Consistent and chronic cochlear implant use partially reverses cortical effects of single sided deafness in children.

Potentially neuroprotective effects of CI use were studied in 22 children with single sided deafness (SSD). Auditory-evoked EEG confirmed strengthened representation of the intact ear in the ipsilateral auditory cortex at initial CI activation in children with early-onset SSD (n = 15) and late-onset SSD occurring suddenly in later childhood/adolescence (n = 7). In early-onset SSD, representation of the hearing ear decreased with chronic CI experience and expected lateralization to the contralateral auditory cortex from the CI increased with longer daily CI use. In late-onset SSD, abnormally high activity from the intact ear in the ipsilateral cortex reduced, but responses from the deaf ear weakened despite CI use. Results suggest that: (1) cortical reorganization driven by unilateral hearing can occur throughout childhood; (2) chronic and consistent CI use can partially reverse these effects; and (3) CI use may not protect children with late-onset SSD from ongoing deterioration of pathways from the deaf ear.

Electrical Vestibular Stimulation in Humans: A Narrative Review.

BACKGROUND: In patients with bilateral vestibulopathy, the regular treatment options, such as medication, surgery, and/or vestibular rehabilitation, do not always suffice. Therefore, the focus in this field of vestibular research shifted to electrical vestibular stimulation (EVS) and the development of a system capable of artificially restoring the vestibular function. Key Message: Currently, three approaches are being investigated: vestibular co-stimulation with a cochlear implant (CI), EVS with a vestibular implant (VI), and galvanic vestibular stimulation (GVS). All three applications show promising results but due to conceptual differences and the experimental state, a consensus on which application is the most ideal for which type of patient is still missing. SUMMARY: Vestibular co-stimulation with a CI is based on "spread of excitation," which is a phenomenon that occurs when the currents from the CI spread to the surrounding structures and stimulate them. It has been shown that CI activation can indeed result in stimulation of the vestibular structures. Therefore, the question was raised whether vestibular co-stimulation can be functionally used in patients with bilateral vestibulopathy. A more direct vestibular stimulation method can be accomplished by implantation and activation of a VI. The concept of the VI is based on the technology and principles of the CI. Different VI prototypes are currently being evaluated regarding feasibility and functionality. So far, all of them were capable of activating different types of vestibular reflexes. A third stimulation method is GVS, which requires the use of surface electrodes instead of an implanted electrode array. However, as the currents are sent through the skull from one mastoid to the other, GVS is rather unspecific. It should be mentioned though, that the reported spread of excitation in both CI and VI use also seems to induce a more unspecific stimulation. Although all three applications of EVS were shown to be effective, it has yet to be defined which option is more desirable based on applicability and efficiency. It is possible and even likely that there is a place for all three approaches, given the diversity of the patient population who serves to gain from such technologies.

Limiting asymmetric hearing improves benefits of bilateral hearing in children using cochlear implants.

Neurodevelopmental changes occur with asymmetric hearing loss, limiting binaural/spatial hearing and putting children at risk for social and educational challenges. These deficits may be mitigated by providing bilateral hearing in children through auditory prostheses. Effects on speech perception and spatial hearing were measured in a large cohort of >450 children who were deaf and used bilateral cochlear implants or bimodal devices (one cochlear implant and a contralateral hearing aid). Results revealed an advantage of bilateral over unilateral device use but this advantage decreased as hearing in the two ears became increasingly asymmetric. Delayed implantation of an ear with severe to profound deafness allowed asymmetric hearing, creating aural preference for the better hearing ear. These findings indicate that bilateral input with the most appropriate device for each ear should be provided early and without delay during development.

Delayed access to bilateral input alters cortical organization in children with asymmetric hearing.

Bilateral hearing in early development protects auditory cortices from reorganizing to prefer the better ear. Yet, such protection could be disrupted by mismatched bilateral input in children with asymmetric hearing who require electric stimulation of the auditory nerve from a cochlear implant in their deaf ear and amplified acoustic sound from a hearing aid in their better ear (bimodal hearing). Cortical responses to bimodal stimulation were measured by electroencephalography in 34 bimodal users and 16 age-matched peers with normal hearing, and compared with the same measures previously reported for 28 age-matched bilateral implant users. Both auditory cortices increasingly favoured the better ear with delay to implanting the deaf ear; the time course mirrored that occurring with delay to bilateral implantation in unilateral implant users. Preference for the implanted ear tended to occur with ongoing implant use when hearing was poor in the non-implanted ear. Speech perception deteriorated with longer deprivation and poorer access to high-frequencies. Thus, cortical preference develops in children with asymmetric hearing but can be avoided by early provision of balanced bimodal stimulation. Although electric and acoustic stimulation differ, these inputs can work sympathetically when used bilaterally given sufficient hearing in the non-implanted ear.

Cortical hemispheric asymmetries are present at young ages and further develop into adolescence.

Specialization of the auditory cortices for pure tone listening may develop with age. In adults, the right hemisphere dominates when listening to pure tones and music; we thus hypothesized that (a) asymmetric function between auditory cortices increases with age and (b) this development is specific to tonal rather than broadband/non-tonal stimuli. Cortical responses to tone-bursts and broadband click-trains were recorded by multichannel electroencephalography in young children (5.1 ± 0.8 years old) and adolescents (15.2 ± 1.7 years old) with normal hearing. Peak dipole moments indicating activity strength in right and left auditory cortices were calculated using the Time Restricted, Artefact and Coherence source Suppression (TRACS) beamformer. Monaural click-trains and tone-bursts in young children evoked a dominant response in the contralateral right cortex by left ear stimulation and, similarly, a contralateral left cortex response to click-trains in the right ear. Responses to tone-bursts in the right ear were more bilateral. In adolescents, peak activity dominated in the right cortex in most conditions (tone-bursts from either ear and to clicks from the left ear). Bilateral activity was evoked by right ear click stimulation. Thus, right hemispheric specialization for monaural tonal stimuli begins in children as young as 5 years of age and becomes more prominent by adolescence. These changes were marked by consistent dipole moments in the right auditory cortex with age in contrast to decreases in dipole activity in all other stimulus conditions. Together, the findings reveal increasingly asymmetric function for the two auditory cortices, potentially to support greater cortical specialization with development into adolescence.

Cortical organization restored by cochlear implantation in young children with single sided deafness.

Early treatment of single sided deafness in children has been recommended to protect from neurodevelopmental preference for the better hearing ear and from social and educational deficits. A fairly homogeneous group of five young children (≤3.6 years of age) with normal right sided hearing who received a cochlear implant to treat deafness in their left ears were studied. Etiology of deafness was largely cytomegalovirus (n = 4); one child had an enlarged vestibular aqueduct. Multi-channel electroencephalography of cortical evoked activity was measured repeatedly over time at: 1) acute (0.5 ± 0.7 weeks); 2) early chronic (1.1 ± 0.2 months); and 3) chronic (5.8 ± 3.4 months) cochlear implant stimulation. Results indicated consistent responses from the normal right ear with marked changes in activity from the implanted left ear. Atypical distribution of peak amplitude activity from the implanted ear at acute stimulation marked abnormal lateralization of activity to the ipsilateral left auditory cortex and recruitment of extra-temporal areas including left frontal cortex. These abnormalities resolved with chronic implant use and contralateral aural preference emerged in both auditory cortices. These findings indicate that early implantation in young children with single sided deafness can rapidly restore bilateral auditory input to the cortex needed to improve binaural hearing.

Children With Single-Sided Deafness Use Their Cochlear Implant.

OBJECTIVES: To assess acceptance of a cochlear implant (CI) by children with single-sided deafness (SSD) as measured by duration of CI use across daily listening environments. DESIGN: Datalogs for 7 children aged 1.1 to 14.5 years (mean ± SD: 5.9 ± 5.9 years old), who had SSD and were implanted in their deaf ear, were anonymized and extracted from their CI processors. Data for all available follow-up clinical appointments were included, ranging from two to six visits. Measures calculated from each datalog included frequency and duration of time the coil disconnected from the internal device, average daily CI use, and both duration (hr/day) and percentage of CI use (% daily use) in different intensity ranges and environment types. Linear mixed effects regression analyses were used to evaluate the relationships between CI experience, daily CI use, frequency of coil-offs, and duration of coil-off time. Nonlinear regression analyses were used to evaluate CI use with age in different acoustic environments. RESULTS: Children with SSD used their CI on average 7.4 hr/day. Older children used their CI for longer periods of the day than younger children. Longitudinal data indicated consistent CI use from the date of CI activation. Frequency of coil-offs reduced with CI experience, but did not significantly contribute to hours of coil-off time. Children used their CI longest in environments that were moderately loud (50 to 70 dB A) and classified as containing speech-in-noise. Preschoolers tended to spend less time in quiet but more time in music than infants/toddlers and adolescents. CONCLUSIONS: Children with SSD consistently use their CI upon activation in a variety of environments commonly experienced by children. CI use in children with SSD resembles reported bilateral hearing aid use in children but is longer than reported hearing aid use in children with less severe unilateral hearing loss, suggesting that (1) the normal-hearing ear did not detract from consistent CI use; and (2) a greater asymmetry between ears presents a significant impairment that may facilitate device use to access bilateral sound.

Lateral graft type 1 tympanoplasty using AlloDerm for tympanic membrane reconstruction in children.

OBJECTIVES: To describe the lateral graft type 1 tympanoplasty technique using AlloDerm for tympanic membrane reconstruction in children and to compare its surgical and audiometric outcomes with the traditional underlay type 1 tympanoplasty. METHODS: The records of 34 consecutive children undergoing type 1 tympanoplasty between 2004 and 2005 were reviewed; 18 received lateral graft tympanoplasty with AlloDerm and 16 received underlay tympanoplasty (8 AlloDerm and 8 temporalis fascia). Pre- and post-surgical audiograms, speech reception threshold, closure rate and complication rate were evaluated using one-way and repeated measures ANOVAs. RESULTS: Children who underwent lateral graft type 1 tympanoplasty pre-operatively had larger tympanic membrane perforations, worse pure tone averages, air bone gaps and speech reception thresholds as compared with children undergoing underlay type 1 tympanoplasty (P<0.001). Pure tone averages and air bone gaps improved significantly with surgery in both lateral and underlay type 1 tympanoplasty groups (P<0.05), with both groups achieving comparable postoperative audiometric outcomes (P>0.01). The lateral graft group demonstrated a higher perforation closure rate (94%) as compared with both underlay groups (88%). Complication rates were virtually non-existent. CONCLUSIONS: Despite larger perforations and worse pre-operative audiometric scores, children who underwent lateral graft type 1 tympanoplasty achieved comparable postoperative audiometric results and perforation closure rates as compared with children who underwent underlay type 1 tympanoplasty. Results suggest that lateral graft type 1 tympanoplasty using AlloDerm is effective for tympanic membrane reconstruction in children and should be used when temporalis fascia is not available or the extent of the perforation limits its use.

Ethnicity and mutations in GJB2 (connexin 26) and GJB6 (connexin 30) in a multi-cultural Canadian paediatric Cochlear Implant Program.

OBJECTIVE: To determine the relationship between ethnicity and mutations in the GJB2 and GJB6 genes in multi-cultural patients enrolled in a Canadian paediatric Cochlear Implant Program. METHODS: Blood was analyzed from 65 paediatric cochlear implant users by direct sequencing of the coding region and intron/exon boundaries of the GBJ2 gene. Individuals heterozygous for one mutation in GJB2 or in whom mutations in GJB2 were not detected were analyzed for the common 342 kb deletion mutation D13S1830 in the GJB6 gene. Information regarding ethnicity of patients' families was obtained from patient records and/or interview. RESULTS: GJB2 mutations were found in 36.9% of paediatric cochlear implant users tested. Nine different GJB2 mutations were identified among individuals from 14 different countries of origin. Seventy-eight percent of all identified pathogenic GJB2 mutations were 35delG. Biallelic GJB2 mutations were found in 16 cochlear implant users (66.7% of GJB2 mutations). Three novel GJB2 sequence changes were identified: (1) a missense mutation T107C (L36P) in an individual of African decent; (2) a missense mutation G475T (D159Y) in an individual of Caribbean decent; (3) a regulatory region change 1-34C to T in an individual of African decent. GJB6-D13S1830 mutations were not found in any of the patients tested. Individuals of African, Caribbean and East Indian decent had different GJB2 mutations than the remainder of individuals tested. Patients of Asian, Italian, Spanish, Polish and Armenian decent were not found to carry mutations in GJB2 or the common GJB6-D13S1830 mutation. CONCLUSIONS: This study represents the largest number of biallelic GJB2 mutations isolated in a group of paediatric cochlear implant users to date. Numerous and diverse GJB2 mutations were found in this multi-cultural group of children. Even though GJB2 mutations have been widely reported in the literature, this discussion represents the first report of GJB2 mutations in a multi-ethnic population (Canadian), as compared with previous studies that investigated fairly homogeneous populations. The diversity of GJB2 mutations identified reinforces the importance of testing for changes in GJB2 by direct sequencing of the entire coding region rather than testing only for common mutations.

Congenital conductive hearing loss.

Congenital conductive hearing loss due to ossicular deformities can be treated by either rehabilitation with a hearing aid or surgical reconstruction. We present the results of exploratory tympanotomy performed in a large paediatric otolaryngology centre in 67 patients with non-serous congenital conductive hearing loss. Forty-two children had malformation of one or more ossicles without fixation of the stapes, and 19 had fixed stapes. In 26 cases, the surgeon decided not to perform surgical correction. Seven operated patients were lost to follow-up. As a group, 47 per cent of the patients who underwent reconstruction showed no significant benefit from surgery, with post-operative air-bone gaps (ABG) greater than 30 dB. Assessment of the results by pathology showed that 64 per cent of the patients with mobile stapes had an air-bone gap within 30 dB compared to only 33 per cent of the patients with fixed stapes. One patient sustained severe sensorineural loss after the procedure. Considering that exploratory tympanotomy is a relatively minimal, benign procedure but that findings during exploration may exclude the option of reconstruction (in 39 per cent of our patients), we suggest exploring the ear, but in a more realistic, informed way.