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  • 1 Általános Orvostudományi Kar, Fül-, Orr-, Gégészeti és Fej-Nyaksebészeti Klinika, Pécsi Tudományegyetem, Pécs
  • 2 Fül-Orr-Gégészeti Osztály, Tolna Megyei Balassa János Kórház, Szekszárd, Béri Balogh Ádám u. 5–7., 7100
  • 3 Fül-Orr-Gégészeti Osztály, Kanizsai Dorottya Kórház, Nagykanizsa

Összefoglaló. Halláspanasszal számos beteg fordul orvoshoz. A rutinszerűen elvégzett súgottbeszéd-, hangvilla- és tisztahangküszöb-audiometriai vizsgálat alapján vezetéses, sensorineuralis, illetve a kettő együttes fennállása esetén kevert típusú halláscsökkenést különböztetünk meg. Vezetéses halláscsökkenés létrejöhet mind a külső, mind a közép- vagy a belső fül veleszületett vagy szerzett rendellenességei esetén. Amennyiben a stapediusreflex kiváltható, ugyanakkor a betegnél nincs jelen a külső fület, valamint a középfület érintő kórfolyamat, felmerül a harmadikablak-szindróma lehetősége. A kórkép okaként egy, a belső fül csontos tokján „harmadik ablakként” funkcionáló laesio van jelen, amely az ovális ablakon keresztül beérkező hangenergia egy részét elvezeti, mielőtt az a kerek ablakon át kivezetődik a középfülbe. A diagnózis felállítása gyakran nehéz feladat elé állítja a klinikust, melyhez a megfelelő audiológiai, illetve képalkotó vizsgálatok elvégzése elengedhetetlen. Tekintettel arra, hogy a panaszok megszüntetésére számos műtéti módszer került leírásra, közleményünkben átfogó képet adunk a kórkép etiológiájáról, diagnosztikájáról, terápiájáról, valamint bemutatjuk saját kezdeti tapasztalatainkat is 2 eset prezentálásával. Orv Hetil. 2020; 161(46): 1944–1952.

Summary. Patients frequently present to the physician with hearing loss. Routine hearing tests include speech field (whisper test), tuning forks and pure tone threshold audiometry, which can identify the presence of sensorineural hearing loss, conductive hearing loss or a combination of both (mixed type). Conductive hearing loss can be a symptom of many different conditions. These include congenital or acquired malformations of the outer, middle and inner ear. If a conductive hearing loss with intact stapedial reflexes are recorded and in the absence of outer or middle ear pathology, then the third window syndrome should be considered. The cause is a bony defect on the otic capsule that acts as a ’third window’, dissipating the incoming sound energy. Without the appropriate audiological and imaging tests, the diagnosis of the condition is challenging in clinical setting. Several surgical techniques have been described to treat the condition. The authors give a comprehensive review of the etiology, diagnosis and treatment of the disease presenting their initial experiences with 2 cases. Orv Hetil. 2020; 161(46): 1944–1952.

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  • 1

    Bess FH, Miller GW, Glasscock ME 3rd, et al. Unexplained conductive hearing loss. South Med J. 1980; 73: 335–338.

  • 2

    House JW, Sheehy JL, Antunez JC. Stapedectomy in children. Laryngoscope 1980; 90: 1804–1809.

  • 3

    Al Muhaimeed H, El Sayed Y, Rabah A, et al. Conductive hearing loss: investigation of possible inner ear origin in three cases studies. J Laryngol Otol. 2002; 116: 942–945.

  • 4

    Tonndorf J, Tabor JR. Closure of the cochlear windows: its effect upon air- and bone-conduction. Ann Otol Rhinol Laryngol. 1962; 71: 5–29.

  • 5

    Minor LB, Carey JP, Cremer PD, et al. Dehiscence of bone overlying the superior canal as a cause of apparent conductive hearing loss. Otol Neurotol. 2003; 24: 270–278.

  • 6

    Halmagyi GM, Aw ST, McGarvie LA, et al. Superior semicircular canal dehiscence simulating otosclerosis. J Laryngol Otol. 2003; 117: 553–557.

  • 7

    Mikulec AA, McKenna MJ, Ramsey MJ, et al. Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo. Otol Neurotol. 2004; 25: 121–129.

  • 8

    Stenfelt S, Goode RL. Bone-conducted sound: physiological and clinical aspects. Otol Neurotol. 2005; 26: 1245–1261.

  • 9

    Wever EG, Lawrence M. Physiological acoustics. Princeton University Press, Princeton, NJ, 1954.

  • 10

    Voss SE, Rosowski JJ, Peake WT. Is the pressure difference between the oval and round windows the effective acoustic stimulus for the cochlea? J Acoust Soc Am. 1996; 100: 1602–1616.

  • 11

    Minor LB, Solomon D, Zinreich JS, et al. Sound- and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg. 1998; 124: 249–258.

  • 12

    Merchant SN, Nakajima HH, Halpin C, et al. Clinical investigation and mechanism of air-bone gaps in large vestibular aqueduct syndrome. Ann Otol Rhinol Laryngol. 2007; 116: 532–541.

  • 13

    Modugno G, Brandolini C, Savastio G, et al. Superior semicircular canal dehiscence: a series of 13 cases. ORL J Otorhinolaryngol Relat Spec. 2005; 67: 180–184.

  • 14

    Mikulec AA, Poe DS. Operative management of a posterior semicircular canal dehiscence. Laryngoscope 2006; 116: 375–378.

  • 15

    Monsell EM. The mechanism of hearing loss in Paget’s disease of bone. Laryngoscope 2004; 114: 598–606.

  • 16

    Bance M. When is a conductive hearing loss not a conductive hearing loss? Causes of a mismatch in air-bone threshold measurements or a “pseudoconductive” hearing loss. J Otolaryngol. 2004; 33: 135–138.

  • 17

    Brantberg K, Bagger-Sjöbäck D, Mathiesen T, et al. Posterior canal dehiscence syndrome caused by an apex cholesteatoma. Otol Neurotol. 2006; 27: 531–534.

  • 18

    Juers AL. Observations on bone conduction in fenestration cases; physiological considerations. Ann Otol Rhinol Laryngol. 1948; 57: 28–40.

  • 19

    Snik AF, Hombergen GC, Mylanus EA, et al. Air-bone gap in patients with X-linked stapes gusher syndrome. Am J Otol. 1995; 16: 241–246.

  • 20

    Nance WE, Setleff R, McLeod A, et al. X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher. Birth Defects Orig Artic Ser. 1971; 7: 64–69.

  • 21

    Glasscock ME 3rd. The stapes gusher. Arch Otolaryngol. 1973; 98: 82–91.

  • 22

    Phelps PD, Reardon W, Pembrey M, et al. X-linked deafness, stapes gushers and a distinctive defect of the inner ear. Neuroradiology 1991; 33: 326–330.

  • 23

    Cremers CW, Snik AF, Huygen PL, et al. X-linked mixed deafness syndrome with congenital fixation of the stapedial footplate and perilymphatic gusher (DFN3). Adv Otorhinolaryngol. 2002; 61: 161–167.

  • 24

    Talbot JM, Wilson DF. Computed tomographic diagnosis of X-linked congenital mixed deafness, fixation of the stapedial footplate, and perilymphatic gusher. Am J Otol. 1994; 15: 177–182.

  • 25

    Kim HH, Wilson DF. A third mobile window at the cochlear apex. Otolaryngol Head Neck Surg. 2006; 135: 965–966.

  • 26

    Vlastarakos PV, Proikas K, Tavoulari E, et al. Efficacy assessment and complications of surgical management for superior semicircular canal dehiscence: a meta-analysis of published interventional studies. Eur Arch Otorhinolaryngol. 2009; 266: 177–186.

  • 27

    Chien WW, Carey JP, Minor LB. Canal dehiscence. Curr Opin Neurol. 2011; 24: 25–31.

  • 28

    Rodgers B, Lin J, Staecker H. Transmastoid resurfacing versus middle fossa plugging for repair of superior canal dehiscence: comparison of techniques from a retrospective cohort. World J Otorhinolaryngol Head Neck Surg. 2016; 2: 161–167.

  • 29

    Ziylan F, Kinaci A, Beynon AJ, et al. A comparison of surgical treatments for superior semicircular canal dehiscence: a systematic review. Otol Neurotol. 2017; 38: 1–10.

  • 30

    Teixido M, Seymour PE, Kung B, et al. Transmastoid middle fossa craniotomy repair of superior semicircular canal dehiscence using a soft tissue graft. Otol Neurotol. 2011; 32: 877–881.

  • 31

    Fiorino F, Barbieri F, Pizzini FB, et al. A dehiscent superior semicircular canal may be plugged and resurfaced via the transmastoid route. Otol Neurotol. 2010; 31: 136–139.

  • 32

    Ward BK, Agrawal Y, Nguyen E, et al. Hearing outcomes after surgical plugging of the superior semicircular canal by a middle cranial fossa approach. Otol Neurotol. 2012; 33: 1386–1391.

  • 33

    Goddard JC, Wilkinson EP. Outcomes following semicircular canal plugging. Otolaryngol Head Neck Surg. 2014; 151: 478–483.

  • 34

    Shaia WT, Diaz RC. Evolution in surgical management of superior canal dehiscence syndrome. Curr Opin Otolaryngol Head Neck Surg. 2013; 21: 497–502.

  • 35

    Peng A, Shaia WT. Endoscopic repair of superior canal dehiscence. Presented at the Virginia Society of Otolaryngology Annual Meeting, Charlottesville, VA, May 1–2, 2010.

  • 36

    Silverstein H, Kartush JM, Parnes LS, et al. Round window reinforcement for superior semicircular canal dehiscence: a retrospective multi-center case series. Am J Otolaryngol. 2014; 35: 286–293.