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To analyze the presence of chronic cerebrospinal venous insufficiency parameter and vascular abnormalities, in the internal jugular veins (IJVs) and/or vertebral veins in sitting and supine posture, in patients with Meniere's disease compared to healthy general population. A prospective study on 32 patients affected by definite Meniere was performed from February 2012 to January 2013. All subjects underwent an echo-color Doppler examination of the cerebrospinal venous flow. 21 of the 32 Menieric patients showed a statistically significant reflux in the intracranial veins versus healthy (65.6 vs 25%; P < 0.001). A high prevalence of IJVs stenosis with hemodynamic changes (increased velocity or absence of flow) was observed (66.7 vs 33.3%; P < 0.05). The other parameters considered did not show statistically significant differences among the two groups. The results obtained showed a vascular pattern of cerebrospinal venous system present in patients affected by definite Meniere. This vascular impairment significantly affects the vascular areas more directly involved in the venous drainage of the inner ear. Thus venous stasis may be considered a further pathogenetic mechanism for development of Meniere's disease.

Objective:This retrospective, comparative study aimed to assess anatomical and functional results in a group of adults undergoing type I tympanoplasty for subtotal tympanic membrane perforation, using two different types of graft.Subjects and methods:The study included 106 patients affected by chronic otitis media, who underwent underlay type I tympanoplasty, 53 using an autologous chondro-perichondral tragal graft and 53 using temporalis fascia. Anatomical and functional outcomes were evaluated over time.Results:Audiometric results comparing the cartilage and fascia groups at six months and one year after surgery showed no statistically significant differences. Assessment of anatomical outcomes indicated a greater number of complications in the fascia group.Conclusion:Functional results indicate the validity of the cartilage tympanoplasty, while anatomical results indicate a slightly better outcome in terms of graft re-perforation and retraction, compared with temporalis fascia at one-year follow up. These results suggest that the cartilage technique is preferable for type I tympanoplasty.

Objectives/Hypothesis: To investigate the efficacy of an intratympanic steroid as a first-line therapy in patients affected by moderate idiopathic sudden sensorineural hearing loss (ISSNHL). Study Design: Prospective, randomized, triple-blind, placebo-controlled trial. Methods: Fifty patients presenting with moderate idiopathic sudden sensorineural hearing loss involving all frequencies from 250 Hz to 8,000 Hz (a flat audiogram) were enrolled. Patients were randomized into two groups of 25 each. The first group (intratympanic steroid) underwent a daily intratympanic administration of prednisolone for 3 consecutive days. Subjects in the second group (control) received a daily intratympanic injection of a saline solution for 3 consecutive days. Audiometric tests were performed at day 7 after the beginning of therapy (T1), and then 10 and 30 days after T1. The patients in both groups who did not show a complete recovery at T1 were treated with oral prednisone at a tapering dose. Results: In the intratympanic steroid group, 19 out of 25 patients presented at T1 complete recovery (76%), whereas in the control group the number patients who recovered completely at T1 was five out of 25 (20%). The mean pure-tone average (PTA) recorded at T1 shows a statistically significant improvement in the hearing threshold of the first group compared to the control group (P < .01). Conclusions: The mean PTA recorded after the first-line approach (T1) demonstrated a significant therapeutic action of the short-duration intratympanic steroid therapy on moderate ISSNHL, with a flat audiogram shape, compared to the natural course of the disease and the placebo effect at that time point. Laryngoscope, 2013

This study was designed to investigate the indication and advantages of contralateral implantation after postimplant meningitis (piM). Speech perception assessment, most comfortable levels and high-resolution computer tomography were used to monitor cochlear fibrosis/ossification and clinical changes in outcomes in 5 children affected by meningitis after a variable-time post-cochlear implantation. Ipsilateral ossification was found in 3 children, 1 of whom developed delayed contralateral ossification. These children were implanted on the contralateral side as they all showed deterioration of hearing performance. Results from the present paper and literature analysis suggest that, (1) piM can induce ipsilateral and contralateral ossification, (2) meningitis-induced cochlear ossification is more prone to develop in the presence of a normal cochlear structure and (3) contralateral implantation after piM has proven to be effective in restoring performance when a full electrode insertion is accomplished.

Purpose To evaluate relationship between Meniere disease (MD) and chronic cerebrospinal venous insufficiency (CCSVI) using ultrasound, magnetic resonance (MR) imaging, and venography and to evaluate the effectiveness of angioplasty of the internal jugular vein (IJV) and azygos vein (AV) in reducing symptoms of MD. Materials and Methods Patients with a confirmed diagnosis of MD unresponsive to standard treatment underwent duplex ultrasound and MR imaging to diagnose CCSVI. Healthy volunteers were also studied to evaluate CCSVI in asymptomatic subjects. Patients with CCSVI and MD underwent venography and percutaneous transluminal angioplasty (PTA) of IJV and AV. Results There were 182 patients with no clinical benefit from standard treatments evaluated. CCSVI was diagnosed in 175 (87.5%) patients with MD. Venography was performed in 69 patients to confirm the diagnosis of CCSVI. In 80% of these patients, PTA of the IJV and/or AV was effective for treating signs and symptoms of MD. In the healthy cohort, CCSVI was observed in only 12% of subjects. Conclusions These results suggest a possible etiologic relationship between CCSVI and MD that warrants further investigation.

Revision stapes surgery requires intense patient counseling about possible outcome and potential hazards due to the surgery, and written consent is needed. Most frequent causes of the postoperative hearing loss and vertigo are displacement of the piston, incus necrosis, immobilization of the ossicular chain, obliteration of the oval window, too long piston, perilymph fistula, and fibrous tissue in the niche. Precise audiological workup and documentation help in the evaluation of the hearing outcome.

Cyclo-oxygenase-2 (COX-2) enzyme would not appear to be constitutively expressed in human perilymph while it is always induced in the perilymph of patients affected by sensorineural hearing loss (SNHL). The COX-2 isoform may be involved in hearing loss and, therefore, pathological states of the inner ear should possibly be further analyzed to clarify the clinical relevance of prostaglandin and selective COX-2 antagonist therapy.Perilymph samples from a group of patients with bilateral SNHL and another with conductive hearing loss were collected to evaluate the presence of the COX-2 enzyme. The possible correlation between different causes of deafness and the expression of COX-2 in the human ear was studied.A prospective clinical study of 14 patients with severe or profound hearing loss who underwent cochlear implant surgery and 4 patients with conductive hearing loss who underwent stapes surgery was carried out. Western blot analysis of perilymph samples was performed with monoclonal anti-human COX-2 antibody.COX-2 enzyme was detected in all patients affected by SNHL and was absent in all those with conductive hearing loss due to otosclerosis.

The image quality of 64-MDCT provided excellent definition of the fine osseous structures and individual electrode contacts. Evaluation of electrode distances revealed a more focused stimulation for the Helix contacts, with better optimization of pulse width and frequency of stimulation.A multi-slice CT scan was performed postoperatively to evaluate electrode distance from the modiolus and variability of fitting parameters (M level) for two different types of cochlear implant electrode carriers, CII and 90K implants with 1J and Helix electrode carriers.The electrode's position in different cochlear implant (CI) electrodes, Advanced Bionics 90K 1J and Helix, was assessed postoperatively in 20 adult patients by means of a 64-MDCT scanner. Axial, coronal, and oblique 0.3 mm multiplanar reconstructions (MPRs) were obtained and datasets were analyzed to assess the intracochlear position and distance from the surface of the electrodes to the bony edge of the modiolus. Patients' fitting characteristics were gathered at the time the CT was performed and correlated to intracochlear measurements.Determination of contact distances confirmed smaller average values for the Helix at the apex and medial segments. Helix electrodes were closer to the modiolus in all segments. Likewise, M level determination showed lower values for the Helix carrier, confirming a more focused stimulation and better optimization of pulse width and frequency of stimulation.

Objectives: To assess the therapeutic effectiveness of an intratympanic (IT) steroid protocol compared to a systemic steroid protocol. Methods: A total of 265 consecutive patients presenting unilateral idiopathic sudden sensorineural hearing loss were divided into 2 groups. One group comprised 131 patients enrolled between May 2009 and May 2010, and the other consisted of 134 patients enrolled between June 2010 and June 2011; a total of 48 patients were excluded among the 2 groups. The first group received oral prednisone for 8 days in tapering doses; the second group had IT prednisolone at a dose of 62.5 mg/ml once a day for 3 consecutive days. Audiological examinations were performed at study entry and 30 days after the beginning of therapy. Mean pure tone audiometry (PTA) of both groups and hearing outcomes following the criteria of Furuhashi et al. [Clin Otolaryngol 2002;27:458-463] and Siegel [Otolaryngol Clin North Am 1975;8:467-473] were investigated. Results: The strong efficacy of steroid therapy was evident in both groups, observing both PTA and hearing threshold improvement. The evaluation of the hearing outcomes shows a significantly better result for the short-term IT protocol; this result is ascribable to two types of audiometric curves: down- and up-sloping. Conclusion: The results show a significant efficacy of both steroid therapeutic approaches. There was no significant difference in PTA improvement between the 2 study groups; the short-term IT protocol led to better results in the evaluation of the hearing outcomes (following the criteria of Siegel and Furuhashi et al.) for up- and down-sloping audiometric curves.

Objectives To assess skills in inferences during conversations and in metaphors comprehension of unilaterally cochlear implanted children with adequate abilities at the formal language tests, comparing them with well-matched hearing peers; to verify the influence of age of implantation on overall skills. Methods The study was designed as a matched case–control study. 31 deaf children, unilateral cochlear implant users, with normal linguistic competence at formal language tests were compared with 31 normal hearing matched peers. Inferences and metaphor comprehension skills were assessed through the Implicit Meaning Comprehension, Situations and Metaphors subtests of the Italian Standardized Battery of “Pragmatic Language Skills MEDEA”. Differences between patient and control groups were tested by the Mann–Whitney U test. Correlations between age at implantation and time of implant use with each subtest were investigated by the Spearman rank correlation coefficient. Results No significant differences between the two groups were found in inferencing skills ( p = 0.24 and p = 0.011 respectively for Situations and Implicit Meaning Comprehension). Regarding figurative language, unilaterally cochlear implanted children performed significantly below their normal hearing peers in Verbal Metaphor comprehension ( p = 0.001). Performances were related to age at implantation, but not with time of implant use. Conclusions Unilaterally cochlear implanted children with normal language level showed responses similar to NH children in discourse inferences, but not in figurative language comprehension. Metaphors still remains a challenge for unilateral implant users and above all when they have not any reference, as demonstrated by the significant difference in verbal rather than figurative metaphors comprehension. Older age at implantation was related to worse performance for all items. These aspects, until now less investigated, had to receive more attention to deeply understand specific mechanisms involved and possible effects of different levels of figurative language complexity (presence or absence of contextual input, degree of transparency and syntactic frozenness). New insight is needed to orient programs in early intervention settings in considering and adequately responding to all these complex communicative need of children with hearing loss.

Herpes simplex virus (HSV) encephalitis is a life-threatening consequence of HSV infection of the central nervous system. Although HSV encephalitis is rare, mortality rates reach 70 per cent in the absence of therapy and only a minority of individuals return to normal function. Antiviral therapy is most effective when started early, necessitating prompt diagnosis.A case of atypical HSV encephalitis is reported. The appearance of a strong headache followed by impairment of consciousness and hypertone of arms and legs complicated the post-operative course in a 33-year-old patient who underwent surgical removal of an acoustic neuroma. Several brain magnetic resonance imaging (MRI) and computed tomography scans performed in the first week after onset of symptoms of infection did not establish a proper diagnosis. Diffusion-weighted MRI detected brain abnormalities on the fourth day after onset of symptoms, and polymerase chain reaction identification of HSV 1 DNA confirmed the diagnosis. A positive prognosis was achieved due to the decision to start specific, high-dose antiviral therapy based on clinical suspicion, before a firm diagnosis was established.

Idiopathic causes of retroauricular pain are rarely seen in clinical practice. We present a 9-year-old child who suffered from atypical retroauricular pain resistant to conventional treatment. After excluding any other cause of retroauricular pain, a nerve block was performed with a 0.3 ml lidocaine 1% injection into the trigger point. We believe that this case report is important because in the literature there are no similar cases described in children.

The aim of this study is to present a patient who developed a pneumolabyrinth following intratympanic steroid injection performed one year after stapes surgery. The patient started a medical treatment but since no improvement of his symptoms occurred, 10 days after the intratympanic injection a surgical treatment was proposed. An exploratory tympanotomy showed a displacement of the stapes prosthesis from the platinotomy, a dislocation of the incus long process, a fracture of the footplate and a depression into the vestibule. The fragment of the broken footplate was removed, a new prosthesis was located. Nausea, vertigo and nystagmus resolved immediately following surgery with a hearing threshold unchanged. In the present case report, the pathogenesis of pneumolabyrinth may be connected to an IT steroid injection proposed to the patient for the onset of sudden sensorineural hearing loss. To our knowledge, no case of a pneumolabyrinth provoked by intratympanic steroid injection has been previously described.

The aim of the present study is to assess long-term outcomes of CI in prelingual deafened adolescents and adults, describing, where present, differences in performance, self perceived benefit and highlighting specific characteristics. Twenty-three patients were enrolled: 10 adolescents, 13 young adults. Each patient underwent speech perception/language development, psychological evaluation and structured interviews on self perception concerning CI. 70 % adolescents and 100 % adults used their cochlear implant for most of the day; two adolescents were partial users and one was a non-user. Adolescents' average word recognition and comprehension scores improved respectively from 7 to 29.8 % (p = 0.01) and 3 to 26 % (p = 0.1). Adults' average scores improved significantly from 1.5 to 41.9 % (p = 0.01) and from 18.5 to 52.7% (p = 0.001), respectively. None of the subjects showed a linguistic age adequate to the chronological one: average linguistic age was 7.6 years for adolescents and 19.3 for adults. Structured interviews showed improvement in self-esteem. Adults and most adolescents were fully or moderately satisfied with their implant. Cochlear implantation can be considered a valid option for the rehabilitation of highly motivated and well-selected pre-lingual deafened adolescents and adults. Although there is a substantial variability in both groups of patients and language skills are only marginally influenced by CI, there is still a significant improvement in speech perception. CI was described by both groups as having had a positive impact on their lives; nevertheless adolescents were the ones with a tendency to under-use CI, even those with better hearing outcomes.

Objective: To evaluate, with a long-term follow-up, the speech perception and language development in children with cytomegalovirus (CMV)-related deafness after cochlear implantation. Study Design: A retrospective study on CMV-related profound deafness and cochlear implantation was performed from 1995 to 2010. Six children with an average follow-up of 10 years were included in this research. Medical history, imaging, cognitive delay, speech perception and production data were reviewed. Results: Two of the 6 patients developed a functional language with the use of phrases and word sequences based on morphological and syntactic rules; the others demonstrated the development of a preverbal or transitional language with the use of single words only. Conclusion: Patients with CMV-related deafness benefit from cochlear implantation; however, the expectations of the parents must be evaluated in a series of counseling efforts prior to the surgery.

The excellent tolerability of intratympanic (IT) steroid offers the possibility to use a high dose, which would appear to be more effective than intravenous (IV) steroid treatment, when both are associated with hyperbaric oxygen (HBO) therapy.The purpose of the study was to assess for the first time the efficacy of the association of IT steroid and HBO therapy in patients presenting idiopathic sudden sensorineural hearing loss (ISSNHL), comparing this protocol with another consisting of IV steroid administration and HBO therapy.A total of 48 patients presenting ISSNHL were recruited. Patients were divided into two categories: the severe ISSNHL group with a pure-tone average (PTA) between 70 and 90 dB, and the profound ISSNHL group with a PTA90 dB. The first protocol consisted of 10 days of HBO therapy together with IV methylprednisolone 1 mg/kg body weight for 7 days; the second protocol consisted of HBO therapy for 10 days, associated with an IT injection of prednisolone at a dose of 62.5 mg/ml, once a day for 3 consecutive days, performed 2 h before the HBO therapy.The overall success rate was superior in the group submitted to IT steroid and HBO therapy. Nevertheless, these clinical results were not statistically significant.

Lateral sinus thrombosis (LST) is an uncommon, but life-threatening complication of both acute and chronic otitis media. There is some evidence that acquired or hereditary prothrombotic disorders are risk factors for LST. The aim of this work was to evaluate the role of thrombotic screening, anticoagulant therapy or prophylaxis in patients with either acute or chronic otitis media and LST. The medical records of five children hospitalized at Pediatric Hospital Bambino Gesu of Rome because of acute or chronic otitis media complicated by mastoiditis and LST were reviewed. All children underwent laboratory workup for hypercoagulability. All the five children were found to be heterozygote for the C677T MTHFR mutation and a child presented also heterozygosity for factor V Leiden mutation. They have been successfully treated with anticoagulant therapy without sequels. Children with acute or chronic otitis media may have a prothrombotic tendency that becomes clinically evident because of the inflammatory state. Patients with a family and/or personal history of thrombosis and/or thrombophilic conditions need anticoagulant prophylaxis also in the absence of clear signs of LST. Treatment with low molecular weight is successful in patients with LST.

Objective:We report a case of a rare cochlear implant complication: the introduction of the electrode array into the superior semicircular canal, with intra-operative measurements of neural response reactions suggesting reasonable functioning of the implant.Case report:A two-year old patient affected by congenital, profound, sensorineural deafness underwent bilateral cochlear implantation at the ENT clinic of the ‘La Sapienza’ University of Rome. Two Clarion 90k devices were implanted, and electrophysiological and radiological checks were performed. After the introduction of the array in the right side, neural response imaging was performed, and a neural potential was found only on two apical electrodes, at a stimulation intensity of 431 clinical units. The situation differed on the left side, where neural response imaging was present at a stimulation intensity of 300 clinical units on the two electrodes tested (one apical electrode (number three), and one middle electrode (number nine)). Intra-operative radiological assessment with a transorbital plain films was performed as usual in order to assess the position of the electrodes inside the cochlea. This radiography showed the electrode array to be in the superior semicircular canal in the right ear.Conclusion:Intra-operative monitoring tests during cochlear implant surgery play different roles; measurement of impedances and neural response imaging can evaluate the integrity of implant electrodes and the status of the electrode–cochlea interface, but it must not be the sole way in which correct positioning of the array is confirmed. In our opinion, intra-operative radiological assessment is mandatory during cochlear implant surgery.

Objective: To identify patients developing positional vertigo after cochlear implantation. Study Design: Prospective study on a cohort of patients undergoing cochlear implantation. Setting: Academic tertiary referral center. Patients: The study included 70 consecutive patients who underwent vestibular evaluation before and after cochlear implantation. Intervention: Medical record review. Main Outcome Measure: Recorded vestibular symptoms after cochlear implantation. Patients with positional vertigo were considered case subjects, whereas those without vestibular symptoms were considered case controls. Results: Benign paroxysmal positional vertigo (BPPV) occurred in 8 patients (on the cochlear implant [CI] side in 7 patients, and in the other ear in 1). One patient had BPPV of the lateral semicircular canal on the implanted side, and 7 patients had BPPV of the posterior semicircular canal (on the same CI side in 6 patients, and on the opposite side in 1), which were detected and presented during the last examination. In 5 patients, the onset of symptoms varied from 7 to 130 days after implant activation; in 2 patients, the onset occurred before activation. Conclusion: Three different mechanisms are proposed for the occurrence of BPPV in patients with CI. The first focuses on the fall of bone dust particles into the cochlea during cochleostomy. In the second, the vibration caused by drilling the cochlea would be sufficient to dislodge otoconia into the labyrinth. The third hypothesis suggests dislodging of an otolith because of the electric stimulation. In our patients, conservative approaches have been used with a minimal invasive cochleostomy and without perilymph suction. Thus, the vibratory trauma affecting the cochlea during cochleostomy seems to play a fundamental role in the development of paroxysmal vertigo in patients with implant. Key Words: Positional vertigoVCochlear implantation. Otol Neurotol 28:764Y767, 2007.

The aim of the study was to investigate changes in middle ear dynamic characteristics caused by both otosclerosis and stapes surgery (platinotomy, prosthesis positioning, ossicular chain maneuver) and to evaluate distortion product otoacoustic emissions (DPOAEs) before and following surgery. The study included 15 patients (12 women, 3 men; mean age 51 years; range 32-69 years) with advanced otosclerosis. All the patients were evaluated with the use of pure-tone audiograms (preoperatively, 5 and 30 days after surgery), stapedial reflexes (preoperatively), and DPOAE recordings (preoperatively, at the end of surgery, and 5 and 30 days after surgery). Changes in the hearing thresholds and in the DPOAE amplitudes were compared. Preoperative tests showed conductive hearing loss, with a mean air-bone gap of 36.6 dB HL ranging from 0.25 to 1 kHz, and no stapedial reflexes were detected. DPOAEs were not measurable preoperatively, and they were detected only in 2 patients at the end of surgery, with low amplitudes in a narrow frequency range. No significant changes occurred in DPOAEs 5 days postoperatively. A month after surgery, improvement in conductive hearing loss was observed; the mean air-bone gap from 0.25 to 1 kHz was 12.9 dB HL, whereas the higher frequencies were still affected by the disease. DPOAEs increased in amplitude in 4 patients, but this was not significant. It remains unclear why DPOAEs are not detected despite a subjective hearing improvement and a sufficiently closed air-bone gap at least in middle and low frequencies. The results of our study show that DPOAEs cannot replace behavioral threshold tests; they may only be included in a battery of tests for a complete clinical follow-up for efficiency monitoring after stapes surgery.