Clinical and Audiological Features of Ménière's
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Clinical and audiological features of Ménière’s disease: Insight into the diagnostic process. by Alida Naudé In fulfillment of the requirements for the degree M Communication Pathology, Department Communication Pathology, Faculty of Humanities University of Pretoria Supervisor: Ms. C Avenant Co-supervisor: Dr. M Soer February 2006 Title: Clinical and audiological features of Ménière’s disease: Insight into the diagnostic process. Name: Alida Naude Study leaders: Ms. C Avenant & Dr. M Soer Department: Communication Pathology Degree: M Communication Pathology ABSTRACT Ménière’s disease is the third most common inner ear disorder. The individual course of Ménière’s disease in different patients makes it difficult to diagnose on the basis of symptomatology alone. The impact of Ménière’s disease on quality of life has highlighted the importance of an additional tool to support the diagnosis of Ménière’s disease. Apart from the patient’s history, audiological data provide the most relevant information for confirming the diagnosis. The aim of this study was to analyse and describe the clinical and audiological features of a cohort of subjects diagnosed with Ménière’s disease, in order to develop understanding of the pathophysiology of the disease and to facilitate the diagnostic process. The research is based on a retrospective study of the medical records of 135 subjects with Ménière’s disease which were selected according to a non-probability sample. Descriptive statistics were used to organize, analyse and interpret the data. Sixty one percent of subjects presented with definite Ménière’s disease, 14 % with probable Ménière’s disease and 25 % with possible Ménière’s disease. The results showed a higher incidence of Ménière’s disease in females especially in the vestibular type. Three percent of subjects indicated a family history of Ménière’s disease. Bilateral Ménière’s disease presented in 39 % of subjects. The results confirmed that vertigo was the most debilitating symptom in Ménière’s disease. Correlating the clinical features of subjects with audiometric and vestibular tests highlighted the clinical value of an audiological test battery including the following tests: Pure tone audiometry, Speech discrimination, Oto-acoustic emissions, Electronystagmography and Electrocochleography. This confirms the role of the audiologist in the diagnostic and rehabilitation process in patients with Ménière’s disease. Terminology: Clinician, diagnostic process, differential diagnosis, endolymphatic hydrops, etiology, idiopathic, Ménière’s disease, pathogenesis, recurrent vestibulopathy. Titel: Clinical and audiological features of Ménière’s disease: Insight into the diagnostic process. Naam: Alida Naude Studieleiers: Me. C Avenant & Dr. M Soer Departement: Kommunikasiepatologie Graad: M Kommunikasiepatologie OPSOMMING Ménière se siekte is die derde algemeenste binne-oor afwyking. Die individuele verloop van Ménière se siekte in verskillende pasiënte maak dit moelik om ‘n diagnose alleenlik op grond van die simptomatologie te maak. Die impak van Ménière se siekte op kwaliteit van lewe beklemtoon die belangrikheid van ‘n addisionele hulpmiddel om die diagnose van Ménière se siekte te bevestig. Naas die gevalsgeskiedenis bied oudiologiese data die mees toepaslike inligting vir die diagnose van Ménière’s se siekte. Die doel van die studie was om ‘n reeks proefpersone met Ménière’s se siekte se simptome te evalueer in verhouding tot oudiologiese en vestibulêre toeste, om kennis uit te brei ten opsigte van die patofisiologie van die afwyking, asook om die diagnostiese proses te vergemaklik. Die navorsing is gebaseer op ‘n retrospektiewe studie van die mediese verslae van 135 proefpersone met Ménière se siekte, wat volgens ‘n nie-waarskynlikheids steekproef geselekteer is. Beskrywende statistiek is gebruik om die data te organiseer, te analiseer en te interpreteer. Een en sestig persent van die proefpersone is gediagnoseer met definitiewe Ménière se siekte, 14 % met waarskynlike Ménière se siekte en 25 % met moontlike Ménière se siekte. Volgens die resultate is daar ‘n hoër voorkoms van Ménière se siekte onder vrouens, veral ten opsigte van vestibulêre Ménière’s se siekte. ‘n Familie geskiedenis van Ménière se siekte is in 3 % van die proefpersone vasgestel. Bilaterale Ménière se siekte was verteenwoordig deur 39 % van proefpersone. Die resultate het bevestig dat vertigo die hoofsimptoom is wat lei tot ongeskiktheid. Die verhouding tussen die kliniese kenmerke van pasiënte en oudiometriese, asook vestibulêre toetsresultate beklemtoon die kliniese waarde van ‘n oudiologiese toetsbattery insluitende die volgende toetse: Suiwertoonoudiometrie, Spraakdiskriminasie, Oto-akoestiese emissies, Elektronistagmografie en Elektrokogleografie. Dit bevestig die rol van die oudioloog in die diagnostiese- en rehabilitiasie proses in pasiënte met Ménière se siekte. Sleutelwoorde: Klinikus, diagnostiese proses, differensiaaldiagnose, endolimfatiese hidrops, etiologie, idiopaties, Ménière’s se siekte, patogenese, herhalende vestibulopatie. "When you are inspired by some great purpose, some extraordinary project all your thoughts break their bonds your mind transcend limitations your consciousness expands in every direction and you find yourself in a new, great and wonderful world." - Patanjali ACKNOWLEDGEMENTS I would like to express my sincere gratitude and appreciation to the following people: My Heavenly Father, for His love and grace. Ms Carina Avenant, for her interest, encouragement, support and guidance throughout the past two years. Dr. M Soer for her guidance and valuable contribution derived from years of experience. Mr. Emmanuel Sibanda for the statistical analysis of the data. Mrs. L Buchner for the language and technical editing. Dr. Brian Wolfowitz for giving me the opportunity to work with him and develop a passion for otoneurology. My husband, Michael, for his patience, prayers and support. TABLE OF CONTENTS CHAPTER 1: INTRODUCTION AND RATIONALE 1 1.1 INTRODUCTION 1 1.2 PROBLEM STATEMENT AND RATIONALE 5 1.3 TERMINOLOGY 6 1.4 ABBREVIATIONS 7 1.5 ORGANISATION OF THE STUDY 9 1.6 SUMMARY 10 CHAPTER 2: PERSPECTIVES ON THE NATURE OF MÉNIÈRE’S DISEASE 12 2.1 INTRODUCTION 12 2.2 INCIDENCE OF MÉNIÈRE’S DISEASE 12 2.3 ETIOLOGY OF MÉNIÈRE’S DISEASE 14 2.4 CHARACTERISTICS AND SYMPTOMS OF MÉNIÈRE’S DISEASE 15 2.4.1 Non-auditory characteristics and symptoms of Ménière’s disease 15 2.4.2 Auditory characteristics and symptoms of Ménière’s disease 16 2.4.2.1 Sensorineural hearing loss 17 2.4.2.2 Tinnitus 18 2.4.3 Course and development of Ménière’s disease 19 2.4.4 Emotional impact of Ménière’s disease 19 2.5 DIAGNOSIS OF MÉNIÈRE’S DISEASE 20 2.5.1 Audiometric tests 22 2.5.1.1 Pure tone audiometry results 22 2.5.1.2 Speech discrimination results 23 2.5.1.3 Oto-acoustic emission testing 23 2.5.1.4 Electronystagmography 24 2.5.1.5 Electrocochleography 26 2.5.2 Clinical application of audiometric tests 27 2.6 MANAGEMENT OF MÉNIÈRE’S DISEASE 27 2.6.1 Surgical treatment 28 2.6.2 Medications 28 2.6.3 Dietary changes 29 2.6.4 Behavioral therapy 29 2.6.5 Hearing rehabilitation 30 2.6.6 Treatment of tinnitus 31 2.6.7 Conclusion 32 2.7 SUMMARY 32 CHAPTER 3: METHODOLOGY 34 3.1 INTRODUCTION 34 3.2 AIMS 34 3.2.1 Sub-aims 34 3.3 RESEARCH DESIGN 36 3.4 ETHICAL CLEARANCE 37 3.5 SAMPLE 38 3.5.1 Population 38 3.5.2 Selection criteria 39 3.5.3 Sample size 40 3.5.4 Description of sample 40 3.6 MATERIAL AND APPARATUS 41 3.6.1 Material and apparatus during subject selection 41 3.6.2 Material and apparatus during data collection 41 3.6.2.1 Audiometry results 42 3.6.2.2 Oto-acoustic Emission Analyzer 42 3.6.2.3 Electronystagmography system 42 3.6.2.4 Electrocochleography system 43 3.6.3 Material and apparatus during data analysis and processing 43 3.7 PROCEDURES 44 3.7.1 Procedures during subject selection 44 3.7.2 Data collection procedures 44 3.7.2.1 Pure tone audiometry test procedure 45 3.7.2.2 Speech discrimination test protocol 47 3.7.2.3 Distortion product oto-acoustic emission test protocol 49 3.7.2.4 Electronystagmography test protocol 51 3.7.2.5 Electrocochleography protocol 54 3.7.3 Data recording procedures 57 3.7.3.1 Type of Ménière’s disease (codebook column one) 57 3.7.3.2 Pure tone average (codebook column five) 59 3.7.3.3 Audiogram configuration (codebook column six) 59 3.7.4 Data analysis procedures 60 3.8 SUMMARY 61 CHAPTER 4: RESULTS AND DISCUSSION 63 4.1 INTRODUCTION 63 4.2 PATIENT ANALYSIS 64 4.2.1 Clinical profile of a group of subjects diagnosed with Ménière’s disease 64 4.2.1.1 Type of Ménière’s disease 65 4.2.1.2 Sexual preponderance 66 4.2.1.3 Age of onset 67 4.2.1.4 Family history 67 4.2.1.5 Summary of the clinical profile of subjects with Ménière’s disease with reference to type of Ménière’s disease, gender, age of onset and family history 68 4.2.1.6 Laterality 68 4.2.1.7 Summary of laterality in Ménière’s disease 72 4.2.2 Investigation into the incidence of symptoms experienced in each type of Ménière’s disease 73 4.3 THE SIGNIFICANCE OF AUDIOLOGICAL AND VESTIBULAR TEST RESULTS IN THE DIAGNOSIS OF MÉNIÈRE’S DISEASE 77 4.3.1 The significance of pure tone audiometry and speech discrimination scores in the diagnosis of Ménière’s disease 77 4.3.1.1 Pure tone audiometry results in relation to patient history 78 4.3.1.1.1 The relationship between pure tone audiometry results and the type of Ménière’s disease 78 4.3.1.1.2 The influence of age on pure tone audiometry results 80 4.3.1.2 Relationship between pure tone audiometry results and the symptoms