Autoimmune Inner Ear Disease (AIED) is caused by one’s own immune system attacking the inner ears. People with AIED experience progressive, bilateral, typically step-wise sensorineural hearing loss as well as ringing in the ears called tinnitus that worsens over a period of weeks to a couple of months. At least half of such patients also describe balance problems. AIED typically begins in one ear but progresses to involve the second ear quickly. This differentiates this disease from idiopathic Sudden Sensorineural Hearing Loss.
The diagnosis of AIED requires (1) a hearing test confirming bilateral, new-onset, progressive sensorineural hearing loss, (2) the appropriate symptom time-course as determined by patient history, and (3) an MRI Brain with Internal Auditory Canal protocol that finds no evidence of retrocochlear mass lesions. Laboratory tests such as Lyme Titers, FTA-abs (syphilis test), and HIV tests could be obtained to rule out treatable causes of inner ear dysfunction, and laboratory markers of an activated immune response such as erythrocyte sedimentation rate (ESR) and C-reactive Protein (CRP) may be ordered.
In many cases, AIED is steroid responsive and early intervention is recommended. Oral steroids at high dose are typically prescribed for at least one month followed by a slow taper. Those who relapse after termination of oral steroids are referred to Rheumatology for a discussion regarding long-term oral steroid use. Intra-tympanic steroids (injected directly into the middle ear space) may be an adjunct or alternative treatment, depending on the particular circumstance. Unfortunately, not all patients are improved or stabilized by use of steroids and some progress to bilateral severe to profound sensorineural hearing loss. Many such patients are excellent candidates for cochlear implantation.
Why Choose Us
Dr. Abraham Jacob, Medical Director for Ear & Hearing (E&H) at the Center for Neurosciences (CNS), is fellowship trained in Otology, Neurotology, and Cranial Base Surgery. He is the first and most experienced Neurotologist in Tucson.Dr. Jacob was a founding member of the University of Arizona (UA) Department of Otolaryngology prior to his departure and transition to CNS. At UA, he was Vice Chair of ENT and held the rank of full Professor with Tenure. He has an international reputation as an expert for treating ear diseases.Dr. Jacob transitioned his practice to the Center for Neurosciences in early 2017 as he felt that the new environment helped him to optimize delivery of personalized ear and lateral skull base care for his patients.Dr. Jacob is currently the only ear, nose, throat (ENT) physician in Tucson able to offer the full range of diagnostic testing and treatment options for Autoimmune Inner Ear Disease. Audiologic testing, laboratory evaluation, MR imaging options, referrals to Rheumatology, oral steroid treatments, intra-tympanic steroids (injected under the ear drum) and cochlear implantation are all available under the comprehensive, multi-disciplinary umbrella of “Ear & Hearing at the Center for Neurosciences.”
Ear & Hearing Appointments
Our Otologist/Neurotologist specializes in the medical and surgical care of patients with disorders of the ear and lateral skull base. Otology/Neurotology and Audiology work together to offer complete hearing health solutions.
To schedule an appointment with our Ear & Hearing department, please call us at (520) 795-7750.
Our Physicians’ Goal
Our physicians’ goal is to provide the highest-quality neurological care– the same level of care we would want for ourselves or our loved ones.
We will help our patients to understand their condition, listen to their concerns, answer their questions, and provide the best clinical treatment available.