Ear, Nose & Throat

Why does my nephew have profound hearing loss?

How should I manage nasal allergy with migraine?

What is the cause of severe itching in the ears?

How to minimise pain in the ears while air travelling?

 

Why does my nephew have profound hearing loss?

Q. My 18-month-old nephew recently underwent a BERA test. He was diagnosed with profound hearing loss due to damage to celiae in cochlea and was recommended a hearing aid. What are the reasons behind this condition? Nobody in our near and extended family suffers from hearing loss. Is there any surgical procedure to alleviate the condition? Where is a good speech therapy or hearing aid centre in Mumbai?

A. The ear has three parts - the external, the middle and the inner. The inner part can be further subdivided into the cochlea and the labyrinth. The cochlea subserves the hearing function. The labyrinth subserves the function of balance. The ciliae are brush like organs situated within both parts of the inner ear; they play a role in the process of transforming the vibratory form of sound energy into electrical energy. They are very minute and can only be seen under the electron microscope. They are very delicate and can get damaged very easily due to various reasons. Sometimes, they may be absent right from conception due to genetic causes, the mother might have had some fever or other illnesses during pregnancy; during the process of delivery, some delay or trauma might have occurred; in the new born period jaundice or some other illness might have occurred; certain drugs also might cause such damage.

The term genetic only means that the cause resides in the genes. The label genetic does not imply that one of the parents or grandparents must have manifested the same illness in them. Certain genetic changes arise unpredictably and suddenly. These sudden changes in the composition of the genes are called mutations. Several tests are now available to find out the cause, to pinpoint the site of damage and to quantify the degree of the hearing loss. However, in the present state of the art, this task is extremely difficult and also, only probabilities can be arrived at. The priorities, as far as this particular child is concerned, are as follows:
1. The first priority is to rehabilitate the child. Do a hearing level test and assess the degree of loss from the functional point of view. Fit a hearing aid. Ideally this should have been done at the age of 3-6 months. Care must be taken to fit an appropriate hearing aid. In essence, the hearing aids are amplifiers of sound energy. If the child does not hear at low volumes, these hearing aids increase the sound volume and render the hearing possible. In the case of hearing aids, expensiveness does not necessarily mean that they are either better or more appropriate. Of course, this is true of every other thing in life! Many times, a less expensive hearing aid may be the most suitable. It is the training that is given to child after fitting that marks the difference between success and failure. Rehabilitation of hearing is not a "fit and forget" proposition.
2. Sometimes, such absence of ciliae may occur in other organs also and result in other defects like defective teeth, malposition of heart and other organ systems, sperm dysmotility, absence of sweating, etc. A systematic search must be made to look for their presence. If such are present, they must be treated.
3. Advanced testing could be done to ascertain the cause, if possible. Such testing may also give an indication whether this loss will be progressive. Another benefit of such testing will be to predict the possibility of similar defect in subsequent pregnancies.
4. Cochlear implants are a recent discovery in hearing aids. Unlike the hearing aids, in this piece of equipment, a microphone collects the sounds, changes them into electrical signals and feeds these electrical signals directly into the cochlea. Thus, any defect in the ability of the cochlea to transform sound energy into electrical energy is bypassed. Cochlear implants can bypass a defective cochlea, which the hearing aids cannot achieve. The disadvantages are - they are expensive; they are still being developed. By the time you find the finance and fit the current cochlear implant, another better model might have arrived. You cannot replace the old with the new one, as it costs even more. Besides, it may be impossible because of the earlier surgery, and would be certainly more risky. The technique of operation is not very difficult. Most of the ENT surgeons in India have the skill to do similar surgery. It is the post operative audiological facilities, which are lacking in several hospitals and this is the main stumbling block. The immediate post operative training of the child takes about one year. During this period, the child and the parents will have to be under the constant supervision and guidance of the speech trainer. Otherwise, the entire exercise comes to naught. This period of support, in most cases, extend to the entire lifetime. The President of India, Shri Abdul Kalam has initiated a project to manufacture cochlear implants at an affordable price and the fruition is expected in a few years. When such implants become available, a cascade effect might occur, resulting in betterment of audiological services and speech training facilities.

The child is already receiving competent medical attention. The treating doctor can give you further details. You can request your audiologist to give you the address of a nearby institutional facility e.g. Ali Yavar Jung National Institute of Hearing and Speech.

How should I manage nasal allergy with migraine?

Q. I am 33 years old and suffering from bronchial asthma and allergic rhinitis since the age of 10. I had high eosinophil in blood during childhood. The bone in my nose is also protruded. But asthma symptoms subsided. Yet nasal allergy, sneezing, headache continue to trouble me. I also have migraine four times a month. Two years back, I took a medicine for migraine for about 5 months (sibelium tab-nasal spray) under the supervision of a neurologist. I use the Asthalin inhaler and sometimes Cetirizine tablets help. But my main problem is headache, due to nasal blockage or severe migraine with nausea. I consulted a neurophysician, who suggested that I should go for a comprehensive test like a CT scan of brain, face, chest x-ray, ECG, ESR, CBP, etc. for treatment. Kindly evaluate my case and give advice regarding next course of action.

A. Your problems are Bronchial asthma, allergic rhinitis, deviated nasal septum and probable migraine.
I am not sure if you have features of sinusitis (which at times can cause headache and needs to be differentiated from migraine). In fact, sinusitis is often diagnosed by lay persons or even by GPs in patients who have cough and cold or rhinitis (as in your case) quite often. Distinguishing headache due to sinusitis and migraine is very important because treatments are different. Migraine headaches have throbbing, pulsatile quality, usually occurs in half of head (may involve both halves at times), associated with nausea and vomiting and intolerance to bright light and loud sound. It typically increases with activity and makes the patient unable to carry on with his/her work. It usually lasts for hours.
Migraine is a primary headache (meaning that there is no structural pathology in the brain) and usually CT/ MRI is normal. However, before being sure of migraine, the physician has to exclude serious intracranial pathology by considering certain specific symptoms and / or signs (so called red flags) by careful history taking and examination. If he feels that there are indeed red flags, some investigations may be requested.
Nasal blockage can at times cause headache (usually due to acute / acute on chronic sinusitis). You need to check up with your ENT doctor. Radiological finding of sinus involvement (by X-ray, CT-sinus, MRI) is a common finding even in asymptomatic patients and always needs to be clinically correlated.
Thus, my advice will be to get an opinion from a Neurologist, ENT specialist, Chest Physician (for asthma and allergic rhinitis) and get a comprehensive strategy for tackling your problems.

What is the cause of severe itching in the ears?

Q. I have severe itching in my ears everyday. I have to use ear buds to get rid of the itching. There is no dirt in my ear as I have been using ear buds for the last one ear. The itching subsides after using ear buds. Please advise.

A. There are various reasons for itching in the ear. Fungal infection, seborrhoeic dermatitis, inflammation of external ear, wax formation or ear discharge may cause itching. Irritation or inflammation in throat and teeth also could be the cause of irritation in the ear. Consult an ENT doctor and get your ears checked.

How to minimise pain in the ears while air travelling?

Q. I work abroad in the oil industry and am a frequent air traveller. I see most of the children crying during plane landing due to the pain in the ears. I myself experience severe ear pain occasionally. Now my wife and children will be travelling by air for the first time. What is the correct technique to avoid or minimize the ear pain that may occur due to the variation of the cabin pressure while landing?

A. The pain in the ear during travelling is due to pressure difference in the middle ear and outside. It is more if you travel with nose or throat infection. The problem can be avoided by:

Staying awake especially during descent.

Repeated swallowing movements which can be facilitated by chewing sweets.

Taking proper decongestants if you are suffering from cold. See ENT specialist before journey.

If ear blockage occurs manoeuvres like wide yawning, swallowing or blowing out through nose with mouth and nose closed.