Evaluation of Audiometric Thresholds and Speech Perception Sentence Test in Adults and Elderly After Cochlear Implantation.

AIM: To compare the audiometric thresholds and speech perception sentence test, between two groups with bilateral post-lingual, severe to profound sensorineural hearing loss. METHODS: Retrospective and analytical study, with 59 patients divided into 2 groups (under 60 years and above 60 years old) implanted between May/2002 and February/2007. RESULTS: The first group (control) included 30 patients with a mean age of 44 years. The audiometric threshold value in this group was 26 dB, and the average value of speech perception test was 94%. The second group included 29 patients with a mean age of 69 years. The average audiometric threshold was 29 dB, and the average value of SPT was 90%. The Mann-Whitney U-test was considered significant (P<0.05) only for 6-8 KHz frequencies and for SPT. CONCLUSION: Both groups had excellent outcomes in audiometric and speech testing with the use of CI, but with a significantly better performance in the adult group.

Reduction of Cellular Expression Levels Is a Common Feature of Functionally Affected Pendrin (SLC26A4) Protein Variants.

Sequence alterations in the pendrin gene (SLC26A4) leading to functionally affected protein variants are frequently involved in the pathogenesis of syndromic and nonsyndromic deafness. Considering the high number of SLC26A4 sequence alterations reported to date, discriminating between functionally affected and unaffected pendrin protein variants is essential in contributing to determine the genetic cause of deafness in a given patient. In addition, identifying molecular features common to the functionally affected protein variants can be extremely useful to design future molecule-directed therapeutic approaches. Here we show the functional and molecular characterization of six previously uncharacterized pendrin protein variants found in a cohort of 58 Brazilian deaf patients. Two variants (p.T193I and p.L445W) were undetectable in the plasma membrane, completely retained in the endoplasmic reticulum and showed no transport function; four (p.P142L, p.G149R, p.C282Y and p.Q413R) showed reduced function and significant, although heterogeneous, expression levels in the plasma membrane. Importantly, total expression levels of all of the functionally affected protein variants were significantly reduced with respect to the wild-type and a fully functional variant (p.R776C), regardless of their subcellular localization. Interestingly, reduction of expression may also reduce the transport activity of variants with an intrinsic gain of function (p.Q413R). As reduction of overall cellular abundance was identified as a common molecular feature of pendrin variants with affected function, the identification of strategies to prevent reduction in expression levels may represent a crucial step of potential future therapeutic interventions aimed at restoring the transport activity of dysfunctional pendrin variants.

Satisfaction of Children with Auditory Neuropathy and Cochlear Implant.

OBJECTIVE: In auditory neuropathy (AN) a dyssynchrony in the nerve conduction of the auditory nerve fibers is observed. Typically, patients with AN exhibit moderate to profound sensorineural hearing loss, and treatment using cochlear implants (CIs) or hearing aids should be performed as early as possible for a better hearing rehabilitation. The aim of this study is to evaluate the satisfaction level of patients with AN spectrum disorder treated using CIs. The Satisfaction with Amplification in Daily Life questionnaire was selected to evaluate 10 patients with AN treated using CIs. MATERIALS AND METHODS: Clinical study of patients with AN spectrum disorder submitted to CI. A retrospective data analysis, genetic and clinical evaluation in a tertiary referral center was done. RESULTS: The means of the subscales for positive effects, services and costs, negative factors, and personal image were 6.15, 4.6, 3.26, and 3.33, respectively. CONCLUSIONS: Patients with AN treated using CIs consider themselves satisfied.

Hearing Preservation after Cochlear Implantation: UNICAMP Outcomes.

Background. Electric-acoustic stimulation (EAS) is an excellent choice for people with residual hearing in low frequencies but not high frequencies and who derive insufficient benefit from hearing aids. For EAS to be effective, subjects' residual hearing must be preserved during cochlear implant (CI) surgery. Methods. We implanted 6 subjects with a CI. We used a special surgical technique and an electrode designed to be atraumatic. Subjects' rates of residual hearing preservation were measured 3 times postoperatively, lastly after at least a year of implant experience. Subjects' aided speech perception was tested pre- and postoperatively with a sentence test in quiet. Subjects' subjective responses assessed after a year of EAS or CI experience. Results. 4 subjects had total or partial residual hearing preservation; 2 subjects had total residual hearing loss. All subjects' hearing and speech perception benefited from cochlear implantation. CI diminished or eliminated tinnitus in all 4 subjects who had it preoperatively. 5 subjects reported great satisfaction with their new device. Conclusions. When we have more experience with our surgical technique we are confident we will be able to report increased rates of residual hearing preservation. Hopefully, our study will raise the profile of EAS in Brazil and Latin/South America.

Auditory brainstem response and otoacoustic emission assessment of hearing-impaired children of mothers who contracted rubella during pregnancy.

CONCLUSIONS: Based on the auditory brainstem response (ABR) results obtained in this study, the incidence of deafness in children whose mothers had rubella during pregnancy is high (29.5%), and deafness is profound in most cases (80%). Vaccinating women of childbearing age against rubella is essential to reduce the number of cases of childhood sensorineural hearing loss caused by gestational rubella. OBJECTIVE: It has been shown in the literature that, in Brazil, gestational rubella is responsible for approximately 21% of cases of deafness in children. The purpose of this study was to assess the incidence of deafness in children whose mothers had rubella during pregnancy. MATERIAL AND METHODS: Between February and July 2001, we conducted a prospective study assessing hearing status in 17 children (mean age 6 months). The mothers had serologically (ELISA) confirmed gestational rubella. We recorded ABRs and analysed distortion product otoacoustic emissions (DPOAEs). RESULTS: In 5 children (29.5%), ABRs revealed sensorineural hearing loss, which was moderate to severe in 1 (20%) and profound in 4 (80%). The hearing loss was bilateral in 3 children (60%) and asymmetrical in 4 (80%). Regarding DPOAEs, 7 children (41%) presented no response, and this occurred bilaterally in 4 (57%). All children with abnormal ABRs also presented abnormal DPOAEs. Two children with normal ABRs presented abnormal DPOAEs.

Tele-radiografia lateral de crânio e radiografia De cavum: estudo comparativo em crianças com obstrução nasal / Lateral Teleradiograph of Cranium and Lateral Radiograph of the Nasopharynx: comparative study in children with nasal obstruction

A obstrução nasal é queixa freqüente na clínica diária, sendo a hipertrofia da tonsila faríngea (adenóide) uma das causas principais em crianças. A avaliação radiológica da parte nasal da faringe (rinofaringe) é exame não invasivo, de acesso universal e objetivo, existindo vários trabalhos que mostram correlação entre os achados radiográficos, o quadro clínico e o volume de adenóide retirado em cirurgia. Objetivo: O objetivo do estudo foi o de comparar a radiografia de cavum (RC) e a telerradiografia lateral de crânio MO na avaliação de crianças com obstrução nasal. Material e método: Foram estudados 26 pacientes entre três a 11 anos de idade com quadro de obstrução nasal e/ou respiração bucal há mais de três meses, submetidos às duas técnicas radiológicas em estudo no mesmo dia, analisadas pelo método de Cohen e Konak (permeabilidade da via aérea) e com relação ao posicionamento do paciente. Resultados: A idade média dos pacientes foi de 6,4 anos, sendo 16 do sexo feminino e 10 do masculino, sendo encontrada concordância na análise da permeabilidade da parte nasal da faringe em ambos os exames, em 73,1% dos casos. Em 61,5% das RC havia alteração no posicionamento do paciente. Conclusão: Os autores concluem que a TLC deve ser o exame radiológica de escolha na avaliação de crianças com obstrução nasal, pois avaliam a parte nasal da faringe como a RC, porém com a vantagem de posicionamento adequado do paciente na maioria dos casos.

Nasal obstruction is a common complaint in daily practice and adenoidal obstruction is one of the main causes of that in childhood. Radiological assessment constitutes a noninvasive, generally available and objective means of estimating the nasopharynx airway. Some authors describe correlations among radiographic evaluation, clinical and intraoperative findings. Purpose: This study intends to compare lateral teleradiograph of cranium (LTC) and lateral radiograph of the nasopharynx (LRN) in the evaluation of nasal obstruction children. Material e methods: We studied 26 patients between 3 to 11 years old with nasal obstruction and/or mouth breathing for more than 3 months. Both radiographs were taken in the same day and they were evaluated by Cohen and Konak approach. Results: The mean age was 6.4 years and 16 patients were female and 10 male. We found the some interpretation in both radiographs in 73.1% of children. 61.5% of the LRN presented head rotation in the sagital plane. Conclusion: Authors conclude LTC is the radiograph of choice in the nasal obstruction children approach because it evaluates the nasopharynx airway as the LRN but it minimizes changes in head positioning.