Comparison of speech perception performance between the Nucleus 24 and Nucleus 24 Contour cochlear implant systems

OBJECTIVE: To compare the 1-, 3-, and 6-month postoperative speech perception scores obtained by a group of subjects who received the new perimodiolar array (Nucleus Contour) cochlear implant with those obtained by a group of subjects implanted with the straight electrodes of the previous-generation Nucleus 24 device. MATERIAL AND METHODS: The speech performance of 10 postlingually deafened adults implanted with the Nucleus Contour device was compared with that of matched controls who received the Nucleus 24 model. Objective measures included word and sentence speech recognition scores. RESULTS: Patients implanted with the Nucleus Contour device obtained significantly higher word and sentence recognition scores after short-term use of the implant compared to those obtained by patients implanted with the Nucleus 24 model. CONCLUSION: Further long-term studies are required to determine whether the Nucleus Contour CI recipients continue to improve over time.     

Comparative speech recognition results in eight subjects using two different coding strategies with the Nucleus 22 channel cochlear implant.

This study was designed to compare the results of several speech tests administered to eight subjects who used two types of speech-coding strategies with the Nucleus 22 channel cochlear implant. The subjects had an average experience of 40 months with the F0F1F2 coding strategy implemented in their previous wearable speech processor. Three subjects were good performers (showing significant open-set understanding without lip-reading) and five were moderate performers (not able to do speech-tracking by auditory means alone). All subjects were evaluated again after 1 month and 6 months of experience with the MULTIPEAK coding strategy of the miniature speech processor. The test materials included vowel and consonant identification, monosyllabic words, everyday sentences and numbers in noise. All eight subjects showed an improvement on more than three of five measures. The group of moderate performers showed a larger improvement in vowel (+16%) and consonant (+17%) identification scores than the group of good performers. For the open-set sentence test, the better patients were able to increase their score from 52% to 80% correct; two of the moderate performers did not improve. Six subjects achieved significantly higher scores at moderate signal-to-noise ratios (up to 10 dB S/N) in the (Freiburger) number test. Results of information transmission analysis are also discussed.     

Zur Indikationserweiterung des „cochlear-implant”Freiburger Ergebnisse bei Patienten mit Resthörigkeit

The usual indication for a cochlear implant (CI) is acquired deafness in patients for whom conventional hearing aids are of no benefit. The question is whether CI is superior to the best conventional hearing aids for patients with some residual hearing, but who achieve only minimal speech recognition (< 30% in the Freiburg monosyllable word test at 70 dB (I) SPL) with optimal hearing aids. We report our experience with five patients with residual hearing who underwent cochlear implantation (Nucleus Mini 22 and 24) on the worse side. The patients were examined preoperatively and at 1, 6 and 12 months following activation of the implant. The Freiburg monosyllabic word test, the G?ttingen sentence test and consonant recognition were used to assess postoperative results. All patients benefitted from CI when test scores were compared with preoperative ones. All patients achieved a score in the Freiburg monosyllabic word test of more than 60% at 70 dB (I) SPL 12 months post switch-on. Four patients achieved a score of more than 85% in the G?ttingen sentence test. These results and the progress made in cochlear implant technology are an impetus to continue discussions of various considerations of criteria for cochlear implants and possibly extend these for patients with severe hearing impairment.     

Comparison of speech perception performance between the nucleus 24 and nucleus 24 contour cochlear implant systems

Objective—To compare the 1-, 3-, and 6-month postoperative speech perception scores obtained by a group of subjects who received the new perimodiolar array (Nucleus Contour) cochlear implant with those obtained by a group of subjects implanted with the straight electrodes of the previous-generation Nucleus 24 device.

Material and Methods—The speech performance of 10 postlingually deafened adults implanted with the Nucleus Contour device was compared with that of matched controls who received the Nucleus 24 model. Objective measures included word and sentence speech recognition scores.

Results—Patients implanted with the Nucleus Contour device obtained significantly higher word and sentence recognition scores after short-term use of the implant compared to those obtained by patients implanted with the Nucleus 24 model.

Conclusion—Further long-term studies are required to determine whether the Nucleus Contour CI recipients continue to improve over time.     

Early results using the nucleus CI24M in children

OBJECTIVE: To report early postimplantation speech recognition results in children who received Nucleus CI24M cochlear implants. STUDY DESIGN: The study group consisted of 19 consecutively implanted children. PATIENTS AND SETTING: Congenitally deaf children (20 months to 15 years old) were implanted with the Nucleus CI24M and followed-up at New York University Medical Center for a period of 3 to 12 months. MAIN OUTCOME MEASURES: Speech perception was evaluated preoperatively and postoperatively using the Early Speech Perception (ESP) test, the Glendonald Auditory Screening Procedure (GASP) word and sentence tests, Phonetically Balanced Kindergarten (PBK) monosyllabic word lists, Common Phrases test, the Multisyllabic and Lexical Neighborhood (MLNT, LNT) tests, and the Banford-Kowal-Bench (BKB) sentence test. RESULTS: One-way analyses of variance revealed significant improvement in open-set speech recognition in children able to perform measurement tasks. CONCLUSIONS: The Nucleus CI24M cochlear implant provides significant benefit to children after short-term use.     

Speech perception of Mandarin Chinese speaking young children after cochlear implant use: effect of age at implantation

OBJECTIVE: The purposes of this study were to examine (1). whether the benefit of cochlear implants in Mandarin-speaking congenitally deaf children persists over a 2-year follow-up period, and (2). whether the age of the child at implantation affects the acquisition of speech perception by young deafened children. METHOD: Sixteen congenitally deaf children received implants of Nucleus CI24M devices. Speech perception tests were conducted using the Mandarin Auditory Perception Test Battery at 6-month intervals after connection of the device. We determined the benefit of cochlear implants by monitoring percent correct scores between 12 and 24 months after implantation. Regression analysis was used to correlate the measures of progress and the age at implantation. RESULTS: In the comparison of performance at 12 and 24 months post-connection, subjects performed significantly better at 24 than at 12 months in spondee, vowel, and consonant tests (P<0.05). In phrase and sentence tests, the mean score improved over time with device use, but the difference was not significant (P=0.066 and 0.067, respectively). However, no obvious improvement in average score was found in tone tests (P=0.386). Progress (improvement in speech perception) at 12 and 24 months had moderate negative correlations with age at implantation in spondee, vowel, phrase and sentence tests (P<0.05), but no correlation with age in consonant (P=0.20) and tone tests (P=0.26). CONCLUSION: The result, in accordance with the reports on Western language-speaking children, showed cochlear implants increasingly benefit Mandarin-speaking congenitally deaf children over a 2-year post-implantation period. Because age at implantation negatively correlates with improved speech perception after implantation, congenitally affected children with profound deafness (if they cannot obtain enough benefit from hearing aids) should receive implants as early as possible.     

Combining acoustic and electrical speech processing: Iowa/Nucleus hybrid implant

OBJECTIVES: In this paper we test the concept of combining electrical stimulation for high-frequency sound with acoustic hearing for low-frequency information in the same ear. In addition, we test whether residual hearing can be preserved when an electrode is placed up to 10 mm into the inner ear, and whether the site of electrical stimulation influences speech perception. MATERIAL AND METHODS: Nine post-lingual adults with severe high-frequency hearing impairment were recruited to participate in the study. A single-subject clinical trial design was employed. A unique six-channel cochlear implant was designed for this clinical trial. The intracochlear electrodes were either 6 or 10 mm in length based on a Nucleus CI-24 multichannel implant. Monosyllabic word understanding and consonant identification in a recorded sound-only condition were used to assess changes in speech perception. Follow-up was > 12 months. RESULTS: Acoustic hearing was preserved in all nine subjects. Preoperative monosyllabic word and sentence scores were unchanged in all subjects following implantation. A 30-40% improvement in consonant recognition occurred with the 10-mm electrode. The 10-mm electrode subjects were able to understand 83-90% of the monosyllabic words using the implant plus binaural hearing aids. Scores were more than doubled when compared to preoperative scores achieved with hearing aids only. CONCLUSIONS: The human ear has the capability to integrate both acoustic and high-frequency electrically processed speech information. Placement of a short 10-mm electrode does not appear to damage residual low-frequency inner ear hair cell function, interfere with the micro-mechanics of normal cochlear vibration or decrease residual speech perception. The improvement in speech recognition was due primarily to the increased perception of higher-frequency consonantal speech cues. Such a device can provide a substantial benefit in terms of speech understanding to those with severe high-frequency hearing loss, while still maintaining the benefits of the residual lower-frequency acoustic hearing. The position of the electrode and the site of frequency information within the cochlea are shown to be important factors in the success of such a device.     

Cochlear implantation in prelingually deafened children with residual hearing

OBJECTIVE: The purpose of this study was to examine the speech perception skills of prelingually deafened French children with preoperative residual hearing who received multichannel cochlear implants. DESIGN: The design of the study incorporated a within-subject, repeated measures design for assessing speech perception skills. SETTING: Montpellier, Toulouse and Lyon Pediatric Cochlear Implant Centers. SUBJECTS: Seven prelingually deafened children demonstrating marginal benefit from conventional amplification prior to implantation with a Nucleus multichannel cochlear implant, served as subjects for the speech perception assessment (a speech recognition score less than 30% defines marginal benefit from acoustic amplification on open set materials). The mean age at implantation was 7 years, 9 months. OUTCOME MEASURES: Speech perception skills were assessed using open set materials and the MUSS and MAIS questionnaires. RESULTS: Open-set speech recognition averaged 21.4% before implantation, and 83.6% after 1 year's cochlear implant experience. All children demonstrated an open-set score over 60% after 12 months of CI use. MAIS test scores averaged 18.1/40 before implantation and 35.1/40 after 9 months of CI use. MUSS test scores averaged 24.4/40 before implantation and 34.1/40 after 9 months of CI use. CONCLUSIONS: Cochlear implantation should be considered for prelingually hearing impaired children demonstrating marginal benefit from hearing aids, with a speech recognition score less than 30% on open set materials, in order to improve their speech discrimination skills.     

Speech perception with a cochlear implant used in conjunction with a hearing aid in the opposite ear

The aim of this study was to determine the improvement in speech recognition provided by a cochlear implant (CI) in conjunction with a hearing aid (HA) in the opposite ear. The study was a retrospective cohort study in the context of a university teaching hospital CI programme. Seven CI patients who still use their HA in the opposite ear were tested. The scores with the CI alone and the CI in conjunction with an HA were evaluated by using three speech perception tests in quiet (Freiburger Numbers, Freiburger Monosyllables, and Innsbrucker Sentence Test). In the majority of tests and subjects, the CI alone performed better than the HA alone, and the bimodal (CI+HA) condition was superior to the CI alone. On the sentence test, the patients as a group improved from 47-96% (mean: 79%; CI alone) to 50-100% (mean: 88.1%; CI+HA, pv<0.05). With the more difficult monosyllable test, the scores improved from 15-52% (mean: 37.2%; CI alone) to 15-82% (mean: 48.7%; CI+ HA, p<0.05). On the numbers test, scores increased from 65-98% (mean: 83%; CI alone) to 75-98% (mean: 88.7%; CI+ HA, p<0.05). All patients in this study were implanted in the poorer ear. The results of the present study suggest the advantage of CI usage in conjunction with an HA in the opposite ear.     

Speech Discrimination Scores of Postlingually Deaf Adults Implanted with the Combi 40 Cochlear Implant

The aim of the study was to assess the speech discrimination ability of postlingually deaf adults implanted with the Combi 40 cochlear implant and to compare the results with the postoperative data published for other devices. The postoperative open and closed set speech perception performance of 21 consecutive patients was tested using a standardized test battery comprising a number, monosyllable, sentence, consonant and vowel discrimination test as well as a rhyme test in the sound only condition. Mean values achieved for each test 1, 6 and 12 months after ?switch on? were evaluated. The results demonstrate that all patients have a substantial benefit from their implant and show a continuous improvement in their speech perception abilities with increased device experience. The mean percentages of correct answers after 12 months were 93.4 for numbers, 44.6 for monosyllables, 78.5 for sentences, 67.6 for the rhyme test, 59.8 for vowel, and 67.3 for consonant discrimination. Preoperatively, the mean discrimination score for monosyllables was 0%. The speech discrimination scores of our patients were similar or higher than described for similar patient groups implanted with other devices. The high stimulation rate of the implant system using the continuous interleaved speech processing strategy as well as a deep atraumatic electrode insertion into the apicalmost regions of the scala tympani may be the reason for good performance.     

Speech perception with a cochlear implant used in conjunction with a hearing aid in the opposite ear

The aim of this study was to determine the improvement in speech recognition provided by a cochlear implant (CI) in conjunction with a hearing aid (HA) in the opposite ear. The study was a retrospective cohort study in the context of a university teaching hospital CI programme. Seven CI patients who still use their HA in the opposite ear were tested. The scores with the CI alone and the CI in conjunction with an HA were evaluated by using three speech perception tests in quiet (Freiburger Numbers. Freiburger Monosyllables, and Innsbrucker Sentence Test). In the majority of tests and subjects. the CI alone performed better than the HA alone, and the bimodal (CI + HA) condition was superior to the CI alone. On the sentence test, the patients as a group improved from 47 96% (mean: 79%; CI alone) to 50-100% (mean: 88.1%; CI + HA, pv < 0.05). With the more difficult monosyllable test, the scores improved from 15-52% (mean: 37.2%; CI alone) to 15 82% (mean: 48.7%; CI + HA, p < 0.05). On the numbers test. scores increased from 65-98% (mean: 83%; CI alone) to 75-98% (mean: 88.7%; CI + HA, p < 0.05). All patients in this study were implanted in the poorer ear. The results of the present study suggest the advantage of CI usage in conjunction with an HA in the opposite ear.     

Mandarin speech perception in nucleus CI 24 implantees using MAPs based on neural response telemetry

The purpose of this study is to compare speech perception performance in Mandarin-speaking Nucleus CI24 implantee using standard behavior MAPs and NRT-based MAPs. Eight Nucleus CI24 users (5 years and older) participated in the study. They all fulfilled the following criteria: (1) behavioral MAP and NRT thresholds can be reliably obtained; (2) had more than 18 functioning electrodes; (3) had at least 6 months experience using CI. All subjects received speech evaluation under three different MAPs: a traditional behavioral MAP, a MAP predicted from the NRT thresholds of the E22 (electrode 22), E19, E15, E11, E8, E5, E1 and a combined MAP based on the information of NRT thresholds and behavioral threshold/comfortable levels of the E11. The speech evaluation package included word recognition test in quiet, in noise, and a Mandarin sentence test in quiet. Results showed that three MAPs are similar in some subjects, but different in other subjects. Compared to the NRT MAPs, the combined MAPs are more similar to the behavioral MAPs. There was no significant difference in the mean score of the word recognition test in quiet, in noise and sentence test under these three MAP conditions. In conclusion, although the behavioral MAPs and the NRT-based MAPs are not identical, the speech performance of Mandarin-speaking CI24 implantee using MAPs predicted from NRT thresholds appeared to be no worse than the traditional behavioral MAPs. Therefore, in certain cases that behavioral MAPs are difficult to obtain (such as in very young or multiple handicapped children), NRT-based MAPs may serve reliably as an initial estimation.     

Combining acoustic and electrical hearing

OBJECTIVES/HYPOTHESIS: The concept of combining electrical stimulation for high-frequency sound with acoustic hearing for low-frequency information was tested. In addition, whether residual hearing can be preserved when an electrode is placed into the inner ear up to 10 mm and whether place of electrical stimulation influences speech perception were tested. STUDY DESIGN: A single-subject clinical trial design was employed. METHODS: Six postlingual adults with severe high-frequency hearing impairment were recruited to participate in the study. A new six-channel cochlear implant was designed for the clinical trial. The intracochlear electrodes were either 6 or 10 mm in length based on a Nucleus CI-24 multichannel implant. Monosyllabic word understanding and consonant identification testing in a recorded sound-only condition were used to assess changes in speech perception. Follow-up was greater than 12 months. RESULTS: Acoustic hearing was preserved in all six subjects (n = 3, 6-mm electrodes; n = 3, 10-mm electrodes). Preoperative monosyllabic word and sentence scores were unchanged in all subjects following implantation. A 30% to 40% improvement in consonant recognition occurred with the 10-mm electrode. The subjects with 10-mm electrodes were able to understand 83% to 90% of the monosyllabic words using the implant plus binaural hearing aids. Scores were more than doubled when compared with preoperative scores with hearing aids only. CONCLUSION: The human ear has the capability to integrate both acoustic and high-frequency electrically processed speech information. Placement of a short, 10-mm electrode does not appear to damage residual low-frequency inner ear hair cell function, interfere with the micro mechanics of normal cochlear vibration, or decrease residual speech perception. The improvement in speech recognition was due primarily to the increased perception of higher-frequency consonantal speech cues, and this improvement took several months to become apparent. Such a device can provide a substantial benefit in speech understanding to individuals with severe high-frequency hearing loss, while still maintaining the benefits of the residual lower-frequency acoustic hearing. The position of the electrode and the place of frequency information within the cochlea were shown to be important factors in the success of such a device.     

Speech perception scores in cochlear implant recipients: An analysis of ceiling effects in the CUNY sentence test (Quiet) in post-lingually deafened cochlear implant recipients

Objectives: To evaluate the clinical utility of the City University of New York sentence test in a cohort of post-lingually deafened cochlear implants recipients over time.

Methods: 117 post-lingually deafened, Australian English-speaking CI recipients aged between 23 and 98 years (M?=?66 years; SD?=?15.09) were recruited. CUNY sentence test scores in quiet were collated and analysed at two cut-offs, 95% and 100%, as ceiling scores.

Results: CUNY sentence scores ranged from 4% to 100% (M?=?86.75; SD?=?20.65), with 38.8% of participants scoring 95% and 16.5% of participants reaching the 100% scores. The percentage of participants reaching the 95% and 100% ceiling scores increased over time (6 and 12 months post-implantation).

The distribution of all post-operative CUNY test scores skewed to the right with 82% of test scores reaching above 90%.

Discussion: This study demonstrates that the CUNY test cannot be used as a valid tool to measure the speech perception skills of post-lingually deafened CI recipients over time. This may be overcome by using adaptive test protocols or linguistically, cognitively or contextually demanding test materials.

Conclusion: The high percentage of CI recipients achieving ceiling scores for the CUNY sentence test in quiet at 3 months post-implantation, questions the validity of using CUNY in CI assessment test battery and limits its application for use in longitudinal studies evaluating CI outcomes. Further studies are required to examine different methods to overcome this problem.     

Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation.

OBJECTIVE: The present article investigates on an individual basis the performance achieved with the auditory brainstem implant in patients who had been treated unsuccessfully with a cochlear implant. STUDY DESIGN: An intrasubject comparison between results achieved with the cochlear implant and the auditory brainstem implant is reported. SETTING: Tertiary referral care. PATIENTS: Five subjects were fitted with an auditory brainstem implant in our department because of the poor results achieved with cochlear implants. Two were children, one with bilateral cochlear nerve aplasia and one suffering from auditory neuropathy. Three were adults with complete cochlear ossification. INTERVENTION: A retrosigmoid approach was used in all subjects. Electrically evoked auditory brainstem responses and neural response telemetry were used to monitor electrode positioning. RESULTS: No complications were observed due to implantation surgery or related to activation or long-term use of the auditory brainstem implant. Auditory sensations were induced in all patients with varying numbers of electrodes (from 9-16). In all three adults, the cochlear implant did not allow either word/sentence discrimination or speech tracking, whereas the auditory brainstem implant permitted discrimination of two- or three-syllable words with scores from 85 to 100%. In the two adults with a follow-up of 5 and 6 months after auditory brainstem implant activation, the open-set sentence recognition scores (auditory-only mode) were 70% and 100%, respectively, and the speech-tracking scores were 27 and 40 words/min, respectively. One patient with a follow-up of only 3 months scored 0% in both sentence recognition and speech tracking. The two children who had achieved no hearing ability with the cochlear implant were already able to detect sounds and words as early as 2 months after activation of the auditory brainstem implant and are showing progressive improvement in their performance. CONCLUSION: Auditory brainstem implantation may be a very powerful rehabilitative treatment after cochlear implant failure. The possibility of using the auditory brainstem implant as first-choice therapy in some categories of deaf patients (e.g., subjects with auditory neuropathy or cochlear ossification) who are currently treated with cochlear implantation is discussed.     

Cochlear implant in postlingual children: functional results 10 years after the surgery

The benefits of cochlear implants (CI) for communication skills are obtained over the years. There are but a few studies regarding the long-term outcomes in postlingual deaf children who grew up using the electronic device.AimTo assess the functional results in a group of postlingual children, 10 years after using a CI.MethodsTen postlingual deaf children, implanted before 18 years of age, participated in this study. We assessed: sentence recognition and speech intelligibility. We documented: device use and function and the patient's academic/occupational status. Study design: series.ResultsThe mean scores were 73% for sentence recognition in silence and 40% in noise. The average write-down intelligibility score was 92% and the average rating-scale intelligibility score was 4.15. There were no cases of device failure. Regarding educational/vocational status, three subjects graduated from the University. Five quit education after completing high school. Eight subjects had a professional activity.ConclusionThis study showed that cochlear implantation is a safe and reliable procedure. The postlingual profoundly hearing-impaired children after 10 years of CI use developed satisfactory levels regarding speech perception and intelligibility, and completed at least high school and were inserted in the labor market. Clinical Trials Registry: NCT01400178.     

Variables affecting speech perception in postlingually deaf adults following cochlear implantation

OBJECTIVE: To evaluate the time span over which there is greatest improvement in postlingually deaf adults undergoing cochlear implantation. Additionally, to quantify potential patient and device factors that may predict the postoperative results. MATERIAL AND METHODS: A longitudinal study was conducted. Numbers, monosyllables and sentence test results were collected for 66 cochlear implant subjects [Combi 40/40 +, n = 60; Clarion HF2, n = 2; Nucleus 24m/k, n = 4] at regular intervals for up to 6 years following cochlear implantation. RESULTS: All patients showed a steady improvement over time on all tests. Progress during the first 12 months was statistically significant, with further improvements being recorded after the 12-month testing period. The duration of deafness and the number of electrodes (8 for the Combi 40, 12 for the Combi 40 + ) appeared to be weakly correlated with postoperative performance. Re-implantation after device failure had no negative effect on speech reception. Subjects who were "upgraded" from an analogue to a digital cochlear implant improved their test results almost twofold. CONCLUSION: All the patients in our study gained substantial benefit from their cochlear implants. It is encouraging to note that the factors examined were not deemed to be relevant predictors of performance. Even long-term deaf subjects and re-implantees are able to achieve an excellent level of speech perception.     

Tinnitus before and 6 months after cochlear implantation

In this prospective multicenter study, tinnitus loudness and tinnitus-related distress were investigated in 174 cochlear implant (CI) candidates who underwent CI surgery at a Swiss cochlear implant center. All subjects participated in two session, one preoperatively and one 6 months after device activation. In both sessions, tinnitus loudness was assessed using a visual analogue scale and tinnitus distress using a standardized tinnitus questionnaire. The data were compared with unaided pre- and postoperative pure tone thresholds, and postoperative speech reception scores. 71.8% of the subjects reported tinnitus preoperatively. Six months after CI surgery 20.0% of these reported abolition of their tinnitus, 51.2% a subjective improvement, 21.6% no change and 7.2% a deterioration. Of the 49 (28.2%) subjects with no tinnitus preoperatively, 5 developed tinnitus 6 months after CI. These 5 had poorer speech understanding after CI surgery with their device than the group who remained tinnitus free. We found no correlation between tinnitus improvement, age, duration of tinnitus, or change in unaided hearing thresholds between the two sessions.     

Intra- and intersubject comparison of cochlear implant systems using the Esprit and the Tempo+ behind-the-ear speech processor

A patient with bilateral profound deafness was implanted with a Nucleus CI24M cochlear implant (CI) and used an Esprit behind-the-ear (BTE) speech processor. Thirteen months later, the implant had to be removed because of a cholesteatoma. As the same electrode could not be reinserted, a Medel combi40s CI was implanted in the same ear, and the patient used a Tempo+ BTE processor. After 1 year of use of the Combi40s/Tempo+ system, speech recognition was better and was rated better subjectively than with the CI24M/Esprit system. Speech recognition and subjective ratings were also assessed for two matched groups of nine CI users each, using either an Esprit or a Tempo+ processor. On average, speech recognition scores were higher for the group of Tempo+ users, but the difference was not statistically significant. Users of the Esprit processors rated their device higher in terms of cosmetic appearance and comfort of wearing.     

Favorable outcome of cochlear implant in VIIIth nerve deficiency.

OBJECTIVE: To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center, University hospital. PATIENTS: An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging. INTERVENTION: Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months. MAIN OUTCOME MEASURES: Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation. RESULTS: Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthur's questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The child's cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age. CONCLUSION: We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.