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.

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.

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.

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.

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.

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.

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.

Unilateral Cochlear Implants for Severe, Profound, or Moderate Sloping to Profound Bilateral Sensorineural Hearing Loss: A Systematic Review and Consensus Statements.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine. Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL. Design, Setting, and Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019. Main Outcomes and Measures: A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting. Results: In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement). Conclusions and Relevance: These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

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.

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.

Predictors of Reading Comprehension in Children With Cochlear Implants.

Children with a profound hearing loss who have been implanted with cochlear implants (CI), vary in terms of their language and reading skills. Some of these children have strong language skills and are proficient readers whereas others struggle with language and both the decoding and comprehension aspects of reading. Reading comprehension is dependent on a number of skills where decoding, spoken language comprehension and receptive vocabulary have been found to be the strongest predictors of performance. Children with CI have generally been found to perform more poorly than typically hearing peers on most predictors of reading comprehension including word decoding, vocabulary and spoken language comprehension, as well as working memory. The purpose of the current study was to investigate the relationships between reading comprehension and a number of predictor variables in a sample of twenty-nine 11-12-year-old children with profound hearing loss, fitted with CI. We were particularly interested in the extent to which reading comprehension in children with CI at this age is dependent on decoding and receptive vocabulary. The predictor variables that we set out to study were word decoding, receptive vocabulary, phonological skills, and working memory. A second purpose was to explore the relationships between reading comprehension and demographic factors, i.e., parental education, speech perception and age of implantation. The results from these 29 children indicate that receptive vocabulary is the most influential predictor of reading comprehension in this group of children although phonological decoding is, of course, fundamental.

Correlates of Orthographic Learning in Swedish Children With Cochlear Implants.

This study set out to explore the cognitive and linguistic correlates of orthographic learning in a group of 32 deaf and hard of hearing children with cochlear implants, to better understand the factors that affect the development of fluent reading in these children. To date, the research about the mechanisms of reading fluency and orthographic learning in this population is scarce. The children were between 6:0 and 10:11 years of age and used oral language as their primary mode of communication. They were assessed on orthographic learning, reading fluency and a range of cognitive and linguistic skills including working memory measures, word retrieval and paired associate learning. The results were analyzed in a set of correlation analyses. In line with previous findings from children with typical hearing, orthographic learning was strongly correlated with phonological decoding, receptive vocabulary, phonological skills, verbal-verbal paired-associate learning and word retrieval. The results of this study suggest that orthographic learning in children with CI is strongly dependent on similar cognitive and linguistic skills as in typically hearing peers. Efforts should thus be made to support phonological decoding skill, vocabulary, and phonological skills in this population.

Executive Functions, Pragmatic Skills, and Mental Health in Children With Congenital Cytomegalovirus (CMV) Infection With Cochlear Implants: A Pilot Study.

Congenital cytomegalovirus (cCMV) infection is the most common cause of progressive hearing impairment. In our previous study around 90% of children with a cCMV infection and CI had severely damaged balance functions (Karltorp et al., 2014). Around 20% had vision impairment, 15% were diagnosed with Autism-Spectrum-Disorder, and 20% with ADHD. One clinical observation was that children with cCMV infection had problems with executive functioning (EF), while controls with a genetic cause of deafness (Connexin 26 mutations; Cx26) did not have similar difficulties. A follow-up study was therefore initiated with the main objective to examine EF and pragmatic skills in relation to mental health in children with a cCMV infection and to draw a comparison with matched controls with Cx26 mutations (age, sex, hearing, non-verbal cognitive ability, vocabulary, and socioeconomic status level). Ten children with a cCMV infection and CI (4.8-12:9 years) and seven children with CI (4:8-12:8 years) participated in the study, which had a multidisciplinary approach. Executive functioning was assessed both with formal tests targeting working memory and attention, parent and teacher questionnaires, and a systematic observation by a blinded psychologist during one test situation. Pragmatics and mental health were investigated with parent and teacher reports. In addition, the early language outcome was considered in non-parametric correlation analyses examining the possible relationships between later EF skills, pragmatics, and mental health. Children with cCMV had a statistically significant worse pragmatic outcome and phonological working memory than controls despite their groups having similar non-verbal cognitive ability and vocabulary. However, there were no statistical differences between the groups regarding their EF skills in everyday settings and mental health. There were associations between early language outcomes and later EF skills and pragmatics in the whole sample. Conclusion: Children with a cCMV infection are at risk of developing learning difficulties in school due to difficulties with phonological working memory and pragmatic skills in social interactions.

Hearing preservation cochlear implantation in children: The HEARRING Group consensus and practice guide.

OBJECTIVES: To provide multidisciplinary cochlear implant teams with a current consensus statement to support hearing preservation cochlear implantation (HPCI) in children, including those children with symptomatic partial deafness (PD) where the intention is to use electric-acoustic stimulation (EAS). The main objectives are to provide guidelines on who is a candidate, how to assess these children and when to implant if Med-El Flex electrode arrays are chosen for implantation. METHODS: The HEARRING group reviewed the current evidence and practice regarding the management of children to be considered for HPCI surgery emphasizing the assessment needed prior to implantation in order to demonstrate the benefits in these children over time. The consensus statement addresses following three key questions: (1) Should these children be treated? (2) How to identify these children? (3) How to manage these children? SUMMARY: The HEARRING group concludes that irrespective of the degree of residual hearing present, the concepts of hearing and structure preservation should be applied in every child undergoing cochlear implantation and that HPCI is a safe and reliable treatment option. Early detection and multidisciplinary assessment are key to the identification of children with symptomatic PD, these children should undergo HPCI as early as possible.

X-linked Malformation and Cochlear Implantation.

OBJECTIVE: To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. STUDY DESIGN: Retrospective patient review in combination with a multidisciplinary follow-up. SETTING: Tertiary referral hospital and cochlear implant program. PATIENTS: Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities. RESULTS: In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases. CONCLUSION: Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.

Quality standards for bone conduction implants.

CONCLUSION: Bone conduction implants are useful in patients with conductive and mixed hearing loss for whom conventional surgery or hearing aids are no longer an option. They may also be used in patients affected by single-sided deafness. OBJECTIVES: To establish a consensus on the quality standards required for centers willing to create a bone conduction implant program. METHOD: To ensure a consistently high level of service and to provide patients with the best possible solution the members of the HEARRING network have established a set of quality standards for bone conduction implants. These standards constitute a realistic minimum attainable by all implant clinics and should be employed alongside current best practice guidelines. RESULTS: Fifteen items are thoroughly analyzed. They include team structure, accommodation and clinical facilities, selection criteria, evaluation process, complete preoperative and surgical information, postoperative fitting and assessment, follow-up, device failure, clinical management, transfer of care and patient complaints.