Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease

ABSTRACT. We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted diffusion in the diffusion-weighted imaging and hypointense areas corresponding to the apparent diffusion coefficient in the cerebral cortex. The autopsy revealed positive immunohistochemistry for the PrPSc protein. Creutzfeldt–Jakob disease presenting with hearing loss is unusual.

Ototoxicity and Teprotumumab

Objectives: Teprotumumab, a novel monoclonal antibody, targets the insulin-like growth factor 1 (IGF-1) receptor. IGF-1 receptors, found in muscle and fat adjacent to the eye and implicated in Graves Ophthalmopathy, are also in the cochlea. In clinical trials, 5 participants reported self-limited audiologic symptoms but there are no objective data in the literature. The aim of this report is to describe one of the first known cases of teprotumumab-induced irreversible sensorineural hearing loss. Methods: Case report at a tertiary referral center Results: A 61 year old female with Graves ophthalmopathy presented with bilateral hearing loss, sound distortion, and tinnitus following treatment with teprotumumab. Audiogram showed mild sloping to moderately-severe sensorineural hearing loss. Repeat audiometry obtained 4 months after cessation of teprotumumab and treatment with oral corticosteroids was unchanged. Conclusions: This is one of the first descriptive cases of ototoxicity resulting in irreversible sensorineural hearing loss in the setting of treatment with teprotumumab. Periodic audiologic evaluations should be recommended to patients on teprotumumab.

Detection of unknown ototoxic adverse drug reactions: an electronic healthcare record-based longitudinal nationwide cohort analysis

Ototoxic medications can lead to significant morbidity. Thus, pre-marketing clinical trials have assessed new drugs that have ototoxic potential. Nevertheless, several ototoxic side effects of drugs may remain undetected. Hence, we sought to retrospectively investigate the potential risk of ototoxic adverse drug reactions among commonly used drugs via a longitudinal cohort study. An electronic health records-based data analysis with a propensity-matched comparator group was carried out. This study was conducted using the MetaNurse algorithm for standard nursing statements on electronic healthcare records and the National Sample Cohort obtained from the South Korea National Health Insurance Service. Five target drugs capable of causing ototoxic adverse drug reactions were identified using MetaNurse; two drugs were excluded after database-based analysis because of the absence of bilateral hearing loss events in patients. Survival analysis, log-rank test, and Cox proportional hazards regression models were used to calculate the incidence, survival rate, and hazard ratio of bilateral hearing loss among patients who were prescribed candidate ototoxic drugs. The adjusted hazard ratio of bilateral hearing loss was 1.31 (1.03–1.68), 2.20 (1.05–4.60), and 2.26 (1.18–4.33) in cimetidine, hydroxyzine, and sucralfate users, respectively. Our results indicated that hydroxyzine and sucralfate may cause ototoxic adverse drug reactions in patients. Thus, clinicians should consider avoiding co-administration of these drugs with other well-confirmed ototoxic drugs should be emphasized.