Abstract
Purpose :
Teprotumumab is a novel IGF-1R inhibitor used to treat thyroid eye disease (TED). Hearing loss is a known adverse event, with an incidence of 10% in the clinical trials. However, recent reports suggest that the incidence of otologic symptoms, including hearing loss, may be up to 81%. To date, an effective protocol for managing hearing loss in this setting has not been determined. Here we present the first report of the successful treatment of teprotumumab-related hearing loss with prompt oral prednisone.
Methods :
Case report with pre- and post-steroid therapy audiometric data.
Results :
A 70-year-old woman with a 1-year history of Graves’ disease controlled with methimazole presented with a 6-month history of active, moderate-to-severe TED. Initial treatment with oral steroids was ineffective. Five months later, teprotumumab was initiated due to worsening diplopia and a clinical activity score of 4. Ten days after teprotumumab initiation, the patient reported sudden hearing loss and tinnitus in the right ear. Audiogram demonstrated a mild down-sloping to moderately severe mixed conductive and sensorineural hearing loss with a pure tone average (PTA) of 33 dB HL (the conductive component was minimal and ranged from 10-15 dB). Hearing was normal in the left ear (Figure 1). She had no history of prior hearing problems, ototoxic medication use, exposure to loud noise, or infectious symptoms. Two days after hearing loss onset, she was treated with prednisone 60mg for 6 days with a 1-week gradual taper. Three weeks following steroid therapy a repeat audiogram demonstrated PTA improvement of 20 dB HL in her right ear which brought her to a normal level that was symmetric with her left ear (Figure 2). Word recognition scores improved from 92% at 70dB before steroid therapy to 100% at 55dB post-treatment. After improvement in hearing symptoms, the patient completed the 8-infusion treatment with no further hearing symptoms at 52-week follow-up.
Conclusions :
Hearing loss is a concerning adverse event of teprotumumab therapy. Effective management of teprotumumab-related hearing loss should include risk factor assessment, patient education, baseline audiometric testing, early identification of hearing symptoms with repeat audiogram testing. If hearing loss is detected, prompt oral prednisone burst therapy should be considered.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.