Pediatric Cochlear Implant Candidates Research Articles

The effect of ventilation tube insertion in pediatric cochlear implantation candidates with otitis media with effusion on postoperative complications

ObjectiveOtitis media with effusion (OME) is a common finding in pediatric cochlear implant(CI) candidates and may be managed by inserting ventilation tubes. This study aimed to compare postoperative complication rates in children who underwent CI without and with OME, including patients who were treated without and with ventilation tube insertion. MethodsA population-based retrospective cohort study was conducted, including all CI patients, under ten years of age, at our institution, between 2007 and 2020. The study's population was divided into three groups based on their middle ear status at CI: 1) OME previously treated with VT, 2) untreated OME, and 3) normal-aerated ears. Postoperative complications of the groups were reviewed and served as our primary outcome measure. ResultsOf the 257 implanted ears included, 53, 42, and 162 ears belonged to the VT-treated OME, untreated OME, and aerated groups, respectively. Acute mastoiditis (AM) rate was significantly higher in the OME group compared to the aerated groups (9.5 % vs. 2.5 %, p = 0.0134) and in the VT-treated compared to the untreated OME groups (15.1 % vs. 2.3 %, p = 0.0356). Similarly, the rate of developing chronic suppurative otitis media without cholesteatoma (CSOMWC) was significantly higher in the OME compared to the aerated groups (12.6 % vs. 2.5 %, p = 0.0011) and in the VT-treated compared to the untreated OME groups (18.8 % vs. 4.7 %, p = 0.0366). Other complications rated were very low and similar between the groups. No other statistical difference was found between the groups. ConclusionVT insertion in pediatric CI candidates with OME increased postoperative AM and CSOMWC. We believe that, at least in our population, VT introduction prior to CI, for OME, surgery should be avoided.

Diffusion Tensor Imaging of Auditory Pathway: A Comparison of Pediatric Cochlear Implant Candidates and Healthy Cases.

We aimed to investigate the changes that may occur in the auditory neural network in pediatric congenital hearing loss cases. Fifty-four cochlear implant candidates and 47 normal-hearing controls were included in this retrospective study. Fractional anisotropy, radial diffusivity, and apparent diffusion coefficient maps were generated. We placed region of interest on the cochlear nucleus, superior olivary nucleus, lateral lemniscus, medial geniculate body, auditory radiation, Heschl's gyrus, inferior fronto-occipital fasciculus, superior longitudinal fascicle, and corpus callosum splenium. The area of the cochlear nerve was measured. Diffusion tensor imaging metrics, children's ages, and cochlear nerve area were compared. Apparent diffusion coefficient and radial diffusivity values of patients were higher than the control group in all places except the radial diffusivity values of medial geniculate body. The fractional anisotropy values of the patients in lateral lemniscus, auditory radiation, Heschl's gyrus, inferior fronto-occipital fasciculus, superior longitudinal fascicle, and corpus callosum splenium were lower than the control group. There is a positive correlation between fractional anisotropy and age in both patient and control groups for all locations. The cochlear nerve area is lower in patients (0.88 ± 0.29) than in the control group (1.18 ± 0.14) (P = .000). The cochlear nerve area has a positive correlation with age in the patient group (P = .000) but has not in the control group. The cochlear nerve area positively correlates with fractional anisotropy values of all locations except fractional anisotropy values of medial geniculate body. The alterations of diffusion tensor imaging metrics on the auditory pathway reflect the microstructural changes of white matter tracts.

Olfactory bulb volume in cochlear implant candidates as compared to age-matched control subjects: A potential marker of cognitive function for auditory information processing.

To evaluate magnetic resonance imaging (MRI)-based olfactory bulb (OB) volumes in cochlear implant (CI) candidates with sensorineural hearing loss as compared to age-matched control subjects with normal hearing. A total of 31 pediatric CI candidates (mean±SD age: 7.0±2.5 years, 51.6% were boys) with sensorineural hearing loss and 35 age-matched control subjects (mean±SD age: 7.1±2.5 years, 54.3% were boys) with normal hearing were included in this study. Data on demographic characteristics (age, gender) and right and left OB volume (mm3) on MRI using planimetric contouring method were recorded in patients and control groups. Median (min-max) values for right OB volume (80(50-120) vs. 90(50-160) mm3, p=0.006) and left OB volume (70(50-120) vs. 90(50-170) mm3, p=0.007) were significantly lower in CI candidates vs. controls, regardless of the gender and age. No significant difference was noted between right and left OB volume in CI candidate and control groups. Hearing loss subgroups of CI candidates including hereditary familial (n=8), hereditary non-familial (n=14) and mixed syndromic (9) subgroups were also similar in terms of patient demographics and OB volumes. There was a tendency for having lower left OB volume (60(50-120) vs. 80(60-110) mm3) in girls vs. boys in the CI candidate group, along with a tendency for lower left and right OB volume in candidates vs. controls, particularly at age 11 (median 120 vs. 80mm3 and 120 vs. 60mm3, respectively). No significant correlation of age was noted with right and left OB volume overall and in the study groups. In conclusion, our findings revealed lower left and right OB volumes in CI candidates compared to control subjects, regardless of age and gender, indicating the presence of baseline olfactory dysfunction in patients with hearing loss planned to undergo CI. Accordingly, MRI-based measurement of OB volume in the pre-surgical workup of CI candidates may serve as a marker of cognitive function enabling auditory information processing that may also correlate with post-operative CI outcomes.

Intra- and Interrater Reliability of CT- versus MRI-Based Cochlear Duct Length Measurement in Pediatric Cochlear Implant Candidates and Its Impact on Personalized Electrode Array Selection.

Radiological high-resolution computed tomography-based evaluation of cochlear implant candidates' cochlear duct length (CDL) has become the method of choice for electrode array selection. The aim of the present study was to evaluate if MRI-based data match CT-based data and if this impacts on electrode array choice. Participants were 39 children. CDL, length at two turns, diameters, and height of the cochlea were determined via CT and MRI by three raters using tablet-based otosurgical planning software. Personalized electrode array length, angular insertion depth (AID), intra- and interrater differences, and reliability were calculated. Mean intrarater difference of CT- versus MRI-based CDL was 0.528 ± 0.483 mm without significant differences. Individual length at two turns differed between 28.0 mm and 36.6 mm. Intrarater reliability between CT versus MRI measurements was high (intra-class correlation coefficient (ICC): 0.929-0.938). Selection of the optimal electrode array based on CT and MRI matched in 90.1% of cases. Mean AID was 629.5° based on the CT and 634.6° based on the MRI; this is not a significant difference. ICC of the mean interrater reliability was 0.887 for the CT-based evaluation and 0.82 for the MRI-based evaluation. MRI-based CDL measurement shows a low intrarater difference and a high interrater reliability and is therefore suitable for personalized electrode array selection.

Developmental performance among pediatric candidates for cochlear implantation

Background Developmental evaluation of deaf children before cochlear implantation (CI) has not been given enough attention. Objectives This study was designed to evaluate the comprehensive developmental performance of deaf children who are CI candidates. Materials and methods The medical records of pediatric candidates for CI were reviewed. Five hundred children (287 boys; median age: 21.00 months; range: 6–72 months) with a diagnosis of severe-to-profound hearing loss were included. Preoperative developmental evaluation, including gross motor, fine motor, adaptability, language and social skill were retrieved. Comprehensive developmental performances including verbal and nonverbal skill were assessed. Multivariate linear regression analysis was used to analyze the related developmental factors. Results Compared with normal developmental metrics, deaf children had developmental delay (p < .001), which occurred in not only the verbal but also nonverbal skill (all p < .05). Of the 500 deaf children, 50 (10%) had normal performance; the majority (51.6%) had mild neurological dysfunction. Of all the sub-developments, language developed worst (normal rate: 4.2%) and gross motor developed best (normal rate: 42%). Age of intervention was a risk factor for the developmental level of deaf children (β = −0.340, p < .05). Conclusions Pediatric candidates for CI had both verbal and nonverbal developmental delay. Age of intervention was a risk factor for the developmental level. Significance Comprehensive developmental evaluation of deaf children before cochlear implantation (CI) should be paid enough attention. Early intervention for improving hearing was of significance.

Bilateral Cochlear Implants or Bimodal Hearing for Children with Bilateral Sensorineural Hearing Loss

This review describes speech perception and language outcomes for children using bimodal hearing (cochlear implant (CI) plus contralateral hearing aid) as compared to children with bilateral CIs and contrasts said findings with the adult literature. There is a lack of clinical evidence driving recommendations for bimodal versus bilateral CI candidacy and as such, clinicians are often unsure about when to recommend a second CI for children with residual acoustic hearing. Thus the goal of this review is to identify scientific information that may influence clinical decision making for pediatric CI candidates with residual acoustic hearing. Bilateral CIs are considered standard of care for children with bilateral severe-to-profound sensorineural hearing loss. For children with aidable acoustic hearing-even in just the low frequencies-an early period of bimodal stimulation has been associated with significantly better speech perception, vocabulary, and language development. HA audibility, however, is generally poorer than that offered by a CI resulting in interaural asymmetry in speech perception, head shadow, as well as brainstem and cortical activity and development. Thus there is a need to optimize "two-eared" hearing while maximizing a child's potential with respect to hearing, speech, and language while ensuring that we limit asymmetrically driven auditory neuroplasticity. A recent large study of bimodal and bilateral CI users suggested that a period of bimodal stimulation was only beneficial for children with a better-ear pure tone average (PTA) ≤ 73 dB HL. This 73-dB-HL cutoff applied even to children who ultimately received bilateral CIs. Though we do not yet have definitive guidelines for determining bimodal versus bilateral CI candidacy, there is increasing evidence that 1) bilateral CIs yield superior outcomes for children with bilateral severe-to-profound hearing loss and, 2) an early period of bimodal stimulation is beneficial for speech perception and language development, but only for children with better-ear PTA ≤ 73 dB HL. For children with residual acoustic hearing, even in just the low-frequency range, rapid sequential bilateral cochlear implantation following a trial period with bimodal stimulation will yield best outcomes for auditory, language, and academic development. Of course, there is also an increasing prevalence of cochlear implantation with acoustic hearing preservation allowing for combined electric and acoustic stimulation even following bilateral implantation.

Prelinguistic Vocal Development in Children With Cochlear Implants: A Systematic Review.

This systematic review is designed to (a) describe measures used to quantify vocal development in pediatric cochlear implant (CI) users, (b) synthesize the evidence on prelinguistic vocal development in young children before and after cochlear implantation, and (c) analyze the application of the current evidence for evaluating change in vocal development before and after cochlear implantation for young children. Investigations of prelinguistic vocal development after cochlear implantation are only beginning to uncover the expected course of prelinguistic vocal development in children with CIs and what factors influence that course, which varies substantially across pediatric CI users. A deeper understanding of prelinguistic vocal development will improve professionals' abilities to determine whether a child with a CI is exhibiting sufficient progress soon after implantation and to adjust intervention as needed. We systematically searched PubMed, ProQuest, and CINAHL databases for primary reports of children who received a CI before 5 years 0 months of age that included at least one measure of nonword, nonvegetative vocalizations. We also completed supplementary searches. Of the 1916 identified records, 59 met inclusion criteria. The included records included 1125 total participants, which came from 36 unique samples. Records included a median of 8 participants and rarely included children with disabilities other than hearing loss. Nearly all of the records met criteria for level 3 for quality of evidence on a scale of 1 (highest) to 4 (lowest). Records utilized a wide variety of vocalization measures but often incorporated features related to canonical babbling. The limited evidence from pediatric CI candidates before implantation suggests that they are likely to exhibit deficits in canonical syllables, a critical vocal development skill, and phonetic inventory size. Following cochlear implantation, multiple studies report similar patterns of growth, but faster rates producing canonical syllables in children with CIs than peers with comparable durations of robust hearing. However, caution is warranted because these demonstrated vocal development skills still occur at older chronological ages for children with CIs than chronological age peers with typical hearing. Despite including a relatively large number of records, the evidence in this review regarding changes in vocal development before and after cochlear implantation in young children remains limited. A deeper understanding of when prelinguistic skills are expected to develop, factors that explain deviation from that course, and the long-term impacts of variations in vocal prelinguistic development is needed. The diverse and dynamic nature of the relatively small population of pediatric CI users as well as relatively new vocal development measures present challenges for documenting and predicting vocal development in pediatric CI users before and after cochlear implantation. Synthesizing results across multiple institutions and completing rigorous studies with theoretically motivated, falsifiable research questions will address a number of challenges for understanding prelinguistic vocal development in children with CIs and its relations with other current and future skills. Clinical implications include the need to measure prelinguistic vocalizations regularly and systematically to inform intervention planning.

The feasibility, validity and reliability of a child friendly vestibular assessment in infants and children candidates to cochlear implant

ObjectivesThe pediatric vestibular assessment has developed significantly in the past two decades, especially in terms of assessment of cochlear implant (CI) candidates. Different methods and test protocols have been applied, however without a general consensus. We present here the feasibility, validity and reliability of a child friendly vestibular testing in use at our department for the assessment of pediatric CI candidates. MethodsThe test battery consisted of head impulse test (HIT), video head impulse test (vHIT), cervical evoked myogenic potentials (cVEMP) and mini ice water caloric test (mIWC), all adapted from previous methods, mentioned in literature. We defined specific criteria for test feasibility, for test validity and test positivity (i.e. indicating vestibular insufficiency). The reliability of the whole protocol was assessed with test agreement analysis. ResultsComplete data from 35 children, all CI candidates, age ranging 4–79 months (67% under 2 years) and recruited over two years, were obtained. All but one child could complete at least one test with valid responses bilaterally, with the best compliance for HIT (97,1%) and least for cVEMP (68,6%). Feasibility did not appear to be affected by age or hearing loss etiology. Among the valid responses there was a substantial test agreement between HIT and vHIT, moderate agreement between vHIT/HIT and mIWC and no apparent agreement between the canal tests and cVEMP. Simple clinical guidelines were introduced to solve the tests’ disagreement and to improve the protocol reliability: a) a pathological response had to be confirmed in at least two different canal tests and in at least three cVEMP trials; b) a canal/otolith disagreement was interpreted as a partial vestibular loss to be opposed to a complete vestibular insufficiency. ConclusionsThe search for vestibular insufficiency in infants and young children can be attained with an opportunely adapted vestibular assessment, such the test battery proposed here. That assessment resulted easy to conduct and to interpret in a representative sample of CI candidates in preschool age, the most of whom were younger than 2 years. This method appears to particularly suit the demands of a vestibular assessment in young children CI candidates.

Imaging of Pediatric Hearing Loss.

Temporal bone high-resolution computed tomography (HRCT) and magnetic resonance (MR) imaging are valuable tools in the evaluation of pediatric hearing loss. Computed tomography is important in the evaluation of pediatric conductive hearing loss and is the imaging modality of choice for evaluation of osseous abnormalities. MR imaging is the modality of choice for evaluation of sensorineural hearing loss. A broad spectrum of imaging findings can be seen with hearing loss in children. HRCT and MR imaging provide complementary information and are often used in conjunction in the preoperative evaluation of pediatric candidates for cochlear implantation.

Evaluation of pediatric cochlear implant care throughout Europe: Is European pediatric cochlear implant care performed according to guidelines?

Objectives: International guidelines indicate that children with profound hearing loss should receive a cochlear implant (CI) soon after diagnosis in order to optimize speech and language rehabilitation. Although prompt rehabilitation is encouraged by current guidelines, delays in cochlear implantation are still present. This study investigated whether European countries establish timely pediatric CI care based on epidemiological, commercial, and clinical data.Methods: An estimation of the number of pediatric CI candidates in European countries was performed and compared to epidemiological (Euro-CIU), commercial (Cochlear®), and clinical (institutional) age-at-implantation data. The ages at implantation of pediatric patients in eight countries (the Netherlands, Belgium, Germany, the United Kingdom, France, Turkey, Portugal, and Italy) between 2005 and 2015 were evaluated.Results: From 2010 onwards, over 30% of the pediatric CI candidates were implanted before 24 months of age. Northern European institutions implanted children on average around 12 months of age, whereas southern European institutions implanted children after 18 months of age. The Netherlands and Germany implanted earliest (between 6 and 11 months).Discussion: Implemented newborn hearing screening programs and reimbursement rates of CIs vary greatly within Europe due to local, social, financial, and political differences. However, internationally accepted recommendations are applicable to this heterogeneous European CI practice. Although consensus on early pediatric cochlear implantation exists, this study identified marked delays in European care.Conclusion: Regardless of the great heterogeneity in European practice, reasons for latency should be identified on a national level and possibilities to prevent avoidable future implantation delays should be explored to provide national recommendations.

What is the role of preoperative imaging for cochlear implants in adults with postlingual deafness?

What is the role of preoperative imaging for cochlear implants in adults with postlingual deafness?

Delayed-onset hearing loss in pediatric candidates for cochlear implantation.

The objective of this study was to evaluate the clinical significance of delayed-onset hearing loss in children. Seventy-three children who underwent cochlear implantation (CI) were included. They were divided into a congenital hearing loss group (n = 50) and a delayed-onset hearing loss group (n = 23). The age at diagnosis of hearing loss, age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the two groups. Children in the congenital hearing loss group were confirmed to have hearing loss at a mean age of 0.3 years, and those in the delayed-onset hearing loss group were diagnosed with hearing loss at a mean age of 2.0 years. Auditory habilitation began at a mean age of 0.4 and 2.0 years, and CI was performed at a mean age of 1.4 and 2.6 years, respectively. Children in the congenital hearing loss group had better scores on speech perception tests than those in the delayed-onset hearing loss group, but the differences were not significant. About half of the children with delayed-onset hearing loss (57 %) had risk factors associated with delayed-onset hearing loss. A high prevalence of delayed-onset hearing loss was noted in the group of children who underwent CI. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed-onset hearing loss needs to be performed during early childhood.

Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation.

Cochlear implantation is the only U.S. Food and Drug Administration-approved treatment for children with marked bilateral sensorineural hearing loss. It provides auditory benefits that range from simple sound detection to substantial word understanding. Improved hearing through cochlear implantation has been demonstrated to enhance the rate of language acquisition, enable development of spoken language, and advance literacy in deaf children. Magnetic resonance imaging and computed tomography both have roles in the preoperative assessment of inner-ear abnormalities, cochlear nerve deficiency, and variant anatomy that may affect the decision to implant and the prognosis for auditory improvement and increase the risk for complications. Most cochlear abnormalities may be successfully treated with cochlear implantation, but the presence of a cochlear malformation may increase the risk for intraoperative cerebrospinal fluid leakage and postoperative bacterial meningitis. Eighth-nerve deficiency correlates with poor auditory outcomes and may affect eligibility for cochlear implantation. Another important consideration for implantation is the presence of labyrinthitis ossificans in some children with deafness resulting from bacterial meningitis, which may cause obstruction that limits electrode insertion. Anatomic variations of the facial nerve or middle-ear cavity, which are more common in syndromic patients, may also affect the surgical approach and make implantation difficult.

Subtotal Petrosectomy and Mastoid Obliteration in Adult and Pediatric Cochlear Implant Recipients

To investigate the effect of subtotal petrosectomy and mastoid obliteration (SPMO) on the overall success of adult and pediatric cochlear implant (CI) recipients. Retrospective case series. Tertiary care referral center. Thirty-nine ears in 36 patients (23 adults and 13 children) received both surgeries between 1990 and 2012. CI candidates underwent SPMO to permit implantation and minimize the risks of infectious complications in the recipient ear. SPMO was performed before (69.3%), at the time of (25.6%), and after CI (5.13%). Mastoids were obliterated with fat (30.8%), muscle (66.7%), and bone pate (2.56%). Feasibility, complications, and success of SPMO and CI were assessed with standard statistical analysis and Fisher's exact test with 2-sided p values. Ear disease was definitively managed, and CI was successfully placed in all but one case. Complications including abscess (n = 3), subcutaneous emphysema (n = 1), ear canal granulation formation (n = 1), and electrode extrusion (n = 1) occurred in 15.4% of patients. Predisposing syndromes were present in children more often than adults (43.8% versus 13.0%, p = 0.0598). Adults more often than children had previous mastoid surgery for middle ear disease (30.4% versus 0.0%, p = 0.0288). CIs were placed under local anesthetic and sedation (n= 3) and after radiation treatment for nasopharyngeal cancer (n = 2) in adult ears. SPMO is an effective and safe procedure for definitively managing middle ear disease and implanting adult and pediatric CI candidates.

Prognosis Tool Based on a Modified Children's Implant Profile for Use in Pediatric Cochlear Implant Candidacy Evaluation

We developed a prediction tool to assist in evaluation of pediatric candidates for cochlear implantation (CI) and to help plan for preoperative and postoperative support. Between 1995 and 2005, 277 patients underwent CI at Children's Hospital Boston. Of these 277 patients, 250 had at least 2 years of post-CI follow-up and adequate pre-CI information for rating by our prediction tool. Of the 250, 106 were randomly selected for inclusion. The patients were divided into group A (auditory/oral communicator); group B (auditory/oral communicator with visual assistance), group C (visual/manual communicator with auditory/oral skills assistance), and group D (will not derive communicative benefit from implant). Predictions were performed with clinical assessment and two statistical techniques: regression modeling and classification and regression tree (CART) analysis. Among patients who became auditory/oral communicators (group A), clinical assessment predicted that outcome accurately 65% of the time, CART analysis had intermediate sensitivity (79%), and regression modeling was the most sensitive (95%). Groups B through D were predicted 45% of the time by regression modeling, 90% of the time by clinical assessment, and 100% of the time by CART analysis. A combination of speech-language, medical, and educational constructs can provide a reliable prediction of the communication outcome. Our goal for the prognosis tool is to make it part of the overall candidacy process in supporting decision-making about CI and planning for post-CI therapy.

Limitations of Universal Newborn Hearing Screening in Early Identification of Pediatric Cochlear Implant Candidates

To determine whether implementation of universal newborn hearing screening (UNHS) in the state of Illinois has affected the ages at diagnosis of hearing loss and implantation in children receiving cochlear implants and to determine how often children undergoing implantation had UNHS results with no indication of hearing loss (pass). Retrospective case review of 417 randomly selected pediatric implant recipients born before and after UNHS was mandated by law in Illinois. Data analyzed included hearing screening status, ages at initial diagnosis of sensorineural hearing loss (SNHL) and severe to profound SNHL, and age at implantation. Tertiary care medical center. Children receiving implants from 1991 through 2008. Ages at diagnosis of SNHL and implantation. Children born after legally mandated UNHS had significantly younger ages at diagnosis and implantation. However, a younger age at diagnosis of SNHL was not achieved in children who had passed UNHS or who were not screened. Approximately 30% of pediatric implant recipients passed UNHS, regardless of the cause of hearing loss or the presence or absence of known risk factors. Almost one-third of our pediatric implant recipients pass UNHS and are older at the time of initial diagnosis and implantation than their peers who fail UNHS. Delayed onset of SNHL limits our ability to achieve early diagnosis and implantation of a significant number of deaf children. This problem will not be solved by the current design of universal hearing screening programs.

Preoperative cerebral magnetic resonance imaging and white matter changes in pediatric cochlear implant recipients

Preoperative magnetic resonance imaging (MRI) can yield valuable information regarding the status of the inner ear in pediatric cochlear implant (CI) candidates. Incidentally, cerebral white matter changes are at times noted on these MRI scans. They may indicate post-infectious/postinflammatory changes or progressive neurodegenerative processes. To evaluate the clinical significance of cerebral white matter changes in pediatric CI patients. Retrospective chart review of pediatric CI recipients with preoperative MRI between March 2004 and January 2009 was performed. MRI reports along with postoperative audiology and speech and language outcomes were assessed. Fifty-seven patients were included in the study. There were 29 females and 28 males. Mean age was 58 months (range of 12-194 months). Abnormal MRI with varying degrees of white matter changes was noted in 10 (18%) children. Neurological assessments did not reveal any serious central nervous system diseases. Two of the 10 patients demonstrated significant delays and difficulties with postoperative CI performance. Cerebral white matter abnormalities are not uncommon incidental findings in preoperative MRI scans in pediatric CI candidates. These changes may reflect remote or active pathology which may require neurological assessment. The significance of these findings is still uncertain and should be better clarified as we study more patients.

Vestibular impairments pre- and post-cochlear implant in children

Determine prevalence and types of vestibular impairments in sensorineural hearing loss (SNHL) in a large population of pediatric candidates for cochlear implants. Evaluate impact of cochlear implants on vestibular function. Retrospective and prospective study. Children with profound SNHL (n=224) underwent complete vestibular testing (clinical vestibular examination, bicaloric test, earth vertical axis rotation, off vertical axis rotation and vestibular evoked myogenic potentials) before cochlear implant. Changes in vestibular responses were measured after implants in 89 of these patients. In the SNHL population only 50% had normal bilateral vestibular function, while 20% had bilateral complete areflexia, 22.5% partial asymmetrical hypoexcitability and 7.5% partial symmetrical hypoexcitability. In the 71/89 follow-up patients showing vestibular responses prior to implant, 51 (71%) had changes in vestibular function including 7 (10%) who acquired ipsilateral areflexia. Others developed ipsilateral hypo- or hyperexcitability. Vestibular modifications occurred during the 3 months after surgery and were not clearly associated with clinical signs except for ipsilateral areflexia cases. In long-term follow-up, two of the 7 patients with ipsilateral areflexia partially recovered vestibular function. Since half of pediatric cochlear implant candidates have vestibular deficits and 51% of implants induce modifications of existing vestibular function, each implant should be preceded by canal and otolith functional tests to assure that the least functional vestibule is implanted. The tests provide baselines for follow-up monitoring of subsequent losses and recovery. This could be easily implemented with a clinical vestibular examination including the head thrust test associated with a bicaloric test and vestibular-evoked-myogenic-potentials.

Predominance of Genetic Diagnosis and Imaging Results as Predictors in Determining the Speech Perception Performance Outcome After Cochlear Implantation in Children

To investigate the roles of genetic diagnosis and imaging studies, as well as other prognostic factors, in predicting outcomes in children with cochlear implant. Prospective cohort study. Tertiary referral center. Sixty-seven consecutive children with sensorineural hearing impairment who had at least 3 years of experience with cochlear implant. Imaging of the inner ear was done with high-resolution computed tomography, and mutations were screened in 3 genes commonly associated with pediatric hearing impairment: GJB2, SLC26A4, and the mitochondrial 12S ribosomal RNA gene. Speech perception performance was compared according to genetic diagnosis and imaging data. A general linear model was constructed to demonstrate the predictive values of specific genetic and imaging results after adjusting for other factors. Main Outcome Measure Recognition scores on speech perception tests. Twenty-two children (33%) showed genetic mutations: 18 with SLC26A4 and 4 with GJB2 mutations. According to imaging findings, 33 children (49%) showed inner ear malformations: 9 with a narrow internal auditory canal and 24 with other malformations. All children with SLC26A4 or GJB2 mutations exhibited excellent speech recognition scores, whereas a narrow internal auditory canal was associated with poorer outcomes (P < .001 in all recognition scores). The general linear model confirmed that both a narrow internal auditory canal (P < .001) and SLC26A4 mutations (P = .04) correlated with speech perception outcome. Genetic diagnosis and imaging results are the 2 predominant factors determining the outcome in children with cochlear implants. In pediatric candidates for cochlear implantation, both genetic examination and imaging studies should be included in the battery of preoperative evaluations.

我々の臨床に於ける幼児の人工内耳適応の考え方と療育指導の方法論について

我々の臨床に於ける幼児の人工内耳適応の考え方と療育指導の方法論について