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Purpose To report a case series of subjective and objective hearing function changes associated with teprotumumab treatment for thyroid eye disease. Observations A 74-year-old female with a history of Graves' disease with thyroid eye disease was treated with teprotumumab. She had a history of bilateral tinnitus and noticed a subjective improvement in her tinnitus after the second infusion. Audiology testing obtained before, during, and after completion of infusions showed symmetric and rapidly progressive worsening of the patient's sensorineural hearing loss. In contrast, a 42-year-old male with a history of Grave's disease endorsed worsening intermittent tinnitus and low-pitched hearing loss after initiation of teprotumumab. Audiology testing before, during, and after completion of infusions showed stable and normal hearing function bilaterally. Conclusion and importance This case series highlights the importance of objective testing in patients prior to and after teprotumumab initiation as subjective hearing changes may not accurately reflect objective hearing function. In addition, this report suggests that teprotumumab may play a role in potentiating sensorineural hearing loss.

Purpose To study post-interventional findings in patients with dysthyroid optic neuropathy (DON) treated with teprotumumab. Observations In this multicenter observational Case series, patients with DON were treated with teprotumumab, an insulin-like growth factor I receptor inhibitor (10 mg/kg for the first infusion then 20 mg/kg for subsequent infusions, every three weeks for a total 8 infusions). This study included patients with acute and chronic thyroid eye disease (TED) with DON who had failed conventional therapies and were not candidates for surgical decompression. Data collected included best corrected visual acuity (BCVA), color vision, RAPD when present, and orbital CT or MRI. Proptosis, clinical activity score (CAS), Gorman diplopia score (GDS), and Humphrey visual fields (HVF) were also evaluated. Ten patients (6 women, 4 men) with an average age 64 years old were included in this study. Mean follow up after completion of infusions was 15 weeks. Baseline visual acuity (VA) impairment ranged from hand motion (HM) to 20/25 in affected eyes. All patients had pre-treatment orbital CT or MRI that confirmed orbital apex compression. Seventy percent of patients had objective improvement in DON after 2 infusions of teprotumumab measured as significant improvement in visual acuity, resolution of RAPD, or both. After completion of treatment, affected eyes had a mean BCVA improvement of 0.87 logMAR (p=0.0207), proptosis reduction of 4.7 mm (p, Highlights • Teprotumumab is effective for the treatment of dysthyroid optic neuropathy. • Most patients demonstrated rapid objective improvement after 2 infusions. • Visual acuity improved and relative afferent pupillary defect resolved after treatment. • Orbital imaging showed improvement of orbital apex crowding after treatment. • Patients with longstanding severe vision loss had limited improvement in visual acuity.

Purpose To report the first case of a patient with chronic thyroid eye disease (TED) treated with teprotumumab. Observations A 50-year-old female with a 3-year history of Graves’ disease presented with bilateral exophthalmos greatest on the left side. She was followed for 2 years with stable proptosis measurements (23mm OD, 28mm OS). Her clinical activity score (CAS) was 1 and there were no examination findings reflective of active inflammation. The patient underwent systemic treatment with teprotumumab and despite chronic TED and low CAS, she had notable improvement in proptosis (18mm OD, 22mm OS) and decrease in extraocular muscle volume as noted on orbital imaging. Conclusion and importance This case report suggests that teprotumumab may be used in patients with chronic TED and low CAS. Improvement in the proptosis and reduction in extraocular muscle volume suggest that teprotumumab may alter disease course even in patients with inactive or quiescent TED.

Thyroid eye disease (TED) is a potentially sight-threatening and cosmetically disfiguring condition arising in 25–50% of patients with Graves’ hyperthyroidism. CIRTED is the first study to evaluate the long-term role of radiotherapy and prolonged immunosuppression with azathioprine in treating TED, one aim of which was to validate the use of the English version of GO-QOL in an UK population with TED. In a three stage design over a 48 week period, the GO-QOL was tested and compared to a general measure of quality of life (WHOQOL-Bref). In stage 1 utilising a standard 14 day test-retest design both GO-QOL subscales achieved Cronbach’s alphas demonstrating excellent validity and internal reliability (Visual Function 0.929 and 0.931; Appearance 0.888 and 0.906). In stage 2, Repeated Measures ANOVA demonstrated longitudinal validity, with both subscales of the GO-QOL showing significant change over time (Visual Function, η2 = 0.114, p < .001; Appearance, η2 = 0.069, p < .002). In stage 3 the GO-QOL showed discriminant validity at the week 48 time point, with the visual function subscale being able to detect changes in groups identified by clinicians (using BCCOM ratings of improvement or deterioration), while both subscales could detect group differences when based on participants’ subjective ratings of TED noticeability and severity. The results of this project provide support for the English translation of the GO-QOL as an outcome measure for patients with moderately severe active Graves’ orbitopathy/TED