Hiperacusia en trastornos del espectro autista: una revisión de la literatura / Hyperacusis in autism spectrum disorders: a review of the literature

Resumen La hiperacusia se define como la intolerancia a ciertos sonidos cotidianos que causa angustia y discapacidad significativas en las actividades sociales, ocupacionales, recreativas y otras actividades cotidianas. Los sonidos pueden percibirse como incómodamente fuertes, desagradables, atemorizantes o dolorosos. Se encuentra presente en aproximadamente un 3% población general, y aumenta significativamente en trastornos del espectro autista (TEA), alcanzando entre un 15% a 40%. Los mecanismos fisiopatológicos no son del todo claros, pero se ha propuesto, una alteración en el funcionamiento de mecanismos reflejos y de regulación, tanto a nivel de la vía auditiva periférica, como central, incluyendo estructuras no relacionadas directamente con la vía auditiva. El siguiente texto tiene como objetivo analizar la relación entre hiperacusia y TEA, enfatizando en la frecuencia en que se presentan como comorbilidades, en los posibles mecanismos fisiopatológicos, y en actualizaciones en el abordaje diagnóstico y terapéutico. Se realiza una revisión bibliográfica cualitativa en Pubmed con artículos entre los años 2008-2020 utilizando los términos: "hyperacusis autism", "sistema olivococlear", arrojando 39 artículos, de los cuales se seleccionaron en base a la temática de cada uno, evaluada por los autores. A pesar de una significativa relación entre hiperacusia y TEA, los mecanismos fisiopatológicos de ambas patologías siguen siendo un misterio. Existen estudios que sugieren pruebas de screening no invasivas que relacionan ambas patologías, pero debido a los sesgos de selección, todavía no son factibles de usar en forma universal. El abordaje terapéutico ha sido poco explorado, y no se dispone de fármacos que hayan demostrado su efectividad, por el contrario, algunos de ellos empeoran la sintomatología. Se recomienda al tratante, seguir un camino largo, en conjunto con el paciente, donde las terapias no farmacológicas como la terapia cognitivo conductual han mostrado tener buenos resultados.

Abstract Hyperacusis is defined as intolerance to certain sounds that causes significant distress and disability in social, occupational, recreational and other activities. Sounds can be perceived as uncomfortably loud, unpleasant, frightening, or painful. It is present in approximately 3% of the general population, and increases significantly in autism spectrum disorders (ASD), between 15% and 40%. The pathophysiological mechanisms are not entirely clear, but an alteration in the functioning of reflex and regulatory mechanisms has been proposed, both at the peripheral and central auditory pathways, including structures not directly related to the auditory pathway. The therapeutic approach has been little explored as there are no drugs that have demonstrated their effectiveness, on the contrary, some of them worsen the symptoms. The practitioner is recommended to follow a long path, in conjunction with the patient, where non-pharmacological therapies such as cognitive behavioral therapy have been shown to have good results. The following text shows a review of the literature with articles referring to the subject between the years 2008-2019.

Relación entre hiperacusia y desorden del procesamiento auditivo central: Una revisión de la literatura / Relationship between hyperacusis and auditory processing disorder: a literature review

Resumen La hiperacusia corresponde a la excesiva sensibilidad auditiva o intolerancia a ciertos sonidos cotidianos que para la mayoría de las personas parecerían habituales. Considerando los mecanismos fisiológicos involucrados en el origen de la hiperacusia, es lógico pensar que su presencia podría afectar algunas habilidades del procesamiento auditivo central, sin embargo, la evidencia en torno al tema es escasa y no existe actualmente una revisión de la literatura que agrupe las investigaciones sobre esta temática. Por ello, el presente estudio pretende identificar y analizar la evidencia científica disponible sobre la relación entre hiperacusia y desorden del procesamiento auditivo central. Se realizó una revisión de la literatura guiada por protocolo PRISMA en las bases de datos Proquest, Ebsco, Pubmed, ScienceDirect, Cochrane Library y Scielo de acuerdo con términos claves. Fueron incluidos artículos originales de investigación, revisiones sistemáticas y metaanálisis, publicados desde el año 2010, realizados en animales y humanos, escritos en idiomas inglés, español y portugués. Se encontraron 323 estudios relacionados con los términos claves utilizados, de los cuales 13 cumplieron con los criterios de inclusión y fueron analizados. Se puede concluir que la evidencia científica en torno al tema es escasa e incipiente. Estructuras de la vía auditiva central como núcleos cocleares, lemnisco lateral, colículos inferiores, cuerpo geniculado medial y corteza auditiva primaria estarían relacionadas con la hiperacusia, así como también habilidades de procesamiento auditivo de figura/fondo, ordenación temporal y transferencia interhemisférica se verían afectadas.

Abstract Hyperacusis has been defined as the excessive auditory sensitivity or intolerance to certain everyday sounds that seem common for most people. Considering the underlying physiological mechanisms of hyperacusis, it is reasonable to think that it could affect some abilities involved in the central auditory processing. However, there is lack of evidence about this topic, and there is no literature review that gathers all the existing research. Therefore, the current study intends to identify and analyze the available scientific evidence regarding the relationship between hyperacusis and central auditory processing disorder. The review of the literature followed the PRISMA protocol, using key words in Proquest, Ebsco, Pubmed, ScienceDirect, Cochrane Library and Scielo databases. Original research articles, systematic reviews and meta-analyses of studies made with human and animals that have been published since 2010 in English, Spanish and Portuguese were included. Among them, 323 studies were related to the key terms, out of which 13 met the inclusion criteria and were analyzed. It is possible to conclude that there is little and incipient scientific evidence on the topic. Structures of the central auditory pathway such as cochlear nuclei, lateral lemniscus, inferior colliculi, medial geniculate body and primary auditory cortex seem to be related to hyperacusis; auditory processing skills such as figure/ground discrimination, temporal ordering and interhemispheric transfer appear to be affected as well.

Hiper-responsividade auditiva no transtorno do espectro autista, terminologias e mecanismos fisiológicos envolvidos: revisão sistemática / Auditory hyper-responsiveness in autism spectrum disorder, terminologies and physiological mechanisms involved: systematic review

RESUMO Objetivo identificar as terminologias mais utilizadas para designar o comportamento desproporcional a determinados sons (CDS) no TEA e sua relação com as respectivas ferramentas para sua investigação, assim como sua ocorrência e desfechos. Estratégia de pesquisa Foram utilizadas as bases de dados: PubMed, PsycINFO, Web of Science, Scielo e Lilacs. As palavras-chave utilizadas foram "autism", "hyperacusis" e "auditoryperception", com as seguintes combinações: "autism AND hyperacusis" e "autism AND auditoryperception". Critérios de seleção Foram incluídos os trabalhos com diagnóstico de TEA, de qualquer faixa etária; resumo disponível; Artigos em inglês, espanhol e português brasileiro; série de casos, estudos de prevalência e incidência, coorte e ensaios clínicos. Análise dos dados Foram analisados estudos com sujeitos com diagnóstico de TEA de qualquer faixa etária; referência no título e/ou resumo da ocorrência do CDS, aceitando os termos hiper-responsividade, hipersensibilidade e hiperacusia; resumo disponível; artigos em inglês, espanhol e português brasileiro; série de casos, estudos de prevalência e incidência, coorte e ensaios clínicos. Resultados Dos 692 estudos resultantes da consulta, foram identificados 13 que atendiam aos requisitos estabelecidos. Conclusão O termo hipersensibilidade auditiva foi o mais empregado para designar o CDS, seguido da hiperacusia. Não houve relação entre os termos e a respectiva ferramenta de investigação, sendo os questionários os mais utilizados para designar o referido comportamento, cuja frequência relatada foi de 42,1% a 69,0%. Os testes auditivos, quando realizados, mostraram o envolvimento das vias neurais auditivas, aferente e eferente.

ABSTRACT Purpose this paper aims to identify the most used terminologies to designate the disproportional behavior to sounds in the autism spectrum disorder (ASD) and its relationship with the respective tools for its investigation, as well as its occurrence and outcomes. Research strategies the databases used were PubMed, PsycINFO, Web of Science, Scielo and Lilacs. The keywords used were "autism", "hyperacusis" and "auditory perception", with the following combinations: "autism AND hyperacusis" and "autism AND auditory perception". Selection criteria individuals diagnosed with ASD of any age group; available abstract; papers in English, Spanish and Brazilian Portuguese; case series, prevalence and incidence studies, cohort and clinical trials. Data analysis we analyzed studies with individuals diagnosed with ASD of any age group; reference in the title and/or summary of the occurrence of disproportional behavior to sounds, accepting the terms hyper-responsiveness, hypersensitivity and hyperacusis; summary available; papers in English, Spanish and Brazilian Portuguese; series of cases, prevalence and incidence studies, cohort and clinical trials. Results Of the 692 studies resulting from the consultation, 13 studies could achieve the established requirements. Conclusion The term auditory hypersensitivity was the most commonly used to designate disproportional behavior to sounds, followed by hyperacusis. There was no relationship between the terms and the respective research tool, and the questionnaires were the most used to designate the referred behavior, whose reported frequency was from 42.1% to 69.0%. The auditory behavior tests when performed showed the involvement of the auditory, afferent and efferent neural pathways.

Evaluation of selective attention in patients with misophonia / Avaliação da atenção seletiva em pacientes com misofonia

Abstract Introduction: Misophonia is characterized by the aversion to very selective sounds, which evoke a strong emotional reaction. It has been inferred that misophonia, as well as tinnitus, is associated with hyperconnectivity between auditory and limbic systems. Individuals with bothersome tinnitus may have selective attention impairment, but it has not been demonstrated in case of misophonia yet. Objective: To characterize a sample of misophonic subjects and compare it with two control groups, one with tinnitus individuals (without misophonia) and the other with asymptomatic individuals (without misophonia and without tinnitus), regarding the selective attention. Methods: We evaluated 40 normal-hearing participants: 10 with misophonia, 10 with tinnitus (without misophonia) and 20 without tinnitus and without misophonia. In order to evaluate the selective attention, the dichotic sentence identification test was applied in three situations: firstly, the Brazilian Portuguese test was applied. Then, the same test was applied, combined with two competitive sounds: chewing sound (representing a sound that commonly triggers misophonia), and white noise (representing a common type of tinnitus which causes discomfort to patients). Results: The dichotic sentence identification test with chewing sound, showed that the average of correct responses differed between misophonia and without tinnitus and without misophonia (p = 0.027) and between misophonia and tinnitus (without misophonia) (p = 0.002), in both cases lower in misophonia. Both, the dichotic sentence identification test alone, and with white noise, failed to show differences in the average of correct responses among the three groups (p ≥ 0.452). Conclusion: The misophonia participants presented a lower percentage of correct responses in the dichotic sentence identification test with chewing sound; suggesting that individuals with misophonia may have selective attention impairment when they are exposed to sounds that trigger this condition.

Resumo Introdução: A misofonia é caracterizada pela aversão a certos sons muito seletivos, que evocam uma forte reação emocional. Tem sido demonstrado que a misofonia, bem como o zumbido, está associada à hiperconectividade entre os sistemas auditivo e límbico. Indivíduos com zumbido incômodo podem ter comprometimento da atenção seletiva, mas isso ainda não foi demonstrado em caso de misofonia. Objetivo: Caracterizar uma amostra de indivíduos misofônicos e compará-la com dois grupos controle, um com indivíduos com zumbido (sem misofonia) e o outro com indivíduos assintomáticos (sem misofonia e sem zumbido) em relação à atenção seletiva. Método: Avaliamos 40 participantes com audição normal: 10 com misofonia, 10 com zumbido (sem misofonia) e 20 sem zumbido e sem misofonia. Para avaliar a atenção seletiva, o teste de identificação de sentenças dicóticas foi usado em três situações: no primeiro, aplicou-se o teste em português do Brasil. Então, o mesmo teste foi aplicado em duas outras situações, combinado com dois sons competitivos: som de mastigação (representa um som que geralmente desencadeia misofonia) e ruído branco (representa um tipo comum de zumbido que causa desconforto nos pacientes). Resultados: No teste de identificação de sentenças dicóticas com som de mastigação, observou-se que a média de respostas corretas diferiu entre os grupos misofonia e sem zumbido e sem misofonia (p = 0,027) e entre misofonia e zumbido (sem misofonia) (p = 0,002) e, em ambos os casos, foi menor em misofonia. Em relação ao teste feito isoladamente e com ruído branco, nenhuma diferença foi observada na média das respostas corretas nos três grupos (p ≥ 0,452). Conclusão: Os participantes do grupo misofonia apresentaram uma menor porcentagem de respostas corretas no teste de identificação de sentenças dicóticas com som de mastigação, sugeriu que indivíduos com misofonia podem ter comprometimento da atenção seletiva quando expostos a sons que desencadeiam a condição.

Constipation is associated with tension type headache in women / Constipação está associada à cefaleia do tipo tensional em mulheres

ABSTRACT Tension-type headaches (TTH) and irritable bowel syndrome (IBS) are comorbid diseases affecting, especially, women. One of the underlying mechanisms for both is autonomic dysfunction in the brain-gut axis. We aimed to evaluate the factors accompanying TTH and their relation to functional gastrointestinal disorders. Methods: Women diagnosed with TTH were questioned about headache pattern and severity, and accompanying factors, as well as being screened for IBS according to the Rome III criteria. The participants were divided into two groups: constipation-dominant IBS (IBS-C) group and "others", with a total of 115 individuals included in the study. Results: Of the 115 patients; 48 (41.8%) of the women had IBS-C criteria while 67 (58.2%) described mixed-type IBS or diarrhea-dominant IBS. There were no significant differences in terms of mean age (p = 0.290), body mass index (p = 0.212), visual analog scale (p = 0.965), duration of attacks (p = 0.692), and episodic/chronic type (p = 0.553). Osmophobia was seen in 43.5%; phonophobia in 68.7%, and photophobia in 47.0% of the patients, and only osmophobia was significantly associated in women in the IBS-C group (p = 0.001). Conclusion: In female patients with TTH, a higher level of constipation was detected. The relationship of these two diseases suggests that they may share common mechanisms. This is the first study showing the relationship of osmophobia with constipation.

RESUMO As cefaléias do tipo tensional (TTH) e a síndrome do intestino irritável (IBS) são comorbidades que afetam especialmente as mulheres. O mecanismo subjacente para ambas é a disfunção autonômica no eixo cérebro-intestino. Nosso objetivo foi avaliar os fatores que acompanham as TTH e sua relação com distúrbios gastrointestinais funcionais (FGID). Métodos: Mulheres com diagnóstico de TTH foram questionadas quanto ao padrão e gravidade da cefaleia, fatores acompanhantes e triadas para IBS de acordo com os critérios de Roma III e foram divididas em dois grupos: grupo com IBS com predominância de constipação (IBS-C) e as demais. Um total de 115 mulheres foram incluídas no estudo. Resultados: Do total de 115 pacientes; 48 (41,8%) das mulheres tiveram os critérios da IBS-C, enquanto 67 (58,2%) tiveram IBS do tipo misto ou IBS com predominância de diarreia. Não houve diferenças significativas em termos de idade média (p = 0,290), índice de massa corporal (IMC) (p = 0,212), escala visual analógica (VAS) (p = 0,965), duração dos ataques (p = 0,692), e tipo episódico / crônico (p = 0,553). Osmofobia foi observada em 43,5% das pacientes; fonofobia em 68,7% e fotofobia em 47,0%, e apenas osmofobia foi significativamente associada no grupo de mulheres com predominância de constipação (p = 0,001). Conclusão: Em pacientes do sexo feminino com TTH, encontra-se uma maior constipação. A relação dessas duas doenças sugere que elas podem compartilhar mecanismos comuns. Além disso, este é o primeiro estudo mostrando a relação da osmofobia com a constipação.

Differences in Characteristics and Comorbidity of Cluster Headache According to the Presence of Migraine

BACKGROUND AND PURPOSE: Cluster headache (CH) can present with migrainous symptoms such as nausea, photophobia, and phonophobia. In addition, an overlap between CH and migraine has been reported. This study aimed to determine the differences in the characteristics of CH according to the presence of comorbid migraine. METHODS: This study was performed using data from a prospective multicenter registry study of CH involving 16 headache clinics. CH and migraine were diagnosed by headache specialists at each hospital based on third edition of the International Classification of Headache Disorders (ICHD-3). We interviewed patients with comorbid migraine to obtain detailed information about migraine. The characteristics and psychological comorbidities of CH were compared between patients with and without comorbid migraine. RESULTS: Thirty (15.6%) of 192 patients with CH had comorbid migraine, comprising 18 with migraine without aura, 1 with migraine with aura, 3 with chronic migraine, and 8 with probable migraine. Compared to patients with CH without migraine, patients with CH with comorbid migraine had a shorter duration of CH after the first episode [5.4±7.4 vs. 9.0±8.2 years (mean±standard deviation), p=0.008], a lower frequency of episodic CH (50.0% vs. 73.5%, p=0.010), and a higher frequency of chronic CH (13.3% vs. 3.7%, p=0.033). Psychiatric comorbidities did not differ between patients with and without comorbid migraine. The headaches experienced by patients could be distinguished based on their trigeminal autonomic symptoms, pulsating character, severity, and pain location. CONCLUSIONS: Distinct characteristics of CH remained unchanged in patients with comorbid migraine with the exception of an increased frequency of chronic CH. The most appropriate management of CH requires clinicians to check the history of preceding migraine, particularly in cases of chronic CH.

Severe Temporal Hyper-Activated States Caused by Noise in Tinnitus and Hyperacusis with Normal Hearing

Lots of neuroimaging and animal studies have revealed that tinnitus and hyperacusis share the same patterns in the bottom up central auditory process. The aim was to identify the abnormal central patterns commonly observed in both tinnitus and hyperacusis in humans. We investigated two cases of normal hearing: a tinnitus patient and a hyperacusis patient. We compared the differences between the severe temporal hyper-activated state (STHS), with spikes, fast beta and gamma frequencies after noise exposure, and the mild temporal hyper-activated state (MTHS), in no sound exposed condition. The power of the gamma band in the two cases was increased in both auditory cortices compared to the other brain regions. Our results of human with normal hearing were the first to identify how tinnitus and hyperacusis caused by sound are abnormally active and how they maintain constant pathological states.

The effectiveness and tolerability of oral acetaminophen/aspirin/caffeine (AAC) combination regimen as an acute treatment for migraine in adults: A meta-analysis of randomized trials

BACKGROUND: Migraine is a highly common disorder that can cause significant disability on an individual, which collectively may lead to a substantial burden for the society. Various expert societies have recommended Acetaminophen/Aspirin/Caffeine (AAC) combination regimen as the first-line drug treatment for migraine attacks; however, there were no pooled evidences summarizing the effectiveness and tolerability of this regimen.OBJECTIVE: To determine the effectiveness and tolerability assessment of oral AAC combination regimen as an acute treatment for migraine in adults.METHODS: Relevant studies from inception to March 2014 were searched in Cochrane CENTRAL, MEDLINE, LILACS, Scopus and metaRegister of Controlled Trials. The Cochrane Collaboration's tool for the assessment of risk of bias was employed. Trials that were randomized, double-blind, parallel-group, placebo and active-controlled were included and the data were employed for meta-analysis. To evaluate the quality of evidence, the GRADE approach was utilized for outcomes with sufficient studies and data.RESULTS: From 225 records identified, 4 trials were included in this review, with a total of 3,608 participants with recorded baseline characteristics. Patient-reported migraine intensity was moderate-severe and the AAC dose used was at 500/500/130 mg. At 2 hours, AAC regimen was statistically different and found to be superior to placebo in terms of pain-free, headache relief, nausea-free, photophobia-free, phonophobia-free and functional disability reduction rates using intension-to-treat analysis. Missing data did not alter the outcome measures generating robust results. Sumatriptan 100 mg was found to be better than AAC in pain-free rate, and phonophobia-free rates at 2 hours. Statistically more patients in the AAC arm experienced "any adverse event" compared to placebo and complaints were commonly nausea and nervousness.CONCLUSION: For adult individuals with moderate-severe migraine, a fixed oral dose of Acetaminophen/Aspirin/Caffeine (AAC 500/500/130 mg) may be used as first-line therapy for the acute treatment of migraine and is only associated with mild, infrequent adverse events.

Degree of Sigmoid Sinus Compression and the Symptom Relief Using Magnetic Resonance Angiography in Venous Pulsating Tinnitus

OBJECTIVES: To show that mechanical compression of sigmoid sinus is effective for treatment of pulsatile tinnitus caused by sigmoid sinus enlargement, and to evaluate the relationship between the compression degree of sigmoid sinus and the tinnitus symptom relief using magnetic resonance angiography. METHODS: Medical records of twenty-four patients who were diagnosed with venous tinnitus caused by sigmoid sinus enlargement and underwent mechanical compression of sigmoid sinus were reviewed between April 2009 and May 2013. All these patients received computed tomography and magnetic resonance venography study before undergoing surgery and were followed for at least 4 months. RESULTS: Twenty-three patients felt relief from tinnitus three months after the surgery, and the cross-sectional area of the sigmoid sinus on the tinnitus side was compressed approximately by half (46%-69%) after the surgery. There were 4 patients whose tinnitus suddenly disappeared while lying on the operating table before operation, which may be a result of the patient's emotional tension or postural changes from standing. One of the four patients felt no relief from tinnitus after the surgery, with the cross-sectional area of the sigmoid sinus only compressed by 30%. And two patients of them had a recurrence of tinnitus about 6 months after the surgery. Seven patients had sigmoid sinus diverticula, and tinnitus would not disappear merely by eliminating the diverticulum until by compressing the sigmoid sinus to certain degree. There were 3 minor complications, including aural fullness, head fullness and hyperacusis. The preoperative low frequency conductive and sensorineural hearing loss of 7 subjects subsided. CONCLUSION: Mechanical compression of sigmoid sinus is an effective treatment for pulsatile tinnitus caused by sigmoid sinus enlargement, even if it might be accompanied by sigmoid sinus diverticulum. A compression degree of sigmoid sinus about 54% is adequate for the relief of tinnitus symptom. Cases in which patients' tinnitus suddenly disappeared before the surgery might be excluded to improve the efficacy of surgery.

Estudo da relacao entre o grau de incomodo de pacientes com zumbido e a presenca de hiperacusia / Study of the relationship between the degree of tinnitus annoyance and the presence of hyperacusis

Introdução: A hiperacusia pode ser definida como uma manifestação de ganho central aumentado das vias auditivas, compreendida como um estado pré-zumbido. Em alguns casos, o zumbido pode ser secundário a esse ganho aumentado. Objetivo: Avaliar a prevalência da hiperacusia em pacientes com zumbido e sua associação com o incômodo do zumbido. Materiais e métodos: Estudo retrospectivo envolvendo pacientes do ambulatório de otoneurologia com queixa principal de zumbido que foram submetidos a avaliação clínica, audiológica e a questionário de avaliação da hiperacusia e do zumbido. O grau de incômodo da hiperacusia e do zumbido foi classificado utilizando a Escala Visual Analógica. Resultados: Foram analisados prontuários de 309 pacientes, 169 (54,7%) do sexo feminino e 140 (45,3%) do sexo masculino, com idade média de 53 anos. O grau de incômodo do zumbido apresentou mediana de sete. A hiperacusia esteve presente em 57 (18,4%) pacientes, com mediana de grau de incômodo de cinco. O grau de incômodo pelo zumbido nos pacientes com hiperacusia foi semelhante ao dos pacientes sem hiperacusia. Conclusão: A hiperacusia esteve presente em 18,4% dos pacientes com zumbido. O grau de incômodo do zumbido não teve correlação com a presença da hiperacusia. .

Introduction: Hyperacusis can be defined as a manifestation of an increased of central auditory pathways gain and can be considered a pre-tinnitus state. In some cases tinnitus can be caused by such increased gain. Aim: To evaluate the prevalence of hyperacusis in patients with tinnitus and its relation to the annoyance of tinnitus. Materials and methods: Retrospective study with patients from the neurotology service complaining of tinnitus in the first consultation were submitted to clinical evaluation, a questionnaire and audiological evaluation of tinnitus and hyperacusis. The degree of annoyance of tinnitus and hyperacusis was measured using a visual analog scale. Results: We analyzed medical records of 309 patients, 169 (54.7%) females and 140 (45.3%) males. The mean age was 53 years. The median degree of tinnitus annoyance was 7. Hyperacusis was present in 57 (18.4%) patients, with a median degree of 5. The degree of annoyance due to tinnitus patients with hyperacusis was similar to that of patients without hyperacusis. Conclusion: Hyperacusis was present in 18.4% of patients with tinnitus. The degree of annoyance due to tinnitus had no correlation with the presence of hyperacusis. .

Headache Associated with Moyamoya Disease in a Child: Pain Pattern in MRA Progression

Headache associated with moyamoya disease (HAMD) has been reported in about 20-30% of pediatric cases with moyamoya disease. However, the characteristic patterns of HAMD during disease progression are not completely known, although much is known on the incidence of HAMD and on the treatment effects of bypass surgery. In a child who presented with HAMD and had no infarction or hemorrhage till bypass surgery, we described the characteristic patterns of headache progression. At first presentation, the patient complained of severe bilateral headache despite mild stenosis only in the left internal carotid artery on brain magnetic resonance angiography. During all stages of progression, headache was severe and pressing in nature rather than throbbing. Nausea and vomiting were present even in the early stage, but photophobia, phonophobia and neck stiffness developed in the late stage. Headache was aggravated by menstruation and was relieved by analgesics and topiramate in the early stage, although the medication failed to provide relief when the headache increased in frequency and duration.

Superior Semicircular Canal Dehiscence Syndrome Presenting with Sudden Deafness and Vertigo after Trauma / 대한평형의학회지

Superior semicircular canal dehiscence syndrome (SCDS) is characterized by cochleovestibular hyper-responsiveness symptoms including sound- and pressure-evoked vertigo and oscillopsia, autophony, hyperacusis and ear fullness. The typical audiometric feature of SCDS is known as conductive hearing loss at low frequency. A 43-year-old man presented with unilateral sudden deafness after several events of heading during soccer game. High-resolution temporal bone computed tomography revealed a dehiscence of superior canal encased by superior petrous sinus. We reviewed audio-vestibular findings in this patient and speculated potential pathogenic mechanisms of sudden deafness in SCDS with literature review.

Ocorrência e efeito de supressão das Emissões Otoacústicas em adultos normo-ouvintes com zumbido e hiperacusia / Occurrence and suppression effect of Otoacoustic Emissions in normal hearing adults with tinnitus and hyperacusis

A associação entre zumbido e hiperacusia é frequente na literatura. OBJETIVOS: Verificar a ocorrência e o efeito de supressão das emissões otoacústicas transientes (EOATs), a existência de associação entre graus de zumbido e de hiperacusia, entre efeito supressor das EOATs e lateralidade, graus de zumbido e de hiperacusia, em adultos normo-ouvintes com queixas de zumbido e hiperacusia. MATERIAIS E MÉTODOS: Foram incluídos, nesta forma de estudo transversal, 25 indivíduos normo-ouvintes com queixas de zumbido e hiperacusia. Utilizou-se o Tinnitus Handicap Inventory (THI) para classificação do grau do zumbido e o Loudness Discomfort Level (LDL) para o da hiperacusia. RESULTADOS: A ocorrência das EOATs variou de 33 por cento a 88 por cento. Houve 63,7 por cento de presença de efeito de supressão na orelha direita e 81,7 por cento na orelha esquerda. Não ocorreu correlação significativa entre os graus de zumbido e os graus de hiperacusia em ambas as orelhas e não houve associação significativa entre efeito de supressão das EOATs e lateralidade, grau de zumbido e de hiperacusia. CONCLUSÃO: A ocorrência de EOATs foi inferior à encontrada em adultos normo-ouvintes. Obteve-se maior percentual de presença do efeito de supressão das EOATs em ambas as orelhas. Não houve associação entre as variáveis analisadas.

The association between tinnitus and hyperacusis is common according to the literature. AIM: To verify the occurrence and the suppression effect of transient otoacoustic emissions (TEOAE), the existence of association between tinnitus degrees and hyperacusis degrees, and between the suppressive effect of TEOAE and laterality, tinnitus and hyperacusis degrees in normal hearing adults with complaints of tinnitus and hyperacusis. MATERIALS AND METHODS: 25 normal hearing subjects with complaints of hyperacusis and tinnitus were studied in this cross-sectional study. The Tinnitus Handicap Inventory (THI) was used for the classification of tinnitus degrees, and the Loudness Discomfort Level (LDL) for the hyperacusis classification. RESULTS: The occurrence of TEOAE ranged from 33 to 88 percent. We observed the presence of TEOAE suppression effect on 63.7 percent in the right ear and 81.7 percent in the left ear. There was no significant correlation between the degrees of tinnitus and hyperacusis in both ears. No statistically significant associations between the TEOAE suppression effect and laterality, tinnitus degrees and hyperacusis degrees were found. CONCLUSION: The occurrence of TEOAE was lower than that found in normal hearing adults. A higher percentage of the presence of TEOAE suppression effect has been found in both ears. No association between the variables was observed.

Decreased sound tolerance and tinnitus / 临床耳鼻咽喉头颈外科杂志

Decreased sound tolerance includes hyperacusis and/or misphobia (phonophobia). As a commonly subjective symptom, tinnitus is defined to be a sound perceived in the ear or head without corresponding sound or electrical stimulus in the surrounding environment. It is usually occurred with or without hearing loss or harmful psychic reaction, which includes dyssomnia, annoy, rage, anxiety, depress, hard to concentrate, etc. Frequently, tinnitus is accompanied by decreased sound tolerance, while this text is aimed to review the correlated concepts of decreased sound tolerance,and focus on the hyperacusis which is a more common symptom.

Reconhecimento de fala de indivíduos normo-ouvintes com zumbido e hiperacusia / Recognition of speech of normal-hearing individuals with tinnitus and hyperacusis

Introdução: O zumbido e a hiperacusia são sintomas audiológicos cada vez mais frequentes que podem ocorrer na ausência do comprometimento da audibilidade, mas não de menor impacto ou incômodo aos indivíduos acometidos. O Sistema Olivococlear Medial auxilia no reconhecimento de fala no ruído e pode estar relacionado com a presença do zumbido e hiperacusia. Objetivo: Avaliar o reconhecimento de fala de indivíduos normo-ouvintes sem e com queixas de zumbido e hiperacusia, e comparar os resultados entre os mesmos. Método: Estudo descritivo, prospectivo e transversal em que foram avaliados 19 indivíduos normo-ouvintes com queixas de zumbido e hiperacusia do Grupo Estudo (GE), e 23 indivíduos normo-ouvintes sem queixas audiológicas do Grupo Controle (GC). Os indivíduos de ambos os grupos foram submetidos ao teste Listas de Sentenças em Português, elaborado por COSTA (1998), para determinar o Limiar de Reconhecimento de Sentenças no Silêncio (LRSS) e a relação Sinal-Ruído (S/R). O GE também respondeu ao Tinnitus Handicap Inventory para análise do zumbido, e para caracterizar a hiperacusia, foram determinados os limiares de desconforto. Resultados: O GC e o GE apresentaram LRSS e relação S/R médios de 7,34 dB NA e -6,77 dB, e de 7,20 dB NA e -4,89 dB, respectivamente. Conclusão: Os indivíduos normo-ouvintes com ou sem queixas audiológicas de zumbido e hiperacusia apresentaram desempenho semelhante no reconhecimento de fala no silêncio, o que não foi observado quando avaliados na presença de ruído competitivo, uma vez que o GE apresentou desempenho inferior nessa situação de comunicação, inclusive com diferença estatisticamente significante.

Introduction: Tinnitus and hyperacusis are increasingly frequent audiological symptoms that may occur in the absence of the hearing involvement, but it does not offer a lower impact or bothering to the affected individuals. The Medial Olivocochlear System helps in the speech recognition in noise and may be connected to the presence of tinnitus and hyperacusis. Objective: To evaluate the speech recognition of normal-hearing individual with and without complaints of tinnitus and hyperacusis, and to compare their results. Method: Descriptive, prospective and cross-study in which 19 normal-hearing individuals were evaluated with complaint of tinnitus and hyperacusis of the Study Group (SG), and 23 normal-hearing individuals without audiological complaints of the Control Group (CG). The individuals of both groups were submitted to the test List of Sentences in Portuguese, prepared by Costa (1998) to determine the Sentences Recognition Threshold in Silence (LRSS) and the signal to noise ratio (S/N). The SG also answered the Tinnitus Handicap Inventory for tinnitus analysis, and to characterize hyperacusis the discomfort thresholds were set. Results: The CG and SG presented with average LRSS and S/N ratio of 7.34 dB NA and -6.77 dB, and of 7.20 dB NA and -4.89 dB, respectively. Conclusion: The normal-hearing individuals with or without audiological complaints of tinnitus and hyperacusis had a similar performance in the speech recognition in silence, which was not the case when evaluated in the presence of competitive noise, since the SG had a lower performance in this communication scenario, with a statistically significant difference.

Analysis of Associated Symptoms of Bell's Palsy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: We aimed to evaluate associated symptoms in patients with Bell's palsy and prognosis according to associated symptoms. SUBJECTS AND METHOD: Seventy-four patients with Bell's palsy were enrolled in this study. We evaluated the degree of facial palsy with Facial Nerve Grading System 2.0. The grade of facial palsy was determined as initial facial palsy at admission and as final facial palsy at visit after 3 months. Afterwards, we examined symptoms (such as otalgia, facial numbness, taste disturbance, eye problems, hyperacusis, and tinnitus) and co-morbid diseases (such as diabetes mellitus and hypertension) associated with facial palsy. After 3 months of the onset of facial palsy, we examined the changes in the associated symptoms. RESULTS: At admission, 71.6 percent of all patients had symptoms associated with facial palsy. Of the associated symptoms, facial numbness and taste disturbance were the most common, which were still found in 23% of the patients after 3 months of the onset of facial palsy. Patients with a high initial grade of facial palsy were accompanied by taste disturbance and hyperacusis more than by other symptoms. Also patients who had auricular pain during the recovery period of Bell's palsy showed poor prognosis. Compared to non-hypertension patients, for patients with hypertension, taste disturbance was more common during the early period whereas eye problems were more common during the recovery period. CONCLUSION: Patients with Bell's palsy had diverse symptoms associated with facial palsy. During the early period of Bell's palsy, we concluded that there was no correlation between the symptoms and the prognosis. However, patients with pain around the ear during the recovery period showed poor recovery of facial palsy.

Clinical Analysis of Bell's Palsy in One University Hospital

BACKGROUND: Bell's palsy (BP) is the most frequent disease of the seventh cranial nerve, and has a relatively good prognosis. However, the precise etiology of the disorder has not been well understood. During the last decade, many researchers have focused on the conditions that are associated with BP, including diabetes, hypertension, and viral infection. We therefore analyzed that the etiology and clinical course of acute BP in patients in one university hospital. METHODS: The study comprised 241 BP patients who were examined over a 60-month period. Their clinical history was taken, and neurologic examinations, laboratory tests, electrophysiologic studies, and brain imaging was performed. The clinical severity of the facial palsy was assessed using the House-Brackman (HB) facial nerve grading scale. Patients were followed up once a week during the first month, and for up to 2 months. RESULTS: Of the 241 patients, 103 (43.5%) were men and 138 (56.5%) were women, whose ages were 46.4+/-16.6 and 48.4+/-17.6 years, respectively (mean+/-SD). The initial examination revealed that 36.7% of the cohort had an HB grade below 4, while in 63.3% the HB grade was above 3. Combined symptoms were as follows: postauricular pain (77.5%), increased tear flow (23.4%), taste change (15.6%), and hyperacusis (15.1%). The initial facial nerve conduction study performed within 1 week of presentation revealed a low compound muscle action potential amplitude in 140 (57.8%) and an absent blink reflex in 225 (93%). On follow-up examination, 134 (59%) of the patients exhibited a partial improvement by 4 weeks, and 166 (79.8%) had recovered completely within 2 months. CONCLUSIONS: We describe the epidemiologic, clinical, electrophysiologic, and radiologic characteristics of BP patients at one university hospital.

Determining of migraine prognosis using latent growth mixture models / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>This paper presents a retrospective study to classify patients into subtypes of the treatment according to baseline and longitudinally observed values considering heterogenity in migraine prognosis. In the classical prospective clinical studies, participants are classified with respect to baseline status and followed within a certain time period. However, latent growth mixture model is the most suitable method, which considers the population heterogenity and is not affected drop-outs if they are missing at random. Hence, we planned this comprehensive study to identify prognostic factors in migraine.</p><p><b>METHODS</b>The study data have been based on a 10-year computer-based follow-up data of Mersin University Headache Outpatient Department. The developmental trajectories within subgroups were described for the severity, frequency, and duration of headache separately and the probabilities of each subgroup were estimated by using latent growth mixture models. SAS PROC TRAJ procedures, semiparametric and group-based mixture modeling approach, were applied to define the developmental trajectories.</p><p><b>RESULTS</b>While the three-group model for the severity (mild, moderate, severe) and frequency (low, medium, high) of headache appeared to be appropriate, the four-group model for the duration (low, medium, high, extremely high) was more suitable. The severity of headache increased in the patients with nausea, vomiting, photophobia and phonophobia. The frequency of headache was especially related with increasing age and unilateral pain. Nausea and photophobia were also related with headache duration.</p><p><b>CONCLUSIONS</b>Nausea, vomiting and photophobia were the most significant factors to identify developmental trajectories. The remission time was not the same for the severity, frequency, and duration of headache.</p>

15th Yahya Cohen Memorial Lecture - the relationship between the air-bone gap and the size of superior semicircular canal dehiscence

<p><b>INTRODUCTION</b>This study aimed to examine the relationship between the air-bone gap (ABG) and the size of the superior semicircular canal dehiscence (SSCD) as measured on a computed tomography (CT) scan.</p><p><b>MATERIALS AND METHODS</b>The study design was a case series with chart review. Twenty-three patients (28 ears) from a tertiary referral centre were diagnosed with SSCD. The size of the dehiscence on CT scans and the ABG on pure-tone audiometry were recorded.</p><p><b>RESULTS</b>The size of the dehiscence ranged from 1.0 to 6.0 mm (mean, 3.5 ± 1.6 mm). Six ears with a dehiscence measuring less than 3.0 mm did not have an ABG (0 dB). The remaining 18 ears showed an average ABG at 500, 1000, and 2000 Hz (AvABG(500-2000)) ranging from 3.3 to 27.0 dB (mean, 11.6 ± 5.7 dB). The analysis of the relationship between the dehiscence size and AvABG(500-2000) revealed a correlation of R(2) = 0.828 (P <0.001, quadratic fit) and R(2) = 0.780 (P <0.001, linear fi t). Therefore, the larger the dehiscence, the larger the ABG at lower frequencies on pure-tone audiometry.</p><p><b>CONCLUSION</b>In SSCD patients, an ABG is consistently shown at the low frequency when the dehiscence is larger than 3 mm. The size of the average ABG correlates with the size of the dehiscence. These findings highlight the effect of the dehiscence size on conductive hearing loss in SSCD and contribute to a better understanding of the symptomatology of patients with SSCD.</p>