Hearing disability in patients with Fuchs’ endothelial corneal dystrophy: unrecognized co-pathology?

Abstract

Marilette Stehouwer, Ward R Bijlsma, Allegonda Van der LelijDepartment of Ophthalmology, University Medical Center Utrecht, Utrecht, The NetherlandsPurpose: To investigate a possible association between Fuchs’ endothelial corneal dystrophy (FECD) and hearing disability.Methods: A cross-sectional observational study was performed at the University Medical Center Utrecht. Cases and controls were patients who were treated by a cornea specialist between 2004 and 2008. FECD patients had either already undergone or were planned for a keratoplasty procedure. All controls were patients treated for cataract without any corneal pathology. Cases and controls were matched by age group and gender. A structured telephone interview combined with a validated self-perceived hearing function test (the Hearing Handicap Inventory for the Elderly, screening version; HHIE-S) was used for data collection.Results: Hearing disability was reported in 33 patients (45.8%) in the FECD-group (n = 72, average age: 73 years) versus 50 patients (34.7%) in the control group (n = 144, average age: 73 years). Hearing disability was significantly associated with FECD after adjustment for age, noise exposure, and diabetes mellitus (odds ratio 1.97 95% confidence interval 1.04–3.75).Conclusion: This case–control study reports a significant association between FECD and hearing disability. The causal relation was not evaluated in this study, though mutations in the SLC4A11 gene could have played an important role. This gene encodes for an ion transporter, which has been found in the cornea and inner ear. With the lack of examination with a pure tone or speech audiometry in this study, further studies need to be performed in order to support the association and should include a complete ENT examination with audiometry and genetic research.Keywords: hearing disability, hearing impairment, Fuchs’ endothelial corneal dystrophy