A fascinating journey through Audiovestibular Medicine

1. A fascinating journey through Audiovestibular Medicine Development of AVM and Audiology in UK Ewa Raglan St. George’s / Great Ormond Street Hospitals, London XVIthSymposium in Audiological Medicine, IAPA Beijing, CHINA October 2012

3. AVM - bird’s eye view • Audiovestibular Medicine • Audiovestibular Physician- role, training,manpower • Audiologists - role, training, manpower • Scientific discoveries in Audiology • Development in technology • Development of knowledge of medical conditions • Development of service provision • Development of care pathways • Now and future: translational research

4. Audiovestibular Medicine A medical speciality concerned with investigating, diagnosing and managing disorders of hearing and balance in both ,children and adults Training : AVM + + MSc in AVM Developmental Paediatrics Geriatrics Medical Genetics Neurology Ophthalmology Otolaryngology Psychiatry

5. Dizziness Hearing Loss Tinnitus WHY MEDICAL FOUNDATION TO THE SPECIALITY? Endocrinology Ophthalmology Renal medicine Haematology Neurology Cardiovascular medicine Psychiatry Genetics Psychology Rheumatology

6. Prevalence in the UK • Hearing loss:most common sensory disability in developed world • 9 millions hard of hearing adults in UK,only 2mln have a hearing device(Davis 2007) • 3 in 1.000 children (aged 0-16) suffer permanent hearing loss,those with severe impairment 4 times more likely to be unemployed (AoHL 2011) • 80% of children under 4 years have an episode of middle ear dysfunction • Tinnitus: 5 millions sufferers • Dizziness/Balance: most common reason for visits to a doctor by patients over 65 • affects 30-40% population by 60 years of age. • Social, economic, occupational costs of hearing and balance disorders on the individual, society, health services are profound • The collective goal of all employed in audiology( from basic sciences, applied research, service development, service delivery) is to improve the lives of adults and children with hearing, communication and vestibular problems.(Munro 2012)

7. WHAT IS THE SIZE OF THE PROBLEM? ESTIMATE OF WORLD BURDEN OF DISABLING HEARING IMPAIRMENT = 250 million persons From World Health Assembly Resolution WHA48.9,1995 on Prevention of hearing impairment

8. Presbyacusis

9. PROGRESS in Audiovestibular Medicine 1990’s 2004/5 2007/8 2012 Surgery vs. medicine Scientific discoveries Development of technology 1970’s Development of new diagnoses ENT surgery vs. AVM Development of services MDT 46 (+20) AVP 3 MMC (medical) training changes 3000 Technician in Audiology Audiologist MSC training changes Consultant Clinical Scientist

10. Progress in AVM 1970’s - 2012… • Audiovestibular Physician- role,training, • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

11. Role of Audiovestibular Physician • Diagnosis,aetiology of hearing loss, tinnitus, imbalance in Isolated ear disease or multisystem disease • Provide specific medical management, holistic medical care • Ensure AVM input in service provision and rehabilitation via MDT • Initially: +hands-on testing, hearing aid provision • Now: diagnosis, management, procedures

12. Training in the UK - AVP • In the 1970’s • ENT (FRCS) or General Medicine (MRCP) + 5years AVM • Currently • Foundation training for all(FY1/FY2) • Core Medical/ or Paediatric Training • 2 years in CMT or ST1/ST2 • 3 years in core paediatric training (ST1, ST2, ST3) • Specialty Training (ST3 – ST7) • 5 years in AVM + Diploma/ Degree in AVM • Assessments - CCT

13. Progress in AVM 1970’s - 2012… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

14. Audiologists • 1970 - Technicians (shortage, inconsistent quality, O levels + courses + practical training) Career progression on duration of years worked • 2000 – Audiologists BSc in Audiology - 10 schools (300/yr) MSc in Audiological sciences • 2010/12 - Modernisation of scientific careers Change of training (BSc, MSc, HSST, PhD - career progression path towards Consultant Audiological Scientist on merit (knowledge & skills)

15. Modernising Scientific Careers:Career and Training Pathways

16. Audiovestibular Multidisciplinary Team( MDT) Audiovestibular Physician Audiologist Scientist ENT Specialist Other members of MDT (specialists doctors and therapists) ENT Specialist

17. Progress in AVM 1970’s - 2011… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

18. Recent Developments • Phenotypic, molecular study  genetic hearing impairment • Discovery of molecular mechanisms of the cochlea lead to understanding of damage, regeneration & treatment • Discovery of OAE  allow detailed study of cochlea function • Stem cell research (future auditory applications)

19. Genetics of deafness • Identification of genes associated with deafness/balance disorder, understanding the pathological mechanisms underlying deafness, determination of their function (Karen Steel & others) • Role of AVP- careful description of the phenotype- clinical audiovestibular status, which help geneticist with a diagnosis

20. For clinical practice-REACHING THE DIAGNOSIS • Describe the clinical presentation: • Hearing loss, vestibular status, radiology • Exclude syndromic causes of Hearing loss • Consider GJB2 mutation screen in all nonsyndromic cases with unknown aetiology • Consider mitochondrial inheritance in multigeneration families unless there is clear evidence of transmission from a male

21. Molecular mechanisms of the cochlea Understanding the mechanism of hearing damage in the cochlea helps in discovery of new treatments or protection against noxious agents

22. Auditory System Ext Ear MiddleEar Inner Ear VIIIth Nerve SOC…IC Cochlea Retrocochlea Conductive Sensorineural Sensory Neural VIIIth n Central Brainstem Temp lobe Lower Upper

23. OAE

24. Otoacoustic emissions in baby with auditory neuropathy [ANSD] Cochlear microphonic and ABR in baby with ANSD

25. OAE- use • Screening for cochlear damage cisplatin treatment, ototoxic drugs mobile users/ noise exposure • Screening for hearing loss- neonates, difficult to test, learning disability • Progression of disease- Meniere’ s disease • Impaired cochlea- normal PTA+ Tinnitus • Malingering • Diagnosis of auditory neuropathy

26. From Tuning Fork to……

27. ….. video head impulse test (vHIT) • Detects vertical semicircular canal dysfunction as well as horizontal • H. MacDougall; L McGarvie, M Halmagyi, I Curthoys, K. Weber (2012)

28. Important research - findings • Yoshinaga- Itano = critical time for language development = first 6 months of life • Anu Sharma = congenital deafness leads to an atypical organisation of the auditory nervous system, there is cross modal plasticity of the nervous system. There is a defined period (sensitive period) of central auditory reorganisation benefiting children with CI

29. Progress in AVM 1970’s - 2011… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

30. Development of new technology • Hearing aids - digital • Cochlear implants • Brainstem implants • BAHA( bone anchored hearing aids) • Vestibular implants • Emerging technologies of assessing vestibular mechanisms • VEMP (vestibular evoked myogenic potentials) • HTT (Head thrust test) • VAT (vestibular-auto rotation test) • New range of motorised Barany chairs with • computerised analysis

31. Recent Service Development • MHAS/MCHAS – Modernisation of Hearing Aid Services for Adults and Children • Digital Sound Processing • Bone Anchored Hearing Aids programme • Cochlear Implant programme • Newborn Hearing Screening Programme [NHSP]

32. Earlier identification of hearing loss - 2000 • In 2000, the Joint Committee on Infant Hearing issued a new position statement, which included a recommendation that hearing loss in infants be identified by the age of 3 months and that intervention be initiated by the age of 6 months

33. UK Perspective 2012 • All areas of England have been offered neonatal hearing screening since March 2006 • Over 4 million babies have been screened since the programme started • A rigorous Quality Assurance Programme ensures that all hearing screening services around the country are assessed on a regular basis to check that they are effective and safe

34. Identification • Screening at birth – TOAE and Automated ABR (99.7% of all births) • Follow up TEOAE and diagnostic ABR as necessary and screening output set < 12 weeks

35. Fitting of Hearing Aids • Target – amplification within 4 weeks of identification • Upper limit – 4 months but usually less NHSP Annual Report 2010-11

36. Amplification-Hearing Aids

37. Amplification-Implantable Devices

38. Progress in AVM 1970’s - 2011… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

39. Development of new diagnoses and treatment • Auditory neuropathy • Auditory processing disorders • Migraine related dizziness • Dehiscence of semicircular canal • New methods of vestibular rehabilitation - from Cawthorne-Cooksey (1940’s) to customised / visual vertigo exercises • Positioning manoeuvre for treatment of BPPV (Epley’s / Semont / Gans etc…) • Virtual reality and OKN desensitisation treatment for visual vertigo • Brainport ?

40. Visual vertigo treatment (Pavlou)

41. Progress in AVM 1970’s - 2012… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of pathways • Developments: translational research

42. Development of services • Modernisation of NHS Hearing Aid Programme  introduction of digital hearing aids • Introduction of NHSP  OAE + ABR new  auditory neuropathy • Paediatric balance/dizziness services • Central auditory processing services • Triaging for vestibular disorders (head thrust test) • Screening for hearing disorders (hearing screening device)

43. Recent Developments (Cont) • Development of Paediatric Vestibular Science and Services • Development of a screening test battery in children • Development of new testing techniques allows more precise assessment of child’s vestibular organ and plan better customised vestibular therapy • Assessment of vestibular function in patients with hearing impairment complements phenotypic presentation of a disorder thus helping with a diagnosis.

44. Vestibular Science / Disorders

46. Diagnostic Tests of VOR Caloric Test (low frequency) [0.001Hz] Whole body passive rotation [0.01- 0.6-1Hz] VAT (active rotation) [2-6Hz] A H OVAR SVV Otolith P VAT active rot [2-6Hz] VEMP – cervical & ocular

47. Somatosensory Foot sole pressure Vision CNS Interpretation Learning Adaptation Compensation Hearing Spatial orientation Balance control Graviceptors Labyrinths Image stabilisation

48. Diagnostic Process Symptoms Assessment Diagnostics Management Vestibulometry AudiometryRadiology Haematology Biochemistry Dizziness Vertigo Disequilibrium ± Auditory ± Neurological ± Cardiac/ Vascular ± Other Site of lesion Dehiscency of SCC, dilated vestibular aqueduct Medical Surgical Rehabilitative  Underlying Medical Condition Cognitive behaviouralTherapies Physical

49. Clinical service quality assurance • Clinical Governance • Protocols / Guidelines • Audit • Clinical Research • Appraisal / Revalidation

50. Progress in AVM 1970’s - 2012… • AVP- role, training, manpower • Audiologists- role, training, manpower, career • Scientific Discoveries in Audiology • Developments in technology • Developments - knowledge of medical conditions • Developments in service provision • Development of patient pathways • Developments: translational research