Cochlear Implantation in Acquired ANSD: 2 Case Reports

1
Cochlear Implantation in Acquired ANSD 2 Case
Reports

• 3 July 2009
• Neil Heslop, MNZAS-CCC
• Southern Cochlear Implant Programme

2
Case Studies

• Child KS (F) - Personal Information
• Age now 155
• Referred 04.10.2005 Age at referral 109
• Implanted 26.10.2006 Age at implant 119
• Currently 28 post-implant
• Child GV (M) - Personal Information
• Age now 117
• Referred 05.09.2006 Age at referral 89
• Implanted 16.05.2007 Age at implant 95

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Child KS
4
History

• Bouts of serious illness at 1 and 3 years with
  convulsions, balance disturbance, and hearing
  loss
• (? Encephalitis)
• Intermittent hearing aid use over several years
  relies heavily on speech reading and the written
  word no benefit from hearing aids, not worn
  reliably as a result
• EEG at 5 years of age the EEG continues to show
  more beta than one normally sees in a child of
  this age
• Further bout of illness resulting in
  deterioration in hearing, 3 months prior to
  cochlear implant referral

5
Classroom and Social

• Appears to comprehend very little spoken word
  although she copes a lot better when things are
  written down
• Watching what her peers are doing and trying to
  follow their example she watches very closely
  and looks very worried she is appearing very
  stressed in class is now very isolated

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Audiology Pre-Implant
Serial Audiometry 2001 2005
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Audiology Pre-Implant

• robust OAE responses in both ears consistent
  with good cochlea sensory cell function
• Cortical responses right but not left
• ABR Reliable and repeatable cochlear
  microphonics were found in both ears

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CI Assessment
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Speech Perception Pre-Implant

• HINT-C sentences A-V A-A
• Unaided 88 33
• Aided 96 0
• CNC Words A-V A-A
• Unaided 44 16
• Aided 48 32

10
Habilitation Pre-Implant

• No pattern perception
• No ability to detect voice on / voice off
  (difficulty monitoring own voice as a result)
• functionally K is profoundly deaf

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Habilitation Test Battery

• MAIS
• Pre-Implant 2 / 40
• EVT
• Pre-Implant CA 119 LA 76
• PPVT
• Pre-Implant CA 119 LA 79

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Implantation

• Decision to implant made in conjunction with
  family
• Childs school and social circumstances
  increasingly desperate
• Implanted with (L) Nucleus Freedom no
  complications

13
NRT in Theatre
14
MAP Dynamic range
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Speech Perception Post-Implant

• HINT-C sentences A-V A-A
• CI Aided at 6 months 94 99
• CI Aided at 12 months 100 98
• CNC Words A-V A-A
• CI Aided at 6 months 96 52
• CI Aided at 12 months 88 72

16
Habilitation Post-Implant

• MAIS (out of 40)
• Post-Implant
• 3 months 35
• 12 months 40
• PPVT
• 12 months CA 1310 LA 97
• 2 years CA 1411 LA 101
• EVT
• 12 months CA 1310 LA 1110

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Child GV
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Child GV

• Personal Information
• Age now 117
• Referred 05.09.2006 Age at referral 89
• Implanted 16.05.2007 Age at implant 95
• Currently 22 years post-implant

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History

• Acute meningitis-like illness with severe
  head-ache, cough, and high fever at 5 ½ years
• Sudden onset hearing loss with balance
  disturbance and dysarthria
• Further sudden deterioration in hearing
  subsequent to an URTI treated aggressively with
  steroids
• Local audiological testing revealed poor speech
  discrimination following the last drop in hearing
  levels
• Aided KTT 60 A-V
• Aided KTT 10-20 A-A
• Referral to CI Programme from AODC initiated

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Classroom and Social

• experiencing significant barriers to all
  communication
• unable to access classroom instruction and
  discussion without the support of the written
  word
• Finding it difficult to monitor voice levels and
  has to be prompted to turn on his voice
• Needs to know of changes in classroom routine in
  advance
• Can no longer hear the school bell or fire alarm
• Looks around the room and is ill at ease watching
  the other children

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Audiology Pre-Implant
2003
2006
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CI Assessment
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Speech Perception Pre-Implant

• deVault Common Phrases A-V A-A
• Unaided 64 56
• Aided 73 54
• Hearing aids make no difference auditory-alone.
  There is possibly some benefit for speech-reading
• many responses were word perfect but were then
  followed almost immediately by a number of errors
  that bore no relation to the original stimulus

24
TEOAE
25
DPOAE
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Audiology Pre-Implant

• ABR
• no synchronous neural activity in any of the
  traces beyond 2 ms repeatable potential in each
  ear between 0 and 1 ms would reverse with a
  reversal of the polarity of the stimulus not
  apparent with an alternating stimulus

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Habilitation Pre-Implant

• MAIS (out of 40)
• Pre-Implant 15 / 40
• NSW Auditory Skills Placement Test
• (Sound Awareness, Discourse, Sentence, Word)
• Pre-Implant No scores on any subtest

28
Implantation

• Decision to implant made in conjunction with
  family as per child KS
• Implanted with (R) Nucleus Freedom no
  complications
• No NRT response in theatre

29
MAP Dynamic range
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Speech Perception _at_ 6 months

• HINT-C sentences A-V A-A
• CI 100 94
• CIHA - 96
• CNC Words A-V A-A
• CI 88 76
• CIHA 80 64

31
Speech Perception _at_ 12 months

• HINT-C sentences A-A
• CI 99
• CIHA 97
• CNC Words A-A
• CI 72
• CIHA 56

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Habilitation Post-Implant

• MAIS (out of 40)
• 6 months Post-Implant 32 (15)
• NSW Auditory Skills Placement Test
• (Sound Awareness, Discourse, Sentence, Word)
• All 6 Ling sounds at 6 metres
• Passed all other sub-tests

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Habilitation Post-Implant

• CELF
• 1 month CA 97 LA 80
• PPVT
• 1 month CA 97 LA 80
• 12 months CA 106 LA 81
• EVT
• 12 months CA 106 LA 95

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Summary and Conclusions

• Both children continue to be good users of their
  devices and continue to make gains
• These 2 cases of demonstrate successful use of CI
  as an intervention in ANSD secondary to acute
  illness
• Neurodiagnostic ABR and OAE should be considered
  routine in individuals with sudden hearing loss
  secondary to acute illness, particularly where
  the audiogram is atypical or there is discrepancy
  between hearing levels and school and social
  progress
• Concerns about destroying significant residual
  hearing through the CI process should not deter
  this as a possible intervention

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The End