Endolymphatic Sac Tumor as a Ménière-Like Vertiginous Syndrome: A Case Report.

Endolymphatic sac tumors are rare benign neoplasms with locally aggressive behavior located in the posterior petrous ridge of the temporal bone. They cause sensorineural hearing loss and may develop vestibular damage. A 24-year-old male patient arrived at our office with a history of acute vertiginous syndrome, left hearing loss, and tinnitus 1-year ago. His chief complaint was an increase in auditory symptoms. A CT scan and MRI showed an endolymphatic sac tumor. Complete resection of the lesion was achieved by a transmastoid and translabyrinthine approach. Low-grade adenocarcinoma was confirmed by histopathology. The patient remained without clinical vestibular symptoms. However, a small residual tumor was addressed by gamma-ray radiosurgery. Postoperative deep left sensorineural hearing loss was identified, without any vestibular sequelae. Radiologic imaging is the most useful tool for this diagnosis. Endolymphatic sac tumors should be in the differential diagnosis of recalcitrant audio-vestibular symptoms. Complete surgical resection is the most appropriate management.

Extended high-frequency audiometry as early detection of hearing loss in primary Sjögren syndrome.

The aim of the study is to evaluate hearing function by extended high-frequency audiometry in primary Sjögren Syndrome (pSS) and to correlate it with disease activity. All patients diagnosed with pSS were recruited within 1 year. Patients underwent otologic evaluation, extended high-frequency audiometry, tympanogram, and the ESSPRI scale evaluation. A total of 63 (60 female and 3 male) patients with mean age 49.5 ± 9.9 years with pSS were included. We detected a hearing loss prevalence of 95.2% (60 patients) in both ears in very high frequencies (10000-16,000 Hz). The hearing loss of all patients was sensorineural. There was no significant difference between the degree of disease activity and the hearing thresholds. We enrolled 188 subjects with a mean age of 47.20 ± 9.7 years as a healthy control group. We compared the hearing thresholds of both ears of patients with pSS versus the healthy control group and observed a significant difference in frequencies above 500 Hz. Sensorineural hearing loss was found in pSS patients in frequencies above 4000 Hz. Sensorineural hearing loss was highly prevalent in pSS patients, predominantly in very high frequencies. We claim that sensorineural hearing loss could be an associated symptom of primary Sjögren syndrome and suggest further research to support this conclusion.