Table 1.
Patient third window syndrome symptoms, physical findings, and results of diagnostic studies in 16 patients with cochlea-facial nerve dehiscence.
| Group 1: Cochlea-facial nerve dehiscence and third window syndrome patients who underwent round window reinforcement surgery | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Patient (age at surgery) | Sex | Sound-induced | Hearing internal sounds | 128 and 256 Hz tuning forks | Pseudoconductive hearing loss | Electrocochleography (SP/AP ratio) | cVEMP threshold (dB nHL)/amplitude (μV) | Surgery performed (length of follow-up) | High-resolution TB CT |
| 1* (12.75) | M | Dizziness and nausea, increased headache | Heartbeat | Negative | None | L 0.36 R 0.32 |
L 75 dB/437 μV R 95 dB/458 μV |
L RWR (52 months) | L CFD |
| 2* (12.92) | M | Increased HA, dizziness, pain | Eyes moving and blinking (R > L) | Positive (back of head) | Left, Right (true conductive hearing loss) | L 0.43 (ELH) R 0.38 |
L 80 dB/77 μV R 95 dB/301 μV (with true conductive hearing loss) |
L RWR R RWR (71 months) |
L CT– TWS R CFD |
| 3 (15.17) | F | Dizziness, headache | Eyes blinking, chewing, heel strike | Positive | Bilateral | L 0.38 R 0.46 (ELH) |
L 80 dB/121 μV R 75 dB/148 μV |
R RWR (10 months) | R CFD L near-CFD |
| 4 (16.5) | F | Increased headache, no dizziness | Voice resonant (left) | Positive (back of head) | Left | L 0.33 R 0.34 |
L 75 dB/466 μV R 95 dB/358 μV |
L RWR (49 months) | L CFD |
| 5* (17.19) | F | Dizziness, migraine; severe sound sensitivity/pain | Eyes blinking, autophony | Positive | Bilateral | L 0.28 R 0.32 |
L 90 dB/415 μV R 70 dB/619 μV |
R RWR (69 months) | R CFD |
| 6* (19.0) | F | Dizziness, nausea, agitated, worsens postural dyscontrol | Voice resonant (L > R), eyes moving and blinking (R), heartbeat (R), chewing (R) | Positive | Bilateral | L 0.39 R 0.37 |
L 70 dB/194 μV R 70 dB/206 μV |
R RWR (11 months) | R CFD |
| 7* (51.42) | F | Dizziness, nausea, HA | Voice resonant, heartbeat | Positive | Left | L 0.36 R 0.35 |
L 75 dB/277 μV R 75 dB/296 μV |
L RWR (12 months) | L CFD |
| 8 (52.92) | F | Dizziness, nausea | Voice resonant | Negative | Bilateral | L 0.14 R 0.37 |
L 95 dB/3.3 μV R 80 dB/22 μV |
R RWR (37 months) | R CFD |
| Group 2: Cochlea-facial nerve dehiscence and third window syndrome patients who did not undergo round window reinforcement surgery | |||||||||
| Patient (age at presentation) | Sex | Sound-induced | Hearing internal sounds | 128 and 256 Hz tuning forks | Pseudoconductive hearing loss | Electrocochleography (SP/AP ratio) | cVEMP threshold (dB nHL)/amplitude (μV) | Surgery performed | High-resolution TB CT |
| 1 (6.65) | M | Dizziness; pain | Heel strike, face being touched | Positive | Left | L 0.36 R 0.17 |
L 70 dB/1,093 μV R 70 dB/531 μV |
None | Bilateral CFD (R > L) |
| 2 (7.58) | F | Dizziness; pain | Voice resonant | Positive | Left | L 0.26 R 0.30 |
L 70 dB/430 μV R 80 dB/387 μV |
None | L CFD |
| 3 (27.88) | F | Dizziness | Voice resonant | Positive | Left (small) | L 0.30 R 0.29 |
L 75 dB/180 μV R 80 dB/170 μV |
None | L CFD |
| 4 (28.71) | F | Dizziness, increased headache | No | Positive | Bilateral | L 0.39 R 0.23 |
L 70 dB/554 μV R 90 dB/539 μV |
None | Bilateral CFD (L > R) |
| 5 (30.27) | F | Dizziness, confusion, overwhelmed, headache | Eyes blinking, voice resonant, chewing, heartbeat | Positive | Bilateral | L 0.37 R 0.31 |
L 80 dB/134 μV R NR |
None | Bilateral CFD |
| 6 (34.61) | M | Head pain, bitter taste | No | Positive | Left | L 0.37 R 0.39 |
L 75 dB/400 μV R 80 dB/229 μV |
None | L CFD or near-CFD |
| 7 (54.71) | M | Agitated, sense of foreboding | Chewing | Positive | Bilateral | L 0.36 R 0.35 |
L 80 dB/100 μV R 80 dB/75 μV |
None | L CFD |
| 8 (55.67) | F | Dizziness | Chewing | Positive | Left | L NR R NR |
L 85 dB/41 μV R 95 dB/20 μV |
None | L CFD |
See video links in references (15–19) [Cohort 1: subject 1 (15), subject 2 (16), subject 5 (17), subject 6 (18), subject 7 (19)]; 128 and 256 Hz = ability to hear or feel the vibration of the tuning fork in the head when applied to knees and elbows; CFD, cochlea-facial nerve dehiscence; CT, computed tomography scan; CT–, CT negative (no dehiscence seen on CT); cVEMP, cervical vestibular evoked myogenic potential; dB nHL, decibel above normal adult hearing level; Dizziness, gravitational receptor asymmetry type of vertigo (e.g., as if on a boat, rocky, wavy, tilting, being pushed, pulled, flipped, or sense of floor falling out from under them); ELH, endolymphatic hydrops (abnormal summating potential/action potential [SP/AP] ratio >0.42 by electrocochleography); F, female; HA, headache; L, left; M, male; TB, temporal bone; R, right. The classification of headache and migraine used in this study followed the International Headache Society's International Classification of Headache Disorders, 3rd edition (ICHD3).