Abstract
Introduction: The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL). Methods: Fifty-three patients with AIHL were retrospectively recruited, and a tapering schema of steroid treatment was administered in all these patients. The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden (sensorineural hearing loss [SSHL]), fluctuating, or quickly progressing (<12 months) SSHL (uni-/bilateral), in association with the coexistence of autoimmune diseases, high antinuclear antibodies (ANA) and the presence of human leukocyte antigen (HLA) B27, B35, B51, C04, and C07. Logistic regression analysis was applied to correlate the clinical data and laboratory features of AIHL with final outcomes. Results: The onset of AIHL was mainly progressive (49%), followed by SSHL (39.6%) or fluctuating (11.3%). The pure-tone audiogram showed more commonly a downsloping pattern (42.6% of ears), but also an upsloping, flat, cookie-bite, or inverse cookie-bite shape. Bilateral progressive AIHL was more frequently simultaneous (23 patients) than heterochronous (4 patients). Nineteen patients (35.8%) showed a favorable response to steroid therapy. The presence of recurrent, bilateral SSHL versus recurrent, unilateral SSHL had statistically negative effect on hearing recovery (OR = 0.042, p < 0.05). The heterochronous bilateral SSHL may have better prognosis than simultaneous bilateral SSHL (OR = 10.000, p = 0.099). The gender, age, concomitant autoimmune disease, high ANA, HLA alleles, tinnitus, and vestibular symptoms had no statistical effect on a favorable outcome of AIHL. Conclusions: A bilateral, simultaneous, and progressive hearing loss combined with downsloping audiogram occurred more often in patients with AIHL. Bilateral simultaneous SSHL with recurrences represents the worse prognostic form of AIHL.
References
1.
Agrup
C
, Luxon
LM
. Immune-mediated inner-ear disorders in neuro-otology
. Curr Opin Neurol
. 2006 Feb
;19
(1
):26
–32
. .2.
Amor-Dorado
JC
, Paco
L
, Martin
J
, Lopez-Nevot
MA
, Gonzalez-Gay
MA
. Human leukocyte antigen-DQB1 and -DRB1 associations in patients with idiopathic sudden sensorineural hearing loss from a defined population of Northwest Spain
. Acta Otolaryngol
. 2005 Dec
;125
(12
):1277
–82
. .3.
Arduc
A
, Isık
S
, Allusoglu
S
, Iriz
A
, Dogan
BA
, Gocer
C
, Evaluation of hearing functions in patients with euthyroid Hashimoto’s thyroiditis
. Endocrine
. 2015 Dec
;50
(3
):708
–14
. .4.
Atturo
F
, Colangeli
R
, Bandiera
G
, Barbara
M
, Monini
S
. Can unilateral, progressive or sudden hearing loss be immune-mediated in origin?
Acta Otolaryngol
. 2017 Aug
;137
(8
):823
–8
. .5.
Bovo
R
, Aimoni
C
, Martini
A
. Immune-mediated inner ear disease
. Acta Otolaryngol
. 2006 Oct
;126
(10
):1012
–21
. .6.
Bovo
R
, Ciorba
A
, Martini
A
. The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls
. Eur Arch Otorhinolaryngol
. 2009 Jan
;266
(1
):37
–40
. .7.
Bowman
CA
, Nelson
RA
. Human leukocytic antigens in autoimmune sensorineural hearing loss
. Laryngoscope
. 1987 Jan
;97
(1
):7
–9
. .8.
Brant
JA
, Eliades
SJ
, Ruckenstein
MJ
. Systematic review of treatments for autoimmune inner ear disease
. Otol Neurotol
. 2015 Dec
;36
(10
):1585
–92
. .9.
Broughton
SS
, Meyerhoff
WE
, Cohen
SB
. Immune-mediated inner ear disease: 10-year experience
. Semin Arthritis Rheum
. 2004 Oct
;34
(2
):544
–8
. .10.
Campbell
KC
, Klemens
JJ
. Sudden hearing loss and autoimmune inner ear disease
. J Am Acad Audiol
. 2000 Jul–Aug
;11
(7
):361
–7
.11.
Ciorba
A
, Corazzi
V
, Bianchini
C
, Aimoni
C
, Pelucchi
S
, Skarżyński
PH
, Autoimmune inner ear disease (AIED): a diagnostic challenge
. Int J Immunopathol Pharmacol
. 2018 Mar‒Dec
;32
:2058738418808680
. .12.
Choi
HG
, Park
KH
, Seo
JH
, Kim
DK
, Yeo
SW
, Park
SN
. Clinical and audiologic characteristics of acute low-tone sensorineural hearing loss: therapeutic response and prognosis
. Korean J Audiol
. 2011
;15
(1
):8
–13
.13.
Das
S
, Bakshi
SS
, Seepana
R
. Demystifying autoimmune inner ear disease
. Eur Arch Otorhinolaryngol
. 2019 Dec
;276
(12
):3267
–74
. .14.
Di Stadio
A
, Ralli
M
. Systemic Lupus Erythematosus and hearing disorders: literature review and meta-analysis of clinical and temporal bone findings
. J Int Med Res
. 2017 Oct
;45
(5
):1470
–80
. .15.
Fetterman
BL
, Luxford
WM
, Saunders
JE
. Sudden bilateral sensorineural hearing loss
. Laryngoscope
. 1996 Nov
;106
(11
):1347
–50
. .16.
García-Berrocal
JR
, Trinidad
A
, Ramírez-Camacho
R
, Lobo
D
, Verdaguer
M
, Ibáñez
A
. Immunologic work-up study for inner ear disorders: looking for a rational strategy
. Acta Otolaryngol
. 2005 Aug
;125
(8
):814
–8
. .17.
García Berrocal
JR
, Lobo
D
, García López
F
, Ramírez-Camacho
R
. [Immunomediated inner ear disease: diagnostic validation by means of a systematic analysis of the scientific literature]
. Acta Otorrinolaringol Esp
. 2007 May
;58
(5
):208
–16
. .18.
Harris
JP
, Sharp
PA
. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss
. Laryngoscope
. 1990 May
;100
(5
):516
–24
. .19.
Hida
K
, Takano
K
, Yoshimitsu
K
, Fukae
J
, Hokao
K
. Inner ear enhancement on gadolinium-enhanced 3D FLAIR images in a patient with Vogt-Koyanagi-Harada disease
. BJR Case Rep
. 2016 Sep 16
;3
(1
):20160090
. .20.
Hwang
M
, Zuccoli
G
. Rapidly destructive autoimmune labyrinthitis associated with ulcerative colitis
. Pediatr Neurol
. 2015 Oct
;53
(4
):375
–6
. .21.
Kulahli
I
, Balci
K
, Koseoglu
E
, Yuce
I
, Cagli
S
, Senturk
M
. Audio-vestibular disturbances in Behcet’s patients: report of 62 cases
. Hear Res
. 2005 May
;203
(1-2
):28
–31
. .22.
Lobo
D
, Tuñón
M
, Villarreal
I
, Brea
B
, García-Berrocal
JR
. Intratympanic gadolinium magnetic resonance imaging supports the role of endolymphatic hydrops in the pathogenesis of immune-mediated inner-ear disease
. J Laryngol Otol
. 2018 Jun
;132
(6
):554
–9
. .23.
Loveman
DM
, de Comarmond
C
, Cepero
R
, Baldwin
DM
. Autoimmune sensorineural hearing loss: clinical course and treatment outcome
. Semin Arthritis Rheum
. 2004 Oct
;34
(2
):538
–43
. .24.
Mata-Castro
N
, García-Chilleron
R
, Gavilanes-Plasencia
J
, Ramírez-Camacho
R
, García-Fernández
A
, García-Berrocal
JR
. Analysis of audiometric relapse-free survival in patients with immune-mediated hearing loss exclusively treated with corticosteroids
. Acta Otorrinolaringol Esp
. 2018 Jul-Aug
;69
(4
):214
–8
. .25.
McCabe
BF
. Autoimmune sensorineural hearing loss
. Ann Otol Rhinol Laryngol
. 1979 Sep-Oct
;88
(5 Pt 1
):585
–9
. .26.
Mijovic
T
, Zeitouni
A
, Colmegna
I
. Autoimmune sensorineural hearing loss: the otology-rheumatology interface
. Rheumatology
. 2013 May
;52
(5
):780
–9
. .27.
Monsanto
RC
, Neto
AC
, Lorenzetti
FT
. Reiter’s syndrome and hearing loss: a possible association?
Clin Case Rep
. 2014 Dec
;2
(6
):310
–2
. .28.
Ralli
M
, D’Aguanno
V
, Di Stadio
A
, De Virgilio
A
, Croce
A
, Longo
L
, Audiovestibular symptoms in systemic autoimmune diseases
. J Immunol Res
. 2018 Aug 19
;2018
:5798103
. .29.
Rauch
SD
. Clinical practice. Idiopathic sudden sensorineural hearing loss
. N Engl J Med
. 2008 Aug 21
;359
(8
):833
–40
. .30.
Rossini
BAA
, Penido
NO
, Munhoz
MSL
, Bogaz
EA
, Curi
RS
. Sudden sensorioneural hearing loss and autoimmune systemic diseases
. Int Arch Otorhinolaryngol
. 2017 Jul
;21
(3
):213
–23
. .31.
Wong
AC
, Ryan
AF
. Mechanisms of sensorineural cell damage, death and survival in the cochlea
. Front Aging Neurosci
. 2015 Apr 21
;7
:58
. .32.
Wu
CM
, Lee
KJ
, Chang
SL
, Weng
SF
, Lin
YS
. Recurrence of idiopathic sudden sensorineural hearing loss: a retrospective cohort study
. Otol Neurotol
. 2014 Dec
;35
(10
):1736
–41
. .33.
Xenellis
J
, Nikolopoulos
TP
, Stavroulaki
P
, Marangoudakis
P
, Androulakis
M
, Tsangaroulakis
M
, Simultaneous and sequential bilateral sudden sensorineural hearing loss: are they different from unilateral sudden sensorineural hearing loss?
ORL J Otorhinolaryngol Relat Spec
. 2007
;69
(5
):306
–10
. .34.
Yeom
K
, Gray
J
, Nair
TS
, Arts
HA
, Telian
SA
, Disher
MJ
, Antibodies to HSP-70 in normal donors and autoimmune hearing loss patients
. Laryngoscope
. 2003 Oct
;113
(10
):1770
–6
. .35.
Young
YH
. Contemporary review of the causes and differential diagnosis of sudden sensorineural hearing loss
. Int J Audiol
. 2020 Apr
;59
(4
):243
–53
. .36.
Zeitoun
H
, Beckman
JG
, Arts
HA
, Lansford
CD
, Lee
DS
, El-Kashlan
HK
, Corticosteroid response and supporting cell antibody in autoimmune hearing loss
. Arch Otolaryngol Head Neck Surg
. 2005 Aug
;131
(8
):665
–72
. .37.
Ziu
T
, Ajvazi
H
. Management of patient with Cogan’s syndrome: a case report
. Oftalmologia
. 2010
;54
(4
):73
–5
.38.
Zorzetto
M
, Ricevuti
G
, Martinetti
M
, Gritti
D
, Gasparetto
C
, De Silvestri
A
, HLA and hypocomplementemia: the disadvantage of carrying the HLA-B35 and the silent alleles of the C4 complement component
. Int J Immunopathol Pharmacol
. 2004 Sep‒Dec
;17
(3
):307
–16
. .© 2021 S. Karger AG, Basel
2021
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.
