Auditory Function in Patients with Surgically Treated Superior Semicircular Canal Dehiscence

Limb, Charles J.; Carey, John P.; Srireddy, Sharmila; Minor, Lloyd B.

doi:10.1097/01.mao.0000235376.70492.8e

Cited by 119 publications

(115 citation statements)

References 17 publications

(10 reference statements)

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“…1). Therefore, supranormal thresholds for bone conduction may be evident, as in many patients with SCD (11,(13)(14)(15)(16). Such improvements of bone-conducted thresholds produced by the third window may be masked by an accompanying true sensorineural hearing loss.…”

Section: Mechanism Of Conductive Hearing Lossmentioning

confidence: 99%

“…A low-frequency air-bone gap with bone conduction thresholds that are better than 0 dB (−5 to −20 dB) can be a clue especially in dehiscence of the semicircular canals (11,(13)(14)(15)(16)32). Therefore, it is important to accurately assess audiometric bone conduction thresholds to levels less than 0 dB hearing level.…”

Section: Diagnosismentioning

confidence: 99%

“…Low-frequency (<2,000 Hz) bone conduction thresholds are sometimes at supranormal levels, 0 to −20 dB, or better. The lack of middle ear pathologic findings as a cause for the CHL in SCD has been well documented by a variety of diagnostic tests such as tympanometry, acoustic reflexes, laser Doppler vibrometry, air-conducted VEMP testing, otoacoustic emission (OAE) testing, and by exploration of the middle ear (9)(10)(11)(12)(13)(14)(15)(16). Definitive evidence that the SCD can cause a CHL is demonstrated by resolution of the air-bone gap upon patching or plugging the dehiscence, as has been reported by various investigators (9,12,20,21).…”

Section: Superior Canal Dehiscencementioning

confidence: 99%

“…The report of Minor et al is noteworthy because these patients did not have any features of genetic deafness or apparent malformations of the inner ear, and their clinical presentation was similar to patients with middle ear disease such as otosclerosis. Several authors have now reported CHL as a manifestation of SCD (9)(10)(11)(12)(13)(14)(15)(16). Our research group has conducted a series of studies to investigate the mechanisms by which an SCD results in a CHL (11,(17)(18)(19)(20)(21)(22).…”

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confidence: 99%

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Conductive Hearing Loss Caused by Third-Window Lesions of the Inner Ear

Merchant

Rosowski

2008

Otology &Amp; Neurotology

299

223

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Section: Mechanism Of Conductive Hearing Lossmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Superior Canal Dehiscencementioning

confidence: 99%

mentioning

confidence: 99%

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Conductive Hearing Loss Caused by Third-Window Lesions of the Inner Ear

Merchant

Rosowski

2008

Otology &Amp; Neurotology

299

223

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“…The major surgical treatments involve a number of risks inherent in the type of surgery, but in addition to these risks and sequel, access by the middle cerebral fossa could cause sensor neural hearing loss in patients who have to be revised in the future or have been surgically treated previously by stapedotomy [24]. In addition, Transmastoidea and endaural approach can produce vestibularhypo function in addition of sensor neural hearing loss [25].…”

Section: Diagnosismentioning

confidence: 99%

Superior Semicircular Canal Dehiscence Syndrome as Diagnosis of Vestibular Pathology; Diagnosis and Treatment: Review of the Literature

Valdés-Pons¹,

Jc²,

Eiroa-Breijo³

et al. 2017

JOENTR

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Introduction: Superior Semicircular Canal Dehiscence Syndrome is a pathological entity that causes incapacitating auditory and vestibular symptoms. It is common that these patients are erroneously diagnosed with simulators or psychiatric diseases, or with any other vestibular pathologies.Etiology: It seems to have a genetic predisposition supported in a higher frequency of Semicircular Canal Dehiscence and bone defects in the tegmen tympani in patients with DFNA 9 (gen COCH). In other cases there is no specific cause justifying communication between the SSC and cranial cavity. Over the years, and the progressive thinning of the bone, the communication between membranous labyrinthine and cranial cavity, will occur.Pathophysiology: Superior Semicircular Canal Dehiscence Syndrome is characterized by thinning or loss of bone that covers the SSC and which separates it from of the cranial cavity. A pathologic third window on the vestibular side of the cochlea allows for a portion of acoustic energy to be shunted away from the cochlear partition, producing a decrease in the sound pressure within the vestibule, thus resulting in a loss of hearing sensitivity to air-conducted sound (conductive hearing loss) and increases the pressure difference between the two sides of the cochlear partition by lowering the impedance on the vestibule side, thereby improving the cochlear response to bone conduction (supraliminal levels). Diagnosis:Is based on the clinical history (auto phony, fluctuating hearing loss, acute or short-term vertigo). Typically patients complain of auto phony and feeling of being "in a box". The audio vestibular tests will confirm the clinical diagnosis: PTTA with GAP in low frequencies, VEMPs with threshold elevation in affected ear and CT with SSC dehiscence. Treatment:The initial treatment of this pathology should be conservative. If the symptoms are disabling it may raise a surgical solution that is the closure of the communication between the SSC and the floor of the middle fossa. The different types of surgical treatment are: access to the middle cranial fossa by temporal craniotomy; access to the SSC by upper Tran's mastoid access, and finally, strengthening of the oval and round windows membranes by endaural access.Case Report: Present four patients diagnosed and treated surgically in our hospital. Comment their post operatory evolution and the functional results. Conclusion:The SCD is a pathologic entity that must be present in the differential diagnosis of all patients with audio logical and vestibular symptoms. Their initial treatment should be conservative, but if the symptoms are disabling, surgery should be recommended since results are very good, compensating risks.

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