The European accreditation of Istituto Tumori Giovanni Paolo II of Bari.

The National Cancer Institute of Bari (Istituto di Ricovero e Cura a Carattere Scientifico, IRCCS) has been involved since the conception of the project of the Italian Ministry for Health aimed to validate the applicability of the Organisation of European Cancer Institutes (OECI) accreditation and designation (A&D) model to the Network of Italian Cancer Centers, IRCCS, of Alleanza Contro il Cancro. The self-assessment phase of the Institute started in September 2013 and ended in June 2014. All documents and tools were transferred to the OECI A&D Board in June 2014 and a 2-day peer review visit was conducted in October 2014 by an international qualified audit team. The Institute received its final designation and certification in June 2015. The OECI A&D Board, in its final report, came to the conclusion that Istituto Tumori "Giovanni Paolo II" of Bari has a strong research component with some essential elements of comprehensive cancer care still under development; the lack of a system for using outcome data for the strategic management approach to decision-making and missing a regular internal audit system eventually helping further quality improvement were reported as examples of areas with opportunities for improvement. The OECI A&D process represented a great opportunity for the cancer center to benchmark the quality of its performance according to standard parameters in comparison with other international centers and to further develop a participatory group identity. The common goal of accreditation was real and participatory with long-lasting positive effects. We agree with the OECI comments about the next areas of work in which the Institute could produce future further efforts: the use of its powerful IT system as a means for outcome analysis and empowerment projects for its cancer patients.

Quality of life after cholesteatoma surgery: intact-canal wall tympanoplasty versus canal wall-down tympanoplasty with mastoid obliteration.

OBJECTIVES: The aim of this study was to evaluate, by means of the Chronic Ear Survey (CES), the quality of life of patients who had undergone either intact-canal wall tympanoplasty (ICWT) or canal wall-down tympanoplasty (CWDT) with mastoid obliteration. METHODS: This was a retrospective case review study performed at a tertiary referral center. Among 379 patients affected by middle ear and mastoid cholesteatoma operated on between November 2000 and December 2009, 50 patients who underwent ICWT and 50 who underwent CWDT with mastoid obliteration were randomly selected. The CES scores were analyzed for both groups. RESULTS: The mean scores on the CES were 6.5 ± 2.1 in patients who underwent CWDT and 6.9 ± 2.2 in patients treated with ICWT (t = -0.93; p > 0.05). No significant differences between the two groups were found on the activity restriction, symptom, or medical resource subscales (p > 0.05). CONCLUSIONS: The results of this study demonstrate that CWDT with mastoid obliteration resulted in a quality of life comparable with that after ICWT. Postoperative hearing loss is the most frequently reported problem for both techniques.

[Oncological outcomes of laparoscopic and open treatment (nephroureterectomy) for urothelial tumors of the upper urinary tract]. / Risultati oncologici del trattamento open e laparoscopico (nefroureterectomia) per i tumori uroteliali dell'alta via escretrice.

Currently, the treatment of choice in urothelial tumors of the upper urinary tract is nephroureterectomy (NU) as an Open procedure (ONU), though the laparoscopic treatment is now routinely performed as a minimally invasive therapy (LNU). LNU has demonstrated oncologic safety at least equivalent to open, but some issues dealing with cancer still remain. We retrospectively analyzed data from 36 LNU performed between 2006 and 2010, compared with data of 32 ONU performed in 2002-2005 (pre-laparoscopy era). The mean follow-up was 23 months in patients undergoing LNU and 42 months for those treated with ONU. In particular, we evaluated cancer recurrence, the site of recurrence and survival rates. We had local recurrence in 3 patients (8.3%) after LNU and 2 after ONU (6.25%). 2 patients who underwent LNU (5.5%) died of metastatic disease at 9 and 12 months; 3 patients who underwent ONU (9.3%) died of metastasis at 12, 16 and 23 months, respectively. Bladder recurrence was observed in 3 patients after ONU and in 4 after LNU. The most frequent sites of cancer recurrence were: local recurrence (3 LUN, 2 ONU), 1 laparoscopic port recurrence, 3 regional lymph node recurrences (2 LNU, 1ONU), bladder recurrences (3 LNU, 4 ONU). There were no significant differences in disease recurrence and even survival rates at 1 and 3 years were not very different between the two techniques. The grade and stage of cancer affecting the incidence of metastatic disease, as well as the localization of early disease (pelvis-ureter-both) is a negative prognostic factor, rather than the surgical technique used. Therefore, there is no evidence that the control is compromised in cancer patients treated with LNU rather than with ONU.

Hearing results using titanium ossicular replacement prosthesis in intact canal wall tympanoplasty for cholesteatoma.

CONCLUSIONS: Titanium proved to be a valuable alternative to ossicles in ossiculoplasty procedures. Complication rates are comparable to those obtained by other authors with titanium and nontitanium prostheses. Hearing results were worse compared with other reports; however, no conclusion can be drawn on the basis of the literature because of the heterogeneity of the populations studied. OBJECTIVES: To evaluate the results and complications of total (TORP) and partial (PORP) titanium ossicular replacement prostheses in middle ear cholesteatoma. METHODS: Fifty-seven patients affected by acquired cholesteatoma of the middle ear undergoing titanium ossiculoplasty during second stage intact canal wall tympanoplasty were evaluated. Postoperative hearing gain, complication rate, and revision rate were analyzed. RESULTS: Average postoperative gain was 13.6 dB HL for PORP and 17.9 dB HL for TORP. After ossicular reconstruction the mean postoperative ABG was 24.1 dB HL in PORPs and 27.2 dB HL in TORPs. The difference in air-bone gap (ABG) between the two groups after ossiculoplasty was not significant. The number of patients with an ABG > 30 dB was higher in the TORP group compared with the PORP group (p = 0.024) after ossicular reconstruction. The total extrusion rate was 5.2% and the total revision rate was 10.5%.

Endothelial progenitor cells in sudden sensorineural hearing loss.

CONCLUSIONS: Endothelial progenitor cells (EPCs) are a unique subtype of circulating cells with properties similar to those of embryonal angioblasts. They have the potential to proliferate and to differentiate into mature endothelial cells. EPCs are reduced in patients with vascular risk factors due to a decreased mobilization, an increased consumption at the site of damage or a reduced half-life. The results of this study confirm the existence of an endothelial dysfunction in patients with sudden sensorineural hearing loss (SSHL) and support the vascular involvement in the pathogenesis of the disease. OBJECTIVE: The aim of this study was to evaluate the concentration of EPCs in patients affected by SSHL. METHODS: Twenty-one patients affected by SSHL were evaluated. The number of EPCs was analyzed by flow cytometry analysis of peripheral blood CD34+KDR+CD133+ cells. RESULTS: Circulating levels of EPCs were significantly lower in SSHL patients compared with controls. In particular, CD34+KDR+ cells and CD34+CD133+KDR+ cells were significantly reduced (p < 0.05).

Surgical treatment of labyrinthine fistula in cholesteatoma surgery.

OBJECTIVE/HYPOTHESIS: Evaluate the treatment of labyrinthine fistula in a large series of middle ear cholesteatomas. STUDY DESIGN: Case series in a tertiary referral center. METHODS: Between January 2001 and December 2007, 361 ears affected by mastoid and middle ear cholesteatoma were operated at our institution. The incidence of labyrinthine fistula, preoperative and postoperative hearing function, preoperative symptoms, type of surgery, and intraoperative findings were all analyzed. RESULTS: The incidence of labyrinthine fistula was 12.7 percent. During surgery the matrix over the fistula was removed in all but one case. A labyrinthine fistula occurred in larger cholesteatomas as demonstrated by the higher number of cases with more than two sites involved (P < 0.001), facial nerve exposed (P < 0.001), and stapes superstructure eroded (P = 0.010). Postoperative change of bone conduction threshold and postoperative dead ears were not significantly different between fistula and nonfistula cases. CONCLUSIONS: The preservation of the bone conduction threshold is a common finding in small fistulas and can be obtained also in "large" fistulas when appropriate surgical technique is used. In fistulas involving the promontory the matrix should be left in situ when the endosteum is involved.

The effect of unilateral multichannel cochlear implant on bilaterally perceived tinnitus.

CONCLUSIONS: Available multichannel cochlear implants (CIs) provide effective tinnitus suppression. More sophisticated speech strategies are more effective than analogue or slow strategies. The mechanisms by which tinnitus is suppressed by CIs are unclear; however, both acoustic masking and reorganization of the right auditory association cortex induced by the CI are possible mechanisms. CI significantly reduced the tinnitus-related Handicap as assessed by the Tinnitus handicap Inventory (THI). OBJECTIVE: The objective of the study was to evaluate the effects of a unilateral CI on bilaterally perceived tinnitus. PATIENTS AND METHODS: Forty-one profoundly deaf patients implanted with a multichannel CI reporting bilateral tinnitus were evaluated. All patients were asked to complete a questionnaire that evaluated the presence, location and intensity of tinnitus before and after cochlear implantation. RESULTS: Seven patients (17%) reported the perception of a 'new tinnitus' after surgery. With the CI off tinnitus was abolished in 23 patients (56.1%) in the implanted ear and in 22 patients (53.6%) in the contralateral ear. With the CI on tinnitus was abolished in the ipsilateral ear in 27 patients (65.8%) and in the contralateral ear in 27 patients (65.8%). Statistical analysis showed a significant reduction of the total THI score and of each subscale score (p < 0.001).

A rare case of jugular foramen schwannoma arising from Jacobson's nerve.

The jugular foramen (JF) region is a complex area of the cranial base where venous structures such as the jugular bulb and the inferior petrosal sinuses are strictly related to the lower cranial nerves IX, X and XI. The most common tumours include glomus jugulare, schwannomas of the mixed cranial nerves (IX-XI) and meningiomas. Schwannomas involving the jugular foramen are rare neoplasms and in most of the cases are thought to originate from the X cranial nerve. We report a case of a schwannoma of the JF diagnosed at an early stage, allowing radiological and surgical evidence to support its origin from the tympanic branch of the IX cranial nerve. To our knowledge this is the first case reported in the literature of such a tumour.

Facial paralysis associated with cholesteatoma: a review of 13 cases.

OBJECTIVE: Report the clinical presentation and outcome of the cases of 13 patients with facial paralysis and acquired middle ear and mastoid cholesteatoma. PATIENTS: Patients with acquired cholesteatoma of the middle ear presenting with facial paralysis. INTERVENTIONS: Surgical treatment of the cholesteatoma and decompression of the facial nerve. MAIN OUTCOME MEASURES: The type and the timing of surgery, the intraoperative findings, and the postoperative facial nerve results were analyzed and related to the preoperative facial nerve function. RESULTS: All patients treated less than 7 days after the onset of the paralysis showed a normal facial function at long-term follow-up; patients who were operated on 7 days or more after the onset of paralysis showed a variable outcome. Statistical analysis showed a bigger number of House-Brackmann grade I in patients operated on within the first week of paralysis (p = 0.031). CONCLUSION: The prognosis of facial paralysis is related to the time of intervention. Early diagnosis and treatment of cholesteatoma represent, however, the mainstay of treatment.

Spindle cell carcinoma of the external auditory meatus with intracranial extension: histological, immunohistochemical and electron microscopic evaluation.

We present a case of squamous spindle cell carcinoma of the external auditory meatus in a 38-year-old man. The tumour was extended to the inner ear, the temporal bone, the middle cranial fossa and the meningo-cerebral tissue. The surgical intervention of temporo-occipital craniotomy removed most of the neoplasia. At pathologic examination, the tumour showed an undifferentiated spindle cell pattern. Immunohistochemistry with a large antibody panel found a weak positivity only to EMA. The diagnosis was made when the electron microscopy showed rare junctional structures and tonofilaments.

Cochlear implantation in otosclerosis.

OBJECTIVE: The purpose of this study was to evaluate and compare the results obtained in a group of implanted otosclerotic patients with a group of cochlear implant (CI) patients not affected by otosclerosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Nine patients affected by profound sensorineural hearing loss caused by otosclerosis and nine patients affected by profound sensorineural hearing loss not caused by otosclerosis were evaluated. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Preoperative imaging, intraoperative findings, postoperative facial nerve stimulation, and speech perception performance measures were performed and the data analyzed. RESULTS: Otosclerosis patients showed signs of cochlear ossification both on high-resolution computed tomography scans and intraoperatively. The incidence of facial nerve stimulation was higher in the otosclerosis group, three out of nine, and was generally related to the use of electrical stimulation from the Nucleus 22 cochlear implant. Psychophysical and speech perception measures did not show significant differences between the two groups, despite some otosclerosis patients showing increased electrical thresholds and comfort levels and slightly poorer speech perception performance scores. CONCLUSIONS: Patients with otosclerosis who have progressed to profound hearing loss derive significant benefit from cochlear implants; however, an increased risk of cochlear ossification and facial nerve stimulation has to be taken in account during preoperative counseling. The advance in imaging techniques, CI technology and the possibility to stimulate precise regions of the cochlea with lower intensities make it possible for the surgeons and audiologists to readily and successfully manage these complications as they arise.

Effects of efferent acoustic reflex activation on psychoacoustical tuning curves in humans.

CONCLUSIONS: The results show that, in humans, activation of the contralateral EAR makes the PTC narrower at 1 kHz but wider at 4 kHz. These data are consistent with those reported previously in animals and demonstrate that, during medial efferent stimulation in humans, frequency resolution is improved at low frequencies but impaired at high frequencies. OBJECTIVE: To evaluate, in humans, the effect of activation of the contralateral efferent acoustic reflex (EAR) on the psychoacoustical tuning curves (PTCs) recorded for 1- and 4-kHz probe tones. MATERIAL AND METHODS. Ten young (20-30 years) volunteers served as subjects. They had normal hearing (thresholds <20 dB HL in the frequency range 0.25-8 kHz) and a functioning EAR (contralateral suppression of transient-evoked otoacoustic emissions > or = 0.8 dB). Frequency resolution was evaluated using PTCs. PTCs were recorded at 1 and 4 kHz using a simultaneous masking method. Q10 and Q20 were calculated as the ratio between the test frequency and the bandwidth of the PTC at 10 and 20 dB above the tip of the curve, respectively. The EAR was activated with a 40-dB SL contralateral narrow-band noise centered on the characteristic frequency of the PTC (1 or 4 kHz). Q10 and Q20 were measured in the presence and absence of the contralateral noise. RESULTS: Activation of the EAR led to a significant increase (p < 0.001) in Q10 at 1 kHz and a significant decrease (p <0.001) at 4 kHz. Changes in the value of Q20 were not significant.

Air and bone conduction change after stapedotomy and partial stapedectomy for otosclerosis.

OBJECTIVE: The aim of this study was to analyze and compare the results obtained in otosclerosis patient undergoing stapedotomy and partial stapedectomy. STUDY DESIGN AND SETTINGS: Retrospective review of surgical series. The guidelines of the American Academy of Otolaryngology-Head and Neck Surgery for the evaluation of results of treatment of conductive hearing loss were used. RESULTS: Pure tone average (0.5 to 3 kHz) air-bone gap was 6.1 dB in the partial stapedectomy and 6 dB in the stapedotomy group. The air-bone gap (ABG) closure rate did not differ between the 2 groups, except at 4 kHz, where stapedotomy group showed greater closure (P 0.003). Mean postoperative ABG gain was significantly (P < 0.05) higher in the stapedotomy group at 2, 3, and 4 kHz. Mean postoperative air-conduction gain did not differ significantly. Mean postoperative bone conduction (BC) change (1, 2, and 4 kHz) was 3.68 dB in partial stapedectomy and -0.02 dB in stapedotomy group, the difference being significant (P 0.007). Differences in BC change between the 2 groups were significant at each frequency. CONCLUSIONS: Similar good results can be obtained in experienced hands using either partial stapedectomy or stapedotomy technique. ABG closure rates were analogous in the 2 techniques as well as the complication rate. Although stapedotomy obtain better results at high frequencies, partial stapedectomy is associated with increased BC threshold at all frequencies.

The effects of 'supra-physiological' vitamin B12 administration on temporary threshold shift.

The aim of this study was to evaluate, in 20 young volunteer subjects, the effects of supra-physiological vitamin B12 administration on noise-induced temporary threshold shift (TTS). All subjects had hearing thresholds within 15 dBHL and type A tympanograms. The subjects were randomly assigned to two different groups. Experimental group subjects received cyanocobalamin, 1 mg daily for 7 days, and 5 mg on the eighth day. Control group subjects received a placebo injection daily for 8 days. The vitamin B12 concentration, hearing thresholds and TTS2 (10 min of exposure, narrowband noise centred at 3 kHz, bandwidth of 775 Hz, 112 dBSPL) were measured before and 8 days after treatment. At the end of treatment, the serum vitamin B12 concentration was significantly increased in the experimental group. After 8 days of treatment, the control group showed the same hearing thresholds and TTS2 degrees. Statistical analysis showed that TTS2 decreased significantly at 3 and 4kHz when cobalamin was used to increase the serum concentration of vitamin B12 to > 2350 pg/ml. In addition, a protective effect at 3 kHz in the experimental group was evident when compared with the placebo group. These results suggest that elevated plasma cyanocobalamin levels may reduce the risk of hearing dysfunction resulting from noise exposure in healthy, young subjects.

Cochlear implants: indications in groups of patients with borderline indications. A review.

Cochlear implants (CI) represent the current treatment for patients affected by profound sensorineural hearing loss (SNHL). Initially only deaf adult patients were considered to be candidates for a CI; however, the development of technology and matured experience have expanded the indications for cochlear implantation. Today, CIs are implanted in adults and children and broader indications are followed. There are, however, a number of patients who do not completely fulfill the current indications and who are potential candidates for CI. The duration of deafness and residual hearing represent prognostic indicators for CI performance; however, the candidacy of children with residual hearing and prelingually deafened adults are still under debate. Anatomical variants such as cochlear ossification, cochlear malformation and chronic otitis media represented and still represent for some surgeons a contraindication to CI. The otological experience of CI surgeons and the advent of auditory brainstem implants have changed the approach to these patients, who may still benefit from hearing rehabilitation. This paper briefly analyses and reviews the results obtained in these groups of patients, who were not, at least initially, considered to be candidates for cochlear implantation.

Cochlear implants: indications in groups of patients with borderline indications. A review.

Cochlear implants (CI) represent the current treatment for patients affected by profound sensorineural hearing loss (SNHL). Initially only deaf adult patients were considered to be candidates for a CI; however, the development of technology and matured experience have expanded the indications for cochlear implantation. Today, CIs are implanted in adults and children and broader indications are followed. There are, however, a number of patients who do not completely fulfill the current indications and who are potential candidates for CI. The duration of deafness and residual hearing represent prognostic indicators for CI performance; however, the candidacy of children with residual hearing and prelingually deafened adults are still under debate. Anatomical variants such as cochlear ossification, cochlear malformation and chronic otitis media represented and still represent for some surgeons a contraindication to CI. The otological experience of CI surgeons and the advent of auditory brainstem implants have changed the approach to these patients, who may still benefit from hearing rehabilitation. This paper briefly analyses and reviews the results obtained in these groups of patients, who were not, at least initially, considered to be candidates for cochlear implantation.

Effect of ipsilateral and contralateral low-frequency narrow-band noise on temporary threshold shift in humans.

OBJECTIVE: To evaluate the effect of ipsilateral and contralateral low-frequency (250 Hz) narrow-band noise (NBN) on the temporary threshold shift (TTS) induced by a 2 kHz tone in young normally hearing subjects with a functioning auditory efferent system. MATERIAL AND METHODS: Sixteen young volunteers served as subjects. All the subjects were young (20-30 years), disease-free and had a medical history negative for otological disease, noise exposure and use of ototoxic drugs. They had normal hearing [thresholds of 125-8,000 Hz below 20 dB hearing level (HL)], transient-evoked otoacoustic emissions and contralateral suppression. The subjects were randomly assigned to one of three different groups. Subjects in Group A (n = 5) were exposed to a 90 dB HL 2 kHz pure tone for 10 min. Subjects in Group B (n = 6) were exposed to a 90 dB HL 2 kHz pure tone and an ipsilateral 45 dB HL 250 Hz NBN for 10 min. Subjects in Group C (n = 5) were exposed to a 90 dB HL 2 kHz pure tone and a contralateral 45 dB HL 250 Hz NBN for 10 min. The right ear served as the test ear. The TTS 2 min after the end of the exposure (TTS2) was measured in all subjects at 2, 3 and 4 kHz. RESULTS: TTS, in Group A was significantly higher at 3 kHz (p = 0.011) and at 4 kHz (p = 0.003) than TTS2 in Group B. At 4 kHz, TTS2 in Group C was significantly higher (p = 0.013) than TTS2 in Group B. Although TTS2 in Group C was lower than TTS2 in Group A, this difference was not significant. The presence of an ipsilateral low-frequency NBN significantly reduced TTS2 induced by a 90 dB HL 2 kHz tone. A contralateral low-frequency NBN reduced TTS2 in this group of subjects; however, the reduction was not significant. CONCLUSION: The results of this study show that an ipsilateral low-intensity, low-frequency (250 Hz) NBN can give protection from a TTS induced by a 2 kHz tone. Contralateral low-frequency NBN did not induce any protective effect.

Cochlear function in ears with immunomediated inner ear disorder.

The aim of the present study was to evaluate the performance of ears with inner ear disorder, responsive to immunosuppressive drugs, in advanced tests designed to assess primary cochlear functions (temporal integration, frequency selectivity, cochlear mechanics). The results of this study suggest that immunomediated inner ear disease results, in the acute clinical stage, in the development of endolymphatic hydrops, which increases the stiffness of the vibrating structures within the inner ear and causes dysfunctions of the outer hair cells. Our patients presented with upsloping or flat sensorineural hearing loss, absence of evoked otoacoustic emissions and distortion-product otoacoustic evoked emissions and abnormal temporal integration, frequency selectivity and cochlear mechanics. Following immunosuppressive treatment, hydrops recovered, hearing subsequently returned to normal, the audiometric curve became flat at low-to-middle frequencies and primary cochlear function tended to normalize. This study seems to support the usefulness of testing primary cochlear functions in order to monitor the clinical course of immunomediated inner ear disorders.

Cochlear implants in systemic autoimmune vasculitis syndromes.

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Beçhet's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.