Nicola Quaranta

Tinnitus and cochlear implantation

Tinnitus is a common experience, but there is very marked heterogeneity of aetiology, perception and the extent of distress among individuals who experience tinnitus. In view of this, a modern approach to tinnitus should consider homogeneous groups of individuals. This review considers tinnitus experiences in patients undergoing cochlear implantation, this being of interest because the prevalence of tinnitus in this patient group prior to surgery may shed some light on the link between cochlear dysfunction and tinnitus. Second, any change in tinnitus experience as a result of electrode placement surgery or cochlear implant activation has relevance for patient counselling and informed consent. Finally, in recent tinnitus retraining therapy literature there has been the suggestion that unilateral sound therapy for tinnitus patients may set up an unhelpful asymmetry of input to the auditory system, with possible exacerbation of contralateral tinnitus. Unilateral cochlear implant use represents the most dramatic asymmetry possible and hence is a test of that hypothesis. Relevant papers (n = 32) were identified from literature databases. The standard of reporting tinnitus results was inconsistent. Tinnitus is experienced by up to 86% of adult cochlear implant candidates, but is not universal and is only troublesome in a small proportion (reported as 27% in one study). Electrode insertion may induce tinnitus in a small (up to 4%) number of patients, but this is rare. Cochlear implant device use is associated with reduction of tinnitus intensity and awareness in up to 86% of patients, and rarely with exacerbation (up to 9%). There are some indications in the literature that the more complex the simulation strategy, the larger that effect. Specifically, unilateral cochlear implant use was generally associated with reduction of contralateral tinnitus (in up to 67% of individuals) rather than exacerbation, and so the assertion that unilateral sound therapy for tinnitus is contraindicated is not proven. Sumario El acúfeno es una experiencia común, pero existe una marcada heterogeneidad en cuanto a la etiología, la percepción y el grado de incomodidad experimentado por los individuos que lo padecen. Ante esto, un enfoque moderno para el estudio del acúfeno debe involucrar grupos homogéneos de individuos. Esta revisión considera las experiencias con relación al acúfeno en pacientes sometidos a implantación coclear; hecho que resulta de interés pues la prevalencia del acúfeno en este grupo de pacientes antes de la cirugía puede aportar información sobre el vínculo entre la disfunción coclear y el acúfeno. En segundo lugar, todo cambio sobre la percepción del acúfeno como resultado de la colocación quirúrgica de un electrodo o la activación de un implante coclear tiene relevancia en la asesoría al paciente y para el consentimiento informado. Finalmente, en la literatura reciente sobre terapia de re-entrenamiento en acúfeno, se ha sugerido que la terapia sonora unilateral en estos pacientes puede crear una asimetría en la estimulación de ingreso al sistema auditivo, con una posible exacerbación del acúfeno contra-lateral. El uso de un implante coclear unilateral representa la más dramática asimetría posible, y por lo tanto, se constituye en una prueba para dicha hipótesis. Se identificaron reportes relevantes (n = 32) a partir de bases de datos en la literatura. La norma para reportar el acúfeno fue inconsistente. El acúfeno es experimentado por un 86% de los candidatos adultos para implante coclear, pero este hecho no es universal y resulta molesto solamente para una pequeña proporción de ellos (un estudio reporta un 27%). La inserción del electrodo puede producir un acúfeno en un pequeño número de pacientes (hasta un 4%), pero es raro. El uso del implante coclear se asocia con una reducción en la intensidad y la conciencia del acúfeno en hasta un 86% de los pacientes, y raramente con una exacerbación (hasta 9%). Existen algunas indicaciones en la literatura que aluden a que cuánto más compleja es la estrategia de estimulación mayor es ese efecto. Específicamente, el uso unilateral de un implante coclear se asoció con una reducción en el acúfeno contra-lateral (hasta en un 6% de los individuos) más que con una exacerbación, y por lo tanto, no se demuestra la afirmación de que la terapia sonora unilateral para el acúfeno está contraindicada.

Facial Nerve Paralysis in Temporal Bone Fractures: Outcomes after Late Decompression Surgery

The aim of this paper was to address some of the unanswered questions regarding management of facial nerve paralysis in temporal bone fractures (TBF), such as the outcomes after late facial nerve decompression surgery. The study design was a retrospective review of a consecutive clinical series. Thirteen patients who underwent late decompression surgery for facial nerve paralysis due to TBF involving the perigeniculate ganglion region were analyzed. Patients were operated on 27-90 days after trauma. A transmastoid extralabyrinthine approach was used in all cases. Facial nerve-sheath slitting was performed routinely. Normal or subnormal facial nerve function (HB 1 or HB 2) was achieved in 7/9 cases (78%) evaluated at > or = 1 year after surgery. Good functional results were also obtained in two patients operated on 3 months after trauma. Bases on the outcomes observed in the present series, in patients unable to be operated on early, presenting 1 to 3 months with >95% denervation on EnoG, facial nerve decompression may have a beneficial effect.

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).

Age-related decline of auditory function in the chinchilla (Chinchilla laniger)

The aim of this study was to examine the functional consequences of aging in the chinchilla, a rodent with a relatively long life span and a range of hearing similar to that of humans. Subjects were 21 chinchillas aged 10-15 years, and 23 young controls. Thresholds were determined from auditory evoked potentials (EVPs), and outer hair cell (OHC) functioning was assessed by measuring 2f1-f2 distortion product otoacoustic emissions (DPOAEs). Six cochleas from 11-12-year-old animals were examined for hair cell loss and gross strial pathology. The results show that the chinchilla exhibits a small but significant decline of auditory sensitivity and OHC functioning between 3 and 15 years of age, with high-frequency losses exceeding and growing more rapidly than low-frequency losses. Compared to rodents with shorter life spans, the chinchilla has a rate of loss that is more similar to that of humans, which could make it a valuable model for understanding the etiology of human presbycusis.

Endothelial function and cardiovascular risk in patients with Idiopathic Sudden Sensorineural Hearing Loss

OBJECTIVE To evaluate cardiovascular risk factors and pre-clinical atherosclerosis in subjects affected by idiopathic sudden sensorineural hearing loss (ISSHL). METHODS In this study, 29 ISSHL patients and 29 healthy controls were evaluated. All of the patients underwent a complete audiovestibular and clinical evaluation. Carotid intima-media thickness (C-IMT) and flow-mediated dilation (FMD) of the brachial artery were assessed as early markers of atherosclerosis. RESULTS Our results showed that FMD was significantly lower in the ISSHL patients than in the controls (5.6 ± 1.6% vs. 7.7 ± 3.7%, p < 0.01). Moreover, the total cholesterol and low density lipoprotein cholesterol were significantly higher in the ISSHL patients than in the controls (p < 0.05). The two groups did not differ with regards to C-IMT and other cardiovascular risk factors. Vestibular involvement was shown to be associated with lower FMD values (4.1 ± 1.7% vs. 5.8 ± 1.5%, p < 0.05). No relationship was found between C-IMT and vestibular involvement. Finally, multiple logistic regression highlighted the finding that only FMD values seemed to predispose individuals to developing ISSHL (p = 0.03, OR: 1.4). CONCLUSIONS ISSHL seemed to be associated with vascular endothelial dysfunction and an increased cardiovascular risk, which supports the hypothesis of a vascular aetiology for this disease.

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.

Intratympanic therapy for Ménière's disease: effect of administration of low concentration of gentamicin.

We present results at 2 years follow-up of a group of 15 patients with Ménières disease treated with a low concentration of intratympanic gentamicin (IG group), compared with a group of 15 patients who refused any surgical treatment (NH group). IG was administered according to a predetermined and fixed schedule consisting of 2 doses of 0.5 ml gentamicin solution, pH 7.8, injected once a week, with a drug concentration of 20 mg/ml; the total dose of gentamicin was < or = 20 mg. Additional doses of IG were administered only in patients who had recurrence of vertigo. The results were evaluated following the American Academy of Otolaryngology Head and Neck Surgery 1995 criteria for reporting Ménières disease treatment results by means of interviews, audiologic and vestibular evaluations, and a questionnaire based on a six-point functional level scale. Seven of the 15 IG patients had recurrence of vertigo after the second injection and received a third dose of IG. Four patients had recurrence of vertigo after the third infiltration; three subjects received a fourth dose and one refused additional injection. At 2 years follow-up, 93% of the IG patients had complete (class A) or substantial (class B) control of vertigo. Only 47% of the NH patients had no vertigo or were substantially improved. Hearing deteriorated in 7% of the IG group and in 40% of the NH group. Tinnitus disappeared or improved in 20% of the IG patients and in 27% of the NH patients; 40% of the IG patients and 27% of the NH patients reported that their aural pressure was abolished. The present study demonstrates that, in patients with Ménières disease, 0.5 ml doses of gentamicin solution, with a concentration of 20 mg/ml, injected intratympanically once a week minimize the risk of hearing loss in the treated ear, permitting complete control of vertigo in more than half of cases after 2 doses and in almost all subjects (93%) after 4 doses.We present results at 2 years follow-up of a group of 15 patients with Méniè

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.

Closed tympanoplasty in cholesteatoma surgery: long-term (10 years) hearing results using cartilage ossiculoplasty

Abstract The aim of this retrospective study was to evaluate the long-term hearing results of using costal cartilage prostheses in ossicular chain reconstruction procedures in subjects operated on for a middle ear cholesteatoma with an intact canal wall tympanoplasty. Thirty-six patients (four with bilateral disease) followed up for 10 years who underwent an ossiculoplasty with a cartilage prostheses between January 1987 and December 1989 constituted the population studied. All the subjects underwent a staged intact canal wall tympanoplasty with mastoidectomy. Ossiculoplasty with total or partial chondroprosthesis was performed during the second stage. The long-term outcome was evaluated in terms of hearing according to the guidelines of the Committee on Hearing and Equilibrium (1995), and in terms of complications (anatomical and functional). In 18 patients a partial cartilage ossicular replacement prosthesis (PORP) was used, while in 22 a total cartilage ossicular replacement prosthesis (TORP) was used. In the PORP group the mean preoperative air–bone gap (ABG) was 22.4 dB hearing level (HL); before the second stage the ABG was 37.9 dB HL, at 2 years it was 12.1 dB HL, at 5 years 15.3 dB HL and at 10 years 15.8 dB HL. In the TORP group the mean preoperative ABG was 31.6 dB HL; before the second stage the ABG was 41.1 dB HL, at 2 years it was 14.4 dB HL, at 5 years 17 dB HL and at 10 years 18.5 dB HL. In both groups the number of cases with a postoperative ABG of < 20 dB HL remained stable (P > 0.05) over time. The failure rate was 17.5%, but only in 5% of cases was a functional revision needed. No cases of extrusion of the prostheses were encountered. The use of a chondroprosthesis is associated with functional results similar to those obtained by other authors. The efficacy of the prostheses remains stable over time and is associated with a very low rate of complications and failures. In this series no extrusion occurred and in no case did an infectious disease develop after cartilage transplantation.

Hereditary hemorrhagic telangiectasia: arteriovenous malformations in children.

OBJECTIVE To evaluate the clinical features in a large cohort of pediatric patients with genetically confirmed hereditary hemorrhagic telangiectasia (HHT) and to identify possible predictors of arteriovenous malformation (AVM) onset or clinical significance. STUDY DESIGN Prospective cross-sectional survey of all children subjected to screening for AVMs in the multidisciplinary HHT center. All patients proved to be carriers of endoglin mutations or activin A receptor type-II-like kinase 1 mutations, defined as HHT1 and HHT2, respectively. A full clinical-radiological protocol for AVM detection was adopted, independent from presence or absence of AVM-related symptoms. RESULTS Forty-four children (mean age, 10.3 years; range, 1-18) were subjected to a comprehensive clinical-radiologic evaluation. This investigation disclosed cerebrovascular malformations in 7 of 44 cases, pulmonary AVMs in 20 of 44 cases, and liver AVMs in 23 of 44 cases. Large visceral AVMs were found in 12 of 44 children and were significantly more frequent in patients with HHT1. Only large AVMs were associated with symptoms and complications. CONCLUSIONS Children with HHT have a high prevalence of AVMs; therefore, an appropriate clinical and radiological screening protocol is advisable. Large AVMs can be associated with complications in childhood, whereas small AVMs probably have no clinical risk.