360 Degree EVALUATION OF

PEDIATRIC C.I.

-DR. LEENA JAIN

PROFESSOR & HOD, DEPT OF ENT,
RVRS MEDCAL COLLEGE,
BHILWARA.
 INTRODUCTION :

 Hearing loss is the most common sensory deficit in humans today. As per WHO

estimates in India, there are approximately 63 million people, who are suffering from

Significant Auditory Impairment; this places the estimated prevalence at 6.3% in Indian

population.

 As per NSSO survey, currently there are 291 persons per one lakh population who are

suffering from severe to profound hearing loss (NSSO, 2001). Of these, a large

percentage is children between the ages of 0 to 14 years.

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 For many, hearing aids provide a viable solution, but if one experiences significant

hearing loss, hearing aids may offer little or no benefit. This is where cochlear

implants (CI) come into role.

 A cochlear implant is a hearing prosthesis designed to restore or provide a level of

auditory sensation to adults and children who have severe to profound bilateral

sensorineural hearing impairment and who get limited benefit from hearing aids .

 https://nhm.gov.in/index1.php?lang=1&level=2&sublinkid=1051&lid=606
 STATISTICS :
• More than 1.5 billion people worldwide are currently affected by
hearing loss in at least one ear.

• Around 430 million people worldwide require rehabilitation for

disabling hearing loss.

• Approximately 13% of adults ages 18 and older experience some

difficulty hearing even when using a hearing aid.

• On average, people with hearing loss wait seven years before seeking
help.
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• Around 22 million Americans are exposed to hazardous noise levels in

their workplaces.

• Nearly 60% of hearing loss in children is due to causes that can be

prevented through proper health interventions, such as improved
maternal and neonatal care.

• Among adults between the ages of 45 and 64, 2.8% of men and 1.9% of
women use a hearing aid.

• More than 14% of adults ages 65 and over use a hearing aid.
.

• Over 25% of adults worldwide ages 60 and older are affected by

disabling hearing loss.

• An estimated 700 million people worldwide will be affected by

disabling hearing loss by 2050.

 https://www.forbes.com/health/hearing-aids/deafness-statistics/
 ORIGIN OF COCHLEAR IMPLANTS :

 In 1800, Alessandro Volta first reported that electrical stimulation to

metal rods inserted in his ear canal created an auditory sensation. He

described this sensation as “a boom within the head.” In 1957, Djourno

and Eyries placed a wire on the auditory nerve of someone who was

undergoing surgery.

 They used this wire to stimulate the auditory nerve directly with

electrical current and the person reported a clear auditory percept.

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 This observation lent impetus to the search for a treatment of profound

deafness

 The first single channel cochlear implant was introduced in 1972. Over

1000 people were implanted from 1972 to the mid 1980s including

several hundred children.

 In 1984, Cochlear Corporation introduced the first multi-channel

cochlear implant system.

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 This device, the Nucleus 22, consisted of an implanted

receiver/stimulator and an intracochlear electrode array that consisted of

22 banded contacts.

 Later versions of the Nucleus device used magnets to hold the

transmitting and receiving coils in close proximity.

 https://www.asha.org/policy/tr2004-00041/
 Guidelines for Pediatric C.I :
 In Children 12–24 months -

 Bilateral, profound sensorineural hearing loss (>90 dB HL)

 Limited benefit from appropriate binaural hearing aids.

 Lack of progress in development of simple auditory skills.

 Participation in intensive aural rehabilitation over 3–6 mo.

 Quantified by Meaningful Auditory Integration Scale or Early

Speech Perception Test.
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 In Children 25 months to 17 yr and 11 months-

 Severe to profound sensorineural hearing loss (>65 dB HL)

 Limited benefit from appropriate binaural hearing aids

 3–6 mo hearing aid trial

 Scoring < 20-30% on open-set Multisyllabic Lexical

Neighborhood Test or Lexical Neighborhood Test depending on

the child’s cognitive and linguistic skills.

 Teagle HF, Roush PA, Woodard JS, et al. Cochlear implantation in children with auditory neuropathy
spectrum disorder. Ear Hear 2010;31:325–35
PEDIATRIC CANDIDACY EVALUATION FOR C.I :

 Patient selection is one of the most important determinants of

cochlear implant success within the pediatric population.

Therefore, comprehensive candidacy evaluation is critical to

the patient success.

 The purpose of the candidacy evaluation is to determine the

medical and audiometric suitability of the patient for cochlear

implantation.
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 Within the pediatric population, the candidacy evaluation varies

slightly by age, but maintains a core of essential components.

 The pediatric cochlear implantation candidacy evaluation should

comprise a battery of testing, including a medical evaluation,

imaging evaluation, audiologic evaluation, speech and language

evaluation, and patient/family counseling.

 Table 1 provides a synopsis of the components of the cochlear

implantation evaluation.
 Teagle HF, Roush PA, Woodard JS, et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear
2010;31:325–35

.
First, abnormal NBHS results must be followed by confirmatory

audiometric testing, typically diagnostic auditory brain stem response

(ABR).

 The next step is typically medical evaluation by an otolaryngologist, often

the first point of contact for children with newly diagnosed hearing loss.

 A genetic cause is identified in up to 60% of children with congenital

bilateral severe-to-profound sensorineural hearing loss (SNHL).

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 Other children referred for complete diagnostic assessment regardless

of NBHS result often include:

• Children considered to have a high chance of hearing loss for reasons such

as a family history of hearing loss, a syndrome associated with hearing loss

(e.g., trisomy 21 or cleft lip and palate), or exposure to specific

environmental risk factors (e.g., care in a neonatal intensive care unit

[NICU], exposure to aminoglycoside antibiotics);

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• School-age children who typically have milder hearing loss that was not

detected on NBHS and are often identified following:

(1) parental concern regarding hearing;

(2) delayed speech or language development; and/or

(3) hearing screening as part of a well-child examination or school

program
 Patient and Family Counseling :

• The goal of the patient and family counseling is to summarize the

findings of the cochlear implant evaluation and provide the family
with recommendations from the cochlear implant team.

• At this time, expectations for cochlear implantation outcome are

established. Explanation and assurance of understanding regarding the
components and extent of postoperative aural rehabilitation are
explained to the family.
• The level of commitment of the patient and family to the

requirements of aural rehabilitation is assessed.

• The family should be provided with literature regarding the

available devices for implantation, and with the assistance of the

cochlear implantation team, a decision regarding desired device for

implantation are made.

 Evolving
.
Criteria for Cochlear Implantation :

• Decreased age of implantation (<9 months)

• Patients with residual hearing

• Single-sided deafness (<5 years old)

• Cochlear nerve aplasia

• Inner ear malformations

• Bilateral Cochlear Implantation

• Cochlear Implantation in Adolescents with Congenital Long-term

Deafness
 ETIOLOGY OF HEARING LOSS :

 Prenatal period :

• genetic factors including hereditary and non-hereditary

hearing loss

• intrauterine infections – such as TORCH infections.

• intrauterine exposure of teratogens like alcohol, drug abuse,

thalidomide, etc.
.

 Perinatal period :

• birth asphyxia (a lack of oxygen at the time of birth)

• hyperbilirubinemia (severe jaundice in the neonatal period)

• low-birth weight , low APGAR score , prematurity , use of

ototoxic medicines

• other perinatal morbidities and their management.

.

 Childhood and adolescence :

• chronic ear infections (chronic suppurative otitis media)

• collection of fluid in the ear (chronic nonsuppurative otitis

media)

• meningitis and other infections.

* Other causes include: congenital non-genetic malformations and other maternal prenatal causes
.

 Hereditary sensorineural hearing loss may be categorized as

Non-Syndromic SNHL or Syndromic SNHL;

 Non-Syndromic SNHL accounts for most hereditary congenital

hearing loss. Mutations within the connexin 26 genes, namely

the GJB 2 and GJB 6 genes, are responsible in most cases.

 .

• Patients with deafness associated with mutations in the connexin 26

genes have been found to be excellent candidates for cochlear

implantation because they perform equal to or better than other

cochlear implant patients in reading comprehension, nonverbal

cognition, speech performance, language perception, speech

perception, and speech intelligibility.

.

• Nonsyndromic hearing loss is not associated with visible abnormalities

of the external ear or related medical findings; however, it can be

associated with abnormalities of the middle ear and/or inner ear.

 Syndromic SNHL includes syndromes like Usher syndrome, Pendred

syndrome, Refsum disease, Jervell Lange-Nielsen syndrome, Waardenburg

syndrome, branchio-oto-renal syndrome, and CHARGE syndrome.

 SUMMARY :
 Since the introduction of cochlear implantation in clinical practice,

its preoperative evaluation, implementation, and criteria for use

continue to evolve.

 The preoperative evaluation is designed to identify appropriate

candidates for implantation and should include medical evaluation,

cochlear imaging, audiologic and hearing aid evaluation, speech

and language evaluation, electrophysiologic testing, psychological

testing, and speech perception testing.

 .

 Within the evaluation of pediatric patients, the cause of SNHL

should be identified because it may affect peri-op care, surgical

techniques during implantation, and post-op rehabilitation.

 Within the pediatric population, the criteria for implantation

continue to evolve. Most recently, implantation is being

undertaken in patients who are younger than 12 months.

 B/L cochlear implantation is often used in the pediatric

population to improve directional hearing and hearing in noise.

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 REFERENCES :
 Siem G, Fagerheim T, Jonsrud C, et al. Causes of hearing impairment in the Norwegian paediatric cochlear
implant program. Int J Audiol 2010;49:596–605.
 Kenneson A, Van Naarden Braun K, Boyle C. GJB2 (connexin 26) variants and nonsyndromic sensorineural
hearing loss: a HuGE review. Genet Med 2002;4: 258–74.
 Dahl HH, Sauders K, Kelly TM, et al. Prevalence and nature of connexin 26 mutations in children with non-
syndromic deafness. Med J Aust 2001;175:191–4.
 Dahl HH, Wake M, Sarant J, et al. Language and speech perception outcomes hearing-impaired children with
and without connexin 26 mutation. Audiol Neurootol 2003;8:263–8.
 Sinnathuray AR, Toner JG, Clarke-Lyttle J, et al. Connexin 26 (GJB2) generelated deafness and speech
intelligibility after cochlear implantation. Otol Neurotol 2004;25:935–42.
 Loundon N, Marlin S, Busquet D, et al. Usher syndrome and cochlear implantation. Otol Neurotol 2003;24:216–
21.
 Teagle HF, Roush PA, Woodard JS, et al. Cochlear implantation in children with auditory neuropathy spectrum
disorder. Ear Hear 2010;31:325–35.
 Donaldson AI, Heavner KS, Zwolan TA. Measuring progress in children with autism spectrum disorder who have
cochlear implants. Arch Otolaryngol Head Neck Surg 2004;130:666–71.
Thank
You.