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date: 22 September 2019

(p. 437) Index

(p. 437) Index

10 nm particle formation, prestin 207
acetylcholine
activation of synaptic currents 292–4, 293
effect on hair cells 206, 291–2, 297–8
as efferent neurotransmitter 286–7, 290
acoustic brainstem response (ABR) 21–2, 100, 132
applications 132
newborn screening 379
acoustic cochleography 104–5
acoustic coupling 59, 60
acoustic–electric model, external and middle ear function 54–5
acoustic impedance mismatch 2, 4
acoustic neuromas, ABR testing 22
acoustic reflex (stapedius muscle reflex) 18, 20–1, 52
AC potentials 103
actin filaments, hair cells 180, 182, 238–9, 240, 241, 250
‘active’ cochlear model 154–7, 159–61
active impedance component 156
adaptation
afferent synapses 273–4, 275–6
mechanotransduction 236–8
nerve fibres 260
outer hair cells 162
adaptive directional microphones 414
addition of signals 140
adherens junction (fasciae occludentes) 310
adult stem cell therapy 369
afferent innervation patterns, development 337–8
afferent neurons, spontaneous activity 344, 345
afferent synapses 259
adaptation 273–4, 275–6
genetics 276–7
minimum signal for transmission 274–5
postsynaptic mechanisms
EPSPs and EPSCs 271–3
neurotransmitter clearance 273
presynaptic mechanisms 266
endocytosis 270
imaging synaptic release 270–1
ionic currents and neurotransmitter release 266–8
vesicle exocytosis 268–70
type II 261
unsolved problems 278
age-related changes 73
age-related hearing loss see presbycusis
air–bone gap 16, 74
air conduction testing 15–16
alleles 382
α9 nicotinic subunit 299–300
role in inner ear development 341
α10 subunit 206
Alport’s syndrome 382
Ames waltzer mice 392
amiloride sensitivity, MET channels 247
aminoglycosides, ototoxicity 26–9
AMPA receptors 273
amphibians, new hair cell production 356
amplification, hearing aids 410–12
ampullae 214
analog hearing aids 414
analysis into sinusoidal components 140
anion counterporter model, outer hair cells 192
anions, role in electromotility 202–4
anion shuttle model, outer hair cells 192
ankle links, stereocilia 244–5
annular ligament 52
anterior ligament of the malleus 52, 53
antidepressants, effect on tinnitus 37
APEX (Arrayed Primer Extension) 399
apical cochlea, non-linearity 152
apical end, outer hair cells 180–1
apoptosis 357–60
timeline of events 361
aquaporins 223, 313
arbitration phase, apoptosis 357
area change determination, membrane motor 194
area ratio, tympanic membrane and stapes footplate 60, 105
artefact reduction, cochlear response measurement 128–30
ASICs (acid-sensing channels), stereocilia 247
aspirin (acetylsalicylic acid/ ASA), ototoxicity 26
assistive listening devices 415
assistive training 417
atelectasis 50
ATOH1 335
transfection studies 365, 366
Atoh1 expression
direct transdifferentiation 361, 362, 363
mitotic regeneration 363
Atonal1, role in inner ear development 332, 334
ATP, as a signalling agent 318
ATP-dependent activity during development 320
audiograms 16, 17
patterns of hearing loss 16, 19–20
auditory brain stem implants 431
auditory cortex, role in tinnitus 36–7
auditory evoked brainstem response see acoustic brainstem response (ABR)
auditory filter 96
bandwidth 115
auditory nerve response 98–100, 259–60
auditory neuropathy
cochlear implantation 423
non-syndromic causes 384
(p. 438) auditory responses 97
auditory skills assessment, cochlear implantation 423
autoimmune inner ear disorders 32–3
diagnostic evaluation 32
sudden hearing loss 34
autosomal dominant inheritance 381, 384
pedigree 380
autosomal recessive inheritance 381, 382, 384
connexin hearing loss 384–5
linkage analysis 389–90
pedigree 380
axial displacement, OHCs, reciprocal relationship 186, 187
axial stiffness, outer hair cells 184–5, 205
effect of acetylcholine 206
voltage dependence 197–9
azithromycin, ototoxicity 29
babies, hearing screening 106, 110, 133
pass criteria 131
background noise, impact on hearing aid use 409–10, 416
Baha implantable system 417–20
band analysis, OAE screening 130
bandwidth 115
BAPTA studies, tip links 246–7
Barhl1, role in inner ear development 335
barrier function, cochlear tight junctions 310
basal end, outer hair cells 181
basilar membrane 4, 5, 93, 94, 330
basic model 153–4
effect of efferent activity 295
frequency discrimination 95
impedance 141, 154–6, 155
micromechanics 161–2
non-linearity 157–61
resonance 114
stiffness 154
bassoon 263
Bassoon 276
Bcl-2 proteins, role in apoptosis 357
behind-the-ear hearing aids 415
Békésy, G von 61, 76, 143, 144–5
best frequencies 144
bifurcation, tip links 242
bilateral cochlear implantation 430–1
bilateral hearing aids 417
birds, hair cell regeneration 356–60
supporting cell responses 360–5
BK channel 266
blood-labyrinthine barrier 32
‘blowing’ tinnitus 35
body noise minimization, OAE measurement 129
Boltzmann function 183, 191
bone anchored hearing devices 417–20
bone conduction hearing, mechanisms 77
bone conduction sensitivity, superior canal dehiscence 76–7
bone conduction testing 15
bone morphogenetic proteins, role in inner ear development 331, 332
brain-derived neurotrophic factor (BDNF), role in inner ear development 340
brainstem auditory evoked response (BAEP) 21–2
branchio–oto–renal syndrome 386
BrdU labelling, hair cell regeneration 363
BSND1 mutations 395
bumetanide, ototoxicity 219
Ca2+ absorption 217, 221–2
cadherins 23 241
in lateral links 244
role in development 336
in tip links 244, 250
calbindins 221
location 249
calcitonin gene-related peptide (CGRP) 288–9, 290
studies in genetically modified mice 301
calcium channels, hair cells 267, 274
calcium dependence, hair cells 8
MET adaptation 237–8
calcium handling mechanisms, hair bundle 249–51
calcium-induced calcium release (CICR) 294
calcium influx, stereocilia 247
calcium stores, hair cells 294, 295
calcium wave activity
damage signalling 320
in developing cochlear 321
calibration, OAE measurement systems 126–7
calmodulin 244, 248, 249–50
calretinin 250
cancellation experiments 56–7
capacitance measurements, afferent synapses 264–6, 270–1
carboplatin, ototoxicity 31
cardiac bypass surgery, hearing loss 34
caspases, role in apoptosis 357, 359
cation absorption 223
Cav1.3 276, 277
cavum minor 58
CDH23 mutations 386
cell body, OHCs 180
cerebellopontine angle surgery, hearing loss 34
cerebrospinal fluid, composition 215
characteristic frequencies, nerve fibres 259
charge transfer, electromotility 183, 184
chemically induced mutations, mouse models 392–3
children, cochlear implantation 422, 423
results 429–30
chlorpromazine, effect on electromotility 205
cholesterol, relationship to prestin dimerization 207
choline acetyltransferase (ChAT) 286
cholinergic receptors, hair cells 299–301
Chrna9 and Chrna10 300–1
cisplatin, ototoxicity 26, 31
Cl- concentration, role in electromotility 181, 202–4, 203
Cl- secretion 222
clarithromycin, ototoxicity 29
claudins 219, 310
CLDN11 loss 220
Claudius’ cells 308
CLCNKA mutations 395
CLCNKB mutations 395
CLIC5 242
(p. 439) click evoked otoacoustic emissions (CEOAEs) 108, 110, 165–6
click stimuli
CAP thresholds 99
intensity 127
CNG (cyclic-nucleotide-gated) channels 247
COCH, genetic studies 390, 391, 396–7
cochlea 2, 7, 94, 214
‘active’ modelling 154–7, 159–61
basic modelling 152–4, 153
computed tomography 23, 423
development
afferent innervation patterns 337–8
efferent innervation patterns 338
gross differentiation 329–34
hair cell differentiation 334–5
hair cell maturation 342–7
neurons 338–9
onset of hearing 341–2
regulation of hair cell survival, number and patterning 335–6
role of growth factors 339–41
stereocilia 336
tectorial membrane 337
frequency selection 95
magnetic resonance imaging 23, 24, 423, 425
ossification 425, 426
cochlear amplification 93, 114–15, 156–7, 206
role of OHC motility 162
role of stereocilia 162
cochlear compressibility 56–7
cochlear dysgenesis 425
cochlear ‘echo’ 112
cochlear efferent effect, TEOAEs 113
cochlear electrophysiology 142–3
cochlear fluid, incompressibility 55, 105
cochlear function changes, detection by OAEs 133
cochlear hydrops 223
audiometric pattern 16
cochlear implantation 420–1
bilateral 430–1
candidacy 421–5
complications
device failure 426
infections 426–7
device programming 427
measures of neural response 427–8
MRI 423, 425
results in adults 428–9
results in children 429–30
surgery 425–6
cochlear input impedance 54
cochlear mechanics 139, 142
frequency selectivity 147–8
frequency-to-place transformation 145–6
measurement methods 143–4
von Békésy’s work 144–5
non-linearity 148–52
cochlear microphonic (CM) 100–2, 120, 143, 202
development 342
distortions 122
cochlear partition 4, 5, 6, 139
cochlear pattern 146
cochlear reflex 113
cochlear responses 97–8
applications 132–3
auditory nerve response 98–100
electrocochleography 103–4
noise reduction 128–9
signal enhancement 129–31
summating potential 102–3
cochlear tuning, efferent inhibition 295–6, 297
cochleovestibular ganglion (CVG) development 338
coherent reflection 116, 166–8
combination tones (CTs, intermodulation distortions) 117–19, 118, 142
complex numbers 141
compound action potential (CAP) 98–100, 101
CAP click threshold 99
comparison with cochlear microphonic response 102
diagnostic use 103–4
response averaging 129
compression technologies, hearing aids 410–12
compression waves 152
compressive non-linearity 119, 148, 150, 151–2, 158, 159
computed tomography (CT) 22, 23
concha 49, 51
conchal resonances 63
conductive hearing loss 4, 16, 74
causes 74–7
middle ear fluid 74
ossicular disorders 74
superior canal dehiscence 75–7
tympanic membrane perforation 74–5
congenital hearing loss, epidemiology 24–5
connexin expression, supporting cells 316–17
connexin gene mutations 6, 395
connexin-mediated deafness 220, 384–5
contralateral routing of signals (CROS) amplification 417
contralateral suppression, TEOAEs 113
‘cookie-bite’ deformity 16, 19–20
coupling coefficient, piezoelectric materials 197
cross-linkages, stereocilia 240, 242–5, 243
CRYM, genetic studies 390
CtBP1 263
cupula 214
curvature motor model 204–5
cutaneous–implant interface, Baha system 418–19
cuticular plate, cytoskeleton 238–42
‘Cys-loop’ receptors 299
cytochrome c, role in apoptosis 357, 359
cytoskeleton
hair cell apex 238–42
outer hair cells 199–200
supporting cells 315, 316
damage signalling 320, 322–3
DC potentials 103
Deaf culture 400
deafness genes 11, 394–6
(p. 440) Deiters’ cells 165, 180, 182, 214, 307–8
connexin expression 316, 317
epithelial repair 313
innervation 314
ion channels 319
K+ buffering 216
structural roles 314
Delta 1, role in inner ear development 332, 334–5
depolarization, hair cells 266–7
depression, association with tinnitus 37
development 10–11, 329
afferent innervation patterns 337–8
afferent vesicle exocytosis 278
efferent innervation patterns 290–1, 338
gross differentiation 329–34
hair cell differentiation 334–5
hair cell maturation 342–7
neurons 338–9
onset of hearing 341–2
regulation of hair cell survival, number and patterning 335–6
role of growth factors 339–41
role of purinergic receptors 318–19
role of supporting cells 319–20
stereocilia 336
supporting cells 308–9, 315
tectorial membrane 337
device failure, cochlear implantation 426
DFMO, ototoxicity 31–2
DFNA5 protein 397
diamide, effect on OHC axial stiffness 205
difference tone 118
difluoromethylornithine (DMFO), endocochlear potential inhibition 220
digital hearing aids 414
dimerization, prestin 207
dipyridamole, effect on electromotility 205
directionality
external ear gain 62–3
head-related transfer function (HRTF) 63–4
directional microphones 412–14, 413
direct pressure stimulation hypothesis 56
direct transdifferentiation 356, 360–2
delayed myosin VI expression 363–4
induction by ATOH1 transfection 366
timeline of events 361
discordant drum theory, tympanic membrane 78
distortion product otoacoustic emissions (DPOAEs) 4, 166, 168
applications 132
efferent inhibition 297–8
generation 119–22
isolation and validation 127–8
measurement 122–6
pass criteria 131
signal-to-noise ratio 131
distortion products (DPs) 141–2, 158–9, 160
DNA sequencing 398
dopamine, as efferent neurotransmitter 288
dorsal cochlear nucleus (DCN), role in tinnitus 36–7
downsloping audiometric pattern 16, 19–20
DP-grams 123, 124–6
DPOE threshold 124–6, 125
DP threshold 124
dynamic range problem 267–8
dynamic visual acuity testing 29
dynorphins 288, 289
ear canal 49, 50, 51
acoustic reflectance measurements 132–3
sound pressure variations 70–2, 71
ear canal closure, OAE measurement 126, 129
Early Hearing Detection and Intervention (EHDI) mandates 379
EDN3 mutations 385
EDNRB mutations 385
efferent innervation 283–6, 301
development 290–1, 338, 347
effects on cochlear output 294–5
DPOAE suppression 297–8
inhibition of cochlear tuning 295–6, 297
time course of inhibition 296–7, 298
hair cell receptors 299–301
hair cell responses 291–2
effect on internal calcium stores 294, 295
synaptic currents 292, 293, 294
efferent neurotransmitters
acetylcholine 286–7
co-localization within neurons 289–90
dopamine 288
GABA 287–8
neuropeptides 288–9
efferent stimulation, role in electromotility 205–6
efferent synaptic structure 285
elastase studies, tip links 246–7
elastic displacement, outer hair cells 189, 191
elderly people, cochlear implantation 428
electrical stimulation, organ of Corti 162–3
electroacoustic hearing 428–9
electrocochleography (ECOG) 101, 103–4
electromotility 8, 10, 179, 209
axial stiffness and force production 184–5
charge transfer 183, 184
and efferent inhibition 295
frequency response 187–9, 190, 200
length changes 183, 184
modeling 189
cell geometry and membrane tension 189, 191
membrane motor, incorporation into cell 193–6
membrane motor models 191–3, 192
piezoelectric description 196–200
molecular mechanism and regulation
lipid environment 204–5
significance of anions 202–4
non-linear capacitance 183
organ of Corti displacement 201
RC time constant problem 201–2
reciprocal effects 186–7
role of Cl concentration 181
role of efferent stimulation 205–6
see also prestin
embryology 10–11
see also development
embryonic stem cell therapy 369
ENaC 220
(p. 441) endocochlear potential (EP) 6, 93, 180
and deafness genes 395
generation 218, 219
loss of 219–20
maintenance 310
modulation 100–1
postnatal development 342
summating potential 102–3
endocytosis, inner hair cell synapses 270
endolymph 6, 180
composition 213, 215, 221
postnatal maturation 341–2
volume 223
endolymphatic duct 214
endolymphatic hydrops 6
endolymphatic sac 214
immune responses 32
enkephalins 288, 289
enlarged vestibular aqueduct syndrome 77
ENU mutagenesis project 393
environmental causes of hearing loss 378
environmental noise minimization, OAE measurement 129
Ephrins, role in inner ear development 339, 341
epithelial cells 213, 214
apoptosis 357
ion transport 215, 217
epithelial development 332–4
hair cell differentiation 334–5
epithelial repair 313–14
erythromycin, ototoxicity 29–30
espins 240, 241
role in development 336
ESRRB2 mutations 395
N-ethyl-N nitrosourea (ENU), use as mutagen 392–3
eustachian tube 50
effect of tensor tympani 53
eustachian tube dysfunction, tympanometry 18, 20
evoked otoacoustic emissions see stimulated otoacoustic emissions
excitability, maturation of hair cells 344–6
excitatory postsynaptic potentials (EPSPs) 264, 271–2
excitotoxicity 266, 288
execution phase, apoptosis 357, 359
exocytosis 263–4, 268–70
development 278
measurement techniques 264–6, 265
external ear 2, 49–50, 51
functional interdependence with middle ear 54–5
functions 61–3
external ear gain 62
extracellular linkages, stereocilia 242–5, 243
extracellular matrix molecules, role in inner ear development 340
extracellular regulated kinases 1 and 2 (ERK1/2)
damage-induced activation 322
role in hair cell death 313–14
extrusion of hair cells 357, 359
EYA1 mutations 386
fasciae occludentes (adherens junction) 310
fast adaptation 179
FBXO11 mutations 393
fence function, tight junctions 213, 215
fibroblast growth factors (FGFs), role in inner ear development 330, 332, 336
filtering action of cochlea 151–2
finite-element models (FEMs), middle ear 73
fish, new hair cell production 356
fitting procedures, hearing aids 415–16
flat spectrum stimuli 149
fluid homeostasis 223–4
Ca2+ absorption 221–2
cation absorption 223
Cl secretion 222
endolymph volume 223
K+ buffering 216
K+ cycling 215–16
K+ secretion 218–19
Na+ absorption 220–1
pH regulation 222–3
FM dyes, in study of vesicle dynamics 266, 270, 271, 272
force production, outer hair cells 184–5
Fourier analysis 141
FOXI1 mutations 387
frequency analysis, otoacoustic emissions 127–8
frequency dependence, middle ear 61
frequency discrimination 97
frequency dispersion 115
TEOAEs 110, 111
frequency places 96, 114–15
frequency place specificity, TEOAEs 112
frequency ratios, DPOAEs 121–2
frequency resolution 97
frequency response 141, 146
electromotility 187–9, 190, 200
hearing aids 414
frequency selectivity 147–8, 168–9
development 342
frequency sensitivity 4, 95, 96–7
frequency-specific pass criteria, OAEs 131
frequency spectrum, click TEOAE responses 108, 112–13
frequency threshold curves (FTCs) 147–8
frequency-to-place transformation 145, 146
FSP method 130
furosemide, ototoxicity 30–1, 219
fusion, synaptic vesicles 268–70
GABA
as efferent neurotransmitter 287–8, 290
studies in genetically modified mice 301
gain, hearing aids 410–11
gap junctions
epithelial 310–11, 312, 315–17
stria vascularis 219
GATA transcription factors, role in inner ear development 338–9
gate function, tight junctions 213
gating, OAE measurement 128
gating-spring hypothesis, mechanotransduction 235–6, 242, 245–6
(p. 442) gender differences, congenital hearing loss 24
GeneChip 399
genes, naming conventions 382
GeneTests website 399
gene therapy 366, 368
genetic counseling 399–400
genetic hearing loss 377
age-related 388–9
incidence 377–8
inheritance patterns 380
mitochondrial mutations 387–8
mutations affecting endocochlear potential 395
non-syndromic 382–4
connexin hearing loss 384–5
syndromic 382, 385
branchio-oto-renal syndrome 386
Pendred’s syndrome 387
Usher’s syndrome 386–7
Waardenburg’s syndrome 385
unknowns 396–7
genetics 11–12
afferent synapse 276–7
modes of inheritance 381
genetic studies
genome-wide association studies 394
linkage analysis 389–90
mouse models 390
chemically induced mutations 392–3
spontaneously occurring mutations 391–2
targeted mutations 393–4
reverse molecular genetics 390
genetic testing 397–9
and Deaf culture 400
genome-wide association studies (GWAS) 394
gentamicin, ototoxicity 27, 28–9
gentamicin-induced apoptosis 357–60, 361
Gfi1, role in inner ear development 335
GJB2 mutations 384–5
molecular genetic testing 397–8
GJB6 220
GJB6 mutations 385
GLAST (glutamate-aspartate transporter) 273, 311–12
glia cell line-derived neurotrophic factor (GDNF), role in inner ear development 339, 340
glucose uptake, outer hair cells 181
GLUT5 181
glutamate, clearance from synaptic cleft 273
glutamate decarboxylase (GAD) 287, 288
greater epithelial ridge 333
GRHL2, association studies 394
group delay 141
growth factors, role in inner ear development 339–41
GTTR studies, avian hair cells 358–60
hair bundles
calcium handling mechanisms 249–51
development 240–1
reactive motions 236
hair bundle stiffness 235, 236
hair cell apex, cytoskeleton 238–42
hair cell death, role of supporting cells 313–14
‘hair cell precursors’ 364
hair cell regeneration
birds, stimulation by hair cell loss 356
definition 355–6
in embryonic mammalian cochlea 365–6
future direction 369
gene therapy 366, 368
genetic manipulation studies 366
stem cell therapy 368–9, 370
supporting cell responses 360–5
vertebrates 355–6
hair cells 231–2
afferent innervation 260, 261, 337–8
apoptosis 357–60
cholinergic receptors 299–301
development 11, 333
differentiation 334–5
effects of aminoglycosides 27–8
efferent inhibition 291–2, 294–5, 296
efferent innervation 283–6, 284, 301, 337, 338
innervation and functional differentiation 8–10
internal calcium stores, ACh response 294, 295
K+ cycling 215–16
maturation 342
of efferent innervation 347
of excitability 344–6
onset of mechanotransduction 343–4
spontaneous activity of afferent neurons 344, 345
minimum signal for transmission 274–5
regeneration 10, 11, 323
regulation of survival, number and patterning 335–6
response to trauma 313
stereocilia development 336
structure and function 6, 8
synaptic currents 292, 293, 294
harmonics 141–2
harmonin 241, 244, 395, 396
in tip links 244
head-related transfer function (HRTF) 63–4
head thrust maneuver 29
hearing aids 409
amplifier and compression technology 410–12
analog versus digital technology 414
assessment of benefits 416
binaural fitting 416–17
bone anchored devices 417–20
combination with cochlear implants 428
contralateral routing of signals amplification 417
fitting procedures 415–16
microphone technology 412–14
multiple channels 414
perceptual issues 409–10
reverberation suppression 416
telecoils 414–15
hearing assessment 15
acoustic brainstem response testing 21–2
application of auditory responses 132–3
immitance testing 18, 20–1
pure tone audiometry 15–16
speech audiometry 16–18
hearing loss (p. 443)
causes 11–12, 103
determination of cause 379–80, 382
genetic testing 397–9
incidence 377–8
inheritance patterns 380
patterns 16, 19–20
severity 16, 18
TEOAE loss 112, 113
hearing screening 132
babies 106, 110, 133, 379
pass criteria 131
helicotrema 139
Helmholtz, HL von 61
Hensen’s cells 308
connexin expression 316, 317
innervation 314
ion channels 319
Hensen’s stripe 232
Hereditary Hearing loss Homepage 382
Hes1, Hes5, role in inner ear development 332, 335
heteroplasmy 381, 387
high-frequency bias, CAP click thresholds 99
high frequency motion
ossicles 68–70, 69, 78
tympanic membrane 78
high-frequency sound specification 70–2
hinge point, organ of Corti 233
homeostasis
barrier function and adherence 310
gap junctions 310–11
K+ and neurotransmitter clearance 311–13
osmotic and pH balance 313
homogenization approximation, OHC membrane 193
homologous synteny 391
Hopf bifurcation 160–1
human studies, middle ear function 72–3
hydrodynamics 152
hydrops 223
audiometric pattern 16
hyperpolarization, outer hair cells 9
hypothyroidism
association with Pax8 deficiency 277
effect on inner ear development 335–6, 337
ideal fluids 152
idiopathic sudden sensorineural hearing loss (ISSHL) 33–5
imaging, prior to cochlear implantation 423–5, 424
immitance testing 18, 20–1
impedance, basilar membrane 141, 154–6, 155
impedance matching 105
incompressibility, inner ear fluids 55, 105
incudomalleal joint 67
effect on high frequency middle ear response 78
incus 2, 3, 52, 53
motions 67, 68
inheritance
modes of 381
inheritance patterns of hearing loss 380
inhibitory postsynaptic potentials (IPSPs) 291–2
initiation phase, apoptosis 357
inner border cells 307
inner ear 2, 4, 5, 214
cochlear partition 4, 6
hair cell innervation and functional differentiation 8–10
hair cell structure and function 6, 8
inner ear fluids, incompressibility 55, 105
inner hair cells 95–6, 214, 231–2, 233, 307, 330
afferent innervation 260, 261, 337–8
depolarization 266–8
development 333, 334
efferent inhibition 295, 296
efferent innervation 283–5, 284, 301, 337, 338
development 290–1, 347
endocytosis 270
excitation 161
frequency sensitivity 96–7
vesicle exocytosis 268–70
see also hair cells
inner phalangeal cells 307
inner sulcus 214, 330
development 333
inner sulcus cells 307
interdental cells 214
intermodulation distortions (IMDs) 117–19, 118, 142
in-the-ear hearing aids 415
intratympanic steroids, in idiopathic sudden sensorineural hearing loss 34–5
inverse-solution technique, cochlear modelling 154–7, 155
ion channels, supporting cells 319
ion transport, epithelial cells 215, 217
IP3 transfer, supporting cells 315–16
‘iPOD’ ear 378
irregularities, as cause of reflections 166–7
Jagged, role in inner ear development 332, 334–5
Jeff mouse strain 393
Jervell syndrome 223
Jumbo mouse strain 393
K+ absorption 223
K+ buffering 216
K+ channels, hair cells 266
K+ clearance, supporting cells 312–13
K+ concentration, endolymph 213
K+ cycling 215–16, 311
K+ secretion 218–19
K/Cl co-transporter (KCC) family 312–13
KCNE1 mutations 395
KCNJ10 channels 219, 220
KCNMA1 channel 216
KCNQ1 mutations 395
KCNQ4 channel 216
KCNQ4 mutations 395
Kearns–Sayer syndrome 387
KIF3A 263
kinocilium 336
‘kiss and run’ kinetics, synaptic vesicles 269, 278
Klein–Waardenburg syndrome 385
(p. 444) knock-out/knock-in mouse models 393
Kölliker’s cells 309, 318–19, 320
Kölliker’s organ 308–9
spontaneous calcium waves 321
Lange–Nielsen syndrome 223
language acquisition, children with cochlear implants 429–30
laser-Doppler vibrometry 72–3, 79
in ossicular disorders 74
laser interferometry 143–4
laser self-mixing method 144
lateral compartment, organ of Corti 307
lateral links, stereocilia 243, 244–5, 250
lateral-olivocochlear neurons (lOCs) 9, 283–6, 284, 301
co-localization of neurotransmitters 289–90
lateral wall, outer hair cells 181
length changes, electromotility 183, 184
lesser epithelial ridge 333
Leu-enkephalin 289
lever ratio, malleus and incus 60–1
ligaments of middle ear 52, 53
linear systems 140–1
linkage analysis 389–90
lipids, role in electromotility 204–5
localization of sound 62–3
head-related transfer function 63–4
loci 382
logarithmic (octave) scaling 115
longitudinal fluid flow 163
longitudinal stiffness, tunnel of Corti 165
loop amplification systems 415
loop diuretics, ototoxicity 30–1
macrolide antibiotics, ototoxicity 29–30
magnetic resonance imaging (MRI) 22–3, 24, 423, 425
vestibular schwannoma 25
malleus 2, 3, 52, 53
coupling with tympanic membrane 51
motions 66, 67, 68
malleus fixation 74
manubrium 52, 53
masking 142
maternally inherited diabetes and deafness (MIDD) 387
MDEG (mammalian degenerin) channels 247
mechanical response curves 145
mechanotransducer currents 234
mechanotransduction 232–5
adaptation 236–8
development 342, 343–4
gating-spring hypothesis 235–6, 242
MET channels 179, 180, 182
function 248–9
identity 247–8, 248–9
location 245–7
medial compartment, organ of Corti 307
medial-olivocochlear neurons (MOCs) 9–10, 284–6, 301
co-localization of neurotransmitters 289–90
MELAS (myoclonic epilepsy, lactic acidosis and stroke-like episodes) 387
membrane area constraint, outer hair cells 187, 188
membrane breakup theory, tympanic membrane 78
membrane motor 191–3, 192
in corporation into cell 193–6
membrane tension modeling, OHCs 189, 191
Ménière’s disease
audiometric pattern 16
gentamicin, therapeutic use 29
hydrops 223
pathogenesis 6, 313
meningitis, as complication of cochlear implantation 426–7
MERRF (mitochondrial encephalomyopathy with ragged red fibres) 387
MET channels 179, 180, 182, 233–4
adaptation 236–8
function 248–9
gating-spring hypothesis 235–6
identity 247–8
location 234–5, 245–7
and tip links 244
Met-enkephalin 285, 289
methotrexate, in autoimmune inner ear disease 33
microchamber configuration, OHC studies 185, 188
microdomains, calcium channels 267
microphone technology, hearing aids 412–14
microtubule arrays, supporting cells 315, 316
middle ear 2–4, 50–1, 53
diagnostic procedures 72–3, 79
frequency dependence 61
functional interdependence with external ear 54–5
ligaments 52
models of function 73
muscles and tendons 52–4
ossicles 52
reverse transmission 73
sound transmission 59–61
tympanic membrane 51–2
middle ear fluid, effects on sound transmission 74
middle ear function, measurement in live humans 72–3
middle ear gain 60
middle ear input impedance 54
middle ear reconstruction 58–9, 74
migration of cells, cochlear development 336
minimum signal, hair cells 274–5
MITF mutations 385
mitochondrial inheritance 381
pedigree 380
mitochondrial mutations 387–8
mitotic hair cell regeneration 356, 360, 362–3
timeline of events 361
mixed hearing loss 16
audiometric pattern 19–20
modeling of cochlear 152–4, 153
‘active’ cochlea 154–7, 159–61
modeling of electromotility 189
cell geometry and membrane tension 189, 191
membrane motor 191–3, 192
incorporation into cell 193–6
piezoelectric description 196–200
molecular genetic testing 397–8
molecular selectivity, gap junctions 315–16
(p. 445) Mössbauer technique 143
motility, outer hair cells 162
motor charge 207
motor molecules, anion movements 203–4
mouse models of hearing loss 390–1
chemically-induced mutations 392–3
spontaneously occurring mutations 391–2
targeted mutations 393–4
multicomponent cochlear models 160
multiline trick 275
multiple channels, hearing aids 414
multiple spontaneous otoacoustic emissions 166
multistage compression, hearing aids 411, 412
MUNC13-1 263
muscarinic receptors 299
muscles of middle ear 52–4
mutations, naming conventions 382
mutation scanning 398
mutual suppression 142, 149
myelination, efferent neurons 286
Myo6 242
MYO15A 396
myosins
hair cell apices 239
role in development 336
in tip links 242
myosin 1c 242, 244, 248–9, 250
myosin VI 242
myosin VIIa 241, 244, 250, 395–6
myosin VI and VIIa expression
apoptosis 359, 360
regenerated hair cells 362, 363–4
myosin XVa 240–1, 244, 250
myosin XV gene therapy 366, 368
myosin-dependent adaptation, mechanotransduction 237
myosin gene mutations 396
MYO6 mutations 392
MYO7A mutations 386
MYO15 mutations 392
Na+ absorption 220–1, 223
nanodomains, calcium channels 267, 274
NARP (neurogenic weakness, ataxia and retinitis pigmentosa) 388
negative cooperativity, motor molecules 195
negative resistance, basilar membrane 156
neomycin 27
Netrins, role in inner ear development 339, 341
neural cell adhesion molecule (NCAM), role in inner ear development 339, 340
neural stem cell therapy 369, 370
neurofibromatosis, auditory brain stem implants 431
neurogenins 338, 339
neurons, development 338–9
neuropeptides as efferent neurotransmitters 288–9
neurotransmitters
clearance from afferent synaptic cleft 273, 311–12
efferent neurons
acetylcholine 286–7
co-localization 289–90
dopamine 288
GABA 287–8
neuropeptides 288–9
neurotrophin-3, role in inner ear development 339–40
newborn screening 133, 379
new hair cell production 356
nicotinic receptors 299–300
N-methyl-d-aspartate (NMDA) receptors 273
noise-induced hearing loss 12, 378
ATP-dependent activity 320
audiometric pattern 16
epidemiology 25
excitotoxicity 266
noise reduction, cochlear response measurement 128–30
nompC (no mechanoreceptor potential C) 247–8
non-linear capacitance, outer hair cells 183
non-linearity 117–19, 148–52
basilar membrane response 157–61
benefits 119
TEOAEs 113
non-linear systems 141–2
non-linear TEOAE method 128
non-syndromic hearing loss 12, 377, 382, 384
chromosomal location of genes 383
connexin hearing loss 384–5
nortriptyine, effect on tinnitus 37
Notch 1 receptor, role in inner ear development 335
Nuel, spaces of, development 334
objective tinnitus 35–6
occludin 310
octave (logarithmic) scaling 115
oligomerization, prestin 207
olivocochlear efferent neurons 283–6
omnidirectional microphones 412, 414
onset of hearing 341–2
open mold hearing aid design 415
optical low-coherence interferometry (OCT) 144
optimum stimulus frequency ratio, DPOAEs 121
organ of Corti 4, 5, 6, 9, 93, 94–5, 231–2, 233, 307, 308
development 332–4
displacement by electromotility 201
electrical stimulation 162–3
mechanics 141
micromechanics 161–5
oscillations, hair bundles 236, 237
osmotic balance, supporting cells 313
osseointegration, Baha system 418
ossicles 2, 3, 52
effects of middle ear muscles 53–4
high frequency motion 68–70, 69, 78
motions 65–8
non-ideal behaviour 61
stapes, non-piston-like motions 78–9
ossicular chain interruption 57–8
ossicular coupling 59–60
ossicular delay 70
ossicular disorders, mechanisms of hearing loss 74
ossification, cochlea 425, 426
otic pit 329
otic placode 329
otic vesicle (otocyst) 10, 329–30
promotion of nerve fibre growth 339
(p. 446) otoacoustic emissions (OAEs) 4, 10, 93, 105, 106–7, 108–9, 144, 165–8
applications 132–3, 168
calibration of measuring systems 126–7
as cause of tinnitus 36
distortion product otoacoustic emissions
generation 119–22
measurement 122–6
identification, isolation, and validation 127–8
newborn screening 379
noise minimization 129–30
pass criteria 131
production 115–16
recording 106
signal enhancement 129–31
spontaneous 107, 109
stimulated 109–10
transient evoked otoacoustic emissions 110–13
two-tone suppression 117
otoconia 214
otocyst 10, 329–30
promotion of nerve fibre growth 339
otoferlin gene (OTOF) 276, 277, 398
otogelin, role in inner ear development 332, 337
oto-reflectance 72–3, 79
otosclerosis 384
tympanometry 18, 20
ototoxicity 12, 26
aminoglycosides 26–9
audiometric pattern 16
causative agents 27
and cochlear amplification 156
DFMO 31–2
endocochlear potential inhibition 219–20
loop diuretics 30–1, 219
macrolide antibiotics 29–30
platinum chemotherapeutic compounds 31
salicylates 26
vancomycin 30
outer hair cells 93, 180, 214, 231–2, 233, 307, 330
afferent innervation 260, 261, 337–8
axial displacement, reciprocal effects 186, 187
axial stiffness 184–5, 205
voltage dependence 197–9
charge transfer and non-linear capacitance 183–4
and cochlear amplification 114–15, 157
comparison with piezoelectric materials 197
cytoskeleton 199–200
development 333
effect of poor function 104
efferent inhibition 294–5, 297, 298
efferent innervation 205–6, 284–6, 301, 337, 338
excitation 161, 162
force production 185
length changes 183–4
membrane area constraint 187, 188
membrane motor, incorporation into cell model 193–6
membrane motor models 191–3, 192
membrane tension modeling 189, 191
motility 162
non-linearity 119
pressure change response 194
pressure-induced membrane tension 186–7
spontaneous otoacoustic emissions 107, 109
stimulation studies 163
structure 180–2
viscoelastic relaxation 200
voltage change response 194–6
outer pillar cells 307
outer sulcus 214, 330
pH regulation 222–3
oval window 55, 56
P2X receptors 318
P2Y receptors 318
p19 knock-out mice studies 366
p27kip1, role in inner ear development 332, 334
p27kip1 knock-out mice studies 366, 367
Paget’s disease 77
pars flaccida, tympanic membrane 51–2
pars tensa, tympanic membrane 51
parvalbumins 250
pass criteria, screening programmes 131
passive reflection model, OAEs 116
patch-clamp recording, afferent synapse 264–6, 265
PAX3 mutations 385
Pax8 276, 277
PCDH15 mutations 386–7, 392
PCNA expression, hair cell regeneration 362–3
PDK (TRPP) channels 248
pedigree analysis (linkage analysis) 389–90
pejvakin 277
Pendred’s syndrome 220, 387
hydrops 223
pendrin 221, 222
perforation of tympanic membrane 74–5
perilymph 6, 214
composition 215, 221
K+ buffering 216
periodic signals, analysis into sinusoidal components 140
phase 141
phase jitter, nerve fibres 275
phase locking, nerve fibres 260
phase shift, stereocilia motion 164
pH balance, supporting cells 313
PHR1 241
pH regulation 222–3
pH-sensitive fluorescent proteins 266
pH sensitivity, Ca2+ absorption 221
piccolo 263
piezoelectric coefficient 197
piezoelectricity 196–7
voltage dependence of axial stiffness 197–9
pigmentary anomalies, Waardenburg’s syndrome 385
pillar cells 307, 333
development 336
structural roles 314
pinna flange 49, 50, 51
PJVK 276
place-fixed otoacoustic emissions 167
plasma, composition 215
(p. 447) plastins 240
platinum chemotherapeutic compounds, ototoxicity 31
PMCAs (plasma membrane calcium-ATPases) 248, 250
posterior ligament of the incus 52, 53
postsynaptic mechanisms
adaptation 273–4
EPSPs and EPSCs 271–3
neurotransmitter clearance 273
Pou4f3, role in inner ear development 332, 335
prenatal diagnosis 400
presbycusis 25, 388–9, 394
genetic studies 11
pattern of hearing loss 16
pressure change response, outer hair cells 194
pressure-induced membrane tension, outer hair cells 186–7
prestin 10, 93, 162, 181, 206–7
co-variation with Chrna10 300
dimerization 207
loss of 220
mutants 207–8
prestin knock-out mice 208–9
presynaptic mechanisms 266
endocytosis 270
imaging synaptic release 270–1
ionic currents and neurotransmitter release 266–8
vesicle exocytosis 268–70
pretension, middle ear muscles 54
prevention of hearing loss 12
procaine, effect on electromotility 205
progenitor cells 323
programmed cell death see apoptosis
programming, cochlear implants 427
proliferation inhibitors 366
‘proliferative center’, cochlea 336
Prosaposin 276
protocadherin 15 241, 244, 250
pure tone audiometry 15–16
pure-tone average (PTA) 16
relationship to success with Baha device 419
pure tones 140
purinergic receptors 318–19
Q 99, 115
radiographic imaging 22–3
radixin 241–2, 250
Rasmussen, GL 283
Rb knock-out mice studies 366
RC (resistor–capacitor) time constant problem 201–2
reactive motions, hair bundles 236
readily releasable pool (RRP), ribbon synapses 262
recruitment 410
compression strategies 411
rectification 158
inner hair cells 96
reflection OAEs 115–16, 166–8
reflectometry 72–3
regeneration of hair cells 10, 11, 323
in birds 356
definition 355–6
in embryonic mammalian cochlea 365–6
future direction 369
gene therapy 366, 368
genetic manipulation studies 366
stem cell therapy 368–9, 370
supporting cell responses 360–5
in vertebrates 355–6
Reissner’s membrane 5, 6, 214
Na+ absorption 220–1
releasable pool (RP), ribbon synapses 262
repair 10–11
reproducibility, cochlear responses 129–30, 131
resequencing chips 399
resonance, tectorial membrane 164–5, 168–9
resonant frequency, ear canal 2
response averaging 129
resting activity, nerve fibres 259, 267
reticular lamina, role of supporting cells 314
retinitis pigmentosa
NARP 388
Usher’s syndrome 386
retinoic acid, role in inner ear development 332, 335
reverberations, impact on hearing aid use 410
reverberation suppression, hearing aids 416
reverse middle ear transmission 73
reverse molecular genetics 390
reverse transduction, outer hair cells 179
ribbon synapses 8–9, 261–4, 262
ribeye 262–3
Ribeye/CtBP2 276
RIM2 263
rising (up-sloping) audiometric pattern 19–20
room loop amplification systems 415
round window 55, 56
function 105
round window reflex 55
saccule 214
sac endolymph
composition 215
see also endolymph
salicylates
effects on electromotility 203, 204, 205
ototoxicity 26
SANs 241
SANS mutations 386
scala media 4, 5, 6, 7, 100, 214, 330
scala tympani 4, 5, 6, 7, 139, 214, 330
electrode placement 425
scala vestibuli 5, 6, 139, 214, 330
‘scar’ formation, Dieters’ cells 313
Scheibe’s deformity 223
screening programmes 132
infants 106, 110, 133, 379
pass criteria 131
semaphorins, role in inner ear development 339, 341
semicircular canal duct
Ca2+ absorption 221–2
Cl- secretion 222
Na+ absorption 220–1
semicircular canals 214
(p. 448) sensorineural hearing loss 4, 16
association with tinnitus 35, 36
audiometric pattern 16
autoimmune causes 32–3
epidemiology 23–6
idiopathic sudden (ISSHL) 33–5
ototoxicity 26–32
in vitamin D deficiency 221–2
sequence analysis of selected exons 398
serous otitis media, tympanometry 18, 20
shaker mice 391, 392
signal analysis
linear systems 140–1
non-linear systems 141–2
signal enhancement, cochlear responses 129–31
signal-to-noise ratios, cochlear responses 130–1
single nucleotide polymorphisms (SNPs), genome-wide association studies 394
sinusoidal signals 140
Six1 gene 332
SIX5 mutations 386
SK channels 291, 292, 293, 294
SLC17A8 276
SLC26A4 mutations 387
SLC26A5 see prestin
slit/Robo, role in inner ear development 339, 341
SNAI2 mutations 385
SNARE complex 263–4
Snell’s waltzer mice 392
somatic motility, outer hair cells 162
sonic hedgehog gene (Shh) 332
sound, gathering and transduction 50, 51
sound pressure, variations along ear canal 70
sound shadows 2
sound transmission, middle ear 59–61
Sox2, role in inner ear development 332, 334
SOX10 mutations 385
spectral filtering, hearing aids 410
spectrin 205
speech audiometry 16–18
speech discrimination, candidacy for cochlear transplantation 422
speech discrimination score (SDS) 18
speech reception threshold (SRT) 17–18
speech recognition, children with cochlear implants 429
spike timing analysis 275
spiral ganglion neurons (SGNs) 8–9, 10
development 11
spiral ligament 214
spiral limbus 214, 330
spiral prominence 214
spontaneous activity
during development 320, 344, 345
nerve fibres 259, 267
spontaneous otoacoustic emissions 107, 108, 109, 166
sprouty2 (spry2) gene, role in inner ear development 336
stapedius muscle 52, 53
stapedius muscle reflex (acoustic reflex) 18, 20–1
stapes 2, 3, 52, 53
effect of stapedius muscle 53
motions 66–7
non-piston-like motions 78–9
velocity transfer functions 69–70
stapes fixation 74
stapes footplate, area ratio to tympanic membrane 60, 105
statoacoustic ganglion (SAG) development 338–40
‘stem-cell-like’ cells 364
stem cell therapy 368–9, 370
stereocilia 6, 7, 94–5, 231–2
alignments 165
candidate proteins for mechanotransduction 250
cytoskeleton 238–42
deflection 161
development 240–1, 336
extracellular linkages 242–5, 243
MET channels 234–7
outer hair cells 180
role in cochlear amplification 162
stereocilia motion, phase shift 164
stereociliary bundle, anomalies in Usher’s syndrome 395–6
steroids
in autoimmune inner ear disease 33
in idiopathic sudden sensorineural hearing loss 34–5
stimulated otoacoustic emissions 109–10
transient evoked otoacoustic emissions 110–13
stimulus calibration, OAE measurement 126–7
stimulus frequency otoacoustic emissions (SFOAEs) 116–17, 166
identification, isolation, and validation 128
noise minimization 129–30
stimulus levels, DPOAE production 122
streptomycin, ototoxicity 27, 28–9
stress granule formation 358–9
stretch-sensitive channels, outer hair cells 181
strial marginal cells, K+ secretion 218
stria vascularis 6, 94, 100, 214
K+ transport 218, 219
subjective tinnitus 36–7
summating mechanical response 132
summating potential (SP) 102–3
diagnostic use 103–4
superior canal dehiscence (SCD) 20–1, 75–7, 76
superior olivary complex 283
supporting cells
ATOH1 transfection studies 365, 366
barrier function 310
cell types 307–8
changes during development 308–9
connexins 316–17
cytoskeleton 315, 316
damage signalling 320, 322–3
development 333
epithelial repair 313–14
gap junctions 310–11, 312, 315–17
hair cell regeneration 323, 356, 360, 364–5
direct transdifferentiation 360–2
mitotic division 362–3
innervation 314
ion channels 319
K+ and neurotransmitter clearance 311–13
(p. 449)
non-mammalian 309–10
osmotic and pH balance 313
purinergic receptors 318–19
role in development 319
structural roles 314
surface waves 152
tympanic membrane 64–5, 66
sustained operation, nerve fibres 260
syllabic compression 412
synapses
physiological recording techniques 264–6
structure 260–4
synapsin 264
synaptic currents, hair cells 292, 293, 294
synaptophysin 264
synaptotagmins 263–4
synchronous averaging, cochlear responses 129
syndromic hearing loss 377, 382, 385
branchio-oto-renal syndrome 386
Pendred’s syndrome 387
Usher’s syndrome 386–7
Waardenburg’s syndrome 385
targeted mutation analysis 398–9
targeted mutations, mouse models 393–4
Tecta, knock-out and knock-in mouse models 393
tectorial membrane 4, 5, 6, 214, 231, 233
development 337
micromechanics 161, 163, 164–5
pH sensitivity 223
role in frequency selectivity 168–9
role of supporting cells 314
tectorin, role in inner ear development 332, 337
tectorin gene mutations 164
telecoils, hearing aids 414–15
temporal bone CT scans 423, 424
temporal bone preparation studies 74
tenascin, role in inner ear development 340
tensor tympani muscle 52–4, 53
terminal mitosis, cochlear cells 334
third window model, superior canal dehiscence 75–7, 76
‘third window’ paths 56–7
threshold stimulus 274–5
effect of efferent activity 295
thyroid hormone, role in inner ear development 332, 335–6, 337, 346–7
thyroid hormone deficiency 277
TIAR (T-cell restricted intracellular antigen-related protein) translocation, apoptosis 358–9
tight junctions 213, 310
time delay, otoacoustic emissions 128
tinnitus 35
objective 35–6
role of ATP-dependent activity 320
salicylates as cause 26
spontaneous otoacoustic emissions 109
subjective 36–7
treatment 37
tip links, stereocilia 8, 234–5, 242–4, 243, 250
and MT channel location 245–7
TMC1 (transmembrane channel 1) 276, 277
tobramycin, ototoxicity 27, 28
tonal stimulation, signal filtering 129
tone burst evoked TEOAEs 110, 111, 112, 116
tone-pips 100
tonotopicity 95
tragus 51
transcranial magnetic stimulation (TMS), in tinnitus 37
transduction function 158, 159
transfer functions, afferent synapses 274
transformer mechanisms, middle ear 60–1
transient evoked otoacoustic emissions (TEOAEs) 108–9, 110–12
applications 132, 133
calibration of measurement systems 127
clinical value 112–13
identification, isolation, and validation 127–8
noise minimization 129
non-linearity 113
pass criteria 131
production 115–16
reproducibility 130
size variation 113
transport proteins 215
trans-tympanic electrocochleography 103
transversal waves 93, 94, 96, 114–15, 152
effect of poor outer hair cell function 104
reflection 115–16
signal analysis 139
tricellulin 310
trimodal theory of sound localization 63–4
trinitrophenol, effect on electromotility 205
TRIOBP mutations 390
TRPML3 (transient receptor potential mucolipin 3) 245
TRPP (PDK) channels 248
TRP (transient receptor potential) channels 247–8
TRPV5/ TRPV6 221, 222
trypsin treatment, outer hair cells 199
tuning of the cochlea 146
tunnel of Corti, development 333
two-state motor model, outer hair cells 191–3, 192
two-tone suppression 142
otoacoustic emissions (OAEs) 117
tympanic membrane 2–4, 51–2, 53
area ratio to stapes footplate 60
areas of continuing research 77–8
atelectasis 50
curvature 61
impedance 105
non-ideal behaviour 61
perforations 74–5
sound-induced motion 54, 64–5, 66
tympanometry 18, 20, 105–6
tympanoplasty
diagnosis of causes of failures 79
type IV 58–9
type II afferents 261
unilateral deafness 417
unitary motor charge 183
up-sloping (rising) audiometric pattern 16, 19–20
urocortin 288, 289
(p. 450) USH1C mutations 386
USH2A, mutations 386, 387, 398
USH3 mutations 386
usherin 245
Usher protein complex 395–6
Usher’s syndrome 11, 386–7, 395–6
utricle 214
utricular endolymph
composition 215
see also endolymph
vancomycin, ototoxicity 30
van der Pol oscillators 160–1
vectorial transepithelial transport 215
vesicle dynamics, mathematical models 276
vesicle exocytosis 268–70
development 278
vesicle fusion, measurement techniques 264–6
vesicles, ribbon synapses 261–2, 263–4
vesicular vending machine, ribbon synapses 262
vestibular dark cells 214
K+ secretion 218
vestibular–occular reflex 29
vestibular schwannoma
magnetic resonance imaging (MRI) 25
sudden hearing loss 34
vestibular transition cells, pH regulation 222–3
vestibulotoxicity, aminoglycosides 28–9
vezatin 241
VGLUT3 263
viscoelastic relaxation, outer hair cells 200
viscous drag 201–2
vitamin D deficiency, hearing impairment 221–2
vitamin D receptor, role in Ca2+ absorption 221
VLGR1b mutations 386
VLGR1 (very large G-protein-coupled receptor) 244–5
voltage changes, outer hair cell response 194–6
voltage-dependence, gap junctions 315
Waardenburg–Shah syndrome 385
Waardenburg’s syndrome 385
waltzer mice 242
wave-fixed otoacoustic emissions 167
wave types 152
Wever–Bray effect 143
whirlin 241, 244, 250, 336, 395, 396
WHRN mutations 386
wideband reflectance 106
wide noise band stimulation studies 149–51, 150
windowing, OAE measurement 128
window pressure-difference hypothesis 55–7
clinical evidence 57–9
wnt 336
X-linked inheritance 381
pedigree 380
zone of non-proliferating cells (ZNPC) 365