Endogenous α1-antitrypsin levels in the perilymphatic fluid correlates with severity of hearing loss

Clin Otolaryngol. 2020 Jul;45(4):495-499. doi: 10.1111/coa.13541. Epub 2020 Apr 26.

Abstract

Objectives: 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.

Study design: Retrospective study.

Setting: Tertiary care university hospital.

Participants: 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 = 70 dB), severe hearing loss (N = 11; PTA 70-90 dB) and profound hearing loss (N = 19; PTA > 90 dB).

Main outcome and measure: 1 to 12 μL perilymphatic fluids were collected by micropipette. α1-antitrypsin levels were determined, and current and historic audiological parameters were obtained.

Results: 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).

Conclusion: 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.

Keywords: autoinflammation; congenital deafness; hearing loss; perilymph; α1-antitrypsin.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Female
  • Hearing Loss / congenital
  • Hearing Loss / surgery*
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Peptide Fragments / metabolism*
  • Perilymph / chemistry*
  • Retrospective Studies
  • Severity of Illness Index
  • alpha 1-Antitrypsin / metabolism*

Substances

  • C105Y peptide
  • Peptide Fragments
  • alpha 1-Antitrypsin