Otosclerosis



What is Otosclerosis?

This is a progressive disease that often commences in early adult life, particularly in females, producing advancing deafness, accompanied by tinnitus (ringing in the ears) and occasionally giddiness. There is a tendency, apparently, for this problem to run in families, transmitted by the female to her daughters. The condition may proceed to almost total deafness, but often sensori-neural deafness may also supervene.

Otosclerosis Symptoms

Deafness is the main symptom. It progresses until it may be nearly complete. The patient tends to speak in a quiet voice. Many find they can hear better in a noisy environment. The disorder is caused by spongy bone being laid down around the oval window of the middle ear, fixing the stapes, one of the vital bones required for normal movements in hearing. It is uncertain what produces this, but a genetic factor is definitely present.
Some of the points to look for with little ones include whether there is a history of “risk” factors with the child (eg did the mother have rubella during early months of pregnancy?); suspicion by the mother 54 of the child being deaf; and other defects in the child that may have resulted from the risks mentioned.
The child may also not appear to respond to sound in daily activities (eg failure to arouse when noise is present when very young; failure to answer to its own name; failure to vary the tone of speaking as it becomes older; strident sounds for certain, simple vowel sounds; retardation in speech and language development etc).
Some of these factors may be evident as early as three months of age. They will gradually become more apparent. Hearing is as important to speech as sight is to reading. It is imperative that a child suspected of having a hearing disability be referred to a special centre where it can be thoroughly and expertly checked promptly.
In older people, gradual loss of hearing is part of the aging process. Some note it re severely than others. Conversely, many older people have remarkable raring acuity well into old age. Nevertheless, in this electronic era, much can be done for many people with distress of hearing loss.
Sudden reduction of hearing may be caused by blockage of the outer ear canals by and debris, and foreign bodies can produce a sudden mechanical deafness to normal sound. Hearing diminution is sometimes associated with a ringing sensation in one or both ears. This is called “tinnitus.” It can be very disturbing in older people.
This is of particular importance in small children. Children anywhere from age of three months onwards should alert parents to recognize abnormal hearing. Immediately there is any suspicion of this, the child must be on to an appropriate place where diagnosis and therapy may be initiated without further delay.



Otosclerosis Treatment

There was no satisfactory treatment until the advent of the operating microscope and perfection of a microsurgical technique that has revolutionized procedures. This is in the province of the ENT surgeon, who has specialised in this form of surgery. Carried out by a trained surgeon, it is not a serious procedure.
There are several forms of the operation, but removal of the damaged footplate bone and replacing this with a suitable prosthesis is the final outcome. This restores the normal continuity of the growth and can result in a near-normal upbringing, but if it is missed, it can retard them and remain a tremendous drawback that may have a permanent detrimental effect on their physical and mental welfare.
There are many deaf children. Before the advent and widespread use of vaccination for rubella (German measles), many women sustained the disease during pregnancy. They subsequently produced deaf (completely or partially deaf) children. Now vaccination of women is more readily available, this cause of juvenile deafness should commence to reduce in the near future. Past epidemics resulted subsequently in enormous numbers of deaf children.

Infants and children

These should be taken immediately the family doctor who will most probably refer the patient to an Ear, Nose and -oat (ENT) specialist for further pinion and tests on hearing. The vast majority of these children in Australia attend a branch of the Cornnwealth Acoustic Laboratories. Children may be taken there directly, and no formal referrals are required. These clinics are situated in all State capitals and in many larger provincial towns. Tests are carried out, and if the patient requires hearing aids, these are supplied free of charge. They are subsequently maintained and serviced without charge to the child or the parents, and no means test is applied. Almost the entire deaf child population of Australia is catered for by these laboratories.
Approximately two children for every 1,000 born require hearing aids. These are generally fitted around the age of 18 months. But this age is decreasing with an earlier awareness of the child’s defect. Many are now fitted in the 3-6 month age group.



Older persons

As soon as there is an awareness of a hearing loss, medical assistance should be sought. Your own family doctor is usually the best starting point. He can carry out a preliminary assessment and check if there is any local cause. If there is, this will be rectified (eg syringing ears or removing foreign matter or objects). Alternatively, he may refer the patient to an ENT specialist for further investigation. The suitability of a hearing aid can be assessed. This may be carried out in conjunction with the laboratories already mentioned.
An assessment may also be made as to whether other medical disorders are producing some symptoms. Noises in the head, ringing bells, tinnitus etc may have other reasons, and these must be checked out. Suitable measures to correct these should be taken. Often “tinnitus” has no curable cause. The person must learn to “live with it.”