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Review
June 25, 2020

Hearing Preservation in Patients Who Undergo Labyrinthectomy and Translabyrinthine Procedures: A Case Report and Systematic Review

Author Affiliations
  • 1Department of Otolaryngology–Head and Neck Surgery, Medical University of South Carolina, Charleston
JAMA Otolaryngol Head Neck Surg. Published online June 25, 2020. doi:10.1001/jamaoto.2020.1292
Key Points

Question  Does disruption of the vestibule of the inner ear always lead to hearing loss?

Findings  In this systematic review of 10 studies and 1 case report, hearing was preserved after labyrinthectomy for Meniere disease, cholesteatoma, or vestibular schwannoma.

Meaning  This finding appears to contradict the principle that preservation of a fluid-filled vestibule is essential to hearing preservation; it suggests that an occlusion of the ductus reuniens with granulation tissue or bone dust may be sufficient for preserving hearing after labyrinthine destruction.

Abstract

Importance  Preservation of endolymphatic fluids, maintenance of a fluid-filled vestibule, and preservation of the cochlear nerve and its vasculature are believed to be necessary to retaining hearing after an inner-ear operation. However, some studies have reported no hearing loss despite the violation of the vestibule, questioning the importance of maintaining a fluid-filled vestibule in preserving hearing.

Objective  To report on the preservation of hearing after a complete labyrinthectomy for Meniere disease and after disruption of the vestibule.

Evidence Review  This systematic review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) reporting guidelines. A search of PubMed, Scopus, Ovid, and Cochrane Library databases was conducted to identify English-language articles on hearing preservation after labyrinthectomy, published from 1947 through December 11, 2019. The search strategy used a combination of boolean operators and included the following Medical Subject Heading terms and keywords: hearing preservation, labyrinth surgery, labyrinthectomy, vestibule violation, vestibule disruption, translabyrinthine approach, schwannoma removal, and semicircular canal ablation. Studies that included disruption of the vestibule and hearing preservation were included.

Findings  This systematic review identified 10 studies with 10 patients who underwent surgical removal of cholesteatoma or vestibular schwannoma and displayed postoperative hearing preservation. This study also reported on 1 patient with Meniere disease who retained hearing after undergoing a complete labyrinthectomy. Among these 11 patients, the mean (range) age was 45.1 (27-55) years, and 8 patients (73%) were women. Multiple theories exist that explain the mechanism behind hearing preservation, such as sealing of the ductus reuniens or closure of the remaining vestibule.

Conclusions and Relevance  This systematic review describes a set of patients who did not experience hearing loss after a labyrinthectomy or surgical violation of the vestibule, which seems to contradict prevailing principles for retaining hearing after inner-ear surgical procedures. This finding suggests that hearing preservation is possible after labyrinthine destruction despite the absence of a fluid-filled vestibule and that other mechanisms, such as occlusion of the ductus reuniens with granulation tissue or bone dust, may be sufficient to achieve that outcome.

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