Throat Cancer – Not Just A Smoker’s Disease

Throat cancer refers primarily to malignant tumours of the voice box and the lining of the muscular tube that lies behind it. The main risk factors are smoking and alcohol and these are synergistic when both factors are present. Throat cancer usually affects older males but it can also affect younger patients of any gender who are non-smokers and non-drinkers too. This article aims to inform the reader of the structure, disease process and management of this type of cancer and what to watch out for to avoid unnecessary delay in treatment.

Anatomy and physiology

The throat is a complex structure consisting of the muscular tube (pharynx) that connects the back of the nose and throat to the gullet (oesophagus) and voice box (larynx). It allows for the safe flow of food and fluids to the gullet and of air through the larynx into the lungs. The larynx consists of muscles, cartilage and nerves which are designed to safeguard the airway, protecting it from potential entry of food and drinks by “closing” itself off. This reflex is paramount in survival and is what allows us to thereafter generate immense force from our lungs to produce a cough to clear the airway. Failure to do so can result in choking and lead to chest infections such as pneumonia.

Other functions of the larynx include voicing and the capacity to strain and raise the abdominal pressure. There are three main parts of the larynx (Fig 1): the upper part is the supraglottis; the middle, the glottis – where the vocal folds/cords are located; and the lower part is the subglottis, where the voice box joins the windpipe (trachea). The lining of the throat is known as its mucosa. It is the toxic irritation of cigarettes and alcohol with their chemical compounds that alter the cells of the mucosa causing them to change their appearance and behaviour leading to an irreversible genetic alteration and to cancer. Genetic factors, exposure to certain chemicals and chronic exposure to acid, viral infections and other irritants have been postulated as causes in other patients.

Symptoms and signs

Patients with throat cancer present in several ways. Hoarseness in patients with no obvious precipitating cause exceeding a fortnight should be referred on for specialist investigation. Lesions on the true vocal folds affect their vibratory capacity and result in an abnormal rough voice. If the mass of the tumour or its extension reduce the mobility of either or both cords, the voice can sound breathy too. Swallowing difficulties and choking are also symptoms that can be present as signs of possible throat cancer.

Throat or ear pain on swallowing are also important complaints that warrant investigation. Blood in the saliva and increasing noise while breathing and shortness of breath can be later features of throat cancer and warrant rapid referral to an ENT surgeon. Patients with extension of their cancers may, rarely, present with disease that has spread to the lymph nodes in their neck which are often painless and hence should be seen urgently for further assessment.

History and examination

A full history is sought and risk factors ascertained. The state of the patient is appraised to assess the severity of the disease, especially in cases where the airway is either compromised physically, potentially leading to asphyxia, or in its inability to protect the lungs from aspiration of ingested solids and liquids. Thereafter, patients are examined with particular attention paid to their larynx, back of the tongue and side of their throats. This is achieved using a flexible fiberoptic endoscope known as a nasendoscope which is passed via the nostril, through the nose and above the voice box. Other methods of examining the larynx include using a laryngeal mirror and rigid endoscopes that are angled so they can visualise the throat through the mouth.

Investigations

Imaging of the neck and throat are achieved using sophisticated cross-sectional imaging modalities such as an MRI or CT scan. Further investigation includes examining the patient’s upper aerodigestive tract under a general anaesthetic with concomitant biopsies of the lesion/s in question with analysis by the pathologist. Thereafter, the confirmed cancer is staged and treatment recommended after discussion in a multi-disciplinary tumour board made up of specialist oncologists in surgery, radio and chemotherapy, pathology, radiology and specialist nurses, speech therapists and dietitians.

Treatment

Throat cancer may present at various stages, often divided into early or late. There are also pre-cancerous stages that if untreated may develop into cancer. Hence, treatment is tailored to the disease and patient along with risk factors such as persistent smoking, drinking etc. Early cancers can be treated with a single modality of treatment – that is, either by surgery alone or radiotherapy.

The decision to use a particular modality is made based on the site and accessibility of the  tumour, the ensuing risks to the voice and swallow and cost. Minimally invasive surgery utilising lasers (Figure 2A and 2B) has been well established as an effective method to treat laryngeal cancer. It is very accurate and can be repeated unlike radiotherapy that is often a one-off treatment and therefore rarely repeatable.

More advanced cancers require more aggressive treatment including the incorporation of chemotherapy where applicable to radiotherapy. Transoral robotic surgery has also been used to great success and in cases where patients may have already had radiotherapy and salvage surgery is advocated but carries higher risks of complications. Open surgery has become less common currently as “laryngeal preservation” is advocated to avoid what is generally a mutilating removal of the voice box with subsequent permanent changes to the patient. Total laryngectomy, however, in cases of advanced cancer is still a very oncologically sound and effective operation.

Voice rehabilitation

With the loss of the larynx comes the obvious loss of the capacity to speak. Along with this is the fact that the patient’s throat is no longer at the junction box between the airway (trachea) and food way (oesophagus). The patient therefore has a complete separation between the two tubes with the windpipe now attached to the skin of the neck below the removed voice box.

Various ways of voice rehabilitation have been developed including “oesophageal speech” where the patient swallows air and belches it out in a controlled manner causing vibration of the back of his tongue, mouth and lips while moving his tongue to speak. Other ways include the use of a speech valve – a purpose-built device that is inserted through the back wall of the windpipe into the oesophagus. This valve allows for the channelling of air from the lungs into the oesophagus and similarly out through the mouth causing vibrations that result in sound.

An electronic device that resembles a microphone known as an electrolarynx can be used and this transfers vibrations from the cheek to the device.

Finally, some patients rely on sign language and writing to communicate.

Conclusion

Laryngeal cancer is a cancer that can affect the young and old. It is seen in non-smokers and non-drinkers too. Symptoms that affect the voice box and throat that do not improve after a fortnight or so such as hoarseness, pain, swallowing problems, cough and shortness of breath should be referred on for specialist input quickly.

Children’s Ear, Nose and Throat Problems

Children commonly have issues with their ears, nose and throat. This is partly because of the natural physiological process of development which can make them prone to viral infections and allergies to name a few. While the common cold and ‘flu’ affect children and adults alike, the complications of these often ‘minor’ ailments can lead to more serious problems. An example would be tonsillitis which in the past when untreated could cause problems with the heart valves!

Thankfully this is very rare now. Nasal congestion and ‘sinus’ issues may spread to the eye (potentially causing blindness) through the thin bone that separates it from the nose. Commonly, children can have a blocked or painful ear which may affect their hearing, reducing their attention span, affecting speech and language and causing behavioural problems too. Allergy to dust mite, pets and different plant pollen is very common.

Sneezing, itching of the eyes and nose as well as a runny or blocked nose are typical symptoms. Referral to an ear, nose and throat (ENT) surgeon for an allergy test as well as treatment for their symptoms is highly recommended. Currently, treatment using a tablet placed daily under the tongue can help cure patients with some of these allergies and in many cases it helps avoid other conditions such as asthma later on in life.

Some pointers you can use to check on your child:
1. Does your child snore?
2. Does your child have recurring colds that progress into a sinus infection?
3. Does your child always complain of headache, and/or giddiness?
4. Does you child have a short attention span?
5. Does your child sneeze?
6. Does your child always feel tired and sleepy?

Ear, Nose & Throat Cancer
There are several types of cancers that an ENT and head and neck surgeon manages. They tend to affect adults and generally men who smoke and drink although this is not always the case. Cancers of the back of the nose (Nasopharyngeal cancer, NPC) tend to affect non-smokers and drinkers of Chinese ethnicity more than other populations. These cancers can be screened for in high risk groups. They present without much warning – with pain or bleeding being uncommon symptoms. A lump in the neck, blocked nose and blocked ears with some hearing loss may be the initial complaint. Quick referral to an ENT surgeon is strongly advised to assess the back of the nose with a special video telescope known as a nasendoscope.

Biopsies and scans may be necessary thereafter. Other cancers such as tonsillar (oropharyngeal) cancer are becoming more common partly due to a viral driven pathway – the human papilloma virus (HPV). Patients may present with an enlarged tonsil, swallowing issues or a neck lump. They tend to be younger and the condition is not caused by smoking and alcohol. Thyroid cancers are commoner amongst younger women and have a good prognosis usually. Surgery is the main treatment and in some cases it is done in combination with radio-iodine treatment.

Salivary tumours are rare. They are also treated surgically as the main management option. These tumours can affect the ‘major’ glands but are more aggressive in the minor ones that are dotted all over our oral cavity and throat. Any lump that is new within the mouth or neck should therefore be investigated if it does not regress or disappear after a fortnight or so.

Hearing Loss
Hearing loss affects the young and old. Childhood hearing loss can be due to genetic causes or birth issues. Certain drugs can also cause hearing loss. The commonest causes in children tend to be less serious and usually improve and disappear as the child develops. Often, the cause is a developing Eustachian tube which is short and less angled compared to an adult. Blockage or failure to clear mucus in the middle ear results in hearing loss which can also cause ear infections. Large adenoids (lymphoid structures similar to tonsils) can act as physical barriers to the opening of the Eustachian tubes and be a nidus for infection too. Surgery can help treat these conditions and is commonly performed in recalcitrant cases.

Age-related hearing loss is becoming a global concern as research continues to demonstrate its link with dementia. Many elderly patients become socially isolated and are not provided with the right recommendation for treatment – be it hearing aids or even cochlear implants. As the hearing begins to weaken, other functions take over the hearing centre in the brain creating a situation where the hearing areas of the brain do not ever recover from. Early referral to an audiologist and ENT surgeon is recommended.

Not all wheezing is asthma-related

Asthma is a common medical condition affecting the lower respiratory tract. It affects all age groups, genders and is seen globally. It has several causes – namely genetic and environmental factors. However, in the absence of an obvious precipitant, it is often idiopathic (when the cause is unknown). Asthma is characterised by breathing difficulty, often at night and associated with wheezing, shortness of breath (hence its name from the Greek “to pant”), chest tightness and coughing. There are however other medical conditions which present in a similar vein which are characterised by noisy breathing when the patient is awake, strained/rapid breathing (and in severe cases) leading to cessation of breathing and death. Some of these conditions manifest very early in life and others in adulthood.

The aim of this article is to provide the reader with a few causes of breathing problems affecting children and adults that may occasionally be treated as asthma but affect the upper airway as opposed to the lungs.

Anatomy

The airway can be divided into two broad parts – the upper and lower. The upper part provides a common passage for food, fluid and air to pass through the oral cavity (such as from the nose for air) and into the back of the mouth and thereafter into the throat (pharynx). It is at this crossroads that our physiological control mechanisms separate what enters the lungs and what enters the gullet (oesophagus). The upper airway descends into our voice box (larynx) through the windpipe (trachea) and into the lungs which are found within the chest (thorax).

The narrowest portion of the child’s upper airway is just below the vocal folds (level of the cricoid cartilage) and above the start of the trachea while the adult upper airway is narrowest at the level of the vocal folds. The vocal folds are found within the voice box which is a cartilaginous structure comprising a shield-like structure and a complete cartilaginous ring. The level at which the vocal folds are is described as the glottis and following on, the area above the supragottis and below – the subglottis before descending into the trachea (Figures 1 and 2).

Noisy breathing

Not all noisy breathing is wheeze. Narrowing of the lower air passages with a characteristic high-pitched noise due to the vibration of the walls of the partially obstructed “air tubes” is effectively wheeze. A frequently confused sound which arises from laryngeal obstruction is called stridor. This sound is also high-pitched, often higher pitched than a wheeze and can vary depending on the location of the obstruction in the larynx and trachea i.e. being present when breathing in (inspiration), out (expiration) or both.

Another noise which is low-pitched and related to obstruction of air above the voice box, at the level of the back of the tongue or tonsils, is called stertor. These noises are present when the patient is awake, unlike snoring which is low-pitched noisy breathing when asleep.

Childhood conditions

Paediatric causes of breathing difficulties can be congenital and present very early in life. Severe cases of upper airway obstruction can be due to the incomplete canalisation of the tubular structures that eventually become the larynx and trachea. These conditions are rare and the milder forms may not present that early in life and become more evident as the child becomes more active or starts to make noises or speaks. Other conditions affecting the child but not present at birth are acquired and can be due to a variety of causes including infections, tumours, foreign body inhalation and neurological conditions.

Laryngeal webs

Mild laryngeal webs in children may cause noisy breathing which can be confused for a wheeze. As the child grows, parents may notice that their child has a hoarse voice and on exertion starts to “wheeze”. Mild laryngeal webs can be asymptomatic and go unnoticed for years, while more severe ones will manifest earlier. Surgery in symptomatic cases is the treatment of choice and is generally successful.

Laryngeal papillomatosis

Recurrent respiratory papillomatosis (RRP) is a viral infection of the upper airway – most commonly involving the voice box but occasionally the trachea and rarely (but potentially fatally) – the bronchioles (lung air passages). It is caused by the human papilloma virus (HPV) which causes skin warts and other tumours such as the cancers of the female cervix and palatine tonsil. The condition affects both adults and children although the disease profile is somewhat different with remission generally a feature in the childhood form. Despite this, many children with this condition can be misdiagnosed for having noisy breathing commonly ascribed to wheezing and if associated with a hacking cough – to croup (laryngotracheobronchitis).

Recalcitrant “wheeze” with hoarseness in children should therefore be a consideration for specialist ENT referral to assess the airway. Treatment of RRP can be fraught with multiple operations to reduce the bulk of viral growths and restore the airway while making every effort not to cause damage to the vocal folds that are very delicate and scarring of the airway.

Adult conditions

Unlike childhood causes of laryngeal obstruction, most adult onset upper airway conditions are acquired. Throat cancer is often seen in smokers and when tumours of the larynx narrow the air passage, the ensuing “noise” can be mistaken for a lower airway pathology. Operations to the thyroid, carotid arteries and upper spine can affect the nerves (causing various degrees of paralysis) that control the movement of the vocal folds and may be subtle – only becoming evident if a co-existing problem such as a throat infection worsens the airway resulting in noisy breathing/stridor. Here are some other interesting causes that may be mistaken for asthma:

Exercise-induced laryngeal obstruction

This condition is seen in young adults who are usually involved in high intensity sporting activities or exercise. The attacks of noisy breathing and breathlessness only come on when performing certain tasks but otherwise the subject is well when not under such duress. The confusion with asthma is common and diagnosis in subjects who already have asthma – difficult. Special investigations including a real-time examination of the patient’s larynx (continuous laryngoscopy examination, CLE) with a flexible nasendoscope while performing the said exercise is indicative but, in many instances, impractical.

Idiopathic subglottic stenosis

This condition is characterised by a deposition of scar tissue in the region of the subglottis below the true vocal folds and above the start of the trachea. It is almost exclusively seen in Caucasian women and is, as the name implies, of unknown cause. Treatment options include minimally invasive “closed” techniques which are performed through the nose using flexible endoscopes.

Conclusion

Upper airway narrowing in the context of the voice box and windpipe can be confused with asthma and leads to delays in the diagnosis and subsequent management of the root cause. It is worthwhile reconsidering whether patients who have been erroneously diagnosed with asthma really suffer from this condition. Recalcitrant cases or cases where two or more co-existing pathologies are present should be referred on to ENT surgeons for the necessary investigations and management promptly.

Dr Vyas Prasad
Senior Consultant Otolaryngologist Head and Neck Surgeon
MB BCh BAO BA (Dublin), MSc DIC (London), DLO (England), MRCS (Edin), FRCS (ORL-HNS)(Edin), FICS

THE BUSINESS TIMES WEEKEND, OCTOBER 19-20, 2019

Age-related hearing loss has wider ramifications

Age-related hearing loss has wider ramifications

The Impact of hearing loss on health Issues such as cognition and dementia should be recognized and managed

Hearing loss affects an estimated 5 percent of the world’s population, and will affect twice that number (900 million) in 30 years. The majority of these will be adults and especially the elderly because of the declining population (in the developed world) with increasing longevity.

Besides the obvious disability that hearing loss is associated with are other equally compelling health issues that are important to recognise, prevent or manage. Cognitive impairment, dementia, depression and social isolation are several of the conditions that are associated with hearing loss in the elderly.

The aim of this article is to explain the fundamentals of the ear, mechanisms of hearing and the causes of hearing loss in the elderly briefly. Thereafter, a discussion on the impact of hearing loss on cognition and dementia is provided with ways of delaying these conditions.

Anatomy

The human ear consists of three parts, the external, middle ear and inner ear. The external and middle ear are air-filled, while the inner ear lies within the substance of the side bone of the skull – the temporal bone. The ear drum (tympanic membrane) separates the external ear from the middle. The Eustachian tube connects the front of the middle ear to the back of the nose.

Besides the three smallest bones of the human body – the malleus (hammer), incus (anvil) and stapes (stirrup) – the middle ear also contains important nerves that control facial movement, taste and salivation.

The inner ear has two main parts, the bony outer part and soft (membranous) inner part. Fluid bathes these two parts and important receptors for balance and hearing are activated according to the stimuli that reach them. Similarly, the nerves of balance and hearing pass back into the skull where they connect to the brainstem and brain.

How we hear

Sound is a form of energy created by cyclical compression and rarefactions (decompression) of any medium it travels through and is transmitted as waves across different media – air being the most obvious. As the waves pass by, they enter the ear canal cause the intact ear drum to vibrate and transmit the energy to three small bones in the middle ear – a purely mechanical process that aims to increase the efficiency of sound transmission.

This mechanical process transfers to the inner ear via the flat footplate of the stapes bone into the inner ear’s membranous part. Movement of this fluid excites specialized cells with hairs on their end changing their properties and causing the movement of charged electrical atoms, thereby generating electric current that flows in the nerve of hearing to the brain.

Assessing hearing loss

Hearing is assessed in several ways. History taking and examination can provide some clues as to how poor the patient’s hearing can be. Genetic causes, medication, trauma, noise exposure and infection can all affect hearing. Simple clinical assessment in the form of free field hearing tests provide quick and fairly useful information of the hearing capacity. Tuning forks are also used to assess hearing loss and whether or not it is related to conduction of sound through the external and middle ear (air conductive hearing) or to the nerve of hearing (sensorineural hearing).

Formal hearing assessment (eg. a pure tone audiogram) is done in a soundproof room and assesses the ability to hear pure tones (pitches / frequencies) at different sound levels)

Speech audiometry is performed in cases where hearing loss is severe to profound and when the subject is not benefiting enough from hearing aids. Other tests measure the compliance of sound energy transfer from external ear to the middle.

Finally, “electrical” testing of the inner ear and brain can be done to objectively assess hearing where the other “subjective” tests cannot provide an accurate gauge of the hearing loss.

Severity of hearing loss

Sound is measured using a special scale called the decibel (dB) scale. It is a mathematically derived scale and ranges from -10dB to 120dB in most settings. If the sound level at any frequency is 20dB or less (i.e. -10dB to 20dB), it is normal. The other categories are: mild hearing loss (20dB – 40dB), moderate (40dB – 70dB), severe (70dB – 90dB) and profound – any value beyond that. The World Health Organization defines a disabling hearing loss as any hearing above 40dB in adults and 30dB in children.

The ageing ear

As we age, it may seem unsurprising that our ears age too – the nerves of hearing and balance deteriorate due to “unavoidable” wear and tear.

The difficulty however is predicting who, why and when this process affects and whether or not it is purely a function of chronology. For example, not all individuals at 80 years are profoundly deaf, while some patients present at 50 and are already showing signs of severe hearing loss.

Ageing does need not be the single critical factor in what is often attributed as “age-related” hearing loss. Reduced blood flow to the brain, genetics, medication and other neurological conditions may have  an impact on hearing.

Mild cognitive impairment and dementia

By 2050, an estimated 131.5 million patients worldwide will have dementia. Recent studies have shown that cognition is significantly poorer in individuals with untreated hearing loss.

Hearing loss has been consistently shown to be associated with increased risk of developing dementia in the western world but the link with mild cognitive decline has yet to be established given the paucity of work in this regard.

The Singapore Longitudinal Aging Study is a multi-centre project involving several key opinion leaders who have varied backgrounds including ENT surgery and Gerontology / Geriatric Medicine (ref. Heywood and Ng). This study includes nearly 2,600 subjects and a follow up of eight years and has established that like the West, the Asian population is affected equally vis-a-vis cognitive impairment in hearing impaired subjects.

Treatment options

The results of the aforementioned study suggest several treatment options. Hearing aids have been the mainstay of treatment but in cases where they are clearly unhelpful given the severity of the loss, a speech audiometry may demonstrate that the patient, despite being “aided”, is still missing out on a lot of the sound stimuli.

Only 4.3 percent of 50- to 59-year-old and 22.1 percent of those above 80 years with hearing impairment wear hearing aids in the US.

Lack of awareness, stigma, persistently high cost and lack of reimbursement by insurance companies all contribute to the poor uptake of hearing rehabilitation.

In some cases where conventional hearing aids do not help, cochlear implantation is recommended. Cochlear implants have become more accessible and are being recommended to older patients with severe to profound hearing loss that need not affect all the hearing frequencies.

Given that hearing rehabilitation may slow down the progress of dementia and cognitive decline, recommendation for hearing rehabilitation should be made.

Conclusion

Hearing loss in the elderly has ramifications beyond just day to day sound perception and conversation. The increased cognitive load required to process sound in individuals with hearing loss may direct the innate cognitive resources away from centres for memory processing to centres for speech perception.

A focused approach to the elderly with this condition is essential in promoting health, social well-being and reducing cognitive decline and dementia.

Business Times
14-15 September 2019

Childhood Hearing Loss – Often Avoidable, Preventable and Treatable

Childhood Hearing Loss – Often Avoidable, Preventable and Treatable

Hearing loss affects over 350 million people worldwide with over 30 million being children. The World Health Organisation (WHO) has issued a paper in line with the principles of the Convention on the Rights of People with Disabilities focusing on ‘improving hearing and access to communication to facilitate education and employment and fostering social inclusion and psychological well-being in people with hearing loss’. A three-prong approach comprising Prevention, Identification and Intervention has been established1. Paediatric hearing loss affects children the world over with 60% of cases being preventable. A greater number of children are affected from low-resource settings (low and middle-income groups).

This article provides several cases of children with hearing loss and aims to highlight the importance of early diagnosis and treatment that is appropriate for the stage of life, degree of hearing loss and economic factors that impact families with these children.

Case 1

History

A 4-year girl presented with repeated right ear discharge and hearing loss. She is known to snore and mouth breathes. She has a family history of asthma. She has been seen by her primary care practitioner thrice in the last year with similar complaints and treated with oral antibiotics and eardrops. Her teachers have mentioned that she is easily distracted and can be disruptive.

Diagnosis and management

Glue ear (Otitis Media with Effusion with possible perforation of the ear drum). Cases of ear discharge and hearing loss in pre-school children are very common. The vast majority of children in this age group have or will have issues pertaining to their middle ear simply due to the incomplete development of their mid-face and structures associated with it. The tube that connects the back of the nose to the middle ear (Eustachian or pharyngotympanic tube) is shorter, more horizontal and less sturdy in a young child. Lymphoid tissue similar to the tonsils at the back of the throat known as the adenoids also swell up (contributing to nasal blockage and resulting in snoring) with infections of the upper respiratory tract and increase the infective load and /or physically block the opening.

Most often, this condition if left alone for a few months or treated symptomatically resolves as the child develops. In instances where the condition recurs regularly and is affecting the speech development of the child, a specialist opinion for surgical treatment of the adenoids (adenoidectomy) and glue ear or, for hearing aids – may be necessary. Insertion of small tubes – some of which are akin to a yo-yo / dumb bell (called grommets) are inserted using a microscope by an ENT specialist to help equalize pressure across the ear drum and improve hearing and speech and in so doing, concentration and possibly other behavioural issues.

Case 2

History

A thirty-five year-old mother of two children is pregnant with her third child. She develops a rash and is unwell for a week during her first trimester of pregnancy. Her son is born slightly before term and is found to have hearing loss on the newborn hearing screen. She notices that he doesn’t respond at all to sounds.

Diagnosis and management

Congenital rubella infection. One in every 1000 children is born deaf. Infective causes of deafness are the most common causes of preventable deafness despite immunization programs worldwide. Rubella is a highly contagious viral infection which can be transmitted from mother to fetus. Other congenital infections such measles, mumps, cytomegalovirus (CMV), toxoplasmosis and herpes can cause profound deafness. There have been spikes in measles cases in Singapore and worldwide with deaths from the disease mainly due to lack of vaccination for a variety of reasons.

Congenital rubella syndrome is often picked up when the child has had its newborn hearing screening and later if it does not display the normal reactions to sounds. Infected children should be managed in a multi-disciplinary setting with specialist eye, heart and ENT care. Cataracts, congenital heart defects and sensorineural hearing loss are amongst the more common conditions associated with in utero transmission to the child. Prompt specialist audiological and ENT input with the need to provide hearing aids or other devices (cochlear implants – Figure 1) may be advised.

Case 3

History

After a protracted bout of the ‘flu’ Millie, who was only 8 years old, developed viral meningitis. She was admitted to the intensive care unit and made a slow recovery. Her hearing in both ears was badly affected. She could barely hear loud noises and only felt them as vibrations.

Diagnosis and management

Profound sensorineural hearing loss secondary to viral meningitis. She was referred to the ENT and audiology department for specialist input. A trial of hearing aids failed and she was offered cochlear implantation. Given the urgency in performing the procedure before calcification of the cochlea (similar to hardening) occurred, an operation was performed within a few weeks to help restore her hearing.

Cochlear implants have revolutionised hearing rehabilitation in both the young and old. Indications vary accordingly but age at implantation, at either extreme, is becoming less rigid. Results in children who are born congenitally deaf are very good in most cases when implantation is performed before the child should have started to verbalise (becomes lingual). In certain instances (cultural or due to financial constraints), sign language is the preferred modality of communicating. The Deaf community has its own well-established practices for signing and there are schools, libraries and other social institutions for the Deaf to engage in (i.e. Deaf Culture).

Case 4

History

John was born with multiple defects affecting the development of his eyes, ears, facial bones and teeth. His ears were badly misshapen and very small. He could barely hear and testing his hearing was difficult.

Diagnosis and management

Treacher Collins Syndrome.  This syndrome is a genetic disorder which affects approximately 1:50,000 people. Children born with this condition require hearing rehabilitation and surgery depending on the severity of the condition. In general, the external and middle ear are affected. This means that the shape and size of the outer ear, ear canal and the three small bones (malleus, incus and stapes aka hammer, anvil and stirrup) of the ear are abnormal. Reconstruction of the external can be done in stages using the patient’s own rib cartilage and shaping it into an external ear. Another option is the use of a prosthetic ear which can be designed bespoke and can be inserted using titanium screws attached to the side of the head (Figure 2). Hearing aids that attach to the side of the head onto the skull are also very helpful (Figure 3. Bone-Anchored Hearing Aids, BAHA). They transmit sound waves by vibrating the skull and thereafter transmitting the energy to the inner ear.

Summary

Paediatric hearing loss manifests in many ways. It can be mild, moderate, severe or profound. The causes vary and treatment options depend on the degree of hearing loss, the age at presentation, what structural defects are present, the resources available to the patient and potential for rehabilitation. Modern surgical techniques such as cochlear implantation have transformed hearing restoration. Better hearing aids with the capacity to filter sound, be programmed to suit the needs of the individual and provide other modern conveniences (e.g. Bluetooth) are available too. Early prevention, identification and intervention are essential in making in-roads in the treatment and rehabilitation of children with this disability.

Vyas M.N Prasad
Consultant, Ear, Nose & Throat (ENT) – Head & Neck Surgery
Singapore Medical Specialists Centre

Specialties / Sub-specialties
Otorhinolaryngology / ENT
Specialty Interests
Voice & Swallowing Disorders, Head & Neck Surgery

The Business Times, Sunday, 11 April 2019