Congenital cytomegalovirus (cCMV) infection can cause fluctuating hearing loss and vestibulopathy. The pathogenesis is unknown. This report describes a 13-year old boy with cCMV and severe hearing... Show moreCongenital cytomegalovirus (cCMV) infection can cause fluctuating hearing loss and vestibulopathy. The pathogenesis is unknown. This report describes a 13-year old boy with cCMV and severe hearing loss in the right ear since age 3, presenting with fluctuating hearing loss in the left ear and vestibular symptoms. 3D fluid attenuated inversion recovery (FLAIR) MRI showed endolymphatic hydrops in the acutely affected ear. This is the first description of a child with cCMV subjected to this imaging technique, raising the question whether endolymphatic hydrops could play a role in the development of late-onset symptoms and demonstrating the possibilities of this MRI sequence. Show less
Dit proefschrift is gericht op het onderzoeken van verschillende aspecten van de huidige revalidatie rondomgehoorverlies. In de verschillende hoofdstukken wordt ingegaan op de selectiecriteria voor... Show moreDit proefschrift is gericht op het onderzoeken van verschillende aspecten van de huidige revalidatie rondomgehoorverlies. In de verschillende hoofdstukken wordt ingegaan op de selectiecriteria voor volwassen CI-kandidaten (hoofdstuk 2 en 3), de taalontwikkeling bij kinderen met een ABI (hoofdstuk 4), enverschillende ontwikkelingsuitkomsten na revalidatie voor kinderen met gehoorverlies, zoals het sociaalemotioneel functioneren (hoofdstuk 5) en het opleidingsniveau (hoofdstuk 6). Show less
Objective: To evaluate the long-term ipsi- and contralateral hearing of patients with a unilateral enlarged vestibular aqueduct (EVA). Study design: Multicenter retrospective cohort study. Setting:... Show moreObjective: To evaluate the long-term ipsi- and contralateral hearing of patients with a unilateral enlarged vestibular aqueduct (EVA). Study design: Multicenter retrospective cohort study. Setting: Three tertiary otology and audiology referral centers. Patients and diagnostic interventions: A total of 34 children with a unilateral enlarged vestibular aqueduct as identified on CT and/or MR imaging were evaluated with pure tone and speech perception audiometry. Mean outcome measures: Radiologic measurements of the vestibular aqueduct, ipsi- and contralateral hearing loss, ipsi- and contralateral hearing loss progression over time and DNA test results. Results: All patients in this cohort with unilateral EVA presented with hearing loss. Hearing loss was progressive in 38% of the ipsilateral ears. In 29% of the children, hearing loss was also found in the contralateral ear without EVA. In 90%, the contralateral hearing was stable, with a mean follow up of 4.2 years. We found a significant correlation between the severity of the hearing loss and the size of the EVA. A genetic diagnosis associated with EVA and/or SNHL was found in only 7%. Conclusion: About a third of the children with unilateral EVA are at risk of developing hearing loss in the contralateral ear. This indicates that at least in some patients with a unilateral EVA, a bilateral pathogenic process underlies the hearing loss, in contrary to what the imaging results suggest. These findings are important for counseling of EVA patients and their parents and have implications for follow up. Show less
Cochlear implants (CI) are implantable devices to rehabilitate hearing for people with severe to profound hearing loss. Over recent years, introduction of new designs have not lead to significant... Show moreCochlear implants (CI) are implantable devices to rehabilitate hearing for people with severe to profound hearing loss. Over recent years, introduction of new designs have not lead to significant improvements in outcomes. Tests to evaluate new sound coding strategies and recording methods are time-consuming, and yield results that are difficult to interpret due to a relatively low number of patients and a large heterogeneity in etiology. As an alternative approach to test new designs, a computational model can be used. In this thesis two computational models are presented and validated for simulation of peripheral responses in cochlear implant users. One model simulates spike times in response to electrical pulse trains. In this model, a three-dimensional volume conduction of the cochlea and an active nerve model were extended with stochasticity, adaptation, and accommodation. It can be used to simulate single fiber action potentials (SFAP) and evoked compound action potentials (ECAP). The second model simulates electrocochleography (eCochG) potentials as recorded intracochlearly. Both models can be used to test developments in sound coding and new recording methods. Furthermore, it can be tested how differences in the individuals’ auditory peripheral system contributes to differences in objective recordings obtained from the individuals. Show less
In a social environment composed mostly of people with typical hearing, deaf or hard of hearing (DHH) children experience social interactions differently from their typically hearing (TH) peers,... Show moreIn a social environment composed mostly of people with typical hearing, deaf or hard of hearing (DHH) children experience social interactions differently from their typically hearing (TH) peers, which could guide them towards different patterns for processing other people’s emotions. This thesis aimed to unravel whether hearing status affects how children encode, interpret, and react to others’ emotions in a social context, and whether their responses are associated with psychosocial functioning, using a variety of measures that included eye tracking, pupillometry, behavioral tasks, parent reports, and longitudinal follow-up. DHH children’s skills for perceiving others’ basic emotions were on par with their TH peers. Improved emotional functioning was associated with improved psychosocial functioning to a similar degree in DHH and TH children alike. Yet, DHH children still faced difficulties when they had to process an emotion with adequate knowledge about social rules and causes of emotions. Moreover, DHH children used a visual cue-based encoding strategy to compensate for ambiguous or unavailable information in social situations, and recruited more cognitive resources to process unfamiliar emotional expressions. The findings underscore the need to look into possible qualitative differences between typical and atypical development. These individual differences reflect compensatory strategies to support daily living, or signal a need for support in a certain domain. Show less
Experiencing shame and guilt is painful but these emotions have an important social function. They prevent socially inappropriate behaviors as they make us aware of norms and values that are... Show moreExperiencing shame and guilt is painful but these emotions have an important social function. They prevent socially inappropriate behaviors as they make us aware of norms and values that are necessary for creating and maintaining social harmony. Participation in the social world is crucial for the development of shame and guilt, and less access to the social world could therefore be detrimental to this development. In this thesis, the influence of access to the social world on the development of self-conscious emotions is examined in two groups with less access to the social world: adolescents with an autism spectrum disorder (ASD) and adolescents with hearing loss. In addition, this thesis aimed to unravel the longitudinal contributions of shame and guilt on the development of adolescent aggression. Adolescents with diminished access to the social world reported lower levels of shame and guilt. The longitudinal relationships applied to both adolescent with diminished access and typically developing adolescents. It was demonstrated that shame is a risk factor for the development of reactive aggression, and that guilt is an inhibiting force on the development of bullying and proactive aggression Show less
Linge, A. van; Os, R. van; Hoekstra, N.; Heijmen, B.; Stienstra, L.; Dallenga, A.; ... ; Romero, A.M. 2018
BackgroundAlthough stereotactic radiotherapy (SRT) for vestibular schwannoma has demonstrated excellent local control rates, hearing deterioration is often reported after treatment. We therefore... Show moreBackgroundAlthough stereotactic radiotherapy (SRT) for vestibular schwannoma has demonstrated excellent local control rates, hearing deterioration is often reported after treatment. We therefore wished to assess the change in hearing loss after SRT and to determine which patient, tumor and treatment-related factors influence deterioration.MethodsWe retrospectively analyzed progression of hearing loss in patients with vestibular schwannoma who had received stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) as a primary treatment between 2000 and 2014. SRS had been delivered as a single fraction of 12Gy, and patients treated with FSRT had received 30 fractions of 1.8Gy. To compare the effects of SRS and FSRT, we converted cochlear doses into EQD(2). Primary outcomes were loss of functional hearing, Gardner Robertson (GR) classes I and II, and loss of baseline hearing class. These events were used in Kaplan Meier plots and Cox regression. We also calculated the rate of change in Pure Tone Average (PTA) in dB per month elapsed after radiationa measure we use in linear regressionto assess the associations between the rate of change in PTA and age, pre-treatment hearing level, tumor size, dose scheme, cochlear dose, and time elapsed after treatment (time-to-first-audiogram).ResultsThe median follow-up was 36months for 67 SRS patients and 63months for 27 FSRT patients. Multivariate Cox regression and in linear regression both showed that the cochlear V90 was significantly associated with the progression of hearing loss. But although pre-treatment PTA correlated with rate of change in Cox regression, it did not correlate in linear regression. The time-to-first-audiogram was also significantly associated, indicating time dependency of the rate of change. None of the analysis showed a significant difference between dose schemes.ConclusionsWe found no significant difference between SRS and FSRT. As the deterioration in hearing after radiotherapy for vestibular schwannoma was associated with the cochlea V90, restricting the V90 may reduce progression of hearing loss. The association between loss of functional hearing and baseline PTA seems to be biased by the use of a categorized variable for hearing loss. Show less
For decades children with moderate hearing loss (MHL) were “forgotten children”. Most research focused on deaf children and the needs of children with MHL were underestimated. Children with MHL... Show moreFor decades children with moderate hearing loss (MHL) were “forgotten children”. Most research focused on deaf children and the needs of children with MHL were underestimated. Children with MHL have inconsistent access to linguistic input and social-emotional experiences and that place them at risk for developmental difficulties. In the present thesis the psychosocial functioning of toddlers with moderate hearing loss was examined within the context of the caregiving environment. No risk factors were found in the parent-child affective domain: Toddlers with MHL were affected by the emotions of others, they were affectively available to their parents and their parents to them, and their parents did not feel more parental stress than parents of toddlers without hearing loss. Challenges were found in the domain of meaningful social interactions: Toddlers with MHL had more difficulties in understanding the intentions of others and exchanged fewer social-communicative signals. The episodes of joint engagement during parent-child interactions were briefer and parents used less rich language during these interactions than parents of toddlers without hearing loss. To overcome these challenges parents could ready storybooks with their children. An interactive reading program for parents of toddlers with hearing loss showed to be effective in increasing joint engagement. Show less
The aim of this thesis was to study the link between hearing loss, language skills, and social functioning in deaf and hard of hearing (DHH) children. Sufficient language skills are an... Show moreThe aim of this thesis was to study the link between hearing loss, language skills, and social functioning in deaf and hard of hearing (DHH) children. Sufficient language skills are an essential prerequisite to develop appropriate communication skills, in order to join in conversations with others. Both their hearing loss and their diminished communication skills prevent DHH children from learning by observing their surroundings (incidental learning). As a result, DHH children showed more difficulty in understanding others’ thoughts and wishes (Theory of Mind or ToM). DHH teenagers reported to have difficulties with understanding others’ emotions and showed lower levels of prosocial behavior. Higher communication skills, but not language skills, were related to better ToM development and higher empathic abilities. Second, the role of early identification and intervention of hearing loss on the social-emotional development of DHH children was studied. This was illustrated in a longitudinal study showing that early cochlear implantation resulted in higher language and communication skills. In turn, these improved skills prevented the development of early signs of psychopathology. To conclude, this thesis shows that in order to stimulate the social-emotional development of DHH children, their opportunities for incidental learning have to be increased. Show less
Background: Radiation to the inner ear may lead to (irreversible) sensorineural hearing loss. The purpose of this study was to evaluate the long-term effect of radiotherapy on hearing in patients... Show moreBackground: Radiation to the inner ear may lead to (irreversible) sensorineural hearing loss. The purpose of this study was to evaluate the long-term effect of radiotherapy on hearing in patients treated with Intensity Modulated Radiation Therapy (IMRT), sparing the inner ear from high radiation dose as much as possible.Methods: Between 2003 and 2006, 101 patients with head and neck cancer were treated with IMRT. Audiometry was performed before, short-term, and long-term after treatment. Data were compared to normal hearing levels according to the International Organisation for Standardization (ISO). Statistical analysis was done using repeated measurements. None of the patients received chemotherapy.Results: In 36 patients an audiogram at long-term follow-up (median 7.6 years) was available. The mean dose to the cochlea was 17.8 Gy (1.0 66.6 Gy). A hearing deterioration of 1.8 dB at Pure Tone Average (PTA) 0.5 1 2 kHz (p = 0.11), 2.3 dB at PTA 1-2-4 kHz (p = 0.02), and 4.4 dB at PTA 8-10-12.5 kHz (p = 0.01) was found. According to the ISO, the expected age-related hearing loss was 2.7, 4.8, and 8.8 dB at PTA 0.5-1-2 kHz, 1-2-4 kHz, and 8-10-12.5 kHz, respectively.Conclusions: After IMRT with radiation dose constraint to the cochlea, potential long-term adverse effects of IMRT remained subclinical. The progressive hearing loss over time was mild and could be attributed to the natural effects of ageing. Therefore, we recommend that a dose constraint to the cochlea should be incorporated in the head and neck radiotherapy protocols. Show less
In comparison to hearing children, children with hearing loss more often experience social-emotional problems. This thesis aimed to assess whether this was also true for 1-to-5-year-old children... Show moreIn comparison to hearing children, children with hearing loss more often experience social-emotional problems. This thesis aimed to assess whether this was also true for 1-to-5-year-old children who had received a cochlear implant (CI) to remediate their hearing loss. In comparison to hearing peers, children with CI were found to be delayed on some but not all aspects of social-emotional functioning. No delays were found regarding social behavior or empathy. However, children with CI did show delays on theory of mind understanding and on the development of moral emotions. Against expectations, language skills did not have a major influence on children's social-emotional development. Parents in both groups had adopted similar parenting styles. Positive parenting was associated with better social-emotional functioning in children, whereas negative or uninvolved parenting were associated with lower social-emotional functioning. Based on this thesis we can conclude that a CI is beneficial for the social-emotional development of children with a hearing loss. However, these children are still at risk of developing delays in some areas of social-emotional functioning. Furthermore, we should not assume that children with CI who have a strong language development will also show a healthy social-emotional development. Show less
Children with hearing loss are at risk for developing psychopathology, which has detrimental consequences for academic and psychosocial functioning. Therefore, the aims of this thesis were to... Show moreChildren with hearing loss are at risk for developing psychopathology, which has detrimental consequences for academic and psychosocial functioning. Therefore, the aims of this thesis were to objectify levels of psychopathology in hearing-impaired children, and to investigate the influence of various risk and protective factors on psychopathology. The first result of this large multi-center study was that children with hearing loss were more prone to developing psychopathology than their normally hearing counterparts. The second outcome entailed that cochlear implants recipients equaled to or outperformed children with hearing aids involving their levels of psychopathology. Moreover, for several psychopathological symptoms, children that received cochlear implants were comparable to normally hearing children, which is a very positive outcome for the cochlear implant children. Thirdly, several factors that impacted levels of psychopathology were identified. For example, children at mainstream schools using well-developed spoken language, have low risks for developing psychopathology. On the basis of this thesis, it was concluded that it is not the severity of hearing loss that contributes to the genesis of psychopathology, but many other factors. This necessitates clinicians to always consider the heterogeneity of the HI population, in order to improve personalized screening and treatment trajectories. Show less
In this study socio-demographic, deafness-related and diagnosticcharacteristicsofhearingimpairedchildrenandadolescentsreferred to... Show moreIn this study socio-demographic, deafness-related and diagnosticcharacteristicsofhearingimpairedchildrenandadolescentsreferred to anationalmentalhealthservicefordeafandhardofhearingchildrenandadolescentswere examined. Socio-demographic and diagnosticcharacteristicswere compared to correspondingcharacteristicsofhearingreferred peers with identifiedmentalhealthproblems. The difference incharacteristicsbetween them andhearingreferred peers with identifiedmentalhealthproblems was analyzed. A total of 389deafandhardofhearingand 3361hearingchildrenandadolescentswas extracted from a database, all first referrals of patients of a center for child and adolescent psychiatryovera15-yearperiod. Withdeafandhardofhearingpatients we found higher rates of environmental stress, as indicated by conditions such as more one parent families (38.6% versus 25.8%), and more parents with a low educational level (44.2% versus 31.1%). Moreover,deafandhardofhearingpatients were older at their first referral (10.8 versus 9.4years) and had higher rates of pervasive developmental disorders (23.7% versus 12.3%) andmentalretardation (20.3% versus 3.9%). Within the target group ofdeafandhardofhearingpatients, most patients weredeaf(68.9%; 22.3% was severelyhardofhearing), relatively few (13.7%) had a non-syndromal hereditaryhearingimpairment, and more (21.3%) had a disabling physicalhealthcondition, especially those with a pervasive developmental disorder (42.6%). These findings illustrate both the complexity of the problems ofdeafandhardofhearingchildrenandadolescentsreferred to specialistmentalhealthservices, and the need for preventive interventions aimed at early recognition. Show less
Cochlear implants (CIs) are a well accepted treatment for hearing impaired people. In pre- and postoperative assessment of CI-candidates imaging plays an important role to analyze anatomy, rule out... Show moreCochlear implants (CIs) are a well accepted treatment for hearing impaired people. In pre- and postoperative assessment of CI-candidates imaging plays an important role to analyze anatomy, rule out pathology and determine intracochlear positioning and integrity of the implant. Developments in CI-design, differences in surgical approach and broadening of treatment indications have raised new questions to radiologists, which were the subject of several studies described in this thesis. For optimal, a-traumatic positioning of a CI precise information about the inner ear anatomy is mandatory. We describe the development, validation and application of a method for 3-dimensional medical image exploration of the inner ear. This renders a tool to obtain cochlear dimensions on clinical computer tomography (CT) images. This will be useful for patientspecific implantplanning. It also shows an anatomical substrate for cochlear trauma during insertion. For postoperative imaging we studied the value of multislice-CT for optimal visualization of the implant within the cochlea. Its role to evaluate operation technique and electrode design, to study frequency mapping and to assess cochlear trauma is discussed. Moreover an international consensus for an objective cochlear framework is presented, forming a common ground for clear and easy exchange of findings in scientific and clinical studies. Show less
