To determine the levels of endogenous α1-antitrypsin in the perilymph of patients undergoing cochlear implant (CI), and its reverse association with the severity of hearing loss. Retrospective study. Tertiary care university hospital. The study includes 38 patients undergoing CI surgery, 11 patients diagnosed with congenital deafness and 27 non-congenital deafness, eight patients diagnosed with moderate hearing loss (N=8; PTA=70dB), severe hearing loss (N=11; PTA 70-90dB) and profound hearing loss (N=19; PTA>90dB). 1 to 12μL perilymphatic fluids were collected by micropipette. α1-antitrypsin levels were determined, and current and historic audiological parameters were obtained. The congenital and non-congenital group exhibited AAT concentrations of 2.5±1.9×106 LFQ and 3.2±1.2×106 LFQ, respectively (mean±SD; P=.38). Mean levels of α1-antitrypsin in the perilymph fluid within the moderate group was 3.64×106 ±2.1×106 LFQ vs 3.5×106 ±1.2×106 in severe hearing loss (P=.81) and 2.4×106 ±1.1×106 LFQ in the profound hearings loss group (P=.06). The difference in levels of AAT in samples from the severe hearings loss group vs the profound hearings loss group reached statistical significance (P=.04). Insufficiency in α1-antitrypsin levels in the perilymph fluid of the inner ear appears to display a relationship with the severity of hearing loss. The prospect of introducing clinical-grade plasma-purified α1-antitrypsin directly onto the site of cochlear injury deserves thorough investigation.