Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes

• Published in: The Laryngoscope Vol. 126; no. 6; p. 1446
• Main Authors: Sone, Michihiko, Yoshida, Tadao, Morimoto, Kyoko, Teranishi, Masaaki, Nakashima, Tsutomu, Naganawa, Shinji
• Format: Journal Article
• Language: English
• Published: United States 01.06.2016
• Subjects: Adolescent; Adult; Audiometry, Pure-Tone; Cochlear Diseases - complications; Cochlear Diseases - diagnostic imaging; Cochlear Diseases - physiopathology; Contrast Media; Ear Canal - diagnostic imaging; Ear Canal - physiopathology; Endolymphatic Hydrops - diagnostic imaging; Endolymphatic Hydrops - etiology; Endolymphatic Hydrops - physiopathology; Female; Gadolinium DTPA; Hearing Loss, Sensorineural - diagnostic imaging; Hearing Loss, Sensorineural - etiology; Hearing Loss, Sensorineural - physiopathology; Humans; Magnetic Resonance Imaging - methods; Male; Middle Aged; Perilymph - physiology; Round Window, Ear - diagnostic imaging; Round Window, Ear - physiopathology; Syndrome; Vestibular Aqueduct - diagnostic imaging; Vestibular Aqueduct - physiopathology; Vestibular Diseases - complications; Vestibular Diseases - diagnostic imaging; Vestibular Diseases - physiopathology; Young Adult; third window; Hydrops; MRI; large vestibular aqueduct; endolymphatic hydrops; superior canal dehiscence
• ISSN: 1531-4995
• DOI: 10.1002/lary.25747

Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI). Case series at university hospital. Seventeen ears from nine subjects who were diagnosed as having SCDS (five ears from three cases) or LVAS (12 ears from six cases) were studied. Ears were evaluated by 3-T MRI performed 4 hours after intravenous injection of gadodiamide hydrate. Imaging data concerning the degree of EH in the cochlea and the vestibule were compared with clinical symptoms and hearing levels for all ears. All ears showed air-bone gaps at low frequencies on pure tone audiometry. None of the subjects with SCDS had episodes of acute sensorineural hearing loss (SNHL) or vestibular symptoms, except for one patient who complained of head vibration induced by loud noise. Conversely, five of six subjects with LVAS had episodes of acute SNHL or vestibular symptoms. Four of five ears with SCDS showed severe EH in the cochlea, and two ears showed mild EH in the vestibule. All ears with LVAS showed mild to severe EH in both the cochlea and vestibule. The present study demonstrated the existence of EH in ears with pathologic third window lesions, which might affect patients' auditory or vestibular symptoms. 4 Laryngoscope, 126:1446-1450, 2016.