OTOGL (otogelin-like) encodes a large secreted protein structurally related to gel-forming mucins 1 that functions as a critical component of inner ear acellular membranes, particularly the tectorial membrane, supporting cochlear amplification 2. OTOGL is transiently expressed in spiral ganglion neuron subpopulations during cochlear development 3 and plays a role in mechanical stimulation of cochlear outer hair cells 3. Biallelic OTOGL variants cause autosomal recessive nonsyndromic hearing loss (DFNB84B), characterized by mild-to-moderate, non-progressive sensorineural hearing impairment with early childhood onset 2. Additionally, OTOGL variants associate with vertigo susceptibility, particularly benign paroxysmal positional vertigo, independent of hearing loss phenotype 4. OTOGL mutations are implicated in Ménière's disease pathogenesis through disruption of extracellular matrix and cell-cell adhesion pathways in inner ear hair cells 5. Clinically, OTOGL-associated hearing loss patients benefit from counseling regarding non-progressive hearing decline, though careful vestibular monitoring for adult-onset symptoms is recommended 2. While neuropathy affecting spiral ganglion neurons processing loud sounds manifests as auditory hyperexcitability 3, OTOGL appears nonessential for spermatogenesis despite expression in developing testes 6.