Download Audiologic Management of Auditory Neuropathy Spectrum Disorder

AJA
Review
Audiologic Management of Auditory
Neuropathy Spectrum Disorder in Children:
A Systematic Review of the Literature
Patricia Roush,a Tobi Frymark,b Rebecca Venediktov,b and Beverly Wangb
Purpose: This review summarizes current evidence related
to the audiologic management of children with auditory
neuropathy spectrum disorder ( ANSD).
Method: A systematic search of the literature was conducted
in 25 electronic databases (e.g., PubMed, CINAHL, and ERIC)
using key words such as auditory neuropathy, auditory
neuropathy spectrum disorder, auditory neuropathy/
dyssynchrony, and hearing loss. Eighteen studies met
the inclusion criteria by addressing 1 or more of 8 clinical
questions. Studies were evaluated for methodological quality,
and data regarding participant, intervention, and outcome
variables are reported.
Results: Fifteen of the 18 studies addressed the use of
cochlear implantation, and 4 addressed conventional
acoustic amplification. All participants demonstrated improved
auditory performance; however, all 18 studies were considered
exploratory, and many had methodological limitations.
Conclusion: The clinical evidence related to intervention for
ANSD is at a very preliminary stage. Additional research is
needed to address the efficacy of acoustic amplification and
cochlear implantation in children with ANSD and the impact
of this disorder on developmental outcomes.
A
Although it was recognized that “neuropathy” does not apply
in all cases, the term has become widely used in reference
to this population. The etiology of ANSD is multifactorial
and includes genetic, congenital, and acquired conditions.
Both syndromic (e.g., Charcot-Marie-Tooth disease and
Friedreich’s ataxia) and nonsyndromic genetic forms of ANSD
(DFNB9/OTOF; Varga et al., 2003; Yasunaga et al., 1999)
have been reported. Risk factors reported include prematurity,
hyperbilirubinemia, ototoxic drug exposure (Madden, Rutter,
Hilbert, Greinwald, & Choo, 2002; Rance et al., 1999), and
various neurological disorders such as mitochondrial disease
(Deltenre, Mansbach, Bozet, Clercx, & Hecox, 1997; Starr
et al., 1996).
Initially, ANSD in children was thought to be a rare form
of sensorineural hearing loss (SNHL). However, a recent
systematic review by Vlastarakos, Mikopoulous, Tavoulari,
Papacharalmous, and Korres (2008) indicated that ANSD
accounts for approximately 8% of the newly diagnosed cases
of hearing loss in children each year. Other studies suggest
that ANSD occurs in 2.4% to 15% of pediatric cases identified with permanent hearing loss (Rance, 2005; Sininger,
2002; Tang, McPherson, Yuen, Wong, & Lee, 2004).
Among the clinical characteristics reported are speech
perception difficulties that are often disproportionate to
hearing threshold levels (Starr et al., 1996), and difficulty
hearing in noise (Kraus et al., 2000; Rance et al., 2007).
Furthermore, degree of hearing loss, based on detection
levels, ranges from normal to profound and in some cases
may be transient or degenerative in nature (Deltenre et al.,
uditory neuropathy, a condition first described in
the mid-1990s by Starr, Picton, Sininger, Hood, and
Berlin (1996), is a disorder characterized by absent
or severely abnormal auditory brainstem response (ABR)
with intact outer hair cell function, as evidenced by the
presence of evoked otoacoustic emissions and /or cochlear
microphonics. Although current terminology implies dysfunction of the auditory nerve, possible causes include
damage to the inner hair cells, to the synapse between
the inner hair cell and auditory nerve, or in the auditory
nerve itself (Buchman et al., 2006; Rance, Cone-Wesson,
Wunderlich, & Dowell, 2002).
Recently the term auditory neuropathy was expanded
to auditory neuropathy spectrum disorder (ANSD) to acknowledge the heterogeneous and multifaceted nature of
this condition (Guidelines Development Conference, 2008).
a
University of North Carolina, Chapel Hill
American Speech-Language-Hearing Association,
Rockville, MD
Correspondence to Tobi Frymark: [email protected]
Editor: Sheila Pratt
Associate Editor: Ruth Litovsky
Received August 4, 2010
Revision received March 7, 2011
Accepted July 18, 2011
DOI: 10.1044/1059-0889(2011/10-0032)
b
Key Words: auditory neuropathy, auditory neuropathy
spectrum disorder, cochlear implantation, acoustic
amplification, hearing loss
American Journal of Audiology • Vol. 20 • 159–170 • December 2011 • A American Speech-Language-Hearing Association
159
1999; Franck, Rainey, Montoya, & Gerdes, 2002; Kraus
et al., 2000; Madden et al., 2002; Starr et al., 1998). As
universal newborn hearing screening has expanded and as
audiologists have become more familiar with the disorder
and its diagnosis, additional information has emerged regarding treatment for children with ANSD. Still, the heterogeneous nature of the disorder, compounded by the broad range
of auditory abilities, makes the audiologic management for
these children challenging and at times uncertain.
Currently, two technologies—acoustic amplification and
cochlear implantation—are recommended for children with
ANSD. Not surprisingly, there has been considerable debate among clinicians and investigators regarding the choice
of and expected benefit from these technologies. Those
opposed to conventional amplification emphasize the presumed limited benefits of hearing aid (HA) use for individuals
with normal outer hair cell functioning, arguing that acoustic
amplification offers only louder, more distorted signals
(Berlin, 1999). Others have expressed concern regarding the
potential consequence of acoustic trauma to existing outer
hair cells (Doyle, Sininger, & Starr, 1998). Concern has also
been raised regarding cochlear implant (CI) candidacy for
children with ANSD based on the reported involvement of
the auditory nerve (Miyamoto, Kirk, Renshaw, & Hussain,
1999). Others have expressed concern regarding cochlear
implantation because of spontaneous improvement in hearing
thresholds reported in a subset of children, especially those
with hyperbilirubinemia (Madden et al., 2002).
Although much has been learned about ANSD since its
original description by Starr and colleagues (1996), many
gaps remain in our knowledge of the clinical presentation
and audiologic management of the disorder in children. In
its most recent position statement, the Joint Committee on
Infant Hearing (JCIH, 2007) recommended that children
with auditory neuropathy-type hearing loss undergo a trial
with amplification and that decisions regarding continued
HA use be guided by the benefits derived from amplification.
In an effort to provide further guidance to clinicians working
with this population, consensus guidelines were recently
developed by an expert panel at an international conference
(Guidelines Development Conference, 2008). Although the
guidelines generated by the panel were not based on a systematic review of the evidence, panel members examined
the literature in their areas of expertise and prepared summary statements as a resource for clinicians involved with
the identification and management of infants presenting
with ANSD. The panel noted that many of the recommendations made for assessment and management of ANSD
were consistent with those recommended by the JCIH
(2007); however, additional considerations for acoustic
amplification, cochlear implantation, and habilitation were
outlined. The panel also emphasized the need for further
research on all aspects of the disorder, including a more
in-depth examination of the scientific literature. In light of
the diverse nature of ANSD, the unresolved controversies
surrounding intervention recommendations, and the high
degree of interest among audiologists (American SpeechLanguage-Hearing Association [ASHA], 2008), a systematic
review was undertaken to examine the current state of the
evidence.
This report documents the findings of an evidence-based
systematic review (EBSR) conducted by ASHA’s National
Center for Evidence-Based Practice in Communication Disorders. The primary aim of this review was to synthesize
and analyze existing evidence pertaining to audiologic treatment of children, birth to 18 years of age, diagnosed with
ANSD. The EBSR highlights the current state of evidence
for children diagnosed with ANSD and characterizes findings by severity and type of intervention. The report is
intended to serve as a resource to clinicians as they weigh
options for and against various treatment approaches for
children with ANSD. The findings reported here, along
with other crucial factors such as social/emotional development, speech-language skills, and parent preference, will
assist clinicians in determining what type of intervention
approach best meets the needs of children with ANSD.
The findings also elucidate the quality and level of existing
evidence in order to highlight areas where further study
is needed.
Eight clinical questions were established, a priori, to address the specific population, interventions, and outcomes
of interest (see Table 1). The population reviewed included
children diagnosed with ANSD from birth to 18 years of
age. The targeted interventions were (a) conventional acoustic amplification including HAs or frequency modulated
(FM) systems and (b) cochlear implantation. Several outcomes were considered, including the impact of HAs and CIs
on a child’s functional hearing and communication skills,
speech and language skills, academic performance, and social
skills. Auditory outcomes included, but were not limited to,
Table 1. Clinical questions included in the evidence-based
systematic review.
Question
number
1
2
3
4
5
6
7
8
160 American Journal of Audiology • Vol. 20 • 159–170 • December 2011
Clinical question
Amplification
For children with the diagnosis of auditory neuropathy,
what is the effect of acoustic amplification on
auditory outcomes?
For children with the diagnosis of auditory neuropathy,
what is the effect of acoustic amplification on speech
and language outcomes?
For children with the diagnosis of an auditory neuropathy,
what is the effect of acoustic amplification on
academic outcomes?
For children with the diagnosis of an auditory neuropathy,
what is the effect of acoustic amplification on social
emotional/parent outcomes?
Cochlear implantation
For children with the diagnosis of an auditory neuropathy,
what is the effect of cochlear implantation on auditory
outcomes?
For children with the diagnosis of auditory neuropathy,
what is the effect of cochlear implantation on
speech and language outcomes?
For children with the diagnosis of an auditory neuropathy,
what is the effect of cochlear implantation on
academic outcomes?
For children with the diagnosis of an auditory neuropathy,
what is the effect of cochlear implantation on social
emotional/parent outcomes?
unaided and aided pure-tone and speech detection thresholds,
unaided and aided speech perception measures, hearing in
noise, dichotic listening, and temporal processing (e.g., gap
detection). Examples of speech and language outcomes
included receptive and expressive language abilities and
speech intelligibility. With increased access to speech, language, and other environmental sounds and signals, it was
considered important to examine the impact of these interventions on academic performance. Academic outcomes
included, but were not limited to, reading, written language,
spelling, and educational achievement. Finally, as with any
intervention, it was considered important to examine the
effects of conventional acoustic amplification or cochlear
implantation on social and emotional well-being. Social /
emotional outcomes targeted measures of the child’s communication attitude or behavior (e.g., communication attitude
test, severity rating scales, self-report rating, quality of life
indicators, and social and communication measures). Parent
outcomes focused on any measure of parent attitude on communication (e.g., parent rating scales and parent surveys)
or a change in parental communication (e.g., pre/post parent
communication patterns).
Method
A review of the literature was conducted from October
2009 to July 2010 using search parameters and inclusion/
exclusion criteria set a priori. Approximately 25 databases
were searched (e.g., CINAHL [Cumulative Index to Nursing
and Allied Health Literature], ERIC [Education Resources
Information Center], and PubMed) using key words pertaining to auditory neuropathy, ANSD, auditory dyssynchrony,
and hearing loss. A full list of databases and key words is
provided in the supplementary material (Appendix A) affiliated with this article. Eligibility criteria for inclusion of
studies were as follows: treatment studies published in peerreviewed journals from 1990 to July 2010; publications
in English; and studies including children ages ≤18 years
diagnosed with ANSD receiving acoustic amplification
(HA or FM system) or cochlear implantation as a primary
audiologic intervention. Exclusionary criteria were studies based on animal models, pharmacological models, or
studies with mixed ages, mixed populations, or mixed treatments (e.g., combined HA and CI) unless data allowed for
separate analyses. Studies in which pretreatment and posttreatment measures or raw data were not reported were also
excluded.1 It is important to note that studies were not
excluded based on study design. Although limiting studies to group designs or randomized clinical trials would
reduce the potential for bias, the inclusion of all studies provides a more comprehensive look at the current body of
evidence.
Based on the aforementioned criteria, the second and
third authors independently reviewed all citations for relevance and completed a hand search of all references. The
first author reviewed the full bibliography of studies for
1
When applicable, correspondence with primary author was attempted to
obtain missing data.
completeness prior to final inclusion/exclusion. Any disagreement among authors regarding study eligibility was resolved
by consensus and documented as such. Additionally, the
second and third authors, blinded to each other’s results,
evaluated studies for methodological rigor based on ASHA’s
levels of evidence scheme (Frymark et al., 2009; Mullen,
2007). Studies were evaluated for methodological quality
based on the following indicators: (a) adequate description
of protocol for sufficient study replication, (b) blinding
of assessors, (c) random sampling/allocation procedures,
(d) evidence of treatment fidelity, (e) reporting significance
( p values) of findings or providing adequate data to allow
for statistical calculations, (f ) reporting of effect size and
confidence intervals or providing sufficient data to calculate
precision of treatment effects, and (g) use of intention-totreat analyses. In addition to evaluating methodological
quality, pertinent demographic, intervention, and outcomes
data were extracted from each study.
Results
Figure 1 outlines the systematic findings of the literature
search, which yielded a total of 202 citations, of which 37
were preliminarily accepted. Half of these studies (51%,
19/37) were further excluded upon review of the full text.
In total, 184 studies were eliminated from the review for the
following reasons: (a) did not examine a target intervention
(40%, 73/184); (b) was not the population or age under
review (22%, 40/184); (c) was not a study or systematic
review of the literature (12%, 22/184); (d) did not provide
original data or adequate data for analyses (12%, 22/184);
(e) was not a peer-reviewed study (9%, 17/184); or (f ) did not
directly address a clinical question (5%, 10/184). Eighteen
studies were included in the final analysis (see Appendix B
in the supplementary material for a full list of excluded studies and reasons for exclusion). Interrater reliability of study
eligibility agreement between coders was good, K = .787
(Cohen, 1960; Landis & Koch, 1977).
Participant Characteristics
Table 2 details the 115 participants across 18 studies;
60 were male, 39 were female, and gender was not reported
for 16. The total number of participants accounts for overlapping subjects reported in two Shallop studies (Shallop,
2002; Shallop, Peterson, Facer, Fabry, & Driscoll, 2001)
and two of three cohorts reported in the Teagle et al. (2010)
study. Age of ANSD diagnosis was early (≤4 years) for
66 participants. One study (Santarelli, Scimemi, Dal Monte,
Genovese, & Arslan, 2006) presented a single case report of a
female diagnosed with ANSD at age 14. For the remaining
48 participants, age at diagnosis was not reported. A number
of studies (n = 14) provided medical comorbidities or risk
factors associated with the disorder (e.g., hyperbilirubinemia,
Friedreich’s ataxia, or antibiotic treatments). The majority
of the participants (75%, 86/115) exhibited hearing loss ranging from severe to profound, while 18% (21/115) exhibited
moderate to severe or moderate hearing loss. Four participants (3%) exhibited mild hearing loss. Hearing severity was
Roush et al.: ANSD Systematic Review
161
Figure 1. Literature search selection and identification process for inclusion in the evidencebased systematic review (EBSR).
not reported for four participants. Severity of hearing loss
was primarily based on author report.
Acoustic Amplification
Table 3 outlines the intervention characteristics and major
findings of the 28 participants contributing data to Clinical
Question 1 (see Table 1); the majority of these (n = 26) were
in two case series reports (Rance et al., 1999, 2002). Severity of hearing impairments ranged from mild to profound
(mild = 14%, moderate = 29%, moderate to severe = 14%,
severe = 7%, and profound = 36%), and age of ANSD diagnosis ranged from 2 to 36 months. The authors from three
studies (Deltenre et al., 1999; Lin, Chen, & Wu, 2005; Rance
et al., 1999) reported that participants were fitted with binaural amplification. Participants from the two Rance et al.
studies (1999, 2002) were fitted using the National Acoustics
Laboratory prescriptions for gain, frequency response, and
output limiting levels for HA fitting (Byrne & Dillon, 1986).
The authors also reported that verification of HA settings
was completed. Limited information was provided regarding
prescriptive fitting method or verification strategy for the
Deltenre et al. (1999) and Lin et al. (2005) studies. Of the four
included studies, three (Deltenre et al., 1999; Lin et al., 2005;
Rance et al., 2002) provided pre- and posttreatment data to
investigate the effects of acoustic amplification on pure-tone
thresholds. All four studies examined the effects of amplification on speech perception. No studies addressed speech,
language, or academic outcomes (Clinical Questions 2, 3,
and 4; see Table 1).
Unaided and aided pure-tone thresholds were provided
for 20 participants with ANSD: 18 from the Rance et al.
(2002) case series study and two from the Deltenre et al.
(1999) and Lin et al. (2005) case study reports. Children
from Rance et al. (2002) were identified with ANSD at a
mean age of 6 months, with pure-tone thresholds ranging
from mild to profound. Mean pure-tone average (PTA)
was 70 dB HL at 0.5, 1, and 2 kHz prior to amplification.
HAs were fitted at a mean age of 8 months, with audiologic
follow up ≥12 months postintervention. Although aided PTA
thresholds were not reported at posttesting, the authors
noted improved aided detection thresholds of approximately
30 dB for individual frequencies (see Table 3). The two additional case studies (Deltenre et al., 1999; Lin et al., 2005)
revealed similar findings. Deltenre et al. (1999) investigated
the unaided and aided performance of a female with auditory
neuropathy-type hearing loss with pure-tone thresholds in
the moderate range diagnosed at 2 months of age, while
Lin and colleagues (2005) examined a male diagnosed with
auditory neuropathy-type hearing loss with pure-tone thresholds in the profound range at age 3. Both authors reported
improvement in aided PTAs of approximately 20 dB and
60 dB, respectively.
The four included studies (Deltenre et al., 1999; Lin et al.,
2005; Rance et al., 1999, 2002) also assessed aided speech
perception outcomes of participants with ANSD. Of the
24 of 28 participants with probability data reported, half
(50%, 12/24) demonstrated no significant difference in aided
speech perception abilities. The single case report by Lin
et al. (2005) reported no significant difference ( p > .01)
on the Mandarin Auditory Perception Test Battery (Wu &
Yang, 2003) during a 2-year treatment period; however, it
should be noted that the participant in this study had unaided
pure-tone thresholds of 111 dB HL and >112 dB HL (fourfrequency PTA). Rance and colleagues (2002) reported
mixed speech perception scores for children with ANSD
who used amplification for a minimum of 12 months prior to
participating in the study. Seven of 15 participants tested
exhibited no significant difference in Phonetically Balanced
Kindergarten test (PBK; Haskins, 1949) phoneme scores
in the aided condition. The remaining eight showed a mean
difference in aided versus unaided PBK phonemes of 57%
with a mean aided PBK phoneme score of 67%. Interestingly, cortical evoked event-related potentials were present
162 American Journal of Audiology • Vol. 20 • 159–170 • December 2011
Table 2. Subject variables—all included studies.
Audiologic findings
N
Gender
(M / F )
Age at
diagnosis
Buss et al. (2002)
4
NR
Birth
Deltenre et al. (1999)
1
0/1
2 months
Jeong et al. (2007)
9
5/4
M = 20 months
Lin et al. (2005)
Madden et al. (2002)
1
2
1/0
1/1
Mason et al. (2003)
Miyamoto et al. (1999)
1
1
Rance & Barker (2008)
Rance et al. (1999)
Rance et al. (2002)
Citation
Risk factors/
medical comorbidities
ABR
OAE
CM
HL severity
Previous
intervention
Roush et al.: ANSD Systematic Review
P3 = Mondini’s malformation
P1, P2, P4 = NR
Hyperbilirubinemia,
respiratory distress
2 hyperbilirubinemia
–
–
+
Severe-profound
HA
–
–
+
Moderate
NR
–
+
NR
None
–
–
NR
NR
6 severe-profound,
3 CNT
Profound
Profound
HA
36 months
NR
3–
6+
+
+
0/1
1/0
NR
48 months
None
Friedreich’s ataxia
–
–
+
+
+
NR
Moderate
Profound
10
8
7/3
NR
≤3 months
≤9 months
–
–
+
NR
+
+
18
14/4
M = 6 months
–
7–
11+
+
Raveh et al. (2007)
Rouillon et al. (2006)
4
2
NR
0/2
–
NR
+
NR
+
NR
Santarelli et al. (2006)
Shallop (2002)a
Shallop et al. (2001)a
Teagle et al. (2010)
1
10
5
Group B = 15
0/1
6/4
3/2
9/6
NR
P1 = 22 months
P2 = 10 months
14 years
M = 14 months
38 months
NR
6 jaundice, 4 hypoxia
4 jaundice, 3 hypoxia,
1 low birth weight, 1 none
7 jaundice, 6 hypoxia,
1 hydrocephalus,
1 meningitis, 2 none
NR
Genetic
Systemic sclerosis
Congenital deafness
NR
9 prematurity,
7 bronchopulmonary dysplasia,
3 hyperbilirubinemia,
4 retinopathy
–
–
–
–
+
+
+
NR
+
+
+
+
Group C = 26
15/11
NR
–
NR
+
1
1
1/0
0/1
35 months
Birth
10 prematurity,
2 bronchopulmonary dysplasia,
1 hyperbilirubinemia,
1 Turner’s syndrome,
1 sensorimotor neuropathy,
11 none
NR
Hyperbilirubinemia
–
NR
–
NR
+
NR
Trautwein et al. (2000)
Vermeire et al. (2003)
Severe-profound
2 mild, 2 moderate,
4 profound
2 mild, 5 moderate,
4 moderate-severe,
2 severe, 5 profound
Severe-profound
Profound
Severe
Severe-profound
Severe-profound
2 moderate,
1 moderate-severe,
3 severe,
2 severe-profound,
6 profound, 1 CNT
5 moderate, 5 severe,
16 profound
Severe-profound
Profound
HA
HA
FM
NR
HA
FM
HA
None
NR
HA
HA
None
NR
HA
HA
HA
HA
HA
Note. ABR = auditory brainstem response; OAE = otoacoustic emissions; CM = cochlear microphonics; HL = hearing level; NR = not reported; P = participant; + = present; – = absent;
HA = hearing aid; CNT = could not test; FM = frequency modulated system.
a
Shallop (2002) and Shallop et al. (2001) had overlapping participants.
163
Table 3. Studies examining acoustic amplification.
Citation
Deltenre et al.
(1999)
N
1
Age at
intervention
48 months
Fitting
method/model
NR/binaural
Duration/
compliance
1
NR
NR/binaural
30 months
Rance et al.
(1999)
8
NR
BTE/binaural
≤12 months
18
8 months
BTE/ NR
a
Unaided
50 dB
PTA
Speech identification at 55 dB
Phonemes
0%
Words
0%
a
111 dB/>112 dB
PTA (R/L)
MAPTB
46%
PBK words
P13
8%
P16
32%
Picture Vocabulary Test b
P8
3/12
P10
1/12
P12
2/12
P14
2/24
P15
1/12
P17
3/12
c
70 dB
PTA
5 kHz
NR
1 kHz
NR
2 kHz
NR
4 kHz
NR
PBK phonemes (n = 15)
8%
36 months
Lin et al. (2005)
Rance et al.
(2002)
Outcomes measured
≤12 months
Aided
29 dB
96%
80%
50 dB/51 dB
51%
43%
88%
3/12
1/12
8/12
4/24
8/12
2/12
NR
42 dB
42 dB
44 dB
44 dB
32%
Note. PTA = pure-tone average; MAPTB = Mandarin Auditory Perception Test Battery (Wu & Yang, 2003); BTE = behind the ear; PBK =
Phonetically Balanced Kindergarten test (Haskins, 1949).
a
PTA threshold at 0.5, 1, 2, and 4 kHz.
Subtest of the PLOTT Test (Plant & Westcott, 1983).
c
PTA threshold at 0.5, 1, and 2 kHz.
b
in the group of children with significant open-set speech
abilities but were absent in those who had no open-set abilities. In comparison, a matched control group of children
with non-auditory neuropathy SNHL from the same study
showed a mean difference score of 49%, with a mean aided
PBK phoneme score of 66%. Similar findings were reported
by Rance et al. (1999), with significant differences found
between unaided and aided speech recognition ( p < .01) in
four of the eight participants tested. Although probability
data were not reported by Deltenre et al. (1999), the authors
also reported gains of ≥80% in aided speech recognition
for speech presented at 55 dB HL.
While outcome data showing the effects of acoustic amplification for children with ANSD are limited, the results of
this review suggest that some children with ANSD benefit
from improved aided detection levels with use of HAs. There
appears to be a subset of children who experience benefits
in aided speech perception abilities in addition to improved
pure-tone detection thresholds.
Cochlear Implantation
Fifteen studies (see Table 4) examined the effects of CI
interventions for 88 children who had been diagnosed with
ANSD from birth to 14 years of age. Eighty-two participants
received CI intervention at ≤5 years of age (range = 21–
68 months), and two participants (one each from Miyamoto
et al., 1999, and Santarelli et al., 2006) received a CI at
ages 10.9 and 18 years, respectively. Two studies (Lin et al.,
2005; Raveh, Buller, Badrana, & Attias, 2007) did not
provide information regarding age at intervention. The
majority of studies (80%, 12/15) reported unsuccessful trials
of acoustic amplification (HA and /or FM systems) prior to
CI intervention. Of the remaining three studies, two (Mason,
De Michele, Stevens, Ruth, & Hashiasaki, 2003; Shallop,
2002) did not provide previous intervention history, while
one (Santarelli et al., 2006) indicated no HA trial prior to
CI intervention. Contrary to the heterogeneity among children receiving hearing amplification interventions, 85% of
the children who received cochlear implantation exhibited
severe to profound hearing loss (n = 75).
All 15 studies contributed data to Clinical Question 5
(see Table 1). Six studies reported changes in pure-tone thresholds (Buss et al., 2002; Jeong, Kim, Kim, Bae, & Kim, 2007;
Mason et al., 2003; Miyamoto et al., 1999; Trautwein,
Sininger, & Nelson, 2000; Vermeire, Brokx, Van de Heyning,
Cochet, & Carpentier, 2003), and 12 reported speech perception outcomes (Jeong et al., 2007; Lin et al., 2005; Madden
et al., 2002; Miyamoto et al., 1999; Rance & Barker, 2008;
Raveh et al., 2007; Rouillon et al., 2006; Santarelli et al.,
2006; Shallop, 2002; Shallop et al., 2001; Teagle et al., 2010;
Trautwein et al., 2000). Raveh et al. (2007) also reported speech
and language outcomes (Clinical Question 6). No studies
were found that examined social/emotional or academic outcomes after cochlear implantation (Clinical Questions 7 and 8).
Similar to the findings pertaining to HA interventions,
all participants with ANSD exhibited improved pure-tone
detection thresholds after CI. Six studies reported improvement in PTA thresholds ranging from 40 to 87 dB, five
of which assessed PTAs at 0.5, 1, 2, and 3 or 4 kHz (Buss
164 American Journal of Audiology • Vol. 20 • 159–170 • December 2011
Table 4. Studies examining cochlear implantation (CI).
Citation
N
Buss et al.
(2002)
4
Jeong et al.
(2007)
Age at intervention
Duration/
compliance
Model
Clarion
12 months
9
P1 = 25 months
P2 = 28 months
P3 = 68 months
P4 = 62 months
M = 4 years
Nucleus
M = 24 months
Lin et al.
(2005)
1
NR
Clarion
NR
Madden et al.
(2002)
2
P1 = 39 months
Clarion
7 months
Mason et al.
(2003)
Miyamoto et al.
(1999)
1
P2 = 21 months
32 months
Clarion
NR
24 months
5 months
1
10.9 years
Nucleus
12 months
Rance & Barker
(2008)
Raveh et al.
(2007)
10
M = 33 months
Nucleus
>24 months
4
NR
NR
NR
Rouillon et al.
(2006)
Santarelli et al.
(2006)
2
P1 = 35 months
P2 = 48 months
18 years
Nucleus
Nucleus
Nucleus
1 months
18 months
NR
d
1
Shallop (2002)
10
M = 45 months
Shallop et al.
(2001)d
Teagle et al.
(2010)
5
M = 53 months
15
Group B
M = 30 months
26
Group C
M = 63 months
Trautwein et al.
(2000)
1
39 months
Vermeire et al.
(2003)
1
R = 29 months
L = 52 months
9 nucleus
1 clarion
Nucleus
NR
Outcomes measured
Pre-CI
Post-CI
101 dB
101 dB
104 dB
90 dB
83 dB
0
5%
3%
36 dB
31 dB
36 dB
26 dB
29 dB
5
59%
46%
35%
25%
70%
70%
50%
64%
90%
90%
100%
100%
100%
88%
0/40
70–80 dB
27/40
35–40 dB
PTAc
PBK
Words
Phonemes
MW test
97 dB
4%
4%
12%
<40%
IT-MAIS
Ling Test
CAP
MUSS
MAIS
MAIS
TIPI
Vowel
identification
Consonant
identification
MAIS
18%
.75
1
3%
4/40
4/40
39 dB
4%
4%
20%
>55%
M = 59.6%
56%
5
3.5
29%
40/40
31/40
52%
95%
25%
55%
8%
91%
1
4
26% (21%)
79% (16%)
14%
49%
54% (34%)
76% (22%)
22/30
1
104 dB
110 dB
27/30
4
40 dB
23 dB
a
P1 PTA
P2 PTAa
P3 PTAa
P4 PTAa
PTAb (n = 6)
CAP
MW test
Common phrase test
MAPTB
Spondee
Vowel
Tone
Phrase
Sentence
Closed-set
discrimination
IT-MAIS
PTAa
M = 18 months
ESP
10 nucleus
4 HiRes90
1 sonota
5 Nucleus
8 NucleusFreedom
8 HiRes90
3 Clarion
2 Med El
Nucleus
M = 20 months
IT-MAIS
M = 50 months
PPK
Words
Phonemes
Nucleus
9 months after
2nd CI
12 months
Ling Test
ESP
PTAb (18 months)
PTAb
Note. CAP = Categories of Auditory Performance (Archbold et al., 1995); MW = monosyllabic word; IT-MAIS = Infant-Toddler Meaningful
Auditory Integration Scale (Zimmerman-Phillips, Osberger, & Robbins, 1997); Ling Test = Ling 6 Sound Test (Ling, 1976); MUSS = Meaningful
Use of Speech Scale (Robbins & Osberger, 1991); MAIS = Meaningful Auditory Integration Scale (Robbins, 1998); TIPI = Test di Identificazione
Parole Infantili (Test of Identification of Words for Children; Arslan et al., 1997); ESP = Early Speech Perception Test (Moog & Geers, 1990);
L = left; R = right.
a
PTA threshold at 0.5, 1, 2, and 4 kHz.
PTA threshold at 0.5, 1, 2, and 3 kHz.
c
PTA threshold at 0.5, 1, and 2 kHz.
d
Shallop (2002) and Shallop et al. (2001) had overlapping participants.
b
Roush et al.: ANSD Systematic Review
165
et al., 2002; Jeong et al., 2007; Mason et al., 2003; Trautwein
et al., 2000; Vermeire et al., 2003), and one (Miyamoto et al.,
1999) that assessed PTA thresholds at 0.5, 1, and 2 kHz.
Improvements were also noted across studies examining
speech perception outcomes. However, it is important to
note that studies varied greatly in type of speech perception
measure reported, making it difficult to interpret the results
across studies.
Two studies (Rance & Barker, 2008; Shallop et al., 2001)
reported significant differences in speech perception ability
after cochlear implantation. Rance and Barker (2008) examined the speech perception skills of 10 children with a mean
age at CI of 33 months, nine of whom were diagnosed with
ANSD at ≤3 months of age. The authors reported significant
improvement in consonant-nucleus-consonant (CNC) phoneme scores ( p = .006) after CI. Additionally, 10 participants in
the Shallop (2002) study who completed pre- and posttesting
demonstrated significant improvement on the Meaningful
Auditory Integration Scale (MAIS; Robbins, 1998; p < .0001).
These participants were diagnosed with ANSD at 14 months
of age, receiving CI at a mean age of 45 months. Although
pre-CI data were not provided, Shallop et al. (2001) also
reported significant improvements in speech awareness thresholds and/or speech reception thresholds of five participants
with auditory neuropathy-type hearing loss. In this study, mean
age of implantation was 53 months, and mean diagnosis was
38 months of age. Mean duration of CI use was reportedly
18 months. Additionally, the authors reported that participants
were unable to discriminate speech (Early Speech Perception
Test [ESP] Category 1; Moog & Geers, 1990) prior to CI
intervention and demonstrated consistent ability to discriminate words (ESP Category 4) postimplantation.
Lastly, the recent longitudinal study by Teagle et al. (2010)
followed the largest cohort of participants receiving CI interventions to date (n = 52). In this study, participants were
identified with ANSD through newborn hearing screening protocols. The mean preoperative PTA threshold was
88 dB HL, and the mean duration of HA use prior to CI
was reportedly 26 months. Of the 52 participants studied,
11 (Group A) did not have sufficient pre- and postdata for
analyses; however, the remaining 41 were included and
demonstrated improved speech perception abilities. One
cohort (Group B) consisted of 15 participants with CI use for
>6 months who were unable to participate in open-set speech
perception testing due to young age or development delays;
however, 13 of 15 participants in this group provided preand postdata on the Infant-Toddler Meaningful Auditory Integration Scale (Zimmerman-Phillips, Osberger, & Robbins,
1997) with a mean improvement of 53% postimplantation.
The other cohort (Group C) had 26 CI users who could perform open-set measures. Although pre-CI data were not
reported for all 26 participants who completed PBK testing, the authors reported a mean improvement of 40% and
27%, respectively, for PBK words and phonemes, with
mean PBK phoneme and word scores postimplantation of
76% (SD = 22%, range = 24%–100%) and 54% (SD = 34%,
range = 4%–100%), respectively.2
2
The first author was a coauthor on Teagle et al. (2010) and provided the
PBK data for Cohort C.
The findings detailed in Table 4 suggest that children
with ANSD with thresholds in the severe to profound range
may benefit from CI interventions. Interestingly, Rance
and Barker (2008) found no significant difference in performance on CNC phoneme scores between children with
ANSD who received CI intervention compared to a group
who received HA intervention, d = 0.20, 95% CI [–0.69,
1.07]. They did note that children in their cohort who were
among the poorer performers with HAs had already been
referred for CI. Furthermore, the authors went on to report
that 10 age-matched CI users with SNHL performed significantly better than both the CI and HA users with ANSD
( p = .0006).
Finally as mentioned above, only one study (Raveh et al.,
2007) examined the effect of CI intervention on speech and
language outcomes of children with ANSD. Of the 18 participants with severe to profound loss initially included
in the study, only four had sufficient follow-up testing
post-CI using the Meaningful Use of Speech Scale (Robbins
& Osberger, 1991) allowing for inclusion in the review.
Mean performance was 3% preimplantation and 29% postimplantation. Although sufficient pre- and postdata were
not provided by Buss and colleagues (2002) for inclusion
in Clinical Question 6 (see Table 1), the authors noted that
participants with ANSD using CI performed comparably
to age-matched CI users with SNHL on a speech production
test (Paden–Brown Test; Paden & Brown, 1992). The authors
stated that three of the four participants performed “within
or slightly above the confidence interval one standard deviation around the control group mean” (Buss et al., 2002,
pp. 330–331).
Methodological Quality
The methodological quality of the included studies is
outlined in Table 5. Level of interrater agreement between
authors for appraisal of study quality was good (K = .742;
Landis & Koch, 1977). All studies were case series reports
or single case studies. While the majority of studies (67%,
12/18) provided an adequate description of the treatment
protocol, all 18 studies recruited participants using a method
of convenience sampling and did not report blinding of assessors. Approximately one third of the studies (39%, 7/18)
reported effect size or probability data. Two quality indicators
(evidence of treatment fidelity and use of intention-to-treat
analysis) were not applicable to the types of studies included
in this review and therefore not considered.
Discussion
The purpose of this EBSR was to investigate the impact
of two hearing technologies, acoustic amplification and cochlear implantation, on outcomes in children with ANSD.
A systematic review of the literature from 1990 to 2010
yielded 18 studies that met the predetermined inclusion
criteria. Only four studies contributed data to address the
impact of acoustic amplification on auditory outcomes,
while the majority of studies (83%, 15/18) employed cochlear implantation. Only one study (Raveh et al., 2007) provided data on speech and language outcomes after cochlear
166 American Journal of Audiology • Vol. 20 • 159–170 • December 2011
Table 5. Methodological quality of included studies.
Citation
Study
design
Adequate
description
of protocol
Assessors
blinded
Sampling/
allocation
Significance
( p values)
Precision
(ES/CI)
Clinical
question(s)
Buss et al. (2002)
Deltenre et al. (1999)
Jeong et al. (2007)
Lin et al. (2005)
Madden et al. (2002)
Mason et al. (2003)
Miyamoto et al. (1999)
Rance & Barker (2008)
Rance et al. (1999)
Rance et al. (2002)
Raveh et al. (2007)
Rouillon et al. (2006)
Santarelli et al. (2006)
Shallop (2002)
Shallop et al. (2001)
Teagle et al. (2010)
Trautwein et al. (2000)
Vermeire et al. (2003)
Case study
Case study
Case series
Case study
Case study
Case study
Case study
Case series
Case series
Case series
Case series
Case study
Case study
Case series
Case series
Case series
Case study
Case study
+
–
+
+
+
–
+
+
–
–
–
–
+
+
+
+
+
+
–
–
–
–
–
–
–
–
–
–
–
–
–
–
–
–
–
–
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
Convenience
–
–
–
+
–
–
+
+
+
+
–
–
–
–
+
+
–
–
–
–
–
–
–
–
–
+
–
–
–
–
–
–
–
+
–
–
5
1
5
1, 5
5
5
5
5
1
1
5, 6
5
5
5
5
5
5
5
Note.
ES = effect size; CI = confidence interval.
implantation. No studies reported social, academic, or parent
outcomes for either technology.
For both the CI and the HA groups, the majority of
participants studied (75%, 86/115) had pure-tone thresholds
in the severe or profound range. Yet, Sanyelbhaa Talaat,
Kabel, Samy, and Elbadry (2009) found that only 13% of
children diagnosed with ANSD exhibited severe to profound
hearing loss. The majority of participants (93%, 26/28)
studied with HAs were from two studies by Rance et al.
(1999, 2002). Two case studies compose the remainder
of reports on outcomes with amplification (Deltenre et al.,
1999; Lin et al., 2005). Although not directly addressed
in this review, Rance et al. (2002) found no correlation
between pure-tone thresholds and performance on speech
perception tasks for participants with ANSD. This suggests
that degree of pure-tone hearing loss may not be a useful
predictor of aided speech perception abilities. That is, some
children with greater pure-tone hearing loss may experience
improved speech perception with amplification, while others
with milder degrees of pure-tone loss may not. This finding
is in contrast to studies of children and adults with cochlear
hearing loss who have been shown to demonstrate poorer
speech perception ability with increasing degree of pure-tone
hearing loss (Bamford, Wilson, Atkinson, & Bench, 1981;
Boothroyd, 1997; Walden, 1984; Yellin, Jerger, & Fifer, 1989).
In this EBSR, most of the children with ANSD who
received CIs had at least a severe hearing loss (85%, 75/88);
only nine with mild or moderate degrees of pure-tone hearing
loss were implanted. The majority of CI studies were with
children who had reportedly experienced a failed attempt with
acoustic amplification; however, information was often limited
regarding type of amplification, prescriptive fitting method,
verification strategy employed, or tests used to evaluate aided
benefit. Although most studies reported improved auditory
outcomes for both pure-tone detection thresholds and speech
perception scores following cochlear implantation, as is typical
in children with cochlear hearing loss, a broad range of postimplantation speech perception abilities was observed. For
example, in the Teagle et al. (2010) study, of the 41 of
52 implanted children who had speech perception measures,
only 26 were able to perform open-set word recognition testing despite >2 years of device use. Twenty-seven percent of
those able to perform open-set testing achieved a score of
<30% on PBK word testing, which is below the current level
of performance established by CI manufacturers in cooperation with the U.S. Food and Drug Administration as criteria
level for determining implant candidacy (U.S. Department
of Health and Human Services, 2009).
The findings from this review do not resolve the controversies surrounding the audiologic treatment of ANSD
in children. The review indicates that some children with
ANSD benefit from acoustic amplification, while others
benefit from cochlear implantation. Furthermore, the methodological limitations of the studies, the heterogeneity of
the participants, and the varied outcomes reported provide
insufficient clinical evidence to guide the practicing clinician.
Especially lacking are HA performance data for children
with ANSD whose pure-tone thresholds are in the mildto-moderate range. Of the four studies that evaluated HA
outcomes, only 12 participants had pure-tone thresholds in
this range. These findings do, however, provide a foundation
for further study.
Most of the studies reviewed in this report focus on
auditory outcomes obtained from measures of audibility
for speech, and the ability to perceive and recognize sounds
or words. Further research is needed to address other functional aspects including speech, language, learning, social/
emotional development, and psycho-educational performance. Moreover, considering the heterogeneity of this
population and the varied etiologies associated with ANSD
(e.g., prenatal, genetic, and neurological), there is a need
for additional studies with more homogeneous grouping
Roush et al.: ANSD Systematic Review
167
of children to assess the impact of various comorbidities
on auditory, speech, language, and learning outcomes.
It is important to note that the reports we have cited, and
indeed many of those not included in this review, have made
important contributions to the existing body of knowledge
regarding ANSD. Considering the relatively recent discovery
of this clinical population and its low prevalence, it is not
surprising that most of the studies investigating ANSD in
children represent exploratory research from case series or case
study reports. To determine the efficacy of acoustic amplification and cochlear implantation with this population, wellcontrolled, prospective, longitudinal studies are needed on larger
groups of children with detailed descriptions of participants.
It is important to remember that predicting benefit from
cochlear implantation or amplification in young children is
difficult, not only for the population of children with ANSD
but also for young children with non-auditory neuropathytype hearing loss of varying degrees. Several factors appear
to affect outcomes: age at identification and device fitting,
quality of the fitting, consistency of use, type of intervention,
and degree of parental involvement. Furthermore, there are
numerous barriers to obtaining accurate outcome measures in
the pediatric population, including age at time of assessment,
attention span, speech and language abilities, and cognitive
status (Boothroyd, 1997). Despite these challenges, the
application of age-appropriate, evidence-based measures
should make it possible to acquire additional knowledge
regarding the benefits and limitations of currently available
hearing technologies for children with ANSD. Other factors needing further investigation include risk factors for
ANSD and the role of electrophysiological measures such
as cortical event-related potentials and electrocochleography
to predict the benefit from various interventions. Key points
in this EBSR are summarized as follows:
• As many as 10% of children born with permanent hearing
loss present with clinical evidence of ANSD, yet few
studies are available to guide clinicians in the management of this disorder or to guide families in the decisionmaking process.
• Most of the children studied to date have severe or
profound hearing loss. Further research with children
who have milder degrees of sensitivity loss is needed.
• Group studies often include children with a variety of
medical histories and etiologies. There is a need for
well-controlled, longitudinal prospective studies that
provide homogeneous grouping of participants (i.e.,
genetic, neurological, prematurity/low birth weight,
and hyperbilirubinemia).
• For children who use amplification and for those using
CIs, there is a need for studies that combine electrophysiological measures (i.e., cortical evoked potentials,
electocochleography, and ABR) with medical/radiologic
evaluation and performance.
• Studies of children with CIs who were implanted based
on a history of failed attempts using amplification must
include a detailed description of the fitting method, verification strategies, and outcome measures employed. This
information is needed to assist clinicians and researchers
with interpretation of the data and evaluation of the
criteria for unsuccessful HA use.
• There is a need for studies that go beyond auditory
performance to include a full range of developmental
outcomes.
Although our understanding of ANSD and its management is at an early stage, we hope this EBSR will be useful to
clinicians and researchers. Until further scientific evidence
is available to direct the clinical decision-making process,
audiologists should be guided by the peer-reviewed literature
and by the recommendations of national and international
professional organizations, in combination with clinical
experience, empirical observation, and family preference.
Acknowledgments
This work was supported by the ASHA National Center for
Evidence-Based Practice in Communication Disorders. We thank
the authors of the articles studied for their contributions to the
research on ANSD.
References
References marked with an asterisk indicate studies included
in the review.
American Speech-Language-Hearing Association. (2008).
Knowledge, attitudes and practice survey on evidence-based
practice in audiology and speech-language pathology.
Rockville, MD: Author.
Archbold, S., Lutman, M., & Marshall, D. (1995). Categories
of auditory performance. Annals of Otology, Rhinology, and
Laryngology, 104(Suppl. 166), 312–314.
Arslan, E., Genovese, E., Orzan, E., & Turrini, M. (1997). Test
di Identificazione di Parole Infantili [Test of Identification of
Words for Children]. In Amplifon Valutazione della percezione
verbale nel bambino ipoacusico [Amplifon assessment of verbal
perception in the hearing impaired child]. Bari, Italy: Ecumenica
Editrice.
Bamford, J., Wilson, I., Atkinson, D., & Bench, J. (1981).
Pure tone audiograms from hearing-impaired children. II.
Predicting speech-hearing from the audiogram. British Journal
of Audiology, 15, 3–10.
Berlin, C. I. (1999). Auditory neuropathy: Using OAEs and ABRs
from screening to management. Seminars in Hearing, 20,
307–315.
Boothroyd, A. (1997). Auditory development of the hearing child.
Scandinavian Audiology, 26, 9–16.
Buchman, C. A., Roush, P. A., Teagle, H., Brown, C. J.,
Zdanski, C. J., & Grose, J. H. (2006). Characteristics of
auditory neuropathy in children with cochlear nerve deficiency.
Ear and Hearing, 27, 399–408.
*Buss, E., Labadie, R., Brown, C., Gross, A., Grose, J., &
Pillsbury, H. (2002). Outcome of cochlear implantation in
pediatric auditory neuropathy. Otology and Neurotology, 23,
328–332.
Byrne, D., & Dillon, H. (1986). The National Acoustic Laboratories (N.A.L.) new procedure for selecting the gain and
frequency response of a hearing aid. Ear and Hearing, 7,
257–265.
Cohen, J. (1960). A coefficient of agreement for nominal scales.
Educational and Psychological Measurement, 20, 37–46.
168 American Journal of Audiology • Vol. 20 • 159–170 • December 2011
*Deltenre, P., Mansbach, A. L., Bozet, C., Christiaens, F.,
Barthelemy, P., Paulissen, D., & Renglet, T. (1999). Auditory neuropathy with preserved cochlear microphonics and
secondary loss of otoacoustic emissions. International Journal
of Audiology, 38, 187–195.
Deltenre, P., Mansbach, A. L., Bozet, C., Clercx, A., & Hecox,
K. (1997). Auditory neuropathy: A report on three cases with
early onsets and major neonatal illnesses. Electroencephalography and Clinical Neurophysiology, 104, 17–22.
Doyle, K. J., Sininger, Y., & Starr, A. (1998). Auditory neuropathy in childhood. Laryngoscope, 108, 1374–1377.
Franck, K. H., Rainey, D. M., Montoya, L. A., & Gerdes, M.
(2002). Developing a multidisciplinary clinical protocol to
manage pediatric patients with auditory neuropathy. Seminars
in Hearing, 23, 225–237.
Frymark, T., Schooling, T., Mullen, R., Wheeler-Hegland, K.,
Ashford, J., McCabe, D., . . . Hammond, C. S. (2009).
Evidence-based systematic review: Oropharyngeal dysphagia
behavioral treatments. Part I—Background and methodology.
Journal of Rehabilitation Research and Development, 2, 175–184.
Guidelines Development Conference on the Identification and
Management of Infants with Auditory Neuropathy. (2008).
Auditory neuropathy spectrum disorder (ANSD) guidelines.
Retrieved from www.thechildrenshospital.org/conditions/
speech/danielscenter/ANSD-Guidelines.aspx.
Haskins, H. (1949). A phonetically balanced test of speech
discrimination for children (Unpublished master’s thesis).
Northwestern University, Evanston, IL.
*Jeong, S. W., Kim, L. S., Kim, B. Y., Bae, W. Y., & Kim,
J. R. (2007). Cochlear implantation in children with auditory
neuropathy: Outcomes and rationale. Acta Oto-Laryngologica,
127(Suppl. 558), 36–43.
Joint Committee on Infant Hearing: American Academy of
Audiology, American Academy of Pediatrics, American
Speech-Language-Hearing Association, and Directors of
Speech Hearing Programs in State Health and Welfare
Agencies. (2007). Year 2007 position statement: Principles
and guidelines for early hearing detection and intervention
programs. Pediatrics, 120, 898–921.
Kraus, N., Bradlow, A., Cheatham, M., Cunningham, J.,
King, C., Koch, D., . . . Wright, B. (2000). Consequences of
neural asynchrony: A case of auditory neuropathy. Journal of
the Association for Research in Otolaryngology, 1, 33–45.
Landis, J. R., & Koch, G. G. (1977). The measurement of observer agreement for categorical data. Biometrics, 33, 159–174.
*Lin, C. Y., Chen, Y. J., & Wu, J. L. (2005). Cochlear implantation in a Mandarin Chinese-speaking child with auditory
neuropathy. European Archives of Otorhinolaryngology, 262,
139–141.
Ling, D. (1976). Speech and the hearing-impaired child: Theory
and practice. Washington, DC: Alexander Graham Bell Association for the Deaf.
*Madden, C., Rutter, M., Hilbert, L., Greinwald, J. H., Jr., &
Choo, D. (2002). Clinical and audiological features in auditory
neuropathy. Archives of Otolaryngology—Head & Neck Surgery, 128, 1026–1030.
*Mason, J. C., De Michele, A., Stevens, C., Ruth, R. A., &
Hashisaki, G. T. (2003). Cochlear implantation in patients with
auditory neuropathy of varied etiologies. Laryngoscope, 113,
45–49.
*Miyamoto, R., Kirk, K. I., Renshaw, J., & Hussain, D. (1999).
Cochlear implantation in auditory neuropathy. Laryngoscope,
109, 181–185.
Moog, J., & Geers, A. (1990). The Early Speech Perception Test
for profoundly hearing-impaired children. St. Louis, MO:
Central Institute for the Deaf.
Mullen, R. (2007, March 6). The state of the evidence: ASHA
develops levels of evidence for communication sciences and
disorders. The ASHA Leader. Retrieved from www.asha.org /
about/publications/leader-online/archives/2007/070306/
f070306b.htm.
Paden, E. P., & Brown, C. J. (1992). Identifying early phonological needs in children with hearing loss. Innsbruck, Austria:
Med-El.
Plant, G., & Westcott, S. (1983). The PLOTT Test. Chatswood,
Australia: National Acoustic Laboratories.
Rance, G. (2005). Auditory neuropathy/dys-synchrony and its
perceptual consequences. Trends in Amplification, 9, 1–43.
*Rance, G., & Barker, E. J. (2008). Speech perception in children with auditory neuropathy/dyssynchrony managed with
either hearing aids or cochlear implants. Otology & Neurotology,
29, 179–182.
Rance, G., Barker, E., Mok, M., Dowell, R., Rincon, A., &
Garratt, R. (2007). Speech perception in noise for children
with auditory neuropathy/dys-synchrony type hearing loss.
Ear and Hearing, 28, 351–360.
*Rance, G., Beer, D. E., Cone-Wesson, B., Shepherd, R. K.,
Dowell, R. C., King, A. M., . . . Clark, G. M. (1999). Clinical
findings for a group of infants and young children with auditory
neuropathy. Ear and Hearing, 20, 238–252.
*Rance, G., Cone-Wesson, B., Wunderlich, J., & Dowell, R.
(2002). Speech perception and cortical event related potentials
in children with auditory neuropathy. Ear and Hearing, 23,
239–253.
*Raveh, E., Buller, N., Badrana, O., & Attias, J. (2007).
Auditory neuropathy: Clinical characteristics and therapeutic
approach. American Journal of Otolaryngology, 28, 302–308.
Robbins, A. M. (1998). Meaningful Auditory Integration Scale
(MAIS). In W. Estabrooks (Ed.), Cochlear implants for kids
(pp. 373–378). Washington, DC: Alexander Graham Bell
Association for the Deaf.
Robbins, A. M., & Osberger, M. I. (1991, November). Meaningful Use of Speech Scale (MUSS). Paper presented at the Annual
Convention of the American Speech-Language-Hearing
Association, Atlanta, GA.
*Rouillon, I., Marcolla, A., Roux, I., Marlin, S., Feldmann, D.,
Couderc, R., . . . Loundon, N. (2006). Results of cochlear
implantation in two children with mutations in the OTOF gene.
International Journal of Pediatric Otorhinolaryngology, 70,
689–696.
*Santarelli, R., Scimemi, P., Dal Monte, E., Genovese, E., &
Arslan, E. (2006). Auditory neuropathy in systemic sclerosis:
A speech perception and evoked potential study before and after
cochlear implantation. European Archives of Otorhinolaryngology, 263, 809–815.
Sanyelbhaa Talaat, H., Kabel, A., Samy, H., & Elbadry, M.
(2009). Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss.
International Journal of Pediatric Otorhinolaryngology, 73,
937–939.
*Shallop, J. (2002). Auditory neuropathy/dys-synchrony in adults
and children. Seminars in Hearing, 23, 215–223.
*Shallop, J., Peterson, A., Facer, G., Fabry, L., & Driscoll, C.
(2001). Cochlear implants in five cases of auditory neuropathy:
Postoperative findings and progress. Laryngoscope, 111,
555–562.
Sininger, Y. S. (2002). Identification of auditory neuropathy in
infants and children. Seminars in Hearing, 23, 193–200.
Starr, A., Picton, T. W., Sininger, Y., Hood, L. J., & Berlin, C. I.
(1996). Auditory neuropathy. Brain, 119, 741–753.
Starr, A., Sininger, Y., Winter, M., Derebery, M. J., Oba,
S., & Michalewski, H. (1998). Transient deafness due to
Roush et al.: ANSD Systematic Review
169
temperature-sensitive auditory neuropathy. Ear and Hearing,
19, 169–179.
Tang, T. P., McPherson, B., Yuen, K. C., Wong, L. L., & Lee,
J. S. (2004). Auditory neuropathy/auditory dys-synchrony in
school children with hearing loss: Frequency of occurrence.
International Journal of Pediatric Otorhinolaryngology, 68,
175–183.
*Teagle, H., Roush, P., Woodard, J. S., Hatch, D. R., Zdanski,
C. J., Buss, E., & Buchman, C. A. (2010). Cochlear implantation in children with auditory neuropathy spectrum disorder.
Ear and Hearing, 31, 325–335.
*Trautwein, P., Sininger, Y., & Nelson, R. (2000). Cochlear
implantation of auditory neuropathy. Journal of the American
Academy of Audiology, 11, 309–315.
U.S. Department of Health and Human Services, U.S.
Food and Drug Administration. (2009). Cochlear
implants. Retrieved from www.fda.gov/ MedicalDevices/
ProductsandMedicalProcedures/ ImplantsandProsthetics/
CochlearImplants/default.htm.
Varga, R., Kelley, P. M., Keats, B. J., Starr, A., Leal, S. M.,
Cohn, E., & Kimberling, W. J. (2003). Non-syndromic recessive auditory neuropathy is the result of mutations in the
otoferlin (OTOF) gene. Journal of Medical Genetics, 40, 45–50.
*Vermeire, K., Brokx, J. P., Van de Heyning, P. H., Cochet, E.,
& Carpentier, H. (2003). Bilateral cochlear implantation in
children. International Journal of Pediatric Otorhinolaryngology,
67, 67–70.
Vlastarakos, P., Mikopoulous, T., Tavoulari, E., Papacharalmous,
G., & Korres, S. (2008). Auditory neuropathy: Endocochlear
lesion or temporal processing impairment? Implications for
diagnosis and management. International Journal of Pediatric
Otorhinolaryngology, 72, 1135–1150.
Walden, B. E. (1984). Validity issues in speech recognition testing.
In E. Elkins (Ed.), Speech recognition by the hearing-impaired
(ASHA Reports No. 14, pp. 16–18). Rockville, MD: American
Speech-Language-Hearing Association.
Wu, J. L., & Yang, H. M. (2003). Speech perception of Mandarin
Chinese speaking young children after cochlear implant use:
effect of age at implantation. International Journal of Pediatric
Otorhinolaryngology, 67, 247–253.
Yasunaga, S., Grati, M., Cohen-Salmon, M., El-Amraoui, A.,
Mustapha, M., Salem, N., . . . Petit, C. (1999). A mutation
in OTOF, encoding otoferlin, a FER-1-like protein, causes
DFNB9, a nonsyndromic form of deafness. Nature Genetics,
21, 363–369.
Yellin, M., Jerger, J., & Fifer, R. (1989). Norms for disproportionate loss in speech intelligibility. Ear and Hearing, 10,
231–234.
Zimmerman-Phillips, S., Osberger, M. J., & Robbins, A. M.
(1997). Infant-Toddler Meaningful Auditory Integration Scale.
Sylmar, CA: Advanced Bionics.
170 American Journal of Audiology • Vol. 20 • 159–170 • December 2011