人工耳蜗植入术中圆窗耳蜗电图评价耳蜗残余听功能

目的应用术中圆窗耳蜗电图评估极重度感音神经性聋患者耳蜗残余听功能。方法20例患者全麻下人工耳蜗植入手术过程中,行术中圆窗耳蜗电图测试,测出的复合动作电位（CAP）阈值与术前纯音测听或其他听力测试之阈值进行比较。结果20例患者术中圆窗耳蜗电图测试测出的复合动作电位（CAP）阈值与术前1、2、4kHz听力测试阈值分别有较好的相关系数（0．20429,0．04076,0．38163）。结论术中圆窗耳蜗电图可以较准确客观地评估极重度感音神经性聋患者耳蜗残余听功能,且是人工耳蜗植入术前听力学评估方法的有意义的补充。     

Auditory brainstem response results as predictors of behavioral auditory thresholds in severe and profound hearing impairment

Pediatric cochlear implantation is restricted to patients with stable, bilateral profound sensorineural hearing losses who derive no benefit from conventional amplification. Obtaining reliable audiologic thresholds in a young child with sudden or early-onset hearing loss can be challenging. This study examines the accuracy with which auditory brainstem response evaluation can predict unaided and aided behavioral thresholds in a child with severe-to-profound hearing loss. Reliable behavioral thresholds were obtained on 119 children who had no measurable click-evoked auditory brainstem responses at instrumentation limits of 100 dB HLn. These data show that an absent auditory brainstem response at 100 dB HLn does not necessarily indicate the absence of measurable unaided hearing for test frequencies ranging from 250 Hz to 4000 Hz. Average aided thresholds of better than 60 dB were present in 43% of the children for 500, 1000, and 2000 Hz and in 53% for 500 and 1000 Hz. Therefore, the absence of a click-evoked auditory brainstem response at 100 dB HLn in a young child is not prima facie evidence of the child's cochlear implant candidacy.     

Multiple auditory steady-state responses in children and adults with normal hearing, sensorineural hearing loss, or auditory neuropathy

OBJECTIVES: We tested the clinical effectiveness of multiple auditory steady-state responses (ASSRs) for the objective assessment of hearing thresholds in patients with and without hearing loss, candidates for cochlear implants, and children with auditory neuropathy. METHODS: The study sample included 29 subjects with sensorineural hearing loss (SNHL), 18 candidates for cochlear implants, 11 subjects with auditory neuropathy, and 18 subjects with normal hearing thresholds. Behavioral hearing thresholds and ASSRs to carrier frequencies of 0.5, 1, 2, and 4 kHz were obtained. Special care was taken to minimize possible aliasing and high-intensity multiple stimulation effects. Differences and correlations between the ASSRs and the behavioral thresholds were determined. RESULTS: The ASSR estimation of behavioral thresholds in the normal-hearing group was elevated, whereas very close predictions were found for the SNHL group. The correlations between the two measures ranged from 0.86 at 0.5 kHz carrier frequency to 0.94 at 2 kHz. In the cochlear implant candidates and the auditory neuropathy group, the ASSR thresholds generally overestimated the behavioral audiogram. In these groups the number of detected ASSRs was higher than the number of behavioral responses, especially for the high-frequency carrier stimuli. CONCLUSIONS: Multiple ASSRs may reliably predict the behavioral threshold in subjects with SNHL and may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in candidates for cochlear implants and children with auditory neuropathy.     

Assessment of basal sound identification skills and communication abilities in profoundly deaf children fitted with hearing aids or a cochlear implant

Basal auditory functions and early verbal communication skills were examined in young, profoundly deaf children with hearing aids or a cochlear implant. The hearing aid users (n = 23) were subdivided on the basis of their (unaided) hearing thresholds into: group A (pure tone average (PTA) at 0.5, 1 and 2 kHz: 90–100 dB HL); group B (PTA: 100–110 dB HL); and group C (PTA > 110 dB HL). All the children with a cochlear implant (n = 20) had a profound sensorineural hearing loss with a PTA that exceeded 120 dB HL. Functional hearing was evaluated by means of basal sound identification. The child’s communication abilities with hearing aids or a cochlear implant were assessed using structured observations on the Scales of Early Communication Skills for Hearing Impaired Children. The basal auditory functions on a sound identification level improved over time in the cochlear implant users and groups A and B. Hardly any improvement was seen in group C. The performance of all the groups (either hearing aid or cochlear implant) on the Scales of Early Communication Skills for Hearing Impaired Children at 6 months after fitting the device and at later evaluations, was close to the average level for their age.     

不同听觉干预手段对聋儿声调辨别能力的影响

目的了解聋儿对声调的识别能力,以指导聋儿的言语康复训练。方法将受试对象分为3组:人工耳蜗组、助听器组和对照组。人工耳蜗组和助听器组均为双耳重度或极重度听力损失患儿。人工耳蜗组43例,其中男20例,女23例,人工耳蜗植入年龄在2.5～7岁;助听器组41例,男22例,女19例;对照组28例为听力正常儿童,男13例,女15例,年龄3～6岁。测试3组儿童对单音节词声调、扬扬格词声调的识别能力。结果人工耳蜗组对单音节词、扬扬格词声调的识别能力显著优于助听器组(P<0.01),但弱于正常对照组(P<0.01)。结论不同的听觉干预手段对聋儿声调识别能力有影响,人工耳蜗对重度或极重度听力损失的儿童较助听器有更好的声调识别能力。     

Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection

The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech–language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 ± 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.     

Bilateral cochlear implants in children: localization acuity measured with minimum audible angle

OBJECTIVE: To evaluate sound localization acuity in a group of children who received bilateral (BI) cochlear implants in sequential procedures and to determine the extent to which BI auditory experience affects sound localization acuity. In addition, to investigate the extent to which a hearing aid in the nonimplanted ear can also provide benefits on this task. DESIGN: Two groups of children participated, 13 with BI cochlear implants (cochlear implant + cochlear implant), ranging in age from 3 to 16 yrs, and six with a hearing aid in the nonimplanted ear (cochlear implant + hearing aid), ages 4 to 14 yrs. Testing was conducted in large sound-treated booths with loudspeakers positioned on a horizontal arc with a radius of 1.5 m. Stimuli were spondaic words recorded with a male voice. Stimulus levels typically averaged 60 dB SPL and were randomly roved between 56 and 64 dB SPL (+/-4 dB rove); in a few instances, levels were held fixed (60 dB SPL). Testing was conducted by using a "listening game" platform via computerized interactive software, and the ability of each child to discriminate sounds presented to the right or left was measured for loudspeakers subtending various angular separations. Minimum audible angle thresholds were measured in the BI (cochlear implant + cochlear implant or cochlear implant + hearing aid) listening mode and under monaural conditions. RESULTS: Approximately 70% (9/13) of children in the cochlear implant + cochlear implant group discriminated left/right for source separations of 相似文献   

Hearing impairment in patients with acoustic neuroma—analysis by electrocochleography

Objective: In patients with acoustic neuroma, the site and severity of hearing impairment are important in discussing surgical approaches. Since the effectiveness of conventional auditory psychological testing is limited, we studied objectively hearing impairment of the cochlea and the cochlear nerve due to the tumor. Methods: Electrocochleography (ECochG) was carried out in 21 patients with acoustic neuroma. Cochlear microphonic potential (CM) and action potential (AP) in ECochG evoked with clicks and short tone bursts were recorded through a transtympanic needle electrode technique. Cochlear function was studied using the detection thresholds of CM, and cochlear nerve involvement was analyzed by differences between AP and CM detection thresholds. Results: The 1 kHz CM detection threshold was elevated in 17 (81.0%) of 21 patients indicating cochlear impairment. Of seven patients with normal hearing or mild sensorineural hearing loss in pure tone audiometry, three had a slightly elevated CM detection threshold. Of five patients with pronounced pure tone levels, four showed a CM response and were thought to have mild cochlear dysfunction. Cochlear nerve impairment was confirmed in three of four patients with well-developed CM based on elevated AP detection thresholds. Three patients had CM response but no AP response, suggesting severe cochlear nerve impairment. Conclusion: Disorders of the cochlea and the cochlear nerve can be evaluated with ECochG AP and CM measurement. The findings of ECochG are thought to be important information to judge hearing prognosis, thereby enhancing its clinical utility.     

Audiological and electrocochleography findings in hearing-impaired children with connexin 26 mutations and otoacoustic emissions

We recorded cochlear potentials by transtympanic electrocochleography (ECochG) in three hearing-impaired children with GJB2 mutation who showed otoacoustic emissions. Pure tone thresholds, distortion product otoacoustic emissions (DPOAEs) and, auditory brainstem responses (ABRs) were also obtained. Subjects 1 (35delG/35delG) and 3 (M34T/wt) had profound hearing loss and showed the picture of auditory neuropathy (AN) as DPOAEs were detected with absent ABRs in both ears. The hearing impairment found in subject 2 (35delG/35delG) was profound in the right ear and moderate in the left ear. Both DPOAEs and ABRs with normal latencies and morphology were recorded only from the left ear. On the ECochG recording the cochlear microphonic was obtained from all children. No compound action potential (CAP) was detected in subject 1. A neural response was recorded only from the left ear in subject 2 with a threshold corresponding to the audiometric threshold while no CAP was detected on the right side. The ECochG obtained from subject 3 showed a low-amplitude broad negative deflection which was identifiable down to low stimulus levels. This response decreased in amplitude and duration when utilizing a high-rate stimulation paradigm. The amount of amplitude reduction was close to that calculated for normal ears, thus revealing the presence of an adapting neural component. These findings indicate that patients with GJB2 mutations and preserved outer hair cells function could present with the picture of AN. The hearing impairment is underlain by a selective inner hair cell loss or a lesion involving the synapses and/or the auditory nerve terminals. We suggest that neonatal hyperbilirubinemia may play a role in protecting outer hair cells against the damage induced by GJB2 mutations.     

Effects of early auditory experience on the spoken language of deaf children at 3 years of age

OBJECTIVE: By age 3, typically developing children have achieved extensive vocabulary and syntax skills that facilitate both cognitive and social development. Substantial delays in spoken language acquisition have been documented for children with severe to profound deafness, even those with auditory oral training and early hearing aid use. This study documents the spoken language skills achieved by orally educated 3-yr-olds whose profound hearing loss was identified and hearing aids fitted between 1 and 30 mo of age and who received a cochlear implant between 12 and 38 mo of age. The purpose of the analysis was to examine the effects of age, duration, and type of early auditory experience on spoken language competence at age 3.5 yr. DESIGN: The spoken language skills of 76 children who had used a cochlear implant for at least 7 mo were evaluated via standardized 30-minute language sample analysis, a parent-completed vocabulary checklist, and a teacher language-rating scale. The children were recruited from and enrolled in oral education programs or therapy practices across the United States. Inclusion criteria included presumed deaf since birth, English the primary language of the home, no other known conditions that interfere with speech/language development, enrolled in programs using oral education methods, and no known problems with the cochlear implant lasting more than 30 days. RESULTS: Strong correlations were obtained among all language measures. Therefore, principal components analysis was used to derive a single Language Factor score for each child. A number of possible predictors of language outcome were examined, including age at identification and intervention with a hearing aid, duration of use of a hearing aid, pre-implant pure-tone average (PTA) threshold with a hearing aid, PTA threshold with a cochlear implant, and duration of use of a cochlear implant/age at implantation (the last two variables were practically identical because all children were tested between 40 and 44 mo of age). Examination of the independent influence of these predictors through multiple regression analysis revealed that pre-implant-aided PTA threshold and duration of cochlear implant use (i.e., age at implant) accounted for 58% of the variance in Language Factor scores. A significant negative coefficient associated with pre-implant-aided threshold indicated that children with poorer hearing before implantation exhibited poorer language skills at age 3.5 yr. Likewise, a strong positive coefficient associated with duration of implant use indicated that children who had used their implant for a longer period of time (i.e., who were implanted at an earlier age) exhibited better language at age 3.5 yr. Age at identification and amplification was unrelated to language outcome, as was aided threshold with the cochlear implant. A significant quadratic trend in the relation between duration of implant use and language score revealed a steady increase in language skill (at age 3.5 yr) for each additional month of use of a cochlear implant after the first 12 mo of implant use. The advantage to language of longer implant use became more pronounced over time. CONCLUSIONS: Longer use of a cochlear implant in infancy and very early childhood dramatically affects the amount of spoken language exhibited by 3-yr-old, profoundly deaf children. In this sample, the amount of pre-implant intervention with a hearing aid was not related to language outcome at 3.5 yr of age. Rather, it was cochlear implantation at a younger age that served to promote spoken language competence. The previously identified language-facilitating factors of early identification of hearing impairment and early educational intervention may not be sufficient for optimizing spoken language of profoundly deaf children unless it leads to early cochlear implantation.     

语前聋患儿一侧人工耳蜗对侧助听器效果分析

目的:通过对人工耳蜗植入对侧耳不同听力损失的患儿联合使用助听器与人工耳蜗语前聋患儿的听觉、语言及学习能力进行评估和比较,探索对患儿更为有效的助听方法,帮助患儿获得最大限度的言语交流。方法:将30例3～6岁语前聋患儿按照植入人工耳蜗对侧耳听力损失程度及是否佩戴助听器,分为一侧人工耳蜗+对侧重度听力损失助听器组(CI+SHA组)、一侧人工耳蜗+对侧极重度听力损失助听器组(CI+PHA组)、单耳人工耳蜗组(CI组)。评估各组在康复3、6、9、12、15、18个月时听觉、语言及学习能力,并记录结果。结果:随着术后康复时间的延长,聋儿听觉、语言及学习能力逐渐提高(P<0.05),CI+SHA组听觉能力优于CI+PHA组及CI组(均P<0.05),语言能力及学习能力无明显差异(P>0.05)。结论:语前聋患儿单耳人工耳蜗植入后,若对侧耳尚有残余听力,佩戴助听器后听觉能力效果显著,长期佩戴有助于患儿的康复。     

Evaluation of residual hearing in cochlear implants candidates using auditory steady-state response

Conclusion: The correlations between behavioral and auditory steady-state response (ASSR) thresholds were significant at 500, 1000, 2000, and 4000 Hz. ASSR presented high sensitivity and specificity in the detection of residual hearing in cochlear implant candidates when compared with warble-tone audiometry. Objectives: To assess residual hearing in cochlear implant candidates by comparing the electrophysiological thresholds obtained in dichotic single-frequency ASSR with behavioral thresholds at 500, 1000, 2000, and 4000 Hz. Methods: This was a comparative study between ASSR and warble-tone audiometry thresholds in 40 cochlear implant candidates (80 ears) before cochlear implantation with bilateral severe-to-profound sensorineural hearing loss. Results: Thresholds were obtained in 62.5% of all frequencies evaluated in warble-tone audiometry and in 63.1% in the ASSR. ASSR sensitivity was 96% and specificity was 91.6%. Mean differences between behavioral and ASSR thresholds did not reach significance at any frequencies. Strong correlations between behavioral and ASSR thresholds were observed in 500, 1000, and 2000 Hz and moderate in 4000 Hz, with correlation coefficients varying from 0.65 to 0.81. On 90% of occasions, ASSR thresholds were acquired within 10 dB of behavioral thresholds.     

Auditory neuropathies in infants

Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory evoked potentials, and normal cochlear outer hair cell function. It is believed that a variety of processes are involved in its pathophysiology and their influence on hearing can differ. We describe the diagnostic sequence and management of two new cases of auditory neuropathy in infants. The first case was a girl with no risk factors for hearing loss. Her absence of response to sounds was compared with her twin's reactions. Otoacoustic emissions were present but no auditory evoked potential response was detected. Her evolution was characteristic of deep sensorineural hearing loss without a hearing aid. At present she is awaiting a cochlear implant. The second case was a boy who underwent hearing loss screening for hyperbilirubinemia. Examinations were repeated three months later because the mother suspected hearing loss. Objective tests showed a pattern of auditory neuropathy. The boy's evolution was different: in spite of the presence of destructured auditory evoked potentials; it was evident that the boy conserved some hearing and had thresholds suggestive of moderate hearing loss.     

Aided auditory thresholds in children with postmeningitic deafness

Postmeningitic hearing loss is often bilateral, profound, and rapid in onset. Children with postmeningitic hearing loss may be identified as potential candidates for cochlear implantation. The length of time required for a definitive trial with conventional amplification, which must precede a final decision regarding cochlear implant surgery, has been the subject of debate. Twenty-five children with bilateral severe-to-profound postmeningitic sensorineural hearing loss were fitted with appropriate amplification and results of serial-aided tests were analyzed to determine the degree of benefit and changes in aided thresholds over time. Twelve children gave aided responses that clearly indicated the presence of residual hearing and good aided benefit. Ten youngsters have responded consistently in the vibrotactile range when aided, thus receiving limited benefit from conventional amplification. Of particular interest are three children who initially exhibited limited or no auditory responses but who, after a period of 16 to 25 months, showed excellent benefit with binaural hearing aids. These children are all in total communication programs and have received good auditory training. Caution should be exercised when concluding that such a child does not benefit from amplification.     

Subjective deafness in case of peri-synaptic audiopathy. Isolated defects of the inner haircells?

BACKGROUND: Damage to or functional impair of the inner hair cells, synapsis or dendrites of the ganglion cells of the auditory nerve result in specific audiometric findings. Due to the normal function of the outer hair cells otoacoustic emissions can be registered, ABR and ECochG show at least elevated thresholds or are absent. PATIENTS: We demonstrate 5 cases with these audiological findings described in the literature as Auditory Neuropathy. RESULTS: All patients have profound to severe hearing loss with poor speech understanding under best aided conditions with conventional hearing aids. 3 patients, which were implanted with a cochlear implant have speech understanding but one prelingually adult, also implanted, has only sound identification. CONCLUSIONS: Hypoxia, carboplatin, ototoxicity and metabolic disorders are possible etiologies for damage to the inner hair cells or synapsis. The results will be discussed with reference to the localisation of the pathology and the definition as Auditory Neuropathy.     

Hearing threshold estimation in infants using auditory steady-state responses

Successful early intervention in children with permanent hearing loss requires assessment techniques that can accurately reflect the behavioral audiogram in infancy. This retrospective study compared auditory steady-state response (ASSR) findings from subjects tested in the first three months of life with subsequently obtained behavioral hearing levels. ASSR audiograms were established using amplitude and frequency modulated tones at octave frequencies (500 Hz to 4 kHz). Results obtained from 575 subjects including 285 with normal hearing, 271 with sensorineural hearing loss, and 19 with auditory neuropathy-type hearing loss are presented. ASSR and behavioral hearing thresholds for subjects in the normal and sensorineural groups were highly correlated, with Pearson r values exceeding 0.95 at each of the test frequencies. In contrast, ASSR thresholds in children with AN-type hearing loss did not accurately reflect the behavioral audiogram. Overall, the findings indicate that ASSR testing can offer useful insights into the hearing acuity of children tested in infancy.     

Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review

IntroductionCurrently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants.ObjectiveReview the available evidence in the literature to answer the following: “What is the performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants?”MethodsSystematic review of the literature through electronic database consultation, considering publications in the period 2002–2013.ResultsTwenty-two studies met the criteria and were included in the systematic review.ConclusionThe analyzed studies demonstrated that after cochlear implant surgery, individuals with auditory neuropathy spectrum disorder improved their performance of hearing skills and had similar performance to that of children with sensorineural hearing loss using cochlear implant.     

Auditory sensitivity in children using the auditory steady-state response

OBJECTIVE: To determine the effectiveness of auditory steady-state response (ASSR) as a measure of hearing sensitivity in young children suspect for significant hearing loss. DESIGN: Within-subject comparisons of click auditory brainstem response (ABR) thresholds and ASSR thresholds. SUBJECTS: The study population comprised 42 children suspect for hearing loss and subsequently referred for hearing assessment using electrophysiologic techniques. MAIN OUTCOME MEASURES: Electrophysiologic threshold responses for click ABR and ASSR stimuli (0.5, 1, 2, and 4 kHz) for right and left ears. RESULTS: Based on ABR and ASSR thresholds, 50% of the subjects demonstrated significant hearing loss in the severe to profound range. In some subjects, ASSRs were present at higher stimulus levels when click ABRs were absent. Significant correlations (P<.05) were found between high-frequency ASSR and click ABR thresholds for this study sample. For some subjects, ASSR findings suggested differences between ears that were not observable from the no-response click ABR results. CONCLUSIONS: Auditory steady-state response testing may provide additional information for children who demonstrate hearing levels in the severe to profound range. This information may be helpful when selecting the ear for cochlear implantation for a young hearing-impaired child. Multiple objective methods, such as ABR and ASSR testing, may be needed to determine accurate hearing sensitivity for young children being considered for sensory devices, and in particular, cochlear implants.     

Some comparisons between auditory brain stem response thresholds, latencies, and the pure-tone audiogram

Aspects of auditory brain stem responses (ABR) and pure-tone behavioral audiograms were compared in patients with cochlear hearing loss. Click-evoked ABR thresholds appeared to be related most closely to the audiometric thresholds at 2000 and 4000 Hz, with relatively poor agreement at either 1000 or 8000 Hz. These results were related to the amplitude spectrum of the eliciting stimulus. The slope of the wave V latency-intensity function appeared to be related to the configuration of the hearing loss. Patients with high-frequency sensorineural losses had steeper slopes than normal subjects, whereas patients with flat sensorineural losses had shallower slopes. These results were related to the principle that response latency is determined by the cochlear region that predominates the ABR for different stimulus intensities.     

What to do with the other ear after cochlear implantation

Unilateral cochlear implantation has become a widely accepted surgical intervention for both deaf children and adults. It is a reliable and effective method to rehabilitate profound deafness. Recently the benefits of the use of a contralateral hearing aid (bimodal stimulation) with a cochlear implant became clear. Bilateral cochlear implantation benefits bilateral input into the auditory system for adults and children. To provide the binaural advantages experienced by normal hearing subjects bilateral cochlear implantation or bimodal stimulation is probably indicated. Whether to choose between both possibilities depends on many factors. Cortical auditory evoked potential (CAEP) measurements can be an important tool to decide bilateral implantation in young children. Enough residual hearing in the non-implanted ear might benefit from bimodal stimulation. New protocols are needed for the audiological management for recipients of cochlear implants.