RESUMO
Modern magnetic resonance imaging has significantly improved the diagnosis of cochlear nerve deficiencies. A careful assessment of all clinical, imaging, and auditory data is of utmost importance in such cases in order to properly establish the site of the abnormality. We report the case of a 3-year-old girl with unilateral cochlear nerve aplasia, normal middle and inner ear anatomy, and an absence of otoacoustic emissions, all of which erroneously suggested cochlear damage. We also briefly review the embryogenesis of the inner ear and auditory pathway. A patient with similar findings in the setting of a bilateral hearing loss and insufficient imaging would be at risk of inappropriate cochlear implantation.
Assuntos
Nervo Coclear/anormalidades , Meato Acústico Externo/anatomia & histologia , Perda Auditiva Neurossensorial/etiologia , Doenças do Nervo Vestibulococlear/complicações , Audiometria de Tons Puros , Pré-Escolar , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Doenças do Nervo Vestibulococlear/diagnósticoRESUMO
Objetivo: Mostrar los resultados obtenidos mediante el uso del Implante Auditivo de Tronco Cerebral (IATC) en niños con imposibilidad de recibir un implante coclear (IC), con hipoacusia no asociada a Neurofibromatosis tipo II (NFII). Tipo de estudio: Presentación de casos. Material y método: Se muestran los resultados obtenidos en los dos primeros casos en América en niños (3 y 10 años, respectivamente) a los que se les colocó un I A TC por agenesia de nervio y cóclea. Resultados: Se logró niveles de 30db a 40 db en las audiometrías posteriores a la activación en frecuencias de 250-6.000 Hz, lograron detectar el test de Ling en su totalidad, con buena y rápida respuesta al nombre en una situación estructurada y sin pistas visuales dentro del primer mes de activación. Conclusiones: De acuerdo con nuestra reciente experiencia los casos de niños con agenesia de cóclea y/o nervio, pueden ser considerados para la colocación de un IATC porque la anatomía de los núcleos auditivos está intacta.
Aim: To discuss the results obtained with auditory brainstem implant (ABI) in children non-eligible for cochlear implant, presenting with non-NFII related hypoacusia. A case report study. Materials and Method: Two cases are presented, both of children (aged 3 and 10 years old, respectively), who underwent ABI because of nerve and cochlear agenesis Results: Post-implant audiometric levels were 30 to 40 dB for frequencies between 25 and 6000 Hz. Both patients were able to fully detect the Ling sound test with good and fast answers in a structured non-visual cues-situation within the first post-activation month. Conclusions: In our experience, children with nerve and/or cochlear agenesis could be considered for ABS implantation, because the auditory nuclei's anatomy is intact.