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BACKGROUND The Say-Barber-Biesecker-Young-Simpson (SBBYS) variant of Ohdo syndrome is characterized by congenital hypothyroidism, facial dysmorphism, postaxial polydactyly, and mental retardation. The SBBYS variant of Ohdo syndrome is extremely rare with only 19 cases previously reported in the literature. A case is presented of chronic otitis media associated with cholesteatoma in a six-year-old boy with the SBBYS variant of Ohdo syndrome. CASE REPORT A 6-year-old boy presented with perforation of the tympanic membrane and a cholesteatoma in the mesotympanic-attic region associated with chronic otitis media. The child had previously been diagnosed with the SBBYS variant of Ohdo syndrome. Following computed tomography (CT) and magnetic resonance imaging (MRI), tympanoplasty was performed with removal of the lesion. CONCLUSIONS This is the first case described in the literature of chronic otitis media associated with cholesteatoma in a patient with the SBBYS variant of Ohdo syndrome. This case demonstrates the importance of specialist otolaryngology referral for patient management.

Objective: To compare the Galletti-Contrino manoeuvre with the more widely used Semont-Toupet in overweight subjects presenting with benign paroxysmal positional vertigo (BPBV) of vertical semicircular canals (posterior and anterior canals)., Study Design: Prospective cohort study., Patients: 204 patients (BMI range 25-30) with a diagnosis of BPPV of vertical semicircular canals were randomly divided in two groups treated with two different maneuvers: Galletti-Contrino (Group A) and Semont-Toupet manoeuvre (Group B). The results were compared with those obtained from a control group (204 non-overweight subjects with BPV of vertical semicircular canals.) INTERVENTION: Galletti Contrino/ Semont Toupet manoeuvres., Main Outcome Measure(s): Liberatory nystagmus or vertigo after maximum 2 maneuvers. Vertigo and dizziness intensity scores (Visual analogue scale VAS 0-10) from day 0 to day 5 following the repositioning manoeuvre were also recorded in responsive patients., Results: While in non-overweight subjects no significant difference comparing the effectiveness of the two manoeuvres was found, liberatory nystagmus and vertigo were more frequently observed after Galletti Contrino manoeuvre in overweight subjects; this difference was statistically significant when posterior canals were involved (P < 0.03). Vertigo and dizziness VAS scores reduced significantly from day 0 to day 5 after therapy in all groups. A more significant reduction of dizziness VAS was recorded in patients undergoing Galletti-Contrino manoeuvre at days 4-5 (P < 0.005)., Conclusion: Galletti-Contrino manoeuvre seems to be significantly more effective than Semont-Toupet manoeuvre in the treatment of BPPV of posterior semicircular canal and may be preferential in patients with limited body movements.

The role of cVEMPs and vHIT in the evaluation of otosclerosis and its eventual vestibular impairment: preliminary findings., Objectives: Otosclerosis is one of the most common causes of hearing loss in adults, with a prevalence of 0.3% to 0.4% in Caucasians. Vestibular symptoms may occur, with an incidence ranging between 5% and 57% of patients. The aim of our study is to evaluate the vestibular function and its eventual changes after stapes surgery in patients affected by otosclerosis., Methods: Prospective case-control study. Twenty patients (17 females; age range 33-58; mean age 44) who underwent surgery for otosclerotic disease between April 2012 and February 2014 were prospectively studied. These patients underwent preoperative and postoperative audiological tests. Furthermore, vestibular function was evaluated using the cervical evoked myogenic potentials test (cVEMPs) and video head impulse test (vHIT), preoperatively and postoperatively. A case-control study was also performed. Quantitative and statistical analysis of patients' vestibular function was carried out both before and after stapes surgery., Results: The means of the vHIT gains in the case group were 1.03 on the right side and 1.01 on the left side. A significant difference between case and control groups was seen, with a lower left gain registered in the control group. No cases with a gain of less than 0.8 were found in either group. Moreover, a significant postoperative reduction in P1/NI amplitude was seen in patients complaining of postoperative dizziness or vertigo., Conclusions: These findings indicate a probable traumatic saccular impairment in patients with vestibular symptoms. However, a longer follow-up may help in understanding the behaviour of cVEMPs in post-stapes surgery vertigo.