The French Cochlear Implant Registry (EPIIC): Perception and language results in infants with cochlear implantation under the age of 24 months.

OBJECTIVE: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016. PATIENTS AND METHODS: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared. RESULTS: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling. CONCLUSION: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling.

The French Cochlear Implant Registry (EPIIC): General indicators.

Cochlear and brainstem implants have been included on the list of reimbursable products (LPPR) in France since March of 2009. The implants were initially inscribed for 5 years, after which an application for renewal with the French National Commission for the Evaluation of Medical Devices and Health Technologies (Commission Nationale d'évaluation des dispositifs médicaux et des technologies de santé - CNEDiMTS) was required [Haute Autorité de santé, 2009]. Upon registration to the list of reimbursable products, the companies and the reference centers for cochlear and brainstem implants were asked to set up a post-registration registry called EPIIC. This article reports the evolution in the EPIIC registry of the general indicators for 5051 patients over the five years from 2012-2016.

The French National Cochlear Implant Registry (EPIIC): Cochlear explantation and reimplantation.

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation.

The French National Cochlear Implant Registry (EPIIC): Results, quality of life, questionnaires, academic and professional life.

This study concerns the results of cochlear implantation in children and adults from French cochlear implantation centers, monitored at one, two and three years by the Cochlear Implant French Registry EPIIC. This multicenter study enrolled 2603 subjects (1667 adults and 936 children) implanted in one ear. The following parameters were studied: hearing overall performances, monosyllabic or dissyllabic word perception, speech intelligibility, self-assessment questionnaire of Cochlear Implant (CI) benefits (Abbreviated profile of Hearing aid Benefit); professional activity and schooling. This study confirms the ceiling effect in adults' performances after the 1st year and the progressive growth in children's performances. It also shows that the contralateral hearing aid enhances performances compared to the CI alone condition, in all follow-up sessions. The French register of CIs is the only worldwide register of systematic follow-up on a period of three years and more of all adults and children implanted in a country.

The French National Cochlear Implant Registry (EPIIC): Cochlear implantation in adults over 65years old.

OBJECTIVES: To analyze the performance of cochlear implants in French patients aged 65 and over, implanted between 2012 and 2016, using data from the French national registry for cochlear implants (EPIIC). MATERIALS AND METHODS: The French national registry incorporates patient data from before implantation and for three years after implantation, stratified in different age groups (18-39, 40-64years, 65-74years and>75years). Here, we assessed the latter two categories. Hearing was assessed using mono- and disyllabic words in a silent background. The Category of Auditory Performance (CAP) scale was also implemented and subjects took the Abbreviated Profile of Hearing Aid Benefit (Aphab) questionnaire. RESULTS: The population aged over 65 accounted for 38% (n=1193) of the 3178 adult implanted patients. The performance for mono- and disyllabic words in silence, the CAP scores and the APHAB questionnaire answers for ease of communication, background noise and reverberation were dramatically improved at one year post-implantation (P<0.0001 for each score) and remained stable between one and three years thereafter. The percentage improvement was similar across all age groups. The scores for loud-noise intolerance did not change after cochlear implantation in any age group. CONCLUSION: Cochlear implants improve hearing and communication in subjects aged 65 and over, with comparable efficiency to that achieved in younger subjects. Cochlear implantation should thus be proposed whenever hearing aids provide only limited benefit. However, between 2012 and 2016, cochlear implantation was given to less than 1% of the French population aged 65 and over with profound deafness.

The French Cochlear Implant Registry (EPIIC): Cochlear implant candidacy assessment of off-label indications.

OBJECTIVE: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label. MATERIAL AND METHODS: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded. RESULTS: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients. CONCLUSION: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid.

The French National Cochlear Implant Registry (EPIIC): Bilateral cochlear implantation.

OBJECTIVES: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France. MATERIALS AND METHODS: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations. RESULTS: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02). CONCLUSION: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise.

The French Cochlear Implant Registry (EPIIC): Cochlear implantation complications.

OBJECTIVES: The aim of this study was to evaluate peri- and post-operative complications related to cochlear implantations. We searched for risk factors predicting these complications and analyzed the complications in the youngest and most elderly. STUDY DESIGN: Retrospective analysis of cochlear implant patients. MATERIALS AND METHODS: All patients who underwent cochlear implantation in France between January 2012 and December 2016 were anonymized and registered in the EPIIC database. This population included 3483 adults and 2245 children. Their demographic and surgical data and their incidence of peri- or post-operative complications, including their severity, whether major or minor, were all indicated. RESULTS: The global complication rate was 6.84%. The risk of complication was higher in initial implantation versus reimplantation (P<0.0001). The risk was also higher for bilateral implantation versus unilateral (P<0.0001). Complications were more frequent for patients with cochlear malformation (P=0.002). There was no difference in complication rates across age groups; babies under 1 year old, and the elderly over 80 and even over 90, did not have more complications than the rest of the population. Patients treated in the daily care unit had no more complications than those who were hospitalized for one night or more (P=0.64). CONCLUSION: Cochlear implantation is a safe technique with a low incidence of complications. The absence of increased risk in patients at the extremes of the age spectrum justifies offering this solution to all, without age limitation.

Schooling of hearing-impaired children and benefit of early diagnosis.

OBJECTIVE: To assess the impact of moderate-to-severe bilateral hearing loss on schooling and the factors influencing this impact, and to evaluate special schooling needs in addition to speech therapy. MATERIAL AND METHODS: Retrospective study including children with moderate-to-severe bilateral hearing loss, born between 1992 and 2006, diagnosed and managed in our institution. The age and degree of hearing loss in the better ear, the type of schooling and the level of schooling at the time of the last visit were recorded for each patient. RESULTS: Two hundred and twenty-five hearing-impaired children were included: 161 attended a regular school (58% of the 55 children with severe hearing loss and 76% of the 170 children with moderate hearing loss). The percentage of children with moderate hearing loss attending a regular school increased over time. This study did not demonstrate any difference in terms of grade retention according to the age at diagnosis for children with moderate hearing loss. No child with comorbidity affecting intellectual capacities attended a regular school. CONCLUSION: This study confirms that moderate-to-severe congenital bilateral hearing loss has an impact on the child's schooling, with grade retention that depends, but not exclusively, on the degree of hearing loss. A growing number of children with moderate bilateral hearing loss fitted with a hearing aid now attend a regular school.

SOX10 mutations mimic isolated hearing loss.

Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations.

[Audiophonological evaluation of 16 children fitted with cochlear implants for sensorineural hearing loss induced by bacterial meningitis]. / Implants cochléaires dans les surdités après méningite bactérienne: suivi audiologique de 16 enfants.

BACKGROUND: Bacterial meningitis (BM) is the primary etiology of acquired sensorineural hearing loss (SNHL) in children and may compromise language development. Since the 1990 s, cochlear implants (CIs) have become part of the management of children with profound SNHL with encouraging results. The aim of this study was to analyze the audiophonological performance of children before and after cochlear implantation for SNHL following bacterial meningitis. METHODS: Retrospective study of all children fitted with CIs for bilateral severe to profound SNHL after bacterial meningitis in the Robert-Debré pediatric ENT department between August 1990 and March 2009. Audiophonological performance was assessed using the APCEI profile. RESULTS: Of the 283 children receiving implants during that period, 16 children (6%; 6 boys, 10 girls) underwent CI implantation after bacterial meningitis (Streptococcus pneumoniae in 8 cases, Neisseria meningitidis in 2 cases, and Haemophilus influenzae in 4 cases). The mean time from meningitis to SNHL was 8.3 months (median, 1.5 months; range, 1 day to 13 years). The mean time from meningitis to cochlear implantation was 2 years and 3 months (median, 7 months; range, 1 month to 13 years 3 months). Twelve children (75%) presented partial cochlear and/or vestibular ossification on presurgical CT scan. Three children received bilateral implants. DISCUSSION: Thirteen children (81%) developed early SNHL in the first 3 months, whereas 3 children developed SNHL more than 10 months after meningitis. As for the benefits of cochlear implantation, 11 children presented near to normal intelligibility and optimal use of their cochlear implant; 5 children presented partial benefits due to neurological sequelae (1), a long delay before implantation (1), technical problems (2), or a social problem in relation to low socioeconomic status (1). CONCLUSION: After bacterial meningitis, audiological evaluation must be made carefully during the first 3 months to detect early SNHL, but SNHL may also develop several years later. In case of profound SNHL and a modified signal of the labyrinth on the MRI, cochlear implantation must be performed without delay before cochlear and/or vestibular ossification. Cochlear implantation is an effective technique with good long-term audiologic results. The coexistence of neurological lesions may compromise the results, but it should not contraindicate a cochlear implantation.

Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations.

BACKGROUND AND PURPOSE: Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. MATERIALS AND METHODS: Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. RESULTS: Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). CONCLUSIONS: In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation.

[Total unilateral deafness in children. Etiologies and long-term consequences]. / Surdité unilatérale totale chez l'enfant. Etiologies et conséquences à long-terme.

A retrospective study of 72 children with a profound (average hearing loss > 90 dB) unilateral sensorineural hearing loss (PUSNHL) was carried out in the ENT department at the Rouen hospital in 1988 and 1994. This study intended to investigate the etiologies, how it was recognized, the age of the onset and the results of the medical evaluation of these PUSNHL. We found, in 2 cases, that vestibular lesions of the healthy ear were present before the onset of a hearing loss on this side, turning the PUSNHL into a bilateral deafness. We tried to correlate the etiologies, age of onset of the PUSNHL, status of the opposite ear and possible scholar problems in the group of the 42 children followed-up for more than 2 years (mean follow-up time = 7 years). Among children suffering from PUSNHL, we specified a group with higher risk of school failures, allowing ENT physicians to propose more accurate management to these children.