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404                                          THE EAR

vii. Alporfs syndrome is found mainly in boys in whom a progressive
glomerulonephritis is associated with deafness.

viii. Refsurrfs syndrome which consists of ichthyosis, ataxia, retinitis
pigmentosa, night blindness, mental retardation and a perceptive hearing

B.  Endemic congenital deaf-mutism is associated with goitre and cretinism."
The pathological changes mainly affect the middle ear and the window niches
become narrowed or occluded and the stapes more or less immobile. In many'
cases, however, deaf-mutism probably depends mainly on the poor mental
development of the child.

C.  Maternal rubella. Children born of mothers who have suffered from
rubella during the first 3 months of pregnancy have a 35 per cent chance of
being born deaf and may also have heart defects, cataract and dental abnor-
malities. Other virus illnesses in the first trimester, e.g. glandular fever and
influenza, may have a similar effect.

D.  Other maternal diseases have been blamed for deafness in the baby,
e.g. measles, nephritis, diabetes and syphilis.

E.  Drugs taken during pregnancy. The results of thalidomide are now well
known but deafness has also been attributed to the mother being treated with
streptomycin and quinine.


Prematurity, dysmaturity, birth injuries, anoxia during delivery, prolonged
labour and especially hyperbilirubinaemia can cause deafness.


Deafness in early infancy is mainly due to infection, e.g. meningitisódue to
meningococcus, pneumococcus or tuberculosisómeasles, mumps, influenza,
etc. It may be also due to treatment of infections by drugs such as strepto-
mycin, neomycin or kanamycin.

DIAGNOSIS OF CONGENITAL DEAFNESS. If a deaf child is to make the best
use of its residual hearing, if it is to learn to speak well and if it is going to
benefit from education the diagnosis must be made at as early a stage as
possible so that suitable treatment and education may be commenced early
enough. Where any of the causative factors exist during pregnancy, at the
time of delivery or if illnesses which might cause deafness occur in infancy the
possibility of deafness must be excluded. This will require screening tests by
doctors or health visitors, which may have to be repeated on several occasions
until deafness can be eliminated with certainty in those 'At Risk' children.

Deafness may be suspected by the parents who notice that their baby does
not respond to sound. This suspicion must be treated seriously and hearing
tests applied until there is no doubt that the hearing is normal. Unfortunately,
in many cases still, deafness is not suspected until there is delay in the develop-
ment of speech. In these cases also the hearing must be thoroughly and
accurately tested.

Whenever deafness is suspected a careful history must be taken and this
will include the antenatal history as well as the nature of the confinement and
postnatal health.