Table 3 Auditory nerve responses and auditory and speech function evaluations in children with neurocristopathy-related hearing loss.

From: Analysis of the clinical features of neurocristopathy-related hearing loss and how these relate to outcomes after cochlear implantation

ID

CI side

 

CI NRT

CAP

  

SIR

  
   

Response

Pre CI

1 year after CI

3 years after CI

Pre CI

1 year after CI

3 years after CI

WS01

Right

MEDEL synchrony

Good

1

4

N/A

1

2

N/A

WS02

Right

Cochlear CI622

Good

1

4

N/A

1

2

N/A

WS03

Bilateral

MEDEL synchrony

Good

1

4

N/A

1

1

N/A

WS04

Right

Cochlear CI512

Good

1

5

7

1

2

4

WS05

Right

Cochlear CI512

Good

1

6

8

1

3

4

WS06

Right

Cochlear CI512

Good

1

5

7

1

2

4

NS01

Bilateral

Cochlear CI512

Good

1

5

8

1

3

4

NS02

Bilateral

Cochlear CI622

Good

1

4

N/A

1

4

N/A

NS03

Bilateral

MEDEL synchrony

Good

1

4

N/A

1

4

N/A

KS01

Bilateral

Cochlear CI512

Good

1

5

6

1

2

3

CS01

Bilateral

Cochlear CI512

Partial, right 5/22#;

no response on left

1

2

2

1

1

1

CS02

Right

MEDEL sonata

Partial, 6/12#

1

2

6

1

2

3

CS03

Right

MEDEL concerto

Partial, 5/12#

1

2

4

1

1

2

CS04

Right

MEDEL synchrony

Partial, 10/12#

1

1

N/A

1

1

N/A

CS05

Left

Cochlear CI622

Partial, 10/22#

1

2

N/A

1

2

N/A

CS06

Bilateral

Cochlear CI512

no response

1

1

1

1

1

1

  1. N/A indicates data not available due to incomplete follow-up.
  2. # value indicates the number of active recording electrodes relative to the total electrode array (e.g., 6/12 = 6 active electrodes out of 12 total).
  3. CAP: Categories of Auditory Performance, CI: cochlear implant, CS: CHARGE syndrome, ID: identification number, KS: Kabuki syndrome, MEDEL: MED-EL Medical Electronic, NRT: Neural Response Telemetry, NS: Noonan syndrome, SIR: Speech Intelligibility Rating, WS: Waardenburg syndrome.
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