Preliminary application of combined auditory monitoring technique in resection of vestibular neurinoma / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the value of electrically evoked auditory brainstem response (EABR) monitoring combined with brainstem auditory evoked potential (BAEP) and compound action potential (CAP) monitoring during vestibular schwannoma resection for the protection of the cochlear nerve. Methods: Clinical data from 12 patients with vestibular schwannomas who had useful hearing prior to surgery were analyzed at the PLA General Hospital from January to December 2021. Among them, there were 7 males and 5 females, ranging in age from 25 to 59 years. Before surgery, patients underwent audiology assessments (including pure tone audiometry, speech recognition rate, etc.), facial nerve function evaluation, and cranial MRI. They then underwent vestibular schwannoma resection via the retrosigmoid approach. EABR, BAEP, and CAP were simultaneously monitored during surgery, and patients' hearing preservation was observed and analyzed after surgery. Results: Prior to surgery, the average PTA threshold of the 12 patients ranged from11 to 49 dBHL, with a SDS of 80% to 100%. Six patients had grade A hearing, and six patients had grade B hearing. All 12 patients had House-Brackman grade I facial nerve function prior to surgery. The MRI indicated tumor diameters between 1.1 and 2.4 cm. Complete removal was achieved in 10/12 patients, while near-total removal was achieved in 2/12 patients. There were no serious complications at the one-month follow-up after surgery. At the three-month follow-up, all 12 patients had House-Brackman grade I or II facial nerve function. Under EABR with CAP and BAEP monitoring, successful preservation of the cochlear nerve was achieved in six of ten patients (2 with grade B hearing, 3 with grade C hearing, and 1 with grade D hearing). Successful preservation of the cochlear nerve was not achieved in another four patients (all with grade D hearing). In two patients, EABR monitoring was unsuccessful due to interference signals; however, Grade C or higher hearing was successfully preserved under BAEP and CAP monitoring. Conclusion: The application of EABR monitoring combined with BAEP and CAP monitoring during vestibular schwannoma resection can help improve postoperative preservation of the cochlear nerve and hearing.

Bilateral symmetrical maculopathy and heterochromia in Waardenburg syndrome / Maculopatia simétrica bilateral e heterocromia na síndrome de Waardenburg

ABSTRACT Waardenburg syndrome is a rare congenital genetic disorder characterized by sensorineural hearing loss and pigmentary abnormalities of the hair, skin, and eyes. Based on the different clinical presentations, it is divided into four subtypes as in WS1 to WS4. This report describes a 15-year-old boy who presented with low vision and bilateral hearing loss. His visual acuity was 20/200 in both eyes. Slit-lamp examination revealed complete iris heterochromia, with one blue iris and one brown iris. Fundus examination showed symmetrical pigmentation of the retina and choroid, with atrophy of the pigment epithelium in the macular region, notably also in the eye with normal iris pigment illustrating the broad spectrum of the iris and fundus pigmentation as part of this syndrome. A carefully clinical and ophthalmological evaluation should be done to differentiate various types of Waardenburg syndrome and other associated auditory-pigmentary syndrome. Early diagnosis in some cases may be crucial for the adequate development of patients affected with this condition.

RESUMO A síndrome de Waardenburg é uma doença genética congênita rara caracterizada por perda auditiva neurossensorial e anormalidades pigmentares do cabelo, da pele e dos olhos. Com base nas diferentes apresentações clínicas, é dividida em quatro subtipos (WS1 a WS4). Este relato descreve o caso de um menino de 15 anos que apresentava baixa visão e perda auditiva bilateral. Sua acuidade visual era de 20/200 em ambos os olhos. O exame em lâmpada de fenda revelou heterocromia completa da íris, com uma íris azul e uma íris marrom. A fundoscopia mostrou pigmentação simétrica da retina e coroide, com atrofia do epitélio pigmentar na região macular, notadamente também no olho com pigmento de íris normal, ilustrando o amplo espectro de pigmentação de íris e fundo como parte dessa síndrome. Uma avaliação clínica e oftalmológica criteriosa deve ser feita para diferenciar os vários tipos de síndrome de Waardenburg e outras síndromes auditivo-pigmentares associadas. O diagnóstico precoce em alguns casos pode ser crucial para o desenvolvimento adequado dos pacientes acometidos por essa condição.

Emissões otoacústicas evocadas na doença de Ménière / Otoacoustic emissions evoked in Ménière's disease

RESUMO Objetivo Verificar as respostas das emissões otoacústicas (EOA) evocadas por estímulo transiente e produto de distorção em indivíduos com doença de Ménière. Métodos Estudo transversal com casuística composta por 60 indivíduos de 19 a 75 anos de idade, distribuídos em dois grupos: grupo estudo, com 32 indivíduos com diagnóstico médico de doença de Ménière, sem outros riscos, e grupo controle formado por 28 indivíduos com perda coclear, sem doença de Ménière, pareado por idade e gênero ao grupo estudo. Critério de elegibilidade: curva tipo A, sem perda condutiva ou mista ou suspeita de alteração retrococlear. A avaliação audiológica foi composta por anamnese, inspeção do meato acústico externo, audiometria tonal limiar, logoaudiometria, medidas de imitância acústica e emissões otoacústicas evocadas por estímulo transiente e produto de distorção. Resultados Os indivíduos com Ménière apresentaram maior ocorrência de perda unilateral, zumbido pitch grave, vertigem e plenitude auricular em relação ao controle. Nesses indivíduos, houve maior incompatibilidade entre os resultados das EOA e da audiometria tonal: nas perdas unilaterais, observaram-se alterações nas EOA nas orelhas com limiares auditivos normais do lado contralateral, caracterizando disfunções cocleares. Nas orelhas com perda coclear, houve presença de EOAT (por estímulo transiente) e ausência de EOAPD (produto de distorção), contrapondo-se ao grupo controle, que apresentou ausência de EOAT e de EOAPD, como o esperado em perdas cocleares de outras etiologias. Conclusão A pesquisa das emissões na doença de Ménière identificou disfunção coclear na orelha contralateral nos casos unilaterais e presença de EOAT com ausência de EOAPD nas orelhas com perda auditiva, diferenciando-se das perdas cocleares de outras etiologias.

ABSTRACT Purpose To verify the responses of Evoked Otoacoustic Emissions by transient stimulus and distortion product in individuals with Ménière's Disease. Methods Cross-sectional study with a sample composed of 60 individuals, aged 19 to 75 years, divided into two groups: study group, with 32 individuals with a medical diagnosis of Ménière's disease, without other risks and a control group formed by 28 individuals with cochlear loss without Meniere's disease, age and sex matched to the study group. Eligibility criteria: type A curve, without conductive or mixed loss or suspected retrocochlear alteration. The audiological evaluation consisted of anamnesis, inspection of the external acoustic meatus, pure tone audiometry, logoaudiometry, measures of acoustic immittance and transient evoked otoacoustic emissions and distortion product. Results Individuals with Ménière's disease had a higher occurrence of unilateral hearing loss, low pitch tinnitus, vertigo and ear fullness in relation to the control. In these individuals, there was greater incompatibility between the results of OAE and pure tone audiometry: in unilateral hearing loss, alterations in OAE were observed in ears with normal hearing thresholds on the contralateral side, characterizing cochlear dysfunctions. In the ears with cochlear loss, there was the presence of TEOAE and absence of DPOAE, in contrast to the control group, which showed the absence of TEOAE and DPOAE, as expected in cochlear losses of other etiologies. Conclusion The investigation of emissions in Ménière's disease identified cochlear dysfunction in the contralateral ear in unilateral cases and the presence of TOAE with absence of DPOAE in ears with hearing loss, differentiating from cochlear losses of other etiologies.

Hipoacusia sensorioneural unilateral: Revisión de los manejos posibles en nuestra realidad nacional / Unilateral sensorineural hearing loss: Review of possible management in our national reality

La hipoacusia unilateral (HUL) definida como la pérdida auditiva de cualquier grado en un solo oído tiene prevalencias en edad escolar de 3% a 6%. La etiología es desconocida en la mayoría de los casos, pero destacan las anormalidades anatómicas severas a diferencia de las hipoacusias bilaterales, lo que le otorga importancia al estudio de imágenes. También se recomienda realizar evaluación oftalmológica. El impacto de la HUL se observa en la localización sonora, reconocimiento del habla, desarrollo del lenguaje, desempeño social y conducta, pero por sobre todo en el rendimiento académico. En este sentido es relevante mencionar que además de existir mayor probabilidad de repitencia de curso, es necesario mayor apoyo educacional individualizado para evitar dicha repitencia o lograr mismo éxito académico que un normoyente. En relación al diagnóstico aún faltan normas o protocolos, siendo éste un desafío en las zonas donde no hay screening universal. Se discute la importancia de las encuestas para realizar evaluaciones y seguimiento en estos casos. Finalmente se abordan las estrategias de intervención disponibles, además de un plan individualizado considerando al niño, su familia y sus expectativas, particularmente se discuten las características o factores a tomar en cuenta en el momento de la adaptación, para así lograr un tratamiento tendiente al éxito.

Unilateral hearing loss (UHL) defined as hearing loss of any degree in only one ear has a school-age prevalence of 3% to 6%. The etiology is unknown in most cases, but severe anatomical abnormalities stand out as opposed to bilateral hearing loss, which gives importance to the study of images. Ophthalmological evaluation is also recommended. The impact of UHL is observed in sound localization, speech recognition, language development, social performance and behavior, but above all in academic performance. In this sense, it is relevant to mention that in addition to having a higher probability of repeating the course, it is necessary to have more individualized educational support to avoid this repetition or achieve the same academic success as a normal listener. Regarding the diagnosis, there are still missing norms or protocols, this being a challenge in areas where there is no universal screening. The importance of surveys to carry out evaluations and follow-up in these cases is discussed. Finally, the available intervention strategies are discussed, in addition to an individualized plan considering the child, his family and his expectations, particularly, the factors to be taken into account at the time of adaptation, in order to achieve a success treatment.

Jugular Foramen's Paraganglioma in a Patient with Von Hippel-Lindau Disease: Case Report

Glomus jugular tumors, also known as paragangliomas (PGLs), are rare and related to several clinical syndromes described. These are located in the carotid body, the jugular glomus, the tympanic glomus and the vagal glomus. The symptoms are directly related to the site of involvement and infiltration. These lesions have slow growth, are generally benign and hypervascularized, have a peak incidence between the age of 30 to 50 years old; however, when associated with hereditary syndromes, they tend to occur a decade earlier. Several familial hereditary syndromes are associated with PGLs, including Von Hippel- Lindau disease (VHL) in< 10% of the cases. The diagnosis and staging of PGLs are based on imaging and functional exams (bone window computed tomography [CT] with a "ground moth" pattern and magnetic resonance imaging (MRI) with a "salt and pepper" pattern). The cerebral angiography is a prerequisite in patients with extremely vascularized lesions, whose preoperative embolization is necessary. The histopathological finding of cell clusters called "Zellballen" is a characteristic of PGLs. Regarding the jugular foramen, the combination of two or three surgical approaches may be necessary: (1) lateral group, approaches through themastoid; (2) posterior group, through the retrosigmoid access and its variants; and (3) anterior group, centered on the tympanic and petrous bone. In the present paper, we report a case of PGL of the jugular foramen operated on a young female patientwho underwent a surgery with a diagnosis ofVonHippel-Lindau Disease (VHL) at the Neurosurgery Service of the Hospital Heliópolis, São Paulo, state of São Paulo, Brazil in 2018, by the lateral and posterior combined route.

Sensorineural hearing loss in the acute phase of a single episode of acute otitis media / Perda auditiva neurossensorial no início de um único episódio de otite média aguda

Abstract Introduction: Acute otitis media is a disease with high global prevalence, that can lead to several acute complications and auditory sequelae. Data regarding the auditory evaluation in the acute phase of acute otitis media are scarce. Objective: To evaluate the main audiometric changes (air and bone conduction thresholds) in the initial phase of an acute otitis media episode. Methods: A case-control study was performed. Patients diagnosed with acute otitis media with less than 7 days of evolution in relation to the complaint onset were selected, and healthy volunteers were selected as controls. The acute otitis media and control groups were submitted to pure tone and vocal audiometry. Results: The acute otitis media group included a total of 27 patients (30 ears). Hearing loss was present in 90.0% of the ears with acute otitis media, with conductive loss in 14 (46.67%) and mixed loss in 13 (43.33%). Both the air and bone conduction thresholds obtained with the tonal audiometry in the acute otitis media group were significantly worse than the controls at all tested frequencies (p< 0.05). In patients with acute otitis media, we observed that the thresholds for frequency >1 kHz (bone conduction) and 3 kHz (air conduction) were significantly worse in patients with tinnitus compared to patients without tinnitus. Conclusion: During the first 7 days of evolution after the onset of an isolated episode of acute otitis media, we observed significant increases in bone and air thresholds at all frequencies, especially >2 kHz, compared to healthy ears.

Resumo Introdução: A otite média aguda é uma doença de elevada incidência global, que pode levar a diversas complicações agudas e sequelas auditivas. Dados referentes à avaliação auditiva na fase aguda da otite média aguda são escassos. Objetivo: Avaliar as principais alterações audiométricas (limiares em via aérea e óssea) na fase inicial de um episódio de otite média aguda. Método: Realizou-se estudo de caso-controle. Selecionamos pacientes com diagnóstico de otite média aguda, com menos de sete dias de evolução em relação ao início das queixas, e voluntários saudáveis foram selecionados como controles. Os grupos otite média aguda e controle foram submetidos a audiometria tonal, vocal e audiometria. Resultados: O grupo otite média aguda incluiu 27 pacientes (30 orelhas). Observou-se presença de perda auditiva em 90% das orelhas com otite média aguda, condutiva em 14 (46,67%) e mista em 13 (43,33%). Tanto os limiares auditivos por via aérea quanto os limiares por via óssea obtidos com audiometria tonal do grupo otite média aguda eram significativamente piores em relação aos controles, em todas as frequências testadas (p < 0,05). Em pacientes com otite média aguda, observamos que os limiares das frequências acima de 1 kHz (via óssea) e 3 kHz (via aérea) eram significantemente piores entre pacientes com zumbido em comparação a pacientes sem zumbido. Conclusão: Nos primeiros sete dias de evolução do quadro inicial de um episódio isolado de otite média aguda, observamos aumentos significativos dos limiares ósseos e aéreos em todas as frequências, principalmente nas acima de 2 kHz, em comparação a orelhas sadias.

Application of Cervical Vestibular-Evoked Myogenic Potentials in Adults with Moderate to Profound Sensorineural Hearing Loss: A Preliminary Study

Abstract Introduction The cochlea and the vestibular receptors are closely related in terms of anatomy and phylogeny. Patients with moderate to profound sensorineural hearing loss (MPSHL) should have their vestibular organ functions tested. Objective To evaluate the incidence of vestibular abnormalities in patients with MPSHL and to study the correlation between the etiology of hearing loss (HL) and a possible damage to the labyrinth. Methods A case-control retrospective study was performed. In the case group, 20 adults with MPSHL of known etiology were included. The control group was composed of 15 adults with normal hearing. The case group was divided into 4 subgroups based on the etiology (bacterial meningitis, virus, vascular disease, congenital). Cervical vestibular-evoked myogenic potentials (cVEMPs) were used to rate the saccular function and lower vestibular nerve. Results The study was performed in 70 ears, and it highlighted the presence of early biphasic P1-N1 complex in 29 (71.5%) out of 40 ears in the study group, and in all of the 30 ears in the control group (p = 0.001). Regarding the presence or absence of cVEMPs among the four subgroups of patients with MPSHL, the data were statistically significant (p < 0.001). The comparison between the latencies and amplitude of P1-N1 in case and control groups from other studies and in the four subgroups of cases in the present study did not detect statistically significant differences. Conclusion The present study demonstrates that patients with MPSHL have a high incidence of damage to the labyrinthine organs, and it increases the current knowledge about the etiopathogenesis of sensorineural HL, which is often of unknown nature.

Achados audiológicos na Síndrome de Vogt-Koyanagi-Harada / Audiological findings in the Vogt-Koyanagi-Harada Syndrome

RESUMO A síndrome de Vogt-Koyanagi-Harada (SVKH) é rara, multissistêmica e autoimune. Atinge principalmente os olhos, provocando uma panuveíte crônica bilateral, porém traz afecções em outras áreas e tecidos que são ricos em melanócitos, como olhos, orelha interna, meninges e a pele. Sua origem ainda não é totalmente conhecida. Geralmente, a SVKH atinge indivíduos de origem hispânica, do Oriente Médio, indianos, nativos americanos e asiáticos. Descrição dos aspectos audiológicos acometidos pela síndrome e as possíveis intervenções fonoaudiológicas para um caso específico. Paciente de 53 anos, sexo feminino, compareceu à Clínica de Fonoaudiologia, Faculdade de Odontologia de Bauru (FOB) com queixas audiológicas e diagnóstico médico da SVKH. A paciente apresentou perda auditiva sensório-neural bilateralmente, emissões otoacústicas evocadas ausentes e queixas vestibulares de vertigem postural e desequilíbrio ao andar, bem como queixa de zumbido agudo contínuo. O caso apresentado mostrou perda auditiva sensório-neural, vertigem, zumbido e acometimento ocular bilateral. Apesar do tratamento com corticoesteroide, a perda auditiva se manteve. Desta forma, precedente à indicação do AASI, o fonoaudiólogo deve atentar-se para o acompanhamento audiológico do caso, realização ou não de tratamento medicamentoso e ocorrência de sintomas sugestivos da síndrome, favorecendo o encaminhamento para o médico e participando ativamente do processo terapêutico envolvendo a audição.

ABSTRACT The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic and autoimmune disease. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis, however, the syndrome may also affect the melanocytes tissues, for example, the eyes, inner ear, meninges and skin. The syndrome origin mechanism is not yet completely known. Commonly, the specific ethnic groups that are affected by the VKHS are as follows: Hispanics, Asians, Indians, Native Americans and ethnic groups from the Middle East. The audiological characteristics of the syndrome and the possible audiologist interventions for a specific case will be reported. The patient was attended at the Clinic of Speech Therapy, Faculdade de Odontologia de Bauru (FOB). She is 53 years old and presented audiological complaints. She was diagnosed with VKHS by a specialist doctor. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, complaints about postural vertigo and acute tinnitus. The specific case reported presented sudden sensorineural hearing loss, vertigo, tinnitus and bilateral ocular disease. Even though drug treatment was performed, the hearing loss remained. Therefore, before the hearing aid (HA) fitting, the audiologist should perform the hearing management, investigate if the patient takes the drug treatment and the occurrence of suggestive symptoms of the syndrome. These are some points that help in the reference to the specialist doctor and the audiologist strongly participates in what concerns the hearing.

Estenosis congénita aislada del conducto auditivo interno como causa de hipoacusia neurosensorial unilateral y paresia del nervio facial / Congenital internal auditory canal stenosis presenting with unilateral sensorineural hearing loss and facial nerve dysfunction

RESUMEN La estenosis del conducto auditivo interno con aplasia/hipoplasia del nervio cocleovestibular es una patología muy infrecuente. Suele ser unilateral y puede acompañarse de aplasia/hipoplasia del nervio facial y otras malformaciones del oído interno. Se presentan aquí dos casos clínicos de pacientes pediátricos con estenosis del conducto auditivo interno unilateral con compromiso del séptimo y octavo par craneal ipsilateral. Se describen las historias y evaluaciones clínicas, hallazgos audiovestibulares, hallazgos imagenológicos, tratamientos indicados y sus resultados.

ABSTRACT Congenital internal auditory canal stenosis associated with aplasia/hypoplasia of the cochleovestibular nerve is a very infrequent pathology. It is usually unilateral and may be accompanied by aplasia/hypoplasia of the facial nerve and other malformations of the inner ear. We hereby present two clinical cases of pediatric patients with congenital internal auditory canal stenosis, with involvement of the seventh and eighth ipsilateral cranial nerve. The medical histories and clinical evaluations, audiovestibular findings, imaging findings, treatments and their results are described.

Ototoxicidad inducida por quimio-radioterapia basada en platinos: una revisión / Ototoxicity induced by platinum compounds-based chemoradiotherapy: a review

RESUMEN Los análogos del platino, como el cisplatino, y la radioterapia son usados de forma individual y en conjunto para el tratamiento de diversas neoplasias en población adulta y pediátrica. Sin embargo, el uso de estos tratamientos puede generar ototoxicidad, especialmente cuando son usados de forma combinada para neoplasias que comprometen cabeza y cuello, manifestándose principalmente como una hipoacusia progresiva e irreversible que compromete la calidad de vida. Diversos mecanismos han sido propuestos para explicar el daño en las estructuras auditivas generado por estos tratamientos, incluyendo la producción de especies reactivas del oxígeno y la inflamación, desencadenando muerte celular. Si bien distintas estrategias otoprotectoras han sido probadas en humanos, es aún incierta su efectividad y seguridad en combinación con los tratamientos oncológicos. El objetivo de la siguiente revisión es proporcionar una visión general y actualizada de la ototoxicidad inducida por quimio-radioterapia basada en platinos, discutiendo sus bases, características clínicas, potenciales tratamientos y estrategias preventivas que se han desarrollado en los últimos años.

ABSTRACT Platinum analogues, such as cisplatin, and radiotherapy are used separately or in combination to treat several neoplasms in pediatric and adult populations. Nonetheless, the use of these treatments may lead to ototoxicity, especially when these treatments are concomitantly used to treat head and neck cancers, which can manifest as progressive and irreversible hearing loss that decreases quality of life. Several mechanisms have been proposed in order to explain the damage to the auditory structures induced by these treatment modalities, including: reactive oxygen species production and inflammation, leading to cell death. Although several otoprotective strategies have been attempted in humans, their effectiveness and security are unclear. The objective of this review is to provide an updated and general overview on platinum-based chemoradio-therapy induced ototoxicity, discussing its basis, clinical features, potential treatments and preventive strategies developed in recent years.

Cholesteatoma labyrinthine fistula: prevalence and impact / Fístula labiríntica por colesteatoma: prevalência e impacto

Abstract Introduction: Labyrinthine fistula is one of the most common complications associated with cholesteatoma. It represents an erosive loss of the endochondral bone overlying the labyrinth. Reasons for cholesteatoma-induced labyrinthine fistula are still poorly understood. Objective: Evaluate patients with cholesteatoma, in order to identify possible risk factors or clinical findings associated with labyrinthine fistula. Secondary objectives were to determine the prevalence of labyrinthine fistula in the study cohort, to analyze the role of computed tomography and to describe the hearing results after surgery. Methods: This retrospective cohort study included patients with an acquired middle ear cholesteatoma in at least one ear with no prior surgery, who underwent audiometry and tomographic examination of the ears or surgery at our institution. Hearing results after surgery were analyzed according to the labyrinthine fistula classification and the employed technique. Results: We analyzed a total of 333 patients, of which 9 (2.7%) had labyrinthine fistula in the lateral semicircular canal. In 8 patients, the fistula was first identified on image studies and confirmed at surgery. In patients with posterior epitympanic and two-route cholesteatomas, the prevalence was 5.0%; and in cases with remaining cholesteatoma growth patterns, the prevalence was 0.6% (p = 0.16). In addition, the prevalence ratio for labyrinthine fistula between patients with and without vertigo was 2.1. Of patients without sensorineural hearing loss before surgery, 80.0% remained with the same bone conduction thresholds, whereas 20.0% progressed to profound hearing loss. Of patients with sensorineural hearing loss before surgery, 33.33% remained with the same hearing impairment, whereas 33.33% showed improvement of the bone conduction thresholds' Pure Tone Average. Conclusion: Labyrinthine fistula must be ruled out prior to ear surgery, particularly in cases of posterior epitympanic or two-route cholesteatoma. Computed tomography is a good diagnostic modality for lateral semicircular canal fistula. Sensorineural hearing loss can occur post-surgically, even in previously unaffected patients despite the technique employed.

Resumo Introdução: A fístula labiríntica é uma das complicações mais comuns associadas ao colesteatoma. Representa uma perda erosiva do osso endocondral que recobre o labirinto. As razões para a ocorrência da fístula labiríntica induzida pelo colesteatoma ainda são mal compreendidas. Objetivo: Avaliar pacientes com colesteatoma, a fim de identificar possíveis fatores de risco ou achados clínicos associados à fístula labiríntica. Os objetivos secundários foram determinar a prevalência de fístula labiríntica no estudo de coorte, analisar o papel da tomografia computadorizada e descrever os resultados auditivos após a cirurgia. Método: Este foi um estudo de coorte retrospectivo. Foram incluídos pacientes com colesteatoma adquirido de orelha média em pelo menos um lado sem cirurgia prévia que haviam sido submetidos à audiometria e tomografia computadorizada de orelha ou cirurgia em nossa instituição. Os resultados auditivos após a cirurgia foram analisados de acordo com a classificação de fístula labiríntica e da técnica empregada. Resultados: Analisamos um total de 333 pacientes, dos quais 9 (2,7%) apresentavam fístula labiríntica no canal semicircular lateral. Em 8 pacientes, a fístula foi identificada na tomografia computadorizada e confirmada durante a cirurgia. Em pacientes com colesteatomas epitimpânicos posteriores e de via dupla, a prevalência foi de 5,0%; e nos casos com padrão de crescimento de colesteatoma remanescente, a prevalência foi de 0,6% (p = 0,16). Além disso, a taxa de prevalência de fístula labiríntica entre pacientes com e sem vertigem foi de 2,1. Dos pacientes sem perda auditiva neurossensorial antes da cirurgia, 80,0% permaneceram com os mesmos limiares de condução óssea, enquanto 20,0% progrediram para perda auditiva profunda. Dos pacientes com perda auditiva neurossensorial antes da cirurgia, 33,33% permaneceram com a mesma deficiência auditiva, enquanto 33,33% apresentaram melhora da média de dos limiares de condução óssea aos tons puros. Conclusão: A fístula labiríntica deve ser descartada antes do procedimento cirúrgico, particularmente nos casos de colesteatomas epitimpânicos posteriores e de dupla via. A tomografia computadorizada é uma boa modalidade diagnóstica para a fístula do canal semicircular lateral. A perda auditiva neurossensorial pode ocorrer pós-cirurgicamente, mesmo em pacientes previamente não afetados, a despeito da técnica empregada.

The Relationship between the Etiology of Profound Prelingual Sensorineural Hearing Loss and the Results of Vestibular-Evoked Myogenic Potentials

Abstract Introduction Cervical vestibular-evoked myogenic potentials (cVEMPs) are biphasic, short latency potentials, which represent the inhibition of the contraction of the sternocleidomastoid muscle (SCM) mediated by the saccule, the inferior vestibular nerve, the vestibular nuclei and the medial vestibular spinal tract. Objective To evaluate the response of cVEMPs in individuals with profound prelingual bilateral cochlear hearing loss. Methods A prospective case-control study. A total of 64 volunteers, divided into a study group (31 patients with profound prelingual sensorineural hearing loss) and a control group (33 subjectsmatched for age and gender with psychoacoustic thresholds of ≤ 25 dB HL between 500 and 8,000 Hz) were submitted to the cVEMP exam. The causes of hearing loss were grouped by etiology and the involved period. Results The subjects of the study group aremore likely to present changes in cVEMPs compared to the control group (35.5% versus 6.1% respectively; p = 0.003), with an odds ratio (OR) of 8.52 (p = 0.009). Itmeans that they had 8.52-fold higher propensity of presenting altered cVEMP results. There were no statistically significant differences between the latencies, the interamplitude and the asymmetry index. Regarding the etiology, there was a statistically significant difference when the cause was infectious, with an OR of 15.50 (p = 0.005), and when the impairment occurred in the prenatal period, with an OR of 9.86 (p = 0.009). Conclusion The present study showed abnormalities in the sacculocolic pathway in a considerable portion of individuals with profound prelingual sensorineural hearing loss due to infectious and congenital causes, as revealed by the cVEMP results. (AU)

Hipoacusia en enfermedad de Vogt-Koyanagi-Harada: caso clínico y revisión de literatura / Hearing loss in Vogt-Koyanagi-Harada Disease: case report and literature review

RESUMEN La patología autoinmune de oído interno se caracteriza por presentar hipoacusia sensorioneural bilateral, asimétrica y progresiva, que responde a terapia médica, presentándose aislada o como parte de una enfermedad sistémica como la enfermedad de Vogt-Koyanagi-Harada. Se presenta el caso de una paciente que consulta por cefalea, visión borrosa y sintomatología audiológica, con diagnóstico de enfermedad de Vogt-Koyanagi-Harada. Revisamos la literatura disponible respecto a la enfermedad y sus manifestaciones otológicas.

ABSTRACT Autoimmune inner ear disease presents a bilateral, asymmetric and progressive sensorineural hearing loss, that responds to medical therapy, presenting alone or associated to a systemic disease such as Vogt-Koyanagi-Harada disease. We present the case of a patient with headache, blur vision and audiologic symptoms, diagnosed with Vogt-Koyanagi-Harada disease. We review the literature about the disease and its otologic manifestations.

Hipotireoidismo congênito como fator de risco para os transtornos do processamento auditivo central / Congenital hypothyroidism as a risk factor for central hearing process disorders

RESUMO Objetivo: Investigar a manifestação de sintomas do transtorno do processamento auditivo central em crianças com hipotireoidismo congênito. Métodos: Estudo de caráter exploratório, descritivo e transversal com 112 pacientes com hipotireoidismo congênito com idade ≥5 anos. Realizou-se entrevista com os pais/cuidadores no momento da espera da consulta médica. Portadores de outras afecções médicas foram excluídos. Como instrumento de pesquisa utilizou-se o protocolo estruturado de anamnese para avaliação do processamento auditivo rotineiramente empregado por audiologistas. A análise estatística utilizou o teste Qui-quadrado. Resultados: A distribuição por sexo foi semelhante (meninas: 53,3%). Os casos não-disgenesia constituíram a forma fenotípica mais prevalente para o hipotireoidismo congênito (88,4%), e verificou-se que 65,3% das crianças apresentavam algum episódio de níveis séricos irregulares de hormônio tireoestimulante. Dentre as manifestações mais frequentes dos sintomas do transtorno do processamento auditivo central, as queixas relaciondas às funções cognitivas auditivas, como: figura-fundo (83,0%), atenção auditiva (75,9%) e memória auditiva (33,0%) foram as mais evidentes. Reclamações relacionadas ao rendimento escolar foram reportadas em 62,3%. Conclusões: Os dados obtidos evidenciaram altas frequências de sintomas de defasagem nas funções cognitivas relacionadas ao processamento auditivo central, em especial na atenção auditiva, figura-fundo e memória auditiva nos portadores do hipotireoidismo congênito.

ABSTRACT Objective: To investigate the presence of central auditory processing disorder symptoms in children with congenital hypothyroidism. Methods: An exploratory, descriptive, cross-sectional study of 112 patients with congenital hypothyroidism aged ≥5 years old. An interview was held with the parents/caregivers at the time of the medical consultation. Patients with other medical conditions were excluded. As a research instrument, the structured protocol of anamnesis was used to evaluate the auditory processing routinely used by audiologists. For statistical analysis, the chi-square test was used. Results: Sex distribution was similar in both boys and girls (girls: 53.3%). The most prevalent phenotypic form of congenital hypothyroidism was no dysgenesis (88.4%), and 65.3% of the children had an episode of irregular serum thyroid-stimulating hormone (TSH) levels. Among the manifestations of the most frequent central auditory processing disorder symptoms, problems were reported with regard to cognitive functions, as they related to hearing, such as figure-background ability (83.0%), auditory attention (75.9%) and auditory memory (33.0%). Complaints related to school performance were reported in 62.3% of the cases. Conclusions: The data obtained show a high frequency of lag symptoms in cognitive functions related to central auditory processing, particularly with regard to auditory attention, figure-background ability and auditory memory in patients with congenital hypothyroidism.

Alterações auditivas na esclerose sistêmica / Hearing alterations in systemic sclerosis

RESUMO Objetivo Descrever as queixas e alterações auditivas em indivíduos com esclerose sistêmica (ES), bem como verificar a evolução do quadro audiológico. Método Trata-se de estudo seccional, com uma fase prospectiva, realizado no período de 2012 e 2015, com pacientes com diagnóstico médico de ES. Foram coletados dados sociodemográficos, ano de início da doença, ano de diagnóstico e subtipo da enfermidade. Posteriormente, foram realizadas a anamnese audiológica, para identificação de queixas e sintomas e para a investigação de realização de audiometria pregressa ao estudo, e, em seguida, a avaliação audiológica básica. Resultados Participaram do estudo 50 indivíduos. Tontura e zumbido foram os sintomas mais frequentes. A perda auditiva foi identificada em 23 (46%) indivíduos, sendo a maioria do tipo sensorioneural, de grau e configurações variáveis. A análise dos limares auditivos obtidos na avaliação audiológica realizada em 2012 e, posteriormente, em 2015 indicou desencadeamento ou progressão da perda auditiva, com piora de 10dB na maioria das frequências avaliadas, sendo mais expressiva nas frequências agudas. Conclusão Elevada frequência de queixas e alterações auditivas em indivíduos com ES e desencadeamento e/ou progressão da perda auditiva naqueles que realizaram avaliação audiológica sequencial.

ABSTRACT Purpose Describe hearing complaints and alterations in individuals with systemic sclerosis (SS) and to verify the development of audiological manifestations. Methods This is a cross-sectional study with a prospective phase, conducted in the period from 2012 to 2015, with patients with medical diagnosis of SS. Sociodemographic data, year of disease onset, year of diagnosis and disease subtype were collected. Later, audiological anamnesis was performed to identify complaints and symptoms and to investigate the performance of audiometry before the study and, after that, a basic audiological evaluation was conducted. Results Fifty individuals participated in the study. Dizziness and tinnitus were the most frequent symptoms. Hearing loss was identified in 23 (46%) individuals; most of them were of sensorineural type, of variable degrees and configurations. The analysis of hearing thresholds obtained in the audiological evaluation performed in 2012 and, later, in 2015, indicated onset or progression of hearing loss, with aggravation of 10dB in most frequencies evaluated, being more expressive in acute frequencies. Conclusion High rate of hearing complaints and alterations in individuals with SS and onset and/or progression of hearing loss in those who underwent serial audiological evaluation were observed.

Citomegalovirus congénito: ¿El diagnóstico y el tratamiento precoz pueden revertir una discapacidad permanente? / Congenital cytomegalovirus: Can early diagnosis and treatment revert permanent disability?

Introducción: La infección congénita por citomegalovirus (CMV) es la causa más frecuente de hipoacusia neurosensorial (HNS) no genética en países desarrollados. La incidencia de HNS en los lactantes sintomáticos oscila entre el 30 y el 65%. Objetivos: Describir las formas de presentación clínica de la infección por CMV congénita en pacientes sintomáticos y la evolución auditiva en los pacientes tratados con antivirales y aquellos sin tratamiento. Diseño: Estudio retrospectivo, descriptivo, observacional y longitudinal. Población: Se incluyeron niños, menores de 2 meses, con CMV congénito (confirmado por viruria positiva con método de PCR), sintomáticos, internados en la Unidad de Neonatología, desde el año 2005 al 2013. Método: Diagnóstico y seguimiento auditivo utilizando otoemisiones acústicas (OEA), potenciales evocados auditivos de tronco cerebral (PEAT) y audiometría (AT) según edad e indicación en cada caso en particular. El tratamiento antiviral se realizó con ganciclovir (GCV) y/o valganciclovir (VGCV). Resultados: Clínicamente se estudiaron 16 pacientes con diagnóstico de CMV congénito sintomáticos. Se excluyeron tres. Se describen los motivos de internación más frecuentes. Para el estudio y seguimiento audiológico los pacientes se dividieron en dos grupos de acuerdo a que recibieran o no tratamiento: A: no recibieron tratamiento antiviral (n: 5) y B: recibieron tratamiento antiviral (n: 8). En los pacientes que recibieron tratamiento, las secuelas auditivas fueron menores y en dos de los casos se produjo una mejoría importante en la audición. Conclusiones: El tratamiento de neonatos con infección congénita por CMV con GCV y/o VGCV ofrece resultados alentadores en la prevención de la hipoacusia (AU)

Introduction: Congenital cytomegalovirus (CMV) infection is the most common cause of non-genetic sensorineural hearing loss (SNH) in developed countries. The incidence of SNH in symptomatic infants ranges between 30% and 65%. Objectives: To describe different forms of clinical presentation of congenital CMV infection in symptomatic patients as well as outcome in patients treated with antiviral drugs and those in whom treatment was withheld. Study design: A retrospective, longitudinal, observational, descriptive study. Population: Symptomatic infants younger than 2 months of life with congenital CMV infection (confirmed by positive viruria using PCR), admitted to the Neonatology Unit between 2005 and 2013. Method: Diagnosis and audiological follow-up with otoacoustic emissions (OAE), brainstem auditory evoked potentials (BAEP) and audiometry (AT) according to age and indication for the individual patient. Antiviral treatment consisted of ganciclovir (GCV) and/or valganciclovir (VGCV). Results: Sixteen patients with symptomatic congenital CMV infection were clinically studied. Three were excluded. Main reasons for admission are described. For evaluation and audiological follow-up the patients were divided into two groups according to whether or not they received treatment. Group A: did not receive antiviral treatment (n: 5) and B: received antiviral treatment (n: 8). In patients that received treatment hearing sequelae were less severe and in two patients significant hearing improvement was observed. Conclusions: Treatment with GCV and/or VGCV of neonates with congenital CMV was found to have promising results for the prevention of hearing loss (AU)

Resultados del programa de implantes cocleares de Clínica Las Condes a 20 años de su inicio: serie clínica 1994-2015 / Cochlear implant program results in Clinica Las Condes 20 years since the beginning: clinical series 1994-2015

RESUMEN Introducción: La hipoacusia sensorioneural es la tercera causa de discapacidad en Chile, con incidencia en recién nacidos de 1 a 3 casos cada 1.000 recién nacidos vivos, y prevalencia en adultos de hasta 70%, siendo muchos casos susceptibles de ser tratados mediante implante coclear. Objetivo: Describir resultados de los pacientes sometidos a cirugía de implante coclear en Clínica Las Condes (CLC). Material y método: Estudio longitudinal de cohorte retrospectiva, se analizaron a 237 pacientes implantados en Clínica Las Condes desde 1994 al año 2015. Resultados: Se analizan 237 pacientes, 106 mujeres (44,7%) y 131 hombres (55,3%). El 65,5% de los pacientes se implantaron entre los 2-6 años de vida. Las etiologías más frecuentes fueron hipoacusia congénita no sindrómica (45,9%), genética tardía (11,8%) y posmeningitis (11,4%). Se encontró asociación significativa entre promedio tonal de la palabra (PTP) y presencia de malformación coclear (p =0,008). Sólo 9,9% de los pacientes presentaron otra discapacidad asociada a la hipoacusia. La tasa de reimplante fue 16,3% y sólo la variable malformación coclear se asoció a ella (p =0,016). Conclusión: El implante coclear es una herramienta beneficiosa en pacientes hipoacúsicos que no se benefician de otros dispositivos auditivos ofreciendo la posibilidad de reinsertarlos social y laboralmente con bajo riesgo quirúrgico y alta tasa de éxito.

ABSTRACT Introduction: Sensorineural hearing loss is the third leading cause of disability in Chile, with incidence in infants 1 to 3 cases per 1,000 newborn and prevalence in adult up to 70%, with many cases that can be treated with cochlear implant. Aim: to describe outcomes of our series of patients undergoing cochlear implant surgery at Clinica Las Condes (CLC). Material and method: Retrospective longitudinal cohort study, 237 patients implanted in our center since 1994 to 2015 were analyzed. Results: 237 patients, 106 women (44.7%) and 131 men (55.3%) received a cochlear implant during this period. 65.5% of patients were implanted between 2-6 years of age. The most common etiologies were; non-syndromic congenital deafness (45.9%), late genetic onset (11.8%) and meningitis (11.4%). A significant association between the average pure tone average and presence of cochlear malformation (p =0.008) was demonstrated. Only 9.9% of patients had other disabilities associated with hearing loss. Re-implantation rate was 16.3% and only the variable cochlear malformation was associated to it (p =0.016) Conclusions: The cochlear implant provides clear benefits to patients with hearing impairment who do not benefit from other hearing devices offering the possibility of social and labor reintegrating, with low surgical risk and high success rate.

Hearing Disorders in Congenital Toxoplasmosis: A Literature Review

Abstract Introduction Several studies show correlations between congenital toxoplasmosis and hearing loss, with a broad diversity of levels of hearing loss and specifications of hearing disorders. Objective To describe the studies found in the literature regarding hearing disorders in congenital toxoplasmosis. Data Synthesis A literature review was conducted on the Lilacs, SciELO, PubMed and Scopus databases by combining the following keywords: congenital toxoplasmosis and hearing. Based on this search strategy, 152 papers were found, the majority published on the Scopus and PubMed databases from1958 to 2015. After theapplication of the inclusion criteria, 8 articles published between 1980 and 2015 were included in the present study. Conclusion This review showed a moderate evidence of the association between hearing disorders and congenital toxoplasmosis, which is characterized by sensorineural hearing loss. However, there are gaps in the description of the specific characteristics of the type and level of hearing loss, or of other possible disorders involved in the auditory processing.

Significant association between osteoporosis and hearing loss: a systematic review and meta-analysis / Associação significativa entre osteoporose e perda auditiva: uma revisão sistemática e metanálise

Abstract Introduction: There is inconclusive evidence whether osteoporosis increases risk of hearing loss in current literature. Objective: We conducted this meta-analysis to determine whether there is an association between hearing loss and osteoporosis. Methods: This systematic review and meta-analysis was conducted from studies of MEDLINE, EMBASE, and LILACS. Osteoporosis was defined as having a bone mineral density with a T-score of less than −2.5 standard deviation. The outcome was hearing loss as assessed by audiometry or self-reported assessment. Random-effects model and pooled hazard ratio, risk ratio, or odds ratio of hearing loss with 95% confidence intervals were compared between normal bone mineral density and low bone mineral density or osteoporosis. Results: A total of 16 articles underwent full-length review. Overall, there was a statistically significant increased odds of hearing loss in the low bone mineral density or osteoporosis group with odds ratio of 1.20 (95% confidence intervals 1.01-1.42, p = 0.04, I 2 = 82%, Pheterogeneity = 0.01). However, the study from Helzner et al. reported significantly increase odds of hearing loss in the low bone mineral density in particular area and population included femoral neck of black men 1.37 (95% confidence intervals 1.07-1.76, p = 0.01) and total hip of black men 1.36 (95% confidence intervals 1.05-1.76, p = 0.02). Conclusion: Our study proposed the first meta-analysis that demonstrated a probable association between hearing loss and bone mineral density. Osteoporosis could be a risk factor in hearing loss and might play an important role in age-related hearing loss.

Abstract Introduction: There is inconclusive evidence whether osteoporosis increases risk of hearing loss in current literature. Objective: We conducted this meta-analysis to determine whether there is an association between hearing loss and osteoporosis. Methods: This systematic review and meta-analysis was conducted from studies of MEDLINE, EMBASE, and LILACS. Osteoporosis was defined as having a bone mineral density with a T-score of less than −2.5 standard deviation. The outcome was hearing loss as assessed by audiometry or self-reported assessment. Random-effects model and pooled hazard ratio, risk ratio, or odds ratio of hearing loss with 95% confidence intervals were compared between normal bone mineral density and low bone mineral density or osteoporosis. Results: A total of 16 articles underwent full-length review. Overall, there was a statistically significant increased odds of hearing loss in the low bone mineral density or osteoporosis group with odds ratio of 1.20 (95% confidence intervals 1.01-1.42, p = 0.04, I 2 = 82%, Pheterogeneity = 0.01). However, the study from Helzner et al. reported significantly increase odds of hearing loss in the low bone mineral density in particular area and population included femoral neck of black men 1.37 (95% confidence intervals 1.07-1.76, p = 0.01) and total hip of black men 1.36 (95% confidence intervals 1.05-1.76, p = 0.02). Conclusion: Our study proposed the first meta-analysis that demonstrated a probable association between hearing loss and bone mineral density. Osteoporosis could be a risk factor in hearing loss and might play an important role in age-related hearing loss.

Hearing handicap in patients with chronic kidney disease: a study of the different classifications of the degree of hearing loss / O Handicap auditivo em pacientes com doença renal crônica: um estudo das diferentes classificações do grau da perda auditiva

Abstract Introduction: The association between hearing loss and chronic kidney disease and hemodialysis has been well documented. However, the classification used for the degree of loss may underestimate the actual diagnosis due to specific characteristics related to the most affected auditory frequencies. Furthermore, correlations of hearing loss and hemodialysis time with hearing handicap remain unknown in this population. Objective: To compare the results of Lloyd's and Kaplan's and The Bureau Internacional d'Audiophonologie classifications in chronic kidney disease patients, and to correlate the averages calculated by their formulas with hemodialysis time and the hearing handicap. Methods: This is an analytical, observational and cross-sectional study with 80 patients on hemodialysis. Tympanometry, speech audiometry, pure tone audiometry and interview of patients with hearing loss through Hearing Handicap Inventory for Adults. Cases were classified according to the degree of loss. The correlations of tone averages with hemodialysis time and the total scores of Hearing Handicap Inventory for Adults and its domains were verified. Results: 86 ears (53.75%) had hearing loss in at least one of the tonal averages in 48 patients who responded to Hearing Handicap Inventory for Adults. The Bureau Internacional d'Audiophonologie classification identified a greater number of cases (n = 52) with some degree of disability compared to Lloyd and Kaplan (n = 16). In the group with hemodialysis time of at least 2 years, there was weak but statistically significant correlation of The Bureau Internacional d'Audiophonologie classification average with hemodialysis time (r = 0.363). There were moderate correlations of average The Bureau Internacional d'Audiophonologie classification (r = 0.510) and tritone 2 (r = 0.470) with the total scores of Hearing Handicap Inventory for Adults and with its social domain. Conclusion: The Bureau Internacional d'Audiophonologie classification seems to be more appropriate than Lloyd's and Kaplan's for use in this population; its average showed correlations with hearing loss in patients with hemodialysis time ≥ 2 years and it exhibited moderate levels of correlation with the total score of Hearing Handicap Inventory for Adults and its social domain (r = 0.557 and r = 0.512).

Resumo Introdução: A associação entre perda auditiva e doença renal crônica e hemodiálise tem sido bem documentada. Porém, a classificação usada para o grau da perda pode subestimar o real diagnóstico devido a características específicas em relação às frequências auditivas mais acometidas. Além disso, correlações da perda auditiva e do tempo de hemodiálise com o handicap auditivo permanecem desconhecidas nessa população. Objetivo: Comparar os resultados das classificações de Lloyd e Kaplan e do Bureau Internacional d'Audiophonologie em pacientes com doença renal crônica e correlacionar as médias calculadas por suas fórmulas com o tempo de hemodiálise e com o handicap auditivo. Método: Estudo analítico, observacional e transversal com 80 pacientes em hemodiálise. Todos os pacientes foram submetidos a timpanometria, logoaudiometria, audiometria tonal limiar e os pacientes com perda auditiva foram entrevistados através do Hearing Handicap Inventory for Adults. A classificação dos casos foi feita de acordo com o grau da perda. Foram verificadas as correlações das médias tonais com o tempo de hemodiálise e com as pontuações totais do Hearing Handicap Inventory for Adults e seus domínios. Resultados: Em 48 pacientes que responderam ao Hearing Handicap Inventory for Adults, 86 orelhas (53,75%) apresentaram perda auditiva em pelo menos uma das médias tonais. A classificação do Bureau Internacional d'Audiophonologie identificou maior número de casos (n = 52) que apresentavam algum grau de deficiência do que a classificação de Lloyd e Kaplan (n = 16). No grupo com tempo de hemodiálise a partir de dois anos, houve correlação fraca, mas estatisticamente significante, da média da classificação do Bureau Internacional d'Audiophonologie com o tempo de hemodiálise (r = 0,363). Houve correlações moderadas das médias da classificação do Bureau Internacional d'Audiophonologie (r = 0,510) e tritonal 2 (r = 0,470) com pontuações totais do Hearing Handicap Inventory for Adults e com seu domínio social. Conclusão: A classificação do Bureau Internacional d'Audiophonologie mostra-se mais adequada do que a de Lloyd e Kaplan nessa população, sua média apresentou correlações com perdas auditivas em pacientes com tempo de hemodiálise ≥ 2 anos e manteve níveis moderados de correlação com a pontuação total do Hearing Handicap Inventory for Adults e seu domínio social (r = 0,557 e r = 0,512).