الفهرس 
IntroductionOnly 14 pages are availabe for public view
 |  | from | 145 |  |  |
| | | from | 145 | | |
Abstract
- 105 -
SUM MAR Y
Congenital sensorineural deafness forms one of the
problems that face the otologist as it is difficult to
manage and has its grave sequelae which must be prevented.
It may arise as a result of genetic disorders or teratogenic
factors. The teratogenic factors include prenatal
infections with rubella, cytomegalovirus, measles,chicken
pox, toxoplasmosis and syphilis, ototoxic drugs taken
during pregnancy such as streptomycin, kanamycin, gentamycin,
ethacrynic acid and thalidomide and perinatal
teratogenic factors such as cerebral anoxia, birth trauma,
low, birth weight and hyperbi1irubinaemia. Genetic hearing
loss represents 50 percent of all cases of sensorineural
hearing loss and this population will become increasingly
larger as other causes come under control.
Sensorineural hearing loss may occur as an isolated
anomaly where the diagnosis can be made by its mode
of transmissior.4age of onset, stability or progression
and audiometric findings or it may occur as a part of
syndromes which can be distinguished by their associated
abnormalities such as Penderd’s and Small’s syndromes.
- 106 -
For identifying dea~n~s in newborn infants; there
are two basic steps; first the application of high risk
register, and second, the actual hearing screening tests.
The medical diagnostic work up of the child with
hereditary deafness should go through, history, general
physical examination, head and neck examination, routine
laboratory tests and otoscopic examination.
Hearing screening tests are described in relation
to different age groups:
from 0 - 4 months
from 5 - 15 months
from 15 - 30 mOnths
from 30 months - 5 years
School age child.
Hearing screening tests include sound field behavioural
observation audiometery, conditional orientation reflex,
visual reinforcement audiometery, attempt identification
of body parts, and simple pictures (through air and bone
conduction). A.C., B.C. pure tones, speech detection
level, and speech discrimination level.
Electroacoustic impedance and stapedius reflex
measurements are important objective tools in the diagnosis
and have several values.
- 107 -
Laboratory tests for evaluation of congenital sensorineural
hearing loss are important especially in certain
syndromes as Hurler’s syndrome.
Radiographic investigations including plain films,
poly tomography and high resolution C.T. scanning are of
great value in evaluating a case of congenital sensorineural
deafness.
Management of a child having congenital sensorineural
deafness aims ·for the development of speech and
language which is the main problem. Several factors are
cons;ldered in management including counseling of parents
of the deaf child, sensory aids, and surgical interference.
Counseling of the p~fents of the deaf child is a very
important factor as they supposed to spend a great deal
of the day with him and can have a good part in the plan
of his management. By far hearing aids are the most beneficial
tools in speech and language development. The
early,appropriate and supervised use of a hearing aid is
the single, mostirnportant therapeutic way to assist
the hearing impaired child whose condition is not surgically
or medically correctable. Surgical interference
is restricted to a very small number of cases and it aims
only to prevent further deterioration of the level of
hearing.