Table 3.
Spectrum of symptoms, signs or exacerbating factors seen in third window syndrome and diagnostic tools and metrics available to measure these clinically observed phenomenon.
| Category | Symptom, sign, or exacerbating factors | Diagnostic tools and metrics |
|---|---|---|
| Sound-induced | Dizziness or otolithic dysfunction (see vestibular dysfunction below); nausea; cognitive dysfunction; spatial disorientation; migraine/migrainous headache; pain (especially children); loss of postural control; falls | History; 128 and 256 Hz tuning forks applied to ankles, knees and/or elbows heard or felt in the ear or head; pneumatic otoscopy; cVEMP/oVEMP with reduced threshold with or without increased amplitude, auditory stimuli inducing symptoms; Romberg test while pure tones delivered to individual ear or low frequency tuning fork applied to elbow |
| Autophony | Resonant voice; chewing; heel strike; pulsatile tinnitus; joints or tendons moving; eyes moving or blinking; comb or brush through hair; face being touched | History |
| Vestibular dysfunction | Gravitational receptor (otolithic) dysfunction type of vertigo (rocky or wavy motion, tilting, pushed, pulled, tilted, flipped, floor falling out from under); mal de débarquement illusions of movement | History; Dizziness Handicap Inventory (DHI); cVEMP/oVEMP; computerized dynamic posturography; Romberg/sharpened Romberg; head tilt; nuchal muscle tightness |
| Headache | Migraine/migrainous headache; migraine variants (ocular, hemiplegic or vestibular [true rotational vertigo]); coital cephalagia; photophobia; phonophobia; aura; scotomata | History; Headache Impact Test (HIT-6); Migraine Disability Assessment Test (MIDAS) |
| Cognitive dysfunction | General cognitive impairment, such as mental fog, dysmetria of thought, mental fatigue; Impaired attention and concentration, poor multitasking (women > men); Executive dysfunction; Language problems including dysnomia, agrammatical speech, aprosidia, verbal fluency; Memory difficulties; Academic difficulty including reading problems and missing days at school or work; Depression and anxiety | History Cognitive Screen: MoCA and Schmahmann syndrome scale IQ: WRIT or WAIS2 Attention: NAB, Attention Module and/or CPT3 Memory: CVLT2, WMS4, or WRAML2 Executive Functioning: WCST, TMT, D-KEFS Language: NAB, Naming Visuospatial: Benton JLO Mood/personality: Clinical interview, PHQ-9, GAD-7, ACES, BDI2, BAI, Personality Assessment Inventory (PAI), or Millon Behavioral Diagnostic |
| Spatial disorientation | Trouble judging distances; detachment/passive observer when interacting with groups of people; out of body experiences; perceiving the walls or floor moving | History; subjective visual vertical |
| Anxiety | Sense of impending doom | History; GAD-7; BAI |
| Autonomic dysfunction | Nausea; vomiting; diarrhea; lightheadedness; blood pressure lability; change in temperature regulation; heart rate lability | History; autonomic testing |
| Endolymphatic hydrops | Ear pressure/fullness not relieved by the Valsalva maneuver; barometric pressure sensitivity | History; Electrocochleography, tympanometry |
| Hearing | Pseudoconductive hearing loss (bone-conduction hyperacusis) | Comprehensive audiometric evaluation including tympanometry, stapedial reflex testing, speech perception testing, air-conduction and bone-conduction thresholds; magnitude varies by site of dehiscence |
ACES, Adverse Childhood Experiences Scale; BAI, Beck Anxiety Inventory; BDI2, Beck Depression Inventory, 2nd edition; Benton JLO, Benton Judgment of Line Orientation; CPT3, Continuous Performance Test, 3rd edition; CVLT2, California Verbal Learning Test, 2nd edition; D-KEFS, Delis-Kaplan Executive Function System; DHI, Dizziness Handicap Inventory; GAD-7, Generalized Anxiety Disorder Screener; HIT-6, Headache Impact Test; MoCA, Montreal Cognitive Assessment; NAB, Neuropsychological Assessment Battery; PAI, Personality Assessment Inventory; PHQ-9, Patient Health Questionnaire; TMT, Trail Making Test; WAIS2, Wechsler Adult Intelligence Scale, 2nd edition; WCST, Wisconsin Card Sorting Test; WMS4, Wechsler Memory Scale, 4th edition; WRAML2, Wide Range Assessment of Memory and Learning, 2nd edition; WRIT, Wide Range Intelligence Test.