Auditory Neuropathy

Spectrum Disorder (ANSD)

Presentation by : Igor Hutabarat, dr.
Consultant
: DR. Wijana, dr. SpTHT-KL(K)

Dept. of Otorhinolaryngology Head & Neck Surgery

Hasan Sadikin Hospital
2016

Introduction
Synonyms :
- Paradoxical
- Central Auditory Dysfunction
- Brainstem Auditory Processing Syndrome
(BAPS)
- Auditory Dysynchrony (AD)
- Auditory Neuropathy (AN) starr et al,96

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
2
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Terminology
Auditory neuropathy (AN) is a hearing loss

damage:
- auditory portion of the eighth nerve
- located between the inner ear (cochlea)
and the brainstem.

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
3
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Epidemiology
1 in 10 of those with severely abnormal ABR
(Sininger, 1998 )
22/428 children with hearing loss had AN
(Madden et al, 2002)
700 children attending schools for the deaf or
receiving services for moderate profound
hearing loss76 OAE response + possible
diagnosis of ANSD. Cheng et al. (2005)
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy, International
4
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Pathophysiology
Hearing loss can result from:
- Reduced sound conduction in to the cochlea.
- Reduced transduction of inner hair cells.
- Abnormal transmission impulses of auditory
nerve.
- Disorder processing in the central auditory
pathways.

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
5
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Pathophysiology
- synaptic junction Injury:
between inner hair cells
of the cochlea &
dendrites of spiral
ganglion neurons
- Direct injury to the spiral
ganglion neurons

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
6
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Pathophysiology
- Direct damage to
the dendrites of the
spiral ganglion
neurons
- Direct axonal
damage to the
auditory nerve

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Etiologies
Congenital
Peripheral neuropathies and hearing loss:
Friedreichs ataxia (spoendlin 1974)
Charcot-merie-Tooth disease
Ushers syndrome
Refsums disease
Mohr-Tranejaerg syndrome
8

Etiologies
Congenital:
- Charcot-Marie Tooth
Disease
- Lebers Hereditary
Optic Neuropathy
- Fredreichs Ataxia
- Mohr-Tranebjaerg
Syndrome
- Refsums Disease
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Etiologies
Infection:
- Herpes zoster oticus
- Fungal meningitis
- CMV
Metabolic:
- Diabetes mellitus
- Organic mercury poisoning
- Uremia
- Alcoholic neurophaty
- Hyperbilirubinnemia

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
10
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Risk Factors
- Neonatal anoxia
- Neonatal hyperbilirubinemia
- Neonatal mechanical ventilation,
hypoxia, or both
- Congenital brain abnormalities
- Low birth weight
- Extreme prematurity (< 28 weeks)
- Genetics or family history of ANSD
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Screening
The Joint Commission on Infant Hearing (JCIH,
2007) recommends auditory brainstem response
(ABR) as part of the screening protocol for NICU
babies admitted for greater than 5 days. NICU
infants who do not pass the automated ABR
screen should be referred directly to an audiologist
for rescreen or comprehensive testing.

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

1-36
Screening
RUL
E by 1
The 1-3-6 rule of screening for hearing loss
month of age, confirmation of presence of hearing
loss by 3 months of age, and intervention by 6
months of age is the goal of early detection and
intervention of hearing impairment programs.
A diagnostic test battery of immittance, otoacoustic
emissions, and threshold ABR testing will correctly
diagnose cases of ANSD.
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Diagnostic Evaluation
Infants and children with suspected hearing loss
should be referred to a pediatric audiologist for
comprehensive testing, which should include the
following :
- Auditory Brainstem Response (ABR/BERA)
Testing
- Otoacoustic Emissions (OAE)

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Auditory Brainstem Response (ABR)

Testing

Otoacoustic Emissions (OAE)

Management
Regular repeated assessments
Electrophysiology - repeat ABR, CM and OAE
recording at 8-10 weeks and 9-15 months
Assess development of communication
Speech language therapist
Speech Perception Test with
picture pointing

Guidelines Development Conference on the Identification and

Management of Infants with Auditory Neuropathy, International
17
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Intervention
Hearing Aids
FM system
Cochlear implant

Mode of communication
Cued speech/ signing crucial
Auditory-verbal therapy (AVT)
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy, International
18
Newborn Hearing Screening Conference, Como, Italy, June 19-21,

Goal Of Treatment
Development of language
Inform the family of resources available to
not only educate but to provide emotional
support

19

Conclusion
ANSD is a complex hearing disorder
requiring methodic identification and
management techniques to achieve proper
diagnosis and clinical outcomes.
Auditory neuropathy is a recently
described hearing disorder characterized
by present OAEs and absent or abnormal
ABRs.

 Each case should be approached

individually by the multidisciplinary team.
In addition to management according to
speech/ language progress,

THANK YOU