Otoneurological findings prevalent in hereditary ataxias / Alterações otoneurológicas prevalentes nas ataxias hereditárias

ABSTRACT Objective To describe and compare the vestibular findings most evident among the hereditary ataxias, as well as correlate their clinical features with the nervous structures affected in this disease. Methods Seventy-five patients were evaluated and underwent a case history, otorhinolaryngological and vestibular assessments. Results Clinically, the patients commonly had symptoms of gait disturbances (67.1%), dizziness (47.3%), dysarthria (46%) and dysphagia (36.8%). In vestibular testing, alterations were predominantly evident in caloric testing (79%), testing for saccadic dysmetria (51%) and rotational chair testing (47%). The presence of alterations occurred in 87% of these patients. A majority of the alterations were from central vestibular dysfunction (69.3%). Conclusion This underscores the importance of the contribution of topodiagnostic labyrinthine evaluations for neurodegenerative diseases as, in most cases, the initial symptoms are otoneurological; and these evaluations should also be included in the selection of procedures to be performed in clinical and therapeutic monitoring.

RESUMO Objetivo Descrever e comparar os achados vestibulares mais evidentes entre a ataxia hereditária, bem como correlacionar seus aspectos clínicos com o estudo das estruturas nervosas afetadas nesta doença. Métodos 75 pacientes foram avaliados e submetidos aos seguintes procedimentos: anamnese, avaliação otorrinolaringológica e vestibular. Resultados Clinicamente, os pacientes apresentaram sintomas de distúrbios da marcha (67,1%), tonturas (47,3%), disartria (46%) e disfagia (36,8%). No teste vestibular, as alterações foram predominantemente evidentes no teste calórico (79%), dismetria sacádicas (51%) e no teste rotatório (47%). A presença de alterações ocorreu em 87% dos pacientes. A maioria das alterações observadas foram da disfunção vestibular central (69,3%). Conclusão O estudo ressalta a importância da contribuição da avaliação labiríntica no topodiagnóstico para doenças neurodegenerativas, uma vez que, na maioria dos casos, os sintomas iniciais são otoneurológicos, e essas avaliações também devem ser incluídas na seleção de procedimentos a serem realizados no monitoramento clínico e terapêutico.

Otoneurological Abnormalities in Patients with Friedreich's Ataxia

Abstract Introduction Friedreich's ataxia is a neurodegenerative disease and progressive by nature. It has autosomal recessive inheritance and early onset inmost cases. Nystagmus and hearing loss (in some cases) make up some of the common symptoms seen in this disorder. Objective The objective of this study is to examine vestibular disorders in patientswith Friedreich ataxia. Methods We conducted a retrospective cross-sectional study. We evaluated 30 patients with ages ranging from six to 72 years (mean age of 38.6 ( 14.7). The patients underwent the following procedures: anamnesis, ENT, and vestibular evaluations. Results Clinically, the patients commonly had symptoms of incoordination of movement (66.7%), gait disturbances (56.7%), and dizziness (50%). In vestibular testing, alterations were predominantly evident under caloric testing (73.4%), gaze nystagmus testing (50.1%), rotational chair testing (36.7%), and optokinetic nystagmus testing (33.4%). The presence of alterations occurred under examination in 90% of subjects, with the majority occurring in those with central vestibular dysfunction (70% of the examinations). Conclusion The most evident neurotological symptoms were incoordination of movement, gait disturbances, and dizziness. Alterations in vestibular examinations occurred in 90% of patients, mostly in the caloric test, with a predominance of deficient central vestibular system dysfunction.

Neurotological Findings at a Health Unit for Adults with Cervicalgia

Abstract Introduction The cervical spine is a flexible link between the sensory platform of the skull and torso. The fundamental principle of its operation is due to the balance between muscle strength and flexibility, and any dysfunction of this balance causes neck pain, known as cervicalgia. Objective The objective of this study is to analyze the most prevalent neurotological findings in adults with neck pain. Method A cross-sectional study in which 33 adults from 50 to 83 years of age with neck pain were evaluated and underwent the following procedures: anamnesis, as well as ENT, audiological, and vestibular exams. Results The most evident neurotological symptoms were dizziness (75.7%), tinnitus, neck cracking, tingling in the extremities, and auditory problems (36.3% for each). The most frequently reported clinical symptoms were related to cardiovascular (69.7%), endocrine-metabolic (48.5%), and rheumatic (30.3%) systems. In the audiological assessment, 30 subjects (91.0%) presented hearing impairment in at least one ear, with sensorineural impairment being the most prevalent (88.0%). In the vestibular assessment, there were alterations in 13 subjects (39.0%) found in the caloric test. There was a prevalence of alterations in the peripheral vestibular system with a predominance of irritative peripheral vestibular dysfunction. Conclusion Neurotological complaints were frequent in this population, verifying the importance of these tests in the dysfunctions of the cervical region or the craniocervical junction.

Ataxia espinocerebelar tipo 6: relato de caso / Spinocerebellar ataxia type 6: a case report

O objetivo deste estudo foi verificar as alterações vestibulococleares observadas em um caso de ataxia espinocerebelar tipo 6. O caso foi encaminhado do Hospital de Clínicas para o Laboratório de Otoneurologia de uma Instituição de Ensino e foi submetido aos seguintes procedimentos: anamnese, inspeção otológica, avaliações audiológica e vestibular. O caso retrata uma paciente com diagnóstico genético de ataxia espinocerebelar tipo 6, do sexo feminino, com 57 anos de idade, que referiu desequilíbrio à marcha com tendência a queda para a esquerda, disartria e disfonia. Na avaliação audiológica apresentou configuração audiométrica descendente a partir da frequência de 4kHz e curva timpanométrica do tipo "A" com presença dos reflexos estapedianos bilateralmente. No exame vestibular observou-se na pesquisa da vertigem posicional presença de nistagmo vertical inferior e oblíquo, espontâneo e semiespontâneo múltiplo com características centrais (ausência de latência, paroxismo, fatigabilidade e vertigem), nistagmooptocinético abolido e hiporreflexia à prova calórica. Constataram-se alterações labirínticas que indicaram afecção do sistema vestibular central evidenciando-se a importância dessa avaliação. A existência da possível relação entre os achados com os sintomas vestibulares apresentados pela paciente apontou a relevância do exame labiríntico neste tipo de ataxia uma vez que a presença do nistagmo vertical inferior demonstrou ser frequente neste tipo de patologia.

The aim of this study was to investigate the vestibulocochlear alterations observed in a case of spinocerebellar ataxia type 6. The case was referred from the Hospital das Clinicas to the Otoneurology Laboratory of an educational institution and was subjected to the following procedures: anamnesis, otologic examination, as well as audiological and vestibular assessments. The case shows a 57-year-old female patient with a genetic diagnosis of spinocerebellar ataxia type 6 who presented unsteadiness of gait with tendency to fall to the left, dysarthria, and dysphonia. The audiological assessment presented sloping audiometric configuration from 4.0 kHz and tympanogram type “A” with the presence of acoustic reflexes bilaterally. Observed during the survey of positional vertigo in the vestibular assessment were the presence of oblique and vertical downbeat nystagmus, spontaneous and semispontaneous with multiple core features (absence of latency, paroxysm, fatigue and vertigo), abolished optokineticnystagmus and hyporeflexia in the caloric test. We found labyrinthic alterations that indicate central vestibular system disorders and lend credence to the importance of this evaluation. The existence of a possible relationship between the findings and vestibular symptoms displayed by the patient indicated the relevance of the labyrinthine evaluation for this type of ataxia once the presence of vertical downbeat nystagmus proved to be frequent in this type of pathology.

Vestibular Findings in Military Band Musicians

Introduction: Exposure to music is the subject of many studies because it is related to an individual's professional and social activities. Objectives: Evaluate the vestibular behavior in military band musicians. Methods: A retrospective cross-sectional study was performed. Nineteen musicians with ages ranging from 21 to 46 years were evaluated (average = 33.7 years and standard deviation = 7.2 years). They underwent anamnesis and vestibular and otolaryngologic evaluation through vectoelectronystagmography. Results: The most evident otoneurologic symptoms in the anamnesis were tinnitus (84.2%), hearing difficulties (47.3%), dizziness (36.8%), headache (26.3%), intolerance to intense sounds (21.0%), and earache (15.7%). Seven musicians (37.0%) showed vestibular abnormality, which occurred in the caloric test. The abnormality was more prevalent in the peripheral vestibular system, and there was a predominance of irritative peripheral vestibular disorders. Conclusion: The alteration in vestibular exam occurred in the caloric test (37.0%). There were changes in the prevalence of peripheral vestibular system with a predominance of irritative vestibular dysfunction. Dizziness was the most significant symptom for the vestibular test in correlation with neurotologic symptoms. The present study made it possible to verify the importance of the labyrinthine test, which demonstrates that this population should be better studied because the systematic exposure to high sound pressure levels may cause major vestibular alterations...