Microangiopathy of the inner ear, deafness, and cochlear implantation in a patient with Susac syndrome.

CONCLUSION: The cochlear implant was beneficial as an attempt to restore hearing and improve communication abilities in this patient with profound sensorineural hearing loss secondary to Susac syndrome. OBJECTIVE: To report the audiological outcomes of cochlear implantation (CI) in a young woman with Susac syndrome after a 6-month follow-up period. Susac syndrome is a rare disorder. It is clinically characterized by a typical triad of sensorineural deafness, encephalopathy, and visual defect, due to microangiopathy involving the brain, inner ear, and retina. METHODS: This was a retrospective review of a case at a tertiary referral center. After diagnosis, the patient was evaluated by a multidisciplinary team and received a cochlear implant in her right ear. RESULTS: The patient achieved 100% open-set sentence recognition in noise conditions and 92% monosyllable and 68% medial consonant recognition in quiet conditions after 6 months of implant use. She reported the use of the telephone 3 months after activation.

Implante coclear: correlação da recuperação neural, privação auditiva e etiologia / Cochlear implant: correlation of nerve function recovery, auditory deprivation and etiology

TEMA: a Função de Recuperação do Nervo Auditivo (REC) pode ser extraída do potencial de ação das fibras neurais - ECAP (Eletrically Evoked Compound Action Potential). O ECAP pode ser influenciado pela estimulação recebida pelo nervo e pela etiologia de uma perda auditiva e, consequentemente, afetar a REC. OBJETIVO: verificar se há correlação entre REC e os fatores: etiologia, tempo de surdez e tempo de uso do AASI antes do Implante Coclear (IC). MÉTODO: estudo retrospectivo transversal. Foram coletados dados sobre etiologia, tempo de surdez, tempo de uso do Aparelho de Amplificação Sonora Individual (AASI) e REC de 50 indivíduos, 26 crianças e 24 adultos, submetidos à cirurgia de IC e usuários do dispositivo multicanal Nucleus®24. As medidas da função de recuperação do nervo auditivo foram calculadas e os pacientes foram divididos em grupos (GI: recuperação rápida, GII: recuperação intermediária e GIII: recuperação lenta) para posterior análise de relação com os demais dados coletados. RESULTADOS: a análise dos dados não mostrou correlação estatisticamente significante entre a recuperação e os aspectos pré-cirúrgicos estudados. Entretanto, foi possível observar maior concentração de ambos, crianças e adultos, nas REC intermediárias. GI não agrupou indivíduos com surdez de etiologias infecciosas, tais como a meningite, rubéola e citomegalovírus. A média de REC apresentou-se mais lenta para as etiologias infecciosas, tanto para o grupo de crianças, como para o grupo de adultos. CONCLUSÃO: não houve correlação estatisticamente significante entre função de recuperação do nervo auditivo e os fatores: etiologia, tempo de surdez e tempo de uso do AASI antes do IC.

BACKGROUND: the Auditory Nerve Recovery Function (REC) may be extracted from the Electrically Evoked Compound Action Potential (ECAP). ECAP may be influenced by the stimulation received (or the deprivation of stimulation) and by the etiology of the hearing loss, consequently it might affect the REC. AIM: to verify whether there is a correlation between the REC and each of the following factors: etiology, time of auditory deprivation and time of hearing aid use before cochlear implantation (CI). METHOD: retrospective study. Data regarding etiology, time of auditory deprivation, time of hearing aid use before cochlear implantation were collected in children and adults who received a Nucleus®24. All patients who presented neural response at surgery and whose REC was assessed intraoperatively were included in this study. Fifty patients were selected, 26 children and 24 adults. Patients were divided according to the REC classification into three groups (GI: fast recovery; GII: intermediate recovery and GIII: slow recovery) to allow correlation analysis. RESULTS: data analysis did not show any statistically significant correlation between the recovery function and the pre-implant studied characteristics. Nevertheless, it was observed that there was a greater concentration of both, children and adults, in the intermediate recovery function values. GI did not present individuals with infectious etiologies, such as meningitis, rubella and cytomegalovirus. REC average scores were slower in infectious etiologies for both children and adults. CONCLUSION: there was no statistically significant correlation between the recovery function and factors such as etiology, time of auditory deprivation and time of hearing aid use prior to CI.

Characterization of language and phonological working memory in patients with myoclonic astatic epileptic syndrome.

The course of myoclonic astatic epileptic syndrome (MAES) is variable and little information is available about cortical functions in the presence of the disease. The objective of the present study was to assess the phonological working memory (PWM) and the verbal language of six patients between 8 and 18 years old, on treatment for at least 5 years, and good control of seizures, diagnosed in the Service of Epilepsy of Hospital of Clinics of Ribeirão Preto, São Paulo University in Brazil. The Test of Repetition of Meaningless Words was used to assess PWM. Video-recorded of language samples were collected during spontaneous and directed activities for the study of verbal language and pragmatics. A qualitative analysis showed that all patients presented deficits in the execution of the PWM test and only one patient showed poor mastery of all aspects studied. These finds contribute to strategies of treatment for language problems of patients with MAES, focusing on PWA.

Characterization of language and phonological working memory in patients with myoclonic astatic epileptic syndrome / Caracterização da linguagem e memória de trabalho em pacientes com síndrome epiléptica mioclônica astática

The course of myoclonic astatic epileptic syndrome (MAES) is variable and little information is available about cortical functions in the presence of the disease. The objective of the present study was to assess the phonological working memory (PWM) and the verbal language of six patients between 8 and 18 years old, on treatment for at least 5 years, and good control of seizures, diagnosed in the Service of Epilepsy of Hospital of Clinics of Ribeirão Preto, São Paulo University in Brazil. The Test of Repetition of Meaningless Words was used to assess PWM. Video-recorded of language samples were collected during spontaneous and directed activities for the study of verbal language and pragmatics. A qualitative analysis showed that all patients presented deficits in the execution of the PWM test and only one patient showed poor mastery of all aspects studied. These finds contribute to strategies of treatment for language problems of patients with MAES, focusing on PWA.

A evolução da síndrome epiléptica mioclônica astática (SEMA) é variável e há poucas informações sobre funções corticais. Este estudo pretendeu avaliar a memória de trabalho fonológica (MTF) e a linguagem verbal de pacientes com SEMA. Foram avaliados seis pacientes entre 8 e 18 anos em tratamento há mais de 5 anos e bom controle das crises, do Serviço de Epilepsia do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo. A Prova de Repetição de Palavras Sem Significado foi utilizada para MTF, e coletaram-se amostras de linguagem, gravadas em vídeo, durante atividades espontâneas e dirigidas para estudo da linguagem verbal e pragmática. Análise qualitativa mostrou que todos apresentaram deficiência na MTF e, exceto um, mostraram domínio dos aspectos verbais estudados. Os achados contribuem para estratégias de tratamento de problemas de linguagem de pacientes com SEMA, com maior enfoque na MTF.

Cochlear implant: correlation of nerve function recovery, auditory deprivation and etiology.

BACKGROUND: The Auditory Nerve Recovery Function (REC) may be extracted from the Electrically Evoked Compound Action Potential (ECAP). ECAP may be influenced by the stimulation received (or the deprivation of stimulation) and by the etiology of the hearing loss, consequently it might affect the REC. AIM: To verify whether there is a correlation between the REC and each of the following factors: etiology, time of auditory deprivation and time of hearing aid use before cochlear implantation (CI). METHOD: Retrospective study. Data regarding etiology, time of auditory deprivation, time of hearing aid use before cochlear implantation were collected in children and adults who received a Nucleus®24. All patients who presented neural response at surgery and whose REC was assessed intraoperatively were included in this study. Fifty patients were selected, 26 children and 24 adults. Patients were divided according to the REC classification into three groups (GI: fast recovery; GII: intermediate recovery and GIII: slow recovery) to allow correlation analysis. RESULTS: Data analysis did not show any statistically significant correlation between the recovery function and the pre-implant studied characteristics. Nevertheless, it was observed that there was a greater concentration of both, children and adults, in the intermediate recovery function values. GI did not present individuals with infectious etiologies, such as meningitis, rubella and cytomegalovirus. REC average scores were slower in infectious etiologies for both children and adults. CONCLUSION: There was no statistically significant correlation between the recovery function and factors such as etiology, time of auditory deprivation and time of hearing aid use prior to CI.