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. The speech recognition performance was rated before cochlear implantation and at 1 and 5 years after implantation. The speech materials were presented in quiet only condition. The mean 12-month post-operative word and sentence recognition scores were 91.4 and 93.1%, respectively. Five years after implantation, the group means for word and sentence recognition tests were 94 and 96.3%, respectively. No patients in this series experienced flap complication or other local or systemic complications. This long-term study on 12 subjects with CS over 5 years of cochlear implant use reveals that cochlear implantation is safe in the long term and provides excellent and stable hearing results.

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. Our study confirmed the higher prevalence of the closed type, as well as, a younger age at initial diagnosis compared with the open type congenital cholesteatoma. Other differences between the two forms were: modality of diagnosis (pathognomonic otoscopy in 100% of the closed type and in 40% of the open type), positive history for otitis media with effusion (51.4% in closed type vs 20% in open type), involvement of the tympanic membrane quadrants (anterior quadrants were more frequently involved in the closed forms, whereas posterior quadrants were more frequently involved in the open forms), disease extension and aggressiveness. A residual cholesteatoma was found in 6 out of the 71 patients (8.4%) with a closed type congenital cholesteatoma and in 10 out of the 25 patients (40%) with an open type congenital cholesteatoma. After adjusting for potential confounders, open-type congenital cholesteatoma was significantly associated with residual cholesteatoma compared to the closed-type (odds ratio [OR] 7.39, 95% confidence interval [CI] 1.10-49.77, p=0.03). CONCLUSION: This study confirmed that the open congenital cholesteatoma has global clinical features that are uniquely different from the classical closed form. These differences could reflect a distinct pathogenesis, but there is no proof of this to date. The classification of the congenital cholesteatoma could be further refined by adding the morphologic type of the disease.

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. Treatment options include conservative management (bed rest, antibiotics, corticosteroids) or surgery (exploratory tympanotomy). We present the case of a 31-year-old female who had pneumolabyrinth secondary to a temporal bone fracture. The patient was treated surgically and made a full clinical recovery.

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. Cochlear implants are partially implantable electronic devices designed to provide profoundly deafened patients with hearing sensitivity within the speech range. Since their introduction more than 30 years ago, cochlear implants have improved their performance to the extent that are now considered to be standard of care in the treatment of children with severe to profound deafness. Over the years patient candidacy has been expanded and the criteria for implantation continue to evolve within the paediatric population. The minimum age for implantation has progressively reduced; it has been recognized that implantation at a very early age (12-18 months) provides children with the best outcomes, taking advantage of sensitive periods of auditory development. Bilateral implantation offers a better sound localization, as well as a superior ability to understand speech in noisy environments than unilateral cochlear implant. Deafened children with special clinical situations, including inner ear malformation, cochlear nerve deficiency, cochlear ossification, and additional disabilities can be successfully treated, even thogh they require an individualized candidacy evaluation and a complex post-implantation rehabilitation. Benefits from cochlear implantation include not only better abilities to hear and to develop speech and language skills, but also improved academic attainment, improved quality of life, and better employment status. Cochlear implants permit deaf people to hear, but they have a long way to go before their performance being comparable to that of the intact human ear; researchers are looking for more sophisticated speech processing strategies as well as a more efficient coupling between the electrodes and the cochlear nerve with the goal of dramatically improving the quality of sound of the next generation of implants.

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). OBJECTIVES: To assess the correlation between anti-Hsp70 antibodies and specific ASNHL subgroups. METHODS: We divided 112 subjects into four groups: 14 subjects with typical CS, 24 with atypical CS, 55 with ASNHL, and 19 control subjects (healthy subjects and patients with systemic autoimmune diseases but no sensorineural hearing or audiovestibular alterations). Patients were tested for serological autoimmunity markers including anti-Hsp70. RESULTS: Positivity of the anti-Hsp70 antibody test was highest in the typical CS group (92.9%) and lowest in the control group (5.2%). The test was positive in 52.7% of patients in the ASNHL group and 16.6% in the atypical CS group. The paired comparison analysis between groups showed that sensitivity of anti-Hsp70 in the typical CS group was significantly higher, as compared to the other three study groups. CONCLUSIONS: Anti-Hsp70 antibodies can be considered a serological marker of "typical" CS. "Atypical" CS is conceivably a sort of "melting pot" of different forms of autoimmune diseases still characterized by ocular inflammation and sensorineural hearing loss but whose antigenic characteristics need to be further defined.

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. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. RESULTS: Follow-up ranged from 5 to 12 years (mean 8 years). Twelve children underwent planned staged canal wall up mastoidectomy: a residual cholesteatoma was found and removed during the second-look procedure in 2 ears (16.6%); two children (16.6%) showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A modified Bondy technique was chosen in two children with an epitympanic cholesteatoma with an intact tympano-ossicular system, while in the remaining four subjects a canal wall down mastoidectomy was performed because of an irreparable erosion of the postero-superior canal wall: no cases of recurrent cholesteatoma were observed in these 6 children; revision mastoidectomy was needed in one patient for cavity granulation. A postoperative air-bone gap result of 0-20dB was achieved in 11 children (61.1%); in 5 cases (27.7%) postoperative air-bone gap was between 21 and 30dB, while in 2 (11.1%) was >30dB. Bone conduction thresholds remained unaffected in all cases. CONCLUSIONS: Our results indicate that most cleft palate children with cholesteatoma can be managed with a canal wall up mastoidectomy with low complication rates. In extensive disease with large erosion of the canal wall as well in presence of a retraction pocket in the contralateral ear, a canal wall down mastoidectomy should be considered. In epitympanic cholesteatomas with an intact tympano-ossicular system and mesotympanum free of disease, the modified Bondy procedure is an effective surgical option. As in the general pediatric population, improvement or preservation of hearing can be obtained in most patients.

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. In this study we present the results of very long-term (mean 12 years) clinical and radiological follow-up in 12 patients who underwent external auditory canal closure associated with mastoid and Eustachian tube obliteration to facilitate cochlear implantation. To date, with a mean ± SD follow-up of 12 ± 4.7 years (range 5-21 years), the only complication experienced was the breakdown of the EAC closure in one patient, successfully treated by performing a rotation skin flap. The results of this study confirmed that external auditory canal closure is a reliable technique in cochlear implantation after radical mastoidectomy provided that a rigorous surgical technique is performed. A right balance between the need to reduce costs and to avoid unnecessary doses of radiation to patients and the task of a radiological surveillance may be represented by performing computed tomography 12-18 months postoperatively and then, only if clinically warranted.

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. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months' postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person's speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration.

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. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab. DISCUSSION: in CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.

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. The study was conducted prospectively as a within-subject repeated measures (ABA protocol) between January 2007 and January 2008. Each subject was exposed to and sequentially tested with two different rates of stimulation (260 pps/e and 600 pps/e), in quiet and in a noisy environment. Statistical analysis was performed on the data obtained. RESULTS: Subjects using the MPIS strategy with a stimulation rate of 600 pps/e performed significantly better in words and phrases recognition tests in both a noisy and a quiet environment.

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. We found a significant long-term reduction of tinnitus loudness in subjects treated with notched noise but not in those treated with control stimulations. These results point to the importance of the personalized sound treatment of tinnitus sufferers for the development of an effective tinnitus sound therapy.

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. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. RESULTS: At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf person's speech, and one child had connected speech intelligible to all listeners. CONCLUSIONS: Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.

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. STUDY DESIGN: Retrospective analysis. SETTING: Department of Otolaryngology and Department of Clinical Medicine, Nephrology and Health Science, University of Parma. METHODS: Twenty-nine patients with CSS were identified. Of the 29 patients, 17 (58.6%) had nasal polyposis and were enrolled in this study. The nasal polyps were graded according to the Lund and Mackay endoscopic and radiological classifications. RESULTS: At diagnosis, endoscopic intranasal evaluation identified nasal polyposis of grade 3 in nine cases (52.9%), grade 2 in six cases (35.2%), and grade 1 in the remaining case (5.8%). After corticosteroid and immunosuppressive therapy, clinical remission was achieved in 14 patients (82.3%), whereas 3 patients experienced a relapse. Posttreatment endoscopic evaluation showed a permanent disappearance (grade 0) of nasal polyps in eight patients (47%). The other nine patients (52.92%) were found to have a small polyp situated in the middle meatus (grade 1). CONCLUSIONS: Nasal polyposis in patients with CSS may represent the initial phase of the syndrome, though patients often have concurrent pulmonary disease. Corticosteroid therapy either alone or combined with immunosuppressive drugs usually yielded improvement or stabilization.

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. In ten randomly selected ears, biopsies from the newly built outer attic wall were taken during the second-look operation and were microscopically analyzed. At the second stage operation, the bone paté graft was found normal in 64.9% of cases. In 39 (29.1%) patients there was a remarkable hypertrophy of the bone paté which was subsequently thinned out by using a diamond burr. A partial resorption of the bone paté was observed in 4.4% of ears and a total resorption in 1.4%. Residual cholesteatoma was found in 13.4% of ears. At the last follow-up visit, recurrent cholesteatoma was observed in 5.2% of ears. Self cleaning attic retraction pocket occurred in 4.4% of ears. Twelve months after implantation, histologic examination on the newly built bone showed a well-structured bony tissue. In particular, osteoblasts lined bone trabeculae with signs of an active tissue remodeling. In conclusion, the reconstruction of the outer attic wall should be considered a fundamental step when performing a CWU procedure in order to prevent attic retractions and recurrences of cholesteatoma. In our experience, autogenous bone paté graft has demonstrated good long-term results in outer attic wall reconstruction being adaptable, well tolerated, stable and of low cost.

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. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by activated eosinophils. Patients affected by CSS frequently have ENT manifestations, which are often present at the time of disease onset and may represent relevant clues for the diagnosis. Thus, our objective was to present the ENT manifestations at the onset, at the diagnosis and at some point during the course of the disease in a series of patients with CSS collected at a single center. MATERIALS AND METHODS: Twenty-eight patients with CSS, as defined according to the 1990 American College of Rheumatology classification criteria, were identified. Twenty-one (75%) of these patients had ENT involvement. We evaluated the clinical course, laboratory data, histologic findings, treatment and outcomes. RESULTS: Of the 21 patients, 13 (61.9%) had ENT involvement at asthma onset and 8 (38%) at diagnosis or during follow-up. The most common ENT manifestations were allergic rhinitis in 9 (42.8%) patients and nasal polyposis in 16 (76.1%). Three (14.2%) patients developed chronic rhinosinusitis without polyps, three (14.2%) had nasal crusting, one (4.7%) serous otitis media, one (4.7%) purulent otitis media, two (9.5%) progressive sensorineural hearing loss, and one (4.7%) unilateral facial palsy. Corticosteroid therapy associated with immunosuppressive drugs usually yielded improvement or stabilization.

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. PATIENTS: Nine patients with Down syndrome were surgically treated for cholesteatoma. Two patients had bilateral disease, resulting in a total of 11 ears surgically treated. INTERVENTION: A canal-wall-up mastoidectomy was performed in two ears; in eight of the ears, a canal-wall-down mastoidectomy was carried out and a modified Bondy procedure was performed in one ear. RESULTS: Residual cholesteatoma was found in one ear after the canal-wall-up mastoidectomy and recurrent cholesteatoma developed in another ear, also after canal-wall-up mastoidectomy. The recurrence required conversion to canal-wall-down mastoidectomy. One patient developed a perforation of the neotympanic membrane that had to be revised. CONCLUSIONS: Cholesteatoma in children with Down syndrome is a challenging entity for the otologic surgeon. Otolaryngologists should always suspect a cholesteatoma in each child with Down syndrome presenting warning symptoms such as otorrhea and hearing loss. If there is any doubt on inspection, further imaging studies (high-resolution computed tomography) are necessary. To the best of our knowledge, the current study is the first report to document the surgical treatment of cholesteatoma in subjects with Down syndrome.

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. METHODS: A retrospective chart review of a HIV type 1-seropositive profoundly deafened patient who underwent cochlear implantation. RESULTS: To date, with a follow-up of 4 years, the patient has not experienced any complication and has regained useful open-set speech perception. CONCLUSIONS: Cochlear impairment with preserved auditory pathways can be responsible for profound SNHL in HIV-infected patients. Cochlear implantation can restore a social hearing in these patients, dramatically improving their quality of life. The surgical procedure can be safely performed when keeping in mind that the general condition of the patient is the decisive factor for or against surgery.

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. RESULTS: Patients implanted with the Nucleus Contour device obtained significantly higher word and sentence recognition scores after short-term use of the implant compared to those obtained by patients implanted with the Nucleus 24 model. CONCLUSION: Further long-term studies are required to determine whether the Nucleus Contour CI recipients continue to improve over time.

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. PATIENTS: Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. MAIN OUTCOME MEASURES: Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. RESULTS: In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. CONCLUSIONS: Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.