fMRI in Bell's Palsy: Cortical Activation is Associated with Clinical Status in the Acute and Recovery Phases.

BACKGROUND AND PURPOSE: Using functional magnetic resonance imaging (fMRI), we explored cortical activation in patients with acute Bell's palsy (BP) and analyzed its correlates with clinical status in the acute phase, and with 6-month outcome. METHODS: Twenty-four right-handed patients with acute BP within 15 days of onset and 24 healthy controls underwent fMRI during performance of unilateral active (hemi-smiling) and passive lip movement tasks with both the paretic and the normal lip. The degree of paresis was evaluated during the acute stage and at the 6-month follow up using the House-Brackmann (HB) grading scale. Complete recovery was defined as HB grade II or less at the end of the 6-month period. The difference in the HB grade (ΔHB) between the acute stage and the 6-month follow up was used to evaluate clinical improvement. RESULTS: There were 24 patients with unilateral acute BP. HB grades ranged from III to VI. At 6 months, 11 patients (46%) had completely recovered and 12 (50%) were partially improved. Compared with healthy subjects, BP patients had a significantly greater activation of the frontal areas and the insula ipsilateral to the paretic side. In BP patients, there was an inverse correlation between the activation of the ipsilateral hemisphere when moving the paretic side and the degree of paresis at baseline. An association was also observed between activation and clinical outcome (both complete recovery and ΔHB). CONCLUSIONS: In patients with BP, fMRI may represent a useful tool to predict long-term outcome, guide therapeutic approach, and monitor treatment response.

Temporal Fine Structure Processing, Pitch, and Speech Perception in Adult Cochlear Implant Recipients.

OBJECTIVES: The aim of the study was to investigate the link between temporal fine structure (TFS) processing, pitch, and speech perception performance in adult cochlear implant (CI) recipients, including bimodal listeners who may benefit better low-frequency (LF) temporal coding in the contralateral ear. DESIGN: The study participants were 43 adult CI recipients (23 unilateral, 6 bilateral, and 14 bimodal listeners). Two new LF pitch perception tests-harmonic intonation (HI) and disharmonic intonation (DI)-were used to evaluate TFS sensitivity. HI and DI were designed to estimate a difference limen for discrimination of tone changes based on harmonic or inharmonic pitch glides. Speech perception was assessed using the newly developed Italian Sentence Test with Adaptive Randomized Roving level (STARR) test where sentences relevant to everyday contexts were presented at low, medium, and high levels in a fluctuating background noise to estimate a speech reception threshold (SRT). RESULTS: Although TFS and STARR performances in the majority of CI recipients were much poorer than those of hearing people reported in the literature, a considerable intersubject variability was observed. For CI listeners, median just noticeable differences were 27.0 and 147.0 Hz for HI and DI, respectively. HI outcomes were significantly better than those for DI. Median STARR score was 14.8 dB. Better performers with speech reception thresholds less than 20 dB had a median score of 8.6 dB. A significant effect of age was observed for both HI/DI tests, suggesting that TFS sensitivity tended to worsen with increasing age. CI pure-tone thresholds and duration of profound deafness were significantly correlated with STARR performance. Bimodal users showed significantly better TFS and STARR performance for bimodal listening than for their CI-only condition. Median bimodal gains were 33.0 Hz for the HI test and 95.0 Hz for the DI test. DI outcomes in bimodal users revealed a significant correlation with unaided hearing thresholds for octave frequencies lower than 1000 Hz. Median STARR scores were 17.3 versus 8.1 dB for CI only and bimodal listening, respectively. STARR performance was significantly correlated with HI findings for CI listeners and with those of DI for bimodal listeners. CONCLUSIONS: LF pitch perception was found to be abnormal in the majority of adult CI recipients, confirming poor TFS processing of CIs. Similarly, the STARR findings reflected a common performance deterioration with the HI/DI tests, suggesting the cause probably being a lack of access to TFS information. Contralateral hearing aid users obtained a remarkable bimodal benefit for all tests. Such results highlighted the importance of TFS cues for challenging speech perception and the relevance to everyday listening conditions. HI/DI and STARR tests show promise for gaining insights into how TFS and speech perception are being limited and may guide the customization of CI program parameters and support the fine tuning of bimodal listening.

Adaptation of the STARR test for adult Italian population: A speech test for a realistic estimate in real-life listening conditions.

OBJECTIVES: To introduce the Italian adaptation of the STARR test based on a roving-level adaptive method to mimic challenging real-life listening conditions for use in people with auditory prostheses. DESIGN: Normative data were collected and interlist-variability, as well as learning effects, were investigated using a within-subject design with repeated measures. STUDY SAMPLE: A group of 32 normal-hearing (NH) adults participated in the study. RESULTS: The average speech reception threshold (SRT) for NH subjects was -8.4 dB SNR. The variability of mean SRTs across test lists was relatively small (≤1 dB for all test lists). The statistically significant differences between lists were eliminated after applying correction factors. On the basis of variability for the corrected SRTs within each subject, a difference of 2.8 dB in SRT was meaningful for outcome comparisons using one test list per condition and 2 dB using two lists per condition. Statistical analysis did not show any significant learning effects. CONCLUSIONS: Findings in NH listeners suggested that the Italian STARR test could be a promising supplement to existing speech assessment tools. Further studies in populations with hearing impairment could contribute to cross-language studies.

Tinnitus in patients with temporo-mandibular joint disorder: Proposal for a new treatment protocol.

The present study was designed to verify the correlation between tinnitus and temporomandibular joint dysfunction.86 consecutive patients were enrolled in the study, all affected by subjective tinnitus without hearing impairment, from both genders, age between 18 and 60 years old. The final number of patients included in the study was 55. All patients received a temporo-mandibular joint examination. All the patients were asked to rate the severity of their symptoms before and after treatment using a VAS scale and the Tinnitus Handicap Inventory (THI) and they followed a standardized protocol for the investigation of tinnitus. All the subjects were monitored by the same researcher and they underwent the same splint treatment. The comparison between pre- and posttreatment phase scores showed in patients with predisposition of TMD and with TMD a statistically significant decrease of THI and VAS values. The characteristics of tinnitus and the degree of response to treatment confirmed the relationship between tinnitus and TMD. The authors believe that, when the most common causes of tinnitus, such as otologic disorders and neurological diseases are excluded, it is correct to evaluate the functionality of the temporo-mandibular joint and eventually treat its pathology to obtain tinnitus improvement or even resolution.

Adequate formal language performance in unilateral cochlear implanted children: is it indicative of complete recovery in all linguistic domains? Insights from referential communication.

OBJECTIVES: Referential communication (RC) is a key element in achieving a successful communication. This case series aimed to evaluate RC in children with unilateral cochlear implants (CIs) with formal language skills within the normal range. METHODS AND MATERIALS: A total of 31 children with CIs, with language development within the normal range, were assessed using the Pragmatic Language Skills test (MEDEA). RESULTS: Of the children with CIs, 83.9% reached performance levels appropriate for their chronological ages. The results confirmed a positive effect of cochlear implantation on RC development, although difficulties remained in some CI users. CONCLUSIONS: The outcomes emphasize the need to pay greater attention to the pragmatic aspects of language, assessing them with adequate testing in the early phase after cochlear implantation. Clear knowledge of children's communicative competence is the key in optimizing their communicative environments in order to create the basis for future successful interpersonal exchanges and social integration.

Bell's palsy: symptoms preceding and accompanying the facial paresis.

This individual prospective cohort study aims to report and analyze the symptoms preceding and accompanying the facial paresis in Bell's palsy (BP). Two hundred sixty-nine patients affected by BP with a maximum delay of 48 hours from the onset were enrolled in the study. The evolution of the facial paresis expressed as House-Brackmann grade in the first 10 days and its correlation with symptoms were analyzed. At the onset, 136 patients presented postauricular pain, 114 were affected by dry eye, and 94 reported dysgeusia. Dry mouth was present in 54 patients (19.7%), facial pain, hyperlacrimation, aural fullness, and hyperacusis represented a smaller percentage of the reported symptoms. After 10 days, 39.9% of the group had a severe paresis while 10.2% reached a complete recovery. Dry mouth at the onset was correlated with severe grade of palsy and was prognostic for poor recovery in the early period. These outcomes lead to the deduction that the nervus intermedius plays an important role in the presentation of the BP and it might be responsible for most of the accompanying symptomatology of the paresis. Our findings could be of important interest to early address a BP patient to further examinations and subsequent therapy.

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.

Basal cell carcinoma masked in rhinophyma.

Rhinophyma, the advanced stage of rosacea, is a lesion characterized by progressive hypertrophy and hyperplasia of sebaceous glandular tissue, connective tissue, and blood vessels. Rhinophyma can lead to a significant facial disfigurement and severe emotional distress, but it is not only an aesthetic problem, since rare cases of simultaneous presence of malignant tissue are described in the literature. The case of an 84-year-old farmer affected by basal cell carcinoma (BCC) and diagnosed in the context of rhinophyma is presented. The anatomical distortion produced by the chronic inflammation and fibrous scarring makes the BCC diagnosis difficult and uncertain. The histological examination of the entire mass and its margins is fundamental. A partial biopsy can lead to a false negative result, and the histological examination must be repeated intra- or postoperatively.

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.

New development in intraoperative video monitoring of facial nerve: a pilot study.

OBJECTIVE: The present study proposes an evolution of an intraoperative video-based facial nerve monitoring system for otologic and otoneurosurgical procedures in comparison with classical electromyographic (EMG) monitoring method. STUDY DESIGN: Single-subject design study. SETTING: Tertiary referral center, university clinic. PATIENTS: Fifteen patients undergoing a translabyrinthine approach for removal of 2 cm or less acoustic neuroma. INTERVENTION: Intraoperative monitoring during acoustic neuroma removal. MAIN OUTCOME MEASURES: Measurement of mouth angle displacement and EMG peak amplitude at different stimulation intensities were measured and compared to evaluate the validity and reliability of the proposed new system. RESULTS: The electrophysiologic method has been shown to be slightly more sensitive, although it shows limitations especially in terms of electrical artifacts during cauterization, totally masking the EMG monitoring. CONCLUSION: The present version of video system can be considered as valid and reliable as EMG; its main advantages are the absence of electrical artifacts, less invasivity, and much less cost.

Haematoma after cochlear implantation: management of a minor complication.

CONCLUSIONS: The dimensions of the implant receiver and the material used have influenced the surgical approach leading to a reduction in complications. Ultrasonography of the haematoma is useful in the evaluation of dimensions, entity of fluid component and therapeutic options. OBJECTIVE: Haematoma arising in the receiver area is considered a minor complication, nevertheless it can be complicated by infection and/or flap necrosis or fibrosis leading to difficulties in magnetic adherence of the receiver and rarely to explantation of the receiver. The objective of the study was to evaluate the clinical outcome of postoperative haematoma arising after cochlear implant surgery. PATIENTS AND METHODS: This was a retrospective case series of 22 cochlear implant patients who developed post-implant haematoma over the receiver area. Haematoma extension and fluid collection were analysed via ultrasonography, implant type and predisposing factors such as trauma, coagulation disorders and type of skin incision. RESULTS: Patients were divided into four groups on the basis of the main predisposing factor: coagulation disorders (n=7), trauma (n=3), revision surgery (n=6) and haematoma of unknown origin (n=6). The main factors correlated to haematoma onset were coagulation disorder, type of skin incision and flap revision. In all, 21 subjects had complete recovery and the speech perception performance was not compromised over time, while 1 subject (0.3%) with an extensive haematoma due to a pharmacologically induced coagulation disorder required explantation of the device.

Indication for surgery in otosclerotic patients with unilateral hearing loss.

OBJECTIVE: The aim of this study is to evaluate postoperative spatial speech discrimination in noise in a group of otosclerotic patients with unilateral hearing loss in a 6-month follow-up. Moreover, an additional objective is to verify if our routine criteria (air-bone gap >30 dB at 250-2,000 Hz) for surgical indication in such patients can be acceptable. STUDY DESIGN: Prospective evaluation in 20 patients divided into 2 groups: unilateral otosclerosis and bilateral otosclerosis already successfully operated on 1 side and planned for stapedotomy on the contralateral ear. SETTING: Tertiary referral center, University clinic. PATIENTS: Otosclerotic patients with unilateral hearing loss. INTERVENTION: Evaluation of functional outcome. MAIN OUTCOME MEASURE: Spatial test based on speech discrimination in noise. RESULTS: All patients reach postoperative air-bone gap closure within 10 dB. Average postoperative gain in discrimination under noise was 11.5% for unilateral otosclerosis group and 19.3% for the second ear group. CONCLUSION: Free-field discrimination in noise closely reflects the usual listening conditions in everyday life. Improvement in postoperative free-field discrimination in noise can justify our routine criteria for surgical indication in the patients with unilateral otosclerosis and with bilateral otosclerosis already operated on 1 side. The surgical choice for each patient, apart from audiologic evaluation, is linked to a truly informed consent regarding possible advantages and risks mainly based on an analysis of the real auditory needs of the patient and individual results of the surgeon.

A new integrated system for neural stimulation and bipolar coagulation: preliminary study.

CONCLUSION: The proposed device can be useful for promoting safety during bipolar coagulation in many surgical procedures and/or regions. OBJECTIVE: A new system that integrates a neural stimulator and a bipolar coagulator is proposed, to improve neural (facial nerve) safety during ENT surgical procedures. This study aimed to evaluate the system. MATERIALS AND METHODS: A home-made constant current neural stimulator (0.1-2 mA, pulsed, duration 200 micros), was connected via an automatic switch to a commercial bipolar microcoagulator (Medicon Instrumente mod. 101, Germany). Under general anesthesia, the extra-petrous portion of the facial nerve was identified in three rabbits. The adequate functioning of the device was tested via: 1) neural stimulation and EMG monitoring in basal conditions; 2) bipolar coagulation of connective tissue surrounding the facial nerve; 3) final assessment of the functional integrity of the nerve, via an intraoperative EMG and a postoperative evaluation. RESULTS: Data obtained from all three rabbits provided adequate results in terms of neural stimulation, correct coagulation of juxta-nervous tissue, and functional integrity of the facial nerve, both intraoperatively and postoperatively.

Follow-up of cochlear implant use in patients who developed bacterial meningitis following cochlear implantation.

OBJECTIVES/HYPOTHESIS: The present study is a long-term follow-up of speech perception outcomes and cochlear implant use in three cases of meningitis that occurred after cochlear implantation. STUDY DESIGN: Case series study. METHODS: Study was performed on three children implanted with different models of Clarion devices, two of them with positioner. Recognition and comprehension were assessed via the Italian adaptation of GASP (TAP) test, and phonetically balanced bi-syllabic words in open-set. High resolution computed tomography scan acquisition was performed to obtain axial coronal and oblique multiplanar reconstructions of the cochlea. RESULTS: Two patients were affected by enlarged cochlear acqueduct and Mondini malformation the first carrying positioner. One patient had a normal cochlea, and the positioner could have been the main cause of bacterial spread. As a consequence of meningitis the child with normal cochlea and the other with enlarged vestibular acqueduct developed cochlear ossification, increased M-level and worsening of hearing outcomes. The child with Mondini malformation developed facial nerve stimulation. Contralateral implantation was performed in the first two patients. CONCLUSION: Bacterial meningitis occurring after cochlear implantation may induce cochlear ossification, facial nerve stimulation, and permanent or temporary loss of implant use. Planned follow-up with high resolution computed tomography and evaluation of M-levels could be useful prognostic tools in the management of these patients.

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.