OBJECTIVE:To demonstrate the surgical technique and audiological results of a middle ear implant for restoring hearing in patients with bilateral aural atresia and microtia with conductive or mixed hearing loss. MATERIALS and METHODS:In this prospective study, 12 subjects aged 12 years and older presenting with hearing loss and bilateral congenital aural atresia underwent surgical insertion of a middle ear implant. The patients underwent tympanomastoidectomy with a wide opening of the attic and preservation of the roof of the atresic tympanic bone to expose the middle ear and position the floating mass transducer of the implant.RESULTS: There were no intraoperative or postoperative complications. The hearing threshold averages in a free field were 53.5 dB preoperatively and 25.6 dB postoperatively. Monosyllabic word recognition averaged 61% preoperatively and 91.3% postoperatively. The average speech perception in quiet conditions during the hearing in noise test improved from 67.11 dB to 45.99 dB, and the signal-to-noise ratio improved from 5.64 to 1.31. CONCLUSION:The tested system is an excellent option for auditory rehabilitation of conductive hearing loss due to bilateral ear atresia. The surgery is well structured and safe and provides several alternatives to the surgeon, which is valuable in difficult cases.

RESUMO Objetivo Descrever a perda auditiva sensorioneural unilateral e bilateral assimétrica em crianças quanto às características etiológicas, audiológicas e demográficas. Método Estudo retrospectivo transversal, desenvolvido na Seção de Implante Coclear do Hospital de Reabilitação de Anomalias Craniofaciais, por meio da análise de prontuários. Resultados Foram analisados os dados de 1152 pacientes, sendo 424 (37%) adolescentes, adultos ou idosos e 728 (63%) crianças, dentre as quais, 691 (95%) apresentavam perda auditiva bilateral simétrica e 37 (5%) perda auditiva sensorioneural unilateral (n=10) ou bilateral assimétrica (n=27). A idade média ao diagnóstico na perda auditiva sensorioneural unilateral foi de 33,58±21,69 meses e na bilateral assimétrica de 33,12±21,69 meses, com prevalência de 1,4% e 3,7%, respectivamente. O indicador de risco para a deficiência auditiva de maior ocorrência para ambos os grupos foi o de antecedente familiar. A maioria dos familiares das crianças com perda auditiva sensorioneural unilateral apresentaram a classificação socioeconômica baixa superior (50%), enquanto que as crianças com perda auditiva sensorioneural bilateral assimétrica se subdividiram igualmente em baixa superior (37%) e média inferior (37%). Conclusão Houve uma maior prevalência da perda auditiva sensorioneural bilateral assimétrica em relação à unilateral, bem como do indicador de risco de hereditariedade, com predomínio do grau profundo na pior orelha e preponderância do sexo feminino, em ambos os grupos. Apesar de a triagem auditiva neonatal propiciar a identificação precoce da perda auditiva sensorioneural unilateral, a idade no diagnóstico audiológico ainda se encontra acima do recomendado. Adicionalmente, a maioria dos familiares das crianças apresentou nível de rendimento baixo.

Our results suggest that most patients with EEC syndrome and middle ear cholesteatoma should be considered for a canal wall down mastoidectomy due to extensive disease and a high rate of recidivism. In addition, a high percentage of postoperative stenosis of the external auditory canal was found in this group.

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<p>Hearing loss may be related to several factors, including hearing loss resulting from certain genetic syndromes. Kearns-Sayre syndrome is characterized by mutations in mitochondrial DNA (deoxyribonucleic acid), responsible for the production of energy (adenosine triphosphate-ATP), which is extremely important for the development of structures that require it, such as the cochlea. The case was followed up at the hospital since 2000, due to the progressive characteristic of hearing loss found in audiological tests and findings in cases related to the syndrome. The intervention with individual sound amplification devices proved to be of little benefit for good oral communication of one of the patients, who was diagnosed with bilateral profound hearing loss. Thus, after discussions in clinical meetings, the team opted for the indication of the cochlear implant for the patient, according to the current criteria for indication of this surgery, and with which it obtained good results. His twin sister, who presented good results with hearing aids, will continue in audiological follow-up, to verify the evolution of the case and discuss a new approach, if necessary. Patients with suspected or diagnosed Kearns-Sayre syndrome should seek audiological diagnosis, because it is a possible progressive hearing loss, requiring rehabilitation with the use of hearing devices. Maintaining oral communication is extremely important because, in these cases, other functions will be impaired, such as muscle tone and vision.</p>