Examination of hearing loss in
newborns
Hearing defect
• 6-12/1000
• 1 / 1000 has a very severe hearing loss
• Higher incidence than in phenylketonuria, congenital hypothyroidisms
Hearing defect
• congenital (genetic)
• obtained (during pregnancy, childbirth or shortly after, during the life-infection, trauma, tumor..)
Hearing defect
• prelingually (created before the end of the child's speech development, 6th year of age)
• postlingually (persons with advanced speech)
Genetic hearing loss
• several genes
(mostly gene for connexin)
• these are the different types disability of cochlea
• (Michel's, Mondini's, Scheibe's type ...)
Scheibe's dyspalsia
• Scala media disappeared
• Bony labyrinth is normal
• Dyspalsia is seen in the cochlea and saccule
• Autosomal-recesive, non syndromic
Michel's aplasia
• Complete absence of bony and membranous labyrinth
• External and middle ear may be unaffected
Mondini's dysplasia
• May be seen in many syndroms
• Only basal coil is present
Genetic hearing loss
• part of syndromes
• Alport syndrome (a kidneys)
• Uscher syndrome (an eye)
• Turner syndrome (45X)..
• incidence can be reduced
Acquired hearing defect
• Prenatal- before birth
• Perinatal- during birth
• Postnatal- after birth
Acquired hearing defect
• "Sensitive" period is around 20 day after conception
• maternal infectious diseases (rubeolla, measles, mumps, scarlet fever, flu, toxoplasmosis in the first trimester)
• ototoxic drugs, X-ray
• maternal metabolic diseases (diabetes, uncontrolled high blood pressure ..)
Acquired hearing defect
• premature birth - risk neonate
• risk increase 20-40/1000
• at birth (neonatal prematurity, Rh-incompatibility, a difficult birth, which may cause bleeding into the inner ear or to the brain centers for the hearing, asphyxia or hypoxia of the child)
• after birth (mechanical ventilation longer than 4 days, severe neonatal jaundice, severe fetal infection, meningitis)
Identifying hearing loss
• difficult, if not profound deafness
• parents perceive deviation after several months (2-3 years), delayed speech, poor pronunciation, or disobedience
Identifying hearing loss
hearing test, reaction to sound
lack of adequate response
undeveloping speech
the incidence of congenital deafness in family
followed by an objective hearing tests
Screening
Screen = sieve
search method of early forms of disease, < deviations from the norm in a population through tests
TEOAE
• In the neonatal ward
• 1st and 4th day
• quiet room, usually after feeding
• Into ear insert a small probe (transmitting sounds and records the otoacoustic emissions - the sound of the probe evoked "response" of outer hair cells in the cochlea (inner ear).
• emissions are present ("PASS"), confirmed the proper function of outer hair cells -probably is not present any hearing defect.
• emissions are absent ("REFER"), which may indicate a hearing defect.
• continual monitoring the child and his speech development
• rare and unlikely hearing disorders (disorder of the auditory nerve), which may begin to develop after birth
• hearing may be damaged in the child development
• For PASS TEOAE is needed not only proper function of outer cells in the cochlea, but also air and function of the middle ear and ear canal.
• REFER do not always mean (sensorineural) hearing loss.
• absence of emission (30%), it is appropriate to repeat the test after two days, or after a month.
Tympanometry
• Absence of emissions (sebaceous plug in the ear canal, middle ear ossicles changes, fluid in the middle ear)
• perform tympanometry-objective examination
• examination of eardrum compliance, which is dependent on the ratios of the middle ear
• for the accuracy of the TEOAE result is needed curve A.
BERA
• Repeatedly absented TEOAE, Tympanometry A
• suspected hearing loss incurred in the cochlea
• BERA (Brainstem Evoked Responses Audiometers)
• objective audiological test
• records the electrical potentials of the brain stem, which arise as a brain response to sound signals coming from the surroundings
• Headphone tones are bringing the patient to which a hearing brainstem patient responds to electrical activity (recorded by the probe attached to the patient's head)
rehabilitation of hearing
• first hearing aids (from 3 months)
• on both ears
• effect is considered ideally around the 6th -9th months of age
• inadequate compensation for hearing loss with hearing aids, cochlear implant
• „Learn to hear"
First Moravian Centre for cochlear
implantation
• 19th July 2012
• It is intended for patients with severe sensorineural hearing loss
• allow patients to understand speech
First Moravian Centre for cochlear
implantation
• It was established at the University Hospital St. Anna in Brno (adults) + FN Brno, KDORL (children)
• close cooperation with reputable implanting center in Vienna (Prof. univ. Dr. Wolf-Dietrich Baumgartner from Universitätsklinik für Hals-, Nasen-und Ohrenkrankheiten, Medizinische Universität Wien)
First Moravian Centre for cochlear
implantation
• there is no economically challenging commute
• Brno center offers quality and subsequent operative, rehabilitation needs across the spectrum
satisfaction guarantee not only adults, but also implanted children and their parents.
1st implant child