Journal

auditory neuropathy

by:

Name : Nur wahyuliasti

Nim : 12.06.0038

MEDICAL SCHOOL

ISLAM AL-AZHAR UNIVERSITY

MATARAM

2018
 NEUROPATHY AUDIOTORI

Abstract

Background: Auditory neuropathy is a hearing loss is rare with a prevalence that is

not certain and require early identification and diagnosis. Objective: To clarify the picture of

audiology and electrophysiology auditory neuropathy that can determine an effective

treatment and intervention. Literature: auditory neuropathy is part of sensorineural deafness,

where the sound can sign up to the inner ear, but the transmission of signals from the inner

ear to the brain is disrupted in specific pathways. This disorder can be of all ages ranging

from infants to adults. Patients with auditory neuropathy may have some degree of hearing is

normal or decreased from mild to severe deafness, but always had the ability to talk bad

perception. Auditory neuropathy is characterized by abnormal results on brainstem evoked

response audiometry (BERA), but otoacoustic emission (OAE) are normal. These disorders

require a different management approach to the problem of hearing and deaf communication

than any other peripheral. Conclusion: The clinical evaluation and an accurate audiological

required in auditory neuropathy, and ultimately, the right diagnosis provides therapeutic

strategies and better rehabilitation. Keywords: auditory neuropathy, BERA, OAE, speech

perception

Abstract

Background: Auditory neuropathy is a rare hearing disorder the prevalence of the

which is not well established and need an early identification and diagnosis. Purpose: To

describe the audiological and electrophysiological features of auditory neuropathy in order to

Determine the effective treatment and intervention. Literature Review: Auditory neuropathy

is a kind of sensorineural hearing loss, in the which sounds enter the inner ear normally, but

the signal transmission from the inner ear to the brain is impaired in some ways. It can Affect
people of all ages from infant to adult. Patients with auditory neuropathy may have normal

hearing or hearing loss ranging from mild to profound hearing loss, but they always have

poor speech perception abilities. Auditory neuropathy is Characterized by the abnormal result

of the auditory brainstem response (BERA), but in the presence of preserved otoacoustic

emissions (OAE). It requires a different management approach to the auditory and

communication problems that used for usual peripherals hearing losses. Conclusion: An

accurate clinical and audiological evaluations are needed in auditory neuropathy, and finally,

a correct diagnosis allow better treatment and rehabilitative strategies.

Keywords: Auditory neuropathy, BERA, OAE, speech perception

Preliminary

Auditory neuropathy is a term first introduced by Starr et al in 1996. However, this

disorder is not something new as it has been reported by several researchers sebelumnya.1,2

case of auditory neuropathy was first discovered by Davis and Hirsch in 1970 as an invention

paradoxically because there are differences between the results of brainstem evoked response

audiometry (BERA) is abnormal with the results of otoacoustic emission (OAE) and the

hearing threshold is normal. Similar findings were also reported by Worthington and Peters in

1980, Lenhardt in 1981 and Kraus in 1984. Another term for auditory neuropathy is

dissinkronisasi auditory (Berlin et al, 2002), neural deafness (Rapin and Gravel, 2003), or de

-Synchronization auditory (Ray et al, 2006).

Their latest technologies and procedures make these abnormalities can be

distinguished from other sensorineural deafness. Gravel and Rapin (2006) describes a variety

of sensorineural hearing loss based on the location of the lesion that is deaf sensory (the hair

cells inside), neuropathy auditory (pathology ganglion cell spiralis and axon nerve cochlear),

deafness central (about pathways auditory central) and impaired nerve conduction ( if no
abnormality as mentioned above). Starr et al (1996) Auditory neuropathy divide into two

types: pre-synaptic (type I) if there is involvement of the hair cells and post-synaptic (type II)

if there is a cochlear nerve involvement. 1 This paper will discuss the causes and pathological

mechanisms of occurrence of auditory neuropathy, diagnostic examination required to

enforce this condition and choice of appropriate treatment to improve hearing function.

Definition

Auditory neuropathy including part of sensorineural hearing loss, which is a fairly

broad term and illustrates interference in the activity of afferent nerves in the peripheral and

central auditory pathways. The term dis-synchronization is defined as the inability of

synchronization of neuronal activity in the temporal region, causing limitations in auditory

perception. Auditory neuropathy is characterized by cochlear outer hair cell function in

electrophysiology is still normal or close to normal, but there is a disruption in nerve

conduction along the auditory pathway.

Frequency

Data on the prevalence of auditory neuropathy has so far not known. Various

literature reporting the figure with a high degree of variation is between 0.5 to 15% of

sensorineural hearing loss. In one study in Hong Kong reported auditory neuropathy

prevalence of 2.44%, while Germany reported 0.94% .3 Rance et al (1999) and Madden et al

(2002) reported a prevalence of higher auditory neuropathy, respectively by 11% and 5.1%.

Berg et al (2005) found the incidence of auditory neuropathy among at-risk populations by

24%. Meanwhile, in research conducted by Khairi et al (2009) were obtained from 211

children with sensorineural hearing loss as much as 3 children (1.42%) suffered from

auditory neuropathy.
 From research by Lotfi and Mehrkian (2007) in school children with hearing loss

obtained 1.54% of children suffering from auditory neuropathy and 53% experienced a

unilateral auditory neuropathy. The case of auditory neuropathy have been reported in all

ages ranging from newborns to adults over the age of 60 years. But the majority of cases are

found in less than 10 years of age. Distribution of sex in this same disorder between men and

women.

Etiology

Auditory neuropathy may occur in the general population, but is more often found in

children with high risk for hearing loss. Foerst et al (2006) found out of 32 children with

auditory neuropathy, as many as 27 children at high risk for hearing loss and only five

children do not have a high risk. Prematurity with postpartum complications are most

common risk factor for the occurrence of auditory neuropathy and followed by

hiperbilirubinemia.7 causes auditory neuropathy can be divided into two: the congenital form

of genetic abnormalities (mutations of genes otoferlin / OTOF, Charcot-MarieTooth

syndrome, Friedrich's ataxia) and obtained, covering the risk of perinatal (prematurity,

hyperbilirubinemia, hypoxia / perinatal asphyxia, use of a mechanical ventilator, intracranial

hemorrhage post-natal), exposure to drugs ototoxic, the process of infection (mumps and

meningitis), immune disorders (Guillain-Barre syndrome), polyneuropathy in diabetes

mellitus, as well as head trauma. However, approximately 50% of cases of auditory

neuropathy of unknown etiology. Lotfi and Mehrkian (2007) to get as much as 73% of

patients with auditory neuropathy have a family history of hearing loss that leads to auditory

neuropathy and 62% have risk factors such as anoxia, hyperbilirubinemia, meningitis, and

exposure to ototoxic drugs. Madden et al (2002) found out of 22 patients with neuropathy

auditory, 11 (50%) had a history of hyperbilirubinemia, 10 (45%) with a history of preterm, 9

(41%) with drug exposure ototoxic, 8 (36%) with hearing loss family, 8 (36%) with a history
of mechanical ventilator use, and 2 (9%) with cerebral palsy. 10 From screening with OAE

performed by Dowley et al (2009) in 40 050 infants, showed as many as 30 babies suffering

from sensorineural hearing loss, and 12 (40%) infants included in auditory neuropathy. All

infants with neuropathy auditory being treated in the neonatal intensive care unit (NICU) and

10 (83%) using a ventilator for more than five days, 9 (75%) are exposed to gentamicin, 8

(67%) suffered from sepsis, 7 (58% ) with premature births and 4 (33%) suffered from

hyperbilirubinemia.

Pathophysiology

At first, auditory neuropathy is described as a single disorder characterized by

disorders of the cochlear nerve with the outer hair cells are still normal. But this turned out to

be a disorder that affects the spectrum of the various pathways auditory hair cells starts from

within, the synapse between nerve cells and cochlear hair on, until the cochlear nerve itself.

The clinical picture with wide variation in auditory neuropathy may be caused by differences

in location of the lesion and the underlying causes. Auditory neuropathy affects the activity

of the normal synchronization of auditory pathways, without affecting the function of the

outer hair cell amplification. Auditory neuropathy is caused by damage to the transmitter

simultaneously release of synaptic vesicles that attaches to the hair cells in the produce

disturbances in afferent nerves. Disorders of the cochlear nerve demyelination may arise due

to the lowering of action potentials and inhibit the electric current, or primary axonal disease

with loss of nerve fibers and small action potentials. Both these disorders affect the action

potential of nerve fibers along the longest due to the degeneration of nerve fibers and nerves

provide the supply at the apex of the cochlea which is thought to cause interference at low

frequencies. Cochlear nerve deficiency can occur due to failure of both partial development

(hypoplasia) and complete (aplasia or agenesis). Hair cells in specifically sensitive to hypoxia

than the outer hair cells, and also against some toxic substances such as karbopentin and
gentamicin. Synapse damage can cause interference on the saturation of the response, for

example, a given stimulus 3-11 times a second can be detected in full, but not so in the

stimulus provided as many as 20 times a second.

Diagnosis

History The patient with auditory neuropathy often complain they can hear the sound,

but could not understand the conversation. The lack of recognition of this language due to the

severe disruption in the ability of the process of discrimination in the temporal region. In the

auditory neuropathy impairment in speech perception abilities are not in accordance with the

degree of deafness. Some patients have difficulty in communicating, while others are

functionally deaf. Patients usually experience difficulty in hearing in a noisy situation.

Diagnostic

A comprehensive evaluation is necessary in diagnosing auditory neuropathy involving

various fields such as audiology, radiology, pediatric and neuropediatrik, as well as genes.

Audiological assessments are recommended for neuropathy auditory is audiometry

with audiometric pure tone or behavioral audiometry (visual reinforcement audiometry /

VRA, behavioral observational audiometry / BOA, audiometric play), acoustic immitance

includes tympanometry and examination of the acoustic reflex, otoacoustic emmission

(OAE), brainstem evoked response audiometry (BERA), elektrokokleografi (EcochG) and

inspection of speech perception.

1. examination audiometry

In the auditory neuropathy, hearing threshold of pure tone (pure tone

threshold) may range from at or near normal to severe deafness. Auditory processing

capabilities typically impaired in these patients, especially in noisy environments. In

 infants, behavioral audiometric examination by BOA or VRA. For infants aged less

than 6 months can be examined by observing BOA infant reflex response to sound,

but it is not interpreted as the hearing threshold or minimum limit hear the response.

Limitations BOA is the only measure awareness and the baby can not determine with

certainty the hearing threshold with a high degree of variability (depending on the

condition, awareness and attention of the patient) and can not be used as a benchmark

for the installation of hearing aids. VRA examination done when the baby has been

able to sit and have good head control. In this examination used visual media such as

toys, light or video to condition the child's response to sound. This examination

begins in children aged 6-7 months. For an older child, about 5 years old, can play

audiometric examination. Accurate audiogram for both ears are usually obtained after

at least two visits. The frequency of behavioral audiometric evaluation depends on the

status of children's development and cooperation, but should be evaluated at least

once every three months to children aged 6 years.

2. examination Tympanometry

In the auditory neuropathy, usually acoustic reflex does not appear either in

the ipsilateral and contralateral stimulation, although in some cases this reflex can

emerge. 4,10 acoustic stapedius reflex does not appear or abnormal due to

disturbances in nerve conduction of auditory signals.

3. OAE examination

Lotfi and Mehrkian (2007) 3 found as many as 69.23% of patients with

auditory neuropathy have a good OAE response, 19,23% of the patients there was no

response on the OAE and 11.53% have a bad OAE response. From research Shehata

et al (2008) 2 of the 16 children with auditory neuropathy gained as much as 80% still

shows a normal OAE. Auditory neuropathy diagnosis is confirmed by the results of

 which are still normal OAE indicating the outer hair cells of the cochlea functions still

baik.4,6 options OAE examination in diagnosing auditory neuropathy is

distortionproduct OAE (figure 8). Distortion-product OAE (DPOAE) were measured

at each ear for two primary tones (f1 and f2), with a combined ratio f2 / f1 is 1.2 and

the combined level of 65 dB SPL (L1) and 55 dB SPL (L2). Frequency f2 is

specifically increased gradually from 1500 to 6000 Hz. Their DPOAE at each

frequency is determined by a combination of criteria include the ratio of signal-

tonoise absolute ≥10 dB and -15 dB noise level ≤ SPL.12

4. BERA examination

Typical features of auditory neuropathy in the investigation was the discovery

picture BERA BERA abnormal, elongated or non-existent, with the wave mikrofonik

cochlea (Figure 9). 6.10 Mikrofonik preneural cochlea is a response generated by the

polarization and depolarization of cochlear hair cells (appear before the wave I in

BERA). Mikrofonik Cochlear can be found in a normal ear, typical sensorineural

hearing loss and auditory neuropathy. Mikrofonik cochlea on auditory neuropathy is

accompanied by abnormal neural response or no. The amplitude of the wave is greater

in patients with disorders of the central nervous system. Cochlear mikrofonik wave

distinguished by a neural response through two criteria: cochlear mikrofonik polarity

will reverse the polarity inversion mikrofonik cochlea stimulus and latency will be

constant with changes in the level of stimulus. If a suspect is mikrofonik cochlear

response (especially at relatively high stimulus), the analysis must be confirmed by

the stimulus rarefaction and condensation. To distinguish this wave of stimulus

artifacts, sound tube coupled with the transducer on earphones released without

changing the position of electrodes and transducers. When disappears, then this wave

is mikrofonik cochlea. However, if settled, this wave is a stimulus artifact. BERA

 examination assessed on two main types of stimuli minimum of 100 μsec click and

250 Hz tone burst. BERA physiological hearing threshold is obtained at the lowest

stimulus level where wave V response can be detected visually. At least two waves at

each stimulus level recorded for verification in the identification of the waves.

Examination ASSR Auditory steady-state response (ASSR) is an alternative in

assessing an objective examination of the peripheral auditory pathways central to

combine the specificity of various frequencies and high-level stimulation. Auditory

steady-state response generate continuous tones on the amplitude and / or a certain

frequency. This check is performed in cases of very severe sensorineural deafness

where BERA response does not appear. Only a few studies have reported ASSR

application in children with neuropathic auditori.15 ASSR response obtained at a

higher signal level (> 80 dbHL) in auditory neuropathy, but this response will

increase despite the behavioral audiogram still showed normal results. This

examination can not be used to determine the hearing threshold in auditory

neuropathy.

5. examination Elektrokokleografi

In elektrokokleografi (EcochG) obtained mikrofonik wave cochlear long and

fluctuating amplitude increased and normal hearing threshold. In the study conducted

by Shehata et al (2008), of the 16 children were examined by trans-tympanic EcochG,

13 (81.2%) showed a long cochlear mikrofonik wave and fluctuates with the cochlea

mikrofonik threshold value ranges between 40-60 dB , Waves in the cochlear

mikrofonik EcochG2.

6. Radiological examination

Radiological examination (MRI / CT) was performed to see malformations of

the inner ear and cochlear nerve integrity. Of the 140 patients with neuropathy
 auditory conducted MRI examination, a total of 35 (25%) were found deficient nerve

cochlear form of hypoplasia or aplasia nerve cochlear and on the ears as much as 24

(69%) and both ears as much as 11 (31%). 1 Buchman et al ( 2006) reported a 9 out of

51 patients with auditory neuropathy (18%) had the cochlear nerve aplasia or

hypoplasia were identified by MRI (magnetic resonance imaging). MRI examination

is recommended in patients with cochlear implant candidates.

7. Perception examination Talk

Tests to assess the perception of speech can be done by using a questionnaire

ITMAIS (Infant-Toddler Meaningful Auditory Integration Scale) or MAIS

(Meaningful Auditory Integration Scale), words and phonemes MLNT (Multisyllabic

Lexical Neighborhood Test) / LNT (Lexical Neighborhood Test), words and

phonemes PB-K (Phonetically Balanced Kindergarten), and hint sentence (Hearing in

noise Test) in quiet and noisy environments. Variability of speech perception ability

in adult patients with auditory neuropathy have been reported in several studies.

Speech perception data obtained in adult patients with auditory neuropathy is not as

easy as that obtained in pediatric patients. Auditory neuropathy, speech perception

abilities usually disproportionate to the hearing threshold owned. Speech perception

in these patients is worse than sensorineural deafness. Disorders of the cochlear nerve

activity does not result in a significant decrease in sensitivity, but causes difficulty in

understanding speech. Patients with auditory neuropathy have a good auditory ability,

but has the ability to discriminate very bad word.

Diagnoses

Based on the location of the lesion, differentiated auditory neuropathy with deafness

sensory neural deafness, sensorineural hearing loss and deafness sentral.16 At auditory

neuropathy, disorders that are in the hair cells inside, the synapse between nerve cells and
cochlear hair inside, and cochlear nerve. In deaf sensory abnormalities just about the hair

cells inside. In neural deafness, disorders of peripheral auditory pathways are along the locus

of pathology can not be determined. In central deafness, there are abnormalities in the central

auditory pathways. In sensorineural hearing loss, abnormalities of the hair cells in the

auditory pathway to the locus of pathology can not be determined.

Management

Patients with auditory neuropathy requires treatment of hearing problems and the

different communication with sensorineural deafness lainnya.3 management of patients with

auditory neuropathy is still controversial.

Hearing Aid (ABD)

The use of hearing aids (ABD) Conventional provide benefits in some patients with

auditory neuropathy, while other patients with severe disorders showed no improvement.

Various studies have been conducted in pediatric patients and adults with auditory

neuropathy and obtained various degrees of benefits in the use of amplification ABD, but

have not been able to answer systematically whether ABD provides advantages in auditory

neuropathy. Limitations in the prevalence and heterogeneity makes it difficult to analyze

abnormalities.

Many audiologist found ABD can not help in cases of auditory neuropathy. Hearing

aids can eliminate the perception and high sound intensity can damage the cochlea which is

still intact. But there is another discovery which reported a loss of spontaneous OAE response

in patients with auditory neuropathy even without the installation of ABD. It also reported a

patient with extensive amplification ABD still shows a normal OAE response.
 ABD installation should be selected carefully, especially in the setting amplifikasinya.

Installation of ABD with gain and output is limited to the hearing threshold level, especially

in patients with mild deafness should be considered in order to get the benefits without

causing significant risk. Although Instrument fitting with a light gain has been clinically

acceptable, but it is difficult to assess whether there is the right benefits in the use of ABD in

these patients. In general, conventional amplification unable to improve understanding of

speech in patients with auditory neuropathy during interrupted cochlear nerve. Therefore, the

use of ABD as the management of these patients is still unclear. From the results of research

conducted by Rance et al (2002) compared the ability of speech perception after the

installation of hearing aids in 15 children with auditory neuropathy, the result of 50% showed

an improvement in speech perception and 50% did not experience significant improvement.

Auditory speech perception neuropathy after installation ABD

ABD installation can help in some cases, but it must be ensured that patients use them

correctly and consistently. Before installation ABD, should be given counseling to parents

that ABD may be or will not improve speech and language function in children with auditory

neuropathy. Using the IT-MAIS questionnaire and Early Listening Function (ELF) may assist

in the evaluation of ABD amplification at younger children. Children with auditory

neuropathy should be monitored every month for changes in hearing sensitivity which is

useful in setting ABD amplification and evaluation of speech development. The length of

time needed to evaluate the benefits or disadvantages of the amplification is determined by

the child's developmental level and consistent use of amplification.

In some cases, children do not show an improvement soon in response to the

amplification. Various data include the results of the audiological, parent report, and

improvements in speech and language development can assist in determining the length of
trials for amplification. When ABD showed little benefit, further evaluation is needed to

determine whether the child can be a candidate in the use of cochlear implants. Their negative

effect on understanding speech noisy environments patients with auditory neuropathy,

technology frequency modulation (FM) can be considered in patients with ABD installation

or cochlear implant.

Cochlear implant

After several years of auditory neuropathy is known, there are various studies that

claim the benefits of a cochlear implant. In the case of auditory neuropathy were first

reported by the cochlear implant, there is a progressive improvement in speech and language

skills in the first year. Sininger and TRAUTWEIN (2000) reported the case with a normal

BERA results after cochlear implant. The same was reported by Fabry (2000) that there were

improvements in auditory function after cochlear implant. In some cases, auditory

neuropathy, cochlear implants can create a shortcut on the location of the lesion (hair cells

inside or synapse). In addition to the electrical stimulation can restore the cochlear nerve

synchronization. Electrical stimulation is more effective in the production of neural

synchronization response than acoustic stimulation. Furthermore, the resulting biphasic

pulsatile stimulation of implanted electrodes can improve the synchronization of cochlear

nerve activity. From research conducted by Rance and Barker (2008) 17 in 20 patients with

auditory neuropathy comparing speech perception in 10 patients with ABD installation and

10 patients with cochlear implants, showed the same improvement between the two

(Table 1). Table 1. Comparison of speech perception after cochlear implant

installation of ABD and auditory neuropathy in 17 subject phoneme CNC Score (%) NA

(ABD) 55.1 ± 24.8 NA (cochlear implant) 59.6 ± 20.6 CNC Consonant / Nucleus -Vowel /

Consonant Shehata et al (2008) conducted a study on 16 children with auditory neuropathy,

gained as much as two children can heal spontaneously, two children using the ABD and 12

children using cochlear implants after not obtained sufficient benefits by ABD. From the

speech audiometric examination, the result of discrimination word greater than ABD cochlear

implants. The results of said discrimination auditory neuropathy in patients with ABD

installation and cochlear implants. Although some patients showed improvement with

cochlear implants, but there are some cases that did not show improvement. Besides talking

to neuropathy auditory perception with cochlear implants worse than those with sensorineural

cochlear implant. Recommendations cochlear implant in patients with auditory neuropathy is

not automatically done. In the case of amplification still give good results, cochlear implants

have not need to be used.

Implant Brain Stem Auditory neuropathy is a heterogeneous disease in which damage

can occur from synchronized cells in the hair, cochlear nerve neurons primary, until the

cochlear nerve more proximally. Some patients showed improvement after the installation of

cochlear implants, while some others did not show success with this intervention. In patients

with severe auditory neuropathy that fail to implant cochlear implants do the brainstem

(auditory brainstem implant). Because of the location of stimulation with cochlear implants

possibility spiral ganglion cell, a given signal can not get to the central, particularly in

auditory neuropathy involving the cochlear nerve fibers. To be effective, the electrical signal

must pass through lesions in the neurons and reach the central auditory pathway directly,

obtained with a brain stem implant. Electrodes from the brain stem implant is placed in the

lateral ventricle resesus 4th through the foramen Luschka placed on the surface of the

cochlear nucleus.
Prognosis

Various factors affect prognosis auditory neuropathy include age at diagnosis and

manage, the accuracy of hearing aids, the consistent use of hearing aids, quality intervention,

family involvement, cognitive ability, and the presence of other medical conditions.

However, in some patients with auditory neuropathy auditory function can be improved

spontaneously within one or two years of life.

Conclusion

1. Auditory neuropathy is a hearing loss is rare with a prevalence rate varying between 0.5 to

15% and has been reported at all ages, especially children.

2. In the case of auditory neuropathy dis-synchronization in the auditory pathway to the outer

hair cells of the cochlea function was normal.

3. Various factors cause auditory neuropathy such as prematurity, perinatal disorders, genetic

disorders, infections, immune disorders, diabetes mellitus and head trauma.

4. Typical features for auditory neuropathy is the degree of hearing varies from normal to

very severe deafness and disorders of speech perception, the OAE is still normal and there's

disruption to BERA with cochlear mikrofonik picture. Usually the acoustic reflex does not

appear on this disorder.

5. The heterogeneity in the etiology, location of the lesion and auditory functions in auditory

neuropathy cause various considerations in determining the choice of handling this case.

6. To improve auditory function in patients with auditory neuropathy, may be the installation

of ABD, cochlear implants, or if they fail to do brainstem implant.

7. The strategy needed rehabilitation and education specifically in patients with auditory

neuropathy, it is important to identify this disorder at a younger age.

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