[Effectiveness of cochlear implantation in children with auditory neuropathy and cochlear nerve aplasia].

OBJECTIVE: The aim of this study is to assess of cochlear implantation in children with auditory neuropathy and cochlear nerve aplasia by using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR). METHODS: Twenty one children with cochlear implants participated in this study. They all received cochlear implant surgery at our hospital from January 2004 to October 2010. All children had hearing aid trial and hearing and speech rehabilitation before surgery at least three months.Nine children (7 male, 2 female) were diagnosed with auditory neuropathy, twelve (7 male, 5 female) with cochlear nerve aplasia. Twenty children (10 male, 10 female) with sensorineural hearing loss served as a control group. All the children received cochlear implant for more than six months. Forty two children with normal hearing served as another control group which were divided into three subgroups according to their age.Group A included 18 children aged under two yrs, group B consisted of 16 children aged from two to four yrs and group C comprised eight children aged above four yrs. CAP and SIR were used to evaluate among all the children and the scores were compared. RESULTS: The CAP scores of children with auditory neuropathy, cochlear nerve aplasia, sensorial neural hearing loss and the three subgroups children with normal hearing were 4.44 ± 1.50, 4.83 ± 1.69, 4.55 ± 1.66, 5.22 ± 1.11, 6.75 ± 0.45 and 7.00 ± 0.00 respectively, and SIR scores were 2.66 ± 1.11, 2.33 ± 1.15, 2.40 ± 0.75, 2.56 ± 1.04, 4.12 ± 0.81 and 5.00 ± 0.00 respectively. There were significant differences among the six groups for CAP scores(χ(2) = 35.481, P < 0.001) and SIR scores(χ(2) = 40.549, P < 0.001).No significant differences for CAP and SIR scores were observed between children with auditory neuropathy/cochlear nerve aplasia and sensorial neural hearing loss as well as group A (P > 0.05 for each), and there were significant differences were shown between children with auditory neuropathy/cochlear nerve aplasia and group B as well as group C (P < 0.01 for each aplasia). CONCLUSIONS: The auditory and speech capabilities of children with auditory neuropathy and cochlear nerve deficiency can can get benefits from cochlear implants as children with sensorineural hearing loss, however not achieve the level of those with normal hearing after cochlear implantation. The long term effects still need follow-up and evaluation.

[Vibrant soundbridge implantation (two cases report)].

OBJECTIVE: To investigate the effect of Vibrant Soundbridge (VSB) implantation. METHODS: In accordance with the indications for VSB implantation, surgeries were done for two patients who suffered from either a sensorineural or conductive hearing loss (microtia). Their preoperative auditory thresholds (0.5, 1, 2 and 4 kHz) were 56 dB HL and 61 dB HL. The VSB was turned on and adjusted seven weeks after surgery. RESULTS: Postoperative auditory thresholds of the two patients were improved. Their pure tone thresholds were 32 dB HL and 40 dB HL, and the respective improvement was 24 dB HL and 21 dB HL. There was no facial paralysis, vertigo and tinnitus. CONCLUSION: Patients with a sensorineural or conductive hearing loss may benefit from VSB implantation.

[Quality of life after cochlear implantation in postlingually deaf adults].

OBJECTIVE: To evaluate the quality of life after cochlear implantation in adult cochlear implant recipients and to assess the applicability of Nijmegen Cochlear implant Questionnaire (NCIQ). METHODS: Thirty-two postlingually deaf patients, 19 males and 13 females, underwent cochlear implantation with the age at operation of 35.27 (18-66) during the period from 1996 to the end of 2006. NCIQ was used to investigate the application of the equipment, hearing ability, and emotional exchange ability. RESULTS: All of the patients used their cochlear implants for 16 h every day on average with an experience in using such implant of more than 6 months. Improvement was seen in sound recognition, speech recognition, confidence, daily life, social exchange, and emotional behavior ability at different degrees. The improvement of confidence was very obvious. The speech communication ability of those with a hearing deprivation time longer than 5 years was lower than that of those with a shorter hearing deprivation time. Age and educational level did not influence the efficiency. CONCLUSION: Cochlear implantation is a cost effective intervention in the postlingually deaf adults. NCIQ test can be used to evaluate the quality of life among postlingually deaf adult cochlear implant recipients in China.

[Cochlear implantation in the ossified cochlear].

OBJECTIVE: To explore the problems and describe clinical experiences associated with multichannel cochlear implantation in patients with cochlear ossification. METHODS: Seven cochlear implant cases with bilateral cochlear ossification from 1996 to 2006 in Beijing Tongren Hospital were retrospectively reviewed, 4 of which were the consequence of meningitis. Three patients' cochlear were completely ossified, 1 patient's tympanic scala was completely ossified, 1 patient's cochlear was partially ossified, and 2 patients' cochlear were partially fibrotic. This article addressed the components of the preoperative evaluation, surgical decision-making, and specific techniques for cochlear implant array insertion in all kinds of ossified cochlea. RESULTS: Gusher was found in 1 case but less serious than that with inner ear malformations. The electrodes were inserted in the cochleostomy in full length in 4 cases, 1 case gave up, and the cochlear implant array were partially inserted in the remains. No serious complications occurred after implantation. All patients had auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold were slightly higher than that of the normal cochlear implantation. CONCLUSIONS: The key influencing factor of ossified cochlear patients were the degree of the disease and whether the electrode implanted completely or not. So, the profound sensorineural hearing loss patients after meningitis should be paid more attention. Patients with ossified cochlear could be benefit from cochlear implantation.

[Outcome of cochlear implantation in prelingual pediatric auditory neuropathy].

OBJECTIVE: To explore the electrophysiological results and rehabilitation outcome of two prelingually deafened pediatric cochlear implant patients with auditory neuropathy. METHODS: Preoperative audiological evaluation, intra-postoperative electrically evoked auditory brainstem response (EABR) and neural response telemetry (NRT) record for the two cases were conducted in Beijing Tongren Hospital. A one year follow-up was performed. Data collected before and at 6,12-month intervals after implantation were compared with that from control pediatric cochlear implant patients matched for the same duration of implant use as this two cases. RESULTS: The two children implanted had not had any postoperative medical or cochlear implant device complications. Intraoperative EABR and NRT were elicited in case 1 with unrepeatable waveforms. After 12 months of training, Case 1 had shown significant improvements in sound detection, speech perception abilities and communication skills, which was better than the control group, and the electrophysiological results became normal. Case 2 had also benefited from cochlear implantation, even though no recognizable NRT was found until he returned 12 month after the operation. CONTUSIONS: The desynchronization of auditory path had been changed after the electrical stimulation ongoing 12 months for children with auditory neuropathy. The two children had not had any complications postoperatively, and each child had shown improved listening and communication skills. Cochlear implantation could help patients with auditory neuropathy to improve their communication skill and go back to the main stream.

[Comparison of sensitivity of audiological tests to identify otitis media with effusion in newborn infants].

OBJECTIVE: To investigate the sensitive factors which were used in routine audiological tests to find out otitis media with effusion (OME) in newborn infants. METHODS: Subjects of this study were 48 infants, including 31 males and 17 females, who failed in the universal newborn hearing screening. The age ranged from 1.5 to 12 months with the average age of 4.3 months. All subjects accepted temporal bone CT and routine audiological assessments, including air-conduction and bone-conduction auditory brainstem response (ABR), 40 Hz-auditory event related potential (40 Hz-AERP), distortion-product otoacoustic emission (DPOAE), acoustic reflex, tympanometries using 226 Hz and 1000 Hz probe tone. Nine factors were statistically analyzed using Kappa test, Univariate chi(2) test and multivariate condition Logistic stepwise regression analysis, which included the results of acoustic immittance, the air-conduction and bone-conduction ABR thresholds, the difference between air-conduction and bone-conduction ABR thresholds, the latency of ABR wave I, duration between ABR wave I and V, 40 Hz-AERP thresholds, amplitudes and thresholds of DPOAE, and acoustic reflex thresholds (ART). RESULTS: Seventy-seven ears were diagnosed with OME, and 19 ears were normal. CT scan of temporal bone was set as a comparative standard. Kappa test indicated that the results of tympanometry with 1000 Hz probe tone (Kappa = 0.745, P < 0.001), the air-conduction ABR threshold (Kappa = 0.453, P < 0.001), the latency of ABR wave I (Kappa = 0.430, P < 0.001), the threshold of 40 Hz-AERP (Kappa = 0.582, P < 0.001), and DPOAE (Kappa = 0.495, P < 0.001) had agreement with the results of temporal bone CT on evaluating the function of middle ear. Univariate analysis indicated that sensitive factors of middle ear function in newborn infants were tympanometry with 1000 Hz probe tone (P < 0.001), ART (P < 0.001), the air-conduction ABR threshold (P < 0.001), the difference between air-conduction and bone-conduction ABR thresholds (P < 0.001), the latency of ABR wave I (P < 0.001), the threshold of 40 Hz-AERP (P < 0.001) and DPOAE (P < 0.001). And multivariate conditional Logistic stepwise regression model showed that tympanometry with 1000 Hz probe tone (P < 0.001) and 40 Hz-AERP threshold (P = 0.004) can be substituted into Logistic stepwise regression equation. CONCLUSIONS: Tympanometry with 1000 Hz probe tone and are sensitive factors to find out OME in newborn infants. The air conduction ABR threshold, ABR wave I latency, 40 Hz-AERP threshold and DPOAE could reflect the middle ear function of newborn infants effectively.

[Auditory capability evaluation for children after cochlear implantation using meaningful auditory integration scale].

OBJECTIVE: To analyze the auditory capability of preschool children before and after cochlear implantation using meaningful auditory integration scale (MAIS) questionnaire. METHODS: Eighty-two prelingually deaf patients participated in this study. They received a cochlear implant at the age of 3 to 6 years and 11 months. The audiologists who were trained for the research used the MAIS questionnaire. Audiologists asked for the parents' answers and recorded all the information about the device using (Q1,2) and the patient's spontaneous auditory behavioural responses including spontaneous alerting to sound Q3 approximately 6 and deriving meaning from sound (Q7 approximately 10). The evaluation was performed before operation and 1 , 3, 6 months, 1, 1.5, 2 years after switch-on. RESULTS: The scores of question 1a and 1b were not significantly different among the different periods after switch-on. The scores of question 2 to 10 were significantly different among the different periods after switch-on. CONCLUSIONS: Considerable variability across subjects' auditory ability after cochlear implantation was noted. Most of the patients showed no consistent response to sound in everyday life before implantation. After cochlear implantation, a significant increase in auditory capability occurred. The children demonstrated faster development of device using relative to spontaneous alerting to sound and deriving meaning from sound.

[Follow-up study for newborns and infants who failed hearing screening].

OBJECTIVE: To study the audiological characteristics of newborns and infants who failed hearing screening. METHODS: One hundred and six infants failed hearing screening received follow-up study with routine audiological evaluations (auditory brainstem response, distortion product otoacoustic emission, tympanometry and visual reinforcement audiometry). RESULTS: Sixty-five infants (61.3%) of this group were normal hearing subjects and 39(36. 8% ) of the infants had hearing loss. Two cases (1.9%) received follow-up by phone. Fifteen cases (14.2%) with conductive hearing loss and 24 cases (22.6%) with sensorineural hearing loss. Thirteen (12.3%), 14 (13.2%), 6 (5.7%), and 6 (5.7%) cases were found to be mild, moderate, severe and profound hearing loss respectively. Diagnosis of hearing loss in the thirty-nine infants conducted a prevalence of 0.264% (39/14 785) of congenital hearing loss (both binaural and monaural). The hearing level of those cases with severe and profound hearing loss basically did not change, but that of cases with mild and moderate hearing loss changed. CONCLUSIONS: Early identification and intervention of infants with severe and profound hearing loss by 6 months of age were successful. Infants with mild and moderate hearing loss should be followed up to six or eight months and received routine audiologic evaluations.

Comparisons between neural response imaging thresholds, electrically evoked auditory reflex thresholds and most comfortable loudness levels in CII bionic ear users with HiResolution sound processing strategies.

CONCLUSIONS: The data collected in this study indicated that first Neural Response Imaging (NRI) thresholds had a better correlation with HiResolution most comfortable loudness (M) levels than tNRI thresholds. Electrically evoked auditory reflex thresholds (EARTs) had a higher correlation with HiResolution M levels than tNRI thresholds and a lower correlation than first NRI thresholds. NRI is a very useful method for programming the cochlear implants of young children who cannot demonstrate a reliable judgment of loudness. OBJECTIVE: To investigate how HiResolution sound processing, designed to deliver high-rate stimuli, relates to EARTs and electrically evoked compound action potential measurements produced by low-rate stimuli. MATERIAL AND METHODS: Nine profoundly hearing-impaired children and adults aged 6-29 years participated in the study. NRI responses were elicited using pulse trains consisting of biphasic pulses at a pulse width per phase of 32 micros delivered at a frequency of 30 Hz using SoundWave programming software. Stimuli were delivered to the odd electrodes (1, 3, 5, 7, 9, 11, 13 and 15) along the array. tNRI (NRI threshold) and first NRI thresholds were recorded for each stimulating electrode. "Speech bursts" stimuli used in EARTs recording were delivered to four electrodes at a time and stapedial reflexes were recorded from the impedance bridge. The M levels used were those used by each patient in their everyday HiResolution programs. RESULTS: For 8 patients (53 stimulating electrodes) the correlation between tNRI threshold and M level was r=0.675 (p=0.000) and that between first NRI thresholds and M level was r=0.741 (p=0.000). On average the M-level value was 20 CU (Current Unit) lower than the first NRI threshold value and 12 CU higher than the tNRI threshold value. The M-level patterns across the electrode array overall were similar to the tNRI or first NRI threshold patterns. For 7 patients (112 stimulating electrodes) the correlation between EART and M levels was r=0.710 (p=0.000). On average the EART value was 14 CU higher than the M-level value.

[Multi-channel cochlear implants in patients with Mondini malformation].

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with Mondini malformation. METHODS: Among 300 patients who received multi-channel cochlear implants from 1996 to 2002 in Beijing Tongren Hospital, 15 patients were diagnosed with Mondini malformation. A retrospective analysis was performed dealing with the surgical techniques, mapping and rehabilitations characteristics after surgery. 15 patients with normal cochlear structure are consider as control group. RESULTS: Gusher is found more common than the normal cochlear implantation, most of them are serious. The electrodes are inserted in the "cochleostomy" in full length of 13 Patients, 2 pairs of electrodes remains outside of "cochleostomy" in 2 patients. No serious complications occurred after implantation. All patients have auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold are similar with the normal cochlear implantation group. The results have no significant difference in compare with normal cochlear group(P > 0.05). CONCLUSION: Multi-channel cochlear implantation could be performed safely in patients with Mondini malformation. The primary outcome for patients with Mondini malformation are similar to those with normal cochlear structure following the multi-channel cochlear implantation.

[Cochlear implantation in patients with inner ear malformations].

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHODS: Among 410 patients who received multi-channel cochlear implantations from 1996 to 2004 in Beijing Tongren Hospital, 82 patients were diagnosed with inner ear malformations and implanted. A retrospective analysis was performed about the surgical characteristics and mapping characteristics after implantation. RESULTS: (1) All patients had auditory sensations. (2) Gusher was more common than the normal cochlear implantation. (3) The electrodes were inserted in the "cochleostomy" in full length of 80 Patients, but 2 pairs of electrodes remained outside of "cochleostomy" in 2 patients. (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, the T level and C level were similar with the normal cochlear implantation. The results had no significant difference in compare with normal cochlear group (P > 0. 05). (6) The abilities of speech discrimination and spoken language were improved through rehabilitation. CONCLUSIONS: The cochlear implantation can be performed safely in inner ear malformations. The outcome of hearing rehabilitation for patients with inner ear malformations are similar to those children with normal cochlear structure followed the multi-channel cochlear implantation.

[Cochlear implants in children with enlargement of vestibular aqueduct].

OBJECTIVE: To describe the clinical experiences with multi-channel cochlear implantation in the children with enlargement of vestibular aqueduct. METHOD: Fifteen patients were diagnosed with enlargement of vestibular aqueduct and received multi-channel cochlear implantation at Beijing Tongren Hospital. A cochleostomy was performed for all patients by a standard facial recess approach to anterior inferior to the round window niche. RESULTS: (1) Most of the electrodes were totally inserted in the cochlear, and gusher appeared in 6 implantations. (2) No serious complications occurred after implantation. (3) All patients have auditory sensations after implantations. (4) The hearing threshold in this series were similar to that of the normal cochlear implantation (the average threshold was 30 dB). (5) The rehabilitation concept was developed by Rehabilitation Research Center for Deaf Children in Beijing and Beijing Tongren Hospital. The training sessions included sound detection, word discrimination, auditory comprehension and spoken language skill development. The abilities of speech discrimination and spoken language were improved comparing with that of pre-operation. Two of the patients could go to the normal school after implantations. CONCLUSION: The outcome of hearing rehabilitation for children with enlargement of vestibular aqueduct were similar to those children with "normal" cochlear structure followed the multi-channel cochlear implantation.

[Cochlear implantation in patients with inner ear malformations, clinical analysis of 25 cases].

OBJECTIVE: To explore the clinical experience of multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHOD: Multi-channel cochlear implantation was conducted among 180 patients from 1997 to 2001. Twenty-five of them, being with inner ear malformations, were given hearing and speech rehabilitation including sound detection, word discrimination, auditory comprehension and spoken language skill development. A follow-up lasting 4 - 24 months was conducted after the operation. A retrospective analysis was performed among these 25 patients to observe the surgical outcomes and mapping characteristics after surgery. RESULTS: (1) All patients restored their auditory sensations after the operation. (2) Stapedial gusher was found in 13 cases with inner ear malformations, especially those with Mondini malformation, much more frequently than among the patients with other malformations. (3) Most of the action electrodes were inserted in the "cochleostomy" in full length, only 2 pairs of them remained outside of the "cochleostomy". (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, T level and C level were higher in the 25 cases than in the normal cochlear implantation, however, with the hearing similar hearing threshold. (6) After training, the abilities of speech discrimination and spoken language were improved in comparison with those before operation. CONCLUSION: The cochlear implantation can be performed safely in patients with bilateral inner ear malformations. The primary outcome of cochlear implantation in hearing rehabilitation is similar for both the patients with inner ear malformations and those with normal cochlear structure.