Hearing loss is the most prevalent disabling disease. Cochlear implantation（CI） serves as the primary intervention for severe to profound hearing loss. This consensus systematically explores the value of genetic diagnosis in the pre-operative assessment and efficacy prognosis for CI. Drawing upon domestic and international research and clinical experience, it proposes an evidence-based medicine three-tiered prognostic classification system（Favorable, Marginal, Poor）. The consensus focuses on common hereditary non-syndromic hearing loss（such as that caused by mutations in genes like GJB2, SLC26A4, OTOF, LOXHD1） and syndromic hereditary hearing loss（such as Jervell & Lange-Nielsen syndrome and Waardenburg syndrome）, which are closely associated with congenital hearing loss, analyzing the impact of their pathological mechanisms on CI outcomes. The consensus provides recommendations based on multiple round of expert discussion and voting. It emphasizes that genetic diagnosis can optimize patient selection, predict prognosis, guide post-operative rehabilitation, offer stratified management strategies for patients with different genotypes, and advance the application of precision medicine in the field of CI.
Humans ; Cochlear Implantation ; Prognosis ; Hearing Loss/surgery* ; Consensus ; Connexin 26 ; Mutation ; Sulfate Transporters ; Connexins/genetics*

Objective:To explore the application value of P1 response threshold of cortical auditory evoked potential（CAEP） in evaluating the rehabilitation effect of cochlear implant in young children. Methods:Thirty-three young children after cochlear implantation were divided into groups according to hearing age: Group A（hearing age 1-<2 years old） 10 people; Group B（hearing age 2-<3 years old） 13 people; Group C（hearing age 3-<4 years old） 10 people. The subjective assessment was carried out using the assessment tool for hearing-impaired children- "Criteria and Methods for assessing Auditory and language ability of hearing-impaired children" and objective electrophysiological examination was carried out using CAEP to evaluate the rehabilitation effect. SPSS 25.0 software was used for statistical analysis. Results:The results of subjective assessment of auditory ability and language ability in each group showed an increasing trend with the increase of auditory age. In this study, the P1 response threshold of CAEP in CI implanted children had a significant positive correlation with the 2 kHz hearing threshold after intervention, and the P1 response threshold of CAEP was negatively correlated with many items in subjective auditory ability and language ability assessment. Conclusion:The P1 response threshold of CAEP has a stable correlation with the results of speech audiometry, which can effectively and objectively evaluate the postoperative rehabilitation effect of young children with cochlear implantation.
Humans ; Child, Preschool ; Infant ; Male ; Female ; Evoked Potentials, Auditory ; Cochlear Implantation/rehabilitation* ; Cochlear Implants ; Auditory Threshold

BACKGROUND

The National Cochlear Implant Program (NCIP) is a national program to address the increasing prevalence of hearing loss, especially in the pediatric population here in the Philippines. In its pilot implementation, it included three tertiary hospitals to represent Luzon, Visayas, and Mindanao and was able to enroll 20 patients who successfully underwent cochlear implantation.

The aim of this study is to evaluate the auditory outcomes of the patients who underwent cochlear implantation under the NCIP using the Parents' Evaluation of Aural/Oral Performance of Children (PEACH) Questionnaire and Categories of Auditory Performance (CAP) score questionnaire.

The study included all recipients of the cochlear implants, under NCIP from December 2019 to December 2021, except 1 with incomplete data during the course of his followup. The outcomes measured included the PEACH Questionnaire score and CAP Questionnaire score and were compared on various factors which included patient's sex and age, parents'  socioeconomic status, duration of hearing aid use prior to CI, pre CI imaging findings and CI electrode placement using nonparametric statistical tests .

The mean PEACH score of the 19 patients was 53.59% ± 12.76% (range: 30% - 75%) while the mean CAP score was 3.16 ± 1.04 (range: 1 - 4.3). Parents of the included patients who have a higher educational background and those in which the electrode was located on the ideal location, scala tympani, have a statistically significant higher PEACH score (p-value of 0.017 and 0.012, respectively). In comparing the CAP scores, those who have unremarkable or normal preoperative imaging have a statistically significant higher score (p-value 0.013)

Patients who had normal preoperative imaging, proper placement of electrodes and those patients with parents belonging to a higher educational background had statistically significant better auditory outcomes after cochlear implantation. Patients who had the cochlear implantation before 36 months of age and hearing aid use of 7 to 18 months prior to cochlear implantation had higher PEACH and CAP scores, however these were not statistically significant. Further studies with a larger sample size is recommended.

Cochlear nerve deficiency（CND） is a rare inner ear malformation characterized by a hypoplastic or absent cochlear nerve, resulting in variable hearing loss or total deafness, depending on the quantity of nerve fibers present. About 18% of congenital hearing loss are associated with CND. It is a disease of uncertain cause. The outcome of auditory implant in CND patients varies widely. This article will discuss the related issues of CND.
Humans ; Cochlear Nerve/abnormalities* ; Cochlear Implants ; Child ; Cochlear Implantation/methods* ; Deafness ; Hearing Loss

Objective:This study aimed to compare the effects of cochlear implantation（CI） on vestibular function in patients with large vestibular aqueduct syndrome（LVAS） and in patients with extremely severe deafness with normal inner ear structure. Methods:A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials（cVEMP）, bone conduction evoked ocular vestibular-evoked myogenic potentials（oVEMP） and video head impulse tests（v-HIT） were compared between the two groups before and after CI. The data were analyzed using SPSS 26.0 software. Results:There was no significant difference in the results of the preoperative caloric test, v-HIT, and oVEMP（P1, N1） between the LVAS group and the control group（P>0.05）. Compared to the control group, the LVAS group exhibited a shorter cVEMP P1[（13.41±0.71）ms vs （16.28±0.89）ms, P<0.000 1], shorter N1[（19.83±0.54）ms vs （28.18±1.56）ms, P<0.000 1], higher amplitude[（123.60±83.80）μV vs （73.92±79.85）μV, P=0.049 4] and higher oVEMP amplitude[（16.60±13.87）μV vs （9.96±10.47）μV, P=0.028 5] before CI. The abnormal rate of caloric test increased in both groups after CI（25.00% vs 57.14%, P=0.028 8, 32.14% vs 82.14%, P=0.000 3, respectively）. There was no significant difference in the v-HIT parameters in both groups before and after the operation. As for the LVAS group, there was no statistically significant difference in cVEMP and oVEMP induction rates before and after operation. In the control group, there was a decrease in cVEMP induction rate（96.42% vs 64.28%, P=0.005 2） and oVEMP induction rate（96.42% vs 57.14%, P=0.000 9） after CI. LVAS group showed a shorter cVEMP P1[（13.41±0.71）ms vs （10.30±0.60）ms, P<0.000 1]; shorter cVEMP N1[（19.86±0.53）ms vs （18.97±1.33）ms, P=0.004 7]; decreased amplitude[（124.50±84.86）μV vs （64.35±61.57）μV, P=0.001 0] and shorter oVEMP amplitude[（15.92±13.03）μV vs （9.16±9.20）μV, P=0.009 9] after CI. The oVEMP N1 in the control group was longer than that before operation[（11.73 ± 0.91）ms vs （13.35 ± 2.60）ms, P=0.019 6], whereas there was no significant difference in other VEMP parameters after CI. Conclusion:Before CI, there was no significant difference in the results of the caloric test and v-HIT between the LVAS group and the control group, but the LVAS group exhibited increased sensitivity to acoustic stimulation-induced myogenic potentials. After CI, the function of the semicircular canal was impaired in both groups in the low-frequency area, and remained largely unaffected in the high-frequency area. Additionally, the function of the otolith in the LVAS group was less affected than that in the control group after CI, which may be related to the fact that the enlarged vestibular aqueduct of the LVAS patients acted as the third window of the inner ear.
Humans ; Vestibular Aqueduct/physiopathology* ; Cochlear Implantation ; Male ; Female ; Vestibular Evoked Myogenic Potentials ; Deafness/physiopathology* ; Child ; Adolescent ; Adult ; Young Adult ; Hearing Loss, Sensorineural/physiopathology* ; Vestibular Function Tests

Objective:This study aims to investigate the perception of Mandarin aspirated and unaspirated consonants by children with cochlear implants （CIs） under quiet and noisy conditions. It also examines factors that may affect their acquisition, such as auditory conditions, place of articulation, manner of articulation, chronological age, age at implantation, and non-verbal intelligence. Methods:Twenty-eight CI children aged 3 to 5 years who received implantation from 2018 to 2023 were recruited. Additionally, 88 peers with normal hearing （NH） were recruited as controls. Both groups participated in a perception test for aspirated/unaspirated consonants under quiet and noisy conditions, along with tests for speech recognition, speech production, and non-verbal intelligence. The study analyzed the effects of group （CI vs. NH）, auditory condition, and consonant characteristics on children's perception of aspirated/unaspirated consonants in Mandarin, as well as the factors contributing to CI children's acquisition of these consonants. Results:①CI children's ability to perceive aspirated/unaspirated consonants was significantly poorer than that of their NH peers （χ²= 14.16, P<0.01）, and their perception accuracy was influenced by the acoustic features of consonants （P<0.01）; ②CI children's consonant perception abilities were adversely affected by noise （P<0.01）, with accuracy in noisy conditions particularly influenced by the manner of articulation （P<0.05）; ③The age at implantation significantly affected CI children's ability to perceive aspirated/unaspirated consonants （β= -0.223, P=0.012）, with earlier implantation associated with better performance. Conclusion:It takes time for CI children to acquire Mandarin aspirated/unaspirated consonants, and early implantation shows many advantages, especially for the perception ability of fine speech features.
Humans ; Cochlear Implants ; Child, Preschool ; Speech Perception ; Cochlear Implantation ; Male ; Female ; Language

Objective:Over the past decades, minimally invasive cochlear implantation（CI） have achieved significant advancements, evolving from initial emphasis on incision miniaturization to comprehensive strategies for preserving intracochlear structures and functions, as well as optimizing overall minimally invasive surgical procedure. However, current academic debates persist regarding standardized definitions and consensus on technical protocols. Future research should prioritize innovations in surgical-assistive robots, refinement of hidden cochlear implant, and exploration of drug deliver electrodes. These efforts aim to advance surgical methodologies toward enhanced minimally invasive approaches, functional preservation, and personalized therapeutic interventions.
Humans ; Cochlear Implantation/trends* ; Minimally Invasive Surgical Procedures/trends* ; Cochlear Implants

Objective:To evaluate the perioperative safety and long-term complications of simultaneous bilateral cochlear implantation（BCI） in young infants, providing reference data for clinical BCI in young children. Methods:Seventy-four infants aged 6-23 months with congenital severe to profound sensorineural hearing loss who were candidates for cochlear implantation at the Department of Otolaryngology, Chinese PLA General Hospital between August 2018 and August 2019 were consecutively enrolled. Parents made the decision to implant either unilaterally or bilaterally. Participants were divided into unilateral cochlear implantation（UCI） group（before and after 12 months of age） and simultaneous BCI group（before and after 12 months of age）. Safety indicators, including perioperative risk variables, complications, and other postoperative adverse events were monitored, with complications followed up for 5-6 years. Comparisons were made between the BCI and UCI, as well as between implantation before and after 12 months of age regarding perioperative safety and long-term complications. Results:A total of 40 BCI patients（23 before 12 months, 17 after 12 months） and 34 UCI patients（20 before 12 months, 14 after 12 months） were included in the study. Regarding perioperative risk variables, the BCI group showed significantly longer anesthesia duration, operative time, and greater blood loss compared to the UCI group, though less than twice that of the UCI group; no anesthetic complications occurred in either group; and there was no significant difference in postoperative hospital stay between the groups. Regarding surgical complications during the 5-year follow-up period, the BCI group experienced 7 complications（2 major, 5 minor）, while the UCI group had 7 complications（1 major, 6 minor）, with no statistical differences between groups. Regarding other postoperative adverse events, the BCI group demonstrated significantly higher total adverse event rates than the UCI group（80.0% vs 38.2%）, with higher rates of moderate to severe anemia（60.0% vs 20.6%） and lower mean hemoglobin levels[（92.35±12.14） g/L vs（102.39±13.09） g/L]. No significant differences were found in postoperative fever rates（50.0% vs 52.9%） or C-reactive protein levels between groups. Within the BCI group, patients implanted before 12 months indicated notably higher rates of total adverse events（91.3% vs 64.7%）, high fever（26.1% vs 0）, and moderate to severe anemia（78.3% vs 35.3%） compared to those implanted after 12 months. Conclusion:Simultaneous BCI in young children under 2 years of age demonstrates controllable overall risks. Compared to UCI, while it shows no increase in anesthetic or surgical complications, it presents higher perioperative risks and adverse event rates, especially in patients implanted before 12 months of age, warranting special attention from medical staff.
Humans ; Cochlear Implantation/methods* ; Infant ; Postoperative Complications ; Hearing Loss, Sensorineural/surgery* ; Follow-Up Studies ; Male ; Perioperative Period ; Female ; Cochlear Implants

Objective:The aim of this study was to present an institution's experience with cochlear reimplantation（CRI）, to assess surgical challenges and post-operative outcomes and to increase the success rate of CRI. Methods:We retrospectively evaluated data from 76 reimplantation cases treated in a tertiary center between 2001 and 2022. Clinical features include caused of CRI, type of failure, surgical issues, and auditory speech performance were analyzed. Categorical Auditory Performance （CAP） and Speech Intelligibility Rating （SIR） scores were used to evaluate pre-and post-CRI outcomes. Our center's consecutive cohort of 1 126 patients had seven patients, while 69 patients were from other cochlear implant centers. Device failure was the most common cause of CRI（68/76）, with the remaining cases including flap complications（3/76）, magnet displacement（3/76）, secondary meningitis（1/76）, and foreign bodies around the implant（1/76）. Postoperative auditory and speech outcome improved in 31.6%（24/76） of patients, remained unchanged in 63.2%（48/76）, and decreased in CAP and SIR scores in 5.2%（4/76） of patients. Postoperatively, the seven patients with cochlear ossification and fibrosis scored lower on the overall CAP and SIR scale than non-ossification individuals, which is a significant factor in surgical success rates and auditory-speech outcomes. Conclusion:CRI surgery is a challenging but relatively safe procedure, and most reimplanted patients experience favorable postoperative outcomes. Medical complications and intracochlear damage are the main causes of poor postoperative results. Therefore, minimally invasive CI has a positive significance for reducing the difficulty of CRI surgery and improving the CI performance.
Humans ; Cochlear Implantation/methods* ; Retrospective Studies ; Cochlear Implants ; Male ; Female ; Postoperative Period ; Treatment Outcome ; Adult ; Speech ; Middle Aged ; Postoperative Complications ; Replantation ; Cochlea/surgery*

Objective:To investigate the application value of intraoperative electrocochleography（ECochG） monitoring technique and insertion techniques in cochlear implant（CI） and analyze its relationship with postoperative residual hearing（RH） preservation. Methods:Thirty-one patients（35 ears） who received CI in our hospital from June 2022 to July 2024 were enrolled. The Advanced Bionics Active Insertion Monitoring（AIM） system was used for real-time ECochG monitoring during surgery. Intraoperative cochlear microphonics （CM） waveform changes were recorded and analyzed in relation to postoperative RH preservation. Results:①ECochG recordings were successfully obtained in 34 of 35 ears （97.1%）. ②According to Harris classification, there were 7 ears（20.6%） of Type A（rising）, 7 ears（20.6%） of Type C（declining）, 8 ears（23.5%） of Type CC（fluctuating）, and 12 ears（35.3%） of Type D（no response）. ③The total CM amplitude decrease was significantly moderately correlated with postoperative low-mid frequency hearing loss（r=0.67, P=0.017）. The total CM amplitude decrease was significantly moderately correlated with postoperative low frequency hearing loss（r=0.65, P=0.023）. ④For the mean amplitude variation, the Amax was 30.70 μV, the Amin was 8.64 μV, and the Aend was 18.27 μV. ⑤Sixteen cases completed postoperative follow-up, with an average low-mid frequency（125-1 000 Hz） residual hearing loss of 15.25 dB HL and a RH preservation rate of 87.5%. Conclusion:Intraoperative ECochG monitoring can effectively predict postoperative residual hearing changes, effectively guide surgical manipulation, and improve residual hearing preservation rate.
Humans ; Cochlear Implantation/methods* ; Audiometry, Evoked Response ; Cochlear Implants ; Male ; Female ; Adult ; Middle Aged ; Monitoring, Intraoperative ; Adolescent ; Young Adult ; Minimally Invasive Surgical Procedures ; Child ; Aged ; Postoperative Period