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Abstract
There are over 87 000 Deaf people in the UK with British Sign Language (BSL) as their first language.1 Few healthcare professionals receive training in Deaf awareness or in BSL, and missed diagnoses and inadequate treatment of Deaf patients are estimated to cost the National Health Service £30 million per year.2 Neurologists are likely to encounter Deaf BSL users in their practice, but without prior experience may find consultations challenging, especially within the time constraints and pressure of a standard clinic. In this article, we provide guidance on consulting with Deaf people in a neurology clinic, drawing on experience from our cognitive clinic for Deaf BSL users where effective communication is essential.
- dementia
- cognition
- clinical neurology
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Introduction
The art of communication is crucial for a therapeutic patient–doctor relationship and for the neurologist to intervene successfully. This is challenging at any time but presents unique issues in patients with deafness. Clinicians risk missing some presentations due to communication difficulties and subtle differences in the way symptoms are expressed in both spoken and sign languages.
Deaf patients often report communication difficulties when accessing healthcare. In a UK survey of primary care, over a third of patients reported uncertainty about the doctor’s management of their case.3 Research by the Deafness, Cognition and Language Research Centre at University College London identified that existing services were not adequately serving Deaf British Sign Language (BSL) users with acquired cognitive deficits,4 there being late and missed diagnoses and poor access to services. Therefore, in 2011 the National Hospital for Neurology and Neurosurgery (London, UK) set up a specialist cognitive disorders clinic for Deaf BSL users.
BSL is a visual language that combines hand movements, facial expressions and body language to convey meaning. It has its own grammar, making it distinct from English, and there are many UK regional variations. It also differs from that used in most other countries, including English-speaking ones such as America and Ireland, which have developed their own sign languages. Understanding and communicating with a Deaf person also require appreciation of differences in Deaf culture, access to education and lived experience as a Deaf person, as these form the context for the responses received in clinical interviews.
Here we focus on communication with prelingually deaf people who use BSL. Middleton et al 5 previously covered how to communicate with people with hearing loss. Box 1 outlines commonly used terms relating to deafness.
Commonly used terms
Deaf written with a capital ‘D’ refers to the community of Deaf people who use sign language and regard themselves as a collective identity with a shared history and culture. Conversely, deaf with a lowercase ‘d’ refers to those with a hearing impairment who generally use spoken language.
Prelingual deafness refers to those who became deaf before acquiring spoken language and may rely on sign language to communicate.
Preparation before clinic
We aim to create a welcoming and accessible environment for Deaf people. Our administrative staff are trained in Deaf awareness, and our clinic website has a video in BSL explaining what to expect from an appointment and how to change an appointment time. Deaf people often prefer visual rather than written information (eg, a map or photo of the hospital) as well as communication by email, text messaging or video calls in BSL. We provided an email address for changing appointments, as phone calls are not accessible except via Next Generation Text Relay or BSL Video Relay.
Ideally, outpatient waiting rooms should have visual displays to alert patients when the doctor is ready to see them, as waiting for an appointment can be highly stressful for people who cannot hear their name being called. If there is no visual display, then it is essential to meet the patient in the waiting room. Making eye contact and showing your name badge help to reassure the patient, and knowledge of a few signs in BSL goes a long way in establishing trust and rapport. We recommend booking a longer appointment slot for new patients if possible to incorporate the extra time it takes when working with an interpreter. In addition, Deaf people often bring many family members who are keen to discuss the problems, and describe their symptoms using narratives, rather than specific examples, both of which increase the length of the consultation.
Room set-up
Try to ensure the clinic room has sufficient space and good lighting. Do not sit in front of a window or strong light that might create shadows or silhouette your face. The positioning of individuals can significantly affect the flow of the consultation; the aim is to ensure clear lines of sight between those using sign language (see figure 1). The interpreter is positioned close to and slightly behind the doctor so that the patient and relatives can see both in their same line of sight. This enables understanding without them having to turn away, assisting rapport. Having BSL-fluent staff in the room, such as our Deaf neuropsychologist, allows monitoring for additional information, such as subtle changes in signing ability that might indicate early aphasia.
Aerial diagram of the clinic set-up illustrating lines of sight.
Using an interpreter
The clinician should establish a patient’s preferred method of communication before the clinic. UK General Medical Council standards of care require the use of a registered BSL interpreter with a Deaf BSL user.6 It is essential to use an interpreter familiar with neurology settings to ensure accurate translation and understanding. Using the same interpreter for all consultations has been invaluable in the cognitive clinic, where the team has developed awareness of each other’s preferences and style of communication.
If you have not worked with a particular interpreter before, it helps to spend a few minutes discussing the plan for the consultation with them before seeing the patient. For example, you may wish to discuss how you would want certain questions to be framed during neurological history taking, and emphasise the need to interpret carefully to avoid altering meaning in a neurological context. This is a common problem when working between two languages, as interpreters are trained to find the meaning, rather than to translate verbatim. Due to the variation in signing ability among users, interpreters often ‘repair’ disordered or dysfluent communication and can inadvertently mask underlying language difficulties.
In the cognitive context, spotting changes such as paraphasias, prosodic difficulties, semantic and phonological errors, and changes to facially expressed grammar or spatial syntax is usually only possible by highly skilled and experienced BSL interpreters7 or specialist clinicians who are fluent signers. When performing cognitive testing, it is important to be aware that using an interpreter can introduce variability as well as bias. For example, an inexperienced interpreter may inadvertently help the patient by giving additional clues to questions, or conversely not explain instructions clearly enough and so disadvantage the patient.
Family members should not be asked to interpret as this can have pitfalls. Interpreting is a unique skillset and it is not enough simply to have language fluency. Interpreting may put undue pressure on relatives (particularly if this is the patient’s child) and they may not fully understand questions. Family members may mistranslate information and are unlikely to be impartial. Patients may also not wish to disclose sensitive information when family members are interpreting. Finally, it can be very traumatic to interpret bad news to a loved one.
Using pen and paper is not an adequate method of communication with a patient who uses BSL. Deaf patients report difficulty in understanding doctors when they use written communication3 and unfamiliarity with medical terms can cause confusion.8 The average reading age of prelingually Deaf people is estimated to be 9 years.9 This is likely due to a lack of accessible education and the difficulties associated with learning a written language that they cannot hear, rather than being due to any learning disability.
Likewise, clinicians should not rely on lip reading as a sole means of communication, as only 30%–40% of words are likely to be understood.10 You are more likely to obtain a detailed history and build a relationship with the patient by using a BSL interpreter, even if the patient also has good speech and lip reading ability.
The consultation
We have listed recommendations for optimising communication, getting the most from the history/examination, and pointers to bear in mind when discussing the diagnosis and management (see Boxes 2–4).
Optimising communication
Always show your name badge when introducing yourself and explain the role of other people in the room.
Confirm the preferred method of communication at the outset, and discuss the expected length of consultation and what is likely to be covered (this can help to minimise patient anxiety).
Inform the patient that they can ask you to slow down or pause if they become fatigued. Constantly watching, lip reading and signing can be tiring for patients, which can impact on cognitive testing in particular.
Use short sentences and be aware that the interpretation will not be temporally synchronised with what you say or with the patient’s facial expression. This is because a good interpreter will strive to find the best way to elicit the information you are seeking. Give the interpreter time to explain.
Some Deaf people may find conditional questions difficult to answer (eg, ‘what would happen if…’); therefore, keep sentence structure simple.
For patients who can lip-read, look towards the patient, avoid covering your mouth and speak at a normal pace and volume (speaking loudly and overemphasising words can actually distort the lip pattern, making it difficult to lip-read).
Remember to look at the patient and not the interpreter. Maintain eye contact and do not look away while communicating to take notes or use the computer. Deaf people perceive this as not paying attention to them or listening.
Always talk with the Deaf person rather than their hearing relatives, deferring to them only with permission of the patient and make sure the Deaf person remains fully included in the conversation about them.
Optimising the history and examination
Take time at the outset to establish the patient’s level of education and likely comprehension: often unfamiliar terms such as ‘dementia’ may need explaining.
If Deaf patients do not understand the question, they may provide a response that is tangential or not relevant rather than asking for clarification. This is true for many Deaf people without impairments but can particularly be the case in those with learning difficulties, language delay or acquired cognitive impairments such as aphasia.
Superficial questioning may not immediately yield an answer and further probing of specific areas is often required.
Deaf people often use visual demonstrations, stories or metaphors to explain their symptoms—see box 5 for an example.
Seizure disorders and abnormal movements can pose particular difficulties. Although physical symptoms may be easier to express in British Sign Language, it can be difficult to translate them into the spoken word. Asking the patient or relatives to video seizure events can therefore help when the diagnosis cannot be reached from the history.
There are many language-specific and culturally-specific aspects to consider when asking about mental health disorders. For example, there is no single sign for anxiety—instead there are signs meaning ‘anxious’ that describe the different symptoms of anxiety, such as ‘worry’, ‘rumination’, ‘shaky legs’, ‘churning stomach’ and ‘palpitations’. It is therefore important to be clear on understanding with the patient and interpreter.
Collateral history from family members is very helpful. Be aware of your limitations when using an interpreter, such as the potential for miscommunication, particularly if the diagnosis rests on key parts of the history, and always double-check that the patient is clear on what you are discussing.
When conducting a physical examination, ensure that both the interpreter and the patient understand what you are testing with short, clear instructions, and demonstrate movements as necessary. Signpost your next actions to prevent any unexpected moves that may provoke anxiety.
Discussing diagnosis and management
Deaf people tend to be direct in their communication, and it is important to get straight to the point when delivering the diagnosis.
Patients respond well to visual communication, for example showing them their brain scan or using a diagram to explain their condition.
Visual information can also help when discussing treatment plans, such as taking a photograph of medication that the patient needs to take.
Use plain English when writing clinic letters or correspondence to aid understanding.
Provide information in an accessible format such as ‘Easy Read’. The online supplementary file to this paper lists some online resources for Deaf people with videos in British Sign Language.
Supplemental material
Example of a visual metaphor used to describe memory symptoms by a Deaf patient (adapted from Young et al, p4421)
‘We wanted to describe the decrease in short-term memory capacity in dementia, while long-term memory is retained and of greater importance to the individual. We signed this by showing with one hand a small sphere and with the other a large sphere. The former represented distant childhood memories when the store of memories was small. The latter was the adult brain full of a lifetime of memories and therefore larger. As the effects of dementia increased, we showed how the larger sphere shrank and the smaller sphere grew representing the growth in the importance of distant memories. The visual representation of the complex cognitive process was immediately graspable’.
Cognitive assessment of Deaf patients
Cognitive assessment of Deaf patients can be challenging for many reasons, and a lack of familiarity with healthy Deaf people can make it difficult for neurologists to know what is normal.4 Prelingually Deaf people score within the normal range on non-verbal tests of cognition11; however, there can be subtle differences in cognition compared with hearing individuals, such as relative strengths in visual attention.12 From a cultural perspective, some Deaf people will not necessarily know who is the current prime minister, be able to identify pictures of famous people in the hearing world or to name common musical instruments, due to a lack of exposure or relevance in the Deaf community.7 Questions should therefore be tailored appropriately to their cultural experience.
Standard cognitive tests are not validated or normalised for use with Deaf people, and tasks that depend on language can be affected due to lower levels of literacy.7 Picture naming can also be challenging to assess, as the sign in BSL for certain objects often resembles their use (iconicity), for example the BSL sign for ‘pen’ is the motion of writing. Cognitively normal Deaf adults scored in the abnormal range when tested with the Mini Mental State Examination translated by an interpreter.13 In view of these difficulties, members of our team developed and validated a screening test for detecting cognitive impairment for use in Deaf people.7
Assessing language in a Deaf person is complex and aphasia can manifest in many ways, such as using incorrect signs, not matching the sign used with mouthed words, using incorrect sentence structures or signing in the incorrect space.14 It is therefore important to enquire about such changes with relatives who sign, as well as specifically asking the interpreter. Many cases require a specialist assessment with a psychologist or speech and language therapist highly familiar with manifestations of aphasia in BSL, since non-signing clinicians and interpreters will often miss these.
Examining for apraxia can also be challenging; a full explanation of this is beyond the scope of this article, but it is important to be aware of the difficulties. As an example, asking a patient to pantomime an action can reveal the action itself, for example asking how to use a screwdriver in BSL would demonstrate the action. One way around this is to write instructions such as ‘blow a kiss’, ‘wave goodbye’ or ‘use a hammer’, provided the patient has sufficient literacy, or to combine these with pictures.
Comorbidities in Deaf patients
In general, Deaf people tend to have higher levels of underdiagnosis, reduced awareness and undertreatment of medical conditions. They have higher rates of obesity and hypertension compared with the hearing population. Although rates of diabetes are similar, they are less likely to be aware of their diagnosis and to have poorer rates of control.15
Certain causes of prelingual deafness can be associated with other neurological manifestations; figure 2 illustrates examples of these. Although many of these causes are now rare, they are relevant to Deaf adults who are now in middle to older age. Of note, many causes of prelingual deafness are associated with an increased risk of vestibular dysfunction and learning disability in particular.16 17
Causes of prelingual hearing loss with examples of conditions with associated neurological manifestations in brackets. Flow chart on the left taken from Shearer et al.22 Copyright 1993–2019, University of Washington. CMV, cytomegalovirus; HSV, herpes simplex virus; miRNA, micro-RNA; QTc, corrected QT interval.
Deaf adults also have a higher incidence of mental health disorders (see figure 3) as well as higher rates of abuse in both childhood and later life.18–20 In our clinic, several patients referred with cognitive symptoms were subsequently found to have significant underlying mental health conditions. Sensitive enquiry into a patient’s mental health should therefore be considered, particularly if patients present with features of a functional disorder. There are specialist mental health services for Deaf people in the UK, with inpatient and community treatment available in London, Birmingham and Manchester, as well as dedicated community services offered in Bristol, Nottingham, Yorkshire and the North-East of England.
The burden of mental health problems on Deaf people is symbolised by rucksacks, which everyone carries and from which they seek relief. The rucksacks of Deaf people are bigger, but the entrance to services is smaller because accessibility is poor. Reprinted from T he Lancet, Fellinger et al, p1042.18 Copyright 2012 with permission from Elsevier.
Conclusion
Communicating with Deaf patients and their families presents unique challenges in a busy, pressured outpatient clinic. Understanding this patient group, ensuring the environment and form of communication are appropriate, and following some simple guidelines reduce anxiety not only for the patient but also clinical staff. Such changes can enhance the therapeutic relationship, providing a more positive experience for all and increasing the chances of a beneficial patient outcome.
Illustrative case studies
Case 1
A Deaf woman in her 60s was referred with family concerns around memory and self-care. She herself reported long-standing difficulties with memory, concentration and vision. She had trouble seeing things in front of her and used coloured notes around the house to catch her attention and remind her what to do. Her son commented that she could be obsessive and emotionally detached.
She had been diagnosed with meningitis as a child (from which she became Deaf) and with chronic fatigue syndrome as a young adult. No prior cause for her visual symptoms had been found. Throughout the consultation she had rapid, pressured signing and the interpreter had difficulty keeping up. On cognitive testing her memory recall and concentration were impaired and reading of fragmented letters was effortful and slow.
MR scan of the brain (see figure 4) showed asymmetrical volume loss and T2 hyperintensity within both the anterior temporal lobes consistent with prior encephalitis. Subsequent review of a CT scan of the head from 7 years before showed pre-existing low attenuation and atrophy of the temporal lobes. Presumably, the episode thought to be meningitis as a child was in fact encephalitis, which had left her with long-standing cognitive difficulties. The family found having a clear diagnosis very helpful in explaining her condition and she was referred to a psychologist to help manage her symptoms.
MR scan of the brain, axial T2 (A) and coronal FLAIR (B) sequences, showing asymmetrical volume loss and hyperintensity within both the anterior temporal lobes. FLAIR, fluid-attenuated inversion recovery.
There is an increased incidence of undiagnosed conditions among Deaf adults compared with the hearing population. The detailed cognitive history and imaging were crucial in this case.
Case 2
A Deaf woman in her 70s was referred with a 2-year history of worsening communication and becoming more withdrawn. She had no insight into her difficulties and her husband did not sign; however, her daughter (who did sign) had noticed a significant reduction in her output. Throughout the consultation she showed hesitant sign language with reduced utterance length and complexity. Focal testing identified impaired grammar, but with preserved semantic knowledge and other cognitive domains. Neurological examination was otherwise normal. We made a provisional diagnosis of progressive non-fluent aphasia.
An MR scan of the brain (see figure 5) showed generalised volume loss and slightly asymmetrical hippocampi. Cerebrospinal fluid analysis showed normal concentrations of amyloid beta 1–42 and tau.
MR scan of the brain, coronal T1 imaging, showing generalised volume loss with slightly asymmetric hippocampi.
On follow-up assessment, her daughter reported several falls, difficulties with comprehension and binary reversals, which had not come to light before. On examination, she had developed restricted vertical eye movements and saccades, dysarthria, axial rigidity and limb bradykinesia. She was diagnosed with progressive supranuclear palsy associated with progressive non-fluent aphasia.
This case highlights the importance of obtaining a collateral history from relatives or friends that sign and using a specialist neuropsychologist fluent in BSL to assess for language difficulties.
Case 3
A Deaf woman in her 40s was referred with memory difficulties. She reported forgetting appointments and details of conversations. She needed to take a list when shopping alone, but would usually remember to buy everything on the list. She could easily become distracted and had burnt food many times previously. She reported depression since she was a teenager and was taking citalopram 20 mg daily. Her friend felt that her difficulties were long-standing and had not progressed.
On examination, she appeared euthymic. She often asked her husband to repeat things and commented that others signed too quickly for her to understand. Her processing was clearly slowed with impairments in comprehension, signing and executive function. Her memory was comparatively good on testing. Overall, her cognitive profile showed substantially impaired non-verbal IQ and processing speed, with low performance across all abilities suggestive of long-standing learning disability.
An MR scan of the brain (see figure 6) showed right-sided, perisylvian polymicrogyria affecting the frontal, insular, temporal and parietal lobes associated with chronic volume loss. Subsequent enquiry did not identify any history of seizures. She was diagnosed with long-standing moderate learning disability and referred to the learning disability team for ongoing support in managing her difficulties.
MR scan of the brain, sagittal T1 imaging, showing right-sided, perisylvian polymicrogyria.
Comprehensive neuropsychological evaluation in BSL combined with the collateral history and imaging in this case allowed a clear diagnosis of long-standing learning disability. Comorbidities are more frequent in this population, although no clinical sequelae of the polymicrogyria were evident.
Key Points
Providing the appropriate time and environment will allow a thorough assessment and will help to put the Deaf person at ease.
Spending a short time discussing the plan for the consultation with the interpreter before seeing the patient, and using a consistent interpreter make all the difference in accurate communication.
An understanding of Deaf culture as well as the differences in language is important when assessing patients to understand the context of their difficulties.
Be aware of the potential for undiagnosed or undertreated medical or mental health disorders in Deaf patients.
Acknowledgments
We thank Dr Helen Grote for her valuable comments on the manuscript.
References
Footnotes
Contributors MJH drafted the original article. JRA, KJ, MB and CJM provided clinical expertise and critical appraisal of the article.
Funding CJM is supported by funding from the National Institute for Health Research, University College London Hospitals Biomedical Research Centre.
Competing interests None declared.
Patient consent for publication Next of kin consent obtained.
Provenance and peer review Commissioned; externally peer reviewed by Mark Manford, Cambridge, UK, and Nicola George, Cardiff, UK.