PP1602 - Dysfibrinogenemia: A Key Component in Fluctuating Sensorineural Hearing Loss

This poster presents a case of fluctuating, bilateral sensorineural hearing loss (SSNHL) eventually attributed to Dysfibrinogenemia. Fluctuating hearing loss often occurs along with several other symptoms including aural fullness, vertigo and tinnitus. These symptoms typically occur during an attack and subside when the hearing improves or returns to normal. Meniere’s disease is the hallmark diagnosis considered in those cases. The purpose of this poster is to discuss an interesting case of a 22 year old female subject presenting with bilateral fluctuating sensorineural hearing loss with hearing that returns to baseline between episodes for several months.

Summary:

A number of etiologies of fluctuating sensorineural hearing loss have been proposed but the association of fluctuating hearing loss and the diagnosis of Dysfibrinogenemia has been minimally investigated. Sudden sensorineural hearing loss is defined as sensorineural hearing loss of 30dB or greater over at least three frequencies within 72 hours. Several otologic conditions can present with sudden or fluctuating hearing loss but typically correlate with other symptoms such as dizziness/vertigo, fullness, tinnitus, etc. Dysfibrinogenemia is a clotting disorder resulting from an abnormal or decreased level of fibrinogen, a protein produced by the liver that helps form blood clots and control bleeding. This poster will review a case study of a 22yo female presenting with unexplained isolated bilateral fluctuating hearing loss who failed all usual therapeutics and continued to experience those symptoms over a period of two years. She was eventually diagnosed with Dysfibrinogenemia.

The patient’s audiograms documenting fluctuations in hearing from 2021 to 2023 will be included and reviewed. The hearing fluctuations are bilateral and do not follow a specific pattern consistent with other disorders such as Meniere’s Disease. While there is research regarding the relationship of fibrinogen with hearing prognosis following idiopathic sudden sensorineural hearing loss, fibrinogen disorders were never investigated regarding sudden and fluctuating SNHL. The patient eventually developed petechial rash and was diagnosed with dysfibrinogenemia which is thought to be the cause of her otologic presentation. The hearing loss is isolated, and the patient has failed Meniere’s disease treatment, migraine treatment, hormonal treatment as well as plasmapheresis. Future research is necessary in regarding to the association of Dysfibrinogemia  and other clotting disorders with some of the most challenging otologic conditions (Idiopathic sudden sensorineural hearing loss and fluctuating hearing loss).