Deafblindness

Deafblindness is the combination, in one person, of hearing loss and sight loss. Reports and translations also call it dual sensory loss or multisensory impairment; all of these mean the same thing. Total deafness together with total blindness is rare. Usually both senses keep some function, and that is exactly what misleads the people around: the person hears something and sees something, so surely it cannot be that bad. In reality the difficulties are far greater than for someone who has lost only hearing or only sight, and the reason is worth spelling out.

Why two losses add up to more than two

Hearing and sight cover for each other constantly, and none of us notices it happening. Someone hard of hearing reads lips, watches faces, catches the gesture that goes with a sentence, and finds their way around a room by eye. Someone with poor sight recognises people by voice, works out from sound where the door is and whether a car is coming, and listens to books. When the second sense weakens, that whole system of substitution collapses: there is nowhere left to borrow the missing clue from.

This is why moderate hearing loss combined with moderate sight loss disrupts a life more than severe damage to a single sense. Three things suffer at once: communication, access to information, and the ability to get about independently. From which follows a practical rule — when one sense is already impaired, the other needs unusually close attention and regular testing, without waiting for complaints.

What relatives notice first

In older people everything creeps up slowly, and the person concerned is the last to register it: to them, other people have started mumbling and the lighting is poor everywhere. From the outside it shows sooner.

Pointers to hearing:

• the television keeps getting louder and the household keeps asking for it down;
• conversation is hard work, particularly in a group, in a café, on the phone;
• no response when you speak from behind;
• the doorbell, a knock, the microwave beep all go unheard;
• frequent asking to repeat, requests to speak slower and more clearly, answers that miss the question;
• speaking louder than before and starting to avoid noisy places.

Pointers to sight:

• a book or phone held right up to the face, a chair pulled closer to the screen;
• markedly worse vision at dusk and also in bright light;
• familiar people not recognised until they speak, facial expression no longer read;
• bumping into furniture more often, missing a step, catching door frames;
• feeling for objects on the table instead of finding them by eye;
• no longer looking at the person speaking, no eye contact held.

In children it looks different: a baby does not turn towards a voice or follow a toy with the eyes, an older child lags in speech, fears the dark more than others and stumbles in unfamiliar places. One pairing is worth memorising: if a child with reduced hearing becomes clumsy in dim light and complains of seeing as though through a tube, the retina needs examining without delay.

Where it comes from

There are many causes, and they fall into two groups. The first is what is present from birth: extreme prematurity with its effects on eyes and ears, infections caught in the womb — rubella, cytomegalovirus, toxoplasmosis — severe damage to the nervous system such as cerebral palsy, and the effects of alcohol drunk during pregnancy.

The same group contains the inherited syndromes, which are worth knowing about because they are so often recognised late. The commonest is Usher syndrome: the child is born with hearing loss or deafness, and vision begins to fail later, in adolescence or young adulthood, from retinitis pigmentosa. The first signs are poor night vision and a visual field that narrows gradually, as if looking down a pipe. Another is CHARGE syndrome, where hearing and vision problems come with malformations of other organs. Early genetic diagnosis changes a great deal here: the family has time to teach the child tactile ways of communicating while there is still sight to learn with.

The second group is what arrives with age, and this accounts for most cases. Age-related hearing loss lands on top of age-related eye disease: macular degeneration, cataract, glaucoma. Diabetic retinopathy joins them when diabetes has been present for years. Hearing and sight can be damaged together by meningitis, encephalitis, a stroke or a serious head injury. Medicines contribute too: some antibiotics and certain cancer drugs harm the inner ear, and a few drugs taken over years harm the retina, which is why such courses come with scheduled checks.

When it needs checking immediately

Slow decline is discussed with a doctor at a routine appointment. But some situations are measured in hours and days, and waiting costs something that does not come back.

• Hearing in one ear disappears abruptly, over hours or a day, with no pain and no cold. This is sudden sensorineural hearing loss, and treatment is worth anything only in the first few days.
• Vision in one eye drops suddenly and painlessly, or a dark curtain appears across it, or floaters and flashes arrive all at once. That is how retinal detachment and a blocked vessel in the eye announce themselves: examination the same day.
• An eye turns red and painful, vision clouds over, coloured halos appear around lights, and headache and vomiting follow. This is an acute attack of glaucoma and needs urgent care.
• Someone over fifty develops a new, persistent headache, a scalp that hurts when combed, jaw pain on chewing, and then one eye goes dark. This can be temporal arteritis — a rare but extremely dangerous cause of sudden blindness which, treated quickly, can be prevented in the second eye.
• Loss of sight or hearing arrives together with weakness in an arm or leg, a drooping face or trouble speaking: call an ambulance, 112 is the single European number, because that is how a stroke begins.

How it is identified

In newborns, hearing and vision problems are looked for at the very first screening after birth and then at the routine checks of the first year. If the baby was born early or came through a serious infection, the checks are repeated more often, because some of these problems do not show up straight away.

In adults the order is different. Eyes are checked routinely every one or two years, and immediately once any symptom appears; with diabetes, the retina is examined on the schedule the doctor sets. Hearing is measured by audiometry whenever there is a complaint, and it can be requested directly, without waiting for a referral. A diagnosis of deafblindness is made once loss of both senses is confirmed, but the assessment does not stop there: hearing and sight are re-tested periodically, because both the support needed and the route of communication depend on exactly how much of each sense remains.

Separately there is an assessment of needs, and it should be done by a professional trained specifically in deafblindness rather than only in blindness or only in deafness. It covers how the person communicates best, how they move about, how they manage the household, study or work, their mood and their circle, and what equipment they need. How that assessment is arranged and who pays for it differ from country to country, so the route is worth checking with your own doctor or with a local organisation working on sensory impairment.

What helps in living with it

Full recovery of the senses is rare, but the range of what a person can do unaided grows very noticeably. The starting point is always the same: preserve and get the most out of whatever hearing and sight remain. Cataract is operated on with a replacement lens, glaucoma is treated with drops or laser, diabetic retinopathy with laser and injections into the eye, wax plugs and ear infections are cleared — and sometimes that alone transforms the situation.

Then come the equipment and the ways of communicating:

• hearing aids and, for profound loss, a cochlear implant or a bone conduction implant;
• low-vision aids: spectacles, illuminated magnifiers, electronic magnification, large print, contrast marking, bright directed light;
• clear speech without shouting, the simplest and most overlooked resource when some hearing remains: face to face, in good light, in short sentences, with the background noise switched off;
• the deafblind manual alphabet spelled into the palm, and capital letters traced on the hand with a finger, understood by almost anyone without lengthy training;
• tactile sign language, where the person rests their hands on the hands of the signer, and signing within a reduced frame when the visual field has narrowed;
• raised scripts read with the fingertips, and refreshable displays that render text as dots for a computer;
• phones and keyboards with large keys, alarms and doorbells that vibrate and flash.

Training in independent travel — with a cane and in some cases a guide dog — is badly underrated: it is what gives a person back the ability to leave the house. Many are entitled to a communicator-guide, who relays what is happening around and interprets the conversation at the same time; for those born deafblind there are specialists who support the discovery of the world through touch from early childhood.

The home is adapted to the person, not the other way round: remove thresholds and cables, mark the edges of steps and the light switches in contrasting colour, add light in the kitchen and hallway, keep every object in a fixed place. And one request to relatives: do not decide on the person's behalf or guess what they want. Ask what suits them, and say what you are about to do before taking them by the arm.

Online consultation

A remote appointment is a good way to take the first step and sort out where to go. The doctor will listen to exactly what has changed in hearing and vision and over what period, set it against the illnesses already known and the medicines being taken, and say which tests are worth doing and in what order, and who to see first — ear specialist, eye specialist or neurologist. It is also the place for what worries the family: how to speak to a relative so that they understand, which equipment to try, whether genetic testing is worth raising when a deaf child starts having night blindness, and where to apply for an assessment of needs. Testing hearing and vision themselves cannot be done through a screen: that takes instruments and a room. And the signs listed above are not handled online at all: sudden loss of hearing or sight needs examining the same day, and a suspected stroke needs an ambulance.

This material is for information only and does not replace medical advice.