The Prevalence of Chronic Cerebrospinal Venous Insufficiency in Meniere Disease: 24-Month Follow-up after Angioplasty.

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.

Oral versus short-term intratympanic prednisolone therapy for idiopathic sudden hearing loss.

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.

Inferences and metaphoric comprehension in unilaterally implanted children with adequate formal oral language performance.

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.

A 9-year-old boy with atypical retroauricular pain: a case report.

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.

Pneumolabyrinth after intratympanic steroid injection in patient with prosthesis of the stapes: a case report.

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.

Intratympanic steroid therapy in moderate sudden hearing loss: a randomized, triple-blind, placebo-controlled trial.

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.

Long term results in late implanted adolescent and adult CI recipients.

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.

Long-term follow-up of implanted children with cytomegalovirus-related deafness.

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.

Hyperbaric oxygen therapy with short duration intratympanic steroid therapy for sudden hearing loss.

CONCLUSION: 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. OBJECTIVE: 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. METHODS: 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 PTA >90 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. RESULTS: The overall success rate was superior in the group submitted to IT steroid and HBO therapy. Nevertheless, these clinical results were not statistically significant.

Genetic prothrombotic factors in children with otogenic lateral sinus thrombosis: five case reports.

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 Gesù 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.

Cyclo-oxygenase enzyme in the perilymph of human inner ear.

CONCLUSIONS: 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. OBJECTIVES: 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. METHODS: 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. RESULTS: 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 importance of intra-operative imaging during cochlear implant surgery.

Two unusual cases of cochlear implant (CI) surgery complication are reported: introduction of the electrode array into the superior semicircular canal with normal morphology and a growing amplitude of neural potential (neural response imaging, NRI) during intra-operative monitoring control. In the first case, a two-year-old patient affected by congenital sensorineural profound deafness was bilaterally implanted with two Clarion 90 k devices and intra-operative electrophysiological and radiological controls were performed. After introduction of the array in the right side NRI was performed and a neural potential was found only on two apical electrodes. Radiological intra-operative control with antero-posterior trans-orbital plain films was performed to assess the position of the electrodes inside the cochlea. Radiography showed the electrode array in the superior semicircular canal in the right ear. The electrode array was removed and reinserted correctly. In the second case, a 72-year-old man underwent left cochlear implantation for sensorineural profound deafness of unknown origin. Intra-operative electrophysiological testing (NRI) showed the presence of neural potential on three tested channels. In this case, as routinely employed since 2006, an intra-operative static fluoroscopy control was performed, this showed the electrode array in the superior semicircular canal. The electrode array was removed and reinserted correctly. In conclusion, intra-operative monitoring tests during CI surgery play different roles: measurement of impedances and NRI can evaluate the integrity of implant electrodes and the status of the electrode cochlea interface, but they cannot be the only way to confirm correct positioning of the array. In our opinion the intra-operative radiological check is helpful during CI surgery, especially when there is any doubt about correct electrode insertion.

Positional vertigo and cochlear implantation.

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.

Post-operative Herpes simplex virus encephalitis after surgical resection of acoustic neuroma: a case report.

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.