Caracterização do potencial evocado auditivo de tronco encefálico em recém-nascidos infectados pelo Zika vírus / Characterization of auditory brainstem response in newborns infected by Zika virus

RESUMO Objetivo Caracterizar as avaliações seqüenciais do potencial evocado auditivo de tronco encefálico em recém-nascidos infectados pelo Zika vírus, correlacionando com a presença de microcefalia e com os sintomas de Zika nas mães durante a gestação. Métodos Estudo descritivo, longitudinal e quantitativo, do qual participaram 20 recém-nascidos, filhos de mães infectadas pelo Zika vírus no período gestacional. Foram analisados os prontuários desses bebês, que passaram por duas avaliações eletrofisiológicas, uma no primeiro mês de vida e outra, após 6 meses. Os dados comparativos foram tabulados e analisados por meio de estatística descritiva e inferencial. Resultados Setenta por cento dos bebês apresentaram microcefalia e 55% das mães tiveram os sintomas da infecção pelo Zika no primeiro trimestre de gestação. Não houve alteração significativa dos limiares eletrofisiológicos em nenhum dos momentos. Houve mudança estatisticamente significativa, principalmente das latências das ondas III e V, entre os exames, caracterizando maturação da via auditiva nos bebês. Não foi encontrada correlação entre a microcefalia e alterações nas latências do PEATE. Conclusão Bebês portadores de Zika apresentaram limiares eletrofisiológicos dentro da normalidade e diminuição das latências absolutas das ondas III e V e interpicos, confirmando a ação citotóxica do Zika. Houve dois casos de piora significativa do limiar eletrofisiológico. Não foi observada correlação entre resultados do PEATE e época de aparecimento dos sintomas durante a gestação, ou a presença de microcefalia.

ABSTRACT Purpose To characterize sequential assessments of auditory brainstem responses in newborns infected by zika virus, correlating with presence of microcephaly and with Zika virus symptoms in mothers during pregnancy. Methods A descriptive, longitudinal and quantitative study, in which 20 newborns, children of mothers infected by Zika virus during pregnancy, participated. Medical records of these babies were analyzed, and they underwent two electrophysiological assessments, one in the first month of life and the other, after 6 months. Comparative data were tabulated and analyzed using descriptive and inferential statistics. Results Seventy percent of babies had microcephaly and 55% of mothers had symptoms of Zika infection in the first trimester of pregnancy. There was no significant alteration in electrophysiological thresholds at any moments. There was a statistically significant change, mainly in the latencies of waves III and V, between the tests, characterizing maturation of the auditory pathway in babies. No correlation was found between microcephaly and changes in ABR latencies. Conclusion Babies with Zika had normal electrophysiological thresholds and decreased absolute latencies of waves III and V and interpeaks, confirming the cytotoxic action of Zika. There were two cases of significant worsening of the electrophysiological threshold. There was no correlation between ABR results and time of onset of the symptoms during pregnancy, or presence of microcephaly.

Alterações retrococleares na esclerose sistêmica: relato de casos / Retrocochlear impairments in systemic sclerosis: a case report study

RESUMO Objetivo Relatar três casos de pacientes portadores de Esclerose Sistêmica e que apresentaram alterações retrococleares. Método Trata-se de um estudo de relato de casos de três indivíduos com esclerose sistêmica e alteração retrococlear, acompanhados em um serviço de reumatologia. Todos os pacientes realizaram o Potencial Evocado Auditivo de Tronco Encefálico e, quando necessário, nova audiometria. Resultados Todos os indivíduos apresentaram perda auditiva do tipo sensorioneural. Não foi identificado na avaliação audiológica básica qualquer resultado que sugerisse alteração retrococlear, porém o PEATE apresentou-se alterado. Conclusão Pode-se concluir que o estudo revela alterações retrococleares nesta população, ocorrendo tanto nas latências absolutas quanto no intervalo interpico. E, neste contexto, reumatologistas e fonoaudiólogos, ao acompanharem pacientes com esclerose sistêmica, devem estar atentos para a possibilidade da ocorrência dessa alteração nessa população. Revela também, a necessidade de estudos epidemiológicos sobre o tema.

ABSTRACT Purpose To report three cases of patients with Systemic Sclerosis (SSc) and retrocochlear impairments. Methods This is a case report of three individuals with SSc and retrocochlear impairments assisted at a rheumatology outpatient clinic. All individuals underwent Brainstem Auditory Evoked Potential (BAEP) and, when necessary, audiometry. Results All three individuals presented sensorineural hearing loss. Although no retrocochlear impairment was identified in the basic audiologic evaluation, the BAEP results were altered. Conclusion Retrocochlear impairments were present in the individuals under study, both in the absolute latencies and interpeak interval, thereby demanding the attention of rheumatologists and speech-language pathologists to such changes during the monitoring of SSc patients. The results also show a need for epidemiological studies on the theme.

Monitoring the Hearing Handicap and the Recognition Threshold of Sentences of a Patient with Unilateral Auditory Neuropathy Spectrum Disorder with Use of a Hearing Aid

Abstract Introduction Treatment for auditory neuropathy spectrum disorder (ANSD) is not yet well established, including the use of hearing aids (HAs). Not all patients diagnosed with ASND have access to HAs, and in some cases HAs are even contraindicated. Objective To monitor the hearing handicap and the recognition threshold of sentences in silence and in noise in a patient with ASND using an HA. Resumed Report A 47-year-old woman reported moderate sensorineural hearing loss in the right ear and high-frequency loss of 4 kHz in the left ear, with bilateral otoacoustic emissions. Auditory brainstem response suggested changes in the functioning of the auditory pathway (up to the inferior colliculus) on the right. An HA was indicated on the right. The patient was tested within a 3-month period before the HA fitting with respect to recognition threshold of sentences in quiet and in noise and for handicap determination. After HA use, she showed a 2.1-dB improvement in the recognition threshold of sentences in silence, a 6.0-dB improvement for recognition threshold of sentences in noise, and a rapid improvement of the signal-to-noise ratio from +3.66 to -2.4 dB when compared with the same tests before the fitting of the HA. Conclusion There was a reduction of the auditory handicap, although speech perception continued to be severely limited. There was a significant improvement of the recognition threshold of sentences in silence and in noise and of the signal-to-noise ratio after 3 months of HA use.

A Case of Acute Bilateral Retrocochlear Hearing Loss as an Initial Symptom of Unilateral Thalamic Hemorrhage

A speech discrimination test is a test using a list of 25 phonetically balanced monosyllables. It is often overlooked but significant enough for pure tone audiometry. Many physicians have performed pure tone audiometry but without a speech discrimination test. A 73-year-old woman visited our clinic complaining of sudden bilateral hearing loss. Pure tone audiometry showed only bilateral high frequency loss. However, speech discrimination had decreased markedly. We decided to follow-up after 1 week of Ginexin-F(R) (ginkgo leaf extract) and Nafril(R) (nafronyl oxalate). She felt a gait disturbance within 2 days. Magnetic resonance imaging revealed a left thalamic hemorrhage. After a 1 month hospitalization, the hematoma subsided, and speech discrimination recovered 3 months later. Acute hearing loss due to thalamic hemorrhage that recovered has never been reported. We report the first case of retrocochlear hearing loss that occurred with a thalamic hemorrhage in a patient who recovered.

An Audit of Otorhinolaryngological Practice in a Tertiary Institution in the Niger Delta Region of Nigeria

The Niger Delta University Teaching Hospital (NDUTH) is a young tertiary hospital with recently established department in various specialties. In spite of this it caters for the health needs of a lot of the patients in Bayelsa state and its environs. The aim of this study therefore is to audit the otorhinolaryngological (ORL) practice in this center with the aim of establishing the pattern of ORL disease which is useful for the planning of effective ORL services Methods: This is a retrospective study of patients that presented to the ear; nose and throat department of NDUTH from January 2007 to December 2011. The patient's folders and clinic records were the source of data.Results: There were 2;275 patients seen within the period under study. The age ranged from 3months to 80 years. Patients who were in the 21-30 year age group (21.1) were the most frequently seen. There were 1;150(50.55) males and 1;125(49.45) females with a male female ratio of 1.02:1. The adults were 1;725(75.82) while the children were 550(24.18). Otologic conditions ranked highest in the clinic presentations with wax impaction being the commonest ear disorder encountered. There were about 573 (25.19) procedures done within this period. Aural syringing was the commonest procedure. There was no major theatre procedures carried out.Conclusion: The otorhinolaryngologic practice in the centre is growing and otologic diseases appear to be the most common conditions seen. There is therefore need to equip both the theatre and clinic so as to offer effective care. Audiololgical services also are relevant for the rehabilitation of these patients in view of the considerable number with hearing loss. The ORL practice here appears quite elementary. Therefore there is a need to vigorously equip the department

Clinical Application of 3D-FIESTA Image in Patients with Unilateral Inner Ear Symptom

BACKGROUND AND OBJECTIVES: Unilateral auditory dysfunction such as tinnitus and hearing loss could be a warning sign of a retrocochlear lesion. Auditory brainstem response (ABR) and internal auditory canal magnetic resonance image (MRI) are suggested as novel diagnostic tools for retrocochlear lesions. However, the high cost of MRI and the low sensitivity of the ABR test could be an obstacle when assessing patients with unilateral ear symptoms. The purpose of this study was to introduce the clinical usefulness of three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) MRI in patients with unilateral ear symptoms. SUBJECTS AND METHODS: Two hundred and fifty-three patients with unilateral tinnitus or unilateral hearing loss who underwent 3D-FIESTA temporal bone MRI as a screening test were enrolled. We reviewed the abnormal findings in the 3D-FIESTA images and ear symptoms using the medical records. RESULTS: In patients with unilateral ear symptoms, 51.0% of the patients had tinnitus and 32.8% patients were assessed to have sudden sensory neural hearing loss. With 3D-FIESTA imaging, twelve patients were diagnosed with acoustic neuroma, four with enlarged vestibular aqueduct syndrome, and two with posterior inferior cerebellar artery aneurysm. Inner ear anomalies and vestibulocochlear nerve aplasia could be diagnosed with 3D-FIESTA imaging. CONCLUSIONS: 3D-FIESTA imaging is a highly sensitive method for the diagnosis of cochlear or retrocochlear lesions. 3D-FIESTA imaging is a useful screening tool for patients with unilateral ear symptoms.

Aplicação da audiometria troncoencefálica na detecção de perdas auditivas retrococleares em trabalhadores de manutenção hospitalar expostos a ruído / Auditory brainstem responses evaluation of retrocochlear disease in workers from a general hospital exposed to noise

O objetivo principal deste estudo seccional foi averiguar a presença de comprometimento auditivo retrococlear num grupo de trabalhadores de manutenção de um hospital de grande porte com histórico de exposição a ruído. Foram avaliados 31 trabalhadores de três setores da divisão de engenharia entre 25 e 60 anos e com exposição contínua a ruído entre 2 e 45 anos. O processo avaliativo contou com triagem audiométrica ocupacional e audiometria troncoencefálica (ATE). Foram detectadas anormalidades na ATE em sete pacientes (22,6 por cento), caracterizadas por aumento de latências de ondas III (14,3 por cento) e V (28,6 por cento); prolongamento dos interpicos I-III (71,4 por cento), III-V (28,6 por cento) e I-V (85,7 por cento). Das 35 orelhas com audição normal (três unilateralmente e 32 bilateralmente), quatro (11,4 por cento) apresentaram comprometimento retrococlear. A alta prevalência de comprometimento retrococlear no grupo induz supor que tal distúrbio seja mais frequente que o encontrado e esteja sendo subestimado na investigação diagnóstica desses trabalhadores. A ocorrência desses resultados sem a presença de alterações audiométricas sugere que a ATE seja mais sensível que a audiometria tonal na investigação de perda auditiva provocada por ruído, por isso sua utilização deva ser incentivada.

The main purpose of this cross-seccional study was to investigate the presence of retrocochlear disease in a group of maintenance workers from a general hospital, who presents a history of noise exposure. Thirty one workers of three engineering sections with age range from 25 to 60 years and continuous noise exposure from 2 and 45 years, were examined. The evaluation included an audiometric occupational selection and auditory brainstem responses (ABR). ABR abnormalities were detected in seven patients (22.6 percent) and it was found latency increase of waves III (14.3 percent) and V (28.6 percent), and interpeak prolongation I-III (71.4 percent), III-V (28.6 percent) and I-V (85.7 percent). Among 35 ears with normal audition right, left or both , four (11,4 percent) ears presented retrocochlear disease. The high retrocochlear disease prevalence in workers exposed to noise lead us to suppose that this disturbance is more frequent than usually found; therefore it is underestimated in workers diagnostic evaluation. The presence of this kind of disturbance, even with the absence of audiometric alterations, suggests that ABR is more sensitive than the tonal audiometry for noise induced hearing loss investigation.

Outcome of cochlear implantation in prelingual pediatric auditory neuropathy / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To explore the electrophysiological results and rehabilitation outcome of two prelingually deafened pediatric cochlear implant patients with auditory neuropathy.</p><p><b>METHODS</b>Preoperative audiological evaluation, intra-postoperative electrically evoked auditory brainstem response (EABR) and neural response telemetry (NRT) record for the two cases were conducted in Beijing Tongren Hospital. A one year follow-up was performed. Data collected before and at 6,12-month intervals after implantation were compared with that from control pediatric cochlear implant patients matched for the same duration of implant use as this two cases.</p><p><b>RESULTS</b>The two children implanted had not had any postoperative medical or cochlear implant device complications. Intraoperative EABR and NRT were elicited in case 1 with unrepeatable waveforms. After 12 months of training, Case 1 had shown significant improvements in sound detection, speech perception abilities and communication skills, which was better than the control group, and the electrophysiological results became normal. Case 2 had also benefited from cochlear implantation, even though no recognizable NRT was found until he returned 12 month after the operation. CONTUSIONS: The desynchronization of auditory path had been changed after the electrical stimulation ongoing 12 months for children with auditory neuropathy. The two children had not had any complications postoperatively, and each child had shown improved listening and communication skills. Cochlear implantation could help patients with auditory neuropathy to improve their communication skill and go back to the main stream.</p>

Audiological characteristics and localization of lesions in children with retrocochlear auditory nerve impairment / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To explore the clinic characteristics, audiological characteristics and location of lesions in children with retrocochlear auditory nerve impairment which, including auditory neuropathy characterized by severely abnormal results of ABR and normal results of DPOAE.</p><p><b>METHODS</b>Between 2002 and 2006, eighty-six cases (165 ears) with severely abnormal ABR but normal results of DPOAE were enrolled in the study group. The mean patient age was one year and one-month-old, with a range of 8 days to 7 years. The cases with abnormal conductive function were excluded. The cases with severely abnormal ABR and normal cochlear functions as measured by DPOAE and without abnormal conductive function were selected as the cochlear lesion group. Some same age healthy children without hearing loss were subjected as normal control group. The latency and amplitude of waves I, III and V, the inter peak latency I-III was compared among the three group.</p><p><b>RESULTS</b>(1) Fifty-one cases (59.3%) had a history of hypercholesterolemia during neonatal period, but 40 cases (46.51%) had a severe hypercholesterolemia and 11 cases (12.79%) had mild or moderate hypercholesterolemia. Clinical features common among the population included a history of dyskinesia [n = 40 (46.51%)], hearing and language disorder [n = 10 (11. 63%)]. Thirty-two cases (37.2%) were accompanied by cerebral palsy . (2) Among the 165 ears, absent ABRs to click stimuli presented at 103 dB was in 103 ears, only wave I was developed in 27 ears and only wave V was developed in 19 ears,wave I and III in 13 ears and differentiated wave I and V in 3 ears. (3) When compared to control group, the latency of wave I was prolonged and amplitude of wave I was lower in cases with only wave I developed (t = -6.75 and 2.58, P < 0.05). For for cases with only wave I and III differentiated, the latency and amplitude of wave I was the same but the latency of wave III was prolonged and amplitude of wave III was lower while interpeak latency I-III was prolonged.</p><p><b>CONCLUSIONS</b>Auditory neuropathy which was characterized by severely abnormal ABR was the most common type of retrocochlear auditory nerve impairment. It was mainly due to a disorder of VIII nerve. The pathologies that affect higher levels of the auditory pathway, from the brainstem to the auditory cortex, might be the main sites of lesion in cases with only wave I developed. Superior olivary nucleus where wave III was generated and higher levels of the auditory pathway might be the main sites of lesion in cases with wave I and III differentiated. The low-amplitude wave V was not characteristics of auditory neuropathy. Cerebral cortex, brain stem auditory nucleus and VIII nerve might be damaged successively in cases with retrocochlear auditory nerve impairment induced by hypercholesterolemia.</p>

Identification of cochlear and Retro cochlear causes in patients with tinnitus

This study was designed to identify the possible underlying site of lesion in patients with tinnitus through using Transient Evoked otoacoustic Emissions [TEOAEs], Auditory Brainstem Response [ARR] and Electrocochleography [ECOchG]. Forty subjects complaining of tinnitus were examined. The control group consisted of'24 ears with normal hearing sensitivity not involved with tinnitus. The study group comprised of 36 ears with hearing loss and 20 ears with normal hearing thresholds. TEOAEs revealed that 88.8% of tinnitus ears with hearing loss partially passed or failed to pass the emissions. Interestingly, 20% of tinnitus ears with normal hearing full in the fail to pass category. There was statistically significant difference between control amid study group for absolute latency of wave 1. In ABR test. EcochG results showed that there was no statistically significant difference of the summation potential [SP], action potential [AP] and SP/AP ratio. On individual analysis, two subjects showed increased SP/AP ratio who were finally diagnosed as having Meniere's disease. One subject revealed manifestations of retrocochlear lesion in ABR test results and proved later to have cerebello-pontine angle mass. The combination of TEOAEs, ABR and EcochG is useful to identify site of lesion in tinnitus subjects. EcochG is valuable in diagnosis of endolymphatic subjects. EcochG is valuable in diagnosis of endolymphatic hydrops, ABR for detection of retrocochlear lesion while TEOAEs is very promising for early detection of hidden cochlear pathology

Estudo da supressão das emissões otoacústicas com a utilização de estímulos sonoros contralaterais em indivíduos de audição normal e em pacientes com doenças retrococleares / Study of Suppression of Otoacoustic Emissions Using Contralateral Sound Stimuli in Normal Hearing Subjects and in Patients with Retrocochlear Diseases

As emissões-otoacústicas captadas no meato acústico externo fornecem informações a respeito da integridade dos mecanismos receptores cocleares pré-sinópticos; e, portanto, da função coclear. Tem sido estudada a participação do sistema nervoso olivococlear eferente, principalmente de seu feixe medial, na modulação dessas respostas. A estimulação contralateral por ruídos de banda larga ou estreita é considerada ativadora desse sistema, podendo, como conseqüência, produzir a diminuição dos valores das amplitudes dessas emissões, desde que o controle neural esteja íntegro e com função normal. Material e métodos: Para se verificar a existência de supressão dessas respostas, foi feita a comparação dos registros das emissões otoacústicas, produto de distorção é transientes, usando-se click linear e não-linear, com e sem estimulação contralateral, em uni grupo de 48 indivíduos com audição normal; e em outro grupo, constituído de nove pacientes com doenças retrococleares. Resultados: No grupo normal, encontrou-se supressão de 1 a 3 decibéis, em média, para emissões transientes e de 0,5 a 2 decibéis, em média, para emissões, produto de distorção. No grupo com lesão retrococlear, ora havia supressão discreta, ora não havia nenhuma ou, então, até podia ocorrer intensificação das emissões. Conclusão: Ao se comparar os dois grupos, verificaram-se diferenças estatisticamente significantes, nas emissões transientes, em 1.000 Hertz, 1.500 Hertz, 2.000 Hertz e 3.000 Hertz, com click linear e em 2.000 Hertz e 3.000 Hertz, cone click não-linear. Estas diferenças nas emissões produto de distorção, foram verificadas em 750 Hertz, 1.000 Hertz e 2.000 Hertz.

The otoacoustic emissions captured in the external acoustic meatus afford information concerning the integrity of pre-synaptic cochlear receptor mechanisms and, therefore, of cochlear function. Participation of the efferent olivocochlear - nervous system has been studied, mainly of the medial bundle, in modulating these responses. Contralateral stimulation by wide or narrow band noise is considered an activator of this system; and may, as a consequence, produce diminished values of the amplitudes of these emissions, provided the neural control is integral and of normal function. Material and methods: To verify, the existence of suppression to these responses was made a comparison between the registers of distortion product and transientotoacoustic emissions using a linear and nonlinear click, with and without contralateral stimulation, in a group of 48 individuals with normal audition and another group consisting of 9 patients with retro cochlear diseases. In the normal group, suppression was found, on average, of 1 to 3 decibels, for transient emissions and of 0.5 to 2 decibels, on average, for distortion product emissions. Results: In the group with retrocochlear lesion, either there was discreet suppression or there was none, or else, there might even be an intensifying of the emissions. Conclusion: When we compare the two groups, we verified there was a statistically significant differences, in transient emissions, in 1,000 Hertz,1,500 Hertz, 2,000 Hertz, and 3,000 Hertz, when we used a linear click, and in 2,000 Hertz and 3,000 Hertz with nonlinear click. These differences, in distortion product emissions, were in 750 Hertz, 1,000 Hertz and 2,000 Hertz.

Efficacy of Audiologic Tests in the Differential Diagnosis of Cochlear and Retrocochlear Hearing Loss / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: There are many audiological tests to distinguish between the hearing loss patients of cochlear lesion and the retrocochlear lesion. We started this research to compare the efficacy of the audiological tests and to make helpful selections of the auditory hearing tests in diagnosis of the sensorineural hearing loss patients. MATERIALS AND METHOD: Our study was conducted on 198 patients with hearing loss. They received otologic physical examination, neurological examination, and MRI. We evaluated SISI, STAT, ARDT, DPOAE, and ABR data values and analysed their sensitivity and specificity. RESULTS: Sensitivity of SISI was 55% and specificity was 74%. Sensitivity of STAT was 58% and specificity was 67%. Sensitivity of ARDT was 44% and specificity was 71%. Sensitivity of DPOAE was 50% and specificity was 64%. Sensitivity of ABR was 90% and specificity was 74%. CONCLUSION: Among the tests performed above, ABR showed the highest sensitivity and specificity value, which implies it to be the most meaningful screening test in the sensorineural hearing loss patients. But for the patients with severe hearing loss, such that the auditory hearing test results are suspicious in its accuracy, or for the patients who are suspicious of retrocochlear lesions based upon multiple tests, MRI should be performed to make the final diagnosis.

Emisiones otoacústicas en disfunciones auditivas retrococleares / Otoacustic emissions in retrocochlear auditory disfunctions

Se analizan las Emisiones Otoacusticas Espontáneas y de Productos de Distorsion obtenidos en enfermos con hipoacusias progresivas o subitas, acompanadas de tinnitus y algunos de mareos. Y se describe la utilidad clinica práctica de ellas en el diagnostico diferencial entre lesiones cocleares o retrococleares