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1.
Ear Hear ; 44(3): 494-505, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36607743

RESUMO

OBJECTIVE: A prospective, longitudinal, randomized controlled trial with an original crossover design for 1 year was conducted to compare manual fitting to artificial intelligence-based fitting in newly implanted patients. DESIGN: Twenty-four patients who received their first cochlear implant (CI) were randomly assigned to the manual or Fitting to Outcome eXpert (FOX) arm; they followed the corresponding fitting procedures for 1 year. After 1 year, each patient was switched to another arm. The number of fittings, auditory outcomes (pure-tone thresholds, loudness scaling curves, spectral discrimination scores, bisyllabic word recognition in quiet and noise, and speech tracking), fitting session questionnaire, and CI parameters (T level, C level, Threshold Sound Pressure Level (T-SPL), Comfortable Sound Pressure Level (C-SPL), and loudness growth value) were compared between the two groups. Differences between the two groups were analyzed using the Mann-Whitney test, and Holm corrections were applied for multiple statistical tests. At the end of the crossover session, patients were offered the choice to continue with their old or new map. RESULTS: As early as 3 mo postactivation, the FOX group showed less variability and significantly better speech intelligibility in quiet conditions at 40 and 55 dB SPL and noise ( p < 0.05) with median phoneme scores of 50%, 70%, and 50% at 55, 70, and 85 dB SPL compared with 45%, 50%, and 40%, respectively. This group showed better results at 12 mo postactivation ( p < 0.05). In the manual group, 100% of the patients decided to keep the new FOX map, and 82% performed better with the FOX map. In the FOX group, 63% of the patients decided to keep the manual map, although the measurable outcome had not improved. In this group, participants reported to prefer the manual map because it felt more comfortable, even if the FOX map gave better measured outcome. CONCLUSION: Although the study size remains relatively small, the AI-FOX approach was equivalent to or even outperformed the manual approach in hearing performance, comfort, and resources. Furthermore, FOX is a tool capable of continuous improvement by comparing its predictions with observed results and is continuously learning from clinicians' practice, which is why this technology promises major advances in the future.


Assuntos
Implante Coclear , Implantes Cocleares , Percepção da Fala , Humanos , Inteligência Artificial , Percepção da Fala/fisiologia , Implante Coclear/métodos , Computadores
2.
Ear Hear ; 43(3): 941-948, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34611119

RESUMO

OBJECTIVES: To compare the effects of Narrow band CE-Chirps (NB CE-Chirps) and tone bursts (TBs) at 500 Hz and 1000 Hz on the amplitudes and latencies in cervical vestibular evoked myogenic potentials (cVEMPs). DESIGN: Thirty-one healthy adult volunteers of varying ages were tested by air conduction at 95 dB nHL. Recording conditions were randomized for each participant and each modality was tested twice. RESULTS: NB CE-Chirps showed larger corrected amplitudes than TBs at 500 Hz (p < 0.001) which were themselves larger than NB CE-Chirps and TBs at 1000 Hz (p < 0.001). In older volunteers, NB CE-Chirps 500 and 1000 Hz had significantly higher response rates than TBs 500 Hz (p = 0.039). A negative correlation was observed between the corrected amplitudes and the age of the participants regardless of the stimulus and the frequency studied. The p13 and n23 latencies were not correlated with the age of the subjects. CONCLUSIONS: NB CE-Chirps at 500 Hz improved the corrected amplitudes of waveforms in cVEMPs as a result of a better frequency specificity compared with TBs. In the elderly, eliciting cVEMPs at a frequency of 1000 Hz might not be necessary to improve response rates with NB CE-Chirps. Additional studies including a higher number of healthy participants and patients with vestibular disorders are required to confirm these observations.


Assuntos
Doenças Vestibulares , Potenciais Evocados Miogênicos Vestibulares , Estimulação Acústica , Adulto , Idoso , Voluntários Saudáveis , Audição , Humanos , Potenciais Evocados Miogênicos Vestibulares/fisiologia
3.
Eur Arch Otorhinolaryngol ; 279(10): 4861-4869, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35230509

RESUMO

PURPOSE: The classical surgical approach for superior semicircular canal dehiscences (SSCD) is via the extradural middle cranial fossa. This pathway is used to resurface or to plug the SSC. In this paper, we present long-term data on an alternative route: the transmastoid pathway. The predictive factors for a successful surgery are equally presented in this paper. METHODS: Thirty reports of patients operated between September 2007 to January 2020 were analysed. SSCD was confirmed by the association of concordant complaints, audiometric data, cervical vestibular evoked myogenic potentials (cVEMP) responses and computerized tomography findings. Before and after surgery, the following factors were analysed: auditory and vestibular subjective symptoms, Tullio phenomenon, pure-tone audiometry thresholds for air and bone conduction, air-bone gap, cVEMP threshold, and computerized tomography data, for instance the size of the dehiscence. RESULTS: The follow-up is 21 months on average. The transmastoid approach significantly improves all symptoms (although there were less probing results for the vestibular symptoms). Objectively, we can observe, a closure of the audiometric air-bone gap on the low frequencies and an improvement in the cVEMP. The only correlation that was identified was between the preoperative cVEMP results and the postoperative air conduction. CONCLUSIONS: The originality of this study is the long postoperative follow-up. It allowed us to evaluate the symptoms in the long term and to determine a predictive factor of postoperative complication, which has not yet been described until today.The transmastoid plugging technique is safe and effective. Additional long-term data with a larger cohort are needed to confirm our results and correlation studies.


Assuntos
Procedimentos Cirúrgicos Otológicos , Deiscência do Canal Semicircular , Potenciais Evocados Miogênicos Vestibulares , Audiometria de Tons Puros , Humanos , Procedimentos Cirúrgicos Otológicos/métodos , Estudos Retrospectivos , Canais Semicirculares/diagnóstico por imagem , Canais Semicirculares/cirurgia , Potenciais Evocados Miogênicos Vestibulares/fisiologia
4.
Eur Arch Otorhinolaryngol ; 278(12): 4671-4679, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33388985

RESUMO

OBJECTIVE: As a follow-up to the studies by Vaerenberg et al. (Sci World J 501738:1-12, 2014) and Browning et al. (Cochlear Implant Int 21(3):1-13, 2020), who used questionnaires, we determined whether there are between-centre variations in the fitting of cochlear implants by analysing the methodology, fitting parameters and hearing results of patients from four centres with real data. The purpose of this study is to highlight the lack of streamlined mapping guides and outcome measures with respect to cochlear implant (CI) fittings. METHODS: A retrospective study with ninety-seven post-lingual adults with a nucleus cochlear implant placed between 2003 and 2013 was included to ensure at least 5 years of follow-up. The studied data were as follows: the methodology, including the fitter's professional background, the method of activation, the sequence of fitting sessions, the objectives measures and hearing outcomes; and the fitting parameters, including the speech processors, programming strategy, stimulation mode, T and C levels, T-SPL and C-SPL, maxima, pulse width, loudness growth and hearing results. RESULTS: This investigation highlights some common practices across professionals and CI centres: the activation of a CI is behavioural; impedances are systematically measured at each fitting; and some parameters are rarely modified. However, there are also differences, either between centres, such as the sequences of fitting sessions (p < 0.05) or their approach to spectral bands (p < 0.05), or even within centres, such as the policy regarding T and C levels at high frequencies compared to those at low and mid-frequencies. CONCLUSION: There are important variations between and within centres that reflect a lack of CI-related policies and outcome measures in the fitting of CI. CLINICAL TRIALS REGISTRY: NCT03700268.


Assuntos
Implante Coclear , Implantes Cocleares , Percepção da Fala , Adulto , Testes Auditivos , Humanos , Ajuste de Prótese , Estudos Retrospectivos
5.
Behav Res Methods ; 53(5): 1910-1922, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-33674990

RESUMO

Recent data collected on adult patients with vestibular loss (VL) tend to demonstrate possible cognitive impairments in visuospatial working memory, mental rotation, selective attention, and space orientation. However, the neuropsychological profile of children with VL remains largely under-investigated in the scientific literature. Although previous research has shown that children with VL may experience some degree of delayed motor development, it is not yet clear if VL could also lead to specific delayed cognitive development. In this study, we will present the development and validation of a new tablet-based computerized test battery (VSAD) that evaluates visuospatial working memory, mental rotation, selective attention, and space orientation abilities. Thirteen children with VL and 54 average-age matched healthy children performed the VSAD and classical paper-and-pencil neuropsychological tasks twice within a 1-month interval. Our results demonstrated a good concurrent validity with strong correlations between the visuospatial working memory, mental rotation, and space orientation tests of the VSAD and classical tasks. Test-retest reliability was also supported through good intra-class coefficients. However, the test of selective attention showed no concurrent validity with the matched classical task. The discriminant validity of the VSAD was partially supported for visuospatial working memory and mental rotation performance accuracy. The VSAD shows good concurrent validity and reliability for measuring visuospatial working memory, mental rotation, and space orientation in children with VL. Future studies are needed to extend discriminant validity with other populations.


Assuntos
Navegação Espacial , Adulto , Criança , Cognição , Humanos , Memória de Curto Prazo , Testes Neuropsicológicos , Reprodutibilidade dos Testes
6.
Hum Mutat ; 38(5): 581-593, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28236341

RESUMO

Waardenburg syndrome (WS) is a genetic disorder characterized by sensorineural hearing loss and pigmentation anomalies. The clinical definition of four WS types is based on additional features due to defects in structures mostly arising from the neural crest, with type I and type II being the most frequent. While type I is tightly associated to PAX3 mutations, WS type II (WS2) remains partly enigmatic with mutations in known genes (MITF, SOX10) accounting for only 30% of the cases. We performed exome sequencing in a WS2 index case and identified a heterozygous missense variation in EDNRB. Interestingly, homozygous (and very rare heterozygous) EDNRB mutations are already described in type IV WS (i.e., in association with Hirschsprung disease [HD]) and heterozygous mutations in isolated HD. Screening of a WS2 cohort led to the identification of an overall of six heterozygous EDNRB variations. Clinical phenotypes, pedigrees and molecular segregation investigations unraveled a dominant mode of inheritance with incomplete penetrance. In parallel, cellular and functional studies showed that each of the mutations impairs the subcellular localization of the receptor or induces a defective downstream signaling pathway. Based on our results, we now estimate EDNRB mutations to be responsible for 5%-6% of WS2.


Assuntos
Estudos de Associação Genética , Heterozigoto , Mutação , Receptor de Endotelina B/genética , Síndrome de Waardenburg/diagnóstico , Síndrome de Waardenburg/genética , Adolescente , Adulto , Substituição de Aminoácidos , Criança , Pré-Escolar , Biologia Computacional/métodos , Análise Mutacional de DNA , Exoma , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Espaço Intracelular/metabolismo , Iris , Masculino , Taxa de Mutação , Linhagem , Fenótipo , Transporte Proteico , Sítios de Splice de RNA , Receptor de Endotelina B/metabolismo , Adulto Jovem
7.
Eur Arch Otorhinolaryngol ; 273(9): 2495-502, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26676874

RESUMO

The influence of age on adaptation to cochlear implant (CI) is still being contested in the literature. The aim of this study was twofold. First, hearing outcomes in quiet conditions were compared between CI users implanted over and under the age of 70 years. Second, the effect of the duration of auditory deprivation was investigated. The study design is a retrospective review and the setting is in academic tertiary referral center. One hundred and twenty-one postlingually deafened implanted adults participated in this study. Hearing outcomes were compared between 121 postlingually deafened adults implanted under 40, between 40 and 70, and over 70 years of age. Speech audiometry measurements were taken at 1, 3, 6, 12, 24 and 60 months post-cochlear implantation (pCI), in quiet conditions only. Hearing outcomes were significantly better only at 1 year pCI in the youngest group compared to the two older groups. No significant difference was observed between the middle-aged and eldest subjects at any time. The influence of the severe-to-profound hearing loss (SPHL) duration was investigated and found to be equally distributed among the different age groups. Good hearing outcomes in elderly patients are not secondary to a difference in SPHL duration. Age should not be a limiting factor for cochlear implantation decision.


Assuntos
Fatores Etários , Audiometria da Fala , Implantes Cocleares , Adulto , Idoso , Surdez/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados da Assistência ao Paciente , Estudos Retrospectivos
8.
Eur Arch Otorhinolaryngol ; 273(12): 4241-4249, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27334526

RESUMO

Vertigo patients frequently complain of emotional and associated cognitive problems, yet currently, there is no satisfactory questionnaire to measure these associated problems. In the present paper, we propose a new internet-based Neuropsychological Vertigo Inventory (NVI; French) that evaluates attention, memory, emotion, space perception, time perception, vision, and motor abilities. The questionnaire was created using four steps: (1) open interviews with patients suffering from vertigo; (2) semi-structured interviews with an analysis grid to quantify and define the various cognitive and emotional problems reported by the patients; (3) a first version of an internet questionnaire tested on 108 vertigo participants; and (4) the selection of subscale items using principal component analyses (PCA). From the development phase, the revised NVI was composed of seven subscales, each with four items (28 items). In the validation phase, Cronbach's alphas were performed on the revised NVI for total and each subscale score, and to test extreme groups validity, the analyses of covariance (ANCOVAs) taking into account age were performed between 108 vertigo and 104 non-vertigo participants. The Cronbach's alphas showed good to satisfactory coefficients for the total and for all subscale scores, demonstrating acceptable reliability. The extreme groups validity analyses (ANCOVAs) were reliable for the total scale and for four subscales. Supplementary analyses showed no effect of hearing difficulties and an inverse age effect for attention and emotion subscales, with reduced problems with increased age in the vertigo participants. The NVI provides a useful new questionnaire to determine cognitive and emotional neuropsychological complaints that are associated with vertigo.


Assuntos
Transtornos Cognitivos/diagnóstico , Inquéritos e Questionários , Vertigem/psicologia , Adulto , Fatores Etários , Atenção , Bélgica , Cognição , Emoções , Feminino , Transtornos da Audição/diagnóstico , Humanos , Internet , Entrevista Psicológica , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Reprodutibilidade dos Testes
9.
Eur Arch Otorhinolaryngol ; 272(7): 1629-35, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24615652

RESUMO

The main objective of this study is to analyze results obtained with hydroxyapatite bone cement (HABC) ossiculoplasties. This is a retrospective study of a case series. This study was conducted in an academic hospital and tertiary referral center. A total of 127 ossiculoplasties using HABC were evaluated. Ears were divided into three groups according to procedure: group 1 involved reinforcement of the incudostapedial joint with cement and reconstruction of an incus long process defect with cement. Group 2 involved partial ossicular reconstruction between the stapes and malleus handle with HABC. Group 3 was divided into two subgroups. Group 3B entailed reconstruction of the stapes with a mobile footplate (Austin-Kartush type B = group 3B) and group 3F with a fixed footplate (Austin-Kartush type F = group 3F) using a K-Helix piston (Grace Medical, Memphis, TN, USA) or a classical titanium piston (Kurz, Fuerth, Germany) glued to the incus remnant or malleus handle with cement. Anatomical and pre- and postoperative audiological results were assessed. The mean follow-up was 26 ± 14 months. Percentages of average postoperative air-bone gap ≤ 20 dB were 95, 82.5, 50 and 83.3%, and for air-bone gap ≤ 1 0 dB, 80, 50.9, 16.6 and 50% for groups 1, 2, 3B and 3F, respectively. No complications related to the cement or extrusion occurred. Hearing outcomes also remained stable over time. In our experience, ossiculoplasty with cement provides good and stable functional results, is safe, cost effective, and easy to use. HABC with or without biocompatible ossicular prostheses allows repair of different types of ossicular defects with preservation of the anatomical and physiological ossicular chain, as well as improved stability. Reconstruction of the incus long process or incudostapedial joint defect with cement is preferred over partial ossicular reconstruction.


Assuntos
Ossículos da Orelha/cirurgia , Hidroxiapatitas/uso terapêutico , Doenças do Labirinto/cirurgia , Procedimentos de Cirurgia Plástica , Complicações Pós-Operatórias , Cirurgia do Estribo , Bélgica , Cimentos Ósseos/uso terapêutico , Ossículos da Orelha/patologia , Feminino , Perda Auditiva Condutiva/etiologia , Perda Auditiva Condutiva/cirurgia , Humanos , Doenças do Labirinto/complicações , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/cirurgia , Período Pós-Operatório , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos de Cirurgia Plástica/instrumentação , Procedimentos de Cirurgia Plástica/métodos , Reoperação , Estudos Retrospectivos , Cirurgia do Estribo/efeitos adversos , Cirurgia do Estribo/instrumentação , Cirurgia do Estribo/métodos , Resultado do Tratamento
10.
Eur Arch Otorhinolaryngol ; 272(2): 327-31, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24337878

RESUMO

To evaluate the success rate and the surgical procedure of two different transcanal myringoplasty techniques using the Tutopatch(®) (Tutogen Medical, Inc., Alachua, FL, USA), a xenograft produced from bovine pericardium or the butterfly, an inlay tragal cartilage autograft. This is a retrospective study. We studied all cases of transcanal myringoplasty with Tutopatch and butterfly, performed by the same surgeon between April 2005 and May 2013. Perforations were secondary to chronic otitis media without cholesteatoma, perforation post ventilation tube or trauma. They were not exceeding one-third of the tympanic membrane surface for the Tutopatch and one quarter for the butterfly. We evaluated the anatomical success rate, complications and postoperative hearing results in both techniques. A total of 106 myringoplasties were performed: 66 with Tutopatch and 40 with butterfly with a mean follow-up of 16.5 and 5.2 months, respectively. Successful closure rates of Tutopatch and butterfly were 75.8% (P < 0.0001) and 85.0% (P < 0.0001), respectively. Myringitis controlled with topical antibiotics treatment occurred in 8 (12.1%) and 5 (12.5%) cases. Eighty percent of patients with Tutopatch had a mean residual air-bone gap within 10 dB, compared to 85.0% in patients with butterfly. When anatomically feasible, a transcanal approach myringoplasty with a Tutopatch(®) graft or butterfly appears to provide good anatomical and functional results. We show that both techniques provide good anatomical and functional results. The butterfly has the advantage to use an autograft, which is surgically easier because it does not require tympanomeatal flap elevation. We recommend the butterfly technique for non-marginal perforation not exceeding one quarter of the tympanic membrane after excision of the perforation edge and Tutopatch for bigger perforation or when standard autografts are not available. Myringitis is the only described complication without specific incidence.


Assuntos
Bioprótese , Cartilagem/transplante , Pericárdio/transplante , Perfuração da Membrana Timpânica/cirurgia , Timpanoplastia/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Autoenxertos , Bovinos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Miringoplastia/métodos , Complicações Pós-Operatórias , Estudos Retrospectivos , Adulto Jovem
13.
Otol Neurotol ; 44(6): e412-e418, 2023 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-37231530

RESUMO

OBJECTIVE: To compare ocular vestibular evoked myogenic potentials (oVEMPs) obtained with three different electrode montages (infra-orbital vs belly-tendon vs chin) in a group of healthy subjects. To assess the electrical activity recorded at the level of the reference electrode in the belly-tendon and chin montages. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Twenty-five healthy adult volunteers. INTERVENTIONS: Each ear was tested separately via air-conducted sound (500 Hz Narrow Band CE-Chirps at 100 dB nHL) for recording contralateral myogenic responses. Recording conditions were randomized. MAIN OUTCOME MEASURES: n1-p1 amplitudes values, interaural amplitude asymmetry ratios (ARs) and response rates. RESULTS: The belly-tendon electrode montage (BTEM) produced larger amplitudes than the chin ( p = 0.008) and the IOEM (infra-orbital electrode montage; p < 0.001). The chin montage displayed larger amplitudes than the IOEM ( p < 0.001). The interaural amplitude asymmetry ratios (ARs) were not affected by the different electrode montages ( p = 0.549). In 100% of cases, oVEMPs were detected bilaterally with the BTEM which is higher than with the chin and the IOEM ( p < 0.001; p = 0.020, respectively). We did not record any VEMP when placing the active electrode on the contralateral internal canthus or the chin and the reference electrode on the dorsum of the hand. CONCLUSIONS: The BTEM increased the amplitudes recorded and response rate in healthy subjects. No positive or negative reference contamination was observed with the belly-tendon or chin montages.


Assuntos
Potenciais Evocados Miogênicos Vestibulares , Adulto , Humanos , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Estudos Prospectivos , Eletrodos , Som , Voluntários Saudáveis , Estimulação Acústica
15.
Front Neurosci ; 16: 850245, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35418829

RESUMO

Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human brain. However, it is of high importance since hearing loss is a growing problem due to aging population. Moreover, knowing these brain changes could help to understand some disappointing results with cochlear implant, and therefore could improve hearing rehabilitation. A systematic review and a coordinate-based meta-analysis were realized about the morphological brain changes highlighted by MRI in severe to profound hearing loss, congenital and acquired before or after language onset. 25 papers were included in our review, concerning more than 400 deaf subjects, most of them presenting prelingual deafness. The most consistent finding is a volumetric decrease in gray matter around bilateral auditory cortex. This change was confirmed by the coordinate-based meta-analysis which shows three converging clusters in this region. The visual areas of deaf children is also significantly impacted, with a decrease of the volume of both gray and white matters. Finally, deafness is responsible of a gray matter increase within the cerebellum, especially at the right side. These results are largely discussed and compared with those from deaf animal models and blind humans, which demonstrate for example a much more consistent gray matter decrease along their respective primary sensory pathway. In human deafness, a lot of other factors than deafness could interact on the brain plasticity. One of the most important is the use of sign language and its age of acquisition, which induce among others changes within the hand motor region and the visual cortex. But other confounding factors exist which have been too little considered in the current literature, such as the etiology of the hearing impairment, the speech-reading ability, the hearing aid use, the frequent associated vestibular dysfunction or neurocognitive impairment. Another important weakness highlighted by this review concern the lack of papers about postlingual deafness, whereas it represents most of the deaf population. Further studies are needed to better understand these issues, and finally try to improve deafness rehabilitation.

16.
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
17.
Front Hum Neurosci ; 15: 732974, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34658819

RESUMO

Previous research in vestibular cognition has clearly demonstrated a link between the vestibular system and several cognitive and emotional functions. However, the most coherent results supporting this link come from rodent models and healthy human participants artificial stimulation models. Human research with vestibular-damaged patients shows much more variability in the observed results, mostly because of the heterogeneity of vestibular loss (VL), and the interindividual differences in the natural vestibular compensation process. The link between the physiological consequences of VL (such as postural difficulties), and specific cognitive or emotional dysfunction is not clear yet. We suggest that a neuropsychological model, based on Kahneman's Capacity Model of Attention, could contribute to the understanding of the vestibular compensation process, and partially explain the variability of results observed in vestibular-damaged patients. Several findings in the literature support the idea of a limited quantity of cognitive resources that can be allocated to cognitive tasks during the compensation stages. This basic mechanism of attentional limitations may lead to different compensation profiles in patients, with or without cognitive dysfunction, depending on the compensation stage. We suggest several objective and subjective measures to evaluate this cognitive-vestibular compensation hypothesis.

18.
J Child Neurol ; 36(2): 152-158, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33016209

RESUMO

Chudley-McCullough syndrome, a rare autosomal recessive disorder due to pathogenic variants in the GPSM2 (G-protein signaling modulator 2) gene, is characterized by early-onset sensorineural deafness and a typical combination of brain malformations, including ventriculomegaly, (partial) agenesis of the corpus callosum, cerebellar dysplasia, arachnoid cysts, frontal subcortical heterotopia, and midline polymicrogyria. When hearing loss is managed early, most patients have minor or no impairment of motor and cognitive development, despite the presence of brain malformations. We report 2 cases of Chudley-McCullough syndrome, one presenting with congenital deafness and normal development except for speech delay and one presenting prenatally with ventriculomegaly and an atypical postnatal course characterized by epileptic spasms, deafness, and moderate intellectual disability. These highlight the challenges faced by clinicians when predicting prognosis based on pre- or postnatal imaging of brain malformations. We have also reviewed the phenotype and genotype of previous published cases to better understand Chudley-McCullough syndrome.


Assuntos
Agenesia do Corpo Caloso/diagnóstico , Agenesia do Corpo Caloso/patologia , Cistos Aracnóideos/diagnóstico , Cistos Aracnóideos/patologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/patologia , Adolescente , Agenesia do Corpo Caloso/genética , Cistos Aracnóideos/genética , Encéfalo/anormalidades , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Criança , Surdez/genética , Surdez/patologia , Diagnóstico Diferencial , Feminino , Perda Auditiva Neurossensorial/genética , Humanos , Imageamento por Ressonância Magnética , Masculino
19.
Audiol Neurootol ; 15(1): 7-17, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-19451705

RESUMO

We present sound localization results from 30 children with bilateral cochlear implants. All children received their implants sequentially, at ages from 6 months to 9 years for the first implant and 1.5-12 years for the second implant, with delays of 10 months to 9 years. Localization was measured in the sound field, with a broadband bell-ring presented from 1 of 9 loudspeakers positioned in the frontal horizontal plane. The majority of the children (63%) were able to localize this signal significantly better than chance level. Mean absolute error scores varied from 9 to 51 degrees (root mean square error scores from 13 to 63 degrees ). The best scores were obtained by children who received their first implant before the age of 2 years and by children who used hearing aids prior to implantation for a period of 18 months or longer. Age at second implantation was important in the group of children who did not use a contralateral hearing aid during the unilateral implant period. Additionally, children who attended a mainstream school had significantly better localization scores than children who attended a school for the deaf. No other child or implantation variables were related to localization performance. Data of parent questionnaires derived from the Speech, Spatial and Qualities of Hearing Scale were significantly correlated with localization performance. This study shows that the sound localization ability of children with bilateral cochlear implants varies across subjects, from near-normal to chance performance, and that stimulation early in life, acoustically or electrically, is important for the development of this capacity.


Assuntos
Implante Coclear/métodos , Implantes Cocleares , Surdez/cirurgia , Localização de Som/fisiologia , Adolescente , Análise de Variância , Atenção/fisiologia , Limiar Auditivo/fisiologia , Criança , Pré-Escolar , Feminino , Testes Auditivos , Humanos , Lactente , Masculino , Percepção da Fala/fisiologia , Inquéritos e Questionários , Resultado do Tratamento
20.
Cochlear Implants Int ; 21(5): 299-305, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-31530099

RESUMO

Objective: To assess whether CI programming by means of a software application using artificial intelligence (AI), FOX®, may improve cochlear implant (CI) performance. Patients: Two adult CI recipients who had mixed auditory results with their manual fitting were selected for an AI-assisted fitting. Even after 17 months CI experience and 19 manual fitting sessions, the first subject hadn't developed open set word recognition. The second subject, after 9 months of manual fitting, had developed good open set word recognition, but his scores remained poor at soft and loud presentation levels. Main outcome measure(s): Cochlear implant fitting parameters, pure tone thresholds, bisyllabic word recognition, phonemic discrimination scores and loudness scaling curves. Results: For subject 1, a first approach trying to optimize the home maps by means of AI-proposed adaptations was not successful whereas a second approach based on the use of Automaps (an AI approach based on universal, i.e. population based group statistics) during 3 months allowed the development of open set word recognition. For subject 2, the word recognition scores improved at soft and loud intensities with the AI suggestions. The AI-suggested modifications seem to be atypical. Conclusions: The two case studies illustrate that adults implanted with manual CI fitting may experience an improvement in their auditory results with AI-assisted fitting.


Assuntos
Inteligência Artificial , Implantes Cocleares , Correção de Deficiência Auditiva/métodos , Surdez/reabilitação , Ajuste de Prótese/métodos , Idoso , Limiar Auditivo , Feminino , Humanos , Masculino , Software , Percepção da Fala
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