The Outcome of Cochlear Implantations in Deaf-Blind Patients: A Multicenter Observational Study.

OBJECTIVE: This multicenter study aimed to evaluate the auditory and speech outcomes of cochlear implantation (CI) in deaf-blind patients compared with deaf-only patients. STUDY DESIGN: Retrospective cohort study. SETTING: Multiple cochlear implant centers. PATIENTS: The current study was conducted on 17 prelingual deaf-blind children and 12 postlingual deaf-blind adults who underwent CI surgery. As a control group, 17 prelingual deaf children and 12 postlingual deaf adults were selected. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory and linguistic performances in children were assessed using the categories of auditory performance (CAP) and Speech Intelligibility Rating (SIR) scales, respectively. The word recognition score (WRS) was also used to measure speech perception ability in adults. The mean CAP, SIR, and WRS cores were compared between the deaf-only and deaf-blind groups before CI surgery and at "12 months" and "24 months" after device activation. Cohen's d was used for effect size estimation. RESULTS: We found no significant differences in the mean CAP and SIR scores between the deaf-blind and deaf-only children before the CI surgery. For both groups, SIR and CAP scores improved with increasing time after the device activation. The mean CAP scores in the deaf-only children were either equivalent or slightly higher than those of the deaf-blind children at "12 months post-CI" (3.94 ± 0.74 vs 3.24 ± 1.25; mean difference score, 0.706) and "24 months post-CI" (6.01 ± 0.79 vs 5.47 ± 1.06; mean difference score, 0.529) time intervals, but these differences were not statistically significant. The SIR scores in deaf-only implanted children were, on average, 0.870 scores greater than the deaf-blind children at "12 months post-CI" (2.94 ± 0.55 vs 2.07 ± 1.4; p = 0.01, d = 0.97) and, on average, 1.067 scores greater than deaf-blind children at "24 months post-CI" (4.35 ± 0.49 vs 3.29 ± 1.20; p = 0.002; d = 1.15) time intervals. We also found an improvement in WRS scores from the "preimplantation" to the "12-month post-CI" and "24-month post-CI" time intervals in both groups. Pairwise comparisons indicated that the mean WRS in the deaf-only adults was, on average, 10.61% better than deaf-blind implanted adults at "12 months post-CI" (62.33 ± 9.09% vs 51.71 ± 10.73%, p = 0.034, d = 1.06) and, on average, 15.81% better than deaf-blind adults at "24-months post-CI" (72.67 ± 8.66% vs 56.8 ± 9.78%, p = 0.002, d = 1.61) follow-ups. CONCLUSION: Cochlear implantation is a beneficial method for the rehabilitation of deaf-blind patients. Both deaf-blind and deaf-only implanted children revealed similar auditory performances. However, speech perception ability in deaf-blind patients was slightly lower than the deaf-only patients in both children and adults.

Hyperbilirobinemia and Its Effect on Auditory Perception and Speech Intelligibility in Cochlear Implanted Children (Cochlear Implantation Outcomes in Children Who Have Experienced Hyperbilirobinemia).

The goal of this study was to investigate the probable difference in auditory perception and speech intelligibility performance amongst cochlear implanted children who experienced hyperbilirubinemia or auditory neuropathy in comparison to the cochlear implanted children with unknown etiology for hearing loss. This case-control study was carried out on 106 cochlear implanted children with mean age of 32.36 ± 11.98 months who were purposively selected and allocated into four groups. Out of the total, 30 had no specific etiology for hearing loss, while the others had experienced auditory neuropathy or hyperbilirubinemia with/without blood exchange. The auditory perception and speech intelligibility performance of all the participants who had received auditory verbal therapy were assessed after 6 and 12 months of rehabilitation. Then, the data was analyzed, using the Statistical Package for Social Sciences-version 21(SPSS-21). Results indicated poor auditory perception and speech intelligibility performance of the cochlear implanted children with hyperbilirubinemia and blood exchange (P ≤ 0.05), while the participants in the control group with no specific etiology for hearing loss, the children with hyperbilirubinemia with no blood exchange, and those who suffered from auditory neuropathy performed better, respectively. Also, a significant correlation between auditory neuropathy and hyperbilirubinemia was observed. Despite lower improvement of auditory perception and speech intelligibility of the hearing impaired children who were experiencing moderate to severe degrees of hyperbilirubinemia or auditory neuropathy, cochlear implantation is highly recommended not only for children with unknown etiology for severe hearing loss but also for this group of hearing impaired children.

Influence of Cochlear Implantation on Balance Function in Pediatrics.

BACKGROUND AND OBJECTIVE: Patients with profound Sensorineural Hearing Loss (SNHL) are susceptible to vestibular disturbances following Cochlear Implant (CI) surgery. This study aimed to evaluate vestibular dysfunctions following unilateral CI in the congenitally deaf children. METHODS: This was a cross-sectional study conducted on 24 children (mean age: 10.56 ± 5.49 years old) who underwent unilateral CI and 24 age-matched controls (mean age: 11.13 ± 6.21 years old). Vestibular functions were assessed by Vestibular Evoked Myogenic Potential (VEMP) and Computerized Dynamic Posturography (CDP). The VEMP test was performed for otolith's function (especially saccule) evaluation. Sensory Organization Test (SOT) protocol of CDP was also utilized to differentiate the role of various sensory systems contributing to postural stability. In addition, total equilibrium score was calculated. The variables were comparatively assessed between the two groups. RESULTS: The mean p13-n23 amplitude in the CI users was significantly lower than the controls (p<0.05). However, the two groups showed no significant difference in cVEMP latency values (p>0.05). The SOT analysis revealed that 45.83% (11/24) of the CI subjects had some kind of sensory abnormalities: 7 cases (29.17%) vestibular, 2 cases (8.33%) visual, 2 cases (8.33%) vestibular and somatosensory involvements. Furthermore, total equilibrium score was significantly reduced in implanted group than the controls (p<0.001). At least, 70.59% (12/24) CI patients showed abnormal values in the CDP or cVEMP examinations. CONCLUSION: This study shows functional vestibular impairments in children who underwent CI. These patients showed significantly increased postural instability which was more evident in dynamic conditions. These findings provide the basis for better pre-operative counseling and postoperative vestibular rehabilitation to CI recipients.

Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes.

OBJECTIVES: This multicenter study evaluated the auditory performance and speech production outcomes of cochlear implantation in children with inner ear anomaly and compared the outcomes of patients with different kinds of malformation. METHODS: Cochlear implantation was performed in 107 children with inner ear malformation at four tertiary academic centers. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated preoperatively and one year and two years postoperatively. RESULTS: Types of inner ear malformation and their frequencies were: incomplete partition type-I, 19 (17.8%) patients; incomplete partition type-II, 31 (29%), common cavity, 17 (15.9%), cochlear hypoplasia, 17 (15.9%), and isolated enlarged vestibular aqueduct (isolated EVA), 23 (21.5%) patients. EVA was the coexisting anomaly in 27(25.2%) subjects. The median CAP and SIR scores improved significantly during the first two years after cochlear implantation in all groups (p-values <0.001 and < 0.001, respectively). No significant difference was seen in CAP and SIR scores of children with different inner ear malformations (p-value = 0.147 and 0.570, respectively) or in patients with isolated EVA compared to coexisting EVA (p-value = 0.538 and 0.075, respectively). CONCLUSION: The median CAP and SIR scores two years after surgery were 5 (Understanding of common phrases without lip-reading) IQR: 4-6, and 3 (Connected speech is intelligible to a listener who concentrates and lip-reads within a known context) IQR: 3-4, respectively. Auditory performance and speech production were significantly improved in all inner ear malformation patient groups, and no significant difference was observed between the scores of patients with different types of anomaly.

Cochlear implantation in children with auditory neuropathy spectrum disorder: A multicenter study on auditory performance and speech production outcomes.

OBJECTIVES: To evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated. METHODS: Cochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children ≤24 months, and Group II: subjects >24 months. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated after the first and second years of implantation. The differences between the CAP and SIR scores in the two groups were assessed. RESULTS: The median CAP scores improved significantly after the cochlear implantation in all the patients (p value < 0.001). The improvement in the CAP scores during the first year in Group II was greater than Group I (p value: 0.007), but the improvement in CAP scores tended to be significantly higher in patients who were implanted at ≤24 months (p value < 0.001). There was no significant difference between two groups in SIR scores at first-year and second-year follow-ups. The evaluation of the SIR improvement revealed significantly higher values for Group I during the second-year follow-up (p value: 0.003). CONCLUSION: The auditory performance and speech production skills of the children with ANSD improved significantly after cochlear implantation, and this improvement was affected by age at the time of implantation.