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    Outcomes of long-term audiological rehabilitation in charge syndrome

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    The aim of this paper is to assess the long-term audiological features and outcomes of hearing rehabilitation in a large group of individuals with CHARGE Syndrome. The study has been conducted retrospectively, on a paediatric patient database, at the Audiology Department of the University Hospitals of Ferrara and Padua. The study sample included 31 children presenting with different degrees of hearing impairment associated with CHARGE syndrome. Hearing was assessed using auditory brainstem responses (ABRs) and/or electrocochleography, or conditioned audiometry (visual reinforcement audiometry [VRA] or play audiometry). Auditory-perceptual outcomes in terms of communication skills and expressive language were also recorded. The effects of hearing rehabilitation (with hearing aids or cochlear implants) in this group of children and language outcomes after rehabilitation were monitored during long-term follow-up. The outcomes of rehabilitation measures differed in relation to the heterogeneous and often severe disabilities associated with CHARGE syndrome, e.g. developmental delay, intellectual delay, visual impairment, thin 8th nerve with retrocochlear auditory dysfunction (as described in cases of auditory neuropathy/dyssynchrony). Oral expressive language was severely impaired in most cases, even after lengthy follow-up, suggesting the need for alternative augmentative communication modes. The early identification of sensorineural hearing loss, and carefully planned rehabilitation treatments, can be of some benefit in children with CHARGE syndrome

    Cochlear-vestibular disfunction and sudden cardiac sopraventricular arrhytmia: case report

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    Fractures to the petrous bone are a subject of great clinical interest and are often responsible for a large number of complications. Petrous bone fractures result from cranial injuries and, less frequently, can result from sporting injuries. The authors describe the case of a 55-year-old patient who presented with an episode of subjective vertigo, nausea, right ear fullness and tinnitus, associated with haemorrhage from the ear, auricular pain and retroauricular tension on the right. An initial ECG revealed a supraventricular tachysystolia with a ventricular response of 150 bpm, right bundle branch block (RBBB) and incomplete left bundle branch block (ILBBB) with subsequent spontaneous recovery of the normal sinus rhythm with RBBB and the presence of ILBBB. Audiological evaluation showed the presence of a right cochleovestibulopathy. A successive CT of the petrous/mastoid bones revealed the presence, on the right, of a series of multiple fractures to the mastoid, without osseous compression. Investigations carried out during hospitalization correlated with the audiological symptoms resulting from a fracture of the right petrous bone provoked by a fall due to a syncopal episode. The authors wish to stress the importance of a careful multidisciplinary evaluation in cases of disordered cochleovestibular function of a post-traumatic nature which cannot be immediately linked to known sources. © 2006 Taylor & Francis

    Immune-mediated inner ear disease

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    The incidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for <1% of all cases of hearing impairment or dizziness. Nevertheless, the diagnosis of AIED might be overlooked because of the lack of a specific diagnostic test. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss (SNHL) over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25-50% of patients also have tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15-30% of patients
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