Three cases of pediatric Gorham's disease with hearing loss

Abstract

We experienced three cases of Gorham's disease, a rare and difficult-to-diagnose condition that causes progressive bone destruction throughout the body. In all three cases, temporal bone destruction was observed, and the hearing threshold was raised; furthermore, in one case, spinal fluid leakage was observed, requiring spinal fluid leakage repair. In one case, the patient required interferon and propranolol to repair the cerebrospinal fluid leak; surgery was considered difficult, so the patient continued to be followed without any worsening of hearing. Most patients with Gorham's disease who have been diagnosed and followed are referred to an ear-nose-throat (ENT) clinic after their hearing has already deteriorated, so regular follow-up of the hearing may provide an early indication of the extent of the disease before it becomes symptomatic. If Gorham's disease is diagnosed, it is important to refer the patient to an ENT clinic for regular temporal bone computed tomography and hearing tests.