Progressive Loss of Sensitivity to Electrical Stimulation After Cochlear Implantation in X-Linked Incomplete Partition Type III Deafness.

OBJECTIVES: Patients with X-linked incomplete partition type III (IP3) deafness treated with cochlear implants exhibit higher "Most Comfortable Loudness" (MCL) levels of stimulation and more electrode deactivation than patients with normal morphology. We endeavored to analyze the progression of the MCL levels and electrode deactivation over time and assess those factors that could have led to deactivation. Furthermore, we aimed to assess whether speech perception was affected by a progressive loss of neural contact. DESIGN: All 13 patients with the IP3 malformation in our clinical database were analyzed retrospectively with regard to impedance, stimulation levels, deactivated electrodes, and speech perception. A control group of patients with normal anatomy was included. RESULTS: MCL levels increased over time by 2.5 charge units (qu) per year, which was not seen in the control group. Electrode deactivation was more common in IP3 malformation, and it was estimated that 25% of electrodes would be deactivated by 15 years of age. Impedance was stable but higher in the study population. Speech perception was lower in IP3 malformation generally and was correlated to the number of deactivated electrodes. CONCLUSIONS: Patients diagnosed with IP3 malformation deafness may suffer a greater risk of cochlear implant discontinuation compared with those with normal anatomy. A progressive loss of sensitivity to electrical stimulation may indicate a form of neural degradation in the abnormal cochlea. With time, patients in this group, even with cochlear implant technology, may experience gradual deterioration of speech perception. This has clinical implications for the counseling of parents.

X-linked Malformation Deafness: Neurodevelopmental Symptoms Are Common in Children With IP3 Malformation and Mutation in POU3F4.

OBJECTIVE: Incomplete partition type 3 (IP3) malformation deafness is a rare hereditary cause of congenital or rapid progressive hearing loss. The children present with a severe to profound mixed hearing loss and temporal bone imaging show a typical inner ear malformation classified as IP3. Cochlear implantation is one option of hearing restoration in severe cases. Little is known about other specific difficulties these children might exhibit, for instance possible neurodevelopmental symptoms. MATERIAL AND METHODS: Ten 2; 0 to 9; 6-year-old children with IP3 malformation deafness (nine boys and one girl) with cochlear implants were evaluated with a retrospective chart review in combination with an additional extensive multidisciplinary assessment day. Hearing, language, cognition, and mental ill-health were compared with a control group of ten 1; 6 to 14; 5-year-old children with cochlear implants (seven boys and three girls) with another genetic cause of deafness, mutations in the GJB2 gene. RESULTS: Mutations in POU3F4 were found in nine of the 10 children with IP3 malformation. Children with IP3 malformation deafness had an atypical outcome with low level of speech recognition (especially in noise), executive functioning deficits, delayed or impaired speech as well as atypical lexical-semantic and pragmatic abilities, and exhibited mental ill-health issues. Parents of children with IP3 malformation were more likely to report that they were worried about their child's psychosocial wellbeing. Controls, however, had more age-typical results in all these domains. Eight of 10 children in the experimental group had high nonverbal cognitive ability despite their broad range of neurodevelopmental symptoms. CONCLUSIONS: While cochlear implantation is a feasible alternative for children with IP3 malformation deafness, co-occurring neurodevelopmental anomalies, such as attention deficit hyperactivity or developmental language disorder, and mental ill-health issues require an extensive and consistent multidisciplinary team approach during childhood to support their overall habilitation.

Identical twins affected by congenital cytomegalovirus infections showed different audio-vestibular profiles.

This study explored whether there were long-term hearing and vestibular outcome differences between five pairs of identical twins who had been infected with the congenital cytomegalovirus (CMV) infection before birth. Data were collected from the medical records at the Audiological Clinic, Karolinska University Hospital, Stockholm. The congenital CMV infection resulted in high variations in vestibular and hearing function within, and between, the genetically identical twin pairs. Clinicians need to be aware that treatment and interventions may need to differ substantially when identical twins have hearing issues related to the congenital CMV infection.

Two-phase survey on the frequency of use and safety of MRI for hearing implant recipients.

PURPOSE: Magnetic resonance imaging (MRI) is often used to visualize and diagnose soft tissues. Hearing implant (HI) recipients are likely to require at least one MRI scan during their lifetime. However, the MRI scanner can interact with the implant magnet, resulting in complications for the HI recipient. This survey, which was conducted in two phases, aimed to evaluate the safety and performance of MRI scans for individuals with a HI manufactured by MED-EL (MED-EL GmbH, Innsbruck, Austria). METHODS: A survey was developed and distributed in two phases to HEARRING clinics to obtain information about the use of MRI for recipients of MED-EL devices. Phase 1 focused on how often MRI is used in diagnostic imaging of the head region of the cochlear implant (CI) recipients. Phase 2 collected safety information about MRI scans performed on HI recipients. RESULTS: 106 of the 126 MRI scans reported in this survey were performed at a field strength of 1.5 T, on HI recipients who wore the SYNCHRONY CI or SYNCHRONY ABI. The head and spine were the most frequently imaged regions. 123 of the 126 scans were performed without any complications; two HI recipients experienced discomfort/pain. One recipient required reimplantation after an MRI was performed using a scanner that had not been approved for that implant. There was only one case that required surgical removal of the implant to reduce the imaging artefact. CONCLUSION: Individuals with either a SYNCHRONY CI or SYNCHRONY ABI from MED-EL can safely undergo a 1.5 T MRI when it is performed according to the manufacturer's safety policies and procedures.

Cochlear implants before 9 months of age led to more natural spoken language development without increased surgical risks.

AIM: Evidence suggests that cochlear implants are beneficial for language development, but there is no consensus about the ideal age for surgery. We investigated how language development and surgical safety were affected by patients' ages. METHODS: This study comprised 103 children (52 boys) aged 4.3-16 years who received cochlear implants at 5-29 months at the Karolinska University Hospital, Stockholm, Sweden, between 2002 and 2013. All showed typical development and were from monolingual homes. Bilateral implants were common (95%). The children were regularly assessed on language understanding, vocabulary and speech recognition by a multi-disciplinary team for 10.0 ± 3.7 (4.7-16.0) years. RESULTS: There were no associations between complications after surgery and the age when children had their first implant. Children implanted at 5-11 months reached an age-equivalent level of language understanding and better vocabulary outcome sooner than subgroups implanted later. Children who had surgery at 12-29 months demonstrated more atypical and delayed language abilities over time. Early implantation, preferably before 9 months, may lead to a more typical trajectory of spoken language development. CONCLUSION: Our findings showed that cochlear implantation before 9 months was safe. Early implantation may reduce the negative effects of auditory deprivation and promotes more natural and synchronised language development.

Chorioretinal scars and visual deprivation are common in children with cochlear implants after congenital cytomegalovirus infection.

AIM: The aim of this study was to compare visual function and ocular characteristics in children with cochlear implants, due to severe hearing impairment caused by the congenital cytomegalovirus (CMV) infection, with control children fitted with cochlear implants due to connexin 26 mutations (Cx26), a genetic cause of hearing impairment. METHODS: We carried out ophthalmological assessments, including visual acuity, ocular alignment, Ocular Motor Score, biomicroscopy and fundus photography, on 26 children with congenital CMV (median age 8.3 years, range 1.4-16.7) and 13 Cx26 controls (median age 5.6 years, range 1.7-12.5). RESULTS: We found unilateral chorioretinal macular scars that reduced best-corrected visual acuity ≤0.3 in five (19%) of the children with congenital CMV, but in none of the children with Cx26 (p = 0.15). Ocular motility problems were more common among children with congenital CMV, but the difference was not significant (p = 0.20). The vestibulo-ocular reflex was more frequently pathological in children with congenital CMV (p = 0.011). CONCLUSION: Ocular complications with central chorioretinal scars and ocular motility disturbances were common in children treated with cochlear implants due to severe hearing impairment caused by the congenital CMV infection. Ophthalmological assessments are advisable in such children for early identification, intervention and follow-up.

A longitudinal study of the bilateral benefit in children with bilateral cochlear implants.

OBJECTIVE: To study the development of the bilateral benefit in children using bilateral cochlear implants by measurements of speech recognition and sound localization. DESIGN: Bilateral and unilateral speech recognition in quiet, in multi-source noise, and horizontal sound localization was measured at three occasions during a two-year period, without controlling for age or implant experience. Longitudinal and cross-sectional analyses were performed. Results were compared to cross-sectional data from children with normal hearing. STUDY SAMPLE: Seventy-eight children aged 5.1-11.9 years, with a mean bilateral cochlear implant experience of 3.3 years and a mean age of 7.8 years, at inclusion in the study. Thirty children with normal hearing aged 4.8-9.0 years provided normative data. RESULTS: For children with cochlear implants, bilateral and unilateral speech recognition in quiet was comparable whereas a bilateral benefit for speech recognition in noise and sound localization was found at all three test occasions. Absolute performance was lower than in children with normal hearing. Early bilateral implantation facilitated sound localization. CONCLUSIONS: A bilateral benefit for speech recognition in noise and sound localization continues to exist over time for children with bilateral cochlear implants, but no relative improvement is found after three years of bilateral cochlear implant experience.

Impaired balance and neurodevelopmental disabilities among children with congenital cytomegalovirus infection.

AIM: Although cytomegalovirus (CMV) is the most common congenital infection, existing research has not provided us with a full picture of how this can affect children in the future. The aim of this case-control study was to evaluate disabilities in a well-defined group of children with congenital cytomegalovirus (CMV) infection, who had been fitted with cochlear implants because of severe hearing impairment. METHODS: A multidisciplinary team assessed 26 children with congenital CMV infection for balance difficulties, neurodevelopmental disabilities and language and visual impairment. We also included a control group of 13 children with severe hearing impairment due to connexin 26 mutations. RESULTS: The majority of the children with congenital CMV infection (88%) displayed balance disturbances, including walking at a later age, but there were no cases in the control group. The CMV group also displayed frequent neurodevelopmental disabilities and feeding difficulties. CONCLUSION: Congenital CMV infection affects the general development of the brain and gives rise to a complex pattern of difficulties. Identifying comorbid conditions is very important, as children with associated difficulties and disabilities need more support than children with just hearing impairment. Congenital CMV infection needs to be considered in children with hearing impairment and/or balance disturbance and/or neurodevelopmental disabilities.

Long-term linguistic outcome in adults with congenital cytomegalovirus infection.

BACKGROUND: Congenital cytomegalovirus (cCMV) is the most common prenatal infection and the main infectious cause of neurodevelopmental abnormalities in developed countries. Long-term neuropsychological outcome of cCMV infection is still not well understood. This is the first study that presents linguistic follow-up data performed on adults who were infected in utero. METHOD: All individuals from a universal newborn CMV screening study in Sweden sampled from 1977 to 1985 were invited to participate in a follow-up study. 34/71 persons (48%) with cCMV and 22/46 controls (48%) were enrolled. Participants were between 34 and 43 years. Linguistic ability was evaluated with two-word fluency tasks (FAS letter fluency and verb fluency), and a qualitative analysis of the participants' word retrieval strategies was conducted. RESULTS: No statistically significant group differences were found in the total number of retrieved words. When related to Swedish norm data, 43% of participants with cCMV infection, all asymptomatic at birth, had adequate results on both FAS and verb fluency tasks, compared to 86% of the controls. Education level was the most important factor for word fluency ability in both groups. Adults with cCMV infection and higher education levels used less effective retrieval strategies on FAS letter fluency than controls. CONCLUSION: This study suggests that adults with cCMV infection may have deficits in the word retrieval process, even in the absence of known neurodevelopmental disorders. Long-term effects of cCMV infection may exist even in those with asymptomatic infection at birth.

HEARRING group genetic marker study: genetic background of CI patients.

BACKGROUND: While cochlear implantation (CI) and electric acoustic stimulation (EAS) have a positive outcome in most cases, their effectiveness varies depending on the etiology of the hearing loss. Among the various etiologies, genetic factors are the leading cause of hearing loss and may impact CI and EAS outcomes. AIMS/OBJECTIVES: To reveal the genetic background of the hearing loss in CI/EAS patients in each ethnic population, we undertook a multi-center study involving the genetic testing of hearing loss in CI/EAS patients from 10 centers. MATERIAL AND METHODS: Saliva samples and clinical information for the patients and their family members were obtained and next-generation sequencing analysis using a panel carrying 63 deafness genes was then performed. RESULTS: Genetic testing successfully identified the causative gene variants in 54.5% (48/88) of patients with pre-lingual onset hearing loss (onset under 6 years) and in 12% (12/95) of those with late-onset hearing loss (onset at 6 years or more). CONCLUSIONS AND SIGNIFICANCE: We clearly indicated that genetic factors are the most common cause of hearing loss regardless of ethnic background. Saliva-based genetic testing is a useful tool for multi-center studies seeking to clarify the genetic causes of hearing loss in CI or EAS patients between countries separated by distance.

Vestibular Loss in Children Affected by LVAS and IP2 Malformation and Operated with Cochlear Implant.

This is a single center cohort study regarding the prevalence of vestibular loss in hearing impaired children affected by large vestibular aqueduct syndrome (LVAS) with incomplete cochlear partition malformation type II (IP2), fitted with cochlear implant (CI). Twenty-seven children received CI operations at 0.4-13 years on one or both ears and tested for vestibular loss with head impulse test, video head impulse test, mini ice-water test and cervical VEMP. Vestibular loss was found in 19% of operated ears and in 13.9% of non-operated ears. The difference was not statistically significant and was not significantly modified by age at implantation, age at testing, sex, presence of SLC26A4 gene mutation or bilaterality. However, the presence of anatomic anomalies at the level of the vestibulum or semicircular canals was significantly associated with a higher incidence of vestibular loss in CI operated children but not in those non-operated. No other factors, such as the surgical access, the electrode type, the presence of Gusher perioperatively, or post-operative vertigo modified significantly the prevalence of vestibular loss. In conclusion, LVAS/IP2 appears to be the major determinant of vestibular loss in these children, with a less obvious impact of CI, excluding the cases with vestibulum/canal anomalies: this group might have a higher risk for vestibular loss after CI surgery.

A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss.

Children with unilateral sensorineural hearing loss (uSNHL) have a high risk of speech-language delays and academic difficulties. Still, challenges remain in the diagnosis of uSNHL. With a prospective cross-sectional design, 20 infants were consecutively recruited from a universal newborn hearing screening program and invited to genetic testing. Eighteen of the subjects agreed to genetic testing, 15 subjects with OtoSCOPE® v.9 screening 224 genes, and four subjects underwent targeted testing, screening for chromosomal abnormalities or 105-137 gene mutations. The genetic results were described together with the 20 infants' previously published auditory profiles and imaging results. Genetic causes for the uSNHL were found in 28% of subjects (5/18) including CHARGE syndrome (CHD7), autosomal recessive non-syndromic hearing loss (GJB2), Townes-Brocks syndrome (SALL1), Pendred Syndrome (SLC26A4) and Chromosome 8P inverted duplication and deletion syndrome. In subjects with comorbidities (malformation of fingers, anus, brain, and heart), 100% were diagnosed with a genetic cause for uSNHL (3/3 subjects), while 13% (2/15 subjects) were diagnosed without comorbidities observed at birth (p = 0.002). Genetic testing for congenital uSNHL is currently efficient for alleged syndromes, whereas genetic variants for non-syndromic congenital uSNHL need further research.

Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia.

BACKGROUND: Infants and young children with vestibulocochlear nerve (VCN) hypoplasia/aplasia present with severe hearing loss and are candidates for cochlear implantation (CI). It is unknown whether vestibular function is related to CI outcome and if vestibular tests can guide the operation decision. AIMS/OBJECTIVES: Our aim was to describe the vestibular function in patients with VCN hypoplasia/aplasia before a possible CI. MATERIALS AND METHODS: Forty-two ears in 23 patients were tested between 2019 and 2022 with bone-conducted cervical vestibular evoked myogenic potentials (BCcVEMP), video head impulse test (vHIT) and miniice-water caloric test (mIWC). RESULTS: All ears could be tested with at least one vestibular test and 83% could be tested with more than one method. Twenty-nine ears (61%) showed normal function with at least one method. The presence of a normal response to any test doubled the likelihood of a measured hearing threshold after CI, the best predictors being the BCcVEMP and vHIT (p < 0.05). CONCLUSION: Canal function may represent a predictor of auditive pathway integrity with a possible favourable audiological outcome after CI operation. SIGNIFICANCE: Our results demonstrate high vestibular response rates suggesting a functioning pathway despite the radiological diagnosis.

Congenital cytomegalovirus infection - a common cause of hearing loss of unknown aetiology.

AIM: The aim of this study was to investigate the role of congenital cytomegalovirus (CMV) infection as a cause of various types of sensorineural hearing loss (SNHL) in a group of nonsyndromic children with otherwise unknown aetiology of hearing loss. Furthermore, the occurrence of combined congenital CMV infection and connexin 26 (Cx26) mutations was investigated. METHODS: The dried blood spot (DBS) cards of 45 children with various degrees of hearing deficits and 46 children with severe/profound hearing loss were tested for CMV DNA with polymerase chain reaction (PCR) technique. The DBS cards of the 46 children with severe/profound hearing loss were also analysed for Cx26 mutations. RESULTS: Of the 45 children with various degrees of hearing loss, nine were positive for CMV DNA (20%). The nine children represented severe/profound, mild and unilateral hearing loss. From the 46 children with severe/profound hearing loss, nine of 46 (20%) were positive for CMV DNA. In addition, three of the CMV DNA-positive children were carriers of mutations of Cx26. CONCLUSION: Congenital CMV infection is a high risk factor in hearing impairment among children.

Bilateral versus unilateral cochlear implants in children: speech recognition, sound localization, and parental reports.

OBJECTIVE: To compare bilateral and unilateral speech recognition in quiet and in multi-source noise, and horizontal sound localization of low and high frequency sounds in children with bilateral cochlear implants. DESIGN: Bilateral performance was compared to performance of the implanted side with the best monaural speech recognition in quiet result. Parental reports were collected in a questionnaire. Results from the CI children were compared to binaural and monaural performance of normal-hearing peers. STUDY SAMPLE: Sixty-four children aged 5.1-11.9 years who were daily users of bilateral cochlear implants. Thirty normal-hearing children aged 4.8-9.0 years were recruited as controls. RESULTS AND CONCLUSIONS: Group data showed a statistically significant bilateral speech recognition and sound localization benefit, both behaviorally and in parental reports. The bilateral speech recognition benefit was smaller in quiet than in noise. The majority of subjects localized high and low frequency sounds significantly better than chance using bilateral implants, while localization accuracy was close to chance using unilateral implants. Binaural normal-hearing performance was better than bilateral performance in implanted children across tests, while bilaterally implanted children showed better localization than normal-hearing children under acute monaural conditions.

Development of Sound Localization in Infants and Young Children with Cochlear Implants.

Cochlear implantation as a treatment for severe-to-profound hearing loss allows children to develop hearing, speech, and language in many cases. However, cochlear implants are generally provided beyond the infant period and outcomes are assessed after years of implant use, making comparison with normal development difficult. The aim was to study whether the rate of improvement of horizontal localization accuracy in children with bilateral implants is similar to children with normal hearing. A convenience sample of 20 children with a median age at simultaneous bilateral implantation = 0.58 years (0.42−2.3 years) participated in this cohort study. Longitudinal follow-up of sound localization accuracy for an average of ≈1 year generated 42 observations at a mean age = 1.5 years (0.58−3.6 years). The rate of development was compared to historical control groups including children with normal hearing and with relatively late bilateral implantation (≈4 years of age). There was a significant main effect of time with bilateral implants on localization accuracy (slope = 0.21/year, R2 = 0.25, F = 13.6, p < 0.001, n = 42). No differences between slopes (F = 0.30, p = 0.58) or correlation coefficients (Cohen's q = 0.28, p = 0.45) existed when comparing children with implants and normal hearing (slope = 0.16/year since birth, p = 0.015, n = 12). The rate of development was identical to children implanted late. Results suggest that early bilateral implantation in children with severe-to-profound hearing loss allows development of sound localization at a similar age to children with normal hearing. Similar rates in children with early and late implantation and normal hearing suggest an intrinsic mechanism for the development of horizontal sound localization abilities.

A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss.

Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices.

Gender differences in caregiver's use of spoken language with young children who are hard-of-hearing.

PURPOSE: Shared parenting among caregivers of different gender is common in the Swedish society. It is unclear if this includes shared contribution for children's language development. The objective of this cross-sectional study was to explore the natural language environment of children who were hard-of-hearing compared to typically hearing controls. METHODS: Seventy-two families with children aged 7-35 months participated; 22 children who were hard-of-hearing (Cochlear implants, n=11; Hearing aids, n=11) and 50 controls with typical hearing. The majority of caregivers had higher education background level, especially in the control group. Families conducted a daylong recording with the Language Environment Analysis technology, when both parents were present at home. An Interpreted Time Segmental analysis was performed to extract information about female versus male caregivers quantitative word use. RESULTS: The results showed significant gender differences related to number of adult words, with less male words than female words (p <0.001). Male caregivers of children who were hard-of-hearing contributed with around 27 % of adult words during the recordings while males in the control group contributed with 37 %. There was a larger variation in number of female words in the study group than for controls, especially in mothers of children with cochlear implants. CONCLUSIONS: Female caregivers talk significantly more close to young children than male caregivers, and especially in the subgroup of children with cochlear implants. Children who are hard-of-hearing are dependent on a rich language environment, and might be especially vulnerable if male caregivers are less involved as language facilitators. More studies are needed to explore caregiver gender differences, both related to quantitative and qualitative language stimulation.

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

OBJECTIVES: The 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. METHODS: The 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. RESULTS: Complete 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. CONCLUSIONS: The 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.

The reliability of hearing implants: report on the type and incidence of cochlear implant failures.

Objectives: This study presents the data collected through a database on the type and incidence of cochlear implant device failures and major complications and quantifies the risk of failures across time based on the Association for the Advancement of Medical Instrumentation (AAMI) CI86:2017 standard. Methods: Information on reliability of MED-EL cochlear implants was collected from the MED-EL complaint database between 2003 and2013. Explants were categorized and device reliability was calculated according to the AAMI CI86:2017 standard principles. Results: Data were collected for 11662 devices (5462 children, 6200 adults). The mean duration of follow up was 46.16 months. The total failure rate for all devices and all subjects was 2.41%. Medical related explants (MRE) were significantly worse for children than for adults with the ceramic implants, C40+ (p = 0.008) and PULSAR (p = 0.020). Device failure explants (DFE) were significantly worse for children than for adults with all four devices in the study, the C40+ (p < 0.001), PULSAR (p < 0.001), SONATA (p < 0.001), and CONCERTO (p = 0.023). The mean annual failure rate for all subjects and devices was 0.63% (1.03% for children, 0.28% for adults). The mean annual failure rate was 0.90% for the C40+; 0.57% for the PULSAR; 0.46% for the SONATA; and 0.39% for the CONCERTO. Conclusions: Compared to adults, children had significantly worse MRE and DFE due to a higher risk of head trauma and more vulnerable skull anatomy. Further, the authors conclude that the AAMI standard will ensure a more comprehensive and transparent evaluation of cochlear implant reliability in the future.