David A. Fabry

Open trial of methotrexate as treatment for autoimmune hearing loss.

OBJECTIVE To assess the efficacy of low-dose methotrexate (MTX) administered for the treatment of autoimmune hearing loss. METHODS This was a prospective, 12-month, open-label study of 17 patients with refractory autoimmune hearing loss. All patients had ongoing episodic worsening of hearing in one or both ears prior to enrollment despite traditional medical therapy. The MTX dose was 7.5-25 mg/week. Hearing loss and vertigo were evaluated at baseline and at completion of the study. Hearing improvement was defined as an improvement in pure tone threshold (PT) average of >10 dB or an increase in speech discrimination (SD) of >15%; worsening was defined as a decrease of >10 dB in PT or a decrease of >15% in SD in at least one ear. RESULTS MTX was well tolerated. Among patients with Menieres disease, 5 of 9 had improvement or resolution of vertigo. Equilibrium improved in all 3 patients with Cogans syndrome and improved in 2 out of 3 patients with idiopathic hearing loss and this symptom. According to the parameters defined above, hearing improved in 11 patients (65%), was unchanged in 4 patients (23%), and worsened in 2 patients (12%). CONCLUSION Long-term low-dose MTX therapy may be a useful therapy for at least some patients who have hearing loss with a presumptively autoimmune-mediated component that is refractory to traditional therapies.

The utility of positron emission tomography in the evaluation of autoimmune hearing loss

Objective To evaluate positron emission tomography as an imaging tool in the diagnosis, evaluation, and management of autoimmune inner ear disease. Background Autoimmune inner ear disease is a form of cochleovestibular disease associated with variable hearing loss and vertigo for which no reliable diagnostic tests are available. Methods Pilot study of 10 patients with autoimmune inner ear disease and 5 sex-matched and age-matched control subjects without any history of autoimmune inner ear disease, who underwent limited positron emission tomography of the inner ear. Five patients with new or active autoimmune inner ear disease underwent serial positron emission tomography before and after 4 to 6 weeks of a high-dose tapering course of prednisone. The subjects had cranial magnetic resonance imagining, audiometric and vestibular studies, and heat-shock protein (HSP-70) measurements. Reading of the positron emission tomography scans was blinded. Results Positron emission tomography was normal in 4 of 5 normal control subjects and abnormal in 1 with normal audiometric and vestibular studies and positive HSP-70. Of patients with established and stable autoimmune inner ear disease, 4 of 5 had no positron emission tomography abnormalities and negative HSP-70, and the one with abnormal positron emission tomography shortly thereafter manifested clinically active disease. Of the 5 patients with active autoimmune inner ear disease monitored serially, 4 had an initial abnormal positron emission tomography in at least one ear, which became normal in all but 1 patient after therapy. HSP-70 correlated with disease activity. Only 1 patient with clinically active autoimmune inner ear disease had a normal positron emission tomography before and after therapy (the HSP-70 was positive before therapy and negative after the therapy). Conclusions Positron emission tomography, especially when combined with HSP-70 determination, may be a useful technique for assessing disease in patients with autoimmune inner ear disease.

Hearing loss and phacoemulsification.

Purpose: To determine the acoustic spectra of currently used phacoemulsification units and to contrast phacoemulsification‐generated acoustic spectra with representative audiograms of common types of sensorineural hearing loss. Setting: Mayo Clinic, Rochester, Minnesota, USA. Methods: The acoustic spectra of 3 phacoemulsification systems (Alcon Series 20,000 Legacy, Storz Millennium, and AMO Diplomax) were recorded in an acoustically soundproofed room using a Roland VS‐880 Digital Studio Workstation and analyzed with a Hewlett‐Packard 35660A Dynamic Signal Analyzer. Results: Phacoemulsification handpiece‐generated harmonic overtones produced during ultrasound mode (6.0, 12.0, and 18.8 kHz for the 20,000 Legacy and Diplomax; 7.0 and 14.2 kHz for the Millennium) were outside the range of minimal decibel loss in individuals with hearing loss. Supplemental, low‐frequency, console‐generated tones produced during ultrasound mode (0.4 to 2.0 kHz for the Diplomax; 0.1 to 1.5 kHz for the Millennium) were within the range of minimal decibel loss in individuals with hearing loss. Conclusion: Phacoemulsification systems with console‐generated, low‐frequency tones were audible to ophthalmologists with common types of sensorineural hearing loss.