Neonatal Respiratory Distress and Airway Emergency: Report of Two Cases.

We discuss two cases of congenital airway malformations seen in our neonatal intensive care unit (NICU). The aim is to report extremely rare events characterized by immediate respiratory distress after delivery and the impossibility to ventilate and intubate the airway. The first case is a male twin born at 34 weeks by emergency caesarean section. Immediately after delivery, the newborn was cyanotic and showed severe respiratory distress. Bag-valve-mask ventilation did not relieve the respiratory distress but allowed for temporary oxygenation during subsequent unsuccessful oral-tracheal intubation (OTI) attempts. Flexible laryngoscopy revealed complete subglottic obstruction. Postmortem analysis revealed a poly-malformative syndrome, unilateral multicystic renal dysplasia with a complete subglottic diaphragm, and a tracheo-esophageal fistula (TEF). The second case is a male patient that was vaginally born at 35 weeks. Antenatally, an ultrasound (US) arose suspicion for a VACTERL association (vertebral defects, anal atresia, TEF with esophageal atresia and radial or renal dysplasia, plus cardiovascular and limb defects) and a TEF, and thus, fetal magnetic resonance (MRI) was scheduled. Spontaneous labor started shortly thereafter, before imaging could be performed. Respiratory distress, cyanosis, and absence of an audible cry was observed immediately at delivery. Attempts at OTI were unsuccessful, whereas bag-valve-mask ventilation and esophageal intubation allowed for sufficient oxygenation. An emergency tracheostomy was attempted, although no trachea could be found on cervical exploration. Postmortem analysis revealed tracheal agenesis (TA), renal dysplasia, anal atresia, and a single umbilical artery. Clinicians need to be aware of congenital airway malformations and subsequent difficulties upon endotracheal intubation and must plan for multidisciplinary management of the airway at delivery, including emergency esophageal intubation and tracheostomy.

Cognitive improvement after cochlear implantation in deaf children with associated disabilities.

AIM: To monitor functional auditory and non-verbal cognitive skills in children with cochlear implants who had associated disabilities over a 24-month period and define how cochlear implantation may impact on non-verbal cognition by restoring functional auditory skills. METHOD: Sixty-four children with cochlear implants (36 females, 28 males; mean age 4y 3mo, SD 3y 5mo, 9mo-14y 5mo) were recruited and divided into three groups: children with typical development group (TDG); children with associated disabilities not linked to non-verbal cognitive disorders group (ADG1); and children with associated disabilities linked to non-verbal cognitive disorders group (ADG2). Tests of functional auditory, communicative, and non-verbal cognitive skills were performed before cochlear implantation and at 12 and 24 months after cochlear implantation. RESULTS: Functional auditory and communicative skills improved similarly in the three groups at 12 and 24 months after implantation. An increase in non-verbal cognitive scores was present in children in the ADG2 from baseline to 12 and 24 months (p<0.01), whereas scores remained stable in children in the TDG and ADG1. The increased functional auditory skills scores after cochlear implantation corresponded to an increase in non-verbal cognitive scores (p=0.032) in children in the ADG2. INTERPRETATION: Children with associated disabilities, especially if linked to non-verbal cognitive disorders, benefitted from cochlear implantation. They improved their comprehension of acoustic information inferred from the environment, improving not only functional auditory skills but also non-verbal cognition.

Primary mucocele of the mastoid: An incidental finding.

Mucocele is an accumulation of secretion products, desquamation, and inflammation within a body cavity: Localization in the mastoid is extremely rare. Erosion of bony walls and invasion of surrounding structures expose a patient to intra- and extracranial complications. Proper imaging work-up and complete removal through mastoidectomy is warranted.

Cognitive function and quality of life in older adult patients with cochlear implants.

Objective: This study aimed to evaluate the benefits of cochlear implant (CI) on speech perception, quality of life (QoL), and cognitive function in profoundly deafened older adults.Design: Longitudinal and cross-sectional cohort study.Study sample: Participants in this study were CI recipients aged 65 years or older (experimental group), CI recipients aged 50 years or younger (control group 1), and normal-hearing participants aged 65 years or older (control group 2).Results: Speech perception significantly improved in both groups of implanted patients (preoperative vs. 1-year median [IQR] sentence recognition: older patients, 0 (0-20) vs. 90 (70-95), p = 0.001; younger patients, 35 (0-50) vs. 90 (80-100), p = 0.001). There were no significant differences in QoL (Glasgow Benefit Inventory) between younger and older patients (median [IQR] overall benefit score, 42 (31-64) vs. 53 (39-69), p = 0.2). Mini-Mental State Examination scores showed no significant differences between elderly CI recipients and normal-hearing participants (median [IQR], 28 (26-28) vs. 28 (27-29), p = 0.5).Conclusions: Cochlear implantation rehabilitation allows the profoundly deafened older adult to lead a QoL comparable to that of a younger patient. The correlation between hearing restoration and cognitive function preservation in older adults suggest a possible role for CI as a useful resource in limiting age-related cognitive decline.

Sialoendoscopy for treatment of juvenile recurrent parotitis: The Brescia experience.

OBJECTIVE: To evaluate the role of sialoendoscopy associated with steroid irrigation for juvenile recurrent parotitis (JRP) at a tertiary referral hospital. METHODS: Clinical records of patients affected by JRP and treated with operative sialoendoscopy between June 2011 and April 2017 were retrospectively reviewed. Data on demographics, number of acute episodes per year before and after surgery, characteristics of the surgical procedure, hospitalization time, and rate of complications were collected. The outcome of the procedure was measured by comparing the number of episodes of parotid swelling before and after salivary endoscopic treatment. RESULTS: Twenty-three patients for a total of 34 operative sialoendoscopies were included in the study. Before the surgical endoscopic procedure, the mean number of parotid swelling was 10 episodes per year. At sialoendoscopy, typical endoscopic findings such as mucous plugs, stenosis of the duct, intraductal debris, and pale ductal appearance were evident. All patients were discharged on the first postoperative day. A significant decrease in the number of swelling episodes per year was observed compared to the preoperative rate (p = .0004). Complete resolution of the disorder was obtained in 35% of patients. CONCLUSIONS: Operative sialoendoscopy with steroid irrigation can be considered a valid therapeutic treatment for JRP. The technique is conservative, effective, safe, and, potentially repeatable. Short hospitalization time, rapid recovery, absence of peri-operative complications, and a high rate of good outcomes are the main advantages of this treatment.

Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease.

BACKGROUND: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. METHODS: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. RESULTS: Median age was 36 years (range, 25-52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70-139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis. CONCLUSION: Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended. © 2016 Wiley Periodicals, Inc. Head Neck 39: E45-E50, 2017.

Titanium vs hydroxyapatite ossiculoplasty in canal wall down mastoidectomy.

OBJECTIVE: To report hearing results using a titanium ossicular replacement prosthesis during canal wall down mastoidectomy with tympanoplasty to treat cholesteatoma. DESIGN: Retrospective medical record review. SETTING: Referral university hospital. PATIENTS: Patients with cholesteatoma treated with primary or revision canal wall down mastoidectomy with tympanoplasty in a single stage. Patients with implanted hydroxyapatite prostheses composed a matched control group. MAIN OUTCOME MEASURES: Medical records were reviewed for type of ossicular condition, type of prosthesis, and hearing threshold at 1-year follow-up. RESULTS: Results are reported as the 5-frequency average air conduction gain, bone conduction gain, and air-bone gap. The malleus handle was present in 24 patients, and the stapes superstructure in 22 patients. Mean (SD) air conduction gain was 7.6 (14.7) dB (P = .001); it was 8.7 (12.0) dB in the group with titanium prostheses and 6.3 (17.4) dB in the group with hydroxyapatite prostheses (P = .54). Bone conduction gain was 1.1 (4.9) dB (P = .19). No patients experienced postoperative impairment of bone threshold greater than 5 dB. Postoperative air-bone gap was 26.5 (15.3) dB; it was 23.8 (15.7) dB in the titanium group and 29.8 (14.6) dB in the hydroxyapatite group (P = .18). Air-bone gap closure was 40%; it was 46.2% in the titanium group and 33.3% in the hydroxyapatite group (P = .35). CONCLUSION: Titanium is a satisfactory material for use in ossicular reconstruction and is comparable to hydroxyapatite, although at present, no definitive conclusion about the superiority of titanium can be drawn.

Sensorineural hearing loss in primary antibody deficiency disorders.

To evaluate the hearing function in patients affected by primary antibody deficiency disorders. Forty-seven patients, 25 of whom were affected by X-linked agammaglobulinemia and 22 of whom were affected by common variable immunodeficiency were evaluated with audiologic tests that included pure tone audiometry, acoustic immittance assessment and auditory brainstem-evoked response. Eighteen patients (38%), 7 with X-linked agammaglobulinemia and 11 with common variable immunodeficiency, showed sensorineural hearing loss, bilateral in 12 and unilateral in 6. Our data underline the high frequency of hearing loss in patients with antibody deficiency and suggest that a systematic audiologic evaluation should be part of the clinical care of these patients.

Benign vascular lesions involving the external ear canal.

Benign vascular lesions include various forms whose classification has created some controversies in the literature. The observation of two rare cases of vascular bulge of the external ear canal prompted us to analyze the essential features of these lesions. One case was observed during an episode of otitis media, the other one was an incidental finding, and both are still on follow-up without treatment. The lesions are unmodified after 4 and 10 years, respectively. Vascular malformations can be differentiated from vascular tumors since they are present at birth, are generally stable, and do not involute. An attending strategy is justified unless symptoms occur.

Diode laser myringotomy for chronic otitis media with effusion in adults.

OBJECTIVE: To analyze the closure time of diode laser-assisted myringotomies, the incidence of complications, and the hearing results in comparison with the "cold" procedure in adults with otitis media with effusion (OME). STUDY DESIGN: Prospective case-control study. SETTING: Tertiary referral center, university hospital. PATIENTS: Twenty-eight adult patients (39 ears), 13 men and 15 women, age 13 to 76 years (mean, 51.9). Inclusion criteria included 3 months (or more) history of OME resistant to medical therapy. Twenty-two control patients (34 ears) underwent cold myringotomies with knife and ventilation tubes (VT). INTERVENTION: Diode laser myringotomy performed in an office setting under local anesthesia with topical EMLA ointment. MAIN OUTCOME MEASURES: Timing of closure of the myringotomy, hearing results, incidence of complications, recurrence of OME. RESULTS: No intra- or postoperative pain nor complications were observed. Otomicroscopic daily monitoring documented the healing patterns of the tympanostomies, which remained patent for 7 to 25 days (average, 15.6 +/- 4.8 days). Immediate improvement of hearing was achieved in every patient. Recurrence of OME was observed in 36 ears (92.3%) within 1 month from healing. In the control group with VTs, healing of the eardrum was observed between 126 and 301 days (average, 183.2 +/- 44.8 days), and recurrence of OME was observed in 8 ears (23.5%) (p < 0.001). One month after healing, the air-bone gap was retained within 10 dB in 10.3% (4/39) of the diode laser group and in 50% (17/34) of the standard procedure group (p=0.0001). CONCLUSIONS: Diode laser myringotomy is a straightforward, painless procedure simplified by the thin fiberoptic cables available. Functional benefit is comparable to conventional tympanostomies plus VTs, but the duration of patency is too short to achieve long-term clearance of the effusion in "glue" ears of adult patients. Selected indications could be acute or recurrent otitis media or the prevention of barotraumas in tubal dysfunction.

Acute otitis media and facial nerve paralysis in adults.

OBJECTIVE: The pathophysiology and treatment of facial nerve paralysis associated with acute otitis media are still under debate. The objective of this study was to review treatment strategies and extent of recovery in adult patients with the aim of defining a standard treatment protocol for this rare pathologic condition. STUDY DESIGN: Retrospective chart review. SETTING: University hospital, tertiary referral center. PATIENTS: Between 1993 and 2000, 11 patients were admitted for facial nerve paralysis secondary to acute otitis media. There were six women and five men without a history of chronic middle ear disease, who ranged in age from 21 to 71 years. Facial palsy was graded with the House-Brackmann scale: four patients had Grade III palsy, six had Grade IV palsy, and one patient had Grade V palsy. Bacteriologic examination of middle ear fluid was performed in four patients Streptococcus pneumoniae was observed in one patient, and the remaining three cultures were negative. INTERVENTIONS: All patients were treated with parenteral ampicillin-sulbactam or a third-generation cephalosporin in conjunction with oral or intravenous corticosteroids, except in a single patient with diabetes mellitus who received antibiotics alone. Myringotomy alone or with ventilation tube application was performed in eight patients. A simple mastoidectomy without facial nerve decompression was used in a patient with sudden impairment to Grade VI paralysis and worsening otitis after an initial improvement. RESULTS: Normal facial function returned in all patients, independently of the grade of the paralysis, the treatment strategy, or the outcome of the middle ear disease. The time of recovery varied from 2 weeks to 3 months, except for one patient who underwent mastoidectomy and in whom normal function returned in 10 months. CONCLUSIONS: The treatment of facial nerve paralysis secondary to otitis media should be as conservative as possible, using antibiotics and corticosteroids. Myringotomy and a ventilation tube should be added when spontaneous perforation of the tympanic membrane is not present. Mastoidectomy should be performed only when it is necessary to treat otitis media. Facial nerve decompression should not be necessary.

The distribution of lymph node metastases in supraglottic squamous cell carcinoma: therapeutic implications.

BACKGROUND: The treatment of the neck in cancer of the upper aerodigestive tract is still a matter of controversy, even though nowadays there is a trend in the literature toward elective surgery in the N0 neck when the probability of occult lymph node metastasis is greater than 20%. In the elective setup, every effort is made for preservation of uninvolved nonlymphatic structures in positive neck. The aim of this study is to analyze in a large cohort of patients treated for supraglottic carcinoma the prevalence of lymph node metastases and their distribution through various neck levels to redefine our policy of neck treatment. METHODS: A retrospective review of 402 consecutive patients, who underwent surgery in the Department of Otolaryngology of the University of Brescia (Italy) for supraglottic squamous cell carcinoma in a 14-year period, has been performed. The prevalence of neck metastases was assessed by pT category and site (marginal vs vestibular) of the primary tumor. The side(s) of neck disease was related to the side of the primary tumor, whether lateral or central. The distribution of involved lymph nodes through the neck levels was determined. RESULTS: Overall lymph node metastases accounted for 40%; their prevalence rate increased with pT category from 10% to 57% (p =.0001). Occult metastases were found in 26% of N0 patients from 0% in pT1 to 40% in pT4 (p =.02). There was no difference in metastases rate between marginal vs vestibular, and central vs lateral neoplasms, whereas bilateral metastases were more frequent in central tumors (20% vs 5%; p <.0001). Level IV was involved only in association with level II and/or level III. Levels I and V were rarely involved when overt metastases were present and never by occult metastases. CONCLUSIONS: Elective lateral neck dissection (levels II-IV) is recommended in T2-T4 N0 supraglottic cancers; clearance of both sides of the neck is indicated whenever the lesion is not strictly lateral. We still perform a selective neck dissection including levels II-V whenever there is clinical, radiologic, or intraoperative evidence of metastases at any level.