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Recent Advances in Cochlear
    Implant Candidacy


       BY: DR AMIRA EL
          SHENNAWY


     ASSISTANT PROFESSOR
              OF
          AUDIOLOGY
       CAIRO UNIVERSITY
History Of Cochlear Implantation



 1800 Alessandro Volta
 1957 Djourno & Eyries
 1961 Dr Wiliam F House
 1970s
 1984 FDA approved single channel device in adults
What is a
Cochlear
Implant ?
Evolution of Cochlear implant
Current Implant Technology

Three companies currently have FDA approved implants
   Advanced Bionics (California) —HR90 K
   Cochlear (Australia) —Nucleus 5
   Med-El (Austria) —Sonata ti100
 Candidacy for CI has changed gradually but
 significantly since the first multichannel devices
 were implanted in the late 1970s .

 Due to increasing experience, improvement of
 technology and the proven reliability, the selection
 criteria are broadened with shifting borders.

 Main extensions are related to age, additional
 handicaps, residual hearing & special etiologies of
 deafness.
 The obvious goal for careful selection of CI
 patients is to never have a single patient
 perform more poorly with their CI than they
 did with their hearing aids (Gifford,H 2011).
Stages in Patient Selection

 I. Questionnaire
      Age - Aetiology
      Onset of HL ( pre , peri , postlingual )
      Duration of sensory deprivation ( plasticity )
      Hearing aid use
  II. Audiological investigations
  III. Language assessment
  IV. Radiological examination
 V. Psychological assessment
     IQ testing
 VI. Vestibular testing
 VII. Medical & Otological examination
 VIII. Electrical stimulation of the cochlea


                Counseling…………..
Pediatric CI Candidacy
Team Approach
                Teacher

                              Surgeon &
Psychologist
                             Pediatrician

                 Child


    SLP                      Caregivers


               Audiologist
 FDA approval for implanting children:
    1990 > 2 years
    1998 = 18 months
    2000 = 12 months
 Changes in age of implantation are due to:
1.Better / early identification of HL (universal
hearing screening )
 2.Increased public awareness
 3. Increased professional awareness
 4.Changes in technology :
  Electrode array – Programming – Processors
    Telemetry
 The literature has demonstrated that in terms of
 speech development and language acquisition , the
 best results come from children implanted under the
 age of 2 years similar to normal hearing children
 (Sharma et al ., 2002 ).

 All CI devices can be safely indicated for children 12
  months or older .
 However, implanting children younger than 12
  months remains controversial.
 Further reductions in age at implantation are currently
  limited by the nature of audiologic testing in very young
  children.

 In cases of HL due to meningitis or hereditary hearing loss ,
  implantation should be considered before the age of 12
  months.
Audiological evaluation


 Behavioral audiometry ( age appropriate )
 Tympanometry & acoustic reflexes
 ABR , ear specific & frequency specific
 ASSR
 OAE
 Aided free field testing
 Special tests : EABR , EMLR , ESR, Electrical late
 event related potentials.
Audiometric Thresholds


 For children aged 12-23 months hearing threshold
 for both ears should equal or exceed 90 dB.

 For individuals older than 24 months hearing
 threshold should equal or exceed 70 dB.

 Speech detection with best fit hearing aids in a sound
 field equal to or worse than 55 dB.
 Hearing aid trial for 3 months.


 During HA trial , child should be making at least
 month to month auditory progress as well as speech
 and language progress. If this is not the case then, CI
 evaluation should be considered ( Gifford, H 2011 ).

 Teenagers ???
 Speech & Language assessment


 Psychological assessment , IQ testing


 Counseling , family support , motivation & realistic
 expectations.

 Medical and Otological examination
Radiological assessment:
CT scan traditionally is the gold-standard imaging modality
    Superior visualization of the bony structure of the otic capsule
    and the course of the facial nerve .
   Weakness: can miss cochlear fibrosis, retrocochlear pathology, CNS
    abnormalities, and cochlear nerve hypoplasia/absence


Magnetic resonance imaging (MRI)
   More effective at identifying cochlear fibrosis
   Able to identify presence/absence of cochlear nerve and caliber
   Weakness: inferior visualization of bony anatomy, inability to detect
    the presence of the round window, oval window, or an enlarged
    vestibular aqueduct; often requires anesthesia for young patients
Vaccination

 Children with cochlear implants are at higher risk for
  meningitis, though overall rate is low (<0.6%)

 Streptococcus pneumoniae has been the most common
  organism isolated in the children with cochlear implants who
  developed meningitis

 Current vaccine recommendations:
     Patients <2 years old
         Prevnar (7-valent) only
     Patients 2-5 years old
         Prevnar and Pneumovax (23-valent)
     Patients >5 years old
         Pneumovax only
     Additionally, all patients <5 year old should receive the Hib vaccine
 Vaccination should be completed at least 2 weeks prior to
  surgery
Adult CI Candidacy criteria
 History :
 Onset & course of hearing loss
 Duration (plasticity)
 Aetiology ( fever , trauma )
 History of HA use
 Means of communication
 Education
 Motivation , realistic expectations
                    Prelingual adults ???????
When to refer an Adult for a CI?
• Bilateral severe to profound sensorineural hearing
  loss
• Limited benefit from appropriate hearing aids i.e.
  poor speech recognition
• Telephone use is difficult, limited or impossible
• Patient relies heavily on speech reading or note
  writing to understand speech
• Patient is distressed by the inability to communicate
  efficiently on a daily basis
• No medical contraindications
 Speech recognition testing is the cornerstone in adult
 CI patient selection,

 Where the best-aided scores on open-set sentence
 tests of <50% in the ear to be implanted and <60%
 in contralateral ear is considered as an indication for
 implantation.
Choosing the most appropriate ear

 Audiological factors :
1. Residual hearing
  In the early years of CI , the audiologically worse ear was
 chosen so that (un) expected iatrogenic cochlear damage
 would have fewer consequences.

  In the late 1990s the better ear with the most residual
 hearing was preferred as studies had shown that better
 preserved peripheral neural pathways would lead to better
 results after CI.
2. Duration of HL
It is generally accepted that duration of deafness has
a negative effect on CI performances.

Most clinicians choose the ear with the shortest
duration of deafness.

 3. Only functioning labyrinth
 Surgical Factors
   1.Anatomic variations : cochlear ossification,
 cochlear malformation and cochlear nerve
 malformation.

  2.Otological medical history : otitis media ,
 cholesteatoma and temporal bone fracture.
 Personal factors
   Handedness , (right ear advantage)
Contraindications for implantation

 Completely atretic VIII nerve
   Small internal auditory canal syndrome

 Agenesis of cochlea: Michel deformity
 Active middle ear/mastoid infection
 Tympanic membrane perforation
 Severe organic brain dysfunction
 Severe mental retardation
 Psychosis, unrealistic expectations
Hybrid CI (Electro Acoustic Stimulation)


 The expanded criteria have led to research questions
 centering on advanced uses of the technology.

 Specifically, could an implant benefit other users
 previously not considered to be a candidate?

 A group that was felt to be underserved with
 conventional amplification were those patients with
 the following audiometric profile
 Audiogram
  Below 1.5 kHz – No or moderate HL
  Above 1.5 kHz – Severe to profound sensorineural
  hearing impairment.
  Dead regions of the cochlea (elderly) .
 Speech scores
  The patient's monosyllable word score should be ≤
  60% at 65dB SPL in the best aided condition.
 Hearing preservation surgery


 Two methods are commonly used for inserting the
 electrode into the cochlea:
 Round-window insertion VS
 Cochleostomy insertion.

 Round-window insertion has found a wider
 acceptance because it is considered to be less
 traumatic (controversial).
 EAS electrodes for cochlear implants :
 Long-term research has shown that mechanical flexibility
  of the electrode array is one of the key factors for
  preserving residual hearing.

 Studies with different lengths of electrodes have shown that
  an insertion depth of 10 mm has a good chance of
  preserving residual.

 Electrodes that can be inserted to a depth of 18–
  22 mm are a good compromise.
EAS audio processors
 Combines cochlear implant technology with a digital
  hearing aid. This device uses one microphone for the
  input, but has two separate digital sound processors
  for differentiated processing.
 The parallel processing of these signals, however, is
  performed separately and optimized for both
  acoustic hearing (focusing on low-frequency hearing)
  and cochlear implant stimulation (focusing on high-
  frequency hearing).
The hearing aid is integrated in the ear hook and the
  amplified signals are forwarded to the auditory
  pathway via an ear mould.
 The ear mould used for the acoustic component is
  similar to a conventional hearing aid ear mould and
  can be exchanged.
Bilateral CI

 Recent Trend towards BILATERAL use of CI/s
   -- 1992: 0-1%
   -- 2007: 14-15%

 70% of bilateral CI usage is among 18 years and under age
  group.

 Simultaneous CI


 Sequential CI
 Advantages of bilateral implantation

 Improved hearing in quiet (binaural summation)
 Improved hearing in noise (binaural squelch, head shadow
    effect, and binaural redundancy)
   Improved sound lateralization
   Improved sound localization
   Assurance that the ―better hearing ear‖ is
    implanted/‖captured‖
   Qualitative listening improvement (more ―balanced‖;
    ―richer quality‖; more ―confident‖ feeling; and less fatigued)
Disadvantages
 Increased costs (2 devices, batteries, etc.)
 Multiple pieces of equipment to manage
 Surgical and medical risks
 Future developments
 No or limited ―natural‖ hearing remaining
 Different processing strategies & speech
  processors (with sequential bilateral CIs)
Bimodal stimulation

  CI in one ear and HA in the other.
 Binaural stimulation
 Residual hearing in contralateral ear
 After established electrical stimulation
 Balancing between the two ears
 Future technology
 Cost effectivness
Special Etiologies
 Meningitis:

 9% of childhood deafness.
 Commonest organism to cause HL is S pneumoniae.
 Labyrinthitis ossificans.
 Implantation before 12 months of age.

Trauma:
  BILATERAL OTIC CAPSULE FRACTURES ARE
  UNCOMMON

 Intraluminal fibrosis or ossification may occur which
 makes electrode insertion difficult.
 Hyperbilirubinemia
   risk of auditory neuropathy.

Auditory neuropathy /dyssynchrony:
  Many clinicians have been conservative about the
 outcome.
  Sydney CIC has the most experience.
  They reported variable outcome due to wide
 variability of impairments.
Many of the children had successful implantation
  with a smaller number failing to gain significant
  benefit.
75 % of the patients benefited from the CI due to
  surviving OHCs when IHCs are compromised.
Patients who did not benefit ,may have dysfunction
  in afferent neural synapses, CN or higher auditory
  systems.
During patient selection, electrically evoked CAP
  should be tested .
Usher Syndrome
 Most common cause of blindness in humans.
 Autosomal recessive
 Type I (USH1) most severe 30- 40 % :
 Severe to profound congenital HL , motor
  developmental delay & progressive retinopathy.
 Early implantation is critical to developing
  effective oral – auditory skills prior to visual loss.
Autosomal syndrome.


Hyperplasia of
eyebrow,

heterochromia iridis,
white forlock,
Variable SNHL.




Increased incidence
of auditory             Waardenburg Syndrome
neuropathy
 Keratitis Icthiosis Deafness Syndrome (KID)
Rare congenital disorder of the ectoderm.
Heterogeneous mutation in the Connexin 26 gene
Autosomal dominant.
Congenital icthyosis , vascular keratits , SNHL ,
 alopecia and squamous cell carcinoma may occur.
CI produces good audiological results BUT Wound
 complications are very common , failure to heal,
 partial extrusion of the implant.
Multi handicapped

 Patients with additional disabilities such as mild
  motor disability, cerebral palsy , cognitive
  disabilities, specific learning disabilities, behavioral
  disorders and sight impairment have been
  implanted.
 Multi-handicapped children receive benefit from
  cochlear implantation. The rate of this improvement
  is slow but offers better quality of life due to better
  auditory-communication skills, better self-
  independence and social integration.
Recent Trends
 RECENT TRENDS
C I in Unilateral Deafness

 Up to now treatment modalities for single sided
  deafness are; NO treatment , Conventional
  contralateral routing of signal or BAHA.
 CI makes a new treatment modality for those
  patients.
 Study done by Arndt et al., 2011 revealed that CI
  improved hearing abilities in single sided HL &
  superior to alternative options. CI didn’t interfere
  with speech understanding in the normal ear.
CI in Unilateral deafness and tinnitus

 Tinnitus is a frequent often disabling condition.
 In patients who are deaf with tinnitus in the affected
  ear, treatment based on acoustic input are
  impossible.
 Tinnitus suppression using electric stimulation has
  been reported to be successful (Buechner et
  al.,2010).
 Several studies (Kleinjung 2009: Van de Heyning et
  al., 2008 and Moller 2003 ) concluded that CI may
  represent a chance for complete suppression of
  tinnitus in selected cases.
CHALL
ENGES
Audiological


 Mapping very young children:
  difficulty in obtaining behavioral results’
 evolution of recent technology helps assist in
 mapping through the use of ECAP measurements.


 Mapping the multi handicapped.
Surgical Challenges

           Implanting very young children
 Children below 12 months usually have poorly pneumatized
  mastoid bones leading to greater intraoperative blood loss
  and risk of facial nerve injury.

 Greater anesthesia risk ,size of airway & difficulty
  maintaining cardiovascular fluid & temp homeostasis.

 Thin scalp : care in drilling well for body of device.

 Increased incidence of otitis media

 Fortunately, cochlea is adult size at birth.
Hearing preservation surgery
 A special surgical technique to preserve the residual
  hearing of the patient (in most routine cochlear
  implant surgeries, any residual hearing will likely be
  destroyed).
 This is a very realistic goal for many patients with
  sloping hearing loss (EAS ).

 Achieved by performing ―Soft surgery‖
Dysplastic Cochlea
Due to increased knowledge of temporal bone
  anatomy and improved imaging techniques more
  patients with Mondinin dysplasia , Common
  cavity, hypoplastic cochlea and large vestibular
  aqueducts are implanted.
Modifications in surgical techniques.
Likelihood of CSF gusher .
Labyrinthitis Ossificans
Consequence of meningitis
Ossification partially or completely block the lumen
 of scala tympani & or scala vestibuli.
Several techniques:
Drilling a basal tunnel , circum-modiolar drill-out,
 use of double or split electode array.
Bilateral Implantation
Lengthy, bilateral loss of vestibular function ,
 contamination of the field.

Monopolar cautery can not be used for the second
 side
New Devices
If and when the totally implantable cochlear
  implant (TICI) becomes a reality, it will
  require a modification of current surgical
  techniques to implant a microphone and
  possibly adding hardware to the ossicles.
 Overall the selection criteria have
  been broadened with increasing
  experience and technological
  improvement.
 This development may continue
  and the borderline between HA &
  CI will shift further.
 However, the basis for success
  still remains good rehabilitation,
  a team approach and the
  willingness of the patient to
  undergo the whole process of CI.
Recent Advances in Cochlear Implant Candidacy

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Recent Advances in Cochlear Implant Candidacy

  • 1. Recent Advances in Cochlear Implant Candidacy BY: DR AMIRA EL SHENNAWY ASSISTANT PROFESSOR OF AUDIOLOGY CAIRO UNIVERSITY
  • 2. History Of Cochlear Implantation  1800 Alessandro Volta  1957 Djourno & Eyries  1961 Dr Wiliam F House  1970s  1984 FDA approved single channel device in adults
  • 4.
  • 5.
  • 7. Current Implant Technology Three companies currently have FDA approved implants Advanced Bionics (California) —HR90 K Cochlear (Australia) —Nucleus 5 Med-El (Austria) —Sonata ti100
  • 8.  Candidacy for CI has changed gradually but significantly since the first multichannel devices were implanted in the late 1970s .  Due to increasing experience, improvement of technology and the proven reliability, the selection criteria are broadened with shifting borders.  Main extensions are related to age, additional handicaps, residual hearing & special etiologies of deafness.
  • 9.  The obvious goal for careful selection of CI patients is to never have a single patient perform more poorly with their CI than they did with their hearing aids (Gifford,H 2011).
  • 10. Stages in Patient Selection  I. Questionnaire Age - Aetiology Onset of HL ( pre , peri , postlingual ) Duration of sensory deprivation ( plasticity ) Hearing aid use II. Audiological investigations III. Language assessment IV. Radiological examination
  • 11.  V. Psychological assessment IQ testing  VI. Vestibular testing  VII. Medical & Otological examination  VIII. Electrical stimulation of the cochlea Counseling…………..
  • 13. Team Approach Teacher Surgeon & Psychologist Pediatrician Child SLP Caregivers Audiologist
  • 14.  FDA approval for implanting children: 1990 > 2 years 1998 = 18 months 2000 = 12 months
  • 15.  Changes in age of implantation are due to: 1.Better / early identification of HL (universal hearing screening ) 2.Increased public awareness 3. Increased professional awareness 4.Changes in technology : Electrode array – Programming – Processors Telemetry
  • 16.  The literature has demonstrated that in terms of speech development and language acquisition , the best results come from children implanted under the age of 2 years similar to normal hearing children (Sharma et al ., 2002 ).  All CI devices can be safely indicated for children 12 months or older .  However, implanting children younger than 12 months remains controversial.
  • 17.  Further reductions in age at implantation are currently limited by the nature of audiologic testing in very young children.  In cases of HL due to meningitis or hereditary hearing loss , implantation should be considered before the age of 12 months.
  • 18. Audiological evaluation  Behavioral audiometry ( age appropriate )  Tympanometry & acoustic reflexes  ABR , ear specific & frequency specific  ASSR  OAE  Aided free field testing  Special tests : EABR , EMLR , ESR, Electrical late event related potentials.
  • 19. Audiometric Thresholds For children aged 12-23 months hearing threshold for both ears should equal or exceed 90 dB.  For individuals older than 24 months hearing threshold should equal or exceed 70 dB.  Speech detection with best fit hearing aids in a sound field equal to or worse than 55 dB.
  • 20.  Hearing aid trial for 3 months.  During HA trial , child should be making at least month to month auditory progress as well as speech and language progress. If this is not the case then, CI evaluation should be considered ( Gifford, H 2011 ).  Teenagers ???
  • 21.  Speech & Language assessment  Psychological assessment , IQ testing  Counseling , family support , motivation & realistic expectations.  Medical and Otological examination
  • 22. Radiological assessment: CT scan traditionally is the gold-standard imaging modality Superior visualization of the bony structure of the otic capsule and the course of the facial nerve .  Weakness: can miss cochlear fibrosis, retrocochlear pathology, CNS abnormalities, and cochlear nerve hypoplasia/absence Magnetic resonance imaging (MRI)  More effective at identifying cochlear fibrosis  Able to identify presence/absence of cochlear nerve and caliber  Weakness: inferior visualization of bony anatomy, inability to detect the presence of the round window, oval window, or an enlarged vestibular aqueduct; often requires anesthesia for young patients
  • 23.
  • 24. Vaccination  Children with cochlear implants are at higher risk for meningitis, though overall rate is low (<0.6%)  Streptococcus pneumoniae has been the most common organism isolated in the children with cochlear implants who developed meningitis  Current vaccine recommendations:  Patients <2 years old  Prevnar (7-valent) only  Patients 2-5 years old  Prevnar and Pneumovax (23-valent)  Patients >5 years old  Pneumovax only  Additionally, all patients <5 year old should receive the Hib vaccine  Vaccination should be completed at least 2 weeks prior to surgery
  • 25. Adult CI Candidacy criteria
  • 26.  History :  Onset & course of hearing loss  Duration (plasticity)  Aetiology ( fever , trauma )  History of HA use  Means of communication  Education  Motivation , realistic expectations Prelingual adults ???????
  • 27. When to refer an Adult for a CI? • Bilateral severe to profound sensorineural hearing loss • Limited benefit from appropriate hearing aids i.e. poor speech recognition • Telephone use is difficult, limited or impossible • Patient relies heavily on speech reading or note writing to understand speech • Patient is distressed by the inability to communicate efficiently on a daily basis • No medical contraindications
  • 28.
  • 29.
  • 30.  Speech recognition testing is the cornerstone in adult CI patient selection,  Where the best-aided scores on open-set sentence tests of <50% in the ear to be implanted and <60% in contralateral ear is considered as an indication for implantation.
  • 31. Choosing the most appropriate ear  Audiological factors : 1. Residual hearing In the early years of CI , the audiologically worse ear was chosen so that (un) expected iatrogenic cochlear damage would have fewer consequences. In the late 1990s the better ear with the most residual hearing was preferred as studies had shown that better preserved peripheral neural pathways would lead to better results after CI.
  • 32. 2. Duration of HL It is generally accepted that duration of deafness has a negative effect on CI performances. Most clinicians choose the ear with the shortest duration of deafness. 3. Only functioning labyrinth
  • 33.  Surgical Factors 1.Anatomic variations : cochlear ossification, cochlear malformation and cochlear nerve malformation. 2.Otological medical history : otitis media , cholesteatoma and temporal bone fracture. Personal factors Handedness , (right ear advantage)
  • 34. Contraindications for implantation  Completely atretic VIII nerve  Small internal auditory canal syndrome  Agenesis of cochlea: Michel deformity  Active middle ear/mastoid infection  Tympanic membrane perforation  Severe organic brain dysfunction  Severe mental retardation  Psychosis, unrealistic expectations
  • 35. Hybrid CI (Electro Acoustic Stimulation)  The expanded criteria have led to research questions centering on advanced uses of the technology.  Specifically, could an implant benefit other users previously not considered to be a candidate? A group that was felt to be underserved with conventional amplification were those patients with the following audiometric profile
  • 36.
  • 37.  Audiogram Below 1.5 kHz – No or moderate HL Above 1.5 kHz – Severe to profound sensorineural hearing impairment. Dead regions of the cochlea (elderly) .  Speech scores The patient's monosyllable word score should be ≤ 60% at 65dB SPL in the best aided condition.
  • 38.  Hearing preservation surgery  Two methods are commonly used for inserting the electrode into the cochlea: Round-window insertion VS Cochleostomy insertion.  Round-window insertion has found a wider acceptance because it is considered to be less traumatic (controversial).
  • 39.  EAS electrodes for cochlear implants :  Long-term research has shown that mechanical flexibility of the electrode array is one of the key factors for preserving residual hearing.  Studies with different lengths of electrodes have shown that an insertion depth of 10 mm has a good chance of preserving residual.  Electrodes that can be inserted to a depth of 18– 22 mm are a good compromise.
  • 40. EAS audio processors  Combines cochlear implant technology with a digital hearing aid. This device uses one microphone for the input, but has two separate digital sound processors for differentiated processing.  The parallel processing of these signals, however, is performed separately and optimized for both acoustic hearing (focusing on low-frequency hearing) and cochlear implant stimulation (focusing on high- frequency hearing).
  • 41. The hearing aid is integrated in the ear hook and the amplified signals are forwarded to the auditory pathway via an ear mould.  The ear mould used for the acoustic component is similar to a conventional hearing aid ear mould and can be exchanged.
  • 42. Bilateral CI  Recent Trend towards BILATERAL use of CI/s -- 1992: 0-1% -- 2007: 14-15%  70% of bilateral CI usage is among 18 years and under age group.  Simultaneous CI  Sequential CI
  • 43.  Advantages of bilateral implantation  Improved hearing in quiet (binaural summation)  Improved hearing in noise (binaural squelch, head shadow effect, and binaural redundancy)  Improved sound lateralization  Improved sound localization  Assurance that the ―better hearing ear‖ is implanted/‖captured‖  Qualitative listening improvement (more ―balanced‖; ―richer quality‖; more ―confident‖ feeling; and less fatigued)
  • 44. Disadvantages Increased costs (2 devices, batteries, etc.)  Multiple pieces of equipment to manage  Surgical and medical risks  Future developments  No or limited ―natural‖ hearing remaining  Different processing strategies & speech processors (with sequential bilateral CIs)
  • 45. Bimodal stimulation CI in one ear and HA in the other.  Binaural stimulation  Residual hearing in contralateral ear  After established electrical stimulation  Balancing between the two ears  Future technology  Cost effectivness
  • 47.  Meningitis: 9% of childhood deafness. Commonest organism to cause HL is S pneumoniae. Labyrinthitis ossificans. Implantation before 12 months of age. Trauma: BILATERAL OTIC CAPSULE FRACTURES ARE UNCOMMON Intraluminal fibrosis or ossification may occur which makes electrode insertion difficult.
  • 48.  Hyperbilirubinemia risk of auditory neuropathy. Auditory neuropathy /dyssynchrony: Many clinicians have been conservative about the outcome. Sydney CIC has the most experience. They reported variable outcome due to wide variability of impairments.
  • 49. Many of the children had successful implantation with a smaller number failing to gain significant benefit. 75 % of the patients benefited from the CI due to surviving OHCs when IHCs are compromised. Patients who did not benefit ,may have dysfunction in afferent neural synapses, CN or higher auditory systems. During patient selection, electrically evoked CAP should be tested .
  • 50. Usher Syndrome  Most common cause of blindness in humans.  Autosomal recessive  Type I (USH1) most severe 30- 40 % :  Severe to profound congenital HL , motor developmental delay & progressive retinopathy.  Early implantation is critical to developing effective oral – auditory skills prior to visual loss.
  • 51. Autosomal syndrome. Hyperplasia of eyebrow, heterochromia iridis, white forlock, Variable SNHL. Increased incidence of auditory Waardenburg Syndrome neuropathy
  • 52.  Keratitis Icthiosis Deafness Syndrome (KID) Rare congenital disorder of the ectoderm. Heterogeneous mutation in the Connexin 26 gene Autosomal dominant. Congenital icthyosis , vascular keratits , SNHL , alopecia and squamous cell carcinoma may occur. CI produces good audiological results BUT Wound complications are very common , failure to heal, partial extrusion of the implant.
  • 53. Multi handicapped  Patients with additional disabilities such as mild motor disability, cerebral palsy , cognitive disabilities, specific learning disabilities, behavioral disorders and sight impairment have been implanted.  Multi-handicapped children receive benefit from cochlear implantation. The rate of this improvement is slow but offers better quality of life due to better auditory-communication skills, better self- independence and social integration.
  • 55. C I in Unilateral Deafness  Up to now treatment modalities for single sided deafness are; NO treatment , Conventional contralateral routing of signal or BAHA.  CI makes a new treatment modality for those patients.  Study done by Arndt et al., 2011 revealed that CI improved hearing abilities in single sided HL & superior to alternative options. CI didn’t interfere with speech understanding in the normal ear.
  • 56. CI in Unilateral deafness and tinnitus  Tinnitus is a frequent often disabling condition.  In patients who are deaf with tinnitus in the affected ear, treatment based on acoustic input are impossible.  Tinnitus suppression using electric stimulation has been reported to be successful (Buechner et al.,2010).  Several studies (Kleinjung 2009: Van de Heyning et al., 2008 and Moller 2003 ) concluded that CI may represent a chance for complete suppression of tinnitus in selected cases.
  • 58. Audiological  Mapping very young children: difficulty in obtaining behavioral results’ evolution of recent technology helps assist in mapping through the use of ECAP measurements.  Mapping the multi handicapped.
  • 59. Surgical Challenges Implanting very young children  Children below 12 months usually have poorly pneumatized mastoid bones leading to greater intraoperative blood loss and risk of facial nerve injury.  Greater anesthesia risk ,size of airway & difficulty maintaining cardiovascular fluid & temp homeostasis.  Thin scalp : care in drilling well for body of device.  Increased incidence of otitis media  Fortunately, cochlea is adult size at birth.
  • 60. Hearing preservation surgery  A special surgical technique to preserve the residual hearing of the patient (in most routine cochlear implant surgeries, any residual hearing will likely be destroyed).  This is a very realistic goal for many patients with sloping hearing loss (EAS ).  Achieved by performing ―Soft surgery‖
  • 61. Dysplastic Cochlea Due to increased knowledge of temporal bone anatomy and improved imaging techniques more patients with Mondinin dysplasia , Common cavity, hypoplastic cochlea and large vestibular aqueducts are implanted. Modifications in surgical techniques. Likelihood of CSF gusher .
  • 62. Labyrinthitis Ossificans Consequence of meningitis Ossification partially or completely block the lumen of scala tympani & or scala vestibuli. Several techniques: Drilling a basal tunnel , circum-modiolar drill-out, use of double or split electode array.
  • 63. Bilateral Implantation Lengthy, bilateral loss of vestibular function , contamination of the field. Monopolar cautery can not be used for the second side
  • 64. New Devices If and when the totally implantable cochlear implant (TICI) becomes a reality, it will require a modification of current surgical techniques to implant a microphone and possibly adding hardware to the ossicles.
  • 65.  Overall the selection criteria have been broadened with increasing experience and technological improvement.  This development may continue and the borderline between HA & CI will shift further.  However, the basis for success still remains good rehabilitation, a team approach and the willingness of the patient to undergo the whole process of CI.

Editor's Notes

  1. Sound is received by a microphone located on the BTE sound processor (1); it is processed and coded, then sent via the transcutaneous radiofrequency link to the implanted receiver-stimulator (2); data are decoded and sent to the multi-electrode array (3), stimulating spiral ganglion neurons, which then transmit the signal via the auditory nerve (4) toward higher processing centers
  2. Advanced Bionics—HR90 KCochlear—Nucleus 5Med-El Sonata ti100
  3. Vestibular testing
  4. Promontry and electrical stimulation
  5. ELDERLY
  6. Explain what AN is
  7. Special atraumatic soft electrodes; small cocleostomy care during drilling and insertion. Avoid infection, blood