ORIGINAL ARTICLE

Effects of Central Nervous System Residua

on Cochlear Implant Results in Children
Deafened by Meningitis
Howard W. Francis, MD; Margaret B. Pulsifer, PhD; Jill Chinnici, MA, CCC-A; Robert Nutt;
Holly S. Venick, MS, CCC-A; Jennifer D. Yeagle, MS, CCC-A; John K. Niparko, MD

Background: This study explored factors associated with
speech recognition outcomes in postmeningitic deaf-
ness (PMD). The results of cochlear implantation may
vary in children with PMD because of sequelae that ex-
tend beyond the auditory periphery.
Objective: To determine which factors might be most
determinative of outcome of cochlear implantation in chil-
dren with PMD.
Design: Retrospective chart review.
Setting: A referral center for pediatric cochlear implan-
tation and rehabilitation.
Subjects: Thirty children with cochlear implants who
were deafened by meningitis were matched with sub-
jects who were deafened by other causes based on the
age at diagnosis, age at cochlear implantation, age at which
hearing aids were first used, and method of communi-
cation used at home or in the classroom.
Main Outcome Measure: Speech perception perfor-
mance within the first 2 years after cochlear implanta-
tion and its relationship with presurgical cognitive mea-
sures and medical history.
Results: There was no difference in the overall cognitive
or postoperative speech perception performance be-
tween the children with PMD and those deafened by other
causes. The presence of postmeningitic hydrocephalus,
however, posed greater challenges to the rehabilitation pro-
cess, as indicated by significantly smaller gains in speech
perception and a predilection for behavioral problems. By
comparison, cochlear scarring and incomplete electrode
insertion had no impact on speech perception results.
Conclusions: Although the results demonstrated no sig-
nificant delay in cognitive or speech perception perfor-
mance in the PMD group, central nervous system re-
sidua, when present, can impede the acquisition of speech
perception with a cochlear implant. Central effects as-
sociated with PMD may thus impact language learning
potential; cognitive and behavioral therapy should be con-
sidered in rehabilitative planning and in establishing ex-
pectations of outcome.
Arch Otolaryngol Head Neck Surg. 2004;130:604-611

From the Department of
Otolaryngology–Head and
Neck Surgery, The Johns
Hopkins University, Baltimore,
Md (Drs Francis, Pulsifer, and
Niparko; Mss Chinnici, Venick,
and Yeagle; and Mr Nutt); and
the Department of Psychiatry,
Massachusetts General
Hospital, Harvard Medical
School, Boston (Dr Pulsifer).
Mr Nutt is now with Dartmouth
Medical School, Lebanon, NH.
The authors have no relevant
financial interest in this article.
H
EARING IMPAIRMENT IS
one of the most frequent
complications of menin-
gitis and is reported to
occur in 6% to 16% of
cases.1-6 Postmeningitic deafness (PMD)
threatens the normal development of
verbal communication and carries impli-
cations for literacy and educational
achievement. Even in the absence of hear-
ing impairment, delayed language devel-
opment, and neurologic deficits, subse-
quent educational difficulties are
recognized complications of childhood
meningitis.2,7
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at www.archoto.com
Cochlear implantation in children
who fail to reach communication mile-
stones because of the severity of their sen-
sorineural hearing loss has generally been
demonstrated as an efficacious interven-
tion for childhood hearing impairment,
yielding verbal language growth curves
that approach those of hearing children.8
The resulting impact on quality of life9 and
educational placement10 suggests that this
is a cost-effective intervention with du-
rable generic benefits. The task of com-
munication rehabilitation in children deaf-
ened by meningitis, however, may be
complicated by language and cognitive
deficits that result from liquefactive and
inflammatory brain insults.
Neurologic complications of bacte-
rial meningitis result predominantly from
immune response to the invading patho-
gen, with less injury resulting from di-
rect effects of the offending organism.11 Cy-
totoxic injury of neurons that induces
cerebral edema and hydrocephalus can
provoke further increased intracranial

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 Table 1. Comparison of Cochlear Implantation (CI) Results in Children Deafened by Meningitis
and a Matched Group of Children Deafened by Congenital Causes*
Meningitis Group
Communication method, No.
Oral
Total communication
Sign
Age at diagnosis, y
Age at amplification, y
Age at CI, y
Interval between diagnosis and CI, y
No. with open-set speech discrimination
Preoperatively
Postoperatively
Interval to postoperative speech discrimination testing, mo
*Data are mean (SD) unless otherwise indicated.
†P ⬎.05 for all.
pressure and secondary cerebral ischemia with further
neuronal injury.
Central neuropathologic correlates of acute bacte-
rial meningitis imply that expectations of benefit from a
cochlear implant may differ in children with PMD com-
pared with children with an isolated auditory disorder.
Although cochlear implantation has yielded levels of
speech perception in children with PMD that vary com-
pared with those of children deafened by other
causes,12-15 strict comparisons of the effect of pathogen-
esis on outcome are difficult to make owing to a number
of intervening variables and confounders. There has
been no systematic examination of how clinical features
of the acute illness and its neurologic sequelae affect per-
formance.
Deafness and cognitive delay are complications of
meningitis that correlate with the severity of illness.6,16
Illness severity is linked to delayed diagnosis and treat-
ment5 and is marked by the presence of central nervous
system (CNS) deficits and low levels of cerebrospinal fluid
(CSF) glucose.4,5,17 The severity of auditory pathway in-
jury and neurocognitive deficits within the PMD child-
hood population has implications for language out-
come. The identification of clinical predictors of cochlear
implant outcome in children with PMD may help direct
appropriate rehabilitation plans and expectations by the
implant team, school, and family. This study tests the hy-
pothesis that children with PMD experience slower rates
of speech perception gain after implantation than age-
matched children deafened by other causes, because of
neurocognitive residua of the illness.
METHODS
SUBJECTS
Our study included 30 children with PMD and 30 children deaf-
ened by other, predominantly congenital causes (the compari-
son group), all of whom received multiple-channel cochlear im-
plants at The Johns Hopkins Hospital, Baltimore, Md, between
1991 and 2002. All candidates had severe-to-profound levels
of sensorineural hearing loss based on the average of preim-
plantation unaided pure-tone thresholds at 500, 1000, and 2000
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Congenital Group
(n = 30) (n = 30) Statistics†
14 11
10 15 ␹2 = 1.8
6 4
1.4 (1.2) 1.4 (1.0) t = 0.03
1.8 (1.1) 1.6 (0.8) t = 0.62
4.3 (4.1) 4.2 (3.3) t = 1.3
2.9 (3.9) 2.8 (3.1) t = 1.3
2 2 ...
14 17 ␹2 = 0.6
20.8 (5.4) 22.4 (3.8) t = 1.3
Hz of 75- to 90-dB hearing level (HL) (severe) and over 90-dB
HL (profound). All subjects failed to experience speech per-
ception benefit from appropriately fit power hearing aids. Each
subject with PMD was matched with a child deafened by other
causes based on age at diagnosis, age at which hearing aids were
first used, age at cochlear implantation, and method of com-
munication used at home or in the classroom (Table 1).
PROCEDURES
All subjects at The Listening Center, The Johns Hopkins Hos-
pital, received audiological, speech perception, and psycho-
logical evaluations immediately before cochlear implantation.
Speech perception testing was conducted using age-
appropriate instruments (see below) in the best-aided condi-
tion both before and after surgery, using live voice presenta-
tion at conversational levels. All subjects received audiological
evaluations at 6, 12, and 24 months after activation. Informa-
tion about the acute meningitis illness and its medical se-
quelae were obtained from medical records requested with per-
mission from outside hospitals and from Hopkins records. This
study was approved by the institutional review board at The
Johns Hopkins University School of Medicine, Baltimore.
SPEECH PERCEPTION MEASURES
The speech perception tests used in the study included the Early
Speech Perception Test, Northwestern University–Children’s
Perception of Speech, Word Intelligibility by Picture Identifi-
cation (WIPI), Glendonald Auditory Screening Procedure, Lexi-
cal Neighborhood Test, and Phonetically Balanced Word Lists-
Kindergarten. The Early Speech Perception Test is a 4-level,
closed-set instrument that examines pattern perception, spon-
daic word identification, and monosyllabic word identifica-
tion using toy objects or pictures to represent the stimulus item.
It is most appropriately used with children 2 years of age and
older. The Northwestern University–Children’s Perception of
Speech is a 4-choice, closed-set picture test that is adminis-
tered to children 3 years of age and older. The WIPI is a 6-choice,
closed-set picture test with phonemically similar words for chil-
dren aged 6 years and older. The Glendonald Auditory Screen-
ing Procedure is an open-set task consisting of 10 common (3-
to 7-word) questions and 12 common words administered to
children aged 6 to 13 years. The Lexical Neighborhood Test is
an open-set list of 25 phonetically balanced words and is most
appropriate for children 3 years of age and older. The Phoneti-
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 score is identified in the data analysis as the SB/BSID Nonver-
1 2 3 4 5 6 bal IQ.
Detection Pattern Closed-Set Words Open-Set Recognition The Stanford-Binet yields 3 factor scores (each with a mean
ESP Pattern 50%-100%
[SD] of 100 [16]). The present study used only the Nonverbal
Reasoning/Visualization factor score, which reflects the abil-
ESP Spondee 33% ----- 50% ----- 100%
ity to interpret and organize visually perceived material, to per-
ESP Mono 33% ----- 50% ----- 100% form basic mathematical operations using visual cues, and to
NU-CHIPS 36% ----- 50% ----- 100% demonstrate visual-motor skills.22
WIPI 28% ---------- 100% The Bayley Scales yield 4 facet scores (expressed as a devel-
GASP-W 16% ----- 25% ----- 100%
opmental age, which is based on the highest developmental age
level at which the child displays mastery). The present study used
PBK-W 4% ------- 8% ----- 100%
only the Cognitive facet score, which assesses nonverbal problem-
GASP-S 20% ----- 30% ----- 100% solving skills. A developmental quotient (DQ) was calculated by
dividing the estimated developmental age by the chronological
Figure 1. Speech distribution categories.18 ESP indicates Early Speech age and multiplying by 100 (DQ = [developmental age/
Perception Test; NU-CHIPS, Northwestern University–Children’s Perception chronological age] ⫻ 100).
of Speech; WIPI, Word Intelligibility by Picture Identification; GASP,
Glendonald Auditory Screening Procedure (W, word; S, sentences); Adaptive Functioning
and PBK, Phonetically Balanced Word Lists-Kindergarten.
The Vineland Adaptive Behavior Scales (Survey Form) assess
adaptive skills and social competence in individuals from birth
9 through the age of 19 years.23 The Vineland is completed through
a structured interview with a parent or other respondent who
8
is familiar with the individual’s daily living activities. It as-
7 sesses an individual’s capabilities in 4 domains: communica-
tion, daily living skills, socialization, and motor. The combi-
6
nation of these 4 domains forms the Adaptive Behavior
No. of Subjects

5 Composite. All 4 domains and the Adaptive Behavior Com-

4
posite yield standard scores (mean [SD]=100 [15]).

3 Emotional/Behavioral Functioning
2
The Child Behavior Checklist is a written parent-report mea-
1 sure that was designed to assess behavioral and emotional prob-
0 lems in children aged 2 to 18 years,24,25 or as young as 18 months,
0 1 2 3 4 5 6 7 for serial assessments. It yields normalized scores (mean
Age at Diagnosis, y [SD]=50 [10]) in several specific areas as well as a total score.
Higher scores indicate greater behavior problems, with scores
Figure 2. Distribution of ages at which meningitis illness and resulting higher than 60 denoting clinically significant problems.
deafness occurred (n=30). Postmeningitic deafness was more prevalent
within the first 18 months of life; only 7 children developed postmeningitic
deafness in the postlingual period (after 2 years of age).
STATISTICAL METHODS

To compare characteristics before and after implantation be-

cally Balanced Word Lists-Kindergarten is a 50-item, open-set
word list derived from the spoken vocabulary of kindergarten-
age children and is administered to children 5 years of age and
older.
Based on original work by Geers and Moog,18 speech per-
ception measured using a variety of different instruments was
categorized on a common ordinal scale consisting of 6 levels
of performance (Figure 1). The use of this scale in a meta-
analysis of the pediatric cochlear implant literature19 has dem-
onstrated its validity as a tool for assessing the emergence of
open-set speech perception across a wide age range. The scale
incorporates several measures. Closed-set speech perception,
in which visual cues accompany auditory information, encom-
passes levels 1 to 4 inclusively, whereas open-set speech per-
ception, which depends solely on auditory information, cor-
responds to levels 5 and 6.
PSYCHOLOGICAL MEASURES
Nonverbal Cognitive Functioning
Nonverbal cognitive functioning was assessed using a subset
of the Stanford-Binet Intelligence Scale: Fourth Edition20 or, in
subjects younger than 2 years, a subset of the Bayley Scales of
Infant Development, Second Edition, Manual.21 The appropriate
tween the postmeningitic and comparison groups, 1-way analy-
sis of variance or the unpaired t test was conducted. One-way
analysis of variance was used to compare preimplantation scores
between groups in nonverbal cognition, adaptive functioning,
and emotional/behavioral functioning. The PMD and compari-
son groups were compared for the incidence of categorical out-
comes such as open-set speech perception and the incidence
of neurologic complications using ␹2 analysis. All statistical
analyses were performed using Statview (Version 5.0.1; SAS In-
stitute Inc, Cary, NC) and SPSS (Version 10.0; SPSS Inc, Chi-
cago, Ill) software. All P values less than .05 were considered
significant. All data are presented as mean (SD).
RESULTS
Approximately 30 (8.7%) of 343 children who under-
went implantation at our center over an 11-year period
between January 1992 and January 2003 were deafened
by meningitis. Most children acquired postmeningitic
deafness in infancy (Figure 2); only 7 were affected in
the postlingual period (⬎24 months of age). The bacte-
rium cultured from the CSF was known in 15 cases.
Twelve children were infected by Streptococcus pneumo-
niae, and 1 each by Haemophilus influenzae, Neisseria men-

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ingitides, and group B streptococci. A similar distribu-
tion of preoperative speech perception performance was
observed for both the children with PMD and the com-
parison group (Figure 3A). Closed-set speech percep-
tion was demonstrated by 27 and 28 children in the PMD
and the comparison groups, respectively, the majority of
whom performed at the lowest level of function. De-
tailed information was available regarding the cochleos-
tomy and array insertion in 27 of 30 children with PMD,
9 of whom required an extended cochlear drill-out or
trough. Of 28 cases in which the extent of electrode in-
sertion was known, 26 had full insertion.
Detailed behavioral and cognitive testing, which has
been routinely performed during the candidacy process
since 1995, was available for 18 children with PMD and
their matched counterparts. There were no significant dif-
ferences between the 2 groups in age at diagnosis, at first
hearing aid use, at implant surgery, or at evaluation. As
shown in Table 2, subjects generally demonstrated av-
erage nonverbal cognitive functioning (SB/BSID Non-
verbal IQ) before cochlear implantation. Overall adap-
tive abilities before implantation (Vineland) were in the
low-average range, with significant delays noted in the
communication and socialization domains. There were
no overall emotional/behavioral problems reported be-
fore implantation. The unpaired t test revealed no sig-
nificant group differences in preimplantation nonverbal
intelligence, adaptive abilities, or emotional/behavioral
functioning.
At an average of 21.5 (5.0) months after cochlear
implantation, there was a significant shift in the distri-
bution of speech perception performance toward open-
set hearing (categories 5 or 6, Figure 3) in both the PMD
group (␹2 = 34.3; P⬍.001) and the comparison group
(␹2 =40.1; P⬍.001). There was no difference in the dis-
tribution of speech perception levels between the PMD
and comparison groups at this postsurgical interval
(␹2 =3.1; P⬎.05). As a test of intermediate difficulty, the
WIPI test provided longitudinal data for the largest num-
ber of children over the longest duration. The applica-
bility of other speech recognition testing was limited to
the highest or lowest performers over shorter periods.
The WIPI scores over time suggested greater variability
in the rate of acquisition of speech perception among chil-
dren with PMD compared with children in the compari-
son group (Figure 4).
We assessed the incidence of clinical features of men-
ingitis to determine their impact on speech perception
after cochlear implantation. We compared children with
PMD and open-set hearing with those with only closed-
set hearing for clinical features of the acute illness
(Table 3). There was, on average, a 4-month differ-
ence in the postimplantation interval at which speech per-
ception data were available for subjects in the the open-
and closed-set groups. The only clinical feature found to
be significantly different between open-set and closed-
set listeners was the significantly higher incidence of hy-
drocephalus as a complication of meningitis in the closed-
set group (␹2 = 6.8; P⬍.01). Longitudinal WIPI scores
suggest that children with PMD who sustained neuro-
logic complications, including stroke syndrome, brain ab-
scess, cranial nerve deficits (other than deafness), and
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A
30
PMD
Congenital
25
20
No. of Subjects
15
Open Set
10
5
0
B 30
25 Open Set
20
No. of Subjects
15
10
5
0
1 2 3 4 5 6
Speech Perception Categories
Figure 3. Distribution of speech perception categories before (A) and 12 to
24 months after (B) cochlear implantation. Speech perception performance
was comparable in the meningitis and nonmeningitis (congenital) groups
both before and after surgery (␹2 = 3.1; P ⬎.05). Both groups experienced
improved speech perception performance after surgery (meningitis group,
␹2 = 34.3, P⬍.001; nonmeningitis group, ␹2 = 40.1, P⬍.001), including a
significant increase in the proportion of children with open-set speech
discrimination (meningitis group, ␹2 = 11.8, P⬍.001; nonmeningitis group,
␹2 = 17.3, P⬍.001). PMD indicates postmeningitic deafness.
hydrocephalus, were delayed in the acquisition of speech
perception abilities compared with other children with
PMD and the comparison group (compare Figure 4A and
B). These data also suggest that functional gains were not
rapid enough for 4 months of additional implant expe-
rience to explain the superior performance of the open-
set group.
We observed a trend toward lower CSF cell counts
in the closed-set group (t = 2.3; P = .06) (cell counts
⬍100/mm3: 5 of 6 in the closed-set group; 2 of 6 in the
open-set group). Communication method, age at ill-
ness, age at cochlear implantation, and presurgical cog-
nitive measures were not significantly different between
open-set and closed-set groups. Also, there were no dif-
ferences in the severity of cochlear obstruction or rate
of full electrode insertions between children with open-
set (5 of 13 subjects and 1 of 14 subjects, respectively)
and closed-set (4 of 14 subjects and 1 of 14 subjects, re-
spectively) speech perception abilities. There was no
significant difference in the distribution of cochlear im-
plant devices used by children with open- vs closed-set
hearing (␹2 =0.95; P⬎.05).
A comparison of all 6 subjects with postmeningitic
hydrocephalus with 18 subjects with PMD without this
complication confirmed that there was a significantly
lower speech perception performance in the hydrocepha-
lus group despite a mean postsurgical testing interval that
was comparable to that of the nonhydrocephalus group
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 Table 2. Comparison of Preoperative Cognitive Function in Children Deafened by Meningitis
and Matched Children With Congenital Deafness*

Meningitis Group Congenital Group

(n = 18) (n = 18) t Statistic†
Age at preoperative evaluation, mo 41.7 (40.3) 45.1 (36.9) 0.26
Nonverbal intelligence
Stanford-Binet/Bayley IQ/DQ 90.3 (13.4) 92.8 (11.8) 0.6
Adaptive abilities
Vineland Composite SS 82.2 (11.8) 79.8 (22.2) 0.4
Emotional/behavior
Child Behavior Checklist Total T-score 47.0 (6.9) 48.9 (5.5) 0.9

Abbreviations: DQ, developmental quotient; SS, standard score.

*Data are mean (SD). Cognitive data only available for children who received cochlear implants after 1996.
†P ⬎.05 for all.

rologic complications of meningitis were more preva-

A 100 lent in the hydrocephalus group, particularly stroke
(␹2 =5.8; P⬍.05). There were no significant differences
80
in the results of CSF studies (P⬎.05) or in the preva-
60 lence of cochlear scarring (1 of 5 subjects vs 7 of 16 sub-
jects) and incomplete electrode insertion (0 of 5 sub-
Score, %

40
jects vs 1 of 17 subjects) between children with and
20
without hydrocephalus (␹2 =0.9; P⬎.05).

0
COMMENT
–20

The results of our study suggest that neurologic se-

B 100 quelae of meningitis impede the development of speech
perception after cochlear implantation in children with
80
PMD. Cochlear implantation increases auditory sensi-
60 tivity by direct electrical activation of auditory nerve fi-
bers, enabling phonemic awareness, discrimination, and
Score, %

40 identification and ultimately yielding speech understand-

20
0 information to generate the physiologic substrate of speech
–20
–10 0 10 20 30 40 50 60 70 80
Time After CI, mo
Figure 4. Word Intelligibility by Picture Identification (WIPI) scores of
children deafened by meningitis at different times after cochlear implantation
(CI) in comparison to children deafened by other causes (open circles). In
both figures, each child with postmeningitic deafness (PMD) is assigned a
unique symbol. A, Despite some variability in the increase of WIPI scores
among children with PMD without neurologic complications, the progression
of open-set speech perception skills is similar to that of children in the
comparison group (open circles), most of whom approach maximum scores
within the first 12 to 24 months. B, Compared with children who are not
deafened by meningitis (open circles), neurologically involved children with
PMD are more likely to experience a gain in speech perception scores that is
delayed in onset and more gradual.
(Table 4). Also, there was no significant difference in
age at diagnosis of illness (t= 0.8; P⬎.05), age at implan-
tation (t=1.1; P⬎.05), or prevalence of oral communi-
cation method (t = 2.1; P⬎.05). Three children with hy-
drocephalus for whom cognitive test scores were available
before cochlear implantation demonstrated similar non-
verbal intelligence and adaptive abilities compared with
other children with PMD, but they scored more poorly
on the behavioral assessment (t=2.4; P⬍.05). Other neu-
ing. These data indicate, however, that central auditory
stations must be capable of processing implant-encoded
comprehension. The presence of clinically evident men-
ingitic residua within the CNS appeared to hold sway over
cochlear ossification and associated degeneration of the
auditory nerve as a predictor of attenuated growth rates
of speech perception skills after implantation. When the
burden of early auditory deprivation is combined with
the increased risk of cognitive and behavioral deficits as-
sociated with meningitis, children with PMD can face sig-
nificant challenges in acquiring spoken language skills
after implantation. Nonetheless, auditory benefit mani-
fests even in this high-risk group.
Despite an average of 1.4 years of postnatal audi-
tory experience before the onset of PMD, children with
implants who were prelingually deafened by meningitis
acquired levels of open-set speech discrimination simi-
lar to those of the comparison group, most of whom were
deaf at birth. Variability in the growth of WIPI scores in
children with PMD (Figure 4), however, suggests that
meningitis poses an additional burden on the acquisi-
tion of speech perception by these children, even though
many of them eventually achieved the same level of per-
formance as their congenitally deafened peers, albeit af-
ter delays of as much as 5 years. As previously reported
in prelingually deafened children with cochlear im-

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 Table 3. Comparison of PMD Children With and Without Open-Set Hearing After Cochlear Implantation*

Open-Set Hearing Closed-Set Hearing

(n = 14) (n = 16) Statistics
Oral communication, No.† 9/14 5/16 ␹ = 3.3, P⬎.05
2

Age at PMD, y 1.8 (1.5) 1.1 (0.7) t = 1.8, P⬎.05

Age at amplification, y 2.2 (1.4) 1.4 (0.6) t = 2.29, P⬍.05
Age at CI, y 4.9 (4.3) 3.8 (4.0) t = 0.9, P⬎.05
Interval without CI, y 2.7 (3.5) 3.1 (4.4) t = 0.02, P⬎.05
Preoperative open-set discrimination, No. 1/13 1/16 ...
Interval to postoperative speech discrimination test, mo 23.1 (3.2) 18.8 (6.1) t = 2.4, P⬍.05
Cognitive measures (n = 8) (n = 10)
PreIQ 90.5 (10.5) 90.2 (16.0) t = 0.8, P⬎.05
Vineland 80.7 (11.6) 81.6 (11.6) t = 0.1, P⬎.05
CBCL total 44.3 (8.1) 49.2 (5.1) t = 1.4, P⬎.05
Neurologic complications, No.
Any present 5/11 9/13 ␹2 = 1.4, P⬎.05
Seizure 4/11 7/11 ␹2 = 1.6, P⬎.05
Hydrocephalus 0/11 6/13 ␹2 = 6.8, P⬍.01
Stroke syndrome 2/11 6/12 ␹2 = 2.6, P⬎.05
Brain abscess 1/10 3/12 ␹2 = 1.0, P⬎.05
CSF studies (n = 6) (n = 6)
Glucose, mg/dL 10.3 (14.9) 11.0 (7.0) t = 0.1, P⬎.05
Protein, mg/dL 232.3 (84.3) 335 (258.5) t = 0.9, P⬎.05
Cell count, /mm3 267.3 (240.8) 54.2 (45.4) t = 2.1, P = .06
Cochlear patency, No.
Obliterative scar‡ 5/13 4/14
␹2 = 0.3, P⬎.05
Incomplete insertion 1/14 1/14
Device, No.
ABC Clarion 3 6
ABC HiFocus 1 1
␹2 = 0.95, P⬎.05
CC Nucleus 22 7 6
CC Nucleus 24 3 3

Abbreviations: ABC, Advanced Bionics Corporation; CBCL, Child Behavior Checklist; CC, Cochlear Corporation; CI, cochlear implantation; CSF, cerebrospinal
fluid; PMD, postmeningitic deafness.
SI conversion factor: To convert glucose to millimoles per liter, multiply by 0.0555.
*Data are mean (SD) unless otherwise indicated.
†Of 14 children with open-set hearing after CI, sign, total communication, and oral methods of communication were used in 1, 4, and 9 children, respectively,
whereas 5, 6, and 5 children with closed-set hearing used these methods.
‡Requiring drill-out to at least the first turn.

plants,12,26,27 auditory experience before PMD does not
confer a functional advantage, possibly because of in-
jury to the auditory pathway. In fact, a slower improve-
ment in speech perception skills has been observed in
children with PMD in the first year of implant experi-
ence.13 In a study of electrically evoked auditory brain-
stem responses in deaf children, Nikolopoulos et al28 at-
tributed the inferior performance of children with PMD
to a reduction in the electrical responsivity of the coch-
lea as reflected in higher thresholds and poorly defined
electrically evoked auditory brainstem response wave-
forms. In children with PMD, responses were, on aver-
age, significantly attenuated and longer in latency and
the growth of the response was slower.
A central question concerns whether PMD effects
manifest peripherally (cochlear scalae and auditory nerve)
or centrally (auditory nerve structure and conductivity
of central pathways) to produce lower-than-expected gains
in auditory perception after implantation. Constraints on
auditory rehabilitative benefit may also represent a con-
tinuum of injuries that includes central auditory sta-
tions that are responsible for the development of linguis-
tic function. The similar incidence of cochlear scarring
and ossification and incomplete electrode insertions be-
tween children with PMD who have open-set and those
who have closed-set hearing suggests that peripheral ef-
fects of meningitis may have relatively little influence on
ultimate speech recognition results. One caveat is that a
minimal number of electrode channels must be avail-
able for auditory nerve activation. The peripheral ef-
fects of meningitis on the parameters of electrical stimu-
lation that are required for optimal speech perception were
not addressed in the present study and require further
evaluation.
Even in the absence of postmeningitic deafness and
clinically obvious neurologic compromise, language defi-
cits, lower IQs, and increased behavioral problems oc-
cur as a result of CNS changes after meningitis.3,16 Since
neurologic complications due to meningitis are more
prevalent in children with hearing deficits than in normal-
hearing children,4,5 our results reassert that children with
PMD have the added burden of CNS injury and its ef-
fects on central processing of auditory inputs.
The present study also extends observations that
children with PMD suffer from neurologic complica-
tions that can pose particular rehabilitation challenges
after cochlear implantation. Children with PMD and
CNS residua exhibited a slower acquisition of speech

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 Table 4. Comparison of Children Deafened by Meningitis With and Without Hydrocephalus Complication*

Hydrocephalus Present Hydrocephalus Absent

(n = 6) (n = 18) Statistics
Oral communication, No.† 1/6 9/18 ␹ = 2.1, P⬎.05
2

Age at PMD, y 0.9 (0.6) 1.2 (0.8) t = 0.8, P⬎.05

Age at CI, y 2.1 (0.9) 4.1 (4.4) t = 1.1, P⬎.05
Interval without CI, y 1.2 (1.1) 2.9 (4.2) t = 1.0, P⬎.05
Preoperative open-set discrimination, No. 0/6 2/18 ␹2 = 0.7, P⬎.05
Postoperative open-set discrimination, No. 0/6 11/18 ␹2 = 6.8, P⬍.01
Interval to postoperative speech discrimination test, mo 24.0 (0) 21.3 (5.1) t = 1.3, P⬎.05
Cognitive measures
PreIQ 87 (26.9); n = 3 89.9 (11.7); n = 3 t = 0.4, P⬎.05
Vineland 78.6 (10.9); n = 3 81.6 (13.2); n = 11 t = 0.4, P⬎.05
CBCL total 54.0 (3.7); n = 3 44.6 (6.8); n = 12 t = 2.3, P⬍.05
Coincidence with other complication, No.
Seizure 4/5 7/17 ␹2 = 2.3, P⬎.05
Stroke syndrome 4/5 4/18 ␹2 = 5.8, P⬍.05
Brain abscess 2/5 2/18 ␹2 = 2.3, P⬎.05
CSF studies
Glucose, mg/dL 12.0 (8.7) 10.0 (12.6) t = 0.3, P⬎.05
Protein, mg/dL 394.3 (311.4) 228.4 (72.7) t = 1.5, P⬎.05
Cell count, /mm3 58.8 (54.2) 211.8 (228.5) t = 1.3, P⬎.05
Cochlear patency, No.
Obliterative scar‡ 1/5 7/16 ␹2 = 0.9, P⬎.05
Incomplete insertion 0/5 1/17

Abbreviations: See Table 3.

SI conversion factor: See Table 3.
*Data are mean (SD) unless otherwise specified.
†Of 6 children with hydrocephalus, sign, total communication, and oral methods of communication were used in 3, 2, and 1 children, respectively, whereas 3,
6, and 9 children without hydrocephalus used these methods.
‡Requiring drill-out to at least the first turn.

perception (Figure 4B) and a lower rate of open-set
hearing after 2 years of implant experience (Tables 3
and 4). Hydrocephalus was likely to be both a sequela
of severe CNS insult and a potential cause of further
compromise. The increased incidence of stroke in chil-
dren with hydrocephalus reflects the severity of brain
injury. Adaptive behaviors were poor in 3 children with
hydrocephalus for whom cognitive testing was per-
formed. Although this correlation between postmenin-
gitic hydrocephalus and poor adaptive behaviors needs
to be confirmed and further elucidated in a larger
sample of subjects, it nonetheless points to behavioral
problems as a potential impediment of the postimplan-
tation rehabilitation process. The design of the rehabili-
tation plan should therefore take into consideration the
need for behavioral therapy.
The long-term effects of hydrocephalus and other
central complications of meningitis on language devel-
opment are unknown, but substantial delays are likely.
Auditory and language rehabilitation should therefore be
sustained, intensive, and frequently evaluated to appro-
priately focus the therapeutic effort on the long-term goal
of language development. Children with PMD and CNS
complications are at increased risk of behavioral and emo-
tional problems that may complicate efforts by profes-
sionals to develop the hierarchy of listening skills that
are required for the development of speech perception
after cochlear implantation. Although progress is likely
to be slower than for the average pediatric implant re-
cipient with PMD, appropriate intervention is likely to
effect meaningful gains in communication.
Submitted for publication January, 30, 2004; final revision
received February 12, 2004; accepted February 12, 2004.
This study was presented at the Eighth Symposium on
Cochlear Implants in Children; March 3, 2001; Los Ange-
les, Calif.
Corresponding author and reprints: Howard W. Fran-
cis, MD, 601 N Caroline St, Johns Hopkins Outpatient Cen-
ter, Sixth Floor, Baltimore, MD 21287-0910 (e-mail:
hfrancis@jhmi.edu).
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