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The Laryngoscope C 2012 The American Laryngological, V 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 Laryngoscope 122: November 2012 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. BIBLIOGRAPHY 1. McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88:585–589. 2. Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum 2004;34:544–548. 3. Rauch SD. 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Predictors of cochlear implant performance. Audiology 1999; 38:109–116. 23. Francis HW, Buchman CA, Visaya JM, et al. Surgical factors in pediatric cochlear implantation and their early effects on electrode activation and functional outcomes. Otol Neurotol 2008;29:502–508. 24. Rauch SD, Herrmann BS, Davis LA, Nadol JB Jr. Nucleus 22 cochlear implantation results in postmeningitic deafness. Laryngoscope 1997;107: 1606–1609. 25. Ma C, Billings P, Harris JP, Keithley EM. Characterization of an experimentally induced inner ear immune response. Laryngoscope 2000;110: 451–456. 26. Nadol JB Jr. Patterns of neural degeneration in the human cochlea and auditory nerve: implications for cochlear implantation. Otolaryngol Head Neck Surg 1997;117:220–228. Malik et al.: Immune-Mediated Inner Ear Disease