Facial nerve stimulation in adult cochlear implant recipients with far advanced otosclerosis.

Objectives: The objective of this study was to predict occurrence of facial nerve stimulation (FNS) in cochlear implanted patients for far-advanced otosclerosis (FAO) by correlating preoperative computed tomography (CT)-scan data to FNS and to evaluate FNS impact on hearing outcomes. Methods: Retrospective analysis on 91 ears (76 patients) implanted for FAO. Electrodes were straight (50%) or perimodiolar (50%). Demographic data, extension of otosclerosis on preoperative CT scan, occurrence of FNS, and speech performance were analyzed. Results: Prevalence of FNS was 21% (19 ears). FNS appeared during the first month (21%), 1-6 months (26%), 6-12 months (21%), and over 1 year (32%) postimplantation. Cumulative incidence of FNS at 15 years was 33% (95% CI = [14-47%]). Extension of otosclerotic lesions on preimplantation CT-scan was more severe in FNS ears compared to No-FNS (p < .05): for Stage III, 13/19 (68%) and 18/72 (25%) ears for FNS and No-FNS groups, respectively (p < .05). Location of otosclerotic lesions relative to the facial nerve canal was similar whatever the presence or not of FNS. Electrode array had no impact on FNS occurrence. At 1 year post-implantation, duration of profound hearing loss (≥5 years) and previous stapedotomy were negatively associated with speech performance. FNS did not impact hearing outcomes, despite a lower percentage of activated electrodes (p < .01) in the FNS group. Nevertheless, FNS were associated with a decrease of speech performance both in quiet (p < .001) and in noise (p < .05). Conclusion: Cochlear implanted patients for FAO are at greater risk of developing FNS affecting speech performance over time, probably due to a higher percentage of deactivated electrodes. High resolution CT-scan is an essential tool allowing FNS prediction but not time of onset. Level of evidence: 2b, Laryngoscope Investigative Otolaryngology, 2022.

Internal auditory canal osteoma: Case report and review of the literature.

We report a case of internal auditory canal osteoma and discuss this entity's etiology, natural history, and treatment options. The internal auditory canal osteoma is a rare entity with only a few reports published in the medical literature. Its diagnosis is based on two complementary imaging modalities: thin-slice computed tomography and magnetic resonance imaging. No consensus exists regarding the treatment of this entity, and treatment should be tailored to each patient depending on that patient's initial complaints, as well as his or her medical findings.

Temporal bone central giant-cell granuloma presenting as a serous otitis media.

Central giant cell granuloma is a benign intraosseous lesion that most commonly occurs in the facial bones. Its location in the temporal bone is extremely rare and only 20 cases have been reported in the literature. We report a case of an adult female patient presenting with a right serous otitis media and mastoiditis associated with a mixed hearing loss during 6 months. CT-scan and MRI revealed a temporal bone tumor involving the mastoid, and surrounding the right temporo-mandibular joint. Tumor was totally removed after a canal-wall-down mastoidectomy and middle ear exclusion. Pathology revealed a central giant cell granuloma. Seven months following the surgery there was no evidence of recurrence. Central giant cell granuloma is a rare temporal bone lesion, with non specific clinical and imaging signs but characteristic pathological features. Today, a total surgical removal and regular MRI follow-up is the best management option.

Diagnosis and management of intracochlear schwannomas.

OBJECTIVE: To describe and analyze a series of intracochlear schwannomas. METHODS: From 1987 to 2005, 19 patients with schwannomas involving the cochlea were included in this series. Clinical, audiovestibular, and imaging data concerning initial and follow-up visits were collected. RESULTS: The mean age was 54 years (range, 25-71 yr). The series comprised 10 women and 9 men, 18 solitary tumors and 1 neurofibromatosis Type 2 patient. The mean diagnosis delay was 11 years (range, 1-31 yr). At diagnosis, a total or profound hearing loss (Class D of American Academy of Otolaryngology-Head and Neck Surgery classification) was noted in 17 patients (89%), and a severe loss (Class C) was reported in 2 patients (11%). Facial paresis was reported in 2 patients (11%; Grades 2 and 4 of House and Brackmann classification). Magnetic resonance imaging showed an involvement of the posterior labyrinth in 8 patients (37%), an extension of the schwannoma to the internal auditory meatus in 8 patients (37%), and a cerebellopontine angle extension in 7 patients (32%). In 11 patients (58%), the schwannoma was removed through a transotic route. In the 8 remaining patients, a watch-and-rescan policy was decided. The postoperative course was uneventful. Postoperative follow-up period was 27 months (range, 6-88 mo). Postoperative facial function was assessed as Grade 1 in 7 patients, as Grade 2 in 4, and as Grade 4 in 1 patient. CONCLUSION: The diagnosis of intracochlear schwannomas can be difficult on magnetic resonance imaging. The diagnosis should be considered in all unilateral hearing losses.

Atypical haemangioma of the internal auditory meatus: a case report.

Haemangiomas involving the internal auditory meatus (IAM) are rare and can mimic other frequent lesions of the IAM such as schwannomas by their clinical and imaging aspects. The case of a patient with an atypical IAM haemangioma fluctuating in size is reported to highlight this diagnostic possibility. A 36-year-old female presented with a sudden and recurrent left sensorineural hearing loss (SNHL). Three consecutive MRIs were performed in a 10-month period of preoperative observation. They showed a fluctuation of the tumour signal and size. The surgical removal was performed via a translabyrinthine approach. Pathological findings were consistent with the diagnosis of a heamangioma. MRI and CT findings suggested the diagnosis of IAM heamangioma, but the rapid variation in size and signal was misleading. This phenomenon may be due to haemorrhage or oedema, and can be accompanied by a deterioration of the hearing function. Early surgical resection is the treatment of choice and allows to confirm the diagnosis.

Langerhans' cell histiocytosis of the labyrinth in adults.

OBJECTIVE: To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS: Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone. INTERVENTION: Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy. RESULTS: The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred. CONCLUSION: Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.

Auditory brainstem implant in bilateral and completely ossified cochleae.

OBJECTIVE: To report and evaluate the results of auditory brainstem implantation in a case of postmeningitis hearing loss with totally ossified cochleae on both sides. STUDY DESIGN: Case report. SETTING: Tertiary referral center. PATIENT: A 49-year-old man was referred to the authors' department for bilateral total hearing loss subsequent to bacterial meningitis 2 years earlier. Pure tone audiometry, auditory brainstem response tests, and promontory tests did not reveal any cochlear activity. Computed tomography showed bilateral and totally ossified cochleae. Magnetic resonance imaging confirmed the diagnosis by showing no cochlear signal on T2-weighed images and ruled out brainstem and cerebellopontine angle abnormalities. INTERVENTION: A left auditory brainstem implantation was performed through a translabyrinthine route, using a Nucleus 22-channel device. MAIN OUTCOME MEASURES: Word and sentence recognition tests in sound-only and sound plus vision modes. RESULTS: No postoperative complication was observed. Twelve electrodes could be activated, and their tonotopy was defined. Nine other electrodes were inactivated because of an absence of auditory response (4 electrodes) or paresthesia (5 electrodes). At the last follow-up visit, 26 months after the implantation, 50% of disyllabic word scores and 60% of sentence scores were achieved using auditory brainstem implant sound only. These scores reached 80% and 93%, respectively, with lip-reading. CONCLUSION: Auditory brainstem implantation is an efficient means of auditory rehabilitation in cases of bilateral total hearing loss with totally ossified cochleae. It should be considered in cases of predictable failure in cochlear implantation.

Management of cholesterol granulomas of the petrous apex based on clinical and radiologic evaluation.

OBJECTIVE: The purpose of this study was to analyze the imaging characteristics of 12 cholesterol granulomas as a function of their clinical symptoms. The results of the different surgical approaches and the management of these lesions are discussed. STUDY DESIGN: Retrospective case review. SETTING: Five tertiary referral centers. PATIENTS: Twelve patients managed for a cholesterol granuloma of the petrous apex. INTERVENTIONS: All patients were evaluated via computed tomography and magnetic resonance imaging. Eight patients required surgical drainage: through a conservative approach in seven patients (infralabyrinthine, n = 5; infracochlear, n = 2) and a transotic approach in one patient. Clinical and radiologic follow-up without surgery was the mode of treatment for four patients. The mean follow-up period was 18 months for patients who underwent operations and ranged from 6 months to 10 years for patients without operations. RESULTS: Four patterns of clinical symptoms were noted: retrocochlear signs by an involvement of the internal auditory meatus (n = 8), headaches by a traction of the dura (n = 4), serous otitis media by a compression of the eustachian tube (n = 2), and asymptomatic lesions with no involvement of the adjacent structures (n = 2). Hearing and facial functions were preserved in all the cases treated by a noninvasive procedure. No recurrence or complication was reported in the patients who underwent operations. None of the noninvasively treated patients with cholesterol granulomas showed significant enlargement on follow-up imaging. CONCLUSION: Clinical manifestations of cholesterol granulomas depend on their anatomic location and the involvement of the adjacent structures. Aggressive lesions in patients with residual hearing can be drained via an infralabyrinthine or an infracochlear approach with minimal morbidity. Follow-up must be preferred for patients with nonaggressive lesions. Although magnetic resonance imaging provides a specific diagnosis tool for cholesterol granulomas, computed tomography is essential for an accurate evaluation of the location of the cyst and choice of the surgical procedure.