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 acoustic amplification. All participants demonstrated improved
to the audiologic management of children with auditory auditory performance; however, all 18 studies were considered
neuropathy spectrum disorder ( ANSD). exploratory, and many had methodological limitations.
Method: A systematic search of the literature was conducted Conclusion: The clinical evidence related to intervention for
in 25 electronic databases (e.g., PubMed, CINAHL, and ERIC) ANSD is at a very preliminary stage. Additional research is
using key words such as auditory neuropathy, auditory needed to address the efficacy of acoustic amplification and
neuropathy spectrum disorder, auditory neuropathy/ cochlear implantation in children with ANSD and the impact
dyssynchrony, and hearing loss. Eighteen studies met of this disorder on developmental outcomes.
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. Key Words: auditory neuropathy, auditory neuropathy
Results: Fifteen of the 18 studies addressed the use of spectrum disorder, cochlear implantation, acoustic
cochlear implantation, and 4 addressed conventional amplification, hearing loss

Although it was recognized that “neuropathy” does not apply

A
uditory neuropathy, a condition first described in
the mid-1990s by Starr, Picton, Sininger, Hood, and in all cases, the term has become widely used in reference
Berlin (1996), is a disorder characterized by absent to this population. The etiology of ANSD is multifactorial
or severely abnormal auditory brainstem response (ABR) and includes genetic, congenital, and acquired conditions.
with intact outer hair cell function, as evidenced by the Both syndromic (e.g., Charcot-Marie-Tooth disease and
presence of evoked otoacoustic emissions and /or cochlear Friedreich’s ataxia) and nonsyndromic genetic forms of ANSD
microphonics. Although current terminology implies dys- (DFNB9/OTOF; Varga et al., 2003; Yasunaga et al., 1999)
function of the auditory nerve, possible causes include have been reported. Risk factors reported include prematurity,
damage to the inner hair cells, to the synapse between hyperbilirubinemia, ototoxic drug exposure (Madden, Rutter,
the inner hair cell and auditory nerve, or in the auditory Hilbert, Greinwald, & Choo, 2002; Rance et al., 1999), and
nerve itself (Buchman et al., 2006; Rance, Cone-Wesson, various neurological disorders such as mitochondrial disease
Wunderlich, & Dowell, 2002). (Deltenre, Mansbach, Bozet, Clercx, & Hecox, 1997; Starr
Recently the term auditory neuropathy was expanded et al., 1996).
to auditory neuropathy spectrum disorder (ANSD) to ac- Initially, ANSD in children was thought to be a rare form
knowledge the heterogeneous and multifaceted nature of of sensorineural hearing loss (SNHL). However, a recent
this condition (Guidelines Development Conference, 2008). systematic review by Vlastarakos, Mikopoulous, Tavoulari,
Papacharalmous, and Korres (2008) indicated that ANSD
accounts for approximately 8% of the newly diagnosed cases
a of hearing loss in children each year. Other studies suggest
University of North Carolina, Chapel Hill
b that ANSD occurs in 2.4% to 15% of pediatric cases iden-
American Speech-Language-Hearing Association,
tified with permanent hearing loss (Rance, 2005; Sininger,
Rockville, MD
2002; Tang, McPherson, Yuen, Wong, & Lee, 2004).
Correspondence to Tobi Frymark: [email protected] Among the clinical characteristics reported are speech
Editor: Sheila Pratt perception difficulties that are often disproportionate to
Associate Editor: Ruth Litovsky hearing threshold levels (Starr et al., 1996), and difficulty
Received August 4, 2010 hearing in noise (Kraus et al., 2000; Rance et al., 2007).
Revision received March 7, 2011 Furthermore, degree of hearing loss, based on detection
Accepted July 18, 2011 levels, ranges from normal to profound and in some cases
DOI: 10.1044/1059-0889(2011/10-0032) may be transient or degenerative in nature (Deltenre et al.,

American Journal of Audiology • Vol. 20 • 159–170 • December 2011 • A American Speech-Language-Hearing Association 159

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 1999; Franck, Rainey, Montoya, & Gerdes, 2002; Kraus This report documents the findings of an evidence-based
et al., 2000; Madden et al., 2002; Starr et al., 1998). As systematic review (EBSR) conducted by ASHA’s National
universal newborn hearing screening has expanded and as Center for Evidence-Based Practice in Communication Dis-
audiologists have become more familiar with the disorder orders. The primary aim of this review was to synthesize
and its diagnosis, additional information has emerged regard- and analyze existing evidence pertaining to audiologic treat-
ing treatment for children with ANSD. Still, the heteroge- ment of children, birth to 18 years of age, diagnosed with
neous nature of the disorder, compounded by the broad range ANSD. The EBSR highlights the current state of evidence
of auditory abilities, makes the audiologic management for for children diagnosed with ANSD and characterizes find-
these children challenging and at times uncertain. ings by severity and type of intervention. The report is
Currently, two technologies—acoustic amplification and intended to serve as a resource to clinicians as they weigh
cochlear implantation—are recommended for children with options for and against various treatment approaches for
ANSD. Not surprisingly, there has been considerable de- children with ANSD. The findings reported here, along
bate among clinicians and investigators regarding the choice with other crucial factors such as social/emotional develop-
of and expected benefit from these technologies. Those ment, speech-language skills, and parent preference, will
opposed to conventional amplification emphasize the pre- assist clinicians in determining what type of intervention
sumed limited benefits of hearing aid (HA) use for individuals approach best meets the needs of children with ANSD.
with normal outer hair cell functioning, arguing that acoustic The findings also elucidate the quality and level of existing
amplification offers only louder, more distorted signals evidence in order to highlight areas where further study
(Berlin, 1999). Others have expressed concern regarding the is needed.
potential consequence of acoustic trauma to existing outer Eight clinical questions were established, a priori, to ad-
hair cells (Doyle, Sininger, & Starr, 1998). Concern has also dress the specific population, interventions, and outcomes
been raised regarding cochlear implant (CI) candidacy for of interest (see Table 1). The population reviewed included
children with ANSD based on the reported involvement of children diagnosed with ANSD from birth to 18 years of
the auditory nerve (Miyamoto, Kirk, Renshaw, & Hussain, age. The targeted interventions were (a) conventional acous-
1999). Others have expressed concern regarding cochlear tic amplification including HAs or frequency modulated
implantation because of spontaneous improvement in hearing (FM) systems and (b) cochlear implantation. Several out-
thresholds reported in a subset of children, especially those comes were considered, including the impact of HAs and CIs
with hyperbilirubinemia (Madden et al., 2002). on a child’s functional hearing and communication skills,
Although much has been learned about ANSD since its speech and language skills, academic performance, and social
original description by Starr and colleagues (1996), many skills. Auditory outcomes included, but were not limited to,
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 Table 1. Clinical questions included in the evidence-based
Infant Hearing (JCIH, 2007) recommended that children systematic review.
with auditory neuropathy-type hearing loss undergo a trial
with amplification and that decisions regarding continued Question
HA use be guided by the benefits derived from amplification. number Clinical question
In an effort to provide further guidance to clinicians working Amplification
with this population, consensus guidelines were recently 1 For children with the diagnosis of auditory neuropathy,
developed by an expert panel at an international conference what is the effect of acoustic amplification on
(Guidelines Development Conference, 2008). Although the auditory outcomes?
2 For children with the diagnosis of auditory neuropathy,
guidelines generated by the panel were not based on a sys- what is the effect of acoustic amplification on speech
tematic review of the evidence, panel members examined and language outcomes?
the literature in their areas of expertise and prepared sum- 3 For children with the diagnosis of an auditory neuropathy,
mary statements as a resource for clinicians involved with what is the effect of acoustic amplification on
the identification and management of infants presenting academic outcomes?
4 For children with the diagnosis of an auditory neuropathy,
with ANSD. The panel noted that many of the recommen- what is the effect of acoustic amplification on social
dations made for assessment and management of ANSD emotional/parent outcomes?
were consistent with those recommended by the JCIH Cochlear implantation
(2007); however, additional considerations for acoustic 5 For children with the diagnosis of an auditory neuropathy,
amplification, cochlear implantation, and habilitation were what is the effect of cochlear implantation on auditory
outlined. The panel also emphasized the need for further outcomes?
research on all aspects of the disorder, including a more 6 For children with the diagnosis of auditory neuropathy,
what is the effect of cochlear implantation on
in-depth examination of the scientific literature. In light of speech and language outcomes?
the diverse nature of ANSD, the unresolved controversies 7 For children with the diagnosis of an auditory neuropathy,
surrounding intervention recommendations, and the high what is the effect of cochlear implantation on
degree of interest among audiologists (American Speech- academic outcomes?
8 For children with the diagnosis of an auditory neuropathy,
Language-Hearing Association [ASHA], 2008), a systematic what is the effect of cochlear implantation on social
review was undertaken to examine the current state of the emotional/parent outcomes?
evidence.

160 American Journal of Audiology • Vol. 20 • 159–170 • December 2011

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 unaided and aided pure-tone and speech detection thresholds, completeness prior to final inclusion/exclusion. Any disagree-
unaided and aided speech perception measures, hearing in ment among authors regarding study eligibility was resolved
noise, dichotic listening, and temporal processing (e.g., gap by consensus and documented as such. Additionally, the
detection). Examples of speech and language outcomes second and third authors, blinded to each other’s results,
included receptive and expressive language abilities and evaluated studies for methodological rigor based on ASHA’s
speech intelligibility. With increased access to speech, lan- levels of evidence scheme (Frymark et al., 2009; Mullen,
guage, and other environmental sounds and signals, it was 2007). Studies were evaluated for methodological quality
considered important to examine the impact of these inter- based on the following indicators: (a) adequate description
ventions on academic performance. Academic outcomes of protocol for sufficient study replication, (b) blinding
included, but were not limited to, reading, written language, of assessors, (c) random sampling/allocation procedures,
spelling, and educational achievement. Finally, as with any (d) evidence of treatment fidelity, (e) reporting significance
intervention, it was considered important to examine the ( p values) of findings or providing adequate data to allow
effects of conventional acoustic amplification or cochlear for statistical calculations, (f ) reporting of effect size and
implantation on social and emotional well-being. Social / confidence intervals or providing sufficient data to calculate
emotional outcomes targeted measures of the child’s com- precision of treatment effects, and (g) use of intention-to-
munication attitude or behavior (e.g., communication attitude treat analyses. In addition to evaluating methodological
test, severity rating scales, self-report rating, quality of life quality, pertinent demographic, intervention, and outcomes
indicators, and social and communication measures). Parent data were extracted from each study.
outcomes focused on any measure of parent attitude on com-
munication (e.g., parent rating scales and parent surveys)
or a change in parental communication (e.g., pre/post parent Results
communication patterns).
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%,
Method 19/37) were further excluded upon review of the full text.
A review of the literature was conducted from October In total, 184 studies were eliminated from the review for the
2009 to July 2010 using search parameters and inclusion/ following reasons: (a) did not examine a target intervention
exclusion criteria set a priori. Approximately 25 databases (40%, 73/184); (b) was not the population or age under
were searched (e.g., CINAHL [Cumulative Index to Nursing review (22%, 40/184); (c) was not a study or systematic
and Allied Health Literature], ERIC [Education Resources review of the literature (12%, 22/184); (d) did not provide
Information Center], and PubMed) using key words pertain- original data or adequate data for analyses (12%, 22/184);
ing to auditory neuropathy, ANSD, auditory dyssynchrony, (e) was not a peer-reviewed study (9%, 17/184); or (f ) did not
and hearing loss. A full list of databases and key words is directly address a clinical question (5%, 10/184). Eighteen
provided in the supplementary material (Appendix A) af- studies were included in the final analysis (see Appendix B
filiated with this article. Eligibility criteria for inclusion of in the supplementary material for a full list of excluded stud-
studies were as follows: treatment studies published in peer- ies and reasons for exclusion). Interrater reliability of study
reviewed journals from 1990 to July 2010; publications eligibility agreement between coders was good, K = .787
in English; and studies including children ages ≤18 years (Cohen, 1960; Landis & Koch, 1977).
diagnosed with ANSD receiving acoustic amplification
(HA or FM system) or cochlear implantation as a primary
audiologic intervention. Exclusionary criteria were stud- Participant Characteristics
ies based on animal models, pharmacological models, or Table 2 details the 115 participants across 18 studies;
studies with mixed ages, mixed populations, or mixed treat- 60 were male, 39 were female, and gender was not reported
ments (e.g., combined HA and CI) unless data allowed for for 16. The total number of participants accounts for over-
separate analyses. Studies in which pretreatment and post- lapping subjects reported in two Shallop studies (Shallop,
treatment measures or raw data were not reported were also 2002; Shallop, Peterson, Facer, Fabry, & Driscoll, 2001)
excluded.1 It is important to note that studies were not and two of three cohorts reported in the Teagle et al. (2010)
excluded based on study design. Although limiting stud- study. Age of ANSD diagnosis was early (≤4 years) for
ies to group designs or randomized clinical trials would 66 participants. One study (Santarelli, Scimemi, Dal Monte,
reduce the potential for bias, the inclusion of all studies pro- Genovese, & Arslan, 2006) presented a single case report of a
vides a more comprehensive look at the current body of female diagnosed with ANSD at age 14. For the remaining
evidence. 48 participants, age at diagnosis was not reported. A number
Based on the aforementioned criteria, the second and of studies (n = 14) provided medical comorbidities or risk
third authors independently reviewed all citations for rele- factors associated with the disorder (e.g., hyperbilirubinemia,
vance and completed a hand search of all references. The Friedreich’s ataxia, or antibiotic treatments). The majority
first author reviewed the full bibliography of studies for of the participants (75%, 86/115) exhibited hearing loss rang-
ing from severe to profound, while 18% (21/115) exhibited
1
When applicable, correspondence with primary author was attempted to moderate to severe or moderate hearing loss. Four partici-
obtain missing data. pants (3%) exhibited mild hearing loss. Hearing severity was

Roush et al.: ANSD Systematic Review 161

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 Figure 1. Literature search selection and identification process for inclusion in the evidence-
based systematic review (EBSR).

not reported for four participants. Severity of hearing loss was 70 dB HL at 0.5, 1, and 2 kHz prior to amplification.
was primarily based on author report. 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
Acoustic Amplification noted improved aided detection thresholds of approximately
Table 3 outlines the intervention characteristics and major 30 dB for individual frequencies (see Table 3). The two addi-
findings of the 28 participants contributing data to Clinical tional case studies (Deltenre et al., 1999; Lin et al., 2005)
Question 1 (see Table 1); the majority of these (n = 26) were revealed similar findings. Deltenre et al. (1999) investigated
in two case series reports (Rance et al., 1999, 2002). Sever- the unaided and aided performance of a female with auditory
ity of hearing impairments ranged from mild to profound neuropathy-type hearing loss with pure-tone thresholds in
(mild = 14%, moderate = 29%, moderate to severe = 14%, the moderate range diagnosed at 2 months of age, while
severe = 7%, and profound = 36%), and age of ANSD diag- Lin and colleagues (2005) examined a male diagnosed with
nosis ranged from 2 to 36 months. The authors from three auditory neuropathy-type hearing loss with pure-tone thresh-
studies (Deltenre et al., 1999; Lin, Chen, & Wu, 2005; Rance olds in the profound range at age 3. Both authors reported
et al., 1999) reported that participants were fitted with bin- improvement in aided PTAs of approximately 20 dB and
aural amplification. Participants from the two Rance et al. 60 dB, respectively.
studies (1999, 2002) were fitted using the National Acoustics The four included studies (Deltenre et al., 1999; Lin et al.,
Laboratory prescriptions for gain, frequency response, and 2005; Rance et al., 1999, 2002) also assessed aided speech
output limiting levels for HA fitting (Byrne & Dillon, 1986). perception outcomes of participants with ANSD. Of the
The authors also reported that verification of HA settings 24 of 28 participants with probability data reported, half
was completed. Limited information was provided regarding (50%, 12/24) demonstrated no significant difference in aided
prescriptive fitting method or verification strategy for the speech perception abilities. The single case report by Lin
Deltenre et al. (1999) and Lin et al. (2005) studies. Of the four et al. (2005) reported no significant difference ( p > .01)
included studies, three (Deltenre et al., 1999; Lin et al., 2005; on the Mandarin Auditory Perception Test Battery (Wu &
Rance et al., 2002) provided pre- and posttreatment data to Yang, 2003) during a 2-year treatment period; however, it
investigate the effects of acoustic amplification on pure-tone should be noted that the participant in this study had unaided
thresholds. All four studies examined the effects of amplifi- pure-tone thresholds of 111 dB HL and >112 dB HL (four-
cation on speech perception. No studies addressed speech, frequency PTA). Rance and colleagues (2002) reported
language, or academic outcomes (Clinical Questions 2, 3, mixed speech perception scores for children with ANSD
and 4; see Table 1). who used amplification for a minimum of 12 months prior to
Unaided and aided pure-tone thresholds were provided participating in the study. Seven of 15 participants tested
for 20 participants with ANSD: 18 from the Rance et al. exhibited no significant difference in Phonetically Balanced
(2002) case series study and two from the Deltenre et al. Kindergarten test (PBK; Haskins, 1949) phoneme scores
(1999) and Lin et al. (2005) case study reports. Children in the aided condition. The remaining eight showed a mean
from Rance et al. (2002) were identified with ANSD at a difference in aided versus unaided PBK phonemes of 57%
mean age of 6 months, with pure-tone thresholds ranging with a mean aided PBK phoneme score of 67%. Interest-
from mild to profound. Mean pure-tone average (PTA) ingly, cortical evoked event-related potentials were present

162 American Journal of Audiology • Vol. 20 • 159–170 • December 2011

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 Table 2. Subject variables—all included studies.

Audiologic findings
Gender Age at Risk factors/ Previous
Citation N (M / F ) diagnosis medical comorbidities ABR OAE CM HL severity intervention

Buss et al. (2002) 4 NR Birth P3 = Mondini’s malformation – – + Severe-profound HA

P1, P2, P4 = NR
Deltenre et al. (1999) 1 0/1 2 months Hyperbilirubinemia, – – + Moderate NR
respiratory distress
Jeong et al. (2007) 9 5/4 M = 20 months 2 hyperbilirubinemia – 3– + 6 severe-profound, HA
6+ 3 CNT
Lin et al. (2005) 1 1/0 36 months NR – + NR Profound HA
Madden et al. (2002) 2 1/1 NR None – + NR Profound HA
FM
Mason et al. (2003) 1 0/1 NR None – + + Moderate NR
Miyamoto et al. (1999) 1 1/0 48 months Friedreich’s ataxia – + NR Profound HA
FM
Rance & Barker (2008) 10 7/3 ≤3 months 6 jaundice, 4 hypoxia – + + Severe-profound HA
Rance et al. (1999) 8 NR ≤9 months 4 jaundice, 3 hypoxia, – NR + 2 mild, 2 moderate, None
1 low birth weight, 1 none 4 profound
Rance et al. (2002) 18 14/4 M = 6 months 7 jaundice, 6 hypoxia, – 7– + 2 mild, 5 moderate, NR
1 hydrocephalus, 11+ 4 moderate-severe,
1 meningitis, 2 none 2 severe, 5 profound
Raveh et al. (2007) 4 NR NR NR – + + Severe-profound HA
Rouillon et al. (2006) 2 0/2 P1 = 22 months Genetic NR NR NR Profound HA
P2 = 10 months
Santarelli et al. (2006) 1 0/1 14 years Systemic sclerosis – + + Severe None
Shallop (2002)a 10 6/4 M = 14 months Congenital deafness – + + Severe-profound NR
Shallop et al. (2001)a 5 3/2 38 months NR – + + Severe-profound HA
Teagle et al. (2010) Group B = 15 9/6 NR 9 prematurity, – NR + 2 moderate, HA
7 bronchopulmonary dysplasia, 1 moderate-severe,
3 hyperbilirubinemia, 3 severe,
4 retinopathy 2 severe-profound,
Roush et al.: ANSD Systematic Review

6 profound, 1 CNT
Group C = 26 15/11 NR 10 prematurity, – NR + 5 moderate, 5 severe, HA
2 bronchopulmonary dysplasia, 16 profound
1 hyperbilirubinemia,
1 Turner’s syndrome,
1 sensorimotor neuropathy,
11 none
Trautwein et al. (2000) 1 1/0 35 months NR – – + Severe-profound HA
Vermeire et al. (2003) 1 0/1 Birth Hyperbilirubinemia NR NR NR Profound 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

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 Table 3. Studies examining acoustic amplification.

Age at Fitting Duration/

Citation N intervention method/model compliance Outcomes measured Unaided Aided
a
Deltenre et al. 1 48 months NR/binaural 36 months PTA 50 dB 29 dB
(1999) Speech identification at 55 dB
Phonemes 0% 96%
Words 0% 80%
a
Lin et al. (2005) 1 NR NR/binaural 30 months PTA (R/L) 111 dB/>112 dB 50 dB/51 dB
MAPTB 46% 51%
Rance et al. 8 NR BTE/binaural ≤12 months PBK words
(1999) P13 8% 43%
P16 32% 88%
Picture Vocabulary Test b
P8 3/12 3/12
P10 1/12 1/12
P12 2/12 8/12
P14 2/24 4/24
P15 1/12 8/12
P17 3/12 2/12
Rance et al. 18 8 months BTE/ NR ≤12 months PTA c
70 dB NR
(2002) 5 kHz NR 42 dB
1 kHz NR 42 dB
2 kHz NR 44 dB
4 kHz NR 44 dB
PBK phonemes (n = 15) 8% 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.
b
Subtest of the PLOTT Test (Plant & Westcott, 1983).
c
PTA threshold at 0.5, 1, and 2 kHz.

in the group of children with significant open-set speech provide information regarding age at intervention. The
abilities but were absent in those who had no open-set abil- majority of studies (80%, 12/15) reported unsuccessful trials
ities. In comparison, a matched control group of children of acoustic amplification (HA and /or FM systems) prior to
with non-auditory neuropathy SNHL from the same study CI intervention. Of the remaining three studies, two (Mason,
showed a mean difference score of 49%, with a mean aided De Michele, Stevens, Ruth, & Hashiasaki, 2003; Shallop,
PBK phoneme score of 66%. Similar findings were reported 2002) did not provide previous intervention history, while
by Rance et al. (1999), with significant differences found one (Santarelli et al., 2006) indicated no HA trial prior to
between unaided and aided speech recognition ( p < .01) in CI intervention. Contrary to the heterogeneity among chil-
four of the eight participants tested. Although probability dren receiving hearing amplification interventions, 85% of
data were not reported by Deltenre et al. (1999), the authors the children who received cochlear implantation exhibited
also reported gains of ≥80% in aided speech recognition severe to profound hearing loss (n = 75).
for speech presented at 55 dB HL. All 15 studies contributed data to Clinical Question 5
While outcome data showing the effects of acoustic ampli- (see Table 1). Six studies reported changes in pure-tone thresh-
fication for children with ANSD are limited, the results of olds (Buss et al., 2002; Jeong, Kim, Kim, Bae, & Kim, 2007;
this review suggest that some children with ANSD benefit Mason et al., 2003; Miyamoto et al., 1999; Trautwein,
from improved aided detection levels with use of HAs. There Sininger, & Nelson, 2000; Vermeire, Brokx, Van de Heyning,
appears to be a subset of children who experience benefits Cochet, & Carpentier, 2003), and 12 reported speech per-
in aided speech perception abilities in addition to improved ception outcomes (Jeong et al., 2007; Lin et al., 2005; Madden
pure-tone detection thresholds. 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;
Cochlear Implantation Trautwein et al., 2000). Raveh et al. (2007) also reported speech
Fifteen studies (see Table 4) examined the effects of CI and language outcomes (Clinical Question 6). No studies
interventions for 88 children who had been diagnosed with were found that examined social/emotional or academic out-
ANSD from birth to 14 years of age. Eighty-two participants comes after cochlear implantation (Clinical Questions 7 and 8).
received CI intervention at ≤5 years of age (range = 21– Similar to the findings pertaining to HA interventions,
68 months), and two participants (one each from Miyamoto all participants with ANSD exhibited improved pure-tone
et al., 1999, and Santarelli et al., 2006) received a CI at detection thresholds after CI. Six studies reported improve-
ages 10.9 and 18 years, respectively. Two studies (Lin et al., ment in PTA thresholds ranging from 40 to 87 dB, five
2005; Raveh, Buller, Badrana, & Attias, 2007) did not 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

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 Table 4. Studies examining cochlear implantation (CI).

Duration/
Citation N Age at intervention Model compliance Outcomes measured Pre-CI Post-CI
a
Buss et al. 4 P1 = 25 months Clarion 12 months P1 PTA 101 dB 36 dB
(2002) P2 = 28 months P2 PTAa 101 dB 31 dB
P3 = 68 months P3 PTAa 104 dB 36 dB
P4 = 62 months P4 PTAa 90 dB 26 dB
Jeong et al. 9 M = 4 years Nucleus M = 24 months PTAb (n = 6) 83 dB 29 dB
(2007) CAP 0 5
MW test 5% 59%
Common phrase test 3% 46%
Lin et al. 1 NR Clarion NR MAPTB
(2005) Spondee 35% 90%
Vowel 25% 90%
Tone 70% 100%
Phrase 70% 100%
Sentence 50% 100%
Madden et al. 2 P1 = 39 months Clarion 7 months Closed-set 64% 88%
(2002) discrimination
P2 = 21 months Clarion 24 months IT-MAIS 0/40 27/40
Mason et al. 1 32 months NR 5 months PTAa 70–80 dB 35–40 dB
(2003)
Miyamoto et al. 1 10.9 years Nucleus 12 months PTAc 97 dB 39 dB
(1999) PBK 4% 4%
Words 4% 4%
Phonemes 12% 20%
Rance & Barker 10 M = 33 months Nucleus >24 months MW test <40% >55%
(2008) M = 59.6%
Raveh et al. 4 NR NR NR IT-MAIS 18% 56%
(2007) Ling Test .75 5
CAP 1 3.5
MUSS 3% 29%
Rouillon et al. 2 P1 = 35 months Nucleus 1 months MAIS 4/40 40/40
(2006) P2 = 48 months Nucleus 18 months MAIS 4/40 31/40
Santarelli et al. 1 18 years Nucleus NR TIPI
(2006) Vowel 52% 95%
identification
Consonant 25% 55%
identification
d
Shallop (2002) 10 M = 45 months 9 nucleus NR MAIS 8% 91%
1 clarion
Shallop et al. 5 M = 53 months Nucleus M = 18 months ESP 1 4
(2001)d
Teagle et al. 15 Group B 10 nucleus M = 20 months IT-MAIS 26% (21%) 79% (16%)
(2010) M = 30 months 4 HiRes90
1 sonota
26 Group C 5 Nucleus M = 50 months PPK
M = 63 months 8 Nucleus- Words 14% 54% (34%)
Freedom Phonemes 49% 76% (22%)
8 HiRes90
3 Clarion
2 Med El
Trautwein et al. 1 39 months Nucleus 12 months Ling Test 22/30 27/30
(2000) ESP 1 4
PTAb (18 months) 104 dB 40 dB
Vermeire et al. 1 R = 29 months Nucleus 9 months after PTAb 110 dB 23 dB
(2003) L = 52 months 2nd CI

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.
b
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.

Roush et al.: ANSD Systematic Review 165

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 et al., 2002; Jeong et al., 2007; Mason et al., 2003; Trautwein The findings detailed in Table 4 suggest that children
et al., 2000; Vermeire et al., 2003), and one (Miyamoto et al., with ANSD with thresholds in the severe to profound range
1999) that assessed PTA thresholds at 0.5, 1, and 2 kHz. may benefit from CI interventions. Interestingly, Rance
Improvements were also noted across studies examining and Barker (2008) found no significant difference in per-
speech perception outcomes. However, it is important to formance on CNC phoneme scores between children with
note that studies varied greatly in type of speech perception ANSD who received CI intervention compared to a group
measure reported, making it difficult to interpret the results who received HA intervention, d = 0.20, 95% CI [–0.69,
across studies. 1.07]. They did note that children in their cohort who were
Two studies (Rance & Barker, 2008; Shallop et al., 2001) among the poorer performers with HAs had already been
reported significant differences in speech perception ability referred for CI. Furthermore, the authors went on to report
after cochlear implantation. Rance and Barker (2008) exam- that 10 age-matched CI users with SNHL performed sig-
ined the speech perception skills of 10 children with a mean nificantly better than both the CI and HA users with ANSD
age at CI of 33 months, nine of whom were diagnosed with ( p = .0006).
ANSD at ≤3 months of age. The authors reported significant Finally as mentioned above, only one study (Raveh et al.,
improvement in consonant-nucleus-consonant (CNC) pho- 2007) examined the effect of CI intervention on speech and
neme scores ( p = .006) after CI. Additionally, 10 participants in language outcomes of children with ANSD. Of the 18 par-
the Shallop (2002) study who completed pre- and posttesting ticipants with severe to profound loss initially included
demonstrated significant improvement on the Meaningful in the study, only four had sufficient follow-up testing
Auditory Integration Scale (MAIS; Robbins, 1998; p < .0001). post-CI using the Meaningful Use of Speech Scale (Robbins
These participants were diagnosed with ANSD at 14 months & Osberger, 1991) allowing for inclusion in the review.
of age, receiving CI at a mean age of 45 months. Although Mean performance was 3% preimplantation and 29% post-
pre-CI data were not provided, Shallop et al. (2001) also implantation. Although sufficient pre- and postdata were
reported significant improvements in speech awareness thresh- not provided by Buss and colleagues (2002) for inclusion
olds and/or speech reception thresholds of five participants in Clinical Question 6 (see Table 1), the authors noted that
with auditory neuropathy-type hearing loss. In this study, mean participants with ANSD using CI performed comparably
age of implantation was 53 months, and mean diagnosis was to age-matched CI users with SNHL on a speech production
38 months of age. Mean duration of CI use was reportedly test (Paden–Brown Test; Paden & Brown, 1992). The authors
18 months. Additionally, the authors reported that participants stated that three of the four participants performed “within
were unable to discriminate speech (Early Speech Perception or slightly above the confidence interval one standard devi-
Test [ESP] Category 1; Moog & Geers, 1990) prior to CI ation around the control group mean” (Buss et al., 2002,
intervention and demonstrated consistent ability to discrimi- pp. 330–331).
nate words (ESP Category 4) postimplantation.
Lastly, the recent longitudinal study by Teagle et al. (2010) Methodological Quality
followed the largest cohort of participants receiving CI inter-
ventions to date (n = 52). In this study, participants were The methodological quality of the included studies is
identified with ANSD through newborn hearing screen- outlined in Table 5. Level of interrater agreement between
ing protocols. The mean preoperative PTA threshold was authors for appraisal of study quality was good (K = .742;
88 dB HL, and the mean duration of HA use prior to CI Landis & Koch, 1977). All studies were case series reports
was reportedly 26 months. Of the 52 participants studied, or single case studies. While the majority of studies (67%,
11 (Group A) did not have sufficient pre- and postdata for 12/18) provided an adequate description of the treatment
analyses; however, the remaining 41 were included and protocol, all 18 studies recruited participants using a method
demonstrated improved speech perception abilities. One of convenience sampling and did not report blinding of as-
cohort (Group B) consisted of 15 participants with CI use for sessors. Approximately one third of the studies (39%, 7/18)
>6 months who were unable to participate in open-set speech reported effect size or probability data. Two quality indicators
perception testing due to young age or development delays; (evidence of treatment fidelity and use of intention-to-treat
however, 13 of 15 participants in this group provided pre- analysis) were not applicable to the types of studies included
and postdata on the Infant-Toddler Meaningful Auditory Inte- in this review and therefore not considered.
gration Scale (Zimmerman-Phillips, Osberger, & Robbins,
1997) with a mean improvement of 53% postimplantation.
The other cohort (Group C) had 26 CI users who could per- Discussion
form open-set measures. Although pre-CI data were not The purpose of this EBSR was to investigate the impact
reported for all 26 participants who completed PBK test- of two hearing technologies, acoustic amplification and co-
ing, the authors reported a mean improvement of 40% and chlear implantation, on outcomes in children with ANSD.
27%, respectively, for PBK words and phonemes, with A systematic review of the literature from 1990 to 2010
mean PBK phoneme and word scores postimplantation of yielded 18 studies that met the predetermined inclusion
76% (SD = 22%, range = 24%–100%) and 54% (SD = 34%, criteria. Only four studies contributed data to address the
range = 4%–100%), respectively.2 impact of acoustic amplification on auditory outcomes,
while the majority of studies (83%, 15/18) employed co-
2
The first author was a coauthor on Teagle et al. (2010) and provided the chlear implantation. Only one study (Raveh et al., 2007) pro-
PBK data for Cohort C. vided data on speech and language outcomes after cochlear

166 American Journal of Audiology • Vol. 20 • 159–170 • December 2011

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 Table 5. Methodological quality of included studies.

Adequate
Study description Assessors Sampling/ Significance Precision Clinical
Citation design of protocol blinded allocation ( p values) (ES/CI) question(s)

Buss et al. (2002) Case study + – Convenience – – 5

Deltenre et al. (1999) Case study – – Convenience – – 1
Jeong et al. (2007) Case series + – Convenience – – 5
Lin et al. (2005) Case study + – Convenience + – 1, 5
Madden et al. (2002) Case study + – Convenience – – 5
Mason et al. (2003) Case study – – Convenience – – 5
Miyamoto et al. (1999) Case study + – Convenience + – 5
Rance & Barker (2008) Case series + – Convenience + + 5
Rance et al. (1999) Case series – – Convenience + – 1
Rance et al. (2002) Case series – – Convenience + – 1
Raveh et al. (2007) Case series – – Convenience – – 5, 6
Rouillon et al. (2006) Case study – – Convenience – – 5
Santarelli et al. (2006) Case study + – Convenience – – 5
Shallop (2002) Case series + – Convenience – – 5
Shallop et al. (2001) Case series + – Convenience + – 5
Teagle et al. (2010) Case series + – Convenience + + 5
Trautwein et al. (2000) Case study + – Convenience – – 5
Vermeire et al. (2003) Case study + – Convenience – – 5

Note. ES = effect size; CI = confidence interval.

implantation. No studies reported social, academic, or parent in children with cochlear hearing loss, a broad range of post-
outcomes for either technology. implantation speech perception abilities was observed. For
For both the CI and the HA groups, the majority of example, in the Teagle et al. (2010) study, of the 41 of
participants studied (75%, 86/115) had pure-tone thresholds 52 implanted children who had speech perception measures,
in the severe or profound range. Yet, Sanyelbhaa Talaat, only 26 were able to perform open-set word recognition test-
Kabel, Samy, and Elbadry (2009) found that only 13% of ing despite >2 years of device use. Twenty-seven percent of
children diagnosed with ANSD exhibited severe to profound those able to perform open-set testing achieved a score of
hearing loss. The majority of participants (93%, 26/28) <30% on PBK word testing, which is below the current level
studied with HAs were from two studies by Rance et al. of performance established by CI manufacturers in cooper-
(1999, 2002). Two case studies compose the remainder ation with the U.S. Food and Drug Administration as criteria
of reports on outcomes with amplification (Deltenre et al., level for determining implant candidacy (U.S. Department
1999; Lin et al., 2005). Although not directly addressed of Health and Human Services, 2009).
in this review, Rance et al. (2002) found no correlation The findings from this review do not resolve the con-
between pure-tone thresholds and performance on speech troversies surrounding the audiologic treatment of ANSD
perception tasks for participants with ANSD. This suggests in children. The review indicates that some children with
that degree of pure-tone hearing loss may not be a useful ANSD benefit from acoustic amplification, while others
predictor of aided speech perception abilities. That is, some benefit from cochlear implantation. Furthermore, the meth-
children with greater pure-tone hearing loss may experience odological limitations of the studies, the heterogeneity of
improved speech perception with amplification, while others the participants, and the varied outcomes reported provide
with milder degrees of pure-tone loss may not. This finding insufficient clinical evidence to guide the practicing clinician.
is in contrast to studies of children and adults with cochlear Especially lacking are HA performance data for children
hearing loss who have been shown to demonstrate poorer with ANSD whose pure-tone thresholds are in the mild-
speech perception ability with increasing degree of pure-tone to-moderate range. Of the four studies that evaluated HA
hearing loss (Bamford, Wilson, Atkinson, & Bench, 1981; outcomes, only 12 participants had pure-tone thresholds in
Boothroyd, 1997; Walden, 1984; Yellin, Jerger, & Fifer, 1989). this range. These findings do, however, provide a foundation
In this EBSR, most of the children with ANSD who for further study.
received CIs had at least a severe hearing loss (85%, 75/88); Most of the studies reviewed in this report focus on
only nine with mild or moderate degrees of pure-tone hearing auditory outcomes obtained from measures of audibility
loss were implanted. The majority of CI studies were with for speech, and the ability to perceive and recognize sounds
children who had reportedly experienced a failed attempt with or words. Further research is needed to address other func-
acoustic amplification; however, information was often limited tional aspects including speech, language, learning, social/
regarding type of amplification, prescriptive fitting method, emotional development, and psycho-educational perfor-
verification strategy employed, or tests used to evaluate aided mance. Moreover, considering the heterogeneity of this
benefit. Although most studies reported improved auditory population and the varied etiologies associated with ANSD
outcomes for both pure-tone detection thresholds and speech (e.g., prenatal, genetic, and neurological), there is a need
perception scores following cochlear implantation, as is typical for additional studies with more homogeneous grouping

Roush et al.: ANSD Systematic Review 167

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 of children to assess the impact of various comorbidities with interpretation of the data and evaluation of the
on auditory, speech, language, and learning outcomes. criteria for unsuccessful HA use.
It is important to note that the reports we have cited, and • There is a need for studies that go beyond auditory
indeed many of those not included in this review, have made performance to include a full range of developmental
important contributions to the existing body of knowledge outcomes.
regarding ANSD. Considering the relatively recent discovery
of this clinical population and its low prevalence, it is not Although our understanding of ANSD and its manage-
surprising that most of the studies investigating ANSD in ment is at an early stage, we hope this EBSR will be useful to
children represent exploratory research from case series or case clinicians and researchers. Until further scientific evidence
study reports. To determine the efficacy of acoustic amplifica- is available to direct the clinical decision-making process,
tion and cochlear implantation with this population, well- audiologists should be guided by the peer-reviewed literature
controlled, prospective, longitudinal studies are needed on larger and by the recommendations of national and international
groups of children with detailed descriptions of participants. professional organizations, in combination with clinical
It is important to remember that predicting benefit from experience, empirical observation, and family preference.
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 neuropathy- Acknowledgments
type hearing loss of varying degrees. Several factors appear
to affect outcomes: age at identification and device fitting, This work was supported by the ASHA National Center for
quality of the fitting, consistency of use, type of intervention, Evidence-Based Practice in Communication Disorders. We thank
the authors of the articles studied for their contributions to the
and degree of parental involvement. Furthermore, there are
research on ANSD.
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 References
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