A Mutation in Tmem135 Causes Progressive Sensorineural Hearing Loss

Area of Science:

• Otolaryngology
• Genetics
• Cell Biology

Background:

• Transmembrane protein 135 (TMEM135) is implicated in mitochondrial dynamics, thermogenesis, and lipid metabolism.
• The function of TMEM135 in the inner ear and auditory system remains unexplored.

Approach:

• Investigated TMEM135 function in hearing using wild-type and Tmem135 ^{FUN025/FUN025} mutant mice.
• Utilized auditory brainstem response (ABR) testing, cochlear histology, and BaseScope in situ hybridization.

Key Points:

• Tmem135 ^{FUN025/FUN025} mice developed progressive hearing loss from 3 months to 12 months of age.
• A severe loss of outer hair cells and spiral ganglion neurons was observed in 13-month-old mutant mice.
• TMEM135 expression was detected in inner hair cells, outer hair cells, and supporting cells of the cochlea.

Conclusions:

• The FUN025 mutation in Tmem135 leads to progressive sensorineural hearing loss.
• TMEM135 plays a critical role in preserving cochlear cell integrity and sensory function in aging