ACTA OTORHINOLARYNGOLOGICA ITALICA Epub 2017 May 22; doi: 10.14639/0392-100X-1426 Pediatric Otorhinolaryngology New trends in rehabilitation of children with ENT disorders Aggiornamenti sulla riabilitazione ORL in età pediatrica R. BOVO, P. TREVISI, E. ZANOLETTI, D. CAZZADOR, T. VOLO, E. EMANUELLI, A. MARTINI Department of Neuroscience, Institute of Otorhinolaryngology, University Hospital of Padua, Padua, Italy SUMMARY In the last 20 years, neonatal survival has progressively increased due to the constant amelioration of neonatal medical treatment and surgi- cal techniques. Thus, the number of children with congenital malformations and severe chronic pathologies who need rehabilitative care has progressively increased. Rehabilitation programs for paediatric patients with disorders of voice, speech and language, communication and hearing, deglutition and breathing are not widely available in hospital settings or in long-term care facilities. In most countries, the number of physicians and technicians is still inadequate; moreover, multidisciplinary teams dedicated to paediatric patients are quite rare. The aim of the present study is to present some new trends in ENT paediatric rehabilitation. KEY WORDS: Children • Rehabilitation • Sinusitis • Laryngeal paralysis • Choanal atresia • Aural atresia • Music • Voice • Cochlear implant RIASSUNTO Negli ultimi 20 anni il miglioramento dell’assistenza neonatale ha determinato un progressivo aumento dei bambini che sopravvivono in presenza di gravi malformazioni o patologie congenite. Questi bambini richiedono una riabilitazione prolungata, talora multidisciplinare e complessa. Purtroppo, un’organizzazione adeguata alla riabilitazione della disfagia, dei disturbi della comunicazione e della respirazione non è sempre disponibile, non è sempre coordinata in equipe multidisciplinari che operino sia negli ospedali che sul territorio e non è facile mantenere tutte le figure professionali coinvolte al passo con le sempre più rapide innovazioni. Scopo del presente lavoro è presentare un aggiornamento su alcuni aspetti tuttora controversi della riabilitazione in età pediatrica. PAROLE CHIAVE: Bambini • Riabilitazione • Sinusite • Paralisi laringea • Atresia coanale • Atresia auris • Musica • Voce • Impianto cocleare Introduction Chronic rhinosinusitis In the last 20 years, neonatal survival has progressively Rhinosinusitis is a very common condition and its preva- increased due to the constant amelioration of neonatal lence has increased in recent years in both children and medical treatment and surgical techniques. These aspects the adult population. By definition in chronic rhinosinusi- have produced an increased incidence of children with tis (CRS), symptoms last more than 12 weeks. Antibiot- congenital malformations and severe chronic pathologies. ics are the most frequently used therapeutic agents in acute Moreover, the immigration of children from underdevel- rhinosinusitis (ARS). Uncomplicated ARS, if no allergies oped countries, where perinatal infections or other risk exist, can be treated with amoxicillin (40 mg/ kg/day or factors are still high, have further increased the number of 80 mg/kg/day). Other reasonable choices are amoxicillin/ paediatric patients who need rehabilitative care. It is worth clavulanate and cephalosporins. If hypersensitivity to any noting that among over 50,000 scientific papers in the of the above antimicrobials is suspected, alternative choices literature regarding paediatric rehabilitation, only about include trimethoprim/sulfamethoxasole, azithromycin, or 1000 are related to ENT arguments. Thus, rehabilitation clarithromycin 1. Surgical intervention in the treatment of of paediatric patients with disorders of voice, speech and nasal polyps is considered in patients who fail to improve language, communication and hearing, deglutition and after a trial of maximal medical treatment or in patients pre- breathing is generally still inadequate to the real needs sented at diagnosis with antrochoanal polyp, cystic fibrosis and rarely is well organised with multidisciplinary teams, (CF), Kartagener and Churg-Strauss syndromes. Function- working both in the hospital setting and in long-term care al endoscopic sinus surgery (FESS) involves the clearance facilities. The aim of the present study is to present some of polyps and polypoid mucosa and opening of the sinuses new trends in ENT paediatric rehabilitation. ostia. If maximum medical therapy is unsuccessful in a 1 R. Bovo et al. child with chronic or recurrent sinusitis, evaluation for un- of cases it occurs bilaterally, thus constituting the main derlying medical disorders (e.g. immunodeficiency, aller- indication for sinonasal surgery in the newborn 6. gic rhinitis, CF and immotile cilia syndrome) is warranted. The association of choanal atresia with craniofacial and Surgery consisted of adenoidectomy with or without antral genetic syndromes is well known. In particular, between irrigation and balloon sinus dilation, and FESS 2. 7% and 29% of patients with choanal atresia are affected Follow-up begins after surgery and can last for years; it is by CHARGE syndrome. In these conditions, the atresia performed with nasal endoscopy and inspection of breath- presents often bilaterally (Fig. 1) 5. ing spaces, eventual nasal secretions, healing of nasal muco- Bilateral choanal atresia presents with respiratory distress in sa, natural ostium and sinusotomy patency. In the first visit the immediate neonatal period. The crises are typically alle- after FESS, there is removal of nasal packing; it is normally viated when the child begins to cry. Surgical intervention is performed one or two days after surgery. Further visits are considered the main therapeutic option. Over the decades, made at 15 days and one month after surgery: the goal is in- various surgical techniques have been described 7-9. spection of nasal cavities and removal of nasal crusting and An “ideal” surgical technique should ensure adequate fibrinous exudate to prevent nasal adhesions. If no com- choanal patency and low restenosis rates, should spare the plications exist, clinical follow-up visits are scheduled 3-6 surrounding anatomical structures and provide low rates and 12 months after surgery and are made to prevent late of morbidity and mortality. It should also ensure the short- complications and relapse. Recurrence rates are higher in est time hospitalisation 8. nasal polyposis stage IV and V (association with CF, Karta- Nowadays there is still a lack of randomised controlled gener and Churg-Strauss syndrome). In these cases, there studies comparing the efficacy of the different surgical ap- is no definitive treatment for nasal polyposis and long fol- proaches, but it is common expert opinion to consider an low-up is necessary. In the youngest patients, endoscopic endonasal endoscopic approach to be the least invasive medications must be scheduled in sedation or under general and probably the safest in terms of compliance 9. anaesthesia: parents should be previously informed about However, restenosis remains the most frequent complica- this requirement and the fact that these are not new surgical tion in choanal atresia surgery, with a prevalence between interventions, but are necessary to improve the first result. 9% and 36%. Adjuvant measures have been introduced All children with CRS should be submitted to allergy tests. to reduce the restenosis rate: nasal stenting, mitomycin Tests for immunodeficiency should be done in children C and balloon dilatation. The use of nasal stenting is still with chronic recurrent disease, poor response to medical debated 10. treatment, a history of other chronic infections (such as re- A stent is generally applied to stabilise postoperative out- current pneumonia or otitis) or when unusual microorgan- comes, but this procedure entails some worrisome com- isms are isolated in the nasal secretion. To obtain the best plications. Nasal and nasopharyngeal infections, mucosal results, surgery in children should be performed by expert ulcerations, tissue ischaemia and necrosis and formation surgeon after a skills trial in phlogistic, malformative and of granulation tissue are only some of the possible conse- neoplastic diseases in adults and using surgical instruments quences of long-term use of a nasal stent 10 11. dedicated to the paediatric population. Recently, some authors performed stentless choanal The health impact of chronic recurrent rhinosinusitis of endoscopic surgery repair. To date, a total of 42 proce- paediatric patients and their parents is severe. Children dures have been carried out, with a restenosis rate of only with rhinosinusitis are perceived by their parents to have 38% 11-13. Nasal irrigation in the postoperative period is significantly more bodily pain and to be more limited in crucial for maintaining neo-choanal patency, in associa- physical activities than children with asthma, juvenille tion to endoscopic medications according to a precise fol- rheumatoid arthritis and other chronic disorders 3. The low-up schema. In our centre, nasal packing is removed SN-5 is a validated symptom score questionnaire for the 24 hours after the surgical procedure, the first postopera- evaluation of CRS in children 4. The self-administered survey is completed by the child’s parent using 7-point ordinal response scales for each item. Domains include si- nus infection, nasal obstruction, allergy symptoms, medi- cation use, emotional distress and activity limitations. Choanal atresia rehabilitation in paediatric patients Choanal atresia is defined as a congenital obstruction of the posterior nasal choanae. It represents one of the most frequently observed congenital nasal defects, with a prevalence of 1:5,000-8,000 live births 5. In about 50% Fig. 1. Endoscopic view of a bilateral choanal atresia. 2 Rehabilitation of children with ENT disorders tive visit is generally performed after 7 days, under seda- A flexible fiberscope is routinely used in the diagnostic tion. Further visits take place after 15, 45 and 90 days, and workup of LP. It is performed without anesthesia, thus al- then at 6 and 12 months. lowing direct visualisation of laryngeal motility. Possible Anti-reflux agents are helpful in avoiding granulation tis- underlying associated malformation, which is present in sue formation, since gastro-oesophageal reflux is a negative about 45% of LP cases, is investigated with a rigid lar- predictive factor for restenosis, with incorrect postoperative yngo-tracheo-bronchoscope under general anaesthesia. management and an early age at surgery (> 10 years) 14. Only after both central and peripheral causes of vocal fold paralysis have been excluded, the condition can be defined Obstructive sleep apnoea as idiopathic. In severe cases of LP, when the risk of liquid inhalation occurs, it is necessary to place a nasal-feeding Sleep disorders include different conditions ranging from tube. In addition, tracheostomy is required in 8% and 53% primary snoring to obstructive sleep apnoea (OSA). OSA of cases of unilateral and bilateral LP, respectively 22. is characterised by both partial and complete airway ob- Above all, idiopathic LP may partially spontaneously re- struction, which induce hypoxaemia with a relevant de- solve within the 6th-12th month of life. When there is no crease in quality of the sleep and day life. evidence of improvement in laryngeal motility after the OSA in children has a prevalence between 1.1% and 2.9% age of 2 years, the lesion could be defined persistent. At 15. The diagnostic gold standard in OSA syndrome is over- this point, surgical intervention is required to create ad- night polysomnography, but home pulse oximetry has also equate respiratory space. A posterior cordectomy is gen- been proposed as a screening test 16. Adenotonsillar hyper- erally performed 23. trophy is the most important aetiologic factor for OSA in Logopedic rehabilitation (LR) is indicated in unilateral paediatric patients: nearly 45% of OSA children are af- LP with the aim of inducing a spontaneous compensation fected by lymphatic Waldeyer’s ring hypertrophy 17. Ade- of vocal fold motility. The earlier LR is begun, the better no-tonsillectomy is therefore indicated as a first therapeutic the functional results that might be obtained. Moreover, option in OSA children without other important comorbidi- LR finds application in children who underwent tracheal ties and has shown good efficacy in improving the patients’ tube or nasal-feeding tube placement. Long lasting tra- quality of life 18. However, the degree of adenotonsillar hy- cheal intubation (> 48 h), as well as nasal-feeding tube ap- pertrophy is not strictly related to the severity of respiratory plication, can provoke vocal and swallowing dysfunctions sleep disorder. Predictors for a persistent elevated postop- when removed. Tracheostomy, moreover, affects larynge- erative apnoea-hypopnoea index (AHI) are: age > 7 years, al motility in indirect ways, determining deafferentation BMI increase, preoperative AHI > 20 and asthma. of the proprioceptive innervation in the transglottic area As reported by Albera et al. in a large cohort of paediatric pa- and progressive muscular atrophy 24. A LR program is de- tients, more OSA children presented with impaired swallow- fined on the basis of the patients’ symptoms. Plurisenso- ing function and impaired speech articulation in comparison rial stimulation touches all the structures involved in res- to non-OSA patients. Interestingly, adeno-tonsillectomy im- piratory, vocal and deglutition functions. proved obstructive respiratory function in the first group of patients, but did not modify secondary dysfunctions such as Juvenille laryngeal papillomatosis atypical swallowing, oral breathing, or dyslalia 19. A rehabilitative program is necessary in addition to surgi- Juvenille laryngeal recurrent papillomatosis (JRRP) is a cal or medical therapy for OSA. No standardised proto- rare viral disease caused by HPV-6 and HPV-11 infection. cols are reported to date 20. The speech therapy centre in Both single and multiple lesions of JRRP affect especially Rome has proposed a valid rehabilitation scheme, which the glottic region but, in 5-28 % of cases, may also ex- consists of neuromuscular and myofunctional therapy for tend caudally into the tracheobronchial three and lungs 25. oro-facial musculature 21. The papillomatosis glottic involvement entails important functional and vocal consequences, with significant im- Laryngeal paralysis pact on the emotional and behavioural sphere of children, Laryngeal paralysis (LP) is the second most common la- and consequently their quality of life. ryngeal congenital malformation after laryngomalacia. Although in JRRP the goal of treatment is obtaining ad- Of all laryngeal malformations, its prevalence is about equate respiratory airway patency to avoid tracheostomy, 15-20%. Unilateral LP (48%) may cause swallowing it is also of utmost importance to define suitable surgical impairments and chronic food inhalation; depending on treatment protocols to reduce the incidence of iatrogenic the position of fixation of the vocal cords, bilateral LP vocal damage. (52%) may present with respiratory symptoms varying The intensity of voice function alteration correlates with from inspiratory stridor to acute respiratory distress with the number of surgical procedures per person, and not cyanosis. The latter condition requires emergent tracheal with age at diagnosis 26. Consequently, voice function intubation 22. preservation surgery should preserve as much healthy la- 3 R. Bovo et al. ryngeal mucosal tissue as possible, without reaching radi- goscopy under general anaesthesia (without muscle relax- cal excision. ant). In order to complete the staging, pulmonary function The gold standard treatment is a mini-invasive approach with tests (PFTs) and polysomnography (PSG) are mandatory 31. CO laser or microdebrider surgical excision of the lesions, Spontaneous resolution of symptoms is the rule; it usually 2 associated or not to adjuvant virostatics 27. Only one prospec- occurs by the age of 2-5 years, so the main treatment con- tive cohort study in 11 patients compared vocal function out- sists in antireflux drugs and follow-up. Surgical interven- comes of the two surgical techniques. Main vocal outcomes tion is indicated only in severe cases (10-12% of all cases) included overall severity rating, jitter, shimmer and noise-to- of laryngomalacia and consists in endoscopic approaches, harmonic ratio. “Cold” dissection with a microdebrider re- namely supraglottoplasty. The aim of surgery is to correct sulted in better immediate and early postoperative voice out- the anatomical cause with minimal tissue damage. A cold comes in children. Additionally, increased exposure to CO steel technique is recommended. Other instruments have 2 laser correlated with worsening voice quality. This can be been used including laser and microdebriders. explained by the potential thermal injuries of the surround- In recent years, paediatric breathing physiotherapy uses ing and deeper tissues induced by the laser 28. various techniques to help removal of mucus from the air- Long-term soft tissue complications including scarring, ways and improvement of pulmonary function. These are: stenosis and web formation can also lead to voice disor- cough, compressions/vibrations, forced expiratory tech- ders. The total number of repeated microsurgical interven- nique (FET), autogenic drainage (AD), prolonged slow ex- tions performed per patient and the lesion site influences piration (PSE), positive expiratory pressure (PEP), positive the rate of these complications. The most frequent laser- continuous periodic pressure (PCPAP), continuous positive induced soft tissue complication is anterior glottic web 25. airway pressure (CPAP), physical exercise, aerosol and na- The rate of soft tissue complications is influenced not only sal unblocking. by the total number of repeated surgical interventions per Evaluation of patient posture is another important issue: patient, lesion site, surgical technique, age at diagnosis chest conformation, postural alignment (chin, shoulder, and advanced stage of disease, but it also correlates with thorax position) and spine alignment on frontal and sagit- the presence of gastro-oesophageal reflux 29. tal plane. The main goal of breathing treatment is remov- The main functional aim in JRRP surgery is therefore al of airway secretions, where physiological clearance avoiding soft tissue deep laryngeal damage in order to mechanisms (cough, cilia, ventilation) are ineffective. prevent voice function injuries in children. A “cold” dis- section of the glottic lesions sparing the lamina propria, Paediatric tracheostomy: a changing trend healthy surrounding tissue and glottal deeper laryngeal structures seems to be the right direction. The use of a In the last 30 years, the role and the indications for tracheos- microdebrider is reaching increasing consent in this field. tomy have remarkably changed. The most common indica- However, in literature there are no prospective randomised tions for paediatric tracheostomy have passed from infective studies to support the superiority of the microdebrider sur- causes (epiglottitis, croup, diphtheria) to airway obstruction gical technique. In the future, the use of tetravalent HPV and anomalies 32, long-term ventilation requirement and un- vaccine might represent a promising option. derlying neuromuscular or respiratory problems 33. Long-term intubation is now the first indication for tra- cheostomy; in the absence of guidelines, the majority of Breathing rehabilitation in patients with authors agree to perform a tracheostomy after 2-3 weeks laryngo-tracheal axis stenosis of intubation to avoid hypoglottic or tracheal stenosis 34. The most frequent diseases causing laryngo-tracheal ste- A literature review shows that 50% of patients with tra- nosis in the paediatric population and requiring breathing cheostomy is less than one year old. This is related to rehabilitation are: laryngomalacia, vocal cord paralysis the improvement of the Paediatric Intensive Care Unit (monolateral or bilateral), hypoglottic stenosis, tracheal (PICU) that allows more premature babies survive, but at stenosis and tracheomalacia. the same time it entails greater risk of complications for Laryngomalacia is a congenital abnormality of the laryn- the smaller diameter and the lower stiffness of the trachea. geal cartilage. It is a dynamic lesion resulting in collapse More generally, there has been in recent years an increase of the supraglottic structures during inspiration, leading of late complications of trachestomy, related to the pro- to airway obstruction. It is thought to represent a delay of longed presence of the tracheostomy tube. maturation of the supporting structures of the larynx 30. These late complications are peristomal granulomas, If present, reflux can worse breath symptoms 31. Laryn- tracheal stenosis, trachea-innominate fistula, trachea-oe- gomalacia is the most common cause of congenital stridor sophageal fistula and trachea-cutaneous fistula. and is the most common congenital lesion of the larynx. The surgical procedure is more complicated in the paediat- Diagnosis is made by flexible naso-pharyngo-laryngoscope ric population. Open surgical tracheostomy and percutane- under topical or no anaesthesia, followed by direct laryn- ous dilatational tracheostomy, described by Ciaglia in 1985 4 Rehabilitation of children with ENT disorders 35, are two possible approaches in the adult population. posterior cordotomy) and open surgery (laryngotracheo- There are different types of tracheostomy tubes that vary in plasty for airway dilatation, laryngotracheal reconstruc- certain features for different purposes; they can be made in tions, cricotracheal resection with removal of stenotic plastic, silicon, PVC and metal. For the first application, it tract and termino-terminal anastomosis). is generally suggested to use a plastic tube. The length and The difficulties in feeding and swallowing after laryngo-tra- angle of the tube should be such as to maintain its end por- cheal reconstruction are multifactorial and to be able to make tion in axis with the trachea. The American Thoracic Society a rehabilitative project is mandatory understand the reason suggests selecting a tube of such a length as to extend to 2 cm of these difficulties. There are specific and non-specific fac- below the stoma and at least 1-2 cm from the carina 36. The tors that contribute to the disease. Non-specific factors are National Guideline Clearinghouse (NGC) 37 recommend that basic diagnosis, health status of the baby and type of sur- skin care of the stoma and under the tracheostomy ties be gery which has undergone. Specific factors can be further provided at least daily, and more often if indicated, to prevent divided in immediate and delayed. The immediate factors pressure necrosis and to maintain intact, clean and dry skin. are anatomical structure transformation, altered protection of Tracheostomy tube suctioning should be performed at least airway and presence or absence of the tracheostomal tube. twice daily and as needed, based on clinical assessment to The delayed factors are development of sensory skills, ac- assure tracheostomy tube patency (ATS). t is recommended ceptance of feeding aids, acceptance of food more consist- that tracheostomy tube changes are performed routinely by ent, development of oral motor skills like chewing, adjusting institutional standards to maintain airwayI patency; the first meal times and appropriate eating behaviour. change is made within 5-7 days for surgical tracheostomy Considering the importance of swallowing and breathing and within 10-14 days for percutaneous tracheostomy. The for general health and in the quality of life of the child, it further changes are routinely made every 2-4 weeks accord- is very important to manage these diseases with a mul- ing to the Cincinnati Children’s Hospital Center (CCHMC) tidisciplinary approach involving surgical, medical and guidelines 38. There is insufficient evidence and a lack of rehabilitative features 42. consensus to make a recommendation on the use of heated In the literature, there are many studies that stress the impor- versus cool humidification in prevention of mucous plug- tance of evaluating feeding and swallowing skills of the child ging (ATS). According to AAO-HNSF decannulation can be before and after surgery; this is done to better understand the made if clinically there is resolution of the primary disease, functional ability of the child and understand if the swallow- no active infection, tolerance of the speaking valve, endo- ing problems are related to the basic diagnosis or a conse- scopically there is a clear tracheobronchial tree without su- quence of the surgery on anatomical structures 43-45. prastomal granuloma and functionally if there is an adequate The evaluation protocol includes the presence of alter- cough reflex. The process of decannulation needs observa- native nutrition; evaluation of the different mechanisms tion for 24-48 in a monitored setting 36. Every child with of sensory and motor parts of deglutition; assessment of tracheostomy should be referred to a phoniatric clinic for the management of oral secretions; the evaluation of the evaluation of the phonation and deglutition. Rehabilitation mechanisms of sucking/chewing and swallowing; breath- of speech relies on phonatory valves, signs language, laryn- ing during the meal; and the amount of food administered. gophone and fenestrated tracheotomy tubes. Evaluation of If swallowing function will be evaluated with instrumen- the deglutition to rehabilitate the patient for feeding is made tal tests, the child may undergo a video-fluoroscopic swal- with administration of methylene blue solution under vide- lowing exam. olaryngoscopy and with scintigraphic study of deglutition. The goal of preoperative evaluation is to understand the anatomical and physiological factors that determine Dysphagia following laryngo-tracheal any difficulty feeding in order to react appropriately and quickly in the postoperative period. After intervention, as- reconstruction sessment will be carried out to evaluate the results and The laryngeal anatomical structures involved in swallow- make a rehabilitation plan with the objective of recover- ing are epiglottis, laryngeal vestibule and vocal cords 39 40. ing the best features. The rehabilitation project must be Anatomical alterations of upper airways causing stenosis customized and targeted, and should not only take into may prevent coordinated process of swallowing, affecting account swallowing function, but also respiratory func- its efficiency and safety 41. Surgery designed to restore tion, and must include weaning from tracheal cannula if the airway in laryngo-tracheal anomalies can cause or ex- de-cannulation is possible, or use of speech valve. acerbate the alterations of swallowing mechanisms and interfere transiently or permanently on airway protection Voice rehabilitation in children: two mechanisms causing dysphagia. Surgery of the airway different possible protocols include both endoscopic (epiglottoplasty, dilatations and balloon surgery, vocal fold lateralisation, partial aryte- The Voice Craft method (Estill Voice Training) was es- noidectomy, closure of laryngo-tracheo-esophageal cleft, tablished in 1988 by American singing voice specialist Jo 5 R. Bovo et al. Estill and is characterised by a series of vocal manoeu- Fig. 2. Proprioceptive-elas- tic therapy (PROEL). vers to develop specific control over individual muscle groups within the vocal mechanism 46. Training comprises 13 vocal exercises or compulsory figures. Each ‘figure’ establishes control over a specific structure of the vocal mechanism, in isolation, by moving the structure through a number of positions. The most important compulsory figure is probably the false vocal folds control, which identifies three possible positions of the false vocal folds: constricted, mid and retracted. Moreover, Estill Voice Training incorporates six ‘voice qualities’ as mechanisms for demonstration of voice production control. The six voice qualities are speech, sob, twang, opera, belting and falsetto. A protocol of 12 training sessions for the voice rehabilitation in children has been described by Fussi and Turlà 47. In the first session, children and parents cooperate in collecting clinical history and receiving together initial makes an “impression” on the brain that allows them to in- and simple counseling about vocal hygiene and possible ternalise a “phonatory pattern” correctly. Perception train- consequences of voice disorders. Children receive a diary ing is thus fundamental even in the PROEL method: chil- to note correct or un-correct vocal use during the day eve- dren should become conscious of their voice qualities, of ry evening. During the second session, several relaxing the mechanisms of voice production, how to use the voice and stretching exercises are carried out. Perceptive train- in the different daily situations and how to avoid voice fa- ing (starting from the intensity parameter) is taught during tigue. Rehabilitative training should be considered com- the third session, using specific games and exercises. The pleted when the child has perfectly consolidated the correct fourth and fifth sessions are dedicated to correct breathing use of voice in relation to total body, is able to recognise the and pneumo-phonic coordination. These aspects are gen- possible risk factors for his/her voice and is able to self-cor- erally boring to children and need to be taught through dif- rect voice misuse or abuse without an external guide. The ferent games (soap balls, wind imitation, etc.). The sixth PROEL method has been modified by De Maio et al 49 for session is dedicated to control of the correct acquisition optimal use in paediatric dysphonic patients. Ludic activi- of breathing and to further auditory perception training ties, on several occasions chosen by the young participants on intensity and pitch. In the seventh session exercises of themselves, have demonstrated to be useful in improving articulation and exercises to experiment and perceive vo- voice production and enjoyable by the children. cal tract resonance are proposed. Several nasalisation and A new instrument for the evaluation of the benefits ob- chewing movements are explained. During the eighth ses- tained with different treatments for the voice disorders in sion, children are invited to sing a simple tune using dif- children is the Children Voice Handicap Index 10 ques- ferent modalities of voice productions, correct breathing tionnaire (CVHI-10). This questionnaire is easily admin- and coordination, hyperarticulation etc. The basic vocal istered, highly reproducible, with good clinical validity qualities described in the Estill method are explained in and responsiveness to treatment 50. the ninth lesson: speech, sob, belting and twang. During the 10th and 11th lessons, children must produce the differ- Aural atresia rehabilitation ent voice qualities by imitating several cartoon characters. Finally, during the last session participants are involved in Patients with bilateral atresia should have either a softband dramatising a simple story by using different voice quali- BAHI (Bone Anchored Hearing Implant) or a conventional ties and productions, depending on the specific context headband bone conduction hearing aid in early life to pro- of the story. Post-treatment evaluation generally demon- vide adequate stimulation for development of the central strates that children have acquired better consciousness nervous system. When the child grows older, percutaneous of their voice instrument, use different voice modalities BAHI becomes an option 51, usually by 5 years old. The depending on the specific situation, significantly reduce surgical technique has been recently simplified with the vocal abuse and enjoy in playing with their voice. linear incision (Fig. 3), instead of the skin flap technique 52. The aim of the “Proprioceptive-Elastic Voice Rehabilita- Canalplasty is a choice for selected candidates after the age tion Program” (PROEL) (Fig. 2) 48 is to achieve a relaxa- of 8. The anatomy of the temporal bone is a major con- tion of muscle stiffness and to obtain elasticity through fa- founding factor in the approach of hearing rehabilitation. cilitating postures, unstable balance, and body movement. Patients with good middle ear anatomy (Jahrsdoerfer score The method of work is “experimental”: a hoarse patient 7 or above) 53 may be suitable for canal reconstructions, compares his/her voice before and after exercise, and thus although this kind of surgery is less frequently carried out 6 Rehabilitation of children with ENT disorders Fig. 3. The linear incision and the insertion of a new-profiled abutment. Fig. 4. Apparent good surgical outcomes of atresiaplasty. However, recur- rent external otitis exacerbated by whatever kind of ear moulds required the implantation of BAHI. than in the past. In most studies comparing BAHI and pa- tients with EAC reconstruction, BAHI has generally re- bone is required. Despite some reports of successfully im- sulted in significant hearing gain vs. reconstruction 54. The planted paediatric patients, this procedure requires a great mean postoperative speech reception threshold is 25-35 dB deal of caution. HL, which is the range of mild hearing loss and around For those with unilateral disease, time is allowed for a 30% of patients still need a conventional hearing aid to as- thorough discussion and consideration of treatment op- sist with hearing after surgery 54. In addition, otologists are tions. In fact, it has been well established that patients often frustrated with the relatively common occurrence of with unilateral hearing loss may have difficulties in com- restenosis and recurrent infections of the canal (Fig. 4). The munication and at least 25% of the patients’ parents and reported rate of restenosis ranges from 5 to 29% and around teachers report behavioural problems and academic per- 26% of the population requires reoperation. Serious com- formance issues 57. plications may also occur during this surgery, such as wors- There is still controversy about how much we should do for ening of hearing loss and facial nerve palsy in 1% of cases, patients with unilateral congenital aural atresia. Restoring as up to 30% of this group of patients have an anomaly in binaural hearing can avoid the development of an ear domi- the course of their facial nerve 55 56. nance syndrome and numerous studies have shown that it Vibrant sound bridge (VSB), on the other hand, is im- brings more benefits than harm, including better hearing in planted inside one middle ear, which provides unilateral noise, improved distance hearing and elimination of head- stimulation to the inner ear system. This unilateral direct shadowing 58. However, to avoid a dominant syndrome, inner ear stimulation completely removes the possibility binaural hearing should be promptly restored, most prob- of signal confusion. VSB is a middle ear implant consist- ably in the first years of life. As every patient’s needs and ing of two parts, the external audio processor (EAP) and expectations vary, detailed discussion should take place to the implantable vibrating ossicular prosthesis (VORP). achieve the best solution for each individual case. The EAP picks up sound signals, amplifies them and In some patients who need repeated magnetic resonance transmits them to the VORP. The floating mass transducer imaging (MRI), for example in patients with neurological (FMT), in the distal part of the VORP, vibrates the at- diseases, the method of hearing rehabilitation has to be tached middle ear structure through a single point of at- carefully considered. This is because MRI is generally not tachment and thereby stimulates the cochlea. recommended for patients with implantable hearing aids, In aural atresia patients, the FMT can either be attached like VSB. BB can tolerate MRI scanning up to 1.5 tesla to the stapes, if it is functioning, or to the round window, and BAHI up to 3 Tesla. These implants also produce ar- in which case the procedure is known as round window tifacts in images of the brain. BAHI is the least disturbing vibroplasty. During VSB implantation, the usually mal- due to its small implant size. formed middle ear cleft has to be entered to insert the In summary, most patients with aural atresia benefit from FMT. It carries a risk of injury to facial nerves and the hearing rehabilitation. The choices are conventional inner ear, while no such complications occur in BAHI. headband bone conduction hearing aid, softband BAHI, Bonebridge (BB) was initially designed for patients over canalplasty, percutaneous BAHI, VSB and BB. Each op- 18 years old because a certain thickness of the cortical tion has its strengths and weaknesses. Early identification 7 R. Bovo et al. and referral for further management are key to obtain a cardinal elements of music, i.e. rhythm, melody and tim- successful long-term outcome. bre, in most hearing aided or implanted deaf children only rhythm perception is reported to be similar to that of lis- Cochlear implant teners with normal hearing. In fact, even with technically sophisticated multiple-channel Cochlear implant in children is a safe procedure with a low sound processors, recognition of melodies is poor, with per- risk of complications as largely reported in recent literature formance at little better than chance levels for many implant 59-61. Immediate and late complications are not significantly users. This can also be observed when the tunes are famil- different when surgery is carried out before one year of age, iar and are played as a sequence of isolated notes without with respect to 12-18 months (P = 1). Moreover, anaesthe- accompaniment or harmony. Thus, discrimination of music siological complications are rare, probably ranging from 0 interval, tone sequences with ascending or descending notes, to 1.5 %, even in syndromic patients 62. Early implantation or simple melodic structures are greatly deteriorated abili- is believed to enable the maximal development of com- ties in profound deaf children. On the other hand, one should munication skills, social exchange and cognitive abilities. consider that often the concepts of high and low are difficult The lower age limit of implantation is therefore not related even for hearing students who confuse the term with loud to surgical or anaesthesiological considerations, but to the and soft. Nevertheless, the ability to differentiate between consideration that very early audiological diagnosis is not notes is necessary to study and learn melodies. Perception always reliable, even when established at tertiary centres, of timbre, which is usually evaluated by experimental pro- and not all children identified as suffering from sensorineu- cedures that require subjects to identify musical instrument ral hearing loss (SNHL) will have permanently impaired sounds, is also generally unsatisfactory 65 68. Only the dis- threshold levels. The absence of any electrophysiological crimination of the different groups of musical instruments is or behavioural reaction to sound does not always reflect generally possible (i.e. string-, wood-, brass-, percussion- in- SNHL. Bovo et al. 61 have recently described a case series struments), while recognising a single instrument among its of 23 newborns with a diagnosis of severe-to-profound group is very difficult. Despite all these limitations, Hash 68 hearing loss at three months, who significantly improved stated, “Considering an ‘average’ profound hearing loss, the even reaching a normal auditory threshold during the first motivated student is capable of learning to play an instru- year of life. All the full term neonates showed a significant ment to at least an intermediate level”. Some instruments improvement in their initial threshold within 6 months of will be easier for the student who is deaf or hard of hearing age. On the other hand, in most of the premature newborns to learn, but under the right conditions nearly all are audible the initial signs of threshold amelioration occurred beyond over most of their ranges 65 69. Generally, instruments with 70 weeks of gestational age, and even beyond 85 weeks fixed pitches, such as clarinet, saxophone, flute, piano and in one case. The authors assumed that when severe-to-pro- organ are recommended. Some instruments produce strong found SNHL is confirmed and persistent after appropriate vibration that can be felt on the lips, on the chest or by touch- follow-up tests for 6-8 months, cochlear implantation can ing them. Percussion instruments, particularly the bass drum, be carried out with no risk of diagnostic error in term-born are great producers of vibration and can therefore provide infants. On the contrary, follow-up for severely pre-term a very tactile experience. On the other hand, whenever the babies should last up until no less than 80 weeks of gesta- student is still having difficulties sensing vibrations, instru- tional age. The lower limit of age must not be respected in ment selection should include one that is capable of produc- all cases of post-meningitic deafness or in other causes of ing sustained rather than percussive sounds, as these may be cochlear fibrosis and calcification. easier to perceive via residual hearing. The xylophone, with its clear and short sounds, is considered one of the easiest Music rehabilitation in children instrument to be perceived by the cochlear implanted child. with hearing aid and cochlear implant For further details about the instrument selection for a deaf child, see Hash 69. Singing activities are also strongly recom- According to Ford 63, “the capacity to perceive and assim- mended for these children. Cochlear implanted children are ilate music resides in the brain, and although hearing loss generally able to sing familiar songs from memory, though may impose certain limitations upon the extent to which their performance is significantly poorer than in children musical potential is realised, it does not negate the pres- with normal hearing on almost all pitch-based assessment of ence of innate musicality”. Furthermore, Darrow 64 stated singing 70. that “for the deaf or hard of hearing children, music can be Musical involvement can help a child to develop a positive in some ways more aurally accessible than speech”. There self-image with opportunities for self-expression and can im- is today a general agreement that these children can enjoy prove social interactions. Moreover, music can serve as moti- and participate in music activities and can increase their vational tool for positive behavior and can relieve the tension aesthetic sensitivity 65-67. and struggle that some children experience during language Nevertheless, it is to be considered that among the three training. The music classroom can play an important role in 8 Rehabilitation of children with ENT disorders promoting acceptance and understanding. Musical activity can aid in breaking down any social barriers, thus helping to diminish misconceptions and fear related to hearing loss. Darrow 64 affirms that almost any aural concept can be visu- ally reinforced. The use of Windows Media Player visuals has been recommended because of their colourful represen- tation of music elements such as rhythm, tempo, and melodic direction. In fact, visual representation of music can help to clarify what children perceive. Moreover, body rhythms can be implemented to symbolise rhythmic structure. Pitch can also be illustrated using the body. For example, younger stu- dents can crouch down for low notes and stand on their tip- toes with arms above their heads for higher notes. It has been found that music combined with speech ther- Fig. 5. The instrument «Sound in Hands» designed by McAdams and Bigand apy can have positive effects on the development of both in 1993, composed of two speakers, a response platform and a computer. good listening habits and auditory skills, and on the devel- opment of the suprasegmental elements of language, au- ditory figure-ground discrimination, sequential memory, tor regulating (inhibiting) growth. voice quality and rhythm of speech 69 71. The crucial aspect of the management of NF2 cases is both Nevertheless, it should be noted that only studies that carried the decision of what to do and, not less important, when out a long and intensive musical training with deaf children to do something, in the frame of a balance between the were able to demonstrate a significant benefit on language natural history of the disease and the consequences of ac- perception and productions 72 73. For example, Rouchette et tive therapy. The goal of therapy cannot be the cure of the al. 74 used training that lasted from 1.5 to 4 years conducted disease, but the setting of pro-active therapy to improve by music teachers with a consolidated experience. In con- quality of life and prevent the early fate of the disease. trast, shorter training carried out by parents or caregivers When proactive therapy is not successful, functional reha- failed to demonstrated significant positive results 75. Train- bilitation is feasible, provided early therapy is performed. ing benefits were evaluated by using the instrument «Sound The role of radiotherapy is limited, for the young age of in Hands» (Fig. 5). Evaluation included four aspects of au- the patient, the low success of long-term preservation of ditory perception: discrimination, identification, auditory function and the risk of radio-induced tumours. Clinical scene analysis and auditory working memory. observation, radiology and genetics support the diagnosis. In conclusion, it is still debatable whether music training The disease is evident in 20% of cases before 15 years might have a benefit on language perception and produc- with the bilateral schwannoma, the majority of cases after tion in deaf children. On the other hand, there is a general 18 years presentation 76. agreement that musical involvement can help a child to The new frontiers in the management of NF2 patient ac- develop a positive self-image with opportunities for self- count for: 1) the awareness of the importance of early-stage expression and can improve social interactions. Moreover, surgery conceived as functional surgery; and 2) the possi- hard of hearing children may enjoy music and motivated bility of rehabilitation, especially of the VIII cranial nerve. students are capable of learning to play an instrument to The most frequent condition in NF2 is bilateral acoustic at least an intermediate level. schwannoma. This involves a relentless growth of the tu- mour and an unavoidable loss of hearing over the years, Neurofibromatosis 2. Hearing-related which may be progressive or sudden but is the functional endpoint. Tumour growth in NF2 schwannoma is more management strategies aggressive and rapid than the sporadic and is often char- Neurofibromatosis 2 (NF2) is a genetic disease (autosomal acterised by multiple growing lobules of tumours. The dominant) with a variable expression due to the different natural history of the tumour is also more associated with kinds of mutations, mosaicism being the less serious con- facial palsy than in sporadic schwannoma. dition. It involves benign tumours of the central nervous The treatment involves two different attitudes: a “conserva- system, schwannoma on nerves, or meningiomas. Other tive” attitude, which means watchful observation of tumour tumours are glioma, ependymoma and posterior sub- growth, in which treatment is delayed until function is lost capsular lenticular opacity. Schwannomas on the nerves (hearing) and tumour growth is no more tolerated due to size involve the unavoidable loss of function of the nerve of and impending risk of brain compression. When surgery is origin. The incidence is about 1 new case/every year/over performed, the size of tumour is such to put facial nerve 100,000 inhabitants. The mutations are on chromosome preservation at risk, not differently from what is the rule in 22 in a protein named merlin, which acts as a growth fac- the sporadic tumour. Late surgery with a large tumour pre- 9 R. Bovo et al. vents the possibility of hearing preservation (which is feasi- this proactive surgery is not to rehabilitate function straight- ble and advisable only at a very early stage of tumour,) and forward, but to cure the disease at an early stage with the of the cochlear nerve (which may anatomically be preserved attempt to preserve natural hearing in the best preoperative only in small-medium size tumours and its possibility of conditions and rehabilitate it only in case of failure. functioning with a cochlear implant seems to be inversely We summarise our guidelines in Table I for sporadic tu- related to the preoperative loss of hearing). The advantage mours. of this “conservative” attitude is to leave natural function In NF2 patients, the choice is more difficult and should be to patients (hearing and facial nerve) as long as this is pos- balanced in the frame of the severity of the disease and the sible. The consequences of a delayed surgical treatment are expectations of the patient and family. higher intraoperative surgical risks, higher morbidity on the Some considerations are preliminary to any decision- facial nerve and no possibility of hearing preservation/hear- making process in the NF2 patient. ing rehabilitation with cochlear implant. Why should we A multidisciplinary nature of the approach (neuro-otol- adopt a conservative strategy, which prevents any possibil- ogist, neurosurgery, genetics, radiotherapy, oncologist); ity of preservation/rehabilitation of function? The answer is the high rates of success of hearing preservation surgery in the complexity of the disease, where every choice pays in sporadic acoustic neuroma are probably less reproduc- a price and has to be balanced very carefully. Sometimes it ible in the NF2 patient 69. The inexorable growth, the mul- may be reasonable, for serious NF2 patients, to leave hear- tilobular pattern of presentation of the tumour relate to ing functions intact (at one side at least) as long as this is more aggressive involvement of the cochlear nerve and, feasible in the so-called crucial years, when education takes to a lesser extent, of the facial nerve; bilateral acoustic its course and postpone the problem of the management of neuroma is often not an isolated condition, but in the NF2 bilateral acoustic neuroma at a later age. It is evident that patient it is associated with other sites as meningioma, this “conservative” attitude, though the most adopted till schwannoma, gliomas. Surgery may involve higher mor- the 2000’s, has limited value nowadays, or rather it is still bidity due to the presence of other adjacent tumours (i.e. an option but has to be balanced in the frame of a more lower posterior fossa, jugular foramen, foramen magnum) updated program of management of the NF2 patient. The that may affect the function of other cranial nerves and the “proactive” attitude involves an early therapy at an early CSF pathways; hearing rehabilitation with ABI, though stage tumour. When good function is still present (hearing) always feasible, should be the last option after any at- it may be preserved with hearing preservation surgery 77 tempt of hearing preservation/hearing rehabilitation with and, if not, may be rehabilitated with cochlear implant 78. cochlear implant. Similarly, good facial nerve preserva- In our experience in sporadic and NF2 acoustic neuromas, tion should be the goal in small-medium size tumours. we observed better results in hearing rehabilitation with When not feasible, intraoperative reconstruction of the cochlear implant when: 1) the tumour was small and the facial nerve with a graft should be the option. cochlear nerve did not suffer longtime from the presence In conclusion, the new trend in NF2 management involves of tumour; 2) hearing was preoperatively good and a func- proactive therapy in the attempt to preserve function or reha- tioning cochlear nerve could be preserved. The rationale of bilitate it in case of failure. The attitude of leaving the disease Table I. Treatment algorithm of sporadic vestibular schwannoma in our centre. T size (mm) Decision factors Treatment Intrameatal or Good hearing class A (B) Hearing preservation surgery T < 10 in CPA (observation) Good hearing class A (B) + risk Observation* Bad hearing Observation* or hearing rehabilitation T 10-15 No growth Observation* or surgery Growth Surgery Growth + risk Radiotherapy T 15-25 - Surgery or Radiotherapy T > 25 - Surgery Risk Partial surgery + radiotherapy Any size Cystic tumour Surgery * Active treatment is planned in case of vertigo or new onset of 7the c.n. defect or C.I. rehabilitation. 10