Giampietro Ricci

Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience.

AIMS Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear. Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved. The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia. METHODS We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7). The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Childrens Benefit Inventory; rate and type of surgical complications. RESULTS The mean preoperative air- and bone-conduction thresholds were 51.2±12.5 and 14.1±6.3dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3±7.2dB HL, and the mean postoperative threshold with the Baha system was 18.1±7.5dB HL. Quality of life improved for all operated patients. CONCLUSIONS The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted in quality of life, while the rate of complications was very low. Therefore, we are convinced that the Baha system is the treatment of choice for hearing loss due to bilateral congenital aural atresia.

Subjective visual vertical before and after treatment of a BPPV episode

OBJECTIVE The study analyses the behavior of subjective visual vertical (SVV) in benign paroxysmal positional vertigo (BPPV) before and after treatment, and offers a clinical-pathogenic interpretation. METHODS We studied 30 consecutive patients with BPPV of the posterior semicircular canal treated with the Epley repositioning maneuver. SVV was determined at three different stages: at the time of diagnosis (1st test), after the repositioning maneuver (2nd test), and then 7 days after the resolution of the clinical picture (3rd test). The main study parameter was represented by the mean of 6 consecutive measurements (SVV(0)) for each patient. SVV was also examined in 20 healthy subjects, who represented the control group. The comparison between mean values and standard deviations showed a statistical significance of p<0.05. RESULTS During the first test, the degree of deviation of SVV was significantly higher in the patient group than in the control group. Tilting towards the affected side was observed in all cases. The 2nd test showed an inversion in the orientation of SVV in 16 patients, and as a result of the Epley maneuver there was a statistically significant variation in SVV(0) values in 20 patients with respect to the previous test (2nd test vs. 1st test). This involved 87% (23 patients) of those who then had a negative Dix-Hallpike test, and none of the ones in whom paroxysmal positional nystagmus persisted. Lastly, no differences emerged in the behavior of the patient group vs. the control group during the third test. CONCLUSIONS SVV is often altered during active BPPV. The degree of otolithic dysfunction is never high and, in all cases, it is brief in duration. Tilting towards the dysfunctional side is essentially a constant in untreated BPPV. This could be due to a substantial loss of otoconia, with a decrease in the density and specific weight of the macula, and thus hypofunction of the receptor. The observation of a significant variation in SVV after therapeutic maneuvers has a favorable predictive value, as it probably reflects the migration of otoliths to the utricle, where saturation mechanisms can often have irritative effects leading to the inversion of SVV.

Universal newborn hearing screening in Umbria region, Italy.

OBJECTIVE In Italy, universal newborn hearing screening (UNHS) was first introduced in some regions from 1997. Umbria Region has launched a UNHS program in all hospitals, which has been implemented throughout the region since July 2010. Before UNHS implementation in Umbria region, the average age of identification of congenital hearing loss was around 32 months of age with an average age of initial amplification treatment at least 2 months later. The coverage rate of newborn screening was only 34.4% in 2006. The aim of this study was to examine the results of this program and its evolution in the first 2.5 years since implementation in our region. METHODS Since July 2010, all 11 birth centers and hospitals in Umbria region have been involved in a UNHS program. The screening involves the automated otoacoustic emissions (AOAE) test and automatic auditory brain stem response (AABR) audiometry. The number of screening stages and tests used were different depending on whether the infants had audiological risk factors or not. RESULTS A total of 20,841 babies were born in the hospitals involved of whom 20,051 were well born babies (WB), while 790 babies (3.8%) presented identified audiological risk factors (BRF). The overall coverage rate in the study period was 93.8%. The prevalence of hearing loss was 2‰ for WB infants and 4.3% for BRF. Mean age at diagnosis was 5.31±3.95 and 11.28±7.73 months in the WB and BRF groups, respectively. CONCLUSIONS UNHS has allowed us to substantially increase the coverage rates and decrease the mean age at diagnosis and subsequent treatment. The identification of audiological risk factors is very important for adequate screening and follow-up. However the Joint Committee on Infant Hearing 2007 quality indicators and benchmarks for screening have not yet been fully achieved and there is still scope for some improvement. This could be achieved with a closer cooperation among institutions, parents, pediatricians, and ENT doctors.

Personal experience with tinnitus retraining therapy

We present the results of tinnitus retraining therapy (TRT) in a group of patients suffering from tinnitus and/or hyperacusia. Based on the scores from a specific questionnaire and the Tinnitus Handicap Inventory (THI), the patients were classified into five categories and began therapy according to Jastreboff’s criteria. Depending on the individual case, therapy envisaged counselling sessions, ambient sound enrichment, sound generators and hearing aids. At the end of the 18-month period, therapeutic success was observed in 79% of the patients. The initial numerical values of the scale of the symptoms and the THI seem predictive of treatment outcome. The use of instruments (sound generators) increases the success rate, but the study also demonstrates the effectiveness of counselling and ambient sound enrichment. Failures mainly involved patients with hypacusia who refused to wear hearing aids, as this influenced the effectiveness of ambient sound enrichment and counselling. Paralleling the data in the literature, the results demonstrate the effectiveness of TRT, which cannot be attributed to a placebo effect given the extended duration of treatment.

“TuNa-saving” endoscopic medial maxillectomy: a surgical technique for maxillary inverted papilloma

The maxillary sinus is the most common site of sinonasal inverted papilloma. Endoscopic sinus surgery, in particular endoscopic medial maxillectomy, is currently the gold standard for treatment of maxillary sinus papilloma. Although a common technique, complications such as stenosis of the lacrimal pathway and consequent development of epiphora are still possible. To avoid these problems, we propose a modification of this surgical technique that preserves the head of the inferior turbinate and the nasolacrimal duct. A retrospective analysis was performed on patients treated for maxillary inverted papilloma in three tertiary medical centres between 2006 and 2014. Pedicle-oriented endoscopic surgery principles were applied and, in select cases where the tumour pedicle was located on the anterior wall, a modified endoscopic medial maxillectomy was carried out as described in this paper. From 2006 to 2014 a total of 84 patients were treated. A standard endoscopic medial maxillectomy was performed in 55 patients (65.4%), while the remaining 29 (34.6%) had a modified technique performed. Three recurrences (3/84; 3.6%) were observed after a minimum follow-up of 24 months. A new surgical approach for select cases of maxillary sinus inverted papilloma is proposed in this paper. In this technique, the endoscopic medial maxillectomy was performed while preserving the head of the inferior turbinate and the nasolacrimal duct (“TuNa-saving”). This technique allowed for good visualization of the maxillary sinus, good oncological control and a reduction in the rate of complications.

Residual dizziness after successful repositioning maneuver for idiopathic benign paroxysmal positional vertigo: a review

The benign paroxysmal positional vertigo (BPPV) is a vestibular disorder cause of vertigo. The BPPV may be corrected mechanically by repositioning maneuvers but even after successful maneuvers, some patients report residual dizziness for a certain period afterward. Early recognition and treatment might decrease the incidence of residual dizziness in patients with BPPV, especially in those patients with psychiatric comorbidities and in the elderly, lowering the risk of falling. Many pathogenetic hypotheses for residual dizziness are under debate. The purpose of this review was to identify, evaluate and review recent researches about possible causal factors involved in residual dizziness and the implications on clinical practice. A literature search was performed using different databases such as Pubmed and Scopus. The following search terms were used: residual dizziness, otolithic membrane and BPPV. The search found a total of 1192 titles, which were reduced to 963 after a procedure of de-duplication of the found titles. The research was then restricted to an interval of time comprised between 2000 and 2016 for a total of 800 titles. Among these titles, only those including the terms benign paroxysmal positional vertigo were considered eligible for this review. Only publications in English language were taken into consideration and we excluded those with not available abstract. Finally, 90 abstracts were obtained and critically evaluated by two different Authors, and additional studies were identified by hand searching from the references of artiche of interest. Only 53 were included in this work.

Impact of identifying factors which trigger bothersome tinnitus on the treatment outcome in tinnitus retraining therapy.

The aim of this work was to ascertain any differences in the effectiveness of rehabilitation therapy in relation to the presence or absence of a known negative reinforcement responsible for the tinnitus-related pathology. Between 1 January 2001 and 31 December 2008, we recruited 294 subjects suffering from incapacitating tinnitus and/or hyperacusis. The patients underwent tinnitus retraining therapy (TRT) according to the methods described by Jastreboff and Hazell [Tinnitus Retraining Therapy: Implementing the Neurophysiological Model. Cambridge, Cambridge University Press, 2004, pp 121-133]. We clinically assessed the presence or absence of known phenomena of associative learning, regarding the presence of adverse events temporally correlated with tinnitus and the treatment outcome. The separate analysis of the 2 subgroups shows a statistically significant difference in the improvement rate between the group with a known triggering factor and the group without a triggering factor, with a preponderance of the former with a 91% improvement rate versus approximately 56% for the latter. In our study, the inability to identify factors triggering bothersome tinnitus negatively affected the treatment outcome in TRT.

Study of vestibular evoked myogenic potentials in unilateral vestibulopathy: Otolithic versus canal function testing.

Objective: The study provides a qualitative evaluation of unilateral vestibulopathy by comparing otolithic and canal function, to establish possible relationships between the type of dysfunction observed and the evolving clinical pictures associated with it. Study Design: Retrospective study of a series of cases. Setting: Department of Medical-Surgical Specialization, Otolaryngology and Cervicofacial Surgery Division, University of Perugia, Perugia, Italy. Patients: Twenty patients whose medical history showed at least one episode corresponding to the clinical parameters of acute vestibulopathy. Interventions: Study of vestibular function by recording VEMPs and repeating canal function testing at least 6 months after the first episode of vertigo. Main Outcome Measures: Relationship between the type of vestibulopathy (canal and otolithic) and the clinical pictures observed. Results: Paroxysmal positional vertigo, observed in 4 patients, was correlated with the presence of vestibular evoked myogenic potentials (VEMPs) and the absence of an ipsilateral canal response in all cases (100%). Persistent dizziness was observed in nine patients, and VEMPs were absent in all of them (100%); three (33.3%) showed the recovery of previously absent canal function. Comparison of responses in six patients with recurrent acute vestibulopathy showed persistent and complete loss of canal function in five cases (83.3%), whereas impairment of otolithic response was less constant (40%). Conclusion: The combined VEMPs-canal test study shows predictive value regarding certain evolving clinical pictures of vestibulopathy. The absence of VEMPs confirms the role of otolithic dysfunction in the onset of dizziness. Likewise, it suggests that a vestibular origin of these disorders should be considered in cases that have shown aspecific symptoms since onset, without frank vertigo and with normal vestibular response to canal function testing.

Evoked otoacoustic emissions behaviour in retinitis pigmentosa

The hearing function was studied in 26 patients affected by retinitis pigmentosa (RP) and in their relatives. Sixteen patients showed bilateral normal hearing when examined with traditional audiometric methods. In these normoacusic patients evoked otoacoustic emissions (EOE) have been studied. The EOE offer a unique opportunity to measure objectively the function of outer hair cells: they record the amplitude of the energy produced by the outer hair cells of the coclea following an acoustic stimulation. The data have been statistically compared, using the Students t-test, with those obtained in a homogeneous control-group of normal subjects. In normoacusic subjects with RP the average values of EOE intensity are statistically lower than those of normal subjects in 64 of the 127 frequency bands examined. Moreover, the distribution of the EOE in patients with retinitis pigmentosa proved to be more discontinous than that observed in the normal subjects. The EOE recorded in 14 normoacusic relatives show in some cases small anomalies but the data, on account of the limited sample group, cannot be statistically evaluated. Therefore a subclinical alteration of the Organ of Corti is found in 100% of the patients affected by RP, although they appear to be normoacusic to usual audiometric tests.

Ocular and oto-syphilis: not a thing of the past

IgG positive. HIV and HCV antibodies tested both negative. The patient reported engaging in unsafe sex 5 months prior. Secondary syphilis with ocular, otologic and mucous manifestations and hepatitis B infection were diagnosed [1–5]. Aqueous penicillin G 24 million U a day was administered for 14 days along with prednisone. The hepatosplenomegaly, skin rash, oral lesion and visual acuity improved; while the hearing deficit slightly regressed. After the course of intravenous penicillin, penicillin benzathine 2.4 million U weekly for 3 weeks was prescribed [1]. Four months later, RPR titer was 1:2, visual capacity and the tongue were normal (Fig. 1b), while the hearing deficit persisted (Fig. 2b). Ocular and oto-syphilis is not a thing of the past [1–5]. These manifestations may also occur in the first few months of infection, when T. pallidum can cause skin and mucous membrane lesions. During this stage of the disease other types of systemic manifestations can develop; also, rare, uncommon findings [6] and malignant forms have been reported [7]. Other sexually transmitted diseases must be simultaneously investigated for when syphilis is diagnosed.