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.

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.

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.

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.

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.

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 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.

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.

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.

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.

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.

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.

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.

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

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. 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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.