Base of Audiology Anatomy and Physiology of the organ of hearing. Examinations of hearing losses with different origin.

Transcription

1 UNIVERSITY of SZEGED Department of Oto-Rhino- Laryngology and Head- Neck Surgery Base of Audiology Anatomy and Physiology of the organ of hearing. Examinations of hearing losses with different origin. Dr. habil. József Géza Kiss Research Professor

2 Structure of the ear I.

3 Structure of the ear II.

4 The cochlea

5 The organ of Corti I.

6 The organ of Corti II.

7 The organ of Corti III.

8 Frequency distribution inside of the cochlea topology Traveling wave Traveling wave

9 How the outer hearing cells are functioning

10 The sound Source of sound Propagation medium

11 The sound Source of sound: vibration of elastic bodies causes pressure difference (for example: tuning fork, string, vocal chords) Propagation medium: vibration of the sound source spreads as a pressure wave (longitudinal wave) Physical parameters: frequency, amplitude, phase, etc.

12 The sound are longitudinal wave

13 The spread of sound

14 Period time The sound I t[s] Wave lenght I x[m]

15 Frequency(f or ν) Periods taking place per time unit Unit: f=1/s = 1 Hz We hear different sounds with different frequencies as different pitched sounds.

16 Amplitude Intensity of sound Intensity of sound: Magnitude of sound energy passing perpendicularly through one unit of the spreading surface: [I] = 1 W/m 2 Amplitude: air-pressure change caused by vibration: [P] = 1 Pa Formula: I = P 2 /d*v d: medium density, v: spreading velocity of sound

17 The decibel scale (db) Bel: log 10 of two sound intensities: 1 Bel = 10 db (SPL = Sound Pressure Level) Base level: the human ear can sense it at 1 khz: I 0 = W/m 2, P 0 = 20 µpa Discomfort level: I ucl = 1 W/m 2, P ucl = 20 Pa Dynamic range: log 10 (10-12 ) = 120 db

18 Sound and wibration

19 Doppler-effect

20 Vibration type

21 Superposition

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23 Harmonic waves

24 The vibrations of the membranes

25 The vibrations of the eardrum

26 The vibrations of the eardrum

27 The vibrations of the eardrum

28 Vibration of the eardrum and the ossicular chain (500 Hz)

29 Vibration of the eardrum and the ossicular chain (2000 Hz)

30 Sensitivity of the ear

31 db SPL and db HL

32 Sensation of sound Loudness Phon-scale: equal loudness curves (1 khz) Son-scale: comparative loudness 40 phon = 1 son Pitch mel scale

33 Complex sounds pure tone tone noise

34 Audiometry Subjective audiometry Objective audiometry

35 Subjective audiometry Pure-tone audiometry threshold audiometry above threshold examination examination of recruitment Fowler-test SISI-test examination of adaptation Békésy-audiometry Speech-audiometry Speech-threshold examination (numbers) Speech recognition examination (words)

36 Normal audiogram (Right) Jobb (Left) Bal db Hz db Hz

37 Sensorinaural hearing loss db Hz

38 Noise-induced hearing loss db Hz

39 Presbyacusis db Hz

40 Incipient Meniere disease db Hz

41 Advanced Meniere disease db Hz

42 Conductive hearing loss db Hz

43 Otosclerosis db Hz

44 Mixed type hearing loss db Hz

45 Conductive hearing loss % számpróba numbers szópróba words db

46 Sensorineural hearing loss % számpróba numbers szópróba words db

47 Sensorineural hearing loss with discrimination loss % számpróba numbers szópróba words db

48 Sensorineural hearing loss with rollover recruitment % számpróba numbers szópróba words db

49 Objective audiometry Tympanometry Otoacoustic emission Auditory evoked potentials

50 Tympanometry Dinamic complianceexamination Statik compliance-examination Stapedius-reflex test Tuba-function test

51 Tympanometer

52 The stucture of the tympanometer

53 Acoustic impedance

54 Tympanogram

55 Multifrequency tympanometry

56 Multifrequency tympanometry

57 Multifrequency tympanometry

58 Multifrequency tympanometry LEFT

59 Multifrequency tympanometry RIGHT

60 Multifrequency tympanometry

61 Stapedius reflex ipsilateral contralateral

62 Reflex decay

63 ARLT ipsilateral contralateral

64 Otoacoustic emission Spontaneous otoacoustic emission (SOAE) Evoked otoacoustic emission (EOAE) transient EOAE (TEOAE) stimulus frequency emission (SPOAE) distortion product otoacoustic emission (DPOAE)

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66 Spontaneous otoacoustic emission

67 Transient otoacoustic emission

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69 Distortion product otoacoustic emission

70 F1 M F2

71 Distortion product otoacoustic emission

72 Distortion product otoacoustic emission (F 1 *F 2 ) 1/2

73 Auditory evoked potentials Cochlear evoked potentials Brainstem evoked potentials Cortical evoked potentials

74 Auditory evoked potentials Electrocochleographia (ECoG) Brainstem Evoked Response Audiometry (BERA) Cortical Evoked Response Audiometry (CERA)

75 Auditory evoked potentials Early evoked potentials (ECog, BERA) Middle latency responses(mlr) Late evoked potentials (CERA, SVR) Contingent negative variation (CNV)

76 Auditory evoked potentials transient potentials (ON) sustained potentials cognitive potentials

77 Averager technic without stimulus with stimulus

78 BERA examination Signal generator GAIN Display / Printer A/D converter, averaging GAIN PRE- GAIN

79 BERA examination

80 Condensation, rarefaction

81 The type of the stimuli

82 The setup of BERA examination

83 The setup of the stimulus

84 The parameters of the BERA waves

85 The localisation of the BERA waves

86 The localisation of the BERA waves

87 The ear-lobe electrode

88 Amplitude 4 electrode, ipsilateralis Amplitúdó [nv] Latency Latencia [ms] L.I. L.II. L.III. L.IV. L.V.

89 Amplitude [nv] 4 elektrode, contralateralis Amplitúdó [nv] L.I. L.II. L.III. L.IV. L.V Latency Latencia [ms]

90 Amplitude [nv] 3 electrode Amplitúdó [nv] L.I. L.II. L.III. L.IV/V Latency Latencia [ms]

91 BERA waves in case of different stimulus rate (normal) 64 Hz 52 Hz 40 Hz 28 Hz 16 Hz

92 BERA waves in case of different stimulus rate (pathological) 64 Hz 52 Hz 40 Hz 28 Hz 16 Hz

93 Acoustic neurinoma

94 Acoustic neurinoma

95 Cochlear laesio

96 Cochlear laesio

97 Cochlear laesio

98 Cochlear laesio

99 Cochlear laesio

100 Cochlear disturbance

101 Cochlear disturbance

102 Cochlear disturbance

103 Cochlear disturbance

104 Cochlear disturbance

105 Brainstem disturbance

106 Brainstem disturbance

107 Brainstem disturbance

108 Brainstem disturbance

109 Brainstem disturbance

110 ASSR: Auditory Stady-State Response

111 Superpozition

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118 The Cortical Auditory Evoked Potential (CAEP) HearLAB Cortical Evoked Response System The end of the road Auditory cortex orientation NAL: Dillon, Van Dun, Carter, Gardner-Berry NAL: Dillon, Van Dun, Carter, Gardner-Berry

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120 CAEP The main components of cortical evoked potential are the P1-N1-P2 complex. As we know the P1 wave represented in the deepest layer IV of the neurons activity in the secondary auditory cortex. The N1-P1 complex represented neurons activity in the other upper layer (primary and secondary auditory cortex).

121 CAEP Infants Adult P2 10 µv 5 P µv P1 N1 0-5 N ms NAL: Dillon, Van Dun, Carter, Gardner-Berry NAL: Dillon, Van Dun, Carter, Gardner-Berry

122 NAL: Dillon, Van Dun, Carter, Gardner-Berry

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125 NAL: Dillon, Van Dun, Carter, Gardner-Berry

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127 Hearing genetics Deaf children 1/1000 birth Assessing the quality of hearing is a demanding task with infants and in childhood. Unknown 25 % Acquired 25% Genetic origin 50% Yet it it is very important as the earlier we begin with the rehabilitation the better are the results (social integration, etc). Autosomal recessive 75-85% Nonsyndromic 70% Autosomal dominant 15-25% Syndromic 30% X-linked 1-2% HELP: Molecular biology!! 35 delg 50% Other recessive 50% GVOP /3.0 GENE PROTEIN GENE PROTEIN GJB2 BETA-2 GAP JUNCTION PROTEIN GJB6 BETA-6 GAP JUNCTION PROTEIN 12SrRNA Sensitivity to ototoxic drugs KCNQ4 K + transporter protein COCH cochlin SLC26A4 pendrin (anion transporter) GJA1 ALFA-1 GAP JUNCTION PROTEIN POU3F4 transcription factor 4 GJB3 BETA-3 GAP JUNCTION PROTEIN MYO6 unconventional myosin-6

128 Dried Blood Spots & dhplc SIBLINGS! SrRNA (TRNS1-TRND) Right Left Right Left SrRNA (TRNS1-TRND) & KCNQ4