Wideband absorbance for the assessment of pressure equalizing tubes patency in children.

OBJECTIVES: To analyze the feasibility of using wideband absorbance to verify the patency of pressure equalizing tubes (PETs) in clinical practice and to present the response pattern of this measure for ears with patent PET. METHODS: This observational case-control type study evaluated 48 ears of 30 children with severe or profound hearing loss, aged 10-44 months, of both sexes. The subjects were subdivided into two groups: 24 ears with Sheppard type PET (experimental group - EG) and 24 ears with normal middle ear (control group - CG), paired with the EG, according to age, sex, and ear evaluated. To obtain the wideband absorbance, a Middle-Ear Power Analyzer, version 5.0 (Mimosa Acoustics), was used, and absorbance values for pure tone and chirp stimuli were analyzed. RESULTS: There was no influence of ear (right or left) on the measurements obtained. The EG showed higher absorbance values at low frequencies. Although the two stimuli made it possible to identify the difference in acoustic transfer function between the groups studied, compared to pure tone, the chirp stimulus allowed identification of differences in a higher number of frequencies. CONCLUSIONS: Ears with a patent PET present an acoustic transfer pattern that differs from that obtained for normal middle ears, with a higher absorbance at low frequencies. Both pure tone and chirp stimuli can be used to identify such differences, nevertheless, the use of chirp stimulus is recommended, since it allows differentiation over a wider frequency range.

Unilateral and asymmetric hearing loss in childhood. / Perda auditiva unilateral e assimétrica na infância.

PURPOSE: To describe unilateral and bilateral asymmetric sensorineural hearing loss in children and its etiological, audiological and demographic characteristics. METHODS: Retrospective cross-sectional study developed in the Seção de Implante Coclear of Hospital de Reabilitação de Anomalias Craniofaciais, through the analysis of medical records. RESULTS: Data from 1152 patients were analyzed: 424 (37%) adolescents, adults or elderly, and 728 (63%) children, of whom 691 (95%) had bilateral symmetrical hearing loss, and 37 (5%) had unilateral hearing loss (n=10) or bilateral asymmetric (n=27) sensorineural hearing loss. The mean age at diagnosis of unilateral sensorineural hearing loss was 33.58±21.69 months, and for asymmetric bilateral it was 33.12±21.69 months, with a prevalence of 1.4% and 3.7%, respectively. The highest risk indicator for hearing loss for both groups was the family history of permanent deafness, which began in childhood. The majority of the relatives of children with unilateral sensorineural hearing loss presented the highest low socioeconomic classification (50%), while children with bilateral asymmetric sensorineural hearing loss were also be subdivided into upper (37%) and lower (37%). CONCLUSION: We observed a greater occurrence of asymmetric bilateral sensorineural hearing loss compared to unilateral hearing loss, as well as the hereditary risk indicator, with a predominance of the deep ear and female preponderance in both groups. Although neonatal hearing screening provides early identification of unilateral sensorineural hearing loss, the age at the audiological diagnosis is still above the recommended level. In addition, the majority of the children's family members presented a low level of income.

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.

Perda auditiva unilateral e assimétrica na infância / Unilateral and asymmetric hearing loss in childhood

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.

ABSTRACT Purpose To describe unilateral and bilateral asymmetric sensorineural hearing loss in children and its etiological, audiological and demographic characteristics. Methods Retrospective cross-sectional study developed in the Seção de Implante Coclear of Hospital de Reabilitação de Anomalias Craniofaciais, through the analysis of medical records. Results Data from 1152 patients were analyzed: 424 (37%) adolescents, adults or elderly, and 728 (63%) children, of whom 691 (95%) had bilateral symmetrical hearing loss, and 37 (5%) had unilateral hearing loss (n=10) or bilateral asymmetric (n=27) sensorineural hearing loss. The mean age at diagnosis of unilateral sensorineural hearing loss was 33.58±21.69 months, and for asymmetric bilateral it was 33.12±21.69 months, with a prevalence of 1.4% and 3.7%, respectively. The highest risk indicator for hearing loss for both groups was the family history of permanent deafness, which began in childhood. The majority of the relatives of children with unilateral sensorineural hearing loss presented the highest low socioeconomic classification (50%), while children with bilateral asymmetric sensorineural hearing loss were also be subdivided into upper (37%) and lower (37%). Conclusion We observed a greater occurrence of asymmetric bilateral sensorineural hearing loss compared to unilateral hearing loss, as well as the hereditary risk indicator, with a predominance of the deep ear and female preponderance in both groups. Although neonatal hearing screening provides early identification of unilateral sensorineural hearing loss, the age at the audiological diagnosis is still above the recommended level. In addition, the majority of the children's family members presented a low level of income.

Evolution of Acquired Middle Ear Cholesteatoma in Patients With Ectrodactyly, Ectodermal Dysplasia, Cleft Lip/Palate (EEC) Syndrome.

OBJECTIVE: To review an institutional experience with the surgical and clinical management of acquired middle ear cholesteatoma in patients with ectrodactyly, ectodermal dysplasia, cleft lip/palate (EEC) syndrome. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Eight patients with medical history significant for EEC syndrome who underwent surgery for acquired middle ear cholesteatoma between 1996 and 2016. INTERVENTION(S): Appropriate surgical interventions at the time of admission. MAIN OUTCOME MEASURE(S): History of ventilation tube insertion, status of the contralateral ear, surgical technique, cholesteatoma recidivism, presence of postoperative external auditory canal stenosis, pre and postoperative audiograms. RESULTS: Cholesteatoma was diagnosed in all patients, 3 (37.5%) unilateral and 5 (62.5%) bilateral, totalizing 13 ears. Six ears (46.2%) underwent a canal wall up mastoidectomy but required conversion to a canal wall down technique in a second procedure due to recurrent cholesteatoma. In the remaining seven ears (53.8%) a canal wall down mastoidectomy was performed. Of all meatoplasty performed, seven (53.8%) evolved with stenosis of the external auditory canal. CONCLUSIONS: 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.

Implante coclear na síndrome Kearns-Sayre: estudo de caso de irmãs gêmeas / Cochlear implant in Kearns-Sayre syndrome: case study of twin sisters

RESUMO A deficiência auditiva pode estar relacionada com diversos fatores, entre eles, a perda auditiva decorrente de determinadas síndromes genéticas. A síndrome Kearns-Sayre é caracterizada por mutações no DNA (deoxyribonucleic acid) mitocondrial, responsável pela produção de energia (trifosfato de adenosina - ATP), que é de extrema importância para o desenvolvimento de estruturas que dela necessitam, como a cóclea. O caso foi acompanhado no hospital, desde o ano 2000, devido à característica progressiva da perda auditiva constatada nos exames audiológicos e nos achados em casos relacionados à síndrome. A intervenção com os aparelhos de amplificação sonora individual (AASI) se mostrou pouco benéfica para a boa comunicação oral de uma das pacientes, que passou a ter o diagnóstico de perda auditiva profunda bilateral. Assim, após discussões em reuniões clinicas, a equipe optou pela indicação do implante coclear para a paciente, segundo os critérios atuais para indicação desta cirurgia, e com o qual obteve bons resultados. Sua irmã gêmea, que apresentou bons resultados com AASI, continuará em acompanhamento audiológico, para se verificar a evolução do caso e discutir uma nova conduta, caso necessário. Pacientes com suspeita, ou diagnóstico de síndrome Kearns-Sayre devem buscar o diagnóstico audiológico, pois se trata de uma possível perda auditiva progressiva, sendo necessária a reabilitação com o uso de dispositivos auditivos. Manter a comunicação oral é de extrema importância, pois, nestes casos, outras funções serão prejudicadas, como o tônus muscular e a visão.

ABSTRACT Hearing impairment may be related to several factors, including hearing loss due to certain genetic syndromes. Kearns-Sayre syndrome is characterized by mutations in mitochondrial DNA (deoxyribonucleic acid), responsible for energy production (adenosine triphosphate - ATP), which is extremely important for the development of structures that need it, such as the cochlea. The case was followed in the hospital, since 2000, due to the progressive characteristic of the hearing loss observed in the audiological examinations and the findings in cases related to the syndrome. The intervention with individual sound amplification devices (AASI) proved to be of little benefit to the good oral communication of one of the patients, who was diagnosed as having bilateral profound hearing loss. Thus, after discussions at 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. Her twin sister, who presented good results with AASI, will continue to undergo audiological follow-up, to verify the evolution of the case and discuss a new conduct, if necessary. Patients with suspected or diagnosed Kearns-Sayre syndrome should seek the audiological diagnosis, since 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.

An Implantable Hearing System As Rehabilitation for Hearing Loss Due to Bilateral Aural Atresia: Surgical Technique and Audiological Results.

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.