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

To analyze the functional change of horizontal semicircular canals after cochlear implantation.Eighteen patients were enrolled in this study.Their vestibular function was evaluated by using the caloric test and video head impulse test before and one week,one month after CI surgery,respectively.The unilateral weakness(UW),slow phase velocity(SPV)in caloric test and gain in video head impulse test(vHIT-G)were observed.Caloric test was abnormal when UW>25% or SPV mean<6°/s,while vHIT was abnormal when vHIT-G<0.8.The SPV of the implanted ear were[(10.36±8.01)°/s;(14.77±14.24)°/s]pre-operatively,[(6.45±7.52)°/s;(5.14±4.67)°/s]1 week post-operatively and[(6.05±3.86)°/s;(6.27±4.17)°/s]1 month post-operatively.Statistically significant difference(<0.05)was found between pre-and post-operative period.The vHIT-G of the implanted ear were(0.73±0.33)pre-operatively,(0.65±0.32)1 week post-operatively and(0.71±0.36)1 month post-operatively.There was no statistically significant difference of vHIT-G between preand post-operative period((pre-operative/1 week post-operative)=0.084,(pre-operative/1 month post-operative)=0.679).Four patients presented with vertigo and one of them manifested slight unsteadiness post-operatively.All symptoms resolved within 7 days.These symptoms had no correlate with age,gender,implantedear and results of vestibular test.Cochlear implantation can affect the horizontal semicircular canal function,and the video head impulse test and caloric test should be used in a complementary fashion.
Caloric Tests ; Cochlear Implantation ; adverse effects ; methods ; Head Impulse Test ; Humans ; Semicircular Canals ; physiopathology ; Vertigo

BACKGROUND AND OBJECTIVES: Auditory brainstem implantation (ABI) is another option for hearing rehabilitation in non-neurofibromatosis type 2 patients who cannot undergo cochlear implantation (CI). However, the average performance of ABI is worse than that of CI. We analyzed the psycho-electrical parameters of each electrode and psycho-acoustic response to different frequency sounds in nontumor patients with ABI. SUBJECTS AND METHOD: Sixteen patients with ABI from July 2008 to May 2013 were included in the study. They were followed up for 4 to 56 months. Among them, 12 were prelingual deaf with a narrow internal auditory canal or cochlear ossification. The remaining four were post-lingual deaf adults with severely ossified cochleae. We analyzed the electrical parameters [impedance, threshold level (T level), and dynamic range] of each of the 12 electrodes. We also evaluated the sound field pure-tone threshold, Ling 6 sound detection-identification test (Ling 6 test), and pitch ranking data of these patients. RESULTS: The impedance, T level, and dynamic range did not significantly differ among electrodes. However, the pure-tone threshold to sound field stimulation was elevated in the high tone area, where more variables were found than in the low frequency area. Patients could not identify /S/ and /Sh/ sounds in the Ling 6 test. The mean T level and the dynamic range of the three highest pitch-perceiving electrodes in each patient was higher and narrower, respectively, than those of the three lowest pitch-perceiving electrodes. CONCLUSION: The nontumor patients with ABI have difficulty perceiving high pitch sound. More sophisticated penetrating type electrodes and, if possible, bimodal stimulation with CI, could be considered.
Adult ; Auditory Brain Stem Implantation* ; Auditory Brain Stem Implants* ; Cochlea ; Cochlear Implantation ; Cochlear Implants ; Electric Impedance ; Electrodes ; Hearing ; Humans ; Methods ; Rehabilitation

BACKGROUND AND OBJECTIVES: This study analyzed short-term clinical outcome and safety of Neuro One® (Oticon Medical) cochlear implantation on postlingually deafened Korean adults. SUBJECTS AND METHOD: A prospective study was performed on five postlingually deafened Korean adults who underwent Neuro One® cochlear implantation between April and June, 2016. All cases were successfully implanted via round window approach. Preoperative pure tone audiometry and speech perception score [phonetically balanced (PB) words, bi-syllable words, Korean version-central institute for the deaf (K-CID)], intraoperative evoked compound action potential (ECAP), postoperative audiometry, aided warble-tone thresholds and speech perception scores after 6 months were evaluated. Residual hearing group was defined as patients with below 40 dB of average audiometric threshold in low frequency (250 and 500 Hz). Preservation of residual hearing and postoperative complication were also evaluated. RESULTS: The mean age of five patients (3 men, 2 women) was 37.6 years. All of the recipients were unsatisfied about the conventional hearing aid. Two patients met the criteria for belonging to a residual hearing group. The audiometric threshold on the implanted side was 102.8 dB, and the opposite side was 89.6 dB. The speech perception score with hearing aid resulted in the PB word score of 35.5%, bi-syllable word score of 50%, and the open set sentence test of 62.5% on the average. The mean operation time was 87 minutes. Three patients showed normal ECAP response throughout the entire electrode intraoperatively, but the other two patients could not detect ECAP response intraoperatively. However, we could eventually identify ECAP responses in all cases postoperatively. All patients of residual hearing group could preserve their residual hearing without deteriorating more than 10 dB on audiometry. At 6 months after implantation, there was significant improvement of speech perception scores (PB word score was 54.1%; bi-syllable word score was 68.1% and K-CID was 86.2%). CONCLUSION: Neuro One® cochlear implant system was able to preserve residual hearing with relative short operation time. Short-term clinical outcome showed no significant difference with other cochlear implant devices. However, intraoperative ECAP measurement was not consistent. Further investigation was necessary.
Action Potentials ; Adult* ; Audiometry ; Cochlear Implantation* ; Cochlear Implants* ; Electrodes ; Hearing ; Hearing Aids ; Humans ; Male ; Methods ; Postoperative Complications ; Prospective Studies ; Speech Perception

BACKGROUND AND OBJECTIVES: ObjectivesZZAlthough many studies have shown that cochlear implantation promotes restoration of adequate hearing and verbal ability, there is little research showing whether patients with cochlear implants (CI) adapted well to the society by forming a good interpersonal relationship. The purpose of this study was to investigate the interpersonal competence of college students who acquired good verbal ability through CI. SUBJECTS AND METHOD: Interpersonal competence was examined using the Korean version of interpersonal competence questionnaire. Participants were 23 college students with CI who had good verbal communication abilities. Their interpersonal competence was compared to that of 28 college students with normal hearing (NH). To identify the factors contributing to the interpersonal competence, regression analysis was performed using speech perception, vocabulary and speech intelligibility as independent variables. RESULTS: College students with CI showed significantly weaker interpersonal competence than those with NH. Among 5 sub-factors of interpersonal competence, assertion and conflict management were not significantly different between two groups and initiation, caring others, and appropriate disclosure of CI group were significantly weaker than NH group. But college students with CI who had normal vocabulary and Speech Intelligibility Ratings score of 5 showed good interpersonal competence, which was comparable to those with NH. CONCLUSION: College students with CI who acquired good verbal communication ability demonstrates lower interpersonal competence than those with NH. Therefore, it is necessary to support them to improve interpersonal competence as well as speech-language ability.
Cochlear Implantation ; Cochlear Implants* ; Communication ; Disclosure ; Hearing ; Humans ; Mental Competency* ; Methods ; Speech Intelligibility ; Speech Perception ; Vocabulary

BACKGROUND AND OBJECTIVES: The objective of this study was to examine the effect of early cochlear implantation (CI) performed in infants less than 12 months of age. SUBJECTS AND METHOD: Twenty-five children who received their first CI before 12 months of age were included in this study (infant group). The speech perception and language outcomes of these children were compared with those of 14 children who received their first CI between 13 and 24 months of age (older group). All children received sequential bilateral CI with the inter-stage interval of less than 2 years. Speech perception was measured using Categories of Auditory Performance, monosyllabic word test and sentence test, and language ability was measured using Sequenced Language Scale for Infants, Preschool Receptive-Expressive Language Scale, or Receptive & Expressive Vocavulary Test, depending on the age at the time of testing. RESULTS: There were no significant differences in speech perception abilities between the infant group and the older group. The mean expressive language score of infant group was higher than that of the older group, but the difference was not statistically significant. However, the receptive language score of infant group was significantly higher than that of the older group. CONCLUSION: Children who received CI before 12 months of age achieved better receptive language ability than those who received it after 12 months of age. Thus CI should be performed as early as before 12 months of age to achieve better language ability.
Child* ; Cochlear Implantation ; Cochlear Implants* ; Humans ; Infant ; Language ; Methods ; Speech Perception*