diverse auditory experiences who were prelingually deafened (deafened at or before 6 years of age)-early implanted (N = 21), prelingually deafened-late implanted (implanted at or after 12 years of age-N = 13), and postlingually deafened (N = 16) as well as 50 age-matched NH controls. All participants completed the same survey, which included 28 emotions and 10 mechanisms (Brainstem reflex, Rhythmic entrainment, Evaluative Conditioning, Contagion, Visual imagery, Episodic memory, Musical expectancy, Aesthetic judgment, Cognitive appraisal, and Lyrics). Data were presented in detail for CI groups and compared between CI groups and between CI and NH groups. RESULTS: The principal component analysis showed five emotion factors that are explained by 63.4% of the total variance, including anxiety and anger, happiness and pride, sadness and pain, sympathy and tenderness, and serenity and satisfaction in the CI group. Positive emotions such as happiness, tranquility, love, joy, and trust ranked as most often experienced in all groups, whereas negative and complex emotions such as guilt, fear, anger, and anxiety ranked lowest. The CI group ranked lyrics and rhythmic entrainment highest in the emotion mechanism, and there was a statistically significant group difference in the episodic memory mechanism, in which the prelingually deafened, early implanted group scored the lowest. CONCLUSION: Our findings indicate that music can evoke similar emotions in CI recipients with diverse auditory experiences as it does in NH individuals. However, prelingually deafened and early implanted individuals lack autobiographical memories associated with music, which affects the feelings evoked by music. In addition, the preference for rhythmic entrainment and lyrics as mechanisms of music-elicited emotions suggests that rehabilitation programs should pay particular attention to these cues.

Musical Mistuning Perception and Appraisal in Cochlear Implant Recipients.

OBJECTIVE: Music is a very crucial art form that can evoke emotions, and the harmonious presence of the human voice in music is an impactful part of this process. As a result, vocals have had some significant effects on contemporary music. The mechanism behind the cochlear implant (CI) recipients perceiving different aspects of music is clear; however, how well they perceive vocal tuning within music it is not well known. Hence, this study evaluated the mistuning perception of CI recipients and compared their performance with normal-hearing (NH) listeners. STUDY DESIGN, SETTING, AND PATIENTS: A total of 16 CI users (7 cisgender men, 9 cisgender women) and 16 sex-matched NH controls with an average age of 30.2 (±10.9; range, 19-53) years and 23.5 (±6.1; range, 20-37) years, respectively, were enrolled in this study. We evaluated the mistuning ability using the mistuning perception test (MPT) and assessed self-perceived music perception and engagement using the music-related quality-of-life questionnaire. Test performance was measured and reported on the item-response theory metric with a z score ranging from -4 to +4. RESULTS: A significant difference in the MPT scores was found between NH and CI recipients, whereas a significant correlation was noted between the music-related quality-of-life questionnaire-frequency subscale and MPT scores. No significant correlations were found between age, CI age, and CI usage duration and MPT performance. CONCLUSIONS: This study revealed that musical mistuning perception is a limitation for CI recipients, similar to previously evaluated aspects of music perception. Hence, it is important to consider this aspect in the assessment of music perception, enjoyment, and music-based auditory interventions in CI recipients, as vocals are paramount in music perception and recreation. The MPT is a convenient and accessible tool for mistuning assessment in CI and hearing-aid users.

Observational Study of Pediatric Cochlear Implant Recipients: Two-year Follow-up Outcomes.

Objective: The aim of this study was to evaluate the improvements in quality of life and auditory performance via a descriptive report using patient-related outcomes measures in a group of children with cochlear implants (CI). Methods: This was a longitudinal and retrospective study based on the analysis of patient records of a total of 28 children with CI, 18 of whom had bilateral (64.3%) and 10 had unilateral (35.7%). The study included repeated within-subject measures of Hearing Implants Quality of Life (CuHI-QoL), Categories of Auditory Performance (CAP-II), Speech, Spatial, and Qualities of Hearing scale-Parents' version (SSQ-P) scales, and Implant Recipient Follow-up (IRF) form at 6-month intervals from baseline up to 24 months. Results: During the 2-year follow-up assessments, all children with CI showed statistically significant improvement over time in their CAPII, SSQ-P, CuHI-QoL, and IRF scores (p<0.001). The most significant increase in scores occurred within the first six months following the baseline and then continued to increase gradually at a slower pace over time. A moderate negative relationship was found between the age of implantation and SSQ-P and family satisfaction scores of IRF (p<0.05). Conclusions: Family expectations, overall well-being, quality of life, auditory, and verbal skills increase positively with earlier CI intervention. Moreover, the subjective assessment results showed that parents-positive attitudes toward implantation, and willingness to recommend it to others have steadily increased in time. Although the cohort is relatively small and variable, the results offer a descriptive view to outcomes in real-world practice.

A longitudinal study of cortical auditory maturation and implications of the short inter-implant delay in children with bilateral sequential cochlear implants.

OBJECTIVE: This longitudinal study aimed to investigate the maturational development of P1 latency and the effects of a short inter-implant delay in children who received bilateral sequential cochlear implants. MATERIALS AND METHODS: Fourteen children (first CI mean age: 1.4 ± 0.4 years and Second CI mean age: 3.3 ± 0.5 years) who had received sequential bilateral cochlear implants during the sensitive period for auditory maturation participated in our study. The speech-evoked cortical P1 response was recorded after the activation of the second CI at four intervals (implant activation, 3 months, 6 months, 12 months) under three listening conditions (first CI, second CI, binaural). RESULTS: Our results showed that the P1 latencies of the second CI reached normative values within 3 months, but did not reach the P1 latency of the first CI until 12 months. We found a strong negative correlation between the inter-implant delay and the P1 latencies of the second CI when the second CI was activated. CONCLUSION: Changes in cortical auditory responses over time resulted in normal auditory maturation in children with sequential bilateral cochlear implants during the sensitive period. The results also provide evidence that the timing of auditory experience in the first ear during the sensitive period may influence the speed of compensation in children receiving sequential cochlear implants.

Psychoacoustic abilities in pediatric cochlear implant recipients: The relation with short-term memory and working memory capacity.

OBJECTIVE: The aim was to investigate school-age children with cochlear implants (CIs) and their typically developing peers in terms of auditory short-term memory (ASTM), auditory working memory (AWM), visuospatial short-term memory (VSTM), visuospatial working memory (VWM), spectral resolution and monosyllabic word recognition in noise. METHODS: Twenty-three prelingually deaf CI users and twenty-three typically developing (TD) peers aged 7-10 years participated. Twelve children with CI were earlier-implanted (i.e., age at implantation ≤24 months). Children with CIs were compared to typically developing peers and correlations between cognitive and psychoacoustic abilities were computed separately for the groups. Besides, regression analyses were conducted to develop models that could predict SMRT (spectral-temporally modulated ripple test) and speech recognition scores. RESULTS: The AWM scores of the later-implanted group were significantly lower than both earlier-implanted and TD groups. ASTM scores of TD children were significantly higher than both earlier-implanted and later-implanted participants. There was no statistically significant difference between groups in terms of VSTM and VWM. AWM performance was positively correlated with ASTM, SMRT scores, and speech recognition under noisy conditions for pediatric CI recipients. The AWM was a statistically significant predictor of the SMRT score and the SMRT score was an indicator of speech recognition score under 0 dB SNR condition. CONCLUSION: Most of children using CI are at risk for clinically remarkable deficits across cognitive abilities such as AWM and ASTM. While evaluating cognitive and psychoacoustic abilities in the clinic routine, it should be kept in mind that they can be influenced by each other.

Listening Effort in Prelingual Cochlear Implant Recipients: Effects of Spectral and Temporal Auditory Processing and Contralateral Acoustic Hearing.

OBJECTIVE: Considering the impact of listening effort (LE) on auditory perception, attention, and memory, it is a significant aspect in the daily hearing experiences of cochlear implant (CI) recipients. Reduced spectral and temporal information on an acoustic signal can make listening more difficult; as a result, it is important to understand the relationship between LE and spectral and temporal auditory processing capacities in CI receivers. STUDY DESIGN, SETTING, AND PATIENTS: This study used spectral ripple discrimination and temporal modulation transfer function to evaluate 20 prelingually deafened and early implanted CI recipients. The speech perception in noise test (primary) and the digit recall task (DRT-secondary) were used to assess LE using the dual-task paradigm. To assess the effects of acoustic hearing, contralateral acoustic hearing thresholds between 125 Hz and 8 kHz with a hearing aid were also acquired. To examine the relationship between the research variables, correlation coefficients were generated. Furthermore, the Mann-Whitney U test was used to compare unilateral and bimodal users. RESULTS: There was a statistically significant correlation between LE and spectral ripple discrimination (r = 0.56; p = 0.011), 125 Hz (r = 0.51; p = 0.020), 250 Hz (r = 0.48; p = 0.030), 500 Hz (r = 0.45; p = 0.045), 1,000 Hz (r = 0.51; p = 0.023), 2000 Hz (r = 0.48; p = 0.031), and 4,000 Hz (r = 0.48; p = 0.031), whereas no statistically significant correlations were observed between temporal modulation transfer function in four frequencies and LE. There was no statistically significant difference between unilateral and bimodal CI recipients ( p > 0.05). CONCLUSION: As a result of the improved signal-to-noise ratio in the auditory environment, CI users with better spectral resolutions and acoustic hearing have a reduced LE. On the other hand, temporal auditory processing, as measured by temporal modulation detection, does not contribute to the LE.

The body image in hearing aid and cochlear implant users in Turkey.

PURPOSE: We investigated the relationship between hearing aid or cochlear implants usage and body image, and quality of life. METHODS: Of the 96 participants studied, 26 were cochlear implant users, 20 hearing aid users, 20 non-device users with hearing loss, and 30 normal hearing. The inclusion criteria for the study for all groups were subjects 18 years or older with no other physical complaints. The average age of groups and gender distributions were similar. The Body Image Scale (BIS) and World Health Organization Quality of Life Scale were applied to all participants, whereas the International Outcome Inventory for Hearing Aids, Turkish Version, was applied only to device users. RESULTS: A significant difference in body image scores was identified between non device users with hearing loss group and normal hearing group, device users group (HA/CI), and cochlear implant users group. In contrast, no significant difference in body image scores between normal hearing group and the device users group was identified. Finally, a positive correlation between body image scores and quality of life scores in all groups was identified. CONCLUSIONS: Non-device users with hearing loss have a lower body image and quality of life than those in the other groups, and device usage has a positive effect on body image and quality of life.

Translation and validation of the music-related quality of life questionnaire for adults with cochlear implant in Turkish language.

PURPOSE: It is important to assess the impact of music on cochlear implant (CI) users' quality of life. The aim of this study was to adapt and validate the music-related quality of life questionnaire into the Turkish language for adult CI users. METHODS: 161 CI users and 162 normal-hearing adults were included in the study. The final Turkish version of the questionnaire was prepared and evaluated for validity and reliability. The internal consistency of the questionnaire and test-retest reliability were evaluated by Cronbach's α and ICC index. Factor analysis and 'know-group' method was used to determine the construct validity. RESULTS: Sampling adequacy for execution of factor analysis was confirmed by the results of Kaiser-Meyer-Olkin (= 0.91) and Bartlett test (p < 0.05). 2 factors for each scale were identified from exploratory factor analysis. Confirmatory factor analysis confirmed the questionnaire met the criteria standards for adequacy of fit. The reliability coefficient was determined at least 0.80. Correlation between items indicated excellent (> .80) internal consistency. CONCLUSION: The Turkish version of the questionnaire has good validity and reliability and can be used to investigate the relationship between music and quality of life and as a diagnostic tool in identifying individuals who need music support and to guide and evaluate music rehabilitation.

Effects of spatial separation with better- ear listening on N1-P2 complex.

OBJECTIVE: The purpose of this study was to determine better- ear listening effect on spatial separation with the N1-P2 complex. METHODS: Twenty individuals with normal hearing participated in this study. The speech stimulus /ba/ was presented in front of the participant (0°). Continuous Speech Noise (5 dB signal-to-noise ratio) was presented either in front of the participant (0°), left-side (-90°), or right-side (+90°). N1- P2 complex has been recorded in quiet and three noisy conditions. RESULTS: There was a remarkable effect of noise direction on N1, P2 latencies. When the noise was separated from the stimulus, N1 and P2 latency increased in terms of when noise was co-located with the stimulus. There was no statistically significant difference in N1-P2 amplitudes between the stimulus-only and co-located condition. N1-P2 amplitude was increased when the noise came from the sides, according to the stimulus-only and co-located conditions. CONCLUSION: These findings demonstrate that the latency shifts on N1-P2 complex explain cortical mechanisms of spatial separation in better-ear listening.

Music Listening Habits and Music Perception Abilities of Prelingually Deafened Adolescent Cochlear Implant Recipients.

BACKGROUND: Music is a very important art form that can evoke strong emotions and involvement with it can have a considerable impact on cochlear implant (CI) recipients' quality of life. It is well known that music perception is limited in CI with fully electric hearing and CI recipients have significantly poorer music-appraisal ability when compared with normal hearing subjects. Considering the importance of music on the life of an adolescent and negative impact of hearing loss on mental health, understanding and improving music listening habits and perception abilities can have a positive impact on the mental health of CI user adolescents. PURPOSE: This study aims to evaluate music listening habits and music perception abilities of prelingually deafened adolescent CI users to reveal the relationship between self-reported assessment and psychophysical measurement. RESEARCH DESIGN: The psychophysical measures of music perception and self-reported music listening habits of adolescent CI users were obtained. STUDY SAMPLE: Twenty-nine prelingually deafened adolescent CI users were evaluated in this study. DATA COLLECTION AND ANALYSIS: The Turkish Clinical Assessment of Music Perception (T-CAMP) test was used to evaluate music perception abilities. These abilities were evaluated with a three-item questionnaire assessing enjoyment of music, listening habits, and comprehension of lyrics. Pearson's correlation coefficients were calculated to assess the relationship between the T-CAMP subtests and questionnaire items. RESULTS: Statistically significant correlations were observed between the melody-recognition subtest of T-CAMP and all three questionnaire items. The correlation between the enjoyment and rate items on the questionnaire was also statistically significant. CONCLUSION: The present study shows that adolescent CI users also enjoy and listen to music in ways similar to postlingually deafened adult CI users. It also demonstrates that melody-recognition abilities have a significant relationship with music enjoyment and listening habits in prelingually deafened adolescent CI users.

The effects of behind-the-ear and off-the-ear sound processors on speech understanding performance in cochlear implant users.

OBJECTIVE: The first aim of this study was to compare speech understanding performance in adult cochlear implant users using behind-the-ear and off-the-ear sound processors in quiet and noise. Second, the impact of sound processor microphone location on speech understanding performance was compared. METHODS: Participants were tested with both types of sound processors in the free field with warble tones, and speech discrimination scores in quiet were obtained. Turkish Matrix Test was used to measure speech understanding in noise with five different loudspeaker settings. Twenty-seven participants of cochlear implants between 16 and 67 years-of-age using behind-the-ear or off-the-ear sound processors were included in the study. The results obtained with two types of sound processors were compared. RESULTS: Aided free field thresholds were significantly better for the behind-the-ear vs. off-the-ear sound processor. The mean difference was 3.3 dB HL. There was no significant difference in speech discrimination scores in quite between the two processors (p > 0.05). Statistically significant differences were not seen for speech intelligibility in five spatial settings in the Turkish Matrix Test (p > 0.05). CONCLUSION: Although both types of sound processors had different microphone locations, the outcomes were found to be consistent with previous results in adult users off-the-ear sound processors, demonstrating equivalent speech understanding in quiet and noise.

Data logging variables and speech perception in prelingually deafened pediatric cochlear implant users.

OBJECTIVES: To investigate the relationship among objectively gathered data logging measurements, patient-related variables, and speech recognition performance of pediatric CI users. METHODS AND MATERIALS: Thirty-two prelingually implanted children who have the ability to perform word discrimination test were included in this study. To reveal the relationship between speech perception abilities and auditory exposure, seven data logging variables were analyzed: "on-air," "off-air," "coil-off," "speech," "speech in noise," "music" and "noise. In addition, implantation age (months) and CI usage duration (months) were taken into account. Finally, it was investigated the differences between unilateral, sequential bilateral, and simultaneous bilateral CI users in terms of all study variables. RESULTS: The average on-air time ranged between 10.52 and 12.30 in the groups. In the case of sequential implantation, smaller on-air and higher coil off values were observed with the second CI. In the case of simultaneous bilateral implantation, data logging measurements were almost the same in both implants. WRS was significantly correlated (p < 0.05) with on-air time (r = 0.62), coil-off count (r = -0.48), chronological age (r = 0.48), and CI duration (r = 0.44). Multiple linear regression model was fit to predict the WRS, with on-air time, CI duration, and chronological age as predictors. CONCLUSIONS: The critical importance of early intervention and long-term use of CI is well-established in the literature and is also corroborated by our findings. However, the key findings of the present study are that consistent CI use and the quality of daily listening environment also exerted a major and positive effect on the speech recognition performance of pediatric CI users. Therefore, during the monitoring of pediatric CI recipients, it is important to know the device usage data in order to detect problems in the early stages after CI.

Music and psychoacoustic perception abilities in cochlear implant users with auditory neuropathy spectrum disorder.

OBJECTIVE: Auditory neuropathy spectrum disorder (ANSD) is a condition wherein the pre-neural or cochlear outer hair cell activity is intact, but the neural activity in the auditory nerve is disrupted. Cochlear implant (CI) can be beneficial for subjects with ANSD; however, little is known about the music perception and psychoacoustic abilities of CI users with ANSD. Music perception in CI users is a multidimensional and complex ability requiring the contribution of both auditory and nonauditory abilities. Even though auditory abilities lay the foundation, the contribution of patient-related variables such as ANSD may affect the music perception. This study aimed to evaluate the psychoacoustic and music perception abilities of CI recipients with ANSD. STUDY DESIGN: Twelve CI users with ANSD and twelve age- and gendermatched CI users with sensorineural hearing loss (SNHL) were evaluated. Music perception abilities were measured using the Turkish version of the Clinical Assessment of Music Perception (T-CAMP) test. Psychoacoustic abilities were measured using the spectral ripple discrimination (SRD) and temporal modulation transfer function (TMTF) tests. In addition, the age of diagnosis and implantation was recorded. RESULTS: Pitch direction discrimination (PDD), timbre recognition, SRD, and TMTF performance of CI users with ANSD were concordant with those reported in previous studies, and differences between ANSD and SNHL groups were not statistically significant. However, the ANSD group performed poorly compared with SNHL group in melody recognition subtest of T-CAMP, and the difference was statistically significant. CONCLUSION: CI can prove beneficial for patients with ANSD with respect to their music and psychoacoustic abilities, similar to patients with SNHL, except for melody recognition. Recognition of melodies requires both auditory and non-auditory abilities, and ANSD may have an extensive but subtle effect in the life of CI users.

The Relationship between Electrical Stapedius Reflex Thresholds and Behaviorally Most Comfortable Levels in Experienced Cochlear Implant Users.

OBJECTIVES: Programming the cochlear implant's speech processor with subjective methods in young, uncooperative children is difficult. Since young children cannot provide adequate feedback to the clinician, objective methods which do not require patient's response were often used. Electrical Stapedius Reflex Test is one of the most common procedures used. The goal of this study was to investigate the relationship between electricalstapedius reflex thresholds and behaviorally most comfortable levels in experienced cochlear implant users. MATERIALS AND METHODS: The patients implanted with two brands of cochlear implants were compared in terms of electrical stapedius reflex thresholds versus most comfortable levels. Speech tests results were also compared between the two groups. 46 cochlear implanted patients who had at least 1 year of cochlear implant experience were included in the study. 28 patients were implanted with Nucleus, 18 patients with Med-El devices. RESULTS: Moderate correlations were obtained between Electrical Stapedius Reflex (ESR) thresholds and C levels in Nucleus users; higher correlations were obtained for Med-El patients. ESR thresholds were present at higher levels than MCL/C levels in both Nucleus and Med-El users. No significant difference was obtained between the two groups in terms of speech tests. CONCLUSION: ESR test can be very informative for programming young and uncooperative patients.

The effect of canal diameter on audiologic results in patients with cochlear implantation with large vestibular aqueduct syndrome.

PURPOSE: To compare audiologic results according to vestibular aqueduct (VA) diameter in patients who have undergone cochlear implantation and were diagnosed with LVAS. METHODS: This was a retrospective study detailing the outcomes of 18 patients with LVAS and 18 patients undergone cochlear implants. VA diameter was assessed by magnetic resonance imaging and computed tomography. Categories of Auditory Perception (CAP) and Speech Intelligibility Rating (SIR) were assessed in all patients, and speech audiometry, including speech recognition thresholds (SRT) and word discrimination scores, was applied for all subjects who were able to perform these tests. All audiologic parameters were compared between patients with and without LVAS, and the relationship of these parameters with VA diameter was investigated. RESULTS: The control group consisted of 18 subjects (5 males, 13 females), ranging in age between 2 and 34 years (mean 13.17 ± 8.97 years). The research group consisted of 18 subjects (8 males, 10 females), ranging in age between 2 and 35 years (mean 13.28 ± 8.96 years). There was a statistically significant difference between the groups in terms of SIR and CAP pre-post differences (Mann-Whitney U test, p < 0.05), with higher averages in the LVAS group. No statistically significant correlations were found between VA diameter on computed tomography and magnetic resonance imaging and the audiologic variables collected. CONCLUSIONS: Patients with LVAS benefit from cochlear implant surgery and VA parameters do not affect audiologic parameters.

Evaluation of Speech, Spatial Perception and Hearing Quality in Unilateral, Bimodal and Bilateral Cochlear Implant Users.

OBJECTIVE: The aim of the study was to conduct a scale-based evaluation of the hearing skills of unilateral, bimodal and bilateral cochlear implant (CI) users, including distinguishing, orientating and locating speech and environmental sounds in their surrounding environment that they are exposed to in different contexts of everyday life. The scale results were compared between groups. METHODS: A total of 74 cochlear implant users, 30 unilateral, 30 bimodal and 14 bilateral, were included in the study. Their ages ranged from 11 to 64 years. Participants were assessed using the Speech, Spatial and Qualities of Hearing Scale (SSQ). RESULTS: Bilateral CI users' subjective ratings of their own hearing skills were found to be significantly better than those of bimodal and unilateral CI users; bimodal users' subjective ratings were also found to be significantly better than those of unilateral CI users. Paired comparisons showed statistically significant differences between the groups in terms of total scores of Speech, Spatial, Qualities of Hearing and General SSQ (p<0.05). CONCLUSION: Our findings show that bilateral use of cochlear implants should be recommended for those presently using bimodal and unilateral devices. Moreover, subjective tests should be used regularly along with objective tests for evaluating CI patients.

Outcomes of cochlear implantation in children with inner ear malformations.

PURPOSE: To evaluate the surgical experience and auditory functions and progress of speech development of cochlear implantation in malformed ears. MATERIALS AND METHODS: Between November 1995 and July 2017, thirty-seven patients (26 females and 11 males; mean age: 138.275 ± 96.24 months) with diverse anomalies of the inner ear were retrospectively examined for surgical and audiological results. Speech Intelligibility Rating (SIR), Categories of Auditory Perception (CAP), Pure Tone Average (PTA), Speech Intelligibility Rating (SRT), and Word Discrimination Score (WDS) were the audiological tests used to evaluate the efficacy of CI in the malformed inner ears. RESULTS: CSF gusher was experienced by six patients (three with LVA (large vestibular aqueduct), one with IP (incomplete partition) I, and two with both IP II Mondini malformations and LVA). Two patients had transient facial paresis after surgery. All patients fully recovered within 6 months. The postoperative PTA, SRT, and WDS test results showed significant differences between progressive and congenital sensorineural hearing loss (p values < 0.05 for all). On the other hand, the postoperative CAP and SIR test results revealed no significant differences between the two groups. According to etiology, the PTA and SRT values were significantly lower in common cavity patients than the LVA patients (p values < 0.01); no significant differences were found among the other etiological groups. CONCLUSIONS: Cochlear implantation is safe in children with inner ear malformations. However, the success rate is low compared to patients with normal anatomy in terms of audiological results; the most successful group of patients with inner ear malformation is large vestibular aqueduct.

A Case with Brown-Vialetto-Van Laere Syndrome: A Sudden Onset Auditory Neuropathy Spectrum Disorder.

The Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disorder that may present at all ages with sensorineural hearing loss, bulbar palsy and respiratory compromise. We describe a 6-year-old male patient who suffered bilateral sudden onset severe hearing loss for two years. Audiological investigations revealed sudden onset auditory neuropathy spectrum disorder bilaterally. He also had neurological complaints. During riboflavin therapy an improvement in hearing loss and the benefit of hearing aids were observed. In BVVLS, it is difficult to plan and apply auditory rehabilitation interventions and the results vary from patient to patient. In audiological evaluation, it should be borne in mind that subjective and objective tests are complemental. Early medical intervention and regular audiological follow-up are very important for effective hearing rehabilitation in the patients with BVVLS.