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Specific anatomic defects include semicircular canal dehiscence, perilabyrinthine fistula, enlarged vestibular aqueduct, dehiscence of the scala vestibuli side of the cochlea, X-linked stapes gusher, and bone dyscrasias.

We discuss these various entities and provide key examples from our institutional teaching file with a discussion of symptomatology, temporal bone CT, audiometry, and vestibular-evoked myogenic potentials. Third window abnormalities are defects in the integrity of the bony structure of the inner ear, first described by Minor et al in Normal sound conduction is transmitted through the oval and round windows, which serve as fluid interfaces between air in the middle ear and perilymphatic fluid spaces of the inner ear.

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Various conditions can enlarge existing bony channels or create additional defects in the bony labyrinth, producing hydrodynamic third windows. Potential third windows include bony dehiscence of the semicircular canals, enlargement of the opening of the vestibular aqueduct, dehiscence of the scala vestibuli side of the cochlea, and abnormal bony thinning between the cochlea and vascular channels.

At audiometry, there is a characteristic low-frequency air-bone gap due to decreased air conduction and increased bone conduction.

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Figure 1 A illustrates the mechanism of air-conducted sound in normal ears, and Fig 1 B demonstrates how third window shunting effects decrease air conduction. The 2 physiologic windows between the middle and inner ear are the oval window, which transmits vibrations from the auditory ossicles, and the round window of the cochlea. With air conduction, there is physiologic entrainment of the oval and round windows due to coupling by incompressible perilymph.

Pressure differences between the cochlear perilymphatic spaces activate hair cells and create the perception of sound. In the presence of a third window, incoming acoustic energy from the oval window is shunted away, decreasing transmission to the round window. This result reduces sound perception because less acoustic energy is available to the hair cells. Mechanisms of air- and bone-conducted sound in healthy and third window anatomy.

Abbreviations:

ANormal air conduction. Vibrations of the tympanic membrane are transmitted inward through the auditory ossicles and oval window.

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Energy is then conducted through the incompressible perilymph, producing equal and outward motion of the round window. The difference in vibration between the oval and round windows generates a pressure gradient across the basilar membrane, activating hair cells and creating the perception of sound. BDecreased air conduction in third window anatomy.

Due to shunting across third windows semicircular canal dehiscence [SCCD], EVAS, cochlear dehiscencethere is decreased energy transmission from the oval window to the round window.

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The decrease in pressure gradient across the basilar membrane yields reduced sound perception. CNormal bone conduction. Vibrations are transmitted throughout the otic capsule. This transmission in differential outward motion of the oval and round windows due to unequal impedance of these 2 structures.

The resulting pressure difference across the basilar membrane enables sound perception. DIncreased bone conduction in third window anatomy. Due to shunting across third windows, there is decreased motion of the oval window on the scala vestibuli side of the cochlea.

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However, the motion of the round window on the scala tympani side is unchanged. This phenomenon artifactually elevates the pressure difference across the basilar membrane, resulting in increased sound perception. TM indicates tympanic membrane; yellow, auditory ossicles; beige, otic capsule; red, oval window; green, round window; blue, perilymph; purple, basilar membrane.

Conductive hearing loss caused by third window lesions of the inner ear. Otol Neurotol ;— In contrast, Fig 1 C illustrates the mechanism of bone-conducted sound in normal ears, and Fig 1 D demonstrates how third window shunting effects paradoxically increase bone conduction. With bone conduction, vibrations throughout the otic capsule produce differential outward motion of the oval and round windows, due to unequal impedance of these 2 structures.

The pressure difference across the basilar membrane creates the perception of sound. The presence of a third window involving the semicircular canals, vestibular aqueduct, or scala vestibuli side of the cochlea lowers the apparent impedance.

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This feature has the opposite effect of increasing sound perception in proportion to the differential acoustic energy across the basilar membrane. In this article, we discuss the spectrum of third window abnormalities, including superior semicircular canal dehiscence SSCCDposterior semicircular canal dehiscence, perilabyrinthine fistula, enlarged vestibular aqueduct, X-linked stapes gusher, and bone dyscrasias.

We review the literature for each disease entity and provide key examples from our institutional teaching file with discussion of symptomatology, temporal bone CT, audiometry, and vestibular-evoked myogenic potentials. Superior semicircular canal dehiscence refers to focal loss of the bony wall of the superior semicircular canal. The prevalence is reported as 2.

SSCCD is idiopathic, though the proposed risk factors include congenital underdevelopment of bone overlying the semicircular canal, shear stress from trauma, increased pressure due to Valsalva maneuvers, and gradual erosion by vascular pulsations. Neurovascular foramina can serve as potential windows between the middle and inner ear but are normally not associated with abnormalities Bone Gap adult chat lines sound transmission due to their small cross-sectional area.

It is theorized that acoustic decompression through the dura mater acts as a third window equivalent, permitting shunting of acoustic energy into the subarachnoid space or into the vessel itself. Patients with SSCCD typically present with vertigo and nystagmus induced by loud noises Tullio phenomenon or increases in external auditory canal pressure Hennebert.

At audiometry, a characteristic air-bone gap from increased bone and decreased air conduction. This phenomenon occurs most ificantly at lower sound frequencies below 1 kHza range at which acoustic energy is readily dissipated Fig 3. At higher frequencies, there is a small or no gap because proportionally less acoustic energy is shunted by the third window. Vestibular-evoked myogenic potential testing may show abnormally low response thresholds on the side of pathology.

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The effective impedance is reduced; this reduction in increased transmission of acoustic energy at the saccule Fig 4. Because middle ear pathology can also produce an air-bone gap, tympanometry and acoustic reflexes may be tested to verify that the air-bone gap does not result from an inefficient middle ear.

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AAudiogram of the right superior semicircular canal dehiscence with increased bone conduction brackets and decreased air conduction crosses. BAudiogram of left superior semicircular canal dehiscence into the superior petrosal sinus. There is increased bone conduction brackets and decreased air conduction circlessimilar to typical SSCCD.

Vestibular-evoked myogenic potentials in superior semicircular canal dehiscence. Compared with the normal right ear triangle range linesthe left ear demonstrates an abnormally sensitive response threshold squares across all tested stimulus frequencies. The lower thresholds indicate increased excitation of otolithic organs due to third window acoustic transmission.

Posterior semicircular canal dehiscence is uncommon, with a reported frequency of 0. The condition can occur sporadically or in association with superior canal dehiscence. Clinically, patients may also demonstrate the Tullio and Hennebert s.

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Thin-collimation CT demonstrates the focal bony defect in the posterior semicircular canal Fig. Audiometry reveals an air-bone gap at frequencies below 1 kHz Fig 6. Posterior semicircular canal dehiscence. Temporal bone CT in the coronal plane demonstrates a defect arrow in the roof of the posterior semicircular canal.

Audiogram of posterior semicircular canal dehiscence with increased bone conduction brackets and decreased air conduction circles.

Spectrum of third window abnormalities: semicircular canal dehiscence and beyond

Findings are compatible with posterior semicircular canal dehiscence described in the text. At Massachusetts Eye and Ear Infirmary, 5 patients 8 ears with posterior semicircular canal dehiscence were included in the teaching file from to Destructive middle ear processes that erode the attenuated otic capsule can produce inadvertent communication with the inner ear, known as a perilabyrinthine fistula. The lateral semicircular canal is most frequently involved due to its location directly adjacent to the middle ear Fig 7.

On audiometry, cholesteatoma demonstrates a characteristic air-bone gap of middle ear origin, which is present at both low and high sound frequencies due to superimposed ossicular chain pathology Fig 8. On audiometry, this condition demonstrates an air-bone gap that is greater at lower frequencies, similar to other third windows Fig Inflammatory causes of perilabyrinthine fistula.

ACholesteatoma. Axial CT shows an expansile, widely destructive soft-tissue mass centered in the middle ear with erosion into the cochlea, vestibule, and posterior semicircular canal arrows.

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BRecurrent otitis media. Axial CT identifies residual soft-tissue opacity in the middle ear, with a fistula arrow extending through the otic capsule into the lateral semicircular canal. CInvasive fungal infection. Axial CT illustrates heterogeneous soft tissue centered in the middle ear, with invasion through the otic capsule into the lateral semicircular canal arrow.

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Audiogram of a cholesteatoma shows an air-bone gap of middle ear origin. There is decreased air conduction crosses relative to normal bone conduction brackets. The resulting air-bone gap is present at both high and low sound frequencies. Given the background abnormality, it is not possible to detect the presence of superimposed third-window effects.

Carotid-cochlear dehiscence. Audiogram of carotid-cochlear dehiscence. There is increased bone conduction brackets and decreased air conduction circleswith a progressively larger air-bone gap at lower frequencies. In this case, the ipsilateral acoustic reflex was present, indicating that the ossicular chain was free to move with the action of the stapedius muscle.

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Therefore no middle ear pathology such as serous otitis, otosclerosis, or cholesteatoma was responsible for the air-bone gap. Other potential causes of perilabyrinthine fistula include trauma Fig 11an operation, and benign and neoplastic masses Fig Transverse temporal bone fractures are more likely to involve the petrous pyramid and violate the otic capsule.

With this background abnormality, it is difficult or impossible to identify superimposed third window effects. In such cases, hearing is generally unrecoverable and audiometry is not performed, so a typical third window effect cannot be demonstrated.

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