ObjectiveEstablish normative data for tympanic electrocochleography (TM ECochG) parameters in normal hearing adults without Ménière's disease's (MD) symptoms. Describe TM ECochG variables that help to distinguish normal from MD ears.Material and methodsWe enrolled 100 subjects (N = 200 ears), 59 females, aged between 19 and 71 years from 09/2010 to 04/2014. Inclusion criteria: normal otomicroscopy, hearing thresholds ≤25 dB nHL from 250 to 4000 Hz, normal tympanogram, no symptoms of MD according to the AAO-HNS 1995 criteria and Gibson's score <7. We excluded subjects with dizziness, aural fullness or other symptoms of endolymphatic hydrops. The following parameters were analyzed: SP/AP amplitude ratio, SP/AP area ratio and the difference between AP latency with rarefaction and condensation stimuli.ResultsThere was no significant difference between right and left ears (Intraclass correlation coefficient < 0.6). SP/AP amplitude ratio varied between 0.084 and 0.356 and SP/AP area ratio between 0.837 and 1.671 (percentiles 5 and 95). The AP latency difference to rarefaction and condensation clicks was between 0.0 and 0.333 ms.ConclusionNormative data for TM ECochG parameters were established in 100 normal hearing subjects without MD. These data can be used to distinguish normal from pathological findings and in follow-up of MD patients.

genetic mutations identified were the SLC26A4 gene, known to encode the pendrin protein, followed by the GJB2 gene, responsible for gap-junction protein Connexin 26.Conclusion: This DNA-based genetic hearing screening approach has allowed us to identify genes that are responsible for deafness and may be able to identify children at risk for developing delayed-onset hearing loss that current screening methods miss. It may have widespread applications in clinical practices beyond hearing screening.

Objective. To evaluate Auditory Steady-State Responses (ASSR) at high intensities in pediatric cochlear implant candidates and to compare the results to behavioral tests responses. Methods. This prospective study evaluated 42 children with suspected severe-to-profound hearing loss, aged from 3 to 72 months. All had absent ABR and OAE responses. ASSR were evoked using binaural single frequency stimuli at 110 dB HL with a 10 dB down-seeking procedure. ASSR and behavioral test results were compared. Results. Forty-two subjects completed both ASSR and behavioral evaluation. Eleven children (26.2%) had bilateral responses. Four (9.5%) showed unilateral responses in at least two frequencies, all confirmed by behavioral results. Overall 61 ASSR responses were obtained, most (37.7%) in 500 Hz. Mean thresholds were between 101.3 and 104.2 dB HL. Among 27 subjects with absent ASSR, fifteen had no behavioral responses. Seven subjects showed behavioral responses with absent ASSR responses. No spurious ASSR responses were observed at 100 or 110 dB HL. Conclusion. ASSR is a valuable tool to detect residual hearing. No false-positive ASSR results were observed among 42 children, but in seven cases with absent ASSR, the test underestimated residual hearing as compared to the behavioral responses.

RESUMO Introdução:As fístulas liquóricas otológicas espontâneas (FLOEs) são entidades raras. Geralmente manifestam-se em crianças com meningite e perda sensorioneural profunda e em adultos com quadro de otite média com efusão. Objetivo:Descrever o quadro clínico, o diagnóstico e o tratamento cirúrgico de uma paciente com FLOE. Relato do Caso:Paciente do sexo feminino, 57 anos, com história de hipoacusia, plenitude aural e tinnitus à direita há 10 anos. Após colocação de tubo de ventilação à direita em outro serviço, iniciou otorreia líquida, transparente e constante em grande quantidade. O diagnóstico foi realizado através da cisternocintilografia, sugestiva de fístula liquórica; e da cisternotomografia que mostrou área de deiscência óssea em região de tegmen tympani à direita, velamento parcial de células mastóideas e de orelha média à direita, além concentração do meio de contraste no espaço sub-aracnoídeo à direita menor em relação ao lado esquerdo. Através de via transmastoídea foi localizada fístula liquórica e meningoencefalocele na região de tegmen tympani à direita. O fechamento da fístula foi realizado através do uso de retalho de músculo temporal, cola de fibrina e Surgicel ® . Comentários Finais: Em adultos com história clínica sugestiva de otite média com efusão recorrente, a hipótese diagnóstica de fístula liquórica deve ser levantada. A investigação deve prosseguir com exames de imagem, destacando-se a cisternotomografia. O tratamento cirúrgico nesta paciente, através da técnica transmastoídea, se revelou eficaz a curto e a longo prazo. Palavras-chave: otorreia de líquido cefalorraquidiano, fistula, meningite. SUMMARY Introduction:The spontaneous otological CSF leaks are rare entities. Usually they manifest themselves in children with meningitis and deep sensorineural hearing loss and in adults with middle otitis and effusion clinical picture. Objective:To describe the clinical picture, diagnosis and surgical treatment of a patient with CSF otological. Case Report:A female sex patient, 57 years old, with hearing loss, aural fullness and right ear tinnitus case history 10 years ago. After insertion of tympanostomy tubes on the right in another service, it began liquid, transparent and constant otorrhea in large quantities. The diagnosis was performed by suggestive cisternoscintigraphy of CSF leak, and by cisternography that showed that the area of bone dehiscence in the tegmen tympani region to the right, partial opacification of mastoid cells and middle ear to the right, besides concentration of contrast medium in sub-arachnoid space to the lower right compared to the left side. Through transmastoid via was located the CSF leak and meningoencephalocele in the region of the right tegmen tympani. The closure of the fistula was accomplished through the use of the temporal muscle flap, fibrin glue and Surgicel ® . Final Comments:In adults with symptoms suggestive of recurrent otitis media with effusion, the diagnosis of CSF fistula should be raised. The research should proceed with imaging, highlightin...