Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence
Objective: This communication is the first assessment of outcomes after surgical repair of cochlea-facial nerve dehiscence (CFD) in a series of patients. Pre- and post-operative quantitative measurement of validated survey instruments, symptoms, diagnostic findings and anonymous video descriptions o...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-12-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2019.01281/full |
| _version_ | 1818034825885384704 |
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| author | P. Ashley Wackym Carey D. Balaban Pengfei Zhang David A. Siker Jasdeep S. Hundal |
| author_facet | P. Ashley Wackym Carey D. Balaban Pengfei Zhang David A. Siker Jasdeep S. Hundal |
| author_sort | P. Ashley Wackym |
| collection | DOAJ |
| description | Objective: This communication is the first assessment of outcomes after surgical repair of cochlea-facial nerve dehiscence (CFD) in a series of patients. Pre- and post-operative quantitative measurement of validated survey instruments, symptoms, diagnostic findings and anonymous video descriptions of symptoms in a cohort of 16 patients with CFD and third window syndrome (TWS) symptoms were systematically studied.Study design: Observational analytic case-control study.Setting: Quaternary referral center.Patients: Group 1 had 8 patients (5 children and 3 adults) with CFD and TWS who underwent surgical management using a previously described round window reinforcement technique. Group 2 had 8 patients (2 children and 6 adults) with CFD who did not have surgical intervention.Interventions: The Dizziness Handicap Inventory (DHI) and Headache Impact Test (HIT-6) were administered pre-operatively and post-operatively. In addition, diagnostic findings of comprehensive audiometry, cervical vestibular evoked myogenic potential (cVEMP) thresholds and electrocochleography (ECoG) were studied. Symptoms before and after surgical intervention were compared.Main outcome measures: Pre- vs. post-operative DHI, HIT-6, and audiometric data were compared statistically. The thresholds and amplitudes for cVEMP in symptomatic ears, ears with cochlea-facial nerve dehiscence and ears without CFD were compared statistically.Results: There was a highly significant improvement in DHI and HIT-6 at pre- vs. post-operative (p < 0.0001 and p < 0.001, respectively). The age range was 12.8–52.9 years at the time of surgery (mean = 24.7 years). There were 6 females and 2 males. All 8 had a history of trauma before the onset of their symptoms. The mean cVEMP threshold was 75 dB nHL (SD 3.8) for the operated ear and 85.7 dB (SD 10.6) for the unoperated ear. In contrast to superior semicircular canal dehiscence, where most ears have abnormal ECoG findings suggestive of endolymphatic hydrops, only 1 of 8 operated CFD ears (1 of 16 ears) had an abnormal ECoG study.Conclusions: Overall there was a marked improvement in DHI, HIT-6 and symptoms post-operatively. Statistically significant reduction in cVEMP thresholds was observed in patients with radiographic evidence of CFD. Surgical management with round window reinforcement in patients with CFD was associated with improved symptoms and outcomes measures. |
| first_indexed | 2024-12-10T06:45:19Z |
| format | Article |
| id | doaj.art-7a735a58676d43e2b094dd6e5204b45e |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-10T06:45:19Z |
| publishDate | 2019-12-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-7a735a58676d43e2b094dd6e5204b45e2022-12-22T01:58:40ZengFrontiers Media S.A.Frontiers in Neurology1664-22952019-12-011010.3389/fneur.2019.01281502787Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve DehiscenceP. Ashley Wackym0Carey D. Balaban1Pengfei Zhang2David A. Siker3Jasdeep S. Hundal4Department of Otolaryngology–Head and Neck Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United StatesDepartments of Otolaryngology, Neurobiology, Communication Sciences & Disorders, and Bioengineering, University of Pittsburgh School of Medicine, Pittsburgh, PA, United StatesDepartment of Neurology, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United StatesSiker Medical Imaging and Intervention, Portland, OR, United StatesDepartment of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United StatesObjective: This communication is the first assessment of outcomes after surgical repair of cochlea-facial nerve dehiscence (CFD) in a series of patients. Pre- and post-operative quantitative measurement of validated survey instruments, symptoms, diagnostic findings and anonymous video descriptions of symptoms in a cohort of 16 patients with CFD and third window syndrome (TWS) symptoms were systematically studied.Study design: Observational analytic case-control study.Setting: Quaternary referral center.Patients: Group 1 had 8 patients (5 children and 3 adults) with CFD and TWS who underwent surgical management using a previously described round window reinforcement technique. Group 2 had 8 patients (2 children and 6 adults) with CFD who did not have surgical intervention.Interventions: The Dizziness Handicap Inventory (DHI) and Headache Impact Test (HIT-6) were administered pre-operatively and post-operatively. In addition, diagnostic findings of comprehensive audiometry, cervical vestibular evoked myogenic potential (cVEMP) thresholds and electrocochleography (ECoG) were studied. Symptoms before and after surgical intervention were compared.Main outcome measures: Pre- vs. post-operative DHI, HIT-6, and audiometric data were compared statistically. The thresholds and amplitudes for cVEMP in symptomatic ears, ears with cochlea-facial nerve dehiscence and ears without CFD were compared statistically.Results: There was a highly significant improvement in DHI and HIT-6 at pre- vs. post-operative (p < 0.0001 and p < 0.001, respectively). The age range was 12.8–52.9 years at the time of surgery (mean = 24.7 years). There were 6 females and 2 males. All 8 had a history of trauma before the onset of their symptoms. The mean cVEMP threshold was 75 dB nHL (SD 3.8) for the operated ear and 85.7 dB (SD 10.6) for the unoperated ear. In contrast to superior semicircular canal dehiscence, where most ears have abnormal ECoG findings suggestive of endolymphatic hydrops, only 1 of 8 operated CFD ears (1 of 16 ears) had an abnormal ECoG study.Conclusions: Overall there was a marked improvement in DHI, HIT-6 and symptoms post-operatively. Statistically significant reduction in cVEMP thresholds was observed in patients with radiographic evidence of CFD. Surgical management with round window reinforcement in patients with CFD was associated with improved symptoms and outcomes measures.https://www.frontiersin.org/article/10.3389/fneur.2019.01281/fullcochlea-facial nerve dehiscencecognitive dysfunctiondizzinessperilymph fistulaspatial disorientationsuperior semicircular canal dehiscence syndrome |
| spellingShingle | P. Ashley Wackym Carey D. Balaban Pengfei Zhang David A. Siker Jasdeep S. Hundal Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence Frontiers in Neurology cochlea-facial nerve dehiscence cognitive dysfunction dizziness perilymph fistula spatial disorientation superior semicircular canal dehiscence syndrome |
| title | Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence |
| title_full | Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence |
| title_fullStr | Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence |
| title_full_unstemmed | Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence |
| title_short | Third Window Syndrome: Surgical Management of Cochlea-Facial Nerve Dehiscence |
| title_sort | third window syndrome surgical management of cochlea facial nerve dehiscence |
| topic | cochlea-facial nerve dehiscence cognitive dysfunction dizziness perilymph fistula spatial disorientation superior semicircular canal dehiscence syndrome |
| url | https://www.frontiersin.org/article/10.3389/fneur.2019.01281/full |
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