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

BACKGROUNDThe auditory brainstem implants (ABIs) have been used to treat deafness for patients with neurofibromatosis Type 2 and nontumor patients. The lack of an appropriate animal model has limited the study of improving hearing rehabilitation by the device. This study aimed to establish an animal model of ABI in adult rhesus macaque monkey (Macaca mulatta).

METHODSSix adult rhesus macaque monkeys (M. mulatta) were included. Under general anesthesia, a multichannel ABI was implanted into the lateral recess of the fourth ventricle through the modified suboccipital-retrosigmoid (RS) approach. The electrical auditory brainstem response (EABR) waves were tested to ensure the optimal implant site. After the operation, the EABR and computed tomography (CT) were used to test and verify the effectiveness via electrophysiology and anatomy, respectively. The subjects underwent behavioral observation for 6 months, and the postoperative EABR was tested every two weeks from the 1 st month after implant surgery.

RESULTThe implant surgery lasted an average of 5.2 h, and no monkey died or sacrificed. The averaged latencies of peaks I, II and IV were 1.27, 2.34 and 3.98 ms, respectively in the ABR. One-peak EABR wave was elicited in the operation, and one- or two-peak waves were elicited during the postoperative period. The EABR wave latencies appeared to be constant under different stimulus intensities; however, the amplitudes increased as the stimulus increased within a certain scope.

CONCLUSIONSIt is feasible and safe to implant ABIs in rhesus macaque monkeys (M. mulatta) through a modified suboccipital RS approach, and EABR and CT are valid tools for animal model establishment. In addition, this model should be an appropriate animal model for the electrophysiological and behavioral study of rhesus macaque monkey with ABI.

Sensorineural hearing loss is one of the most common chronic clinical disorders that we can easily encounter. The etiology of sensorineural hearing loss is multifactorial: congenital, idiopathic, traumatic, noise-induced, head injury induced, infectious disease, drug induced, degenerative, immune disorder, vestibular schwannoma and Meniere's disease. Many people are living with the discomfort of hearing loss because fundamental treatment is has not yet been found. Also due to the progress of medical science, human life span has been extended. As the result, the number of patients suffering from hearing loss has increased. But the present situation does not measure up to the demand for recovery of hearing loss. Hearing loss has a great influence on the quality of life. To overcome this situation, neural prostheses such as the cochlear implant and auditory brainstem implant are helpful for the rehabilitation of total deaf patients. Recently, due to the advancement of studies related to hair cell regeneration and the field of gene therapy on the inner ear has made big progress during the last few years. The purpose of this study is to describe the latest known causes and rehabilitation of sensorineural hearing loss.
Auditory Brain Stem Implants ; Cochlear Implants ; Communicable Diseases ; Correction of Hearing Impairment ; Craniocerebral Trauma ; Ear, Inner ; Genetic Therapy ; Hair ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hearing* ; Humans ; Immune System Diseases ; Meniere Disease ; Neural Prostheses ; Neuroma, Acoustic ; Quality of Life ; Regeneration ; 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: 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

Between one and three of every 1,000 neonates have sensorineural hearing loss (SNHL). It is of utmost importance to minimize the duration of auditory deprivation between the onset of bilateral deafness and intervention using hearing devices such as hearing aids and cochlear implants for achieving the best speech percpetion ability. To fit amplification accurately for children with SNHL, hearing thresholds for frequencies in the range of human communication should be evaluated. However, infants and young children are difficult to test using conventional behavioral tests, and hearing thresholds of them can be predicted using auditory brainstem response (ABR) and auditory steady-state response (ASSR). ABR is best evoked by applying a click stimulus, which allows an estimate over a broad range of high frequencies. ABR elicited by tone burst stimulus provides frequency-specific audiometric information. However, it can be difficult to record and observe at near-threshold levels, especially at lower frequencies. ABR thresholds for click and tone burst stimuli are highly correlated with behavioral thresholds, and often give an idea of the shape of an audiogram. ASSR is an auditory evoked potential, elicited with modulated tones. It provides frequency-specific hearing thresholds across the audiometric frequencies, which are well correlated with behavioral thresholds. However, the accuracy of threshold prediction decreases directly with the decrease of degree of hearing loss, and hearing thresholds cannot be predicted for auditory neuropathy. ASSR is most useful for estimating auditory thresholds for patients with no evidence of auditory neuropathy by the click ABR and OAEs, and who have an ABR only at high intensities or no ABR at a maximum stimulus level. Even if hearing thresholds are predicted through ABR and ASSR, behavioral testing including behavioral observation audiometry, visual reinforcement audiometry, or play audiometry should be employed repeatedly to verify the predicted thresholds, becasue the thresholds of ABR and ASSR are not true measure of hearing acuity but just responses generated at the brainstem.
Audiometry ; Auditory Threshold ; Brain Stem ; Child ; Cochlear Implants ; Deafness ; Evoked Potentials, Auditory ; Evoked Potentials, Auditory, Brain Stem ; Hearing ; Hearing Aids ; Hearing Loss ; Hearing Loss, Central ; Hearing Loss, Sensorineural ; Humans ; Infant ; Infant, Newborn ; Reinforcement (Psychology)

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