Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 37
Filtrar
1.
Hear Res ; 450: 109076, 2024 Jul 05.
Artigo em Inglês | MEDLINE | ID: mdl-38991628

RESUMO

As part of a longitudinal study regarding the benefit of early cochlear implantation for children with single-sided deafness, the current work explored the children's daily device use, potential barriers to full-time device use, and the children's ability to understand speech with the cochlear implant (CI). Data were collected from 20 children with prelingual SSD who received a CI before the age of 2.5 years, from the initial activation of the sound processor until the children were 4.8 to 11.0 years old. Daily device use was extracted from the CI's data logging, while word perception in quiet was assessed using direct audio input to the children's sound processor. The children's caregivers completed a questionnaire about habits, motivations, and barriers to device use. The children with SSD and a CI used their device on average 8.3 h per day, corresponding to 63 % of their time spent awake. All children except one could understand speech through the CI, with an average score of 59 % on a closed-set test and 73 % on an open-set test. More device use was associated with higher speech perception scores. Parents were happy with their decision to pursue a CI for their child. Certain habits, like taking off the sound processor during illness, were associated with lower device use. Providing timely counselling to the children's parents, focused on SSD-specific challenges, may be helpful to improve daily device use in these children.

2.
Artigo em Inglês | MEDLINE | ID: mdl-38977473

RESUMO

We report a case of a severe ear infection in a 35-year-old man treated with ixekizumab for psoriasis. Ixekizumab is a humanized monoclonal antibody that selectively prevents the interaction between interleukin 17 A and its receptor. Biologicals like ixekizumab are used to achieve symptom relief in autoimmune diseases including psoriasis. Unlike the mild upper respiratory tract infections usually described as side-effects of this treatment, we report a case of a patient who presented with a severe otitis media, complicated with a facial paresis and nasopharyngeal abscess. To the best of our knowledge, this is the first case presenting a severe, complicated ear infection as a possible side effect of ixekizumab. We conclude that when using ixekizumab, vigilance for upper airway infections is needed and if necessary, interruption of therapy should be considered. However, further research is needed to confirm this hypothesis.

3.
Int J Pediatr Otorhinolaryngol ; 182: 112017, 2024 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-38908259

RESUMO

OBJECTIVES: Increased neonatal referral rate of conductive hearing loss (CHL) related to otitis media with effusion (OME) following universal neonatal hearing screening (UNHS) may cause an unnecessary clinical, emotional, and financial burden. This study analyzes the long-term, audiological, and medical characteristics of CHL associated with OME in neonates in order to establish a standardized protocol following technology-driven improvements in detection and referral rates in UNHS. METHODS: A retrospective study of all neonates with OME-related CHL referred to the University Hospital of Leuven (Belgium) after failing UNHS with the MAICO devices between January 1, 2013 and December 31, 2021 was performed. Follow-up consultations, auditory tests, referral side, birth month, hearing loss degree, underlying pathologies and risk factors, time to normalization, and need for ventilation tubes were assessed. RESULTS: The incidence of CHL related to OME was stable between 2013 and 2021. Of all referred infants with OME, 52.3 % demonstrated spontaneous recovery. The average time to hearing normalization was significantly longer in children with underlying congenital pathologies compared to those without. Moreover, 74.4 % of these children received ventilation tubes compared to 32.0 % of children without underlying pathologies. No correlation was found between the incidence of OME-related CHL with either a hearing loss degree, admission to neonatal intensive care, or history of a nasogastric feeding tube. CONCLUSIONS: In children who failed UNHS due to OME, hearing recovers spontaneously without surgical intervention in 2/3 of the infants without underlying conditions within one year. In children with underlying congenital disorders, the time to hearing recovery is longer and the risk for surgical intervention is higher, underlining the need for implementing a UNHS standardized protocol.

4.
J Belg Soc Radiol ; 108(1): 11, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38371367

RESUMO

Tophaceous gout can rarely present in the middle ear as a mass-like lesion, causing conductive hearing loss. Noncontrast high-resolution computed tomography (HRCT) of the temporal bone plays a significant role in the diagnosis. Awareness of this condition among radiologists is important since it presents a distinctive appearance on HRCT. We present a case of tophaceous gout of the middle ear diagnosed with photon-counting computed tomography (PCCT). Teaching point: The presence of a partially calcified mass with a semolina-like appearance within the middle ear is highly suggestive of tophaceous gout, even in the presence of normal serum uric acid levels.

5.
Eur Arch Otorhinolaryngol ; 281(7): 3433-3441, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38180608

RESUMO

PURPOSE:  Vestibular implant electrode positioning close to the afferent nerve fibers is considered to be key for effective and selective electrical stimulation. However, accurate positioning of vestibular implant electrodes inside the semicircular canal ampullae is challenging due to the inability to visualize the target during the surgical procedure. This study investigates the accuracy of a new surgical protocol with real-time fluoroscopy and intraoperative CT imaging, which facilitates electrode positioning during vestibular implant surgery. METHODS:  Single-center case-controlled cohort study with a historic control group at a tertiary referral center. Patients were implanted with a vestibulocochlear implant, using a combination of intraoperative fluoroscopy and cone beam CT imaging. The control group consisted of five patients who were previously implanted with the former implant prototype, without the use of intraoperative imaging. Electrode positioning was analyzed postoperatively with a high-resolution CT scan using 3D slicer software. The result was defined as accurate if the electrode position was within 1.5 mm of the center of the ampulla. RESULTS: With the new imaging protocol, all electrodes could be positioned within a 1.5 mm range of the center of the ampulla. The accuracy was significantly higher in the study group with intraoperative imaging (21/21 electrodes) compared to the control group without intraoperative imaging (10/15 electrodes), (p = 0.008). CONCLUSION:  The combined use of intraoperative fluoroscopy and CT imaging during vestibular implantation can improve the accuracy of electrode positioning. This might lead to better vestibular implant performance.


Assuntos
Tomografia Computadorizada de Feixe Cônico , Humanos , Fluoroscopia/métodos , Feminino , Masculino , Pessoa de Meia-Idade , Idoso , Estudos de Casos e Controles , Tomografia Computadorizada de Feixe Cônico/métodos , Eletrodos Implantados , Adulto , Tomografia Computadorizada por Raios X/métodos , Cirurgia Assistida por Computador/métodos
6.
JAMA Otolaryngol Head Neck Surg ; 150(1): 30-38, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-37917050

RESUMO

Importance: Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking. Objective: To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss. Design, Setting, and Participants: This multicenter cohort study included patients from 6 secondary and tertiary hospitals enrolled in the Flemish CMV registry (Belgium). Newborns with untreated cCMV infection with at least 4-year audiological follow-up were included. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Data were collected for 15 years (January 1, 2007, to February 7, 2022) and analyzed from September 26, 2022, to January 16, 2023. Main Outcomes and Measures: Primary outcome was hearing evolution (per-ear analysis; described as stable hearing, improvement, or deterioration). The association of gestational characteristics, clinical findings, timing of seroconversion, viral load, and hearing status at birth with hearing evolution was investigated using effect sizes (Cramer V, odds ratio [OR], or Hedges g). Results: Of the 387 children, 205 of 385 with nonmissing data were male (53.2%), 113 (29.2%) had a symptomatic infection, and 274 (70.8%) had an asymptomatic infection. Every child was 4 years or older at final hearing evaluation. A total of 701 of 774 ears (90%) showed stable hearing (normal hearing or stable hearing loss since birth) over time. Late-onset hearing loss (normal hearing at birth followed by hearing loss) was present in 43 of 683 ears (6.3%). Among children with hearing loss present at birth, 24 of 34 ears (70.6%) had hearing deterioration, and 6 of 91 ears (6.6%) had hearing improvement. Prematurity was associated with a higher chance of hearing improvement (OR, 12.80; 95% CI, 2.03-80.68). Late-onset hearing loss was more prevalent in a first trimester infection (OR, 10.10; 95% CI, 2.90-34.48). None of the 104 ears of children with a third trimester seroconversion developed late-onset hearing loss. Conclusions and Relevance: Findings of this cohort study support that ongoing audiological follow-up for untreated children with congenital hearing loss is important, as the majority of patients had hearing deterioration. The timing of seroconversion was associated with the risk of developing late-onset hearing loss. These insights can aid in parental counseling, patient stratification, and follow-up. Future research should focus on the effect of treatment, the influence of determined risk factors, and the study of eventual new risk factors in patients at high risk to develop hearing loss.


Assuntos
Infecções por Citomegalovirus , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Lactente , Criança , Recém-Nascido , Humanos , Masculino , Feminino , Estudos de Coortes , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/congênito , Infecções por Citomegalovirus/tratamento farmacológico , Audição , Perda Auditiva Neurossensorial/complicações , Fatores de Risco , Perda Auditiva/complicações
7.
Sci Rep ; 13(1): 17828, 2023 10 19.
Artigo em Inglês | MEDLINE | ID: mdl-37857664

RESUMO

Prelingual single-sided deafness (SSD) not only affects children's hearing skills, but can also lead to speech-language delays and academic underachievement. Early cochlear implantation leads to improved spatial hearing, but the impact on language development is less studied. In our longitudinal study, we assessed the language skills of young children with SSD and a cochlear implant (CI). In particular, we investigated their narrative skills in comparison to two control groups: children with SSD without a CI, and children with bilateral normal hearing. We found that children with SSD and a CI performed in line with their normal-hearing peers with regard to narrative and verbal short-term memory skills. Children with SSD without a CI had worse narrative (group difference = - 0.67, p = 0.02) and verbal short-term memory (group difference = - 0.68, p = 0.03) scores than the implanted group. Verbal short-term memory scores and grammar scores each correlated positively with narrative scores across all groups. Early grammar scores (at 2-3 years of age) could partially predict later narrative scores (at 4-6 years of age). These results show that young children with prelingual SSD can benefit from early cochlear implantation to achieve age-appropriate language skills. They support the provision of a CI to children with prelingual SSD.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Percepção da Fala , Humanos , Criança , Pré-Escolar , Estudos Longitudinais , Surdez/cirurgia , Audição
8.
JAMA Otolaryngol Head Neck Surg ; 149(2): 122-130, 2023 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-36580312

RESUMO

Importance: With a prevalence between 0.2% and 6.1% of all live births, congenital cytomegalovirus (cCMV) infection is a major cause of congenital nonhereditary sensorineural hearing loss. Despite the large amount of research on cCMV-related hearing loss, it is still unclear which newborns are at risk of hearing loss. Objective: To identify independent risk factors for cCMV-related congenital hearing loss and predictors of hearing loss severity at birth. Design, Setting, and Participants: This cross-sectional study of newborns with cCMV infection used data included in the Flemish CMV registry that was collected from 6 secondary and tertiary hospitals in Flanders, Belgium, over 15 years (January 1, 2007, to February 7, 2022). Data were analyzed March 3 to October 19, 2022. Patients were included in the study after confirmed diagnosis of cCMV infection and known hearing status at birth. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Main Outcomes and Measures: Primary outcome was hearing status at birth. Clinical, neurological, and laboratory findings along with the timing of seroconversion and blood viral load were separately considered as risk factors. Binary logistic regression was performed to identify independent risk factors for congenital hearing loss in newborns with cCMV. Effect sizes were measured using Hedges g, odds ratio, or Cramer V. Results: Of the 1033 newborns included in the study (553 of 1024 [54.0%] boys), 416 (40.3%) were diagnosed with symptomatic cCMV infection and 617 (59.7%) with asymptomatic cCMV infection. A total of 15.4% of the patients (n = 159) presented with congenital hearing loss; half of them (n = 80 [50.3%]) had isolated hearing loss. The regression model revealed 3 independent risk factors for congenital hearing loss: petechiae at birth (adjusted odds ratio [aOR], 6.7; 95% CI, 1.9-23.9), periventricular cysts on magnetic resonance imaging (MRI; aOR, 4.6; 95% CI, 1.5-14.1), and seroconversion in the first trimester (aOR, 3.1; 95% CI, 1.1-9.3). Lower viral loads were seen in patients with normal hearing compared with those with congenital hearing loss (median [IQR] viral load, 447.0 [39.3-2345.8] copies per milliliter of sample [copies/mL] vs 1349.5 [234.3-14 393.0] copies/mL; median difference, -397.0 [95% CI, -5058.0 to 174.0] copies/mL). Conclusions and Relevance: Findings of this cross-sectional study suggest that newborns with cCMV infection and petechiae at birth, periventricular cysts on MRI, or a seroconversion in the first trimester had a higher risk of congenital hearing loss. Clinicians may use these risk factors to counsel parents in the prenatal and postnatal periods about the risk of congenital hearing loss. Moreover, linking clinical features to hearing loss may provide new insights into the pathogenesis of cCMV-related hearing loss. The importance of viral load as a risk factor for congenital hearing loss remains unclear.


Assuntos
Cistos , Infecções por Citomegalovirus , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Masculino , Gravidez , Feminino , Recém-Nascido , Humanos , Criança , Estudos Transversais , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/epidemiologia , Infecções por Citomegalovirus/diagnóstico , Perda Auditiva/complicações , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/diagnóstico , Citomegalovirus/isolamento & purificação , Fatores de Risco , Cistos/complicações
9.
Sci Rep ; 12(1): 9376, 2022 06 07.
Artigo em Inglês | MEDLINE | ID: mdl-35672363

RESUMO

Individuals with single-sided deafness (SSD) have no access to binaural hearing, which limits their ability to localize sounds and understand speech in noisy environments. In addition, children with prelingual SSD are at risk for neurocognitive and academic difficulties. Early cochlear implantation may lead to improved hearing outcomes by restoring bilateral hearing. However, its longitudinal impact on the development of children with SSD remains unclear. In the current study, a group of young children with prelingual SSD received a cochlear implant at an early age. From the age of four, the children's spatial hearing skills could be assessed using a spatial speech perception in noise test and a sound localization test. The results are compared to those of two control groups: children with SSD without a cochlear implant and children with bilateral normal hearing. Overall, the implanted group exhibited improved speech perception in noise abilities and better sound localization skills, compared to their non-implanted peers. On average, the children wore their device approximately nine hours a day. Given the large contribution of maturation to the development of spatial hearing skills, further follow-up is important to understand the long-term benefit of a cochlear implant for children with prelingual SSD.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Localização de Som , Percepção da Fala , Criança , Pré-Escolar , Implante Coclear/métodos , Surdez/cirurgia , Humanos , Resultado do Tratamento
10.
Pediatrics ; 150(1)2022 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-35698886

RESUMO

OBJECTIVES: Although vestibular deficits are more prevalent in hearing-impaired children and can affect their development on many levels, a pediatric vestibular assessment is still uncommon in clinical practice. Since early detection may allow for timely intervention, this pioneer project has implemented a basic vestibular screening test for each six-month-old hearing-impaired infant in Flanders, Belgium. This study aims to report the vestibular screening results over a period of three years and to define the most important risk factors for abnormal vestibular screening results. METHODS: Cervical Vestibular Evoked Myogenic Potentials with bone-conduction were used as a vestibular screening tool in all reference centers affiliated to the Universal Newborn Hearing Screening Program in Flanders. From June 2018 until June 2021, 254 infants (mean age: 7.4 months, standard deviation: 2.4 months) with sensorineural hearing loss were included. RESULTS: Overall, abnormal vestibular screening results were found in 13.8% (35 of 254) of the infants. The most important group at risk for abnormal vestibular screening results were infants with unilateral or bilateral severe to profound sensorineural hearing loss (20.8%, 32 of 154) (P < .001, odds ratio = 9.16). Moreover, abnormal vestibular screening results were more prevalent in infants with hearing loss caused by meningitis (66.7%, 2 of 3), syndromes (28.6%, 8 of 28), congenital cytomegalovirus infection (20.0%, 8 of 40), and cochleovestibular anomalies (19.2%, 5 of 26). CONCLUSIONS: The vestibular screening results in infants with sensorineural hearing loss indicate the highest risk for vestibular deficits in severe to profound hearing loss, and certain underlying etiologies of hearing loss, such as meningitis, syndromes, congenital cytomegalovirus, and cochleovestibular anomalies.


Assuntos
Infecções por Citomegalovirus , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Potenciais Evocados Miogênicos Vestibulares , Vestíbulo do Labirinto , Criança , Infecções por Citomegalovirus/complicações , Perda Auditiva/diagnóstico , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Humanos , Lactente , Recém-Nascido , Síndrome
11.
JAMA Netw Open ; 4(8): e2122591, 2021 08 02.
Artigo em Inglês | MEDLINE | ID: mdl-34432009

RESUMO

Importance: Pediatric single-sided deafness (SSD) can seriously affect development, causing impaired spatial hearing skills, speech-language delays, and academic underachievement. Early cochlear implantation likely improves hearing-related outcomes, but its association with language development remains unclear. Objective: To investigate whether early cochlear implantation is associated with language outcomes for children with prelingual SSD. Design, Setting, and Participants: The Cochlear Implant for Children and One Deaf Ear study was initiated in 2015 and recruited participants at 4 academic hospitals in Flanders, Belgium, through 2019. This cohort study included 3 groups of children aged 2 to 5 years: children with SSD and a cochlear implant, children with SSD without a cochlear implant, and a control group with normal hearing. Language and hearing skills were assessed 1 to 2 times per year until the age of 10 years. Study completion rates were high (82%). Data analysis was performed from October to December 2020. Exposure: Unilateral cochlear implant. Main Outcomes and Measures: Longitudinal vocabulary, grammar, and receptive language scores. The implanted group was hypothesized to outperform the nonimplanted group on all language tests. Results: During the recruitment period, 47 children with prelingual SSD without additional disabilities were identified at the participating hospitals. Fifteen of the 34 children with an intact auditory nerve received a cochlear implant (44%, convenience sample). Sixteen of the remaining children were enrolled in the SSD control group (50%). Data from 61 children (mean [SD] age at the time of enrollment, 2.08 [1.34] years; 26 girls [42%]) were included in the analysis: 15 children with SSD and a cochlear implant, 16 children with SSD without a cochlear implant, and 30 children with normal hearing. Children with SSD and a cochlear implant performed in line with their peers with normal hearing with regard to grammar. In contrast, children with SSD without a cochlear implant had worse grammar scores than the group with implants (-0.76; 95% CI, -0.31 to -1.21; P = .004) and the group with normal hearing (-0.53; 95% CI, -0.91 to -0.15; P = .02). The 3 groups had similar vocabulary and receptive language abilities. Conclusions and Relevance: These findings suggest that early cochlear implantation is associated with normal grammar development in young children with prelingual SSD. Although further follow-up will reveal the long-term outcomes of the cochlear implant for other skills, the current results will help clinicians and policy makers identify the best treatment option for these children.


Assuntos
Implante Coclear/métodos , Surdez/complicações , Surdez/cirurgia , Diagnóstico Precoce , Transtornos do Desenvolvimento da Linguagem/etiologia , Transtornos do Desenvolvimento da Linguagem/prevenção & controle , Desenvolvimento da Linguagem , Bélgica , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Estudos Longitudinais , Masculino
12.
Otol Neurotol ; 42(7): 953-958, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-33625195

RESUMO

INTRODUCTION: Girls and women with Turner syndrome (TS) present with multiple ear and hearing problems, ranging from external morphologic abnormalities to sensorineural or conductive hearing loss. The exact pathophysiology behind these otological diseases is not yet completely understood. The aim of this study is to provide a systematic review on the prevalence of otological disease in TS. METHODS: We conducted a systematic review according to the PRISMA guidelines. A database search was performed in PubMed, Embase, Web of Science, and Cochrane library. RESULTS: The prevalence of otological disease as external ear deformities (20-62%), recurrent otitis media (24-48%), and hearing loss (36-84%) is high in TS. The auditory phenotype in TS is complex and seems to be dynamic with CHL due to middle ear disease at young age and sensorineural hearing loss later in life. CONCLUSION: This systematic review of the literature confirms that otological disease is definitely part of the widely variable phenotype in Turner patients. Strong evidence is lacking on the exact prevalence numbers, emphasizing the need for more prospective data gathering. Growing insights in its pathophysiology will help in the understanding and management of hearing problems in TS across lifespan.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva , Síndrome de Turner , Feminino , Perda Auditiva Condutiva/epidemiologia , Perda Auditiva Condutiva/etiologia , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Humanos , Prevalência , Estudos Prospectivos , Síndrome de Turner/complicações , Síndrome de Turner/epidemiologia
13.
Ear Hear ; 42(2): 373-380, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-32769435

RESUMO

OBJECTIVES: Congenital cytomegalovirus (cCMV) infection is the leading cause of nonhereditary sensorineural hearing loss in childhood and is also associated with CNS abnormalities. The main objective is to investigate the prognostic value of neonatal cranial ultrasound (cUS) and cranial magnetic resonance imaging (cMRI) in predicting long-term hearing outcome in a large cohort of cCMV-infected symptomatic and asymptomatic patients. DESIGN: Data were prospectively collected from a multicentre Flemish registry of children with cCMV infection born between 2007 and 2016. Neonatal cUS and cMRI scans were examined for lesions related to cCMV infection. Audiometric results at different time points were analyzed. The imaging and audiometric results were linked and diagnostic values of cUS and cMRI were calculated for the different hearing outcomes. RESULTS: We were able to include 411 cCMV patients, of whom 40% was considered symptomatic at birth. Cranial ultrasound abnormalities associated with cCMV infection were found in 76 children (22.2% of the cUS scans), whereas cMRI revealed abnormalities in 74 patients (26.9% of the cMRI scans). A significant relation could be found between the presence of cUS or cMRI abnormalities and hearing loss at baseline and last follow-up. Cranial ultrasound and cMRI findings were not significantly correlated with the development of delayed-onset hearing loss. Specificity and sensitivity of an abnormal cUS to predict hearing loss at final follow-up were 84% and 43%, respectively compared with 78% and 39% for cMRI. Normal cUS and cMRI findings have a negative predictive value of 91% and 92%, respectively, for the development of delayed-onset hearing loss. CONCLUSIONS: Neuroimaging evidence of CNS involvement in the neonatal period is associated with the presence of hearing loss in children with a cCMV infection. Imaging abnormalities are not predictive for the development of delayed-onset hearing loss.


Assuntos
Infecções por Citomegalovirus , Perda Auditiva , Criança , Infecções por Citomegalovirus/diagnóstico por imagem , Audição , Perda Auditiva/epidemiologia , Testes Auditivos , Humanos , Neuroimagem
14.
Sci Rep ; 10(1): 21011, 2020 12 03.
Artigo em Inglês | MEDLINE | ID: mdl-33273502

RESUMO

Due to the close anatomical relationship between the auditory and vestibular end organs, hearing-impaired children have a higher risk for vestibular dysfunction, which can affect their (motor) development. Unfortunately, vestibular dysfunction often goes unnoticed, as vestibular assessment in these children is not standard of care nowadays. To timely detect vestibular dysfunction, the Vestibular Infant Screening-Flanders (VIS-Flanders) project has implemented a basic vestibular screening test for hearing-impaired infants in Flanders (Belgium) with a participation rate of 86.7% during the first year and a half. The cervical Vestibular Evoked Myogenic Potentials (cVEMP) test was applied as vestibular screening tool to map the occurrence of vestibular (mainly saccular) dysfunction in this population. At the age of 6 months, 184 infants were screened. No refers on vestibular screening were observed in infants with permanent conductive hearing loss. In infants with permanent sensorineural hearing loss, a cVEMP refer rate of 9.5% was observed. Failure was significantly more common in infants with severe-profound compared to those with mild-moderate sensorineural hearing loss (risk ratio = 9.8). Since this is the first regional study with a large sample size and successful participation rate, the VIS-Flanders project aims to set an example for other regions worldwide.


Assuntos
Perda Auditiva Neurossensorial/diagnóstico , Triagem Neonatal/métodos , Potenciais Evocados Miogênicos Vestibulares , Bélgica , Feminino , Perda Auditiva Neurossensorial/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino
16.
Am J Med Genet A ; 179(3): 448-454, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30635960

RESUMO

The 22q11.2 deletion syndrome (22q11.2DS) is the second most common cause of developmental delay after Down syndrome. Impaired cognitive development is highly prevalent, but also motor abnormalities such as hypotonia and delays in achieving motor milestones are described. Instability is frequently detected in children, adolescents, and adults and is mostly attributed to their limited motor performance. Until now, vestibular function has not been investigated in these patients, despite the growing evidence that they often have inner ear malformations. The aim of this prospective study was to identify the presence and character of vestibular dysfunction in 22q11.2DS. We investigated 23 subjects with proven 22q11.2DS, older than the age of 12. We performed caloric testing and pendular rotation chair tests with videonystagmography, cervical vestibular-evoked myogenic potential (c-VEMP)-testing, and posturography. Additional otoscopy and audiometry were performed on all subjects. We found a unilateral caloric hypofunction in 55% of patients, a certain absent c-VEMP response in 15% of ears, an inconclusive c-VEMP response in 33% of ears, and abnormal posturography in 68% of patients, of whom 42% displayed a typical vestibular pattern. Remarkably, 90% revealed uni- or bilateral weak caloric responses, independent of caloric symmetry. Vestibular dysfunction is frequent in subjects with 22q11.2DS. This knowledge should be taken into account when assessing motor performance in these patients. Additional larger studies are needed to determine whether this dysfunction implicates a therapeutic potential.


Assuntos
Síndrome de DiGeorge/diagnóstico , Síndrome de DiGeorge/fisiopatologia , Vestíbulo do Labirinto/fisiopatologia , Adolescente , Adulto , Animais , Criança , Síndrome de DiGeorge/genética , Potenciais Evocados Auditivos , Feminino , Humanos , Masculino , Camundongos , Avaliação de Sintomas , Proteínas com Domínio T/genética , Potenciais Evocados Miogênicos Vestibulares , Adulto Jovem
17.
Hear Res ; 372: 29-41, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29395617

RESUMO

The estimated incidence of sensorineural hearing impairment (>40 dB HL) at birth is 1.86 per 1000 newborns in developed countries and 30-40% of these are unilateral. Profound sensorineural unilateral hearing impairment or single sided deafness (SSD) can be treated with a cochlear implant. However, this treatment is costly and invasive and unnecessary in the eyes of many. Very young children with SSD often do not exhibit language and cognitive delays and it is hard to imagine that neurocognitive skills will present difficulties with one good ear. In the current paper we review the most recent evidence on the consequences of unilateral hearing impairment for auditory and neurocognitive factors. While data of both adults and children are discussed, we focus on developmental factors, congenital deafness and a window of opportunity for intervention. We discuss which etiologies qualify for a cochlear implant and present our multi-center prospective study on cochlear implants in infants with one deaf ear. The large, state-of-the art body of research allows for evidence-based decisions regarding management of unilateral hearing loss in children.


Assuntos
Perda Auditiva Unilateral/congênito , Adulto , Bélgica , Criança , Pré-Escolar , Implantes Cocleares , Função Executiva , Feminino , Perda Auditiva Unilateral/fisiopatologia , Perda Auditiva Unilateral/reabilitação , Humanos , Lactente , Recém-Nascido , Desenvolvimento da Linguagem , Masculino , Memória de Curto Prazo , Testes de Estado Mental e Demência , Pessoas com Deficiência Auditiva/psicologia , Pessoas com Deficiência Auditiva/reabilitação , Estudos Prospectivos , Localização de Som , Percepção da Fala
18.
PLoS Biol ; 16(10): e2005164, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30321166

RESUMO

Frequency tuning and phase-locking are two fundamental properties generated in the cochlea, enabling but also limiting the coding of sounds by the auditory nerve (AN). In humans, these limits are unknown, but high resolution has been postulated for both properties. Electrophysiological recordings from the AN of normal-hearing volunteers indicate that human frequency tuning, but not phase-locking, exceeds the resolution observed in animal models.


Assuntos
Cóclea/fisiologia , Nervo Coclear/fisiologia , Estimulação Acústica/métodos , Adulto , Animais , Feminino , Voluntários Saudáveis , Audição/fisiologia , Humanos , Macaca mulatta , Masculino , Som , Adulto Jovem
19.
Audiol Neurootol ; 23(2): 89-97, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30130747

RESUMO

Recent findings support the efficacy of the direct acoustic cochlear implant (DACI) in patients with advanced otosclerosis whose rehabilitation is very challenging. Standard treatment consists of stapes surgery combined with hearing aids or a cochlear implant (CI). CI surgery, however, is often challenging depending on the grade of otosclerosis. This study aims to compare speech perception scores in quiet and noise of 6 DACI and 12 CI patients with advanced otosclerosis at 3 and 12 months after fitting. Preoperative computed tomographic scans of all patients were scored by experts using an existing otosclerosis grading system (stages 1-3). Speech perception in quiet was significantly better for DACI compared to CI users at 3 months after fitting. At 12 months, no difference was found between DACI and CI patients. Speech perception scores in noise were significantly better in the DACI group. In summary, a DACI system seems to provide an effective treatment option as the acoustic component can be preserved in patients with advanced otosclerosis.


Assuntos
Implante Coclear , Implantes Cocleares , Otosclerose/cirurgia , Percepção da Fala/fisiologia , Estimulação Acústica , Idoso , Idoso de 80 Anos ou mais , Feminino , Auxiliares de Audição , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
20.
J Int Adv Otol ; 12(3): 231-236, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28031153

RESUMO

OBJECTIVE: The purpose of this study was to investigate etiological, clinical, and pathological characteristics of traumatic injuries of the middle ear ossicular chain and to evaluate hearing outcome after surgery. MATERIAL AND METHODS: Thirty consecutive patients (31 ears) with traumatic ossicular injuries operated on between 2004 and 2015 in two tertiary referral otologic centers were retrospectively analyzed. Traumatic events, clinical features, ossicular lesions, treatment procedures, and audiometric results were evaluated. Air conduction (AC), bone conduction (BC), and air-bone gap (ABG) were analyzed preoperatively and postoperatively. Amsterdam Hearing Evaluation Plots (AHEPs) were used to visualize the individual hearing results. RESULTS: The mean age at the moment of trauma was 27.9±17.1 years (range, 2-75 years) and the mean age at surgery was 33.2±16.3 years (range, 5-75 years). In 10 cases (32.3%), the injury occurred by a fall on the head and in 9 (29.0%) by a traffic accident. Isolated luxation of the incus was observed in 8 cases (25.8%). Dislocation of the stapes footplate was seen in 4 cases (12.9%). The postoperative ABG closure to within 10 and 20 dB was 30% and 76.7%, respectively. CONCLUSION: Ossicular chain injury by direct or indirect trauma can provoke hearing loss, tinnitus, and vertigo. As injuries are heterogeneous, they require a tailored surgical approach. In this study, the overall hearing outcome after surgical repair was favorable.


Assuntos
Ossículos da Orelha/lesões , Transtornos da Audição/epidemiologia , Procedimentos Cirúrgicos Otológicos , Ferimentos não Penetrantes/cirurgia , Ferimentos Penetrantes/cirurgia , Adolescente , Adulto , Idoso , Audiometria , Criança , Pré-Escolar , Traumatismos Craniocerebrais/complicações , Feminino , Transtornos da Audição/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Ferimentos não Penetrantes/complicações , Ferimentos Penetrantes/complicações , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA