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Cyclic nucleotide phosphodiesterases (PDEs) refer to a class of enzymes that degrade the phosphodiester bonds in cAMP and cGMP secondary messenger molecules (Tetsi et al., 2017; Weber et al., 2017). Due to their ability to regulate signal transduction, PDEs are vital in the modulation of biological signaling pathways in sensory systems including auditory and visual systems. The importance of PDEs in maintaining homeostatic environments in both the inner ear and the eye is highlighted by the fact that mutations in PDEs have been associated with various phenotypic abnormalities, including sensorineural hearing loss (SNHL), vision disorders, and other related syndromes (Bayés et al., 1995; Saga et al., 1998; Tetsi et al., 2017). PDEs affect many biological systems (Fusco and Paldino, 2017), and PDE genetic mutations have been associated with the pathogenesis of several conditions, including sudden sensorineural hearing loss (SSNHL), retinitis pigmentosa (RP), and Usher Syndrome. While more research must be conducted and it is likely that the mechanisms surrounding SSNHL are complex, current data and several studies strongly supports the notion that PDE specifically PDE4D plays a role in causing SSNHL. PDE4D is expressed in the inner ear (Degerman et al., 2017) potentially playing a role in maintaining cochlear homeostasis and hearing function. In addition, PDE4Ds have been associated with atherosclerosis, which is a risk factor for SSNHL (Fischer et al., 2015; Rajati et al., 2016). While PDE4Ds are proposed as key players in SSNHL, PDE6 genes most likely play a role in the development of RP. PDE6 is an important component of visual phototransduction cascade (Majumder et al., 2015; Lagman et al., 2016) and therefore, low levels of PDE6 due to mutations can lead to rod-cone degeneration. This article will discuss the association of SSNHL, RP and Usher syndrome with PDE mutations and seek to elucidate the potential for common treatment modalities in such syndromes due to their shared etiologies.

An Update on Phosphodiesterase Mutations Underlying Genetic Etiology of Hearing Loss and Retinitis Pigmentosa