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
Hearing loss in patients with vestibular schwannoma (VS) is commonly attributed to mechanical compression of the auditory nerve, though recent studies suggest that this retrocochlear pathology may... Show moreHearing loss in patients with vestibular schwannoma (VS) is commonly attributed to mechanical compression of the auditory nerve, though recent studies suggest that this retrocochlear pathology may be augmented by cochlear damage. Although VS-associated loss of inner hair cells, outer hair cells, and spiral ganglion cells has been reported, it is unclear to what extent auditory-nerve peripheral axons are damaged in VS patients. Understanding the degree of damage VSs cause to auditory nerve fibers (ANFs) is important for accurately modeling clinical outcomes of cochlear implantation, which is a therapeutic option to rehabilitate hearing in VS-affected ears. A retrospective analysis of human temporal-bone histopathology was performed on archival specimens from the Massachusetts Eye and Ear collection. Seven patients met our inclusion criteria based on the presence of sporadic, unilateral, untreated VS. Tangential sections of five cochlear regions were stained with hematoxylin and eosin, and adjacent sections were stained to visualize myelinated ANFs and efferent fibers. Following confocal microscopy, peripheral axons of ANFs within the osseous spiral lamina were quantified manually, where feasible, and with a "pixel counting " method, applicable to all sections. ANF density was substantially reduced on the VS side compared to the unaffected contralateral side. In the upper basal turn, a significant difference between the VS side and unaffected contralateral side was found using both counting methods, corresponding to the region tuned to 20 0 0 Hz. Even spiral ganglion cells (SGCs) contralateral to VS were affected by the tumor as the majority of contralateral SGC counts were below average for age. This observation provides histological insight into the clinical observation that unilateral vestibular schwannomas pose a long-term risk of progression of hearing loss in the contralateral ear as well. Our pixel counting method for ANF quantification in the osseous spiral lamina is applicable to other pathologies involving sensorineural hearing loss. Future research is needed to classify ANFs into morphological categories, accurately predict their electrical properties, and use this knowledge to inform optimal cochlear implant programming strategies. 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
Objectives: For children to understand the emotional behavior of others, the first two steps involve emotion encoding and emotion interpreting, according to the Social Information Processing model.... Show moreObjectives: For children to understand the emotional behavior of others, the first two steps involve emotion encoding and emotion interpreting, according to the Social Information Processing model. Access to daily social interactions is prerequisite to a child acquiring these skills, and barriers to communication such as hearing loss impede this access. Therefore, it could be challenging for children with hearing loss to develop these two skills. The present study aimed to understand the effect of prelingual hearing loss on children's emotion understanding, by examining how they encode and interpret nonverbal emotional cues in dynamic social situations. Design: Sixty deaf or hard-of-hearing (DHH) children and 71 typically hearing (TH) children (3-10 years old, mean age 6.2 years, 54% girls) watched videos of prototypical social interactions between a target person and an interaction partner. At the end of each video, the target person did not face the camera, rendering their facial expressions out of view to participants. Afterward, participants were asked to interpret the emotion they thought the target person felt at the end of the video. As participants watched the videos, their encoding patterns were examined by an eye tracker, which measured the amount of time participants spent looking at the target person's head and body and at the interaction partner's head and body. These regions were preselected for analyses because they had been found to provide cues for interpreting people's emotions and intentions. Results: When encoding emotional cues, both the DHH and TH children spent more time looking at the head of the target person and at the head of the interaction partner than they spent looking at the body or actions of either person. Yet, compared with the TH children, the DHH children looked at the target person's head for a shorter time (b = -0.03, p = 0.030), and at the target person's body (b = 0.04, p = 0.006) and at the interaction partner's head (b = 0.03, p = 0.048) for a longer time. The DHH children were also less accurate when interpreting emotions than their TH peers (b = -0.13, p = 0.005), and their lower scores were associated with their distinctive encoding pattern. Conclusions: The findings suggest that children with limited auditory access to the social environment tend to collect visually observable information to compensate for ambiguous emotional cues in social situations. These children may have developed this strategy to support their daily communication. Yet, to fully benefit from such a strategy, these children may need extra support for gaining better social-emotional knowledge. Show less
Empathy enables people to share, understand, and show concern for others' emotions. However, this capacity may be more difficult to acquire for children with hearing loss, due to limited social... Show moreEmpathy enables people to share, understand, and show concern for others' emotions. However, this capacity may be more difficult to acquire for children with hearing loss, due to limited social access, and the effect of hearing on empathic maturation has been unexplored. This four-wave longitudinal study investigated the development of empathy in children with and without hearing loss, and how this development is associated with early symptoms of psychopathology. Seventy-one children with hearing loss and cochlear implants (CI), and 272 typically-hearing (TH) children, participated (aged 1-5 years at Time 1). Parents rated their children's empathic skills (affective empathy, attention to others' emotions, prosocial actions, and emotion acknowledgment) and psychopathological symptoms (internalizing and externalizing behaviors). Children with CI and TH children were rated similarly on most of the empathic skills. Yet, fewer prosocial actions were reported in children with CI than in TH children. In both groups, affective empathy decreased with age, while prosocial actions and emotion acknowledgment increased with age and stabilized when children entered primary schools. Attention to emotions increased with age in children with CI, yet remained stable in TH children. Moreover, higher levels of affective empathy, lower levels of emotion acknowledgment, and a larger increase in attention to emotions over time were associated with more psychopathological symptoms in both groups. These findings highlight the importance of social access from which children with CI can learn to process others' emotions more adaptively. Notably, interventions for psychopathology that tackle empathic responses may be beneficial for both groups, alike. 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
Objectives: The impact of the newly introduced cochlear implantation criteria of the United Kingdom and Flanders (Dutch speaking part of Belgium) was examined in the patient population of a... Show moreObjectives: The impact of the newly introduced cochlear implantation criteria of the United Kingdom and Flanders (Dutch speaking part of Belgium) was examined in the patient population of a tertiary referral center in the Netherlands. We compared the patients who would be included/excluded under the new versus old criteria in relation to the actual improvement in speech understanding after implantation in our center. We also performed a sensitivity analysis to examine the effectiveness of the different preoperative assessment approaches used in the United Kingdom and Flanders. Design: The selection criteria were based on preoperative pure-tone audiometry at 0.5, 1, 2, and 4 kHz and a speech perception test (SPT) with and without best-aided hearing aids. Postoperatively, the same SPT was conducted to assess the benefit in speech understanding. Results: The newly introduced criteria in Flanders and the United Kingdom were less restrictive, resulting in greater percentages of patients implanted with CI (increase of 30%), and sensitivity increase of 31%. The preoperative best-aided SPT, used by both countries, had the highest diagnostic ability to indicate a postoperative improvement of speech understanding. We observed that patient selection was previously dominated by the pure-tone audiometry criteria in both countries, whereas speech understanding became more important in their new criteria. Among patients excluded by the new criteria, seven of eight (the United Kingdom and Flanders) did exhibit improved postoperative speech understanding. Conclusions: The new selection criteria of the United Kingdom and Flanders led to increased numbers of postlingually deafened adults benefitting from CI. The new British and Flemish criteria depended on the best-aided SPT with the highest diagnostic ability. Notably, the new criteria still led to the rejection of candidates who would be expected to gain considerably in speech understanding after implantation. Show less
Objectives: To compare the quality of life (QoL) of children with hearing loss (HL) and children with normal hearing (NH) and to examine how the QoL of children with HL changes over time,... Show moreObjectives: To compare the quality of life (QoL) of children with hearing loss (HL) and children with normal hearing (NH) and to examine how the QoL of children with HL changes over time, considering language skills, type of hearing device, degree of HL, and type of education.Methods and materials: This longitudinal study included 62 children with HL and their parents. Developmental outcome data were collected at two time points, when the mean ages of the children were 4 and 11 years. The Pediatric Quality of Life (PedsQL (TM)) questionnaire, which includes assessments of Physical, Emotional, Social, and School functioning, was completed by parents at both time points and by the children with HL at the second time point. Receptive and expressive language skills at 4 years were assessed by the Reynell Developmental Language Scale. Results were compared with a Dutch normative sample.Results: The QoL of children with HL was similar to that of children with NH at both time points on two of the four QoL scales, Emotional and Physical functioning. On the other two scales, Social and School functioning, children with HL who attended special education and children who switched to mainstream education showed lower scores than children with HL who were consistently in mainstream education and lower scores than children with NH. The School QoL of children with HL decreased over time, as did the School QoL of children with NH. Social QoL of children with cochlear implants decreased over time, but this was not the case in children with hearing aids. Language skills and the degree of HL did not clinically improve the QoL over time of preschool children with HL.Conclusions: The QoL of children with HL in mainstream education and the Physical and Emotional QoL of all children with HL were satisfactory. It is essential to develop specific guidance regarding school activities for children with HL in special education and for children with HL who switch to mainstream education in order to increase their social QoL. 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
Ketelaar, L.; Wiefferink, C.H.; Frijns, J.H.M.; Rieffe, C. 2017
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 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
