Publicação
IMPLANTE COCLEAR EM CRIANÇA COM AGENESIA IMAGIOLÓGICA DO NERVO AUDITIVO: UM CASO CLÍNICO
| Resumo: | Introduction: Cochlear nerve deficiency represents a challenge in pediatric auditory rehabilitation. Historically, radiological agenesis of the cochlear branch was considered a contraindication for cochlear implantation. However, current evidence suggests the potential persistence of imperceptible functional fibers on Magnetic Resonance Imaging, challenging this paradigm.Case Report: We present the case of a male patient with failed newborn hearing screening and bilateral profound sensorineural hearing loss. A 3-Tesla Magnetic Resonance Imaging revealed bilateral agenesis of the cochlear branches with a common vestibulocochlear nerve and normal labyrinthine morphology, classified as Govaerts Type IIb. At 12 months of age, the patient underwent bilateral cochlear implantation using Cochlear model 635 with the Kanso 2 sound processor. Four months post-activation, moderate functional success was achieved (Categories of Auditory Performance score of 3 on the right), despite inconsistent responses on the left. Given the uncertain auditory prognosis and absent speech intelligibility, Portuguese Sign Language was introduced early as the primary communication framework; at 20 months, the patient demonstrates appropriate bilingual cognitive and communicative competencies. Conclusion: The absence of a cochlear nerve signal on Magnetic Resonance Imaging does not necessarily equate to an absence of function. The achieved sound perception validates the recruitment of alternative neural pathways, such as the Oort anastomosis, underscoring the relevance of the Govaerts Type IIb classification as a viability indicator for cochlear implantation. Early surgical intervention, coupled with a timely bilingual strategy, constitutes a safe and ethical approach, essential for safeguarding the neurocognitive and linguistic development of the child. |
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| Autores principais: | Cuevas, Paulo Manuel Mansilha Xavier Dote |
| Assunto: | Hearing Loss, Sensorineural Vestibulocochlear Nerve Diseases Cochlear Implantation Correction of Hearing Impairment Surdez Neurossensorial Doenças do Nervo Vestibulococlear Implante Coclear Correção da Deficiência Auditiva |
| Ano: | 2026 |
| País: | Portugal |
| Tipo de documento: | dissertação de mestrado |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Universidade de Coimbra |
| Idioma: | português |
| Origem: | Estudo Geral - Universidade de Coimbra |
| Resumo: | Introduction: Cochlear nerve deficiency represents a challenge in pediatric auditory rehabilitation. Historically, radiological agenesis of the cochlear branch was considered a contraindication for cochlear implantation. However, current evidence suggests the potential persistence of imperceptible functional fibers on Magnetic Resonance Imaging, challenging this paradigm.Case Report: We present the case of a male patient with failed newborn hearing screening and bilateral profound sensorineural hearing loss. A 3-Tesla Magnetic Resonance Imaging revealed bilateral agenesis of the cochlear branches with a common vestibulocochlear nerve and normal labyrinthine morphology, classified as Govaerts Type IIb. At 12 months of age, the patient underwent bilateral cochlear implantation using Cochlear model 635 with the Kanso 2 sound processor. Four months post-activation, moderate functional success was achieved (Categories of Auditory Performance score of 3 on the right), despite inconsistent responses on the left. Given the uncertain auditory prognosis and absent speech intelligibility, Portuguese Sign Language was introduced early as the primary communication framework; at 20 months, the patient demonstrates appropriate bilingual cognitive and communicative competencies. Conclusion: The absence of a cochlear nerve signal on Magnetic Resonance Imaging does not necessarily equate to an absence of function. The achieved sound perception validates the recruitment of alternative neural pathways, such as the Oort anastomosis, underscoring the relevance of the Govaerts Type IIb classification as a viability indicator for cochlear implantation. Early surgical intervention, coupled with a timely bilingual strategy, constitutes a safe and ethical approach, essential for safeguarding the neurocognitive and linguistic development of the child. |
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