Download Spectrum of Immune-Mediated Inner Ear Disease and Cochlear

The Laryngoscope
C 2012 The American Laryngological,
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Rhinological and Otological Society, Inc.
Spectrum of Immune-Mediated Inner Ear Disease and Cochlear
Implant Results
Mohammad U. Malik, MD; Vinciya Pandian, PhDc, CRNP; Hamid Masood, MD;
David A. Diaz, MD; Voss Varela, MD; Alfredo Jose D
avalos-Balderas, MD; Martha Parra-Cardenas, MD;
Phillip Seo, MD; Howard W. Francis, MD, MBA
Objectives/Hypothesis: To characterize the progression of hearing loss in patients with immune-mediated inner ear
disease (IMIED), and to identify disease- and patient-specific factors associated with cochlear implant (CI) performance.
Study Design: Retrospective cohort study.
Methods: Subjects consisted of CI patients suspected to have lost their hearing due to IMIED. The primary dependent
variable for functional decline was time to deafness, whereas for CI benefit it was post-CI speech perception scores. Independent
variables included presence or absence of systemic autoimmune disease, age at CI, and insertion depth of the cochlear electrode.
Results: A transient favorable response to immunosuppressive therapy was reported in 16 of 26 patients (66.67%). The
time to deafness differed between an organ (ear)-specific immune-mediated group, a systemic immune-mediated group
including Cogan syndrome and relapsing polychondritis (subgroup A), and a systemic immune-mediated group associated
with other autoimmune diseases (subgroup B; P ¼ .001). Disease group (15.52; P ¼ .04), insertion depth of the CI electrode
(40.71; P ¼ .01), and the age at CI (0.48, P ¼ .05) were associated with speech perception results.
Conclusions: Triaging IMIED cases based on presence and type of systemic autoimmune disease may aid in selecting a
management strategy. Knowledge about the predictors of CI outcome will help clinicians select appropriate patients for CIs.
In the setting of significant and irreversible hearing deficit, the restoration of hearing using a cochlear prosthesis may be
appropriate earlier rather than later.
Key Words: Autoimmune inner ear disease, immune-mediated inner ear disease, cochlear implant, steroid therapy,
speech perception, electrode insertion, ossification.
Level of Evidence: 4
Laryngoscope, 122:2557–2562, 2012
INTRODUCTION
Immune-mediated inner ear disease (IMIED) results in
<1% of all cases of acquired deafness, and can be accompanied by tinnitus and vestibular disturbance. Inner ear
manifestations may occur in isolation or accompany other
autoimmune disorders. McCabe, who studied a cohort of
patients with bilateral progressive hearing loss, supported
the theory of an autoimmune etiology for rapidly progressive
bilateral hearing loss when he found that hearing improved
after treatment with steroids and cytotoxic therapy.1
Systemic steroids form the mainstay of immunosuppressant therapy in cases of IMIED, but there remains a
high rate of eventual treatment failure. Broughton et al.2
demonstrated a short-term response in 70% of patients to
From the Department of Otolaryngology-Head and Neck Surgery,
Johns Hopkins University (M.U.M., H.M., D.A.D.V.V., A.J.D.-B., M.P.-C., H.W.F.);
Percutaneous Tracheostomy Service, Johns Hopkins Hospital (V.P.); and
Johns Hopkins Vasculitis Center, Johns Hopkins University (P.S.),
Baltimore, Maryland, U.S.A.
Editor’s Note: This Manuscript was accepted for publication June
26, 2012.
The authors have no funding, financial relationships, or conflicts
of interest to disclose.
Send correspondence to Howard W. Francis, MD, 601 N. Caroline
St. Suite 6251, Johns Hopkins Outpatient Center, Baltimore, MD 21287.
E-mail: [email protected]
DOI: 10.1002/lary.23604
Laryngoscope 122: November 2012
steroids. This response rate, however, deteriorated to 14%
over a follow-up period of 34 months. Rauch has previously reported a response rate of 60% to steroids.3
Similarly, Loveman et al.,4 in their retrospective analysis
of 30 patients, noted a response rate of 50%, leading the
authors to conclude that IMIED does not reliably respond
to steroids. Eventually, patients with IMIED, after being
placed on several trials of immunosuppressive agents,
reach a level of profound deafness and are deemed candidates for cochlear implantation (CI). Although favorable
CI benefits have been reported, prognostic indicators
unique to this population are lacking.5
The aims of our study were to retrospectively examine the progression of hearing loss in a cohort of adult CI
patients with a diagnosis of IMIED, and to identify the
patient and disease factors that influence speech perception outcome with a CI in this population. We
hypothesized that IMIED progression would be predictive
of CI outcome and should be considered in the management strategy of these patients.
MATERIALS AND METHODS
Patients
After obtaining institutional review board approval, a retrospective chart review was performed on patients who received
Malik et al.: Immune-Mediated Inner Ear Disease
2557
a CI at Johns Hopkins University between 1995 and 2009, and
were suspected of having suffered from IMIED. Demographic
information and details of the hearing loss and its treatment as
well as associated symptoms were obtained from the medical
record.
The patients were selected based on information documented in history of present illness and past medical history, as
well as available laboratory and audiological data. Inclusion criteria included: 1) clinical manifestation of rapidly progressive
sensorineural hearing loss (SNHL) with or without systemic
autoimmune disease; 2) treatment response to steroids or other
immunosuppressant therapy; 3) results of antigen nonspecific
immunological tests; 4) results of antigen-specific immunological tests; and 5) exclusion of other etiologies of hearing loss.6
Antigen nonspecific immunological tests included erythrocyte
sedimentation rate, antinuclear antibody, antineutrophil cytoplasmic autoantibody, and rheumatoid factor. Antigen-specific
immunological tests included anti-68 Kd antibody (HSP-70) and
lymphocyte transformation test (LTT).7,8 Ten patients were
diagnosed on clinical symptomatology, audiological testing of
SNHL, and response to steroids alone. Five of these patients
had concurrent systemic autoimmune diseases, further raising
suspicion of an IMIED diagnosis. The antigen-nonspecific
immunological tests were available for 16 patients. The diagnosis in these patients was further supported by a high index of
clinical suspicion and fluctuating response to the steroids. The
antigen-specific immunological tests were available for nine
patients (anti-68 kd antibody in seven patients, LTT in two
patients). In thirteen patients, otosyphilis was ruled out by performing rapid plasma reagin and fluorescent treponemal
antibody absorption test. In all cases, a retrocochlear etiology of
hearing loss was ruled out by computed tomography or magnetic resonance imaging (MRI) imaging.
a series of two lists with 10 sentences each that is administered
without visual cues in a quiet environment, and may then be
repeated in the presence of speech-shaped noise to evaluate
hearing in noise.10 The CNC test is a measure of single-syllable
words that consists of 10 lists. Each list contains 50 monosyllabic words that can be scored as the percentage of correct
words (CNC-W) or phonemes (CNC-P).11 The duration of deafness was the time interval between the day of CI surgery and
the onset of profound hearing loss based on patient report or
the first available audiological test reporting a PTA >90 dB
SPL. The effects of duration of deafness, cochlear ossification,
and electrode insertion depth were explored using univariate
and multivariate analyses.
Statistical Analysis
Statistical analysis was performed using STATA 12.0 (StataCorp, College Station, TX). Normality of the variables was
assessed by using Shapiro–Wilk test. Continuous data for normally distributed values were compared between the two
groups using t tests, and between three or more groups using
analysis of variance. Categorical variables were compared using
v2 or Fisher exact test. Wilcoxon signed rank test was used to
compare nonparametric continuous data. Time to deafness was
analyzed using Kaplan–Meier curves and log-rank tests. Longitudinal data obtained for auditory tests were analyzed using
generalized linear mixed models controlling for random effects
such as time and the ear affected. Cox regression analyses were
performed to control for influencing variables while exploring
the effects of the covariate time to deafness. Multiple linear
regression and univariate regression analyses were performed
to identify the predictors of post-CI speech perception.
RESULTS
Hearing Outcome Measures
Overall IMIED Patient Characteristics
A favorable response to therapy was defined as the stabilization of functional decline or functional improvement in
hearing after the initiation of pharmacotherapy as demonstrated by auditory test scores and/or subjective improvement
reported by the patient. Time to deafness was defined as the period between the onset of hearing loss symptoms and the
development of severe to profound hearing loss. Changes in
hearing sensitivity were measured by: 1) recorded pure tone average (PTA)—an average of pure tone hearing thresholds
determined at 500, 1,000, and 2,000 Hz; 2) speech reception
threshold—minimum intensity of sound required for a patient
to perceive and repeat 50% of a list of two-syllable words; and
3) speech awareness threshold (SAT)—minimum hearing level
of speech at which an individual can discern the presence of
speech 50% of the time.9 Changes in hearing clarity were measured by speech discrimination score (SDS)—the patient’s ability
to distinguish phonetic elements of speech.9
Post-CI speech perception results were measured at four
time points: preimplant (time 0), 6 to 11 months post-CI (time
1), 12 to 17 months post-CI (time 2), and 18 to 23 months postCI (time 3). Preoperative speech recognition testing was completed in the best-aided condition in sound field. Postoperative
speech recognition testing was completed in the CI only condition in the sound field without use of a contralateral hearing
aid. Speech stimuli were administered via a compact disk of a
recorded male speaker’s voice presented at 70 dB SPL. Stimuli
were presented from a loudspeaker at 0 azimuth to the participant and a distance of approximately 1 m. Speech perception
was assessed using the hearing in noise sentence test presented
in quiet (HINT-Q), and consonant nucleus consonant word
(CNC-W) or phonemes (CNC-P) list. The HINT sentence test is
We identified 26 CI patients with suspected IMIED
with equal numbers of men and women. Of the 26
patients, 18 (69.2%) were Caucasians and six (23.1%)
were African Americans (Table I). The median age of
onset of hearing loss for the first ear was 55 years
(range, 24–84 years). Reports of hearing fluctuations
accompanied progressive hearing loss in 8 (30.8%) subjects. Tinnitus and dizziness accompanied hearing loss
in 11 (42.3%) and 14 (53.9%) subjects, respectively. Hearing loss started unilaterally in 20 (76.9%) patients and
bilaterally in six (23.1%). The time to bilateral deafness
from onset of hearing loss in one or both ears occurred
over a median time period of 4.5 months (range, 0.5–120
months). Seven patients (26.9%) were only treated with
oral steroids, and seven (26.9%) received a combination
of oral and intratympanic steroids. Nine (34.6%) patients
received other immunosuppressive agents in addition to
steroids, such as methotrexate, cyclophosphamide, or
mycophenolate mofetil. Although progressive hearing
loss was ultimately refractory in all patients, a transient
favorable response was reported or documented in 16
patients (66.7%).
The preimplant audiological reports were available
for 20 patients. The softest volume level at which speech
was heard, measured as SAT, increased at the average
rate of 9.5 dB HL per month (P ¼ .02) after onset of
hearing loss. The mean hearing threshold, measured
as PTA, increased on average by 2.6 dB HL per month
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2558
Malik et al.: Immune-Mediated Inner Ear Disease
TABLE I.
Overall Patient Characteristics and Characteristics of Immune-Mediated Groups.
Overall, n ¼ 26
Organ Specific Immune
Mediated, n ¼ 16
Systemic Immune
Mediated, n ¼ 10
P
Female, No. (%)
Race, No. (%)
13 (50)
6 (37.50)
7 (70)
.11
Caucasians
19 (73.08)
11 (68.75)
8 (80)
.81
African Americans
Other
5 (19.23)
2 (7.69)
3 (18.75)
2 (12.50)
2 (20)
0
55 [24–84]
55.5 [28–84]
51.5 [26–76]
.27
.09
Variable
Demographics
Progression of hearing loss
Median age of onset of hearing loss
in first ear, yr [range]
Ear affected first, No. (%)
Right
10 (38.46)
4 (25)
6 (60)
Left
Both
10 (38.46)
6 (23.08)
6 (37.50)
6 (37.50)
4 (40)
0
4.5 [0.5–120]
20.5 [1–120]
3.5 [0–120]
Median time to deafness, mo [range]
Associated symptoms, No. (%)
Hearing loss fluctuation
Tinnitus
Dizziness
Pharmacotherapy, No. (%)
8 (30.77)
6 (37.50)
2 (20)
.31
11 (42.31)
7 (43.75)
4 (40)
.58
14 (53.85)
10 (62.50)
4 (40)
.24
.45
Steroids only
7 (26.92)
4 (25)
3 (30)
Steroids þ intratympanic steroids
Steroids þ intratympanic þ
immunosuppressive agents
7 (26.92)
3 (11.54)
6 (37.50)
2 (12.50)
1 (10)
1 (10)
Steroids þ 1 non–steroid-based
immunosuppressive agents
6 (23.08)
2 (12.50)
4 (40)
Unknown
3 (11.54)
2 (12.50)
1 (10)
Response to therapy, No. (%)
No response
Responsive in some fashion
Unknown
Implant information
.07
8 (33.33)
3 (20)
5 (55.56)
16 (66.67)
12 (80)
4 (44.44)
2
1
1
.07
Age at cochlear implant, yr [range]
54.53 [24–84]
55.31 [24–84]
53.3 [36–84]
.73
Duration of deafness, mo [range]
12.4 [1–53.73]
12.17 [1–49.36]
13.09 [1.5–53.73]
.79
(P < .01), and the percentage of words correctly identified on SDS testing decreased on average by 15.8% per
month (P < .01).
Characteristics of Immune-Mediated Groups
(Organ-Specific vs. Systemic IMIED)
A total of 16 patients had no symptoms or signs of
systemic autoimmune disorders and were categorized as
belonging to an organ (ear)-specific IMIED (OS-IMIED)
group. Ten patients presented with a constellation of
symptoms consistent with systemic autoimmunity or
had pre-existing diagnoses of such disease and were designated to a systemic IMIED (S-IMIED) group. Systemic
disorders found in the S-IMIED group included relapsing polychondritis (RP; n ¼ 3), Cogan syndrome (CS; n
¼ 2), and one patient each with Sj€ogren syndrome (n ¼
1), primary sclerosing cholangitis (n ¼ 1), rheumatoid
arthritis (n ¼ 1), Wegener granulomatosis (n ¼ 1), and
cerebral vasculitis (n ¼ 1).
Laryngoscope 122: November 2012
There were no differences between the IMIED
groups in terms of age of onset and the constellation of
presenting cochleovestibular symptoms (Table I),
although S-IMIED cases were more likely to present
unilaterally (100%) as compared to OS-IMIED cases
(62.5%; P ¼ .05). The median time to deafness was
shorter for S-IMIED compared to OS-IMIED (3.5 vs.
20.5), but this difference barely reached significance
(P ¼ .07). There was a tendency for OS-IMIED patients
to respond more favorably to pharmacotherapy compared to S-IMIED (80% vs. 44.44%), but this only
achieved a P value of .07 (v2 ¼ 3.2).
Characteristics of Systemic Immune-Mediated
Group (Subgroup A vs. Subgroup B)
The S-IMIED group was further examined for any
differences in disease progression between the subgroups
of patients with syndromes that are associated with
inner ear dysfunction (subgroup A), including CS (n ¼ 2)
Malik et al.: Immune-Mediated Inner Ear Disease
2559
associated with a 40.7% higher HINT-Q score on average
compared to incomplete insertion (P < .05). Multivariate
analysis for the above covariates at time 1 did not reveal
any significance. Significant effects were not realized for
the other speech perception tests at either time point.
There was no significant association between baseline
speech perception and post-CI performance on any test.
DISCUSSION
Fig. 1. This figure compares time to deafness between three
immune-mediated inner ear disease (IMIED) groups: 1) organ (ear)
specific immune-mediated (OS-IMIED), 2) a systemic immunemediated group consisting of relapsing polychondritis and Cogan
syndrome (subgroup A), and 3) other systemic autoimmune diseases (subgroup B). Systemic subgroup A demonstrated the fastest deterioration in hearing function, followed by the OS-IMIED
group and systemic subgroup B.
and RP (n ¼ 3), and subjects with other forms of systemic autoimmune disease (subgroup B). Subjects in
subgroup A experienced the fastest progression to deafness, whereas cases in subgroup B had the slowest
progression. The OS-IMIED group progressed at an intermediate rate (P < .001; Fig. 1). In addition, Cox
regression analysis showed a significantly different hazard ratio for time to deafness between the three groups
(P < .001). No significant differences were noted when
the three groups were compared in terms of gender,
race, age of onset of hearing loss, or associated audiovestibular symptoms.
Evaluation of CI Results and Predictors
Mean delay to CI after achieving severe to profound
hearing loss (duration of deafness) was 12.4 months
(standard deviation, 15.53; range, 1–53.73). Four
patients (16%) had cochlear scarring found on MRI scans
prior to CI surgery. With the exception of two patients in
whom advanced cochlear obstruction prevented full electrode insertion, complete insertion was achieved in all
other subjects. There was no significant difference in
baseline pre-CI speech perception scores between OSIMIED and S-IMIED subjects (v2 ¼ 2.43, P ¼ .11). There
were significant gains in speech perception at 6 to 11
months (time 1) post-CI relative to baseline (Fig. 2) for
HINT-Q (mean 6 standard deviation, 14.8623.4 vs.
75.7624.9), CNC-W (9.1612.1 vs. 54.4625.5), and CNCP (19.4621.0 vs. 71.7617.9; all P < .001). Thereafter
group mean scores remained relatively stable. Multivariate regression analysis revealed that at 12 to 17 months
post-CI (time 2), HINT-Q scores were significantly
higher by 15.52 points on average for the OS-IMIED
compared to the S-IMIED group (P ¼ .04; Table II).
HINT-Q scores decreased by 0.48% for every additional
year to the age of CI (P ¼ .05), and a full insertion was
Laryngoscope 122: November 2012
2560
This study describes a cohort of IMIED patients
with asymmetric and rapid progressive sensorineural
hearing loss but otherwise variable clinical presentation.
Differences were found in the rate of functional decline
between patients with isolated inner ear disease, inner
ear disease associated with Cogan’s Syndrome and
relapsing polychondritis, and inner ear disease associated with other systemic autoimmune diseases. Despite
loss of audition to a profound level in all cases, there
was substantial recovery of speech perception using a
cochlear prosthesis that was sustained over a follow-up
of up to 2 years. The OS-IMIED group, younger age at
CI and complete electrode insertion were associated with
better speech perception.
Communication difficulties are the chief cause of
disability in patients with IMIED resulting from an average monthly SDS decline of 15.8% in this cohort
despite treatment.12 Furthermore, vestibular effects of
IMIED (as seen in 54% of our cases) threaten independent function and injury from falls. The research
identifies that both cell-mediated immunity and humoral
immunity might have a role in mediating damage to the
inner ear. Lorenz et al. found that Interferon-gamma
producing T-cells specific to inner ear antigen were elevated when compared to healthy controls.13 Gloddek
et al.14 further demonstrated the production of specific
populations of T cells targeted against inner ear antigens, after in vitro stimulation with appropriate
antigens. Histological manifestations of IMIED include
strial vasculitis, atrophy of the sensory epithelium, and
Fig. 2. This figure shows the speech perception scores of all
immune-mediated inner ear disease patients. These scores were
assessed using hearing in noise test (HINT) and Consonant Nucleus Consonant (CNC) scores. A significant increase in speech
perception scores is seen within 6 to 11 months (time 1) following
implantation for all tests (CNC word, CNC phonemes, and HINT in
quiet). This improvement in speech perception scores is sustained
at later time intervals (times 2 and 3).
Malik et al.: Immune-Mediated Inner Ear Disease
TABLE II.
Predictors of Hearing Outcomes 12 to 17 Months
(Time 2) Post-Cochlear Implantation.
Speech Perception Score Coefficient (P)
Covariates
HINT-Q
CNC-W
CNC-P
15.52 (.04*)
21.37 (.07)
9.57 (.24)
40.71 (.01*)
17.88 (.37)
6.49 (.65)
0.05 (.79)
0.25 (.35)
0.09 (.63)
Age at cochlear
implant, yr
0.48 (.05*)
0.02 (.94)
0.13 (.55)
Constant
46.90
50.40
79.14
S-IMIED group
Full depth of electrode
insertion
Duration of deafness
in months
Speech perception scores are surrogates of postcochlear implant
outcomes. Multivariate model is adjusted for type of group, electrode insertion, duration of deafness, and age at cochlear implant.
*Statistically significant.
HINT-Q ¼ hearing in noise sentence test presented in quiet;
CNC-W ¼ consonant nucleus consonant word; CNC-P ¼ consonant
nucleus consonant phonemes; S-IMIED ¼ systemic immune-mediated
inner ear disease.
new bone formation within the scala tympani.15 The differential diagnosis of IMIED is broad and includes
syphilis, Lyme disease, and ototoxicity from medications
such as hydrocodone/paracetamol, sildenafil, and aminoglycosides.16,17 The continuous systemic use of
glucocorticoids is restricted by adverse side effects.7 Intratympanic glucocorticoid therapy delivers a higher
intralabyrinthine
concentration
while
eliminating
systemic side effects. In steroid-responsive cases, intratympanic glucocorticoids may restore and maintain
function with serial treatments.18 Alternative immunosuppressive strategies are tried in patients who either
fail to respond to glucocorticoids or do not maintain functional improvement when therapy is withdrawn. These
strategies include the use of methotrexate, cyclophosphamide, azathioprine, tumor necrosis factor antagonists,
and plasmapheresis. An overall response rate of 66% for
all immunosuppressant strategies attempted in our study
cohort was comparable to that reported in previous studies.2–4 Loveman et al.4 showed that hearing loss could be
stabilized or even restored in about 40% to 50% of cases
with objective audiometric evidence of improvement. In a
study of 42 patients, Broughton et al. reported an initial
70% response rate to steroids followed by subsequent
decline; Rauch reported a 60% response rate in the treatment of patients with IMIED.2,3
We observed a difference in the rate of progression
and response to intervention between patient groups
(Table I). A relatively rapid course to deafness in
patients with CS and RP (subgroup A) from the SIMIED group could imply a larger burden of immune
dysfunction or resulting tissue injury in this patient
group. Differences in CI results between OS-IMIED and
S-IMIED groups suggest that the effects of IMIED are
not only confined to the sensory epithelium but may also
extend to the spiral ganglion and the fidelity with which
the auditory nerve transmits signals to the central nervous system. Further study is needed to examine a
putative link between indicators of graded immune
response, the spectrum of presenting symptoms, the proLaryngoscope 122: November 2012
gression of functional loss, and subsequent hearing
results with the cochlear prosthesis.
Speech perception results reported in this study are
comparable to other reports in IMIED patients, which
include open-set word recognition and sentence scores of
61% and 69%, respectively, at 6 months, with subsequent scores of 84% and 100% at 1 year.19 In a similar
study, Aftab et al.5 reported no difference in hearing outcomes between 10 patients with IMIED compared to 12
randomly selected CI patients with other diagnoses.
Unlike studies in diverse patient populations, the present study did not reveal a significant effect of baseline
speech understanding or duration of deafness on post-CI
speech perception.20–22 This may result from relatively
little variability in the profound level of preoperative
hearing loss and the short time to CI.
The benefits of CI warrant careful consideration
relative to the risks associated with prolonged immunosuppressive therapy. Optimal long-term hearing function
may be more likely to be achieved if CI is pursued while
there is still residual hearing and an open cochlea. The
tendency for inflammatory disease to induce an endosteal reaction leading to cochlear obliteration by scar
and new bone formation poses an occasional challenge to
CI surgery in the IMIED patient population. Incomplete
insertion or activation of the electrode array particularly
in patients deafened by meningitis is also associated
with poorer speech perception results.23,24 Whereas the
loss of spiral ganglion cells is associated with labyrinthitis ossificans due to meningitis, it is unclear how IMIED
and secondary ossification of the cochlea affect nerve integrity in human cases, although spiral ganglion cell
death is observed in an animal model.25,26 Early CI
should therefore be considered in IMIED patients before
cochlear obstruction limits the ability to achieve optimal
hearing outcomes.
Several limitations of this study should be noted.
First, due to the rarity of this disease, our sample size
was limited. Second, our diagnosis of IMIED relied on
clinical parameters based on a retrospective evaluation
of clinical records from outside facilities, which were not
always complete. A comparison of this cohort with a
group of successfully treated patients with IMIED might
provide additional insight regarding the patient, disease,
and treatment characteristics that predict a successful
outcome, such as timing of therapy. Additional multiinstitutional prospective studies are warranted for such
comparisons.
A better characterization of IMIED subtypes
based on clinical presentation and response to therapy
may reveal important biological variations of the disease and lead to therapeutic innovations. For now,
clinicians are left with limited therapeutic options and
the resolve of the patient–doctor relationship to navigate the medical and psychosocial dimensions of
IMIED. The impetus to win the medical battle at
all costs should be reconsidered in favor of early hearing intervention to regain communication function
using CIs within US Food and Drug Administration
guidelines, while limiting morbidity of prolonged
immunosuppressant therapy.
Malik et al.: Immune-Mediated Inner Ear Disease
2561
CONCLUSION
Categorization of IMIED patients based on type
and presence of systemic autoimmune disease may help
to predict the course of deafness and CI outcomes. Corticosteroids and immunosuppressive interventions remain
the mainstay therapy to slow down the progression of
debilitating hearing deterioration in patients with
IMIED. Early intervention with CI, however, offers the
opportunity to restore hearing function and communication capacity in these patients if pharmacological
therapy fails or cannot be tolerated.
Acknowledgment
The authors thank Stephen P. Bowditch MS, CCC-A
and Mary Z. Rattell for provision and compilation of audiological results for the patients.
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