Marco Mandalà

Infants versus older children fitted with cochlear implants: performance over 10 years.

OBJECTIVES To investigate the efficacy of cochlear implants (CIs) in infants versus children operated at later age in term of spoken language skills and cognitive performances. METHOD The present prospective cohort study focuses on 19 children fitted with CIs between 2 and 11 months (X=6.4 months; SD=2.8 months). The results were compared with two groups of children implanted at 12-23 and 24-35 months. Auditory abilities were evaluated up to 10 years of CI use with: Category of Auditory Performance (CAP); Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS); Peabody Picture Vocabulary Test (PPVT-R); Test of Reception of Grammar (TROG) and Speech Intelligibility Rating (SIR). Cognitive evaluation was performed using selected subclasses from the Griffiths Mental Development Scale (GMDS, 0-8 years of age) and Leiter International Performance Scale-Revised (LIPS-R, 8-13 years of age). RESULTS The infant group showed significantly better results at the CAP than the older children from 12 months to 36 months after surgery (p<.05). Infants PPVT-R outcomes did not differ significantly from normal hearing children, whereas the older age groups never reached the values of normal hearing peers even after 10 years of CI use. TROG outcomes showed that infants developed significantly better grammar skills at 5 and 10 years of follow up (p<.001). Scores for the more complex subtests of the GMDS and LIPS-R were significantly higher in youngest age group (p<.05). CONCLUSION This study demonstrates improved auditory, speech language and cognitive performances in children implanted below 12 months of age compared to children implanted later.

Estimated net saving to society from cochlear implantation in infants: A preliminary analysis†‡

Although it is clear that cochlear implants (CIs) are highly cost‐effective in adults and children, the possible additional economic benefit of implantation at younger ages has to be fully established to verify whether the costs and outcomes of CIs differ between infants and older children.

Long-term Outcome of Round Window Vibrant SoundBridge Implantation in Extensive Ossicular Chain Defects

Objective To evaluate retrospectively the long-term safety and efficacy of the first 50 patients, all suffering from severe ossicular chain defects and with moderate to severe mixed hearing loss, who received the Vibrant SoundBridge with the floating mass transducer located on the round window membrane. To evaluate differences in outcome versus etiology and age of the patient population. Study Design Case series with planned data collection. Setting Tertiary referral medical center. Subjects and Methods Patients eligible for implantation of the floating mass transducer on the round window membrane ranged in age from 2 months to 74 years with a moderate to severe conductive or mixed hearing loss from different etiologies. For each adult patient, preoperative versus postoperative bone and air conduction thresholds, air-bone gaps, and speech understanding scores were evaluated at 24-month follow-up. At 60-month follow-up, data were available from 33 patients. Preoperative and postoperative free-field auditory brainstem responses were studied in infants and children. Intraoperative and short- and long-term postoperative complications are presented. Results There were significant improvements in speech perception and pure-tone audiometry in adults and auditory brainstem response thresholds in infants immediately after surgery and at follow-up examinations (12 to 71 months). No significant complications or device extrusions were observed in the present series. Conclusions Infants, children, and adults with moderate to severe conductive or mixed hearing loss obtained substantial benefit from implantation of the floating mass transducer on the round window membrane regardless of the etiology of hearing loss and previous surgery.

Congenital aural atresia treated with floating mass transducer on the round window: 5 years of imaging experience

PurposeThe aim of this paper is to illustrate imaging features of patients affected by congenital aural atresia (CAA) before and after treatment with a Vibrant SoundBridge (VSB) device implanted on the round window.Materials and methodsTen patients (5 males and 5 females; mean age 22.1 years) with CAA underwent preoperative high-resolution computed tomography (HRCT) to estimate the degree of involvement of the middle- and inner-ear structures and highlight radiological landmarks useful for surgical planning.ResultsBilateral CAA, mostly of the mixed type, was present in 7 patients and ossicular chain abnormalities in 16 ears (94% of cases). The round window region was normal in all patients, whereas facial-nerve course and/or caliber abnormalities were present in 6 ears (35.3%). The tympanic cavity was small in 13 ears (76.5%), whereas the mastoid was well pneumatized in 8/17 (47%).ConclusionsHRCT provides accurate information about anatomy and malformations of the middle and inner ear and can thus assist the surgeon in planning the procedure.RiassuntoObiettivoLo scopo di questo studio è di descrivere le caratteristiche radiologiche pre-operatorie e postoperatorie dei pazienti affetti da atresia congenita aurale (ACA) impiantati mediante Vibrant SoundBridge (VSB) alla finestra rotonda.Materiali e metodiDieci pazienti (5 maschi e 5 femmine; età media: 22,1 anni) con ACA sono stati sottoposti ad un esame di tomografia computerizzata (TC) ad alta risoluzione per stimare il grado di coinvolgimento delle strutture dell’orecchio medio ed interno ed evidenziare utili punti di riferimento per l’intervento.RisultatiSono state riscontrate atresia bilaterale in 7 pazienti, prevalentemente di tipo misto, ed anomalie della catena ossiculare in 16 orecchi (94% dei casi). La finestra rotonda era nei limiti in tutti i soggetti, mentre in 6 orecchi (35,3%) erano presenti anomalie di decorso e/o di calibro del nervo faciale. La cassa timpanica era ipoplasica in 13 orecchi (76,5%), mentre la mastoide era ben pneumatizzata in 8/17 (47%).ConclusioniLa TC ad alta risoluzione fornisce le corrette informazioni circa l’anatomia e le malformazioni dell’orecchio medio ed interno, utili per il chirurgo per la programmazione operatoria.

Lateral Medullary Ischemia Presenting with Persistent Hiccups and Vertigo

ABSTRACT This study describes a patient with lateral medullary ischemia (LMI) presenting with persistent hiccups followed by vertigo with horizontal head-shaking-induced contralesional nystagmus (HSN) and discusses pertinent pathophysiology. A 65-year-old man presented with persistent hiccups and disabling spells of vertigo, lasting 30 seconds that became much more frequent and associated with lateropulsion to the right. A strong left beating HSN was evident. Magnetic resonance imaging and angiography, and intra-arterial cerebral digital subtracted angiography showed subacute ischemic lesions in the right lateral medulla and ipsilateral inferior cerebellar hemisphere, and two tight stenoses of the V1 and V4 segments of the right vertebral artery. Patient was treated by intravenous heparin and oral clopidogrel. After 48 hours, hiccups disappeared. One month later, vertigo spells were less frequent but still disabling. Endovascular stenting of the right vertebral artery stenoses was then performed. In the subsequent four years, the patient had no further episodes of hiccups or vertigo. Less intense HSN persisted. Hiccups followed by vertigo, lateropulsion, and HSN had been the clinical presentation of LMI and cerebellar ischemia, without other major neurologic or ocular motor findings. This unusual clinical variant of LMI could mimic a more benign labyrinthine lesion, and possibly leading to a dangerously delayed treatment.

Intraoperative observation of changes in cochlear nerve action potentials during exposure to electromagnetic fields generated by mobile phones

Background The rapid spread of devices generating electromagnetic fields (EMF) has raised concerns as to the possible effects of this technology on humans. The auditory system is the neural organ most frequently and directly exposed to electromagnetic activity owing to the daily use of mobile phones. In recent publications, a possible correlation between mobile phone usage and central nervous system tumours has been detected. Very recently a deterioration in otoacoustic emissions and in the auditory middle latency responses after intensive and long-term magnetic field exposure in humans has been demonstrated. Methods To determine with objective observations if exposure to mobile phone EMF affects acoustically evoked cochlear nerve compound action potentials, seven patients suffering from Ménières disease and undergoing retrosigmoid vestibular neurectomy were exposed to the effects of mobile phone placed over the craniotomy for 5 min. Results All patients showed a substantial decrease in amplitude and a significant increase in latency of cochlear nerve compound action potentials during the 5 min of exposure to EMF. These changes lasted for a period of around 5 min after exposure. Discussion The possibility that EMF can produce relatively long-lasting effects on cochlear nerve conduction is discussed and analysed in light of contrasting previous literature obtained under non-surgical conditions. Limitations of this novel approach, including the effects of the anaesthetics, craniotomy and surgical procedure, are presented in detail.

Effect of bluetooth headset and mobile phone electromagnetic fields on the human auditory nerve

The possibility that long‐term mobile phone use increases the incidence of astrocytoma, glioma and acoustic neuroma has been investigated in several studies. Recently, our group showed that direct exposure (in a surgical setting) to cell phone electromagnetic fields (EMFs) induces deterioration of auditory evoked cochlear nerve compound action potential (CNAP) in humans. To verify whether the use of Bluetooth devices reduces these effects, we conducted the present study with the same experimental protocol.

Pneumolabyrinth and positional vertigo after stapedectomy

Pneumolabyrinth due to late complications of stapes surgery is a rare entity. Symptoms may include various degrees of hearing loss, tinnitus and dizziness. We report the case of a 67-year-old patient who developed a unique pneumolabyrinth variant affecting the vestibule, cochlea and semicircular canals 28 years after stapedectomy. The patient presented with intractable paroxysmal positional vertigo of the lateral semicircular canal. The pneumolabyrinth was visualized by means of high resolution computed tomography. Exploratory tympanotomy findings were consistent with a perilymphatic fistula. One year after surgical treatment the subject was free of vertigo spells. Pathogenetic mechanisms are discussed.

Induced endolymphatic flow from the endolymphatic sac to the cochlea in Ménière's disease

OBJECTIVE The aim of the present study was to verify whether drugs injected into the endolymphatic sac (ES) can reach the cochlea and possibly treat inner ear disorders. STUDY DESIGN Prospective cohort study. SETTING Tertiary referral center, Otolaryngology Department, University of Verona. SUBJECTS AND METHODS Patients with Ménières disease (MD) who were candidates for ES decompression were selected. Nineteen subjects received dexamethasone (DEX) via injection into the ES. To objectively define whether substances administered into the ES could reach the cochlea, we added gadolinium (GD) in three patients. All subjects had intraoperative electrocorticogram recordings and an audiologic follow-up. The three subjects who underwent injection of the DEX-GD solution were followed-up with magnetic resonance imaging. The audio-logical data are presented during a follow-up period of 12 months. RESULTS Intraoperative electrocochleography recordings revealed no changes in two patients and summating potentials and compound action potential latency and wave-form modifications in all the other subjects. GD distribution was observed from 48 hours to one week after ES injection into the cochlea of the three subjects injected with DEX-GD. GD-related enhancement of inner ear structures lasted more than two weeks in all subjects. Pure tone average results showed hearing improvement of at least 20 dB HL in 42 percent of patients (8 of 19) at the 12-month follow-up. Statistically significant differences emerged between the mean pure tone average of the ES procedure subjects at one and 12 months after surgery (P = 0.0096). CONCLUSION This novel approach might reveal new prospects for treating viral, metabolic, autoimmune, and genetic disorders of the cochlea.

Vestibulotomy with ossiculoplasty versus round window vibroplasty procedure in children with oval window aplasia.

Objective To review the surgical procedures and outcomes in children with bilateral oval window aplasia (OWA). Study Design Retrospective cohort review. Setting Tertiary referral center. Patients Children suffering from OWA between 1990 and 2010. Intervention Vestibulotomy with ossiculoplasty (V-OPL) or round window vibroplasty (RWV). Main Outcome Measures Findings at radiology and surgery, preoperative and postoperative bone conduction (BC), air conduction (AC), and RWV-air conduction (RWV-AC) thresholds and speech discrimination scores (SDSs). Results Among 23 children, 11 underwent V-OPL and 8 RWV. Four children in the V-OPL group had aborted surgery and were excluded from the study. In all the remaining 19 children, the 6-month follow-up time showed postoperative AC and SDS values significantly better than the preoperative thresholds in both groups. At the 36-month long-term follow-up, AC and SDS were stable in the RWV group but showed a significant worsening in the V-OPL children compared with the 6-month follow-up results. Preoperative versus postoperative BC values showed a significant difference between the 2 groups at 36 months; 5 of the V-OPL group underwent revision following the same surgical principles, which did not result in improved outcome. Conclusion In children with OWA, V-OPL provides modest long-term results and carries higher risks of BC degradation compared to RWV. Both procedures are technically challenging but considering the respective hearing results and morbidity of primary and revision surgery, we have abandoned the V-OPL procedure in favor of RWV. In infants and children younger than 5 years with OWA previously not considered candidates for hearing restoration, we consider RWV as the first-choice surgery. It has shown to provide significantly better hearing outcomes than traditional atresia surgery with minimal complication rate.