cochlear implantation for sensorineural hearing...

Cochlear Implantation for Sensorineural Hearing

LossCliff A. Megerian, MD, FACSPogue Chair in Auditory Surgery

Professor of Otolaryngology-Head and Neck Surgery, and Neurological Surgery

Director of Otology and NeurotologyCase Medical Center-UHC

Objectives• Discuss the various degrees of hearing loss

• Review indications for cochlear implantation

• Describe the cochlear implant device• Describe the cochlear implant device• Outline the surgical steps in cochlear implantation

• Discuss postoperative management • Future trends

Etiology for sensorineural hearing loss

• Meningitis• Medications • Genetic - Ushers, Waardenberg• Trauma• Trauma• Congenital inner ear deformities• Autoimmune• Post-natal - NICU, ECMO, anoxia• Aging-Presbyacusis

Hearing loss in the United States• Affects approximately 2 million people• 1:1000 live births• Potential disadvantages– Loss of occupational and educational – Loss of occupational and educational opportunities

– Loss of social opportunity– Isolation– Depression– Personal danger

Newborn screening

• Screening is now universal in Ohio, effective in June 2004

• All newborns have their hearing screened prior to hospital dischargescreened prior to hospital discharge

• Previously only “high-risk” infants were screened, missing nearly one-half of children with significant hearing loss

Newborn screening

• Conducted with automated ABR• Babies who fail their initial screen are re-screened prior to discharge

• Babies who fail their re-screen in • Babies who fail their re-screen in both ears are scheduled for a diagnostic ABR and OAE evaluation prior to discharge

Newborn screening

• Children who are identified with hearing loss and enrolled in an appropriate rehabilitation program by the age of six months develop by the age of six months develop age appropriate language in early childhood and maintain these skills at least through entry in to school

Mild hearing loss

Normal hearing

Moderate hearing loss

Profound hearing loss

Severe hearing loss

Moderate hearing loss

Anatomy

Histopathology of SNHL• Advanced hearing loss related to hair cell loss with reserve of viable nerve fibers

• Average hair cell counts of 14,000 in deafness (normal 35,000 to 40,000)deafness (normal 35,000 to 40,000)

• 65% of surviving hair cells are found between 6 to 22 mm from the round window

• 10,000 neurons with 3,000 apically necessary for speech discrimination

Normal histology

Damaged hair cells

What is a cochlear implant?

• Converts sound energy to electrical energy

• Microphone• External speech processor• External speech processor• Signal transfer hardware• Subdermal receiver• Implantable electrode

How the implant works

History of cochlear implants

• 1950’s Djourno & Eyries – Direct stimulation of the auditory nerve with an electrode

• 1960’s House & Urban • 1960’s House & Urban – Developed a single electrode cochlear implant for stimulation of postlingually deaf adult


• 1970’s – Research showed that intracochlear electrodes could produce sound awareness via spiral ganglion cell awareness via spiral ganglion cell stimulation despite deterioration of the organ of Corti


• 1980’s – First successful implantation of 3M-House single-channel device and Nucleus 22 multi-channel device (tonotopic stimulation)

• 1990’s • 1990’s – FDA approval of Nucleus 22 channel device for children-once language acquisition in pre-lingual children was proven

• 150,000 cases performed to date worldwide

Indications for cochlear implantation

• Infants (12 – 24 mos.)– Profound bilateral SNHL– No benefit from trial of hearing aids– No medical contraindications– No medical contraindications– High motivation and appropriate expectations from family

– Earlier implantation if SHNL secondary to meningitis

Indications for cochlear implantation

• Older Children (25 mos. – 17 yrs.)– Severe-to-profound bilateral SNHL– No benefit from trial of hearing aids– Word recognition scores less than – Word recognition scores less than < 30% in best-aided condition

Indications for cochlear implantationAdults• Prelinguistic or postlinguistic onset of severe to profound hearing loss

• HINT Sentence recognition• < 50% implanted ear • < 50% implanted ear • < 60% nonimplanted ear or binaurally

• No medical contraindications• A desire to be part of the hearing world

The trend appears to be as technology improves, outcomes are better, and as a result, the candidacy criteria expands As seen in the chart, selection criteria have expanded dramatically since clinical trials with cochlear implants began in 1985. Adults and children with increasing amounts of residual hearing, Adults and children with more preoperative open-set speech recognition, Children younger in age, And those with abnormal cochleae, are now considered CI candidates The expanded criteria speaks to the safety of Nucleus systems

Ethics of cochlear implantation

• From the medical point of view, CI is an effective treatment option for a severe disability

• However, to some deaf activists, it represents unwanted technology that threatens their way of lifeof life

• Most viable ethical and moral arguments have resolved once pre-lingual patients shown to develop normal language skills

• Cultural arguments still exist in Deaf community

Preoperative planning• Evaluation by otolaryngologist• Audiology & speech pathology involvement

• Developmental psychologist• Genetic testing - Connexin 26• CT temporal bone– Cochlear abnormalities– Middle ear/mastoid inflammation– Position of jugular bulb/carotid– Internal auditory canal width

Cochlear implant systems

• Nucleus 24 Freedom• Med-El (Medical Electronic) Cochlear ImplantImplant

• Clarion 90-K

Nucleus Freedom Contour Advance

– Cochlear, Ltd. In Sydney, Australia

– Highlights:

• Rounder – easier to implant• Ease of electrode insertion• Ease of electrode insertion• Strong Titanium case• Smaller – less drilling• Removable Magnet MRI compatible up to 1.5 tesla• Self curling, protective ofdelicate structures

ESPrit™

ear level speech processor

SPrint™

body worn

Speech

processor

Nucleus Freedom Contour Advance

• Self-curling electrode array

• 22 half-band electrodeselectrodes

• Adjacent to the inner wall of the cochlea

• Cast pre-curved to regain pre-designed shape & size

Clarion

• Advanced Bionics of California

• Clarion CII Bionic Ear– Platinum digital sound – Platinum digital sound processor

– CII BTE digital microprocessor

Med-El cochlear implant

• Manufactured in Innsbruck, Austria

• Offers three • Offers three types of cochlear implants:– Standard array– Split electrode array

– Compressed array

“This is not brain

surgery”

Incisions for cochlear implant

Planning the incision

Incision exposing bone

Mastoidectomy

Cochleostomy

Drilling the well for the implant

Core design concept

The electrode array is easily inserted with the help of a malleable stylet: 1). The stylet holds the array straight prior to insertion. 2). During insertion the stylet, assumes the shape of the lateral wall and gently guides the array to its final location. 3). After the stylet is removed, the array curls close to the modiolus.

Electrode array in cochlea

Stylet withdrawal

CUT THE BOVIE!!!!

Electrode sewn into bone

Post operative skull film

Surgical considerations

• Cochlear dysplasia– Variable postoperative speech perception results

– Risk for intraoperative complications– Risk for intraoperative complications

• Aberrant facial nerve• Intracochlear ossification

Outcomes

• More than 95% of Deaf children implanted at 1 year obtain open-set speech understanding….Normal language

• Earlier implantation results in greater • Earlier implantation results in greater gains in speech perception

• Approximately 70% of post-lingual deaf CI patients may use the telephone

Cost of cochlear implant ?

Post-operative expectations

• Post-operative anesthesia care • Home going concerns• Questions families may ask• Questions families may ask

Wound care

• Mastoid dressing remains for ~2-3 daysdays

• Patient may shower once dressing is removed

• Absorbable sutures

Questions from families

• When will the patient be able to hear?– The implant is activated 3-4 weeks after surgery by the audiologist

• What type of activities are allowed?– No heavy lifting 2 weeks after surgery– Precautions during contact sports – helmet– Remove CI for water sports, showering

Questions from families

• Is is OK to have an MRI?– Only if there is a removable magnet such as Nucleus 24 device

• Should there be precautions for future surgery?– Monopolar cautery should NOT be used – Biopolar instruments may be used but keep away ~1 cm from implant

Potential complications• Wound complications

Infection HematomaWound dehiscence Skin flap Necrosis

• Facial nerve injury• Facial nerve injury• Exposed electrode• Device failure• Perilymph fistula• Meningitis

Meningitis• US Food and Drug Administration identified a possible association between cochlear implants and bacterial meningitis in 52 people worldwideworldwide

• Ages 21 months to 72 years• Onset < 24 hours to > 5 yrs after implantation

• Majority of organisms S. pneumoniae

Meningitis• Etiology– Nidus to a local infection in patients with bacteremia

– Preoperative otitis media– Immunodeficiencies– Immunodeficiencies– CSF leak

• Immediate workup and treatment• Vaccination

Future Developments

• Smaller devices• Totally implantable cochlear implant• “Short implants” for high frequency hearing losshearing loss

• Bilateral cochlear implantation• Stem cell work• Gene therapy

Electric-Acoustic Stimulation(EAS)

Caution: Investigational device. Limited by Federal (US) law to investigational use.

CAUTION: Investigational device. Limited by law to investigational use

EAS Concept

10

0

-10

0,125 0,50,25 1 2 4 8

frequency [kHz]

110

100

90

80

70

60

50

40

30

20

dB HL

X X

Indication Areas for HA, CI and EAS

• No amplification

necessary

Frequency [kHz]

[dB

HL]

-10

0

10

20

0,125 0,25 0,5 1 2 4 8

77% necessary

• Hearing aid

• EAS indication

• Relative CI indication

• CI indicationN = 23521 Courtesy W. Gstöttner, J. Kiefer, Frankfurt

Thre

shold

[dB

HL]

20

30

40

50

60

70

80

90

100

1100.54%

1.1%

18%

77%

3.4%

New FLEXeas Electrode• Improved mechanical

flexibility

• Requires less insertion

force 1

2

3

Fo

rce

[g] C40+

C40+ flex short

leads to less insertion

trauma

• Thin front-end with 5 single

contacts 1.9mm contact

spacing

01 3 5 7 9 11 13 15 17 19 21 23

Insertion depth [mm]

Insertion Depth in EAS?

• How deep should the electrode be inserted for EAS?

• Deep insertion, covering both the functioning and the non-functioning region of the cochlea?and the non-functioning region of the cochlea?

• Insertion depth covering most of the non-functioning region (abt. 18-20 mm)?

• Insertion only in the straight part of the basal region of the cochlea (abt. 10 mm)?

• Insertion only in the very basal part or only extra-cochlear stimulation ( 6 mm / extra-cochlear)?

Clinical ExperienceFrankfurt Pilot Study

• Residual hearing was preserved in

01020

125 250 500 1000 2000 4000 8000

Frequency [Hz]

Pre-op MeanPre-op Median

N

preserved in 18/21 patients– 13 (0-10 dB)– 5 (11-20 dB)– 3 hearing loss

2030405060708090100110120

dB

HL

Pre-op MedianPost-op MeanPost-op Median

Results

78,0

89,2

76,6

85,0

95,2

80,0

100,0

120,0 Pre- and Postop thresholds for subjects where at least partial hearing was achieved

31,7

43,1

60,6

45,4

58,2

0,0

20,0

40,0

60,0

125 Hz 250 Hz 500 Hz 750 Hz 1000 Hz

FrequencyGstöttner, Adunka, 2004

ResultsAverage speech discrimination scores in EAS subjects

84,4

71,0

83,9

96,0

72,7

60,0

80,0

100,0

Gstöttner,Adunka, 2004

8,4

26,7

13,1

7,0

57,6

37,1

57,9

0,0

20,0

40,0

60,0

monosyllablesquiet

HSM sentences

quiet

HSM sentences15 dB SNR

HSM sentences10 dB SNR

EAS – External Devices

• Two independent devices

• Different number / • Different number / sizes of batteries

• Different battery life-time

• ITE hearing aids at their limits

Design of a Combined EAS-Device

• Combined power supply

• Combined input signal

• Independent compression

(AGC)

• Independent fitting

Combined EAS-Device

• Simplified handling• Increased user comfortcomfort

• Higher amplification than ITE HA

• Flexibility to adapt to EAS learning process

MED-EL EAS Clinical Studies• www.hearingpreservation.com• Frankfurt study (completed) - single-site to explore feasibility, surgical techniques and electrodes

• EAS Europe Multi-Center study (n=30)(ongoing) – to verify efficacy of electric /acoustic stimulation product for EU market

• EAS US Multi-Center IDE Clinical Trial (IDE FDA approved) – to establish efficacy/safety for US market

Fully Implantable Cochlear Implant

• Technically: much more complex than conventional cochlear than conventional cochlear implants

• Surgically: complex but not prohibitively complex

Fully Implantable Fully Implantable


Microphone Design Options

•Acoustic attenuation of the signal as low as possible•Microphone located under the skin •An ear-canal microphone•An ear-canal microphone

Batteries

• Design: 1 day per charge

• Cycle life as long as possible• Charging period as short as possible –• Charging period as short as possible –patient will wear a small external charger for less than one hour a day

• Batteries should not lose significant capacity over time

BilateralBilateral


US Adult Bilateral Cochlear Implantation Study

N=19 at 12 months

13 showed all 3 effects (bilateral advantage)

Head shadow (19), squelch (15) and summation effects (17) were significant

We are very interested in bilateral implantation. To date 150pts have been implanted worldwide, 130 of those, children and adults, were with the MED-EL device in Europe. Early results show improvement in performance in noise, the ability to localize, slightly better performance in quiet and an improved quality of life.Results may have implications for future implant designs that are bilateral specific. We will begin a US protocol post FDA approval of our device.

New Adult Bilateral Studies

• Loudness summation and AGC settings effects

• Extension of SPECT scan study with • Extension of SPECT scan study with bilaterally implanted patients

• Evoked potentials in bilaterally implanted patients

• Psychophysical measures

Pediatric Bilateral Study Pediatric Bilateral Study


Status

•Bilateral vs unilateral performance longitudinally•Speech/language perception and production over time•Localization •Speech in noise

As of Friday, 6 centers have received IRB approval. These include: Nemours Children’s Hospital, Medical College of Wisconsin, Callier, Indiana, and Carle Clinic. Several others are in the final stages. To date, 5 children have been enrolled in the study and have been implanted. 3 have been implanted bilaterally and 2 unilaterally. Study binders were shipped to all centers with IRB approval in early June (they should have received theirs…if not, please let me know).

Drug Delivery SystemsDrug Delivery Systems


Not currently available

Developments in Inner Ear Biology

• New drugs/biological treatments• Genetics• Stem cell implantation • Study of the process of apoptosis

There have been a number of advances in the field of inner ear biology that may open doors to alternative ways to treat deafness or enhance hearing abilities. These include new drugs or biological treatments, genetic treatment, the possibility of stem cell implantation, and intensive study into the process of cell death to better understand how to interrupt that process.

Drug Delivery Systems § Goals: § Preserve spiral ganglion cells§ Prevent apoptosis§ Reduce inflammation and tissue growth§ Reduce inflammation and tissue growth§ Maintain residual hearing

§ Method: § Combine inner ear drug delivery with electrical stimulation in CI patients

The interface between drug delivery and the cochlear implant

• C. Jolly • C. Jolly • G. Reetz• F. Béal • F. Aattendorf• W-D.

Baumgartner • J. Miller •

Not currently available

Implant

System concept

Scala tympani

Scala vestibuli

Spiral ganglion cells Electrode

Drug delivery channel

refillable pump

catheter

2 twin outlets

electrode contacts

Implant housing

connector

Cross section

drug delivery

contactwiredrug delivery channel

double outlets

silicone

outlets

central channel8 mm

ELECTRODE DESIGN(Based on FLEX Technology)

outlets

Modular system is primed and connected

intra-operatively

housingElectrode arrayImplant Catheter

and needlePump

housingElectrode arrayImplant Catheter

and needlePump

Electrode array

housing

--

Embedded micro-port and septum

Electrode array and needleElectrode array

housing

--

Embedded micro-port and septum

Electrode array and needle

Pump and catheter are to be removed after therapy

Conclusions§ Neurotrophic factors preserve spiral

ganglion cells and reduce hearing threshold in animal studies.

§ A fluid-based drug delivery system is technically feasible.technically feasible.

§ Modelling of drug diffusion shows that with two outlets, we achieve a more uniform drug concentration in the cochlea.

§ At 0.5 µl/hr, pressure increase is negligible. § Fluid-based drug delivery offers more

flexibility than surface coating and can be terminated.

Summary

• Cochlear implants are an appropriate sensory aid in deaf children and adults who receive minimal benefit from conventional amplificationconventional amplification

• Future and ongoing research is promising

Thank You!

cochlear implantation for sensorineural hearing...

Documents