Cochlear implantation in a human immunodeficiency virus-infected patient

OBJECTIVES/HYPOTHESIS: Patients infected with HIV have an increased risk of developing sensorineural hearing loss (SNHL), yet pathogenesis of SNHL in HIV infection is still poorly understood. In subjects affected by bilateral profound or total SNHL, cochlear implantation may be the only possibility to restore a hearing level that allows them to have an acceptable quality of life. STUDY DESIGN: Case report. METHODS: A retrospective chart review of a HIV type 1-seropositive profoundly deafened patient who underwent cochlear implantation. RESULTS: To date, with a follow-up of 4 years, the patient has not experienced any complication and has regained useful open-set speech perception. CONCLUSIONS: Cochlear impairment with preserved auditory pathways can be responsible for profound SNHL in HIV-infected patients. Cochlear implantation can restore a social hearing in these patients, dramatically improving their quality of life. The surgical procedure can be safely performed when keeping in mind that the general condition of the patient is the decisive factor for or against surgery.     

Chronic suppurative otitis media following paediatric cochlear implantation

The objective of this study was to report and discuss the management of chronic suppurative otitis media (CSOM) following cochlear implantation in children. The study was a retrospective review of 650 patients receiving an implant at two paediatric tertiary referral centres for cochlear implantation. Nine patients were identified who developed CSOM following cochlear implantation (incidence 1.38%). The mean time interval between implantation and symptom development was 3.66 years (range 2-8 years) and the mean time interval between implantation and CSOM surgery was 5.02 years (range 2.2-8 years). All patients presented with otorrhoea and/or abscess formation over the implant site. Two patients underwent a modified radical mastoidectomy and seven underwent a combined approach tympanoplasty, three of whom required posterior canal wall reconstruction with cortical bone and one with cartilage. In four cases it was possible to remove the cholesteatoma without removing the implant. All but two patients were fitted with a contralateral implant. In the explanted ears the cochlear implant electrode was cut at the cochleostomy site, which was then covered with muscle. Chronic suppurative otitis media following cochlear implantation may occur either as a result of a posterior canal wall defect related to surgery or possibly de novo. Attempts should be made to save the implant, but explantation with reimplantation of the contralateral ear may be the only option. In these cases the intracochlear part of the electrode array should be left in situ to facilitate possible future reimplantation. Surgical options for management of CSOM should be individualized and may include both canal-wall up and canal-wall down techniques. To reduce the incidence of CSOM following implantation the authors recommend: (1) prompt treatment and careful follow-up of patients with a history of otitis media with effusion, (2) avoidance of excessive thinning of the posterior canal wall during mastoidectomy and (3) reconstruction of any accidental trauma to the annulus or posterior canal wall during posterior tympanotomy.     

Chronic suppurative otitis media following paediatric cochlear implantation

Abstract

The objective of this study was to report and discuss the management of chronic suppurative otitis media (CSOM) following cochlear implantation in children. The study was a retrospective review of 650 patients receiving an implant at two paediatric tertiary referral centres for cochlear implantation. Nine patients were identified who developed CSOM following cochlear implantation (incidence 1.38%). The mean time interval between implantation and symptom development was 3.66 years (range 2–8 years) and the mean time interval between implantation and CSOM surgery was 5.02 years (range 2.2–8 years). All patients presented with otorrhoea and/or abscess formation over the implant site. Two patients underwent a modified radical mastoidectomy and seven underwent a combined approach tympanoplasty, three of whom required posterior canal wall reconstruction with cortical bone and one with cartilage. In four cases it was possible to remove the cholesteatoma without removing the implant. All but two patients were fitted with a contralateral implant. In the explanted ears the cochlear implant electrode was cut at the cochleostomy site, which was then covered with muscle. Chronic suppurative otitis media following cochlear implantation may occur either as a result of a posterior canal wall defect related to surgery or possibly de novo. Attempts should be made to save the implant, but explantation with reimplantation of the contralateral ear may be the only option. In these cases the intracochlear part of the electrode array should be left in situ to facilitate possible future reimplantation. Surgical options for management of CSOM should be individualized and may include both canal-wall up and canal-wall down techniques. To reduce the incidence of CSOM following implantation the authors recommend: (1) prompt treatment and careful follow-up of patients with a history of otitis media with effusion, (2) avoidance of excessive thinning of the posterior canal wall during mastoidectomy and (3) reconstruction of any accidental trauma to the annulus or posterior canal wall during posterior tympanotomy. Copyright © 2006 John Wiley & Sons, Ltd.     

Recent epidemiology of pediatric cochlear implantation in the United States: disparity among children of different ethnicity and socioeconomic status

OBJECTIVES/HYPOTHESIS: Congenital severe to profound sensorineural hearing loss (SNHL) is found in higher proportions of children with minority and/or lower socioeconomic status (SES). Cochlear implants were approved by the U.S. Food and Drug Administration for use in children with bilateral severe to profound SNHL in 1990. The objectives of the study were as follows: 1) to study the epidemiology of pediatric cochlear implantation, assessing whether cochlear implant technology is provided to children with severe to profound SNHL in proportion to their racial/ethnic or SES, and 2) to compare data provided by a national health care database with data provided by cochlear implant manufacturers. STUDY DESIGN: Patients aged 0 to 18 years who underwent cochlear implantation in 1997 using a cross-sectional study design. METHODS: Analyses were made of pediatric cochlear implant patients, using data from the 1997 Health Care and Utilization Project/Kids' Inpatient Database. Relative rates of implantation compared with rates of severe to profound SNHL were calculated using national estimates generated from census and Galludet Research Institution data. Logistic regression analysis was carried out to compare implanted children of different racial/ethnic backgrounds. A surrogate measure of socioeconomic status was used based on the median household income of the patient's home ZIP code. Information was also obtained from the two companies producing U.S. Food and Drug Administration-approved cochlear implants in 1997 and used to determine whether the data obtained from the Health Care and Utilization Project/Kids' Inpatient Database were representative of the national cohort of implanted children. RESULTS: The Health Care and Utilization Project/Kids' Inpatient Database identified 124 children who underwent cochlear implant surgery in 1997. White and Asian children were implanted at higher rates than Hispanic and black children. Furthermore, white and Asian children received implants at greater rates than would be expected based on prevalence of severe to profound SNHL. The relative rate (RR) of implantation, defined as the proportion of children who received cochlear implants divided by the proportion of children with severe to profound SNHL (in each race/ethnicity group compared with the same ratio in white children), was similar in white (RR = 1.00) and Asian (RR = 0.93) children but markedly different in Hispanic (RR = 0.28) and black (RR = 0.10) children. Comparison of SES information from the Health Care and Utilization Project/Kids' Inpatient Database population with the manufacturers' database suggested that the Health Care and Utilization Project/Kids' Inpatient Database is representative of all implanted children in the United States. Both sources of information suggested that children receiving cochlear implants in the United States in 1997 resided in above-average SES areas. CONCLUSION: White and Asian children with severe to profound SNHL had higher proportionate rates of cochlear implantation than black and Hispanic children in 1997. Implanted children were more likely to live in areas (represented by ZIP codes) with higher median incomes. Although there was a disparity in rate of cochlear implantation based on race/ethnicity and surrogate measures of SES, these data did not allow the authors to determine the causes for these differences.     

Cochlear implantation in patients with acute or chronic middle ear infectious disease: a review of the literature

Although in the past cochlear implantation was considered contraindicated in patients with acute (AOM) or chronic suppurative otitis media (CSOM) with or without middle ear cholesteatoma, recent developments now make it possible to perform cochlear implantation in these patients. Various procedures are available to make the ears of patients with either acute or CSOM suitable for cochlear implantation and to minimize the risk of recurrence of the disease, device extrusion, or intracranial complications. This review discusses these different approaches for optimizing implant survival and preventing complications related to otitis media. We performed a comprehensive literature search of the MEDLINE database. Cochlear implantation can be safely performed in patients with otitis media. However, the infection should be adequately controlled well before implantation, and all measures should be taken to prevent recurrent disease. Therefore, the procedure used should be tailored to individual clinical findings. This article provides a guideline to optimize the course of action in patients suffering from AOM, CSOM or their sequelae in preparation for cochlear implantation.     

Cochlear implantation following treatment for medulloblastoma

Objectives/Hypothesis:

Medulloblastoma is the most common pediatric malignant tumor of the central nervous system in children. Treatment includes surgical excision, external beam radiation, and multiagent chemotherapy. Otologic sequelae are common and may result from radiation and/or chemotherapy. Profound sensorineural hearing loss (SNHL) is a known complication of neuro‐oncologic treatment and may render these patients eligible for cochlear implantation (CI). Issues of CI in this population, including diagnosis, treatment of preoperative middle ear disease, operative and postoperative course, performance data, and long‐term tumor surveillance are highlighted and reviewed.

Study Design:

Retrospective chart review.

Methods:

Three patients treated for pediatric medulloblastoma with surgical resection, postoperative hyperfractioned craniospinal radiotherapy, and multiagent adjuvant chemotherapy who underwent cochlear implantation were identified. Details of neuro‐oncologic treatment and associated otologic complications are presented and analyzed. Primary outcome assessment includes treatment of middle ear pathology, perioperative cochlear implant course, and postimplantation performance data.

Results:

Each patient required surgical treatment of chronic ear disease 4 to 16 years after chemoradiation. All progressed to profound SNHL and were implanted 8 to 17 years post–neuro‐oncologic treatment. There were no intraoperative complications, and full insertion of the cochlear implant electrode array was achieved in each patient. One patient developed postoperative wound dehiscence requiring operative closure. Postimplantation performance data support significant benefit in all patients.

Conclusions:

Patients treated for pediatric medulloblastoma develop otologic sequelae, including profound SNHL, and may require cochlear implantation. Successful management of middle ear and mastoid pathology involves consideration of potential future cochlear implantation. Postoperative performance data supports cochlear implantation in this population. Laryngoscope, 2010     

Cochlear implants in systemic autoimmune vasculitis syndromes

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Be?het's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.     

Contemporary surgical issues in paediatric cochlear implantation

Abstract

Objective: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. Design: Narrative literature review. Results: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. Conclusion: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.     

Cochlear Implants in Systemic Autoimmune Vasculitis Syndromes

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Beçhet's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.     

慢性化脓性中耳炎与感音神经性聋

目的：观察慢性化脓性中耳炎（CSOM）对感音神经性聋（SNHL）的影响。方法：测量135例（168耳）CSOM患者的骨导听阈,并以66例单侧患者的健耳为对照,比较不同类型、不同病程的CSOM的骨导听阈。结果：CSOM各组骨导听阈均值明显高于对照组,且与中耳炎的类型和病程相关。结论：CSOM可引起SNHL,且中耳病变越重,病程越长,听力下降越明显     

Cochlear implants: indications in groups of patients with borderline indications. A review

Cochlear implants (CI) represent the current treatment for patients affected by profound sensorineural hearing loss (SNHL). Initially only deaf adult patients were considered to be candidates for a CI; however, the development of technology and matured experience have expanded the indications for cochlear implantation. Today, CIs are implanted in adults and children and broader indications are followed. There are, however, a number of patients who do not completely fulfill the current indications and who are potential candidates for CI. The duration of deafness and residual hearing represent prognostic indicators for CI performance; however, the candidacy of children with residual hearing and prelingually deafened adults are still under debate. Anatomical variants such as cochlear ossification, cochlear malformation and chronic otitis media represented and still represent for some surgeons a contraindication to CI. The otological experience of CI surgeons and the advent of auditory brainstem implants have changed the approach to these patients, who may still benefit from hearing rehabilitation. This paper briefly analyses and reviews the results obtained in these groups of patients, who were not, at least initially, considered to be candidates for cochlear implantation.     

前庭水管扩大综合征患者的人工耳蜗植入术

目的 评价前庭水管扩大综合征患者人工耳蜗植入术的安全性和可行性。方法 1995年5月1日～2002年6月1日因双耳重-极重度感音神经性聋在北京协和医院接受人工耳蜗植入术的患者312例中诊断为双耳前庭水管扩大者10例(3．2％)，其中语前聋7例，语后聋3例。回顾性分析这10例患者的临床资料。结果 10例患者人工耳蜗电极植入顺利，8例耳蜗底回开窗时发生轻度井喷，迅速用颞肌筋膜牢固封闭圆窗制止井喷。全部患者术后未出现脑脊液漏、颅内感染、面瘫、中耳炎等并发症。开机6个月时8例患者具有开放性言语识别力，已进入普通学校(幼儿园、小学、大学)就读。另2例语前聋的幼儿视觉强化测听听阈达40dB HL，与其他无内耳畸形的全聋儿童术后效果差异无显著性。全聋前语言能力较好的5例患者术后语言能力明显好于语前聋的患者，语言交流基本听不出聋人特有的语音特征。另5例患者语言均有不同程度的进步。结论 尽管前庭水管扩大患者在人工耳蜗植入术中可能出现井喷，但术后未出现并发症且听力-言语康复效果好，因此重-极重度聋的前庭水管扩大综合征患者行人工耳蜗植入术是安全可行的。     

影响人工耳蜗植入效果因素分析

人工耳蜗植入（cochlear implant,CI）可帮助所有年龄段的感音神经性耳聋（sensorineural hearing loss.SNHL)患者,尤其重度、极重度感音神经性聋患者重获声音信息,可以改善患者的言语感知和生活质量,更好地促进身心健康,因此探究影响耳蜗植入效果的因素是临床的一个重要研究方向。本文通过文献回顾,主要介绍影响人工耳蜗植入术效果的因素。     

1例耳蜗前庭神经畸形患者耳蜗植入术前的功能性磁共振评估并文献复习

目的:探讨功能性磁共振(fMRI)评价耳蜗前庭神经畸形患者是否有人工耳蜗植入指征的可能性。方法:对1例15岁双侧耳聋患者,在人工耳蜗植入前进行听力学、影像学检查及fMRI综合评估。结果:纯音测听和听觉脑干反应显示患者为双侧极重度感音神经性聋,瞬态诱发性耳声发射双侧均未引出。颞骨薄层CT显示内耳畸形(不全分隔Ⅰ型),MRI斜矢状位重建显示耳蜗前庭神经畸形。fMRI显示左耳给声,1000Hz100dBHL,右侧听皮层被激活;右耳给声,双侧听皮层均未见激活。左侧人工耳蜗植入术后1个月开机,患者对声音有反应。结论:结合听力学和影像学检查,fMRI可评估人工耳蜗植入候选者,尤其是在影像学检查显示患者耳蜗前庭神经发育畸形时,更是一种有效的评估方法。     

Cochlear nerve size evaluation in children with sensorineural hearing loss by high-resolution magnetic resonance imaging

PURPOSE: To determine differences in size of cochlear nerves among subjects with deafness due to connexin 26 (Cx26) mutations, subjects with deafness of unknown origin, and normal hearing subjects by sagittal high-resolution magnetic resonance (HRMR) imaging of the temporal bone. MATERIALS AND METHODS: Cross-sectional and surface areas and volumetric measurements of the cochlear nerve and modiolus were made on HRMR images of the internal auditory canal (IAC) and inner ear in the 3 groups of children (groups 1, 2, and 3). Three-way comparisons of in vivo cochlear nerve measurements on HRMR imaging were made among 17 children with sensorineural hearing loss (SNHL) and no obvious etiology for the hearing loss (group 1), 7 children with profound SNHL due to a Cx26 mutation (group 2), and 10 normal hearing children (group 3). RESULTS: Children with profound SNHL of unknown cause and children with profound SNHL due to a connexin mutation displayed hypoplastic cochlear nerves as compared with normal controls. HRMR imaging of the temporal bone was accurately delineated potential problems with cochlear nerves in 2 of 17 instances where high-resolution computed tomography did not do so. CONCLUSIONS: Accurate and specific measurements of the cochlear nerve and related structures is possible on HRMR imaging of the temporal bone. The size of the cochlear nerve is mildly hypoplastic in children with profound SNHL of unknown causes or children with a deafness-causing Cx26 mutation. HRMR imaging is superior to high-resolution computed tomography in the investigation of profound SNHL in children.     

Presence of cytomegalovirus in the perilymphatic fluid of patients with profound sensorineural hearing loss caused by congenital cytomegalovirus infection

Conclusion: Not all patients diagnosed with congenital infection using umbilical cord assay were found to be positive for CMV-DNA by perilymphatic fluid assay. In addition, a CMV-DNA-positive result was observed in one patient who had not been diagnosed with congenital infection. Sampling of perilymphatic fluid from a large population of patients with congenital SNHL caused by congenital CMV infection or of unknown etiology is required to determine the prevalence of CMV-related profound HL. Objectives: Sensorineural hearing loss (SNHL) is one of the most frequent manifestations in patients with congenital cytomegalovirus (CMV) infection. Using dried umbilical cord, a PCR-based assay was recently developed for the retrospective detection of congenital CMV infection. This study analyzed the presence of CMV in the perilymphatic fluid and evaluated differences in the effect of cochlear implantation between CMV-positive and -negative groups. Method: Perilymphatic fluid was collected from each patient at the time of cochlear implantation and analyzed for the presence of CMV using a PCR method. Results: The perilymphatic fluid in two of the five patients suffering from congenital CMV infection and in one of the 17 patients without congenital CMV infection was found to be positive for CMV.     

前庭水管扩大综合征患者的人工耳蜗植入术

目的　评价前庭水管扩大综合征患者人工耳蜗植入术的安全性和可行性。方法　 1995年 5月 1日～ 2 0 0 2年 6月 1日因双耳重 极重度感音神经性聋在北京协和医院接受人工耳蜗植入术的患者 312例中诊断为双耳前庭水管扩大者 10例 (3 2 % ) ,其中语前聋 7例 ,语后聋 3例。回顾性分析这 10例患者的临床资料。结果　 10例患者人工耳蜗电极植入顺利 ,8例耳蜗底回开窗时发生轻度井喷 ,迅速用颞肌筋膜牢固封闭圆窗制止井喷。全部患者术后未出现脑脊液漏、颅内感染、面瘫、中耳炎等并发症。开机 6个月时 8例患者具有开放性言语识别力 ,已进入普通学校 (幼儿园、小学、大学 )就读。另 2例语前聋的幼儿视觉强化测听听阈达 4 0dBHL ,与其他无内耳畸形的全聋儿童术后效果差异无显著性。全聋前语言能力较好的 5例患者术后语言能力明显好于语前聋的患者 ,语言交流基本听不出聋人特有的语音特征。另 5例患者语言均有不同程度的进步。结论　尽管前庭水管扩大患者在人工耳蜗植入术中可能出现井喷 ,但术后未出现并发症且听力 言语康复效果好 ,因此重 极重度聋的前庭水管扩大综合征患者行人工耳蜗植入术是安全可行的     

Inner ear anomalies in cochlear implantees: importance of radiologic measurements in the classification.

OBJECTIVE: To classify the inner ear anomalies of cochlear implantees with profound sensorineural hearing loss. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: For the normative data of the inner ear structures, the temporal bone computed tomography of 60 patients (120 ears) with normal bone-conduction threshold (< 15 dB) were used, and the data were applied to 570 ears of 285 cochlear implantees. INTERVENTIONS: Predesignated inner ear structures were measured in temporal bone computed tomography images from the normal and cochlear implantation groups using a computer-based caliper that formed part of a picture archiving and communication system. MAIN OUTCOME MEASURES: The inner ear anomalies were defined when the structures presented visually obvious malformations or the measurements deviated 2 standard deviations from the means in the normative data. RESULTS: The application of normative data to 570 profound SNHL ears resulted in the identification of 293 individual anomalies in 127 anomalous ears. An enlarged vestibular aqueduct was the most common individual anomaly (49 cases), followed by vestibular enlargement (38 cases) and other semicircular canal dysplasia (37 cases). When the individual anomalies were reaccounted according to the more prominent anomaly where multiple anomalies were present in each ear, incomplete partition type II was the most common (34 ears), followed by cochlear hypoplasia (22 ears) and incomplete partition type I (20 ears). CONCLUSION: We suggested a measurement technique for the inner ear structures using computed tomography and derived normative measurements helpful for diagnosing inner ear anomalies. Using these normative data, we classified the inner ear anomalies of profound SNHL ears in cochlear implantees.     

Cochlear implantation in chronic demyelinating inflammatory polyneuropathy†

Objective: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation.

Methods: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation.

Results: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20?dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss.

Conclusions: Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.     

Cochlear implantation in children with auditory neuropathy: outcomes and rationale

CONCLUSION: Children with auditory neuropathy (AN) obtain considerable benefit from cochlear implantation. Their performance outcomes are as good as those of implanted children with sensorineural hearing loss (SNHL). The neural status of children with AN would be comparable to that of children with SNHL and be suitable for cochlear implantation. OBJECTIVE: The purpose of this study was to evaluate the outcomes of cochlear implantation in children with AN and to assess the status of auditory nerve in these patients. SUBJECTS AND METHODS: Nine children with AN who underwent cochlear implantation were included. Their performance outcomes which were measured by Categories of Auditory Performance (CAP), Monosyllabic Word (MW) test for phonemes, and Common Phrases test were compared with those of matched implanted children with SNHL. To assess the status of auditory nerve in children with AN, the slopes of amplitude growth functions of electrically evoked compound action potentials (ECAPs) in implanted children with AN were compared to those of implanted children with SNHL. RESULTS: There were no statistically significant differences between two groups in performance outcomes (CAP, p=0.1200; MW test, p=0.5768; Common Phrases test, p=0.3337). No significant difference was found in the slopes of ECAP amplitude growth functions (p=0.970) between two groups, which shows that spiral ganglion cell populations may be comparable in these two groups.