Diagnosis Of Autoimmune Inner Ear Disease Research Articles

Ear Involvement in Inflammatory Bowel Disease: A Review of the Literature.

Inflammatory bowel disease (IBD) is a multisystemic disease. The ear is a rare but recognized site of extraintestinal manifestations of IBD. In external ear, the more common manifestations of IBD are pyoderma gangrenosum, metastatic Crohn’s disease and relapsing polychondritis and the treatment includes corticosteroids and anti-TNF agents. Sensorineural hearing loss (SNHL) is the most common ear disease in IBD and especially in patients with ulcerative colitis. In most cases of IBD patients with SNHL, the hearing loss is attributable to autoimmune inner ear disease (AIED). Diagnosis of AIED is based on clinical presentation, the demonstration of a progressive sensorineural hearing loss in periodic audiological tests, a response to immunosuppressive drugs and exclusion of other causes of SNHL. The only diagnostic test that is available for clinical use is the Otoblot test (Western blot for antibodies against 68 kD protein-inner ear antigens). Initial therapy is usually steroids, with a step up to anti-TNF-a therapy and cochlear implantations with failure of treatment. Furthermore, Cogan’s syndrome, a chronic disease characterized by deafness, vertigo keratitis and aortitis, has been associated with IBD and mainly with Crohn’s disease.

The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls

The purpose of this paper is to review the current diagnostic work-up for patients with suspected Autoimmune Inner Ear Disease (AIED). AIED is a rare disease accounting for less than 1% of all cases of hearing impairment or dizziness, characterized by a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. While specific tests for autoimmunity to the inner ear would be valuable, at the time of writing, there are none that are both commercially available and proven to be useful. Thus far, most of the identified antigens lack a clear association with localized inner ear pathology and the diagnosis of AIED is based either on clinical criteria and/or on a positive response to steroids. For clinical practice, we recommend an antigen-non-specific test battery including blood test for autoimmune disorders and for conditions that resemble autoimmune disorders. Nevertheless, if financial resources are limited, a very restricted work-up study may have a similar efficiency.

Cochlear Implantation in Autoimmune Inner Ear Disease

Objective 1) Evaluate the success of cochlear implantation (CI) in patients with autoimmune inner ear disease (AIED). 2) Discuss the impact of CI on consideration of immunosuppressive therapy for patients with fluctuating hearing loss due to AIED. Methods Retrospective chart review was conducted of patients undergoing CI with diagnosis of AIED between 6/02 and 6/07. Patients’ autoimmune and immunotherapy treatment history was explored. Non-operative ear hearing thresholds and speech discrimination scores (SDS) were documented pre- and postoperatively. Objective and subjective implant success in setting of fluctuating hearing in non-implant ear was evaluated. Results Four patients with AIED, aged 5–55, underwent CI. Two had isolated otologic symptoms and 2 had other systemic autoimmune diagnoses. All had been previously treated with steroids. Three had received other immunosuppressive therapy. One declined other treatment due to potential risks. At the time of implantation, 3 patients had functional hearing in the non-implant ear and received benefit from a hearing aid. Each continued to have usable hearing, with fluctuating improvements post-implant at times to much better SDS than would normally meet candidacy requirements for CI. All patients found the implant provided better hearing than the hearing-aid ear. Conclusions CI is a viable option for patients with AIED. Implanting a profoundly deafened ear where opposite ear has fluctuating but usable hearing does not negatively impact outcomes. With the success of CI for AIED, the potential risks of long-term immunosuppressive therapy may not warrant its use in patients with isolated hearing loss symptoms.

Immune-mediated inner ear disease

The incidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for < 1% of all cases of hearing impairment or dizziness. Nevertheless, the diagnosis of AIED might be overlooked because of the lack of a specific diagnostic test. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss (SNHL) over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25–50% of patients also have tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15–30% of patients.

Relationship Between Three Inner Ear Antigens with Different Molecular Weights and Autoimmune Inner Ear Disease

Crude inner ear antigen (CIEAg) can induce autoimmune inner ear disease (AIED) although it is not known which subcomponent of CIEAg is involved. In this study, we investigated the relationship between 3 purified inner ear antigens (31, 42-45 and 60 kD proteins) and AIED, and determined their distribution in normal guinea pig cochlea. Three groups of guinea pigs were immunized with the three inner ear antigens and one group served as a control. The hearing thresholds, serum IgG level and morphological changes in the inner ear were observed. The expression of the three antigens in the cochlea was detected using immunohistochemical techniques. No obvious changes in hearing thresholds or inner ear morphology were observed between the control and 42-45 kD groups. Animals immunized with the 31 or 60 kD proteins showed a significant increase in hearing thresholds ( p < 0.05 vs control), accompanied by morphological changes in the inner ear. The serum IgG level was increased significantly ( p < 0.05) in all immunized animals. The 31 kD protein was distributed in the cochlear nerve and spiral ganglion, while the 42-45 and 60 kD proteins were distributed widely, being found in the spiral ganglion, organ of Corti, stria vascularis and spiral ligament. These results suggest that two subcomponents of CIEAg (the 31 and 60 kD proteins) may induce AIED independently, that several inner ear antigens may contribute to the pathogenesis of AIED and that the 31 kD protein is of high tissue specificity and may be used as a marker protein for the clinical diagnosis of AIED.