Defibrinogenation therapy with batroxobin for idiopathic pediatric sudden sensorineural hearing loss: Report of three cases

Few large-scale prospective studies have examined idiopathic sudden sensorineural hearing loss (SSNHL), which is rare in children. Its pathophysiological characteristics, optimal treatment, prognosis, and other aspects remain unclear. Clinical practice guidelines are non-existent. The medical records of University of Fukui Hospital were analyzed retrospectively during 2006–2021, elucidating details of idiopathic pediatric (under 16 years old) SSNHL. Three cases of idiopathic pediatric SSNHL were identified, which were not caused by known pathogenesis including mumps, meningitis, or hereditary hearing loss. In all cases, systemic steroids (0.3–1.0 mg/kg/day) were administered as the initial treatment within 0–5 days of SSHNL onset. Two cases had progressive hearing loss despite steroid therapy. The other one showed incomplete improvement, whose average improvement was 18 dB of five frequencies (250, 500, 1000, 2000, and 4000 Hz). Defibrinogenation therapy using batroxobin (5 unit/body/day, 2–3 times per week) was added to systemic steroids 8–11 days after SSNHL onset, improving hearing in all cases. Although the low range to middle range of hearing was improved completely with batroxobin therapy, high-range hearing loss remained in two patients who had progressive hearing loss despite steroid treatment (Cases 1 and 2). Complete hearing recovery occurred in Case 3. Because their hearing loss progressed despite systemic steroids and similarly recovered through defibrinogenation therapy, their pathophysiology appears to be similar: possible blood circulation insufficiency in the inner ear. Our report demonstrated the possible use of batroxobin to treat idiopathic pediatric SSNHL, which is sometimes refractory. Although additional findings are necessary, batroxobin can be considered as a treatment option. •Three cases of idiopathic pediatric sudden sensorineural hearing loss are reported.•Their sudden sensorineural hearing loss (SSNHL) was prednisolone-resistant.•Defibrinogenation therapy using batroxobin improved their hearing.•Inner ear blood insufficiency might lead to idiopathic pediatric SSNHL.