Advanced Rehabilitation in Otology

keynote lecture delivered at ORLSON’s Oluyole

2014

Advanced Rehabilitation in Otology

Olusesi A.D (FWACS, FMCORL)

Lecture Format

❖ Concept and models of rehabilitation

❖ Evolution of otological rehabilitation

❖ Advances in Otological Rehabilitation

❖ Global

❖ Africa

❖ Nigeria

❖ Round up

Rehabilitation concept

Definition:

"A set of measures that assist individuals who

experience, or are likely to experience, disability to

achieve and maintain optimal functioning in

interaction with their environment”

- WHO

Who is in need of rehabilitation?

❖ Impairment: loss/abnormality of function

❖ Disability: reduction/lack ability to perform within normal range

❖ Handicap: disadvantages from either limit fulfillment of normal role

❖ Functional limitation: loss of ability to perform tasks/obligations of

usual role/normal daily life

❖ Chronic illness: irreversible presence, accumulation or latency of

disease states/impairments that involve total human environment

Rehabilitation Issues

Quality

Quantity

Care

Cure

High

Cost

Long

Term

Rehab

Rehabilitation Team: Models

Rehab Team

Models

MultiDisciplinaryInterDisciplinary

TransDisciplinary

Rehabilitation Principles: Whatever Model

❖ Client centered therapy

❖ Team approach

❖ Evidence based care

❖ Equitable access

❖ Others

❖ care coordination / service integration / early

intervention / setting

Factors Influencing Effective Rehabilitation

❖ Acute / early intervention

❖ Early supported discharge

❖ Settings: home base with interface btw settings

❖ Therapy intensity associated with better outcomes

❖ Team care

❖ Goal setting

Rehabilitation in Otology: Evolution

❖ Prehistoric to XVI Century: Egypt / Greece / Rome /

❖ XVI Century: Italy

❖ XVII Century: France

❖ XVIII Century: France / Italy

❖ XIX Century: France / England / Germany/ Vienna

❖ XX Century: Scotland / England / Sweden / USA /

France /

Ref: Rev. Bras. Otorrinolaringol. vol.73 no.5 São Paulo Sept./Oct. 2007

Evolution of Otology By Geography

March of Otology Thro’ The Ages

❖ Hx of Otology followed Hx of Medicine

❖ Little know by ancient physicians

❖ Middle ages focused on readily accessible structured

(EAC, trauma, FBs)

❖ Magnification introduced 18th century

❖ Otology specialty started in France (1850s)

Otolaryngol Head Neck Surg. 1996 Feb;114(2):173-96

PreHistoric Otology

❖ 1,500 BC: Egyptian pharmacopedia > ‘Medication for the

hard of hearing ear’

❖ Alcmaeon of Croton (Greek): ‘air movement theory’ of

hearing

❖ Empedocles (Greek): 1st described cochlea

❖ Hippocrates: empirical Rx / relations of ear infection to

tonsils/brain infection

❖ Aristotle (Greek): ‘Pure air implantation theory’ of hearing

Otology in 1st to 16th Century AD

❖ Cornelius Celsius (Roman):

❖ first description of tonsillectomy (by finger dissection)

❖ New Rx options for tinnitus

❖ FBs in EAC

❖ Surgeries for EAC atresias

❖ Galerno (PP of Marcus Aurelius): animal dissection / creta

labyrinths

❖ Anatomical sculptures & paintings

Otology in the 16th Century

❖ Description of ossicles: Berengario de Capri / Ingrassia

❖ Description of tensor tympani, ET, and cord tympani as a nerve -

Eustachio

❖ Description of RW / OW - Versalius, 1543

❖ Fabrizi (student of Fallopius)-

❖ merged Aristotle theory with new concept of auditory stimulation

❖ proper methods of otological surgery lightning / devices to guide

light from sun or candles

Otology in the 17th Century

❖ Riolanus (1649): technique of simple mastoidectomy

❖ Perraut (Architect/Physician): Cochlear theory of

hearing/cochlear membranes vibration/degeneration in

aged

❖ Duverney: detail anatomical drawings / theory of cochlear

resonance predating Helmholtz/cholesteatomatous OM

Otology in 18th Century

❖ Jean Loius Petit: 1st description of tuberculous OM (b4

Kochs) / successful mastoidectomies

❖ Valsalva (of Bologna school): dissected over 1000

human heads / ‘ossicular chain discontinuity’ theory of

hearing loss

❖ Cotugna (from Naples): described perilymph/impressive

study of cochlea structures

❖ Scarpa (from modena) described endolymph

Otology in the 19th Century

❖ Astley Cooper: 1st myringotomy to cure ET occlusion (1801)

❖ Cruveilhier: pearl-like CNS tumor (cholesteatoma of petrous

apex) (1829)

❖ Toulemouche - 1st description of malignant otitis externa

❖ Johannes Muller - pioneer auditory physiology >> Helmholz

>> ME physiology (based on previous studies from

Duverney

❖ Dienffenbach (1845): 1st report of otoplasty for bulging ear

Otology in 19th Century contd.

❖ Huschke described brainstem / dissected birds ears /

erroneously thought he found end of auditor nerve fibers

❖ Albert Corti met Virchow (pathologist) >> to Schroeder van

der Kolk and Pieter Harting >> used innovative methods

with microscopes for anatomical dissection >> description

of Organ of Corti

❖ Evenberg (1860) 1st SHL from mumps

❖ Meniere (1861): MD, inner ear disease, not brain disorder /

deafness incurable

Rest of 19th Century Otology

Adam Politzer ViennaAtlas of Otoscopy/ ME Physiology /

Politzerization / Myringotomy for OME

Joseph Toynbee EnglandStapes footplate ankylosis main cause of

deafness

Mach GermanyPioneering rotatory stimulation for vestibular

assessment

Kessel First Surgery of stapes mobilization

Von Troltsch Germany Modified Schwartzes Mastoidectomy

Retzius Labyrinthine Microdissection

Emanuel Zaufal Radical Mastoidectomy

Ewald Labyrinthine origin of nystagmus

Otology in 20th Century

❖ Otology + Laryngology = Otolaryngology

❖ 1st electrical HA (Alt from Vienna) - micro telephone

❖ 1st VIII nerve neurectomy for MD (Perry, Scotland) - 1901

❖ Perforation of pars flaccid of apical cholesteatoma (Bondy, 1910)

❖ 1st trephination of PSCC without opening the antrum - Barany 1910)

❖ 1st description of tympanoplasty (Kisch, 1912)

❖ 1st trephination of horizontal SCC (Jenkins, 1913)

❖ Nobel Prize for work in vestibular research (Barany, 1914)

20th Century Otology Advances

❖ 1st use of cartilage in pinna reconstruction - Gilles, 1920

❖ Introduction of monocular microscope in ear surgeries - Carl

Nguyen, 1921)

❖ Audiometric exam in screening patients for HL - Fletcher &

Wegel, 1922)

❖ 1st analysis of nystagmus - Schutt & Meyer

❖ 1st MD Surgery - (Didier Portmann, 1926) >> House

❖ Description of ME acoustic muscle activity - Luscher, 1929

2oth Century Otology Advances contd.

❖ Facial nerve decompression technique introduced -

Balance & Duel, 1932

❖ 1st measure of ME impedance - Schuster, 1934

❖ Fenestration of LSCC for otosclerosis - Lempert

❖ 1st description of congenital cholesteatoma in petrous

temporal bone

Further 20th Century Otology

Boetcher, 1940Electrical burr introduced for mastoid

surgery

Glomus jugulare

described same year

Zeiss Optical, 1953Introduced modern binocular

microscope for ear surgery

Rosen, Wullstein

cashed in and improved

ear surgeries

William House,

1953

First case of Cong cholesteatoma of ME

cleft

Armstrong introduced

vent polyethylene tube a

year after

Better control and knowledge about use of microscope and the IAC area

(common btw ORL and Neurosurgery) with pioneering work of William House

contributing to successful removal of pontine cerebellar tumors with use of

microscope

Despite progress so far mentioned, 3 perplexing issues - Tinnitus, Vertigo, and Hypoacusis remain unsolvable.

Desire to conquer them form the basis of subsequent advances in ORL

Otology Advances - 1967 -Date

❖ 1st attempt at recording electrical activity of human

brainstem, evoked by acoustic stimuli - Sohmer &

Feinmesse, 1967

❖ Basis for electroCoch established - Aran & Le Bel, 1968

❖ Origin of brainstem potentials proved - Jewet, Romano &

Wilinston, 1970

❖ Many deafness related genes isolated - 2005

Otological Advances - Global

Global Otological Rehabilitation Advances

❖ Hearing Aids & Implantable Hearing (Aids) Solutions

❖ AudioVestibular implants

❖ CI / ABI / AMI

❖ Vestibular Implants

❖ Robotic Ear Surgeries

❖ Advances in Regeneration Medicine

❖ Stereotactic Ear / Skull Base Surgery

Advances in Hearing Aids

❖ Analog aids gradually giving way to digital HAs

❖ programmable

❖ equipped with more than one listening program

❖ directional microphone technology (better hearing in background

noise) 2 not 1 mic

❖ smart hearing aids - hearing aids that learn a user’s preference

recording them and - auto adjusting

❖ others: wireless connection to phone / waterproof & rustproof /

maskable / bluetooth compatible

Autoadjusting Hearing Aids

❖ Auto-focus on and amplify source of speech in a crowded room

❖ Siemens SpeechFocus / Phonak’s Zoom technology

❖ Auto-adjust to multiple listening environments

❖ e.g. Siemen’s Micon

❖ Learn user’s listening preference in up to 6 acoustic

environments

❖ Automatically detect and eliminate feedbacks (whistling) before

it begins

Bone Anchored Hearing Aids -Advances

❖ Abutment has given way to magnets

2nd BAHA Surgery in Nigeria

Cochlear / AB / AM Implants -Advances

❖ CI in the past 25 years:

❖ Speech feature extractor as coding strategy - 1986

❖ FDA approval for SPEAK strategy - 1994

❖ Implant integrated circuit - 1995

Oooh

Frequency Encoding: Signal-to-Noise Generation

Adult Male: 132 Hz; Adult Female: 223 Hz

Children: 264 Hz ; Most of Hearing: .3 - 3KHz

How Auditory System Encode Speech Sound: Cochlea

as Frequency Analyzer

Place Theory

❖ Bekesy discovered cochlear Traveling waves, 1927: Findings at variance with von

Helmholtz

❖ 1970s - 1980s: Vibration of basilar membrane non-linear

❖ 1985 - 1986: low frequency electromotility responses of OHC

❖ 1998: Mario Rugero et al: At near threshold stimulus level frequency tuning of

auditory nerves closely parallels that of basilar membrane displacement modified

by high-pass filtering

Frequency Theory

Advances in Hearing Theories till Today

Single-channel Implants Multichannel Implants

http://147.162.36.50/cochlea/cochleapages/overview/history.htm

Cochlear Implants Advances

❖ Electrode design: no. of electrodes / configuration /

placement (cochlear Vs extracochlear)

❖ Type of stimulation: analogue or pulsatile

❖ Transmission Link: transcutaneous Vs percutaneous

❖ Signal Processing: waveform Vs feature extraction

Cochlear Implants Electrode Design

❖ Single Vs Multiple Channel

Electrodes?

❖ frequency coding constrained by

❖ No. of surviving auditory

neuron at particular site in the

cochlea (Limited by etiology!!)

❖ Spread of excitation associated

with electrical stimulus

d

d

d

d

d

d

d

d

d

d

surviving neuron spread out

surviving neuron concentrated at a spot

Electrodes Design: Med El ‘Flex’ Electrodes for Different Indications

Electrodes Design: Med El ‘Form’ Electrodes for Deformed Cochlear

Commercially Available implant Devices

DeviceProcessor

Name

Electrodes

Stimulation

Number Spacing

Nucleus EsPrit/Freedom 22 0.7mm Sequential

Clarion Auria 16 1.1mmSequential/si

multaneous

Med El

Combi

40+/Tempo

40+/PULSARci100

12 2.4mmSequential/si

multaneous

Møller A (ed): Cochlear and Brainstem Implants. Loizou

Adv Otorhinolaryngol. Basel, Karger, 2006, vol 64, pp 109–143

Cochlear Implants in Last 25 Years

❖ Improvement in speech coding strategy: Nucleus 22 CI

speech feature extractor >> SPEAK strategy >> Nucleus

24M >> CIS / ACE strategy >> 1st ear level processor >>

dual microphone technology

❖ Functional improvement: Listening in background noise

(directional microphone technology)

❖ Broadening indications: Early implantation (<12

month+profound SNHL bilat), 12-24 month + severe-to-

profound SNHL+no benefit from amplification,

Last 25 years of CI - Broadening Adult Indications

❖ Moderate-to-Profound low-freq hearing loss & mid-to-

high-frequency hearing loss

❖ Aided speech recognition up to 50% in ear to be

implanted, up to 60% in bilateral aided conditions

❖ Pre- and post-lingually deafened adults - no upper limit

age restriction for adults

Last 25 Years of CI - Surgical Advances

❖ Minimally invasive cochlear implants

❖ Direct Cochlear Access (no mastoidectomy, no wide posterior

tympanotomy)

❖ Utilizes robotic drilling + image registration

❖ Residual hearing preservation in CI

❖ atraumatic RW insertion

❖ robotic micro-drill technique

❖ Electric Acoustic Stimulation (EAS)

Assadi et al, 2013; Williamson et al, 2014; Gurbani et al, 2014

‘Slim’ Versus ‘Hybrid’ Electrode CI from Cochlear

The AOS Electrode from Cochlear

Special Electrodes from Cochlear

Otological Rehabilitation Advances: The ABI Implant

❖ 1st implanted 1979 at HEI, for

NF2 cases

❖ Original implantation near

surface of cochlear nucleus

❖ From 2 >> 8 >> 21 electrodes

of Cochlear ABI

❖ 12 electrodes Med El ABI

❖ 16 Electrodes Clarion-1.2

(Advanced Bionics)

❖ Only 1,500 implanted to date

❖ Indications for ABI

❖ Acoustic Neuroma

❖ Cochlear Nerve

Avulsion

❖ Completely ossified

cochlear

Cochlear Vs Med El Auditory Brainstem Implants

Auditory Midbrain Implants

❖ Electrodes inserted to stimulate central nucleus of

inferior colliculus

❖ for those with

❖ little benefit from CI or ABI

❖ after surgery for bilateral acoustic neuroma

❖ Variable outcomes

Advances in Rehabilitation in Otology in Africa Over Past 25 Years - Published Otological Surgeries

0

4.5

9

13.5

18

Nigeria S/Africa Egypt Rest

Tympanoplasty Surgeries in Africa

0

0.25

0.5

0.75

1

1.25

Nigeria Egypt S/Africa Rest

Tympanomastoidectomy in Africa

Otological Rehabilitation in Africa - Published Minor Otological Surgries

0

0.75

1.5

2.25

3

3.75

Nigerian S/Africa

Myringotomy

0

1.75

3.5

5.25

7

8.75

Nigeria Egypt S/Africa Rest

Myringoplasty

Published Studies: Otological Flaps in Otological Rehabilitation in Africa

0

1

2

3

4

5

Nigeria S/Africa Egypt Rest

Hearing Aids Technological Advances in Africa

❖ HA distribution system

❖ Good in Egypt

❖ fair in S/Africa - 25% discount

❖ Non-existent in Nigeria, elsewhere

❖ No personalization of choice & fitting

❖ Personalized assembly still being discussed

Source: WHO

Advances in Rehabilitation in Otology - Vestibular Rehabilitation

❖ Normally functioning vestibular system ensures

❖ upright stance and locomotion

❖ head & eye stabilization

❖ internal spatial representation

❖ Practical advances in rehab of vestibular defective patients targets

❖ rehabilitation at early stage

❖ active retraining

❖ adaptive exercises over stereotyped rehab programs

❖ standardized environment examination

❖ both static & dynamic tests used

❖ avoids/limits use of drugs with sedative effects

Lacour M, 2006 - Curr Med Res Opin. 2006 Sep;22(9):1651-9.

Current Rehabilitation Algorithm

❖ Is it Vertigo?

❖ If yes is it for a peripheral or central vestibular disease?

❖ If peripheral vestibular disease, which one? - Common

thing occur commonly

❖ Is it treatable by medical or surgical means, or is

rehabilitation needed?

❖ Which rehabilitation method should be employed and

how often?

Current Vestibular Assessment

• Balance Test: (vestibulospinal

tract)• Romberg, Tandem Romberg

• Tandem gait (Acute or Chronic?)

• Evaluation of Basic Eye

Movement:• Nystagmus (saccadic, vestibular, pendular,

congenital, alternating)

• Saccades, pursuit, vergence, gaze

• Fixation Suppression

• Vestibulo-Ocular Reflex (VOR)

• Head Shake Test / Rapid Doll

Eye Movt Test

• Fistula Test (labyrinthine fistula)

• Vibration of SCM with Video

Frenzel(Unilateral vest. Lesion)

• Dynamic & Static Positional

Tests: Dix-HallPike Test

Tests carried out in clinician’s office

• ENG/VNG Test battery

(composed of saccadic,

gaze, pursuit, optokinetic-

eye movement, head-shake

nystagmus, positional

nystagmus, positioning

nystagmus, and bithermal

caloric tests)

• Rotational Tests

• Posturography

• CT Scan

• MRI Scan

• Audiometry

• Blood Tests

Additional Tests

Advances in Vestibular Assessment

❖ 3D fluid-attenuated inversion recovery MRI in diagnosis of

Endolymphatic hydrops

❖ potentials of intravenous contrast MRI in imaging of ear

began 2010 with visualization of post gentamicin injection

❖ Since expanded to ELH visualization / comparison of IV

and IT contrast MRI

❖ Vestibular Evoked Myogenic Potential (VEMP)

❖ Cochlear Hydrops Analysis Masking Procedure (CHAMP)

Vestibular Evoked Myogenic Response (VEMP)

❖ neurophysiological means of assessing otolith organs

function, especially saccule (slight sound sensitivity)

❖ Sound-evoked VEMP recorded from the neck usu

unilateral

❖ CHL obliterates VEMP, SNHL does nothing to VEMP

82 YO Male suspected MD: T2W 3D-FLAIR MRI

Courtesy of Naganawa S et al, 2012: Magn Reson Med Sci, Vol 9, No4, pp 237-242, 2010

46 YO Male with Bilateral MD: T2W 3D-FLAIR Compared to Other Protocols

Courtesy of Naganawa S et al, 2012: Magn Reson Med Sci, Vol 9, No4, pp 237-242, 2010

Cochlear Hydrops Analysis Masking Procedure (CHAMP)

❖ Modified standard ABR with click stimulus mixed with increasing

high-pass masking noise

❖ Cut off frequency of the masking successively lowered from one

runs to another, progressively masking lower frequency freq.

regions of cochlea

❖ Interpretation:

❖ Normal: latency of wave V prolonged with increasing masking

❖ Cochlear Hydrops: Little or no shift in latency of Wave V

Advances in Vestibular Rehabilitation

❖ Vestibular Rehabilitation Therapy (VRT)

❖ stable lesion, incomplete recovery

❖ Surgery / Vestibular Implants

❖ Unstable lesion, episodic, spontaneous

Vestibular RT - Basis

❖ Physical Therapy

❖ uses specialized

individualized exercises that

>> gaze/gait stabilization

❖ Use existing neural mechanisms

in human brain for adaptation,

plasticity and compensation

❖ Exercises: habituation, CRP,

VOR, static/dynamic balance

exercises

Ankle Stabilization Strategy

Goals of VRT

• Improve balance

• Minimize falls

• Decrease subjective sensations of

dizziness

• Improve stability during

locomotion

• Reduce overdependency on visual

and somatosensory inputs

• Improve neuromuscular

coordination

• Decrease anxiety and

somatization due to vestibular

disorientation Head Stabilization Strategy

VRT - What is New?

❖ Use of Virtual Reality in VRT

❖ Customized VRT programs can be provided

❖ Situations that cause difficulty in everyday living can

be artificially recreated to help >> vestibular

compensation

❖ Exercises can also be admitted remotely

Virtual Reality Vs Vest. Rehab Protocol

❖ Virtual reality-augumented rehabilitation

❖ Conventional exercises based on VR equipment at

office clinic/home + regime of simulated virtual rehab

exercises

❖ Virtual reality-based therapy

❖ No conventional exercises / New virtual rehab

approaches >> recovery

Virtual Reality Screen Interfaces

❖ Feedback-centered interaction

❖ e.g. threadmill training in front of a screen

❖ Gesture-based interaction

❖ e.g. specialized camera to capture point of reference of a target

during several motions/captured image projected in real time to

facilitate interaction btw target/virtual environment (like Wii)

❖ Haptic stimuli-based interaction

❖ enables user to perceive mechanical stimuli inside a virtual

environment (like sensory gloves)

Coutesy Valeska Gatica-Rojas et al, Neural Regen Res. Apr 15, 2014; 9(8): 888–896.

Virtual Reality Devices Used In Vestibular Rehabilitation

Over 33 publications to date

on Virtual Reality in

Vestibular Rehabilitation!!!

Vestibular Implants

❖ Fairly new approach to bilateral vestibular

failure

❖ Implants device similar to CI / can be hybrid

for SNHL coexisting with bilat vestibular failure

❖ Implanted component either connected

extravestibular or intravestibular (within the

SCC)

❖ External component has gyroscopes and

accelerometers that register and process head

movements.

❖ VI research over a decade old, first successful

human implants 2012

Image courtesy medgadget.com

Regeneration Medicine & Otology

❖ Stem cells research attempts to reconcile ‘Why HCs

regenerate in birds but not in mammals’ was driving force

❖ Several centers in USA / Japan at forefront of

groundbreaking research that could provide ‘cure’ for

hearing loss

❖ Autologous marrow cells / cord blood stem cells have been

implanted into inner ear of lab animals*

❖ Challenges have been getting regenerated stem cells to link

up with auditory nerve

First FDA-approved autologous cord-blood stem cells transplant given 2012

Summary

❖ Otology has advanced, and will continue to advance

based on available medical technology at each age

❖ Our ability to key in and keep abreast of advances in

otology will rely on how much we interface otology with

advances in medical technology

❖ Otolaryngologists should get out of their secluded

pedestal and team up with basic scientists /

technologists to help advance Otology