Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58 kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing’s syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68 kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss.
from Auris Nasus Larynx
Intratympanic administration of methylprednisolone reduces impact of experimental intensive impulse noise trauma on hearing
Conclusion. The present findings demonstrated that intratympanic methylprednisolone (MP) reduces the impact of impulse noise trauma on hearing and in part preserves the hair cells from death 1 h after exposure to intensive impulse noise. Objective. To examine the treatment efficiency of intratympanic MP (IT-MP) with different methods of administration on cochlear injury induced by exposure to intensive impulse noise. Materials and methods. Fifty-five guinea pigs were assigned into six groups and exposed to intensive impulse noise, i.e. 60 impulses at 165 dB SPL peak pressure with 0.5 ms duration and 2 s intervals. The auditory brainstem response (ABR) was used to examine the hearing thresholds. Cochlear morphology was examined to estimate the inner and outer hair cell loss induced by impulse noise exposure. MP was applied as a rescue agent via different modalities of administration. Results. The ABR threshold value of IT-MP1 or IT-MP4 groups significantly decreased at 4 weeks as compared with the IT-NS (IT 0.9% physiological saline) group. The ABR threshold value of the group that received intramuscular administration of MP (IM-MP) also decreased at 4 weeks as compared with the IT-NS group. Significant hair cell loss was observed at the region 40-50% from the apex in the present model. Within this region, the residual hair cell number in the IT-MP1 or IT-MP4 groups was significantly greater than that in the IT-NS group.
1. Determine whether corticosteroid therapy improves hearing thresholds of patients with enlarged vestibular aqueduct (EVA) anomaly. 2. Determine sample size for a future prospective study.
Retrospective chart review hearing loss in EVA patients comparing patients treated with corticosteroids and untreated patients.
Eighty percent (n = 5) of patients treated with steroids for hearing loss demonstrated audiometric improvement, compared to 14.3% of patients (n = 7) not treated. The pure tone average improved by 17.7 dB in the steroid treated group. A prospective, placebo-controlled trial would need between 19 and 45 patients in each group, treatment versus no-treatment, to achieve statistical significance.
Patients with EVA who develop hearing loss have a high rate of hearing improvement when treated with corticosteroid therapy. The hearing improvement appears to be better than spontaneous recovery.