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.

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.

About 20% of children with congenital hearing loss present malformations of the inner ear. In the past few years much has been understood about the morphology and function of the anterior part of the labyrinth, since hearing loss may have a dramatic effect on the overall development of a child. Nowadays, for most of them, a chance for hearing rehabilitation is available, making hearing loss a treatable condition. The anomalies range from the lack of development of the whole inner ear to specific anomalies of isolated structures. Despite the frequent concomitant involvement of the posterior part of the labyrinth, this part of the inner ear is frequently neglected while discussing its morphology and dysfunction. Even though vestibular and balance function/dysfunction may have a significant impact on the global development of children, very little is known about these specific disorders in patients with inner ear malformations. The aim of this review is to summarize the available literature about vestibular anomalies and dysfunctions in children with inner ear malformations, discussing what is currently known about the topic.

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.