title: Vestibular dehiscence syndrome caused by a labyrinthine congenital cholesteatoma.
abstract: A 40-year-old man presented with conductive hearing loss and pressure- and sound-related vestibular symptoms. Computed tomography and diffusion-weighted magnetic resonance imaging revealed the presence of a cholesteatoma involving the vestibular labyrinth. The patient underwent a canal-wall-up tympanoplasty, which revealed evidence of a disruption of the vestibular labyrinth and a wide dehiscence of the vestibule, which was immediately resurfaced. At the 2-month follow-up, the patient's pressure- and sound-related vestibular symptoms had disappeared. Pure-tone audiometry showed a reduction in the air-bone gap with a slight deterioration of bone conduction and an improvement in the air-conduction threshold. Fistulization of the otic capsule produces a "third window," which can lead to a dehiscence syndrome. One possible cause is a cholesteatoma of the middle ear or petrous bone. When the vestibule is invaded by a cholesteatoma, hearing is almost invariably lost, either pre- or postoperatively. However, in our case, wide opening of the vestibule resulted in hearing preservation.
authors: Unità Operativa di Otorinolaringoiatria, Ospedale Civile Maggiore, Azienda Ospedaliera Universitaria Integrata, Piazzale Stefani, 37126 Verona, Italy. franco.fiorino@virgilio.it.
authors: Francesco
authors: F
authors: Fiorino
authors: Francesca B
authors: FB
authors: Pizzini
authors: Barbara
authors: B
authors: Mattellini
authors: Franco
authors: F
authors: Barbieri
mesh_terms: D000328:Adult; D002781:Cholesteatoma / Q000150:complications / Q000151:congenital*; D005402:Fistula / Q000150:complications*; D006314:Hearing Loss, Conductive / Q000209:etiology; D006801:Humans; D007759:Labyrinth Diseases / Q000150:complications*; D008297:Male; D009325:Nausea / Q000209:etiology; D004856:Postural Balance; D012678:Sensation Disorders / Q000209:etiology*; D013577:Syndrome; D014717:Vertigo / Q000209:etiology; D015837:Vestibular Diseases / Q000209:etiology