Summary
Samenvatting bijzondere pathologie van gehoor en evenwicht: complexe casuïstiek
Het onderdeel complexe casuïstiek bestaat uit connexine, TORCHeS, CMV, usher, brancio-oto-renaal (BOR), enlarged vestibular aquaduct syndroom (EVA), rotsbeenfractuur, verstandelijke beperking, auditieve neuropathie. Het bevat alle inhoud van de powerpoints en aanvullingen uit het handboek met mijn...
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Summary
Institution
Katholieke Hogeschool VIVES (VIVES)
Education
Audiologie
Course
Bijzondere pathologie van gehoor en evenwicht (B3C157)
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Samenvatting pathologie van gehoor en evenwicht
1. Summary - Samenvatting bijzondere pathologie van gehoor en evenwicht: pathologie en casuïstiek
2. Summary - Samenvatting bijzondere pathologie van gehoor en evenwicht: complexe casuïstiek
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Bijzondere pathologie van gehoor
en evenwicht: complexe casuïstiek
Inhoud
Congenitaal gehoorverlies................................................................................................................................4
Oorzaken congenitaal gehoorverlies............................................................................................................4
Connexine 26 (Cx26).....................................................................................................................................5
Wat...........................................................................................................................................................5
Handboek gehoorverlies bij kinderen p168-169.......................................................................................6
Diagnostiek...............................................................................................................................................7
Beleid........................................................................................................................................................7
TORCHeS.......................................................................................................................................................8
Wat?.........................................................................................................................................................8
Handboek gehoorverlies bij kinderen p159-163.......................................................................................9
Congenitale cytomegalovirusinfectie (cCMV).............................................................................................11
Wat.........................................................................................................................................................11
Preventie................................................................................................................................................12
Symptomen............................................................................................................................................12
Diagnostiek.............................................................................................................................................13
Beleid......................................................................................................................................................14
Usher..........................................................................................................................................................15
Wat.........................................................................................................................................................16
Types.......................................................................................................................................................16
Symptomen............................................................................................................................................17
Diagnostiek & beleid...............................................................................................................................17
Branchio-oto-renaal syndroom...................................................................................................................18
Genetisch onderzoek..............................................................................................................................20
Wat.........................................................................................................................................................20
Enlarged Vestibular Aquaduct Syndrome (EVA)..........................................................................................21
Wat.........................................................................................................................................................21
Etiologisch protocol bij congenitaal gehoorverlies.........................................................................................22
Congenitaal gehoorverlies..........................................................................................................................22
Overzicht.....................................................................................................................................................22
Vaststelling gehoorverlies...........................................................................................................................23
Neonatale gehoorscreening....................................................................................................................23
Uitgebreide audiologische diagnostiek...................................................................................................30
1
, Etiologisch protocol....................................................................................................................................31
Anamnese & klinisch onderzoek.............................................................................................................32
Geïsoleerd gehoorverlies........................................................................................................................33
Niet-geïsoleerde gehoorverlies...............................................................................................................35
Overzicht.................................................................................................................................................36
Casus.......................................................................................................................................................36
Aandachtspunten...................................................................................................................................36
Rotsbeenfractuur............................................................................................................................................38
Transversale rotsbeenfractuur...................................................................................................................39
Casus.......................................................................................................................................................39
Longitudinale rotsbeenfractuur..................................................................................................................42
Casus.......................................................................................................................................................42
Definitie......................................................................................................................................................47
Gehoor en verstandelijke beperking...............................................................................................................48
Definitie VB.................................................................................................................................................48
Impact gehoorverlies..................................................................................................................................49
Diagnostiek.................................................................................................................................................50
Anamnese...............................................................................................................................................50
Otoscopie................................................................................................................................................50
Audiologische testbatterij.......................................................................................................................50
Behandeling / Revalidatie...........................................................................................................................52
NKO-behandeling....................................................................................................................................52
Geluidsversterking..................................................................................................................................53
Optimalisatie communicatie...................................................................................................................54
Vestibulaire testbatterij..............................................................................................................................55
Testbatterij aanpassen................................................................................................................................56
Tympanometrie......................................................................................................................................56
Oto-akoestische emissies........................................................................................................................56
Brainstem Evoked Response Audiometry...............................................................................................57
Behavioural Observation Audiometry.....................................................................................................57
Visual Reinforcement Audiometry..........................................................................................................58
Conditioned Play Audiometry.................................................................................................................58
Rotatietest..............................................................................................................................................58
Calorisch onderzoek................................................................................................................................59
(Video) Hoofd Impuls Test......................................................................................................................59
Cervicaal Vestibulair Geëvokeerde Myogene Potentiaal........................................................................60
Oculair Vestibulair Geëvokeerde Myogene Potentiaal...........................................................................61
2
,Auditieve neuropathie....................................................................................................................................62
Historiek.....................................................................................................................................................62
Eerste casussen.......................................................................................................................................62
Terminologie...........................................................................................................................................62
Definitie......................................................................................................................................................63
Effect op gehoor.....................................................................................................................................63
Prevalentie..................................................................................................................................................64
Etiologie......................................................................................................................................................64
Lokalisatie probleem...............................................................................................................................64
Risicofactoren.........................................................................................................................................65
OTOF – DFNB9........................................................................................................................................66
Diagnostiek.................................................................................................................................................67
CM, ABR, OAE, reflexen..........................................................................................................................67
Uitbreiding..............................................................................................................................................69
Neonaten, kinderen, volwassenen.........................................................................................................70
Evolutie...................................................................................................................................................71
Klinische testbatterij...............................................................................................................................71
Differentiaaldiagnose..............................................................................................................................71
Revalidatie..................................................................................................................................................72
3
, Congenitaal gehoorverlies
Oorzaken congenitaal gehoorverlies
Prevalentie?
o 2-4/1000 kinderen geboren met bilateraal neurosensorieel gehoorverlies (NS GV) ≥ 40dBHL
o Vlaanderen: 1,74/1000 kinderen
Aangeboren doofheid en slechthorendheid bij 1,74 op de 1000 geteste kinderen Aangeboren
doofheid en slechthorendheid kan al op zeer jonge leeftijd worden vastgesteld. In 2019 werd 94,2%
van de kinderen geboren in Vlaanderen in de eerste levensmaanden getest door Kind en Gezin. In
2018 stellen we bij 2,18 op de 1000 door Kind en Gezin geteste kinderen een aangeboren doofheid
slechthorendheid vast. In 2019 ligt het cijfer lager (1,74).
Van de kinderen geboren in 2019 gaat het bij 72 van de 108 kinderen om gehoorverlies aan beide
oren, van wie 55 kinderen aan beide oren dezelfde graad van slechthorendheid hebben.
Bij 96 kinderen stellen we – aan 1 of beide oren – minstens een matige slechthorendheid vast (>40
decibel), bij 12 kinderen gaat het om lichte slechthorendheid (gehoorverlies van 21-40 decibel). 27
kinderen zijn minstens aan 1 oor doof
Impact
o Taal- en spraakontwikkeling
o Negatieve impact op sociaal & professioneel vlak
Classificatie gehoorverlies
o Congenitaal / prelinguaal vs postlinguaal
o Syndromaal vs geïsoleerd
o Genetisch vs verworven
4