Does parental experience of the diagnosis and intervention process differ for children with auditory neuropathy?

ObjectivesThis study compared parental experience of the audiological diagnosis and intervention process in children with auditory neuropathy spectrum disorder and sensory neural hearing loss.MethodsA matched group survey was used with parents of children with auditory neuropathy spectrum disorder (ANSD) matched with a control group of parents and children with sensorineural hearing loss (SNHL). The two groups were matched in terms of the child's gender, age, amplifications used, social background and utilisation of private or public health care sectors. An interview questionnaire, consisting of 45 questions in six categories (1. biographic information, 2. experiences of audiological diagnosis, 3. hearing aid benefit, 4. parental experience of the rehabilitation decision making process, 5. parental needs for emotional support and 6. parental needs for information) using a 5-point Likert scale for categories 2–7, was administered by the same audiologist.ResultsChildren with ANSD experienced a significantly longer waiting period from diagnosis to hearing aid fitting (p = 0.025) and/or cochlear implantation (p = 0.036). Parents of children with ANSD reported significantly different experiences of the diagnostic process (p = 0.001) with poorer understanding of the diagnosis and reporting insufficient time allowed for asking questions. During the rehabilitation decision-making process 47% of parents with ANSD children (vs. 0% of parents with SNHL children) reported receiving conflicting information. Parents of children with ANSD were also less likely to recommend hearing aids to other parents. Information needs were similar between groups.ConclusionsParents of children with ANSD have different experiences and greater uncertainty during the diagnostic and rehabilitation process. Providing regular consultation and structured timelines through the diagnostic process and decision-making process may facilitate this process with less uncertainty.     

Childhood hearing impairment in northern Finland,etiology and additional disabilities

ObjectivesThe purpose of this study was to determine the prevalence and etiology of hearing impairment (HI) in Finnish children and to evaluate the frequency and type of additional disabilities among children with HI.MethodsSubjects consisted of 214 children with mild to profound HI ascertained until the age of 10 years. They belonged to the birth cohort spanning the years 1993–2002 in northern Finland. The clinical data were collected from the electronic patient records of the Oulu University Hospital. Age at ascertainment, degree and type of HI and audiogram configuration were determined. Risk factors and etiology of HI and co-existing disabilities were recorded.ResultsThe prevalence of childhood HI was 2.3/1000 live births (95% CI; 2.0, 2.7). The etiology of HI was genetic in 47.2%, acquired in 16.4% and unknown in 36.4% children. Among the 214 children with HI, 101 (47.2%) had other minor or major disabilities. The frequency of additional disabilities did not differ between children with mild HI and those with moderate or severe HI (p = 0.78). Additional disabilities were more common (65.7%) in children with acquired HI than in children with genetic or unknown HI (43.6%) (p = 0.035).ConclusionThe prevalence of childhood HI has remained unchanged in northern Finland as compared to previous studies. Genetic causes were the most common (47%) etiology of childhood HI. Among acquired causes of HI, perinatal risk factors were more common than previously. The frequency of additional disabilities was similar among children with different degrees of HI. Because almost 40% of children had one or more additional disabilities affecting development or learning, it is important to take them into consideration in rehabilitation.     

Hearing loss in urban South African school children (grade 1 to 3)

ObjectiveThis study aimed to describe the prevalence and characteristics of hearing loss in school-aged children in an urban South African population.MethodChildren from grade one to three from five schools in the Gauteng Province of South Africa formed a representative sample for this study. All children underwent otoscopic examinations, tympanometry and pure tone screening (25 dB HL at 1, 2 and 4 kHz). Children who failed the screening test and 5% of those who passed the screening test underwent diagnostic audiometry.ResultsA total of 1070 children were screened. Otoscopic examinations revealed that a total of 6.6% ears had cerumen and 7.5% of ears presented with a type-B tympanogram. 24 children (12 male, 12 female) were diagnosed with hearing loss. The overall prevalence of hearing loss was 2.2% with Caucasian children being 2.9 times more (95% confidence interval, 1.2–6.9) likely to have a hearing loss than African children.ConclusionHearing loss prevalence in urban South African school-aged children suggest that many children (2.2%) are in need of some form of follow-up services, most for medical intervention (1.2%) with a smaller population requiring audiological intervention (0.4%).     

Supraglottoplasty outcomes in children with Down syndrome

ObjectiveChildren with Down syndrome have a higher incidence of upper airway obstruction and laryngomalacia. We sought to determine outcomes of supraglottoplasty in this patient population.MethodsA retrospective chart review was performed from January 2000 through January 2014. Children (n = 18) at our institution with the diagnosis of Down syndrome who underwent supraglottoplasty were included. We reviewed patient characteristics, preoperative findings, and surgical outcomes (stridor, feeding problems, respiratory distress, weight, sleep apnea, and tracheostomy or feeding tube dependence).ResultsThe average age at surgery was 7.7 months. Operative indications included feeding difficulties (n = 9), noisy breathing or respiratory distress (or both) (n = 16), and sleep-related symptoms (n = 7). Most patients (89%) were extubated successfully on postoperative day 1. There were 2 major complications (CPAP requirement and aspiration pneumonia) and no perioperative deaths. Fifty percent had improved weight (mean = 18 percentile points). Feedback was available from 88% of parents with 100% reporting improvement in respiratory symptoms and 93% reporting improved feeding. Eight patients (44%) subsequently required either adenoidectomy or adenotonsillectomy. Two patients later underwent tracheostomy, 2 subsequently needed a gastrostomy tube and 2 required revision supraglottoplasty.ConclusionsThe majority of children with Down syndrome and laryngomalacia benefit from supraglottoplasty, with outcomes of improved breathing, feeding, and sleeping.However, approximately half may require additional airway procedures. This procedure is well tolerated and associated with a low risk of complications especially given their high rate of comorbidities.     

Wideband reflectance in Down syndrome

ObjectiveChildren with Down syndrome (DS) have a high incidence of middle ear disorders and congenital abnormalities of the external, middle and inner ear. Energy reflectance (ER), a wideband acoustic immittance (WAI) measurement parameter, can measure the sound energy reflected or absorbed in the ear canal over a wider range of frequencies more efficiently and faster than conventional single-tone 226 Hz tympanometry. The aim of the present study was to compare the WAI measurements of children with DS with those of typically developing, normal-hearing children according to their tympanometric findings.MethodsFour groups of children with Down syndrome (age range: 2 years and 4 months to 16 years and 3 months; mean age: 8.5 yr) with normal tympanograms (19 ears), flat tympanograms (13 ears), mild negative pressure tympanograms (6 ears between −100 and −199 daPa at the admittance peak) and severe negative pressure tympanograms (4 ears at −200 daPa or lower at the admittance peak) were assessed. All findings were compared with data obtained from 21 ears of a healthy control group (age range: 3 years and 1 month to 13 years and 11 months; mean age: 7.9 yr). The subjects underwent tympanometry with a 226-Hz probe tone frequency and ER measurements along the 200–6,000 Hz range with a chirp stimulus using the Middle-Ear Power Analyzer (MEPA3 – HearID) by Mimosa Acoustics (Champaign, IL), software, version 3.3 [38].ResultsStatistically significant differences were observed in the ER curves for some comparisons between the studied groups. There was also a negative correlation between the static acoustic admittance at the tympanic membrane level and ER measured with a chirp stimulus at 500 and 1,000 Hz. The discriminant analysis technique, which used a chirp stimulus at 1,000 and 1,600 Hz to classify the participants' data based on ER values, achieved a correct classification rate of 59.52% for participants with DS.ConclusionWhile groups with abnormal middle ear status, as indicated by tympanometry, showed higher ER values compared to the DS tymp A group and the control group, similar reflectance curves were observed between control group and the DS tymp A group. WAI shows promise as a clinical diagnostic tool in investigating the impact of middle ear disorders in DS group. However, further research is required to investigate this issue in narrower age range group and a larger sample size.     

Characteristics and utility of combined duplex color-coded ultrasonography in the cervical rotational position with head MRI and MRA in the differential diagnosis of apogeotropic direction-changing positional nystagmus

Objective: To determine whether cervical ultrasonography, magnetic resonance imaging(MRI), and magnetic resonance angiography(MRA) are useful in the differential diagnosis of etiology and understanding the pathophysiology in cases of apogeotropic direction-changing positional nystagmus(DCPN).Methods: Thirty patients with apogeotropic DCPN were classified into 11 cases of central disease, seven cases of mixed central and peripheral disease, and 12 cases of peripheral disease by differential diagn...     

Possible role of anti-inflammatory drugs in complications of pharyngitis. A retrospective analysis of 163 cases

ObjectivesComplications of pharyngitis (peritonsillar abscess, retropharyngeal abscess, and cervical cellulitis) are rare, but appear to be on the increase over recent years and many of these patients have been treated by anti-inflammatory drugs prior to admission. The purpose of this study was to review the current epidemiological data concerning these complications and investigate a possible correlation with anti-inflammatory drug use.Material and methodsA single-centre retrospective review of epidemiological, clinical and microbiological data was performed on the medical charts of patients hospitalised for peritonsillar abscess, retropharyngeal abscess or cervical cellulitis between 2005 and 2010.ResultsOver a six-year period, 163 patients were hospitalised for complications of pharyngitis, with a sex-ratio of 1.82 (104/57). The number of cases of peritonsillar abscess (PTA) increased from 13 to 28 cases per year from 2005 to 2010 and the number of cases of retropharyngeal abscess increased from three to six cases per year over the same period. The number of cases of cellulitis remained stable with an average of 1.82 cases per year. Each year, significantly more patients with an abscess were admitted to our unit with a history of anti-inflammatory drug use (13.3 ± 4.6) than without anti-inflammatory drug use (7.8 ± 4.3) (P < 0.01). Micro-organisms were identified in 80% of cases, with mixed strains in 73% of cases, Streptococcus in 72% of samples and Streptococcus pyogenes in 19% of cases of PTA. A favourable outcome was observed in all patients in response to medical and surgical treatment.ConclusionIn line with the literature, we observed an increasing incidence of complications of pharyngitis. The present series comprised significantly more patients admitted for PTA with a history of anti-inflammatory drug use. A multicentre prospective controlled study in Nantes on a large cohort is currently underway and will probably confirm these preliminary results.     

Injuries due to foreign body aspirations in Georgia: A prevention perspective

BackgroundSuffocation due to foreign bodies (FB) is a leading cause of death in children aged 0–3. No data from the former U.S.S.R. are available in the international scientific literature.MethodsConsecutive patients admitted at the Iashvili Central Children Hospital in Tbilisi, Georgia from 1989 to 2011 were analyzed. Injuries in the upper airways due to foreign bodies’ inhalation were collected and compared with the Susy Safe Registry and the pooled estimates of the meta-analysis.Results2896 cases were collected. Distribution of injuries in children younger than 3 years was significantly higher than in the Susy Safe Registry and in the “High-Income” countries in the meta-analysis. Percentage of injuries due to organic objects (86%) was significantly higher than in published data.ConclusionsSince Georgia is not showing any substantial difference, both in epidemiology and treatment of foreign bodies injuries, as compared to the other case series, translation of public health initiatives from other most advanced prevention experiences is possible and it is likely to be effective.Level of evidenceLevel V, Epidemiological case series.     

Long-term complications of the transmeatal approach (Open Transcanal) in cochlear implants: A follow-up study

Objective: Multiple alternative approaches of cochlear implant surgery have been described, such as the suprameatal approach, transcanal approach, transmeatal approach and middle cranial fossa approach.Transmeatal(open trnascanal) approach has not been adapted since first described in the clinical field.we aimed to assess the long-term complications of the transmeatal approach in a series of 131 patients at our center between 2004 and 2008.Methods: This study was a retrospective case series of a...     

Premaxillary abscess without bony erosion: An unusual complication of pediatric acute maxillary sinusitis

ObjectivesTo report an unusual complication of pediatric acute maxillary sinusitis: premaxillary abscess. To describe clinical, radiological and biological presentation, treatment strategy and progression.Material and methodsA retrospective study included all pediatric patients treated for premaxillary abscess complicating acute maxillary sinusitis in two ENT reference centers between 1999 and 2017. Disease history, clinical presentation, biological and radiological findings, treatment modalities and progression were studied.ResultsTen patients were included, with a mean age of 10 ± 4.2 years. All presented with fever, rhinorrhea and premaxillary edema. Contrast-enhanced CT scan systematically found complete opacity of the maxillary sinus, without bone lysis, and extensive effusion along the intersinonasal wall up to the premaxillary region, extending in 3 cases back toward the parapharyngeal space. Bacteriology isolated Streptococcus anginosus most frequently (n = 4; 40%). Treatment comprised intravenous wide-spectrum antibiotics, with surgical drainage of the abscess if > 10 mm (n = 9; 90%). Seven of these 9 patients (78%) had recurrent abscess requiring surgical revision and 3 (33%) required a third drainage. All patients were cured without sequelae at 1 month.ConclusionIn case of acute maxillary sinusitis with premaxillary edema, premaxillary abscess should be suspected. The high recurrence rate argues for maximalist surgery associated to close clinical monitoring with radiological examination.     

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient

ObjectiveTo provide recommendations for the workup of hearing loss in the pediatric patient.MethodsExpert opinion by the members of the International Pediatric Otolaryngology Group.ResultsConsensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss.ConclusionThe workup of children with hearing loss can be guided by the recommendations provided herein.     

Vestibular function in carotid territory stroke patients

Stroke patients may present otoneurological symptoms.ObjectiveTo assess the vestibular function of subjects with a history of carotid territory stroke.MethodThis historical cohort cross-sectional study enrolled 40 patients; subjects answered the Dizziness Handicap Inventory, were interviewed and submitted to ENT examination and vector electronystagmography.ResultsMild saccadic movement anomalies were seen in 20 patients (50.0%); nine complained of imbalance and dizziness. Abnormal smooth pursuit gain was seen in 17 cases (42.5%); six subjects reported imbalance and one complained of dizziness. Abnormal directional preponderance during rotational nystagmus was seen in two cases (5.0%), who also reported imbalance. Three patients (7.5%) and two subjects (5.0%) were found to have abnormal labyrinthine predominance and abnormal nystagmus directional preponderance respectively; all five individuals reported imbalance. Ten of the 11 patients without complaints of disordered balance had altered saccadic and smooth pursuit eye movements, while one had unaltered vestibular function.ConclusionPatients with a history of carotid territory stroke may suffer from dizziness or imbalance and present signs of compromised eye motility and vestibular function.     

“Sitting-up vertigo as an expression of posterior semicircular canal heavy cupula and posterior semicircular canal short arm canalolithiasis”

BackgroundVestibular symptoms on sitting-up are frequent on patients seen by vestibular specialists. Recently, a benign paroxysmal positional vertigo (BPPV) variant which elicits vestibular symptoms with oculomotor evidence of posterior semicircular canal (P-SCC) cupula stimulation on sitting-up was described and named sitting-up vertigo BPPV. A periampullar restricted P-SCC canalolithiasis was proposed as a causal mechanism.ObjectiveTo describe new mechanisms of action for the sitting-up vertigo BPPV variant.MethodsEighteen patients with sitting-up vertigo BPPV were examined with a pre-established set of positional maneuvers and follow-up until they resolved their symptoms and clinical findings.ResultsAll patients showed up-beating torsional nystagmus (UBTN) and vestibular symptoms on coming up from either Dix-Hallpike (DHM) or straight head-hanging maneuver. Sixteen out of 18 patients presented a sustained UBTN with an ipsitorsional component to the tested side on half-Hallpike maneuver (HH). A slower persistent contratorsional down-beating nystagmus was found in eleven out 18 patients tested on nose down position (ND).ConclusionsPersistent direction changing positional nystagmus on HH and ND positions indicative of P-SCC heavy cupula was found in 11 patients. A sustained UBTN on HH with the absence of findings on ND, which is suggestive of the presence of P-SCC short arm canalolithiasis, was found on 5 patients. All patients were treated with canalith repositioning maneuvers without success, but they resolved their findings by means of Brandt-Daroff exercises. We propose P-SCC heavy cupula and P-SCC short arm canalolithiasis as two new putative mechanisms for the sitting-up vertigo BPPV variant.     

The translabyrinthine approach in residency training.“Step by step”surgical anatomy from the dissection laboratory to the operative room

Surgical anatomy training in a dedicated research laboratory and attendance to focused“hands-on”dissection courses are of high educational importance in order to acquire and maintain surgical expertise in skull base surgery,both for young and more experienced surgeons.Nevertheless,transitioning surgical skills and anatomic knowledge from the laboratory to the operative room it is not free of challenges,especially during skull base approaches where the three-dimensional surgical orientation can be quite complex.We present a“step-by-step”and“side-by-side”surgical anatomy report on a translabyrinthine approach that was practiced in the laboratory then performed in the operative room by the surgical team,and we compare surgical anatomy exposures while discussing intraoperative techniques,nuances and challenges,both in the laboratory and the operative room.     

Guidelines of the French Society of Otorhinolaryngology (SFORL), short version. Extension assessment and principles of resection in cutaneous head and neck tumors

Cutaneous head and neck tumors mainly comprise malignant melanoma, squamous cell carcinoma, trichoblastic carcinoma, Merkel cell carcinoma, adnexal carcinoma, dermatofibrosarcoma protuberans, sclerodermiform basalioma and angiosarcoma. Adapted management requires an experienced team with good knowledge of the various parameters relating to health status, histology, location and extension: risk factors for aggression, extension assessment, resection margin requirements, indications for specific procedures, such as lateral temporal bone resection, orbital exenteration, resection of the calvarium and meningeal envelopes, neck dissection and muscle resection.     

Surgical treatment of pulsatile tinnitus related to the sigmoid sinus

Objective: Tinnitus—a common clinical symptom—can be categorized into pulsatile tinnitus(PT) and non-PT. Among these, PT is usually associated with sigmoid sinus symptoms, such as sigmoid sinus wall defect or diverticulum, for which various surgical treatments are available. We have discussed the clinical efficacy of surgery for sigmoid sinus-associated PT via the transmastoid approach in this study.Methods: We conducted a retrospective review of 4 patients who underwent surgery for sigmoid sinu...     

Clinical characteristics for conservative therapy of pediatric parapharyngeal abscesses

IntroductionThe role of surgical drainage versus conservative therapy in treating patients with parapharyngeal abscesses is still a theme of debate.ObjectivesThis study aimed to investigate the characteristics associated with good outcomes in pediatric patients with parapharyngeal abscesses treated with conservative therapy.MethodsThis retrospective chart review was performed on children aged 0.3–14 years with the diagnosis of parapharyngeal abscesses confirmed by computed tomography from January 2013 to March 2018. Patients with a severe upper airway obstruction required early intervention, while those in a stable condition initially received conservative therapy with antibiotics. If the patients appeared unlikely to recover, additional surgical drainage was provided. Multivariate logistic regression models were constructed to investigate the clinical characteristics associated with a good response to conservative therapy. A receiver operating characteristic curve was used to identify the age and abscess size cutoff for predicting a successful response.ResultsA total of 48 children were included in the study. Patient age, antecedent illness, and abscess size were significantly associated with a response to therapy (Odds Ratio = 1.326, 2.314 and 1.235, respectively). The age cutoff associated with the conservative therapy was 4.2 years (76.9% sensitivity, 68.2% specificity), and the abscess size cutoff associated with the conservative therapy was 23 mm (84.6% sensitivity, 77.3% specificity).ConclusionThe findings suggested that younger age, smaller abscess size, and less frequent antecedent illnesses, such as upper respiratory tract infection and lymphadenitis, could predict a successful response to conservative therapy in pediatric patients with parapharyngeal abscesses.     

Effect of minocycline and its nano-formulation on central auditory system in blast-induced hearing loss rat model

Blast injuries are common among the military service members and veterans. One of the devastating effects of blast wave induced TBI is either temporary or permanent hearing loss. Treating hearing loss using minocycline is restricted by optimal drug concentration, route of administration, and its half-life.Therefore, therapeutic approach using novel therapeutic delivery method is in great need. Among the different delivery methods, nanotechnology-based drug delivery is desirable, which can achiev...     

Delayed cochlear implantation in post-meningitic deafness and hereditary complement C2 deficiency

We report the case of an adolescent with post-verbal severe/profound sensorineural hearing loss, occurring as a consequence of two bouts of pneumococcal meningitis at 12 and 32 months of age. A possible immunodeficiency was investigated, revealing hereditary complement C2 deficiency (C2D). Given the insufficient benefit from high-power hearing aids, the boy received a cochlear implant (CI) at age 12. Despite the long interval of partial hearing deprivation and the post-meningitic etiology, improvement in open-set speech perception and quality of life were observed. The C2D did not favour post-operative infections nor meningitis recurrence. The risks and benefit of CI in this peculiar clinical circumstance are discussed after reviewing the literature.