| 1. |
- Benatti, Alice, et al.
(författare)
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Endocochlear inflammation in cochlear implant users : Case report and literature review
- 2013
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Ingår i: International Journal of Pediatric Otorhinolaryngology. - Amsterdam : Elsevier. - 0165-5876 .- 1872-8464. ; 77:6, s. 885-893
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Forskningsöversikt (refereegranskat)abstract
- Objectives: Cochlear implantation is a relatively safe procedure with a low complication rate. The overall rate of complications among cochlear implant patients ranges from 6% to 20%. Major complications are those that are life-threatening or require surgery, whereas minor complications are those that can be medically treated. Nonetheless, certain complications, even if highly rare, may require specific investigations and treatments. Among these rare complications are those with endocochlear involvement, such as cochleitis or labyrinthitis, with fibrosis or ossification that could lead to explantation. The aims of the present study were to report a particular case of post-operative cochleitis and to review the rate of complications after cochlear implantation, emphasising those conditions with proven endocochlear involvement.Methods: We refer to the case of an eight-year-old Italian boy affected by the sudden onset of headache, ipsilateral otalgia and facial paresis, who presented to our clinic for inexplicable worsening of the performance of his implant and his residual hearing, six years after surgery. A complete investigation including (clinical history, routine, autoimmune and serological blood tests, electrophysiological measurements from the cochlear implant and neuroimaging) was performed and is herein described. Additionally, a comprehensive review of the literature was conducted using internet search engines; 274 papers were selected, 88 of which were best suited to our purposes.Results: In our case, the progression of the symptoms and the performance decrement required explantation, followed by a complete recovery. Reviewing the literature revealed only three reports concerning cases of proven endocochlear phlogosis that required revision surgery. Wound swelling/infection and vertigo remain the two most common complications of cochlear implantation. Failure of the device is the third most frequent complication (10.06% of all complications and 1.53% of cochlear implantations). Other rare conditions (such as granulating labyrinthitis with cochlear fibrosis, ossification and erosion, silicone allergy and the formation of a biofilm around the internal device) are possible and unpredictable. Although rare (approximately 1%), such cases may require explantation.Conclusions: Despite efforts by both surgeons and manufacturers, device-related and surgical complications still occur. These and other rare conditions demand specific management, and their frequency may be underestimated. Further studies are needed to assess more realistic rates of complications and devise more efficient strategies for early diagnosis and treatment.
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| 2. |
- Bovo, Roberto, et al.
(författare)
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Cavernous hemangioma of the external ear : case report and literature review
- 2010
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Ingår i: B-ENT. - : Societe Royale Belge d'Oto - Rhino - Laryngologie et de Chirurgie Cervico - Faciale. - 1781-782X .- 1781-782X. ; 6:2, s. 127-130
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Forskningsöversikt (refereegranskat)abstract
- Objectives: To describethe occurrence of a cavernous hemangioma of the external auditory meatus extending to the middle ear and the retroauricolar region in an adult patient, and to review the relevant literature.Methods: Case report. We report the clinical presentation, imaging studies and surgical procedures used in the study.Results: The angiographic study showed that the blood supply of the mass originated from the posterior auricular artery. Via a retroauricular approach, this artery was isolated and ligated in order to control the intra-operative bleeding. A canal wall up mastoidectomy with posterior tympanotomy and a tympano-canaloplasty were performed, permitting a complete excision. At 36 months of follow-up, no clinical and radiological signs of recurrence were detected.Conclusion: Surgical removal is the treatment of choice, with rare recurrence of the hemangiomas. Pre-operative evaluation is based on computed tomography (CT scan), but should be followed by magnetic resonance angiography (MR-angio) or intra-arterial digital subtraction angiography (IA-DSA), especially in case of wider lesions.
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| 3. |
- Bovo, Roberto, et al.
(författare)
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Cochlear implant in Cogan syndrome
- 2011
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Ingår i: Acta Oto-Laryngologica. - : Taylor & Francis. - 0001-6489 .- 1651-2251. ; 131:5, s. 494-497
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Tidskriftsartikel (refereegranskat)abstract
- Conclusions: Despite the need for special fitting strategies, improvements in speech discrimination tests support the use of cochlear implantation (CI) for patients with Cogan syndrome. Adequate preimplant counselling is mandatory, to prevent high expectations and to stress the necessity for bilateral implantation.Objective: In 60% of patients with Cogan syndrome, CI remains the only treatment option. Literature data agree that once the electrode array is properly inserted, functional outcomes are very good. Nevertheless, results may deteriorate due to progressive cochlear ossification. A few studies have documented the outcomes of CI in these patients, but none have reported the long-term results.Methods: This was a retrospective study describing the outcomes of 3 implanted patients with Cogan syndrome – among 300 adult patients who received a cochlear implant, 3 had become deaf due to Cogan syndrome.Results: In one patient the cochlear ossification advanced and the speech perception abilities worsened from the highest category to identification of words in closed set. The second patient complained of an abrupt reduction of loudness at 18 months post-implant, which required an increased electrical stimulation. The third patient reached the identification category probably due to auditory dyssynchrony, as an atypical consequence of the syndrome.
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| 4. |
- Bovo, Roberto, et al.
(författare)
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Hearing impairment in the Sturge-Weber syndrome
- 2009
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Ingår i: European Journal of Clinical Investigation. - Oxford, UK : Wiley-Blackwell Publishing Inc.. - 0014-2972 .- 1365-2362. ; 39:9, s. 837-838
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Tidskriftsartikel (refereegranskat)
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| 5. |
- Brotto, Davide, et al.
(författare)
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Salivary glands abnormalities in oculo-auriculo-vertebral spectrum
- 2018
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Ingår i: Clinical Oral Investigations. - Berlin, Germany : Springer. - 1432-6981 .- 1436-3771. ; 22:1, s. 395-400
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Tidskriftsartikel (refereegranskat)abstract
- Background: Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (OAVS) patients. Salivary gland abnormalities have been reported in OAVS patients but their rate, features, and relationship with phenotype severity have yet to be defined.Material and methods: Parotid and submandibular salivary gland hypo/aplasia was evaluated on head MRI of 25 OAVS patients (16 with severe phenotype, Goldenhar syndrome) and 11 controls.Results: All controls disclosed normal salivary glands. Abnormal parotid glands were found exclusively ipsilateral to facial microsomia in 21/25 OAVS patients (84%, aplasia in six patients) and showed no association with phenotype severity (14/16 patients with Goldenhar phenotype vs 7/9 patients with milder phenotype, p = 0.6). Submandibular salivary gland hypoplasia was detected in six OAVS patients, all with concomitant ipsilateral severe involvement of the parotid gland (p < 0.001). Submandibular salivary gland hypoplasia was associated to Goldenhar phenotype (p < 0.05). Parotid gland abnormalities were associated with ipsilateral fifth (p < 0.001) and seventh cranial nerve (p = 0.001) abnormalities. No association was found between parotid gland anomaly and ipsilateral internal carotid artery, inner ear, brain, eye, or spine abnormalities (p > 0.6).Conclusions: Salivary gland abnormalities are strikingly common in OAVS. Their detection might help the management of OAVS-associated swallowing and feeding impairment.
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| 6. |
- Castiglione, Alessandro, 1976-, et al.
(författare)
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Aging, Cognitive Decline and Hearing Loss : Effects of Auditory Rehabilitation and Training with Hearing Aids and Cochlear Implants on Cognitive Function and Depression among Older Adults
- 2016
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Ingår i: Audiology & neuro-otology. - Basel : S. Karger. - 1420-3030 .- 1421-9700. ; 21:Sup. 1, s. 21-28
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Tidskriftsartikel (refereegranskat)abstract
- A growing interest in cognitive effects associated with speech and hearing processes is spreading throughout the scientific community essentially guided by evidence that central and peripheral hearing loss is associated with cognitive decline. For the present research, 125 participants older than 65 years of age (105 with hearing impairment and 20 with normal hearing) were enrolled, divided into 6 groups according to their degree of hearing loss and assessed to determine the effects of the treatment applied. Patients in our research program routinely undergo an extensive audiological and cognitive evaluation protocol providing results from the Digit Span test, Stroop color-word test, Montreal Cognitive Assessment and Geriatric Depression Scale, before and after rehabilitation. Data analysis was performed for a cross-sectional and longitudinal study of the outcomes for the different treatment groups. Each group demonstrated improvement after auditory rehabilitation or training on short- and long-term memory tasks, level of depression and cognitive status scores. Auditory rehabilitation by cochlear implants or hearing aids is effective also among older adults (median age of 74 years) with different degrees of hearing loss, and enables positive improvements in terms of social isolation, depression and cognitive performance.
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| 7. |
- Castiglione, Alessandro, 1976-, et al.
(författare)
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Correspondence Between Cognitive and Audiological Evaluations Among the Elderly : A Preliminary Report of an Audiological Screening Model of Subjects at Risk of Cognitive Decline With Slightto Moderate Hearing Loss
- 2019
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Ingår i: Frontiers in Neuroscience. - Lausanne : Frontiers Media S.A.. - 1662-4548 .- 1662-453X. ; 13
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Tidskriftsartikel (refereegranskat)abstract
- Epidemiological studies show increasing prevalence rates of cognitive decline and hearing loss with age, particularly after the age of 65 years. These conditions are reported to be associated, although conclusive evidence of causality and implications is lacking. Nevertheless, audiological and cognitive assessment among elderly people is a key target for comprehensive and multidisciplinary evaluation of the subject's frailty status. To evaluate the use of tools for identifying older adults at risk of hearing loss and cognitive decline and to compare skills and abilities in terms of hearing and cognitive performances between older adults and young subjects, we performed a prospective cross-sectional study using supraliminal auditory tests. The relationship between cognitive assessment results and audiometric results was investigated, and reference ranges for different ages or stages of disease were determined. Patients older than 65 years with different degrees of hearing function were enrolled. Each subject underwent an extensive audiological assessment, including tonal and speech audiometry, Italian Matrix Sentence Test, and speech audiometry with logatomes in quiet. Cognitive function was screened and then verified by experienced clinicians using the Montreal Cognitive Assessment Score, the Geriatric Depression Scale, and further investigations in some. One hundred twenty-three subjects were finally enrolled during 2016-2019: 103 were >65 years of age and 20 were younger participants (as controls). Cognitive functions showed a correlation with the audiological results in post-lingual hearing-impaired patients, in particular in those affected by slight to moderate hearing loss and aged more than 70 years. Audiological testing can thus be useful in clinical assessment and identification of patients at risk of cognitive impairment. The study was limited by its sample size (CI 95%; CL 10%), strict dependence on language, and hearing threshold. Further investigations should be cond
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| 8. |
- Castiglione, Alessandro, 1976-, et al.
(författare)
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EYA1-related disorders : two clinical cases and a literature review
- 2014
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Ingår i: International Journal of Pediatric Otorhinolaryngology. - Amsterdam : Elsevier. - 0165-5876 .- 1872-8464. ; 78:8, s. 1201-1210
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Forskningsöversikt (refereegranskat)abstract
- Objectives: To delineate the diagnostic and rehabilitative aspects of syndromes that have overlapping features, we present the cases of two unrelated Caucasian males affected by hearing impairment, preauricular pits and cervical fistulae. Specific findings that are helpful in the diagnosis and management of EYA1-related disorders are highlighted.Methods: Genetic, otologic, imaging, eye and renal evaluations were conducted to achieve a detailed and comprehensive assessment, leading to the most accurate diagnosis and appropriate treatment. A literature review was also carried out.Results: Diagnostic criteria indicated that the two patients were affected by BOS1 (Branchio-Otic Syndrome 1). We also identified a novel sporadic missense mutation in the EYA1 gene: p.G533R (c.1597G>A, NM_000503.4), a highly conserved, heterozygotic amino acid substitution. In the other case, we identified the p.X593QextX6 (c.1777T>A, NM_000503.4) substitution. Both variants lead to isoform 1 (EYA1B and EYA1C) which is composed of 592 amino acids. Clinical and in silico evidence suggests a pathogenic role for the new mutations. Imaging evaluation revealed a complex pathology, characterized by external, inner and middle ear malformations, without renal anomalies.Conclusions: Our results demonstrate the importance of considering the imaging evaluation and the complete DNA sequencing of the EYA1 gene for the differential diagnosis of deafness and related branchio-oto-renal disorders.
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| 9. |
- Ciorba, Andrea, et al.
(författare)
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Sudden bilateral sensorineural hearing loss as an unusual consequence of accidental ingestion of potassium hydroxide
- 2010
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Ingår i: Medical principles and practice. - Basel : S. Karger. - 1011-7571 .- 1423-0151. ; 19:5, s. 406-408
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To discuss the possible etiopathogenetic mechanism of inner ear damage induced by the ingestion of potassium hydroxide (KOH).Clinical presentation and intervention: We report the case of a 37-year-old patient with sudden bilateral sensorineural hearing loss after accidental ingestion of a KOH solution. The first ear, nose and throat examination disclosed only mild edema of the upper airways. He was treated in the intensive care unit and prescribed high-dose steroids, proton pump inhibitors and sucralfate for 2 weeks. Unfortunately, there was no recovery of the hearing loss, and no audiogram changes were noticed after 12 months of follow-up.Conclusion: After exploring the possible etiopathogenetic mechanism involved, the authors believe that in this case, a transient severe hemodynamic imbalance can actually be considered to be the most reliable explanation for the inner ear damage and subsequent onset of permanent bilateral sensorineural hearing loss.
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| 10. |
- Davide, Brotto, et al.
(författare)
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Oculo-auriculo-vertebral spectrum : going beyond the first and second pharyngeal arch involvement
- 2017
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Ingår i: Neuroradiology. - Berlin : Springer. - 0028-3940 .- 1432-1920. ; 59:3, s. 305-316
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: Intracranial vascular, bone, and brain abnormalities might be observed in oculo-auriculo-vertebral spectrum (OAVS) patients even though these structures do not derive embryologically from the first and second pharyngeal arches. This neuroimaging study investigated their type, frequency and phenotype correlations among OAVS patients.Methods: Thirty-five OAVS patients (mean-age 4.8 ± 6.0 years; age range 0.3-30, 14 females) underwent head CT (all patients) and brain MRI (32 patients); 19 patients had a more severe phenotype (Goldenhar syndrome). Internal carotid artery hypoplasia/agenesia and abnormal course, brain abnormalities, internal acoustic canal stenosis/aplasia, cochlear-vestibular malformations, facial nerve bony canal anomalies, and oval window atresia were recorded.Results: Nine of 35 (26%) OAVS patients showed internal carotid artery anomalies; 18/32 (56%) had protean brain MRI abnormalities, ranging from tegmental cap anomaly to mild ventriculomegaly, which were associated with Goldenhar syndrome (p < 0.001) and concomitant cranial nerve abnormalities (p = 0.004); 11/35 (31%) disclosed cochlear-vestibular abnormalities including Michel deformity, common cavity, cochlear hypoplasia, and incomplete partition type-1 that were associated with Goldenhar syndrome (p = 0.01) and ipsilateral VIII cranial nerve abnormalities (p < 0.001); 16/35 (46%, 23 sides) presented facial nerve bony canal abnormalities that were associated with Goldenhar syndrome (p < 0.001) and ipsilateral VII cranial nerve (p < 0.001) and cochlear-vestibular (p < 0.001) abnormalities; and 23/31 (74%, 31 sides) showed oval window atresia, always with concomitant ossicular chain dysplasia.Conclusions: Intracranial vascular, bone, and brain abnormalities in OAVS patients are strikingly common and heterogeneous. As their detection might impact significantly on clinical and surgical management of affected patients, accurate neuroimaging investigations should be included in the diagnostic work-up of OAVS.
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