Clinical case of gusher-syndrome in surgery of a congenital isolated malformation of the middle ear

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Abstract

Gusher syndrome is a rather rare condition complicating surgical interventions on the stapes. It is characterized by profuse effusion of perilymphatic fluid from the vestibule into the lumen of the tympanic cavity during fenestration or removal of the foot plate of the stapes and rapid filling of the volume of the tympanic cavity and the external auditory canal with perilymph. This is due to increased pressure of perilymph fluid in the inner ear cavity in some developmental anomalies. Preoperative diagnosis of gusher syndrome can be difficult when the CT scan does not show any abnormalities in the anatomy of the inner ear canal and inner ear structures. The rarity of this phenomenon greatly complicates the procedure of its study, as well as the development of optimal schemes of therapeutic and diagnostic tactics in a real clinical situation. A clinical case of a combination of congenital isolated middle ear developmental anomaly and gusher syndrome is studied. A patient with suspected otosclerosis underwent stapedoplasty at Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech. During the operation, abundant perilymphorrhea "Gusher" symptom was obtained, as well as thickening of the stapes legs and thin tendon of the stirrup muscle were revealed. The postoperative diagnosis was changed from otosclerosis to congenital isolated anomaly of middle ear development (isolated ankylosis of the stapes), gusher syndrome. The tactics of management of a patient with otosclerosis and isolated middle ear anomaly are similar. CT of temporal bones and virtual CT endoscopy should become the standard of examination of patients before surgical treatment. The use of cartilage and supracartilage is a safe way to stop perilymphorrhea in patients with gusher syndrome.

About the authors

Svetlana V. Astashchenko

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Author for correspondence.
Email: docte@yandex.ru
ORCID iD: 0000-0003-1863-2279

D. Sci. (Med.)

Russian Federation, Saint Petersburg

Igor А. Anikin

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Email: dr-anikin@mail.ru
ORCID iD: 0000-0003-2977-2656

D. Sci. (Med.)

Russian Federation, Saint Petersburg

Sergei N. Ilyin

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Email: docte@yandex.ru

Cand. Sci. (Med.)

Russian Federation, Saint Petersburg

Oleg I. Goncharov

City Hospital №26

Email: entgoncharov@gmail.com
ORCID iD: 0000-0003-3738-4944

otorhinolaryngologist

Russian Federation, Saint Petersburg

Denis D. Kaliapin

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Email: kalyapin92@gmail.com
ORCID iD: 0000-0002-2768-6036

Cand. Sci. (Med.), Res. Assist.

Russian Federation, Saint Petersburg

Ilya M. Dyakov

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Email: ilya.dyakov@mail.ru

otorhinolaryngologist, Res. Assist.

Russian Federation, Saint Petersburg

Anastasiya A. Valkova

Saint-Petersburg Research Institute of Ear, Throat, Nose and Speech

Email: Anastasi.valkova@yandex.ru
ORCID iD: 0009-0000-8792-9450

Clinical Resident

Russian Federation, Saint Petersburg

References

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Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Tonal threshold audiometry. On the left is a mixed form of hearing loss of the 2nd degree, the presence of a Carhart tooth at a frequency of 2000 Hz, on the right is conductive hearing loss 1st degree. Date of the event: 03/10/2023.

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3. Fig. 2. Normal structures of the left VC are visualized on MSCT sections : a - normal dimensions and absence of defects in the bone wall of the middle and apical whorls of the cochlea; b – normal dimensions and absence of defects in the wall of the cochlea conduit and the basal whorl of the cochlea; c - normal dimensions of the vestibule plumbing; d – normal dimensions and absence of defects in the bone capsule of the vestibule.

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4. Fig. 3. Normal structures of the VU and SU are visualized on sections of the MSCT of the left VC : a - normal dimensions and absence of defects in the bone capsule of the sagittal semicircular canal; b – normal dimensions and absence of defects in the bone capsule of the lateral semicircular canal; c – normal dimensions and absence of defects in the bone capsule of the frontal semicircular canal; d – normal dimensions and absence of defects in the wall of the VSP.

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5. Fig. 4. Virtual CT endoscopy of BP. The normal configuration of the SC circuit is visualized.

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6. Fig. 5. Intraoperative otomicroscopy: left BP. Thinned long leg Anvils and tendons SEE The front and rear stirrup legs are thickened. The superstructures of the stirrup are immobile.

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7. Fig. 6. Intraoperative otomicroscopy: left BP. The tendon of the CM is crossed, the superstructures of the stirrup are removed. A stationary gray PPP is visualized . The annular ligament does not contour.

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8. Fig. 7. Intraoperative otomicroscopy: left BP. After perforation of the PPP, abundant perilous trout - gusher syndrome was detected.

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9. Figure 8. Intraoperative otomicroscopy: left BP. The stage of tamponade of the niche of the vestibule window with cartilage and fragments of autochondria.

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10. Fig. 9. Tonal threshold audiometry on the 13th day after surgery. The absence of negative dynamics in comparison with the preoperative audiogram, the absence of a Carhart wave at a frequency of 2000 Hz.

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