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Purpose Most of Superior Semicircular Canal Dehiscence (SSCD) are located in the apical region of the SSC. However, in a small number of cases, it may be situated in the medial wall, causing the SSC to contact with the superior petrosal sinus (SPS). The aim of this study is to describe four patients with SSCD involving the superior petrosal sinus (SSCD-SPS) and to perform a review of the literature. Methods Observational retrospective study of patients diagnosed of SSCD-SPS in a tertiary referral center. A systematic review was made, identifying 7 articles in the literature. Clinical presentation, complementary test (pure-tone audiometry, PTA; vestibular evoked myogenic potential, VEMP; computed tomography, CT), therapeutic management and outcomes were reported. Results Four new cases of SSCD-SPS are reported, in three of them a transmastoid plugging was performed. 54 patients with SSCD-SPS (57 dehiscences) were reported in the literature. The most frequent symptoms were aural pressure (57.41%) and vertigo provoked by pressure/Valsalva (55.55%). Conductive hearing loss was the most common finding in PTA (47.37%). Abnormally low thresholds were observed in 59.46% of reported VEMP. Transmastoid approach was used in ten cases, middle fossa approach in four, round window reinforcement in one, and occlusion of the SPS using coils in two. Conclusions Within SSCD, we have encountered a rare subtype characterized by its medial wall location in close proximity to the SPS. This subgroup needs special consideration as it has shown its own distinct characteristics. Regarding therapeutic management, we advocate a transmastoid approach.

Objectives Facial nerve palsy is one of the most important complications of vestibular schwannoma resection. When VII cranial nerve is damaged, ocular complications might appear, impacting patient´s quality of life. Therefore, standardized eye care must be a priority. This involves three key treatments: topical treatment, rehabilitation and oculoplastic surgery. The aim of this work is to review the results of oculoplastic surgery performed simultaneously with vestibular schwannoma resection in terms of vision-related quality of life, compared to deferred surgery. Design The study involved 177 patients who underwent vestibular schwannoma resection between 2015 and 2022. The incidence of facial palsy was registered. Of those patients who had the palsy, we selected 35 who also had oculoplastic surgery. 5 of them declined participating in the study. The final sample ( n  = 30) was divided in two groups depending on the timing of oculoplastic surgery: simultaneously- first group ( n  = 15) and deferred-second group ( n  = 15). The vision-related quality of life was measured using the NEI VFQ-25 test. Results 26.56% patients developed an immediate postoperative facial dysfunction, decreasing to 18.08% after one year of follow-up. Timing of oculoplastic surgery was associated with better results in quality of life; the first group of patients had a better quality of life (70.27/100) compared to the second group (53.73/100; p  = 0.006). Moreover, worse results in quality of life were also associated with long-term postoperative facial palsy ( p  = 0.042). Current criteria for selecting patients were reliable, proving adequacy as we found worse long-term facial functions in patients who underwent simultaneous surgery ( p  = 0.01). Conclusions Our current criteria for selecting candidates for simultaneous oculoplastic surgery are effective. When long-term facial nerve dysfunction is expected during vestibular schwannoma resection, oculoplastic surgery should be performed simultaneously to preserve the q vision-related quality of life. Long-term severe facial palsy is associated with poor vision-related quality of life.