Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature.

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination.

We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT.

Osteomas of the internal auditory canal (IAC) are rare pathologies and diagnosis requires thin-slice computed tomography imaging of the skull or magnetic resonance imaging. We report a case of bilateral florid osteomas in the IAC resulting in tinnitus and vertigo. A literature review identified 29 publications, the majority case reports, meeting our criteria involving 40 patients. We examined symptomatology, laterality, diagnostic imaging, and management strategies. Regarding diagnosis, our findings affirm the importance of thorough clinical and radiological investigations. Of utmost importance, is the assessment of patient symptoms on an individual basis to determine treatment goals, as both conservative and surgical management options have risks and benefits. While conservative treatment remains appropriate for the majority of patients, those with more severe symptoms do experience benefit from a surgical strategy. Careful case selection and management of patient expectations continue to feature as a mainstay of successful treatment. This report aims to contribute to the growing body of literature on osteomas of the IAC and ultimately guide the establishment of evidence-based nationally recognized guidelines to improve care and outcomes in patients with this rare condition.