Strongly recommanded study literature: Hans Behrbohm, Oliver Kaschke, Tadeus Nawka,Andrew Swift: Ear, Nose, and Throat Diseases: Founding Authors W. Becker, H.H. Naumann, C.R. Pfaltz (Paperback) Publisher: Thieme Publishing Group; 3rd Revised edition edition (12 Aug 2009). 471 pages , Language English. ISBN-10: 313671203X, ISBN-13: 978-3136712030. Ear I Ass.prof. P. Smilek, MD, Ph.D. ENT Clinic of Masaryk university, Brno Faculty St. Ann Hospital Head: Ass.prof. Gál Břetislav, MD, Ph.D. Pekařská 53, Brno , 656 91 The hearing and balance system The hearing and balance system localized in temporal bone The hearing system Two main subdivisions: Peripheral Part ▪ external, middle and inner ear ▪ auditory nerve Central Part ▪ central hearing pathways ▪ subcortical and cortical auditory centers Anatomic boundary - entry the VIIIth nerve into brainstem. Function of hearing system – The external and middle ear transport the stimulus – cochlea distributes the stimulus – the sensory cells transform the stimulus External ear Auricle – abundantly formatted cartilage (localization, protection) External meatus (meatus acusticus externus) External part ▪ cartilage ▪ Lined with skin and down (tragi) and sebaceous gland – cerumen Internal part ▪ Temporal bone ▪ Lined with thinned epidermis (skin) – curved cartilaginous mobile part – must be drawn upward and posteriorly (in adults)– to bring the same axis External meatus skin has 10x higher growth potential than middle ear mucose membrane – theory of development of acquired cholesteatoma Middle Ear Middle Ear cavity – summary name for the whole pneumatic system of temporal bone: tympanic cavity, cells of proc. mastoideus and Eustachian tube (tubotympanal and tympanomastoideal segment) Middle Ear Tympanic membrane (membrana tympani) - a sound pressure receptor and transformer Inclination and declination angle to meatus axis, surface 55 mm2 – sulcus tympanicus; incissura tympanica Rivini – anulus fibrocartilagineus (fixing of tymp.membrane) pars tensa ▪ Three layers: – external- epidermis (stratum cutaneum) – middle– fibrous layer (str. fibrosum) – internal– epithelium (str. mucosum) pars flaccida (membrana Shrapnelli) ▪ Surface 5 mm2 in superior part of ear drum ▪ Fibrous layer is almost missing (lower strength) Tympanic cavity (Cavum tympani; shape of biconcave lens) 6 walls Paries: ▪ membranaceus ▪ labyrinthicus ▪ tegmentalis ▪ jugularis ▪ mastoidea ▪ caroticus Lateral projection of Ear drum divides tympanic cavity Into: 1,2 epitympanic recess 3 mesotympanum 4 hypotympanic recess Tympanic cavity – medial wall Middle ear Tympanic cavity (cavum tympani) ossicular chain: ▪ malleus ▪ incus ▪ stapes Hearing function of tympanic cavity (transfer system) 1. Compensation of loss of acoustic energy (ear- liquid): a/ tympanic membrane - oval window 14x b/ lever-action system of ossicles 1,3x c/ lever-action system due to uneven incurvation of ear drum, all together amplification is 30-35dB 2. Mutual change of deviation and pressure acoustic vibration . Gas = great deviation, low pressure. Liquid = low deviation, great pressure . Eustachian tube • Ventilation function - it serves to equalize the pressure between middle ear and the nasopharynx • Drainage function – removal of secretion from middle ear cavity • Protective function - before secretion penetration into middle ear cavity (microbial barrier guard system in epipharynx vs sterile tympanic cavity) Membranous labyrinth (hearing and balance) ▪ Cochlear Duct (ductus cochlearis) ▪ Saccule and Utricle (saculus et utriculus) ▪ Membraneus semicircular Ducts (canales semicirculares) Cross-section of Cochlear Duct (dct. Cochlearis) A snail shell in shape, two and half turns Cochlear duct cross-section, organon spirale Corti membrana basilaris, reticularis, tectoria HEARING FUNCTION - COCHLEA Cochlear function: •Change of mechanic vibration on neural excitation •Basic frequency analysis Vibration conduction from the ear drum through cochlea, Organon Corti Outer hair cells (OHC) = servomechanism for inner hair cells (IHC) Cochlear septum System of 3 membranes – membrana basilaris, reticularis, tectoria Principle of tonotopy – as higher frequency, the acustic pressure balanced near to stapes. High frequency are perceived in basal thread, low frequencies in apical thread. Wave hydrodynamic theory of hearing (von Bekesy theory of „traveling wave“; still valid ☺) ▪ Acoustic tension is led from ear drum through ossicles into oval window. ▪ Liquid is je not compressible, pressure changes are equalized on round window membrane, which vibrate in anti phase to stapes. ▪ Acoustic pressure is equalized on cochlear septum and it create wave. ▪ a sound impulse sends a wave sweeping along the basilar membrane. ... And as Helmholtz had postulated, Bekesy found that the high-frequency tones were perceived near the base of the cochlea and the lower frequencies toward the apex." (principle of tonotopy). ▪ „ traveling wave“ leads to shift of tectorial membrane of Organon Corti in relation to basilar membrane and deflection of hairs of sensory cells (opening of iont channels) ▪ Thus the mechanic energy is changed into electric potential in VIII cranial nerve. Vestibular system Basic function 1. Equilibrium of human body in stand and in walking 2. Stabilization of retinal picture and keeping visual sharpness in movement Equilibrium Three afferent sources of equilibrium: eye, proprioception and vestibularsemicircular system. Basic reflex circles ▪ vestibulo-ocular reflex (VOR) ▪ Vestibulo-spinal reflex (VSR) - help keep head and body in upright position due to vestibulospinal system. On keeping balance : Interaction of eye, vestibular-semicircular system, proprioception X vestibular nuclei and cerebellum Vestibulo-occular reflex Stabilisation of retinal picture. Create movement of eye, which are opposite to movement of head in some plains. Nystagmus – conjugated, coordinated eye movement around a specific axis. The movement consists of rhythmically alternating slow – and fast beating phases. The direction of the fast components determines the laterality of the nystagmus. Membranous labyrinth ▪ Vestibular apparatus localized in pyramis ossis temporalis; membranous labyrinth: saccule, utricle and three semicircular canal; filled with endolymph. ▪ Every semicircular canal begins with pars ampullaris with ampullary crest, sense organ is static macula (macula statica utriculi et saculi). Section of semicircular canal, schema of membranous labyrinth ( with one canal), sensory organs. Genesis of receptor potential Ampullary crests (cristae ampulares) and maculae utricle and saccule are created by supporting cells and hair cells. On their surface lie the otoliths (statoconia) – calcium carbonate crystal. Linear acceleration changes the otolith pressure, deflecting the sensory hairs. This stimulates the sensory cell by altering the resting potential. Diagnosis, vestibular function tests History – subjective feeling of dizziness, time of start and duration… Evaluation of reflexes – spontaneous vs. experimentally provoked (calorisation, rotation) ▪ Nystagmus (vestibulo-ocular reflex) Detail evaluation of eye movement electronystagmography, video oculography (preferred) ▪ Vestibule-spinal reflexes: – Hautant test (spontaneous deviation test), – Romberg test (patient is asked to stand in basic position/wide stance, and to close eyes. A check is to see whether there is unsteadiness or a tendency to fall.) ▪ Vestibule-cerebellar reflex – Barany test (finger-nose pointing test) ; posturography Vestibular function Tests ▪ Head shaking nystagmus – spontaneous Nystagmus can be provoked by gentle, passive, horizontal shaking of the patients head ▪ Unterberger test, walk „on the rope“ ▪ Stabilometric plain - static and dynamic (vestibule-spinal reflexes) ▪ Head impulse test: – in long lasting dizzines. From mild eccentric head position we provide passive quick rotation movement from side to side, patient fixis our tip of nose…. Vertigo (dizziness) the outcome of the functional tests is to determine whether it is peripheral or central vertigo. Peripheral vertigo is troublesome but not life-threatening, while central vertigo can be life-threatening. • Periferal type – feeling of rotation of itself body or surroundings, direction of rotation is usually into healthy part, loss of stability or feeling of swimming • Central type – ineptitude by walk, inability of walk, vertigines with aura (EPI), disorder of vision "black outs" – diplopia is seen by disorder of oculomotory. Differential diagnosis peripheral vs. central vestibular syndrome Symptom Peripheral (harmonic) VS Central VS Nystagmus Horizontal rotatory on side of most reactive labyrinth Other then horizontal rotatoric: vertical, „gaze“ nystagmus, rebound nystagmus etc) Tonic deviation On side of weak labyrinth (to affected ear) in relation to position of head Without to relation to head position Eye fixation In absence of eye fixation nystagmus is growing In absence of eye fixation nystagmus not changed Cranial nerves Without lesions (excl.: n. VII) Laesion of cranial nerves present Cerebellar symptoms Not present Could be present Disturbance of occulomotoric function Not present Could be present Hearing disorder Usually Could be present Usually Not present Central compensation Gradually compensation, harmonic symptoms: intensity of vertigo correlates with nystagmus intensity and tonic deviations Not present, disharmonic symptoms History of ear disease Hearing disorder Otorhoea Ear discharge Tinnitus Pain Dizzines Physician itself is a „remedy“. (Michael Balint) Ear evaluation, oto(mikro)scopy ▪ Aspection and palpation – Auricle shape, deformities – Skin lesions, scars (also retroauriculars) – Discharge from external meatus – Pain in pressure on tragus (by otitis externa) – Pain in pressure on processus mastoideus (by mastoiditis acuta) ▪ Otoscopy and oto(micro)scopy evaluation – Ear speculum – Illuminated otoscope, pneumo-otoscopy – Otomicroscope, endoscope Otoscopy eye evaluation of deeper parts of external meatus and ear drum The cartilaginous part of ext. meatus is stretched by pulling the auricle upward and backward „Normal“ ear drum • Grey colour • Without perforation • Bezold´s trias Prominentia mallearis Stria mallearis Light reflex Otoscopy – tympanic membrane quadrants and zones Basic pathologic finding on tympanic membrane ▪ Changes on the tympanic membrane: Injection of the vessels ▪ Position of the tympanic membrane – bulgging due to exudate - hyperemia, moist infiltration and opacity of the surface, the contours of the handle of malleus and short process disappear – retraction - injection of blood vessels ▪ integrity of the tympanic membrane perforations – – after injury – inflammatory - acute - chronic - central (mesotympanic) - marginal (peripheral) ▪ changes after inflammation: thickening of the tympanic membrane, scars ▪ changes behind the ear-drum: middle ear effusion, fluid level, air bubbles Retraction of the ear drum, pressure equalizing tube Retrakční kapsa ve Schrapnel. blance, perforace v zad. hor. kv. s cholest. hmotami Various types of ear drum perforations Peripheral perforation Central perforation Periopheral and central perforation Ear drum injury Siegl´s ear speculum Siegl´s ear speculum Radiographs in Schüller wiev Radiographs in Schüller wiev Radiographs in the Stenver view Schema of CT of ossicular chain and middle ear cavity Canalis Fallopi Hearing disorder according to place of lesion ▪ External ear – conductive hearing loss ▪ Inner ear sensorineural intracochlear hearing disorder ▪ Central pathways – retro cochlear hearing disorder Hearing disorder (Hypacusis) Senzorineural Hypacusis perceptiva Weber lat. to better hearing Rinne posit Schwabach shortened Cochlear RetrokochleárníRetro (supra) cochlear Conductive hearing disorder Hypacusis conductiva Weber lat to worse hearing Rinne negat Schwabach prolonged Mixed hearing disorder Hypacusis mixta Schwabach shortened Rinne negat Classification of basic type of hearing dysfunction according to place of lesion Evaluation of hearing function We evaluate on growing level of objectivity: ▪ „Classical“ hearing test ▪ Audiometry ▪ Objective evaluating methods – tympanometry – evoked potentials – otoacoustic emissions Classical hearing test (speech test, tuning fork tests) Important part of hearing tests: + quick, easy, cheap, information about understanding speech – only for orientation Tuning fork tests: – Rinne – Weber – Schwabach, Gellé, … Basic types of hearing disorder P L 4 V 10 O,5 Vs 10 W + R + zkr. Sch norm Hypacusis perceptiva (Sensorineural deafness) Weber unto better hearing ear Rinne posit Schwabach shorter Retrocochlear P L 4 V 10 3 Vs 10 W –– R + prod. Sch norm. Hypacusis conductiva (Conductive hearing loss) Weber unto worse hearing ear Rinne negat Schwabach longer Hypacusis mixta (Mixed hearing loss) Schwabach shorter Rinne negat. Pure-tone audiometry An audiometer is an electric tone generator used to determine the hearing threshold for pure tones; generates tones of specific frequency (Hz) and intensity (dB). ▪ audiometric room ▪ Air conduction: → headphones ▪ Bone conduction: →bone vibrator Normal range – until 20 dB loss Symbols for record of audiometric evaluation right left Air conduction Bone conduction The speech field Region of the best sensitivity for hearing Hearing loss (Hypacusis) Conductive Sensorineural Mixed Speech audiometry Patient repeats words which are reproduced. One correctly repeated word means 10% of comprehension from one set. It is evaluated on increasing levels of intensity till 100% of comprehension or maximally possible per cent of comprehension. Comprehensionin% Speech sound level in % Sensorineural hearing loss Conductive hearing loss Normal hearing Tympanometry The greater the pressure differential (before and behind ear drum), the greater is the impedance of tympanic membrane and more acoustic energy is reflected back into external meatus. The level of testing tone in meatus is measured by sensitive microphone . Tympanometry Evoked response audiometry (ERA) ▪ Human brain works with bioelectric impulses, not sound. ▪ bioelectric impulses could be measured as an electric potential ▪ conditions: motoric and psychic rest of patient, camera silent, electric interference suppression of the room ▪ Patient repeatedly exposed to an acoustic stimulus, an EEG is recorded. Averaging – the individual response can be distinguished by mathematical analysis of numerous individual evoked potentials Evoked response audiometry (ERA) ▪ Cochlear potentials, electrocochleography (Ecochg): latency window 0-4 ms. Probe – in externeal meatus or on promontorium) ▪ Early potentials (brain stem, BERA): 10-12 ms. ▪ Middle potentials MERA (middle latency evoked response audiometry : 40-60 ms – suitable for processing (steady-state evoked potentials) ▪ Late potentials (cortical, CERA-cortical evoked response audiometry): to 200 ms. Before application CI or hearing aids. BERA, BAEP (brainstem auditory evoked potentials) ▪ Early potentials (brain stem, BERA): 10-12 ms. ▪ Complex 7 peaks, so called Jewett waves, most important I-III. ▪ Fixed elektrods temporal, occipital and referent forhead, given earphone ▪ Susp. rektrocochlear laesion – dysmorphy of Jewett complex, missing or shift of latency V wave. ▪ Senzitivity and specifity for dg vestib schwanoma 70-80 % Auditory evoked potentials BERA – prolonged latency of 0,2 ms – suspection on small schwanoma n. VIII Patient repeatedly exposed to an acoustic stimulus, an EEG is recorded. Averaging – the individual response can be distinguished by mathematical analysis of numerous individual evoked potentials Middle potentials MERA Steady-state evoked potentials (SSEP) ▪ Other names: ASSR (auditory steady-state responses), AMFR (amplitude modulated frequency responses) – processing of signals of middle electric response. ▪ Fully automatized ▪ Good correlation with pure tone audiometry ▪ Outcome of measurement – objective frequency – specified threshold audiometry, estimation audiogram Otoacustic emision (OAE) ▪ Sound result from spontaneous or provoked activity of outer hair sells organon Corti. ▪ Spontaneus OAE arise itself, sign of normal function of Corti organ, present in childhood ▪ Evoked OAE – Tranziently evoked otoacustic emision (TEOAE)- as response on acoustic stimulus – Distors products (DPOAE) – emission response on 2 simultaneous perceived tons of nearby freqency and intensity ▪ Usage newborn screening of hearing function and frequency specified determination of hearing threshold Sensorineural hearing loss According to type of audiometry curve: • Basocochlear • Pankochlear • Apicocochlear • Mediocochlear According to lesion localisation: • Cochlear • Retrocochlear Cochlear lesion - bothering, but not life threating Retrocochlear lesion - bothering, but also they could life threaten Sensorineural hearing loss (intra) cochlear = damage of cochlear structures Etiology: – Presbyacusis – Heredo-degenerative – Nois damage – Toxic damage – Menier´s disease – Acute sensorineural hearing loss … etc. Sensorineural hearing loss retro- (supra-) cochlear = damage of structures proximal from cochlea Etiology: Demyelization - atherosclerosis - sclerosis multiplex Inflammation - borreliosis - neuro-viruses - meningitis - meningoencephalitis Tumors -vestibular schwannoma - meningioma - other tumors of cerebellar angle Trauma - commotion, contusion - scull base fractures Differencial diagnosis cochlear/retrocochlear 1. Subjective tests: - time demanding - active cooperation of pt - complicated for understanding - relatively low validity 2. Objective tests : - time usually not so demanding - demand only passive patient cooperation - expansive technical equipment - high validity Subjective tests Based on proof of: mask effect of noise recruitment phenomena wearisomeness of hearing organ Recruitment phenomena = abnormal increase of loudness in above-threshold in damage of OHC and normal function IHC. Fowler test; convergence of perceptions SI-SI Short Increament Sensitivity Index short time 1 dB increase of intensity 20 dB above treshold (20x) assuredly recognize Langebeck test = increase ability to mask tons by hum in supracochlear hearing loss. Thresholds are higher about more than 10 dB as level of ripple. Noise audiometry Langebeck test Supracochlear hearing loss