Humans ; Auditory Pathways ; Hearing Loss, Sensorineural ; Cochlear Implantation ; Cochlear Implants ; Hearing Loss, Central ; Auditory Threshold ; Evoked Potentials, Auditory, Brain Stem

BACKGROUND AND OBJECTIVES: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). SUBJECTS AND METHODS: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. RESULTS: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R² =0.276) and C-level (p=0.002, R² =0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at ≥1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). CONCLUSIONS: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.
Auditory Perception ; Child ; Cochlear Implantation ; Cochlear Implants ; Ear ; Evoked Potentials, Auditory ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss ; Hearing Loss, Sensorineural ; Hearing ; Humans ; Retrospective Studies ; Speech Perception

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

Patients with neurofibromatosis type 2 (NF2) develop bilateral vestibular schwannomas that can cause binaural progressive hearing loss in most individuals. Auditory rehabilitation for bilateral profound sensorineural hearing loss in patients with NF2 poses a great therapeutic challenge. An auditory brainstem implantation may be an option after tumor excision, but its hearing results are still relatively unsatisfactory. A cochlear implantation (CI) may be another option in those cases where the cochlear nerve has been left intact after tumor excision or in those cases that have been kept stable after treating with Gamma-Knife. Here we report a case of undergoing CI after having been treated with Gamma-Knife in NF2 and showing improved open-set speech perception.
Auditory Brain Stem Implantation ; Auditory Brain Stem Implants ; Cochlear Implantation* ; Cochlear Implants* ; Cochlear Nerve ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural ; Humans ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Radiosurgery ; Rehabilitation ; Speech Perception

OBJECTIVE: The purpose of this study is to report surgical skills for CI cases with modiolus ossification and to investigate the relation between post-operational electroneurophysilogical test result and speech recognition result. Further more, we also attempt to confirm indications for CI in this specific population. METHOD: Based on temporal bone HRCT, 7 subjects were identified as modiolus ossification from 101 cases with cochlear ossification. Modiolus ossification is confirmed by CT scan if CT value in modiolus reaches or exceeds 900 HU with the exception of congenital modiolus ossification or modiolus seal off. Electroneurophysiological test was conducted intra- and pos-operationally speech tests were applied for 7 subjects. RESULT: Normal impedance value was observed by intro-operational measurement in 7 subjects. EABR test was conducted and negtive response was observed in only 1 subject, while other 6 subjects were confirmed with atypical EABR waves which were observed in apical and middle turn region. Hearing threshold test (in sound field) was applied, no auditory response was recorded for the subject without EABR waveform, while hearing threshold in average for the other 6 subjects was 75 dB. Results of speech tests (Mandarin) were followed as 0 for the one without EABR wave, while 100% (simple finals test) and 30% (simple initials test) for the other 6 subjects. CONCLUSION Optimal multichannel CI surgery that inserting and locating electrode array spirally is very frequently interrupted by ossification,which was indentified with atypical EABR wave and relative poor speech recognition results, especially in modiolus ossification case. A post-operative negative EABR response may indicate surgical failure following cochlear implantation.
Adolescent ; Adult ; Child ; Child, Preschool ; Cochlea ; pathology ; Cochlear Implantation ; Evoked Potentials, Auditory, Brain Stem ; physiology ; Female ; Humans ; Infant ; Male ; Middle Aged ; Ossification, Heterotopic ; surgery ; Postoperative Period ; Young Adult

OBJECTIVE: To evaluate the electrophysiological characteristics of electrically evoked auditory brainstem responses (EABR) and its application in cochlear implantation, especially in evaluating acoustic nerve survival. METHOD: An auditory evoked potential instrument was used to record responses and Cochlear Nucleus 24CA implants were used to generate electrical stimulation. We measured EABR in 23 patients with cochlear implants and compared EABR with behavioral measures and neural response telemetry (NRT). RESULT: EABR III-V waveforms were recognized in all of the 23 patients. The characteristics and origins of EABR waveforms were similar to those of ABR. The average EABR threshold was (172.61 +/- 14.61) CL. At 20 CL above threshold, the average latencies of Wave III, V were (2.93 +/- 0.18)ms, (4.80 +/- 0.28)ms which were 1-2 ms shorter than ABR latencies. But III-V intervals remained at (1.86 +/- 0.18)ms. EABR thresholds were strongly correlated with behavioral performance and NRT thresholds, while EABR input-output function is correlated with behavioral dynamic range (DR). CONCLUSION EABR is such an effective method to objectively evaluate the function of auditory pathway which can estimate residual spiral ganglion cell count. This is consistent with the foreign study leading to the conclusion that DR reflects spiral ganglion cell survival.
Auditory Threshold ; Child ; Child, Preschool ; Cochlear Implantation ; Cochlear Nerve ; physiopathology ; Deafness ; physiopathology ; surgery ; Evoked Potentials, Auditory, Brain Stem ; physiology ; Female ; Humans ; Infant ; Male

OBJECTIVE: To investigate the intra-operative electrical evoked auditory brain stem response (EABR) characteristics and the hearing and speech rehabilitation effects of cochlear implantation (CI) in patients with internal auditory canal stenosis (IACS). METHOD: A retrospective study was performed on 16 patients with IACS (IACS group) matched with 16 implanted without IACS (control group), who received multi- channel CI because of pre-lingual sensorineural hearing loss. The integrity and functional status of the auditory pathway were assessed by EABR, recording waveforms, thresholds and dynamic ranges intra-operation before CI. Interviewed the implanted parents or teachers, asking them to rate the implanted hearing and speech ability according to Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR). Paired T test was performed to compare scores of CAP and SIR between before and 1 year after CI, while Spearman test was performed to compare correlation between EABR grades and post-operative CAP scores. RESULT: Among the IACS group, 2 cases weren't recorded typical EABR waveforms and without auditory response 1 year after a successful CI. The EABR waveforms in the IACS group were poorer than that in the control group, their EABR thresholds higher than the control group, and their EABR dynamic ranges less than the control group. The hearing and speech rehabilitation after CI showed that the results of CAP and SIR values (3.47 +/- 1.09 and 1.62 +/- 0.50) scored significantly lower than the control group (5.06 +/- 0.79 and 2.59 +/- 0.58) (P < 0.05), but significantly increased compared with pre-operation. Intra-operative EABR grades and post-operative CAP scores showed significant correlation (r = 0.78 , P < 0.05). CONCLUSION Intra-operative EABR can accurately monitor the integrity and functional status of the auditory pathway, be of important clinical value in predicting whether patients can acquire auditory responses with the aid of CI. CI can help patients with IACS to improve the ability of hearing and speech with EABR to screen out compatible implanted.
Adolescent ; Auditory Pathways ; Child ; Child, Preschool ; Cochlear Implantation ; Constriction, Pathologic ; Ear, Inner ; surgery ; Evoked Potentials, Auditory, Brain Stem ; Female ; Humans ; Infant ; Labyrinth Diseases ; physiopathology ; surgery ; Male ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVE: To evaluate the pathological position of auditory neuropathy and investigate the hearing and speech rehabilitation results of cochlear implantation in patients with auditory neuropathy. METHOD: In our hospital, among the patients received cochlear implantation, 8 cases with auditory neuropathy were selected, and 8 cases of non-auditory neuropathy patients with profound deafness were selected as matched control group with the background close to the study group. The preoperative hearing data of these two groups were retrospectively analyzed. During operation, the homemade stimulation electrodes were inserted to test the electric evoked auditory brainstem response(EABR) for assessing the auditory pathway; EABR and neural response telemetry(NRT) were tested after implantation, and T, C value were acquired 1 month later. CAP, SIR and speech recognition rate were used to assess hearing and speech rehabilitation effect 12 months after booting. RESULT: Intra-operative EABR wave can be derived in 8 cases of auditory neuropathy, but the wave pattern exhibited variations compared with normal wave. It needed increased stimulation or adjusted parameters, with variable V latency. After cochlear implantation, the waveforms of NRT and EABR were similar between the two groups, and the post-operative V waveform was close to the intra-operative EABR. 8 pairs of patients can present listening response after booting. There was no statistically significant difference in T, C value, CAP (6.50 +/- 0.94 and 6.90 +/- 0.77) and speech recognition rate (85.00% +/- 11.66% and 89.50% +/- 9.02%) between the auditory neuropathy group and the control group 1 year after booting. CONCLUSION Pre-operative EABR can be used as an effective tool to assess the auditory pathway of auditory neuropathy patients, improving the pre-operative examination and helping with selecting the suitable cochlear implant patient. Cochlear implantation can help patients with auditory neuropathy to improve hearing and speech.
Adolescent ; Adult ; Child ; Child, Preschool ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Central ; physiopathology ; therapy ; Humans ; Infant ; Male ; Monitoring, Intraoperative ; Retrospective Studies ; Young Adult

OBJECTIVES: The aim of this study was to investigate changes in the hearing thresholds during the first year of life in infants who failed the newborn hearing screening (NHS) test and of infants treated in the neonatal intensive care unit (NICU). METHODS: From March 2007 to November 2010, 193 healthy infants who failed the NHS test and 51 infants who were treated in the NICU were referred for evaluation of hearing acuity. Their hearing was evaluated using impedance audiometry, auditory brainstem response (ABR), and otoacoustic emission before 6 months of age, and follow-up hearing tests were administered before 12 months of age. Changes in their hearing thresholds were then analyzed. RESULTS: Of the 193 healthy infants who failed the NHS test, 60 infants (31%) had normal hearing acuity, 126 infants (65%) had sensorineural hearing loss (SNHL, ABR threshold > or =40 dB) and 7 infants (4%) had auditory neuropathy (AN). On the follow-up hearing tests, which were conducted in 65 infants, 6 infants showed a hearing threshold deterioration of more than 20 dB, and 19 infants showed a hearing threshold improvement of more than 20 dB. Of the 51 infants who were treated in the NICU, 38 infants (75%) had normal hearing acuity, 12 infants (24%) had SNHL, and one infant (2%) had AN. In the follow-up hearing tests, which were performed in 13 infants, one infant with normal hearing progressed to severe hearing loss. Five infants who had SNHL showed a hearing threshold improvement of more than 20 dB, and 4 infants recovered to normal hearing. CONCLUSION: The hearing thresholds of infants with congenital SNHL can change during the first year of life; therefore, the importance of administration of follow-up hearing tests is emphasized. Irreversible intervention such as cochlear implantation should be considered with great caution within the first year after birth.
Acoustic Impedance Tests ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory, Brain Stem ; Follow-Up Studies ; Hearing ; Hearing Loss ; Hearing Loss, Central ; Hearing Loss, Sensorineural ; Hearing Tests ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Mass Screening ; Parturition

OBJECTIVE: To investigate the hearing and speech rehabilitation results of cochlear implantation in patients with common cavity deformity. METHOD: A retrospective study was performed on 19 patients with common cavity deformity who received multi-channel cochlear implantation from 1995 to 2010 in Peking Union Hospital, with assisted evaluation of auditory nerve pathways by intraoperative electrical evoked auditory brain stem response (EABR); matched with 19 implantees with no deformity. Paired T test was performed to compare T values, dynamic range; Rank-sum test was performed to compare scores of categories of auditory performance (CAP) and speech intelligibility rating (SIR). RESULT: The T value of common cavity inner ear malformation group (172.59 +/- 14.57) was significantly higher than that of the control group (139.63 +/- 19.45) (P < 0.05), no significant difference in dynamic range (P > 0.05); hearing and speech rehabilitation after implantation showed that the results of CAP and SIR values (5.50 +/- 0.94 and 3.00 +/- 0.82) scored significantly lower than the control group (6.90 +/- 0.77 and 3.90 +/- 0. 57) (P < 0.05), but significantly increased compared with that before. Some children appeared facial twitch at boot time caused by electrical stimulation of the facial nerve (surface pumping rate of 31.58%). CONCLUSION Common cavity is a kind of severe inner ear malformations, but to perform a comprehensive preoperative evaluation, select the appropriate surgical technique, employ personalized postoperative booting, transfer machines and long-term auditory intensive language training, the children can get varying degrees of language and hearing recovery according to the severity of deformity. On the whole, the rehabilitation outcome was significantly worse in patient with deformity than its counterpart without deformity.
Child ; Child, Preschool ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear, Inner ; abnormalities ; Evoked Potentials, Auditory, Brain Stem ; Female ; Humans ; Male ; Retrospective Studies ; Treatment Outcome