Gianluca Piras

Waldenström's macroglobulinemia presenting with bilateral vestibular loss: a case report

Waldenstrom’s macroglobulinemia (WM) is a low-grade lymphoma originally described in 1944. It is a malignant lymphoproliferative disease characterized by the clonal expansion of B cells with lymphoplasmacytic differentiation that secrete monoclonal immunoglobulin M (IgM). Diagnostic criteria are based on specific clinical, morphological, and immunophenotypic parameters, besides the evidence of pathologic plasmacytoid cells in the bone marrow. Symptoms can be related either to tumor infiltration or to the amount and properties of the circulating monoclonal protein: cardiac and renal failure, mucosal bleeding, headache, visual disturbances, ataxia, and eventually coma have been described as part of the clinical spectrum of WM. Peripheral neuropathy occurs in nearly half of the patients and is mostly related to the reactivity of the IgM protein with different neural antigens. Frequently, it represents the initial presentation of the disease; sometimes it could precede the diagnosis of macroglobulinemia by several years.

The treatment of superior semicircular canal dehiscence: A review of the literature about a not completely clarified problem

Abstract Objective: This paper reviews the principal surgical strategies proposed in the literature in approaching the Superior Semicircular Canal Dehiscence (SSCD) syndrome. It examines the controversies regarding each treatment modality including surgical indications, procedures and the main outcomes. Design: A systematic literature research was conducted using a number of resources including electronic databases, books and websites. Relevant citations from current publications were also followed up. Results: Two principal types of surgical procedure for the treatment of superior canal dehiscence were identified: canal plugging and resurfacing. Each can be performed by both a middle cranial fossa (MCF) and transmastoid (TM) approach. An alternative and less invasive option to treat this pathological condition, without repairing the underlying lesion, has recently emerged from the literature: the round window (RW) niche reinforcement through a transmeatal approach. Conclusions: All the reported techniques represent reliable and effective solutions in cases of intolerable symptoms; nevertheless, the controversies still present about the pathophysiological changes linked to dehiscence reflect the difficulties in proposing a universal method of treatment. It is, therefore, probable that a more prolonged knowledge of this affection is necessary to fully understand all its implications.

Color Atlas of Endo-Otoscopy: Examination-Diagnosis-Treatment

This a topic dear to my heart, the end result being my redundant collection of over three-thousand slide transparencies, a few hundred of which I did manage to digitise, and a collection of 35 mm film cameras that do not even have scrap value now. The opening chapter describes how such images are now captured in the digital age. It does carry a nice illustration of ‘a set up used in past years’, exactly the kit I once used. Fortunately, most of us do now have access to the modern visual aids illustrated in the following Figure 1.7. A nice Preface tells us that ‘Otoscopy alone can establish the diagnosis in some cases, parameters such as history and audiological and neuroradiological evaluation are required in others’. Otoscopy is a bit like oral surgery or even dermatology, where pattern recognition and decades of experience can make all the difference to correct and instant diagnosis. The authors do advise of course that what you see, the red blush, the white bulge, the fluid-filled ear, is ‘the tip of the iceberg’. This book is far more comprehensive than the title, or even the cover, suggests. I expected to flick through countless pictures of typical tympanic membrane and middle-ear lesions, and, with over a thousand illustrations, there are plenty of those. There are countless computed tomography scans, nicely printed and clearly labelled; there are operative images, taken through the microscope, that are of superb quality. There is surprisingly detailed text on the underlying pathology, staging and even management of the various disease processes covered. A typical example is surgery of external canal exostoses, and I was relieved to read that the authors share my success rate in surgicalmanagement of post-inflammatory canal stenosis. They advise against it! I was struck by the coverage of external canal carcinoma, offering differential diagnosis, staging and detailed diagrams of tumour extension, in what is called simply an atlas. The blue drum of cholesterol granuloma is not easily captured, but nicely shown here. Indeed, middle-ear effusion is illustrated, but with farmore coverage of the countless weird and wonderful skull base tumours that may be responsible. Again, one expects nice views of ossicular disorders through perforations or retractions, of cholesteatomas and of middle-ear masses. The surprise is the coverage of tympanoplasty techniques and the excellent microscopy images of mastoidectomy (Figures 8.73–8.111). Chapter 12, ‘Rare Retrotympanic Masses’, reports precisely such, the really obscure, probably reflecting the group’s experience over 30 years, of 32 000 operations and 300 000 consultations! The final chapter ‘Postsurgical Conditions’ shows an unconvincing Schwartz sign, which I will forgive as challenging to capture, with a series of failed tympanoplasties and extruding prostheses. I had expected to see more coverage of otoendoscopic surgery of the ear, so increasingly popular amongst the younger surgeons. Instead, this book is very much geared towards the diagnostic uses of otoendoscopy. Overall, this book is invaluable to trainees, from the youngest medical students to the over sixties, who still have much to learn, I find.

Superior canal dehiscence with tegmen defect revealed by otoscopy: Video clip demonstration of pulsatile tympanic membrane

Superior canal dehiscence is a pathologic condition of the otic capsule acting as aberrant window of the inner ear. It results in reduction of inner ear impedance and in abnormal exposure of the labyrinthine neuroepithelium to the action of the surrounding structures. The sum of these phenomena leads to the onset of typical cochleo-vestibular symptoms and signs. Among them, pulsatile tinnitus has been attributed to a direct transmission of intracranial vascular activities to labyrinthine fluids. We present the first video-otoscopic documentation of spontaneous pulse-synchronous movements of the tympanic membrane in two patients with superior canal dehiscence. Pulsating eardrum may represent an additional sign of third-mobile window lesion.