Object Tinnitus is a known presenting symptom of acoustic neuromas, but little is known about the impact of observation or treatment on tinnitus. Most patients experience improvement with treatment, while others may worsen. Therefore, this study was designed to assess the overall impact of observation and treatment on tinnitus outcome in patients with acoustic tumors. Methods Data from the 2007–2008 Acoustic Neuroma Association survey were used. Tinnitus severity was graded both at presentation and at last follow-up for all patients questioned. This data set was analyzed using the Student t-test and a linear regression model adjusted for possible confounders. Results Overall there were more patients receiving intervention (n = 1138) for their acoustic neuromas than observation (n = 289). Presenting tumor size positively correlated with tinnitus severity score. Regardless of treatment (microsurgery or stereotactic radiosurgery), tinnitus improved at last follow-up and worsened in those who were observed (p = 0.02). When comparing microsurgical options, retrosigmoid and translabyrinthine resection improved tinnitus symptoms (both p < 0.01). Stereotactic radiosurgery had a treatment effect similar to microsurgery. Conclusions Presenting tinnitus severity correlates strongly with tumor size. Furthermore, regardless of treatment, there appears to be an overall reduction in tinnitus severity for all forms of microsurgery and stereotactic radiosurgery. Importantly, observation leads to a worsening in symptomatic tinnitus and therefore should be weighed in the treatment recommendation.

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Malformations in either the inner ear, vestibulocochlear nerve (VIIIth) or auditory cortex of the brain can lead to congenital sensorineural hearing loss (SNHL). In most cases the underlying disorders involve the membranous labyrinth at a microscopic level and therefore radiological examinations are entirely normal. In a significant proportion however (up to 20%), there are abnormalities visualized in the inner ear and/or the VIIIth nerve; the type of abnormality is relevant for the surgical planning of a cochlear implant. Imaging and the accurate radiological identification of the affected inner ear structures therefore plays an integral role in the clinical evaluation of sensorineural hearing loss. In this pictorial review, we describe the main malformations of the inner ear in view of recent classifications and briefly explore the surgical implications.

To describe in detail the temporal bone and brain findings in both common and rare syndromic causes of hearing loss, with the purpose of broadening among radiologists and enhance the current understanding of distinct imaging features in paediatric patients with syndromic hearing loss. Methods: A detailed search of electronic databases has been conducted, including PubMed, Ovid Medline, Scopus, Cochrane Library, Google Scholar, National Institute for Health and Care Excellence (NICE), Embase, and PsycINFO. Results: Syndromic causes of hearing loss are characterised by different and sometimes specific abnormalities in the temporal bone. Conclusion: A complete knowledge of the image findings in the temporal bones, brain, skull and other body regions is critical for the optimal assessment and management of these patients. 2.1. Inner ear The inner ear development starts late in the 3rd week of gestation, with the formation of the otic placode (an ectodermal thickening on both sides of the developing head).The otic placode later invaginates to form otic pits which then fuse to form the otic vesicle. By day 26, the ventral region of otic vesicle differentiates into the saccule, the lower pole of which gives rise to the cochlear duct, which will form 2.5 turns by the 8th week. During the 7th week, cochlear duct epithelial cells differentiate to form the Organ of Corti. The mesenchyme surrounding the cochlear

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