Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon?

Objective: The clinical features and surgical results of "closed type" versus "open type" congenital cholesteatoma were compared in order to analyse the differences between the two forms; whether the morphology of the disease may have a role in the staging systems has been also evaluated.

Patients And Methods: We reviewed retrospectively 95 patients (96 ears) who underwent surgery for congenital cholesteatoma over a 15-year period focusing on the clinical differences between open and closed type congenital cholesteatoma.

Results: Seventy-one patients (74%) had a closed-type and 25 (26%) an open type congenital cholesteatoma.

Cochlear implantation in patients with Cogan syndrome: long-term results.

The objective of this study was to evaluate the long-term outcomes of patients with Cogan syndrome (CS) who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of CS. Measures included word and sentence recognition scores.

Pneumolabyrinth secondary to temporal bone fracture: a case report and review of the literature.

Pneumolabyrinth following temporal bone fracture is an extremely rare condition. It results from air entering the inner ear when a communication between the air-filled middle ear spaces and inner ear is established. The imaging modality of choice for pneumolabyrinth is high-resolution computed tomography of the temporal bone.

Pediatric cochlear implantation: an update.

Deafness in pediatric age can adversely impact language acquisition as well as educational and social-emotional development. Once diagnosed, hearing loss should be rehabilitated early; the goal is to provide the child with maximum access to the acoustic features of speech within a listening range that is safe and comfortable. In presence of severe to profound deafness, benefit from auditory amplification cannot be enough to allow a proper language development.

Cogan's syndrome: anti-Hsp70 antibodies are a serological marker in the typical form.

Background: Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss. CS is divided into a "typical" form with non-syphilitic interstitial keratitis and audiovestibular symptoms, and an "atypical" form with ocular involvement affecting structures other than the cornea. Anti-Hsp70 antibodies were found at variable levels in patients presenting with various forms of autoimmune sensorineural hearing loss (ASNHL).

Acquired middle ear cholesteatoma in children with cleft palate: experience from 18 surgical cases.

Objectives: To review an institutional experience with the surgical management of middle ear cholesteatoma in children with cleft palate.

Materials And Methods: We analyzed retrospectively 18 children diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma between 2000 and 2007. The following data were recorded: age, sex, history of ventilation tube insertion, status of the contralateral ear, cholesteatoma location and extension, and surgical technique involved.

Long-term results of external auditory canal closure and mastoid obliteration in cochlear implantation after radical mastoidectomy: a clinical and radiological study.

Cochlear implant candidates with mastoid cavity present a significant challenge to safe cochlear implantation because of possible spread of infection to the inner ear as well as an increased risk of electrode array extrusion. Closure of the external auditory canal is one of the several surgical techniques utilized to block the potential entry routes for infection and to protect the implanted device. The main concern after external auditory canal closure is the risk of developing a cholesteatoma, which can lead to an asymptomatic erosion of the temporal bone and/or cochlear implant failure.

Cochlear implantation in pontine tegmental cap dysplasia.

Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present.

Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report.

Background: Rituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogan's syndrome (CS).

Case Presentation: a 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy.

The windowed sound therapy: a new empirical approach for an effectiv personalized treatment of tinnitus.

We auditorily stimulated patients affected by subjective tinnitus with broadband noise containing a notch around their tinnitus frequency. We assessed the long-term effects on tinnitus perception in patients listening to notched noise stimuli (referred to as windowed sound therapy [WST]) by measuring the variation of subjects' tinnitus loudness over a period of 2-12 months. We tested the effectiveness of WST using non-notched broadband noise and noise of water as control sound therapies.

Main peak interleaved sampling (MPIS) strategy: effect of stimulation rate variations on speech perception in adult cochlear implant recipients using the Digisonic SP cochlear implant.

Conclusion: Data obtained from a cohort of 10 post-lingually deaf adult patients indicated that use of a higher stimulation rate, in the setting of the main peak interleaved sampling (MPIS) strategy coupled with the Neurelec-MXM Digisonic SP cochlear implant system, gives a significant advantage in terms of speech perception if compared with a lower rate, especially in a noisy environment.

Objectives: To evaluate speech recognition performances in post-lingually deaf adult cochlear implant recipients using two different stimulation rates (260 pps/e and 600 pps/e) in the setting of the MPIS strategy combined with the MXM-Neurelec Digisonic SP cochlear implant system.

Patients And Methods: Ten post-lingually deaf adults who consecutively received a Neurelec-MXM Digisonic SP device at the CI Center of the University of Parma participated in the study.

Cochlear implantation in children with cerebral palsy. A preliminary report.

Objectives: The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy.

Methods: This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study.

Nasal polyposis in Churg-Strauss syndrome.

Objectives: Churg-Strauss syndrome (CSS) is a systemic vasculitic disorder of unknown etiology that affects small-to-medium-size blood vessels. Patients affected by CSS frequently show ear, nose, and throat manifestations, which are often present at the time of disease onset. The purpose of this study was to determine the frequency of nasal polyposis in a series of 29 patients with CSS and to correlate the nasal findings to the total health situation of these patients.

Reconstruction of outer attic wall defects using bone paté: Long-term clinical and histological evaluation.

The objective was to evaluate the long-term clinical outcome of the bony outer attic wall reconstruction (scutumplasty) by using autogenous bone paté in a series of patients who underwent canal wall up (CWU) mastoidectomy for middle ear cholesteatoma. In addition, an histological analysis of bone paté graft specimens taken during the second-look procedure 12 months after the first surgery, was carried out. A series of 134 ears from 128 patients with cholesteatoma who underwent staged CWU mastoidectomy and attic reconstruction with bone paté, between 1995 and 2000 inclusive, was retrospectively analyzed.

Ear, nose and throat manifestations of Churg-Strauss syndrome.

Conclusion: Ear, nose and throat (ENT) involvement is common in Churg-Strauss syndrome (CSS), usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. Otolaryngologists may play a pivotal role in making an early diagnosis of this disease.

Objectives: CSS is a systemic vasculitic disorder that affects small to medium-sized blood vessels.

Surgical treatment of middle ear cholesteatoma in children with Down syndrome.

Objectives: To report our personal experience in the surgical treatment of cholesteatoma in children with Down syndrome.

Study Design: Retrospective study.

Setting: Tertiary care otology and skull base centers.

Cochlear implantation in a human immunodeficiency virus-infected patient.

Objectives/hypothesis: Patients infected with HIV have an increased risk of developing sensorineural hearing loss (SNHL), yet pathogenesis of SNHL in HIV infection is still poorly understood. In subjects affected by bilateral profound or total SNHL, cochlear implantation may be the only possibility to restore a hearing level that allows them to have an acceptable quality of life.

Study Design: Case report.

Comparison of speech perception performance between the Nucleus 24 and Nucleus 24 Contour cochlear implant systems.

Objective: To compare the 1-, 3-, and 6-month postoperative speech perception scores obtained by a group of subjects who received the new perimodiolar array (Nucleus Contour) cochlear implant with those obtained by a group of subjects implanted with the straight electrodes of the previous-generation Nucleus 24 device.

Material And Methods: The speech performance of 10 postlingually deafened adults implanted with the Nucleus Contour device was compared with that of matched controls who received the Nucleus 24 model. Objective measures included word and sentence speech recognition scores.

Reflux esophagitis as a possible risk factor in the development of pharyngolaryngeal squamous cell carcinoma.

Aims And Background: To determine the role of reflux esophagitis in the development of pharyngolaryngeal squamous cell carcinoma in non-smoking and non-drinking patients.

Methods: The study population consisted of 92 consecutive non-smoking and non-drinking patients with histologically confirmed squamous cell carcinoma of the pharynx and the larynx. As a control, a group of 125 lifetime non-smoking and non-drinking cancer-free subjects was selected.

Cochlear implantation and Cogan syndrome.

Objective: To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome.

Study Design: Prospective cohort.

Setting: Department of Ophthalmology and Otorhinolaryngology, University of Parma.

Multichannel cochlear implantation in radical mastoidectomy cavities.

Objective: We report on our experience in cochlear implantation in patients with radical mastoidectomy cavities. Study Design, Setting, and Methods: Retrospectively, records of patients from the Department of Otolaryngology, University of Parma between December 1991 and March 2000 were reviewed, and 6 postlingually deafened adults who received a cochlear implant in a radical cavity were identified. Speech performances were evaluated in terms of bisyllabic word and sentence recognition and common phrase comprehension.

The nucleus contour electrode array: an electrophysiological study.

Objective/hypothesis: Positioning intracochlear electrodes adjacent to the modiolus would lead to a reduction in threshold levels and latencies, as measured with electrically evoked auditory brainstems responses. Recently, Cochlear Corporation introduced a new perimodiolar array (Contour electrode) characterized by a platinum stylet inserted into the array's silicone carrier that keeps the electrode in a straightened configuration before insertion. After completion of the insertion, the withdrawal of the stylet allows the array to coil within the cochlea positioning electrodes closer to the modiolus.

Comparison of speech perception benefits with SPEAK and ACE coding strategies in pediatric Nucleus CI24M cochlear implant recipients.

Nine congenitally deaf children who received a Nucleus CI24M cochlear implant and who were fitted with the SPrint speech processor participated in this study. All subjects were initially programmed with the SPEAK coding strategy and then converted to the ACE strategy. Speech perception was evaluated before and after conversion to the new coding strategy using word and Common Phrase speech recognition tests in both the presence and absence of noise.

Nucleus multichannel cochlear implantation in partially ossified cochleas using the Steenerson procedure.

Objective: The purpose of this study was to report hearing results obtained in a group of subjects who received scala vestibuli implantation because of cochlear ossification and to compare these results to those in patients with scala tympani implantation.

Study Design: Retrospective analysis of consecutive cochlear implant procedures.

Setting: Department of Otolaryngology, University of Parma.