Michele Cavaliere

Hearing Impairment in Parkinson's Disease: Expanding the Nonmotor Phenotype

The objective of this study was to evaluate hearing impairment in patients affected by Parkinsons disease compared with hearing scores observed in normal age‐ and sex‐matched controls. One hundred eighteen consecutive patients with a clinical diagnosis of Parkinsons disease were screened. Severity of motor symptoms and staging were measured with the Unified Parkinsons Disease Rating Scale (section III) and the Hoehn and Yahr scale. Audiometric evaluation consisted of a comprehensive audiologic case history and questionnaire, visual otoscopic examination, acoustic immittance measures (tympanogram and acoustic reflexes), pure tone audiometry, and measurement of brain stem auditory‐evoked potentials. Healthy age‐ and sex‐matched subjects were selected as the control group. One hundred six of 118 patients were enrolled. Pure tone audiometry revealed age‐dependent high‐frequency hearing loss in patients with Parkinsons disease compared with both normative values and values for healthy age‐ and sex‐matched controls (75/106 [71%], χ2 = 5.959, P = .02; 92/106 [86.8%] vs 60/106 [56.6%], χ2 = 23.804, P < .001, respectively). Pure tone audiometry scores correlated with Hoehn and Yahr scale scores (P < .05). Brain stem auditory‐evoked potentials were normal in all patients. Our patients with Parkinsons disease showed age‐dependent peripheral, unilateral, or bilateral hearing impairment. Whether these auditory deficits are intrinsic to Parkinsons disease or secondary to a more complex impaired processing of sensorial inputs occurring over the course of illness remains to be determined. Because α‐synuclein is located predominately in the efferent neuronal system within the inner ear, it could affect susceptibility to noise‐induced hearing loss or presbycusis. It is feasible that the natural aging process combined with neurodegenerative changes intrinsic to Parkinsons disease might interfere with cochlear transduction mechanisms, thus anticipating presbycusis.

Diffusion Weighted MR Imaging of Primary and Recurrent Middle Ear Cholesteatoma: An Assessment by Readers with Different Expertise

Introduction and Purpose. Diffusion weighted imaging (DWI) has been proven to be valuable in the diagnosis of middle ear cholesteatoma. The aims of our study were to evaluate the advantage of multi-shot turbo spin echo (MSh TSE) DWI compared to single-shot echo-planar (SSh EPI) DWI for the diagnosis of cholesteatoma. Material and Methods. Thirty-two patients with clinical suspicion of unilateral cholesteatoma underwent preoperative MRI (1.5T) with SSh EPI and MSh TSE. Images were separately analyzed by 4 readers with different expertise to confirm the presence of cholesteatoma. Sensitivity, specificity, diagnostic accuracy, and positive (PPV) and negative predictive values (NPV) were assessed for each observer and interrater agreement was assessed using kappa statistics. Diagnosis was obtained at surgery. Results. Overall MSh TSE showed higher diagnostic accuracy and lower negative predictive value (NPV) compared to conventional SSh EPI. Interreader agreement between the observers revealed the superiority of MSh TSE compared to SSh EPI. Interrater agreement among all the four observers was higher by using MSh TSE compared to SSh EPI. Conclusion. Our findings suggest that MSh TSE DWI has higher sensitivity for detection of cholesteatoma and lower probability of misdiagnosis. MSh TSE DWI is useful in guiding less experienced observers to the diagnosis.

Stapedotomy: functional results with different diameter prostheses.

Developments in surgical technique and, more importantly, the use of increasingly sophisticated biocompatible prostheses have meant that good results can be achieved for otosclerosis sufferers in terms of restored hearing and very little postsurgical discomfort. We set out to assess whether the diameter of the prostheses used for stapedotomy (platinum piston/polytetrafluoroethylene, i.e. Teflon) has any effect on surgical outcome. Two groups of otosclerotic patients were selected, and these underwent stapedotomy surgery during the second phase of the disease. A piston-Teflon type prosthesis was used, 5.50 mm in terms of length but of different diameters (group A: 0.4 mm; group B: 0.6 mm). All the patients underwent the same pure-tone audiometry test before surgery, and then at 1 week and 1 month after surgery, to assess function. We compared air conduction after surgery with bone conduction before surgery. The data collected was analysed using the χ2 (p < 0.05) test. This analysis showed that the results obtained with a 0.4-mm prosthesis or a 0.6-mm prosthesis are almost identical. There was no statistically significant difference in terms of hearing results when comparing either average tonal threshold or when analysing audiometric data frequency by frequency. It can be concluded, therefore, that in stapedotomy surgery, functional recovery is not affected by the diameter of the prosthesis used. A smaller diameter prosthesis is, however, the one of choice when the facial nerve is prominent or the oval window particularly narrow.

Diffusion-Weighted Intensity Magnetic Resonance in the Preoperative Diagnosis of Cholesteatoma

We have analyzed the preoperative diagnosis of cholesteatoma through the use of diffusion-weighted intensity magnetic resonance (DWI-MR) in 16 consecutive patients suffering from chronic otitis media with clinical and radiological (by computed tomography) suspicion of cholesteatoma. In particular, we compared the radiological data with intraoperative ones, verifying the correspondence (in terms of sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy) between what is reported by DWI-MR and what is actually detectable at the time of surgery. Furthermore, we identified the most reliable DWI-MR sequence [single-shot (SSh) echo planar imaging (EPI) vs. multi-shot turbo spin-echo not-EPI] to detect cholesteatoma and reduce the time for examination. The obtained data on computed tomography scans revealed low diagnostic accuracy (56%); DWI-MR, instead, showed higher values, especially using not-EPI sequences (93.75 vs. 68.75% obtained by SSh-EPI sequences).

Endolymphatic hydrops in idiopathic intracranial hypertension: prevalence and clinical outcome after lumbar puncture. Preliminary data

Idiopathic intracranial hypertension is characterized by raised intracranial pressure (ICP) without any underlying pathology, presenting with (IIH) or without papilledema (IIHWOP). Headache, often on daily basis, is the most frequent symptom. Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports. Endolymphatic hydrops (ELH) is the typical histopathologic feature of Ménière disease, a condition featured by episodes of vertigo, dizziness, fluctuating hearing loss, tinnitus, and aural fullness. Evidences suggest that ICP is transmitted to inner ear. The aim of this study is to investigate the prevalence of ELH symptoms in IIH/IIHWOP and the relationship between the raised ICP and ELH. The prevalence of chronic headache and of ELH symptoms was investigated in a consecutive series of IIH/IIHWOP patients, and a standard audiometry with hearing threshold measurement (pure-tone average—PTA) was performed. Differences in chronic headache and ELH symptoms prevalence and changes of PTA threshold were calculated after ICP normalization by lumbar puncture (LP). Thirty-one patients (17 with IIH and 14 with IIHWOP) were included. Before LP, chronic headache was present in 93.5%. The percentages of patients reporting tinnitus, dizziness, vertigo, and aural fullness were 67.7, 77.4, 22.6, and 61.3%, respectively. Headache frequency as well as ELH symptoms and PTA significantly improved after LP. The improvement of PTA and of ELH symptoms observed after LP in this series of IIH/IIHWOP patients indicates that a raised ICP, a condition known to be involved in the progression and refractoriness of migraine pain, has also a role in ELH. We propose that intracranial hypertension may represent the shared pathogenetic step explaining the large epidemiological comorbidity between migraine and vestibular symptoms, at present conceptualized as “vestibular migraine.”

SLC26A4 genotypes associated with enlarged vestibular aqueduct malformation in south Italian children with sensorineural hearing loss

aAnnamaria Franzè and Gabriella Esposito are co-first authors. *Corresponding authors: Elio Marciano, Area di Audiologia, Dipartimento di Neuroscienze, Scienze Riproduttive ed Odontostomatologiche,Via Sergio Pansini, Università di Napoli Federico II, Italy, Via Sergio Pansini 5, 80131 Naples, Italy, Phone: +39 081 746 3875, Fax: +39 081 746 3581, E-mail: marciano@unina.it; and Francesco Salvatore, CEINGEBiotecnologie Avanzate s.c. a r.l., Via Gaetano Salvatore 486, 80145 Napoli, Italy; IRCCS Fondazione SDN, Naples, Italy, Phone: +39 081 746 3133, Fax: +39 081 746 3650, E-mail: salvator@unina.it Annamaria Franzè: CEINGE-Biotecnologie Avanzate s.c. a r.l., Napoli, Italy; and Area di Audiologia, Dipartimento di Neuroscienze, Scienze Riproduttive ed Odontostomatologiche, Università di Napoli Federico II, Naples, Italy Gabriella Esposito: CEINGE-Biotecnologie Avanzate s.c. a r.l., Napoli, Italy; and Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli Federico II, Naples, Italy Carmela Di Domenico, Sandra Iossa and Giuliana Sauchelli: CEINGEBiotecnologie Avanzate s.c. a r.l., Naples, Italy Tiziana Fioretti: IRCCS Fondazione SDN, Naples, Italy Michele Cavaliere: UOC, Otorinolaringoiatria, Dipartimento di Neuroscienze, Scienze Riproduttive ed Odontostomatologiche, Università di Napoli Federico II, Naples, Italy Gennaro Auletta, Virginia Corvino, Carla Laria and Rita Malesci: Area di Audiologia, Dipartimento di Neuroscienze, Scienze Riproduttive ed Odontostomatologiche, Università di Napoli Federico II, Naples, Italy Letter to the Editor

Operative Management of OSAS in a Complex Case of Proteus Syndrome

Obstructive sleep apnea syndrome (OSAS) is a common disorder in childhood with high prevalence in syndromic subjects with craniofacial malformations. Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils. We report a case of a 12-year-old boy diagnosed with Proteus Syndrome (PS) and suffering from OSAS due to asymmetric palatine tonsillar hypertrophy, treated with partial resection of left tonsil. To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed. Recovery of the obstructive respiratory disease was obtained. To our knowledge, this is the first case reported in the literature of partial tonsillar resection performed in a patient with PS suffering from OSAS under local anesthesia.

Exclusion of TNFRSF11B as Candidate Gene for Otosclerosis in Campania Population

The etiology of otosclerosis is unknown. The etiopathogenesis of otosclerosis seems similar to that occurring in Paget’s disease of bone, for which mutations or polymorphisms in several genes have been identified. Among these, TNFRSF11B gene encoding the osteoprotegerin is produced at high levels in the normal inner ear and at low level in active otosclerotic stapes footplates. The aim of this work was to verify the presence of a correlation between the rs2073618 (N3K) polymorphism in the TNFRSF11B gene and otosclerosis. Mutational screening in the TNFRSF11B gene was performed by direct sequencing. SNPs analysis was performed by PCR and by specific restriction enzyme assay with HpaI. The significance of the association was analyzed by statistical specific software. No causative mutation has been identified but the data suggested a strong correlation between the rs2073618 (N3K) polymorphism and otosclerosis. This correlation, however, has been excluded in a case–control study. This study excluded the association between the N3K polymorphism and otosclerosis in Campania region population.

Epidermal cyst of temporal bone as a delayed complication of myringoplasty

Epidermal cysts are benign tumors derived from the epidermis or the epithelial hair follicle filled with keratin and lipid-rich debris, typically occurring in areas with a high-density of sebaceous glands. These cysts commonly occur on the face, scalp, neck and trunk, where the sebaceous glands are more active. Their localization within the bone is extremely uncommon. The current study details the case of a 24-year-old male who presented with right otorrhea and ipsilateral hypoacusia having undergone right overlay myringoplasty for subtotal eardrum perforation. This patient represents a rare case of an epidermal cyst localized in the temporal bone (the fifth described in English-language literature), which may be considered as a complication of myringoplasty.

A huge retropharyngeal bronchogenic cyst displacing the posterior wall of the hypopharynx: Case report and review of the literature.

Lesions of the retropharyngeal space (RPS) are uncommon, and they generally present as solitary, painless masses, which are often cystic. They usually originate from branchial arches anomalies, and only in a few cases do they turn out to be bronchogenic cysts. Generally, these lesions are diagnosed in childhood, but sometimes they can appear in adulthood. We report here a rare case of a bronchogenic cyst expanding into the RPS and causing dysphagia in an adult patient treated surgically. Since the RPS is clinically inaccessible, clinical examination was not crucial in determining the correct diagnosis, and only the additional information provided by radiological examinations led to the final diagnosis, which is essential for accurate surgical planning.