Yael Henkin

Cognitive function in idiopathic generalized epilepsy of childhood

This study evaluated the cognitive profiles of children with idiopathic generalized epilepsy (IGE), uniformly treated with valproic acid with well‐controlled seizures. Twenty‐four were neuropsychologically evaluated. They comprised: 14 females, 10 males: 12 with generalized tonic‐clonic seizures (GTCS), mean age 14y 4mo, SD 1y 7mo, range 12y to 16y 4 mo; 12 with absence seizures (AS) mean age 14y 5mo, SD 1y 10mo, range 11y to 16y 4mo, with intellectual abilities within the normal range and age‐appropriate scholastic skills, and 20 healthy controls (12 females, 8 males; mean age 14y 5mo, SD 1y 10mo, range 10y 7mo to 16y 7mo). As a group, children with IGE performed significantly poorer in all tests (non‐verbal and verbal attention, verbal learning and memory, word fluency, and controlled sequential fine motor responses) excluding nonverbal memory. Analysis according to type of seizure revealed that both patient groups (AS and GTCS) had an attention deficit, whereas only children with AS showed deficits in verbal learning and memory, word fluency, and controlled fine motor responses. These results suggest a long‐term risk of learning impairment for children with IGE, even if they have normal intelligence and their seizures are well controlled.

Changes over time in electrical stimulation levels and electrode impedance values in children using the Nucleus 24M cochlear implant

OBJECTIVE The present study was designed to evaluate changes in psycho-electric parameters, i.e. threshold levels, comfortable levels, dynamic range, and electrode impedance values during the 1st year post-implantation, in children using the Nucleus 24M cochlear implant system. METHODS The maps of 25 pre-lingual children programmed with ACE strategy in Monopolar 1 + 2 mode were examined at five time points: connection, 1, 3, 6, and 12 months post-initial stimulation. Maps and electrode impedance values were analyzed according to three cochlear segments: basal, medial, and apical. RESULTS Significant elevations of thresholds, comfortable levels, and dynamic range were found during the first few months of implant use. Specifically, threshold increased and dynamic range widened until the 3 months visit, whereas comfortable levels continued to increase until the 6 months visit, thereafter levels stabilized. Electrode impedance values decreased significantly from connection to the 1-month visit thereafter a stabilization of values was evident. In addition, thresholds and comfortable levels were found to be significantly lower in the apical segment, whereas dynamic range and electrode impedance values did not differ among the cochlear segments. CONCLUSIONS Significant changes in psycho-electric parameters and electrode impedance values were evident during the first 6 months of implant use. Given the important role of an optimal map for speech perception, frequent programming sessions during the first few months of implant use are essential.

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options

Deafness affects ∼2 in 1000 children and is one of the most common congenital impairments. Permanent hearing loss can be treated by fitting hearing aids. More severe to profound deafness is an indication for cochlear implantation. Although newborn hearing screening programs have increased the identification of asymmetric hearing loss, parents and caregivers of children with single-sided deafness are often hesitant to pursue therapy for the deaf ear. Delayed intervention has consequences for recovery of hearing. It has long been reported that asymmetric hearing loss/single-sided deafness compromises speech and language development and educational outcomes in children. Recent studies in animal models of deafness and in children consistently show evidence of an “aural preference syndrome” in which single-sided deafness in early childhood reorganizes the developing auditory pathways toward the hearing ear, with weaker central representation of the deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, with slow rates of improvement measured over time. Therefore, asymmetric hearing needs early identification and intervention. Providing early effective stimulation in both ears through appropriate fitting of auditory prostheses, including hearing aids and cochlear implants, within a sensitive period in development has a cardinal role for securing the function of the impaired ear and for restoring binaural/spatial hearing. The impacts of asymmetric hearing loss on the developing auditory system and on spoken language development have often been underestimated. Thus, the traditional minimalist approach to clinical management aimed at 1 functional ear should be modified on the basis of current evidence.

Evidence for Atypical Auditory Brainstem Responses in Young Children with Suspected Autism Spectrum Disorders.

Aim  The aim of this study was to characterize the auditory brainstem responses (ABRs) of young children with suspected autism spectrum disorders (ASDs) and compare them with the ABRs of children with language delay and with clinical norms.

Reduced auditory efferent activity in childhood selective mutism.

BACKGROUND Selective mutism is a psychiatric disorder of childhood characterized by consistent inability to speak in specific situations despite the ability to speak normally in others. The objective of this study was to test whether reduced auditory efferent activity, which may have direct bearings on speaking behavior, is compromised in selectively mute children. METHODS Participants were 16 children with selective mutism and 16 normally developing control children matched for age and gender. All children were tested for pure-tone audiometry, speech reception thresholds, speech discrimination, middle-ear acoustic reflex thresholds and decay function, transient evoked otoacoustic emission, suppression of transient evoked otoacoustic emission, and auditory brainstem response. RESULTS Compared with control children, selectively mute children displayed specific deficiencies in auditory efferent activity. These aberrations in efferent activity appear along with normal pure-tone and speech audiometry and normal brainstem transmission as indicated by auditory brainstem response latencies. CONCLUSIONS The diminished auditory efferent activity detected in some children with SM may result in desensitization of their auditory pathways by self-vocalization and in reduced control of masking and distortion of incoming speech sounds. These children may gradually learn to restrict vocalization to the minimal amount possible in contexts that require complex auditory processing.

Self-Reported Listening Habits and Enjoyment of Music Among Adult Cochlear Implant Recipients

Objectives: We sought to assess the associations between self-reported listening habits and enjoyment of music, and the following variables: Age at implantation, gender, prelingual versus postlingual deafness, duration of deafness, duration of cochlear implant (CI) use, type of CI, speech coding strategy, and speech perception abilities. Methods: A questionnaire on listening habits and enjoyment of music before the onset of deafness and after implantation was sent to 85 adult CI recipients who had been using the devices for at least 6 months. Results: Of the 53 responders, 39 (73.6%) listened to music after implantation. Listening to music was not significantly related to age at implantation, gender, duration of deafness, duration of CI use, type of CI device, speech coding strategy, or open-set speech perception abilities. The 14 nonlisteners were postlingually deafened. The ratings of enjoyment were the same for 22.6% of patients, improved for 26.4%, and worse for 50.9%. Only 2 of 13 patients who played a musical instrument and 14 of 24 patients who sang before the onset of deafness resumed their musical activities. Conclusions: Despite the decline in listening habits and in the enjoyment of music after cochlear implantation, most patients do listen to music. The changes in listening habits and enjoyment were not related to the selected background variables.

A longitudinal study of electrical stimulation levels and electrode impedance in children using the clarion cochlear implant

Conclusions. Electrical stimulation levels and electrode impedance values (EIVs) in children using the Clarion cochlear implant (CI) programmed with CIS strategy stabilized after 3 months of implant use. The data presented here may be useful as a general guideline for the programming of infants and young children and may further be of help for the identification of patients who fall outside the “average” range. Objectives. The purpose of the present study was to evaluate changes in electrical stimulation levels, i.e. threshold (T) levels, comfortable (M) levels, dynamic range (DR), and EIVs during the first 18 months of implant use, in children using the Clarion CI. Materials and methods. The maps of 18 pre-lingual children (mean age at implantation 4.2 years; range 1–8), using the Enhanced Bipolar 1.2 or Bipolar standard electrode with the S-Series speech processor programmed with CIS strategy, were examined at five time points: connection, and 3, 6, 12, and 18 months post-initial stimulation. T levels, M levels, DR and EIVs were analyzed according to four cochlear segments: apical, apical-medial, medial-basal, and basal. Results. During the first 3 months of implant use T levels increased to some extent, whereas M levels and DR increased significantly. From 3 months and through the entire follow-up, T and M levels as well as DR were stable. EIVs of current carrying electrodes decreased significantly from connection to the 3-month visit; thereafter a stabilization of values was evident. Electrical stimulation levels and EIVs did not differ among the cochlear segments during the entire follow-up.

Lateralized enhancement of auditory cortex activity and increased sensitivity to self-generated sounds

Performing actions with auditory consequences modulates the response in auditory cortex to otherwise identical stimuli passively heard. Such modulation has been suggested to occur through a corollary discharge sent from the motor cortex during voluntary actions. However, the relationship between the effector used to generate the sound, type of modulation and changes in perceptual sensitivity are unclear. Here we use functional magnetic resonance imaging on healthy subjects and demonstrate bilateral enhancement in the auditory cortex to self-generated versus externally generated sounds. Furthermore, we find that this enhancement is stronger when the sound-producing hand is contralateral to the auditory cortex. At the behavioural level, binaural hearing thresholds are lower for self-generated sounds and monaural thresholds are lower for sounds triggered by the hand ipsilateral to the stimulated ear. Together with functional connectivity analysis, our results suggest that a corollary discharge sent from active motor cortex enhances activity in the auditory cortex and increases perceptual sensitivity in a lateralized manner.

Reduced Auditory Processing Capacity during Vocalization in Children with Selective Mutism

BACKGROUND Because abnormal Auditory Efferent Activity (AEA) is associated with auditory distortions during vocalization, we tested whether auditory processing is impaired during vocalization in children with Selective Mutism (SM). METHODS Participants were children with SM and abnormal AEA, children with SM and normal AEA, and normally speaking controls, who had to detect aurally presented target words embedded within word lists under two conditions: silence (single task), and while vocalizing (dual task). To ascertain specificity of auditory-vocal deficit, effects of concurrent vocalizing were also examined during a visual task. RESULTS Children with SM and abnormal AEA showed impaired auditory processing during vocalization relative to children with SM and normal AEA, and relative to control children. This impairment is specific to the auditory modality and does not reflect difficulties in dual task per se. CONCLUSIONS The data extends previous findings suggesting that deficient auditory processing is involved in speech selectivity in SM.

Hearing Aid Satisfaction and Use in the Advanced Digital Era

To evaluate satisfaction ratings and use patterns of advanced digital hearing aids (HAs) in a group of hearing‐impaired adults by means of self‐report questionnaires.