Middle ear myoclonus associated with forced eyelid closure in children: diagnosis and treatment outcome.

OBJECTIVES/HYPOTHESIS: Forceful eyelid closure syndrome (FECS) was first reported at the Proceedings of the Second International Tinnitus Seminar in 1983. The main symptom of this syndrome is a spontaneous muscular tinnitus related only to forced eye closure, specifically the voluntary contraction of the periorbital muscles. Although investigation of the syndrome was initiated >100 years ago, only four cases have been published in the past 20 years. We report six cases of middle ear myoclonus tinnitus diagnosed as FECS in children and discuss issues surrounding the diagnosis and treatment of this syndrome.

STUDY DESIGN

Retrospective case series.

METHODS

From 2009 to 2011, six children complaining of clicking or crackling sounds in their ears presented at Seoul St. Mary's Hospital. Endoscopic examination and recording of the tympanic membrane were performed while the patients were asked to close their eyes forcefully. Audiologic studies including acoustic reflex decay and static compliance were performed for documentation of the movement of the tympanic membrane. Triggering factors of FECS in the children were carefully evaluated.

RESULTS

Synchronous movement of the tympanic membrane in response to forced eye closure on endoscopic examination was the most reliable finding to diagnose FECS. Acoustic reflex decay and other impedance audiogram findings showed irregular perturbations during forced eye closure, which led to diagnosis of the tinnitus as middle ear myoclonus. Most of the patients had triggering factors for FECS. Reassurance and removal of the triggering or causal factors with or without medication improved clicking sounds coming from middle ear myoclonus.

CONCLUSIONS

FECS is a rare clinical entity and can be easily missed in routine clinical examination. We suggest that patients, especially children, with clicking or crackling tinnitus should be evaluated for FECS using proper diagnostic tools. A possible mechanism of FECS in children postulated from our case review is suggested.