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Objective: Cochlear nerve deficiency (CND) is often combined with modiolar deficiency-type inner ear malformations, which cause variable cochlear implantation (CI) outcomes. We aimed to assess the postoperative development of auditory and speech perception in CND patients with modiolar deficiency-type malformations after 3 years of follow-up to determine the factors correlated with CI outcomes., Methods: Sixty-seven CND patients with modiolar deficiency-type malformations who underwent CI surgery were retrospectively reviewed. Modiolar deficiency-type malformations included common cavity (CC), cochlear hypoplasia (CH) (including CH-I and CH-II) and incomplete partition-I (IP-I). Categorical auditory performance (CAP) and the infant-toddler meaningful auditory integration scale (MAIS) were used to assess auditory ability. The speech intelligibility rating (SIR) and meaningful use of speech scale (MUSS) were used to assess the speech intelligibility of these CI patients. The CI outcomes were evaluated at 0, 12, 24 and 36 months after implant activation., Results: All patients demonstrated improvements in auditory ability and speech intelligibility after CI. There were no significant differences in CI outcomes at any time point according to the malformation type. The number of nerve bundles within the internal auditory canal (IAC) showed significant differences at 12, 24 and 36 months after CI ( p < 0.05). Patients with one nerve bundle had relatively poor CI outcomes., Conclusions: CND patients with modiolar deficiency-type malformations showed continuous improvement in auditory and speech abilities after CI. Compared with malformations, the number of nerve bundles should be given more attention when selecting the side for CI., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: The purpose of the present study was to compare spoken word recognition performance in the presence of speech spectrum-shaped noise and four-talker babbles in Mandarin-speaking children with cochlear implants (CIs)., Methods: Participants included 33 children with unilateral CIs (with a mean age of 10.4 ± 2.9 years old and a mean length of CI use of 7.5 ± 3.0 years). The Standard Chinese version of Lexical Neighborhood Test was implemented in quiet, speech-spectrum-shaped noise (SSN), and four-talker babble (FTB). The signal-to-noise ratios (SNRs) were set at +5 and + 10 dB for both types of maskers. Participants responded by verbally repeating each word they heard and the response was scored as the percentage accuracy of recognition performance. A Generalized Linear Model (GLM) fitting, correlational tests, and a two-way repeated-measures ANOVA were conducted on the percent-correct data., Results: Word recognition in quiet was on average 74.5% correct but dropped to 57.3% and 48.8% correct for SSN and FTB at 10 dB SNR, respectively, and 44.4% and 32.6% correct for SSN and FTB at 5 dB SNR, respectively. In both quiet and noise conditions, the participants showed lower recognition accuracy for the hard words than for the easy words. Disyllabic words were recognized with higher accuracy rates than were the monosyllabic words. The GLM analysis revealed that all four tested factors (masker type, SNR, lexical neighborhood feature, and lexical type) showed significant impacts on word recognition in children with CIs. Word recognition scores in the two types of maskers were significantly correlated for the disyllabic words at both SNRs and monosyllabic words at 10 dB SNR., Conclusions: The present study demonstrated that the lexical features such as the lexical neighborhood characteristics and lexical type had significant effects on speech recognition performance in both quiet and noise conditions in pediatric CI users. Children with years of experience of CI use still encountered remarkable difficulties in everyday listening environment although their speech recognition in quiet reached relatively desired level. Fluctuating noise, such as speech babbles, caused greater challenge than steady-state noise for speech recognition in children with CIs., (Copyright © 2018. Published by Elsevier B.V.)

Background: Cochlear implants (CIs) can improve speech recognition for children with severe congenital hearing loss, and open-set word recognition is an important efficacy measure. This study examined Mandarin open-set word recognition development among Chinese children with CIs and normal hearing (NH)., Methods: This study included 457 children with CIs and 131 children with NH, who completed the Mandarin lexical neighborhood test. The results for children at 1-8 years after receiving their CIs were compared to those from the children with NH using linear regression analysis and analysis of variance., Results: Recognition of disyllabic easy words, disyllabic hard words, monosyllabic easy words, and monosyllabic hard words increased with time after CI implantation. Scores for cases with implantation before 3 years old were significantly better than those for implantation after 3 years old. There were significant differences in open-set word recognition between the CI and NH groups. For implantation before 2 years, there was no significant difference in recognition at the ages of 6-7 years, compared to 3-year-old children with NH, or at the age of 10 years, compared to 6-year-old children with NH. For implantation before 3 years, there was no significant difference in recognition at the ages of 8-9 years, compared to 3-year-old children with NH, or at the age of 10 years, compared to 6-year-old children with NH. For implantation after 3 years, there was a significant difference in recognition at the age of 13 years, compared to 3-year-old children with NH., Conclusions: Mandarin open-set word recognition increased with time after CI implantation, and the age at implantation had a significant effect on long-term speech recognition. Chinese children with CIs had delayed but similar development of recognition, compared to normal children. Early CI implantation can shorten the gap between children with CIs and normal children.

Objective: The objective of the present study was to investigate the longitudinal performance on open-set word perception in Mandarin children with cochlear implants (CIs)., Methods: Prospective cohort study. One hundred and five prelingually deaf children implanted with CIs participated in the study. The Standard-Chinese Version of Monosyllabic Lexical Neighborhood Test (LNT) and Multisyllabic Lexical Neighborhood Test (MLNT) were used as open-set word perception evaluation tools. Evaluations were administrated at 6, 12, 24, 36, 48, 60, 72, and 84 months post CI stimulation, respectively., Results: (1) Spoken word perception performance of congenitally deaf children with CIs improved significantly over time. (2) The fastest improvement occurred in the first 36 months after initial activation, then the improvement slowed down and the final peak score of 81.7% correct was achieved at 72 months after initial activation. (3) Early implanted children exhibited better longitudinal performance. (4) Lexical factors affected consistently in each evaluation session. For lexically harder words, such as monosyllabic hard words, there was substantial room for improvement even after long-term use of CI., Conclusions: (1) CI continuously provided significant benefits in word perception to children with severe/profound sensorineural hearing loss. (2) Age at implantation and Mandarin lexical factor affected longitudinal performance significantly., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Conclusion: This study indicates that Mandarin-speaking subjects acquire significant benefit from a cochlear implant (CI) and audio processor with the fine structure processing (FSP) coding strategy. It can take adult Mandarin CI users time to become accustomed to their CI and appreciate the benefits., Objectives: This study aimed to determine speech perception outcomes in Mandarin-speaking adults who received the FSP coding strategy., Methods: This was a prospective study. Ten adults who were implanted unilaterally with a PULSARCI(100), and received an OPUS 1 or OPUS 2 speech processor, were included in this study. The mean age at implantation was 31.1 years. We determined the benefit to postlingually deafened Mandarin-speaking adults with a severe to profound hearing loss using the tests: monosyllables in quiet, sentences in quiet and the Mandarin Hearing in Noise Test (MHINT). Tone perception was evaluated using a tone perception test. A visual analog scale (VAS) was used to score sound quality and ease of listening., Results: The results showed a significant improvement between first fitting and 6 months in all speech tests and in tone perception. The VAS scores showed a significant improvement over 6 months in sound quality and an immediate benefit in ease of listening.

Objective: To evaluate the abilities speech perception of post-lingual cochlear implant patients by Mandarin Hearing In Noise Test(MHINT), get the performance-intensity function (P-I function)., Method: The material was MHINT adults' version, including 12 lists, 20 sentences per list. Twenty-two cochlear implant patients were the subjects, age from 14 to 56. Three adaptive rules were used during the test. Firstly Rule 3 would be done. If subject could finish the test, then Rule 2 and Rule 1 would be used. Got the Reception threshold of sentences and speech recognition score of subjects, then got performance-intensity function (PI function)., Result: Among those 22 subjects, 5 of them could be tested in all three rules, 3 could be tested in Rule 2 and Rule 3, 7 of them could be tested with Rule 3, and 7 of the CI users could not be tested even with Rule 3. All of the PI functions for CI users are shifted 10 dB or more above the normal PI functions. Most CI users thought that MHINT was hard for them., Conclusion: When use new adaptive scoring rules for MHINT, most CI users could be tested with the modified HINT scoring rules, although three subjects could not be tested. Compared with normal hearing subjects, all CI users required positive S/N ratios to perform the HINT measures.

Cochlear implant (CI) users' speech understanding may be influenced by different speaking styles. In this study, speech recognition was measured in Mandarin-speaking CI and normal-hearing (NH) subjects for sentences produced according to four styles: slow, normal, fast, and whispered. CI subjects were tested using their clinical processors; NH subjects were tested while listening to a four-channel CI simulation. Performance gradually worsened with increasing speaking rate and was much poorer with whispered speech. CI performance was generally similar to NH performance with the four-channel simulation. Results suggest that some speaking styles, especially whispering, may negatively affect Mandarin-speaking CI users' speech understanding., (© 2011 Acoustical Society of America)

Conclusion: Not stimulating the apical cochlear region in tonal language speaking cochlear implantees significantly reduces discrimination of Mandarin vowels. The data presented here suggest that electrode arrays that allow complete cochlear coverage with stimulation pulses seem to be preferable over shorter arrays for use in cochlear implant (CI) indications., Objective: To assess the contribution of electrical stimulation beyond the first cochlear turn on tonal language speech perception., Methods: Twelve Mandarin-speaking users of the MED-EL COMBI 40+ cochlear implant with complete insertion of the standard COMBI 40+ electrode array participated in the study. Acute speech tests were performed in seven electrode configurations with stimulation either distributed over the whole length of the cochlea or restricted to the apical, middle or basal regions. The test battery comprised tone, consonant, and vowel identification in quiet as well as a sentence recognition task in quiet and noise., Results: While neither tone nor consonant identification depended crucially on the placement of the active electrodes, vowel identification and sentence recognition decreased significantly when the four apical electrodes were not stimulated.

Conclusion: The study demonstrates that two methods of segmentation, i.e. word and character segmentations, produce equivalent results in the Mandarin Hearing in Noise Test (MHINT). Potentially, both methods of segmentation can be used clinically. A majority of the Mandarin-speaking cochlear implant subjects could complete MHINT - with the more relaxed adaptive rules. The results make it possible to compare the performance of cochlear implant users across languages., Objectives: The primary purpose of the present study was to evaluate the modified adaptive scoring rules and to develop alternative methods of segmentations in MHINT that are suitable for the Chinese language., Methods: Thirty Mandarin-speaking normal-hearing adults were tested with MHINT using three adaptive rules based on character and/or word segmentation of the sentences. Twenty-three Mandarin-speaking post-lingually deafened cochlear implant patients were also recruited to participate in the testing., Results: There were no significant differences in the reception threshold for sentences and speech recognition scores obtained with either method of segmentation (p > 0.05). Fifteen of the 23 cochlear implant subjects (65%) could be tested with the modified adaptive scoring rules. The performance-intensity functions of the cochlear implant subjects were shifted at least 8-10 dB to higher signal-to-noise ratios as compared with those of the normal-hearing subjects.

Objective: To develop two versions of the Mandarin Hearing In Noise Test (MHINT). These tests are adaptive tests that measure the reception threshold for sentences (RTSs) in quiet and in noise. The RTS is the presentation level at which half the sentences are correctly recognized., Design: Four studies were undertaken to (1) develop sentence materials, (2) equalize sentence difficulty, (3) create phonemically balanced sentence lists; and (4) evaluate within-list response variability, inter-list reliability, and produce normative data. A total of 137 native Mandarin (Putonghua) speaking subjects in Mainland China and 89 native Mandarin speakers in Taiwan participated. They had normal hearing thresholds at 25 dB HL or better. RTSs were measured under four headphone test conditions: Quiet, and in noise with noise originating from the 0 degree (Noise Front), 90 degrees to the right (Noise Right), and 90 degrees to the left (Noise Left). The speech originated from the front (0 degree) in all conditions. The noise level was fixed at 65 dBA, and the speech was varied adaptively to find the RTS., Results: Two versions of the test materials, consisting of 24, 20-sentence lists each in Mandarin spoken in the Mainland (the MHINT-M) and the dialect of Mandarin spoken in Taiwan (the MHINT-T), were created from two sets of 240 sentences containing 10 syllables per sentence. The mean Quiet RTS was 14.7 dBA, using the MHINT-M, and 19.4 dBA, using the MHINT-T. Using the MHINT-M, the mean RTS for Noise Front was -4.3 dB signal-to-noise ratio (SNR), -11.7 dB SNR for Noise Right, and -11.7 dB SNR for Noise Left. Using the MHINT-T, the Noise Front RTS was -4.0 dB SNR, -10.7 dB SNR for Noise Right, and -11.0 dB SNR for Noise Left. Results in noise are slightly better than those seen for the English HINT norms. Response variability within list was low, and inter-list reliability was high, indicating that consistent results can be obtained using any list. Confidence intervals are reported., Conclusions: The two versions of the MHINT are the first standardized Mandarin sentence speech intelligibility tests. Similar to other language versions of the HINT, the MHINT was developed using the same rationale as the English HINT, allowing norm-referenced results for the MHINT to be compared directly with results in other languages. The MHINT would benefit from further evaluation of its validity.

Conclusions: The data collected in this study indicated that first Neural Response Imaging (NRI) thresholds had a better correlation with HiResolution most comfortable loudness (M) levels than tNRI thresholds. Electrically evoked auditory reflex thresholds (EARTs) had a higher correlation with HiResolution M levels than tNRI thresholds and a lower correlation than first NRI thresholds. NRI is a very useful method for programming the cochlear implants of young children who cannot demonstrate a reliable judgment of loudness., Objective: To investigate how HiResolution sound processing, designed to deliver high-rate stimuli, relates to EARTs and electrically evoked compound action potential measurements produced by low-rate stimuli., Material and Methods: Nine profoundly hearing-impaired children and adults aged 6-29 years participated in the study. NRI responses were elicited using pulse trains consisting of biphasic pulses at a pulse width per phase of 32 micros delivered at a frequency of 30 Hz using SoundWave programming software. Stimuli were delivered to the odd electrodes (1, 3, 5, 7, 9, 11, 13 and 15) along the array. tNRI (NRI threshold) and first NRI thresholds were recorded for each stimulating electrode. "Speech bursts" stimuli used in EARTs recording were delivered to four electrodes at a time and stapedial reflexes were recorded from the impedance bridge. The M levels used were those used by each patient in their everyday HiResolution programs., Results: For 8 patients (53 stimulating electrodes) the correlation between tNRI threshold and M level was r=0.675 (p=0.000) and that between first NRI thresholds and M level was r=0.741 (p=0.000). On average the M-level value was 20 CU (Current Unit) lower than the first NRI threshold value and 12 CU higher than the tNRI threshold value. The M-level patterns across the electrode array overall were similar to the tNRI or first NRI threshold patterns. For 7 patients (112 stimulating electrodes) the correlation between EART and M levels was r=0.710 (p=0.000). On average the EART value was 14 CU higher than the M-level value.

Objective: The aim of this research is to analyse and compare the psychophysical tests in different age of patients with a cochlear implant., Method: One hundred and seventy patients with Nucleus CI24M cochlear implant system participated in this study. The patients were divided into four groups according to their age. They were group A (<3 year), B(3-<6 year) C(6-12 year) and D(>12 year). The psychophysical tests included T and C-Level evaluation., Result: C-Levels of each electrode (except electrode No. 20) were significantly different between group A and D at the time of one month after switch-on (P<0.01, P<0.05 for the No. 2, 18, 19, 21, 22 electrode). The normalized differences between used C-Levels and C-Levels for group D were significantly higher than the other three groups at the switch-on session (P<0.01, P<0.05 between group D and C). The normalized differences between used C-Levels and C-Levels were significantly different for the group A and D at one week after first programming session (P<0.05)., Conclusion: T and C-Level for different age groups were different. The normalized differences between used C-Levels and C-Levels were significantly different among the four groups in the few weeks after switch-on.

Objective: To provide an objective method to estimate T-levels and C-levels using neural response telemetry (NRT) thresholds for children, from whom we can not get accurate responses because they are very young or have other disabilities, by comparing NRT thresholds and T/C levels., Methods: Seventy patients implanted with the nucleus CI24M multiple cochlear implant system participated in this study. 329 electrodes were tested. The software used in this study was NRT 2.04. Monopolar stimulation mode was used during NRT threshold measurement. No. 3, 5, 10, 15, 20 electrodes were tested for each patient. The T-levels and C-levels were obtained at the same visit., Results: 92.7% of all the tested electrodes recorded NRT responses. There were variations in the amplitudes and thresholds of NRT responses across subjects and electrodes. NRT thresholds fell within the different points of the MAP dynamic range. The mean of NRT thresholds was shown to fall between the mean of T-levels and C-levels. The NRT thresholds and T/C levels reduced from basal to apical ends of the cochlear., Conclusions: Considerable variability across subjects was noted. So it is difficult to estimate T-levels and C-levels accurately by testing only NRT thresholds only. NRT technology provides a new objective method for estimating T-levels and C-levels for children who are unable to cooperate with audiologists during mapping after operation. The new NRT technology should be improved in the future.

Objective: This study aimed to assess the correlation between residual hearing and audiologic outcomes after cochlear implant (CI) surgery in patients with cochlear nerve deficiency (CND). Methods: This retrospective study included 57 patients with CND who underwent CI surgery. Patients were divided into four groups according to hearing level (80–95, 95–110, 110–120, and >120 dB) and three groups according to residual hearing (entire spectrum hearing, partial spectrum hearing, and no spectrum hearing) based on the measured response at each frequency. Auditory performance (categorical auditory performance [CAP], Infant–Toddler Meaningful Auditory Integration Scale [IT‐MAIS]) and speech perception (speech intelligibility rating [SIR] and meaningful use of speech scale [MUSS]) were assessed before and 2 years after the surgery. Results: Forty‐seven (82.5%) patients had complete or total hearing loss (≥95 dB) and 17 (29.8%) had no spectrum hearing before CI surgery. Twenty‐nine (50.9%) patients did not exhibit residual hearing at 4 kHz. All patients demonstrated an improvement in auditory performance and speech perception: the CAP score in the 80–95 dB group was significantly higher than that in the 110–120 and >120 dB groups, and the entire spectrum hearing group showed significantly higher CAP, SIR, and IT‐MAIS scores than the partial spectrum hearing group and significantly higher CAP, SIR, IT‐MAIS, and MUSS scores than the no spectrum hearing group. Conclusion: For patients with CND, residual hearing, especially high‐frequency residual hearing, was poor and postoperative audiologic outcomes were significantly associated with the range of residual hearing. Level of Evidence: 4 [ABSTRACT FROM AUTHOR]

Cochlear nerve deficiency (CND) is often associated with variable outcomes of cochlear implantation (CI). We assessed previous investigations aiming to identify the main factors that determine CI outcomes, which would enable us to develop predictive models. Seventy patients with CND and normal cochlea who underwent CI surgery were retrospectively examined. First, using a data-driven approach, we collected demographic information, radiographic measurements, audiological findings, and audition and speech assessments. Next, CI outcomes were evaluated based on the scores obtained after 2 years of CI from the Categories of Auditory Performance index, Speech Intelligibility Rating, Infant/Toddler Meaningful Auditory Integration Scale or Meaningful Auditory Integration Scale, and Meaningful Use of Speech Scale. Then, we measured and averaged the audiological and radiographic characteristics of the patients to form feature vectors, adopting a multivariate feature selection method, called stability selection, to select the features that were consistent within a certain range of model parameters. Stability selection analysis identified two out of six characteristics, namely the vestibulocochlear nerve (VCN) area and the number of nerve bundles, which played an important role in predicting the hearing and speech rehabilitation results of CND patients. Finally, we used a parameter-optimized support vector machine (SVM) as a classifier to study the postoperative hearing and speech rehabilitation of the patients. For hearing rehabilitation, the accuracy rate was 71% for both the SVM classification and the area under the curve (AUC), whereas for speech rehabilitation, the accuracy rate for SVM classification and AUC was 93% and 94%, respectively. Our results identified that a greater number of nerve bundles and a larger VCN area were associated with better CI outcomes. The number of nerve bundles and VCN area can predict CI outcomes in patients with CND. These findings can help surgeons in selecting the side for CI and provide reasonable expectations for the outcomes of CI surgery. [ABSTRACT FROM AUTHOR]

Purpose: Owing to the characteristic anatomy, cochlear implantation (CI) for common cavity deformity (CCD) has resulted in varied outcomes and frequent facial and vestibular nerve stimulation. The current study analyzed the correlation among the distance between each electrode and cavity wall (abbreviation, D), programming parameters, and performances outcomes. Materials and Methods: The current, retrospective study included 25 patients (27 ears) with CCD underwent CI. The multiplanar volume reconstruction (MPVR) techniques were employed to reconstruct and evaluate the postoperative temporal bone CT. The D and maximum comfortable level (MCL) 6 months after CI, facial and vestibular nerve stimulation, and outcomes 1, 2, and 3 years after CI pertaining to the questionnaires were documented and analyzed. Results: The patients were divided into symptomatic (10, 37%) and asymptomatic (17, 63%) groups according to with or without facial and vestibular nerve stimulation. The MCL pertaining to the symptomatic group was significantly lower than asymptomatic group, but Categories of Auditory Performance (CAP) scores 1 year after surgery was better (p < 0.05). The subjects were divided into flat (12, 44.4%) and curved (15, 55.6%) groups based on the contour of MCL map. The MCL and D were lower and shorter in the curved group than the flat group, and CAP score 1 year after surgery and Speech Intelligibility Rating (SIR) 3 years after surgery were better (p < 0.05). Conclusion: Although abnormal reactions such as facial and vestibular nerve stimulation were observed to be more frequent, lower MCL and better outcomes were observed in relation to the shorter D. [ABSTRACT FROM AUTHOR]

The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children received cochlear implantation. Based on whether or not there was a Mondini dysplasia as diagnosed with CT scanning, the subjects were divided into 2 groups: (A) 514 young children with radiologically normal inner ears and (B) 31 young children with Mondini dysplasia. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess the children's auditory skills that include vocalization changes, spontaneous alerting to sounds in everyday living environments, and the ability to derive meaning from sounds. The assessment was performed prior to surgery and at 1, 3, 6, 9, 12, 24, and 36 months after implant device switch-on. The mean scores for overall auditory skills were not significantly different between groups A and B at pre-surgery, 1, 12, 24, and 36 months post-surgery, but were significantly different at 3, 6, and 9 months post-surgery. The mean scores for all auditory skills in children with Mondini dysplasia showed significant improvement over time. The mean scores for the three subcategories of auditory skills in children with Mondini dysplasia also showed significant differences at pre-surgery, 1, 3, 6, and 9 months, however, there were no significant differences at 12, 24, and 36 months. Overall, the auditory skills of young children with Mondini dysplasia developed rapidly after cochlear implantation, in a similar manner to that of young children with radiologically normal inner ears. Cochlear implantation is an effective intervention for young children with Mondini dysplasia. [ABSTRACT FROM AUTHOR]

Conclusion: Most of the infants demonstrated rapid improvement in the three different auditory skills within the first year after switch-on. Infants undergoing hearing aid trial and habilitation demonstrated a significant positive effect on the development of auditory skills in comparison with infants without trial and habilitation. Objective: This paper aims to evaluate the auditory performance of infants of different age at cochlear implantation, emphasize the importance of the hearing aid trial and habilitation before implant, and provide baseline data of auditory development. Methods: In all, 259 infants with prelingually profound hearing loss participated in this study. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess auditory skills in infants at different intervals. Results: The mean scores for the auditory skills improved significantly over time. The mean scores of three skills for each group at each interval were significantly different. The mean scores of the auditory skills were significantly superior for infants undergoing hearing aid trial and habilitation in comparison with those of infants without hearing aid trial and habilitation. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development. Method: 435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant–Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills. Results: The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B. Conclusion: Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS. [Copyright &y& Elsevier]