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Unilateral sensorineural hearing loss as immune-­‐mediated inner ear disease S.Monini, R.Colangeli, F.A1uro, C.Filippi, M.Barbara NESMOS Department, Sapienza University, ENT Clinic Rome, Italy INTRODUCTION Immune-­‐mediated inner ear disease (IMIED) is a syndrome of bilateral sensorineural hearing loss (SNHL) characterized by rapid progression over days to months (1), that is caused probably by specific or aspecific anLbodies or immune cells which are a1acking the inner ear (2). Hearing loss involves mostly both ears (80%), with symmetric or asymmetric auditory thresholds (3,4). IMIED is diagnosed aUer clinical suspicion and response to corLcosteroids (3). Therefore the most raLonal strategy for a correct diagnosis of IMIED should consider, in addiLon to the typology of SNHL, differenLal diagnosis and immunosuppression response, also the results of some immunological laboratory tests, that can be specific (OTOblot ®) and aspecific (5,6,7). The aim of our study was to invesLgate the posiLvity of aspecific immunological tests not only in paLents suffering from bilateral SNHL, but also in paLents suffering from progressive or sudden unilateral SNHL. MATERIALS AND METHODS A prospecLve case series study was performed. PaLents with Menière’s disease, retrocochlear pathologies or aged over 65 years were excluded. All the paLents have undergone pure tone audiometry and the following ba1ery of blood exams to evaluate the immunological response: ANA, ENA screening, anL-­‐thyroperoxidase (anL-­‐TPO), anL-­‐thyroglobulin and anLbody against smooth muscle (ASMA). Clinically, the paLents were divided into two groups: the first one, Group A, with bilateral hearing loss and the other one (Group B), with unilateral hearing loss. The incidence of aspecific anLbody posiLvity was evaluated globally, in individual groups and also in the different hearing loss evoluLon. A scale has been assigned to the autoanLbodies with higher posiLvity, ANA and ASMA: 1 for slight posiLvity, 2 for mild posiLvity and 3 for high posiLvity (Fig.3B). A staLsLcal two pair sample test (t-­‐test) has been applied for the evaluaLon of staLsLcal difference between ANA and ASMA posiLvity scores of the two Groups A and B. The results were compared with a control group, Group C, composed of 35 normal subjects. The incidence of systemic autoimmune disease has been evaluated in Group A and Group B: the correlaLon between systemic autoimmune disease and aspecific autoanLbody posiLvity or negaLvity was analyzed. RESULTS 72 paLents were observed at our Clinic because suffering from suspected IMIED. They were 24 males and 48 females, with a mean age of 46,8 years (min 14, max 72). In total, the aspecific immunological tests resulted posiLve in 48 paLents (66,67%). In group A (n.39), 26 paLents resulted posiLve for aspecific immunological tests (66,7%) (Figure 1): 80,8% was affected by symmetric SNHL and 19,2% by asymmetric SHNL. 13 paLents (33,3%) resulted negaLve for aspecific immunological tests, 61,5% affected by symmetric SNHL and 38,5% by asymmetric SNHL (Figure 1). In Group A1, 72,4% of paLents resulted posiLve for aspecific immunological tests and 27,6% were negaLve. In Group A2, 50% resulted posiLve and 50% negaLve (Figure 2A). In group B, aspecific tests were posiLve in 66,7% of paLents and negaLve in 33,3% of paLents (Figure 1). In Group B, among the 22 paLents affected by sudden SNHL (Group B1) 59,1% resulted posiLve for aspecific immunological tests, and 40,9% resulted negaLve; among the 11 paLents affected by progressive SNHL, the 81,8% resulted posiLve for aspecific immunological tests, while the 18,2% resulted negaLve (Figure 2B). Considering together the bilateral progressive and unilateral progressive SNHL (39+11 paLents), a 70% of posiLvity of immunological tests has been found. ANA resulted posiLve in 30,7% of Group A and 33,3% of Group B; ENA resulted posiLve in 12,8% of Group A and 3% of Group B, AnL smooth-­‐muscle resulted posiLve in 23,1% of Group A and 27,3% of Group B; AnL-­‐thyroglobulin anLbodies resulted posiLve in 10,2 of Group A and 24,2% of Group B; AnL-­‐thyroid peroxidase (anL-­‐TPO) anLbodies resulted posiLve in 18% of Group A and 24,2% of Group B. In Group C 17,14% (6) ANA posiLvity was found in 17,14 % of the paLents, while ASMA resulted always negaLve. In regard to the anLbodies Lters scores of ANA and ASMA, significaLve staLsLcal differences have not been assessed between Group A and B, for both parametric and non-­‐parametric tests (Figure 3A). Among Group A paLents with posiLvity for aspecific auto-­‐anLbodies (n.26), 3 (11,5%) had systemic autoimmune disease associated to thyroid disease, 7 (26,9%) had only systemic autoimmune disease and 3 (11,5%) had only thyroid disease. Among Class A paLents with negaLvity for aspecific auto-­‐anLbodies (n.13), nobody had systemic autoimmune disease associated to thyroid disease, 2 (15.4%) had only systemic autoimmune disease and nobody had only thyroid disease. Among Class B paLents with posiLvity for aspecific auto-­‐anLbodies (n.22), 1 (4.5%) had systemic autoimmune disease associated to thyroid disease, 2 (9%) had only systemic autoimmune disease and 7 (31.8%) had only thyroid disease. Among Class B paLents with negaLvity for aspecific auto-­‐anLbodies (n.11), nobody had systemic autoimmune disease associated to thyroid disease, 1 (9%) had only systemic autoimmune disease and nobody had only thyroid disease (Figure 4). As far as the correlaLon between thyroid pathology and posiLvity for anL-­‐thyreoglobulin and anL-­‐TPO, Group B showed this correlaLon in 88,9% of paLents with high value of autoanLbodies, while Group A in 66,7% of paLents with inferior values. DISCUSSION AND CONCLUSIONS Fig.1 Fig.2A % of distribu0on of ANA and ASMA scores (Control Group C ANA: 17,14%; ASMA: 0%) ANA and ASMA Scores referred to plasma0c 0tra0on ANA Titer Posi0vity Score >1:80<1:120 Slight 1 >1:120<1:160 Mild 2 >1:160 High 3 ASMA Titer Posi0vity Score >1:40 Slight 1 >1:80 Mild 2 >1:100 High 3 Fig.3A Fig.3B Correla0ons between the posi0vity and nega0vity for an0bodies and systemic autoimmune diseases. Aspecific tests posiLvity in suspected IMIED showed to be high (66,67%) in unilateral and bilateral groups: ANA e ASMA Lters and distribuLons resulted similar. IMIED can be unilateral or bilateral. The detecLon of serological non specific inner ear autoanLbodies may be useful to suggest a non-­‐organ-­‐specific autoimmune disorder, not only in bilateral SNHL but also in unilateral type, especially in the progressive form. Although there is no consensus about the use of diagnosLc tools to correctly idenLfy an IMIED, an immunological evaluaLon of paLents would be helpful in view of a therapeuLc approach, eventually based on steroids for primary and recurrent disease. REFERENCES Fig.2B 1. McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88(5pt1):585-­‐589. 2. Harris JP, Sharp PA. Inner ear auto-­‐anLbodies in paLents with rapidly progressive sensorineural hearing loss. Laryngoscope 1990;100:516–24. 3. Bovo R, Ciorba A, MarLni A. The diagnosis of autoimmune inner ear disease: evidence and criLcal piialls. Eur Arch Otorhinolaryngol 2009;266:37-­‐40. 4.  Broughton SS, Meyerhoff WE, Cohen SB. Immune-­‐mediated inner ear disease: 10-­‐year experience. Semin ArthriLs Rheum. 2004 Oct;34(2):544-­‐8. 5. Yeom K, Gray J, Nair TS, Arts HA, Telian SA, Disher MJ, El-­‐Kashlan H, Sataloff RT, Fisher SG, Carey TE. AnLbodies to HSP-­‐70 in normal donors and autoimmune hearing loss paLents. Laryngoscope 2003; 113:1770-­‐1776. 6. Go1schlich S, Billings PB, Keithley Em, Weisman MH, Harris JP. Assessment of serum anLbodies in paLents with progressive sensorineural hearing loss and Meniere’s disease. Laryngoscope 1995; 105: 1347-­‐1352 7. Garcia-­‐Berrocal JR, Trinidad A, Ramirez-­‐Camacho R. Immunologic work-­‐up study for inner ear disorders: Looking for a raLonal strategy. Acta Oto-­‐Laryngologica, 2005; 125: 814-­‐818 Fig.4