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: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

Objective:To investigate the application value of intraoperative sliding rail computed tomography （CT） in complicated and difficult cochlear implantation by analyzing the cases of complicated and difficult cochlear implantation. Methods:The clinical data of patients with complicated and difficult cochlear implantation assisted by sliding rail CT were retrospectively analyzed, the intraoperative complications and the number of electrode adjustments were summarized, and the patients were followed up. Results:A total of 51 subjects were included in this study, including 46 patients with inner ear malformation, 2 patients with cochlear ossification, there were 7 patients underwent secondary scanning to adjust the electrode and achieved satisfactory implantation position. Conclusion:Intraoperative CT scanning is a reliable adjunctive tool for determining the placement of complex cochlear implantation, and it improves the accuracy of difficult cochlear implantation surgeries.
Humans ; Cochlear Implantation/methods* ; Retrospective Studies ; Tomography, X-Ray Computed ; Cochlear Implants ; Male ; Female ; Child, Preschool ; Child ; Cochlea ; Electrodes, Implanted ; Infant

Objectives: This study aimed to evaluate the responsiveness of cochlear nerve to electrical stimulation in patients with cochlear nerve deficiency(CND), to compare their results with those measured in implanted children with normal-sized cochlear nerves, and to investigate the characteristics of the cochlear nerve injury of children with CND. Methods: Participants were children who underwent cochlear implantation at Shandong Provincial ENT Hospital from January 2012 to January 2020, including CND group and control group. The CND group included 51 subjects (male:20; female: 31) who were diagnosed with CND and had normal cochlea. For the CND group, four children had been bilaterally implanted, the mean implantation age was (2.7±1.5) years old. The control group included 21 subjects (male:10; femal:11) who had normal-sized cochlear nerve and normal cochlea. For the control group, all children had been unilaterally implanted except one, and the mean implantation age was (3.0±1.9)years old. Three subjects in the CND group used CI422 electrode arrays, and all the other subjects used CI24RECA/CI512 electrode arrays. The electrically evoked compound action potentials (ECAP) had been tried to record for each electrode using Custom Sound EP software (v. 4.3, Cochlear Ltd.) at least six months post first activation. Furthermore, ECAP amplitude growth functions (AGF) were measured at multiple electrode locations across the electrode array. Generalized linear mixed effect models with the subject group and electrode location as the fixed effects and subjects as the random effect were used to compare results of ECAP measurements. Results: In the control group, ECAP could been recorded at all electrodes (100%), but it could only be recorded in 71% (859/1 210) electrodes in the CND group. Additionally, the percentage of electrodes with measurable ECAP decreased from electrode 1 to electrode 22 in the CND group. Compared to the control group, the ECAP thresholds significantly increased, the ECAP amplitudes and AGF slopes significantly decreased, and the ECAP latency significantly increased in the CND group (P<0.01). GLMM showed that the stimulating site had a significant effect on the ECAP threshold, maximum amplitude, and AGF slope (P<0.01), but had no significant effect on the ECAP latency (P>0.05) in the CND group. However, the stimulating site had no significant effects on the ECAP measurements in the control group. Furthermore, the functional status of cochlear nerve varied greatly among CND group. From electrode 1 to electrode 22, the ECAP thresholds gradually increased, the ECAP maximum amplitudes and AGF slopes gradually decreased in the CND group. Conclusion: Compared with patients with normal-sized cochlear nerve, not only the number of residual spinal ganglion neurons reduce,but also the function of spinal ganglion neurons damages in CND patients. The degree of cochlea nerve deterioration varies greatly among CND patients. Generally, the deterioration of cochlear nerve tends to increase from the basal to the apical site of the cochlea.
Child, Preschool ; Female ; Humans ; Infant ; Male ; Cochlea ; Cochlear Implantation/methods* ; Cochlear Implants ; Cochlear Nerve ; Electric Stimulation ; Evoked Potentials, Auditory/physiology*

Objective:To report the experience of using CT-guided cochlear implant surgery in difficult cases such as severe inner ear deformities and anatomical abnormalities, and to discuss the application value of intraoperative CT-assisted localization in difficult cases of cochlear implant surgery. Methods:Retrospectively analyzed the clinical data of 23 cases of difficult cochlear implant surgery cases completed by our team with the assistance of intraoperative CT, and collected their medical data, including preoperative imaging manifestations, surgical conditions, and intraoperative imaging images for evaluation. Results:During the study period, 23 difficult cases（27 ears） underwent cochlear implantation under the guidance of intraoperative CT, and 4 cases were bilaterally implanted. Including 6 cases of incomplete segmentation type Ⅰ（IP-Ⅰ）, 1 case of incomplete segmentation type Ⅱ（IP-Ⅱ）, 10 cases of incomplete segmentation type Ⅲ（IP-Ⅲ）, 3 cases of common cavity deformity（CC） and 3 cases of cochlear ossification after meningitis. Facial nerve anatomy was abnormal in 9 cases, cerebrospinal fluid "blowout" was serious in 14 cases, electrode position was abnormal in 3 cases requiring intraoperative adjustment of electrode position, anatomical difficulties required intraoperative CT to assist in finding anatomical landmarks in 2 cases, and electrodes were not fully implanted in 3 cases. Conclusion:When faced with difficult cases with challenging and complex temporal bone anatomy, intraoperative CT can accurately evaluate the electrode position and provide intraoperative anatomical details, allowing immediate adjustment of the electrode position if necessary, providing safety guarantee for difficult cases of cochlear implant surgery and ensure accurate implantation of electrodes.
Humans ; Cochlear Implantation/methods* ; Retrospective Studies ; Tomography, X-Ray Computed/methods* ; Cochlea ; Cochlear Implants

Objective:The auditory and speech rehabilitation effects were assessed by the Categories of Auditory Performance（CAP） and the speech intelligibility rating scale（SIR） after cochlear implantation（CI） in prelingually elderly patients by telephone follow-up or face-to-face conversation. Methods:The clinical data of the prelingually deaf patients who underwent unilateral CI in the Department of Otorhinolaryngology and Head and Neck Surgery, Shanxi People's Hospital, from December 2016 to December 2021 were collected. Thirty-eight patients were divided into Group A（SIR 1, 17 cases）, Group B（SIR 2, 10 cases） and Group C（SIR 3, 11 cases） according to the preoperative SIR Score. Nineteen patients with post-lingual hearing impairment were selected as the control group（Group D, 19 cases）. The effects of hearing and speech rehabilitation were evaluated using CAP and SIR Scores before surgery, 6 months after startup, and 1 year after startup. Results:There were no significant differences in CAP scores among the three groups of patients with prelingually deaf patients at 6 months and 1 year after startup（P>0.05）, but there were significant differences between group A and group D at 6 months and 1 year after startup（P<0.05）; the SIR Score of group A had statistical difference before surgery and 6 months after startup（P<0.05）, group B had statistical difference before surgery and 1 year after startup（P<0.05）, and group C and D had no statistical difference before surgery and 6 months and 1 year after startup, respectively（P>0.05）. Conclusion:For the prelingually deaf elderly patients, hearing will develop rapidly 6 months after startup, and the effect of postoperative auditory rehabilitation was positively correlated with the preoperative speech ability. In the aspect of speech, the prelingually dear elderly patients who have poor preoperative speech ability could benefit more from CI early after surgery. CI is not contraindicated in prelingually deaf elderly patients, even those with poor preoperative speech function.
Humans ; Aged ; Cochlear Implantation/methods* ; Cochlear Implants ; Speech Perception ; Deafness/rehabilitation* ; Hearing Tests ; Speech Intelligibility ; Treatment Outcome

Objective: To investigate whether pre-lingual deafness adult caused by inadequate auditory compensation in childhood can benefit from cochlear implants and the related influencing factors. Methods: A total of 26 prelingual deafness as experimental group [11 males and 15 females, the age of operation was (24.5±5.7) years] and 13 postlingual deafness as control group [5 males and 8 females, the age at the time of operation was (42.2±11.4) years] were recruited. Objective assessment included hearing threshold and speech recognition rate tests while wearing cochlear implants. Subjective assessment used Nijmegen Cochlear Implant Questionnaire to assess hearing-related quality of life of subjects. The changes of hearing ability in the prelingual deafness group before and after operation and the differences with the postlingual deafness group were compared, and the correlation between speech recognition ability and the age diagnosed as severe or profound deafness, the age of hearing aid invalid, and duration of wearing cochlear implant were analyzed as factor indicators. All statistical results were analyzed by SAS 9.4 software. Results: In terms of objective indicators, the speech recognition rate of pre-lingual deafness was significantly lower than that of post-lingual deafness [(35.4±28.0)% vs (80.9±8.0)%,t=7.67, P<0.001], while there was no statistical difference in hearing threshold between the two groups [(34.8±4.0) dB HL vs (33.1±3.7) dB HL, t=1.30, P>0.05]. The indicators in the subjective questionnaire showed that the prelingual deafness group was only weaker in advanced sound perception, confidence and total mean score than the post-lingual deafness group (P<0.05), and there was no significant difference in other aspects(P>0.05), meanwhile, all indicators of the prelingual deafness group were significantly improved compared with the preoperative level (P<0.001). There was a moderate positive correlation between the hearing quality and the speech recognition rate in the prelingual deafness group(r=0.51, P=0.008). The regression analysis showed that the invalid age of hearing aid was the exact influencing factor of speech recognition rate. Conclusions: Certain prelingual deaf adults can adapt to cochlear implants and obtain different degrees of auditory assistance. Compared with the improvement of objective auditory ability assessment, the patient who received cochlear implantation gain more improvement in auditory related quality of life subjectively. The ineffective age of preoperative hearing aid is an important factor, which needs to be aroused sufficient preoperative attention.
Adolescent ; Adult ; Cochlear Implantation/methods* ; Cochlear Implants ; Deafness/rehabilitation* ; Female ; Humans ; Male ; Quality of Life ; Speech Perception ; Young Adult

BACKGROUND AND OBJECTIVES: Hearing loss is a common complication associated with Noonan syndrome (NS), and the level of hearing loss for NS patients with sensorineural loss ranged from normal to severe. Additional insights into the outcome of cochlear implantation (CI)in children with NS with or without comorbidities are needed. SUBJECTS AND METHOD: In this study, five patients with NS, four with a mutation in PTPN11 and one who tested negative in mutation screening, diagnosed with the clinical scoring systemand underwent CI at ages ranging from 16 to 50 months were retrospectively reviewed. Patientswere evaluated for auditory perception (Categories of Auditory Performance), speech production (Korean Version of the Ling's Stage), and language ability (Receptive and Expressive Vocabulary Test). RESULTS: In five of the children with NS, CI was performed without any complications. Threepatients who received CI before 30 months of age showed outstanding outcomes. One patientwho received CI at 50 months showed limited benefit. One patient who was diagnosed with developmental delay and cochlear nerve hypoplasia underwent CI at 28 months with poor outcome. DISCUSSION: Our report suggests that although the benefit may be influenced by comorbidities associated with NS or delay in hearing rehabilitation, profound hearing loss in patientswith NS may be restored to normal levels in terms of auditory/speech perception and vocabulary/language development. The variable hearing outcomes also underscore the need for earlyscreening and detection of profound hearing loss, and regular follow-up for hearing evaluationin patients with NS.
Auditory Perception ; Child ; Cochlear Implantation ; Cochlear Implants ; Cochlear Nerve ; Comorbidity ; Follow-Up Studies ; Hearing ; Hearing Loss ; Humans ; Language ; Mass Screening ; Methods ; Noonan Syndrome ; Rehabilitation ; Retrospective Studies ; Vocabulary