THE 2021 LIBBY HARRICKS MEMORIAL ORATION
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THE 2021 LIBBY HARRICKS MEMORIAL ORATION Clinical Professor Harvey Coates AO, DM, MS, FRCS(C) FRACS Indigenous Ear and Hearing Health ---Tackling the silent epidemic Introduction Thank you to Deafness Forum and to Audiology Australia for the honour of being invited to give the Libby Harricks Memorial Oration on a subject that I’m passionate about. Firstly, I should like to acknowledge the Whadjuk Noongar land upon which we meet and the elders, past, present and emerging. The exceptional Libby Harricks, who, in spite of progressive hearing loss, accomplished so much in her advocacy for deaf and hearing impaired people, brings to mind Nelson Mandela’s statement. “ What counts in life is not the mere fact that we have lived. It is what difference we have made to the lives of others that will determine the significance of the life we lead.” 1
In this Oration I will discuss the problem of Indigenous ear disease around the world, why it is a silent epidemic, the causes and effects of middle ear disease, its treatment, and culminating in how we can, in the 2020’s, close the gap in ear and hearing health. My distinguished friend and colleague, Professor Andrew Smith who gave this address two years ago emphasised, from his years leading the ear health strategies at the World Health Organisation, the critical role that Public Health initiatives have in detecting, preventing and treating those causes of deafness and hearing loss. In the WHO-UNICEF-Lancet Commission on ‘A future for the world’s children’, chaired by former New Zealand Prime Minister Helen Clark and published in 2020, the multiple threats of climate change, ecological degradation, migrating populations, conflict, pervasive inequalities and predatory commercial practices to health and the future of all children were outlined.(1) They noted childhood is the ideal time to intervene as, though it’s a time of vulnerability, it’s also a time of opportunity. Intervening in childhood has lifelong, intergenerational benefits. Consider that brain plasticity and neurogenesis are at their peak, at the critical time for cognitive and psychosocial skill development. And so it is that in the last decade, recognition of hearing loss has finally been acknowledged, with hearing loss increasing from 11th leading cause of years living with disability (YLD) in 2010 to the 4th leading cause in 2015, ahead of headline grabbing conditions like diabetes and dementia.(2) 2
Apart from the lancet articles, the World Health Assembly (WHA) ratified a 2017 “Resolution and action plan for prevention of deafness and hearing loss’, and a 2019 WHO and the International Telecommunication Union developed standards for noise exposure from devices to protect the young and adolescents from noise induced hearing loss. In Australia Minister Ken Wyatt unveiled a seven year ‘Roadmap for ear and hearing health.’ Half a billion people have disabling hearing loss-7% of the world’s population, while 1.34 billion have a mild-moderate hearing loss. 32 million children have this all too often overlooked ‘invisible disability’, especially those in low and middle income nations. Indigenous populations in particular carry much of the burden, especially in Asia, the South Pacific and uniquely. In first world countries such as USA, Canada and Australia. It is the Indigenous child with hearing loss that we address today. 3
GLOBAL INDIGENOUS EAR HEALTH Indigenous peoples, also known in some regions as First peoples, First Nations, Aboriginal peoples or Native peoples are ethnic groups who are the original or earliest known inhabitants of an area, in contrast to groups that have settled, occupied or colonised the area more recently. Indigenous societies inhabit every climate zone and continent except Antarctica. For the purposes of this talk, Aboriginal relates to Aboriginal and Torres Strait Islander peoples, the main focus of my research into chronic middle ear diseases. As illustrated in Figure 1, the world map of Indigenous peoples displays the multitude of communities, with an estimated total population of 350 million people. Those countries and First Nation peoples where the prevalence, microbiology and aetiological or causative data of ear health and hearing loss in their Indigenous population has been well documented are the Australian Aboriginal, the New Zealand Maori, North American and Greenland Inuits and Native Americans. In addition to first world countries with high prevalence of chronic ear disease ( particularly chronic suppurative otitis media -CSOM or ‘runny‘ ear ) in their Indigenous population, Tanzania, India, Guam and the Solomon Islands have prevalence rates of greater than 4%. ( Table 1) 4
This Photo by Unknown Author is licensed under CC BY-SA Figure 1. Ethnic Global Groups by the end of the 20th Century The WHO informs us that 60% of the world’s hearing loss is preventable especially permanent or sensori-neural hearing loss due to noise exposure, ototoxic drugs, toxic chemicals and vaccine preventable diseases as well as poor nutrition and trauma. The remaining principal preventable cause of conductive hearing loss is middle ear infection or otitis media (OM) and its sequelae. 5
TABLE 1. CSOM PREVALENCE (WHO) Prevalence of CSOM Nation/Population Group Highest (>4%)—urgent attention Tanzania, India, Solomon needed for massive public health Islands, Guam, Greenland, problem ATSI people High (2-4%)-avoidable burden of Nigeria, Angola, Korea, China disease must be addressed Thailand, Malaysia, Vietnam Philippines, Micronesia, Inuit. Low (1-2%) Brazil, Kenya Lowest (>1%) Gambia, Saudi Arabia, Israel, UK, Denmark, Australia, USA Source: WHO: Chronic Suppurative otitis media: Burden of illness and management options. 6
Not unexpectedly, the causation and risk factors for otitis media are very similar in the Indigenous populations of the world particularly as the most severe form of OM, chronic suppurative otitis media (CSOM) is a disease of poverty. Where there is overcrowding, poor nutrition and hygiene, exposure to cigarette or other smoke, lack of running water and lack of ready access to medical care, OM is prevalent in all its forms in up to 90% of young children. In Indigenous children OM appears earlier, more frequently and more severely than in non- Indigenous children. Almost a third of Aboriginal, Greenlandic and Native American children suffer from chronic suppurative otitis media (CSOM), yet the WHO states that a CSOM prevalence of greater than 4% is a serious public health problem requiring urgent attention. OM-related complications cause 21,000 deaths globally every year.(3) Otitis media-presentation and complications Acute otitis media (AOM) is essentially an abscess in the middle ear (Figure 2) which may perforate causing discharge from the middle ear into the ear canal. If the discharge continues for six weeks, then, by definition, we have CSOM (Figure 3). If, however, the eardrum or tympanic membrane does not perforate and the acute infection settles, we may be left with a non-sterile effusion or glue ear (otitis media with effusion-OME) (Figure 4). Chronic OME or some chronic perforations may lead to cholesteatoma (Figure 5) - skin invading the middle ear, mastoid bone or even the brain, a condition seen in over 10% of children with longstanding ear disease (4). 7
This Photo by Unknown Author is Figure 2. Figure 3. Acute Otitis Media (AOM) Chronic Suppurative Otitis Media (CSOM) Figure 5. OME Figure 4. Figure 5. Otitis Media with Effusion. Attic polyp with cholesteatoma. ( OME) 8
Other complications of OM and its sequelae include mastoiditis, facial nerve palsy, vestibulitis, meningitis and brain abscess. These signs are overt, but it is the silent development of hearing loss in the absence of obvious signs or symptoms that may be difficult to notice. In the child under three, this may present as delayed speech and language development due to the blockage of auditory signals to the processing areas in the brain. This, in turn leads to problems learning new tasks relating to hearing and subsequent learning problems at school. ”If you can’t hear, you can’t learn” is the mantra we emphasise over and again in our remote visits But it is the Aboriginal child with longstanding OME and CSOM that suffers the most from the effects of their middle ear disease. The facts are confronting—the average Aboriginal child has OME for 32 months in their first five years of life compared with three months for non- Indigenous children. Almost all remote Aboriginal babies have OME by age 12 weeks and at least one attack of OM by twelve months. (5) The associated hearing loss affecting a third of Aboriginal children throughout childhood leads to inattention, non-attendance at school, social isolation, early school leaving, lack of vocation, interaction with drugs, alcohol and the judiciary. 93% of adult male Aboriginal prisoners in Darwin gaol have a longstanding hearing loss. (6) CAUSATION OF OTITIS MEDIA 9
So, what is causing this mini-epidemic of OM and its sequelae in Indigenous populations? The risk factors for OM are varied, but the essential underlying issue for development of OM, especially CSOM, is poverty. Risk factors include early nasopharyngeal bacterial colonisation, overcrowding, lack of breast feeding, poor nutrition and hygiene, passive smoking, lack of clean running water and lack of access to medical care. (7) Good housing is critical. Urbanisation worldwide has led to slums, where 40% of the world’s children live in overcrowded, unsafe, unhealthy and socially excluding dwellings. Factors for development of otitis media fall into two major categories, host-related factors and environmental factors. Host-Related factors A major cause of early development of middle ear disease in Indigenous babies and infants is the failure of their immature immune systems to elicit a strong response to an overwhelming nasopharyngeal colonisation by bacterial organisms. Three principal bacteria, together with respiratory viruses are present in both the nasopharynx and middle ear, resulting in recurrent acute OM, CSOM as well as OME at an early age, often before 6 months.(8,9) This causes hearing loss at those critical months for language acquisition from shortly after birth to eighteen months of age. This can lead to loss of central auditory neural plasticity and subsequent delay in speech and language acquisition. (10) The same applies to persistent conductive hearing loss in babies and infants with OME. 10
Twin and genomic studies have isolated genes which increase the risk of recurrent OM such as FBOX 11 discovered at TKI. (11). Environmental factors Overcrowding is seen in many Indigenous households, with substandard housing or inadequate numbers of bedrooms. The average number of persons per Indigenous household is almost five, compared to half that rate in non-Indigenous households, and in some remote regions up to nineteen people may share a home. (12). This overcrowding places children at risk for recurrent upper respiratory tract infections with middle ear and lower respiratory tract infections as complications. This is known as the ‘day care effect’. (13). Passive cigarette smoking, or exposure to other environmental smoke has been shown in the Kalgoorlie Otitis Media study to significantly increase the risk of RAOM and chronic OME. (14) Breastfeeding has a protective effect related to the immunological properties of breast milk. (15). Poor nutrition leads to a multiplicity of health disorders including type 2 diabetes mellitus which can impact on the child’s resistance to infection. Lack of access to clean running water, and lack of access to medical care are other concomitant external factors. In addition to these social determinants of health affecting the development of otitis media, historical and cultural determinants play a role as well. IMPACT OF OM ON CHILDREN’S DEVELOPMENT 11
Otitis media by virtue of its longevity in Indigenous children has an impact that affects their language and speech development, with secondary effects on school learning, socialisation, general development and later, vocational outcomes and interaction with the judiciary. The areas of cognitive development most likely to be affected by hearing loss associated with OM and its sequelae are auditory processing skills, attention, behaviour, speech and language. (16) The specific auditory processing skills affecting these children include difficulty with sound localisation, difficulty discriminating speech sounds in noisy backgrounds. Behavioural and attentional difficulties have been noted and speech perception in Indigenous children with OM showed a reduced ability in those children with English as a second language. (17). A study of Aboriginal children with OM showed that, compared with their peers without OM, they had significantly poorer phonological awareness, spelling and reading skills. (18) (19) Williams (16) has noted those myriad of factors that impact on the ability of an Indigenous child with OM to acquire speech and language. The factors that are associated with high risk are in Table 2. • Early onset of OM under 12 months of age 12
• More than one episode of OM before 12 months of age • Long periods of infections • Poor or no access to medical management • Compromised environment (passive smoking, overcrowding etc.) • Pre-existing cognitive or language deficit • Disrupted attachment • Degree of hearing loss TABLE 2 . FACTORS THAT INCREASE THE RISK OF SUSTAINING LONG- TERM SPEECH AND LANGUAGE DEFICITS FROM OM. (After Williams 2009) At school, Indigenous children who have hearing loss and have had difficulty in picking up the sounds of their own language, may find it even harder to follow lessons in the second language, English. For example, high frequency sounds such as s, f, t, th don’t exist in 13
Aboriginal languages and will be hardest to hear if there is a hearing loss. If this hearing loss is compounded by a noisy classroom as well as a secondary central auditory processing disorder, then the child will be significantly disadvantaged educationally. There are subsequent social and emotional issues with long term hearing loss leading to social isolation, reduced self esteem, mental health issues, truancy issues, drug and alcohol issues, self harm, vocational issues and interaction with the judicial system. PREVENTION OF HEARING LOSS “An ounce of prevention is better than a pound of cure.” 14
Benjamin Franklin. The WHO categorises prevention in three tiers, primary prevention to avert an adverse health condition, secondary prevention to detect a condition early and to treat it promptly and tertiary prevention to reduce the impact of an established condition and restore function, where possible. Significant preventable causes of hearing loss include otitis media, maternal rubella, other infectious diseases, perinatal problems, ototoxic medications, consanguinity and loud noise exposure. The prevention of OM in Indigenous children, apart from vaccination, and appropriate and early medical intervention, is related principally to addressing the social determinants of health. Pneumococcal conjugate vaccination has led to a modest drop in AOM episodes in Indigenous children. But it is those underlying fundamentals of public health that are the essential factors requiring attention in order to effectively prevent OM and its sequelae. Attention to noise protection such as from rifle fire and snowmobiles in Indigenous children in Canada is another preventive action, as well as education and legislation to reduce noise damage from personal musical devices. Strategies that address the social determinants of ear health include those in Table 3. Most of these are primary preventions, but early recognition and treatment of disease is secondary, and ear surgery, hearing aids, cochlear implants, sign language are tertiary preventive measures. 15
Table 3; PUBLIC HEALTH INTERVENTIONS FOR OTITIS MEDIA • Avoiding overcrowding - a bed for each child • Clean water for washing and showering. Swimming pools • Improved nutrition and healthy eating • Increasing breastfeeding to at least six months • Reducing passive smoking from cigarettes and campfires • Keeping flies away from food and children’s ears, nose & mouths • Hand and face washing, BBC program (Breathe, Blow, Cough) • Ensuring all vaccinations up to date, especially pneumococcal • Early intervention for infections, regular health checks • Education programs regarding prevention, early diagnosis and treatment of otitis media These preventive measures are where those at the coal-face in Indigenous communities-the primary health care workers, nurses and general practitioners are the most important to address this condition, in association with Aboriginal, Indian or Inuit health services together with Government and non-Government organisations (NGOs). Secondary to these, audiologists, speech 16
pathologists, teachers of the deaf as well as otolaryngologists support and treat those affected children. There are internationally, many groups and organisations dedicated to help manage the global burden of OM, from the WHO, Inuit, American Indian, and Aboriginal medical services, through to innovative award winning programs such as Deadly Ears in Queensland and Earbus Foundation of Western Australia. Globally, it is well known that persistent, consistent, culturally appropriate interventions deliver the best outcomes in terms of disease and hearing outcomes. EAR AND HEARING CARE IN INDIGENOUS POPULATIONS At the global level, stakeholders in the hearing health community at all levels should join forces to ensure member states have access to educational material such as the WHO Ear Health Manual, training, and equipment through existing government, service organisations 17
and NGOs, universities and research organisations and Indigenous health services. Low cost hearing aids, cochlear implants and telehealth services including tele-audiology should be made available. At the country level, attention to the call to action by the World Health Assembly (WHA) in 2017 requires integration of strategies for ear and hearing care within the framework of their health systems. There is need for collection of high quality population-based data, human resource development, early identification and management Services, noise control and improving access to hearing technologies and communication.(20). The services to Indigenous patients should be culturally appropriate and safe.(21) There is a disparity between supply of services such as health clinics, equipment and staff training and the demand which refers to the patient’s ability to access services with cultural appropriateness. rapport with health professionals and awareness of services. At the regional and community level, early and appropriate antibiotic treatment for acute otitis media and topical drops and local treatment for CSOM, with timely referral to surgeons for grommet, and middle ear surgery accompanied by audiologic and habilitation is essential. As hearing health professionals are scarce in low and middle income countries, capacity building and education are a high priority. Task 18
sharing may be an approach to ameliorate this skills shortage. Research and innovation may allow, as in India, trained technicians performing myringoplasty procedures, or the new tissue engineered techniques with reduced operating time.(22,23) In Australia the 2020 otitis media guidelines and the new app improve access to the most up to date evidence on best practice in otitis media and hearing loss prevention and management for ATSI children. DIFFERENT MODELS OF EAR AND HEARING CARE COMMUNITY BASED EAR HEALTH CLINICS Whether by fly in-fly out ear health teams, such as in the Queensland Deadly Ears Program, or mobile units with sound treated buses, (Earbus Foundation of WA, Variety Club ear buses in New Zealand), the essentials of these programs is to provide diagnostic and therapeutic ear health in the local community, especially in rural and 19
remote communities. This is often closely allied to local community health organisations such as Aboriginal Medical Services providing on the ground support and their own ear health teams as well. Aboriginal Health Workers, Community, school and child health nurses are the linchpin providing ongoing care between ear health team visits. Community liaison and education workers are important members of the team, as are health promotion officers. Initiatives such as the MRFF project chaired by Professor Catherine McMahon are exploring innovative methods to co-produce, implement and evaluate a community-based model of ear and hearing care for Aboriginal children with strengthened partnerships between health and early years through to primary education. Eventually, the aim is for each community to have dedicated Aboriginal Ear Health Workers (or Ear, Eye and Dental AHWs as suggested by Minister Ken Wyatt) and Aboriginal community liaison workers with ear nurse specialists, audiologists and general practitioners providing secondary support and ENT surgeons and other groups such as Hearing Australia providing tertiary services. TELEHEALTH OUTREACH The exponential growth in telehealth, especially during the Covid era has significantly enhanced the capabilities of remote consultations, either as ‘live’ or as ‘store and send’ consultations. Complex audiological procedures such as brain stem evoked audiometry or mapping of cochlear implants can be supervised by telemedicine, as can surgical procedures. (24) The stand-out tele-health centres in ear health are in Alaska and Ontario, with validation of significant benefits both clinically with wait times and financially with cost savings. In 20
Australia, some tele-otology and tele-audiology programs have been in service for over ten years, but there is a significant need for more such initiatives. (25) (Figures 7,) This Photo by Unknown Author is licensed under CC BY-ND Figure 7. Telehealth SURGICAL BUSES Globally, fully equipped surgical buses have been used to perform various specialised surgical procedures in urban, rural and remote locations. Between 50-70% of all procedures performed today are day surgical procedures, ideally suited, with appropriate patient screening, for surgical buses. Examples include those in the Solomon Islands, India, and Bali as well as New Zealand. Otologic surgery lends 21
itself to day surgery and the cost and patient satisfaction of having their surgery in their local region rather than often hundreds of kilometres away is undeniable. Hopefully, the next generation of surgeons will continue to advocate for surgical buses, ideal in Australia’s vast outback. (Figure 8) This Photo by Unknown Author is licensed under CC BY-SA Figure 8. Mobile operating bus INDIGENOUS EAR HOSPITALS In Anchorage, Alaska, the Alaska Native Medical Centre has a 173 bed hospital offering comprehensive medical services including otolaryngology to Alaska Native and American Indian people living in Alaska. The 9 staff otolaryngologists provide medical and surgical management as well as perform telemedicine consultations to remote communities in Alaska, with significant cost savings from live on line consultations 22
compared with transferring patients to Anchorage. The Community Health Aides in each village liaise with the patients to ensure this is effective. ADJUNCTIVE SERVICES Services such as Hearing Australia, a government sponsored agency provides innovative diagnostic services including PLUM and HAP-EE, hearing aid fitting and classroom amplification systems. Speech pathologists, teachers of the deaf and other para-medicals are important members of the team habilitating children with hearing loss. The interface of medicine and education is often overlooked and teachers, Aboriginal education workers together with other community staff are critical in the school aged child’s ear and hearing health. CLOSING THE GAP IN INDIGENOUS EAR & HEARING HEALTH So, how do we address this silent epidemic? We start with community- based organisations educating parents and carers and treating the children at the earliest stages of infection, with frequent opportunistic hearing screening by health workers and nurses, utilising telemedicine for the difficult cases, and expedited treatment 23
Including surgery. We should advocate, develop and ensure pathways into addressing professional inequality on vocational training for Indigenous people. In order to close the gap in ear and hearing health equity in Indigenous communities generally, we need to consider the seven steps suggested by respected public health advocate Professor Ian Ring recently. These are relatable to all First Nations globally - 1. Setting of achievable and fully budgeted targets 2. Needs based funding- Funding based on the burden of disease, which is three times greater in ATSI populations than in non- ATSI populations 3. Focus on services--From resourcing and policy issues at the national level though to staffing, training and resourcing at the regional level 4. Training - Development of a national training plan to ensure all involved in the field are trained in culturally appropriate service delivery, with capacity building for Indigenous staff a linchpin 5. Management - Development of a formal, integrated, multi- layered management system, underpinned with information and evaluation systems 6. Continuous quality improvement - The need for proper systems to measure, monitor and evaluate the quality of services. Output assessment is good, outcomes assessment essential 24
7. Learning from national and international experience - Adaptation of successful programs using systematic application of current knowledge to the local regional services IN CONCLUSION Sixty percent of Indigenous hearing loss and ear disease is preventable. The major problem of chronic middle ear disease is preventable in the majority of cases by diligent application of early diagnosis, timely treatment, appropriate ,supportive habilitation with accompanying translational research and attention to the underlying social determinants of health. Hearing loss cannot and must not continue to be a silent epidemic. REFERENCES 1. Clark H et al, A future for the world’s children? A WHO- UNICEF- Lancet Commission. Lancet, 395; 2020:605-614. 25
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