Frequency and Patterns of Hearing Dysfunction in Patients Treated with Teprotumumab.

PURPOSE: To better characterize the frequency and patterns of hearing dysfunction in patients who have received teprotumumab to treat thyroid eye disease. DESIGN: Noncomparative case series. PARTICIPANTS: Patients who underwent audiology testing before and after completion of teprotumumab infusions. METHODS: A review of patients who underwent audiology testing before and after completion of teprotumumab infusions was carried out. Additional audiogram testing during treatment was included when available. Hearing function was analyzed using audiogram data measuring threshold hearing levels at specific frequencies. Basic demographic data as well as information regarding otologic symptoms also were obtained and analyzed. MAIN OUTCOME MEASURES: Hearing loss demonstrated by a significant change in decibel hearing thresholds or that meets criteria for ototoxicity. RESULTS: Twenty-two patients (44 ears) were included in the study, with baseline and most recent audiology testing after treatment ranging from 84 days before to 496 days after treatment. Fifteen patients (30 ears) also underwent testing during treatment starting after the second infusion up until the day of, but before, the eighth infusion. Hearing loss after treatment met criteria for ototoxicity in 17 of the 44 ears (38.6%), with 11 of the 22 patients (50.0%) meeting criteria in at least 1 ear. The pure-tone average decibel hearing levels (HLs) across all 44 ears demonstrated hearing loss after treatment (P = 0.0029), specifically at high (P = 0.0008) and middle frequencies (P = 0.0042), but not at low frequencies (P = 0.8344). Patients who were older also were more likely to experience hearing loss after treatment (P = 0.0048). CONCLUSIONS: Audiometric data demonstrate that teprotumumab influences hearing function, most significantly at higher frequencies and in older patients. Audiometric testing is critical for counseling patients regarding teprotumumab treatment. A protocol for monitoring hearing during treatment is needed to detect and manage hearing changes associated with teprotumumab use. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Elevated Intraocular Pressure in Periorbital Sweet's Syndrome.

A 66-year-old immunocompromised man presented with cellulitis around the left eye that was initially concerning for necrotizing fasciitis. Exam findings were remarkable for exquisite periocular tenderness with rigid, immobile eyelids resulting from severe erythema, edema, and induration. Given the concern for orbital compartment syndrome and a necrotizing infection, the patient was taken urgently to the operating room for debridement of the eyelid skin as well as an urgent lateral canthotomy and cantholysis. His eye exam revealed 360° of hemorrhagic chemosis, no relative afferent pupillary defect, and an ipsilateral elevated intraocular pressure of 35 mm Hg. No visual acuity measurement could be obtained secondary to the patient's altered mental status. His intraocular pressure normalized after treatment with antihypertensive drops and further extension of the canthotomy. Histopathological analysis showed extensive neutrophilic infiltrate of the dermis which was compatible with a diagnosis of Sweet's syndrome.

Adult Versus Pediatric Relapse and Recurrence in Orbital Inflammatory Syndrome.

PURPOSE: Orbital inflammatory syndrome (OIS) is a diagnosis of exclusion that has a variable presentation and unpredictable course. Many studies report incomplete or lack of OIS resolution with high recurrence and relapse rates. No studies to date have investigated the characteristics of both recurrence and relapse in OIS. We sought to determine this in both pediatric and adult patients. METHODS: A retrospective chart review of 56 patients with OIS was performed between 2004 and 2018. Forty-one patients were identified as adults greater than 18 years of age and 15 were identified as pediatric patients less than 18 years of age. RESULTS: Among 56 (41 adult and 15 pediatric) cases of OIS, 18 cases of recurrent disease (32.1%) were identified and 15 (26.8%) patients experienced relapses. All 6 (100%) pediatric patients that had recurrent disease initially suffered from relapses. In contrast, only 1 of the 12 (8.3%) recurrent adult cases initially experienced relapse. Of the 18 patients with recurrent disease, 9 (50%) had multiple recurrences. Underlying etiologies were confirmed in 5 of 18 recurrent cases (27.8%) and 5 of 38 (13.2%) non-recurrent cases. Of the 5 patients with recurrent OIS and an identified etiology, all 5 (100%) demonstrated multiple recurrences. CONCLUSIONS: In pediatric cases, relapse was more common and prior episodes of relapse were predictive of later recurrence. Recurrence was relatively common in both groups with half of the patients having multiple recurrences. Identifiable underlying etiologies were more common in patients with recurrent OIS and those cases all demonstrated multiple recurrences.