OBJECTIVE: Radiographic review of pathologies that associate with third window syndrome.
METHODS: Case series and literature review.
RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct.
CONCLUSIONS: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.

We report the case of a man suffering from superior semicircular canal dehiscence with severe audiovestibular symptoms. The patient had a history of posttraumatic stress disorder and traumatic brain injury, and he had a presumed diagnosis of schizophrenia, with suicidal ideation. The patient was treated surgically with complete resolution of his symptoms and a retraction of his schizophrenia diagnosis. This case highlights the overlap between tinnitus and auditory hallucinations, as both result in auditory perception without an external stimulus. Ascertaining the appropriate cause of a patient\'s auditory symptoms is integral to providing appropriate medical and possibly surgical care.

To evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma.
Case series assessing patients presenting with SSCD after a trauma.
A case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio-vestibular symptoms, 2) mean pre- and post-operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG).
A total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air-conduction PTA of 38.0 dB, bone-conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air-conduction PTA, bone-conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005).
Head trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra-vestibular or intracranial pressures, unmasking SSCD syndrome.
4 Laryngoscope, 131:E2810-E2818, 2021.

UNASSIGNED: Report a series of cases in which patients have concomitant superior semicircular canal dehiscence (SSCD) and a dehiscent tegmen tympani with Dural contact to the malleus head (DCMH).
UNASSIGNED: An analysis of radiologic and audiologic data in 4 patients who presented with SSCD and DCMH at a tertiary care institution. A pertinent literature review was performed.
UNASSIGNED: Four patients (5 ears) had SSCD and DCMH. In 3 patients with unilateral DCMH, the mean maximum air-bone gap was 15 dB in the ear with DCMH compared to 50 dB in the ear without DCMH. Of the 5 ears with DCMH, the mean air conduction threshold at 250 Hz was 17 dB compared to 42 dB in the 3 ears without DCMH.
UNASSIGNED: We report the findings of DCMH in a series of 4 patients with bilateral SSCD. This limited series suggests that ears with SSCD and DCMH have less of an air-bone gap than would be expected, as 1 would expect an additive effect of DCMH and SSCD on the air-bone gap.

Concomitant otosclerosis (OTS) and superior semicircular canal dehiscence (SSCD) is a rare, but difficult-to-identify and treat diagnosis. A systematic review of the literature was performed to analyze the diagnostic and therapeutic approaches of concurrent OTS and SSCD cases and to identify possible factors that may help in predicting the surgical outcome.
PubMed, Scopus, Medscape, Ovid databases.
Studies showing diagnosis of OTS documented by audiometric test with or without associated radiological signs (computed tomography), and concomitant diagnosis of SSCD, documented at least by high-resolution computed tomography (and possibly supported by neurophysiological testing) were included. Both surgically treated and untreated patients were considered for data analysis.
The general characteristics of each study were recorded, when available. Clinical, audiological, vestibular testing, surgical, and radiological data were extracted from the published case reports and series, and recorded on a database. For each case, the diagnostic steps that confirmed OTS and concomitant SSCD in the same ear were extracted.
Twelve studies were included in the review and 18 cases affected simultaneously by the 2 conditions were extracted and analyzed. Seven of 12 ears (58.3%) undergoing stapes surgery experienced a hearing improvement. In four (33.3%) cases, vestibular symptoms (with or without associated acoustic symptoms) of SSCD were unmasked or worsened by stapes surgery.
A reliable preoperative diagnosis of the two concomitant conditions is possible with a proper interpretation of clinical signs, audiometric, and vestibular testing, in association with the radiologic assessment. Despite that the length and the location of the dehiscence may guide the surgical decision, definitive conclusions regarding the appropriate indications for surgical treatment cannot be drawn due to the limited number of cases with adequate data reported in the literature.

Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease\'s as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio\'s phenomenon, and Hennebert\'s sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs.

To conduct a systematic review of posterior semicircular canal dehiscence (PSCD) and to present a series of patients with PSCD with and without classic third-window symptoms.
PubMed, Scopus, and the Cochrane Library from inception until April 2019. Case series of five patients seen in a multidisciplinary, vestibular-focused, neurotology clinic.
Inclusion criteria: PSCD studies of symptomatology, diagnostic testing, radiology, and histopathology.
non-English articles, reviews, letters, animal studies.
Quality evaluated according to Oxford Center for Evidence-Based Medicine criteria and funnel plot via the Stern and Egger method.
Two hundred five studies were found, and 58 studies were included. In 47 total patients, sound-induced vertigo, mixed hearing loss, and tinnitus were the most common presenting symptom. A meta-analysis of proportions using eight radiological and histopathological studies revealed an incidence of 0.38% adult ears [95% CI 0.08, 0.89] and 2.16% of adult patients [0.64, 4.54]. The incidence in pediatric patients ranged from 1.3 to 43%. Jugular bulb abnormalities were common. In our case series, four of five patients presented without third-window symptoms, while one had sound- and pressure-induced vertigo. Hearing loss in these patients was not salvageable.
PSCD is a rare phenomenon most commonly presenting with third-window type symptoms. However, PSCD might also present with dizziness and hearing loss inconsistent with third-window symptomatology. One should be conscious of potentially poorer prognosis for hearing recovery in these patients.