Inner ear dysplasia with agenesis of the corpus callosum

Smith, Lee P.; Karimi, Kian; Angeli, Simón I.

doi:10.1016/j.ijporl.2005.04.032

Cited by 2 publications

(3 citation statements)

References 9 publications

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“…The agenesis of the corpus callosum has been found in association with neurosensorial hearing loss 7,8 . Smith et al 34 reported a case of association between agenesis of the corpus callosum and Mondini dysplasia.…”

Section: Discussionmentioning

confidence: 99%

Avaliação audiológica em lactentes com agenesia de corpo caloso

2014

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Objetivo verificar a ocorrência de alterações auditivas em lactentes com alteração do corpo caloso, comparando-os a crianças sem tal malformação. Métodos estudo de coorte dividido em duas partes: retrospectivo de 2008 a 2011 e prospectivo de 2011 a 2012. O grupo estudo foi constituído por 12 lactentes com diagnóstico de agenesia de corpo caloso e o grupo controlepor12 lactentes, pareados por idade pós concepcional e sexo. Todos realizaram Emissões Otoacústicas Evocadas por Estimulo Transiente, Potencial Evocado Auditivo de Tronco Encefálico (análise da média das latências das ondas I, III e V e interpicos I-III, III-V e I-V a 80dBnNA) e Avaliação do Comportamento Auditivo. Resultados na análise da ocorrência de alteração auditiva na avaliação com Emissões Otoacústicas Transientes e Potencial Evocado Auditivo de Tronco Encefálico, houve diferença em ambos os grupos com maior percentual de resultados normais no grupo controle. Houve diferença significante entre as orelhas, nas latências da onda III e intervalo interpico I-III, menor à direita, nogrupo controle.Naavaliação comportamental, houve diferença significante entre os grupos em relação aos resultados normais e alterados, com maior ocorrência de alteração central no grupo estudo.Ainda neste grupo, a segunda avaliação mostrou maior índice de resultados alterados quando comparados à primeira avaliação, sendo estaticamente significante. Conclusão as alterações auditivas nos lactentes com alteração do corpo caloso não foram identificadas ao nascimento, tendo sido apenas a partir de seis meses de vida. A maioria das alterações ocorreram na via auditiva central, no tronco encefálico.

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Section: Discussionmentioning

confidence: 99%

Avaliação audiológica em lactentes com agenesia de corpo caloso

2014

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“…Doherty et al (10) reported that 3.2% of patients with CCA had profound deafness, and 14.4% had frequent otitis media in at least one ear. Other previous studies are case reports of patients with CCA with other problems that cause hearing loss (11–13). Although CCA is a well-known comorbidity of numerous syndromic diseases related to hearing loss, this study is the first to report the incidence of hearing loss in CCA patients who conducted NHS protocol.…”

Section: Discussionmentioning

confidence: 99%

“…Our results indicated that hearing loss was not observed in 22 patients with isolated CCA. Smith et al (12) once reported hearing loss with isolated CCA in 2006; however, the patient had amblyopia, mild intellectual disability, and motor developmental delay, implying the possibility of syndromic disease. Patient 19 in our study, who had isolated CCA, did not undergo NHS; thus, it is inconclusive, but the caregiver reported that unilateral hearing loss developed after infection.…”

Section: Discussionmentioning

confidence: 99%

Incidence of Hearing Loss in Corpus Callosum Agenesis

Kim,

Jeong,

Yang

et al. 2023

Otol Neurotol

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Objectives Congenital corpus callosum agenesis (CCA) is one of the congenital anomalies in newborns, which usually presents with syndromic features. It can be asymptomatic or have variable neurological deficits. Some studies demonstrated that hearing loss can occur in patients with CCA; however, the exact prevalence remains unclear. Therefore, we aimed to investigate the prevalence of hearing loss in CCA using data from newborn hearing screening in a single tertiary referral center. Methods A total of 126 patients with CCA combined with hearing loss diagnosed at our hospital from November 2005 to November 2022 were retrospectively included in our study. All patients had at least one screening or diagnostic auditory brainstem response result. Brain ultrasonography and magnetic resonance imaging were used to diagnose CCA. Results Among 126 patients, 93 had automated auditory brainstem response within a month from birth. Of the 93 patients, 20 (21.5%) had a “refer” result in the screening tests in at least one ear. The final incidence of hearing loss in patients with CCA was 16.1%. We observed no hearing loss in 22 patients with isolated CCA. Conclusions Patients with CCA have a higher incidence of hearing loss. However, this is likely related to the concurrent condition of patients. CCA seems not to be a risk factor for hearing loss.

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Inner ear dysplasia with agenesis of the corpus callosum

Cited by 2 publications

References 9 publications

Avaliação audiológica em lactentes com agenesia de corpo caloso

Avaliação audiológica em lactentes com agenesia de corpo caloso

Incidence of Hearing Loss in Corpus Callosum Agenesis

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